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UNLABELLED Infants requiring parenteral nutrition (n = 244) were randomized to receive either 1 (group 1, n = 121) or 0.0182 micromol/kg/d (group 2, n = 123) of manganese supplementation. The whole-blood manganese and serum direct bilirubin concentrations of the infants were monitored, as was the development of cholestasis (peak serum direct bilirubin concentration >50 micromol/L). Subgroup analysis was carried out on the data of 78 infants in group 1 and 82 in group 2 who had received manganese supplementation and more than three-quarters of their total daily fluid as parenteral nutrition for >14 d. Of all the infants randomized, the high manganese group (group 1) showed a trend towards developing higher peak whole-blood manganese concentration [group 1 versus group 2: median (interquartile range): 606.0 (421.0; 1005.0) vs 566.0 (336.0: 858.0); p=0.061] and higher peak serum direct bilirubin concentration [37.0 (10.5; 122.5) vs 19.0 (8.0; 112.5); p=0.153], but the differences between the 2 groups did not reach statistical significance. The 2 groups did not differ in terms of the occurrence of cholestasis during parenteral nutrition (63/121 vs 57/123; p=0.444). Subgroup analysis of infants who had received more than three-quarters of their total daily fluid as parenteral nutrition showed, however, that the high manganese group developed significantly higher whole-blood manganese concentration [743.5 (498.0; 1211.0) vs 587.0 (438.0; 982.0); p=0.037] and serum direct bilirubin concentration [84.0 (28.0; 170.0) vs 25.5 (9.0; 117.0): p 100 micromol/L (32/78 vs 20/82; p = 0.038). CONCLUSION We conclude that the pathogenesis of parenteral nutrition-related cholestasis is probably multifactorial, and that high manganese intake is a significant contributory factor.

Structured Abstract Authors – Fok TF, Hon KL, So HK, Wong E, Ng PC, Lee AKY, Chang A Objectives – To provide a database of the auricular measurements of Chinese infants born in Hong Kong. Design – Prospective cross-sectional study. Setting and Sample Population – A total of 2384 healthy singleton, born consecutively at the Prince of Wales Hospital and the Union Hospital from October 1998 to September 2000, were included in the study. The range of gestation was 33–42 weeks. Measurements and Results – Measurements included ear width (EW), ear length (EL) and ear position (EP). The data show generally higher values for males in the parameters measured. When compared with previously published data for Caucasian and Jordanian term babies, Chinese babies have shorter EL. The ears were within normal position in nearly all our infants. Conclusion – The human ear appears to grow in a remarkably constant fashion. This study establishes the first set of gestational age–specific standard of the ear parameters for Chinese new-borns, potentially enabling early syndromal diagnosis. There are significant inter-racial differences in these ear parameters.

Objective: The factors accounting for the low ‘ever breastfeeding’ rate in Hong Kong remain unclear. The objective of this survey was to study the intention and planned duration of breastfeeding in Chinese women in Hong Kong, and to investigate the sociodemographic and atopic factors affecting the intention to breastfeed. Methods: All Chinese mothers who were Hong Kong residents and who delivered their babies in a University teaching hospital were given a self administered and anonymous Chinese questionnaire within 1 day postpartum. The questionnaire included items on sociodemographic data, the presence of allergic diseases in the families, and the intention and planned duration of breastfeeding for their newborn babies. Results: A total of 1374 eligible questionnaires were collected. About one-third of mothers were born outside Hong Kong and the majority (78%) of them were aged between 25 and 40 years. Logistic regression revealed that only socioeconomic factors, namely older maternal age (odds ratio (OR) 2.10, 95% confidence interval (CI) 1.14−3.89), parity (OR 3.08, 95% CI 1.02−9.27) and higher educational background (OR 2.87, 95% CI 1.92−4.27), significantly affected the intention to breastfeed. In addition, mothers born outside Hong Kong and those with first babies, had a longer intended duration of breastfeeding (P-values

Two cases of homozygous alpha-thalassaemia who received active treatment in accordance with parental wishes are reported. One infant survived and the other, although successfully weaned off mechanical respiratory support, unexpectedly developed portal vein thrombosis and died. Homozygous alpha-thalassaemia, a condition previously considered to be universally fatal, and an indication for therapeutic abortion, is now potentially curable with advances in diagnostic technology and treatment. However, active management of these cases raises serious ethical questions and has major financial implications on the health-care system. Invasive prenatal and intensive postnatal interventions should remain experimental and cannot be recommended as routine clinical practice until the questions of long-term neurodevelopmental outcome, and the morbidity and mortality associated with bone-marrow transplantation have been fully addressed. As a result of advances in information technology, more and more parents of affected foetuses are likely to request active treatment.

Objectives: A prospective study comparing the efficiacy and side-effects of oral sulindac with intravenous indomethacin in clinically stable preterm infants (0.25). More renal adverse effects were encountered in the indomethacin group. Significant differences in changes from baseline value for urine output, plasma sodium, urea and creatinine concentrations were noted at 24, 48 and 72 h after commencement of treatment between the two groups (P

Accurate clinical diagnosis of severe acute respiratory syndrome (SARS) based on the current World Health Organization definition is difficult and at times impossible at the early stage of the disease. Both false positive and false negative cases are commonly encountered and this could have far-reaching detrimental effects on the patients, their family and the clinicians alike. Contact history is particularly important in diagnosing SARS in children as their presenting features are often non-specific. The difficulty in making a correct diagnosis is further compounded by the lack of a sensitive rapid diagnostic test. Serology is not particularly helpful in the initial triaging of patients as it takes at least 3 weeks to become positive. Co-infection and other treatable conditions should not be missed and conventional antibiotics should remain as part of the first-line treatment regimen. We report five cases to illustrate the difficulties and dilemmas faced by clinicians in diagnosing SARS in children.

Five cases of moderately severe hypernatraemic dehydration were identified within a 5-month period between two regional hospitals in Hong Kong. Unlike previous reported cases, these exclusively breast-fed infants presented with the unusual triad of fever, absence of overt signs of dehydration and within the first week of life. Three of the cases also had high serum bilirubin concentrations at presentation. The fever subsided quickly and the serum bilirubin concentration fell rapidly within a few hours of rehydration. All infants made an uneventful recovery without permanent neurological sequelae. Fever, presumably secondary to dehydration, is an useful early warning sign. These cases emphasize the importance of early and regular measurement of bodyweight in exclusively breast-fed infants so that prompt identification of affected cases may prevent potentially detrimental complications.

A severe case of aplasia cutis congenita in a preterm infant is described. Although major problems with thermoregulation and fluid balance were anticipated, these parameters were relatively easy to control once the patient was stabilized. Meticulous skin care and rapid formation of a membranous-like fibrous tissue layer covering the denuded areas probably played an important role in minimizing excessive fluid and heat loss. The prognosis in aplasia cutis congenita is determined by the underlying associated anomalies, the severity of skin lesions and, in our case, the maturity of the infant who died from complications of prematurity.

Objective: To report our clinical experience on the use of oral erythromycin for the treatment of severe gastrointestinal dysmotility in preterm infants. Methodology: A case series study of seven preterm infants (six were very low birthweight) with severe intestinal dysmotility in a tertiary neonatal centre. Results: All responded favourably without adverse effects and tolerated full enteral feeding within 1–2 weeks of the commencement of the drug. Conclusions: As prolonged total parenteral nutrition carries significant risk of complications, this therapy could be considered in selected preterm infants who fail to establish enteral feeding after an extended period, and in whom an anatomically obstructive lesion of the gastrointestinal tract has been excluded. Meanwhile, we would caution against the widespread implementation of this therapeutic approach until formal evaluation by randomized controlled trials have established the exact role of erythromycin, or its analogues, in the treatment of intestinal dysmotility in preterm infants.

Isolated congenital tricuspid valve dysplasia is a rare and potentially lethal congenital heart disease that can be easily confused with persistent pulmonary hypertension of the newborn. We describe a neonate with isolated congenital tricuspid valve dysplasia who did not respond to mechanical ventilation but improved by tolazoline. Clinicians should be aware that the initial fulminant course of this condition may be reversed by reducing the pulmonary vascular resistance, thereby allowing time for spontaneous recovery.

We report the first case in a preterm infant given oral sulindac for treatment of symptomatic patent ductus arteriosus who subsequently developed severe acute haemorrhagic gastritis leading to disseminated intravascular coagulation, massive pulmonary haemorrhage and death. The postmortem examination suggested that the mechanism was likely a direct irritant insult causing ischaemia on the gastric mucosa. Although sulindac is supposed to be a renal-sparing non-steroidal anti-inflammatory prodrug associated with minimal renal and gastrointestinal adverse effects, clinicians should be alerted to this potential life-threatening complication in preterm infants. Until the question of safety could be adequately addressed, the use of sulindac for ductal closure should remain experimental.

The plasma concentrations of antithrombin III, protein C and protein S in capillary and venous blood samples obtained simultaneously from 30 neonates were compared in order to determine the suitability of using capillary blood for estimation of these proteins with anticoagulant action. Our findings showed that while capillary and venous blood did not differ significantly in antithrombin III functional activity and protein C antigen levels, the capillary samples had significantly lower protein C functional activity and higher antithrombin III antigen level. Protein S antigen level was also significantly higher in the capillary samples although the difference was relatively small. The capillary and venous concentrations of the binding protein of protein S, C4b binding protein, were almost identical.

A preterm infant developed bilateral tension pneumothoraces and extensive vascular air embolism 6 h after being commenced on nasal continuous positive airway pressure (CPAP). Neonatal clinicians should be aware that catastrophic vascular air embolism could occur in infants receiving nasal CPAP, a modality of respiratory support conventionally considered non-invasive and 'safe'.

A newborn infant who presented with central cyanosis was found to have hereditary methaemoglobinaemia. The pulse oximeter readings and physical findings were incompatible. Clinical assessment remains an important part in the management of such cases.

A baby with congenital heart block and hydrops fetalis diagnosed prenatally was delivered at 36 weeks of gestation. Heart failure was controlled with temporary cardiac pacing which was complicated by perforation of the myocardium. She made an uneventful recovery after implantation of a permanent pacemaker. Sjögren's syndrome A antibodies were detected in her serum. Coincidentally, she also had pulmonary valvular stenosis as a structural abnormality.