Histopathologic characteristics of Steroid-Resistant Nephrotic Syndrome in children in Iran

Nephrotic syndrome is the most common chronic renal disease in children. Mostly, it is controlled by steroids. Many underlying pathologies exist in patients with steroid-resistant nephrotic syndrome [SRNS]. Among them are 'focal segmental glomerulosclerosis [FSGS] and 'minimal change disease' [MCD]. Examining patients' clinicopathologic characteristics can be helpful by giving an insight into the etiology of steroid resistance and determining patient prognosis. This cross-sectional study was performed in 'Children's Medical Center' between 2001 and 2011. From 150 patients biopsied, seventy-one children with SRNS, aged 1-14 years, were included. Among 150 patients biopsied, 71 children [47.3%] had steroid-resistant nephrotic syndrome. Forty-four [62%] of these were boys. Upon pathologic investigation of SRNS cases, FSGS came in first, with the highest prevalence at a rate of 32.4%, and MGN came in last, at a rate of 5.6%. The mean age of disease onset was 4.7 years and the mean age of undergoing biopsy was six years.In this study, the predominant pathologic pattern of steroid-resistant nephrotic syndrome was FSGS, a finding similar to that of most studies conducted in this field. MCD was observed in 21.1% of patients, which indicates the variety in reporting renal lesions, particularly, regarding the diagnoses of MCD, mesangio-proliferative glomerulonephritis and early stages of FSGS

treatment algorithm for children with lupus nephritis to prevent developing renal failure

Chronic kidney disease is one of the most common complication of systemic lupus erythematosus, which if untreated can lead to the end-stage renal disease [ESRD]. Early diagnosis and adequate treatment of lupus nephritis [LN] is critical to prevent the chronic kidney disease incidence and to reduce the development of ESRD. The treatment of LN has changed significantly over the past decade. In patients with active proliferative LN [Classes III and IV] intravenous methylprednisolone 1 g/m[2]/day for 1-3 days then prednisone 0.5-1.0 mg/kg/day, tapered to <0.5 mg/kg/day after 10-12 weeks of treatment plus mycophenolate mofetile [MMF] 1.2 g/m[2]/day for 6 months followed by maintenance lower doses of MMF 1-2 g/day or azathioprine [AZA] 2 mg/kg/day for 3 years have proven to be efficacy and less toxic than cyclophosphamide [CYC] therapy. Patients with membranous LN [Class V] plus diffuse or local proliferative LN [Class III and Class IV] should receive either the standard 6 monthly pulses of CYC [0.5-1 g/m[2]/month] then every 3[rd] month or to a shorter treatment course consisting of 0.5 g/m[2] IV CYC every 2 weeks for six doses [total dose 3 g] followed by maintenance therapy with daily AZA [2mg/kg/day] or MMF [0.6 g/m[2]/day] for 3 years. Combination of MMF plus rituximab or MMF plus calcineurin inhibitors may be an effective co-therapy for those refractory to induction or maintenance therapies. This report introduces a new treatment algorithm to prevent the development of ESRD in children with LN

Preventing kidney injury in children with neurogenic bladder dysfunction

The most common cause of neurogenic bladder dysfunction [NBD] in newborn infants is myelomeningocele. The pathophysiology almost always involves the bladder detrusor sphincter dyssynergy [DSD], which if untreated can cause severe and irreversible damage to the upper and lower urinary tracts. Early diagnosis and adequate management of NBD is critical to prevent both renal damage and bladder dysfunction and to reduce chances for the future surgeries. Initial investigation of the affected newborn infant includes a renal and bladder ultrasound, measurement of urine residual, determination of serum creatinine level, and urodynamics study. Voiding cystogram is indicated when either hydronephrosis or DSD is present. The main goal of treatment is prevention of urinary tract deterioration and achievement of continuance at an appropriate age. Clean intermittent catheterization [CIC] in combination with anticholinergic [oxybutynin] and antibiotics are instituted in those with high filling and voiding pressures, DSD and/or high grade reflux immediately after the myelomeningocele is repaired. Botulium toxin-A injection into detrusor is a safe alternative in patients with insufficient response or significant side effects to anticholinergic [oral or intravesical instillation] therapy. Surgery is an effective alternative in patients with persistent detrusor hyperactivity and/or dyssynergic detrusor sphincter despites of the CIC and maximum dosage of anticholinergic therapy. Children with NBD require care from a multidisciplinary team approach consisting of pediatricians, neurosurgeon, urologist, nephrologists, orthopedic surgeon, and other allied medical specialists