Steroid induced ocular complications in idiopathic nephrotic syndrome: a cross sectional single center study.

Objective: To screen children receiving steroids to identify ocular complications and their prevalence. METHODS: The cross-sectional study was conducted at the Paediatric Nephrology and Ophthalmology departments of the Sindh Institute of Urology and Transplantation, Karachi, from May to October 2022, and comprised patients who received at least 1500mg cumulative steroid dose for a minimum of 3 months. They were screened for steroidsensitive or steroid-resistant nephrotic syndrome. Ocular examinations, including visual acuity, intraocular pressure, slit-lamp biomicroscopy, lens examination and fundus evaluation, were performed. Data was analysed using SPSS 22. RESULTS: Of the 124 subjects with mean age 8.15±2.03 years (range: 6-12 years), 64(51.6%) were boys. Steroidsensitive nephrotic syndrome was present in 97(78%) cases. The mean cumulative steroid dose was 3999.31±1564.22mg. Overall, 36(29%) children developed ocular complications. Blood pressure, number of relapses and the duration of treatment were significantly associated (p<0.05). Conclusion: Refractive errors were the most frequent side effects/complication seen among children with nephrotic syndrome who received prolonged corticosteroids.

Cutaneous Manifestations in Patients with Beta-Thalassemia Major.

BACKGROUND: Beta-thalassemia major is a transfusion-dependent thalassemia. Both ongoing disease-related inflammatory processes and chronic transfusions lead to iron overload, which is depicted by hyperferritinemia. We aimed to report the prevalence of various dermatological manifestations in beta-thalassemia major patients and their relationship with serum ferritin levels. MATERIAL AND METHODS: This was a cross-sectional study conducted over a period of six months. Beta-thalassemia major patients were consecutively enrolled and examined by a dermatologist who charted any skin conditions, if present. A blood sample was also taken at the same time to check for the serum ferritin levels. Data was analysed using SPSSv25. RESULTS: A total of 113 patients were included in the study. The mean age of the cohort was 9.32 ± 4.54 years. The mean ferritin level for the cohort was 3334 ± 1676 micrograms per litre. Cutaneous manifestations were seen in 89.4% (n = 101) patients with the common ones namely xerosis (44.2%), freckles (39.8%) and pruritus (44.2%). We noted that serum ferritin levels were significantly higher in those with freckles (p = 0.00288). The cause of pruritus does not appear to be jaundice (p = 0.973). Lastly, number of skin conditions were higher in those with onset of blood transfusions at age less than one year (p = 0.0011). CONCLUSION: Dermatological manifestations are a frequently encountered problem in beta-thalassemia major patients. It is important to examine these patients for various skin disorders periodically as this can help improve their quality of life and reduce dermatological-associated morbidity.

Transcatheter Palliation of a Young Infant With Obstructed Supracardiac Total Anomalous Pulmonary Venous Connection.

Obstructed total anomalous pulmonary venous connection is a life-threatening pediatric cardiac emergency. Infants usually present in critical condition with marked respiratory distress, severe metabolic acidosis, and central cyanosis. Urgent cardiac surgical intervention, despite its high risk, is necessary in order to save the life of the patient. A two-month-old female infant presented to our tertiary care hospital with dense cyanosis and metabolic acidosis. She required mechanical ventilation, but her oxygen saturation did not improve. Her 2D transthoracic echocardiography revealed obstructed supracardiac total anomalous pulmonary venous connection with adequate interatrial communication and severe pulmonary hypertension. After discussion with the family and pediatric cardiac surgical team, it was decided to offer her transcatheter relief of obstructive ascending channel. She underwent successful balloon angioplasty of stenosed levoatrial cardinal vein (vertical vein) with remarkable improvement in blood flow and vessel caliber. She was extubated and her oxygen saturation rose from the high seventies to low eighties immediately after the procedure. She is scheduled for cardiac surgical repair within the next few days. Transcatheter angioplasty is a workable option in stabilizing very sick young infants with obstructed total anomalous pulmonary venous connection, especially supracardiac ones.