A rare case of bleeding disorder: Glanzmann's thrombasthenia.

BACKGROUND: Glanzmann's thrombasthenia (GT) is a rare bleeding disorder, which is characterized by a lack of platelet aggregation. It is characterized by qualitative or quantitative abnormalities of the platelet membrane glycoprotein IIb/IIIa. Physiologically, this platelet receptor normally binds several adhesive plasma proteins, and this facilitates attachment and aggregation of platelets to ensure thrombus formation at sites of vascular injury. The lack of resultant platelet aggregation in GT leads to mucocutaneous bleeding whose manifestation may be clinically variable, ranging from easy bruising to severe and potentially life-threatening hemorrhages. OBJECTIVE: To highlight this rare but potentially life-threating disorder, GT. CASE REPORT: We report a case of GT that was first detected because of the multiple episodes of gum bleeding. The patient was an 18-year-old girl who presented with a history of repeated episodes of gum bleeding since childhood. Till the first visit to our hospital, she had not been diagnosed with GT despite a history of bleeding tendency, notably purpura in areas of easy bruising, gum bleeding, and prolonged bleeding time after abrasions and insect stings. GT was diagnosed on the basis of prolonged bleeding time, lack of platelet aggregation with adenosine di phosphate, epinephrine and collagen. CONCLUSION: GT should always be considered as differential diagnosis while evaluating any case of bleeding disorder.

Indigenous cost-effective peritoneo-venous shunt for refractory ascites.

About 5% of patients with chronic liver disease develop massive refractory ascites. These patients cease to respond to diuretic therapy and may develop prerenal azotemia. There is a small but definite role for the peritoneo-venous shunt in these patients. In our study of 36 patients, managed with locally made, single-valved peritoneo-venous shunts (GSAIMS shunts), shunt failure and complication rates were assessed postoperatively. There is a definite improvement in quality of life with this cost-effective locally made shunt if patients are selected carefully. Long-term follow-up of these patients is not possible because most of these patients succumb to advanced liver disease.