Systemic lupus erythematosus in childhood--a review of 11 patients at a single center in eastern Nepal.

We retrospectively evaluated the clinico-laboratory features of 11 children and adolescents with Systemic Lupus Erythematosus between the period of 2001 and 2006. All of them (100.0%) had renal involvement at the first visit in the hospital. Female to male ratio was 10:1. Skin and or mucosal involvement (90.9%), periorbital puffiness and or pedal edema (81.8%), fever (72.7%), hypertension (72.7%), and reticuloendothelial involvement (72.7%), were the commonest presentations. All patients had anemia (8.6 +/- 1.5 gm/dl), raised ESR (46.7 +/- 9.4 mm in first hour), proteinuria, and in disease activity as evident by raised ESR and positive anti-dsDNA antibody at the first visit. The mean duration of disease was 7.6 months and the average duration of disease activity was 63.18 days. Renal biopsy was performed in 8 patients: class IV lupus nephritis in 4 patients (50.0%), class II in 2 patients (25.0%), class III and V in patient (12.5%) each. Nephrotic range proteinuria and hypertension was observed in all patients of class IV and V of lupus nephritis. Class II and III lupus nephritis patients' were normotensive and had non-nephrotic range proteinuria. Three out of 11 patients (27.2%) expired. The commonest primary determinant of mortality was uncontrolled disease activity in 2 patients (66.6%). The third one had infection and developed disseminated intravascular coagulation. The mean duration of disease activity in patients who died (mean 30 days) was statistically lower than the survival group (75.6 days) (p < 0.01). Renal involvement during first visit and mortality could be attributed by late referrals and diagnosis at hospital.

Detection of beta-thalassemia (homozygous) by hemoglobin electrophoresis on agar gel and citrate agar medium: a case report.

A seven month old male baby was admitted to NICU of BPKIHS, Dharan with dyspnoea and distress. He was investigated for hemolytic anemia and suspected of beta Thalassemia (Homozygous) based on the low level of Hb and high HbF%. To investigate further, Hb electrophoresis was carried out using agar gel and citrate agar gel at pH 8.6 and 6.0 respectively along with control. Electrophoretogram showed single narrow band lagging behind the healthy control moved toward anode at pH 8.6 and single narrow band moved parallel to healthy control toward cathode at pH 6.0. Thus the diagnosis of betaThalassemia (Homozygous) is favored.