A female infant weighing 2.8 kg was born at 39 weeks, 2 days gestation to a 28-yr-old mother after an uncomplicated pregnancy. On the 3rd day of life, she presented with three brief generalized tonic seizures with upward deviation of the eyes and cyanosis of the lips which lasted approximately one minute. General and neurologic examinations were unremarkable. Electrolytes, calcium, glucose, cerebrospinal fluid examination, brain magnetic resonance imaging and electroencephalography (EEG) were all normal. She was discharged after 3 days without further seizures on phenobarbital. In follow-up, her phenobarbital was weaned at 3 months of age, but her seizures recurred 1 month later. Her EEG showed several spike discharges originating from the left and right central-temporal areas at that time. Phenobarbital was restarted and tapered off successfully without any seizure recurrence at 7 months of age. The child has had a normal neurodevelopmental course thereafter.
Detailed review of her family history revealed five affected family members, in three separate generations, who experienced similar neonatal convulsions, including the patient's grandfather, father, paternal uncle, and cousin ().
Ia (Grandfather): Patient Ia had 3 convulsions within the 1st month of life, but no further seizures after those initial episodes.
IIc (Paternal uncle): This patient experienced 5 convulsions within the first 3 months of his life. No further seizures were noted thereafter. The patient graduated from a postgraduate school.
IIe (Father): He experienced a single convulsion on the 2nd day of life. He also graduated from a postgraduate school and has no neurologic problems.
IIIc (A cousin): Eight months-old at the time of this writing. Although he experienced 6 convulsions within the 6 months of life, he has shown normal development.
In sum, despite multiple infantile seizures, no epilepsy or psychomotor dysfunction occurred in any affected family members even without the use of antiepileptic drugs.
To search for mutations in KCNQ2 in the pedigree, 16 exons and adjacent short intronic sequences of the gene were amplified by polymerase chain reaction (PCR) with DNA obtained from the affected individuals. Sixteen exons of the KCNQ2 gene and their intronic flanking sequences were amplified by PCR using a total of 19 sets of primers with exon 1 split into two and exon 6 into three primer pairs.
Genetic study revealed an R448X mutation located in the C-terminal segment of KCNQ2 in the family members with neonatal seizures (Ia, IIe, IIId, ).


A 64-year-old man visited the emergency department with acute shortness of breath. It was his first episode, and it came on abruptly; twenty minutes earlier, he was asymptomatic and sleeping comfortably at home. His medical history included hypertension for more than five years, but he had not received treatment for it. He was a heavy smoker (45 pack years). Physical examination revealed arterial hypertension (260/140 mmHg). He had widespread wheezes and rales on auscultation, and the chest radiograph demonstrated bilateral interstitial shadowing (). Additional investigations were performed. The serum creatinine level was in the normal range. The N-terminal pro-B-type natriuretic peptide (NT-proBNP) increased by 1,163 pg/mL. His electrocardiogram showed changes consistent with left ventricular hypertrophy and left atrial enlargement but no acute ischemic changes. Echocardiography demonstrated concentric left ventricular hypertrophy, left atrial enlargement, and diastolic dysfunction (grade 2) with an ejection fraction of 73%. He was hypoxic and was intubated and ventilated. After intubation, he improved rapidly without any special medications and was weaned from the mechanical ventilator within 1 hour. He was admitted to the intensive care unit for close observation. The pulmonary edema had subsided in the follow-up chest radiograph (). These findings are characteristic of flash pulmonary edema. Since he had good left ventricular function we suspected a renovascular etiology for this pulmonary edema episode. CT angiography of the renal arteries revealed severe stenosis of the right renal artery ostium (). We performed a selective renal angiography, which revealed a 95% segmental ostial stenosis in the right renal artery (), and a direct stent implantation (6.0×15 mm Genesis; maximum pressure of 12 atm) was performed (). There was 10% residual stenosis and no detectable dissection. He remained stable for the next 48 hours without any complications. He was discharged, and out-patient follow-up was arranged. He has remained free of pulmonary edema and hypertension. Follow-up CT angiography revealed no restenosis two months after the stent placement ().


A 48-year-old male presented at our hospital with typical chest pain. His past medical history was unremarkable. His coronary risk factors only included "current smoker". On physical examination, he had a systolic blood pressure of 120 mmHg and a heart rate of 62 beats per minute. An initial electrocardiogram (ECG) showed no definite ST segment change and sinus rhythm (). Initial creatine kinase-MB and cardiac troponin I levels were in the normal range. Transthoracic echocardiography showed no regional wall motion abnormality and normal left ventricular function. We administered aspirin 300 mg for 1 day. CAG revealed a filling defect of the distal left anterior descending artery (dLAD), distal left circumflex artery (dLCX) and LMA (). We decided to aspirate the LMA thrombus and to do PCI at the dLAD and dLCX. A guiding catheter was positioned in the left coronary artery ostium. At that time, he complained of aggravating chest pain. On CAG, the distal region of the 1st diagonal branch (D1) and the obtuse marginal branch (OM) were not visualized (). We thought that this event had developed due to coronary embolization that resulted from the migration of thrombi into the LMA during CAG. Intravascular ultrasonography showed the LMA thrombus (). We tried to aspirate the thrombus in the LMA using a guiding catheter and to do balloon angioplasty in the dLAD and dLCX. Balloon angioplasty was successful only for D1 (). However, the dLAD, dLCX and OM had Thrombolysis In Myocardial Infarction (TIMI) grade 1 flow in spite of repeated balloon angioplasty. Applied platelet glycoprotein IIb/IIIa receptor antagonist (Abcimixab, Reopro®) and balloon angioplasty of the dLAD, OM and dLCX were done sequentially. However, filling defects in the dLAD, OM and dLCX remained ().
We cared for the patient in the cardiovascular care unit. Creatine kinase-MB and cardiac troponin I levels were elevated to 349.4 ng/mL and 40.91 ng/mL, respectively. Follow up ECG showed a 3 mm ST segment elevation at leads II, III, aVF and V3-5 after angiography (). We thought that the AMI developed due to distal embolization of the LMA thrombus during angiography. Laboratory profiles associated with hypercoaguability such as lupus anticoagulant, protein C activity 102% (NL 70-130%), protein S activity 74% (NL 73.7-146.3%), D-dimer 0.36 µg/mL (NL 0.0-0.4 µg/mL), antithrombin III 91% (NL 80-120%) and homocysteine 12.2 µmol/L (NL 5.0-15.0 µmol/L) were in the normal range.
After 3 days of intravenous unfractionated heparin (25,000 IU/day), follow-up CAG showed only slight resolution of the filling defect and only in the dLAD, not in the OM and dLCX (). He had no chest pain and was discharged on triple antiplatelet agent. After 1 year of this treatment, he experienced no chest pain and, on follow up CAG, had no significant stenosis or intracoronary filling defects ().


A 45-year-old male patient was admitted with effortinduced chest pain for a month. He presented no cardiovascular risk factors except for dyslipidemia. The electrocardiogram was sinus bradycardia without any ischemic changes such as changes in ST-T. Furthermore, the echocardiogram showed no regional wall motion abnormality with normal ejection fraction. However there was significant ST segment depression at Bruce protocol stage 1 during the treadmill test. A multidetector computed tomogram revealed total occlusion with positive vascular remodeling at proximal LAD and middle RCA.
Diagnostic coronary angiography (CAG) was performed. The CAG revealed a total occlusion in distal RCA and significant stenosis in mid RCA and proximal to mid LAD (). The patient underwent percutaneous coronary intervention (PCI) at the mid to distal RCA with a 3.0 by 33 mm Cypher (Cordis, Miami Lakes, FL, USA) with 20 atmospheres {minimal lumen diameter (MLD) 2.72 mm}.
To revascularize the LAD lesion, DESs were successfully implanted with a 3.5 by 23 mm Cypher and 3.0 by 23 mm Cypher with 20 atmospheres covering proximal to mid LAD with overlapping technique (MLD 2.93 mm). Intravascular ultrasound (IVUS, Atlantis Plus 40 MHz; Boston Scientific, Natick, MA, USA) was then performed with a pullback speed of 0.5 mm/sec from distal to middle RCA and from middle to proximal LAD. Nine months later, a scheduled follow-up CAG was performed ( and ).
A subsequent IVUS showed that there was remarkable separation between the stent struts and the vessel wall indicating the extensive development of the lateacquired ISA in the stented segments of both LAD and RCA. The maximum and mean depth of ISA in LAD was 0.89 mm and 0.5 mm respectively, while that of ISA in RCA was 1.09 mm and 0.63 mm respectively. The length of ISA in LAD and RCA was 34.2 mm and 26.2 mm respectively. Furthermore, focal stent fracture also existed within the stent body in RCA. The stent strut-free length was 1.42 mm. The patient received 100 mg of aspirin as well as 75 mg of clopidogrel as part of the antiplatelet therapy. There was no evidence of any major adverse cardiac events during a 25-month followup period, post detection of late-acquired ISA.


A 68-year-old female presented with focal seizures of the left arm. MRI-imaging was done and a lesion with radiological characteristics of a small meningeoma immediately above the right central sulcus was diagnosed. An fMRI demonstrated that left-sided “finger tapping” resulted in activation within the motor cortex immediately adjacent, lateral, and anterior to the tumor (Fig. ). Ri-TMS was performed and demonstrated clear delineation of individual areas of maximal MEP response for the m. brachioradialis, m. abductor pollicis brevis, and m. abductor digiti minimi. The areas of maximum MEP response by Ri-TMS were transferred to the neuronavigation (Fig. ). A neuronavigation-guided craniotomy exposed the tumor and a small area of adjacent cortex which was examined by direct electrical cortex stimulation. The area of maximal MEP response by ECS for the m. digiti was identified just lateral and anterior to the tumor. The cortical area was registered using the neuronavigation. Comparison of data acquired for the maximal MEP response for Ri-TMS and ECS showed that both areas localized to a morphologically distinct area of the proximal right “hand knob” within 5 mm. However, due to the small craniotomy, more lateral aspects of the precental gyrus could only be exposed following resection of the tumor. Repeat cortical mapping by ECS now identified the cortical motor area of the m. abductor pollicis brevis. Comparison of the spatial data of maximal MEP response by Ri-TCM and ECS now showed a mismatch of 16.9 mm, possibly due to brain shift during the course of the operation as a consequence of loss of CSF and the resected tumor volume.


A 2-month-old Korean baby girl developed dyspnea and irritability about a week before admission to our hospital. On physical examination at admission, her temperature was 36.2℃, blood pressure 80/60 mmHg, pulse 145/min, respiratory rate 28/min, and weight 4,200 g. Breathing sound decreased in the right lung field and was accompanied by chest wall retraction. There was no edema signs in extremities. Arterial blood gas analysis result was pH 7.26-pCO2 50.3%-pO2 97.7%-O2 saturation 96.3%. Otherwise physical examination and laboratory results proved unremarkable. On birth history, delivery occurred spontaneously at 37 weeks gestation and her birth weight was 3,000 g. A chest radiograph at admission showed a solitary cystic mass, entirely occupying the upper lobe of the right lung. High-resolution computed tomography (HRCT) of the chest depicted a well-circumscribed, multiseptate, air-filled cystic lesion in the upper lobe of the right lung, mimicking the feature of type I congenital cystic adenomatoid malformation ().
Under the preoperative diagnosis of congenital cystic adenomatoid malformation, she underwent bilobectomy of the upper and middle lobes of the right lung. Grossly, the large part of the upper lobe was replaced by multiple cystic spaces that contained some serous fluid and varied in size from 2-3 mm to 3.5 cm. Remained lung parenchyma is hemorrhagic and revealed emphysematous change. The middle lobe just showed emphysematous change and focal hemorrhage.
Histopathological examination of the upper lobe revealed a relatively circumscribed intrapulmonary cystic lesion composed of multiple cysts or cavities (). The cystic spaces were lined by a monolayer of flat or low cuboidal cells. They were interconnected each other. Their walls were supported by loose fibrous stroma with mild lymphocytic infiltrate (). Neither significant smooth muscle proliferation nor lymphoid follicles were observed in the walls. Lung parenchyma outside the cystic lesion exhibited emphysematous change and some lymphangiectasia in the connective tissues of interlobular septa and subpleural zones. In the immunohistochemical staining the lining cells of the cystic walls were not reactive for epithelial membrane antigen and cytokeratin, but weakly positive for factor VIII-related antigen (). Intrapulmonary cystic lymphangioma was subsequently diagnosed. The microscopic findings of the middle lobe were consistent with the gross finding.
The patient had an eventful recovery after operation. At 4-month follow up she remained well without evidence of recurrence.


A 65-yr-old man was referred to our hospital complaining of pain in the right upper quadrant and vomiting for previous 3 days. His past medical history was unremarkable except for a spinal operation performed due to a falling-down injury 10 yr ago. A physical examination demonstrated tenderness and a soft palpable mass on the right upper quadrant of the abdomen. The laboratory examinations showed a hemoglobin level of 15.3 g/dL (normal range, 14 to 18), a white blood cell count of 12,590/µL (normal range, 4,000 to 10,000), and a platelet count of 254,000/µL (normal range, 140,000 to 400,000). The liver function tests showed a total serum bilirubin level of 3.3 mg/dL (normal range, 0.1 to 1.2), a direct bilirubin level of 2.1 mg/dL (normal range, 0.1 to 0.4), alkaline phosphatase level of 323 IU/L (normal range, 20 to 90), a gamma glutamyl transpeptidase level of 371 IU/L (normal range, 0 to 50), an aspartate aminotransferase level of 267 IU/L (normal range, 10 to 35); alanine aminotransferase, 228 IU/L (normal range, 0 to 40); amylase, 972 IU/L (normal range, 50 to 160) and a lipase level of 2,767 IU/L (normal range, 0 to 200). A duodenoscopy revealed a huge extrinsic mass with an intact mucosa causing almost complete luminal obstruction in the second portion of the duodenum. On palpation with a biopsy forcep, the mass was found to be soft and easily compressible (). A computerized tomography scan showed a 12×6 cm sized elliptic cystic lesion with a distinct wall at the second-to-third portion of the duodenum. The lesion occupied the intramural portion of the duodenal wall. These findings above were consistent with a DDC (). The intrahepatic duct and common bile duct were dilated, and the gallbladder was markedly distended (). A percutaneous transhepatic biliary drainage for the relief of obstructive jaundice was performed one day after admission. pylorus-preserving pancreaticoduodenectomy (modified Whipple's operation) was performed 24 days after supportive treatment. The surgical specimens included the segments of the duodenum and the duplication cyst, the gallbladder and some portions of the pancreas. The duplication cyst was located along the antimesenteric border and was 9 cm in length and 2.8 cm in the inner diameter (). There was no luminal communication between the cyst and the duodenum. The inner surface of the cyst was flat and showed multiple erosions and ulcers. Histologically, the cyst showed denudation of the mucosal layer, which was replaced by granulation tissue, due to the mucosal inflammation, but most parts of the cyst contained a smooth muscle layer (). The post-operative course of the patient was uneventful, and the patient has been doing well for 6 months after surgery without any complication.


A 4-yr-old girl was admitted to the Pediatric Department on May 27, 2002, with a 1-day history of headache, fever (38.1℃), abdominal pain, and vomiting (). Her past medical history was unremarkable and she was up-to-date with her immunizations. Her physical and neurological examinations were unremarkable.
Laboratory tests revealed a peripheral white blood cell (WBC) count of 19,800/µL (91% neutrophils), a hemoglobin level of 11.6 g/dL, and a platelet count of 356,000/µL. Cerebrospinal fluid (CSF) had a hazy appearance with an increased WBC count of 890/µL (99% neutrophils), but no significant change in glucose concentration of 78 mg/dL, and a protein level of 31 mg/dL (). Routine blood chemistry results were within the reference range. A chest radiograph was normal.
The patient was treated with cefotaxime and ampicillin for 1 week. The patient became afebrile after 2 days of treatment, and her other symptoms also improved. The CSF analysis showed a decreased WBC count to 9/µL from 890/µL at hospital day 3. She was discharged on 9th hospital day without complications.


A 15-yr-old boy was admitted to the Neurology Department on May 18, 2005, with a 3-day history of headache and 2 days of generalized petechiae, nausea, and vomiting (). His past medical history was unremarkable. On admission, his oral temperature was 36.9℃, heart rate was 93 beats/min, and blood pressure was 125/82 mmHg. He was alert and complained about neck stiffness. There were no motor or sensory deficits, and the rest of his physical examination was unremarkable.
A laboratory investigation at the time of admission revealed a peripheral WBC count of 30,290/µL (96% neutrophils), a hemoglobin level of 15.4 g/dL, and a platelet count of 151,000/µL. CSF had a cloudy appearance with an increased protein concentration of 575 mg/dL, and a WBC count of 35,500/µL (90% neutrophils) and a decreased glucose concentration of 10 mg/dL (). Routine blood chemistry results were within the reference range. A chest radiograph was normal.
The patient was treated with ceftazidime and netilmicin for 2 weeks. His clinical condition was improved after 3 days, and the CSF analysis showed a decreased WBC count and protein concentration to 4,150/µL and 90 mg/dL from 35,500/µL and 575 mg/dL, respectively. On 13th hospital day, the CSF WBC count decreased to 130/µL, and the patient was discharged without complications on 18th hospital day.


An 81-yr-old man with a known history of pancreatic cancer (March, 2004) and liver cirrhosis due to hepatitis C virus infection (June, 2004) was admitted to the Emergency Department on June 6, 2005, with drowsy mental status for one day. On admission, he had icteric sclera ().
Laboratory investigation revealed a peripheral WBC count of 6,950/µL (73% neutrophils), a hemoglobin level of 12.8 g/dL, and a platelet count of 60,000/µL. CSF was clear with a protein level of 38 mg/dL, a slightly increased WBC count of 9/µL, and a glucose concentration of 76 mg/dL (). Routine blood chemistry showed increased blood urea nitrogen/creatinine of 41.8/2.2 mg/dL, ammonia of 287 µg/dL and total bilirubin of 3.1 mg/dL. The other test results were unremarkable.
The patient was treated with cefotaxime for 1 week. On hospital day 3, the CSF WBC count decreased to 0/µL. He was managed conservatively and was discharged 18th hospital day without complications.


A 66-year-old male patient, who lived in Switzerland and whose parents were originally from Turkey and Azerbaijan, was diagnosed with BD in 2004, based on ocular symptoms, erythema nodosum, positive pathergy test and typical vasculitis described on biopsy specimens from erythematous area. The patient was treated with methotrexate, colchicine and corticosteroids with substantial improvement of ocular and mucocutaneous symptoms. In the absence of classical cardiovascular risk factors, the patient developed myocardial infarction with ST elevation (STEMI) and positive enzyme tests. On coronarography, large aneurismal lesions of left marginal and left descending coronary artery were detected. Coronary angioplasty was unsuccessful, with ventricular fibrillation requiring electrical cardioversion. Later, the patient was discharged from the hospital on aspirin, clopidogrel, beta-blocker and peripheral calcium-channel blocker, corticosteroids and cyclosporine. After 6 months, cyclosporine was discontinued because of worsened glomerular filtration rate and advanced heart failure (NYHA III). Importantly, at that time laboratory tests revealed high levels of proBNP (up to 2,246 pg/mL). On ECG performed in 2008, there were occasional extrasystoles and pathological Q waves (old anteroseptal MI) (Fig. ). Echocardiography revealed decreased left ventricular ejection fraction (EF) (<36%), altered mitral flow [early diastolic/atrial flow (E/A)=0.5, fusion of E and A waves)]. MRI showed apical aneurysm with 2.5x2.3 cm calcified apical thrombus (Fig. ).


A 52-year-old woman (Swiss father, Greek mother), living in Switzerland since the age of 32, was referred for consultation with established diagnosis of BD in 2002 (recurrent oral ulcerations, retino-uveitis, erythema nodosum, perivasculitis in biopsy specimens). The patient was treated with corticosteroids, methotrexate and colchicine. The cutaneous and mucosal pathology improved dramatically. In 2005, she experienced suddenly developed dyspnea (NYHA II-III) and palpitations. New heart murmurs were detected. Besides, there were also several other signs of cardiac pathology: pathologic right apical bulging; marked and permanent splitting of the 2nd heart sounds, 4/6 degree tricuspid, 3/6 degree mitral regurgitation murmur; 2/6 systolic aortic and 2/6 diastolic murmurs over aorta; premature beats (supraventricular and ventricular in origin) and complete right bundle branch block on ECG; aneurysmal motion of the interatrial septum, dilatation of the sinus coronarius, mild mitral regurgitation, myxoid mitral and tricuspid valves, enlarged annulus of the thickened aortic valve, and severe mitral regurgitation on echocardiography (Fig. ). The calculated mean pulmonary pressure was 38 mmHg. Spiral CT excluded pulmonary lesions and embolization. The patient was treated with aspirin, diuretics and carvedilol. Occurrence of symptomatic paroxysmal atrial fibrillation and complex ventricular arrhythmias were successfully treated with amiodarone. Lately, the patient was asymptomatic and continued treatment with aspirin and carvedilol. On echocardiography there were features of myxoid mitral and tricuspid valves with insignificant mitral and moderate tricuspid regurgitation. The interatrial septum was thickened but without aneurysm. The mean pulmonary pressure was normal (18 mmHg).


A 66-year-old female patient (father from Algeria, mother from Afghanistan), who had been living in Switzerland since the age of 24, was diagnosed with BD in 2003 (oral aphths, genital ulcers, phlebitis, erythema nodosum, positive pathergy test). She was treated with high-dose corticosteroids in combination with cyclophospamide and colchicine. Non-cardiac symptoms ameliorated. In 2005, she was diagnosed with heart failure. On physical examination – mitral and aortic regurgitation murmurs, on ECG – I degree AV blockade, left ventricular hypertrophy, non-specific ST changes; on echocardiography – II degree mitral and aortal regurgitation, enlarged sinus coronarius and aortic root (Fig. ), and diastolic dysfunction (Fig. ). Due to the worsening of mitral regurgitation, the patient underwent mitral valvuloplasty 3 years later.


A 54-year-old Italian male patient was diagnosed with BD in 2002 (recurring genital ulcers, iridocyclitis, epididymitis, vasculitis with perivascular mononuclear infiltration on biopsy). He was treated with high-dose corticosteroids, methotrexate and colchicine with a substantial improvement of non-cardiac symptoms. In 2004, the patient presented with dyspnea (NYHA II) and diastolic murmur over the aorta. Echocardiography showed thickened aortic valve and mild dilatation of the ascending aorta (Fig. ). There were also signs of left ventricular diastolic dysfunction with preserved systolic function. Candesartan and carvedilol were added to the treatment. Dyspnea disappeared. On echocardiography, performed 9 months later, there were no signs of aortic dilatation. Diastolic dysfunction disappeared two years later.


A 55-year-old Greek male patient, who lived in Switzerland since the age of 20, was diagnosed with BD in 2002 (recurrent oral aphthous ulcerations, genital ulcers, phlebitis, erythema nodosum, positive pathergic test), and was treated with high-dose corticosteroids, methotrexate, colchicine. Non-cardiac symptoms responded to the treatment. ECG showed mild left ventricular hypertrophy with nonspecific changes of T wave. Two years later, the patient developed non-ischemic chest pain, dyspnea (NYHA II), and ECG revealed left ventricular hypertrophy, with marked horizontal ST depression in I, II and V4-6 leads, and giant, negative T waves in I, II, aVF, V4-6 (resembling changes of apical cardiomyopathy) (Fig. ). On echocardiography there were signs of left ventricular medial and apical endocardial fibrosis with advanced diastolic dysfunction (similar to the changes in Fig. ). There was also blood pressure elevation from 130/80 mmHg (2002) to 160/100 mmHg (2004). Therapy with candesartan, amlodipine and carvedilol was initiated with blood pressure normalization and cardiac symptoms improvement. However, electrocardiographic and echocardiographic findings remained unchanged.


A 64-year-old woman presented with chronic, waxing and waning uveitis in her left eye. She had undergone phacoemulsification and intraocular lens implantation in that eye at a local clinic nine months prior. Two weeks after surgery, mild anterior chamber inflammation developed. At first, the inflammation was well controlled with steroid eye drops. Over time, however, the inflammation repeatedly recurred. Whenever the patient stopped using steroid eye drops or when her general condition deteriorated, her left eye experienced anterior chamber inflammation. After nine months, she was referred to our clinic.
On initial examination in our clinic, her best corrected visual acuity (BCVA) was 20/25 in both eyes. Slit-lamp examination revealed grade 3 anterior chamber inflammation and mild vitreous opacity in the left eye. She was diagnosed with chronic uveitis and was prescribed steroid eye drops.
After seven weeks of steroid therapy, the inflammation worsened, and her visual acuity decreased to 20/40. On biomicroscopic examination, the anterior chamber inflammation had increased to grade 4, and plaque-like material was noted around the intraocular lens in the left eye. A fundus photograph showed mild vitreous opacity obscuring the fundus detail (). Laboratory tests, including complete blood cell count, a treponemal antibody test, rheumatoid factor measurement, an antistreptolysin O test, and measurement of autoimmune antibody, were all negative, except for a slightly increased erythrocyte sedimentation rate.
Pars plana vitrectomy along with a smear and culture of the aqueous and vitreous fluids was conducted, as delayed-onset chronic infectious endophthalmitis caused by P. Acne or fungus was suspected. The plaque-like material along the posterior lens capsule was removed as much as possible, but the intraocular lens was left in place since the patient strongly wi shed to maintain her pseudophakic state. At the end of vitrectomy, 10 µg/0.1 mL amphotericin B, 1 µg/0.1 mL vancomycin, and 2 µg/0.1 mL ceftazidime were injected into the vitreous. Examination of the vitreous fluid revealed WBCs and septated fungal hyphae of an Acremonium species in both KOH-Calcofluor staining () and Gram-staining (). With a diagnosis of Acremonium-related endophthalmitis, intravenous ampotericin B was given daily for post-operative five days. The patient was then discharged with directions to take oral voriconazole medication for four weeks.


A 69-year-old man was referred to our clinic in August, 2006 presenting with decreased visual acuity in his right eye. He had been diagnosed with CIMF in July, 2000 and continued to have supportive care for CIMF throughout his follow-up. He had no specific ocular history and no history of irradiation. Visual acuities were 20/25 in the right eye and 20/20 in the left. Slit lamp examination showed no abnormalities on the conjunctiva, cornea, anterior chamber and iris. Fundus examination disclosed a mild vitreous hemorrhage in the right eye (). The peripheral fundi of both eyes showed marked vessel occlusion and microaneurysms in the temporal area. Fluorescein angiography in both eyes showed arteriovenous anastomosis with a marked area of nonperfusion and multiple sea-fan neovascularization on both eyes (). On laboratory examination, hemoglobin was 4.9 g/dL, platelet count was 63,000/mm3, and white blood cell count was 2,500/mm3 (with no blast cells). A peripheral blood smear test showed teardrop-shaped red cells. His fasting blood sugar value was normal, and the hemoglobin electrophoresis study was negative. Bone marrow examination showed hypocellular marrow with severe fibrosis and osteosclerosis (cellularity 11-20%), and no Philadelphia chromosome or BCR/ABL gene rearrangement was found.
In view of these findings, photocoagulation of the peripheral avascular retina of the left eye and laser photocoagulation with pars plana vitrectomy of the right eye were performed. Intraoperative findings were mild vitreous hemorrhage with a diffuse traction membrane around the temporal avascular retina. The vitreous hemorrhage and traction membrane were removed and laser photocoagulation was performed. Four weeks after surgery, his visual acuities were 20/25 in the right eye and 20/20 in the left, fundus examination of the right eye revealed no vitreous hemorrhage, and the retinal neovascularization had regressed in both eyes with good laser scars ().


A 23-year-old man with a healthy appearance (170 cm, 57 kg) was referred to our clinic for persistent bacteriuria unresolved with prolonged use of antibiotics. He was symptom-free and unremarkable on the physical examination at his 1st visit to our clinic. He had a history of intermittent pain in the right lower abdomen with recurrent UTI for 15 years and had been treated with antibiotics repeatedly. He had a history of hospital admission 10 years ago but he could not hear any underlying cause identified. He had not suffered any other medical or surgical disease. Laboratory tests were normal except for pyuria and growth of Escherichia coli in his urinary samples. Cystoscopy was performed and a small slit-like opening was found on the right lateral wall of the bladder dome without a definite fistulous tract (). Voiding cystography revealed a diverticulum on the right lateral wall of the bladder with a suspicious linear fistula tract heading in the 10 o'clock direction (). We found some air within the bladder and a suspicious communicating tract between the appendix and bladder on the CT scan.
With an impression of appendicovesical fistula, laparoscopic surgery was performed through three abdominal ports. A supraumbilical port (10 mm trocar), a suprapubic port (5 mm trocar), and a right upper quadrant port (12 mm trocar) were placed. The distal tip of the appendix was firmly adhered to the right lateral side of the bladder with thick fibrosis. The body and base of the appendix looked healthy without surrounding fibrosis (). The base of the appendix was ligated with 2-0 Vicryl and the bladder was resected with an Endo-GIA stapler (Ethicon Endo-Surgery, Cincinnati, OH, USA) under simultaneous intravesical monitoring of the bladder with a flexible cystoscope. A urethral catheter was inserted for urinary drainage and was removed 7 days after the surgery. The postoperative course was uneventful. The surgical specimen showed a 7.5×1.0 cm sized appendix attached to a 3.0×2.5 cm sized bladder wall, and a small caliber fistulous tract with some fecalith was identified between them.


A 23-yr-old nulliparous, primigravid woman was referred to our maternal-fetal medicine unit at 18 weeks' gestation. She had been previously seen at our institution in the pediatric clinic as a child of 6 yr, when she presented with two umbilici and an epigastric pulsatile mass present since birth. At that time, she complained of intermittent abdominal pain in the area between the two umbilici. On physical examination, the superior umbilicus was found to be a dimple in the epigastrium connected to the actual umbilicus inferiorly by a fibrous band. A 1×3 cm pulsatile mass was palpable above the dimple.
Chest plain radiograph showed situs solitus and slightly decreased pulmonary vascularity and abdominal plain radiograph was unremarkable. On EKG, left ventricular hypertrophy was suspected. Cardiac catheterization, angiography, and echocardiography were performed and the pulsatile mass was found to be a substernal apical diverticulum of the heart beneath the weakened abdominal wall. Diagnosis of Cantrell's Pentalogy was made. Periodic follow-up studies over the next 6 yr, including chest radiography, EKG, and echocardiography showed no remarkable changes.
At age 23 yr, she presented to our institution at 18 weeks' gestation and was examined by echocardiography. The echocardiogram revealed left ventricular ejection fraction of 63%, suspected mild right ventricular outflow tract compression without a pressure gradient, and insignificant mild narrowing of left pulmonary artery origin. During prenatal evaluation, the patient did not complain of any significant symptoms except for a pulsatile sensation in the epigastrium and mild intermittent dyspnea. There were no abnormal obstetric findings, including antenatal routine laboratory results, midtrimester maternal serum screening test, and vaginal examination. On fetal ultrasonogram, fetal growth appeared normal, and fetal anomaly was not found. An epigastric dimple and the umbilicus were connected by a fibrous band, and the substernal cardiac diverticular beating beneath the abdominal wall was similar as previously described ().
At 39 weeks and 6 days' gestation, she was admitted for oligohydramnios. Induction of labor with vaginal dinoprostone insertion and intravenous oxytocin was performed. During the intrapartum period, she was monitored closely for any abnormal symptoms or signs. She delivered a 3.5 kg male baby without complication. The 1-min and 5-min Apgar Score was 9 and 9 and there was no gross anomaly noted including the umbilical cord and umbilicus. Postpartum uterine contraction was adequate, and she was discharged with the healthy baby without any significant hemorrhage or abdominal pain.
One year after the delivery, she underwent abdomino-pelvic CT. Appreciable on the CT were the apical diverticulum and pericardial defect (), lower sternum and diaphragm and pericardium defect (), and abdominal wall defect (). She had experienced no significant problems since delivery for one year.


A 77-year-old man, who was diagnosed with RCC with peritoneal seeding 2 months previously (), was admitted for severe dyspnea. He had been treated with sunitinib malate (Sutent, Pfizer, Korea) for only 3 days because of severe general weakness and poor oral intake. At the time of admission, his vital signs were unstable; his blood pressure was 78/46 mmHg, heart rate 136 beats per minute, and body temperature 36.7℃. Laboratory tests showed a normal leukocyte count (white blood cell, 7,450/µL), and pO2 of 72 mmHg and pCO2 of 33 mmHg on arterial blood gas analysis.
A 12-lead electrocardiogram revealed sinus tachycardia, and chest radiography findings included right hilar enlargement and reticular opacities in both lungs, in addition to cardiomegaly (). Chest computed tomography (CT) revealed a large left ventricular mass involving the lateral wall; however, evidence of right ventricular or inferior venal caval involvement was not found (). Multiple pulmonary nodules () and right hilar lymphadenopathy () were seen, suggesting pulmonary metastases.
Two-dimensional transthoracic echocardiography showed a very large, lobulated, oscillating mass attached to the lateral wall and occupying the cavity of the left ventricle (LV) (). This mass caused left ventricular outflow tract (LVOT) obstruction. Doppler imaging showed a turbulent color flow jet across the LVOT. The pressure gradient between the aorta and LV was 46 mmHg (). There was no evidence of metastasis to the inferior vena cava or RA (). The patient refused surgical treatment. On the following day, his blood pressure dropped to 56/35 mmHg, and he died from cardiogenic shock.


The first patient, a 52-year-old male, had an ill-understood pain in the left groin for 2 years, worsening at night. He had a psychiatric past with recurrent depression. The anesthesiologist and the neurologist could not find any abnormalities and referred him to the department of orthopedic surgery. There were no abnormalities in the left hip upon physical examination. Technetium bone scan did not show hot spots. MRI showed a small cyst-like lesion, which had a benign appearance (Fig. ). Given the fact that a malignancy was very unlikely, the decision was made to perform a biopsy followed by RFA. Ablation was reached using a Soloisttm single needle electrode (Boston Scientific, Natick, MA, USA) under computed tomography (CT) guidance (Fig. ). The needle was inserted through a 4.5 mm access hole, and a field of 15–20 mm in diameter was the anticipated size of the heated site. Ablation was reached in three cycles of 5–7 min, starting with 2 W and increasing by 1 W/min. Biopsies taken for histopathological evaluation revealed that the lesion was a chondrosarcoma (CS) grade I, which was a surprising finding. The patient recovered from the procedure and was pain-free after 3 months. Initial weight-bearing was 10 kg for 6 weeks, subsequently followed by 6 weeks of 50% body weight, for a total 3-month period of partial weight-bearing. Although clinical follow-up and MRI seemed unremarkable, the patient developed a pathological fracture while climbing up and down a ladder cleaning windows 3 months after the RFA procedure (Fig. ). The fracture occurred at the exact location of the ablated lesion and could be seen retrospectively on earlier MR images (Fig. ). The patient underwent surgery for total hip arthroplasty, with good clinical results over 3 years of follow-up. Follow-up has shown neither local tumor recurrence nor distant metastases in the lungs. Histopathology of tissue that had been retrieved during surgery showed complete necrosis of tumor tissue at the site of the thermal ablation, indicating a successful intervention (Fig. ).


The second patient is a 48-year-old female with a follicular carcinoma of the thyroid with metastases in her left and right femur. A pathological fracture had developed in her left hip, which was initially treated with a gamma nail and subsequently replaced by a Global Modular Resection System (GMRS) tumor prosthesis (Stryker, Limerick, Ireland). Follow-up with MRI showed a lesion at the calcar region of her right femur (Fig. ). For this lesion, RFA with a LeVeen® umbrella needle (Boston Scientific, Natick, MA, USA) under CT guidance was applied. Dimensions of the access hole and the expected ablated area were 4.5 mm and 20–30 mm in diameter, respectively (Fig. ). In a single cycle of 21 min, a maximum of 20 W was delivered starting with 2 W and adding 1 W/min. Three months after treatment, the patient was pain-free with 50% weight-bearing, and MRI scans showed no uptake of contrast fluid (Fig. ). However, a few days later the patient was admitted to our hospital with a pathological fracture of her right hip at the site of the previous ablation (Fig. ). Retrospectively, no lesions could be seen on the MR images that had been made a few days earlier. Following diagnosis, the patient underwent total hip surgery with good functional results until she died of her primary disease, 2.5 years later. Histopathological analysis of the retrieved femoral neck showed that apart from necrosis, there was still viable tumor tissue in selected slides of the tumor.


On 21 May 2009, a 42-yr-old male patient visited our hospital because of malaise and jaundice. He had been working as an office manager of a trading company in China for two years. Two mild fever, and one month before examination, he felt unusual fatigue and jaundice. Twenty days before examination, he had been informed that his acute hepatitis was unusual because he had no hepatitis A, B, or C viral markers. He took herbal medication and received acupuncture in China under the diagnosis of acute hepatitis of unknown origin. His symptoms improved under herbal medication, but remained. He returned to Korea for treatment of the acute hepatitis. He had no other symptoms except for scanty whitish phlegm. He had been healthy until the recent illness. He was not currently on medication, was not an injecting drug abuser. He did not smoke nor drink alcohol. He had received no blood transfusion. He had been married for seven years, but had lived alone in China for two years. He was well oriented and his vital signs were normal. Physical examination revealed icteric sclera and tender hepatomegaly. No mucous membrane lesions nor lymphadenopathy were present and he was afebrile. Cutaneous manifestations consisted of macular and papular lesions, about 0.5 cm in diameter, localized on the trunk, palms, and soles (). Genital examination revealed no lesions nor ulcers. Lymph nodes were not palpable in the neck, axillary, or inguinal areas. Laboratory tests showed the following: alkaline phosphatase (ALP) 2,974 IU/L, gamma glutamyl transpeptidase (GGT) 1,755 IU/L, aspartate transaminase (AST) 89 IU/L, alanine transaminase (ALT) 119 IU/L, total bilirubin 8.3 mg/dL, direct bilirubin 6.9 mg/dL, creatinine 0.8 mg/dL, albumin 2.4 g/dL, total protein 9.2 g/dL, white blood cell (WBC) 13,600/µL, hemoglobin 9.7 g/dL, platelets 752,000/µL, international normalized ratio (INR) 1.06, venereal disease research laboratory (VDRL) was positive with a titer >1:1,024, and his fluorescent treponemal antibody absorption (FTA-ABS) was reactive. A hepatitis panel including IgM anti-HAV, HBsAg, and anti-HCV, antinuclear antibody, ceruloplasmin, copper, alpha-1 antitrypsin deficiency, and human immunodeficiency virus (HIV) were all nonreactive. Computerized tomography didn't show any evidence of bile duct obstruction. It showed also mildly enlarged liver, nonspecifically enlarged lymph nodes at the porta hepatis area, and thickened gall bladder wall (). Chest radiography was normal.
Liver biopsy revealed portal-to-portal or portal to central zone bridge necrosis, and widening by lymphocyte infiltration, accompanied by intracanalicular and intracellular cholestasis. These findings were compatible with acute hepatitis (). A modified Warthin-Starry stain was negative for spirochetes. Skin biopsy revealed a perivascular mixed-cell infiltrate of prominent plasma cells, lymphocytes, and histiocytes around a blood vessel that contained swollen endothelial cells ().
The patient was treated with weekly intramuscular benzathine penicillin, 2,400,000 U, for a total of three doses. One week after treatment, his fatigue and rash were markedly improved. Three weeks after treatment, ALP was 288 IU/L, total bilirubin was 0.3 mg/dL () and four weeks after treatment, the VDRL titer was down to 1:64. He did not develop a Jarisch-Herxheimer reaction. Positive serologic tests and a prompt response to penicillin treatment confirmed the diagnosis of syphilitic cholestatic hepatitis.


This is the case of a 78-year-old woman who presented with signs of myasthenia gravis (MG).
This patient had noticed that there was a change in her ability to swallow and she had lost a significant amount of weight. Further inquiry highlighted that these symptoms were worse at the end of the day.
On examination, she had bilateral ptosis with some fatigability and dysarthria. There was no discernable dysphagia (assessed by speech and language therapy services) or dysphonia, and her cough effort was normal. Her oxygen saturation was 94% on room air and there was some associated weakness in eye closure. There was no diplopia and the rest of her neurological and systemic examination was unremarkable. An ice pack test was performed to aid the diagnosis of MG which was strongly positive.
Her biochemical and haematological profiles were normal although acetylcholine receptor antibodies were significantly raised confirming the diagnosis of MG. At this point MuSK antibodies and the Tensilon test were deemed unnecessary. The presence of a thymoma was ruled out with a CT thorax and she was started on regular pyridostigmine and neostigmine (low dose) as required along with prednisolone and admitted to hospital for monitoring with collaboration of a neurologist with good results.


A 25-year-old Caucasian woman presented to the emergency department via urgent GP referral with a week-long history of deterioration in mental state typical of acute psychosis. The patient appeared extremely anxious and disorientated with paranoid delusions and bizarre ideation. She mentioned hearing voices and being unable to sleep or eat properly. She described hallucinations, thought withdrawal and inability to carry out simple tasks of daily living. She appeared to have impaired memory and concentration and lack of complete insight regarding her current state although she was asking for help continually.
Prior to deterioration she had been fit and well with no history of drug use. She lived with her parents and had a twin brother who was well. There was no known family history of medical or psychiatric illness and she was in a stable relationship with her boyfriend.
The initial impression was of an acute psychosis and she was discharged home on olanzapine (5 mg twice daily) with plans for close community mental health follow-up. However, within a few days she was admitted acutely to the psychiatric ward as her condition worsened and she developed Parkinsonian-like signs with stooped posture, shuffling gait and masked facies. Her thought processing had worsened with slowness and increased paranoia. Physical examination was normal including blood pressure (115/70 mmHg) and fundoscopy. Initial biochemical and microbiology screening was also normal apart from a raised creatinine kinase level (1295 IU/L). There was no history of seizures. Ferritin was normal and atypical infection screening including toxoplasma, treponema pallidum, lyme and HIV was negative. Autoantibody screen, including ANA, ANCA, anti-cardiolipin, serum ACE, immunoglobulins, dsDNA and complement levels were negative. Thrombophilia screen (including lupus anticoagulant) was normal. A porphyria screen was also negative. She was reviewed by a neurologist who was concerned that she had an underlying organic cause for the psychosis and may have developed extrapyrimidal side-effects from anti-psychotic therapy. An MRI brain and lumbar puncture were thus organized. The MRI revealed high intensity signals in the subcortical white region and left parietal region (Figure ). CSF examination was normal. An incidental screening chest radiograph revealed small pneumothoraces and a subsequent high resolution computed tomography scan of the thorax confirmed these and revealed small scattered pulmonary nodules (Figure ). Further testing showed the ck-mb level was 23.7 (ref range 0–3.5). An echocardiogram was performed which showed right ventricular hypokinesia.
The combination of psychosis with an MRI appearance compatible with SLE and evidence of lung and myocardial involvement led to a clinical suspicion of of seronegative lupus. Despite detailed screening no metabolic or infective causes were evident. Thus, after extensive discussion with neuroradiologists, neurologists and lupus experts the decision was made to treat our patient with pulsed cyclophosphamide (500 mg) and methylprednisolone (250 mg). She received a total of nine pulses as per the Lupus Institute recommended regime for neurolupus (first three at weekly intervals then three at fortnightly intervals and three at 3-weekly intervals). She tolerated the infusions well. Clinically, progress was initially slow but she did appear to improve in terms of comprehension and concentration. She was moved on to mycophenolate mofetil (1 g twice daily) as maintenance treatment which she also tolerated well. After six months inpatient stay on the psychiatric ward she was discharged home with day-care arrangements and reduced anti-psychotic requirements (olanzapine 2.5 mg nocte). Three days post discharge she had a witnessed tonic-clonic seizure lasting approximately one minute (no incontinence). She was thus commenced on an anti-epileptic (leviteracetam 500 mg twice daily) on advice from neurologists. MRI appearances remained unchanged and, importantly, there were no new lesions. Clinically, our patient thereafter made remarkable clinical progress and at a recent outpatient review, nine months since the initial psychotic episode, was reported to be almost completely back to her usual self.


A 47-year-old woman working in a car manufacturing plant was pinned between a conveyer belt and the car body during assembly. Her left shoulder and chest were compressed. When released more than 20 minutes later, she was unconscious. Upon arriving at the emergency department, she was still unconscious, with facial cyanosis, severe edema, and petechiae. Radiographs and computed tomography (CT) scans of her chest, abdomen, and head revealed bilateral pneumothoraces, hemothoraces, multiple rib fractures, and a left scapular fracture. Her blood pressure was unstable. There were no other signs or history of head injury. A closed thoracotomy was immediately performed in the emergency room, and she was transferred to the intensive care unit for further management. In a bedside ophthalmologic examination, severe bilateral subconjunctival hemorrhages, chemosis, severe periorbital swelling, and mild exophthalmos were found (). Both pupils reacted sluggishly to light, and there was a relative afferent pupillary defect in the left eye. Intraocular pressure measured by a Tono-Pen was 17 mmHg in the right eye and 16 mmHg in the left eye. There were no signs of orbital compartment syndrome. Funduscopic examination showed bilateral mild optic disc edema. Admission axial CT scans of the orbit demonstrated bilateral retrobulbar hemorrhages, mild proptosis, and severe eyelid swelling (). Follow-up CT scans obtained 3 days later showed reduced exophthalmos and retrobulbar hemorrhages. On the third day after the accident, the patient recovered consciousness and her corrected visual acuity was finger counting at 50 cm in the right eye, and hand motion in the left eye. Visual evoked potentials revealed bilateral delayed latency, decreased amplitude in the right eye, and a flat wave in the left eye. High-dose steroid therapy was begun with the intravenous injection of 1.0 g methylprednisone daily for five days. Seven days after course of steroid treatment, corrected visual acuity improved to 0.1 in the right eye, but there was a marked visual field defect, and the corrected visual acuity of her left eye remained hand motion without recovery ().
Three months later, the patient experienced no interval change in vision, but the visual field defect was worse, and fundus examination revealed bilateral optic nerve atrophy, especially in the left eye. Optical coherence tomography demonstrated that the retinal nerve fiber layer (RNFL) thickness had significantly decreased in the right eye, and there was a near total loss of RNFL in the left eye.


The patient was a 67-year-old male who had inserted a foreign body into his rectum 7 hours before the hospitalization at the Emergency Room (ER). The patient had a second-degree hemorrhoid and was undergoing conservative treatment. He heard that a hemorrhoid could be improved if he used a massager to massage around the anal area. The patient massaged the perianal area with a massager to which differently shaped tips could be attached. The tip was unintentionally separated from the massager body inside the patient's rectum. The patient tried to remove the tip by himself, but came to the ER after feeling the foreign body move inside.
His past medical history showed a right hemicolectomy 2.5 years earlier because of ascending colon cancer. According to the pathologic results at that time, adjuvant chemotherapy was not performed because of the tumor was T2N0M0, stage Ib. He was going through a periodic follow-up and did not have any other significant medical history.
The patient did not have abdominal pain, but felt discomfort and minor pain around the perianal area. The patient's vital signs were stable, with a pulse rate of 70 per minute, a respiratory rate of 12 per minute, a blood pressure of 130/80 mmHg, and a temperature of 36.5℃. According to the physical examination, the abdomen was soft and flat, the bowel sound was normal, and no abdominal pain was being experienced. Upon digital rectal examination, the distal edge of the foreign body, which was located approximately 8 cm from the anal verge, was palpable. The foreign body was hard and had a smooth surface. There were no blood, no tar-colored rectum discharge, and no foul odor.
On a simple abdominal X-ray image, the foreign body was observed to be a radio-opaque object located in the pelvic cavity (). According to the laboratory test, the white blood cell count was 10,440/µL (neutrophils, 84%; normal range, 8,770/µL), which showed an increase in white blood cell count and neutrophil count. There were no other abnormalities on the blood test.
Since there was a possibility of anal sphincter damage if the foreign body was removed at the ER, removal after anesthesia was decided upon. After spinal anesthesia, a digital rectal examination was done in the lithotomy position. Unlike the first digital rectal examination in the ER, the object moving a bit distally was located 5-6 cm from the anal verge. With careful palpation so as not to move the object inside, a hole could be felt in the center of the foreign body, and a Kelly forcep was used to hold the object and to remove it from the rectum (). The surgery was finished after using anoscopy to check for mucosal damage on the distal rectum and the anal canal.
The removed foreign body was a gray-colored hard-rubber-based object which was 7 cm long and 3 m in diameter (). After making sure that there were no anal bleeding and no abnormality on the physical examination of the abdomen for 24 hours, the patient was discharged. Currently, he is undergoing periodic follow up for colon cancer.


A 42-year-old woman (weight: 48.8 kg, height: 159 cm), who underwent an extensive tumor resection for Klatskin tumor, Bismuth type IV, developed acute hepatic failure in the immediate postoperative period and was listed for an emergency LT.
A preoperative laboratory and radiology evaluation before the tumor resection were within the normal limits except for an elevated liver function profile (aspartate aminotransferase 170 U/L, alanine aminotransferase 100 U/L) due to the underlying Klatskin tumor. The bleeding diatheses at the intensive care unit (ICU) were as follows: hemoglobin and platelet count decreased from 10.4 g/dl and 103 × 103/µl to 4.8 g/dl and 60 × 103/µl, respectively, within the first hour at the ICU. Prothrombin time (PT) expressed in the international normal range (INR), activated partial thrombin time (aPTT), fibrinogen, fibrin degradation product, D-dimer, antithrombin III activity, plasminogen activity, protein C activity were 2.03 (normal [nl], 0.9-1.1), 104.5 seconds (nl, 29-42 seconds), 97 mg/dl (nl, 180-380 mg/dl), >5 µg/ml (nl, <5 µg/ml), 3.23 µg/ml (nl, 0-0.5 µg/ml), 41% (nl, 83-123%), 30% (nl, 75-112%), 43% (nl, 80-161%), respectively. Metabolic acidosis of pH <7.15 with a progressively deteriorating base deficit >15 mmol/L and exacerbating hypocalcemia <0.8 mol/L and hyperglycemia >200 mg/dl were observed throughout the ICU stay. Continuous infusions of calcium gluconate at 1 mg/kg/hr and 5% albumin were given. Bicarbonate and insulin were given intermittently at the discretion of the clinician. At the ICU, exsanguinating bleeding of more than 1,000 ml/hr was seen through a drainage immediately after the tumor resection. During the 11 hour stay at the ICU before receiving a total hepatectomy, more than 25 units of packed red blood cells, 16 units of each fresh frozen plasma, cryoprecipitate and platelet concentrates were administered along with a continuous infusion of 10 µg/kg/min dopamine, 0.35 µg/kg/min norepinephrine to maintain the systolic blood pressure between 50 and 80 mmHg, and the diastolic blood pressure between 30 and 65 mmHg. The urine output was maintained between 20 and 100 ml/hr during the ICU stay. The patient was indicated to undergo a total hepatectomy with a temporary portocaval shunt while awaiting a liver graft due to the uncontrollable bleeding and hepatic toxic syndrome, which was postulated to be caused by acute hepatic necrosis and the resultant coagulation abnormality. During the two hour and 40 minute surgical procedure, crystalloid 8,100 ml, packed red blood cells eight units, fresh frozen plasma four units, platelet concentrates eight units and cryoprecipitate six units were administered to replace the estimated blood loss of 4,500 ml. The total urine output was 330 ml.
After the total hepatectomy and portocaval shunt, the patient was placed at the ICU and medical care for the anhepatic state was provided. During the anhepatic period, an hourly monitoring of the arterial blood gas analysis with an intermittent the ionic calcium level and dieresis were the primary concern of the ICU management. Arterial blood gas analyses showed an alleviation of the metabolic acidosis from arterial pH <7.15 and base deficit >15 mmol/L during the acute liver failure status to pH 7.15-7.25 and base deficit of 10-15 mmol/L during the anhepatic period. Metabolic acidosis was accompanied by slight hyperchloremia ranging 110-115 mmol/L (nl, <108 mmol/L). However, the potassium level was within the normal range of 3.5-3.8 mmol/L throughout the ICU stay before the hepatectomy. The magnesium level decreased only slightly ranging 1.6-1.7 mg/dl and the sodium level was maintained between 144 and 146 mmol/L. Persistent ionic hypocalcemia <0.8 mmol/L was observed despite the continuous infusion of calcium gluconate supplementation at 1 mg/kg/hr. Other continuous supplementations including sodium bicarbonate, magnesium, 5% albumin, 20% mannitol and dextrose solution were provided as needed. There was no rapid correction of hyponatremia or an increase in the sodium level owing to the use of sodium bicarbonate in this case. There were no neurological deficits following the abnormal changes in sodium level or signs of hepatic encephalopathy, even though comprehensive neurological examinations were not feasible due to the patient's sedated and intubated condition. The serum glucose level was maintained between 100 and 200 mg/dl. A warm blanket was applied to maintain the normal body temperature. An inotropic support with a continuous infusion of 5 µg/kg/min dopamine maintained the systolic blood pressure between 80 and 100 mmHg and diastolic blood pressure between 60 and 80 mmHg. At the discretion of the ICU specialist, 12 units of packed red blood cells, 10 units of fresh frozen plasma, 16 units of platelet concentrates and 14 units of cryoprecipitate were given over an 11-hour period at the ICU before the liver transplant. An elevated creatinine level of 1.58 mg/dl and urine out of <10 ml/hr were observed, but continuous renal replacement therapy (CRRT) was not applied. The coagulation profile during the anhepatic period showed a prolongation of PT (INR) around 2, aPTT around 100 s and fibrinogen <100 mg/dl. Cytokine or other toxin related laboratory analyses were not performed in this patient. No artificial liver support, such as molecular adsorbent recirculating system (MARS), was applied in this patient because she was not considered a suitable candidate.
The patient was anhepatic for 15 hours before reperfusion of the liver in the living donor liver transplantation. Anesthesia was induced and maintained with desflurane at an oxygen fraction of 0.5 under routine monitoring with electrocardiography, SpO2, radial and femoral arterial pressure, pulmonary arterial pressure and cardiac output. Continuous vasoactive support with 5 µg/kg/min dopamine and 0.1-0.2 µg/kg/min norepinephrine were maintained. The hemodynamic profile was stable throughout the operation before and after reperfusion of the transplanted liver. During the seven hour and 40 minute LT operation, 7,500 ml crystalloid, 500 ml colloid, 650 ml albumin, eight units of packed red blood cell, four units of fresh frozen plasma, nine units of cryoprecipiate and one unit of platelet apheresis were administered.
Postoperative care at the ICU involved cardiovascular and respiratory support with a CCRT. On the ninth day after LT, the patient became alert and on the 16th day she was extubated for one week until reintubated due to pulmonary effusion related respiratory failure. The acute renal insufficiency with urine output of <5 ml/hr and creatinine level between 1.5 and 2.0 mg/dl persisted despite the best efforts to improve the renal function. The patient also suffered peritonitis from bowel anastomosis leakage and underwent two additional operations for anastomosis site repair. The sustained septic conditions throughout the ICU stay aggravated and progressed to multiorgan failure. The patient died on postoperative day 65.


A man in his early 20s presented with aggression, hearing voices and poor sleep for 1 week. He had two previous involuntary psychiatric admissions and was diagnosed with schizophrenia. Prior to his first admission, social interaction and work performance deteriorated. He presented with positive symptoms, including auditory hallucinations.
He had no comorbidities or head injuries. He previously abused methaqualone and was still using cannabis regularly. Developmental and family histories were unremarkable. He functioned well until his first psychotic episode.
He had previously taken on olanzapine 15 mg daily with flupenthixol depot 20 mg per month but suffered extrapyramidal side effects on risperidone. He became non-adherent when visiting family in a rural district.
He was initially treated in a district hospital for a schizophrenia relapse with mood symptoms secondary to his cannabis use (urine positive for tetrahydrocannabinol). Several weeks after his admission, however, he displayed episodic mutism, posturing and urinary incontinence. He reported déjà vu and gustatory sensations preceding the symptoms but could not recall the episodes.
He was transferred to our unit for further work-up. His mental state at admission involved mood lability, rapid speech, tangential thoughts and grandiose delusions. Physical and neurological examinations, as well as special investigations, were normal. He had at least one further episode of déjà vu with altered behaviour in our unit.
CT of the brain showed a Galassi type 1 arachnoid cyst (42 mm×38 mm×39 mm) in the left middle cranial fossa with a mass effect on the adjacent tissue. Electroencephalography (EEG) was normal. Neurosurgery suggested conservative management based on the Galassi classification of the lesion.
The patient was managed for the Diagnostic and Statistical Manual of Mental Disorders, 5th Edition (DSM 5) schizoaffective disorder, bipolar type, which proved to be resistant to treatment. He required sedation with clonazepam 6 mg per day, which could only be weaned after 7 weeks on our ward. We increased olanzapine to 25 mg daily and eventually added flupenthixol as long-acting injectable antipsychotic at 20 mg monthly to augment this. Sodium valproate was added and increased to 1800 mg daily and he required the addition of lithium 750 mg daily (serum lithium 0.6 mmol/L) before his symptoms abated. By the time of discharge, he was euthymic, psychosis resolved and he was not experiencing the episodes anymore.


A man in his early 20s was admitted after a few days of irritability, grandiosity, insomnia and increased energy.
He first presented 2 years prior with periodical staring that started after the deaths of several family members. He reported derealisation. He made two suicide attempts but claimed no recollection of the entire episode, which resolved before he accessed medical care.
Work-up revealed a left anteromedial temporal arachnoid cyst measuring 46 mm×19 mm×28 mm with compressive effects on the left frontal and anteromedial left temporal pole on MRI. Two extended video EEG recordings of 74 hours and 19 hours showed no epileptiform activity. His neurologists felt temporal lobe epilepsy (TLE) was excluded. Two neurosurgeons recommended conservative management because of the absence of TLE.
He subsequently had four psychiatric admissions. Prior to each admission, there were 3 days of a stereotypical progression from blank staring, derealisation, talkativeness with increasing incoherence to mood lability and finally frank mania with misidentification delusions.
During previous admissions, he received trials of sodium valproate, lamotrigine, phenytoin and risperidone. His course was unpredictable. Episodes were eventually resolved, only to recur despite good adherence.
He had a minor head injury during adolescence for which no medical care was sought. He used nicotine only.
He was born preterm as a result of a complicated twin pregnancy (the other twin stillborn). There was no family history of psychiatric illness.
During his latest admission, risperidone was increased to 4 mg and sodium valproate was changed to lithium 500 mg owing to adverse effects. He responded rapidly and at his transfer to the care of a private psychiatrist in another city closer to his father’s residence, he was euthymic, psychosis resolved and he displayed good insight. Lithium levels were not yet taken at the time of his transfer. Based on his atypical picture, we diagnosed him with DSM 5 bipolar and related disorder due to arachnoid cyst.


A 46-year-old Indian female with a six-month history of a rapidly growing left breast mass presented with altered mental status for three days. She was observed to be less responsive and incoherent at times. There were no preceding autonomic symptoms or fever. She was on atenolol for underlying hypertension. She had no previous history of diabetes mellitus. There was no known use of any anti-diabetic medication, insulin or traditional medicine. There was no family history of malignancy.
Upon presentation, she was confused, afebrile and normotensive. She had a Glasgow Coma Scale (GCS) of E4V3M5 and a capillary glucose of 1.9 mmol/L. Her GCS improved after intravenous infusion of 50 mL 50% dextrose, with a capillary glucose of 5.2 mmol/L. Physical examination revealed a large, firm and mobile left breast mass measuring 20 cm x 20 cm, with three ulcerated lesions and peau d’orange skin changes (). There was no lymphadenopathy or hepatosplenomegaly.
Initial laboratory data showed severe hypoglycemia (random blood glucose 1.7 mmol/L) and hypokalemia (serum potassium 2.8 mmol/L). Otherwise, her full blood count, venous blood gas, and function tests of the kidney, liver and thyroid were normal. Random serum cortisol taken before initiation of hydrocortisone was inappropriately low (166 nmol/L) when her random blood glucose was 1.7 mmol/L. During the same hypoglycemic episode, the serum levels of insulin (<0.5 mU/mL) and C-peptide (35 pmol/L) were suppressed, suggesting that the hypoglycemia was not linked to either endogenous or exogenous hyper-insulinism. Plasma insulin-like growth factor I (IGF-I) (67.4 ng/mL, reference range 53 to 192 ng/mL) and IGF-II (481 ng/mL, reference range 333 to 967 ng/mL) were within normal, but the elevated IGF-II/IGF-I ratio (7:1, normal <3:1) supported the diagnosis of NICTH. Computed tomography (CT) of the brain, chest, abdomen and pelvis showed a large, heterogenous left breast mass with no obvious infiltration of the chest wall and no evidence of lymphadenopathy or metastasis. Bone scan was not done as the patient did not have bone pain and alkaline phosphatase level was normal.
During hospitalization, she experienced recurrent daily hypoglycemia, ranging from 1.7 to 3.2 mmol/L. This resulted in recurring confusion and seizures despite multiple 50% dextrose injections and continuous 20% dextrose infusion at 62.5 mL/hour. She also required large amount of potassium supplementation of up to 150 mmol/day to maintain a normal serum level. Intravenous hydrocortisone at 100 mg three times daily was started on day 3 of admission. This was subsequently converted to oral prednisolone 40 mg daily on day 5. After steroid initiation, she was able to achieve euglycemia, ranging from 4.5 to 7.1 mmol/L within two days, with concurrent continuous 5% dextrose infusion at 62.5 mL/hour. There were also resolution of confusion, seizure and hypokalemia.
Core needle biopsy of the breast lesion showed spindle cell neoplasm. A simple mastectomy was then performed to remove the tumor. On cut section, the 6.3 kg mastectomy specimen showed a well-circumscribed tumor measuring 30 cm x 24 cm x 14 cm with a 2 cm clear resection margin. The tumor contained abundant stromal and epithelial components. The hypercellular stroma was composed of spindle-shaped cells with mild nuclear atypia. Mild epithelial hyperplasia and a few cystically dilated ducts were also seen. There was no evidence of malignancy. Hormone receptor tests were positive for both estrogen and progesterone. All these histological features were consistent with a benign phyllodes tumor ().
Infusion with 5% dextrose was continued in the immediate postoperative period until the patient was able to tolerate oral intake. There were no more hypoglycemic episodes subsequently. Prednisolone was converted to hydrocortisone replacement dose in view of possible adrenal insufficiency. After one week, a short Synacthen® test showed adequate cortisol response (baseline, 197 nmol/L; after 30 minutes, 544 nmol/L; after 60 minutes, 587 nmol/L) prompting discontinuation of hydrocortisone. There has been no recurrence of hypoglycemia, confusion or seizure during the subsequent four-month follow-up.


A 30-year-old woman presented to our hospital with a 10-year history of abdominal distension, constipation and intermittent abdominal pain. She used laxatives and enemas. Her past medical history was significant for pancreatic surgery. Five years earlier, she underwent segmental pancreatic resection because of solid pseudopapillary neoplasm. Her family history was negative for gastrointestinal, neurologic or other familial diseases.
The patient was hospitalized several times due to partial bowel obstruction, which was managed conservatively. Abdominal radiographs and computed tomography (CT) scans revealed diffused dilated loops of small and large bowels (). Colon transit time was measured using radiopaque marker test. All markers remained in the bowels 5 days after the ingestion of them. Colonic hypomotility was diagnosed. As the patient was incapacitated by symptoms and did not respond to conservative treatment, subtotal colectomy was performed. Histopathological examination revealed abnormal layering of colonic muscularis propria. Both inner and outer muscular layers were very thin and inadequate (). Nevertheless, the symptoms of pseudo-obstruction persisted and the patient underwent another surgery. Sigmoid resection with preventive ileostomy was done. Histopathological examination showed the same abnormal changes of the colonic muscular layer. Few months later, preventive ileostomy was closed and ileorectostomy was performed. However, adhesive post-operative small bowel obstruction complicated patient’s recovery. Relaparotomy and adhesiolysis were done. After the last surgery, the patient still complained of abdominal distension and constipation. Colonoscopy disclosed anastomotic stricture, ~5 mm in diameter (). Endoscopic dilation was done, but the patient complained of the same symptoms few weeks after the procedure. Endoscopy was performed again and it showed an anastomotic stricture. Endoscopic dilation was done. This procedure was repeated with stent placement for a few more times due to recurrence of anastomotic stricture. Moreover, the patient still complains of constipation. Constipations are associated with abdominal distension and pain and just laxatives and enemas bring some relief for the patient. The patient continues follow-up and endoscopic treatment (repetitive dilatations) at a medical centre in the USA.


In July 2010, a 52-year-old woman was admitted to the Dermatology Department complaining of palpable purpura in both lower legs and arthralgia in her knees. Leukocytoclastic vasculitis was diagnosed based on skin biopsy. In a routine laboratory test HCV antibodies were detected. Then she was referred to the Infectious Diseases Department for further evaluation of hepatitis C infection. Her medical history was not significant besides blood transfusion at age 24 due to anemia after delivery. Serum levels of aminotransferases were slightly elevated, HCV RNA was positive, genotype (GT) 3. Liver biopsy revealed chronic hepatitis with moderate fibrosis (stage 3 in Ishak score) and steatohepatitis (grade 2 on Brunt scale). Anti-HBc-total and antinuclear antibodies were negative. Mixed cryoglobulinemia type II, with the presence of circulating cryoglobulins consisting of polyclonal IgG and monoclonal IgM (with activity of rheumatoid factor), was diagnosed. Antiviral treatment of PEG-IFNα-2b and RBV was initiated in November 2010. In the course of therapy we observed reduction and then resolution of purpura and arthralgia. Baseline HCV RNA was 1.2 × 106 IU/ml and became negative at week 12 as well as the cryoglobulin test. At week 20 treatment was discontinued because of a serious adverse event – bilateral interferon-associated retinopathy with visual impairment; at the time of discontinuation HCV RNA was negative. Then she was treated by an ophthalmologist with a high dose of steroids (four weeks) and pentoxifylline. After 8 weeks of antiviral treatment discontinuation, relapse of HCV RNA and recurrence of symptomatic cryoglobulinemia were observed. She was monitored by an ophthalmologist and a hepatologist for the next few years. In that period gradual improvement, but not full recovery, of the visual acuity was observed. Purpura of the lower legs was recurrent with higher intensity in summer months. In March 2014 she developed glomerulonephritis with peripheral edema and arterial hypertension. Urinalysis showed moderate proteinuria (up to 1.6 g/24 h) and hematuria with sediment red blood cells more than 100 per high power field (HPF). Glomerular filtration rate (GFR) was 49 ml/min/1.73 m2 and kidney dysfunction stage 3 was diagnosed. Renal biopsy was not performed, but since she was HCV positive and had cryoglobulinemia, the diagnosis of HCV-related cryoglobulinemic glomerulopathy was determined by a nephrologist. In the next months arterial pressure normalized on antihypertensive drugs and proteinuria spontaneously decreased to a value below 0.5 g/24 hours. GFR remained in the range 40-59 ml/min/1.73 m2. The patient was waiting for DAA drugs with activity against genotype 3 HCV. Finally, in December 2015 she started sofosbuvir (SOF) 400 mg and RBV 800 mg daily. At baseline she had clinical symptoms of mixed cryoglobulinemia in the form of lower leg purpura, and presence of cryoglobulins. Liver stiffness measured by shear-wave elastography was 7.6 Kpa, and laboratory parameters were as follows: HCV RNA 1.33 × 107 IU/ml, total bilirubin 0.8 mg/dl, albumin 4.2 g/dl, normal aminotransferases: aspartate (AST) 39 IU/l and alanine (ALT) 34 IU/l, creatinine 1.32 mg/dl, GFR 44 ml/min/1.73 m2, glucose 98 mg/dl, hemoglobin 12.1 g/dl, white blood cells 3550/μl, platelets 185 × 105/μl, erythrocyturia 10-20 cells/HPF with proteinuria 1.48 g/24 hours. She took amlodipine, torasemide and spironolactone; no drug interactions with sofosbuvir were expected so no dose modifications were made. By week 4 HCV RNA was negative, purpura of the lower legs disappeared but the patient complained of weakness and the hemoglobin level decreased to 8.3 g/dl. Therefore, the ribavirin dose was reduced to 400 mg until week 12. At that point of therapy the hemoglobin level increased to 11.5 g/dl and the RBV dose returned to baseline. She completed 24 weeks of treatment with no other significant adverse events, kidney function was stable and cryoglobulins were negative at the end of treatment. Then she was followed up every 3 months. The patient achieved a sustained virological response – HCV RNA was negative at weeks 12 and 24 after the end of SOF+RBV therapy. At the most recent follow-up visit, 18 months after antiviral treatment completion, she remains clinically well, with no recurrence of mixed cryoglobulinemia symptoms. She is still aviremic, cryoglobulins in her blood are negative, kidney function is normal and no urinary abnormality is detected.


A 67-year-old Hispanic female with a history of type 2 diabetes mellitus presented with a 6-month history of recurrent visual loss in the left eye (OS). Two years prior to presentation, she had undergone scleral buckle, pars plana vitrectomy, internal drainage of subretinal fluid, fluid air exchange, endolaser, and C3F8 gas in the OS. The patient was lost to follow-up until she returned with progressive visual loss, OS.
Her best-corrected visual acuity was 20/80 OS and the clinical examination demonstrated the presence of recurrent inferior retinal detachment extending posterior to the scleral buckle. A striking subretinal fibrotic “clothesline” membrane extended horizontally from 4 o'clock to 8 o'clock (Fig. ). Because of the visible retinal elevation by the subretinal band, surgical removal of the band was planned. In this patient, there were no visible epiretinal membranes or signs of retinal inflammation.
During a 23-gauge pars plana vitrectomy, a retinotomy was created with diathermy near the central portion of the subretinal fibrosis. ILM forceps were used to enter the subretinal space and to remove the subretinal band, which was submitted for histopathological examination. A fluid-air exchange was performed and internal drainage of subretinal fluid was accomplished, followed by endolaser around the retinotomy site as well as posterior to the scleral buckle inferiorly. Silicone oil was infused into the air-filled eye and a single suture was placed in the sclerotomy used for oil infusion. At the 4-month postoperative follow-up, the patient had retinal reattachment, but visual acuity remained 20/200 in the oil-filled OS.
Cross-sectional examination of the tissue revealed a cable-like configuration composed predominantly of glial differentiation, RPE differentiation and collagen, based on morphology and immunohistochemical staining with glial fibrillary acidic protein (GFAP), cytokeratin and S100, and Gomori trichrome immunohistochemical staining, respectively. The composition of the band consisted predominantly of cells positive for both GFAP and glutamine synthetase, suggestive of Müller cell differentiation. Gomori trichrome staining revealed collagen fibers arranged in a longitudinal fashion lengthwise within the band. There was pigment embedded within the tissue and foci of cells staining positive for S100 and keratin peripherally around the tissue, suggestive of RPE differentiation (Fig. ). Scattered foci of smooth muscle actin-positive cells suggested mild myoblastic differentiation.


A 65-year-old male with bipolar I disorder was admitted to the inpatient psychiatry unit for an acute manic episode. His outpatient medication regimen, past medical history, and admission labs are described in .
On admission, outpatient psychiatric medications were discontinued and VPA solution (500 mg every morning, 1000 mg every evening) and risperidone tablets (1 mg twice daily) were initiated. Valproate and risperidone were titrated to doses of 3000 mg/d and 6 mg/d, respectively, over the course of 5 days. On day 7, the patient became somnolent, which progressed to garbled speech and altered mental status. Laboratory testing revealed a VPA level of 68 μg/mL (lab reference range: 50 to 100 μg/mL) and an ammonia level of 56 μmol/L (lab reference range: 16 to 53 μmol/L). Valproate was discontinued with resolution of symptoms. Following VPA discontinuation, staff noted reemergence of mania symptoms, including agitation and hypersexual behaviors. On day 11 of admission, lithium 900 mg/d was initiated and VPA solution was restarted at a lower dose of 1000 mg/d, which was eventually increased to 1500 mg/d divided twice daily. At the time of VPA dose increase, levocarnitine 990 mg daily was initiated to prevent VHE and was increased 3 days later to 990 mg twice daily. Blood levels were rechecked 2 weeks later, and ammonia was found to be 23 μmol/L and the VPA level was 50 μg/mL. No symptoms of encephalopathy were reported during the admission after levocarnitine was started.
Valproate was continued as an outpatient but for reasons unknown, levocarnitine was not renewed. Approximately 3 months after inpatient discharge, the patient was admitted to the hospital for encephalopathy with an elevated ammonia level of 66 μmol/L and a low VPA level of 19 μg/mL as it was held for somnolence. Valproate was discontinued and not restarted.


A 64-year-old male with schizoaffective disorder, bipolar type, was admitted to the inpatient psychiatric unit for disorganized behavior. Outpatient medications, past medical history, and admission labs are provided in . Valproate level on admission was 82 μg/mL.
Over an 8-week hospital course, the medication regimen was gradually changed to VPA solution 1250 mg every morning and 1500 mg at bedtime, haloperidol 5 mg twice daily, clonazepam 1 mg at bedtime. Valproate level was found to be 141 μg/mL (7.5-hour post-dose level at steady state; true trough calculated to be ∼88 μg/mL) and ammonia was elevated at 63 μmol/L, increased from 47 and 49 μmol/L earlier in the admission. No changes in mental status were noted with elevated ammonia. Levocarnitine 990 mg was given once. Ten hours later, ammonia decreased to 44 μmol/L and 13-hour post-dose VPA level was 64 μg/mL. The patient was discharged on levocarnitine 990 mg daily for 2 days then 990 mg twice a day to prevent hyperammonemia with chronic VPA use. Valproate solution was changed to VPA derivative, divalproex ER 2500 mg/d.
It is unclear if the patient was adherent to levocarnitine as an outpatient. Ammonia was found to be elevated at 67 and 71 μmol/L on 2 separate occasions. The patient was readmitted to the hospital a few months later and found to have a VPA level of 70 μg/mL on divalproex ER 2500 mg/d and an ammonia level of 75 μmol/L. After 9 days of witnessed adherence to levocarnitine 330 mg 3 times daily, ammonia level decreased to 29 μmol/L. Valproate level was 25 μg/mL (23-hour post-dose level at steady state). The patient was discharged on levocarnitine 990 mg twice daily to be continued with divalproex.


A 53-year-old acutely manic male with bipolar I disorder and substance use disorder was transferred from an outside hospital. Outpatient medications, past medical history, and labs after transfer are reported in .
The patient was continued on the medications from the outside hospital. During his admission at our facility, the patient was briefly transferred to the medicine service due to hypernatremia, which the medicine team attributed to lithium and stopped the medication. At time of transfer back to the psychiatric unit, the patient was initiated on VPA solution 1000 mg twice daily. Over the next 4 days, ammonia levels were found to be elevated at 59, 65, and 82 μmol/L. Once at steady state, VPA level was 104 μg/mL. Manic symptoms persisted but no changes in mental status were noted. Despite lack of symptoms, lactulose was started for hyperammonemia and given for 3 days. Two days after lactulose was initiated, levocarnitine was also started at 1000 mg daily for 3 days then increased to twice daily. Ammonia levels decreased but remained elevated during the 2 weeks following levocarnitine initiation at 70, 58, 67, and 68 μmol/L. Levocarnitine was then increased to 1000 mg every morning and 1500 mg every evening during the second week to target hyperammonemia. After 3 weeks, ammonia levels were checked every 8 to 10 days and found to be within normal range or slightly elevated at 46, 46, 59, and 49 μmol/L. The patient was discharged on VPA and levocarnitine. Similar to our second case, it was unclear if the patient was adherent to levocarnitine as an outpatient. He was readmitted to our psychiatry unit again and found to have a VPA level of 115 μg/mL and an ammonia level of 74 μmol/L, which decreased to 30 μmol/L after levocarnitine supplementation.


Mrs B is a 65-year-old, married, white woman with a long-standing history of bipolar disorder type I, who was transferred to our inpatient psychiatric hospital from a general medical hospital in May 2012. She had been treated in the emergency department for mania with psychosis as well as hypertension, hyponatremia, urinary retention with urinary tract infection (UTI), and resulting delirium. Psychosomatic medicine was consulted, and once medically stabilized, she was transferred to an inpatient psychiatric hospital.
Mrs B reported a history of mood symptoms beginning at age 12. Since that time, she noted discrete periods of mania with 4 to 5 days of elevated mood, decreased sleep, racing thoughts, pressured speech, impulsivity, spending sprees, and reckless behavior. She also had previous major depressive episodes but endorsed more periods of mania than depressive episodes. She had multiple psychiatric hospitalizations, typically during times of mania. Her first hospitalization was at age 12, and she required multiple hospitalizations until she began treatment with lithium in her late teenage years.
Her medical history is significant for type 2 diabetes mellitus, hypertension, dyslipidemia, cataracts, history of secondary hyperparathyroidism, and history of anemia. She had a remote history of alcohol abuse but no current substance use. Family psychiatric history was notable for alcohol use disorder and mood disorders in her brother and both parents.
Mrs B's renal transplant was performed in August 2010 after several decades of lithium treatment. Her renal failure was suspected to be due to lithium nephrotoxicity in the setting of long-standing hypertension and diabetes. At the time of transplant, lithium was discontinued, and she was started on quetiapine 150 mg daily.
Between September 2010 and June 2012, she was hospitalized more than 40 times for medical and psychiatric reasons, including medication nonadherence, hypertensive crisis, and multiple episodes of mania with psychosis. The patient was admitted to multiple hospitals during this time, so not all records were available for review. Data from hospitalizations within our system are included in this report. In between hospitalizations, she required nursing home care as her husband was unable to safely care for her at home. A brief account of medication trials, including relative dates are detailed through May 2012 ().
Her final hospitalization was in May 2012. She was hospitalized from her nursing home for a UTI, elevated blood pressure, urinary retention, mania with psychosis, and delirium. She was treated with fluphenazine decanoate but developed QTc prolongation while medically hospitalized. She was then transitioned back to aripiprazole 15 mg twice daily. Her delirium improved after treatment of her UTI. However, she continued to demonstrate significant symptoms of agitation, violence, flight of ideas, word salad, and clang speech. She then required psychiatric hospitalization. Divalproex sodium delayed release (DR) 750 mg once daily was added without resolution of her mania. She continued to experience functional impairments not consistent with her previous baseline and was not considered safe for discharge by her husband or treatment team.
The decision to restart lithium was made in June 2012 by the primary inpatient team after discussion with the patient, husband, primary care physician, renal transplant surgeon, and nephrologist. This decision was based on the patient's previous history of long-standing mood stability with lithium and failed trials of multiple other medications as well as the patient and husband's desire to subvert nursing home placement. Lithium carbonate immediate release (IR) was started at 300 mg daily at bedtime. Within 24 hours, the patient was noted to have improvement in her symptoms. Her dose of lithium carbonate IR was later increased to 600 mg daily, and within 2 days, she had returned to her previous baseline. Prior to discharge, her serum lithium level was 1.0 mEq/L, and her serum creatinine value was 1.0 mg/dL. The discharge plan included follow-up with her primary care provider, geriatric psychiatry clinic, nephrology, and home health care. Complete blood cell count, basic metabolic panel, lithium, and valproic acid level were planned to be drawn 1 week after discharge.
In August 2012, Mrs B presented to the geriatric psychiatry clinic for hospital follow-up and intake assessment. Mrs B and her husband expressed the dramatic positive changes noticed since lithium carbonate IR was restarted, which she described as “a night and day of difference.” She felt she had regained her independence in carrying out daily activities with improved clarity of thought and alertness. She progressively required less assistance, which enabled her to live at home with her husband. At that time, she had no significant mood symptoms or psychosis. Her husband felt she was 50% back to her baseline mental status, and the patient felt she was closer to 80%.
Lithium level at that time was 1.2 mEq/L. There was some concern that this lithium level was exacerbating the cognitive impairment, which had previously developed with her ESRD. The lithium carbonate IR dose was reduced to 450 mg daily at that appointment. She was continued on divalproex sodium DR 750 mg twice daily and aripiprazole 30 mg once daily.
She was seen frequently over the next several months with ongoing continued stability of mood and denial of any symptoms consistent with psychosis or depression. However, she continued to have complaints of cognitive impairment, and lithium carbonate IR was further decreased to 300 mg daily.
Mrs B was able to reach remission of her symptoms on this medication regimen with minimal cognitive symptoms after the final lithium carbonate IR dose decrease. Her renal function continued to be carefully monitored but was stable over time on this medication regimen.


A 16-year-old female patient reported to the Department of Oral and Maxillofacial Surgery, Dr. D. Y. Patil Vidyapeeth's Dr. D. Y. Patil Dental College and Hospital, with a chief complaint of pain, swelling in the right side of face for 5 days and inability to open the mouth for 2 days []. The patient was apparently alright 5 days back when she felt pain in the right side of lower jaw. Pain gradually increased in severity and swelling was noticed. Initially, swelling was small, which gradually increased and attained present size (dumbbell shaped 6 cm × 3 cm). Mouth opening was restricted.
On clinical examination, on the right side, swelling in the cheek and temporal region was palpated. Anteroposteriorly, extending from the commissure of lip till the angle of the mandible and Superoinferiorly, extending from the temporal fossa region till the lower border of mandible. Swelling had typical dumbbell-shaped appearance due to interposition of zygomatic arch. Swelling was hard, tender, and nonfluctuant. Mouth opening was restricted to 8 mm.
On the basis of clinical findings, provisional diagnosis of right buccal, submassetric, and superficial temporal space infection was made.
Radiological investigations revealed a radiolucent lesion with radiopaque mass in the right posterior region of the mandible []. First mandibular molar of the right side was impacted with resorption of roots. This radiographic picture was mimicking various pathological lesions. Based on clinical and radiologic examination, a differential diagnosis of dentigerous cyst, calcifying epithelial odontogenic tumor, keratinizing cystic odontogenic tumor, unicystic ameloblastoma, complex odontome, and impacted mandibular first molar of the right side was considered.
In the first phase, incision and drainage of abscess after empirical antibiotic therapy was done under conscious sedation [Figures and ].
In the second phase, calcified lesion along with soft tissue lining and impacted molar was removed under general anesthesia [Figures –]. Histopathological report showed the evidence of complex odontome associated with dentigerous cyst posterior to the odontome [].
Final diagnosis was made as buccal, superficial temporal, and submassetric space infection due to complex odontome with impacted mandibular first molar of the right side with resorption of roots.


A 63-year-old man with diabetic nephropathy and end-stage renal disease undergoing hemodialysis was transferred from a local hospital to our hospital in November 2016 because of arteriovenous fistula dysfunction. Four months previously, at the local hospital, non-tunneled right IJV hemodialysis tube catheterization failed for an unknown reason, and the patient underwent non-tunneled femoral vein hemodialysis catheterization and right arm arteriovenous fistula surgery. After only four dialysis sessions, the femoral vein hemodialysis tube developed poor flow and was drawn away. He received a non-tunneled right IJV dialysis tube, and the catheterization was successful. However, the blood flow was poor, and the patient began using the arteriovenous fistula 3 months prior to presentation at our hospital. One month before presentation, the blood flow became inadequate and decreased to 130 to 150 ml/min, and cephalic vein stenosis was detected by color Doppler ultrasound.
The cephalic vein stenosis was severe, and the length of the lesion was long. Percutaneous transluminal angioplasty would have been difficult. Considering the patient’s severe cardiac insufficiency (ejection fraction of 36.2% by Simpson’s rule), placement of a tunnel hemodialysis catheter was planned. After exclusion of stenosis and clots in the right IJV, the vein was successfully punctured and venous blood aspirated under ultrasound guidance. However, a J-shaped guidewire could not be introduced via the guiding needle because something seemed to be blocking it until the performer changed the direction of the puncture needle to the lateral cervix. Bedside consultation by an anesthesia specialist was required. Regardless of what the anesthesia specialist did, the guidewire always entered the SV. The patient refused to undergo placement of a femoral vein hemodialysis tube. Non-tunneled IJV cannulation was performed via the left side. We ordered right arm computed tomography (CT) venography to determine the reason for the unsuccessful catheterization.
CT venography showed that the inferior segment of the right IJV was draining into the ipsilateral SV behind the cranial part of the first rib and SV junction (). The angle of the right IJV and the right SV was 35 degrees. The right brachiocephalic vein drained the right SV only.
This case report was approved by the ethics committee of the Second Affiliated Hospital, School of Medicine, Zhejiang University. The patient provided written informed consent to report his medical history and medical images, and the consent form has been archived by the authors.


A 5-month-old previously healthy African-American female was admitted with 3 days of diarrhea and fever. She had a temperature of 103°F and a pulse of 185 beats per minute. She appeared acutely ill; however, skin rash, oral lesions, conjunctival injection, and cervical lymphadenopathy were absent. Her abdomen was soft and non-distended with increased bowel sounds. Inflammatory markers were high (). Urinalysis was positive for leukocytes and nitrites, but culture was negative. Extreme irritability led to lumbar puncture that yielded normal results. Toxigenic C. difficile was detected in the stool by polymerase chain reaction (PCR) for which oral metronidazole was prescribed. Despite therapy, she continued to have fever (100°F–104°F) and developed dryness and reddening of the lips and buccal mucosa, as well as edema of her face, hands, and feet. A diffuse erythematous maculopapular eruption in her groin area and conjunctival injections also appeared. Progressive abdominal distension was noted, and abdominal ultrasound showed nonspecific ascites. Echocardiogram showed a 2–3 mm pericardial fluid collection without evidence of coronary aneurysms. The diagnosis of KD was made on hospital day (HD) 7, and high-dose intravenous immunoglobulin (IVIG; 2 g/kg) was given along with high-dose aspirin. She dramatically improved with resolution of fever and diarrhea. She was sent home on low-dose aspirin 2 days later.