Arteriovenous malformations of the lateral ventricle

Nine cases with arteriovenous malformations (AVM's) predominantly involving the lateral ventricle are presented. All the AVM's were small, but caused intraventricular hemorrhage in eight cases. Only two patients had an intracerebral hemorrhage large enough to warrant evacuation. Eight patients were under the age of 40 years at the onset of their disease. Computerized tomography demonstrated intraventricular hemorrhage in eight patients, and after intravenous administration of contrast medium a small area of enhancement with dilated subependymal draining veins was seen in seven. The lateral ventricles were of normal size in seven cases, and only two patients required a shunting procedure. Angiography demonstrated that the lesion was an AVM in eight patients, and did not visualize the lesion in the ninth. One patient suffered a recurrent intraventricular hemorrhage when the AVM was demonstrated, although repeated angiography had failed to disclose a vascular lesion at his first intraventricular hemorrhage 14 months before. All nine lesions were resected by microsurgical techniques, and the results were excellent in eight patients. Of four caudate lesions, three were resected through a frontal transcortical approach and the other was operated on through an anterior transcallosal approach; the results were excellent in three of these patients. Only one (Case 4) was left with neurological deficits; he had confusion and disorientation following a right frontal transcortical approach. Even in the dominant hemisphere, lesions in the head of the caudate nucleus could be safely resected by an anterior transcallosal approach. Two choroidal lesions located in the temporal horn and trigone on the dominant side were resected through a middle temporal gyrus approach, and three thalamic lesions through a posterior transcallosal approach, all with excellent results. In all cases the brain opening required was about the width of the retractor (maximum 2.0 cm, average 1.5 cm).     

Microsurgical penile revascularization in patients with pure arteriogenic erectile dysfunction

BACKGROUNDS: We report the results of microscopic penile revascularization in patients with arteriogenic erectile dysfunction. METHODS: One patient with localized obstruction of the common penile artery underwent the Michal II penile revascularization, 13 patients underwent the Furlow-Fisher procedure, and 5 patients underwent the Hauri procedure. The mean age was 33.0 years and the mean follow-up period was 32 months (4-80 months). Eight patients were tobacco smokers, one patient was over 50 years old. Surgery was considered successful when the patients had a permeable anastomosis and were able to achieve satisfactory erection resulting in normal sexual intercourse. RESULTS: All surgery was successful except for one patient who had undergone the Furlow-Fisher procedure. In Spite of antithrombotic therapy, graft occlusion occurred in two patients. Post operative glans hypervascularity occurred in two patients. CONCLUSION: Penile revascularization surgery is a highly effective treatment for selected patients. There is a need for further study of graft occlusion and glans hypervascularity.     

De novo autoimmune hepatitis following living-donor liver transplantation for primary biliary cirrhosis

Abstract:  Since first being described in 1998, de novo autoimmune hepatitis (AIH) after liver transplantation has been reported in several cases suffering from non-autoimmune liver diseases and primary biliary cirrhosis (PBC). Glutathione S-transferase (GST) T1 genotype mismatches between donor and recipient have also been suggested to constitute a risk factor for de novo AIH. Here, we report a 33-yr-old woman who presented complaining of marked fatigue and jaundice four yr after living-donor liver transplantation for PBC. On examination, transaminase levels were highly elevated and ANA and antimitochondrial antibody M2 were positive. Histological findings showed zonal necrosis with lymphoplasmacytic infiltration closely resembling AIH. She had pretreatment AIH score of 16 and 19 points after relapse of de novo AIH. Two color fluorescence in situ hybridization with X and Y chromosome-specific probes clearly revealed that the hepatocytes were of donor origin and lymphocytes were of patient origin. The GSTT1 genotype of the patient and the donor were the same null type, suggesting that mechanisms other than GSTT1 mismatches may exist in de novo AIH development. In conclusion, recipient immune cells attacked the allogeneic transplanted liver of the patient via de novo AIH, although the exact participation of autoimmune mechanisms is unclear.     

Rotavirus gastroenteritis‐associated urinary ammonium acid urate crystals

Although ammonium acid urate (AAU) calculi are extremely rare renal stone components, it was recently found that many urinary tract calculi that cause post‐renal renal failure in rotavirus (RV) gastroenteritis are AAU calculi. The mechanism of AAU calculi development in RV gastroenteritis has not been fully elucidated. We analyzed data from eight RV gastroenteritis patients who transiently had AAU crystals in their urinary sediment. In these patients, formation of AAU crystals occurred earlier than the formation of AAU calculi. No difference was observed in serum and urine uric acid levels between RV gastroenteritis patients with or without AAU crystals. Interestingly, fractional excretion of sodium was extremely low among patients with AAU crystals. These results suggest that the formation of AAU crystals might not be due to excretion of uric acid, but excretion of sodium.     

CD147/basigin reflects renal dysfunction in patients with acute kidney injury

Background

Acute tubular necrosis (ATN) describes a form of intrinsic acute kidney injury (AKI) that results from persistent hypoperfusion and subsequent activation of the immune system. A glycosylated transmembrane protein, CD147/basigin, is involved in the pathogenesis of renal ischemia and fibrosis. The present study investigated whether CD147 can reflect pathological features and renal dysfunction in patients with AKI.

Methods

Plasma and spot urine samples were collected from 24 patients (12 controls and 12 with ATN) who underwent renal biopsy between 2008 and 2012. In another study, patients undergoing open surgery to treat abdominal aortic aneurysms (AAAs) were enrolled in 2004. We collected urine and plasma samples from seven patients with AKI and 33 patients without AKI, respectively. In these experiments, plasma and urinary CD147, and urinary l-fatty acid-binding protein (l-FABP) levels were measured, and the former expression in kidneys was examined by immunostaining.

Results

In biopsy tissues of ATN with severe histological features, CD147 induction was strikingly present in inflammatory cells such as macrophages and lymphocytes in the injured interstitium, but not in damaged tubules representing atrophy. Both plasma and urinary CD147 levels were strikingly increased in ATN patients; both values showed greater correlations with renal dysfunction compared to urinary l-FABP. In patients who had undergone open AAA surgery, urinary and plasma CD147 values in AKI patients were significantly higher than in non-AKI patients at post-operative day 1, similar to the profile of urinary l-FABP.

Conclusion

CD147 was prominent in its ability to detect AKI and may allow the start of preemptive medication.     

The Diagnostic Value of Endoscopic Ultrasonography and Magnetic Resonance Imaging for Anorectal Malignant Melanoma: Report of a Case

A 74-year-old Japanese woman presented with a 3-month history of anal bleeding. Proctoscopy revealed an unusual polypoid lesion with focal pigmentation at the dentate line, which was histologically diagnosed as a malignant melanoma. Whole-body clinical and radiographic evaluations revealed no alternative primary source. Endoscopic ultrasonography (EUS) showed well-delineated hypoechoic tumors invading the muscularis propria, and magnetic resonance imaging (MRI) revealed regional lymphadenopathy. Following this evaluation, an abdominoperineal resection with regional lymphadenectomy was performed. The excised tumor was histologically confirmed to be malignant melanoma, and its depth and metastatic lymph nodes proved to have been accurately and precisely evaluated by the preoperative examinations. Thus, EUS and MRI are useful preoperative diagnostic tools for the tumor staging of primary anorectal malignant melanomas, as for other rectal tumors. Received: February 1, 2002 / Accepted: September 3, 2002 Reprint requests to: H. Sashiyama     

A neonate with homozygous protein C deficiency with a homozygous Arg178Trp mutation

Homozygous protein C deficiency affects approximately 1/400,000 to 1/1,000,000 live births. Homozygous protein C deficiency is associated with catastrophic and fatal purpura fulminans-like or thrombotic complications and disseminated intravascular coagulation. In the present patient, genetic study revealed Arg178Trp, a mutation found widely in European population; but this is the first case of homozygous Arg178Trp mutation who suffered from catastrophic purpura fulminans phenotype.