Pediatric focal segmental glomerulosclerosis in Jordan: A tertiary hospital experience.

Our objective is to study the demographical data, clinical course and outcome of children with primary focal segmental glomerulosclerosis (FSGS) in Jordan. A retrospective chart review of patients with a diagnosis of FSGS at a tertiary care hospital from the period July 2010 to July 2016 was conducted. A total of 99 patients were analyzed. The mean age of presentation was 3.71 ± 2.59 years, 66% were male. At presentation, 66.6% of patients were steroid-resistant, 10% had a steroid dependant course and 20.2% had familial FSGS. Cyclosporine was used in 66.6% of children with a response rate of 46.9%. Long-term follow-up showed complete remission in 29.3%, partial remission in 31.3%, end-stage renal disease in 22.2%, and death in 11.1%. There is a high prevalence of familial FSGS in our Jordanian cohort with a high rate of progression to end-stage kidney disease.

The clinical pattern of primary hyperoxaluria in pediatric patient at Queen Rania Abdulla Children Hospital.

INTRODUCTION: Hyperoxaluria is a metabolic disorder that can lead to end stage renal disease (ESRD). It can be either inherited or acquired. Primary hyperoxaluria (PHO) is more common and characterized by an excessive production of oxalate leading to recurrent urolithiasis and progressive nephrocalcinosis. Due to the high rate of consanguineous marriage in Jordan this disease is commonly diagnosed in pediatric nephrology clinics. We aimed to demonstrate the clinical pattern and progression to ESRD in pediatric patients with hyperoxaluria at Queen Rania Abdulla Children Hospital. METHODS: Medical records of all patients followed up in the pediatric nephrology clinic with the diagnosis of PHO during the period between September 2007 and March 2013 were reviewed. RESULTS: There were 70 patients with the diagnosis of PHO, 52.9% were males. The median age at presentation was 3 years ± 3 months with the youngest child being two months old. Diagnosis was made in the first year of life in 15.7% of patients. The most common presenting symptom was hematuria, while 14% of patients were asymptomatic and detected by family screening after the diagnosis of an index case. At the time of initial presentation, 15.7% of patients had ESRD and 25% had impaired renal function. Kidney stones were found in 57% of cases and nephrocalcinosis was found in 37%. CONCLUSION: High index of suspicion is needed to diagnose PHO in children presenting with kidney stone or unexplained hematuria. Twenty-four hour urine collection for oxalate are required to make the proper diagnosis. Family screening, when appropriate, is indicated for early detection of PHO.

Hypercalcemia, hypercalciuria and nephrocalcinosis in a breast-fed term newborn: a rare presentation.

Although hypercalcemia and hypercalciuria are known to occur in breast-fed pre-term infants, to the best of our knowledge, it has never been reported in a term baby previously. We report a term male baby who was followed-up during pregnancy for having bright kidneys, but a follow-up renal ultrasound (US) after birth had revealed normal scan. Laboratory investigations revealed normal serum calcium (Ca), phosphorous (PO4) and alkaline phosphatase (ALP). The baby was being fed by breast milk. Follow-up US two months later showed early nephrocalcinosis along with hypercalcemia and hypercalciuria; by the age of three months, nephrocalcinosis was more extensive and the serum Ca level was more than 12 mg/L with hypercalciuria. Parathyroid hormone (PTH), phosphorous (PO4), ALP and thyroid function tests were all normal. Antenatal history revealed a hypothyroid mother who was maintained on L-thyroxin, calcium and vitamin D supplement during pregnancy. Her blood tests showed normal serum Ca, low PO4 and elevated PTH. The baby was diagnosed to have hypercalciuria and hypercalcemia secondary to maternal hypophosphatemia (maternal vitamin D deficiency). Breast feeding was stopped and the baby was started on formula, whereby he showed remarkable improvement both for his blood chemistry as well as his hypercalciuria.