The patient is a 67-year-old African American male with a past medical history significant for hypertension, coronary artery disease requiring stent placement, mitral insufficiency, hyperlipidemia, tobacco use disorder, and schizophrenia. The patient was initially brought in for hospitalization by family secondary to altered mental status and disorientation. Upon presentation, an extensive medical workup was undertaken, including laboratory analysis (basic metabolic panel, complete blood count, urinalysis, urine drug screen, ammonia concentration, vitamin B12 concentration, folate concentration, thyroid stimulating hormone, liver function tests, and venereal disease research laboratory), computed tomography of the head, and electroencephalography. All workup for a general medical cause of that patient's acute presentation was negative, and a psychiatric consult was subsequently requested given the patient's chronic history of schizophrenia. Upon consultation and assessment by psychiatric services, the patient endorsed nonadherence to clozapine therapy, with which he had been effectively treated for numerous years. As a result of the negative medical workup and medication nonadherence, the patient was subsequently transferred to the acute care psychiatric unit for reinitiation of clozapine. Concurrent scheduled medications at this time included aspirin 81 mg by mouth daily, atorvastatin 80 mg by mouth at bedtime, buspirone 10 mg by mouth every morning and 20 mg by mouth every night, clopidogrel 75 mg by mouth daily, metoprolol succinate 25 mg by mouth daily, and thiamine 100 mg by mouth daily.
Clozapine was titrated up to a dose of 375 mg by mouth nightly over a 14-day period with the ultimate goal of achieving the previously prescribed dose of 450 mg by mouth nightly; however, it was at this time the patient developed edema, erythema, and pruritus to his face and arms along with lip swelling characteristic of and concerning for angioedema. Clozapine was discontinued upon development of this reaction, and the patient was symptomatically treated with hydroxyzine and ranitidine for suspected angioedema. A single 40-mg dose of prednisone was also given to the patient; however, due to resolution of symptoms, further glucocorticoid treatment was not found to be warranted. A complete blood count was drawn and noted to be significant for increased eosinophils to 1.1 10×3/uL, consistent with an inflammatory response. A probability score for this reaction was calculated utilizing the methods described by Naranjo et al, which identified the described as a probable drug reaction to clozapine.
All scheduled antipsychotic therapy was held for 8 days, during which all allergic sequelae improved; all other nonpsychotropic medications were continued without change. Given resolution of the allergic reaction and continued symptoms of uncontrolled psychosis, the decision was made to initiate olanzapine 10 mg by mouth nightly with plans to cautiously increase the dose as tolerated. Two days following the initiation of olanzapine, the patient had a re-emergence of facial edema, and olanzapine was immediately discontinued. Treatment with an antihistamine was again initiated, and the patient was transitioned to haloperidol 5 mg by mouth twice daily for continued treatment of schizophrenia symptoms. A repeat complete blood count indicated a continued increase in eosinophils to 2.9 10×3/uL. Over the next week, haloperidol was titrated to a dose of 5 mg by mouth every morning and 10 mg by mouth nightly, which was well tolerated and appropriately controlled the patient's psychosis, allowing for discharge.
Fifteen days after discharge, the patient presented to acute care services again, this time with complaints of disorganization, confusion, and substantial extrapyramidal symptoms. Haloperidol was promptly discontinued and replaced with quetiapine 100 mg by mouth nightly in an attempt to minimize extrapyramidal symptoms. Yet again, following 2 days of quetiapine therapy, the patient re-experienced facial edema. Following this reaction, the decision was made by the treatment team to no longer trial atypical antipsychotics, and loxapine was cautiously initiated. Over the next 2 weeks, loxapine therapy was maintained and slowly titrated to a final dose of 25 mg by mouth each morning and 50 mg by mouth at bedtime. The patient responded well to loxapine, so much so that, at the time of discharge, he was logical, organized, and had a sustained reduction in auditory hallucinations. Additionally, the patient continued to tolerate loxapine therapy with no further emergence of allergic sequelae. A review of the patient's medical record identified continued treatment with loxapine for an additional 2 years following the events reported above, at which time the patient was transitioned to paliperidone due to a sustained unavailability of loxapine at local pharmacies.


A 65-year-old female referred to a private dental clinic with the history of a painless, non-healing ulcer on the buccal gingiva of anterior area of the mandible extending from right canine to first premolar for about 7 months. She had negative history of smoking or alcohol consumption. No palpable lymph node was found in the neck examination. In addition, computed tomography (CT) with contrast revealed no lymph node, neck, chest, and abdominal involvement by the tumor. No lesion was noted in CBCT study of the head and neck. Incisional biopsy of the lesion showed a well- differentiated SCC. The tumor clinically was assessed as stage II. After preoperative assessment, the patient was subjected to radical excision with safe margins and right side selective neck dissection at the level II and III for prophylactic reasons. The histopathologic examination of frozen sections confirmed the presence of free margins. In the gross examination, there was a tumoral lesion with white color and cauliflower surface in the area of right mandibular canine and the first premolar tooth. The tumor size was 2.5cm in its greater diameter. Post-operative histopathologic examination showed a low-grade SCC composed of nests of neoplastic squamous cells with mild atypia and keratin pearl formation ().
Surgical margins were tumor-free. Two out of the three resected lymph nodes demonstrated papillary and follicular structures lined by cuboidal cells with intranuclear groove
and pseudo- intranuclear inclusion suggestive of metastatic PTC. The lymph nodes were not involved by SCC (). Laboratory
tests and ultrasound-guided fine needle aspiration confirmed the diagnosis of PTC. Subsequently, total thyroidectomy was performed and patient underwent postoperative radionuclide (I131) therapy.
In addition, the patient received post-operative radiotherapy for oral tumor. After 18 months of follow-up, the patient was free of tumors.


A 53-year-old female patient with a history of hypertension and coronary artery bypass grafting (CABG) was admitted to our outpatient clinic. She presented with typical chest pain with increased intensity recently. Her CABG was performed 6 months ago, and her anginal symptoms were continuing since that time. A single left internal mammary artery (LIMA) graft to LAD was harvested because of totally occluded LAD in CABG according to the surgical report. Physical examination and electrocardiogram results and cardiac enzymes were normal. Echocardiography showed hypokinesia of basal inferior septum with normal left ventricle functions and valves. The patient underwent coronary angiography with the diagnosis of unstable angina pectoris. Angiography revealed that the LAD was thin and terminating proximally after giving off a large diagonal branch (, , , ). A LIMA graft was harvested to the diagonal artery instead of the LAD, and the LIMA graft was being maintained by retrograde flow from the diagonal artery (, , ). When RCA was visualized, a vessel was observed from the right coronary sinus traveling toward AIVS (). When this vessel was selectively cannulated, we noticed that the vessel was misdiagnosed as a retrograde collateral flow toward LAD which was supposed to be occluded and the patient had a type IV dual LAD anomaly (, , , ). We performed 3D imaging with CT angiography to confirm our diagnosis (, , ). We presume that early termination of short LAD in AIVS was considered as total occlusion by the surgical team, and probably due to the deep engagement of the catheter to RCA, the vessel from the right coronary sinus was overlooked (, , , ). We planned medical treatment for the patient and assumed that the symptoms were related to competing arteries.


A 25-year-old male patient reported with a history of discomfort and pain in the left lower tooth region for the past 3 months along with difficulty in closing his mouth. On examination, there was a large yellowish hard mass extending from the distal of first molar to the retromolar region with an approximate size of 3 cm × 2 cm in diameter, which was associated with inflammation and purulent discharge [].
Orthopantomograph [] revealed a radiopaque mass involving the embedded tooth (tooth 38) along with incomplete eruption of tooth 28. After routine blood investigations, the calcified mass was surgically removed [] under local anesthesia and sent for histochemical and biochemical study. Histochemical and biochemical examination of the specimen and salivary examination revealed the presence of calcium (42%), phosphates (18%), and calcium phosphate (70%), which was suggestive of dental calculus. The patient was recalled and educated for proper maintenance of oral hygiene and advised to report for regular follow-up [].


C.P. is a 61-year-old right-handed man who was seen in August 2015 after a 3-year history of increasing difficulties perceiving objects and judging distances (e.g., while driving and cycling), dressing, reading, calculating, and word finding. In addition, his wife described C.P. had been tripping over his words for over a year but that his comprehension remained intact. Neuropsychological assessment at the time revealed a Mini-Mental State Examination (MMSE) of 24/30, some impairments in recognition memory for visual material (RMT) (words: 25th percentile; faces: <5th percentile), and visuospatial and visuoperceptual function (i.e., Visual and Object Space Perception Battery fragmented letters: <5th percentile; position discrimination: <5th percentile).
No neurological or other remarkable findings arose from the medical examination. An MRI scan showed marked biparietal atrophy (Fig. ). In November 2015, a clinical diagnosis of PCA was made. Subsequent review confirmed C.P. met research consensus criteria [] for PCA at Classification Level 1 (core clinical/cognitive features), PCA-pure at Level 2 (by not additionally meeting criteria for LPA or any other neurodegenerative syndrome), and PCA-AD at Level 3 (CSF evidence consistent with AD pathology: total tau = 296; A beta1–42 = 311; ratio = 0.95, p-tau = 33).
In 2017, at the time of the current assessment, neuropsychological assessment revealed impaired performance on testing of generalized function (MMSE = 13/30); basic, visuospatial, and perceptual processing deficits (figure-ground discrimination = 12/20; dot counting = 5/10; fragmented letters = 0/20; all <5th percentile); mild visual crowding (letter identification with condensed flankers = 19/24, spaced flankers = 24/24); simultagnosia in the context of normal visual acuity, impaired working memory (maximum digit span: forwards = 4; backwards = 0; both <5th percentile), and signs of limb apraxia. By contrast, episodic memory performance on the RMT words was just within normal limits (20/25; ≥5th percentile). However, he retained independence in most activities of daily living.
C.P.'s speech was fluent and grammatically correct, but with some word finding difficulties (consistent with confrontation naming to verbal description = 14/20; <1st percentile) and only very occasional phonological errors (i.e., “bedroom” → “beldroom”; “expect” → “exquet”; 2 phonological errors out of 207 words in spontaneous speech).
Experiments were performed between May and December 2017. During the cognitive examination, C.P.'s performance on the Schonell reading list [] was noted to be atypical for an individual with PCA, yielding an unexpectedly high proportion of phonological (63.1%) compared to what can be purely classified as visual errors (20.1%). An extensive further examination of pre-lexical, lexical, and post-lexical stages of reading and an error analysis were conducted subsequently. Error classification followed Crutch and Warrington's classification [] also used by Yong et al. [] (Table ). Written informed consent was provided in accordance with the guidelines established by the Declaration of Helsinki.
C.P. was assessed using five corpora of real words (total N = 480; see Table ). Overall, he made a significantly higher proportion of phonological or non-word than purely visual reading errors (151/309 and 79/309 errors, respectively; χ2[1] = 35.9, p < 0.001). As expected for an individual with a diagnosis of PCA, the contribution of a visual impairment to his reading performance was indeed undeniable. However, C.P.'s inability to produce a real-word response suggested an additional impairment at a phonological level, which we then evaluated further.
Comparisons between C.P. and a group of 26 patients with PCA was performed using a modified t test by Crawford and Howell [] to identify differences between individual cases relative to a group. Reading accuracy was analysed using logistic regression and relevant lexical variables used as covariates depending on the corpus properties (i.e., Schonell []: length, frequency-CELEX [], Brown and Ure words []: concreteness, length, frequency, Yong perceptual corpus: see []). For this latter, logistic regression was also covaried for perceptual variables (font size, inter-letter spacing, and case) []. χ2 test was used to compare two conditions when all other variables remained constant.


A 60-year-old woman had been followed elsewhere for over 5 years for an incidentally found lipoma at the craniocervical region. She was then referred to us due to progressing motor weakness in her right extremities, associated with paresthesia for 3 months. Her neurological examination revealed mild hemiparesis in the right extremities and disturbance of deep perception. Bladder and bowel function were normal. Computed tomography (CT) scans showed a low-density mass compressing the dorsal aspect of the medulla and the spinal cord (Fig. ). There was no abnormality of the vertebral bony components (Fig. ). The mass showed a high-intensity signal on T1-weighted magnetic resonance imaging (MRI) (Fig. ), which was consistent with lipoma by fat suppression sequence. This lipoma was detected 5 years ago; however, the previous MRIs at the initial hospital were not available. Surgery was planned to decompress the medulla and the C1–C2 spinal cord by opening of the foramen magnum, performing a laminectomy of the C1 arch and debulking the lipoma.
The patient was placed in the prone position under general anesthesia. Motor evoked potentials were monitored during the surgery. A suboccipital midline skin incision was made from the inion to the C3 level. A standard suboccipital craniotomy with opening of the foramen magnum and a C1 laminectomy were performed. The dura mater of the posterior fossa and the spinal cord looked normal. Opening of the dura mater revealed a yellowish mass covered by the arachnoid membrane, indicating a lipoma (Fig. ). The mass was dissected from the cerebellar tonsils and the medulla at the upper pole, then it was partially removed (Fig. ). It was difficult to establish a dissection plane on the lateral and inferior aspects of the medulla and the spinal cord (Fig. ). Internal decompression was performed with an ultrasound aspirator for the main mass towards the caudal pole. The mass was hemorrhagic, which could be controlled by bipolar coagulation. The incised arachnoid membrane was sutured to avoid later adherent tethering of the cord (Fig. ). An expansile duraplasty was not performed as sufficient subdural space was obtained after the decompression. Histological examination confirmed that the mass consisted of mature adipocytes, confirming a benign lipoma. The postoperative course was uneventful. No neurological deterioration was noted. The right hemiparesis and deep perception improved within a month after surgery. The postoperative CT scans showed adequate decompression of the medulla and the spinal cord (Fig. ). A follow-up MRI taken 12 months after the surgery showed no recurrence or regrowth of the lipoma (Fig. ). Her neurological deficit subsided completely at the follow-up examination.


The patient was a previously healthy 23-year-old Caucasian male who complained of the sudden onset of linear rash on his trunk evolving for 2 days. The patient denied other symptoms, such as pruritus, arthralgia, mucosal abnormalities, and fever. When asked about consumption of shiitake, he confirmed that he had eaten those mushrooms 4 days before he sought medical assistance and stated that he thought the shiitakes had been well cooked. The patient denied the intake of other medications or changes of personal hygiene products.
At physical examination, the patient presented with small, grouped papules forming a linear design, spreading on the trunk and extending to the proximal part of the upper limbs (Fig. , , ). No alterations on the face, scalp, or mucosa were observed. Dexamethasone ointment was applied for 5 days in the area of the lesions, and complete remission was observed after 7 days.


A 22-year-old, right-handed young man who had been practicing soccer at a competition level sustained a left shoulder injury in September 2015 when playing soccer. He described two successive traumatisms: First, he has been hit laterally, with his arm at his side, by another player and immediately experienced intense pain. Then, he fell onto this shoulder, which was injured again. He did not report any sensation of dislocation. This shoulder had never before been injured or painful. Initially, he was conservatively managed by his coach (i.e., he wore a sling and took some medication). However, after a few days, he felt that persistent pain and loss of function were unusual and sought a medical consultation. At that time, 10 days after the trauma, pain was located posteriorly, exacerbated by palpation and in an armed position. Anterior and posterior apprehension tests were both negative. Passive and active shoulder motions were normal. Active external rotation, with the arm at side and in abduction, was painful. There was a slight asymmetry of strength in external rotation compared to the contralateral side, but it was not obvious and could be explained by the pain. There were no hornblower or dropping signs. Passive internal rotation was difficult and painful as well. Radiographs were judged to be normal (). Ultrasound suggested a posterior cuff lesion without concluding in a precise diagnosis. MRI confirmed a very unusual isolated tear of the TM at the myotendinous junction without any other associated lesions (). There was no labral, capsular, or bony lesion in favor of a posterior instability. The other tendons of the rotator cuff all remained inserted. An arthro-computed tomography scan was consecutively performed and did not reveal any contrast dye moving throughout the joint, thus confirming the integrity of the articular capsule and comparable to what has been described in the case of IS myotendinous ruptures (). Knowing the natural evolution of this kind of lesion (based on what we know from IS lesions) [, ] and the potential functional consequences for the shoulder in this young patient, we hypothesized that it was preferable to repair this lesion early. We ultimately performed an open surgical repair after a 3-week delay following the initial trauma.


The first patient was a 36-year-old female, with a prior history of surgery and chemotherapy for a left breast carcinoma about 13 months earlier. She presented with a 6-month history of headaches with intermittent vomiting, progressive bilateral visual loss, and right-sided weakness. Her visual acuity was decreased to perceive only hand movement on the right side and no light perception on the left side with evidence of bilateral optic atrophy and right hemiparesis. There was no evidence of local disease recurrence at the primary site at her left breast. Brain MRI revealed a solitary left frontal mass with moderate contrast enhancement and perilesional edema []. Given the high suspicion for a neoplasm, she underwent awake craniotomy with gross total tumor excision. Histology was reported as metastatic carcinoma from a likely breast primary.


The second patient was a 33-year-old male, who presented with a 3-month history of headache. Prior to presentation, he reported an episode of alteration in the level of consciousness, from which he fully recovered. He was otherwise neurologically intact. Brain MRI revealed a mixed intensity right temporopolar mass with a cystic anterior portion showing peripheral enhancement []. He was a Jehovah’s witness and hence refused any form of blood transfusion. He was operated on employing an awake craniotomy protocol, and we achieved a gross total excision. The histology was reported simply as a low-grade glioma without molecular profiling, as a facility for this was not available.


A 35-year-old male sustained a traumatic brain injury (Glasgow coma scale [GCS] of 13) following a fall of 5 m. On admission, he was delirious and had bilateral pupils measuring 3 mm without attendant cranial nerves palsies. The skull X-ray showed a non- displaced right frontotemporal fracture and fractures of the right orbital floor, lateral wall/roof, and nasal bones. The brain computed tomography (CT) documented a posttraumatic subarachnoid hemorrhage, right frontal subdural hematoma, and retroclival subdural hematoma extending to the C2 level with partial collapse of the infratentorial cisterns [-]. There was also a left mandibular ramus fracture, plus a distal metaphyseal fracture of the left wrist; none of these warranted operative intervention. Following a hospital stay of 11 days, the patient fully recovered (i.e., GCS of 15 points [M6V5O4]) and exhibited no residual neurological deficits. Further, he remained intact 12 months later.


A 77-year-old female had been diagnosed with UC and had undergone total cystectomy 2 years prior to presentation with dizziness for which she was referred to our department. Magnetic resonance imaging revealed a 35-mm sized lesion in the left cerebellum. This lesion showed low intensity on T1-weighted images, high intensity with perifocal edema on T2-weighted images, and avid heterogeneous enhancement on T1-weighted images after gadolinium administration []. The differential diagnosis included metastasis, glioma, and abscess. Surgery was performed using a standard midline suboccipital approach with the patient in the prone position under general anesthesia. The tumor was totally removed and postoperative course was uneventful. The excised specimen was pathologically diagnosed as mucinous adenocarcinoma consisting mainly of proliferating signet ring cells, which was identical to the features seen in the primary urachal adenocarcinoma comprised a mixture of signet ring cells, mucus-producing cells, and duct-forming cells []. This tumor was pathologically, therefore, diagnosed as cerebellar metastasis of the UC. Recurrence was observed at the site of tumor removal 6 months after surgery, and subsequently, gamma knife radiotherapy was performed to remove the lesion.


A 70-year-old man with hypertension initially presented with imbalance and dizziness and underwent magnetic resonance imaging (MRI), which demonstrated a 3.2 cm × 2.8 cm × 3.7 cm heterogeneously enhancing mass in the suprasellar region which extended to the posterior fossa resulting in mild mass effect on the midbrain and ventral pons and displacement of the optic chiasm []. An endonasal endoscopic approach was used for debulking of the mass. However, unbeknown to the surgical team, the patient had been on daily intranasal oxymetazoline for years, and as a result, excessive nasal cavity bleeding was encountered. Thus, only a biopsy was obtained.
The patient was diagnosed with EVN with ganglioid differentiation. Histopathological analysis showed a moderately hypercellular tumor marked by a proliferation of small cells with scant eosinophilic cytoplasm and generally rounded nuclei. These cells were intermixed with occasional large cells with large nuclei and prominent nucleoli resembling neurons or ganglioid cells []. The tumor demonstrated diffuse positive staining with antibodies to synaptophysin (prediluted; Biogenex; Fremont, CA) and occasionally NeuN (1:800 dilution; Millipore; St. Louis, MO). Tumor cells were negative for glial fibrillary acidic protein (GFAP) (1:600 dilution; DAKO; Carpinteria, CA) and isocitrate dehydrogenase 1 (NADP+) mutation (IDH- 1 mutation). A Ki-67 (prediluted; Ventana; Indianapolis, IN) labeling index was 6–7%. FISH testing showed no evidence of 1p/19q codeletion.
He was followed with serial imaging due to the favorable prognosis of the tumor. 1 year later, the patient’s tumor subsequently progressed and the patient developed short-term memory impairment as well as obstructive hydrocephalus. A right modified orbitozygomatic craniotomy and placement of a left-sided external ventricular drain was performed. Most of the tumor in the interpeduncular cistern was resected with some residual tumor in the third ventricle that did not descend. The patient had third nerve palsy postoperatively; otherwise, he was doing well. Weaning him from the external ventricular drain was difficult due to persistent obstruction at the foramen of Monro. The patient then underwent a left frontal trans-sulcal transtubular transventricular debulking of the intraventricular tumor near the foramen of Monro with septum pellucidotomy. He was successfully weaned from his ventriculostomy.
At the 6 weeks postoperative follow-up, the third nerve palsy resolved. His short-term memory and balance were significantly improved. He underwent radiation therapy with a dose of 5400 cGy in 27 fractions 5 months following surgery to treat his residual tumor. His last follow-up was at 1 year after surgery, and he continued to improve. MRI showed a slight decrease in the size of the residual tumor as compared to his preradiation MRI.


A 50-year-old Chinese man presented with a nodule with itchy rashes on the left lower leg, which gradually grew in size for the last 6 months. With the progression of the disease, patient noticed multiple scattered rashes on the right leg. Prior to visiting our clinic, he had been treated as eczema for last 2 months at a local hospital with topical steroid and antihistamine without any appreciable improvement. On skin examination, we observed a dome-shaped crusted nodule measuring about 1.0 × 1.0 cm on the left lower limb and erythematous plaques with brownish silvery scales around the nodule []. On palpation, nodule was single, round, firm, nontender, and immobile. There was mild infiltration of surrounding skin around the nodule. We also observed multiple scattered erythematous maculopapular rashes on right limb []. Oral and genital mucous membranes were not involved. No lymphadenopathy was detected, especially in the inguinal areas. The patient denied any history of systemic symptoms such as fever, vomiting, diarrhea, cough, shortness of breath, bone pain, or polyuria. There was no history of weight loss or of any malignancy. The skin biopsy demonstrated a few large mononuclear cells infiltrated in superficial dermis. The mononuclear cells showed kidney-shaped nuclei and expressed S-100, CD1a, and langerin []. The diagnosis of LCH was confirmed by histopathological and immunohistochemical changes. Routine examinations including complete blood count, erythrocyte sedimentation rate, liver function, and renal function were within normal range. Electrocardiogram, abdominal ultrasound, and Positron emission tomography–computed tomography (PET-CT) showed no abnormalities. Unfortunately, the patient refused treatment. Further development could not be assessed due to no follow-up in our department.


A healthy male 17-year-old person with an uneventful medical history including the prenatal period, birth and childhood/adolescence, and a negative family anamnesis with regard to dental abnormalities, presented four partially erupted third molars with clinical signs of developmental enamel hypomineralization (opacity, brownish, and creamy discoloration and rough surface structure affecting large parts of the occlusal and smooth tooth surfaces). All other patient's teeth showed a yellowish color, and some showed white spot lesions on smooth surfaces (). The occlusal surfaces showed signs of attrition (). The radiographic examination revealed initial approximal and occlusal caries lesions in upper and lower premolars and molars (). Oral hygiene was in need of improvement. For orthodontic reasons, all third molars were extracted (). Domestic hygiene was intensified, caries lesions within enamel or the outer third of dentin in bitewing X-rays were infiltrated (Icon; DMG, Hamburg, Germany), and more profound lesions were treated by composite restorations. The patient was provided with an occlusal splint to prevent excessive attrition during the night. A regular recall for clinical examination, professional hygiene, and eventual radiographs has been arranged.


A 54-year-old man presented to our hospital with precordial pain and hemoptysis.
Physical examination showed occasional premature ventricular contraction and
swelling of the left lower limb. Seven days earlier, the patient had undergone
implantation of an IVCF (OPTEASE; Cordis Corporation, Fremont, CA, USA) under the
renal veins () in
a foreign hospital, but the IVCF had migrated to the right ventricle with the hook
tilted against the anterior wall (). The patient also had a long history of silicosis
(), which
resulted in a low arterial oxygen pressure (PaO2 of 62.5 mmHg) and high
arterial carbon dioxide pressure (PaCO2 of 39.9 mmHg) preoperatively. An
electrocardiogram showed ventricular tachycardia. Echocardiography demonstrated
metal artifacts in the right ventricle with significant tricuspid regurgitation and
pulmonary hypertension (pulmonary arterial systolic pressure of 38.4 mmHg).
Endovascular retrieval of the IVCF failed because the hook was tilted against the
anterior wall, which made the hook difficult to capture by the snare. Hence, the
filter was removed via midline sternotomy and right ventriculotomy. After opening
the pericardium, approximately 100 mL of bloody pericardial effusion gushed out. We
directly visualized and confirmed the hook’s location, which was against the
anterior wall of the right ventricle (). A hybrid technique was used to
retrieve the filter. First, a purse-string suture was placed in the ventricular wall
about 5 mm around the hook (), and a 3-mm microincision was then made to open the right
ventricle. Second, the hook was captured with a snare () and the filter was removed
through a 10-Fr sheath (). A broken filter strut was seen, but it was completely removed
without residue (). The purse-string suture was then tightened and
knotted. The blood loss volume during the procedure was 100 mL. A postoperative
chest radiograph confirmed that the IVCF was entirely removed (). The patient had an uneventful
recovery, and no complications were observed during the 3-year follow-up.
Informed consent was obtained from the patient for the publication of this case
report. The study protocol was approved by the ethics review board of Beijing
Friendship Hospital.


We present a case of an 81-year-old woman who was first admitted to the Department of
Infectious Diseases at the University Clinical Centre Maribor 18 weeks after the
surgical procedure of laparoscopic sacrocolpopexy. This case report represents the
course of her illness during the next 18 months. The patient was admitted on 8
August 2016 because of general malaise, elevated inflammatory markers, and
progressive lumbosacral pain that started 1 week before admission. She denied having
fever or chills. She also denied any trauma or falls in the past. She had a history
of arterial hypertension and ischaemic heart disease. She had undergone vaginal
hysterectomy 1 year previously and laparoscopic sacrocolpopexy due to vaginal
prolapse 18 weeks before this admission. Both surgeries were uneventful, and the
patient did not have any complaints in the pelvic or genital area thereafter.
On admission, the patient was afebrile and stabile, with lumbosacral pain radiating
into the right leg that was aggravated by walking. She presented with positive
Lassegue’s sign of 50° in her right leg in a physical examination, with normal
muscle strength in both legs. No faecal or urinary incontinence or other
neurological deficits were observed. Slight tenderness on lumbosacral spine
percussion was present, but without paraspinal muscular spasms. No other foci of
infection were detected in the clinical examination. Her total leukocyte count was
17,700/mm3, C-reactive protein (CRP) level was 207 mg/L, erythrocyte
sedimentation rate (ESR) was 99 mm/hour, and procalcitonin level was 7.8 ng/mL. A
urinalysis test was normal and no vaginal discharge was observed. An urgent computed
tomography (CT) scan of the thoracolumbar and lumbosacral spine was performed on
admission, which showed age-related degenerative changes without other significant
pathology.
Spondylodiscitis was suspected because of elevated inflammatory markers and lumbar
pain. Antimicrobial therapy with amoxicillin/clavulanic acid 1.2 g four times a day
and intravenous analgesic therapy were started immediately after withdrawal of blood
for culture. Magnetic resonance imaging (MRI) of the lumbosacral spine on the 4th
day of hospitalization (12 August 2016) showed soft tissue swelling and oedema
around the titanium bone anchors in the first sacral vertebra and minimal fluid
collection in the intervertebral disc at the L5–S1 levels. Additionally, axial
images at the L4 level showed fluid collection in the spinal canal with rim
enhancement, which indicated spondylodiscitis with paraspinal phlegmon and a small
epidural abscess. However, MRI showed no major compression on neural elements. On
the same day (4th day of hospitalization), B. fragilis grew in
blood cultures and we switched to targeted antimicrobial treatment with
metronidazole 400 mg three times a day (t.i.d.) and added ceftriaxone 2 g daily
intravenously. A neurosurgeon was consulted immediately and a decision against
urgent surgical intervention was made at that time because of the absence of
neurological deficits, the patient’s comorbidities, and known aetiology (positive
blood cultures). According to MRI, an association of sacral spondylodiscitis and
epidural abscess with remote laparoscopic sacrocolpopexy was suggested, where three
titanium bone anchors for mesh attachment over the sacral promontory were inserted.
A gynaecological examination, and gynaecological and abdominal ultrasound were
performed without pathological findings. The mesh was firm with normal pelvic
support after the gynaecological procedure (Pelvic Organ Prolapse Quantification
stage was −2, −2, −9, 3.5, 3.5, 11, −3, −3, −).
Because of the aggravating lumbar pain and persistently elevated inflammatory
markers, a control MRI was performed on the 8th day of hospitalization (16 August
2016). This MRI showed a similar size of the abscess, but initial infection in the
disc space was even larger and it had spread to the adjacent vertebral body
endplates (.
Deterioration of the lumbar pain, despite the lack of clinical neurological
deficits, forced the decision for neurosurgical intervention with planned collection
of tissue samples for microbiological analysis. Unfortunately, on the same day of
the planned surgery, the patient suffered from dyspnoea and chest pain (22 August
2016). Pulmonary embolism and acute myocardial ischaemia were diagnosed and surgery
was postponed for a few days. During this time, the lumbar pain started to improve.
Because of the high perioperative risk, we continued with conservative treatment,
although tissue samples for microbiological analysis were not obtained. On the 3rd
week of hospitalization, her condition had greatly improved, and inflammatory
markers were decreased (ESR: 56 mm/hour, CRP level: 40 mg/L). We switched to oral
treatment with metronidazole 500 t.i.d. and ciprofloxacin 500 mg twice daily, and
the patient was released home (6 September 2016). A few days later (14 September
2016), she returned to the hospital because of nausea and vomiting, which were
probably caused by the oral form of metronidazole. She still complained of lumbar
pain and required oral analgesic therapy, but was able to walk with an assistance
device. We changed the antimicrobial therapy according to susceptibility testing of
B. fragilis to monotherapy with clindamycin 900 mg t.i.d.
intravenously. The oral form of clindamycin 450 mg t.i.d. was continued a few days
later after resolution of vomiting. A control MRI of the spine was performed during
this time (1 month after the beginning of antimicrobial therapy; 21 September 2016),
where progression of inflammation in the vertebra was observed (. The patient’s clinical condition
further improved and she was discharged home on oral therapy. Because of the
remaining titanium anchors in the sacral vertebra, we decided to prolong the oral
antimicrobial therapy with clindamycin 450 mg t.i.d. to 6 months.
At the patient’s regular follow-up visits in the following months, her condition
slowly improved further. We discontinued the antimicrobial therapy after 6 months (1
March 2017) when her condition was stable and inflammatory markers remained low
(ESR: 40 mm/hour, CRP level: 10 mg/L). The spinal pain gradually subsided and she
was able to perform most activities of daily living. At a follow-up visit 1 year
after discontinuation of the antimicrobial therapy (March 2018), she was well,
without any signs of recurrence of the infection or other complications, and she was
able to walk without assistance. A control MRI of the lumbar spine was performed
during this visit and it showed resolution of the epidural abscess without any
active site of infection in the vertebra (.
The patient signed written informed consent agreeing to publication of this
manuscript and any accompanying images and medical data.


The patient was a previously healthy 73-year-old female who underwent curative resection for GBC (pT2N0M0 according to the eighth International union against cancer TNM classification). We performed next-generation sequencing (NGS)-based genomic profiling of the resected specimen using the NGS gene panel, Oncomine® Comprehensive Assay version 3 (OCA v.3, Thermo Fisher Scientific), which revealed ERBB2 Ser310Phe (c.929C>T; VAF, 18%) and TP53 Ser241Tyr (c.722C>A; VAF, 19%) mutations. One year later, a hepatic lesion was observed on follow-up imaging and she underwent surgical total biopsy for a pathological diagnosis.
A patient had no symptoms and was in good health at the time of total biopsy.
The patient had no previous medical history.
The patient’s physical examination was not remarkable and laboratory testing was within normal limits, including tumor markers, such as CA19-9 and CEA.
Contrasted computed tomography (CT) showed an ill-defined low attenuation lesion in the posterior lobe of the liver (Figure ).
The hepatic lesion was histologically diagnosed as well-differentiated adenocarcinoma and the histological findings of the hepatic lesion were similar to those of GBC (Figure ). Therefore, the lesion was considered a metastasis. Moreover, we performed genomic profiling from the liver tumor using the NGS panel, Oncomine® Target Test system (OTT, Thermo Fisher Scientific). This revealed ERBB2 Ser310Phe (c.929C>T; VAF, 26%), which was identical to the mutation detected in the sequencing result of the primary site; thus, the liver tumor was the most consistent with a metastasis of GBC rather than localized ICC. To evaluate HER2 overexpression in tumor cells, we performed immunohistochemistry of HER2, which was negative (HER2 score 0). Since TP53 was not included in the gene list of OTTs, TP53 mutation status at the metastatic site was not assessed.


A 71-year-old male presented with a deep venous thrombosis and pulmonary embolus. On further workup, contrast-enhanced computed tomography (CT) of the abdomen and pelvis demonstrated a 73 mm × 70 mm × 41 mm hypoattenuating retroperitoneal mass in the right paracaval region medial to the right kidney with 180° abutment of the inferior vena cava ().
Gadolinium-enhanced magnetic resonance imaging (MRI) demonstrated a multiloculated T2-hyperintense extravertebral chordoma at the level of L2 in the right retroperitoneum that extended medially along the medial aspect of the right psoas muscle (). Of note, there was no evidence of extension into the neural foramina and spinal canal or the presence of an epidural component. A CT-guided biopsy demonstrated a primary retroperitoneal chordoma. Immunohistochemistry was positive for pancytokeratin (A1/A3) and focally positive for CK7 and S-100 and negative for PAX8, MART-1, HMB45, CK20, CDX-2, and CD-10, supporting the diagnosis.
He underwent an en-bloc gross total resection. An 85-mm retroperitoneal chordoma was found to be involving the soft tissues adjacent to but not invading into the inferior vena cava, right renal vein, right renal artery, and right psoas muscle with tumor extending to the inked edge of the specimen. The mass was free of any nerve roots. There was no evidence of gross tumor remaining following the resection.
Final pathology demonstrated a microscopic-positive margin. He was referred for post-operative radiation therapy. We recommended proton beam therapy (PBT) to the surgical bed in the setting of a microscopic positive margin. We prescribed a total dose of 72 Gy (relative biological effectiveness (RBE))/(Gy) to be delivered with 2 Gy (RBE)/Gy daily fractions over 36 consecutive treatment days excluding weekends to the surgical bed. Using an Orfit board (Orfit Industries, Wijnegem, Belgium), custom Vac-LokTM, and a thermoplastic mask (Orfit Industries), the patient was immobilized on the treatment table in the supine position with his arms extended over his head. A treatment planning four-dimensional computed tomography (4DCT) scan demonstrated that respiratory motion of the treatment target was less than 2 mm isotropically. A clinical target volume (CTV) included potential sites of microscopic disease. A scanning target volume (STV) was defined as the CTV with a 5-mm isotropic expansion to guide PBT spot placement. Lexicographic-based optimization was performed based on CTV coverage constraints. Theoretically, PBT plans require utilization of an additional optimization target volume (OTV) to account for daily setup uncertainties. However, the proximity of critical structures can limit the amount of margin one can expand on the CTV. Under such space and dose-limiting situations, an alternate method composes of foregoing generation of an OTV volume and creating an STV margin from the CTV for the placement of Bragg peaks around the target. If the lateral margins of the STV account for lateral penumbra of proton spots placed outside the CTV, and the distal margins account for CT based range uncertainties, one can achieve nearly homogeneous dose coverage with adequate robustness in such plans as one would expect otherwise with plans involving an OTV. Treatment in this case was prepared based on the STV margin and CTV-based optimization.
Normal tissues including the spinal cord, kidneys, small bowel, and liver were contoured. A 5-mm isotropic expansion on the spinal cord was used to create a planning organ-at-risk volume for optimization purposes. Given that the CTV was in close proximity to healthy tissue including the duodenum, small bowel, right kidney, spinal cord, and liver, we created comparison plans of spot-scanning PBT versus intensity-modulated radiation therapy (IMRT) (). To adequately treat the target volume to 72 Gy and maximally spare the kidneys and bowel, we elected to treat this patient with spot-scanning PBT. The patient was treated with two equally weighted posterior oblique fields. Daily kV image guidance and a single verification CT scan halfway through treatment was performed.
Both treatment modalities allowed greater than 99% of the CTV with 100% of the prescription dose. However, PBT maximally spared the organs-at-risk, especially the kidneys and bowel. The duodenum would have received a maximum dose of 55 Gy with IMRT versus 0 Gy with PBT. The ipsilateral and contralateral kidney would have received a mean dose of 8 and 4 Gy with IMRT, respectively. With IMRT, approximately 12% of the total kidney volume would have received 12 Gy and approximately 10% of the ipsilateral kidney would have received 18 Gy. Both kidneys received essentially no radiation with PBT ( and ).
The patient completed PBT without any treatment-related toxicity. He has no treatment-related toxicity or evidence of recurrent disease 9 months following treatment.


A 46-year-old man presented with 1-month blurry vision in the right eye (OD) and no other systemic symptoms. He suffered from temporal lobe epilepsy due to a benign astrocytoma, operated in 1993 and controlled with antiepileptic drugs. Visual acuity (VA) was counting fingers in his OD and 20/20 in his left eye (OS). Anterior examination was unremarkable. Funduscopic examination revealed a yellowish placoid lesion occupying the whole posterior pole in his OD, and there was an apparently normal funduscopic examination in his OS (Fig. ). Ultra-wide-field fundus autofluorescence demonstrated hyperautofluorescence corresponding to the placoid lesion in OD and an area of hyperautofluorescence superotemporal to the optic disk in OS (Fig. ). Spectral-domain optical coherence tomography (SD-OCT; Cirrus HD OCT®, Carl Zeiss, USA) B-scan showed a disruption of the ellipsoid zone and external limiting membrane, hyperreflective granular RPE changes, and areas of punctate hyperreflectivity in the underlying choroid in the OD (Fig. ). En face OCT revealed multiple hyperreflective clumps in the outer retina in the OD (Fig. ), and OCT angiography (OCT-A; Angioplex®) showed areas of flow void at the level of the choriocapillaris in both eyes (Fig. ). Blood analysis revealed reactivity to the venereal disease research laboratory (VDRL) (>1/1,024) and positivity antibodies against membrane protein A of T. pallidum by ELISA (IgG and IgM), indicating acute syphilitic infection. Lumbar puncture was negative for VDRL and for IgM anti-T. pallidum but positive for IgG, and pleocytosis was seen in the cerebrospinal fluid.
Brain magnetic resonance imaging was unremarkable. Serum anti-HIV antibodies were negative. The patient underwent the standard treatment for neurosyphilis, i.e., intravenous penicillin G at a dosage of 24 million units per day for 14 days and i.v. pulse of methylprednisolone. After he completed the antibiotic treatment, his VA improved to 20/20 in both eyes, and although the outer retinal lesions were completely restored, choriocapillaris reperfusion remained incomplete at 3 months of follow-up (Fig. ).


A 62-year-old male developed a painless mass under the right jaw in 2017, in which the mass enlarged gradually. He was admitted to a local hospital on July 2017. Positron emission tomography-computed tomography (PET-CT) scan indicated a dubious diagnosis of lymphoma (). He then underwent resection of the right submandibular mass. The pathological report of the mass revealed that small atypical lymphocytes diffusely or follicle-like distributed and follicle-like structure accounted for 75% of the whole tissue. The absolute number of centroblasts was 0–5 for each high-power field (). Immunohistochemistry showed that the tumor cells were positively associated with CD20, CD79α, CD43, CD10, and Bcl-2, and negatively associated with CD3, CD5, and Cycline-D1. In addition, the follicular dendritic cell (FDC) network stained with CD23 showed that 5-10% of tumor cells were positively associated with Ki-67 (). The pathological diagnosis was follicular lymphoma, grade I. The bone marrow aspiration was performed and flow cytometry of the bone marrow revealed B cells with abnormal immunophenotype, which indicated follicular lymphoma invasion to the bone marrow (). Genetic testing revealed a normal karyotype: 46, XY (). The patient was eventually diagnosed with follicular lymphoma (grade 1, stage IV). Given the repeated unexplained fever and night sweating, he received 4 cycles of modified R-CHOP (rituximab (600 mg d0), cyclophosphamide (1.0 g d1), pegylated liposomal doxorubicin (20 mg d1), vincristine (4 mg d1), and prednisone (90 mg d1–d5)).
After 4 cycles of modified R-CHOP, his symptoms, e.g. fever and night sweating, disappeared. An interim PET-CT scan suggested response of stable disease. His bone marrow was re-assessed, and flow cytometry revealed a minimal residual disease of abnormal B cells accounting for 1.11% of all nucleated cells in the bone marrow.
An abnormal elevation of IgM serum concentration was observed both before and after R-CHOP (IgM serum concentration was above 23g/L, ). The serum immunofixation test was performed, that showed the existence of monoclonal IgM and lambda light chain in the blood (). An MYD88 L265P somatic mutation of the bone marrow was indicated by polymerase chain reaction (PCR) (). And, the mutation in the bone marrow was confirmed by next-generation sequencing. The mutant allele frequency was 1%. However, no mutation of MYD88 was detected in the sample of the patient’s lymph nodes using next-generation sequencing. The original tissue of the patient’s lymph node and bone marrow was re-evaluated by a third party pathologist. However, the second pathological diagnosis remained to be follicular lymphoma with bone marrow invasion. An alternative regimen, involving bortezomib (rituximab (600 mg d1), bortezomib (2.3 mg d1, 8, 15), dexamethasone (20 mg d1, 8, 15), which repeated every 28 days, hereafter named R-Vd regimen) was presented. After 2 cycles of R-Vd regimen, serum IgM concentration significantly decreased (). The patient also received continuous R-Vd regimen and waited for another PET-CT scan.


A 58-year-old male patient visited the outpatient clinic complaining of left calf pain after walking more than 50 m and a 4-month history of an unhealed ulcer at the left medial malleolus (). His left leg was swollen, and engorged varicose veins (VVs) as well as hyperpigmentation around the ulcer were noted, but no port wine stains were evident. The circumferences of the right and left mid-thighs were 37.5 cm and 48.5 cm, respectively, while those of the right and left mid-calves were 30.5 cm and 42.5 cm. He previously underwent excision of the vascular tissues in the left popliteal area and multiple embolosclerotherapy sessions to treat AVM of the lower left leg at another hospital. Eight years prior, he underwent surgery to remove a pelvic mass that was pathologically confirmed as an organized hematoma. He denied any relevant family history.
Computed tomography angiography showed a diffuse aneurysmal change from the left common iliac artery (CIA) to the popliteal artery and thrombotic occlusion along the entire femoro-popliteal aneurysm (, ). The maximum diameters of the popliteal artery aneurysm and external iliac artery (EIA) aneurysm were 58 mm and 27 mm, respectively (). Limb hypertrophy, subcutaneous swelling, a varicosed GSV, and calcified lesions of the residual AVM in the lower left leg were also observed. The left leg was perfused by the collateral vessels from the patent deep femoral artery (DFA). On duplex ultrasonography, the diameters of the left GSV at the upper, middle, and lower thigh were 0.72, 0.63, and 0.79 cm, respectively; these were larger than the normal diameters on the right side (0.42, 0.39, and 0.45 cm, respectively). Deep vein thrombosis was not observed. The reflux durations of the left sapheno-femoral junction and posterior branches of the upper thigh were 4,886 ms and 4,866 ms, while the reflux durations of the mid-thigh and mid-calf were 1,034 ms and 1,339 ms, respectively.
Under the presumption that the patient had atypical Parkes Weber syndrome (PWS) lacking port wine stains, we planned an elective open bypass surgery to reconstruct the arterial flow to the calf and remove the refluxed GSV. Using a curvilinear incision on the lower left quadrant of the abdomen, the left EIA was exposed and dissected. Due to severe periarterial adhesions, the CIA aneurysm was not actively exposed. Another longitudinal incision in the left inguinal area was used to expose the common, deep, and superficial femoral arteries. The thrombosed ilio-femoral aneurysm was excised and replaced by a ringed expanded polytetrafluoroethylene interposition graft (Advanta, Maquet, Wayne, NJ, USA) from the EIA to the DFA (). A longitudinal incision along the thigh was used to ligate the ipsilateral autologous GSV at the sapheno-femoral junction and harvest its branches. Since there was no aneurysmal change along the GSV, we decided to use the GSV as a bypass conduit. We performed a bypass from the left femoral graft to the left posterior tibial artery (PTA) using this reversed autologous vein (). At 1 week postoperative, the patient’s symptoms were relieved and the left medial malleolar ulcer had healed remarkably ().


A 68-year-old Caucasian man with a complex medical history including type 2 DM presented to the emergency department with a 3–4-week history of generalized pruritus, new onset diffuse alopecia and diffuse bullae over his trunk, arms and legs. The patient initially had developed bullae and blisters over his legs. Simultaneously, he noticed a significant loss of his scalp and beard hair as well as his eyebrows. This was accompanied by intense pruritus over the abdomen and back for approximately 2 weeks prior to the development of the bullae. The intense itching, development of further bullae, and almost complete alopecia prompted the patient to present to the emergency department.
A review of his history did not reveal any drug allergies and he denied a family history of autoimmune conditions. He had not travelled anywhere recently and did not present with any constitutional symptoms or myalgias. His home medications included linagliptin, allopurinol, amlodipine, atorvastatin, furosemide, hydralazine, levothyroxine, pantoprazole, rivaroxaban, terazosin and insulin.
His vital signs were all within normal limits: he was afebrile at 36.8°C, his heart rate was 59 bpm, blood pressure was 118/73 mmHg, and oxygen saturation was 98% on room air. Physical examination revealed bullae over his back, abdomen and both lower legs and measuring approximately 1–3 cm in diameter (). He also had numerous smaller bullae over both flanks, upper arms and lower legs and measuring approximately 0.5–1 cm in diameter. There was no specific dermatomal distribution and no oral ulcerations or mucositis. The patient had diffuse alopecia on his scalp, eyebrows and beard. There was no erythema, scaling or scarring associated with the hair loss ().
Initial laboratory investigations revealed an elevated creatinine level of 300 mmol/l (baseline in the mid-200s) with normal electrolytes. The patient also had an elevated CRP level of 12.6 mg/l, his white blood cell count was normal at 10.3 g/l, and haemoglobin concentration was 110 g/l.
Skin biopsies performed on admission showed sub-epidermal blisters with multiple eosinophils highly suggestive of BP (). Eosinophils are commonly seen in BP, but the diffuse eosinophilia seen in the biopsy specimen raised the possibility of drug-induced BP. Direct immunofluorescence showed deposition of IgG and C3 along the basement membrane confirming BP (). Interestingly, linagliptin had been introduced a few months before the onset of cutaneous eruptions. At that point, linagliptin was highly suspected as the cause of BP and alopecia and therefore was discontinued. The patient was started on prednisone 40 mg with significant improvement of his skin lesions. He was then followed by the dermatology department and was started on mycophenolic acid (720 mg by mouth twice a day as a steroid-sparing agent) and topical clobetasol cream. At his 6-month follow-up, 80–90% of his skin lesions had resolved and his hair had grown back ().


A 56-year old man with multiple comorbidities (severe obesity, moderate chronic renal failure, chronic obstructive pulmonary disease, smoking, cirrhosis, atrial fibrillation, coronary artery disease) presented to the out-patient clinic complaining intermittent claudication (Rutherford category 3) at right lower limb since 2 weeks. He received mitral valve replacement with coronary artery bypass graft three months before for acute endocarditis (Streptococcus gallolyticus), followed by brain septic embolization.
Physical examination revealed absence of right lower limb pulses with mild hypothermia and abdominal pain at deep palpation of the right lower quadrant without pulsating masses. Duplex ultrasound (DUS) found a perfused abdominal mass with 69mm in diameter in the right lower quadrant with steno-occlusion of right iliac axis. An emergency computer tomography angiography (CTA) revealed a right common iliac artery pseudoaneurysm (62x64 mm) with occlusion of external iliac artery, patency of hypogastric and recanalization of the right common femoral artery.
At admission, the patient had no fever and no leukocytosis (white blood cells=6,76x10^3), with anemia (Hb=9.1 g/dl), mild chronic renal failure (creatinine: 2.1 mg/dl) and high C reactive protein (CRP=97.2 mg/l). No blood culture or positron emission tomography were performed for absence of signs of concurrent systemic infection. No systemic antibiotics was undertaken for the same reason.
The following day, the patient underwent a digital subtraction angiography (DSA) () through left brachial access (6F-sheath introducer, 90cm in length).
The embolization of the hypogastric artery with coils (Balt SPI™ and Cook Mreye™) and of the common iliac artery with Plug (16 mm-Amplatzer Vascular Plug II™) was performed.
The post-procedural DSA revealed the complete exclusion of the hypogastric artery, and the pseudoaneurysm (). The reperfusion of ipsilateral common femoral artery was maintained by collateral circulation.
During the post-operatory period, no hypotension or ischemia of the right lower limb occurred. A CTA confirmed the exclusion of the pseudoaneurysm. Similar findings were highlighted at CTA performed in the third post-procedural day. At discharge (6th post-procedural day), the patient had no fever, no leukocytosis (WBC=5,66x10^3) with stable anemia (Hb=9.1 g/dl) and renal function (creatinine=1.6 mg/dl). The abdominal pain disappeared, the right leg was normothermic and in hemodynamic compensation without rest pain, acute ischemia or neurological deficit.
After one month, a CTA referred the exclusion of the pseudoaneurysm with a gradual reduction in the size. After 10 months, other CTA showed the complete resolution of the pseudoaneurysm () and the revascularization of the right common femoral artery by collateral circulation.


Patient: A 53-year-old man.
Chief complaint: Gynecomastia and abdominal discomfort.
Present illness: A 53-year-old man with a history of hypertension visited the Department of Family Medicine at our hospital with the complaint of a 6-month history of bilateral breast enlargement, as well as right-sided flank pain and abdominal discomfort. After abdominal computed tomography (CT) revealed an abdominal mass, he was referred to an endocrinologist for a hormone assessment and further examination.
Past history: He had been diagnosed with hypertension 5 years earlier and was taking medication.
Physical examination: The patient's height and weight were 173 cm and 72 kg, and his body weight had increased by approximately 10 kg over the last 6 months. His blood pressure was 120/80 mmHg, body temperature was 36.5ºC, pulse rate was 68 beats/minute, and respiration rate was 18 breaths/minute. His consciousness was clear, and he had no symptoms such as palpitation, sweating, or headache. His conjunctiva and sclera were normal, and a head and neck examination detected no moon face, flushing, or other specific symptoms. During a chest examination, a 4-cm-sized gynecomastia was observed bilaterally with tenderness. The patient's respiratory sounds were normal, and no heart murmurs were detected. An abdominal examination revealed no purple striae but did reveal tenderness in the right upper quadrant and a palpable hard, fixed mass with an approximate size of 15 cm. The patient's bowel sounds were normal, and he exhibited no edema or tenderness in his limbs. There were no skin rashes or specific findings of the joints. A lower limb test revealed normal sensory and motor functions. The patient denied erectile dysfunction and reported a normal shaving frequency of once every 2 days.
Laboratory findings: A complete blood cell count during blood analysis revealed a leukocyte count of 9,330/mm³ (neutrophils, 75.3%), hemoglobin level of 11.4 g/dL, and platelet count of 329,000/mm³. A comprehensive metabolic panel indicated the following levels: aspartate aminotransferase and alanine aminotransferase, 31 and 23 IU/L, respectively; alkaline phosphatase, 80 IU/L; total protein, 6.2 g/dL; albumin, 3.3 g/dL; calcium, 8.8 mg/dL; blood urea nitrogen, 9.0 mg/dL; and creatinine, 0.7 mg/dL. A hormonal assessment revealed increases in the levels of dehydroepiandrosterone, estradiol, and 24-hour urine cortisol but decreases in the levels of follicle-stimulating hormone, testosterone, and adrenocorticotropic hormone. The patient's serum cortisol and luteinizing hormone levels were normal (). His morning plasma cortisol level was 16.3 μg/dL (range, <5 μg/dL), and this was not suppressed during an overnight dexamethasone suppression test. The patient rejected an additional planned low-dose dexamethasone suppression test. A 24-hour urine test yielded normal vanillylmandelic acid, metanephrine, and epinephrine levels.
Radiological findings: Simple chest radiography yielded normal findings. Abdominal CT showed a heterogeneously well-defined single mass with dimensions of 18×11×18 cm between the liver and right kidney (). On renal CT, the mass was not observed to have infiltrated the liver, kidney, and surrounding blood vessels, and lymph node metastasis and ascites were not observed. A chest CT scan and bone scan showed no metastasis.
Treatment and progress: The patient underwent open surgery under general anesthesia to remove the right adrenal tumor. The tumor was found to have adhered to the liver, kidney, and inferior vena cava, and a partial liver resection was performed. Inferior vena cava and kidney involvement were not observed. There were no postoperative complications. The final tumor classification was pT2N0M0, stage 2, and the patient received additional adjuvant radiation therapy. Postoperatively, he was given replacement therapy with hydrocortisone for suspected adrenal insufficiency. Three months after surgery, his estradiol level was 70.32 pg/mL and the gynecomastia had almost disappeared. The patient remains in good health without any local recurrence or metastasis at 21 months after diagnosis and treatment, and the gynecomastia has almost resolved.
Pathological findings: A gross pathology examination of the surgically resected tumor revealed a size of 21×15.3×12 cm and a smooth and partially fibrous outer surface. An analysis of the tumor cross-section revealed encapsulated yellowish-brown nodules and widely distributed necrotic and hemorrhagic lesions ().
A microscopic examination revealed tumor necrosis and readily apparent mitosis. The histopathologic examination revealed necrosis, diffuse architecture, a high nuclear grade, high mitotic rate, atypical mitotic figures, and a clear cell frequency of <25% (). The immunohistochemistry results included positive staining for inhibin α, MART-1, and calretinin and a Ki67 proliferation index of 20% (). No tumor invasion was observed in the resected mass.


A 16-year-old neutered female domestic shorthair cat was presented with a history of
progressively worsening lameness of 8 months’ duration. The lameness gradually
worsened, despite the use of non-steroidal anti-inflammatory drugs (NSAIDs).
At presentation, the cat was reluctant to walk but showed an overall stiff gait. On
video excerpts made by the owner, moderate lameness of the right thoracic limb was
observed. On initial physical examination, the cat was bright and alert. The cat was
obese, with a body weight of 5.6 kg and a body condition score of 6/9. Body
temperature (38.1°C) was normal and further general clinical examination revealed no
abnormalities. No deficits could be observed on neurological examination. Abnormal
findings on orthopaedic evaluation included a decrease in muscle mass of the infra-
and supraspinatus muscles of the right thoracic limb, a decreased range of motion
(ROM) of the right and left carpus and a decreased ROM, with pain on manipulation,
of the right shoulder. Medial, lateral, cranial and caudal stability of both
shoulders was assessed and deemed normal.
In accordance with the owner’s wishes, conservative therapy was first elected. The
patient was given cage rest, nutritional supplements (Hill’s Prescription Diet j/d
Feline), caloric restriction and continued treatment with meloxicam 0.05 mg/kg lean
body weight (Metacam; Boehringer Ingelheim) for 1 month.
One month later at the follow-up examination, no improvement of the clinical signs
was noted.
Despite caloric restrictions, the cat’s weight had increased to 5.8 kg. This was
attributed to poor owner compliance. Neurological and orthopaedic examinations were
as before and the patient was scheduled for radiography under general anaesthesia.
Pre-anaesthetic blood screening was unremarkable.
The patient was sedated with an intravenous administration of 1 mg midazolam
(Midazolam Actavis 5 mg/ml; Actavis), 20 mg ketamine (Ketamine 100 inj.; ASTfarma)
and 0.12 mg buprenorphine (Buprecare multidosis 0.3 mg/ml; AST Farma). Radiographic
examination included a mediolateral and craniocaudal view of the right and left
carpal and glenohumeral joints. No abnormalities were noted in the right and left
carpal joints. Radiographic findings indicated a bilateral UCG ( and ). In the right shoulder a large mineralised
body caudal to the humeral head, probably located inside the caudal pouch of the
glenohumeral joint, was observed ().
An arthrogram of the right glenohumeral joint was performed (). The skin over the right shoulder
was aseptically prepared and 2 ml of iodine contrast agent (Iohexol, OMNIPAQUE
140 mg I/ml; GE Healthcare) was injected intra-articularly. The mineralised body was
completely obscured by the contrast agent, indicating the intra-articular position
of the joint mouse. Also, a small filling defect was seen opposite the UCG,
indicative of impingement on the glenohumeral joint by the UCG. Based on the
clinical and radiographic findings, an arthroscopy of the right glenohumeral joint
was recommended to the owner.
At the time of arthroscopy, the cat was premedicated with the same protocol.
Anaesthesia was induced with 8 mg alfaxolone (Alfaxan 10 mg/ml; Dechra) and
maintained with isoflurane (Isoflo 100% isoflurane/ml; AST Farma) in oxygen. A
standard arthroscopic approach of the right shoulder was performed. Arthroscopic
inspection using a 2.4 mm-wide, 30°-angled arthroscope of the joint revealed
synovitis of the joint capsule, an unstable UCG, severe cartilage wear (modified
Outerbridge scoring 4) of the humeral head opposite the UCG, and a large free-floating mineralised
body, which was attached to the UCG by a string of fibrous tissue (). No other
abnormalities were noted inside the joint.
The UCG was embedded in, and connected to, the glenoid with fibrous tissue. It
consisted of necrotic bone with a characteristic yellow crumbly appearance. It was
unstable on gentle probing.
Cartilage wear of the humeral head was only localised opposite the UCG and showed a
focal severe loss of cartilage with the appearance of subchondral bone. It is
described as a kissing lesion with a modified Outerbridge scoring of 4.
Under arthroscopic visualisation, the UCG was carefully dissected free and both it
and the mineralised body () were removed via the arthroscopic portals.
Skin closure was routine and 2.5 mg of bupivacaine (Marcaine 5 mg/ml; Astra Zeneca)
was injected intra-articularly after closure.
The patient was sent home with activity restrictions and a 3-week course of meloxicam
0.05 mg/kg lean body weight (Metacam; Boehringer Ingelheim).
A follow-up examination was performed 3 weeks after surgery. The two incisions for
the arthroscopic portals had healed without complications. The owner reported that
the patient had markedly improved. NSAIDs were discontinued and a follow-up
appointment was made for 3-weeks later. The owners did not meet this appointment and
were interviewed by telephone 2 months later. By then, the patient was not on any
medications and showed no lameness. Three years later, the patient was euthanased
because of signs related to chronic kidney disease, having shown no recurrence of
the lameness.


A 39-year-old man was referred to our clinic with a pituitary mass. He had right-sided vision loss that occurred suddenly. A 45×28×40 mm pituitary adenoma was detected while evaluating vision loss. Hormonal analyses showed hypogonadotropic hypogonadism without any excess or deficient hormone levels as shown in . A transcranial pituitary adenomectomy was performed. The pathology of the surgical specimen was considered as an adenoma that had no immunostaining for thyroid-stimulating hormone (TSH), adrenocorticotropic hormone (ACTH), growth hormone (GH), and prolactin. After the operation, his vision loss improved.
When he was readmitted to our clinic, he was suffering from loss of libido, infertility, headaches, and relapsing of vision loss on the right side of the eye that started 4 years after his pituitary operation. He had no child. His height and weight were 161 cm and 71 kg, respectively, with a body mass index of 27.4 kg/m2. His physical examination was normal, except for bilateral gynecomastia and small testicles. Hormonal analyses showed hypogonadotropic hypogonadism as shown in . Magnetic resonance imaging of the pituitary gland revealed a 37×32×28 mm residual pituitary mass. Second pituitary adenomectomy was performed, but there was still a residual pituitary mass of 24×25×20 mm after the second operation (). Histopathology of the specimen revealed pituitary adenoma without immunostaining for TSH, ACTH, GH, and prolactin. Although the patient had no headaches and vision problems after the operation, he was still suffering from loss of libido and infertility. According to his laboratory results, hypogonadotropic hypogonadism still existed, and there was no any disturbance of other pituitary hormones (). Ultrasonography of the testicles revealed that both testicles were small as shown in . His semen analysis showed no sperm since the patient and his wife desired to have a child. Initially, human chorionic gonadotropin (hCG) (1500 units, 3 days/week) treatment was started. After 3 months, as testicular volume was still too small and there was no improvement on testosterone levels, hCG dose was increased to 3000 units for 3 days/week, and human menopausal gonadotropin (hMG) 75 µg for 3 days/week was added to hCG treatment. Then, hMG treatment was increased up to 150 µg for 3 days/week while he was on hCG (3000 units, 3 days/week). After 12 months, because of no improvement on both testosterone levels, sperm production, and testicular size, a chromosomal analysis was performed to investigate other causes of infertility. As a result of the analysis, GTG banding procedure was performed on metaphase chromosomes obtained from peripheral blood lymphocytes of the patient. The karyotype 46 XX was observed on 100 metaphases to exclude mosaicism and chimerism. Diagnostic testing of deletions of the sex-determining region Y (SRY), ZFY genes, and AZF region located on the long arm of the Y chromosome was performed by real-time polymerase chain reaction (PCR) amplification. The set of PCR primers that was used in multiplex PCRs for the diagnosis of microdeletion of these regions included: sY14 (SRY), ZFX/ZFY, sY84, sY86, sY127, sY134, sY254, and sY255. Molecular analysis revealed that ZFY and SRY genes were intact, but AZF region was deleted. Therefore, SRY+ 46 XX male sex reversal was diagnosed.
Since no treatment could induce spermatogenesis in 46 XX males, testosterone therapy was started instead of gonadotropin therapy. A genetic consultation and alternative ways of having a child (e.g., adoption) were recommended to the couple. In addition, the couple was advised to have psychological support if they needed it.


The patient was a 62-year-old Chinese woman with no previous disease history. She was admitted to our hospital in August 2016 due to intermittent fever with cough for 2 years, left chest wall redness, and swelling for 3 months. She presented with a fever and cough repeatedly from September 2014. Laboratory tests showed increased white cells and chest computed tomography (CT) suggested patchy infiltration in the left lower lobe, with mediastinal lymph node enlargement (–). Empirical treatment with cephalosporin was partially effective, but the symptoms were recurrent. In May 2016, she developed redness and a swelling in the left front chest wall with pain and high fever. She was admitted to our hospital for incision and drainage of the disease site and antibiotic administration. At the time of admission, laboratory tests showed white blood cells counts of 20.61×109/L (3.5–9.5×109/L); neutral cell percentages of 0.744 (0.4–0.75); hemoglobin: 79 g/L (130–175g/L), platelets: 384×109/L (125–350×109/L), blood C-reactive protein (CRP): 43.6 mg/L (0–8 mg/L), high levels of Immunoglobin G (IgG): 50.5 g/L (7.51–15.6 g/L); blood (1, 3)-D glucan (G tests): 146.20 pg/mL (<100.5 pg/mL); and blood galactomannan test (GM) positivity (0.64) (<0.5). HIV serology tests were negative, and normal CD4+ T cell counts and serum globulins levels (including IgA, IgM, and total IgE) were within normal reference ranges. Serum cryptococcal capsular antigen tests and blood tuberculosis infection T cell spot tests (T-SPOT.TB) were negative. Chest CT (2016-8-27) showed alveolar consolidation in the anterior segment of the left upper lobe, and an anterior chest wall with rib destruction and multiple lymphadenopathies in the left axillary and mediastinum (–). Fungal spores were detected in pus from the left chest wall and microbial cultures showed T. marneffei growth. Disseminated T. marneffei (lung, skin, and bone) were established and the patient was administered amphotericin B followed by itraconazole therapy. After 8 months of regular treatment, her condition improved and antifungal drugs were ceased. The patient was followed up regularly in the clinic (–).
In July 2017, she again developed a high fever. Laboratory examinations after hospital admission showed white blood cells counts of 11.66×109/L; neutral cell percentages of 0.647; hemoglobin: 80 g/L; platelets: 308×109/L; blood CRP: 53.9 mg/L; IgG: 30.2 g/L; erythrocyte sedimentation rates of 66 mm/h (0–20 mm/h); and CD4+ T lymphocyte ratios of 32.6% (34–52%). G-tests, GM, and pro-calcitonin were within the normal range. Repeat chest CT scans showed consolidation in the left and right upper lobes. Bronchoscopy examinations were pathogen negative. After 2 weeks of treatment with β-lactam antibiotics combined with oral antifungal drugs, no improvement in symptoms was observed and abnormal lung infiltration was observed in CT scans. CT-guided percutaneous right lung biopsy was performed. Pathological examinations demonstrated lung inflammation in the absence of granuloma formation. Mycobacterium avium (M. avium) was cultured and identified from mass spectrometry. Anti-NTM treatment included azithromycin, ethambutol, moxifloxacin, and rifabutin which were prescribed from August 2017. Due to adverse gastrointestinal and allergic reactions of the skin after 1 month of anti-NTM combination therapy, the patient refused treatment and was followed up in the clinic. No recurrence of the fever was observed.
In May 2018, the fever recurred with hand rashes, and the patient was admitted to the hospital a third time. Her body temperature peaked at 40°C with increased pulmonary infiltration accompanied by left sternoclavicular joint destruction (–). Physical examinations revealed multiple palpable lymph nodes in the left neck, erythematous plaques, and nodules in both hands (). Blood tests were similar to previous examinations in which increased numbers of white blood cells, CRP, and high levels of serum IgG were observed. Immune electrophoresis revealed polyclonal gammopathy, and blood/bone marrow cultures were negative. After biopsy from the left cervical lymph node and left sternoclavicular joint, M. avium was cultured from both sites. Histopathology demonstrated inflammation from the lymph nodes, and small amounts of elastic fibers with small blood vessels. Histopathology of the hand skin showed neutrophil infiltration ( and ). Microorganism cultures were negative. The patient was finally confirmed as disseminated NTM secondary to disseminated T. marneffei. In view of the negative microbial cultures in skin biopsy tissues, and the rapid resolution of hand rashes with steroid ointments, Sweet’s syndrome was diagnosed. Combination anti-NTM therapy was re-prescribed from June 2018. After 1 year of treatment, the patient’s symptoms and pulmonary consolidation improved. Bone destruction and lymphadenopathy, however, remained obvious (–). Laboratory tests were in the normal range except for IgG (). The patient presented multiple intracellular pathogen infections without HIV or immunodeficiency. Serum cytokines including IL-2, IL-4, IL-6, IL-10, TNF-α, and IFN-γ were within the normal range. Serum samples from both patients and controls (blood donors) including anti-IFN-γ autoantibodies, anti-IL-12 autoantibodies, anti-TNF-α, and IL-12 were assessed using ELISA kits (Cloud-Clone Corp). Patient serum was tested as four independent samples and statistical analysis were performed using unpaired t-tests (GraphPad Prism Software). The mean concentration of anti-IFN-γ antibodies in the serum samples of the patient (range: 33.13–47.06 ng/mL) was 2.85-fold higher than healthy subjects (range: 8.41–20.02 ng/mL) (P<0.01) (). No statistically significant differences between patients and controls for TNF-α, IL-12, and anti- TNF-α were observed (data not shown).


A 6-year-old boy, known case of atlantoaxial dislocation, was posted for C1-C2 lateral mass fixation. An adult bougie was used to insert a 5.5 cuffed Portex tube (Portex, Smith’s Medical India Private Ltd., Borivali, Mumbai, India) for a Cormack–Lehane (CL) grade 2b, after laryngoscopy with Macintosh blade (Macintosh Blade, Anaesthetics India Private Ltd., Goregaon, Mumbai, India) no. 2. Prone position was given after confirming bilateral equal air entry. The patient started desaturating and saturation dropped to 78%. Auscultation revealed a decreased air entry on the left side with wheeze. The FiO2 was made 100%, inhaled salbutamol and injectable hydrocortisone were given. The saturation improved to 88% but air entry did not improve on the left side. The patient became hypotensive (78/40 mm Hg). The patient was made supine and a check laryngoscopy was performed to confirm tube position. The tube was withdrawn slightly. Neither the air entry nor the saturation improved, and the hypotension was unresponsive to fluid boluses. On suspecting a pneumothorax, a chest radiograph was taken in the operation theatre with a fluoroscope. A large left-sided pneumothorax was seen, which was treated with intercostal drainage. The hemodynamics improved dramatically and saturation of 98% was attained. The surgery was abandoned. He was extubated after elective ventilation for 6h.
The patient was taken up for surgery 15 days later. Following a C-Mac-guided intubation, he had an uneventful intraoperative course.


A 7-year-old boy, operated case of C1-C2 lateral mass fixation, was posted for a transoral odontoidectomy. A CL grade 2b was visualized, for which an adult bougie was used to railroad a 5.5 number cuffed Portex tube, after laryngoscopy with Macintosh blade no. 2. After the procedure started, persistently high airway pressures (20–25 mm Hg) were recorded. As the entry of air remained equal on both sides, the high pressure was attributed to surgical manipulation. At the end of surgery, air entry was found to be reduced on the right side. Postoperative chest radiograph confirmed the presence of a large pneumothorax, which was relieved by intercostal drainage. The patient was electively ventilated for 1 day in view of transoral surgery and presence of pneumothorax.


A 16-year-old girl was taken to a local physician with complaints of recurrent abdominal pain and behavioral abnormality for two months. She had a history of two episodes of generalized tonic–clonic convulsions one month back with altered sensorium, which lasted for two days. Computed tomography scan of head was performed, which was normal. She was normotensive at presentation. Investigations revealed normal renal and liver parameters, negative antinuclear antibodies, normal urinalysis, and normal ultrasound abdomen. She was put on antituberculosis drugs for her abdominal complaints and on phenytoin for seizures.
After one month, she presented to us with altered sensorium for the last three days along with severe abdominal pain. On examination, she was found to be confused and disoriented. No focal signs, no meningeal signs, and no abdominal tenderness were observed. Contrast enhanced magnetic resonance imaging brain was performed, which showed T2-weighted and FLAIR hyperintensity in bilateral parieto-occipital lobes [ and ]. No diffusion restriction was observed on diffusion-weighted images []. These findings were suggestive of PRES. In view of recurrent abdominal pain, behavioral abnormality, and seizures, she was investigated for porphyria. Biochemical evaluation revealed markedly increased levels of urinary Porphobilinogen and aminolevulinic acid. She was given high carbohydrate diet and intravenous (IV) glucose, to which she responded dramatically over the next three days. In view of significant clinical improvement with IV glucose, heme was not given.


A 54-year-old female patient developed persistent severe pain in the upper abdomen after abdominal trauma combined with general weakness. After admission to a local hospital, she was diagnosed with abdominal hemorrhage with hemorrhagic shock, and emergency laparotomy was performed. During the operation, the liver was found to be enlarged diffusely, and a significant amount of non-coagulant blood was in the abdominal cavity. The right hepatic capsule was ruptured, and there was a large hematoma in the left liver. Liver biopsy was not performed, and the abdomen was closed after placing the abdominal drainage tube. Hemorrhagic ascites outflow continued after the operation. As the patient was in a critical condition, she was transferred to a superior hospital 2 d after the operation. After completing the relevant examinations, the patient was diagnosed with Budd-Chiari syndrome (a type of HVOD), portal hypertension, varicose esophageal veins, and gastric fundus veins. For further treatment, the patient came to our hospital. She had no history of other diseases.
Physical examinations showed temperature: 36.5 °C; pulse rate: 80 times/min; breathing rate: 16 times/min; and blood pressure: 120/80 mmHg. The patient had a poor general condition, emaciated, and weak with severe jaundice on the skin and mucous membranes; however, there were no liver palms or spider moles. Cardiopulmonary examination did not show any obvious abnormality. Abdominal swelling was present. There were no varicose veins in the abdominal wall. There was no obvious tenderness, rebound pain, and muscle tension, and the liver could be touched. Intestinal sound was 4 times/min, and both lower limbs had mild edema.
Laboratory tests revealed the following: White blood cell count: 11.97 × 109/L; routine urinalysis: Bilirubin 2 +, urine protein 3 +; Sed occult blood (-); blood biochemistry: ALT29U/L, AST74U/L, TBIL27 (1.4 µmol/L), DBIL (148.3 µmol/L), ALB (33.9 g/L), BUN (4.91 mmol/L), Cr (29 µmol/L); coagulation: PT15.3S, PTA (61%), INR (1.38); blood ammonia (45.2 µmol/L); HBsAg (-), HBsAb (+); CA125: 202.1; antibodies against autoimmune liver disease (-).
No abnormalities were observed on both chest radiography and electrocardiography.
Hepatic vascular ultrasound revealed that the inferior vena cava of the hepatic segment was compressed and narrowed by the enlarged hepatic tissue. The blood flow was unobstructed, and the three hepatic veins were not visible. The main portal vein and its intrahepatic branches were unobstructed.
Abdominal computed tomography showed that the inferior vena cava of the hepatic segment was narrowed. The left, middle, and right hepatic veins were not clearly visible. The main portal vein and its branches were unobstructed (Figure ).


A 46-year-old postmenopausal female presented to outdoor department of medicine, S.N. Medical College, Agra. She also complaint of heartburn for past 5 months for which she was taking tablet ranitidine 300 mg daily and she was symptomatically relieved on taking the medication regularly. She complained of pain and whitish discharge from both nipples for past 8--10 days. The milky discharge was spontaneous. On the basis of history, she had no pre-existing disease or any history of breast surgery, there was no drug history except ranitidine. On examination, both breasts were slightly engorged and associated generalized mild pain and tenderness, both breasts were mobile and there was no evidence of any lump. Her secondary sexual character and external genitalia were normal. Her rest of the systemic examination was within normal limit. Routine investigation-complete blood count (CBC), liver function test (LFT), renal function test (RFT), and thyroid function test, luteinizing hormone (LH), follicle stimulating hormone (FSH), estradiol, and testosterone were within normal limit. Visual field testing (perimetry) was normal. Serum prolactin was found slightly high 96 ng/dl. Chest radiography and mammography of both breasts were normal. Magnetic resonance imaging brain with contrast showed normal pituitary. Discharge from both the nipples stopped within 5 days as soon as the drug (ranitidine) was stopped. As other causes of hyperprolactinemia were ruled out, it was ascertained that ranitidine was the cause of galactorrhea in this patient.
On the Naranjo's severity assessment scale, the adverse event was 5 indicating a probable reaction to ranitidine.[] The severity of the adverse drug reaction (ADR) falls under level 2 as a mild severe reaction as per Hartwig's assessment.[]


A 27-year-old Saudi male patient known case of ulcerative colitis diagnosed 8 years ago based on history, clinical presentation, and histological features on mesalamine and prednisolone with poor compliance to medications. The patient presented to our hospital complaining of right neck and lip swelling for 10 days. In the history, the patient mentioned that he had bloody diarrhea four to six times day and he lost 10 kg in 1 month.


A 66-year-old Haitian male with a medical history of hypertension and benign prostatic hyperplasia presented with complaints of chronic diarrhea for several months. Diarrhea was associated with night sweats and an unintentional 80-lb weight loss over the past 5 years. He denied fever, chills, myalgias, pruritus, cough, wheezing, chest pain and shortness of breath. Our patient had emigrated from Haiti approximately 10 years previsouly; however, he reported visiting Haiti numerous times since then. Physical examination was unremarkable. Laboratory findings were significant for an elevated eosinophil count of 8%. The patient was negative for HIV. Other laboratory abnormalities included anemia (hemoglobin 11.7 g/dL, mean corpuscular volume 97 fL), basophilia (1.2%), hypoalbuminemia (3.2 g/dL) and hypoproteinemia (5.4 g/dL). Alanine aminotransferase, aspartate aminotransferase, alkaline phosphatase and total bilirubin were within normal reference ranges. He was diagnosed with strongyloidiasis based on the identification of stool larvae. During his hospital course, albendazole therapy was initiated and administered for 4 days.
Given the patient’s history of weight loss (80 lbs) and night sweats in the setting of positive TB Quantiferon test, computed tomography (CT) scan of the chest/abdomen was performed. Pancreatic and common bile duct dilations were incidentally noted on abdominal CT. Chest CT scan showed the presence of pulmonary apical scarring, reticulonodular changes and prominent inguinal lymph nodes. A QuantiFeron test was positive. Taken together, these findings were diagnostic for latent tuberculosis infection. The patient denied ever having been diagnosed or treated for active or latent tuberculosis. In addition, he denied pulmonary symptoms such as cough, wheezing and dyspnea. Abdominal CT revealed a dilated pancreatic duct, intrahepatic and extra-hepatic biliary duct dilation without choledocholithiasis, and a pancreatic cyst.
The patient was referred for esophagogastroduodenoscopy (EGD) and endoscopic ultrasound (EUS) to further characterize his symptoms of diarrhea, weight loss and the abnormally dilated pancreatic duct, which was noted on CT scan. EGD revealed gastritis and a suspicious ampullary lesion (). Multiple ampullary lesion biopsies followed by histopathology showed reactive epithelium with larva of Strongyloidiasis (). TB gram stain and culture was not performed on the ampullary lesion. On EUS, the ampullary wall appeared hyperechoic. The main pancreatic duct was dilated to 3.5 mm at the body and tail of the pancreas. An anechoic intraductal pancreatic main duct cyst measuring 8.9 mm x 13.8 mm was visualized at the head of the pancreas (). Fine needle aspiration showed normal levels of CEA, amylase and lipase, and was negative for malignant cells. Of note, the patient had an EGD and colonoscopy about 4 years previously, which was unremarkable.
A few weeks after discharge, the patient followed up after completion of albendazole therapy and reported complete resolution of diarrhea and night sweats. He also noted a 10-lb weight gain in the span of 2 weeks. The patient was continued on isoniazid and vitamin B6 therapy for latent TB. MRI abdomen/MRCP was performed 3 months later, which noted unchanged biliary and main pancreatic duct dilatations with tapering to normal caliber distally. No discrete biliary filling defect was identified. An unchanged 0.8-cm cyst in the tail of the pancreas was noted—a finding without overtly complex features. No ampullary lesion was identified.


This is 13-year-old girl with a homozygous LDLR c.313+5G>A mutation and evidence of an aortic root plaque. Her parents are first-degree cousins, both with heterozygous FH (HeFH).
This patient was initially treated with statins, ezetimibe and biweekly LA but the patient remained with increased LDL-C and evidence of atherosclerotic disease in the form of an aortic root plaque. Evolocumab was tried, but only for 3 months because of lack of response. With a background therapeutic regimen of atorvastatin 40 mg/day, ezetimibe 10 mg/day and biweekly (Q2W) LA, mean interval LDL-C levels were 263 mg/dL. The patient was commenced on lomitapide 5 mg/day. The patient initially suffered from nausea, occasional vomiting, reduced appetite and increased stool frequency; however, these symptoms became tolerable and no other adverse events were observed. Liver enzymes remained within normal limits. The patient was gradually escalated to lomitapide 20 mg/day and atorvastatin 40 mg/day with cessation of LA. Mean interval LDL-C levels are now maintained at 72 mg/dL (76% decrease) without the need for LA (Fig. ). The patient has been treated for over 18 months with no alteration in liver enzymes (Fig. ).


This is a 12-year-old boy with a homozygous LDLR c.682G>T mutation. Both parents have HeFH. The patient has left ventricle dilatation, mild aortic regurgitation and atherosclerotic plaques (1.5–2.1 mm thick) in both carotid bulbs, and in the common and internal carotid arteries. Imaging revealed diffuse atherosclerotic involvement of all coronary arteries, but this was not clinically significant.
The patient was treated with rosuvastatin 20 mg/day, ezetimibe 10 mg/day, LA Q2W, but mean interval LDL-C levels remained increased at approximately 300 mg/dL. The patient was commenced on lomitapide 5 mg/day following a normal FibroScan test. Rosuvastatin 20 mg/day, ezetimibe 10 mg/day and LA Q2W were maintained.
Mean interval LDL-C levels decreased modestly over 2 months (Fig. ). The patient then took a vacation with no LA, and experienced an increase in LDL-C levels that was gradually brought under control with escalating doses of lomitapide. Overall, LDL-C levels decreased by 50–70%. There has been no evidence of adverse events to lomitapide, and liver function tests (LFTs) remain normal (Fig. ). Gastrointestinal (GI) complaints were only present when dietary advice was not followed. The patient achieved an LDL-C nadir of 98 mg/dL (70% reduction). LA intervals have been gradually extended to Q4W with a modest increase in LDL-C levels to 184 mg/dL (44% reduction from baseline).


This is a 16-year-old boy diagnosed with HoFH at the age of 10 months on the basis of development of xanthomas, LDL-C levels 981 mg/dL, and LDLR genotyping with the finding of homozygosity for the LDLR mutation c.119_1207del. Both parents have HeFH, and the patient has a right coronary lesion evident on computed tomography, which has been treated with percutaneous revascularisation (at the age of 14 years). The patient was commenced on statins at the age of 2 years, which was gradually escalated to rosuvastatin 20 mg/day, ezetimibe 10 mg/day and colesevelam 3250 mg/day. At the age of 5 years, weekly LA was commenced, which resulted in regression of xanthomas by the age of 10 years, but without complete control of LDL-C levels (mean interval LDL-C 168 mg/dL).
In 2016, at age 14 years, the patient was commenced on evolocumab 420 mg QW with no effect on LDL-C levels. The following year, lomitapide was commenced at 5 mg/day, and gradually escalated to 40 mg/day. Mean interval LDL-C levels were reduced to 75–83 mg/dL, which represents a greater than 60% reduction in LDL-C (Fig. ).
The patient began to have some recurrent issues with elevated liver enzymes, possibly related to rosuvastatin, which had caused this in the past. Lomitapide dose was reduced to 30 mg/day, and rosuvastatin briefly stopped while the LFTs resolved. Eventually, LA intervals were extended to every 2–3 weeks and rosuvastatin was permanently stopped. The patient remained on lomitapide 40 mg/day. Increasing lomitapide dose to 45 mg/day was associated with an increase in LFTs that was brought under control with dose reduction and gradual re-escalation. The patient is now doing well on lomitapide 60 mg/day with an LDL-C level of just 90.8 mg/dL and LA Q2W. There has been no evidence of GI adverse events apart from mild flatulence.


This patient is a 7-year-old boy with compound heterozygote mutations in LDLR c.666C>A and c.1646C>A. The patient has a sister with HoFH who is on LA and not part of this patient series. Both parents have HeFH. The patient has a large aortic plaque extending the length of the thorax with 25–50% narrowing of the lumen.
Before lomitapide, the patient was treated with rosuvastatin 40 mg/day, ezetimibe 10 mg/day and colesevelam 625 mg/day. No LA was used as a result of venous access issues. LDL-C levels were increased at 866 mg/dL. The patient underwent dose reduction of rosuvastatin (20 mg) and was commenced on lomitapide 2.5 mg/day, which was gradually escalated to 30 mg/day in 5-mg increments. LDL-C levels reduced by 78% to 360 mg/dL (Fig. ). Notably, this patient has been treated for over 18 months and has not experienced any side effects of lomitapide.


This patient is an 11-year-old girl with a homozygous duplication spanning the promoter region to exon 6 of LDLR (c.-187-? 940+? Dup) []. Family history includes sudden death in an older brother aged 17. Another brother has HoFH with aortic stenosis and a non-critical obstruction of the right coronary artery. The patient presented with non-critical aortic stenosis/supra aortic stenosis, and non-obstructive plaques in the carotid arteries. Her computed tomography angiography showed no signs of coronary artery disease.
The patient commenced LLT at the age of 2 years when LDL-C levels were 1009 mg/dL. Up until the age of 7, she was treated with atorvastatin 40 mg/day and ezetimibe 10 mg/day and her LDL-C level was 453 mg/dL. LA is not available in the patient’s home country.
At age 7, atorvastatin dose was decreased to 10 mg/day, and lomitapide was commenced at a dose of 5 mg/day. Over the next year, lomitapide was escalated to 30 mg/dL but this dose was reduced back to 20 mg/day as a result of diarrhoea. LDL-C levels were effectively reduced with treatment (nadir 231 mg/dL; 40.7% reduction) with no elevations in LFTs (Fig. ). A trip to a remote region of Brazil led to brief discontinuation of lomitapide with a consequent increase in LDL-C levels. Following resolution of the issues, reintroduction of lomitapide resulted in the expected decrease in LDL-C levels.
The patient is now maintained on atorvastatin 60 mg/day, ezetimibe 10 mg/day and lomitapide 20 mg/day and has been treated with lomitapide for 4.5 years. She continues to develop normally and had her menarche at the age of 11 years. LDL-C levels are 266 mg/dL (38% reduction from pre-lomitapide values). A routine check-up revealed no calcification in the coronary arteries; however, there were signs of aortic stenosis and supra aortic stenosis, and some non-obstructive plaques in the carotid arteries, but these are not considered to be life-threatening. There were no elevations of liver enzymes during treatment. A detailed history of this patient has been published by Chacra et al. [].