ABSTRACT
AIM: Analyze epidemiological and evolutive profile of paediatric celiac disease in the region of Sousse. METHODS: We studied retrospectively 80 cases enrolled in the paediatrics' department of Sousse between 1993 and 2003. RESULTS: There were 44 girls and 36 boys (sex-ratio=0.81). The middle age of gluten introduction was 9 months, with extremes going from 1 to 24 months. Free interval between the introduction of gluten and the beginning of the symptoms was meaningfully more elevated in patients who received gluten after the age of 6 months (p=0.036). At the time of the diagnosis, the middle age of our patients was six years with extremes going from nine months to 17 years. The classic form of celiac disease with chronic diarrhoea has been observed in 85% of the cases. The morbid associations with celiac disease were dominated by the diabetes type 1 noted in 5% of the cases. Antigliadin antibodies, practiced in first intension, were positive in 98.6%. At histology, villous atrophy was sub-total to total in 96.25% of the cases and partial in 3.75% of the cases. Follow-up was on average at 18 months. Adhesion to the gluten-free diet (GFD) was judged satisfactory in 81.45% of the cases on average. Catch up growth, although remarkable, was not very satisfactory. Indeed, several patients adhering little or not to the GFD kept, at one year of evolution, a ponderal and stature delay superior to 2SD.
Subject(s)
Celiac Disease/diet therapy , Celiac Disease/epidemiology , Adolescent , Antibodies/analysis , Celiac Disease/diagnosis , Child , Child, Preschool , Diet, Gluten-Free , Female , Gliadin/immunology , Humans , Infant , Male , Retrospective Studies , Tunisia/epidemiologyABSTRACT
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.
Subject(s)
Antibodies, Antineutrophil Cytoplasmic/immunology , Hemorrhage/chemically induced , Lung Diseases/chemically induced , Thiouracil/analogs & derivatives , Vasculitis/chemically induced , Child , Female , Graves Disease/drug therapy , Hemorrhage/immunology , Humans , Lung Diseases/immunology , Thiouracil/adverse effects , Thiouracil/therapeutic use , Vasculitis/immunologyABSTRACT
PURPOSE: To evaluate the incidence of newly presenting seizures in children in the area of Sousse, Tunisia. METHODS: From June 1, 1998, to May 31, 1999, all children aged 1 month to 15 years with first provoked and unprovoked seizures were included. Children with febrile seizures were excluded. All suspected cases were systematically referred to the Department of Functional Explorations of the Nervous System where a detailed questionnaire was filled out by a neurologist. All the patients underwent an EEG. Only 12 patients had a computed tomography (CT) scan. RESULTS: A total of 175 patients were included. Eighteen (10.3%) patients had acute symptomatic seizures, and 157 patients had unprovoked seizures. The incidence rate of first unprovoked seizures was 102.1/100,000. In this latter group, some epileptic syndromes were individualized on strict electroclinical criteria. CONCLUSIONS: However, nearly 75% of the cases remained cryptogenic, one of the major reasons that no predominant risk factor was identified in this population.
Subject(s)
Developing Countries , Epilepsy/epidemiology , Urban Population/statistics & numerical data , Acute Disease , Adolescent , Child , Child, Preschool , Cohort Studies , Cross-Sectional Studies , Electroencephalography , Epilepsy/classification , Epilepsy/diagnosis , Epilepsy/etiology , Female , Humans , Incidence , Infant , Male , Risk Factors , Syndrome , Tunisia/epidemiologyABSTRACT
We report two siblings with purine nucleoside phosphorylase deficiency revealed by isolated spastic paraplegia, whereas symptoms of immune deficiency did not become apparent until 3 years of age. As the concurrence of immunodeficiency and neurologic problems strongly suggests the diagnosis of purine nucleoside phosphorylase deficiency, special attention should be paid to counts of lymphocytes in any infant with spastic paraplegia.
Subject(s)
Purine-Nucleoside Phosphorylase/deficiency , Spastic Paraplegia, Hereditary/etiology , Cerebral Cortex/pathology , Child, Preschool , Female , Humans , Lymphocyte Count , Magnetic Resonance Imaging , Purine-Nucleoside Phosphorylase/genetics , Siblings , Spastic Paraplegia, Hereditary/immunology , Spastic Paraplegia, Hereditary/pathologyABSTRACT
Children with Crigler-Najjar syndrome type I are at increased risk for neurologic deficits. Cerebellar symptoms are not prominent and appear in adolescent or adult patients with this syndrome. We report a 2-year-old female with Crigler-Najjar syndrome type I who presented severe cerebellar symptoms revealing bilirubin encephalopathy. The patient improved slowly with the duration of phototherapy. Cerebellar symptoms can be the initial manifestation of kernicterus in children with Crigler-Najjar syndrome type I.
