Lipoid Proteinosis: Case Report

A 20-month-old Kuwaiti girl had manifestations of lipoid proteinosis, a rare autosomal recessive disorder seen more commonly in Caucasians. This condition is diagnosed based on clinical, histopathologic, and ultrastructural criteria. Its biochemical and genetic aspects are still poorly understood.     

Spontaneous bacterial peritonitis from Salmonella: an unusual bacterium with unusual presentation

Spontaneous bacterial peritonitis (SBP) is a common cause of morbidity and mortality in patients with advanced cirrhosis and portal hypertension. While gram-negative rods and Enterococcus species are the common offending organisms, Salmonella has also been recognized as a rare and atypical offending organism. Atypical features of Salmonella SBP include both its occurrence in cirrhotic patients with immunosuppressive state and its lack of typical neutroascitic response. Diagnosis is often delayed as it requires confirmation from ascitic fluid culture. We report a case of Salmonella SBP occurring in a patient with decompensated cryptogenic cirrhosis with concurrent low-grade non-Hodgkin lymphoma and prior treatment with rituximab. Physicians should be aware of the atypical presentation, especially in cirrhotic patients who are immunosuppressed.     

Diverticular per oral endoscopic myotomy (DPOEM) for esophageal diverticular disease: a systematic review and meta-analysis

The traditional way to tackle Zenker’s diverticulum (ZD) has been flexible endoscopic septum division (FESD). Recently, the concept of per oral endoscopic myotomy has been found useful for managing diverticular diseases of the esophagus and has been termed DPOEM. We performed a systematic review and meta-analysis to evaluate the efficacy and safety of D-POEM in diverticular disease of the esophagus and to compare it with FESD. We systematically searched PubMed and Embase, for studies reporting clinical success, technical success and adverse events in D-POEM alone or D-POEM comparing with FESD. We computed pooled prevalence for D-POEM alone and risk ratio for D-POEM vs FESD using random effect method with inverse variance approach. Subgroup analysis for ZD, non-ZD and mixed diverticulum was conducted. Totally 19 studies including 341 patients were identified reporting on D-POEM. The pooled clinical, technical success and adverse event rates for D-POEM were 87.07%, 95.19% and 10.22%, respectively. The clinical success was significantly better than FESD while the technical success, adverse event rate, procedure time and length of hospital stay were comparable with FESD. The recurrence rate was negligible for D-POEM compared to FESD. On subgroup analysis by dividing into three groups of ZD, non-ZD and mixed, there was no difference between clinical, technical success and adverse event rate among the three groups. D-POEM is an effective and safe technique among both ZD and non-ZD patients and has better clinical success than FESD.