Endogenous mycotic endophthalmitis in an immunocompetent patient

Acute and invasive fungal infections are usually seen in immunocompromised and debilitated patients. We report a young immunocompetent 28-year-old Indian male who presented with unilateral endogenous endophthalmitis in the left eye and was managed with pars plana vitreous surgery. The polymerase chain reaction from the vitrectomy specimen tested positive for the fungal genome while the cytology examination identified aseptate hyphae with wide-angle branching, most likely suggesting Zygomycosis. A detailed systemic evaluation failed to reveal any systemic focus or predisposing factor for fungal infection. The patient had received intravenous dextrose infusions while undergoing a surgical procedure for post-traumatic hydrocele elsewhere a week prior to this episode. The patient was successfully managed with pars plana vitreous surgery along with intravitreal Amphotericin-B and oral Voriconazole.     

Animal bites��should primary reconstruction be the standard treatment?

We present a series of 91 cases caused by both wild and domestic animals over a period of 5?years from 2005?C2009. The objective of the study was to compare the results of primary reconstruction with the traditional method of debridement and secondary healing. A total of 91 patients were studied from January 2005?CDecember 2009. In 54 patients, the injuries were carefully documented, and immediate reconstruction was performed. Thirty-seven patients with contraindications to surgery or who refused to undergo surgery were allowed to heal by secondary intention and were studied as a control group. The patients and their relatives were asked to rate their result and outcome. Among the 54 patients who underwent primary reconstruction, 37 patients were males and 17 were females. Age ranged from 2 to 70?years. Amongst the wild animals, bear was the most common offending animal while dog was the most common street animal. Overall bear bites were the most common presentation to our facility. Four out of 54 cases (7.4%) with primary reconstruction developed infection of the operative site. Of the patients with immediate reconstruction, 57.4% thought that the result was excellent, 35.2% patients were satisfied with the result but wanted further refinement while 7.4% were unhappy and wanted immediate revision. Of the 37 patients who did not undergo primary reconstruction, 16.2% thought that the result was excellent, 45.9% were satisfied and 37.8% wanted immediate revision. We recommend that animal bite wounds should be considered for reconstruction at the earliest to achieve maximal functional and aesthetic results.     

Long live the Liver!

International Journal of Diabetes in Developing Countries -     

Fanconi-Bickel Syndrome - Mutation in SLC2A2 Gene

Fanconi-Bickel Syndrome (FBS) is a rare autosomal recessive disorder of carbohydrate metabolism. The defect in the GLUT 2 receptors in the hepatocytes, pancreas and renal tubules leads to symptoms secondary to glycogen storage, glucose metabolism and renal tubular dysfunction. Derangement in glucose metabolism is classical with fasting hypoglycemia and post-prandial hyperglycemia. The authors report a 4-year-old boy who presented with failure to thrive, motor delay, protuberant abdomen and was noted to have huge hepatomegaly with glycogen deposition in liver, and renal tubular acidosis. Gene sequencing revealed homozygous mutation, c.1330T > C in SLC2A2 gene, thus confirming the diagnosis of FBS. Only three mutations have been reported from India so far. The primary reason for referral to authors’ hospital was for liver transplantation, but an accurate diagnosis led to avoidance of the major surgery and streamlining of treatment with clinical benefit to the child and family.     

Plasma Exchange for Hemolytic Crisis and Acute Liver Failure in Wilson Disease

Wilson disease (WD) is a rare autosomal recessive disorder of copper metabolism which primarily involves the liver and the central nervous system. Rarely, WD can present as acute liver failure (ALF) and this disease is universally fatal in the absence of liver transplantation. The authors report a young girl with WD ALF, who showed signs of recovery after prompt initiation of plasma exchange (PE) and chelation therapy. Though liver transplantation could not be done in this child and the child died 8 d after stopping PE, this case highlights that PE can be a successful medical treatment in WD ALF and should be considered as a therapeutic measure to stabilize a patient by decreasing serum copper, reducing hemolysis, and helping to prevent renal tubular injury from copper and copper complexes until liver transplantation is possible.