[Thromboembolic complications of childhood nephrotic syndrome]

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. The risk of extension of thrombosis and pulmonary embolism is real. To consider the major thrombotic events associated with childhood nephrotic syndrome and to establish an appropriate preventive approach based on objective clinical and laboratory parameters. This is a retrospective study of all cases of patients suffering from idiopathic nephrotic syndrome, during a period of 20 years, starting from January 1990 to December 2009. We selected six patients with vascular thrombosis. The diagnosis was confirmed by appropriate radiological investigation. Six cases of vascular thrombosis were identified among 260 cases of nephrotic syndrome collected during the period study. Patients are divided into five boys and one girl. The mean age was 13.3 years. The localisation of thrombosis is venous in all cases. One patient presented a massively fatal pulmonary embolism. Sinovenous thrombosis has been objectified in 3 patients who were all treated with a good therapeutic response. Vascular thrombosis remains one of the most serious complications of nephrotic syndrome in children. Early diagnosis and precocious anticoagulation are essential for preventing the extension of thrombosis

[Gitelman syndrome revealed by myoclonus pediatric case report]

Gitelman's syndrome is a salt-losing tubulopathy characterized by hypokalemic alkalosis with hypomagnesemia and hypocalciuria. Patients are often asymptomatic, except for short periods of weakness, tetany or abdominal pain. Myoclonus are rarely reported and misleading with juvenile myoclonic epilepsy. We report a case of 10-year-old girl who had frequent myoclonia occur early in the morning. The brain magnetic resonance image was normal. The electroencephalogram showed an epileptiform discharges. The diagnosis of juvenile myoclonic epilepsy was chosen and patient was treated by valproate. Six month later, she was showed continuous nonconvulsive status epilepticus activity, which could not be suppressed with intravenous diazepam. The clinical and laboratory findings were in accordance with Gitelman syndrome. He has been followed up for 6 months and has suffered no myoclonia under magnesium, potassium and thiazidic

[Acute tubulointerstitial nephritis: about two pediatric cases]

We report two pediatric cases of acute tubulointerstitial nephritis [ATIN]. The first case is a 13 year-old boy, with one month history of vomiting and fever treated with Bromure de butylhyoscine [Buscopan] and Sulpiride. He developed oliguric acute renal failure. ATIN was diagnosed by renal biopsy. He needed dialysis for 3 weeks and was treated successfully with steroids for 8 months. The second case is a 7 year-old boy, treated with Ceftriaxone for febrile diarrhaoe. Laboratory studies showed an eosinophilia and eosinophiluria. Renal histopathology revealed features of drug induced ATIN. The patient was treated with steroids and didn't require dialysis

[AA renal amyloidosis in child: a report of 5 cases]

AA amyloidosis is a rare but serious complication of chronic inflammatory diseases and chronic infections. The kidney is the most frequently affected organ in AA amyloidosis. In order to analyse the clinical and histologic characteristics of AA amyloidosis in our pediatric population, to describe their aetiology and to examine clinical out-come. Retrospective study of 5 cases of AA amyloidosis diagnosed from 1997 to 2006 in our department. 5 cases, 3 girls and 2 boys, with median age at diagnosis of 10 years and 9 meonths. The AA amyloidosis aetiologies were: 3 cases with familial Mediterranean fever and 2 with juvenile chronic arthritis. In 2 patients, diagnosis was done through renal biopsy, in 2 patients by rectal biopsy and in one patient by salivary biopsy. All patients progressed to end-stage renal failure 3 years follow up diagnosis. AA renal amyloidosis has a low incidence in our children population but it remains dramatic complication. Rheumatological disorders and familial Mediterranean fever are the principal aetiology. The prognosis tends to be better with hemodiyalisis

Nephrocalcinosis in neonates: report of 5 cases

The nephrocalcinosis is a pathologic entity rarely revealed to the neonatal period. The etiologies are dominated by hypercalciuria of prematurity whose origin is mostly iatrogenic. In full-term newborn, it refers primarily to the early revelation tubulopathy [distal tubular acidosis, Bartter syndrome] and primary hyperoxaluria, pathology common in the North African population. The prognosis depends on the extension of calcium deposits, the etiology and therapeutic options. In the pediatric nephrology department of the Charles Nicolle hopital, and during a period of 7 years from 2002 to 2008, we supported for nephrocalcinosis, 5 full-term newborns who fall into 3 boys and 2 girls. The average age of discovery is 19 days with extremes of 15 to 25 days. Consanguinity was present in 4 cases. The circumstances of discovery are: Acute dehydration with underweight in 2 patients. A urinary tract infection in one patient. NeonataI convulsions secondary to hypomagnesaemia. A chance discovery in the exploration of a symptomatology evocative of a gastrosophageal ref lux disease in a patient. Explorations turned up the diagnosis of distal tubular acidosis in 2 patients, a primary hyperoxaluria in 1 patient, idiopathic hypercalciuria in a patient and a family hypomagnesaemia with hypercalciuria and nephrocalcinosis in another patient

Familial hypomagnesemia with hypercalciuria and nephrocalcinosis: report of two cases

Familial hypomagnesemia with hypercalciuria and nephrocalcinosis is a rare autosomal recessive tubular disorder caused by CLDN-16 mutations. CLND-1 6 gene encodes the renal tight junction protein claudin 16 [formerly paracelline-1]. This is an unusual cause of chronic renal failure. Case 1: a 9-month-old girl was hospitalized for urinary tract infection. The systematic renal ultrasound scanning showed a bilateral medullary nephrocalcinosis. The diagnosis hypomagnesemia with hypercalciuria and nephrocalcinosis has been established after a metabolic investigation. The follow up was marked by the deterioration of renal function. Case n°2: A boy with nephrocalcinosis was referred for investigation at the age of 15 months. in his history, he had seizures at the age of 5 days. The family inquiry revealed the same disease in uncle and maternal grandfather. The age of revelation of this disease is relatively early in our patients compared with those reported in the literature. The nephrocalcinosis and deterioration of renal function seems inevitable despite substitution treatment

[Acute ethmoiditis in children. About five cases]

The acute ethmoiditis in children is a severe bacterial infection which may spread to the orbital or endocranial spaces. The authors report 5 cases, 4 cases with periorbital cellulitis, and a case revealed by fever and convulsions. The CT scam confirm the diagnosis. Two infants have an alone ehtmoiditis, three children have an ethmoiditis associated with paranasal sinusitis, and complicated with an intracranial infection in a case. Bacteria were isolated from only one child [staphylococcus]. The evolution were favorable in all cases, with antimicrobial therapy. The authors insist on the CT imaging in emergency, and the early and effective antimicrobial therapy

[Galloway -Mowat syndrome. about two new cases]

Galloway - Mowat syndrome is a rare autosomal recessive disorder characterized by early onset nephrotic syndrome and central nervous system anomalies. We report two boys, born to consanguineous parents, who presented respectively at 32 months and 9 years of age a steroid-resistant nephrotic syndrome. The kidney biopsy showed, in the 2 cases, a focal and segmental glomerulosclerosis. At presentation they have a microcephaly, a mental retardation. A brain magnetic resonance imaging scan showed cortical atrophy, mega citerne, and white matter anomality in a case. One boy died at 3,5 years of age from end-stage renal failure, the other had 12 year old and on chronic hemodialysis. The particularity of the two cases is the nephrotic syndrome onset after 2 years of age

[Circulating anticoagulant revealing a systemic lupus erythematosus: about an observation]

In various circumstances, lupus anticoagulants [LA] are found in plasma. 40% of patients with systemic lupus erythematosus [SLE] have LA positive plasmas. These latter's are responsible for thrombosis in 10% of cases. This situation is observed in antiphospholipids antibodies syndrome, and it is complicated by thromboembolia. In other situations, LA can be found after viral infections. However, they are not accompaigned by thrombosis or haemorrhage, and they are discovered accidentally in most cases before an operation by abnormal coagulation tests. The authors report the case of an eleven year-old girl admitted for exploration of isolated adenopathy. The coagulation tests done before anesthesia showed the presence of LA leading to the diagnosis of SLE

[Bardet - biedl syndrome - a rare cause of end - stage renal failure]

Bardet - Biedl syndrome is an autosomal recessive condition caracterised by retinitis pigmentosa, polydactylye, obesity, mental retardation, hypogonadism, an renal dysfonction. We report an observation of 14 year old boy wich present an end - stage renal disease