Infantile cystinosis: From dialysis to renal transplantation.

Cystinosis is an autosomal recessive, lysosomal storage disease characterised by the accumulation of the amino acid cystine in different organs and tissues. It is a multisystemic disease that can present with renal and extra-renal manifestations. In this report, we present the first case of transplanted nephropathic cystinosis in a Tunisian child.

Contribution of ultrasound scans in the first episode of urinary tract infection in children.

Background - Vesicoureteral reflux (VUR) is a common pediatric urologic disorder. After the first urinary tract infection (UTI), imaging studies are recommended, starting with a renal ultrasound (US) and voiding cystourethrography (VCUG). We propose to determine whether abnormalities found on US can help indicate the necessity of VCUG in children after the first urinary tract infection.

Mycophenolate mofetil in treatment of childhood steroid-dependent nephrotic syndrome.

Objectives: To establish the efficacy of mycophenolate mofetil (MMF) in steroid dependent nephrotic syndrome and to determine the predictive factors for a good response.

Methods: retrospective hospital-based cohort study in the department of pediatric of Charles Nicolle hospital, between 2005 and 2012 included 30 children with steroid-dependent nephritic syndrome who were treated with MMF.

Results: A total of 30 patients (20 boys and 10 girls) were included.

Primary hyperoxaluria in infants.

The infantile form of primary hyperoxaluria type-1 (PH-1) is characterized by a rapid progression to the end-stage renal disease (ESRD) due to both increased oxalate load and reduced glomerular filtration rate. In the literature, data on this form are limited. The purpose of this study is to analyze retrospectively the clinical, biological, and radiological features of children who were diagnosed with PH-1 during the 1(st) year of life.

[Etiologies of end-stage renal disease of children in Tunisia].

Background: The end-stage renal disease (ESRD) in children has special features in terms of etiologies, therapeutic modalities and access to renal transplantation. In Tunisia, there are no data on the epidemiology of ESRD in children. The aim of our study was to describe epidemiology of ESRD among Tunisian children.

[Peritonitis in pediatric patients receiving peritoneal dialysis].

Background: Peritonitis on catheter of dialysis represents the most frequent complication of the peritoneal dialysis (PD) in the pediatric population. It remains a significant cause of morbidity and mortality. In this study, we investigated the risk factors for peritonitis in children.

[Outcome of rapidly progressive glomerulonephritis post-streptococcal disease in children].

Background: Rapidly progressive glomerulonephritis is a rare form of postinfectious glomerulonephritis. The aim of this study was to describe the outcome of our patients with severe post-streptococcal glomerulonephritis.

Methods: This retrospective study was conducted in the department of pediatrics in Charles-Nicolle Hospital during a period of 13 years (1997-2009).

Acute pancreatitis: a rare complication in a patient with senior loken syndrome.

Introduction: Senior-Loken syndrome is a rare entity that combines familial nephronophthisisand retinal dystrophy. It has an autosomal recessive inheritance pattern and is characterized by a chronic tubulointerstitial nephritis that progress to terminal renal failure during the first or second decades of life. Systemic associations of this syndrome include sensorineural hearing loss, liver fibrosis or cerebral vermis hypoplasia.

[Thromboembolic complications of childhood nephrotic syndrome].

Background: Vascular thrombosis in the childhood nephrotic syndrome is a rare event. It poses major diagnostic and therapeutic problems. The prognosis depends on early diagnosis and on precocious anticoagulation.

[Early infections in children following renal transplantation].

Background: Infections following renal transplantation in children are a major cause of severe morbidity and mortality. Surgery is complex and performed in a subject whose immunological mechanisms are impaired by end stage renal failure and immunosuppressive drugs.

Aim: To evaluate the incidence and the risk factors of early infectious following renal transplantation in children.