Dubin-Johnson syndrome.

A young man presented with recurrent episodes of mild jaundice. Apart from conjugated hyperbilirubinemia, other liver function tests were always normal. Clinical suspicion of Dubin-Johnson syndrome was raised. Liver biopsy showed diffuse deposition of coarse granular dark brown pigment in hepatocytes. Dubin-Johnson syndrome is a benign condition, which results from a hereditary defect in biliary secretion of bilirubin pigments, and manifests as recurrent jaundice with conjugated hyperbilirubinemia. The defect is due to the absence of the canalicular protein MRP2 located on chromosomes 10q 24, which is responsible for the transport of biliary glucuronides and related organic anions into bile. No treatment is necessary and patients have a normal life expectancy.

Maternal morbidity and mortality due to primary PPH--experience at Ayub Teaching Hospital Abbottabad.

BACKGROUND: Postpartum Haemorrhage (PPH) remains a significant cause of maternal mortality and morbidity like hypovolemic shock, anaemia, multi organ failure, consumptive coagulopathy, disseminated intra vascular coagulation (DIC), blood transfusion related complications and hysterectomy leading to loss of childbearing potential. The present study was conducted to determine the frequency of PPH and the associated maternal morbidity at the Department of Gynaecology Unit 'B', Ayub Teaching Hospital Abbottabad. METHODS: The study was carried out in the Department of Obstetrics and Gynaecology Unit B of the Ayub teaching Hospital Abbottabad from 18th April 2006 to 17 July 2006. The study population included all cases admitted with primary PPH during the study period. For calculation of frequencies, the total number of deliveries in the setting during the study period was used. All subjects underwent a complete obstetrical clinical workup comprising of history, general physical examination, abdominal and pelvic examination, relevant laboratory investigations. The maternal condition was assessed and managed according to established hospital protocols which included both pharmacological and surgical intervention. All maternal complications were noted and recorded on pre-designed proformas. Data was entered and analyzed by computer. RESULTS: A total of 50 cases of primary PPH were recorded during the study period. The frequency of PPH was calculated as 7.1%. The major cause of PPH was uterine atony found in 29 (58%) cases, followed by cervical, vaginal and perineal tears in 12 (24%) cases. Initially all patients were managed pharmacologically followed by surgical intervention. Subtotal (haemostatic) hysterectomy was performed in 10 (20%) cases. Maternal morbidity was detected in 31 (62%) of cases; the major morbidities were DIC in 3 (6%) cases, Acute renal failure in 3 (6%) patients and shock in 2 (9.9%) cases and anaemia in 20 (90.1%) cases. CONCLUSION: The study concludes that the frequency of primary PPH in this setting is in keeping with globally cited frequencies. Other findings such as causes of primary PPH and maternal morbidity data also agree with most national and international studies on this topic.

Unusual metastases of hepatocellular carcinoma (hcc) to bone and soft tissues of lower limb.

Hepatocellular carcinoma metastasizing to bones and soft tissues of extremities is an unusual occurrence. The present report describes two such cases, where this unusual happening was the presenting feature. Role of immunohistochemistry for diagnosis is emphasized.