[Idiopathic steroid-resistant nephrotic syndrome in child : study of 20 cases]. / Syndrome néphrotique idiopathique corticorésistant de l'enfant : Etude de 20 cas.

BACKGROUND: Idiopathic steroid-resistant nephrotic syndrome (ISRNS) is rare and represents a significant therapeutic dilemma for paediatricians and paediatric nephrologists. AIM: To analyze characteristics of the ISRNS in the child. METHODS: Retrospective study of 20 cases of ISRNS enrolled in paediatric department of nephrology in Sahloul hospital (Tunisia) between June 1993 and December 2007 (14 years period). RESULTS: There were eight girls and 12 boys (mean age: 5.8± 3.7 years) originating from the center or the south of Tunisia. Eight of them had a minimal-change disease (MCD), 11 a focal and segmental glomerulosclerosis (FSGS) and one a mesangioproliferative glomerulonephritis (MePGN). In this group, no family form could be identified. All patients were treated by cyclosporine associated with low dose of steroid. We noted a complete remission (CR) in nine cases, partial remission (PR) in three cases and no response to cyclosporine in eight cases. Among patients with CR, six presented MCD and three a FSGS. In this group, we observed relapse of nephrotic syndrome in six cases. End stage renal disease (ESRD) was noted in 10 patients of which five not responded to cyclosporine, two initially having presented a RC and three having since the beginning a PR. Among them, two only could be grafted; one relapses on transplant was observed with a single patient initially presenting a secondarily transformed MePGN in FSGS. CONCLUSION: Our study confirms the clinical, histological and evolutive heterogeneity of idiopathic steroid-resistant nephrotic syndrome. Although there is any therapeutic consensus in this domain, cyclosporine remains indicated in first intention in sporadic forms of ISRNS. On the other hand, renal transplantation constitutes the only therapeutic alternate in genetic forms that constantly evolve at ESRD.

Rotavirus strain diversity in the centre coast of Tunisia from 2000 through 2003.

An epidemiological survey investigating rotavirus infection in children was undertaken in the coastal region of Tunisia from January 2000 through September 2003. A total of 309 fecal specimens were screened by enzyme-linked immunosorbent assay and latex agglutination assay for the presence of group A rotavirus antigen. The detection rate was 26.2%. Rotavirus outbreaks showed a temperature-dependant pattern (P = .026) but no significant association with rainfall. Rotavirus strains isolated were analyzed by RNA polyacrylamide gel electrophoresis and were characterized antigenically by monoclonal antibodies to the VP6 subgroup. Eight RNA electropherotypes were identified, with 3 long and 5 short different RNA profiles. Among VP6 typeable strains, all isolates with a long electrophoretic pattern carried the subgroup II specificity, whereas those with a short profile belonged to subgroup I. In total, 48 rotavirus-positive samples were analyzed for G and P typing by reverse-transcription polymerase chain reaction. A total of 8 different G and P combinations were found: G1P[8] (35.7%), G1P[6] (21.4%), G2P[4] (4.8%), G3P[4] (4.8%), G4P[6] (4.8%), G8P[8] (4.8%), G3P[8] (2.3%), and G4P[8] (2.3%). Mixed infections were detected in 19.1% of stool samples. The emergence in Tunisia of unconventional types, such as G8VP7 specificity, highlights the need for a continual survey of the uncommon strains in North Africa.

[Complete primary distal renal tubular acidosis in children: 11 cases]. / L'acidose tubulaire distale complete et primitive de l'enfant à propos de 11 observations.

AIM: Analyze the clinical and evolutive particularities of complete primary distal renal tubular acidosis in children, METHODS: We studied retrospectively 11 cases enrolled in the pediatrics department of Sousse during 10 years period (1993-2002). RESULTS: It is about 9 boys and 2 girls (sex-ratio = 45) aged 3 month to 5 years (mean age: 18 months). Diagnosis was suspected on clinical and biological data of presumption and confirmed by acidification test. Radiological investigation objectified a nephrocalcinosis in eight patients and urinary lithiasis in two other cases. Auditive exploration showed sensorineural deafness in three patients. The illness appears sporadic in two cases and autosomal recessive in nine other cases. After alkali treatment (sodium bicarbonate), evolution was globally favorable.

[Graves's disease in children and adolescent: study of seven cases]. / La maladie de Basedow chez l'enfant et l'adolescent: etude de sept cas.

BACKGROUND: In spite of its rarity in the paediatric age, Graves' disease constitutes the principal aetiology of hyperthyroidism in child. AIM: Our goal is to analyze the clinical and evolutive particularities of Graves's disease in children. METHODS: We studied retrospectively seven cases of Graves' disease in children enrolled in the pediatrics department of Sousse during ten years period (1993-2002). RESULTS: There were six girls and one boy (sex - ratio = 0.16) aged 4.5 to 16 years (mean age: nine years and one month). The diagnosis has been established clinically on the presence of classic symptoms of the illness associated to the biological and radiological findings. As part of research of possible associations with this illness, we observed solely in a case, in addition of Graves's disease, the coexistence of Down syndrome and coeliac disease, rarely described. Among the HLA antigens predisposing the Graves's disease, we only found HLA B8 antigen in a patient. The evolution under antithyroid drug treatment (ATD) has been marked by fast disappearance of functional signs in all patients. However, biological and clinical euthyroidism was more difficult to achieve. The treatment has been stopped in only one patient after 40 months period. CONCLUSION: Graves' disease is usually easy to recognize but difficult to treat. Radical treatments (thyroidectomy or radioactive iodine therapy) are indicated in second intention after having tempted ATD beforehand.

[Lupus nephritis in childhood: clinical and evolutive study of 14 cases]. / La nephropathie lupique de l'enfant: étude clinique et évolutive de 14 cas.

BACKGROUND: Renal involvement is one of the most severe and frequent manifestations of the systemic lupus erythematosus (SLE). Aim : In this study, we analyzed clinical and evolutive particularities of 14 paediatric cases of lupus nephritis (LN). METHODS: It's a retrospective study in 14 children with lupus nephritis followed-up in the paediatrics department of Sousse and Mahdia between 1983 and 2004. RESULTS: There were 12 girls and two boys (sex-ratio = 0.16) aged four to 14 years (mean age =10 years). At the first presentation, we noted proteinuria in all patients with nephrotic syndrome in six cases, hypertension with variable severity in five cases, hematuria in six cases and a variable severity of renal insufficiency in six cases. Histological examination of kidney performed in 10 patients with severe nephropathy, revealed class IV glomerulonephritis in four cases, class V in two cases and class III in four cases. Thirteen patients were treated by corticosteroids associated with immunosuppressive agent in six cases. One patient had not received any treatment. Five patients were died of the continuations of SLE complications or immunosuppressive therapy. For the other patients, one is in clinical and biological remission since six years, four are lost of view, one is in end stage renal failure, two presented relapsing evolution and one presents refractory form of LN. CONCLUSION: Lupus nephritis is severe in our patients with predominance of class III and IV. New therapeutic strategies permitted to improve the renal survival but at the cost of an important iatrogenic morbidity.

ANCA-associated diffuse alveolar hemorrhage due to benzylthiouracil.

Benzylthiouracil has been recently observed to be associated with antineutrophil cytoplasmic antibody-positive vasculitis, resulting in crescentic glomerulonephritis. We report an 8-year-old girl treated with benzylthiouracil for Graves's disease who developed an ANCA-positive vasculitis with pulmonary hemorrhage. She responded to corticosteroids and discontinuation of benzylthiouracil. This represents the first pediatric case of benzylthiouracil-induced diffuse alveolar hemorrhage.

[Mycobacterial infections in children in Central Tunisia. A study of 31 cases including 7 disseminated BCG-osis]. / Infections mycobacteriennes chez l'enfant dans le centre tunisien. Etude de 31 cas dont 7 cas de becegite disseminée.

UNLABELLED: In order to analyze the current epidemiological pattern of mycobacterial infection in children in Central Tunisia, we studied retrospectively the clinical feature of 31 children with mycobacterial infection enrolled in the pediatrics department of Sousse during eight years period (1994-2001). Twenty three boys and eight girls aged two months to 13 years (mean age: 4 years and 8 months) were investigated. Among them, 24 patients suffered of tuberculosis (TBC) and 7 of disseminated BCG-osis. Pleuropulmonary TBC was observed in 12 patients either isolated (7 cases) or in association with at least another localization (5cases). 17 patients had extrapulmonary TBC with variable localisation. The 7 patients with disseminated BCG-osis had an underlying primary immunodeficiency of the cell-mediated immune response. CONCLUSION: The current epidemiology of mycobacterial infections in children in our region indicates a high frequency of severe adverse effects of BCG vaccination occurring in genetically immunodeficient children.

[Tracking of cardiovascular risk factors among school children: a four-year population surveillance in Susa, Tunisia]. / Phenomene de "tracking" des facteurs de risque cardiovasculaires chez l'enfant en milieu scolaire etude de cohorte sur 4 ans a Sousse en Tunisie.

In order to confirm the phenomenon of tracking of cardiovascular risk factors among school children, we undertook in 2003, a prospective survey of a population of 789 pupils aged 13 to 15 years who had participated in a first investigation on cardiovascular risk factors in 1999. We were able to follow and study 453 pupils (57.4% of the initial population). Prevalences of hypertension and obesity were respectively 11.3% and 6.1% without significant difference between sexes. On the other hand, hypercholesterolemia was significantly more elevated among girls than boys (16.1% versus 9.3%, p = 0.039). Children classified initially as hypertensive, obese or having a hypercholesterolemia were identified 4 years later at a subsequent exam as hypertensive, obese or having a hypercholesterolemia in respectively 25%, 48.9% and 59%. The stability of cardiovascular risk factors among children imposes an early screening for a better management and a policy of cardiovascular health promotion since childhood based essentially on prevention of risk factors in order to reduce the cardiovascular disease burden in adulthood.

[Clinical features and course of Kawasaki disease in central Tunisia: a study about 14 cases collected over a period of three years (2000-2002)]. / La maladie de Kawasaki au centre Tunisien: aspects cliniques et évolutifs de 14 cas colligés en trois ans (2000-2002).

OBJECTIVE: To analyze the clinical features and course of Kawasaki disease in central Tunisia. We studied retrospectively 14 cases of children with Kawasaki disease collected in tunisian center during three years (2000-2002). The study is about 11 boys and 3 girls (sex - ratio: 3.6/1) aged from 6 months to 8 years (mean age : 4 years). Twelve patients had at least 5 diagnostic criteria of the illness, the two others had an incomplete form. We noted cardiac complications in seven patients treated belatedly, beyond 10 days of progression, because of atypical clinical presentations. All patients had all a middle caliber coronary aneurysm that was complicated by a thrombus in three cases, associated with pericarditis and minimal mitral insufficiency in a case and with a cardiac rhythm disturbance (block of branch) in another case. Besides the cardiac complications, several other visceral manifestation could be noted: joint symptoms in five cases, GI tract symptomes in three cases, neuro-meningeal in two cases and urinary trad symptomes in two other cases. Specific treatment (aspirin with antiinflammatory dose and intravenous immune globulin (IVIG)) has been instituted in all patients. The course was favorable for 12 patients with fast regression of clinical manifestation and progressive normalisation of biologic values. Two patients did not respond to the initial IVIG treatment, and had to recense received an additional course of IGIV but without clinical nor biological improvement. These two patients were treated with corticosteroids. Cardiac lesions disappeared completely in all patients even for those with thrombosis and in patients with IVIG-resistant Kawasaki disease. Only one patient had kept neurologic sequellae: aphasia, bevavioral problemes and partial epilepsy. CONCLUSIONS: Kawasaki disease is not rare in our region. Incomplete or atypical presentations are frequent and are a source of diagnostic delay. Coronary aneurysm due to the delay of treatment often regresses even in patients with IVIG-resistant Kawasaki disease.

[Accidental caustic ingestion in Tunisian child. Study of 330 cases]. / Les ingestions accidentelles de caustiques chez l'enfant Tunisien. Etude de 330 cas.

We studied retrospectively 330 cases of caustic product ingestion at the child collected in the pediatric department of Sousse (CHU Sahloul and CHU Farhat Hached) during eight years (1993-2000). It is about 194 boys and 136 girls (sex-ratio to 1.42/1) aged of 4 months at 14 years (middle age at 3 years and 5 months). Concerned products are dominated by the water of bleach (55.7%) dilute essentially (49%), caustic soda (27.9%), diluent of painting (8.5%), potash (2.7%) and the acidic products (2.2%). Endoscopy showed oeso-gastric lesions in 89% of cases: oesophagitis stage I (73.5%), stage IIa (11%), stage IIb (4%), stage III (11.2%) and an inflammatory sténose case of straightaway. The associated gastric lesions have been found in 15.1% of the cases. The recovery was the rule for all patient presenting a benign oesophagitis. The 46 cases of severe oesophogitis have been treated according two protocols: --A group (n=1 ), treated by parenteral food with treatment by antibiotics (1993-1994). --B group (n=35), treated by high dose of corticosteroids (Méthyl-prednisolone) aiming to warn esophageal sténosis (1995-2000). Three patients of the A group and five of the B group developed stenosis with statistically meaningful difference (p=0.44). Among these eight patients, six required oesophagoplasty and two had a good evolution after esophageal dilation.