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Objective: Autoimmune psychosis (AP), defined as an isolated schizophreniform psychosis with no or only minimal neurological abnormalities stemming from autoantibody-associated cerebral processes, is a very rare and potentially underdiagnosed disorder in children and adolescents. Methods: This article reports the case of a 13-year-old boy who became acutely ill with a psychotic disorder following a respiratory infection. We describe the diagnosis and course of treatment in detail. Results: Following an extensive interdisciplinary workup, a probable glutamic acid decarboxylase (GAD)-associated AP was diagnosed that ultimately remitted completely with treatment. Conclusions: Autoimmune psychoses remain a relatively uncharted territory for the specialty of child and adolescent psychiatry. Good interdisciplinary cooperation is essential for patients with somewhat atypical psychotic symptoms, both in terms of diagnosis and treatment.

Berardinelli-Seip congenital lipodystrophy (BSCL) is a very rare autosomal recessive disease. In the described patient diagnostic relevant symptoms developed at 3 week age., (Georg Thieme Verlag KG Stuttgart, New York.)

Introduction: Pneumatosis coli is a rare disease with heterogeneous symptoms which can be detected in the course of various acute and chronic intestinal diseases in children, such as necrotizing enterocolitis, intestinal obstruction and intestinal bacteriological infections., Case Presentation: We report the case of a 12-month-old boy who died of pneumatosis coli caused by an acute intestinal gas gangrene after prolonged artificial alimentation., Conclusion: While intestinal gas gangrene is a highly uncommon cause of pneumatosis coli, it is important to consider it as a differential diagnosis, especially in patients receiving a prolonged artificial food supply. These patients may develop intestinal gas gangrene due to a dysfunctional intestinal barrier.

Patients after kidney, heart and lung transplantation differ in their immunosuppressive drug regimens and in susceptibility to infectious complications with cytomegalovirus (CMV). In this study, CMV-specific T-cell responses were characterized in long-term transplant recipients and associated with the frequency of infectious complications. CMV-reactive CD4 T cells from 50 healthy controls, 68 renal, 14 heart and 24 lung transplant recipients were flow cytometrically quantified by the induction of cytokines after specific stimulation. Moreover, the immunosuppressive effect of calcineurin inhibitors on specific T-cell reactivity was quantified in vitro and compared with responses in vivo. Median CMV-specific T-cell frequencies in long-term renal (1.48%; range 0.06-17.26%) and heart transplant recipients (0.90%; 0.13-12.49%) did not differ from controls (1.82%; 0.26-21.00%). In contrast, CMV-specific T-cell levels were significantly lower in lung transplant recipients (0.50%; <0.05-4.98%) and showed a significant correlation with the frequency of infectious episodes (r =-0.57, p = 0.005). The differences within the groups were associated with increasing dosages of immunosuppressive drugs, as exemplified for calcineurin inhibitors that dose dependently reduced specific T-cell reactivity in vitro. In conclusion, monitoring CMV-specific CD4 T cells may serve as a measure for long-term disease susceptibility and may contribute to an improved management of CMV complications after lung transplantation.

The sulfated bile alcohol scymnol sulfate (ScyS), 3 alpha,7 alpha,12 alpha,24 xi, 26,27-hexahydroxy-5 beta-cholestane-26(27)-sulfate, is the major bile salt in bile of an elasmobranch, the little skate. To investigate hepatic transport of bile alcohols in skate liver, [3H]ScyS and a potential precursor, 3 alpha,7 alpha,12 alpha-trihydroxy-5 beta-cholestane (chtriol), were used as model compounds. Their transport into isolated hepatocytes was partially saturable, temperature sensitive, and Na+ independent. The uptake of ScyS was inhibited by cholyltaurine, and uptake of cholyltaurine was inhibited by ScyS in a competitive manner. In contrast, uptake of chtriol was not inhibited by cholyltaurine, suggesting separate transport systems. ScyS and chtriol showed a choleretic effect in isolated perfused livers. When ScyS was added to the perfusate of isolated perfused livers, > 25% was found in bile within 7 h. When chtriol was added to the perfusate, 10% of the dose was secreted into the bile mainly in the form of polar metabolites, whereas only nonmetabolized chtriol remained in the livers. The slow bile flow of 40-50 microliters/h and the high recovery in the liver suggest that metabolism may be the rate-limiting step in the hepatic elimination of chtriol. The major metabolites secreted into bile were identified by mass spectrometry and chromatography as scymnol and ScyS. To study the enterohepatic circulation, [3H]ScyS or [3H]chtriol was administered into the duodenum of free-swimming skates, and bile was collected through exteriorized indwelling cannulas over a 4-day period. More than 90% of the radioactivity was recovered from bile, indicating that there was a highly effective absorption in the intestinal epithelium, as well as specific transport mechanisms for hepatic uptake and biliary secretion of these compounds. This is the first direct demonstration of an enterohepatic circulation for a bile alcohol sulfate in fish liver.