[Long-term follow-up of permanent neonatal diabetes in Tunisian infant]. / Evolution à long terme d'un diabète néonatal permanent chez un enfant tunisien.

Neonatal diabetes mellitus is a rare entity defined as hyperglycaemia occurring within the first 3 months of life that lasts for at least 2 weeks and requiring insulin therapy for unforeseeable duration. We report the case of a full-term female infant with permanent neonatal diabetes mellitus, stemming from consanguineous parents, born with severe intra-uterine growth retardation and birth weight of 1400 g. The patient presented on the 15th day of life a severe dehydration with a fever and ponderal loss of 14 %. The biology showed hyperglycaemia to 15 mmol/L, moderate metabolic acidosis, glucosuria and ketonuria. The diagnosis of neonatal diabetes mellitus was reserved, justifying its stake under insulin. Etiologic investigation showed a type HLA-DR4/DR8; anti-insulin antibodies were weakly positive, Langerhans islet cell and anti-GAD antibodies were negative. Abdominal magnetic resonance imaging scans, karyotype, molecular biology and chromatography of amino and organic acids did not show any abnormalities. During the first 2 years of age, the patient presented a big instability of glycaemia having required several hospitalizations. After 12 years of age, the patient is still under insulin with a satisfactory glycaemia balance and her growth is normal. Besides, she presents a microcephaly with a spastic walking. The search of neonatal diabetes mellitus must be systematic in front of any fetal hypotrophy allowing a premature coverage and a good prognosis.

[Cerebral hydatid cysts in children: 4 cases]. / Kyste hydatique cérébral de l'enfant: à propos de quatre observations.

The cerebral localization of the hydatid disease is rare, under 2%, and it primarily affects children. We report 4 cases of children presenting with a cerebral hydatid cyst (2 boys and 2 girls, mean age 9 years). Clinical symptoms were very progressive, the disease was frequently diagnosed several months (1-12 months) after onset of symptoms most often headaches and vomiting. One patient presented with a right tonic-clonic seizure 3 days before hospitalization. The diagnostic was confirmed in all cases by Cerebral CT scan. All the patients were screened for other localizations. One patient also presented with pulmonary and hepatic hydatid cysts. Hydatid blood tests were positive in only one case. The treatment was surgical for all the patients (using hydropulsion) without complications in 2 cases. One child presented with meningitis, and the other child with signs of secondary ICHT related to residual mass which required its puncture.

[Brachyolmia at autosomal recessive transmission]. / Brachyolmie à transmission autosomique récessive.

BACKGROUND: Brachyolmia is a form of spondylodysplasia; sufferers have a short stature limited to the trunk that is recessively inherited. There may be other minor abnormalities in some cases. CASE REPORTs. Four brothers, born to consanguineous normal parents, developed short stature and scoliosis that were only identified after the age of 5 years (from 8 to 14 years). The three oldest patients had facial anomalies with flattened mid-face and enlarged lips. X-rays showed scoliosis, universal platyspondyly, irregular iliac crests and short, enlarged femoral necks. CONCLUSION: Evidence of abnormalities in other areas than the spine confirms the heterogeneity for the disease.

[Histiocytosis X and sclerosing cholangitis]. / Histiocytose X et cholangite sclérosante.

The case of a seven-year-old with multifocal (type II) Langheran's cell histiocytosis since the age of two years is reported. Despite therapy, biliary cirrhosis with portal hypertension developed gradually. Histologic studies of liver biopsy specimens, computed tomography, and transhepatic cholangiography disclosed dilatation of the bile ducts, suggesting sclerosing cholangitis, a specific complication of Langherans' cell histiocytosis which precipitates the development of biliary cirrhosis.

[Abandonment-adoption: experience at the neonatology service of the University Hospital Center in Sousse, Tunisia]. / Abandon-adoption: expérience du service de néonatalogie du CHU de Sousse, Tunisie.

We studied the outcome of 178 newborns abandoned in the maternity hospital of Sousse, Tunisia during the past eight years. The natural mothers are frequently very young: 40% less than 20 years of age, 10% less than 16 years. In most cases (65%), there is no medical supervision of the pregnancy. There is a high rate of pathological deliveries (11%) and a small for date infants. As a consequence of a long hospitalization in neonatal unit, these infants have important morbidity and mortality. The minimum annual adoption rate is 77%. Most adoptive parents are middle class Tunisians and the large majority of adoptions are successful.