Pulmonary arteriovenous malformation in cryptogenic liver cirrhosis.

The cause in 10 - 20% cases of liver cirrhosis (LC) cannot be elucidated, and are thus termed cryptogenic. Pulmonary arteriovenous malformations (PAVMs) are relatively rare, but the most common anomaly involving the pulmonary tree. Although the rare correlation between LC and PAVM is well-known, there have been no reports of PAVMs occurring in cryptogenic LC. We report a case of PAVM that occurred in cryptogenic liver cirrhosis in a 3 years old male child. The child presented with complaints of malena, hematemesis and variceal bleed. The examination revealed a child with respiratory distress, irritability, tachycardia, clubbing and abdominal distention. He was worked up for recurrent variceal bleeding secondary to portal hypertension but the oxygen saturation during hospital stay kept deteriorating. The diagnosis of hepatopulmonary syndrome as the cause of persistent hypoxemia in the absence of other cardio-pulmonary causes was then made by enhanced echocardiogram using agitated saline. He improved significantly after liver transplantation performed abroad. At a 6 months follow-up, the child was stable with no evidence of intrapulmonary shunting on repeat echo.

Oesophageal duplication cyst presenting as haemoptysis.

Duplications of the alimentary tract include a variety of cysts, diverticula, and tubular malformations, all believed to have embryological origin. The cysts are most commonly found in children, and the diagnosis is made in infancy in the majority of patients. We report a case of a two-and-a-half year old child, presenting with the history of repeated episodes of haematemesis. Upper GI endoscopy was unremarkable and the chest x-ray showed no pathology. Computed tomography (CT) angiogram revealed soft tissue density lesion in the right chest at the level of T6. Right thoracotomy suggested a cystic mass close to the oesophagus which was shown on histopathology to be lined with gastric mucosa consistent with oesophageal duplication cyst. To the best of our knowledge, this is the first case of its kind reported from Pakistan.

Current efficacy of antibiotics against Klebsiella isolates from urine samples - a multi-centric experience in Karachi.

Due to emergence of bacterial resistant strains, the effectiveness of current antibiotic treatment without culture/sensitivity testing is questionable. Our study aims to assess the present sensitivity profiles of Klebsiella isolates from urine samples and provide options for empiric prescription in critically ill patients. Klebsiella pneumoniae isolates collected over a period of 28 months till January 2011 from 1,617 urine samples of subjects presenting with Urinary Tract Infections were identified at a local diagnostic lab using standard protocol and subjected to Kirby-Bauer disk diffusion sensitivity testing. MICs were also estimated by E-nephelometry. Among 20 drugs used, low sensitivity was found to amoxicillin (0.1%), doxycycline (11.5%), nitrofurantoin (15.5%), amoxiclav (18.2%), gentamicin (35.4%), pipemidic acid, cephradine (40.3%) and cotrimoxazole (43.1%). The isolates were more sensitive to cefuroxime (55.9%), cefixime (57.7%), ciprofloxacin (62.5%), ofloxacin (63%), ceftriaxone (66.2%), ceftazidime (66.4%), cefotaxime (66.6%), fosfomycin (77.5%) and amikacin (89.4). Most effective were cefroperazone.sulbactam (95.8%), piperacillin.tazobactam (95.7%) and imipenem (97.7%). Self-medication, lack of awareness, and the misuse of antibiotics by doctors has exacerbated the menace of microbial resistance. The study warrants the prudent choice of drugs in adherence with prevailing sensitivity profiles.

Incidence and outcome of small for gestational age foetuses: an experience from a secondary care hospital.

The objective of the study was to determine the frequency and outcome of Small for Gestational Age (SGA) foetuses, and its association with the mode of delivery, foetal birthweight, maternal age and gestational age. It was a hospital-based descriptive study, conducted at Aga Khan Hospital for Women, Karachi, from January 2009 to December 2010. Mode of deliveries, foetal birthweight, maternal age and gestational age of SGA foetuses were recorded and analszed. Of a total of 6024 deliveries during the study period, 722 (11.98%) had SGA foetuses. Maternal age in such cased ranged between 20-40 years in 588 (81.44%), while 406 (56.23%) of these deliveries were in between 34-37 weeks of gestation. Besides, 396 (54.84%) cases were delivered vaginally. The incidence of SGA increased from 2009 to 2010 (13.13% vs 10.76%). The frequency of SGA foetuses tends to be significantly higher in Pakistan compared to the Western world. Prenatal care and better management can potentially avoid the occurrence of SGA deliveries, perinatal morbidity and mortality.

Idiopathic pulmonary haemosiderosis presenting as severe iron deficiency anaemia--a case from Pakistan.

Idiopathic pulmonary haemosiderosis is an uncommon disorder, which is characterized by iron deficiency, recurrent haemoptysis and diffuse parenchymal infiltration on chest radiograph. We report an 8 year old child who had past history of multiple blood transfusions with diagnosis of iron deficiency anaemia and recurrent chest infection since the age of 21/2 year. At the age of 8 years, the child presented with fever and severe respiratory distress requiring intubation and ventilation. On Chest X-ray, bilateral white out was found and CT scan lung showed diffuse alveolar involvement. Lung biopsy confirmed haemosiderin-laden macrophages. Child was put on steroids and despite severe anaemia (Hb 3.2 g/dl), he showed improvement and survived. To our knowledge, this is the first case of idiopathic pulmonary haemosiderosis reported from Pakistan.

Urinary tract infection presenting as jaundice in neonates.

Neonatal Hyperbilirubinaemia is a common finding during the first postnatal week. Physiological jaundice occurs in first week of life in 60% of term and 80% of premature neonates. Non physiologic or pathologic jaundice occurs in 5-10% of newborns which require intervention. According to AAP guidelines laboratory investigation for jaundice include total serum bilirubin, blood Type and coombs test and if the baby has an elevation of direct reacting or conjugated bilirubin, there should be a urine analysis and urine culture. Here we are presenting 5 cases that developed indirect hyperbilirubinaemia and routine workup done according to AAP guidelines were normal. On extensive investigation all cases found to have urinary tract infection despite of having indirect bilirubin and they needed course of antibiotics according to sensitivities and follow up ultrasound. From our experience we suggest that UTI should be considered as a cause of neonatal jaundice especially when indirect bilirubin peaks after one week of life at mean age of 10.8 +/- 2.38 days.