Prevalence of fetal alcohol syndrome in the Top End of the Northern Territory.

OBJECTIVE: To establish the prevalence of fetal alcohol syndrome (FAS) in the Top End of the Northern Territory (NT), Australia, in both the indigenous and non-indigenous populations. Fetal alcohol syndrome is a preventable disease that is a major cause of intellectual handicap worldwide. The prevalence of FAS in the NT, and in Australia as a whole, is unknown. METHODOLOGY: Cases were identified through retrospective review of medical records and outpatient letters of children seen by Royal Darwin Hospital paediatric staff. Cases were also identified by tracing potentially affected siblings, or incidentally during clinical work. All children were born between 1990 and 2000, and lived in the Top End of the NT. RESULTS: Seventeen children were identified with definite FAS. Twenty-six children with partial FAS or alcohol-related neurodevelopmental disorder (ARND) were also identified. The prevalence of FAS in the Top End of the NT was calculated to be 0.68 per 1000 live births. The prevalence might be as high as 1.7 per 1000 live births, if cases identified as partial FAS or ARND because of insufficient records, were assumed to have full FAS. In indigenous children, the corresponding prevalence was calculated to be between 1.87 and 4.7 per 1000 live births. The difference between indigenous and non-indigenous rates of FAS was significant (P < 0.0001). CONCLUSIONS: The prevalence of FAS in indigenous children of the Top End of the NT is comparable to the high rates in indigenous populations worldwide.

Paracetamol overdose in a preterm neonate.

The first oral overdose of paracetamol in a neonate is reported. A 55 day old neonate, born 29 weeks premature, was accidentally given 136 mg/kg paracetamol. Treatment was with activated charcoal, supportive care, and N-acetylcysteine. There was no biochemical evidence of hepatotoxicity, and no long term sequelae. After modelling of the data, the following pharmacokinetic variables were calculated: absorption half life (t(abs)), 0.51 hours; volume of distribution (V/F(oral)), 0.80 litres/kg; clearance (CL/F(oral)), 0.22 litres/h; they were consistent with population pharmacokinetic studies. The increased plasma half life (Tbeta) of 5.69 hours thus reflected normal slower metabolism in infants, rather than toxicity. The toxicity of paracetamol in neonates is unclear, but appears to be low because of slow oxidative metabolism and rapid glutathione synthesis. In an overdose, estimates of toxicity can be made from dose and Tbeta in neonates, or from maternal toxicity in transplacental poisoning. Treatment includes N-acetylcysteine and supportive care, with activated charcoal for oral poisoning.

Isospora belli infection and chronic electrolyte disturbance in a child with fetal alcohol syndrome.

We report an 11-year-old boy with fetal alcohol syndrome and immunodeficiency, whose longstanding malnutrition, diarrhoea with steatorrhoea, symptomatic electrolyte loss and eosinophilia were attributed to renal tubular disease and recurrent worm infestation. The symptoms resolved with diagnosis and treatment of infection by the protozoan Isospora belli, although immunodeficiency persisted.

Obstructive sleep apnoea presenting as failure to thrive in infancy.

OBJECTIVE: To study the postoperative outcome of infants under the age of 18 months in whom an adenotonsillectomy had been performed, with particular emphasis on the pre- and postoperative weight gain and linear growth velocities, and the resolution of symptoms of obstructive sleep apnoea (OSA). METHODOLOGY: A retrospective study of all infants in whom an adenotonsillectomy had been performed during the 5 year period to January 1990. Details of pre- and postoperative outcome variables were obtained by review of hospital and office records and by telephone calls to the parents. RESULTS: Complete data were available for 29 (76%) of the 38 infants in whom an adenotonsillectomy had been performed. The data from these infants are reported. Pre-operatively, all infants had clinical symptoms of OSA, and 52% of infants also presented with failure to thrive (FTT). Seven infants were dysmorphic: three had Down syndrome, three had a craniofacial anomaly and one infant had Mobius syndrome. Following adenotonsillectomy, 23 infants (79%) had complete resolution of their OSA symptoms. Two infants with Down syndrome required a tracheostomy to relieve persistent upper airway obstruction. Eighty-seven per cent of the infants with pre-operative FTT had a significant increase in weight gain velocity postoperatively (mean 195.1 +/- 80.8 s.d. vs 509.8 +/- 249.1 g/month; P < 0.001), including the infants with mild persistent symptoms of OSA. The weight gain velocity of infants who were not failing to thrive pre-operatively did not change significantly following adenotonsillectomy (328.1 +/- 106.9 vs 333.2 +/- 146.4 g/month; P = 0.82). The linear growth velocity of all infants did not change significantly postoperatively. CONCLUSIONS: OSA should be considered in infants with FTT, as adenotonsillectomy is an effective treatment for OSA in infancy, and the weight gain velocity of these infants may increase significantly postoperatively. Overnight oximetry or other physiological studies may be required if the clinical signs and symptoms of OSA are equivocal.

Hypernatraemic dehydration after Lucozade.

OBJECTIVE: To emphasise the dangers of inappropriate rehydration fluids in the treatment of gastroenteritis. CLINICAL FEATURES: A two-year-old girl was admitted to hospital in shock and unconscious. She had a 36-hour history of diarrhoeal illness and had received Lucozade. Therapy with this hypertonic fluid resulted in worsening diarrhoea and seizures. On examination she had hypernatraemic dehydration and decorticate posturing. INTERVENTION AND OUTCOME: An intravenous line was inserted, stable plasma protein solution was given, and she was admitted to the intensive care unit. Anticonvulsant and antibiotic therapy were begun. Significant neurological impairment was still evident after 14 days, at which time shw was discharged from hospital. Six months later she had made a good recovery, with no persisting neurological deficit. CONCLUSION: The inappropriate use of hypertonic fluids in gastroenteritis may be associated with significant electrolyte imbalances and neurological sequelae.