Primary hyperoxaluria type 1 in Tunisian children.

BACKGROUND: Primary hyperoxaliuria type 1 is an autosomal recessive disorder characterized by increasing urinary excretion of calcium oxalate, recurrent urolithiasis, nephrocalcinosis, and accumulation of insoluble oxalate throughout the body. This inborn error of metabolism appears to be a common cause of end stage renal disease in Tunisia. AIMS: To review the clinical, biological and radiological futures of primary hyperoxaluria type 1 and to correlate these aspects with the development of end-stage renal disease. METHODS: we retrospectively reviewed 44 children with Primary hyperoxaliuria type I who were treated in our department during a period of 15 years between 1995 and 2009. The diagnosis was established by quantitative urinary oxalate excretion. In patient with renal impairment, the diagnosis was made by infrared spectroscopy of stone or by renal biopsy. RESULTS: Male to female ratio was 1.2. The median age at diagnosis was 5.75 years. About 43 % of those were diagnosed before the age of 5 years. Initial symptoms were dominated by uraemia. Four patients were asymptomatic and diagnosed by sibling screening of known patients. Nephrocalcinosis was present in all patients. It is cortical in 34%, medullary in 32% and global in 34%. At diagnosis, twelve children were in end-stage renal disease (27%). Pyridoxine response, which is defined by a reduction in urine oxalate excretion of 60% or more, was found in 27%. CONCLUSION: In the majority of patients, the clinical expression of Primary hyperoxaliuria type 1 is characterized by nephrocalcinosis, urolithiasis and renal failure. Pyridoxine sensitivity is associated with better outcome.

[Early infections in children following renal transplantation]. / Complications infectieuses precoces chez l'enfant transplante renal.

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. METHODS: Infectious complications were retrospectively monitored in 37 children receiving renal transplantation at our center from 1992 to 2008. RESULTS: Infectious complications identified were dominated by urinary tract infections occurred in 12 patients. The clinical symptomatology is dominated by fever and decrease in health. In 4 patients the urinary infection was asymptomatic. Three patients had pneumonia; the diagnosis was suspected clinically and confirmed by the chest radiography. Three other patients developed sepsis. In one of them, the etiology was a peritonitis related to dialysis catheter. Two transplant showed a herpetic cheilitis and one patient developed a parvovirus infection that evolved well after two transfusions. In all cases, the outcome was favourable with no deaths or impact on graft function. CONCLUSION: After renal transplantation, the disturbance of inflammatory reactions explains the often latent or delayed infectious process making early diagnosis difficult. It is imperative to monitor infectious to minimize morbidity and mortality.

[Toxocariasis: under-estimated disease. Report of 9 cases]. / La toxocarose: une pathologie sous-estimée. A propos de 9 cas.

Toxocariasis or visceral larva migrans is a parasitosis due to the migration in the human organism of animal ascarid larvae. Its importance is under-estimated and the reported tunisian cases are rare. We report nine cases of toxocariasis, noted between January of 2000 to March of 2002, in the laboratory of parasitology-mycology in La Rabta Hospital. They were six children and three adults. The clinical forms are varied: ophthalmologic form (5 cases), general syndrome (1 case), oedema (1 case) and hypereosinophilia (2 cases), and confirmed serologically by the presence of anti Toxocara canis antibody using ELISA test. Visceral larva migrans should be kept in mind to avoid severe forms such as ophthalmologic forms.