An 83-year-old female was transferred to our emergency department (ED) from the branch hospital due to hydrochloric acid ingestion about one and a half hours previously. She was a case of major depressive disorder. She drank toilet bowl cleaner containing 9.5% hydrochloric acid. Subsequently, she was sent to our branch hospital ED. She complained about chest pain and 12-lead ECG was done. ST segment elevation was noted at lead II, III, and aVF (Fig. ). The blood examination included complete blood count, glucose, aspartate transaminase, alanine transaminase, blood urea nitrogen, creatinine, sodium, potassium, creatine kinase, creatine kinase-MB, Troponin I, arterial blood gas, prothrombin time, and partial thromboplastin time. Elevated aspartate transaminase (69 IU/L), thrombocytopenia (62000/μL), and acidosis (pH 7.311, pCO2 27 mm Hg, HCO3 13.3 mmol/L) were noted. Cardiac enzyme test did not elevate then (creatine kinase 36 IU/L, creatine kinase-MB 1.8 ng/mL, troponin I 0.02 ng/mL).
Under the impression of STEMI, she was transferred to our ED within 1 hour. Coronary angiography was done within 1 hour after arrival. Angiography showed 60% stenosis in the segment 7 of left anterior descending coronary artery and 30% nonsignificant stenosis in the segment 2 of right coronary artery, with no apical ballooning. No significant lesion consistent with STEMI was found. The following venous blood gas showed rapid progress metabolic acidosis (pH 7.066, HCO3 7.9 mm Hg). Bradycardia was followed by cardiac arrest that developed 4 hours later. Cardiopulmonary resuscitation was applied and the patient became shock status after return of spontaneous circulation. Computed tomograpy did not reveal pneumomediastinum but showed diffuse stomach wall thickening and adjacent fluid accumulation. Family decided on conservative treatment and the patient was admitted to the intensive care unit and expired on next day.


A 70-year-old woman presented at her local hospital with a sticky sensation in her throat and a weight loss of 2 kg over 10 months.
Esophagogastroduodenoscopy (EGD) revealed an elevated lesion 35 cm from the incisors that was diagnosed as malignant melanoma by biopsy. She was referred to our institution for further examination and treatment. Her blood examination was normal, including tumor markers such as CEA and CA19–9, except for a slightly elevated HbA1c level.
A barium swallow test was arranged and showed a filling defect in the lower esophagus (Fig. ). EGD showed a pigmented and elevated lesion 7 mm in diameter, associated with a hemi-circumferential, irregular-shaped, pigmented, and flat lesion in the lower esophagus. The flat lesion ranged from dark brown to black in color, and the black area contained a well-demarcated mucosal abnormality (Fig. ).
A biopsy specimen showed malignant melanoma cells in the esophageal mucosa, which were strongly positive for melanoma-specific antigens S-100 and HMB45 by immunohistochemical staining. A computed tomography (CT) scan also showed an intraluminal mass in the lower esophagus, which was well-defined without infiltration into the surrounding tissues (Fig. ). There was no enlargement of mediastinal lymph nodes or any visible metastatic lesion. 18F–fluorodeoxyglucose (18F–FDG) positron-emission tomography (PET) combined with CT showed abnormal 18F–FDG uptake in the same part of the esophagus identified on EGD and barium swallow as the site of the lesion with a maximum standardized uptake value (SUV max) of 3.1 (Fig. ). Detailed clinical examination of the eyes, oral cavity, nose, and skin did not indicate any malignant melanoma lesions.
Based on these findings, the preoperative diagnosis of the lesion was PMME without metastasis (cT2N0M0, cStage II) according to the UICC TNM classification of esophageal cancer []. Then, thoracoscopic and laparoscopic subtotal thoracic esophagectomy with lymphadenectomy of the neck, mediastinum, and abdomen was performed. The cervical esophagus and the elevated gastric tube were anastomosed via the posterior mediastinal approach. The operation time was 536 min, and blood loss was 95 mL.
The resected specimen showed an elevated, black, and pigmented polyp-like lesion (15 mm × 13 mm × 9 mm) in a flat and pigmented area (57 mm × 38 mm) (Fig. ). A faintly marked depression that was partly tinged with white on the surface of the polyp-like lesion was found (Fig. ). Microscopic examination showed that most of the polyp-like lesion was composed of solid growth, with pseudocircular and spindle-shaped atypical cells containing melanin pigmentation and irregularly demarcated nucleoli. Many melanophages were present in the intervening interstitial stroma (Fig. ). The lesion was immunohistochemically stained strongly for melanoma-specific antigens melan-A, S-100, and HMB45 (Fig. ). Around the polyp-like lesion, the same characteristic cells were spread laterally in the epithelium. Melanophages were also present in the lamina propria beneath the polyp-like lesion. The tumor cells were thought to invade the muscularis mucosae directly and then spread horizontally in the basal layer of the esophageal epithelium, which is called “junctional activity” [] (Fig. ). The proximal and distal margins were considered safe. No lymph node metastases were detected. Pathologically, a diagnosis of pT1a-MM, pN0, pM0, pStage IA [] was rendered.
Her postoperative course was uneventful and favorable. Adjuvant chemotherapy was not administered, and she has survived 39 months so far without any evidence of recurrence. She has been followed up once a half year and underwent blood tests and contrast-enhanced CT to search for metastasis or recurrence.


A 69-year-old male who had undergone left-sided hemi-hepatectomy for HCC was diagnosed with multifocal hepatic recurrence of the tumor 1 year later. His cardiovascular risk profile consisted of smoking (38 pack years), arterial hypertension, insulin-dependent diabetes, obesity, and dyslipoproteinemia. His medical history additionally comprised bilateral carotid artery disease and stenting of the right internal carotid artery, chronic renal impairment, and chronic obstructive lung disease. Chronic occlusion and good collateralization of his left anterior descending artery (LAD) had been described 5 years previously when he underwent PCI for myocardial infarction with stent implantation in the RCX.
After recurrence of the HCC, he suffered a posterior NSTEMI and underwent stenting of a subtotal stenosis of the RCA. In addition to the chronically occluded LAD, coronary angiography now showed 80 and 90% stenosis of the distal RCX and a posterolateral branch, respectively, which were not addressed in the course of the intervention.
In the weeks after the coronary intervention, he suffered from increasing angina and developed ventricular arrhythmia. Echocardiography, MRI (Siemens Magnetom Prisma, 3 Tesla), and CT (256 Multisclice Philips ICT) showed a tissue mass of 72 × 45 × 56 mm infiltrating the myocardium of both ventricles and the interventricular septum (Fig. a, b). When his cardiac symptoms became so debilitating that they limited his quality of life more than the cancer and he expressed an urgent desire for treatment despite his dismal prognosis, he was referred to our department to evaluate surgical treatment options.
As the RCA lesion had already been addressed, the LAD had been occluded with good collateralization for at least 5 years, and we agreed with the cardiologist that it was highly unlikely that such severe symptoms were caused by the remaining RCX lesions alone, we assumed the metastatic tumor to contribute to the patient’s deterioration by stealing perfusion from, compressing, or infiltrating coronary vessels. The imaging performed by this date, however, had not shown large tumor vessels communicating with the coronary arteries so that these phenomena were supposed to be caused by a multitude of small tumor vessels rather than large communicating vessels that would be amenable to interventional treatment such as embolization.
Assuming that a sufficient reduction of the tumor size would not only palliate the patient’s angina but also reduce the frequency and severity of his ventricular arrhythmia, and given both the surgical risk of an aggressive procedure (Euroscore 14.02 for a combined procedure including CABG) and his palliative situation and limited life expectancy, he was offered a restrictive surgical approach consisting of debulking of the metastatic tumor with an option for subsequent coronary intervention, should the surgical procedure not achieve sufficient palliation of his symptoms.
After median sternotomy and opening of the pericardium, the apex of the heart appeared plump and enlarged with an even surface that showed no visible tumor growth. The large metastasis seen on MRI and CT was palpable on and infiltrating the apex. Cardiopulmonary bypass (CPB) was established in customary fashion because exposition of the apex was not possible without provoking severe arrhythmia and hemodynamic instability. Felt strips were placed along the edges of the palpable tumor mass using 12 pledgeted 4.0 prolene sutures (Fig. c) that were snugged down after application of French glue. Without entering the ventricular cavities, a portion of the tumor measuring 27 × 27 × 12 mm was removed in toto (Fig. d). Additionally, an approximately equal quantity of fragmented tumor tissue was removed so that only those sections of the tumor that had grown deep into the ventricular walls and septum were left. This resulted in considerable bleeding from a multitude of small vessels within the tumor. After careful hemostasis, the edges of the crater that was left within the area delimited by the felt strips were approximated by tying down two pledgeted 2.0 prolene sutures that were spanned across the crater through two of the felt strips. Following this, the remaining 4.0 sutures were tied down, and the defect was finally closed by a mattress suture followed by an over-and-over suture. The patient remained hemodynamically stable throughout the procedure, and weaning from CPB, decannulation, and sternal closure were performed in customary fashion.
Histopathologic examination of the removed tissue showed a mostly solid tumor consisting of medium-sized to large cells with a wide eosinophilic cytoplasm surrounding enlarged vesicular nuclei with prominent eosinophilic nucleoli. Further findings consisted of atypical mitotic figures and local invasion of blood vessels. Immunohistochemical examination yielded a profile that was also compatible with a diagnosis of metastatic HCC (co-expression of Hep-Par1, positive for CK8 at least in some sections, cytoplasmatic positivity for TTF1, negative for CK7).
The patient was extubated 5 h after the procedure and discharged from the ICU on postoperative day 2. He took an uneventful further course and was discharged on postoperative day 9 with his symptoms palliated. One and a half months later, he presented for a follow-up examination, this time complaining not of cardiac symptoms but of headaches. MRI of the head showed no cerebral metastases. MRI of the heart showed a mass of felt strips and organized hematoma on the apex as well as the change in configuration of the apical region brought about by the surgical procedure. This made it difficult to assess the size of the tumor which appeared, however, to have regained much of its previous size. Nevertheless, the patient survived five and a half months after the procedure with his cardiac symptoms alleviated.
The patient provided written approval of the publication of his case including any pertinent images for scientific purposes.


A 3-year-old girl was referred to our hospital with a 1-day history of painless bilateral subconjunctival hemorrhage and a 5-day history of running nose, cough, and wheezing. Mycoplasma pneumoniae antibody titer (immunoglobulins IgG and IgM) was 1:640. Mycoplasma pneumonia had been diagnosed based on serum antibody titers and she had been treated with oral azithromycin (10 mg/kg once daily) for three days at presentation. She developed pneumothorax associated with persistent cough and wheezing, so her bilateral subconjunctival hemorrhage may have been caused by coughing. The cornea, anterior chamber, iris, and lens were normal in both eyes. Funduscopy was within normal limits. There was no coagulopathy and no history of trauma or other medical problems. The patient did not have ocular pain, photophobia, itching, discharge, or headache. She was followed without treatment because her subconjunctival hemorrhages were asymptomatic Fig. (). Subconjunctival hemorrhage was still detected in both eyes at one week after the onset, but complete resolution was achieved within two weeks.


An 85-year-old woman was referred to our institution with a 10-day history of breathing difficulties, dysphagia for solids and liquids, and dysphonia. The patient was a former smoker (20 cigarettes/day for 45 years until the age of 60), while no alcohol intake was reported. The patient's medical history was positive for chronic obstructive pulmonary disease, hypertension, anaemia, osteoporosis, goitre, and chronic renal failure.
General otolaryngological examination was normal. Endoscopy revealed a submucosal lesion in the right arytenoid, covered with normal mucosa, extending towards the right pyriform sinus. The right vocal cord was fixed while the left was hypomobile, thus reducing breathing space. There was no clinical evidence of cervical lymphadenopathy. A computed tomography (CT) scan of the head and neck showed a contrast-enhanced, solid hypopharyngeal-laryngeal neoplasm with well-defined margins, originating from the right pyriform sinus with partial contralateral extension. The mass measured 18 × 22 × 12 mm and extended caudally to the right arytenoid and the upper margin of the cricoid cartilage, causing subtotal obliteration of the right pyriform sinus and a reduction in air lumen of the laryngeal vestibule. Other laryngeal structures were preserved ().
The patient underwent complete endoscopic removal of the lesion and temporary tracheostomy for respiratory distress; tracheostomy was closed 32 days later. Neither postoperative complications nor signs of recurrence during an 18-month follow-up were observed.
Histologic examination of the neoplasm revealed a well-circumscribed, not encapsulated, proliferation of large closely packed neoplastic round to polygonal cells in a scant stroma (Figures and ). The cells showed a deeply eosinophilic focally vacuolated cytoplasm and round, predominantly central, nuclei, at times with prominent nucleolus. In some cells, multiple nuclei were found. Cells with a peripheral cytoplasmic clear zone traversed by thin cytoplasmic strands extending from the more central acidophilic cytoplasm to the periphery (so-called “spider” cells) were detected as well. Mitotic activity and necrosis were absent. Neoplastic cells were immunoreactive for desmin (), myoglobin (), and muscle-specific actin. Immunohistochemical staining for cytokeratin (CKAEAE3 and CKMNF116), S-100 protein, CD68R, chromogranin-A, and synaptophysin failed to stain the neoplastic cells. Based on the histologic and immunohistochemical features, a diagnosis of adult-type RM of the larynx was made.


A 20-year-old male who complained of neck pain that radiated to the left shoulder and arm was admitted to the emergency room. His symptoms developed after accidentally hitting his head on a horizontal bar while riding a bicycle with his head in a rotated position. However, physical examination revealed no specific neurological abnormalities. Plain radiographs of the cervical spine showed traumatic C1–2 posterolateral dislocation associated with type II dens fracture that was established on the basis of the Anderson and D’Alonzo classification and anterior subluxation of C6–7 (Fig. ). Computed tomography (CT) scans confirmed the dislocation with type II dens fracture (Anderson and D’Alonzo classification)[ and revealed a unilateral facet fracture with subluxation and lamina fracture of C6–7 (Fig. ). Magnetic resonance (MR) images showed a detached posterior longitudinal ligament from the posterior aspect of the dens and type II TAL (Dickman classification) injury[ (Fig. ). Acute hemorrhagic fluid was seen collecting along the retropharyngeal space of the subaxial spine.
Skeletal traction was gently applied using a Gardner well tong. Seven pounds was used as the initial traction weight, and it was gradually increased to 18 pounds under fluoroscopy for 2 days. Closed reduction of the traumatic C1–2 posterolateral dislocation with type II dens fracture was successfully achieved (Fig. ). After closed reduction, angio-CT scans of the neck showed a normal vertebral artery at the C1–2 level, but some degree of residual subluxation of the C1–2 joint with type II dens fracture (Fig. ). While maintaining skull traction with 7 pounds, we performed anterior discectomy, open reduction, and fusion of C6–7 with plate fixation (Aspiron, U & I, Uijeongbu, Korea) and allograft (Cortical cervical spacer; DCI Donor Service, Albuquerque) packed with demineralized bone matrix (DBX; Musculoskeletal Transplantation Foundation, Edison). The patient was then placed in the prone position, and posterior segmental screw fixation and fusion of C1–2 were performed. Bilateral C1 posterior arch screws and bilateral C2 pedicle screws (Vertex Select; Medtronic, Memphis) were inserted. The cartilaginous portions of the C1–2 facet joints were thoroughly removed. Meticulous decortication of C1–2, including the facet joints, and sufficient bone grafting with autogenous iliac crest were performed in order to obtain solid fusion. Finally, rods and a crosslink set were applied and tightened. At 2 months after surgery, the patient's neck pain and occipital neuralgia had nearly resolved. One year after surgery, plain radiographs of the cervical spine showed solid union, and dynamic radiographs showed no abnormal motion of the C1–2 and C6–7 segments (Fig. ). Reconstructed 2-dimensional and 3-dimensional CT scans also confirmed anatomical reduction and solid fusion of the C1–2 and C6–7 segments (Fig. ).


An 11-year-old girl was referred to the cardiology clinic on account of left hemiplegia of one month duration. The hemiplegia was of sudden onset and was associated with headache, dizziness and vomiting at onset. She had also been having intermittent episodes of palpitations and had just been discharged from hospital two weeks earlier after being managed for an intracranial space-occupying lesion, with raised intracranial pressure, left hemiplegia and multiple cranial nerve palsy. She had a positive history of sudden death in her family (an uncle and her grandmother).
On examination, she had a hemiplegic gait, a left CN V1, VII palsy and decreased power, tone and reflexes in the left upper and lower limb. A regular pulse and wide blood pressure difference was noted in both right and left upper limbs (right 120/50 mmHg, left 60 mmHg/unrecordable).
Previous tests had been done when she was admitted. MRI of the brain showed subacute right thalamic infarct, and her blood lipid profile and random blood glucose results were normal. Her genotype was AA, mantoux was negative, chest radiograph was normal, and PT/PTTK was also normal.
A diagnosis of peripheral artery disease was entertained and magnetic resonance angiography of both carotid arteries was done, which was normal. The ECG showed sinus rhythm with evidence suggestive of left atrial and left ventricular hypertrophy. An echocardiography could not be done immediately but was requested.
She was subsequently placed on aspirin, encephabol and regular physiotherapy while angiography was being awaited. She defaulted from follow up and was seen in hospital three months later when she collapsed at school after complaining of heaviness of the right side of the body and inability to walk. Her history revealed that she had stopped her aspirin two days earlier.
On examination, she was conscious, and the blood pressure in both upper limbs was equal and normal (110/70 mmHg). Her pulse was a good volume but irregularly irregular, both legs were cold to the touch, and the dorsalis pedis was barely palpable. A diagnosis of deep-vein thrombosis was entertained.
She was rehydrated and recommenced on aspirin tablets, while vascular ultrasound of both legs showed a deep focal vein clot in the right common iliac vein. A transthoracic echocardiography was finally done and it revealed a mobile mass in the left atrium (myxoma) measuring 3.9 × 2.6 cm impinging on the mitral valve, a dilated left atrium and multiple ectopic beats ().>
A diagnosis of left atrial myxoma with intermittent arrhythmia and multiple thromboembolic events was made. Anticoagulation was commenced with heparin and warfarin tablets. She was also referred for surgical resection of the atrial myxoma. Due to financial constraints, surgery could not be done and she died 10 months later while awaiting resection of the cardiac tumour.


This 9 year-old male patient had a VH score of 1.5+ units secondary to intermediate uveitis in his left eye. Uveitis initially occurred at age of 6. Sarcoidosis was suspected because of serum IL2- receptor elevation to 969 IU/ml, but no other organ involvement was found during systemic work-up. He had been treated with CSA orally and MTX subcutaneously, but had to discontinue this regimen due to side effects. Upon enrollment, he was prescribed oral Prednisolone at a concentration of 0.2 mg/kg body weight once daily for 3 months without sufficient effect on VH. After DEX injection, VH decreased from 1.5+ units to 0 at the 4 and 6 months time points. BCVA improved from 55 to 83 letters 5 months after DEX injection. Part-time occlusion therapy of the fellow eye was started at month 2. Macular OCT showed diffuse thickening (CRT 320 μm) at baseline but without evidence of CME. CRT decreased to 262 μm at 2 months after DEX injection and then increased to 319 μm at study end. IOP was 17 mmHg prior to DEX injection and 30 mmHg one month after. Timolol-Dorzolamide eye drops were administered twice daily and IOP decreased to 14 mmHg (month 2) and 13 mmHg (month 3), and medication was stopped. At study completion CDR was stable and the lens remained clear. During the following year VA remained stable and VH score increased slightly to 0.5+ units. One year after study completion a relapse occurred, but was effectively controlled with steroid pulse therapy. Currently, no therapy is needed for intraocular inflammation and the patient has a VA of 80 ETDRS letters.


A 6-year- and 9-month-old boy was referred to our hospital with a history of 6-month intractable diarrhea. Diarrhea was characterized by loose, yellow–green, or green, non-bloody, two to three times per day. The boy was born at term, and his family history was normal. Before admission to our hospital, the patient suffered aggravated watery diarrhea for 15 days, 10 times daily, abdominal pain, 5 days of fever, and a weight loss of 3 kg. Laboratory tests showed a normal eosinophil counts, normal immunoglobulin G, IgA, IgM, and IgE levels. Lymphocyte subsets analysis revealed normal proportions of CD3, CD8 T cells, decreased proportions of CD19 T cells (5.74%, reference range: 14.35–22.65%) and CD4 T cells (25.77%, reference range: 29.78–39.94%) (Table ). A slightly low absolute natural killer cells (0.26 × 109/L, reference range: 0.28–0.63 × 109/L) and a low number of CD4 T cells (0.32 × 109/L, reference range: 0.71–1.84 × 109/L) were observed (Table ). Antineutrophil cytoplasmic and other autoantibodies, including diabetes-related autoantibodies, antithyroglobulin were all negative (Table ). The patient was treated with cefoperazone–sulbactam for intestinal infection. Five days later, gastroscopy and colonscopy were performed for no improvement of symptoms. Colonscopy showed mucosal damages and ulceration, biopsy histology showed eosinophil infiltration (20–30/HP), and hyperplasia of lymphoid follicles. The patient was started with methylprednisolone for suspicion of Crohn’s disease based on colonic ulcers and biopsy pathological features. Infliximab (5 mg/kg) was given for no significant remission after methylprednisolone treatment. Then, the patient was discharged for achieving a partial remission of diarrhea. Two weeks later, he was admitted to our hospital again for second course of infliximab. Diarrhea was still presented in the patient, and characterized by bloody, mucus-containing stools. Metronidazole was started due to Clostridium difficile infection (CDI). Oral methylprednisolone and deep hydrolyzed milk were given and discharged for partial improvement of symptom. Two weeks before the third admission, the boy suffered with severe watery diarrhea again, with occasionally bloody, mucus containing stools, more than 10 times per day, accompanied with abdominal pain and fever. During the third admission, vancomycin and teicoplanin were used to treat CDI, fluconazole and mycamine were used to treat Candida albicans infection which found in the mouth, ceftriaxone, and azithromycin were given to treat pneumoniae Mycoplasma. In addition, fecal microbiota transplantation (FMT) was performed to treat CDI (). Albumin, gamma globulin, red cell suspension, and frozen plasma were given due to hypoproteinemia. Parenteral nutrition and enteral nutrition were supported for failure to thrive. Diarrhea, abdominal pain, mucus-containing stools, and recurrent infections were not well controlled after more than 2 months of continuous treatment. Therefore, the patient was suspicious of immunodeficiency disease and extraction of peripheral blood for immune gene panel testing. Finally, the boy was diagnosed as IPEX syndrome due to a c.1190G>A (p. R397Q) mutation in exon 11 of the FOXP3 gene (Figure A). The patient’s mother is a healthy carrier (Figure B). Two healthy siblings were excluded the same mutation by DNA Sanger sequencing (Figures C,D). At 3 months after the genetic diagnosis of IPEX, at the age of 7.5 years, the boy underwent matched sibling peripheral blood HSCT using reduced-intensity conditioning with busulfan, fludarabine, and cyclophosphamide, antithymocyte globulin. The CD34 cell dose was 7 × 106/kg, and the number of neutrophils was 3.09 × 109/L, platelets was 276 × 109/L in the day of engraftment. He continued to receive cyclosporine and methylprednisolone to prevent graft-versus-host disease. At day +14 post-HSCT, the short tandem repeat test showed that he engrafted with 100% of donor cells. Up to date, diarrhea, abdominal pain, and recurrent infections were well controlled after 6 months of HSCT, and Clostridium difficile (CD) test was negative.


A five-month-old male, with no significant past medical history, was brought to the pediatric emergency department by his mother due to refusal to move his left lower limb and irritability for the past few hours prior to arrival. His mother denied any trauma or fever. The patient had no upper respiratory symptoms or recent illness. The patient was exclusively breastfed, and not on vitamin D supplementation. He was making adequate wet diapers, and there were no changes in bowel movements. The patient was born full term and delivered at home via normal spontaneous vaginal delivery. Newborn screening was negative with no evidence of sickle cell disease or immunodeficiencies that were routinely checked. The mother did not receive any prenatal care prior to delivery, and the patient's postnatal course was uneventful. As per the parent's wish, both the patient and his older sister were unimmunized.
Upon arrival to the emergency department, it was noted that the patient was irritable. He had a temperature of 101 Fahrenheit, and his left lower extremity was kept in the frog position. There was no erythema or swelling, but it was noted that he was crying upon palpating the extremity. Other than the left lower extremity findings, his physical exam was unremarkable. Given the findings on his physical exam, initial suspicion for infectious etiology versus child abuse was raised, and laboratory tests were sent including complete blood count with differential, inflammatory markers of C reactive protein (CRP) and erythrocyte sedimentation rate (ESR), blood culture, basic metabolic panel, and liver function tests. Results are as follows: white blood cell count of 9.9 K/uL; manual differential of 34% lymphocyte, 53% neutrophils, and 3% bands; hemoglobin level of 8.2 g/dL; very low level of calcium 5.9 mg/dL (normal range for age and sex 8.7–10.5 mg/dL); CRP of 22.8 mg/L; and ESR 30 mm/HR (). Imaging of the lower extremities was suggestive of rickets. No acute fracture was seen ().
On the pediatric floor, the patient was started on acetaminophen every four hours, as well as calcium, vitamin D, and iron supplementation. The patient continued to be febrile with the highest temperature peaking to 104.4 F on day 2 of admission. Consults were obtained from the pediatric endocrine, orthopedic, and infectious disease specialists. A clinical decision was made to obtain further laboratory work including vitamin D, parathyroid hormone, phosphorus, and alkaline phosphatase levels to investigate the cause of hypocalcemia, which were significant for a vitamin D level of 9.2 ng/mL, parathyroid hormone level of 466 pg/mL, and normal phosphorus level of 3.1 mg/dL (). These labs were consistent with nutritional rickets, and the radiology findings were classical for rickets (). The patient was started on vancomycin after microbiology informed us that the blood and urine cultures were both positive for Gram-positive cocci in clusters and to cover for Methicillin-resistant Staphylococcus aureus. The susceptibilities returned the next day, and the Staphylococcus aureus was methicillin sensitive (MSSA). Vancomycin was switched to cefazolin, and a repeat blood culture was immediately drawn, which was negative.
A magnetic resonance imaging (MRI) of the bilateral extremities was done on day 2 of admission which showed a small left knee effusion, possible myositis or cellulitis; however, it was noted to be a limited study. A repeat MRI was performed on day 6 of admission, which revealed osteomyelitis involving left proximal tibial diaphysis and metaphysis (). On day 10 of admission, a peripherally inserted central catheter (PICC) was placed, and antibiotic was switched to nafcillin every 6 hours. Nafcillin causes increased rates of phlebitis in children with peripheral IVs, and hence cefazolin was preferred when the child had a peripheral IV. Therefore, after placing the PICC, the antibiotic was changed from cefazolin to nafcillin, as nafcillin is the drug of choice for MSSA. We switched the antibiotic to nafcillin from cefazolin, because nafcillin could cause phlebitis if given through a peripheral IV in a child, has a narrow spectrum, and is the drug of choice for MSSA.
He was treated with 16 days of antibiotics as inpatient, and his inflammatory markers (ESR and CRP) and calcium and phosphorus were trended every 2–3 days and were normal at the time of discharge.
He was discharged home with vitamin D supplements and a 4-week-course of oral clindamycin. The patient was followed up three times with the infectious disease and endocrinology clinics. Repeat Ca and phosphorus levels after discharge remained normal with an improved vitamin D level of 16.9 ng/mL. The vitamin D lab was never redone since patient was lost to follow-up after 32 days post discharge.


A 49-year-old woman with a medical history of hypertension, prediabetes, dyslipidemia, and histologically confirmed NASH (80% diffused steatosis with pericellular inflammation) was referred by her primary care provider for endocrine evaluation for complaint of persistent weight gain with central obesity. Two years prior to the referral she was discovered to have a left adrenal adenoma on a routine abdominal CT scan for evaluation of nephrolithiasis. Phenotypic features of overt CS (i.e., moon face, striae, buffalo hump, etc.) were not present, and biochemical evaluation of the adenoma was not performed at that time. At referral, the patient still did not have overt phenotypic features of CS. A follow-up scan revealed a stable benign left adrenal adenoma (2.9 × 1.9 × 2.5 cm) measuring 14 Hounsfield units on noncontrast CT (). The adenoma was described as lobulated with a high fat content. Baseline clinical characteristics and laboratory findings are listed in .
Results of hormonal testing were negative for pheochromocytoma and primary aldosteronism. Testing for autonomous cortisol secretion showed mildly elevated urinary free cortisol (UFC), failure to suppress cortisol on multiple 1-mg overnight dexamethasone suppression tests (DSTs), elevated late-night salivary cortisol, suppressed adrenocorticotropic hormone (ACTH) on multiple tests, and low dehydroepiandrosterone sulfate (DHEA-S) ().
The patient declined adrenalectomy. Ketoconazole was not considered because of the patient's fatty liver and elevated liver enzymes. Medical therapy with mifepristone (Korlym®, Corcept Therapeutics, Menlo Park, CA) was initiated at 300 mg per day and increased to 900 mg during the 34 weeks of treatment. After 4 weeks, her antihypertensive medication (amlodipine/olmesartan medoxomil 10/20 mg) was discontinued and her blood pressure remained stable (). During the course of mifepristone treatment, she lost 16 lbs and marked improvement in her liver enzymes was noted, with complete normalization by week 20 ().
At week 20 she complained of vaginal spotting, and a pelvic ultrasound showed multiple uterine fibroids. Subsequently she elected to undergo a hysterectomy and a left adrenalectomy. Mifepristone was discontinued 2 weeks prior to the adrenalectomy. Her prediabetes remained stable during mifepristone treatment; HbA1c was 40 mmol/mol before surgery.
During the 34-week course of medical therapy with mifepristone, the patient's hypothalamic-pituitary-adrenal (HPA) axis recovered, as indicated by increases in ACTH levels from undetectable at baseline and week 16 to 2.4 pmol/L at week 24 (). She was treated postoperatively with glucocorticoid replacement (hydrocortisone 50 mg), which was tapered and discontinued within 6 weeks. Her liver enzymes remained within the normal range and there were no further changes in blood pressure and weight 1 year postoperatively. Her most recent HbA1c was 33 mmol/mol (5.2%).


A 56-year-old woman presented with left side cheek swelling of 1.5 months in duration. On clinical examination, expansile hard swelling of left hemimandible was noted that was seen to be both intraoral as well as in the neck. A reconstructed radiograph () showed a bone-forming lesion along the left side of the mandible. Computed tomography (CT) imaging of the face and neck was performed for further characterization. CT imaging (, ) revealed a minimally expansile lytic lesion of the left hemimandible involving its angle and body with erosions of the medial cortex and new bone formation perpendicular to the mass forming the typical sunburst pattern. The patient also complained of a lump in the left breast for which bilateral mammograms were performed that revealed a Breast Imaging Reporting and Data System category 4 lesion in the left retroareaolar region. A biopsy of the left breast mass was done, and histopathology showed infiltrating duct carcinoma (grade 2). On the basis of the imaging findings of the mandibular lesion, diagnosis of aggressive bone lesion was made with the possibility of metastasis from breast primary. Primary OS was considered as the differential diagnosis. Needle biopsy of the mandibular swelling was performed, and the pathologic diagnosis was OS. The patient was planned for excision of both the left mandibular lesion and the left breast mass; however, the patient defaulted the treatment.
Four months later, the patient presented with rapid progression of the left mandibular mass. A repeat reconstructed radiograph () and CT imaging (, ) revealed a significant increase in the extent and size of the mass, particularly the osseous periosteal reaction ( show three-dimensional volume-rendered images of the bone beard tumor). Magnetic resonance imaging (MRI; ) of the neck was performed for planning the extent of resection, which revealed an expansile lesion in the left hemimandible extending into the submandibular region, floor of mouth, oral cavity, parapharyngeal space, and masticator space. T1- and T2-weighted images revealed large areas of low intensity with an intervening enhancing soft tissue component within the mass giving a sunburst appearance representing new bone formation, which was suggestive of an aggressive type of periosteal reaction.
The patient underwent left hemimandibulectomy with free fibula osteocutaneous flap reconstruction. The histopathology revealed OS with approximately 75% tumor necrosis. The left breast mass was excised and subjected to histopathology examination, which revealed infiltrating duct carcinoma with predominant ductal carcinoma in situ. Eight months after surgery, the patient presented with diffuse skeletal pain and dyspnea. Chest radiograph revealed pleural effusion, and technetium-99m–methylene diphosphonate bone scan showed multiple-site skeletal metastases with soft tissue tracer uptake in the left-sided pleural space with effusion suggestive of pleural involvement. Oral and neck examination did not show any evidence of recurrent or residual disease. The patient was managed for metastatic disease from the breast primary tumor.


A 76-year-old man was referred to our urology department with repeated symptoms of dysuria, frequent urination, urgency, and painful micturition for one month. His past medical history showed no hypertension, diabetes, or any history of surgery.
Laboratory data on admission showed that the total prostate specific antigen level was 7.76 ng/ml, free prostate specific antigen level was 0.618 ng/ml, and white blood cell count was 10,750/mm3; however, urinalysis and blood chemistry were all within normal range. Digital rectal examination revealed an enlarged prostate, a prominent median sulcus, a smooth rubbery consistency with no palpable nodule, and elastic enlarged prostate.
Ultrasound scan showed an enlarged prostate (38 mm in anteroposterior diameter, 43 mm in longitudinal diameter, and 51 mm in transverse diameter), prostatic hyperplasia, and a gland of 43 g.
Patient’s pelvis was imaged in 3.0-T MRI (Discovery 750, GE Healthcare, Milwaukee, WI, USA) using a surface phased array coil. Conventional MRI sequences included both axial fast spin-echo T2-weighted imaging (T2WI) and DWI. T2WI of the entire prostate and seminal vesicles was performed in axial, coronal, and sagittal planes. Conventional DWI with b-values of 0 and 1000 s/mm2 was also performed in the axial plane. The ultra-high b-value DWI was performed with b-values of 3000 s/mm2 in the axial plane. Both axial T2WI and DWI were obtained at the same slice location. Dynamic contrast-enhanced (DCE) MRI was also performed. Fifteen ml of Gadobenate dimeglumine (MultiHance; Bracco Sine, Shanghai, China) was injected intravenously at a rate of 3 ml/s using a power injector (Spectris; MedRad, Warrendale, PA), followed by a 20 ml saline flush. DCE-MRI was performed in the transverse plane at baseline (pre-contrast) and during arterial, venous, and delayed phases. MR imaging parameters are shown in Table .
Conventional MRI displayed a round tumor in the prostatic bottom protruding into the bladder with an isointense signal compared to the muscle on T1-weighted images (T1WI) with fat suppression and a homogeneous lesion with slightly hypointense signal on T2WI (Fig. ). Ultra-high b-value DWI (b = 3000 s/mm2) features showed slightly homogeneous hyperintense signal compared to the muscle, and the mean ADC value was 0.817 ± 0.016 × 10−3 mm2/s. The tumor was strongly and homogeneously enhanced during the arterial phase with continuous signal increase of mass in the venous and delayed phases (a persistent pattern). Normal prostatic glandular tissue with a non-enhanced capsule around the mass was clearly distinguished from the tumor as a homogeneous isointense signal compared to the muscle. No central necrosis was demonstrated in the tumor.
In addition, a low signal intensity area on T2WI showed significantly higher signal nodule on ultrahigh b-value DWI, and the mean ADC value was 0.517 ± 0.015 × 10−3 mm2/s. It was found in the left transition and peripheral zone. The tumor showed clear enhancement with an irregular boundary and without capsule on DCE (Fig. ). At the same time, prostate benign hyperplasia nodules were also found in the middle area of right transition zone, and these features were similar to the prostatic leiomyoma observed on T2WI, T1WI and DWI. The mean ADC value was 0.710 ± 0.049 × 10−3 s/mm2. The nodular was enhanced heterogeneously during the arterial phase with continuous signal increase of mass in the venous and delayed phases (a persistent pattern). There was no capsule. The preoperative MRI diagnosis revealed prostatic cancer with benign prostatic hyperplasia; however, the prostatic leiomyoma was not considered at the initial diagnosis.
Ultrasound guided systematic transperineal prostatic biopsy and subsequent histological analysis of biopsy samples confirmed prostatic cancer in the left zone (Gleason score 3 + 3 = 6).
A robot-assisted laparoscopic radical prostatectomy was performed to remove the whole prostate. In addition, histopathological examinations revealed leiomyoma based on following features: firstly, the tumor was composed primarily of hyalinized connective tissue containing spindle cells; secondly, spindle cells were arranged with a fascicular and whorled manner in foci. The cancer was found in peripheral and transitional zones of the left posterior lobe.


A 9-year-old male of black African ethnicity was brought by his parents to our primary care hospital because for 2 days he had been experiencing intermittent sudden perceptions of his parents’ heads either “shrinking” or “expanding” although he could still recognize their faces. He also had the same perception of objects around him. The visual perceptions were usually brief, resolved spontaneously and did not involve confusion in colours or individuals. For fear of a magico-religious origin of the illness, his parents took him to a traditional healer where he was given various herbal concoctions to drink for 2 days but there was no regression of the symptoms.
It is worth mentioning that 1 week prior to the onset of the visual symptoms the boy had developed an intermittent high grade fever which persisted in spite of the oral antimalarial (arthemeter and lumefantrin combination) and antipyretic medications (paracetamol and ibuprofen) which his parents gave to him at home. The fever was associated with general weakness, loss of appetite and severe polyarthralgia.
The review of systems revealed severe headache and postprandial vomiting. The child did not have abnormal auditory or tactile perceptions. This was his first time of having visual problems. He was diagnosed with and treated for severe malaria at the ages of 2 and 6 but visual disturbances were not reported on these occasions. His past history was otherwise without particularity.
On admission, the boy still experienced the visual distortions. Clinical examination revealed an extremely lethargic patient with pale conjunctivae and icteric sclerae. His Glasgow coma score was 15/15 and he was oriented in person, place and time. His vital signs were: blood pressure: 70/50 mmHg, pulse: 117beats/min, respiratory rate: 29breaths/min, and temperature: 36 °C. There was mucocutaneous pallor. Neurological examination revealed generalized hypotonia but there were neither focal neurological nor meningeal signs. Head and neck examination as well as the rest of the physical examination was without notable findings as well.
Severe malaria was suspected. Typhoid encephalopathy and hepatic encephalopathy were considered as differential diagnoses. A series of laboratory tests were then requested:To confirm the diagnosis of malaria, a thick blood film analysis was done which was remarkable for hyperparasitaemia at 277,000 trophozoites/μl (obtained from the product of the number of parasites counted per 100 white cells on the thick blood smear and the number of white cells per microliter of blood), A full blood count revealed a haemoglobin value of 8.3 g/dl (moderate anaemia) and a platelet count of 101,000 platelets/mm3 (moderate thrombocytopenia) The random blood sugar was 50 mg/dL (moderate hypoglycaemia) The Widal test was used to check the possibility of typhoid and the titre was weakly positive. Hepatitis B and C antigens were negative and liver enzymes were within normal limits
In view of prostration and hyperparasitaemia, the boy was diagnosed with severe malaria. The patient was placed on quinine 300 mg in 300 ml of glucose infusion to flow over 4 h and to be repeated 8 hourly. He was also placed on intravenous paracetamol at a dose of 450 mg 6 hourly. Two hours after the first quinine and glucose infusion, he had an episode of generalized tonic-clonic seizures that were arrested with slowly administered intravenous diazepam. Meningo-encephalitis was considered as a possible aetiology of the neurological problems. Subsequent lumbar puncture and cerebrospinal fluid analysis were without particularity. The quinine and glucose infusions were continued for 5 days and the clinical progress was good. Oral relay of the antimalarial drug was done with Quinine tablets at a dose of 900 mg daily in three divided doses for 5 days. The patient was discharged after 8 days of hospitalization and reviewed on out-patient basis. A repeat of the thick blood film analysis 2 weeks after discharge was negative for malaria parasites. The visual symptoms which were experienced by the boy were suggestive of AIWS and they had completely resolved after the successful treatment of malaria. No further visual abnormalities were reported during 3 months of follow-up and reassurance.


A 46-year-old woman came to the dental practice in order to change her veneers to improve her smile. The patient had feldspathic ceramic veneers from 1.3 to 2.3 for 28 years. Clinical examination revealed intense tetracycline discoloration (grade IV), gingival retraction, color change of the veneers, the ceramic of 1.1 and 1.3 was cracked and incisal wear in antero-inferior teeth. Radiographic exploration, extraoral and intraoral photographs were performed and preliminary impressions were made for the preparation of the study models.
Treatment plan consisted on veneers from 1.6 to 2.6. Premolars and first molars were included because its dark color intensifies the width buccal corridors. Besides, restorations with veneers from 3.3 to 4.3 were considered due to tetracycline stains and incisal wear.
Firstly, the veneers were removed and the tooth color was selected from the Vitapan 3D Master guide (Vita Zahnfabrick, Bad Säckingen, Germany). Mock up was made to evaluate aesthetic, phonetic and functional parameters. Teeth were prepared through the mock-up in order to reduce the minimal amount of dental tissue posible. (868B 314 020, 881 314 010 y 881 314 016, Komet, Germany). Then, opaque dentine mass of microhybrid composite (Esthet-X A2O, Dentsply Sirona, Konstanz, Germany) was placed to mask the most intense stains. This technique allows the reduction of hyperchromacity and gradually increase the color value of discolored teeth (). Afterwards, the final dental color was selected by the Vitapan 3D Master guide. The glycerine gel was placed to avoid the oxygen-inhaled layer of the composites. The definitive impressions were taken with silicone (Aquasil Ultra XLV, Dentsply Sirona, Konstanz, Germany) to fabricate conventional feldsphatic ceramic veneers (Noritake kisai Co, Ltd. Japan) by the parallel stratification masking technique. For cementing, an opaque cement was selected from the try-in tests (Calibra, Dentsply Sirona, Konstanz, Germany). The composite was silicatized by an air abrasion with a pressure of 2.5 bars from a distance of about 10 mm for 5 seconds. Next, enamel and dentin were etched with phosphoric acid (Detrey® Conditioner 36, Dentsply Sirona, Konstanz, Germany) and adhesive was applied (Prime & Bond® NT, Dentsply Sirona, Konstanz, Germany). On the other hand, the feldspathic veneers were prepared as follows: etched with 9% hydrofluoric acid for 2 minutes (Ultradent, Ultradent Products. Inc.), immersed in an ultrasonic bath with distilled water, silane (Calibra, Dentsply Sirona, Konstanz, Germany) for 2 minutes heated with a dryer and a thin layer of adhesive (Prime & Bond® NT, Dentsply Sirona, Konstanz, Germany). Finally, the cement was placed inside the veneers and placed on the teeth blandly. The palatal and buccal faces were polymerized for 40 seconds each one. The cement remnants were removed and the occlusion was checked, (Figs. -).


In January 2013, an 11-year-old female patient with a chief report of swelling in the left mandibular molar region was referred to our medical center. Two weeks before the first visit, the patient was prescribed antibiotics by a local family medical clinic because of swelling in the left side of the jaw and tenderness of the mandibular angle. For these reasons, she was referred from a local dental clinic and consulted our center. The patient had no systemic health conditions. Extraoral examination revealed swelling in the left mandibular angle area, and intraoral examination revealed mucosal swelling that extended from the lower first molar to the retromolar region, mixed dentition, and an unerupted left mandibular second molar.
A panoramic radiograph revealed a well-circumscribed, unilocular radiolucency in the region of the left mandibular molars, extending from the distal root of the first molar area to the left ascending ramus, with an unerupted second molar, and the dental follicle of the third molar (Fig. ). The impacted left mandibular second molar presented with incomplete root formation and displacement up to the inferior border of the body. The left mandibular third molar was located on the coronoid process of the mandible. Considering the clinical and radiographic findings, a provisional differential diagnosis of UA, dentigerous cyst, and odontogenic keratocyst was considered. With the patient under local anesthesia, an incisional biopsy was conducted; after histopathologic evaluation, the lesion was diagnosed as UA. The parents were informed of the condition, proposed treatment, recurrence, and benefits, after which they provided their informed consent for the conservative treatment. Marsupialization of the gingiva and cystic wall was performed using a silastic drain to maintain continuity between the marsupialized lesion and oral environment and to ultimately reduce the lesion size. The patient was scheduled for follow-up and daily management for the first month, followed by weekly management. The parents were instructed to maintain overall proper hygiene of the oral cavity through self-irrigation after marsupialization. After 1 month, a propensity of the lower left second molar toward eruption was observed (Fig. ). After confirmation of epithelization of the cystic wall, the drain was removed. Three months after marsupialization, the lesion diminished in size, new bone formation was observed, and the lower left second molar was in a more advanced phase of eruption (Fig. ). Radiographs obtained 6 months after marsupialization showed that the lesion margin had lost clarity and that the regenerated bone was replaced by normal trabeculae. The radiolucent area was significantly reduced (Fig. ). Twelve months after marsupialization, a part of the lower left second molar was observed in the oral cavity (Fig. ). Thirty months after marsupialization, the lower left second molar erupted on the same occlusal plane as the lower right second molar. The lower left third molar was impacted. In the following month, enucleation of the lesion was performed to completely remove the lesion along with the impacted third molar with the patient under general anesthesia (Fig. ). The second molar was preserved. The patient began orthodontic treatment for reduction of mild crowding. At 36 months after marsupialization, radiographs showed complete eruption of the lower left second molar, whereas a mild interdental space was observed between the first and second molars. At 48 months after marsupialization, complete occlusion was observed. This tooth was eventually preserved and did not require a root canal (Fig. ). To date, no evidence of tumor recurrence has been observed on examinations during the 49-month follow-up.


The patient is an 80-year-old female with a history of recurrent abdominal pain. Permission was obtained from the patient to allow discussion and publication of his case. The patient had a history of coronary artery disease, atrial fibrillation (on anticoagulation), multiple cerebral vascular accidents, chronic obstructive pulmonary disease, an aortic and mitral valve replacement, and a prior hysterectomy. The patient was noted to have presented to the hospital three times in the prior month with similar complaints of vague, diffuse abdominal pain that would last a few hours and resolve. The patient did note nausea and vomiting with these episodes. The patient re-presented to the emergency room, where a CAT scan was performed () which showed mesenteric swirling secondary to possible internal hernia. The patient underwent a small bowel follow through with gastrografin, which demonstrated no abnormalities. The patient then underwent an obstructive series that was also noted to be normal. The patient's symptoms, however, did not subside. Due to the chronicity and the unresolving symptoms, the patient was brought to the operating room after a long discussion of possible outcomes with the patient.
The operation was started laparoscopically but was soon converted to an open laparotomy due to a large mass of small bowel swirled onto itself that was adhered together (). In mobilizing the small bowel, blunt dissection was used to lyse adhesions and untwist the mesentery. The small bowel mesentery was observed, and no mesenteric defects were noted. It was seen that the mesentery had swirled and twisted upon itself (). While running the bowel, a large, proximal jejunal diverticulum was present. The diverticulum was excised using a 60 mm stapler. The bowel was run from the ligament of Treitz down to the ileocecal valve with no mesenteric defects noted, and all bowel was viable. The small bowel was returned to the abdomen. The patient did well postoperatively and was seen in follow-up without complication.


A 74-year old female farmer had injured her shoulder against the side view mirror of a moving car. After the accident, she was unable to lift her left arm due to shoulder pain and attended our hospital. On physical examination, a deformity was observed on the left clavicle. Standard radiograph revealed a fracture at the distal end of the left clavicle (). Three-dimensional computer tomography (CT) scan revealed in addition a displaced fracture of the proximal end of the clavicle, and a diagnosis of bipolar segmental clavicle fracture was made (). The proximal and distal displaced fractures were extra-articular (Robinson Type 1B1) and intra-articular (Robinson Type 3B2), respectively. The proximal end of the clavicle was displaced anteriorly and the distal end posteriorly (), with floating intermediate fracture fragments. This indicated fracture instability and a high risk of non-union. Therefore, we decided to treat with open reduction, and it was performed seven days after injury with the patient’s consent.
The patient underwent surgery under general anaesthesia in the beach chair position. A skin incision was made over the distal fracture site. The distal end of the clavicle was displaced posteriorly. The floating bone fragment was found rotated opposite to the direction of the displacement. As the distal end of the clavicle was reduced, the proximal end was simultaneously reduced to a good anatomical position. A 3-hole locking plate [LCP™ Superior Clavicle Plate, DePuy Synthes Trauma, West Chester, USA] was used to provide fixation between the distal end of the clavicle, as the intermediate floating fracture fragment was secured by more than two screws to prevent rotational displacement. The proximal clavicle fracture was left alone to avoid any critical complications.
After the operation, the patient’s shoulder was placed in a sling and allowed passive range of motion of the shoulder below 90 degrees for one month as the proximal clavicle fracture was not surgically fixed. Gradually active movement of the shoulder was allowed as tolerated. Four months after the operation, the patient was able to elevate her shoulder forward up to 160 degrees. Radiographs and CT scans revealed bony union of both proximal and distal ends of the fracture ( and ). Because of discomfort due to implant irritation, the implant was removed one year after surgery and the symptoms diminished (, and ). Three years post operatively, the patient had neither pain nor crepitation in both her proximal and distal fracture sites. The ROM of shoulder was 165 degrees in anterior elevation and 80 degrees in abduction. The Oxford shoulder score was 47 points and ASES shoulder score was 91.6 points. The Quick DASH score was 5.0 points, demonstrating a better score than previous literatures (8 points without complication case, 66 points with complication case). The patient has continued her activities as a farmer.


A 76-year old woman had sustained a fall from standing position, resulting in dislocation of her left elbow. She presented at our centre within an hour of injury, with left elbow tenderness and deformity. The hand was warm and fingers were pink with capillary refill time (CRT) less than 2 seconds. Radial and ulnar pulses were not palpable but weak signal was detectable by Doppler ultrasound. Pulse oximetry over the fingers ranged from 80-90%. Sensation and gross motor function were intact. Radiographs () showed a posterolateral dislocation of the elbow. Under monitored sedation, a closed reduction was performed and stability of joint was assessed, revealing no instability within the entire flexion extension arc; no valgus instability but slight varus laxity was noted. The elbow joint was immobilized with forearm in neutral position and 45 degrees flexion with a backslab, and was increased to 90 degrees flexion once swelling subsided. Post-reduction radiographs () showed a concentric reduction of the elbow joint. After reduction, pulses were not palpable but detectable by Doppler, pulse oximetry over fingertips rose to 100%. Neurovascular status remained the same while patient was in the ward.
Four days later, she was re-examined and it was noted that the distal pulses were still not detectable by Doppler device, even though fingers were pink with CRT <2 seconds. A CT Angiogram revealed abrupt termination of contrast in the brachial artery about 3.7cm from elbow joint, with the non-visualized segment measuring 4cm, with no leakage of contrast. Reconstitution of the brachial artery was noted just distal to the elbow joint. There was very faint contrast opacification of the radial and ulnar arteries. CT angiogram suggested possible thrombosis of brachial artery. After consultation with a vascular surgeon, she was planned for non-surgical management. She was started on subcutaneous enoxaparin which was given for a week, followed by lifelong Aspirin 150mg OD. The patient was discharged from ward ten days post trauma.
At one month follow up, the fingers were pink with CRT <2 seconds, sensation and power intact. Distal pulses were still not palpable. Slab was removed; elbow range of motion was 15 to 100 degrees.
At three months follow-up, she had no complaints of ischemic pain or cold intolerance. Circulation appeared good, distal pulses were palpable although slightly weaker than the contralateral side; range of motion was comparable to contralateral limb with no valgus or varus laxity. There were no signs of Volkmann’s ischemic contracture. Radiographs revealed concentric reduction ().


A 41-year-old white European woman presented to our clinic with a 7-year history of unexplained progressive unilateral left thigh pain during cycling, starting at moderate intensity and increasing upon maximal exertion. She had been a spinning instructor for 13 years, with an average of 5 hours per week, before onset of symptoms. In addition, she worked as a firefighter, where she experienced identical complaints during rapid actions, like climbing the stairs. The patient reported a sensation of unilateral muscle fatigue and pain in the left thigh, starting at the vastus medialis and spreading all over the quadriceps, adductors, and biceps femoris, without symptoms in the gluteal region or calf. Complaints vanished within 3 minutes after cessation of exercise and were highly reproducible. There was no history of trauma, while her medical history included right-sided congenital hip dysplasia, primary Raynaud's phenomenon, and attention deficit hyperactivity disorder (ADHD), for which she used clomipramine for 10 years. She was on no other drug therapy at time of presentation and had no history of smoking, diabetes, hypertension, hypercholesterolemia, peripheral artery disease, or coronary heart disease. Family history was positive for familial hyperhomocysteinemia, but this was never diagnosed in her.
The woman presented with a height of 172 cm and body weight of 65 kg (body mass index of 22 kg/m2). Her resting blood pressure was 120/75 mmHg with a heart rate of 61 beats per minute (bpm). Musculoskeletal investigation showed normal back mobility and a full range of motion of hips and knees with normal muscular strength of both legs. In addition, neurological examination was normal in our patient.
Admission laboratory findings included complete blood count, which was unremarkable (). In addition, haemostatic tests, including activated partial thromboplastin time (aPTT), prothrombin time (PT), INR, fibrinogen, D-dimer, DNA testing for Factor V Leiden and prothrombin G20210A carriership, protein C activity, activated protein C resistance, and free protein S level, were all normal, while no lupus anticoagulants or anti-cardiolipin antibodies were detectable.
Transthoracic echocardiography showed normal anatomy with normal left ventricular ejection fraction (LVEF) and systolic and diastolic volumes but suggested a slight increase in total peripheral vascular resistance ().
Since symptoms appeared during exercise, a maximal treadmill exercise test was obtained with increasing resistance, starting at 100 Watt and elevating 15 Watt every minute, until exhaustion. Complaints started at 130 Watt and the test was ceased at 260 Watt due to unilateral thigh pain and muscular fatigue. The exercise electrocardiogram (ECG) showed no abnormalities and systolic pressures were obtained before and after exercise from the left brachial and bilateral calf for ankle-brachial index (ABI) calculation. The ABI before exercise was 1.1 on both sides, whereas directly after exercise ABI was reduced for both legs (right: 0.75; left: 0.59), where no alterations upon exercise were expected in a healthy young woman [] ().
Prompted by bilateral reduced ABI after exercise and the suggested increased vascular resistance, further vascular examinations were performed. Normal lower limb pulses without audible murmurs and a normal capillary refill (<2 sec) were found in both legs. To investigate whether this patient suffered from endofibrosis, resting arterial duplex ultrasonography was done, which revealed no stenosis and normal diameters of the external iliac artery and common iliac artery, where femoral arteries appeared to be relatively wide, as might be expected in well-trained individuals [] (). In addition, femoral intima-media thickness was within normal range (0.78 mm, reference cut off: <0.9 mm), accompanied by normal peak systolic velocity during hip extension (1.41 m/sec) and flexion (1.54 m/sec) []. At the venous level, no signs of (previous) deep vein thrombosis were observed.
Additionally, obstructive vascular pathologies were investigated by multiphase computer tomography angiography (CTA), showing no signs of intraluminal pathologies or aberrant morphology, thereby corroborating the duplex examinations and excluding endofibrosis, popliteal entrapment syndrome, arterial-venous shunts, and venous malformations.
Next, we evaluated arterial function by ultrasonic examination of carotid and brachial artery and tonometric measurement of carotid-femoral pulse wave velocity (cfPWV). Carotid intima-media thickness (0.68 mm, reference cut off: <0.9 mm []) and cfPWV (6.74 m/s, reference cut off: <10 m/s []) were unremarkable. However, carotid distensibility coefficient was lower than expected (16 × 10−3/kPa, reference value: 20–30 × 10−3/kPa) [].
Brachial flow-mediated dilation (FMD, was 1%, reference value: 3.0–8.4% []) and nitroglycerine-mediated dilation, 8%, (reference value: 9.6–18.0% []) were markedly reduced. Microcirculatory studies showed normal capillary density before (74/mm2, reference value: 40.4–85.6/mm2) and after (109 mm2, reference value: 69.5–117.1/mm2) venous congestion []. Furthermore, heat-induced cutaneous microvascular dilatation was within normal limits (+1356%, reference value: 324–1762%).
Based on the symptoms and test results, we postulated impaired arterial vasodilation and therapy with isosorbide mononitrate 30 mg twice daily was started, without any effects on the symptoms. This appeared to exclude a lack of nitrous oxide as major underlying defect. In another approach, we prescribed a vasodilating agent, the long-acting calcium antagonist diltiazem 200 mg once daily, through which claudication symptoms were well controlled and blood pressure remained within normal range (24 h measurement: average 110/80 mmHg) without any symptoms of orthostasis. During 2-year follow-up, she experienced no side effects and was able to continue her work as a fire fighter and spinning instructor.


The patient was a 25-year-old female born to non-consanguineous parents. She was the second child of a family with one brother and two sisters. Her initial symptom began when she was 12 years old and presented as difficulty in climbing stairs. Her weakness was slowly progressive with the wasting of the proximal leg muscles so that she lost her ability to run. After a few years, she also felt weak in her shoulders and arms but to a lesser degree than her legs. She did not have any sensory or sphincter complaints. Her swallowing was normal. A family history of muscle weakness was positive in her grandmother, but it was not debilitating enough to look for a cause. One of her aunts had MS. Her history was negative for alcohol and smoking. She only took multivitamin pills temporarily. On examination, her body-mass index (BMI) was 23 and she was afebrile with no abnormal findings on physical examination of the skin, mucous, head, and neck. Cardiopulmonary and abdominopelvic examinations showed no abnormalities. She was fully obedient and her mental functions were intact with normal speech. An examination of her cranial nerves showed no facial or bulbar muscle involvement. Muscle weakness and atrophy was symmetrical, prominent in the pelvic and shoulder girdle sparing oculofaciobulbar muscles.
The muscles had a normal tonus without any evidence of contracture or calf hypertrophy. She had good strength in her axial muscles (5/5). Weakness was more obvious in the lower limbs than in the upper limbs (3/5 and 4/5, respectively). The power of her distal muscles, wrists, and feet was preserved. Her tendon reflexes diminished proportional to their weakness. Plantar reflexes were down bilaterally. Sensory examination was normal. Laboratory tests performed at that time showed an increased level of transaminases (ALT: 72 U/L, AST: 55U/L) and CPK 10000 U/L. Cardiologic consultation revealed no significant cardiac abnormalities.
The first electromyography (EMG) (2005) showed a myopathic pattern in the lower limbs from which a muscle biopsy was requested but the patient postponed it. Until the patient was 20 years old, her symptoms were progressive; then, she experienced a stabilization of the disease. A biopsy taken in 2011 revealed dystrophic changes (degeneration/ regeneration) with markedly increased endomysial connective tissue; no inflammatory cell infiltration, ragged red fiber, cytoplasmic body and lipid or glycogen accumulation in muscle fibers were seen (). An immunohistochemistry (IHC) study of sarcolemmal proteins showed sarcolemma labeling of all fibers with DYS1, DYS2, DYS3, alpha and gamma sarcoglycan, dysferlin, merosin, and beta-spectrin ().
The second EMG in 2011 showed a myopathic process pronounced in the proximal muscles. With a diagnosis of LGMD, rehabilitation, physical therapy, and stretching exercises were advised to improve her mobility and prevent disused atrophy or contracture deformities. In April 2015, she complained of acute left visual loss for four days with painful eye movements that lasted for several days. Her muscle strength had not changed for 5 years. On examination of her pupils, the left one had 2+ relative afferent pupillary defects (RAPD). Left visual acuity was finger counting from one meter with cecocentral scotoma in the visual field. Extraocular muscles and other cranial nerves were intact. Ophthalmologic consultation revealed no bulbar ophthalmic problems. For further evaluation, we planned a brain and optic pathway MRI that excluded compressive lesions of the optic pathway and disclosed multiple periventricular, centrum semiovale, corpus callosum, juxta cortical, and infra tentorial (brain stem and cerebellum) T2 hypersignal demyelinating plaques with multiple lesion enhancement () that fulfilled the McDonald criteria for MS (, ).
From this, we prescribed intravenous methyl prednisolone (5 gr divided in 5 days) for the treatment of her optic neuritis. She responded significantly and her visual acuity ameliorated so that she could read from 30 cm. Vasculitis tests and anti-aquaporin 4 antibodies were negative. The final diagnosis was relapsing-remitting MS (RRMS). For long term treatment, we decided to put her on a glatiramer acetate daily injection.


We are presenting an 84-year-old male patient who appealed with a painless, left-sided scrotal swelling longer than 2 months. The right testis was normal and no inguinal palpable lymph node was detected. Also, there was no history of previous surgical intervention or trauma to this region reported by the patient. An ultrasonographic evaluation demonstrated irregular thickening and well vascularized, multiple papillary solid lesions of the tunica vaginalis. Testicular tumour markers, alpha-fetoprotein (AFP) and beta human choriongonadotropin (B HCG) levels were within normal ranges. A computed tomography (CT) of the pelvis, abdomen and thorax showed no sign of metastasis to solid organs to lymph nodes. Although a normal level of tumour markers was detected, ultrasonographic examination results caused an inguinal exploration. The skin was incised 2 cm above the inguinal ligament from the deep ring to the superficial ring and the inguinal canal was detected. After clamping the testicular cordal structures, tunica vaginalis was exposed from the adjacent structures. When it was incised vertically, multiple small papillary tumours, both on the tunica vaginalis and the tunica albuginea, were detected (Figure ).
According to these intraoperative findings, a radical orchiectomy was performed. A pathological gross examination of the specimen showed multiple, papillary, tan/white-coloured nodulations on the parietal and visceral leaves of the tunica vaginalis, with an average size of 0.5-1 cm. Similar papillary masses on the outer surface of the tunica albuginea were also found. Macroscopically, the testis showed the normal structure of the parenchyma and no tumour invasion to the tunica albuginea was determined. A microscopic evaluation showed a solid and nesting pattern, which was composed of atypical epithelioid cells with clear and eosinophilic cytoplasms. The nuclei of the tumour cells were pleomorphic and hyperchromatic, with prominent, big nucleoli (Figure ).
Immunohistochemically, tumour cells were extensively positive with calretinin and creatine, focally positive with epithelial membrane antigen (EMA) and showed a focal nuclear reaction with WT-1. But, it was negative with vimentin, cytokeratin (CK5), carcinoembryonic antigen (CEA) and B72.3 (Figure ).


An 88-year-old man with a Mobitz type II heart block was treated with a dual chamber pacemaker placement with active fixation leads. Post permanent pacemaker implantation chest x-ray showed appropriate lead position, and the electrocardiograph (EKG) showed a paced rhythm. The patient was discharged home and was able to continue with his daily activities without limitations until the day of presentation.
On the 12th day after the procedure, the patient developed chest pain. The pain was right-sided, non-radiating, and aching in character. This pain was described to be worse with inspiration. The patient denied palpitations, dizziness, nausea, syncope, or fatigue.
In the emergency department, his vitals were stable, and initial laboratory investigations revealed electrolytes to be within normal limits; cardiac troponin T was negative and the EKG showed intermittent lead capture. A chest X-ray revealed a left chest wall cardiac pacemaker device with the leads overlying the right atrium and right ventricle, and small bilateral pleural effusions. No pneumothorax was identified. Echocardiogram performed at bedside showed a small pericardial effusion, new compared to the study done on previous admission. There was no chamber compression, and the ejection fraction was 56%-60%, same as the previous echocardiogram.
A high-resolution computed tomogram (HRCT) of the chest was performed, and it revealed migration of the right atrial lead into the right lung. The right atrial lead had penetrated through the right atrial appendage wall, and its tip was seen in the middle lobe of the right lung (Figures -). Additionally, there was a small right pneumothorax, a small anterior pneumo-pericardium, a small pneumomediastinum along the ascending aorta, and a small right-sided pleural effusion. The patient underwent urgent right atrial lead revision and tolerated the procedure well. Repeat echocardiogram a day after the revision procedure showed a resolution of the pericardial effusion. The patient's chest pain resolved post procedure and he was discharged from the hospital.


A 45-year old Sinhalese woman from Galle district in Southern Province of Sri Lanka presented to our hospital with a 2 week history of intermittent fever and severely painful left hip for 3 days duration. She had a history autoimmune hepatitis for 6 months, for which she had been on azathioprine 50 mg daily, and poorly controlled type 2 diabetes mellitus for 11 years.
Fever was intermittent, initially low grade and later high grade, which abated with sweating, but not associated with chills and rigors. She also had mild sore throat and cough with constitutional symptoms. She was initially managed with oral co-amoxyclav as for possible upper respiratory tract infection by her family doctor. However, her fever continued and around 3 days prior to the admission, she has noticed severe pain in her left hip joint which has caused her to be increasingly bedbound and restricted left hip movement. Apart from these symptoms, she did not have involvement of other joints or haemoptysis. There was no previous history of tuberculosis or close contact with a patient with tuberculosis. She was a field officer, and had no exposure to soil; however, she occasionally had engaged in gardening.
On examination, she was ill looking and had a temperature of 40 °C. Examination revealed severely restricted movements of left hip. The joint was mildly warm to touch but there was no obvious swelling over it. Initial laboratory investigations revealed raised peripheral white cell count of 12.4 × 109/L with 82% polymorphonuclear leukocytes, hemoglobin of 9.1 g/dL and a normal platelet count. Both erythrocyte sedimentation rate (65 mm/1st h) and C-reactive protein (86 mg/dL) were elevated. Initial radiograph of left hip joint was normal, but subsequent x-ray which was taken 1 week later showed a narrow joint space and destruction of femoral head (Fig. ). Ultra sound scan of hip revealed small effusion with echogenic particles. MRI of hip was also suggestive of septic arthritis (Fig. ).
After taking blood cultures, she was commenced on empirical antibiotic therapy with ceftriaxone IV 1 g 12 hourly and vancomycin IV 1 g 12 hourly. Despite antibiotic therapy, high fever spikes were continued and the clinical condition deteriorated. Repeated blood cultures and urine cultures revealed negative results. Joint fluid aspiration was failed since there was only a minimal amount of fluid. Further investigations including Mantoux test and sputum for acid fast bacilli (AFB) and mycobacterial culture were negative. Transthoracic 2D-echocardiogram did not show vegetations. Chest x-ray, ultrasound scan of abdomen and subsequently contrast CT scan of chest and abdomen did not reveal any significant abnormality such as visceral lymphadenopathy or abscesses. Autoimmune panel including ANA, ANCAs and serology for other less common infective causes like HIV, toxoplasmosis, and brucellosis were also negative.
Due to her immunosuppressive state and unresponsiveness to empirical antibiotic regimen, possibility of uncommon infective conditions was considered at this stage. One of the conditions that we considered was melioidosis since it is known to cause isolated septic arthritis in susceptible patients. Since the blood cultures were repeatedly negative, we performed antibodies to B. pseudomallei, which came positive with a titre of 1/10240 by Indirect Haemagglutination Assay (IHA). The presumptive diagnosis of melioidosis was made based on clinical and microbiological grounds. Definitive treatment was started with intravenous imipenem 1000 mg 6 hourly and oral co-trimoxazole (1920 mg 12 hourly). The patient responded well to the above treatment with fever settling within 72 h of starting treatment. While in the ward, she had intensive physiotherapy to improve mobilisation. At the end of 3 weeks, inflammatory markers came down to normal range and she was discharged on oral cotrimoxazole for another 10 weeks with a plan to have weekly reviews with full blood count and CRP.


In January 2000, a 53-year-old post-menopausal woman underwent core biopsy of the left breast for microcalcifications detected on routine mammography. The results showed an invasive ductal carcinoma; immunohistochemistry showed estrogen receptor positivity in 85%, progesterone receptor positivity in 70%, HercepTest™ (Dako, Denmark) negative, and Ki-67 positivity in 20%. According to the 2000 guidelines, she underwent partial mastectomy and axillary dissection. The surgical specimen showed 1/18 lymph nodes positive for cancer, without penetration of the lymph node capsule (pathological stage T2N1M0). She underwent locoregional radiotherapy and received four cycles of anthracycline-cyclophosphamide-based chemotherapy. After completing treatment, she was started on tamoxifen, 20 mg/day, with a planned treatment duration of five years. In the posterior controls, there was no evidence of metastatic disease or local recurrence.
In December 2002, the patient presented at our center. There was no evidence of disease relapse using CT imaging, bone scan, and mammography. For the detection of minimal residual disease, blood samples for circulating tumor cells and bone marrow biopsy for micrometastasis were taken:
a) Detection of secondary circulating tumor cells: 8 mL of venous blood was collected in an EDTA (ethylenediaminetetraacetic acid) BD-Vacutainer® (Becton, Dickinson & Co., Franklin Lakes, NJ) and the mononuclear cells were obtained using differential gel centrifugation (Histopaque®-1077; Sigma-Aldrich Corp., St. Louis, MO). The cells were washed in phosphate buffered saline pH 7.4 (PBS), then re-suspended in 100 𝜇L of autologous plasma. Twenty-five 𝜇L aliquots were used to make silanized slides (Dako, Denmark), which were dried in air for 24 hours and then fixed in a solution of 70% ethanol, 5% formaldehyde, and 25% PBS for five minutes and finally washed three times using PBS.
Immunochemistry: Secondary CTCs were detected using a monoclonal antibody directed against mammaglobin (Novocastra Laboratory, Newcastle, UK) and identified using an alkaline phosphatase - anti-alkaline phosphatase-based system (LSAB2) (Dako, Denmark) with new fuchsin as the chromogen. Positive samples underwent a second process with anti-CD45 clone 2B11 and PD7/26 (Dako, Denmark) and were identified with a peroxidase-based system (LSAB2; Dako, Denmark) with 3,3′-diaminobenzidine-tetrahydrochloride-dihydrate (DAB) as the chromogen. A secondary CTC was defined as a cell that expressed mammaglobin but not CD45, according to the criteria of the International Society of Hematotherapy and Genetic Engineering (ISHAGE) []. A leukocyte did not express mammaglobin but expressed CD45. A test was considered positive for secondary CTCs when at least 1 cell/8mL of blood sample was detected; the number of CTCs detected/8 ml blood sample was registered.
b) Detection of bone marrow micrometastasis: A bone marrow biopsy was taken from the posterior superior iliac crest and the sample used to prepare four ”touch preps” using silanized slides (Dako, Denmark). The slides were processed in the same way as for CTCs.
The initial evaluation for minimal residual disease showed that the patient was positive for CTCs, with 5 cells/blood sample detected (Figure ) and positive for bone marrow micrometastasis (Figure ).
The results were interpreted as minimal residual disease positive with a high risk of disease progression. Her treatment was changed from tamoxifen to the aromatase inhibitor, letrozole, at a dose of 2.5 mg/day. The oral bisphosphonate, alendronate, was added at a dose of 70 mg once weekly.
After six months of treatment, the patient was reevaluated. There were no CTCs detected (Figure ).
The number of micrometastatic cells had significantly decreased, but the bone marrow was still positive (Figure ).
As such, the dose of alendronate was increased to 70 mg twice weekly and the letrozole continued at the same dose. One year later, there was no evidence of CTCs or bone marrow micrometastasis (Figure ), and treatment was continued at the same dose.
The follow-up evaluation in 2005 showed limited numbers of mammaglobin-positive cells in the bone marrow, but the patient remained negative for CTCs. There was no evidence of metastatic relapse on bone scan, CT scanning, or mammography. The alendronate was suspended and replaced for eight monthly cycles of intravenous zoledronic acid, 4 mg. After eight cycles of zoledronic acid, there was no evidence CTCs or bone marrow micrometastasis. The patient continued treatment with letrozole, 2.5 mg/day, until completing the recommended five years of treatment. The patient has remained negative both for CTCs and bone marrow micrometastasis to date; her last control was in 2016 with no evidence of minimal residual disease or evidence of metastases on imagining studies. The changes in treatment appeared to eradicate both circulating tumor cells and bone marrow micrometastasis.


A 66-year-old man underwent diagnostic colonoscopy because of fecal occult blood study positive twice. He had history of surgery for esophageal cancer. The esophagus and abdominal computed tomography (CT) scan (Fig. ) were normal before colonoscopy. Fecal occult blood study was positive. Complete blood count revealed a decreased RBC count (4.35 cells/L) with a borderline low hemoglobin level (127 g/L) and a slightly elevated HCT (red blood cell specific volume) (39.4%).
During the colonoscopy process, due to descending colon narrowed (Fig. ), colonoscopy could not be advanced from the anus 40 cm despite of trying twice. The scope was withdrawn and the injected air was removed.
Within 3 hours of colonoscopy, the patient complaints of abdominal distension, left epigastric pain, no nausea, and no dyspnea. X-rays showed several loops of dilated colon within the left upper quadrant (Fig. ). A CT scan of the abdomen was showed marked protrusion of the large bowel into the left thoracic cavity along with the elevation of the left diaphragm (Fig. ). It released diaphragmatic hernia occurred. Laboratory analysis revealed the following after colonoscopy: serum WBC 11.5 cell/L; RBC 4.11 cell/L; Hgb 114 g/L; HCT 35.3%; PLT244K; neutrophils 81.9%; monocytes 10.8%; CRP 71 mg/L; Saa 522.971 mg/L; total protein 48 g/L, pH 7.451; pCO2 36.1 mm Hg; pO2 71.2 mm Hg; HCO3 24.8 mmol/L.
An emergency operation revealed that most of the transverse colon and greater omentum had herniated through a “Y” shape diaphragmatic defect about 6 cm, without any gangrenous or strangulation changes. Abdominal extensive adhesions caused by esophageal cancer postoperative. There was about 300 mL of light red fluid in the abdominal cavity. The diaphragmatic defect was repaired by simple closure and intestinal adhesions release surgery. The bowel was withdrawn into the peritoneal cavity. The patient was discharged in good condition 5 days after the operation.


We report the case of a 58-year-old women, actually in post menopausal state, followed in our hospital for ABC, who underwent radical right mastectomy in 1994, when she was 35 year old, for a stage I breast ductal carcinoma, hormone receptor (HR) negative, human epidermal grow factor receptor 2 (HER2) negative. In 1997 a computed tomography (CT) scan showed multiple backbone metastases, confirmed at bone biopsy (breast ductal carcinoma, HR positive, HER2 negative), so she was submitted to a first line chemotherapy with epirubicina 75 mg/m2 plus paclitaxel 75 mg/m2 3 weekly for six cycles. The metastases were evident to the CT-scan, so she was not subjected to bone scintigraphy. Thereafter, she underwent a palliative radiotherapy on the 10th dorsal vertebra (30 Gray in ten fractions, 3 Gray for each fraction) and a maintenance anti-hormonal therapy with gonadotropin releasing hormone (LHRH) analogue and letrozole, a non-steroidal aromatase inhibitor (AI), then replaced with exemestane, a steroidal AI for intolerance, until June 2011.
In 2009 a chronic myeloid leukemia (CML) was diagnosed with a osteomedullary biopsy that showed overexpression of 180% of Breakpoint Cluster Region of Abelson gene (BCR-ABL gene). The patient started imatinib, which was suspended in May 2010 for resistance onset. So, she started dasatinib, a potent orally SRC family kinase (SFK) inhibitor, at a dosage of 100 mg daily.
In July 2011, CT scan showed multiple lesions in different hepatic segments with maximum diameter of 2 cm at CT scan (Fig. ); so a liver biopsy was performed and the histology confirmed the diagnosis of breast ductal carcinoma, HR positive, HER2 negative.
In August 2011, she started a second line chemotherapy with nab-paclitaxel 260 mg/m2 3 weekly supported with granulocyte colony stimulating factor (GCSF); stopping dasatinib for 6 days during the administration of GCSF, as for hematological counseling. The treatment has been continued for 16 cycles with a stable disease (SD) as a best response. In October 2012, after a total of 14 months of therapy, a CT scan showed liver progression with the appearance of a 11 mm new lesion in IVb segment (Fig. ), thus she started a third line chemotherapy with vinorelbina 60 mg/m2 on day 1, 8 plus capecitabine 1000 mg/m2 twice daily from day 1 to day 14 every 21 days.
In March 2013, after eight cycles, the CT scan showed a liver partial response and after another four cycles a further reduction. The major toxicities occurred were grade 2 neutropenia and diarrhea. Hence, capecitabine dosage was reduced by 25%.
In January 2014, after 17 cycles, the CT scan showed a complete response of liver metastases (Fig. ) and bone SD.
In May 2016, after 43 months, a 18-fluorodeoxyglucose positron emission tomography (18-F-FDG PET) scan, performed due to contrast allergy onset, showed a metabolic response of bone metastases without other site of disease.
Currently, these treatments (capecitabine plus vinorelbine and dasatinib) are still ongoing with a PFS of 54 months and good tolerance.
The CML is in complete molecular response and the molecular examination of peripheral blood showed no expression of BCR-ABL gene.


A 24-year-old male was admitted to Peking Union Medical College Hospital, Beijing, China, on 13 May 2015 because he had been experiencing intermittent fever with chills and fatigue for six months. Antibiotic therapy (lack of detailed information) prescribed by the community hospital was completed before he came to our hospital, but the symptoms persisted. On physical examinations, the patient was revealed to be healthy except for an unstable temperature with intermittent fever, hypotension [103/38 mmHg] and weight loss of 15 kg during the course of illness. Laboratory tests revealed that the patient had an increased erythrocyte sedimentation rate (ESR) of >140 mm/h and suspected anemia with Hb of 57 g/L. His symptoms were slightly alleviated after a blood transfusion with 2 units of condensed erythrocytes. Cardiac auscultation revealed consecutive murmurs in the second aortic valve area. Echocardiography was prescribed to determine the cause of the murmurs. Congenital heart disease (CHD) of patent ductus arteriosus (PDA) and IE were diagnosed with evidence of aortic regurgitation and multiple vegetations on the pulmonary valve and pulmonary artery wall. Based on the above evidence and the current physical condition, the patient was hospitalized in the cardiac surgery department on 26 May, and a cardiac surgical operation was performed to rectify the CHD and IE condition on 1 June 2015. A peripheral blood sample was sent to microbiology laboratory for blood culture testing on 26 May and was positive; the pathogen from the blood culture was revealed to be a novel species of the family Flavobacteriaceae on 6 June. Empirical treatment with ceftriaxone (IV, 2 g, qd) for 2 weeks during his hospitalization was prescribed. The patient recovered well after the operation and was discharged on 10 June 2015 with prolonged antibiotic treatment with ceftriaxone for 5 weeks. Since he was discharged, no significant clinical events have occurred during follow-up.