European journal of pediatrics
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A child with the tentative diagnosis of Alagille syndrome is reported. Additional renal abnormalities are unilateral kidney agenesis and a kidney with subcortical cysts with decreased function. At the age of 5 years, insulin-dependent diabetes mellitus developed, with the pancreas being atrophic and negative pancreatic islet cell antibodies. ⋯ This observation extends the picture of Alagille syndrome and suggests an overlap with renal-hepatic-pancreatic dysplasia (Ivemark syndrome).
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Five hundred and twenty term singleton infants delivered by Caesarean section were categorised into six groups according to type of Caesarean section (elective or emergency), type of anaesthesia (epidural or general) and presence of fetal distress. Infants delivered under general anaesthesia had a significantly higher incidence of respiratory depression at birth with Apgar scores < 7 at both 1 and 5 min (P < 0.00001), greater need for active resuscitation (intermittent positive pressure ventilation or bag and mask ventilation) (P < 0.000001) and a higher rate of neonatal unit admission (P < 0.00001). Caesarean sections for fetal distress were associated with a significantly higher incidence of intermittent positive pressure ventilation, but not bag and mask ventilation, for both the general anaesthesia and epidural groups (P < 0.003 and P < 0.02 respectively), indicating severe respiratory depression in some cases. Under epidural anaesthesia, both elective section and emergency section without fetal distress were low risk deliveries. By excluding the non-cephalic presentation cases in these two groups, the incidence of infants requiring active resuscitation was equivalent to the incidence quoted for spontaneous normal delivery. ⋯ Attendance by a paediatrician is not routinely required at epidural Caesarean section when the infant is cephalic and when there is no fetal distress. Every effort should be made to ensure that epidural anaesthesia is provided in preference to general anaesthesia.
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Non-ketotic hyperglycinaemia (NKH) is a severe seizure disorder associated with high glycine levels. Glycine is a major inhibitory neurotransmitter in the CNS, but has also modulating effects at one of the glutamate receptors, the N-methyl-D-aspartate-(NMDA) receptor. Based on this knowledge we treated a female newborn suffering from severe NKH with the NMDA receptor blocker ketamine in association with strychnine and magnesium supplementation. This treatment led to cessation of seizures, reappearance of swallowing and sucking and improved the neurological status. Some pharmacokinetic data of strychnine and ketamine in the infant are given. ⋯ Ketamine in combination with strychnine may be beneficial in non-ketotic hyperglycinaemia.
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Case Reports
Two cases of pulmonary tuberculosis presenting with unilateral pulmonary hyperinflation in infancy.
Two infants with recurrent obstructive symptoms attracted attention because of massive radiologically detected unilateral pulmonary hyperinflation. Further diagnostic procedures including bronchoscopy, revealed a pulmonary tuberculosis with lymph nodes encroaching on the bronchi. Steady improvement of clinical symptoms and hyperinflation was noted under combined antituberculotic therapy including systemic steroids. ⋯ Our two cases demonstrate that the differential diagnosis of unilateral pulmonary hyperinflation and wheezing in infancy should consider valvular stenosis by encroaching lymph node due to pulmonary tuberculosis.