Localized penile bullous pemphigoid of childhood

Bullous pemphigoid is an acquired autoimmune bullous condition that is uncommon in childhood. Genital involvement is extremely rare. We present a case of a 7-year-old boy with bullous lesions confined to the glans penis. Precise diagnosis is based on clinical presentation and specific histopathologic testing. Oral corticosteroid therapy is the treatment of choice.     

Vancomycin resistant enterococcal sepsis in an acute myeloid leukemic patient

Neutropenic fever is an important cause of morbidity and mortality during therapy of acute myeloid leukemia. Patients with acute myeloid leukemia are at increased risk of acquiring vancomycin resistant enterococcal infection and its treatment remains problematic. Vancomycin therapy for more than 7 days is usually associated with inducible vancomycin-resistant enterococcal infections. We report a case of vancomycin resistant enterococcal sepsis in a patient of acute myeloid leukemia.     

Progressive familial intrahepatic cholestasis

Progressive familial intrahepatic cholestasis (PFIC) is an important cause of cholestatic liver disease and biliary cirrhosis in pediatric population. Three cases of PFIC are described that were diagnosed on the basis of family history, pruritus, cirrhosis and / or paucity of interlobular bile ducts on liver biopsy and presence of extrahepatic biliary tree on imaging. These patients were initially labeled as suffering from extra-hepatic biliary atresia and neonatal hepatitis. PFIC-1 and 2 could not be differentiated on histological grounds, since these patients presented late and process of fibrosis was advanced.     

Increased medial thalamic creatine-phosphocreatine found by proton magnetic resonance spectroscopy in children with obsessive-compulsive disorder versus major depression and healthy controls

Altered brain creatine-phosphocreatine levels might reflect changes in brain energy use and have been implicated in the pathogenesis of obsessive-compulsive disorder and major depressive disorder. We used proton magnetic resonance spectroscopy to measure absolute concentrations of creatine-phosphocreatine in the right and left medial thalami in 18 pediatric patients with major depressive disorder 9 to 17 years of age, 18 case-matched healthy controls, and 27 patients with obsessive-compulsive disorder 7 to 16 years old. The two patient groups were psychotropic drug naive and were not comorbid for the diagnosis of the comparison group. We found significantly increased left and right medial thalamic creatine-phosphocreatine concentrations in patients with obsessive-compulsive disorder compared with both healthy controls and patients with major depression. Creatine-phosphocreatine concentrations did not differ significantly between patients with major depression and healthy controls. Our data suggest that increased medial thalamic creatine-phosphocreatine concentrations in patients with untreated obsessive-compulsive disorder reflect altered energy use in the medial thalamus and might differentiate patients with obsessive-compulsive disorder from healthy controls and patients with major depression. Although these results must be considered preliminary, further study of the diagnostic specificity of creatine-phosphocreatine in obsessive-compulsive disorder is indicated.     

Arginine and urea metabolism in the liver graft: A study using microdialysis in human orthotopic liver transplantation

BACKGROUND: Arginine is an amino acid having a central role in the metabolism of urea and nitric oxide in the liver. We present our findings of the behavior of these metabolites during the process of transplantation of the liver. METHODS: Urea, arginine, ornithine, citrulline, gamma-aminobutyric acid, glutamate, and glutamine levels in 15 livers were studied during the process of retrieval, following storage during the backtable procedure, and for 48 hours postreperfusion using microdialysis. Arginase levels in donor and recipient serum were also analyzed using an enzyme-linked immunosorbent assay specific for type I human arginase. Data was analyzed using one-way analysis of variance, with post-hoc comparison to the value at two hours using Dunnett's test (P < 0.05 significant). RESULTS: Levels of metabolites measured in the donor liver were seen to decline significantly in the stored liver. Immediately postreperfusion, there was a significant rise in arginase I levels in the recipient serum with low arginine levels recorded in the liver. The high arginase I levels significantly reduced six hours postreperfusion with a corresponding rise in extracellular arginine levels. Urea levels in the graft increased significantly immediately postreperfusion. CONCLUSIONS: Arginine levels were found to be low with correspondingly high serum arginase I levels in the early postreperfusion phase. High serum arginase I levels in early postreperfusion may influence nitric oxide production in this phase since considering Vmax and Km values, arginase I could compete with inducible nitric oxide synthase for arginine. Urea metabolism in the liver recommences immediately postreperfusion.     

Is serum gentamicin level a good predictor of graft injury in intestinal transplantation?

INTRODUCTION: Following intestinal transplant (SBT), the early diagnosis and treatment of rejection is a major management aim. The diagnosis of rejection is based on histology of stomal biopsies. Oral gentamycin (2.5 mg/kg) was used for selective decontamination of the digestive system. Our hypothesis was that gentamycin might be absorbed in the presence of graft dysfunction. AIM: Our goal was to assess the correlation between serum gentamycin level and the health of the intestinal graft. SUBJECTS AND METHODS: Among 33 SBT performed from 1993 to 2005, serum gentamycin levels were performed once weekly or more often when there was a suspicion of rejection. All data were analyzed retrospectively. RESULTS: Adequate trough levels were achieved for only 23 patients, six of whom had histologically proven rejection and only one did not have a raised gentamycin content. Five patients with raised levels but no rejection included two with severe intestinal ischemia and three with bowel obstruction/ileus. Four of the five patients required laparotomies. CONCLUSION: We concluded that in our study raised serum gentamycin levels were a good predictor of rejection or significant injury to the graft.