ROLE OF MALE GENDER IN LAPAROSCOPIC CHOLECYSTECTOMY OUTCOME

Laparoscopic cholecystectomy (LC) is one of the most commonly performed laparoscopic procedures. Problems occurring during laparoscopic cholecystectomy include bile duct injury, conversion to open operation, and other postoperative complications. Male gender is a risk factor for LC conversion rate.Our goal is to determine the effect of male gender on the outcome of laparoscopic cholecystectomy for Chronic Cholecystitis. We have done that through a retrospective clinical trial was carried out at our Hospital to evaluate the sex difference as predictor for difficult laparoscopic cholecystectomy. From a total number of 638 patients, who underwent laparoscopic cholecystectomy for Chronic Cholecystitis from 1st January 2012 to 1st of January 2015) two hundred and seventeen patients were excluded according to exclusion criteria and the remaining 421 patients were included.All the operation were done according to standard four-port technique through an open method was used, with first entry port in the periumblical region. 1 Anesthetic technique and perioperative management were the same for all patients during the study period. Results: Patients who were candidates for elective cholecystectomy , were mostly females with (F:M ratio= 4/1), mean age 40 years (range 13-101 years) with mean age of 45 years(range 20-78 years) for the males, 40years (range 13-100 years) for the females. There were more difficult cholecystectomies in males in comparison to female patients for chronic cholecystitis. The out come of this study is male gender is a predictor for difficult laparoscopy for symptomatic gallstones presented as chronic cholecystitis

Optic Nerve Involvement in Farber Lipogranulomatosis: Expanding the Phenotypic Spectrum

Farber lipogranulomatosis (Farber disease [FD], MIM 228000) is a rare autosomal recessive lysosomal storage disorder caused by a deficiency of acid ceramidase (AC) and is linked to ASAH1 gene mutations. ASAH1 mutations can lead to severe reduction in AC activity that causes accumulation of ceramides in various body tissues including the retina. The onset of clinical manifestations usually occurs during infancy, and the severity depends on residual ceramidase activity. Classic clinical features of FD include painful joint swelling, progressive dysphonia, and subcutaneous nodules. We report 2 unrelated patients presenting with classic FD phenotype who shared the same homozygous pathogenic mutation in the ASAH1 gene and optic neuropathy, thereby expanding the FD phenotypic spectrum