Schimke immune-osseous dysplasia (SIOD) is a rare autosomal recessive disorder presented with specific facial features, skeletal dysplasia, steroid resistance nephrotic syndrome (SRNS) and cellular immune insufficiency. This is a SIOD case reported from Iran. He was 5 years old boy when evaluated for proteinuria and short stature. In appearance, we detected hyperpigmented macules, kyphosc...

Aim: Focal segmental glomerulosclerosis (FSGS) is one of the common causes nephrotic syndrome (NS) in children. This study aims to determine demographical data, clinical course, treatment and renal outcome children with primary FSGS report experience a single center. Material Methods: A retrospective long-term 38 patients diagnosis at tertiary pediatric care hospital from period July 2005 2019 ...

Nephrotic syndrome is a frequently encountered disease in children. It mostly responsive to high-dose steroids, with some requiring steroid-sparing immunosuppressive regimens, or further, renal biopsy if resistant steroid therapy. However, nephrotic children post-allogeneic bone marrow transplant rarely encountered. The authors report here child who developed for β-thalassaemia major, the suspi...

Syphilitic keratoderma is a rare cutaneous manifestation of secondary syphilis, characterized by symmetrical and diffuse hyperkeratosis of the palms and soles. In addition, no cases of syphilitic keratoderma and uveitis have been reported in the dermatologic literature. A 69-year-old woman presented with steroid-resistant hyperkeratotic patches on the palms and soles and uveitis for 4 months. A...

IMPORTANCE IgG4-related disease, which is newly recognized, is characterized by lymphoplasmacytic infiltration with increased IgG4-secreting plasma cells. Although a favorable response to steroids has previously been reported, the durations of follow-up to confirm the long-term benefits and clinical courses were limited. We describe long-term favorable response of oral methotrexate in a patient...

A prospective study was undertaken to find out the benefit of immunosuppressive therapy(IV methyl prednisolone followed by oral prednisolone therapy for one year along with six doses of pulse monthly IV cyclophosphamide) in children with steroid resistant nephrotic syndrome. Thirty-four children with steroid resistant nephrotic syndrome were treated with above regime. The remission of the disea...

Steroid-resistant nephrotic syndrome (SRNS) in children carries a significant risk of progression to end-stage renal failure (ESRF). We report a two-step protocol adapted in children with SRNS. Thirty-seven SRNS were treated with cyclosporine A (CyA) in association with prednisolone (alternate day) for 6 months (first-step treatment). Twelve patients (32.4%) went into complete remission, and 2 ...