Glanzmann's thrombasthenia: role of angioembolization and factor VII in recurrent bleeding from ulcerated duodenum

Introduction: Glanzmann's thrombasthenia [GT] is a rare, genetically inherited, functional disorder of platelets. The pathology is deficient or dysfunctional platelet glycoprotein IIb/IIIa [GP IIb/IIIa] complex resulting in bleeding diathesis

Case Report: Here in, we report the effectivity of angioembolization with factor VII in a patient with Glanzmann's thrombasthenia [GT], who presented with recurrent bleeding from duodenal ulcer

Conclusion: Angioembolization with added infusion of factor VII, can be considered an equally effective alternate to platelet transfusion in patients of GT, who present with recurrent bleeding

Hepatic artery pseudoaneurysm mimicking Mirizzi syndrome

Acute cholecystitis leading to development of a pseudoaneurysm of the hepatic artery is a very rare complication; however, a pseudoaneurysm resulting in gallbladder neck compression with dilatation of intrahepatic duct giving a Mirizzi syndrome like presentation is virtually unreported to the best of our knowledge. We report a case of a 60 years male patient who presented in emergency department with right hypochondrial pain and mild jaundice. Initial diagnosis of hepatic artery pseudoaneurysm causing compression of neck of gallbladder and common bile duct was made on ultrasound examination. This was resulting in gross distention of gallbladder and mild dilatation of intrahepatic ducts. Findings were confirmed on CT scan. Later successful selective transcatheter arterial embolization of the aneurysm and percutaneous cholecystostomy were performed

Hydatid disease of Bone

A case of primary hydatid disease of the right femur is reported that presented with pathological fracture and was diagnosed at the time of exploration for biopsy. The patient was treated by removal of all cysts, irrigation with scolicidal solution, bone grafting and immobilisation of the fracture followed by four cycles of oral Albendazole. Eosinophilia and serological tests reverted to normal but the patient died due to acute myocardial infarction six months later. This uncommon condition should be considered in the differential diagnosis of pathological fractures, bone pain or osteolytic lesions, especially in patients of rural and farmer background

Omphalocele major: conservative management

In our community, majority of deliveries are at home and most have no intra-uterine fetal monitoring. Many congenital anomalies, including omphaloceles, are diagnosed after birth. Almost all such cases are transported to health facilities, wrapped in dirty clothing, and are heavily contaminated with established sepsis. Even in tertiary centers like ours, resuscitative and ventilatory facilities have several limitations. Under these circumstances, we have devised a line of management on conservative lines and in stages, for contaminated omphalocele major. This seems to offer the best chance of survival to the infants. The present report is of three children presented at the Children's Hospital PIMS, Islamabad. Their mode and state of presentation and method of management are discussed, which highlights several important features of this condition in our set-up. A review of literature is also presented