title: [Long-term follow-up of permanent neonatal diabetes in Tunisian infant].
abstract: 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.
authors.affiliation: Unité de néonatalogie, service de pédiatrie, hôpital universitaire Tahar Sfar, 5111 Mahdia, Tunisie. hechmi.benhamouda@rns.tn
authors.forename: H
authors.initials: H
authors.lastname: Ben Hamouda
authors.forename: F
authors.initials: F
authors.lastname: Chioukh
authors.forename: B
authors.initials: B
authors.lastname: Boussetta
authors.forename: B
authors.initials: B
authors.lastname: Mahjoub
authors.forename: A
authors.initials: A
authors.lastname: Ayadi
authors.forename: Y
authors.initials: Y
authors.lastname: Belkhir
authors.forename: R
authors.initials: R
authors.lastname: Boussoffara
authors.forename: H
authors.initials: H
authors.lastname: Soua
authors.forename: M T
authors.initials: MT
authors.lastname: Sfar
mesh_terms: D003920:Diabetes Mellitus / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D005260:Female; D005317:Fetal Growth Retardation / Q000276:immunology; D005500:Follow-Up Studies; D006684:HLA-DR Antigens / Q000276:immunology*; D059810:HLA-DR Serological Subtypes; D015804:HLA-DR4 Antigen / Q000276:immunology*; D006801:Humans; D006943:Hyperglycemia / Q000188:drug therapy / Q000276:immunology; D007004:Hypoglycemic Agents / Q000276:immunology / Q000627:therapeutic use; D007231:Infant, Newborn; D019102:Infant, Very Low Birth Weight / Q000276:immunology; D007328:Insulin / Q000276:immunology / Q000627:therapeutic use; D007330:Insulin Antibodies / Q000097:blood*; D007515:Islets of Langerhans / Q000276:immunology
chemical_list: D006684:HLA-DR Antigens; D059810:HLA-DR Serological Subtypes; D015804:HLA-DR4 Antigen; C041559:HLA-DR8 antigen; D007004:Hypoglycemic Agents; D007328:Insulin; D007330:Insulin Antibodies