[Aglossia adactylia syndrome with anterior maxillo mandibular fusion: a case report]

The hypoglossia-hypodactylia syndrome [Hanhart syndrome] is a congenital birth defect. The major features of this syndrome are a reduction in tongue size, micrognathia, limb anomalies and many occasional other anomalies. The neurosensorial development is preserved: We report a particular case with anterior maxillo-mandibular fusion. The patient, a male neonate, was the third child of a 42 year old woman. There was parental consanguinity but no similar cases in the family. The physical examination at birth revealed an eutrophic newborn with facial dysmorphia, impossibility of opening the mouth, aplasia of the fingers on the level of the metacarpo-phalangeal articulations and apodia. Computed tomography scan noted anterior maxillo mandibular fusion, glossopalatine ankylosis and cleft palate. The chromosomic chart, cardiac echography and transfontanellar ultrasound scan were normal. The Hanhart syndrome is a rare malformatif syndrome, grave because of the limbs reductionnal anomalies and feeding difficulties. The etiopathogeny is unknown; drug-taking during the pregnancy, genetic and vascular theories has been implicated

[Fifth day fits about a cases report]

Fifth day fits are rare, their incidence is very variable because of a difficult diagnosis. They were first decrived by Dehan and Navelet using the following criteria: occurrence of electro clinical seizures on the fifth day of life in a term newborn, with no pathological event. Inter-ictal tracing showed 'sharp alternant theta', aetiological investigations are negative and long-term outcome is favourable. Causes of fifth day fits are unknown. Responsibility of disturbances of brain maturation are advanced. We report a case complying with the preceded criteria. This case is a female term newborn who presented repetitive clonic seizure appearing on the fifth day of life and resisted to phenobarbital therapy. The special tracing of encephalography showing "sharp alternant theta" is noted. The aetiological research is negative and the outcome is favourable until the age of 18 months

[Nosocomial infections in a neonatal unit epidemiology and risk factors]

Nosocomial infections [NI] are a major problem for neonates especially premature and low birth weight infants The objective of this study was to identify the risk factors and the bacterial epidemiology for nosocomial infection. This was a retrospective study of confirmed nosocomial infections, recorded from January 2003 to December 2005 at the neonatal unit of Charles Nicolle Hospital-Tunis. During the study period, 37 NI were recorded. The diagnosis was performed at a mean age of 7 days. Intra-uterine growth retardation was recorded in 54%of all cases, 70%of all infected infants were prematures and 75%were low birth weight. An antibiotic treatment before NI was recorded in 70%. Peripheral intravenous line placement, administration of intravenous medications, fluids and umbilical vein catheterization were significantly higher at the infected group [p<10[-3]] The pathogens most frequently isolated were klebsiella pneumoniae [73%] coagulase-negative staphylococci [13%]. The mortality rate was 27%[10/37], Nosocomial infections affect specially premature and low birth weight infants and are associated with a high mortality. klebsiella pneumoniae was the most frequently isolated pathogen

[Fetal/Neonatal alloimmune thrombocytopenia [FNAIT], resulting from maternal alloimmunisation-About one case]

Fetal/Neonatal alloimmune thrombocytopenia [FNAIT], resulting from maternal alloimmunisation against fetal platelet antigens, is a serious affection. The frequency has been estimated to be 1 out of 800-1000 live births. We report a new observation of alloimmune neonatal thrombocytopeni due to anti HPA-5b [Bra] antibodies. Case report: A male full term neonate presented at birth with a thrombocytopenia unexplained by usual causes of neonatal thrombocytopenia. Alloimmune neonatal thrombocytopenia was diagnosed by the determination of platelets and neonatal patelets antigens phenotypes HPA-5b [antiBra] antibodies in maternal sera, absent at delivery, were identified four months neonatal later. A favourable evolution was obtained after maternal washed platelet transfusion. Alloimmune thrombocytopenia is a seious affection, which exposs to intracranial haemorrhage. Difficulties in detemining the diagnosis should not delay therapy, depending on exclusion of other causes