A 4-year-old previously healthy White male presented with a 4-day history of fever (103°F) and non-bilious, non-bloody vomiting. Prior to admission, he received intramuscular penicillin for a positive rapid Streptococcus pyogenes antigen test, but he continued to have poor oral intake, increased irritability, and fever. During hospitalization, his abdomen became distended. Abdominal computed tomography showed small bowel distension without obstruction and wall thickening with mucosal enhancement of the rectosigmoid colon. His stool tested positive via PCR for toxigenic C. difficile and he was treated with oral metronidazole. During the hospitalization, he then developed bilateral nonpurulent conjunctivitis, an erythematous rash on his upper extremities, and swelling of toes and fingers. Elevated inflammatory markers and low albumin were noted (). Echocardiogram showed a small pericardial effusion with normal coronary arteries. He was diagnosed with KD and given high-dose IVIG and aspirin on HD 6. He was discharged home after 2 days with resolution of symptoms and decreased C-reactive protein (CRP) at 7.8 mg/dL. Three days after discharge, irritability and fever (101.9°F) recurred. CRP was 5.3 mg/dL, hemoglobin 7.8 g/dL, and platelets 569,000 cells/µL. Repeat echocardiogram demonstrated the left main coronary artery to be 3 mm (upper limit of normal) with continued effusion. He received a second dose of IVIG. Two weeks later, repeat echocardiogram showed the left main coronary to be dilated at 3.5–3.8 mm (Z score 3.84) with CRP even higher at 8.3 mg/dL. A third dose of IVIG was given. Echocardiogram 3 months later revealed normal coronary arteries and no pericardial effusion.


In 2009, a 42-year-old non-smoking male presented to our hospital for the evaluation of a right lung nodule detected during physical examination. He underwent radical resection of the right lung, and the postoperative pathological report revealed a mucoid adenocarcinoma of the inferior lobe of the right lung (3.5 × 3.0 cm), but no positive lymph nodes (0 of 29; T2aN0M0 stage I). The patient relapsed in 2015. A computed tomography (CT) scan revealed a mass in the superior lobe of the right lung and mediastinal lymph node enlargement. He underwent resection of the residual right lung. Postoperative pathological evaluation and hematoxylin and eosin staining showed different histomorphologies in the primary lung site (mucinous type) (Fig a) and the fourth group of lymph node metastases (solid type, T2N2M0 stage III) (Fig b). Immunohistochemistry indicated a pulmonary origin of both tumors: TTF-1 and Napsin A were positive, and CK 5/6 and P63 were negative. We simultaneously detected ALK and EGFR gene status by reverse transcription-PCR (Amoy, Xiamen, China) in two tumor tissue specimens. The results indicated a positive EML4-ALK rearrangement in tissues and wild EGFR (Fig ). The patient received adjuvant chemotherapy and achieved stable disease.


We describe the case of a previously healthy, 31-year-old woman, who presented in the last trimester of her pregnancy with pain between the shoulder blades. She was admitted to the obstetrics department because of this pain, and the neurology department was consulted. The pain had started in the sixth month of her pregnancy with her first child. The pain arose each night 2 h after she went to sleep, and it was described as unbearable. Our patient would wake up every night because of the pain. The pain was not correlated with breathing. There was no pain radiating to her arms or legs. At the first neurological examination, no abnormalities could be found, and no final diagnosis was made. Muscle and joint complaints were thought to be the most likely explanation. After childbirth, she presented to the outpatient clinic. The pain had spontaneously decreased. She still experienced pain, mainly at night, but sometimes also during the day. She still reported no pain in her arms or legs. Her family history was not contributory. She did not smoke or drink alcohol. She did not use any medication. Because she was breastfeeding her newborn baby, she did not take any pain medication.
The neurological examination showed no motor deficits. There was no clear deficit in sensory testing, but a slight decrease in vital sensibility in her arms and legs was suspected. Tendon reflexes were normal. Plantar reflexes were normal on both sides. The pain could not be provoked by manipulation of the spine. As the pain continued at night and no good explanation could be found, imaging of the spine was ordered. However, the a priori chance of finding a tumor was thought to be very low. An MRI scan of the thoracic spine showed an intramedullary, partly cystic/necrotic, partly solid tumor at levels C5–Th1. The solid part of the tumor was 3 cm long. It showed eccentric growth and was surrounded by edema. Using gadolinium, the solid part of the tumor showed enhancement, indicating a disruption of the blood-brain barrier (Fig. ). The patient was referred to a neurosurgeon, who performed a debulking of the tumor. Histopathological examination showed tissue of high cellularity and pseudorosettes. There were hardly any mitoses seen, no necrosis, and some calcification. These findings are consistent with a WHO grade II ependymoma.
At follow-up, the patient had made a full recovery. Repeated MRI scans showed that the debulking was total, and there were no signs of recurrence.


We report the case of a 67-year-old male with kidney dysfunction. His past medical history included thyroidectomy due to hyperthyroidism at the age of 30 years. In 2006, the patient was admitted to our hospital for cerebral infarction, diabetes, hypertension, hyperuricemia, and dyslipidemia. Since that time, the patient had been prescribed the following medicines: levothyroxine sodium hydrate, 25 μg/day; amlodipine basilate OD, 5 mg/day; valsartan, 160 mg/day; feburostat, 10 mg/day; and atorvastatin calcium hydrate, 5 mg/day. Rapid types of insulin, such as the 16-unit Novo RapidN and the 10-unit Novolin RN had been used. The levels of uric acid are 4.90 mg/dL, and blood pressure was 140/70 mm Hg. The levels of FT3, FT4, TSH, and total cholesterol and triglyceride were within normal limits. An ophthalmologist diagnosed him with diabetic proliferative retinopathy and performed photocoagulation therapy. In November 2015, a serum urea nitrogen level of 56.0 mg/dL, a serum creatinine (s-Cr) level of 3.9 mg/dL and an estimated glomerular filtration rate (eGFR) of 13.4 mL/min indicated renal dysfunction at another division.
Due to a recent increase in frothy urine, the patient was transferred to our nephrology division for a further treatment. At the time of the first visit to our division, body height was 160 cm and weight was 68 kg (BMI: 26.6). He showed slight obesity (obesity criteria in Japan: a BMI of more than 25.0). Laboratory data from the time of his first visit to our division in March 2016 showed a fasting plasma glucose level of 121 mg/dL and a HbA1c (national and glycohemoglobin standardization program: NGSP) of 6.9%. Thus, we moved him to a DPP-4 inhibitor, i.e., saxagliptin hydrate of 5 mg/day. High-density urine and a strongly positive proteinuria quantified at 2.62 g/gCr were found. Urine ketone bodies were negative, and there was a negative urine occult blood reaction, which resulted in a clinical diagnosis of diabetic nephropathy. The frothy (with small bubbles) urine meant that there was a high dose of protein in the urine. A serum urea nitrogen level of 60.7 mg/dL, a s-Cr level of 4.9 mg/dL, and an eGFR of 10.2 mL/min showed a progressive renal dysfunction. Antiglutamic acid decarboxylase antibodies in sera were negative (normal value: < 1.5 U/mL).
In the Japanese Society of Nephrology (JSN) classification, the causal disease, eGFR, and albuminuria (proteinuria) levels have to be determined. eGFR is divided into 5 stages, from GFR 1 to 5, and albuminuria (proteinuria) is divided into 3 stages, from A1 to 3, as shown in Figure . According to this classification, he was diagnosed as follows; chronic kidney disease (CKD); cause diabetes (type 2); GFR G5, (eGFR range: < 15 min/mL); and albuminuria, A3 (albuminuria range: > 300 mg/gCr). A definite diagnosis of diabetic nephropathy could not be made without renal biopsy. Kidney function, i.e., eGFR, was not improved by previous medications. We added on a minimal dosage of AST-120 (Kremezin®) of 2 g/day because of his severe constipation. After 3 months of AST-120 therapy, eGFR increased to 18.1 mL/min/1.73m2 (with a s-Cr of 2.90–2.72 mg/dL). Although he used some laxative products (senna 1 g/day and sennnoside 12 mg/day), he could not continue to take AST-120 and then completely stopped the drug 8 months after starting. Kidney function then abruptly declined (eGFR of 7.3 mL/min/1.73 m2; s-Cr of 6.7 mg/dL) and progressed to end-stage kidney disease (ESKD), as shown in Figure . In June 2017, he was introduced to hemodialysis (HD).


A 42-year-old female with history of RYGB performed seven years ago, complicated by GJ anastomotic ulcer three years ago was seen in follow-up for symptomatic GJ anastomotic stricture. She reported weakness, fatigue and a 50-pound weight loss over the past year due to her inability to tolerate both solids and liquids orally. Her BMI was 18.79 and she did not have any medical comorbidities including no obstructive sleep apnea or hypertension. Patient’s GJ anastomotic ulcer healed two years ago after starting a hydrogen-potassium ATPase inhibitor and she reduced her smoking from one pack per day to three cigarettes per day. For her persistent GJ anastomotic stricture she underwent through-the-scope (TTS) dilation twice this year and twice in the year before with triamcinolone injected in the stricture on her second dilation. These measures provided only temporary relief of her symptoms. On her last esophagogastroduodenoscopy (EGD), she had persistent GJ stricture and no gastro-gastric fistula. Her Helicobacter pylori status was negative on multiple occasions on stool antigen testing and on microscopic examination of obtained tissue samples during EGD. We performed a repeat EGD on which the GJ anastomosis was characterized by severe stenosis (2 mm opening) (Figure ). A LAMS (Axios stent 15 mm x 10 mm; Boston Scientific, Marlborough, MA, United States) was placed into the anastomotic stricture (Figures -). A wire-guided TTS balloon dilator (CRE; Boston Scientific, Marlborough, MA, United States) was passed over a guidewire (Hydra Jagwire 0.035 cm x 260 cm; Boston Scientific, Marlborough, MA, United States) through the LAMS. Dilation with 10 mm-11 mm-12 mm anastomotic balloon dilators was performed and held inflated for 2 min under fluoroscopic guidance. Our patient did not develop any immediate or delayed stent-related adverse events. Patient’s symptoms improved remarkably after the procedure, she had gained 5 pounds and was asymptomatic on three-month follow-up. We aim to remove the stent after a total of six months for maximum effect.


A 62-year-old male patient was admitted with a three-day history of abdominal distention along with abdominal pain, bloating, constipation and concomitant itching and burning skin eruption on the left side of the abdominal wall; which appeared 48 h before admission. Though, there were no complaints of nausea, vomiting or fever. The patient was not taking any medications. On admission, the patient was afebrile and hemodynamically stable. Physical examination revealed abdominal distention and cutaneous vesicular eruption on the left abdomen, extending to the back, not crossing the midline and involving the T7-T10 dermatomes (Figure ). The abdomen was tense, with tympanitic percussion note all over, with decreased bowel sounds and no signs of peritoneal irritation.
Hamatologic and biochemical tests were all within normal range. Plain X-rays and abdominal computed tomography scan demonstrated distended small bowel loops and air-fluid levels (Figure ). The diagnosis of intestinal pseudo-obstruction (paralytic Ileus) was made secondary to segmental paresis and visceral neuropathy caused by VZV infection (HZ). Conservative management was then established with nasogastric tube decompression, fasting, intravenous (IV) resuscitation and IV Acyclovir. The patient’s abdominal distention gradually resolved over the next 7 d and pain subsided. The patient was discharged on oral vancyclovir and gabapentin, 8 d after admission. On follow-up, fifteen days later, cutaneous vesicles dried up, changed to scabs and dropped off. The patient showed a full and uneventful recovery.


A 1-year-old boy presented to us with complaints of a reddish patch on the anterior aspect of his neck with deficient skin over the lesion since birth. There was no associated history of any discharge, trauma, or bleeding. On examination, a single anterior cervical midline skin defect measuring 3 cm × 2 cm was present. There was an external opening of a sinus seen at the inferior margin of the defect []. No discharge was reported from the sinus opening. A diagnosis of midline cervical cleft was made, and the patient was taken up for surgery. The entire lesion along with the sinus tract down to the pretracheal fascia was excised. The sinus opening was connected to two small cysts inferiorly, and hence, the dissection was extended till manubrium sterni for complete excision. The skin defect was closed by a Z-plasty to avoid a contracture and flexion deformity of the neck []. Histology revealed two cystic lesions. The smaller cyst was lined by squamous epithelium, while the larger cyst was lined mostly by squamous epithelium and focally by respiratory epithelium []. The subepithelial stroma shows few serous acini. There was no evidence of thyroid tissue or lymphoid follicles. The features were consistent with congenital midline cleft cyst. On follow-up, the wound showed good healing and the child is asymptomatic.


A 55-year-old woman was admitted to the Internal Medicine Department of Razi Hospital in Rasht (a city in the north of Iran) with a 30-year history of rheumatoid arthritis (RA). She declared a hypogastric, nonpositional pain without any relation to defecation or feeding, for 2 months, with previous constipation (had been cured with lactulose). Each pain episode lasted for ~5–10 minutes. She had a weight loss of 20 kg over the last 7 months. Also, had undergone endoscopy the previous year for the symptoms of nausea, diarrhea, and epigastric pain with eradicated Helicobacter pylori gastritis after a 4-week treatment. Although she had chronic anemia, there was no history of drinking alcohol or smoking, diabetes mellitus, cardiovascular disease, hyperlipidemia, or hypertension. In addition, she took prednisolone 5 mg, hydroxychloroquine 20 mg, hematinic, and supplements such as calcium, folic acid, and multivitamins daily. Furthermore, a self-discontinuation of methotrexate (MTX) was noted. The colonoscopy was performed, and only an ulcerated mass in the hepatic flexure was seen, and a biopsy was taken. As the endoscope could not pass through the hepatic flexure, the procedure was stopped (). The pathobiological features revealed ulceration and granulation tissue formation, foci of necrotizing granulomatous inflammation in lamina propria with adjacent mild crypt regenerative changes (). Moreover, Ziehl–Neelsen stained sections () revealed few acid-fast positive bacilli in the granuloma. No evidence of dysplasia or malignancy was found in the received sample. Meanwhile the polymerase chain reaction (PCR) for ITB was negative. In order to rule out pulmonary TB, radiography was performed with no evidence of pulmonary involvement (). Follow-up after a 2 week treatment with anti-TB medication demonstrated the complete disappearance of the symptoms. A written informed consent form has been provided by the patient to have the case details and any accompanying images published.


We present the case of a 31-year-old Caucasian female with a body mass index (BMI) of 22.6 that had a subdermal contraceptive implant (Nexplanon®; Merck & Co., Inc., Whitehouse Station, NJ, USA) inserted into the medial aspect of the upper arm in 2015 for contraceptive action. There were no reported complications at the time of device insertion.
She presented a year later for elective device removal as she planned on starting a family. The implant was not palpable in the upper arm and unable to be localized by ultrasound or X-ray of the arm (). Etonogestrel levels were raised at 176 pmol/mL suggesting active implant in situ.
The patient also reported several weeks’ history of occasional nonproductive cough. She did not suffer from any chest pain or dyspnoea. Chest X-ray (CXR) was undertaken and was reported normal by local radiologists ().
Given the strong clinical suspicion of embolized device, an unused Nexplanon® implant was shown to the reporting radiologists to facilitate an appreciation of the nature and physical characteristics of the foreign body being looked for. On retrospective re-review of the imaging, the radiologists subsequently identified the device implant on the anteroposterior and lateral views of the CXR. Chest CT was undertaken and revealed the presence of the contraceptive implant in a sub-branch of the right lower lobe pulmonary artery.
The patient was referred onward to the interventional cardiologists for an attempt at percutaneous extraction of the implant. Implications of leaving the device in situ include infection, potential pulmonary arterial thrombus formation, irritant cough and reduced fertility until the active hormone ingredient is depleted.
The patient was keen on device extraction in view of a persistent cough as well as her plans for starting a family. As such, she was electively admitted as a day-case procedure for attempt at device extraction. Routine bloods and electrocardiogram were unremarkable. The procedure was undertaken in the cardiac catheter laboratory under local anesthesia from the right femoral vein using biplane angiography (). A coaxial approach was used to introduce a series of catheters into the right pulmonary artery which facilitated the capture of the device using a gooseneck snare allowing subsequent extraction of the intact device (). The procedure was uneventful and the patient discharged later in the day. She successfully became pregnant 1 month later and went on to have an uncomplicated normal delivery.


An 86 year old man who had undergone bilateral primary TKA at another institution 10 years ago visited our outpatient clinic complaining of pain and swelling in his left knee joint. He had no history of trauma. He had medical history of hypertension which was controlled with anti-hypertensive medications. Physical examination revealed heating sense, swelling and draining sinus at anteromedial aspect of proximal tibia []. Radiographs did not demonstrate any lucency [Figure and ]. The aspirated joint fluid showed plenty of polymorph nuclear leukocytes and Gram-positive cocci. The culture yielded methicillin-resistant staphylococcus aureus.
Two-stage procedure including articulated antibiotic-impregnated cement spacer was planned []. Thorough debridement and irrigation were performed. The patient was maintained on an antibiotic regimen (Vancomycin 500mg IV bid) as recommended by the infectious disease consultants for 6 weeks. The eradication of infection was suggested by the return of inflammatory markers to normal. There was no patella subluxation or dislocation.
During the second stage operation (3 months after cement spacer insertion), rectus snip was performed for the extended surgical approach. Specimens of intraarticular soft tissue were taken for frozen biopsy analysis. After confirming that all specimens had <10 polymorph nuclear leukocytes/high power field, the articulating cement spacers were then removed, followed by debridement, and reimplanted using the constrained condylar knee (LCCK, Nexgen® Legacy®, Zimmer, Warsaw, IN). The tibial cut was made perpendicular to the intramedullary canal of the tibia. The reconstruction was commenced by reestablishing the proximal tibia because any change in the tibial height would affect the extension and flexion symmetrically. The goal of the tibial reconstruction was to place the tibial tray within the planned distance proximal to the two anatomic landmarks; the tibia tubercle and the fibular head. It was planned to place the joint line at around “2 finger breadths proximal to the tibia tubercle” and “about 20 mm proximal to the fibular head.” The distal femur was resected at 6° valgus angulation to the femoral shaft, using intramedullary alignment guides. Rotational positioning of the femoral component was guided by the epicondylar axis. Trying to manage the gap mismatch and maintain the joint line, the additional 10 mm femoral block in both distal and posterior femur was placed and increased the femoral component to one size up (size E: Width of 68 mm [the femoral component width was 64 mm at primary surgery], which corresponds to 4 mm increase in antero-posterior length). Slight overhanging was visible on the lateral aspect (2 mm). For the tibia side, bone defect in proximal tibia was managed using the additional 10 mm tibia block. Finally, 14 mm tibia insert was decided. The estimated joint line elevation was about 10 mm. The stems were planned for both femoral and tibial side.
After placing the trial implants, knee range of motion (ROM) was checked for patella tracking. At this stage, snapping was palpated from the lateral aspect []. Hypertrophied thick band-like structure was recognized at the lateral aspect of knee joint during ROM. The snapping was evident at around 20°-30° of knee flexion. This band was identified as ITB which was adhered to the distal insertion site. Even after the release of the fibrotic tissue surrounding the ITB, it was tightened and was caught during knee ROM on the femoral component which was overhanging laterally (anterior and distal aspect) by about 2 mm. To manage snapping, 8-10 times of multiple transverse puncture was made using No. 11 blade. The punctures were made at the joint line level of the ITB. After multiple attempts, snapping disappeared intraoperatively []. Postoperatively, the patient received graduated compression stockings and intermittent pneumatic calf pumps as prophylaxis against deep vein thrombosis. Postoperative analgesics regime was given. The ROM exercise using continuous passive motion devices, an isometric/isotonic quadriceps exercise, a straight leg raising exercise and a walking exercise were initiated under the control of a physiotherapist. Varus-valgus constraint knee brace was worn during rehabilitation which was removed after 6 weeks. At 5 years after revision TKA [], the knee ROM was 0-110 without snapping []. There was no varus instability. There was no patella subluxation or dislocation. The final knee society score was 92.


A 63-year-old woman with history of breast cancer underwent a medical checkup. Ultrasound examination revealed one hypoechoic lesion measuring 0.6 × 0.4 cm in the left thyroid lobe () and two small cystic lesions in the right thyroid lobe. Liquid based cytology (LBC; CellPrep, Biodyne, Seongnam, Korea) FNA of the left lobe nodule showed features of PTC including overlapping enlarged nuclei, occasional nuclear grooves, and intranuclear pseudoinclusions. The lesion was diagnosed as suspicious for papillary carcinoma. Computed tomography revealed a 0.6 cm low density nodule with no evidence of extracapsular invasion and no significant lymph node enlargement (). The surgeon decided to perform left lobectomy based on the assumption that the lesion could be a benign tumor in the final pathologic report, because of uncertain radiologic features.
On gross examination, one well circumscribed yellow to tan firm nodule measuring 0.6 × 0.5 × 0.4 cm was identified (). Histologic examination revealed a well circumscribed nodule composed of trabecular and alveolar patterns of tumor cells. Hyalinization was observed between cell trabeculae. Tumor cells had abundant cytoplasm, low nuclear to cytoplasmic ratio, perinuclear clearing, nuclear grooves, and intranuclear pseudoinclusions (, ).
Immunohistochemistry showed diffuse membrane positive reactivity for Ki-67 (MIB1 clone) at room temperature, which is a distinct immunohistochemical characteristic of HTT (). Immunohistochemical staining also revealed diffuse positivity for CD56, focal weak positivity for galectin 3, and negativity for cytokeratin 19. The lesion was diagnosed as HTT based on these histological and immunohistochemical features.
Retrospectively, there were several points that could have led to the suggestion or diagnosis of HTT on FNA cytology. Ultrasonographic findings are one clue because the lesion was well circumscribed, a benign feature, although it did show hypoechoic features with internal microcalcification. While the LBC of the FNA specimen had similar features to papillary carcinoma, such as occasional intranuclear pseudoinclusions and nuclear grooves, this case had some special cytological characteristics, as described below. Syncytial fragments or loosely cohesive groups of tumor cell clusters had cores of hyaline material among the cells. Tumor cells were usually oval to polygonal with pale to dense cytoplasm. However, spindle-shaped cells were occasionally observed. Unlike PTC, which has pale and clear chromatin, nuclei showed finely dispersed chromatin (, ). Other than these general characteristics of HTT, more unique cytological features of this HTT case are frequent three-dimensional cell clusters and stratified columnar tumor cells in the peripheral rims of clusters. Although tumor cells of PTC also show nuclear overlapping, HTT tumor cells in this case are more often arranged in a ball-like structure. In addition to the distinct spindle-shaped tumor cells, cells with abundant cytoplasm and eccentrically located round nuclei with columnar features were frequently observed and arranged in a stratified trabecular pattern in this case (, ).
This case study was approved by the Institutional Review Board of Seegene Medical Foundation (IRB No. SMF-IRB-2018-001). The need for informed consent was waived.


A 24-year-old woman was admitted to an outside hospital presenting with abdominal discomfort. She was referred to Asan Medical Center and was found to have a huge liver mass that was detected by abdominal ultrasonography. The diagnosis of pre-operative liver biopsy performed by an outside hospital was HCA. Her medical and family histories were unremarkable. She had never taken OCPs and had a body mass index of 21.48 kg/m2. Hepatitis B surface (HBs) antigen, anti-HBs antibody, and anti–hepatitis C virus antibody were all negative. A complete blood count and serum chemistry tests, including coagulation and liver function tests, were normal except for elevated levels of gamma glutamyl transferase (111 IU/L; normal range, 8 to 61 IU/L). Serum tumor marker of prothrombin induced by vitamin K absence-II (PIVKA-II) levels were markedly elevated (2,964 mAU/mL; normal range, ≤ 40 mAU/mL). α-Fetoprotein levels were slightly elevated (11.1 ng/mL; normal range, ≤ 7.0 ng/mL), while those of serum carcinoembryonic antigen (0.9 ng/mL; normal range, ≤ 5 ng/mL) and carbohydrate antigen 19-9 (3.8 U/mL; normal range, ≤ 37 U/mL) were within normal limits. A dynamic liver and pelvis computerized tomography (CT) scan revealed a 20 cm hypervascular mass occupying segments 4, 5, and 8 of the liver (). An enhanced CT image exhibited high attenuation during the arterial phase and decreasing attenuation during the portal and delayed phases. The lesion showed heterogeneous foci of hemorrhage, necrosis, fat, and calcification. An experienced radiologist (S.Y.K.) interpreted the lesion as a huge HCA and suspected a partly malignant transformation. The patient underwent segmentectomy and partial hepatectomy to remove the lesion. Macroscopically, the resected specimen was filled with a well circumscribed 19.9 × 18.4 × 7.9-cm solid mass, and its cut surface showed a heterogeneous yellow-brown color, multinodular growth, partly cystic degeneration, hemorrhage, and necrosis (). Microscopically, the lesion exhibited typical features of a HCA and was characterized by well differentiated hepatocytes arranged in thin cords or sheets with bland cytology and abundant eosinophilic cytoplasm (). The parenchyma showed dilated sinusoids, occasionally with large aberrant arteries, and multifocal inflammation composed of lymphocytes, neutrophils, and eosinophils (, ). Immunohistochemical stains of HCA cells showed diffuse positivity of liver fatty acid binding protein, serum amyloid A (), C-reactive protein (), and glutamine synthetase () antibodies and partial nuclear positivity for β-catenin antibody (), suggesting an inflammatory HCA with β-catenin activation. Although this case showed histologically typical features of inflammatory HCA without histological features of other subtypes, it coexisted with β-catenin nuclear positivity, suggesting a β-catenin–activated inflammatory HCA. It was difficult to grossly distinguish the area of malignant transformation. However, multiple malignant foci were found microscopically that occupied less than 10% of the total tumor area. These malignant foci were all well differentiated HCC that partially resembled the surrounding HCA but also showed nuclear hyperchromasia and pleomorphism and thickened trabeculae (). Glutamine synthetase showed stronger expression in the HCC area than in the HCA area, and heat shock protein-70 () and glypican 3 () were positive in the HCC area. As is typical of HCC, reticulin loss () and diffuse CD34 expression on sinusoidal cells () were observed. The Ki-67 labeling index of the HCC area (15%) was significantly increased compared with that of the HCA area (< 2%) (). Corresponding to visible calcification regions in the CT scan were areas of bone marrow metaplasia () that were characterized by mature lamellar bone forming trabeculae intermingled with fat tissue that contained myeloid and erythroid cells (, ). The patient was discharged 10 days after surgery and showed no evidence of recurrence at the last follow up visit 23 months after surgery.
This study was approved by the Institutional Review Board of Asan Medical Center with a waiver of informed consent (IRB No.2017-0679).


A 13-month-old female child presented to us with a skin dimple over the lumbar region with overlying hairy patch without any associated lower limb weakness or urinary complaints. Her physical examination revealed no other abnormalities. On imaging evaluation, magnetic resonance imaging showed a split cord from L2-L5 with broad-based spur at L4-L5 level with single thickened filum tethered at L5-S1 level with syrinx formation in the right hemicord from L1-L5 []. Noncontrast computed tomography showed deficient posterior elements of L5 to S1 with two bony spurs, one arising from the posterior aspect of L4 vertebral body and another from the hypertrophied L4 posterior arch [].
The child was taken up for operative intervention, and intraoperatively, two bony spurs were confirmed with soft tissue in between—ventral spur based on the L4 vertebral body and a dorsal spur based on the L4 hypertrophied posterior arch. The cord was divided into two hemicords—right side larger than the left—which were reuniting below the spur to end in a single thickened fatty filum. There was a variable distance of split cords above the spurs suggestive of Type Ic SCM.[] The spurs were excised with the help of a high-speed diamond drill, and the fatty filum was divided at S1 level. Dural bands and arachnoid adhesions tethering the cord were released []. Dura was closed primarily and reinforced with muscle patch and tissue glue. Postoperative hospital stay of the child was uneventful with no new onset of neurological deficits and the postoperative computed-tomography scan demonstrated complete excision of the spurs [].


A 48-year-old male with RA presented with bilateral lower extremity rash and swelling, fevers, night sweats, anorexia and unintentional weight loss over the prior 2 months. Due to financial issues, the patient had stopped taking methotrexate for treatment of his RA two years prior to presentation. He was noted to have pitting edema with non-tender palpable purpura on both shins surrounded by non-blanching petechiae extending proximally to the knees. A firm immobile non-tender mass was palpable in the left lower quadrant.
Laboratory work-up revealed a white blood cell count (WBC) of 1.0 K/ul, an absolute neutrophil count of 680, and an absolute lymphocyte count of 310. He was anemic with a hemoglobin of 6.9 g/dl. Rheumatoid factor was elevated to 180 IU/ml. An antinuclear antibody screen was positive at 1:320. Computed tomography (CT) of the abdomen and pelvis showed an enlarged spleen and a large heterogeneous mass in the left abdomen extending into the pelvis (Figs and ). A bone marrow biopsy showed abnormal CD3+ and CD8+ T-cell lymphocytosis with TCR(beta)+ and CD57+, consistent with the diagnosis of T-cell large granular lymphocytic leukemia.
The patient was evaluated by rheumatology, medical oncology and surgical oncology. He received 300 μg of filgrastim and was transfused two units of packed red blood cells pre-operatively. The patient was taken to the operating room for exploratory laparotomy and splenectomy. The left-sided retroperitoneal mass was vascularized by branches from the distal left external iliac artery and the left inferior epigastric artery. The retroperitoneal tissue was removed en-bloc extending from the splenic bed to the distal left external iliac, Gerota’s fascia to posterior abdominal wall, and lateral abdominal wall to the colonic mesentery (Fig. ).
Pathology of the retroperitoneal mass returned as a 50 × 33 × 16 cm high-grade dedifferentiated liposarcoma arising in a background of well-differentiated liposarcoma. Margins were positive for dedifferentiated liposarcoma. The spleen was markedly enlarged (16 × 10 × 22 cm) but had no significant histologic change. The patient’s post-operative course was unremarkable, and he was discharged on post-operative day five, at which time his WBC count had improved from 1.37 K/ul with 21% neutrophils and 62% lymphocytes pre-operatively to 3.22 K/ul with 56% neutrophils and 17% lymphocytes post-operatively. CT imaging obtained nine months after surgical resection of the retroperitoneal mass shows no evidence of recurrence. Routine lab work demonstrates improvement in his anemia (Hgb 12.8 g/dl) and absolute neutrophil count (>1,500) while on maintenance therapy with methotrexate.


A 67-year-old man was referred to our outpatient clinic of geriatric psychiatry because of persistent affective symptoms for years. The onset of symptoms coincided with an unpleasant work situation that, after a year of sick leave, had led to demotion at 49 years of age. From that moment on, he suffered from depressed mood, anhedonia, loss of initiative, increased appetite, worrying, catastrophising, feelings of anxiety with excessive sweating, difficulties staying asleep, nightmares, increased need of sleep, loss of energy and tiredness. Symptoms fluctuated over the years but increased during autumn/winter, after life events and during vacation periods. Over the years, he had gradually withdrawn from social activities.
His general practitioner (GP) had prescribed benzodiazepine hypnotics, but without any improvement of symptoms. Six weeks prior to his referral, he was started on citalopram 20 mg one time a day, a first-line serotonergic antidepressant. He had lost 7 kg bodyweight due to nausea. This weight loss increased his physical fitness, which made him feel slightly better.
His medical history did not reveal other mental or physical symptoms.
The patient was monitored, but not treated, for mild hypertension by his GP. He had no other previous medical or psychiatric history. His family history revealed no psychiatric problems or neurodegenerative diseases like M. Parkinson, M. Alzheimer or M. Huntington. The patient did not smoke or drink alcohol. Besides citalopram, the patient used simvastatin 40 mg one time a day.
Heteroanamnestic information of his wife and son revealed a change in character since the onset of his symptoms; he became more emotional and insecure about himself. Since his retirement, he tended to lose his temper over seemingly insignificant events. His wife explained that they slept apart for years because of his snoring. She nevertheless confirmed that his breathing paused regularly during sleep. Although they consulted their GP several times for his conspicuous sleepiness, they accepted the explanation that it was an inevitable part of his depression.
At psychiatric examination, we saw a tired man with bags under his eyes and reduced facial expression. Higher cognitive functions were intact. He had a depressed mood and flat affect, but denied suicidal ideation. Evaluation of his sleep pattern revealed a rapid sleep onset, but strikingly, he never woke up well rested, regularly dozed off during the day and he was often at risk of falling asleep while driving his car.
A full physical and neurological examination was remarkable only for an overweight body mass index (28.9 kg/m2) and hypertension (130/100 mm Hg). Routine blood chemistry did not reveal any abnormalities.


A 55-year-old hypertensive female from Pauri Garhwal in the Indian state of Uttarakhand presented to the outpatient department of Internal Medicine with forgetfulness for 3 years. The forgetfulness of the patient had progressed from subtle changes like forgetting where she kept her money or other things, to her daily chores such as eating and bathing at the time of presentation. She also had problems in articulation, though perception and comprehension seemed normal. She also had a history of clumsiness of movements, slowness in performing daily tasks, lack of concentration, decreased attention span, and depressed mood.
She had been experiencing unprovoked falls from the last 2 years, the frequency of which had been progressively increasing, with each lasting around 1 min and not associated with the symptoms suggestive of a seizure episode. The consciousness remained intact throughout. There was no history of confusion after the episodes. She had a significant history of minor trauma due to falling at inappropriate places. There was no particular side on which the patient fell.
There was no history of abnormal involuntary movements, cranial nerve involvement, blurring of vision, sensory deficit, muscular weakness, tremors, bowel or bladder incontinence, or head injury. The patient has been a known hypertensive (uncontrolled from 2 years), on and off medication. Family history was not significant.
On general examination, blood pressure of the patient was 180/100 mm of Hg in the right brachial artery in supine position. Other vitals were stable. Body mass index (BMI) was 25.7 kg/m2 (overweight according to the WHO BMI classification). The patient seemed depressed.
On neurological examination, the patient was cooperative, conscious, and responsive to commands. Glasgow coma scale - E 4 V 5 M 6.
Mini-Mental Status Examination revealed inference of Mild cognitive impairment (score: 20/30). The patient exhibited unsteady gait. All the tests for coordination were abnormal on the left side. Intentional tremors and rebound phenomenon were present. All remaining cerebellar signs and signs of meningeal irritation were absent. Cranial nerves, motor, and sensory evaluation was within normal limits. All other systems seemed normal.
On laboratory investigations, the patient was found to have dyslipidemia and type 2 diabetes mellitus []. Her hemogram, liver, and kidney function tests were within the normal range.
On fundoscopy, papilledema, diabetic, or hypertensive retinopathy was ruled out. On lumbar puncture, cerebrospinal fluid pressure was found to be normal. Serum homocysteine and serum fibrinogen levels were well within normal range.
Magnetic resonance imaging (MRI) of the brain showed a hemorrhagic infarct in the right thalamus with extensive chronic ischemic white matter lesions with multiple microhemorrhages in cerebellar and cerebral hemispheres, brainstem, and deep nuclei with a right-sided (2.3 cm × 2.1 cm) infratentorial arachnoid cyst with prominent ventricular system, sulcal spaces, and basal cisterns [Figures –].
The differentials which were kept in mind while investigating the patient were Alzheimer's, Parkinson's disease, dementia with Lewy bodies, frontotemporal degeneration (Pick's disease), and normal pressure hydrocephalus (NPH). All of these causes were ruled out based on the characteristic radiological findings of Binswanger's disease and slight variation in clinical presentation.
In Alzheimer's disease, the patient has a progressively severe memory loss and little or no gross locomotor disturbances until very late in the disease course.
Patients with Parkinsonism present with bradykinesia, rigidity, tremors, and shuffling gait. The patients have mild memory loss and cognitive impairment. Motor disturbances occur early in the course of the disease.
Both NPH and Binswanger have a similar clinical presentation, but NPH progresses gradually, and Binswanger disease progresses in a stepwise fashion with more commonly associated vascular complications.
The patient was advised lifestyle modifications along with antihypertensives (Amlodipine 5 mg), oral hypoglycemic agents, lipid-lowering drugs for control of her risk factors for preventing any further similar episode of complication on a preexisting debilitating disease. In addition to it, cholinesterase inhibitors (Donepezil 10 mg) and multivitamin supplements.
The patient came for a follow-up visit after 15 days. She improved symptomatically for giddiness and depressed mood, but the remaining symptoms persisted. Her blood pressure levels were still on the higher side (150/90 mm Hg), therefore, dose of Amlodipine was increased to 10 mg. Blood glucose and lipid profile were within normal range. Hence, all remaining drugs were continued in same doses. She was referred to psychiatry for counseling regarding her depression. She was explained about the nature of her disease, prognosis and was encouraged to control the risk factors for preventing future complications.


An asymptomatic 65-year-old man presented with a lesion that had been incidentally detected two months prior. Computed tomography (CT) images showed a left lower posterior mediastinal paravertebral tumor measuring 4.2 × 3.0 cm with no observed direct invasion to any adjacent structures. The oval-shaped tumor had a smooth and clear edge and roughly uniform density, with a CT value of approximately 0 HU. It was located in the left posterior region of the descending aorta (Fig a). The patient denied having any symptoms, such as fever, cough, or pain. His medical and family history of disease was unremarkable. Upon admission, his physical examination and blood and urine evaluations were also unremarkable. CT imaging revealed that the tumor was located in the mediastinum (Fig b), and there was no blood vessel connection with the descending aorta (Fig c); thus, pulmonary sequestration was excluded. The patient's symptoms, signs, and CT images also excluded enlarged lymph nodes and lymphomas. The preoperative diagnosis was a neurogenic tumor. Considering the position and benign nature of the lesion, we planned a transdiaphragmatic retroperitoneoscopic resection of the left lower posterior mediastinal tumor.
The patient lay in a modified flank position under general anesthesia. The retroperitoneal space was expanded with an in-house balloon dilatation apparatus, and the trocar was placed as previously reported. A 30° angle laparoscopy was used during the procedure. The pressure of the cavity was maintained at 10–14 mmHg. The Gerota's fascia was opened, and further dissections were all conducted in the non-vascular plane between the renal fat capsule and the Psoas muscle. Care was taken to avoid injury to the renal artery. After wide dissection in the plane, we located the lesion at the midline end of the arcuate line of the diaphragm and under the diaphragm muscle. The diaphragm was longitudinally cut to reveal the whole mass (Fig a). Separation was conducted primarily by blunt dissection. In total, four nerve roots were cut with a Harmonic scalpel. The mass was completely removed (Fig b). The urologist and the anesthetist both confirmed the lack of pleural injuries from retroperitoneal examination and respiratory parameters. The incision to the diaphragm was then closed with a 2-0 barbed suture using a running method (Fig c). A retroperitoneal drainage tube was placed through the trocar point of the anterior axillary line. The operation lasted 50 minutes. In total, 500 mL normal saline and 250 mL 6% hydroxethyl starch 130/0.4 mL were provided, and urine volume was 300 mL. No obvious blood loss occurred. The drainage was removed after two days, and the patient was discharged three days after the operation. No complications were recorded. Pathologic analyses of the resected specimen revealed a benign schwannoma.


A 7-year-old female experienced abdominal pain without an obvious cause in May 2011, showing continuous dull pains, most of which could be self-relieved. Subsequently, the patient underwent imaging examination at our hospital, which indicated multiple intrahepatic and extrahepatic cystic dilatation of bile duct (Figure ). It was clearly diagnosed as type IVa CCs. After the preoperative examinations, we decided to carry out robotic-assisted laparoscopic choledochocystectomy and biliary-intestinal anastomosis.
With the patient in the supine position, the operation bed was in the reverse Trendelenburg position. After the anesthesia was successful, the anesthesiologists conducted internal jugular vein intubation and radial artery catheterization and placed a gastric tube and urinary catheter. Direct trocar puncture was done on the abdominal wall between the umbilicus and pubic symphysis to establish pneumoperitoneum. The pressure was 12 cmH2O, and a 12-mm trocar and robotic lens were inserted.
Being monitored under the lens, at about the level of umbilicus, the 8-mm-diameter trocars of arm 1 and arm 2 of the robotic instrument were inserted into the left and right abdomen, respectively, and the arm 3 trocar was inserted into the right upper abdomen at the level of the axillary line (Figure ). A 12-mm auxiliary hole was created between arm 1 and the lens. The robotic arms were placed from the end of the head and were connected.
No significant ascites was detected in the abdominal cavity. The gallbladder volume was approximately 7.0 cm × 3.0 cm. The common bile duct was dilated with a diameter of 5.5 cm. Its surface showed hyperemia and edema. An electric hook was used to separate the adhesion between the gallbladder and liver. The gallbladder arteries were ligated, and the gallbladder neck was dissected all the way to the common hepatic duct. The adhesions between the outer serosal membrane of the choledochal cyst and the duodenum and the portal vein were separated. The common hepatic duct was cut transversely. The diameter of the common hepatic duct was approximately 3.2 cm. The left and right hepatic duct openings were visible at the proximal end, and the flow of bile was unobstructed. There was no obvious stenosis. The gallbladder and the choledochal cyst to be removed were turned upside down, the common bile duct was dissected till near the head of the pancreas, and the end of the cyst was significantly narrowed with a diameter of approximately 1 cm and was sutured twice.
A stapler (EC-60) was used to cut the jejunum transversely at 15 cm from the duodenojejunal flexure, and 35 cm of the proximal end towards the hilus was saved as ascending arm. After joining the jejunum and the ascending branch by three stitches, we used a stapler to perform end-to-side anastomosis between the ascending arm and the proximal jejunum. The 3-0 absorbable suture was used for full-thickness interrupted suture of the intestinal wall, which was reinforced by interrupted suture. The ascending arm of the distal jejunum was lifted from the front of the colon to the common hepatic duct location to perform anastomosis. A 4-0 absorbable suture was used for full-thickness continuous suture of the posterior wall. Then the anterior wall was sutured, and interrupted suturing was done for seromuscular layer for reinforcement.
Physiological saline was used for rinsing. One abdominal drainage tube was placed on the lateral posterior side of the gallbladder-intestine anastomosis, which came out of the body through the puncture hole of the arm 2 trocar and was connected to a negative-pressure drainage ball. The abdominal cavity was confirmed to have no active bleeding, and the devices and gauzes were all counted. No. 3 absorbable suture was used to suture the muscular layer at each trocar location, 4-0 absorbable suture was used for interrupted suturing of subcutaneous tissue. Biological fibrin glue was used to bond the edges of the skin, and the drainage tube was properly fixed. Tissue aid was applied to the wound.
The surgery went smoothly. The operation time was 420 min, of which the robot operation time was 370 min. The bleeding was approximately 100 mL. There was no perioperative blood transfusion. Two days after the operation, the gastric tube was removed, and fluid and semi-fluid food were given gradually. Two antibiotics were administered for 3 d. Seven days after operation the abdominal drainage tube was removed. The patient was discharged 9 d after the operation. No serious complications occurred. Routine pathological examination further confirmed the diagnosis of cystic dilatation of the common bile duct.


A 28-year-old man was admitted to our hospital because of progressive renal
dysfunction associated with bloody diarrhea. The patient had consumed half-roasted
meat 12 days before this admission. He had been in excellent health during the next
6 days, but then he began to experience abdominal pain with nonbloody diarrhea and
visited a local physician who prescribed fosfomycin, probiotics, and
butylscopolammonium bromide. The next day (8 days after the half-roasted meat
consumption), he presented at and was admitted to another teaching hospital, as the
diarrhea persisted with some stools appearing to consist entirely of blood. A
laboratory analysis revealed elevated levels of lactate dehydrogenase (LDH) and
C-reactive protein (CRP) at 271 IU/L and 3.6 mg/dL, respectively, as well as obvious
leukocytosis of 22 590/μL with a serum creatinine (Cr) level of 0.59 mg/dL,
hemoglobin (Hb) level of 17.6 g/dL, and platelet count of 28.1 × 104/μL.
Diagnostic noncontrast abdominal computed tomography (CT) revealed an inflamed colon
( and ). He was therefore
presumptively diagnosed with infectious colitis and subjected to empirical
ceftriaxone at an intravenous dosage of 1 g every 12 hours combined with volume
resuscitation. Despite these managements, the clinical status of the patient
steadily worsened, and a second abdominal CT scan performed 3 days later revealed
the exaggeration of bowel thickening, distension of the small intestine, and
accumulation of ascites ( and ) with a
serum Cr level of 2.03 mg/dL and LDH level of 2662 IU/L. Other significant changes
in laboratory data were a decrease in the Hb level to 11.6 g/dL and in the platelet
count to 1.4 × 104/μL. He was therefore transferred and admitted to our
hospital for a further workup.
At the time of transfer (hospital day 1), the patient had somnolence with a
temperature of 38.3°C, a pulse of 101 beats/min, and a blood pressure of
108/52 mm Hg. Renal sonography of both kidneys revealed preservation of the size
with normal renal cortex echogenicity. The laboratory data on admission to our
hospital are shown in . A decreased level of haptoglobin of <10 mg/dL and schistocytes on
peripheral smears were also noted. The results of the patient’s bloody fecal culture
were unremarkable; however, antibodies to the lipopolysaccharide (LPS) of E
coli O157 were detected by latex agglutination. The patient was then
diagnosed with E coli O157–mediated hemorrhagic colitis. Based on
the laboratory findings, he was also suspected of being simultaneously complicated
with disseminated intravascular coagulation (DIC).
The day after admission (hospital day 2), a transient session of plasma exchange
(PEX), which replaced 3.5 L of plasma with the same amount of fresh-frozen plasma
(FFP), was commenced. Due to the oliguric nature of his renal failure, we decided to
apply intermittent hemodialysis (HD) concurrently. Prior to dialysis catheter
placement, random-donor platelet concentrates (250 mL, including
400 × 109 platelets) were transfused, resulting in a platelet count
of 9.7 × 104/μL. The device was then placed after confirming the patency
of the right internal jugular vein by duplex Doppler ultrasound, whereas a hollow
fiber polyethylene membrane filter with an average pore size of 0.3 µm and an
effective surface area of 0.8 m2 (OP-08; Asahi medical Co., Tokyo, Japan)
was used as the plasma separator. Before each session of the PEX, the intravenous
administration of 300 mg hydrocortisone sodium succinate was empirically provided as
allergic reaction prophylaxis. The patient was also subjected to supportive
management consisting of infusion of antithrombin III (1500 U/day) on 2 consecutive
days and intravenous recombinant human thrombomodulin (rhTM) at 8000 U/day.
He became alert and oriented on the hospital day 3. At this point, his bloody
diarrhea disappeared, but laboratory tests revealed increased serum levels of
amylase of 310 U/L with pancreatic isoenzyme predominance (99%) and lipase of
801 U/L, indicating concurrent acute pancreatitis; therefore, nafamostat mesilate
(NM) was administered not only as a regional anticoagulant for an extracorporeal
circuit but also as a therapeutic agent for the disease. Thereafter, the patient’s
urine volume gradually increased along with improvements in his platelet counts as
well as serum levels of LDH, so we decided to stop the PEX with HD on hospital day 8
when his platelet count rose to 23.4 × 104/μL. Despite the termination of
the systemic treatments with rhTM and NM, his abnormal clotting and fibrinolytic
profiles steadily improved, except for a slightly elevated prothrombin time
(PT)-international normalized ratio (INR), which was presumptively ascribed to the
reduced factor VII activity of 68.4% (reference range: 80.0%-115.0%) found on hospital day 24, and the
serum levels of amylase and lipase also decreased gradually but slowly ().
He was ultimately discharged on hospital day 31 with an improved sCr level of
0.83 mg/dL, fibrinogen degradation product level of 2.4 μg/mL, and d-dimer
level of 0.4 μg/mL. However, an additional 10 weeks were required to confirm the
settling of the serum levels of lipase and amylase at around 45 and 96 U/L,
respectively. We confirmed the normalized factor VII activity of 80.5%, PT-INR of
1.12, and haptoglobin of 25 mg/dL approximately 8 months after discharge.


A 24 year-old male with no significant past medical history and no history of smoking was transferred to our facility for evaluation of refractory colitis. Four weeks prior to transfer, the patient underwent a dental bone graft procedure and was administered antibiotics to include amoxicillin and clindamycin. Within 1 week the patient developed watery diarrhea up to 10–12 times daily. The patient was initially administered a 5-day course of metronidazole empirically, and initial Clostridium difficile PCR toxin assay while on metronidazole returned negative. The patient had no improvement in clinical symptoms on metronidazole.
Two weeks prior to transfer, the patient was evaluated by a gastroenterologist and second C. difficile PCR toxin assay returned positive. Treatment was initiated with vancomycin without clinical improvement. The patient was subsequently admitted to an outside facility 1 week prior to transfer with a CT scan of the abdomen demonstrating pancolitis (). The patient had no clinical improvement with oral vancomycin and intravenous metronidazole. He further described persistent abdominal pain, night sweats and a 11 kg weight loss.
On transfer to our facility, the patient had persistent bloody diarrhea. On physical examination the patient was afebrile and had diffuse abdominal discomfort. Initial laboratory studies revealed an elevated CRP, 41 mg/L, with a decreased albumin, 2.5 g/dL. Flexible sigmoidoscopy was performed demonstrating active colitis, without rectal sparing, and containing granular mucosa with a complete loss of vascular pattern (). Pathology revealed active colitis without changes of chronic colitis and without evidence for cytomegalovirus infection (). Stool culture on admission returned positive for Klebsiella oxytoca while repeat C. difficile PCR toxin assay following transfer was negative.
Given the presence of acute colitis and concern for antibiotic-associated hemorrhagic colitis secondary to K. oxytoca, antibiotics were withdrawn with clinical improvement in stool frequency. The patient was discharged with 1-week follow-up, at which time he continued to report symptoms of persistent colitis and weight loss. Repeat stool studies were performed to include a negative stool culture and positive C. difficile PCR toxin assay. Without formal diagnostic criteria present for UC, and in concern for recurrent C. difficile infection (CDI), the patient underwent a fecal microbiota transplantation (FMT) utilizing a third party source and instillation via colonoscopy, although no demonstrated clinical benefit was achieved. Colonoscopy at the time of FMT demonstrated persistent active colitis with a Mayo endoscopic subscore of 2 extending from the anal verge to the cecum (). Biopsies revealed moderately active chronic colitis, now consistent with a diagnosis of UC.
Given lack of response to therapies for antibiotic-associated hemorrhagic colitis and CDI, and with biopsies now favoring UC 3 weeks after initial presentation, the patient was hospitalized due to persistent colitis, weight loss and abdominal discomfort. Therapy was initiated for severe UC to include intravenous corticosteroids and cyclosporine. The patient was discharged on oral tapering doses of prednisone and cyclosporine with transition to oral azathioprine. Subsequently, salvage therapies to include infliximab, vedolizumab and tofacitinib were trialed without clinical or endoscopic response despite targeting recommended drug levels resulting in the performance of a total colectomy fifteen months after initial presentation. Pathology from the colectomy specimen was consistent with severely active chronic colitis consistent with a final diagnosis of UC (). Informed consent was obtained from the patient for description of this case.


In September 2015, a 45-year-old Korean female with ALK-rearranged metastatic lung adenocarcinoma was administered ceritinib 750 mg, a second-generation ALK inhibitor. Her brief anticancer treatment history comprised right lower lobectomy (pT3N0M0) in February 2014, followed by adjuvant chemotherapy with vinorelbine and cisplatin. From September 2014, she received pemetrexed and cisplatin as the first-line therapy for the metastatic disease, followed by pemetrexed maintenance.
In the subsequent second-line therapy of ceritinib in October 2015, she exhibited a partial response in accordance with the response evaluation criteria in solid tumor (version 1.1)[ with substantial tumor shrinkage of 78% (Fig. B) compared with that in the baseline scan (Fig. A). She maintained the partial response until a follow-up computed tomography (CT scan in April 2016 (Fig. C), which revealed newly appearing mass-like lesions in the left apex and right middle lobe (Fig. E). Assuming the presumptive disease progression, we performed a rebiopsy of the new lesion in the left apex to consider it for a new clinical trial; however, the transbronchial lung biopsy of the left apex mass revealed organizing pneumonia, non-infectious pneumonia defined as granulation tissue plugs within the lumens of small airways and extending into the alveolar ducts and alveoli.[
She underwent a repeat CT-guided gun biopsy of the left apex mass and a transbronchial lung biopsy of the right middle lobe mass to eliminate inappropriate targeting in biopsy; however, the pathology confirmed chronic inflammation (Fig. A) from the biopsy of the right middle lobe mass and organizing pneumonia (Fig. B) from that of the left apex mass. Because her symptoms included a mild cough without fever, she was presumably diagnosed with drug-induced organizing pneumonia. Thus, ceritinib was withheld, and the patient was treated with intravenous antibiotics, followed by oral antibiotics for two weeks. A 3-week follow-up CT scan revealed improved consolidation in the right middle lobe and left apex. Because the patient exhibited no specific symptoms, ceritinib was readministered at a one-level reduced dose from May 2, 2016. A 1-month follow-up CT scan revealed resolution of the consolidations in the left apex and right upper lobe (Fig. F). In July 2016, she attained a complete response (Fig. D); at present, she is still undergoing ceritinib treatment, having received 55 cycles of the treatment.


A 43-year-old premenopausal woman with Gravida 3 and Para 3 came to gynecology outpatient unit with complaints of irregular vaginal bleeding and lower abdomen pain since 6 months. There was no significant medical history. On clinical examination, vital signs were normal, and abdomen was soft with no palpable mass. Abdominal and pelvic ultrasonography (USG) was performed and revealed heterogeneous echogenic mass each in uterine corpus (3.5 cm × 2.5 cm × 2.5 cm) and cervix (1.2 cm × 0.8 cm). A diagnosis of leiomyoma in uterine corpus and cervix with degenerative change was made. Surgical specimen of hysterectomy was received for histopathological examination. Specimen showed a well-circumscribed gray-white to yellow tumor mass (3 cm × 2.5 cm) at upper pole of uterine corpus and yellow tumor mass (1 cm × 1 cm) in cervix [].
Histopathology examination of the tumor from uterine corpus showed a tumor composed of lobules of mature adipocytes separated by fibrous septae. At places, thin bundles of smooth muscle cells were seen. Small sized normal blood vessels were seen in . There was no nuclear atypia or increased mitotic figures. A diagnosis of lipoleiomyoma was made. Histopathology of tumor from cervix showed a tumor tissue composed of lobules of mature adipocytes, thick fibrous septae [], and few entrapped mucosal glands []. A diagnosis of pure lipoma was made.
Immunohistochemical (IHC) stains such as smooth muscle actin (SMA) and vimentin were done in sections of both the tumors to confirm the nature of cells. Smooth muscle cells, endothelial cells from the tumor of uterine corpus showed immunoreactivity with SMA and adipocytes were negative [] Vimentin staining showed immunoreactivity with smooth muscle, endothelial cells and mature adipocytes, fibrous septae were negative [].
In sections of cervical lipoma, only endothelial cells of blood vessels showed immunoreactivity with SMA, and fibrous septae and mature adipocytes were negative []. Mature adipocytes showed immunopositivity for vimentin []. A diagnosis of lipoleiomyoma in the uterine corpus and pure lipoma in the cervix was confirmed.


Patient is a 65-year-old male, a retired bank manager, who was first seen at the Haematology Day Care Unit in August 2016 having been referred from the Geriatric Center of the University College Hospital on account of a 5-month history of severe (score 7 of 10) and recurrent pain of the rib cage and low back. The pain was nonradiating and severe enough to disturb his normal daily activities. He had no associated constitutional symptoms. He presented to the source of referral at the onset of the illness where his hemoglobin electrophoresis was determined as HbS + C for the very first time ever. Analgesia was prescribed to him and this resulted in significant relief of the pain. Further questioning revealed that he had bone pain crisis in childhood but SCD was not diagnosed. However, the last episode of such was 35 years ago. He was never transfused with blood. He was married in a nuclear family with five children who are all well and alive. He does not smoke cigarette but stopped taking alcohol about 5 years ago.
Examination at presentation revealed a middle-aged man in no obvious distress, afebrile, tinge of jaundice, fair hydration status, no significant peripheral lymphadenopathy, and no pedal edema. Vital signs were within normal and breath sounds were vesicular. Moderate tenderness was elicited over the lower three ribs bilaterally. He was managed as a newly diagnosed HbS + C patient in moderate bone pain crisis and discharged home to complete investigations on outpatient basis and to return in a week for review with results. He, however, defaulted follow-up appointment only to return 4 weeks after with a more terrible pain and inability to stand from sitting and lying positions. He decided to go to a private facility from where he was referred back to the Hematology Department because an abdominal ultrasonography result revealed splenomegaly and para-aortic lymphadenopathy and hence a lymphoma was strongly suspected. Laboratory investigations revealed a full blood count with anemia (packed cell volume 27%), white blood cell 2700/mm3, and platelet count of 186,000/mm3. He had an elevated prostate-specific antigen of 15.6 ng/ml (0–4). Radiological findings include cervical spondylosis; anterior wedging of L2 vertebral body; and reduction in the height of T9, L1, L2, and L3 vertebral bodies. Further physical examination mainly established moderate tenderness over the anterior lower ribs and the flanks bilaterally and over the lumbosacral spine. At this point, working diagnosis was metastatic prostatic carcinoma rule out lymphoma in an HbS + C.
He was admitted for pain control and further evaluation. A bone marrow aspiration carried out revealed bone marrow plasmacytosis of 80% including binucleate forms and a few plasmablasts, which were suggestive of MM []. Further investigations such as serum protein electrophoresis, immunoglobulin quantitation, serum- and urinary-free light chains, skull and pelvic X-rays, urinary Bence Jones protein, and beta-2 microglobulin were requested to further confirm diagnosis. Patient could only afford beta-2 microglobulin and immunoglobulin quantitation. Result of beta-2 microglobulin was 2.7 ng/L (within normal limit) and immunoglobulin quantitation showed elevated immunoglobulin A (IgA) of 17.6 μg/dl; hence, a diagnosis of IgA myeloma was made in International Staging System-stage 1.
He was commenced on chemotherapy with melphalan (PO 7.5 mg/m2 × 5 days), prednisolone (PO 60 mg/m2 × 5 days), and thalidomide 100 mg daily in a 42-day cycle. Neurosurgeons equally co-managed on account of the vertebral and lumbar spine lesions and pain. He was initially kept on strict bed rest to stabilize the spine. He later had magnetic resonance imaging of lumbosacral spine and flexion/extension radiological study of the lumbosacral spine which was found satisfactory. He was subsequently commenced on ambulation, after a thoracolumbar spine orthosis was applied.
He has tolerated so far, five cycles of chemotherapy, as well as, physiotherapy very well and have made a significant improvement. The bone pain has resolved and he is now fully ambulant without support. He has also received three doses of intravenous zoledronic acid 4 mg monthly.


A 70-year-old man patient weighing 55 kg was admitted to emergency department with a complaint of severe weakness, lightheadedness, and lower limb paresthesia. Before this admission, he also presented worsening of symptoms in the last two weeks. He had no family history of cardiovascular disease, sudden cardiac death, or syncope.
On admission, hemodynamic parameters were in acceptable condition. He had blood pressure of 79/99 mmHg, heart rate of 57 beats/minute, and respiratory rate of 20 breath/minute, and he was also afebrile. Oxygen saturation, at rest, with finger pulse oximeter in index finger was 95%.
Primary physical examination was also done and we found the S4 sound in the apex, in cardiac auscultation. He was in class 2 of the New York Heart Association (NYHA) Functional Classification. Laboratory findings showed a mild hypochromic microcytic anemia [hemoglobin = 10.9 g/dl, hematocrit = 30.3%, mean corpuscular volume (MCV) = 73.09 fl, and mean corpuscular hemoglobin (MCH) = 21.67 pg]. He had degrees of hypothyroidism with thyroid stimulating hormone (TSH) of 20 mU/l and serum thyroxine (T4) of 2.4 µg/dl.
Electrocardiography (ECG) showed right bundle branch block (RBBB) with right axis deviation and complete atrioventricular block that diagnosed as a Trifascicular block ().
The patient referred to echocardiography unit and transthoracic echocardiography showed a moderate increase in thickness of left ventricular (LV) wall as a concentric pattern and LV diastolic dysfunction (grade 2). The ejection fraction of LV (LVEF) was 55%. Echocardiography revealed normal right ventricle size and function as well [tricuspid annular plane systolic excursion (TAPSE) = 1.9 cm] (). Cardiac biomarkers revealed that troponin I was negative with elevated N-terminal prohormone of brain natriuretic peptide (NT-ProBNP) equal to 288 pg/ml.
Regard to severe weakness and paresthesia, he referred to neurology department for electrodiagnostic study. The electrodiagnostic evaluation revealed absent sensory nerve action potential (SNAP) with low amplitude compound muscle action potentials (CMAPs) of upper and lower limbs. These findings were compatible with chronic sensory-motor polyneuropathy with axonal features, and evidence of ongoing axonal loss.
According to the sustained anemic situation and severe weakness, the patient underwent bone marrow aspiration. Microscopic evaluation of bone marrow aspiration revealed elevated cellularity with myeloid hyperplasia to erythroid hypoplasia ratio of 3:1, and complete maturation. Plasma cells were 22% of all nucleated cells. This finding was compatible with plasma cell myeloma.
Laboratory investigations including serum and urine electrophoresis and serum free light chain examination as immunofixation assay were also done. The results showed that kappa (κ) chains predominated over lambda (λ) chains in a ratio of 3:2. The hepatic test showed elevated alkaline phosphatase value equal to 215 U/l.
Ultimately, by considering all clinical aspects and paraclinical investigations mentioned above, final diagnosis for the patient was amyloid light-chain (AL) cardiac amyloidosis.
Immediately, the targeted therapy was established. Regarding clinical and paraclinical parameters, the patient received melphalan combined with high-dose dexamethasone. Concurrently, immunotherapeutic strategy with CPHPC and monoclonal antibodies were also prescribed. The patient closely monitored for 2 weeks and during this treatment period, he showed hematologic and organ response to the therapy. After this period, kappa to lambda ratio in urine immunofixation electrophoresis was normal, and serum level had a negative result. Heart response included no change in normal left ventricular ejection fraction (55 %), enhance the NYHA class of 2 to 1 without using any medications such as diuretic, and no wall thickness enhancement. NT-ProBNP was in normal range (100 pg/ml). The hepatic test revealed that alkaline phosphatase value had a dramatic decrease in 101 U/l (more than 50%).
After completion of treatment, the patient was discharged and advised to follow his situation and disease by referring to heart clinic every 2 weeks until 6 months, and referring electrophysiologist to consult the indications of an implantable cardiac defibrillator.


A 32-year-old woman who had been attempting to achieve pregnancy for 6 years was referred to our reproductive medicine centre with a request for intracytoplasmic sperm injection in October 2016. The indication was primary infertility, and her husband had asthenozoospermia and teratospermia. At 3 days after oocyte retrieval, the patient presented to our hospital complaining of 7 hours of right lower quadrant and right flank pain with nausea and vomiting for no known cause. Her temperature and blood pressure were normal. An abdominal examination showed normal bowel sounds, moderate right lower quadrant tenderness, and voluntary guarding. A vaginal examination showed a tender mass of 10 cm at the right iliac fossa with rebound tenderness. An ultrasound scan indicated hyperstimulation with enlarged ovaries that measured 97 × 68 mm (right ovary) and 71 × 50 mm (left ovary), and excessive fluid in the pelvic area. Doppler imaging showed diminished right ovarian vascular flow compared with the left ovary. A diagnosis of OHSS with right ovarian torsion was made and an emergency operation was immediately performed. At laparoscopy, there was approximately 500 ml of clear ascitic fluid. The right ovary was 9 × 6 × 5 cm in size, dark blue, and the pedicle was twisted 360 degrees (). The left ovary appeared healthy, was 5 × 4 × 4 cm in size, and had multiple follicles on the surface. At 5 minutes after untwisting the right pedicle, there was an improvement of colour. We continued observation until the right ovary, as well as the fallopian tube, turned a similar colour to the left ovary 30 minutes later (). At 9 days after operation, an ultrasound scan indicated that the right ovary was 63 × 48 mm in size with good blood flow signals. The patient was then discharged. On 3 April 2017, frozen-thawed embryo transfer was performed, and her pregnancy test 12 days later was positive. Currently, the patients is asymptomatic and in her 7th month of pregnancy.
The patient provided verbal informed consent for publication of this report.


We present a case of pseudoaneurysm in a 40-year-old man with long-standing chronic alcohol pancreatitis.
He presented to the local tertiary referral centre complaining of chest pain and the normal ECG, cardiac markers and chest X-ray suggested a non-cardiac cause for the pain. He remained haemodinamically stable and was further investigated with an abdominal ultrasound (USS) and a contrast enhanced computed tomography (CT). The latter demonstrated a 5 × 3.8 cm2 pseudoaneurysm posterior to the stomach and superior to the body of the pancreas but of unclear origin. It also showed a large subcapsular splenic haematoma measuring 10.8 × 7.7 cm2 and a perisplenic collection (Fig. ).
At this point urgent angiography for embolization was planned. In the meantime increasing complaints of abdominal pain prompted a review and optimization of the medication. In preparation for the procedure the clotting profile was checked and the patient gave written informed consent.
Selective angiogram using the Seldinger technique via left brachial approach to visualize the celiac trunk and superior mesenteric artery branches failed to show the pseudoaneurysm leading to the conclusion that possibly it had a different origin not accessible by angiography (Fig. ).
The next step was an endoscopic ultrasound guided thrombin injection. This was carried out and 2 ml of thrombin was injected into the pseudoaneurysm to good effect with no immediate complications. Also, percutaneous drainage of the perisplenic collection was carried out, where 400 ml of pus was drained. A follow-up duplex ultrasound scan confirmed that there was no flow in the pseudoaneurysm (Fig. ).
At this point it looked like the therapeutic approach was successful and on the ward the patient appeared clinically stable.
However, 4 days after the ultrasound guided injection, the patient collapsed on the ward with haematemesis and hypovolaemic shock. He was resuscitated initially with intravenous fluids and transfusion of blood products and remained alert with no abdominal signs. An emergency gastroscopy showed a 1.5 cm gastric ulcer on the posterior gastric wall with a large clot in the upper stomach but no active bleeding. A repeat CT abdomen demonstrated persistent flow in the pseudoaneurysm (Fig. ).
3D reconstruction images of the CTA revealed that the pseudoaneurysm was localized on an unnamed vessel branching directly from celiac trunk (Fig. ). This deemed not suitable for endovascular embolization due to its size.
As a result the decision was made to proceed with laparotomy. The procedure involved evacuating the haematoma, and over sowing the bleeding vessel and a subtotal gastrectomy with roux-en-y gastro-jejunostomy.
The patient returned to intensive care postoperatively where his progress was delayed by hospital acquired pneumonia, episodes of failed extubation, agitation and moderate malaena managed with blood transfusion. He returned to the ward and was eventually discharged after 5 weeks of overall hospital stay.
At an outpatient review 6 weeks after discharge the patient was generally well but complained of ongoing diarrhoea and failure to gain weight. This was attributed to chronic pancreatitis and managed with enzyme supplements and high calorie additives.


This is a 76-year-old male who came to the Emergency Department with intense periumbilical abdominal pain radiating to the back and vomiting. The patient underwent a right laparoscopic radical nephrectomy 18 days before. There were no complications during the procedure and post-operative period. Physical examination showed right basal hypoventilation with abdominal pain and tenderness in the right upper quadrant. A CT scan showed an orifice in the right diaphragm, through which intestinal loops passed into the thorax, which were dilated and with signs of ischemia (Fig. ), image not present in the pre-operative CT. The patient underwent emergency surgery, via a right subcostal laparotomy. A diaphragmatic hernia ~6 cm in size was found in the right posterior costophrenic angle with a nonviable loop of ileum, reduced and resected after mobilization of the right hepatic lobe. The orifice was closed with interrupted tension-free stitches. Post-operative recovery was uneventful. One year after the surgery, the patient is asymptomatic and shows no signs of hernia recurrence.


This is a 59-year-old woman. She underwent a left laparoscopic nephrectomy 4 years ago. She was admitted into the Emergency Department with anterior chest pain irradiated to the left shoulder changing with respiratory. The oxygen saturation was 90% and auscultation revealed a lack of breath sounds in the lower half of the left side of the chest. The abdomen was soft and not tender. The CT scan showed a large left diaphragmatic hernia, with intra-thoracic dilated stomach, spleen and descending colon, that led to the collapse of a large part of the left lung, with shifting of the mediastinum towards the right side of the chest (Fig. ), not present in the pre-operative CT. An emergency laparotomy was performed, identifying a major left diaphragmatic defect, with herniation of the structures previously described that were reduced and performing a tension-free suture with interrupted stitches. There were no complications during the post-operative period. One year later, the patient is in good condition with no signs of recurrence.


In a 47-year-old Caucasian male (weight 70 kg, height 177 cm), with a known history of hypertension, beta-lactam-documented allergy, and on hemodialysis for 3 years prior to this event, a dialysis catheter was inserted 30 months prior to the incident. Peripheral blood cultures as well as blood drawn from the catheter revealed methicillin-sensitive S. aureus (MSSA) bacteremia. To avoid possible allergic manifestations, the patient was treated with vancomycin 2,000 mg i.v. (28.5 mg/kg) as a loading dose in the last 90 min of dialysis followed by 1,000 mg i.v. (14.2 mg/kg) after each subsequent dialysis (in the last 30–60 min of dialysis) plus combination vancomycin (2.5 mg/mL)/4% citrate antibiotic lock for 3 weeks. Vancomycin pre-hemodialysis concentrations were > 15 mg/L (target 15–20 mg/L) during the 2 weeks of intravenous antibiotic therapy. The treatment was initiated while the patient was an outpatient. The patient experienced complete resolution of bacteremia confirmed by negative blood cultures. The catheter was removed 5 months after transplant surgery.


A 34-year-old white female (weight 46 kg, height 166 cm) with a medical history significant for type I diabetes, hypertension, ischemic heart disease, and on hemodialysis for 3 years prior to this encounter, grew coagulase-negative Staphylococcus from her CVC (oxacillin susceptible), where the catheter was changed on the same day of suspected blood stream infection. The patient was treated with parenteral cefazolin 2 g thrice weekly after each dialysis session in addition to cefazolin (5 mg/mL)/heparin (5,000 units/mL) lock solution for the duration of 1 week as deemed appropriate by the prescribing physician. The treatment was initiated and completed in the inpatient setting. Repeated cultures were all negative and the catheter remained in place with no recurrence in the 3-month follow-up period.


A 37-year old African-Canadian male (weight 77 kg, height 180 cm), with a documented history of hypertension and type II diabetes, had been receiving hemodialysis for 4 months prior to this encounter. MSSA was recovered from blood samples withdrawn via CVC (which was inserted 2 months earlier) and peripheral veins. The treatment regimen consisted of cefazolin 2 g i.v. thrice weekly after dialysis plus cefazolin (5 mg/mL)/heparin (5,000 units/mL) antibiotic lock instilled at the end of each dialysis for 2 weeks; the treatment started as inpatient and continued in the outpatient dialysis unit. All repeat cultures were negative and the permanent catheter was not removed for the follow-up period of 3 months.


An 81-year-old quadriplegic African-Canadian female (weight 64 kg, height 152.5 cm), with a known history of hypertension, type II diabetes, and requiring hemodialysis 55 months prior to this encounter, was diagnosed with MSSA bacteremia, with blood samples collected from the CVC (which was inserted 21 months before the incidence of bacteremia) and peripheral veins. A prescription for cefazolin 2 g i.v. after each dialysis and cefazolin (5 mg/mL)/heparin (5,000 units/mL) lock solution for 2 weeks were administered. Treatment was started in the outpatient dialysis unit. All repeat cultures were negative and the catheter was conserved intact. The patient expired 60 days later after admission; she was found unresponsive at a nursing home.


A 36-year-old white male (weight 71 kg, height 175 cm) with hypertension, type I diabetes, heart disease, and on hemodialysis 51 months prior to this incident, developed MRSA bacteremia as shown by both CVC (which was inserted 1 week before the incidence of infection due to poor blood flow) and peripheral line blood cultures. The patient was treated with vancomycin 1,000 mg i.v. (14 mg/kg) initially, then 1,500 mg (21 mg/kg) after the subsequent dialysis (due to low trough level) followed by 1,000 mg i.v. after each subsequent dialysis with pre-hemodialysis concentrations > 15 mg/L (MRSA MIC was 0.5 mg/mL). Treatment was initiated in the inpatient setting and was completed in the outpatient dialysis unit. The patient was also treated with vancomycin (2.5 mg/mL)/4% citrate lock solution for a 2-week period. Microbiological eradication and resolution of symptoms were observed. The catheter was removed 1 month later after the patient had a successful kidney transplant.


A 67-year-old white female (weight 67 kg, height 160 cm) with a medical history significant for type II diabetes, hypertension, heart disease, and on hemodialysis for 23 months, developed MRSA bacteremia from the arterial line, CVC (which was inserted 4 months prior to this infection incident), and peripheral lines during her hospital admission and was started on both vancomycin 2 g (31.5 mg/kg) loading dose during the last 90 min of dialysis followed by 1 g after each subsequent dialysis with a pre-hemodialysis level between 13 and 15 mg/L (MRSA MIC of ≤0.5 mg/L). The patient also received vancomycin (2.5 mg/mL)/4% citrate lock solution. Due to catheter blockage by a blood clot irresponsive to dissolution by tissue plasminogen activator local treatment, the catheter was removed 6 months later due to loss of function. However, during the interim when the catheter was in use, there was no infection seen.


An 82-year-old Asian female (weight 55 kg, height 167 cm), with a known history of hypertension, heart disease, and on hemodialysis 28 months prior to this incident, developed bacteremia with Acinetobacter baumanii and coagulase-negative Staphylococcus. The patient was treated with vancomycin loading dose of 1,500 mg i.v. (27.2 mg/kg) during the last 60 min of dialysis followed by vancomycin 1,000 mg maintenance dose after each subsequent dialysis, along with parenteral gentamicin 160 mg (2.9 mg/kg) loading dose and 60 mg maintenance dose at the end of each dialysis, oral ciprofloxacin 500 mg daily, plus gentamicin 5 mg/mL solution in normal saline lock. The three antibiotics and lock solution were started in the outpatient dialysis unit and were continued for 2 weeks without requiring hospital admission. The patient did not experience recurrent infection for a follow-up period of 1 year and continued dialysis through the same catheter.


A 62-year-old white male (weight 125 kg, height 167 cm), with a known history of type II diabetes, heart disease, hypertension, stroke (paraplegic), and on hemodialysis 98 months prior to this infection incident, developed bacteremia secondary to coagulase-negative Staphylococcus from the CVC. The patient received vancomycin 2,000 mg i.v. (16 mg/kg) for 2 consecutive dialysis sessions followed by vancomycin 1,000 mg i.v. after each subsequent dialysis plus vancomycin 2.5 mg/mL/4% citrate lock for 2 weeks total with complete cure, and the patient continued using the same catheter in the 6-month follow-up period.


A 68-year-old woman was re-referred to the rheumatology clinic with worsening joint pain and morning stiffness in both hands. She was first diagnosed with seronegative rheumatoid arthritis at 32 years of age. She had previously taken penicillamine and then sulfasalazine which were stopped due to primary inefficacy. Subsequently her disease went into remission and she had been off all DMARDS for 7 years.
Her past medical history included hypertension and a gastric ulcer for which she was taking losartan and ranitidine. She was taking paracetamol and codeine as required. She had never smoked and drank 35 units of alcohol per week.
On examination she had a bronzed appearance but no stigmata of chronic liver disease. She had active synovitis at the left wrist and second MCP joint. The plan was to commence methotrexate but she was found to have a raised ALT of 156 IU/L with otherwise normal liver function tests and clotting. Her liver function tests had been normal 2 years previously.
She underwent a full liver screen and was found to have a serum ferritin of >2000 μg/L and transferrin saturation of 92%. An abdominal ultrasound was normal. Given her increased alcohol intake a fibroscan was performed which showed a score of 4.6 kPa correlating to no significant liver fibrosis. Haemochromatosis genotyping revealed she was YYHH genotype with C282Y mutations in a homozygous state consistent with hereditary haemochromatosis and she was entered into a venesection programme.
Haemochromatosis can result in a small joint polyarthritis and so her diagnosis of RA was revisited. However, on repeat testing she was strongly positive for both rheumatoid factor (>130 U) and anti-CCP (221 U) and previous x-rays of her feet showed typical marginal erosions consistent with RA. Haemochromatosis would be unlikely to result in significant iron overload causing joint symptoms in a premenopausal woman aged 32 due to regular menses.
Genetic liver disorders such as haemochromatosis can mimic RA. Iron overload occurs due to mutations in the haemochromatosis HFE gene leading to haemosiderin deposition throughout the body, including the joints and the liver []. The arthropathy associated with this condition is classically non-inflammatory and centred around the second and third MCP joints, proximal interphalangeal joints (PIP) and wrists []. The key features differentiating haemochromatosis arthropathy from RA are summarized in Table .


A 64-year-old woman with psoriatic arthritis was found to have a raised ALT on routine DMARD monitoring blood tests. Her psoriatic arthritis was in remission on methotrexate 15 mg once weekly, folic acid 15 mg once weekly and paracetamol as required. She had recently returned from a holiday in Spain and was treated for a chest infection with a course of amoxicillin. Clinically she was icteric but examination was otherwise normal. Blood tests revealed a bilirubin of 65 μmol/L, ALP 501 IU/L and ALT 628 IU/L. These had been normal one month previously.
The differential diagnosis for this patient’s presentation was considered to be either drug related (secondary to antibiotics or methotrexate) or a viral infection. The methotrexate was withheld and she underwent a full liver screen. An abdominal ultrasound showed fatty liver infiltrates but no other significant pathology. She was found to be positive for hepatitis E virus (HEV) IgG and IgM antibodies. The LFTs normalized within 4 weeks after which she was recommenced on methotrexate and have subsequently remained normal.
HEV is a ubiquitous single stranded RNA non-enveloped virus with four main genotypes. HEV1 and 2 are usually transmitted via contaminated water in developing countries whilst HEV3 and 4 are zoonotic, commonly infecting wild and domesticated pigs amongst other mammals []. The virus is well known for its severity in pregnant women, where mortality rates can approach 15–20% in contrast to healthy individuals where the infection is usually asymptomatic [, ].
In patients with compromised immune systems, such as patients on chemotherapy or transplant maintenance medication, chronic HEV infection, where HEV RNA persists for >3 months, is well documented []. Although frequently self-limiting these infections can rarely lead to liver cirrhosis [].
Patients with inflammatory arthritis receive similar immunosuppression from DMARD therapy and therefore are at increased risk of chronic HEV infection []. Similar to healthy individuals HEV infected RA patients are frequently asymptomatic however common complaints include jaundice, right upper quadrant abdominal pain and fever [].
A retrospective study, although low powered, showed good rates of clearance of HEV in these patients within three months. This was achieved either spontaneously or through DMARD withdrawal/dose reduction. The antiviral agent, ribavirin, is indicated when LFTs remained persistently raised [].
Follow up virology testing, monitoring viral load and HEV IgM levels (positive in this patient), is important in these patients to confirm HEV clearance especially when LFTs remain abnormal [].
HEV serology should be performed in patients on with DMARDS who develop abnormal liver function tests.


A 60-year-old man with multiple hepatocellular carcinoma and hepatitis B and C cirrhosis (Child-Turcotte-Pugh grade A and Model for End-Stage Liver Disease score, 4) was scheduled for LT. He also had diabetes and a small nodule in the left lower lobe of the lung. His routine laboratory result was unremarkable showing normal electrolyte levels, without thrombocytopenia nor any other bleeding tendencies. Preoperative chest radiograph was unremarkable except for the small nodule described above. ECG showed normal sinus rhythm with heart rate (HR) of 93 bpm and corrected QT (QTc) interval 474 ms. Transthoracic echocardiogram (TTE) showed an ejection fraction of 58%, ratio of mitral peak velocity of early filling to early diastolic mitral annular velocity (E/E’ ratio) of 6.79, right ventricular systolic pressure of 26 mmHg.
Induction and maintenance of general anesthesia were performed with propofol, remifentanil, cisatracurium, and desflurane. Besides standard monitoring which include ECG, noninvasive blood pressure (BP) device and oxymeter, a Vigileo monitor with FloTrac sensor and PreSep catheter (Edwards Lifesciences LLC; One Edwards Way, Irvine, CA) was applied through arterial and central catheterization because LT has a high possibility of profound hemodynamic alterations. Arterial catheters were inserted at both right radial and femoral arteries. Advanced venous access High Flow (AVA-HF, 9-French, Edwards Lifesciences LLC) and PreSep catheter were inserted at the right internal jugular vein. Insertion of pulmonary arterial (PA) catheter was not considered because the patient had no significant dysfunction at right heart and did not seem to have pulmonary hypertension during the preoperative checkup. Initial vital signs were stable, showing BP, HR, CVP, and body temperature (BT) of 142/82 mmHg, 71 bpm, 8 mmHg, and 35.5°C, respectively. A Vigileo monitor with FloTrac and PreSep continuously analyzed and informed the other hemodynamic values. Baseline results of the measurement showed cardiac index (CI), stroke volume variation (SVV), and central venous oxygen saturation (ScvO2) of 2.3 L/min/m2, 6%, and 87%, respectively (Table ). During preanhepatic to anhepatic phase, no significant change was observed in the vital signs. Laboratory results were in the normal range. Although no significant change was observed in the QT interval compared to the preoperative QT interval, 1 g magnesium sulfate was replaced. Because the laboratory value of magnesium was checked in the low normal range, and the preoperative QTc interval was ≥440 ms, the incidence of arrhythmia could be higher.[ We started the continuous infusion of calcium chloride at 450 mg/h since anhepatic phase to maintain ionized calcium concentration at approximately 1.0 mmol/L. Fluid replacement was mainly based on 5% albumin with crystalloid, and urine output was approximately 100 mL/h on average. Arterial blood gas analysis performed before reperfusion showed Hct of 38% and ionized sodium, potassium, and calcium concentrations were 130, 3.88, and 0.92 mmol/L respectively.
During reperfusion, neither vasopressor nor inotropic management was required to maintain BP because it did not drop significantly and recovered to its baseline within 1 min. However, CVP suddenly increased from 6 to 16 mmHg immediately after reperfusion and ST segment depression was also developed, which decreased to almost −5 mm (Fig. A, B). Hemodynamic values at this time showed CI, SVV, ScvO2, and BT of 4.8 L/min/m2, 10%, 90%, and 35.2°C, respectively (Table ). Considering that BP was quite stable after reperfusion while significant changes were found in CVP and ST segment along with slight increase in CI and ScvO2, we could assume that it was caused by sudden volume overload in the right ventricle after reperfusion rather than the possibility of thromboembolism or hypovolemia. Because CVP alone is not sufficient to reflect right ventricular preload accurately, we wanted to apply transesophageal echocardiogram (TEE) to check right ventricular volume or function. However, only TEE at our operating center was used in open heart surgery. The pulmonary arterial pressure (PAP) was not available because the PA catheter was not inserted in this case. We suspected that sudden increased preload from the grafted liver might became a profound burden which might led to right ventricular ischemia with the change of ST segment. Although we lacked of information from TEE or PA catheter, we immediately performed active venesection of 300 mL through one of the AVA-HF lumen and started to infuse isosorbide dinitrates at the rate of 30 μg/min. After this maneuver, both CVP and ST segment returned to baseline figure as they were before reperfusion within 15 to 20 minutes (Fig. A, B).
Postoperative ECG showed normal sinus rhythm with HR and QTc interval of 80 to 90 bpm and 420 to 440 ms without any abnormal signs of ST segment, respectively. Although laboratory tests performed immediately at the day of the surgery showed troponin I of 0.163 ng/mL, it tended to decrease and subsequently became normal. TTE showed no significant changes compared with preoperative values. At postoperative day 21, the patient was discharged without any surgical or cardiovascular sequelae, and he is still clinically stable with regular checkup for the last 7 months.