A 59-year-old female patient, who was monitored at a neurology clinic, was referred to our clinic due to sustained hemifacial pain on the right side. The patient had diagnosed diabetes mellitus type 2, which was under control. There were no pathological findings in the patient's anterior rhinoscopic or endoscopic nasal examination (), although the nasal septum was observed to be deviated to the left. The patient's hematological and biochemical parameters were normal. As an advanced examination, paranasal computed tomography (PNCT) imaging was performed. A radiological examination revealed concha bullosa variation of the bilateral middle turbinates, and soft tissue density in the right concha bullosa (). Concha bullosa was incised by applying topical anesthesia to the patient's right nasal cavity, and a punch biopsy was performed. As the pathology report concluded fungal hyphae and necrobiotic material, the patient was prepared for surgery. Upon receiving written consent from the patient, endoscopic sinus surgery under general anesthesia was planned. Lateral and inferior lamellae of the concha bullosa were excised and the infected material inside was submitted to pathologic examination (). Upon controlling the bleeding, the intervention was finalized. The patient was prescribed nasal wash and anti-inflammatory treatment post-operatively.
The pathological examination report stated fungal organisms and their hyphae in the necrobiotic material submitted to pathology (). "Aspergillus oryzae" was cultured by sequential analysis of the ribosomal DNA genes in the aspirated material submitted for advanced microbiological examination. During the post-operative 6-month follow-up, the patient's headache complaints were diminished and the nasal passage was open.


A 27-year-old man with a 10-year history of BD, which had been left untreated for the preceding 2 years, referred to our cardiovascular surgery department with symptoms of serious aches in the left arm, edema, and apparent veins on the left anterior chest wall. The color Doppler ultrasonography of the left upper extremity venous system showed a total thrombosis of the left internal and external jugular veins and the left subclavian vein.
A single dose of low molecular weight heparin (LMWH) (7500 IU/0.3 mL of bemiparin sodium) treatment for a day, antibiotics, and anti-inflammatory drugs were ordered. One month after the hospital discharge, the patient returned to our clinic with symptoms of dyspnea and coughs. A physical examination revealed decreased occultation sounds in the middle and bottom fields of the left thorax. A posteroanterior direct chest radiograph illustrated a consolidated region, in line with the auscultation findings (). A milky liquid was aspirated via thoracocentesis from the left thorax and was subsequently biochemically analyzed; the results demonstrated total protein of 4.3 g/dL, albumin of 2.2 g/dL, total cholesterol of 47 mg/dL, and triglycerides of 287 mg/dL.
In light of the findings, a diagnosis of chylothorax was established. The patient was hospitalized and received single doses of LMWH, 0.5 mg of colchicine, 40 mg of an oral corticosteroid (prednisolone), 2 doses of oral immunosuppressive therapy (50 mg of azathioprine), and a high-carbohydrate and low-fat dietary regimen. A left thoracic drainage system was established for the patient. On the seventh day of hospitalization, due to a progressing cheilosis flow, a pleurodesis process was applied with talcum powder. However, the chylothorax progression continued for the following 3 days. Venous autologous blood (60 mL) was injected into the left thorax via a drainage tube, which was clamped in order to avoid the leakage of the blood back through it. For the ensuing 3 hours, the patient was moved into different lying positions. Two days later, the cheilosis flow decreased to an acceptable level, and the dietary limitations were canceled. At the end of the 15th day following the autologous blood pleurodesis process, the cheilosis flow totally disappeared. The patient was discharged with a treatment of an oral anticoagulant (5 mg of warfarin) and a single dose of an oral immunosuppressive therapy (50 mg of azathioprine). One month after the hospital discharge, no pathology was observed on a posteroanterior direct chest radiogram ().


The patient was a 46-year-old Hispanic male who presented with a six-month history of productive cough and pleuritic chest pain associated with intermittent episodes of fevers and chills. Approximately three months into his symptoms, the patient began to experience dysphagia and subsequent decreased appetite. His dysphagia progressed from solids to liquids. He denied having trouble breathing. The patient reported an unintentional 40-pound weight loss over the three-month period following the onset of his dysphagia. His vital signs were within normal limits, and the patient appeared his stated age. The physical exam was remarkable for cachexia.
Initial lab work was unremarkable. Initial chest X-ray revealed a prominent right upper lobe cavitary lesion (). Further workup was obtained with a CT chest, which showed “tree-in-bud” opacities in bilateral lung fields, along with “thick walled” cysts of the right upper lobe (). This was reported to be suggestive of TB versus fungal infection. Additionally, the imaging revealed mediastinal lymphadenopathy. Sputum acid-fast bacilli (AFBs) were collected, and the patient started treatment with rifampin, isoniazid, pyrazinamide, and ethambutol (commonly referred to as RIPE therapy), prior to sputum acid-fast bacilli cultures returning positive [].
To determine the etiology of the dysphagia, the patient underwent a swallow evaluation which revealed combined oral and pharyngeal dysphagia. Follow-up studies with an esophagogastroduodenoscopy () and bronchoscopy () revealed bronchoesophageal fistulas, which were presumed to be a result of MTB infection. Given his inability to tolerate oral nutrition, a percutaneous endoscopic gastrostomy (PEG) tube was placed for nutritional purposes. Once he was able to tolerate PEG tube feeds, he was discharged home on RIPE therapy and given a follow-up appointment in an infectious disease clinic. At a three-month follow-up appointment on RIPE therapy, the patient was able to tolerate oral nutrition. He was reevaluated with a bronchoscopy (), which showed interval healing of the bronchoesophageal fistulas. Since the patient was tolerating a regular diet, the PEG tube was scheduled to be removed three months after it was placed. The patient followed up at nine months in the clinic. At that time, he reported no symptoms. He completed a nine-month course of RIPE therapy with no further complications.


A 76-year-old woman was referred to our hospital because of a nodule in the right upper lobe. The patient had no respiratory symptoms. The computed tomography (CT) scan of the chest revealed a 2.3 × 1.6 cm nodule, and the patient was diagnosed with pulmonary adenocarcinoma (cT1bN0M0, stage IA, according to the TNM classification of the Union for International Cancer Control (UICC), 7th edition; cT1cN0M0, stage 1A3, according to the UICC, 8th edition). The patient had no smoking history. Her comorbidities consisted of hypertension and hyperlipidemia. The right upper lobectomy was performed in November 2015, and the pathological stage was pT1bN2M0, stage IIIA. Genomic analysis revealed the epidermal growth factor receptor (EGFR) gene mutation L858R in exon 21. Immunohistochemical analysis revealed a programmed death-ligand 1 (PD-L1) tumor proportion score (TPS) of < 1%. The patient was under watchful observation without adjuvant chemotherapy. Multiple mediastinal and right hilar lymph node metastases were found in February 2018. Radiation therapy alone was selected because the recurrence of the disease was limited to the local region and the patient was 79 years old at the time of recurrence. Radiation therapy amounting to a total dose of 60.0 Gy, distributed in 30 fractions, was performed over a period of 6 weeks. The 3D radiotherapy-planning technique was used. In the first 4 weeks, 40.0 Gy was distributed in 20 fractions using opposing anterior-posterior fields (Fig. a, b). In the subsequent 2 weeks, an additional 20.0 Gy was distributed in 10 fractions using 10 beams (Fig. c, d) for covering multiple mediastinal lymph node metastases but sparing the spinal cord and hilum of the left lung. The planning target volume included multiple mediastinal and right hilar lymph nodes, with a 10 mm margin to account for microscopic disease, internal moving, and setup errors. The percentage volume of lung receiving a dose of more than 20 Gy (V20) was 29.3%. Static radiotherapy was delivered using a 10 MV X-ray and five fractions per week. A chest CT scan performed 6 weeks after the irradiation therapy showed reduction of the lymph node metastases. However, left hilar and right supraclavicular lymph node metastases and multiple pulmonary metastases were newly observed outside of the irradiation field (Fig. ). EGFR tyrosine kinase inhibitor (TKI) treatment was planned as a start. Interestingly, a follow-up chest CT scan performed 12 weeks after the completion of irradiation therapy showed complete disappearance of the multiple pulmonary metastases associated with radiation pneumonitis (Fig. ). However, there was no change in the size of the left hilar and the right supraclavicular lymph node metastases, which was confirmed by 18-fluorodeoxyglucose positron-emission tomography (PET)/CT (Fig. a, b). The patient was therefore under watchful observation, without receiving EGFR-TKI treatment. A follow up CT scan performed 6 months after the completion of irradiation therapy showed a slight increase in the size of the lymph node metastases, but no reappearance of multiple pulmonary metastases nodules (Fig. ). The levels of serum carcinoembryonic antigen (CEA) dropped from 154.5 ng/mL to 30.9 ng/mL after the irradiation therapy and further decreased to 6.8 ng/mL by 3 months post irradiation therapy. The levels, however, increased again to 19.7 ng/mL at 6 months after completion of the irradiation therapy (Fig. ). No cytotoxic chemotherapy or EGFR-TKI was given during the period. The number of CD8+ lymphocytes was 190/μL at 3 months and 356/μL at 4 months after irradiation.


In a 55-year-old female patient (Patient 1, see Table ), a sweat test was performed due to recurrent pneumothoraces and bilateral bronchiectasis with mucoid impaction apparent in thoracic computed tomography (CT). Sweat test showed borderline values of sweat chloride two times (52 and 41 mmol/L); therefore, genetic testing for mutations in the CFTR gene was initiated. Indeed, two mutations, F508del and R117H-7T, were detected. Pulmonary function testing revealed a severe obstructive ventilation disorder with a FEV1 (forced expiratory volume in 1 sec) of 27% predicted and respiratory failure with the need for oxygen therapy. Indications of the presence of a liver or pancreas involvement in the context of the underlying disease were not found. The repeatedly determined pancreatic elastase was in normal range (> 200 μg/g). In sputum samples, P. aeruginosa was cultured several times. Serologically, antibodies (Elastase and Exotoxin A) against P. aeruginosa were significantly increased as an indication of chronic respiratory tract infection. The patient reports a long history of disease with shortness of breath, productive cough and recurrent pulmonary infections with frequent antibiotic therapies until the final diagnosis was made. She had used bronchodilators (short- and long-acting beta 2-sympathomimetic agents) and inhaled corticosteroids for many years assuming she suffers from a chronic obstructive pulmonary disease (COPD) although she was non-smoking and was not exposed to occupational hazards. Due to severe obstructive pulmonary disease the patient has been repeatedly treated with systemic corticosteroids in the past.
In addition to pulmonary involvement, the patient also had a long-standing affection of the paranasal sinuses. Due to chronic infection of the respiratory tract with P. aeruginosa, we initiated an inhalative antibiotic therapy with tobramycin 300 mg twice a day and later changed to colistin 2 MIU twice a day because of an increase of bronchial obstruction. Hypertonic saline 3% and Dornase alfa were introduced for improving airway clearance. Furthermore, with help of the respiratory therapists, the patient learned airway clearance techniques (ACT) to perform this independently. At that time, nearly monthly hospitalization was necessary as the clinical condition worsened.
After diagnosis of the index patient, her twin sister (Patient 2, see Table ) was introduced to us for further diagnostics. Here, too, a borderline sweat test was found (sweat chloride 44 mmol/L). A repeated sweat test showed a nearly normal value (33 mmol/L). Subsequent genetic testing also revealed a compound heterozygosity for F508del and R117H-7T.
This patient had markedly less symptoms such as cough and shortness of breath. Also, no recurrent pulmonary infections were reported. However, chronic nasal congestions and recurrent sinusoidal infections were reported. A CT-scan of the upper respiratory tract revealed chronic pansinusitis with accentuation of the maxillary sinus. The patient has undergone sinus surgery multiple times in the past. In addition, the patient reported to suffer from allergic bronchial asthma, especially in spring. In this regard, there exists an on-demand therapy with an inhaled corticosteroid and a long-acting beta-agonist bronchodilator.
Pulmonary function showed only a moderate obstructive ventilation disorder (FEV1 = 74%pred.). A CT-scan of the chest revealed bronchiectasis in both upper lobes and a consolidation in middle lobe. In repeated sputum samples, Staphylococcus aureus was detected. Serologically, the reciprocal antibody-titres against P. aeruginosa were all negative. Notes on exocrine or endocrine pancreatic insufficiency or liver involvement were not documented.
Both patients grew up together under the same conditions and in the same area. Both were non-smokers and were not exposed to any substances harmful to the lungs, privately and professionally. There was no family history. Because of the severity of the lung disease, Patient 1 was unable to do any work and was retired while her twin sister was doing a full-time job.
After diagnosis, both patients were placed on ivacaftor. Initially, the drug was well tolerated by both. Patient 2 showed two to three times elevated liver enzymes (ASAT, ALAT) shortly after administration of ivacaftor 150 mg twice a day, which is why we were forced to reduce the dose of ivacaftor. At half the dose, a normalization of the liver function was recognized. Patient 1 initially complained of a bronchial tightness, which could not be detected any more after approximately 14 days under symptomatic therapy.
After initiation of ivacaftor therapy, the pulmonarily more affected Patient 1 did not show improvement in lung function (see Fig. ). A clinical response of the ivacaftor therapy could be documented by reduction of exacerbation rate, weight gain (+ 6 Kg), improved airway secretion mobilization, a reduction of hospitalization rate and a lower need for antibiotic therapy. Due to the lack of pulmonary response of standard and potentiator-therapy, we decided to list the patient for lung transplantation. Ivacaftor therapy was continued because of its stabilizing effect on the disease state. A sweat test repeated after 6 months now shows a normal value of sweat chloride (20 mmol/L).
The pulmonarily less affected twin sister showed improvement in lung function with an increase in FEV1 and FVC after initiation of therapy (see Fig. ). In the repetition of the sweat test, a normal value could be determined (19 mmol/L), as with the sister, even under half the dose of ivacaftor. The existing chronic paranasal sinus complaints were also declining. In addition, the patient reported an improved daily workload. Inpatient treatment was not necessary before or after treatment initiation with ivacaftor.


A 57-year-old male with a history of sickle cell disease (HbSC) without long-term indwelling vascular access complicated by recurrent cholecystitis and choledocholithiasis presented to our quaternary care hospital with a chief complaint of right upper quadrant and back pain. His vital signs on presentation included a temperature of 99.2°F, heart rate of 107 beats per minute, blood pressure of 105/58, respiratory rate of 22/min, and oxygen saturation of 96% on room air. His exam was remarkable for scleral icterus and right upper quadrant tenderness. Initial labs were notable for a leukocytosis of 38,200 cells/mm3, direct hyperbilirubinemia of 26.1 mg/dL, aspartate aminotransferase (AST) and alanine aminotransferase (ALT) of 425 IU/L and 303 IU/L, respectively, and a lactate of 7.3 mmol/L. He was admitted to the intensive care unit for management of shock. Blood cultures drawn on the day of admission grew Klebsiella pneumoniae and Citrobacter koseri. He was started on intravenous piperacillin-tazobactam. Imaging at the time of presentation displayed evidence of prior cholecystectomy with obstructive choledocholithiasis and multiple liver abscesses less than 2 cm in diameter. He underwent emergent endoscopic retrograde cholangiopancreatography (ERCP) with balloon dilation of the common bile duct and biliary stent placement. Transthoracic echocardiogram done at that time showed decreased ejection fracture with otherwise normal valve structure and function. Despite biliary stenting, the patient continued to complain of right upper quadrant and lower back pain and his blood cultures remained persistently positive after stenting.
On hospital day 6, the patient was reimaged with magnetic resonance cholangiopancreatography (MRCP) which displayed evidence of possible ongoing biliary obstruction. A midline was placed for ease of access for medication administration. On hospital day 7, repeat ERCP showed a patent stent from his prior procedure, but a second stent was placed to relieve a left hepatic lobe stricture. Even with this attempted source control, polymicrobial bacteremia persisted with both Klebsiella pneumoniae and Citrobacter koseri through hospital day 12. A transesophageal echocardiogram (TEE) was completed to evaluate for infectious endocarditis as an ongoing source of bacteremia. A 10 mm mobile vegetation was visualized on the pulmonic valve with mild pulmonary regurgitation (). The Infectious Diseases consult team advised switching from piperacillin-tazobactam to combination therapy of intravenous ceftriaxone and oral ciprofloxacin. His midline catheter was removed, but tip was not sent for culture. His blood cultures cleared on hospital day 13. Repeat CT abdomen pelvis completed on hospital day 18 revealed an overall improvement of the previous liver abscess, except for one right lobe abscess that was slightly increased in size, and a new subpleural left lower lung lobe nodule concerning for septic embolus. Surgical evaluation was not pursued given his comorbidities and poor surgical candidacy. Ultimately, he was discharged to complete 6 weeks of intravenous ceftriaxone and oral ciprofloxacin. At the time of this case report, he has not required further intervention on the heart valve.


A 29-year-old male was admitted to the intensive care unit with acute respiratory failure requiring intubation in the field. The patient was found unresponsive on the floor of his apartment. His medical history included morbid obesity with a body mass index of 43 kg/m2 and alcohol dependence syndrome.
On initial examination he was sedated on mechanical ventilation, afebrile (98 F), hypertensive with a blood pressure of 167/69 mmHg, tachycardic with heart rate of 111 beats per minute and a respiratory rate of 20 breaths per minute synchronous to the set rate on the ventilator. Peripheral capillary oxygen saturation (SPO2) was 98% with inspired oxygen saturation (FiO2) of 0.5. Lung examination revealed rales in the mid and lower zones of the right lung field. Cardiac examination revealed tachycardia with no murmurs. Abdominal, neurologic and skin exams were unremarkable.
Relevant laboratory on admission included the following: leukocytosis (white cell count of 11.2 × 103 cells/ /μL), serum creatinine of 2.1 mg/dL (baseline 0.6 mg/dL), serum creatine kinase of 39, 552 units/L, serum lactic acid of 4.2 mmoles/L, and serum ethanol level of 316 mg/dL. Initial arterial blood gas analysis revealed a pH of 7.26, an arterial carbon dioxide (PCO2) level of 40.2 mmHg and oxygen level (PO2) of 108 mmHg on a FiO2 of 0.5, and positive end-expiratory pressure (PEEP) of 5. Chest X-ray (CXR) showed right upper, middle, and lower lobe infiltrates and computed tomography (CT) of the head demonstrated no acute infarcts or hemorrhage.
He was started on broad spectrum antibiotics including vancomycin, piperacillin-tazobactam, and azithromycin for pneumonia. On day 2 of admission, the patient underwent fiberoptic bronchoscopy which revealed thick mucopurulent secretions in all segments of right lung. The respiratory cultures from bronchoalveolar lavage were negative as well as initial blood and urine cultures. Transthoracic echocardiogram (TTE) on day 2 revealed an ejection fraction of 57% with normal right ventricular structure and function and normal pulmonary artery systolic pressure (PASP). The patient clinically and radiologically improved, he was awake and tolerating daily spontaneous breathing trials with FiO2 requirements of 0.4 to keep PO2 of 104.
On day nine of admission, he was noted to be hypoxic with a PO2 of 52 mmHg with a SPO2 of 88%. He required a PEEP of 14 cmH20 and a FiO2 of 1 to achieve a PO2 of 66 mmHg. Due to body habitus prone position was not an option. Repeated CXR did not show any new abnormalities- resolving right-side infiltrates. In addition to the subcutaneous heparin started on admission for deep venous thrombosis (DVT) prophylaxis, a therapeutic dose of low molecular weight heparin (LMWH) at 1 mg/kg was administered with a presumptive diagnosis of acute pulmonary emboli as he was too unstable for transportation to radiology. A repeat TTE showed a PASP of 54 mmHg with new moderate dilatation of the RV and moderate tricuspid regurgitation () with a serum troponin T of 0.204 ng/mL and proBNP of 445 pg/mL. Inhaled nitric oxide (iNO) was given via the breathing circuit of the mechanical ventilator at a concentration of 20 parts per million (ppm) with improved oxygenation noted after one hour, PO2 increased to 81 mmHg with a FiO2 of 0.8. His blood pressure during this time ranged from a systolic of 117-134 mmHg and diastolic of 68–87 mmHg.
Chest CT angiogram revealed large embolus in the right main pulmonary artery (). Thrombolytic therapy with alteplase at 100 mg was administered over two hours and the iNO was slowly tapered off over the next four days with concomitant tapering of PEEP to 8 cmH20 and FiO2 of 0.45. Repeat TTE three days after thrombolytic therapy demonstrated a PASP of 32 mmHg with mild dilation of the RV without signs of strain (). The patient was weaned from mechanical ventilation and later discharged to a skilled nursing facility for rehabilitation on oral apixaban for three months with outpatient follow-up provided.


A 19-year-old male, a resident of Mirpur with no known co-morbidities, presented to the emergency department (ED) of Dr. Ruth KM Pfau, Civil Hospital Karachi (CHK) in September 2018 with a history of fever, dizziness, generalized weakness and bleeding of gums since the past three days. According to the patient, he suddenly developed a continuous fever of 102°F, which was sporadic in nature and associated with chills and rigors. The fever temporarily alleviated with intake of antipyretics. He also experienced occasional bleeding from gums and dizziness with generalized weakness, for which he sought symptomatic treatment from a local health care facility, but the symptoms worsened. Therefore, he was then referred to CHK. No history of bleeding from any other site, nor hematemesis or black tarry stools was present. The patient revealed a decreased appetite and past addiction to tobacco.
On examination (O/E), the patient was of average height and built, comfortably lying on the bed, and well oriented to time, place and person. Initial vitals included blood pressure (BP) 120/70 mmHg, a regular pulse of 90 beats/min and a respiratory rate of 20 breaths/min. The patient was anemic and dehydrated. He had a soft, non-tender, non-distended abdomen without hepatosplenomegaly, and bowel sounds were audible with a rate of 3-4/min. All other systems were unremarkable.
Blood investigations revealed a hemoglobin (Hb) of 4.5 gm/dl, mean corpuscular volume (MCV) of 108.5 fl, mean corpuscular hemoglobin concentration (MCHC) of 35.4 gm/dl, total leukocyte count (TLC) of 2.7 x 109 L, hematocrit (HCT) of 12.7%, platelet count (PLT) of 12 x 109/L. The clotting profile showed an international normalized ratio (INR) of 1.11, while prothrombin time (PT) and activated partial thromboplastin time (aPTT) were 11.1 and 21.7 seconds, respectively. The various lab investigations conducted, including those for hepatitis B surface antigen and hepatitis C antibody, both came out normal, as did his X-ray chest, renal and liver function tests, spot urine examination and ultrasound of the abdomen. The need for conducting specific investigations like hepatitis and chest X-ray were to exclude any other likely cause of the fever and presence of an infection. The electrolytes were within the normal range as well. Upon serology testing, dengue antigen came out to be reactive while that of dengue virus-specific antibodies, immunoglobulin M (IgM) and immunoglobulin G (IgG), were not reactive. Malarial parasite (MP) and MP immunochromatographic test (ICT) came out to be positive for Plasmodium (P) vivax with 7% reticulocytes (RET). Therefore, a diagnosis of concurrent malaria and dengue infection was established.
The patient was treated with an oral combination of artemether and lumefantrine 80/480 twice daily for three days, along with oral acetaminophen two tablets if needed. Additionally, he was intravenously (IV) given tranexamic acid 5 mg when necessary and 1000 ml sodium chloride at the rate of 80 ml/hour. Two units of packed cells and six units of platelets were transfused alongside. Malaria and dengue were treated as separate entities with the above-mentioned treatment.


A sixty-four-year-old female with a past medical history of newly diagnosed hypertension (HTN), type two diabetes mellitus, and hyperlipidemia presented with worsening generalized weakness and bilateral lower extremity swelling for two to three months. She also reported occasional flushing and easy bruising. She gained 20 pounds over six months with centrally distributed fat.
She was recently diagnosed with HTN and diabetes type two by her primary care physician. Routine lab work also showed hypokalemia. Examination revealed high blood pressure and tachycardia with two plus pretibial pitting edema bilaterally.
Due to the combination of recently diagnosed HTN and diabetes type two with hypokalemia, the patient underwent a workup for Cushing syndrome. Cortisol level in the early morning was 45 ug/dL (normal range: 7–28 μg/dL), and ACTH was 444 pg/mL (normal range: 10––60 pg/mL). High-dose dexamethasone suppression test was positive. Prolactin, insulin-like growth factor 1 (IGF-1), and thyroid-stimulating hormone (TSH) were all within normal limits. Subsequently, the patient had an MRI of brain which was negative for any mass. However, the CT scan of abdomen/pelvis with contrast revealed a mass in the pancreas with multiple liver lesions and lymphadenopathy (Figure ). Pathology of the pancreatic mass which was obtained via endoscopic ultrasound (EUS) confirmed a well-differentiated intermediate-grade NET. Subsequently tumor markers were checked. Carbohydrate antigen (CA) 19-9 was 41.1 U/mL (normal range: 0–35 U/mL). Carcinoembryonic antigen (CEA) was 4.7 ng/mL (normal range: 0–5 ng/mL). Chromogranin A was 210 ng/mL (normal range: 0–95 ng/mL). The patient was evaluated for possible Whipple procedure. However, she was deemed not a candidate due to significant liver tumor burden. Her clinical course deteriorated rapidly despite medical management. She passed away two months after the diagnosis of the pancreatic NET.


A 48-year-old man with ESRD secondary to diabetic nephropathy was admitted for initiation of renal replacement therapy. He had undergone 36 cycles (36 hours of 1 L/h cycle) of acute PD, through a temporary PD catheter inserted through the infra-umbilical approach, at another center 10 days prior and presented to us for maintenance dialysis. After discussing the options, he was started on hemodialysis through a temporary catheter and was planned to start on CAPD. He had a history of diabetic retinopathy and neuropathy. He did not have any history or symptoms suggestive of neurogenic bladder.
The patient underwent 3 sessions of hemodialysis and was planned for CAPD catheter insertion with vancomycin prophylaxis. As per protocol, he was advised to void his bladder completely and to pass stools before insertion. An infraumbilical midline incision was made under local anesthesia and IV sedation, an 18-G introducer needle was inserted through the rectus sheath until it reached the peritoneal cavity, and 1.5 L of peritoneal dialysate was instilled intraperitoneally. After instillation of fluid, a guidewire was passed through the needle, with the tip directed towards the suprapubic region. The needle was removed, the tract was dilated using a 16 Fr dilator followed by insertion of a 16 Fr peel away sheath/dilator. The guidewire was removed followed by the dilator, and Tenckhoff swan-neck double-cuff straight catheter was inserted through the peel away sheath. The sheath was peeled away, and a subcutaneous tunnel was created with the exit site facing down and out towards the left iliac fossa. The deep cuff was positioned over the peritoneum, and the superficial cuff was placed in the tunnel. The incision site was sutured in layers. Postprocedure, the inflow and outflow were found to flow without resistance, and the catheter was brought out through a left-sided tunnel. A 500-mL flush was given, and good inflow and outflow were noted. The outflow was initially blood-mixed, followed by drainage of clear fluid. There were no complications during the immediate postoperative period. With a plain abdominal radiograph, the position of the catheter tip was confirmed to be in pelvis (). Ultrasonography was not used during the procedure due to logistic reasons.
On the 3rd day after insertion, after instillation of 500 mL of PD fluid, the patient complained of urinary urgency with an increase in urinary volume to 700 – 800 mL/day from his baseline of 300 – 400 mL/day. Also, there was decrease in the outflow volume with drainage of only 100 mL following instillation of 500 mL of PD fluid. With the increase in the inflow volume, there was a significant increase in his urine volume. Urine analysis was done to look for glycosuria, which showed a 3(+) on dipstick, from his baseline glycosuria of 1(+). He was asked to void after instilling 500 mL PD fluid through the PD catheter, and urinary creatinine testing was done, which was found to be absent. A possibility of peritoneovesical fistula was considered, and a cystogram was planned. The instilled fluid presumably entered predominantly into the urinary bladder through the side holes and led us to presume that the PD catheter could be in the preperitoneal location. Urine culture and PD fluid cultures were sent, which revealed growth of enterococcus (> 105 colony forming units/mL), and he was treated with vancomycin intraperitoneally (IP) and broad-spectrum antibiotics intravenously.
The cystogram revealed a smooth-walled bladder, with no intraperitoneal leak of the contrast, and the catheter abutting the bladder wall (). A plain abdominal computed tomography (CT) was performed to confirm the position (). The catheter was seen to be piercing the anterior abdominal wall and entering the bladder anteriorly, perforating and coursing through the bladder before exiting through the posterior wall, with the tip in the rectovesical fossa. PD was discontinued, and he was continued on hemodialysis through a temporary femoral access catheter. After discussion with the urologists, it was planned to remove the catheter, followed by reinsertion at a later date, with conservative management for the bladder perforation. With cessation of PD, there was decrease in urine output to ~ 50 – 100 mL/day, and a repeat urine routine performed 3 days later showed reduction in pyuria (from plenty of white blood cells/high power field to 6 – 8 WBC/HPF).
The options were discussed with the patient, and the need for a vascular access was explained. However, due to logistic reasons, an attempt was planned towards catheter salvage. He was planned for catheter repositioning using fluoroscopic guidance under the cover of intravenous and intraperitoneal broad-spectrum antibiotics.
Using fluoroscopy with contrast (urograffin), first the bladder perforation was confirmed. The infraumbilical incision was re-explored under local anesthesia, while leaving the tunnel in situ. The deep cuff was identified, and the PD catheter was pulled back by ~ 6 cm (as measured by the prior abdominal CT image). Contrast was injected through the catheter, and it was confirmed to be intraperitoneal with no filling up of the urinary bladder. Using a guide wire, the catheter was redirected towards the right iliac fossa, and the position confirmed. On subsequent contrast injection through the PD catheter, the intraperitoneal nature and absence of bladder filling was confirmed. The bladder was later filled up with contrast, and there was no presence of any leaks (). The initial bladder perforation could have been an intraperitoneal perforation since pulling back and re-positioning the catheter, without any new entry into the peritoneum, confirmed it to be in the peritoneal cavity.
The patient was continued on IV antibiotics for 5 days, IP ceftazidime for a week, and IP vancomycin for 21 days, and was continued on hemodialysis. IP vancomycin was given in a single dwell (1,000 mL) for 6 hours every day. One week post repositioning, he was started on low-volume CAPD and discharged, and a repeat urine and peritoneal fluid culture were sterile. He was continued on Foley’s catheter for 4 weeks, with a residual urine output of ~ 200 – 300 mL/day. At 9-month follow-up, the patient is comfortable with CAPD 3 exchanges/day with good ultrafiltration (~ 1.4 L/day), and no change in urinary volume, with no further episodes of peritonitis.


A 36-year-old woman presented in 2006 at the department of surgery, Maria Pia Hospital, Turin, for important chronic constipation and abdominal pain. She had a long history of constipation with an average of one evacuation every four days despite continual use of laxatives and had been hospitalized several times before for intestinal partial obstruction. Furthermore, the patient suffered of left hemiparesis with difficulty speaking because of a subarachnoid hemorrhage at one year of age. She was implanted a neurostimulator in the third sacral nerve root, but the device was removed two years later due to its inefficacy. In 2010, she was hospitalized again after another partial obstruction, and loop ileostomy was performed. Despite this, the symptomatology did not improve, and the obstructive episodes continued. Colonic manometry and abdominal X-ray revealed a picture of inertia coli.
On November 2015, during the programmed closing operation of ileostomy, the ileum appeared distended with brownish serosa. Therefore, a decision was taken not to close the ileostomy, and a diagnostic surgical biopsy of the ileum was made. Histologic examination showed an abnormal accumulation of eosinophilic granules in the cytoplasm of smooth muscle cells with disruption of muscular fibers (). The mucosa was normal. The pigment was interpreted as lipofuscin, and a suspicion of BBS was raised.
Blood levels of vitamins A, D, E, and K were dosed, and vitamins D and E were found to be low (0.3 mg/dl and 6 ng/ml, respectively). Antibodies against transglutaminase were negative, and there was no clinical or laboratory suspicion of coeliac disease.
After 8 months of nutritional supplementation, the vitamin values were at the lower limit of the normal range (0.8 mg/dl and 10 ng/ml, respectively), but the patient still suffered of recurrent intestinal functional obstruction. Abdominal X-ray and CT evidenced severe intestinal dilatation (), indicating the persistence of a severe impairment of colonic motility. Surgery appeared to be the best choice, and after multidisciplinary discussion, on April 2017, the patient underwent subtotal colectomy with maintenance of the rectum as a reservoir. The histological examination confirmed the diagnosis of BBS. The postoperative period was uneventful.
19 months after surgery, the patient is still under vitamin nutritional supplementation, and blood levels of vitamins D and E are still at the lowest acceptable limit, but since then, she did not have any other obstructive episode and has normal daily evacuation without the use of laxatives.


A 30-year-old male with no comorbidities presented to the emergency room in February 2018 with complaints of headache, fatigue, dry cough, and abdominal pain that started three days prior to admission. Apart from occasional alcohol consumption, his past medical history was unremarkable with no history of surgery or trauma.
On presentation, vitals were within normal limits with the exception of a temperature of 102 °F and his physical exam results were as follows: he appeared to be in moderate distress. Skin was jaundice; his abdominal exam was notable for diffuse abdominal tenderness with hepatosplenomegaly. Lower extremities revealed traced edema. Initial laboratory studies revealed a white blood count of 4.4 K/uL with lymphocytic predominance, hemoglobin of 16 g/dL, platelets 150 K/uL, aspartate aminotransferase (AST) 116 U/L, alanine aminotransferase (ALT) 119 U/L, and creatinine level of 1.1 mg/dL. Hepatitis and human immunodeficiency virus (HIV) panels were negative. Herpes simplex virus (HSV) and cytomegalovirus (CMV) were negative. A presumptive diagnosis of infectious mononucleosis was made and confirmed by serological and polymerase chain reaction (PCR). The EBV viral capsid antigen IgM antibody was >160 (normal <0.9); viral capsid antigen IgG antibody was negative, EBV early antigen IgG was 1.54 (normal <0.9), and the EBV nuclear antigen IgG was negative.
On day three of admission, acute worsening of abdominal pain with shortness of breath complicated the hospital course. Repeat labs were white blood corpuscle (WBC) count of 17.1 K/uL with lymphocytic predominance, hemoglobin 7.8 g/dL, platelets 667 K/uL, AST 332 U/L, and ALT 146 U/L. A computed tomography (CT) was performed, which revealed a wedge infarct of the spleen (Figure ). APAs were sent at that time and were positive. He was transferred to the intensive care unit and started on bilevel positive airway pressure (BiPAP) and continuous renal replacement therapy (CRRT) due to anuria. His clinical status improved with supportive therapy; a repeat CT scan showed improvement of splenic infarcts and he was discharged 27 days later. Repeat APA testing six weeks later was negative.


A 9-month-old Caucasian girl presented to our pediatric unit with fever, pallor, bilateral non-secreting conjunctivitis, and rash. Anamnestic records revealed that 12 days before she had remittent fever, which spontaneously resolved in 5 days. Fever started again after 3 days, associated with pharyngitis, and, later, with cervical adenopathy, diarrhea, and vomiting. She was treated with amoxicillin plus clavulanic acid and steroids, without defervescence. At admission, 9 days after fever onset, she showed fever, conjunctivitis, pharyngitis, generalized rash, and bilateral cervical adenopathy. Hematological parameters revealed: leukocytes, 18,000/mm3 with neutrophils of 8520/mm3, lymphocytes of 6250/mm3, and monocytes of 1930/mm3; hemoglobin, 9.1 g/dl; platelets, 318,000/mm3; and transaminases, albumin, natremia, and urine analysis in the normal range. Her C-reactive protein (CRP) was 2.31 mg/dl; her erythrocyte sedimentation rate (ESR) was 120.
An electrocardiogram (ECG) and echocardiography were normal, including coronary Z-scores. IgM and IgG against Epstein–Barr virus, cytomegalovirus, and parvovirus were tested, and she showed positive IgM against parvovirus. This was confirmed at further testing after 10 days. She was treated with clarithromycin and obtained quick defervescence. A diagnosis of parvovirus infection with severe anemia was made. For this reason and because of the prompt defervescence, it was exclusively treated as a viral infection. During follow-up, further cardiologic evaluation was done because of the risk of pericarditis secondary to the parvovirus infection, and at day 26 after fever onset, CAL were documented, with: a proximal right coronary artery Z-score of 6.02; left main coronary Z-score of 5.72; and left anterior descending Z-score of 5.78. Coronary artery Z-scores are commonly used for decisions in KD management and decisions on treatment, even if Z-scores show variations based on the Z-scores formula used for larger coronary artery dimensions []. However, the Z-score value for CAL is useful for the follow-up of a patient and for testing the response to treatment.
The child was promptly treated with IVIG at the dosage of 2 g/kg plus acetylsalicylic acid (ASA) at the dosage of 5 mg/kg per day. Despite treatment, a further echocardiographic evaluation showed worsening of CAL: proximal right coronary artery Z-score of 5.93; left main coronary Z-score of 5.63; and left anterior descending Z-score of 5.39, which showed a saccular aneurysm of 2.9 mm of diameter (Z-score of 5.08).
A laboratory test did not show inflammation, but the girl was treated with three bolus doses of intravenously administered methylprednisolone at 30 mg/kg per dose. The Z-score of CAL did not change. Informed consent by parents was obtained, and our patient was treated with anakinra at the dosage of 4 mg/kg per day. She showed a progressive improvement of CAL and after 25 days of anti-IL-1 treatment, her proximal right coronary artery Z-score was 0.93, left main coronary Z-score 4.02, and left anterior descending Z-score 2.93. Treatment was continued for 2 months, at which point Z-scores normalized (see Table ).
The first diagnosis in this patient was a parvovirus-related infection, which explained the clinical manifestations and the clinical course of the disease. The diagnosis of KD was reached late; a control echocardiogram showed CAL. Mild CAL are described in systemic juvenile arthritis, in febrile diseases, and in infectious diseases such as Mediterranean spotted fever []. Aneurysms, conversely, are typical of KD.
IVIG did not arrest the worsening of CAL, which were stabilized after three doses of methylprednisolone. However, our patient received IVIG 26 days after the fever started and this delay can explain the poor response. In fact, a delay in IVIG infusion is recognized as a risk factor for non-response to first-line treatment, as demonstrated in our population [, ].
A 7-year-old Caucasian boy, the brother of Case number 1, presented fever (38 °C) and vomiting at the same time as his sister, which spontaneously resolved after 4 days. Four days later, he again had fever, abdominal pain, tachycardia, and tachypnea. He was admitted to our cardiologic unit. He showed pallor, tachypnea, stasis at the pulmonary bases, tachycardia (180 beats/minute), gallop rhythm, hypotension, and secondary anuria hepatomegaly with pain at palpation. Hematological tests evidenced: leukocytes of 24,680/mm3 with neutrophils of 19,744/mm3, lymphocytes of 3430/mm3, and monocytes of 960/mm3; hemoglobin, 10.4 g/dl; platelets, 632,000/mm3; CRP, 0.24 mg/dl; aspartate aminotransferase (AST), alanine aminotransferase (ALT), and gamma-glutamyltransferase (gamma-GT) in the normal range; creatine phosphokinase (CPK), 773 mg/dl; creatinine, 0.77 mg/dl; and blood urea nitrogen (BUN), 111 mg/dl. He had elevated myocardial necrotic enzymes (c-troponin T, 91.4 ng/l) and pro-brain natriuretic peptide (BNP) > 70,000.
An echocardiogram revealed that the left ventricle had a normal diameter (telediastolic diameter, 40 mm; Z-score, 0.37) and generalized hypokinesia, with a severe reduction of the ejection fraction (EF) (20–25%); the left atrium was dilated (diameter, 35 mm; Z-score, 3.3) and the mitral valve had a moderate insufficiency. The right ventricle had normal dimension; the tricuspid valve showed a moderate insufficiency. His suprahepatic veins were dilated. No pulmonary hypertension was documented. He received dopamine (5 gamma/kg per minute), dobutamine (7 gamma/kg per minute), furosemide (1 mg/kg) plus steroids (2 mg/kg). Clinical signs, echocardiographic parameters, and plasmatic enzymes showed a progressive but slow improvement. Sixteen days later, his EF was 45%; however, a persistent septal hypokinesia was documented. He continued to receive treatment with furosemide and enalapril. Specific serological tests were performed to exclude Epstein–Barr virus (for the skin rash, associated with fever and hepatic cholangitis) [] and coxsackie virus infection (for fever and myocarditis) []. A nasal swab for influenza and parainfluenza virus was negative. However, the index case of the sister suggested we should run a serology test for anti-parvovirus, and we found increased IgM anti-parvovirus with low IgG.
A cardiologic follow-up revealed: a further EF improvement (50%); left ventricle was 38 mm (normal value: 32.7–45.5); Z-score, 0.15. His right atrium and ventricle were in the normal range for diameters and kinesis.


Our patient is a 58-year-old African American woman who initially presented to an outside facility of difficulty swallowing, neck pain, fatigue, and shortness of breath for 3 months. She had bilateral neck swelling, greater on the left side, that started about a year prior to her presentation. This patient also had a 4-day history of productive cough and nasal drainage. She had a 30-pack year history and consumed approximately 12 beers daily for years.
She reported a history of CLL and having been treated with chemotherapy, but she was unsure of the regimen. Examination findings and computed tomography (CT) scan of neck at the outside facility were concerning for impending airway compromise, and she was transferred to our hospital to be evaluated by an otorhinolaryngology or oral and maxillofacial surgery service.
During her evaluation in our emergency department, she was noted to have a muffled voice with mild gurgling of secretions with speech. Large, fixed cervical and submandibular lymph node, and a large left neck mass were present. Enlarged axillary lymph nodes were palpated bilaterally. The uvula and posterior oropharynx were not visible due to her tongue and left neck mass. White blood cell (WBC) count was 83 600/mm3. She was started on dexamethasone to reduce the compression of the neck mass on the airway. The oral and maxillofacial surgery service team evaluated her and determined that a definitive airway was not indicated. She was admitted to the medical intensive care unit for close airway monitoring.
The oncology team became involved at this time. Her CLL was the suspected etiology of the neck mass and lymphadenopathy, and intravenous fluids and allopurinol were started to prevent tumor lysis syndrome. Further laboratory workup showed the predominantly lymphocytic, elevated WBC count as before and a normocytic anemia, hemoglobin 10.6 g/dL. Smudge cells were present on the peripheral blood smear. Iron studies, B12, and folate were normal. Flow cytometry was pending ( and ).
CT chest showed lymphadenopathy of the mediastinum, right hilum, bilateral axilla, submental, and supraclavicular lymph nodes. Consolidative and ground glass opacities were also present in the bilateral lung fields ().
Partially confluent retroperitoneal, mesenteric, pelvic, and inguinal lymphadenopathy were noted on CT abdomen and pelvis. These findings were consistent with known CLL.
In addition to CLL, diagnoses considered included Richter transformation. We proceeded to obtain biopsies of the left neck mass.
Around this time outside records were received. Our patient had been treated with rituximab and chlorambucil 5 months prior after presenting with similar symptoms. She had been incidentally found with a WBC count of 40 000/mm3 several years before. She was lost to follow-up after both occasions. We started her on rituximab for treating CLL with possible Richter transformation. The tentative plan was to treat with fludarabine, cyclophosphamide, rituximab (FCR) if CLL or rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone (R-CHOP) if Richter transformation had occurred.
Flow cytometry showed a CD5+ mature B-cell lymphoma consistent with CLL, 13q deletion, and mild CD38 positivity. Over the next few days, she had improvement in her neck pain. The mass and lymph nodes decreased in size. The WBC count decreased to 40 000/mm3. Biopsy of the neck mass showed squamous cell carcinoma (SCC) with extensive necrosis involving lymphoid tissue.
With evidence of a second malignancy with possible significant oropharyngeal involvement, direct laryngoscopy with biopsy was pursued ( and ).
Direct laryngoscopy was performed and showed a large mass involving the right tonsil extending to midline of the posterior oropharynx. Biopsies were obtained. Posterior oropharynx, right tonsil, and right nasopharynx biopsy contained invasive keratinizing SCC, moderately differentiated, with basaloid features. The nasopharyngeal biopsy also included atypical lymphoid infiltrate consistent with involvement by CLL.
This patient’s case was discussed at the tumor board. Given the widespread involvement of her disease, she was diagnosed with Stage IV oropharyngeal SCC. She was not at candidate for resection and was to be treated with high-dose cisplatin (25 mg/m2) weekly for 6 cycles and radiotherapy. Treatment was to be delayed for her Rai Stage III CLL out of priority since her airway compromise was attributed to the SCC and the CLL component had been partially treated with rituximab.
At time of discharge, she had continued improvement of her symptoms. The plan was for her to follow with our medical oncology and radiation oncology teams as an outpatient. However, due to long distance from home, she established care at an outside facility.


A 51-year-old woman who presented with diarrhoea containing mucus and blood had initially been diagnosed with acute severe ulcerative pan-colitis and backwash ileitis at the age of 49 years at Peking Union Medical College Hospital (PUMCH) in December 2014. She had poliomyelitis when she was very young, and there was nothing special regarding her family or psychosocial history. Serology was positive for perinuclear antineutrophil cytoplasmic antibody (pANCA) and negative for anti-Saccharomyces cerevisiae antibody (ASCA). Her condition was refractory to steroids and complicated by Cytomegalovirus (CMV) infection. Ultimately, she underwent sub-total colectomy and ileostomy in February 2015. Pathological examination of the resection specimen showed diffuse pan-colitis consistent with UC and no indications of Crohn’s disease (Fig. ). She did well in the following 11 months; prednisone was tapered and stopped within 2 months, and she gained 5 kg of weight after the ileostomy. In January 2016, a scheduled restorative ileal pouch-anal anastomosis (IPAA) with proximal neo-ileostomy was performed.
From one month after the IPAA, her 24-h stool collection slowly increased to 1.5–2 L. Next, she noticed decreased urine output since April. In early May 2016, she presented to our emergency room with repeated unconsciousness over the course of 10 days. Her vital signs were as follows: blood pressure (BP), 74/50 mmHg; heart rate (HR), 90 bpm; additionally, she exhibited a poor nutritional status (160 cm; 39 kg). On physical examination, active bowel sounds were noticed to occur approximately 7–9 times per minute. Her serum creatinine level was 183 μmol/L, indicating acute kidney injury. Treatment with fluid replacement and noradrenaline maintained her BP at 80–90/50–60 mmHg and gradually normalized her creatinine level. However, her 24-h watery stool collection persisted, and she developed fever and vomiting. While many leukocytes were found in stool collected from the diverted ileostomy, repeated stool cultures and tests for Clostridium difficile toxins were negative. Tests for CMV-DNA, CMV-pp65 and EBV-DNA were performed and were all negative. The patient was not on any medications, including NSAIDs, upon verification. Her treatment with steroids was stopped before the end of April 2015. Empirical treatment with antibiotics, including ceftazidime, metronidazole and oral vancomycin, was administered with no response. Due to her reliance on noradrenaline, relative adrenal insufficiency was suspected, and hydrocortisone was initiated at 50 mg q6 h intravenously. Her stool volume decreased to less than 500 ml per day quickly, by which time the treatment with noradrenaline was successfully stopped. The levels of D-lactate, endotoxin and diamine oxidase indicated that the barrier function of the intestine was compromised and that bacterial translocation may have occurred. Oedematous inflamed mucosa with patchy superficial ulcers was observed in the diverted pouch by pouchoscopy. Although an upper endoscopy and an endoscopy through a stoma revealed a normal gross appearance in the stomach, duodenum and pre-stomal ileum (Fig. a, b), the histological examination of tissue biopsies of both the duodenum and pre-stomal ileum revealed enteritis, as indicated by moderate villous atrophy, cryptitis, decreased goblet cells, and severe active inflammation with neutrophil infiltration in the lamina propria, as well as negativity for intraepithelial lymphocytosis (Fig. a-d). From these lines of evidence of histological enteritis presenting in the duodenum, pre-stomal ileum and diverted pouch, we considered pan-enteritis to be present, and we diagnosed the patient with post-colectomy enteritis. The patient was treated with methylprednisolone at 30 mg intravenously once a day with tapering by 5 mg every 7 to 10 days; however, her stool volume from the ileostomy still gradually increased to 3–4 L. After a multidisciplinary team discussion, ileostomy closure was debated as the final rescue treatment and was performed in August 2016. Two months later, her stool volume decreased to less than 1 L per day, and she gained 2.5 kg of weight. Azathioprine at 50 mg/d was prescribed during the tapering of prednisone. Until the last follow-up in March 2018, she performed well, with an increase in body weight to 50 kg, and daily defecation approximately 5–6 times at less than 1 L/day, sometimes with form (Additional file ). Gastroduodenal endoscopy and pouchoscopy were repeated annually and showed normal villi in the descending duodenum and neo-ileum in March 2018 (Fig. c, d). Gradually, the histology changed, showing recovery of the villous atrophy, cryptitis and inflammation in the lamina propria to normal (Fig. e-f).


A 70-year-old male was admitted to our hospital for “abdominal pain, abdominal distension for 1 month, and no exhaustion or defecation for 4 days” as the chief complaint on April 10, 2017. He had no fever, nausea or vomiting.
The physical examination revealed abdominal distension (Fig. a), full abdominal tenderness and weak bowel sounds (1 beat/min). The following laboratory data were observed: WBC: 9.02 × 109/L, NET%: 78.90%, and CEA: > 60.00 μg/L. No obvious electrolyte, coagulation or liver biochemistry abnormalities were noted.
A CT scan of the abdomen revealed peritoneal effusion and bowel dilatation (Fig. b). The admitting diagnoses that were investigated were acute intestinal obstruction and abdominal effusion. On the first day, a transabdominal ultrasound-guided biopsy was performed, and a characteristic yellow jelly-like mucus containing microscopic mesothelial cells, fibrous tissue and lymphocytes with mild atypia was extracted (Fig. a-c). Therefore, PMP was suspected.
Operation: Because the patient complained of increasing abdominal distension and his abdominal pressure reached 35 mmHg, he underwent an emergency exploratory laparotomy. A significant amount of yellow, jelly-like mucus (approximately 5000 mL) was found during the operation (Fig. a). Numerous metastases were noted on the omentum and mesenteric root. After removing the mucus, we identified a hard mass measuring 10 cm × 15 cm with an unclear boundary and an abundant blood supply on the ileocecal junction (Fig. c). After carefully separating the appendix, the gangrenous rupture of the ileocecal tumour was observed, and the appendiceal lumen was interlinked with the abdomen. The patient’s small intestine and colon were expanded, but the colon’s expansion was more obvious, corresponding to low intestinal obstruction (Fig. b). Considering that explanations other than paralytic intestinal obstruction caused by the significant accumulation of intraperitoneal mucus might be plausible, we further explored the pelvic cavity. A hard mass measuring 4 × 5 cm with an unclear boundary infiltrating the rectal muscle layer was identified in the upper rectum (Fig. d). The peritoneal cancer index (PCI) was estimated intraoperatively, and the aggregative score of 13 abdominopelvic regions reached 20. We performed cytoreductive surgery (CRS), enterolysis, intestinal decompression and special tumour treatment to remove the lesions and relieve the obstruction as much as possible. Although some residual cancer remained, there was no nodule larger than 2.5 mm in diameter. Thus, we performed CC1 cytoreduction on the patient. Radical resection of the rectal carcinoma was also performed because the patient had PMP accompanied by rectal cancer.
The postoperative course was uneventful. The patient was discharged on postoperative day 15.
The postoperative histological pathologic diagnoses were appendiceal mucinous neoplasm, rectal cancer and PMP. The rectal cancer was a medium differentiated adenocarcinoma, approximately 50% of which was a mucinous adenocarcinoma. Serosa invasion, intestinal ulcerations and perineural invasion were noted, but vascular invasion was not observed (Fig. a). In the appendiceal mass, a crowded glandular epithelium with mild nuclear abnormalities, including the pseudo-layer arrangement, was noted. The tumour was LAMN (Fig. b). Moreover, numerous cavities containing mucus were observed in the fibrous tissue (Fig. c). The immunohistochemical staining of the rectal tumour revealed the following: PTEN (++), ERRCC1 (++), VEGF (++), TS (−), EGFR (++), HER2 (0), PMS2 (+), MLH1 (++), MSH2 (+++), MSH6 (+++), and MGMT (+) (Fig. d).
Hyperthermic intraperitoneal chemotherapy (HIPEC) was not performed during the surgery because of disagreement among the patient’s family members. We strongly recommended that the patient receives chemotherapy or radiotherapy after surgery. However, to date, the patient did not receive these treatments due to economic difficulties. At the 1-year follow-up visit, no tumour recurrence was discovered by CT.


A 40-year-old woman consulted us with multiple café-au-lait spots, family history of neurofibromatosis, and prior diagnosis of NF1 by her primary doctor. Her chief complaints were numbness of the upper limb and gait disturbance from 1 month prior. Neurological examination revealed a spastic gait. The Romberg test was positive. The one leg standing test showed instability in both legs. Hyperreflexia showed a deep tendon of the biceps, triceps, patella, and Achilles on both sides. In the manual muscle test, only finger extension was reduced to 4 on the left hand. The sense of pain was reduced on the right side of her body.
Radiography showed expansion of the atlanto-dental interval at the neutral position of the cervical spine (Fig. a), while canal stenosis was observed by computed tomography and magnetic resonance imaging (Fig. b, c, e). An abnormality of the left side vertebral artery inside of the C1 lamina was observed by computed tomographic angiography (Fig. d). Magnetic resonance imaging showed dural ectasia from C2 to T2, and AAD. There was no neurofibroma between the atlas and the odontoid (Fig. e, f).
We performed surgery to prevent the progression of myelopathy caused by AAD. We initially planned a long posterior fixation. However, we achieved a good closed reduction of the AAD under general anesthesia. Thus, we tied an ultra-high molecular weight polyethylene cable (Nesplon; Alfresa, Inc., Osaka, Japan) to the C1 lamina and spinous process of C2 to maintain the position of the reduced AAD. Furthermore, we tied two nesplon cables® to the sublamina of C1 and C2 according to the Brooks technique. The iliac bone was grafted on between the C1 and C2 laminae (Fig. ). The operative time was 1 h 35 min, and bleeding was < 50 ml. After the operation, the patient showed improvement of neurological symptoms. She wore a Philadelphia brace continuously. However, at 5 months after surgery she felt neck pain and consulted us again. Computed tomography showed fracture of the C1 lamina and recurrence of AAD (Fig. ).
We reoperated using a long posterior fusion. Because of the existing abnormal vertebral artery inside of the C1 lamina, we gently removed scar tissue using a subperiosteal approach, and revealed the C1/2 facet under direct vision. We then introduced two intra-articular titanium spacers (KiSCO, Kobe, Japan) for fixed bilateral atlanto-axial joints.
Vertex select® (Medtronic, Minneapolis, MN, USA), a plating system for occipital bone, was used for posterior fixation of the occipital bone and cervical spine. Facet screw fixations were inserted on the right side of C2/3 and both sides of C3/4. Lateral mass screw fixation was performed on both sides of C4. Two pre-bending rods were connected to these screws on both sides of the cervical spine, and two rod couplers were connected to the pre-bending rods at the height of C2 and C4. To avoid stress concentration and refractures, sublaminar taping was performed at C2, C3, and C4 using nesplon cables®. Finally, her right side iliac bone was grafted between the occipital bone and the back of the C1 lamina using the Newman technique (Fig. a, b). The operative time was 3 h 56 min, and bleeding was 425 ml. She wore a Philadelphia brace for 1 year after the second operation. At 4-year follow up, there was no AAD recurrence (Fig. c, d) and her neck pain had improved. She could walk independently, and a manual muscle test showed ‘normal’ for every muscle.


A 49-year-old Hispanic female with no significant past medical history presented to the emergency department with progressive dysphagia to liquids and solids and 40-pound unintentional weight loss over the last eight months. She denied a history of alcohol abuse, herbals, supplements or environmental exposures. Upon presentation, blood pressure was 99/57 mmHg and the pulse rate was 122/minute. On examination, she was cachectic, had 4 to 4+ power in all extremities, bilateral wrist swelling, bi-basilar crackles, and bilateral pedal edema. Her body mass index (BMI) was 22; her BMI one year ago was 30. Liver enzymes, a year prior to the presentation, were normal.
Labs were significant for blood urea nitrogen 7 mg/dL, creatinine 0.3 mg/dL, albumin 1.6 g/dL, total bilirubin 1.2 mg/dL, direct bilirubin 0.9 mg/dL, alkaline phosphatase 722 units/L, gamma-glutamyl transferase 958 units/L, aspartate aminotransferase 325 units/L, alanine aminotransferase 82 units/L, hemoglobin 10.3 g/dL, ferritin 2468 ng/mL, transferrin saturation 85%. Her creatine kinase (CK) was 55 units/L (normal range 0-163), aldolase 10.4 units/L (normal range < 8.1) and C-reactive protein was 1.71 mg/dL (normal range < 0.6). Antinuclear antibody (ANA), anti-Jo-1, and anti-topoisomerase I antibody were negative. There were no documented liver function tests prior to presentation. Computed tomography (CT) of the chest, abdomen, and pelvis revealed ground glass opacities involving bilateral lung apices and dependent portions of the lower lobes, consistent with aspiration pneumonia, and hepatomegaly with hepatic steatosis (Figures -). Anti-mitochondrial antibody assay, HFE gene mutation analysis, ceruloplasmin, viral hepatitis panel, alpha-1 antitrypsin level and anti-smooth muscle antibody assay were sent to evaluate the elevated liver enzymes and were negative. Magnetic resonance cholangiopancreatography (MRCP) did not reveal any biliary pathology. A bedside swallow evaluation revealed oropharyngeal dysphagia and X-ray of the hands revealed juxta-articular osteopenia (Figure ).
An ultrasound-guided liver biopsy revealed severe diffuse macrovesicular hepatic steatohepatitis involving 80%-90% of the liver parenchyma, mild intracanalicular cholestasis, prominent Mallory-Denk bodies within ballooning hepatocytes and bridging fibrosis on trichrome stain (Figures -). Neurologic electrophysiology studies showed normal nerve conduction, fibrillation and positive waves in all muscles and low amplitude motor unit action potentials in the majority of the muscles studied, particularly in the proximal muscles. In summary, it showed electrophysiologic evidence of a diffuse myopathy with features of muscle membrane irritability. A subsequent muscle biopsy revealed atrophic fibers with a perimysial distribution (as seen in Figure ), increased immunohistochemistry (IHC) labeling for major histocompatibility complex (MHC) 1 (as seen in Figure ), and capillary complement staining (as seen in Figure ), suggesting an autoimmune myositis. There were no features to suggest autoimmune hepatitis. Near the end of her hospitalization, she developed confusion, progressive hypoxia, and succumbed to multi-organ dysfunction.


A 70-year-old man was admitted to our hospital with a two-week history of diplopia and right ptosis. He had a history of hypertension and dyslipidemia. Five months before admission, he had been diagnosed with SLE, according to the 1997 American College of Rheumatology classification criteria []. Because his arthritis and bicytopenia were mild, treatment with glucocorticoid had been withheld.
Physical examinations revealed ptosis of the right eye. The position of the right eye was laterally deviated. The adduction and upward and downward movements were reduced. Other significant features were mild drooping of the right angle of the mouth, incomplete closure of his right eye, and asymmetrical crease of the forehead. His hearing was normal, and there was no rash on the ear. Consciousness was clear, and the muscle strength of the extremities was normal.
Laboratory findings were as follows: white blood cell count, 2,200/μL (neutrophils 900/μL and lymphocytes 600/μL); red blood cell count, 373 × 104/μL; hemoglobin, 10.9 g/dL; platelet count, 9.5 × 104/μL; and activated partial thromboplastin time, 32.4 sec. The blood glucose level was 99 mg/dL and HbA1c was 6.4%. C-reactive protein was 0.69 mg/dL, serum IgG was 4,450 mg/dL, and complement components C3 and C4 were 25 and 1 mg/dL, respectively. Serologic markers for hepatitis B and C were negative. Cryoglobulin was not detected. Rheumatoid factor was 18.1 IU/mL (normal <15), and antinuclear antibody was 1 : 2,560 with a homogeneous pattern. Anti-dsDNA antibody was 311.7 U/mL (normal <12), and anti-SS-A antibody was positive at a titer of 1 : 4. Anti-cardiolipin antibody (IgG) was positive at 23.8 U/mL (normal <10). Anti-U1-RNP, anti-Sm, anti-SS-B, anti-cardiolipin (IgM), and anti-β2 glycoprotein I antibodies and lupus anticoagulant were all negative. Urinalysis was normal. Cerebrospinal fluid showed no elevation of cell count or protein. Gadolinium-enhanced magnetic resonance imaging of the brain revealed no increased signal intensity on diffusion-weighted images, and there was no abnormal enhancement of cranial nerves or soft tissue mass around the nerves.
The patient had also noticed a dry mouth. A chewing gum test and salivary gland scintigraphy revealed decreased salivary secretion. Schirmer's test was only positive in his left eye. A labial minor salivary gland biopsy demonstrated focal lymphocytic infiltrations, which was consistent with the finding of SS. The patient was also diagnosed with SS according to the Japanese diagnostic criteria [].
Because the patient was elderly, and had a history of hypertension and dyslipidemia, we first considered several common causes of cranial neuropathies including stroke, diabetes, and infections like varicella-zoster virus. However, these conditions did not explain the cranial nerve palsies. Hence, we considered that the cranial nerve palsies were caused by autoimmune mechanisms. The patient was treated with prednisolone 70 mg/day (1 mg/kg), aspirin, and intravenous cyclophosphamide pulse therapy. Thereafter, both the oculomotor and the facial nerve palsies began to improve in a few days. Both palsies recovered almost completely after two weeks of treatment.


On 15 Aug, 2017, a 57-year-old woman with ulcerative colitis, steroid-induced diabetes mellitus, deep vein thrombosis in the lower part of the left thigh, ecchymotic skin lesions, swelling in the left nose, ptosis (i.e., inability to move eyelids), and facial nerve palsy, was admitted to Shafa Hospital in Sari, north of Iran. She was afflicted with intestinal bleeding after Islamic fasting periods (i.e., Ramadan month).
Ulcerative colitis had been confirmed by clinical manifestations and colon biopsy and was managed with the administration of azathioprine (50 mg/day) and high-dose prednisolone (60 mg/day) for 2 months, prior to hospital admission. Due to prednisolone-induced hyperglycemia, the patient was on treatment with insulin for a month before admission to the hospital. She was also on anticoagulant drugs for the treatment of deep vein thrombosis.
One day after hospitalization, the patient developed progressive periorbital ecchymosis, extensive edema of the nasal area, and nasal ulcer (). The eye examination revealed proptosis with 4+ light reaction, evidence of afferent pupillary defect (i.e., Marcus Gunn pupil), no light perception, absolute blindness, ophthalmoplegia, and neurological defects of the cranial nerves 2, 3, 4, and 6.
The results of the laboratory tests and vital signs included a fast blood sugar of 302 mg/dL, white blood cell count of 9460/µl, red blood cell count of 3.53×106/µl, platelets of 60×103/µl, hemoglobin of 8.9 g/dl, blood urea nitrogen of 35 mg/dL, serum creatinine of 2.3 mg/dl, potassium of 2.1 mg/dL, body temperature of 38°C, blood pressure of 120/70 mm/Hg, pulse rate of 80/min, and respiratory rate of 14/min, and glomerular filtration rate of 32. Blood culture and urine culture tests were negative.
On August 18, with regard to the deterioration of the patient's clinical condition and given the high level of suspicion for mucormycosis, the patient was prescribed amphotericin B deoxycholate with a dosage adjustment (0.7 mg/kg/day; 50 mg/day total dosage) coupled with broad-spectrum antibiotics (piperacillin-tazobactam). She was immediately subjected to surgery in which the nasal necrotic tissues were completely removed. The debrided tissues and mucous membranes were immediately sent to the Medical Mycology Laboratory.
The direct examination of the necrotic tissue revealed broad aseptate hyphae with right angle branching compatible to Mucorales (). The cultures grew gray-brown colonies with cottony texture in which Rhizopus species was identified by means of microscopic and macroscopic characteristics. DNA was extracted from the tissue of fresh nasal samples with QIAamp DNA Mini Kit (Qiagen, Hilden, Germany) in accordance with the manufacturer’s instruction.
The diagnosis was confirmed by polymerase chain reaction (PCR) assay using two universal primers, namely ITS1 and ITS4, to amplify the internal transcribed spacer 1 (ITS1) and ITS2 regions and the 5.8S ribosomal DNA (rDNA) region of the fungi as described previously [9]. The amplicons were sequenced and compared with the GenBank database using the Basic Local Alignment Search Tool, and comparative analysis revealed Rhizopus oryzae. The obtained sequences were submitted to GenBank and received the accession number MG946811.
The brain magnetic resonance imaging scan revealed the involvement of the maxillary sinus, ethmoid bone, and left sphenoid sinus with no mass effect. The brain computed tomography scan also demonstrated the involvement of the left maxillary, ethmoid sinuses, and nasopharynx, as well as the destruction of the bone of the medial wall of the left maxillary and ethmoid sinuses ().
Given the rapid progression of the disease and the spread of infection to the eyes and brain, the eye removal was recommended. However, the patient’s family refused the enucleation. Consequently, the infection progressed to the eye, and the patient lost her vision. At the same time, severe oral ulcers appeared. Patient’s respiratory and hemodynamic conditions were stable. The dosage of amphotericin B deoxycolate (75 mg/day) increased, and broad-spectrum antibiotics were continued. Eventually, despite antifungal treatment and special care, the patient expired in September 6 due to the rapid progression of the infection to the brain as a result of the refusal of the patient's family regarding enucleation surgery.
The study protocol was approved by the Ethics Committee of Mazandaran University of Medical Sciences, Sari, Iran (IR.MAZUMS.REC.95.1560). Informed consent was obtained from the patient’s next of kin for each procedure.


A 69-year-old female was referred to our visual electrophysiology clinic. The referring optometrist was concerned that the nature of her visual field defects, specifically a bilateral altitudinal hemianopsia with macular involvement, suggested a malingering disorder (Figures -). The visual field changes were first noted 10 years prior, and magnetic resonance imaging (MRI) at the time revealed a pituitary microadenoma not affecting the optic chiasm. Yearly MRI examinations of the brain along with her visual fields remained stable without evidence of growth of the pituitary lesion. Her medical history was also significant for hyperlipidemia, hypertension, mitral valve prolapse, hypothyroidism treated with levothyroxine, and a 20-year history of migraine headaches without visual aura, treated with botulinum toxin injections and oral eletriptan hydrobromide. Her family history was notable for migraines in her mother and sister, the latter also having had a ruptured cerebral aneurysm.
Her physical examination was unremarkable. Ophthalmic examination revealed visual acuity of 20/20 in each eye. The anterior segments were normal. The cup to disc ratios were 0.3 with healthy appearing nerves bilaterally. Dilated funduscopic examination was normal in both eyes except for lattice degeneration in the inferior periphery of the right eye. Confrontational visual fields were absent superior to the midline. Optical coherence tomography testing of the optic nerve head and macula were normal. Color vision testing was normal in both eyes. A mfVEP was performed and confirmed bilateral superior altitudinal visual deficits with macular involvement (Figure ). Repeated MRI and magnetic resonance angiogram (MRA) testing of the brain and orbit revealed a hypoenhancing lesion of the right side of the sella turcica, consistent with a pituitary microadenoma, measuring 7.8 x 3.6 mm. This was slightly smaller than the previous examination one year before.


A 35-year-old man with a medical history of splenectomy due to splenic artery rupture presented to the hospital with diffuse abdominal pain of one-day duration associated with nausea and two episodes of non-bilious, non-bloody emesis. Patient denied any significant aggravating or relieving factors of the pain, no association with food intake or recent antibiotic exposure, and no fever, chills, rigors or diarrhea. He was not taking proton pump inhibitor. On examination, the patient was afebrile, tachycardic with a blood pressure of 85/61 mm Hg. Abdominal examination revealed diffuse mild tenderness without guarding or rigidity and bowel sounds were present. Lab work up was pertinent for leucocyte count of 32 x 109 cells/L (normal range: 3.7-11 x 109 cells/L) with predominant neutrophils 87% and elevated serum lactate 4 mmol/L (normal range: 0.5-1.0 mmol/L) with no end-organ damage. Urine, blood cultures, and chest X-ray did not reveal any source of infection. Computed tomography (CT) of the abdomen and pelvis with intravenous (IV) contrast showed mild-moderate prominence of adjacent proximal and mid jejunum without bowel obstruction or evidence of colitis, most likely representing enteritis (Figure ). The patient was started on broad-spectrum antibiotics with IV pipercillin-tazobactam and vancomycin due to concern for severe sepsis of unclear etiology. As patient signs and symptoms did not improve with broad-spectrum antibiotics, stool studies were obtained and C.difficile was confirmed on stool polymerase chain reaction (PCR). The patient was started on oral vancomycin 125 mg every six hours and IV antibiotics were discontinued. The patient’s signs and symptoms improved after oral vancomycin, and he was discharged home to complete a 14-day course of oral vancomycin.


A 46-year-old never smoker Malagasy woman was referred by her family doctor to the emergency department due to dyspnoea with inspiratory stridor and inspiratory-expiratory wheezing with insidious onset over a 3-month period. The patient was treated for supposed asthma since 2 weeks without improvement. She had no fever, weight loss or night sweats. Physical examination revealed a heart rate of 96/min, a respiratory rate of 19/min, and an oxygen saturation of 89% on room air with normal chest auscultation. A non-tender mass was detected on the right side of her neck.
CT scans of the neck and thorax showed a large thyroid mass causing tracheal stenosis (Fig. a), and multiple cystic lesions with thin walls in both lungs (Fig. b). Cysts had a diffuse localisation, including the costophrenic recesses. Neither pulmonary nodules nor ground glass opacities were observed. Abdominal CT scan did not show any sign of renal angiomyolipoma.
Blood arterial gases showed mild hypoxemia (PaO2 82 mmHg). Laboratory investigations, including renal function, liver function tests, C-reactive protein, thyroid function tests, complete blood cell counts, and serum IgG4 levels were all within normal limits. HIV and immunological tests (anti-Ro/SSA and anti-La/SSB antibodies, rheumatoid factor and thyroid-stimulating hormone (TSH) receptor antibody) were negative. Plasma levels of vascular endothelial growth factor-D (VEGF-D) were low (347 pg/mL; normal range 0–450 pg/mL). Electrocardiogram (ECG) tracing and complete pulmonary function testing were normal (forced expiratory volume in 1 s - FEV1 94%, total lung capacity – TLC of 95% of predicted and normal carbon monoxide diffusing capacity - DLCO).
Surgical removal of the thyroid mass with subtotal thyroidectomy, tracheal segment (3.5 cm) resection and multiple adenectomies were performed (Fig. ). Microscopic study revealed extensive infiltration of all lymph nodes and the thyroid with partial replacement of follicular parenchyma by a fibro-inflammatory process of mixed cellularity, rich in histiocytes (Fig. a). The latter exhibited epithelioid to xanthomatous morphology and formed ill-defined clusters or confluent sheets without granulomatous pattern. An important fraction of the histiocytic population showed emperipolesis of neutrophil granulocytes and lymphocytes (Fig. b). Immunohistochemistry revealed co-expression of the macrophage-related epitope CD68 along with S100 protein, in the absence of CD1a, thereby identifying the histiocytic elements as of non-Langerhans lineage (Fig.a, b and c). Remarkably, storiforme fibrosis with signs of vasculitis was also observed and immuno-phenotyping of the infiltrate revealed a substantial participation of IgG4-bearing plasma cells. While their absolute density reached up to 50 per high power field, the IgG4:IgG ratio did not exceed 20% (Fig.d). Histological examination of the tissue biopsies for the identification of infectious organisms using several stainings for pathogens were negative. Based on these findings, a histological diagnosis of RDD was made.
Following surgery dyspnoea disappeared indicating that it was related to tracheal compression. The patient was asymptomatic in the absence of any specific treatment; clinical, functional lung testing and radiological follow up 4 years after surgery did not reveal any disease activity or progression underlining the excellent prognosis of the disease after resection.


A 24-year-old married Arab woman had been admitted to a local health center 2 months prior to referral to our urology department. She had been hospitalized there four times in 1 year for acute pyelonephritis. The fourth episode raised the suspicion for an underlying problem and justified her referral to our urology department after management of the acute pyelonephritis.
On admission, she complained of ascending left-sided flank pain during micturition but did not have dysuria or hematuria. She also had a history of frequent urinary tract infections (UTIs) as a young adult.
She was perfectly asymptomatic on the right side. A physical examination was normal. Her temperature was 37.4 °C, her blood pressure was 128/84 mmHg, and her pulse rate was regular at 76 beats per minute. Laboratory tests were normal; in particular, a urine examination showed no leukocyturia or bacteriuria.
She underwent an abdominal ultrasound which showed an asymmetric size of the kidneys and a bilateral chronic pyelonephritis aspect. Her right kidney measured 10 cm while the left measured 12 cm.
A voiding cystourethrography (VCUG) was performed and showed grade IV VUR on the left side and grade I VUR on the right (Figs. and ).
An abdominal and pelvic computed tomography (CT) scan detected a left completely duplicated collecting system with hydroureteronephrosis and poor opacification of the upper pole moiety. In addition, the parenchyma of the upper pole moiety was atrophied with secretory and excretory delay. In association with VCUG findings, it appeared that the refluxing ureter was the one that drains the upper pole moiety and inserts lower into the bladder. On the right, a duplex collecting system was detected with hypotonic calyces, pelvis, and ureter of the upper pole moiety. An atrophic parenchyma and poor opacification of the upper pole moiety was also detected (Figs. and ). Renal scintigraphy was not available.
We carried out a left heminephrectomy because of the poor functioning of the upper pole moiety based on imaging findings associated with recurrent UTIs (Fig. ). On the right side she underwent dextranomer/hyaluronic acid (Deflux®) injections. Dextranomer/hyaluronic acid (Deflux®) was injected submucosally below the ureteral orifice at the 6 o’clock position to create a prominent bulge and raise the distal ureter and ureteral orifice.
A year after the surgery she has no complaints. The symptoms are completely resolved. Biological and radiological follow-up is unremarkable. A timeline of the case is presented in Figure .


A 26-year-old female, period of gestation of 35 weeks and 6 days into her third pregnancy, presented with contraction pain which was increased in intensity and frequency. She denied any leaking liquor nor show. Fetal movements were well felt. She claimed that she had fever, headache, sore throat, and dry cough for one-day duration. She denied any travelling or any contact with live poultry. She also had no history of dysuria and frequency. Her booking visit was at 9th week of gestation and all routine antenatal tests were negative. Latest scan was done at 33-week follow-up and all parameters were corresponding to gestational age.
On physical examination, she appeared alert, conscious, with no tachypnea and no signs of dehydration. She was afebrile, with no cervical lymph nodes and tonsillar enlargement. The lungs were clear. She had tachycardia up to 110/min and blood pressure was 105/57 mmHg. The abdominal findings corresponded to gestational age, single fetus, longitudinal lie, and cephalic presentation with good fetal heart rate. The estimated fetal weight was 2 to 2.2 kg. The vaginal examination noted that os was closed. The ultrasound examination was repeated and it corresponded to respective gestation. The urine dip stick was suggestive of urinary tract infection. The urine specimen was sent for culture and sensitivity (C&S) before starting the antibiotics.
She was observed in the maternity ward. However, the patient was noted to be febrile with temperature of 39°C, tachycardia, and low blood pressure 80/50mmHg on next day leading to septic shock. Her blood pressure was maintained by intravenous noradrenaline infusion. A septic workup was done and initial resuscitative measures were performed. The blood test results came back with white cell count of 15.34 x 109/L and C-reactive protein level of 365mg/L. She went into active phase of labour at the same time.
Multidisciplinary input from anaesthetist for ICU backup, physician, and infectious disease teams was obtained, led by obstetric consultant shared care. The arterial blood gas showed metabolic acidosis. Her condition worsened as she became restless and breathless even with 2L/min oxygen under nasal prong. Four hours in labour, it was noted that the progress of labour was unsatisfactory with features of fetal distress. A decision was made to deliver by caesarean section in collaboration with anaesthetist and ICU team. An alive, female, 2.61 kg baby with Apgar score of 5 in 1 minute, 7 in 5 minutes and 8 in 10 minutes, was delivered and estimated blood loss was about 700 ml. A placental swab was taken for C & S. The anaesthetist noted on lungs auscultation that air entry at the left lower zone and a portable chest X- ray was ordered. The chest X-ray was reported as slight basal lobe collapse at left lobe with reactive pleural effusion (Figures and ). Tamiflu was started and she was diagnosed with Acute Respiratory Distress Syndrome (ARDS). The baby was admitted to the nursery for late prematurity with presumed sepsis. Antibiotic treatment was given. The placenta swab culture result came back as sterile; still the baby needed photo therapy as neonatal jaundice arose.
The patient was extubated after one day but continued support with Venturi mask 50% and 1.5L/min of oxygen. Once stable, she was transferred to High Dependency Unit and continued monitoring there. The throat swab was reported as influenza A positive and the message was relayed to both Infectious Diseases and Paediatric team. The recommendation was to continue tablet Tamiflu (oseltamivir phosphate) for one week. Universal precaution and isolation of the patient were followed. The symptoms subsided with the treatment. The patient and baby were discharged on the fifth day postoperatively without complications.


A 68-year-old male presented to our hospital with complaints of weight loss, fatigue and a progressively increasing mass over the left mandibular area for the past three months. On examination, the mandibular mass was firm, with no overlying skin changes or discharge. His past medical history included type two diabetes mellitus and chronic pancreatitis diagnosed eight months ago on computed tomography (CT) of the abdomen and pelvis from an outside institution. The CT also reported a 1.4 cm mass in the left kidney. Since his renal function tests were normal and there were no systemic complaints, no further investigations were ordered. Three months later the patient noticed bilateral swelling in armpits which were identified as bilateral axillary lymphadenopathy. Fine needle aspiration cytology of the left axillary lymph node revealed chronic lymphadenitis. Consequently, the patient was prescribed antibiotics. As the axillary lymphadenopathy persisted and the patient noticed new onset cervical lymphadenopathy, an otorhinolaryngology consultation was sought and CT of the neck was performed. The CT revealed bilateral cervical lymphadenopathy and small lytic lesions in the scapula, humerus, upper ribs and cervical vertebrae. This raised the suspicion of bone marrow involvement with a differential diagnosis of a lymphoma, multiple myeloma or metastatic disease. The patient's laboratory investigations on presentation are shown in Table .
A serum immunofixation electrophoresis was ordered which revealed normal levels of serum immunoglobulins G, A, and M, decreasing the likelihood of multiple myeloma. Tissue biopsy of the mandibular lesion exhibited a tumor comprised of nests of polygonal cells with abundant and clear cytoplasm. The nuclei were round to oval and hyperchromatic. A tissue biopsy from a lesion in the left rib revealed predominantly necrotic tissue with one fragment showing a tumor. The tumor comprised of atypical, ovoid cells with hyperchromatic nuclei and eosinophilic to clear cytoplasm. An admixed lymphocytic infiltrate was also seen. Figure shows the tissue biopsy of the mandibular lesion.
A bone marrow biopsy was performed to rule out bone marrow involvement. Bone marrow trephine revealed a hypercellular marrow showing infiltration by non-hematopoietic tissue, composed of tubular structures lined by large cells with abundant and clear cytoplasm. Figure shows the bone marrow trephine biopsy.
The infiltrate was positive for cytokeratin AE1/AE3 and CD10 immunostains. CD10 is an immunostain that is found positive in proximal convoluted tubules. Figure shows a section of the bone marrow showing positivity for CD10.
This confirmed the presence of metastatic renal carcinoma, likely clear cell RCC in the bone marrow. The patient was referred to the oncology clinic for further workup to identify the primary neoplasm.


A 21-year-old woman was referred with aggression, irritability, talkativeness, decreased need to sleep, and racing thoughts without psychotic features.
Her symptoms commenced about 3.5 years ago. She had two prior psychiatric hospitalizations 1.5 and three years ago. The first episode was depression and she had history of two suicidal attempts in the depressed phase. In the second episode, she experienced mania with psychotic features. Because of drug noncompliance after the second episode, a long acting antipsychotic agent (Flupentixol Decanoate) was prescribed for her once monthly. The latest injection was 1.5 months before admission, but she stopped taking Biperiden (4mg/day) and lithium carbonate (900mg/day) since five months ago.
She had a family history of major depressive disorder in her mother and elder sister. She had not any medical comorbidities or history of brain trauma or autistic disorder. Moreover, she had no history of benzodiazepine or alcohol withdrawal.
Laboratory data at the admission time were within normal range except for vitamin B12 level which was in the range of severe deficiency (as the result of vegetarianism). The brain neuroimaging (MRI) revealed no abnormality.
At admission, she had non-cooperative attitude, irritable mood with congruent affect, and pressure of speech without any hallucinations or delusions. She was well-oriented. Her vital signs were within normal levels. In medical examination, she had no tremor, rigidity, or any other extra pyramidal signs.
Drug regimen included lithium carbonate up to 1200 mg/day with 1 mg/dL of serum level, Quetiapine up to 600 mg/day, and Biperiden up to 4 mg/day. Within 3 weeks of non-responsiveness, lithium carbonate was tapered and discontinued within a week. The rest of the medications continued. Then, bitemporal ECT was given with energy level of 25% every other day. Duration of seizures was between 22 to 45 seconds. Anesthesia was induced by Atropine 0.5 mg IV, Succynil choline 25 mg IV, and Thiopental sodium 150 mg IV. The reason for the ECT was non-responsive to the medications, and the patient's family insisted for treatment as soon as possible due to fear of possible consequences of mania for the patient.
She developed some negativism after the fourth session of ECT, and after the fifth session, she developed full catatonic state including waxy flexibility, posturing, negativism, mutism, and echolalia.
Her vital signs were normal and she had not any autonomic dysfunction and extrapyramidal signs. The patient had no history of catatonia or neuroleptic malignant syndrome.
ECT and other psychotropic drugs were discontinued as soon as possible, and lorazepam was prescribed and increased up to 4mg/day twice a day.
The fulminant catatonia resolved after 4 days of treatment, and lorazepam was tapered after remission. Because of non-responsiveness to other antipsychotic agents, clozapine was started and its dose was escalated to 200 mg/day during 25 days until the patient’s discharge. After discharge, there was no sign of recurrence of catatonia in the follow- up sessions.
Informed consent was obtained from the patient for presentation.


A 32-year-old G3P3002 African woman came from Yifag Kebele, Amhara Region, to Felege Hiwot referral hospital in Bahir Dar, Northwest Ethiopia in July 2016. She presented with abdominal pain and intractable vomiting of 1 day’s duration. She was also unable to pass feces and flatus and had developed progressive abdominal distension. She had a past medical history notable only for chronic gastritis for which she took unspecified medications and a past surgical history notable for a Cesarean hysterectomy after an intrauterine fetal demise during labor. As she had been told that her uterus was removed, she did not use contraception and had no menses. She was admitted to our surgical ward with a diagnosis of small bowel obstruction due to presumed post-operation adhesions and possible incisional hernia. She also had severe anemia and was resuscitated with 2 liters of normal saline and transfused with 2 units of blood. A plan was made to correct the hernia once she was stabilized. After 2 days in our hospital, however, her condition worsened and a consultation was made to Obstetrics and Gynecology for further evaluation.
On physical examination at the time of consultation, she was confused and irritable, with an undetectable blood pressure and a thready pulse of 132. She had labored breathing, pale conjunctiva, and a distended abdomen with a palpable mass below the midline surgical scar. An abdominal examination also revealed a fluid wave and hypoactive bowel sounds. Laboratory testing showed a white blood cell count of 12.9 × 103 with 88.4% neutrophils and hemoglobin of 5.8 g/dl. Urine human chorionic gonadotropin (hCG) was positive. A transabdominal ultrasound showed a normal liver, spleen, pancreas, and kidneys. There was a significant debris-filled intraperitoneal fluid collection, especially on the right side of her abdomen, with a deepest pocket measuring 5 cm. No lymphadenopathy was seen. A singleton, viable pregnancy was identified measuring 13 weeks of gestational age with no gross abnormality and adequate amniotic fluid.
The Obstetrics and Gynecology team diagnosed hypovolemic shock secondary to ruptured ectopic pregnancy, and our patient was taken to the operating room for a laparotomy. Intraoperative findings included 4.5 liters of hemoperitoneum, a cervical stump pregnancy with well-formed fetus and intact gestational sac, and active bleeding from partially detached placental site. While the left ovary appeared normal, the right ovary and both fallopian tubes were absent.
The ectopic gestation was clamped at its base and resected from the cervical stump. Bleeding sites were ligated to ensure hemostasis. Our patient was deemed too unstable for trachelectomy. Her abdomen was irrigated with warm saline and the incision closed in layers. She was transferred to our intensive care unit (ICU) for aggressive volume resuscitation with 8 liters of normal saline and 5 units of blood, and a dopamine drip was initiated to assist with blood pressure control. After 2 hours, her blood pressure had stabilized and she was discharged 9 days later. On follow-up at fourth week post-operation, she was healing well and had hemoglobin of 11 g/dl.