We report on a case of autoimmune thyroiditis in a 6-month-old patient with cortico-resistant nephrotic syndrome. Normal serum levels of thyroid hormons and thyroid-stimulating hormone were detected with high titers of circulant antithyroid antibodies and a dysomogeneous ultrasound appearance of the gland, typical of autoimmune thyroiditis. The research of maternal thyroid antibodies was negati...

patients presenting with nephrotic syndrome with or without nephritic illness rarely come across with the diagnosis of ‘c1q nephropathy’. this entity is purely diagnosed with the help of immunofluorescence like iga nephropathy. clinical presentation is heterogenous, ranging from nephrotic range proteinuria to sub-nephrotic state; and with or without hematuria / renal insufficiency. similarly, t...

OBJECTIVE To assess the efficacy of levamisole in frequently relapsing nephrotic syndrome and steroid-dependent nephrotic syndrome. STUDY DESIGN Retrospective analysis of hospital case records. SETTING Pediatric nephrology department of a tertiary referral pediatric hospital. PARTICIPANTS 62 children with frequently relapsing nephrotic syndrome and 35 children with steroid-dependent nephr...

Nephrotic syndrome associated with a malignant tumor may remit following resection of the tumor. This report documents a case of esophageal cancer with concurrent nephrotic syndrome in which a surgical resection of the tumor resulted in a complete remission of nephrotic syndrome. A 78-year-old male patient noticed edema of his lower legs in February 2009 and was diagnosed with nephrotic syndrom...

OBJECTIVES Familial steroid-sensitive idiopathic nephrotic syndrome is rare, and only approximately 3% of patients have affected siblings. METHODS Herein, we report seven cases of patients with steroid-sensitive idiopathic nephrotic syndrome from three Chinese families. Mutational screening of the Nphs2 gene was performed in all the patients. RESULTS All seven of the familial steroid-sensit...

Diseases of the glomerular filter of the kidney are a leading cause of end-stage renal failure. Recent studies have emphasized the critical role of the slit diaphragm of podocytes for the size-selective filtration barrier of the kidney and revealed novel aspects of the mechanisms that lead to proteinuria, in both inherited and acquired diseases (1–4). Several critical structural protein compone...

CD2-associated protein (CD2AP) is an 80-kilodalton protein that is critical for stabilizing contacts between T cells and antigen-presenting cells. In CD2AP-deficient mice, immune function was compromised, but the mice died at 6 to 7 weeks of age from renal failure. In the kidney, CD2AP was expressed primarily in glomerular epithelial cells. Knockout mice exhibited defects in epithelial cell foo...

Wheezy bronchitis was originally used to describe preschool children who wheezed only after viral respiratory tract infections and who seemed relatively resistant to sympathomimetic and steroid treatment. It was thought that only a minority of these children went on to develop 'asthma'. With the increasing awareness of the underdiagnosis of asthma in childhood the term was re-examined.2 Wheezy ...

Mutations in NPHS1, which encodes nephrin, are the main causes of congenital nephrotic syndrome (CNS) in Finnish patients, whereas mutations in NPHS2, which encodes podocin, are typically responsible for childhood-onset steroid-resistant nephrotic syndrome in European populations. Genotype-phenotype correlations are not well understood in non-Finnish patients. We evaluated the clinical presenta...

A three week old infant, born at term, weighing 3.15 kg, following an uncomplicated pregnancy (placental size not known), developed lethargy, rapid breathing and poor feeding. He was treated for septicaemia at the local hospital and was transferred to the Lady Ridgeway Children’s Hospital with haematuria and abdominal distension. He was the second child of non-consanguineous parents whose first...