[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

[Medical Management of Pott's disease: a review of 18 cases]

Vertebral tuberculosis is the most frequent osteo-articular location, surgical treatment is rarely indicate. The duration and the modalities of the medical treatment remain however subject to discussion. The purpose of this study is to propose a therapeutic attitude standardized for the pott's disease. The authors report a retrospective analysis of 18 cases of pott's disease. The mean age was 51 years. All the patients have a medical treatment, no surgical indications were carried. Magnetic resonnance imagery and biopsy under scanner allowed to avoid the surgical approach with aim diagnosis. The chemotherapy consisted of an attack phase associating four antituberculosis antibiotics of an average duration of 5 months, followed by a maintenance phase associating only two major antituberculosis antibiotics in daily grip and of an average duration of 11 months. Eight patients had a spinal cast immobilization. The mean follow up was 36 months. Clinical and anatomical evolution was favorable for all the patients. No neurological complication was noted. The antituberculosis medical treatment is always effective especially if diagnosis is premature and allows so to avoid appeal to the rather laborious surgery and results of which are not encouraging

[Treatmentofsteroid-esistantidiopathicnephrosiswithimmunosuppressivetherapy in 30 children]

The aim of this study is to evaluate the efficacity of immunosuppressive therapy in steroid - resistant idiopathic nephrotic syndrome. 30 children present to the renal biopsy a focal and segmental glomerulosclerosis [HSF 18 cases], minimal change disease [LGM: 9 cases] and diffuse mesangial proliferation [PMD: 3 cases]. 9 of 11 patients did not respond to cyclophosphamid. 28 patients received the cyclosporine - prednisone combinaison with a cyclosensibility in 23 patients [80 per cent]. The mycophenolate mofetil [MMF] was used in 7 patients, and 3 were in remission. In this study 7 patients [23 per cent] develop a chronic renal failure. the cyclophosphamid has not beneficial effects. The cyclosporine is an effective therapeutic agent. MMF has emerged as an important new therapeutic option for the treatment of SNCR and HSF

Insulin therapy in diabetes type 2: indications modalities and results

After several years of evolution, insulin therapy becomes necessary in type 2 diabetes mellitus. However there is still an ongoing controversy in the studies devoted to this subject. The objective of our retrospective study was to evaluate the indications, the modalities and the results of insulin treatment in 106 patients with type 2 diabetes. Insulin treatment was temporary in 20 patients [18.86%] and definitive in 86 patients [81.14%]. The therapeutic protocol used a combined treatment with oral hypoglycaemic agents in 11 cases [10.38%], a conventional treatment in 48 cases [45.28%] or an intensive therapy in 47 cases [44.34%]. The outcome could be evaluated in 86patients after 6 months and 1 year. At the first follow-up [6 months], 59 patients [68.6%] had a significant reduction in fasting glycemia [7.8 vs 15.5 mmol/l], post prandial glycemia [8.9 vs 25.8 mmol/l] and glycated haemoglobin [6.9% vs 10.2%]. 69 patients [80.24%] had a mean weight gain of 7 +/- 5 kg and 8 [7.5%] had hypoglycaemic episodes. In the total number of 59 patients who improved with the insulin treatment, 46 patients 77.96% were treated with the intensive insulin protocol. Insulin treatment in type 2 diabetic patients seems to have a limited risk of hypoglycaemia but causes an important weight gain

[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

Acute hematogenous osteomyelitis of the ischio pubic ramus. A case report

Pelvic osteomyelitis is rare. We report the case of a 10-year-old boy presented with a five-day history of pyrexia and left painful lameness. Passive range of hip was normal. Initial biological investigations revealed an inflammatory syndrome. Plain radiographs of the pelvis and ultrasound scan of the left hip joint were normal. Bone scintigraphy showed a tracer uptake in the left obturator ring. The blood cultures were positive for methicillin-sensitive staphylococcus aureus. The diagnosis of pelvic osteomyelitis was held. Magnetic Resonance Imaging [MRI] showed a left ischio-pubic ramus osteomyelitis with a subperiostal abscess decompressing into the surrounding tissues. This case history draw attention to the distinctive clinical and bacteriological features of this rare focus of acute hematogenous osteomyelitis and emphasize the MRI interest in the investigation of deep localizations. It may facilitate early recognition of pelvic osteomyelitis in child

[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

Insuffisance cardiaque chez le jeune enfant: a propos de 82 cas

The authors report 82 cases of Heart failure observed among children aged 0-3 years hospitalized in a paediatric hospital during a 5 years period. The infan represent 76% of cases. The symptomatology is often serious. The main etiologies were the congenital cardiopathies [56%] and the acquired cardiopathies [33.4%]. Rarely an arrythmia [2.4%] or others [4%]. The prognosis varied wth age and etiology: the rate of death is of 51% the treatment is usually symptomatic if possible etiologic