Berardinelli-seip congenital generalised lipodystrophy

Berardinelli-Seip congenital lipodystrophy [BSCL] syndrome is a rare genetic disorder caused by dysregulation of glycemic and lipid metabolism. We report five BSCL cases with typical clinical pictures and complications. These, to the best of our knowledge, represent the first case series from Pakistan. BSCL is characterized by marked atrophy of adipose tissue, acromegaly, acanthosis nigricans and tall stature. We could not perform genetics studies in any patient owing to non-availability of genetic laboratory in Pakistan. All the cases presented hypertriglyceridemia. One case developed hyperinsulinism controlled with metformin. There is no curative treatment and the current approach is low-fat diet and management of insulin resistance and diabetes. Recently published studies showed that leptin-replacement therapy is promising in the metabolic correction of complications of BSCL. This highlights the importance of further research in BSCL treatment

Etiology and pattern of presentation of gastritis in children, our experience at a tertiary care hospital

Objective: This study aimed to look in to the frequency of different causes and pattern of presentation of gastritis in children less than 16 years of age. Study Design: Prospective cross sectional study. Place and Duration of Study: This study was performed in the Children Hospital and the Institute of Child health, Lahore, from Jan 2016 to Jan 2017

Material and Methods: This study was performed in the department of Pediatric Gastroenterology, Hepatology and Nutrition at Children Hospital and the Institute of Child health, Lahore, from Jan 2016 to Jan 2017. One hundred and sixty children fulfilling the inclusion criteria were enrolled in this study. All patients went through endoscopy. Macroscopic findings were noted and gastritis was confirmed by histological analysis of gastric mucosa. Biopsies were taken from gastric antrum, the body and pylorus

Results: The mean age of children was 8.73 +/- 3.70 years [range 2-16years], and majority were in the age range of 6-10 years. Males were affected slightly more than females with a ratio of 1.3:1. Drug induced gastritis was the major contributor in 58 [36.2%] patients followed by food related gastritis in 55 [34.3%]. H.Pylori positive gastritis was seen in 38 [23.7%] patients. No cause of gastritis was found in 5.6% of patients

Conclusion: Our study concluded that drug induced gastritis and food related gastritis was more common than H. Pylori positive gastritis in children in our setting

Antiacanthamoebic properties of natural and marketed honey in Pakistan

Objective: To determine antiacanthamoebic activity of natural and marketed honey samples. Methods: Natural honey samples were collected directly from the bee hive and marketed honey samples were purchased from the local market in Karachi, Pakistan. Both honey samples were tested for their flavonoid content (quercetin equivalent per gram of the extract) and phenolic content (gallic acid equivalent per gram). Furthermore, their anti-oxidant activity was determined by measuring 2,2-diphenyl-1-picrylhydrazyl. Using amoebistatic and amoebicidal assays, the effects of honey samples were tested against growth and viability of Acanthamoeba parasites. Results: Natural honey exhibited potent amoebistatic and amoebicidal effects, in a concentration-dependent manner. Honey-treated Acanthamoeba castellanii showed loss of acanthopodia, following which amoebae detached, rounded up, reduced in size, decreased in cytoplasmic mass and they were observed floating in the culture medium. Importantly, honey-treated amoebae did not revive when inoculated in fresh growth medium, however, glycerol-treated amoebae exhibited viable trophozoite and active growth. In contrast, marketed honey samples varied in their efficacy against Acantha-moeba castellanii. The proportion of flavonoid, as determined by quercetin measurements and the proportion of phenolic, as determined by gallic acid measurements was higher in natural honey compared with marketed honey. Similarly, the antioxidant activity, as determined by 2,2-diphenyl-1-picrylhydrazyl scavenging activity was higher in natural honey vs. marketed honey. Conclusions: This study shows that natural honey has antiacanthamoebic properties and possesses higher flavonoid, phenolic and antioxidant properties compared with the marketed honey. These findings are of concern to the public, health officials, and to the manufacturers regarding production of honey for medical applications.