Unusual Presentation of Wünderlich Syndrome.

Background: Wünderlich syndrome is a rare but important condition because it involves a sudden blood collection in the renal fossa that can cause hemodynamic instability. Case Report: A 38-year-old female with a history of type 2 diabetes mellitus and hypertension with poor adherence to treatment presented to the emergency department with abdominal pain of 2 weeks' duration accompanied by irritative lower urinary symptoms. Abdominal computed tomography (CT) scan showed bilateral pyelonephritis and an abscess in the lower pole of the right kidney. A second CT scan, performed because of the patient's abrupt decrease in hemoglobin and hematocrit, showed active bleeding secondary to the infectious process in the right kidney. The patient was hemodynamically unstable, so a nephrectomy was performed. Conclusion: Wünderlich syndrome is a spontaneous renal hemorrhage, in most cases attributed to a tumorous etiology and rarely of infectious origin. The clinical picture is varied but can present with the Lenk triad of acute onset flank pain, flank mass, and hypovolemic shock. It is diagnosed principally via an imaging study such as abdominal CT scan. Treatment is conservative in principle, but urgent surgical intervention is sometimes necessary depending on the clinical situation of the patient.

Cyfip1 Haploinsufficiency Increases Compulsive-Like Behavior and Modulates Palatable Food Intake in Mice: Dependence on Cyfip2 Genetic Background, Parent-of Origin, and Sex.

Binge eating (BE) is a heritable trait associated with eating disorders and involves episodes of rapid, large amounts of food consumption. We previously identified cytoplasmic FMR1-interacting protein 2 (Cyfip2) as a genetic factor underlying compulsive-like BE in mice. CYFIP2 is a homolog of CYFIP1 which is one of four paternally-deleted genes in patients with Type I Prader-Willi Syndrome (PWS), a neurodevelopmental disorder whereby 70% of cases involve paternal 15q11-q13 deletion. PWS symptoms include hyperphagia, obesity (if untreated), cognitive deficits, and obsessive-compulsive behaviors. We tested whether Cyfip1 haploinsufficiency (+/-) would enhance compulsive-like behavior and palatable food (PF) intake in a parental origin- and sex-dependent manner on two Cyfip2 genetic backgrounds, including the BE-prone C57BL/6N (Cyfip2N/N) background and the BE-resistant C57BL/6J (Cyfip2J/J) background. Cyfip1+/- mice showed increased compulsive-like behavior on both backgrounds and increased PF intake on the Cyfip2N/N background. In contrast, maternal Cyfip1 haploinsufficiency on the BE-resistant Cyfip2J/J background induced a robust escalation in PF intake in wild-type Cyfip1J/J males while having no effect in Cyfip1J/- males. Notably, induction of behavioral phenotypes in wild-type males following maternal Fmr1+/- has previously been reported. In the hypothalamus, there was a paternally-enhanced reduction in CYFIP1 protein whereas in the nucleus accumbens, there was a maternally-enhanced reduction in CYFIP1 protein. Nochange in FMR1 protein (FMRP) was observed in Cyfip1+/- mice, regardless of parental origin. To summarize, Cyfip1 haploinsufficiency increased compulsive-like behavior and induced genetic background-dependent, sex-dependent, and parent-of-origin-dependent effects on PF consumption and CYFIP1 expression that could have relevance for neurodevelopmental and neuropsychiatric disorders.