Abstract

NEPHROTIC SYNDROME IN SULAIMANI PEDIATRIC TEACHING HOSPITAL

Dr. Ali Qais Abdulkafi*, Dr. Adnan Mohammed Hasan Hamawandi and Dr. Baker R. Rasheed

ABSTRACT

Background: Nephrotic syndrome (NS) is clinical manifestation of different histopathological subtypes. (NS) is characterized by proteinuria and hypoalbuminemia leading to edema. Hyperlipidemia, are usually associated. Most patients with (NS) have frequent relapses until disease resolve spontaneously toward the end of second decade of life and the main problem is their complications or side effects of drugs used, they suffer the influence of proteinuria, caused by the increase of the glomerular permeability. The therapy of (NS) is still a matter of controversy. Objective: To analyze the trend of histopathological subtypes in idiopathic nephrotic syndrome & to assess the options of treatment. Patients and Methods: This case series study was performed in Sulaymaniyah pediatric teaching hospital carried out over 10 months study period, started from 1st of Jan. to 30th of Oct. 2015 diagnosed with (NS) &/or treated and followed up in the pediatric consultation clinic through clinical examination and investigation. The following data parameters were collected (age, gender, initial episode, duration of disease, relapses, predisposing factors, family history of (NS), clinical presentation, investigation, treatment and complication). Patients with (NS) were involved in the study & the patients with other renal pathology were excluded from the study. We compare between each patient according to individual type of pathology and to the response to medication used. Results: The total number of patients involved was 17. Ten males (59%) and 7 females (41%) with male to female ratio 1.4:1. Age range between 2-18 years, with peak incidence at age group from 3-5 years. Age at onset ranged between 10 months-8 (median 4) years. Family history of (NS) was reported in 4 cases (23.6%). The numbers of patients presented with initial attack 1 (5.9%), while the rest with relapse. The main type of presentation was periorbital oedema. Hypertension was presented in 11 patients (64.7%), and only 6 patients (35.5%) had normal blood pressure. Steroid therapy was given to all patients, followed by Every Other Day Steroid (EODS). Steroid sensitive (NS) was found in 9 (52.9%), steroid dependent (NS) and steroid resistant (NS) in 6 (35.3%) and 2 (11.8%) respectively. Regarding the type of pathology, 11 patients (64.7%) were unknown biopsy (not down). Three patients (17.6%) showed minimal change disease, and other 3 (17.6%) focal segmental glomerulosclerosis. According to histopathology, the patients with unknown etiology had higher rate of response, patients with FSGS mostly resistant, and patients with MCNS show dependant on steroid. Respiratory tract infection was reported in 9 cases (52.9%), 6 patients (35.3%) had urinary tract infection, one patient (5.9%) developed septicemia. Conclusion: (NS) is a chronic disease with suffering a relapsing course and being at risk of frequent courses of prednisolone therapy with increase risk of side effect & complications. The drugs used are the common drugs and EODS is preferable type of medication used in SRNS. The trend of histopathological patterns has profound prognostic significance and has significant implications in the management of childhood (NS). Our finding is in agreement with the recommendation of performing renal biopsies on children with idiopathic nephrotic syndrome who are steroid dependant in addition to those who are steroid resistant particularly before starting cytotoxic medication.

Keywords: Nephrotic syndrome (NS), every other day steroid (EODS), steroid sensitive nephrotic syndrome (SSNS), steroid resistant nephrotic syndrome (SRNS).