Pseudohypoaldosteronism type-I: a rare cause of hyperkalemia in neonates.

Pseudohypoaldosteronism type I (PHA-I) is a rare disorder with only a few cases reported worldwide. It appears early in life with salt-wasting, failure to thrive, dehydration, hypotension, hyperkalaemia and metabolic acidosis. There is a resistance to aldosterone by the mineralocorticoid receptors. We describe one such case of a 14-day-old female neonate who presented with frequent episodes of dehydration, hyperkalaemia and hyponatraemia. On further workup, she proved to be a case of PHA-I. The aim of this report is to discuss the evaluation and to highlight the difficulties associated with the management of this rare disorder.

Risk factors and types of cerebral palsy.

OBJECTIVE: To determine the risk factors and associated types of cerebral palsy in a squatter settlement of Karachi. METHODS: The pilot cross-sectional study was conducted in Karachi during 2010 and 2011. Data was collected through an interviewed questionnaire from the mothers of cerebral palsy victims children from a population of 6000. Sample sizes of 20 pre-diagnosed victims were selected through snowball sampling. SPSS 20 was used for statistical significance. RESULTS: The mean age of the 20 children was 8.7 +/- 6.4 years. Of them, 16 (80%) were males and 4 (20%) were females. Major risk factors identified were; home and assisted delivery 5 (75%), consanguinity 10 (50%), infections 8 (40%) and lack of antenatal care 6 (30%). Out of 20 cases, 15 (75%) had spastic type of cerebral palsy, which was further classified as diplegia 7 (35%), quadriplegia 6 (30%) and hemiplegia 2 (10%). Mixed and dystonic types were found in 3 (15%) and 2 (10%) children respectively. CONCLUSION: Important risk factors identified were home delivery, consanguinity and infections during pregnancy. Spastic type of cerebral palsy was the most common type in the study population.

Congenital adrenal hyperplasia with cholestatic jaundice.

Congenital Adrenal Hyperplasia describes a group of autosomal recessive disorders characterized by a decrease in Cortisol production. 11 beta hydroxylase deficiencies is the second most common form. However, its presentation with cholestatic jaundice is extremely rare. We present a case of a 29-day-old infant who came to us with unusual dark complexion, persistent jaundice, and electrolyte imbalance. On investigation he was diagnosed as a case of congenital adrenal hyperplasia. Treatment with hydrocortisone and fludrocortisone cleared his jaundice and complexion with subsequent improvement in electrolytes. The aim of this report is to illustrate an unusual presentation of CAH with Cholestatic jaundice. This is the first case to be reported from Pakistan. The case outlines the difficult workup that was encountered in the diagnosis and management of the patient.

Maintenance of empathy levels among first and final year medical students: a cross sectional study.

OBJECTIVES: The purpose of this study was to quantify the levels of empathy amongst medical students in the first year and final year of the medical curriculum at a medical university in Karachi, Pakistan. METHODS: A cross-sectional study, comprising of participating students in their first year and final year of the medical curriculum at Ziauddin University Medical College, was carried out, using the Empathy Quotient (EQ) scale consisting of 60 questions through a self-administered questionnaire. The results were collected anonymously over a time period of six months from a sample of 171 participants. Results : According to our analysis, we found 82.67% of fifth year students and 80.21% of first years showing average or above average levels of empathy. Female mean scores were 42±9.60 while males were 38.7±9.358 (P=0.03). No association was found between empathy and age of the participants (p=0.77). CONCLUSION: We found no significant difference in the levels of empathy between the first and fifth year medical students. However, it was shown that females exhibited higher levels of empathy than males.