Cerebro-costo-mandibular syndrome (CCM) is a very rare entity with oro-facial features closely resembling Pierre-Robin anomaly (1,2). We describe a patient with typical features: severe micrognathia, glossoptosis, central cleft soft palate and multiple posterior rib-gap defects seen on chest radiograph. Respiratory distress which was evident soon after birth was relieved by an oro-pharyngeal airway. He suffered from a cardiorespiratory arrest and succumbed, presumably secondary to aspiration of milk feeds, before a tracheostomy could be performed.
Pregnancies conceived through assisted reproduction can present considerable management problems to the obstetric and paediatric staff. Multiple pregnancies are common. The complication of prematurity increases the morbidity and mortality rates of the neonates.
AIMS: To examine the pattern of and the influence of some socio-demographic factors on infant milk feedings, and the protective role of breastfeeding against infections.
METHODS: Mothers who breastfed their infants (exclusively or partially) at 6 weeks postpartum, and who had singleton pregnancies and healthy infants at birth, were interviewed when their infants had reached 6 months of age.
RESULTS: Of the 234 mothers studied, only 31 (13%) mothers were practising exclusive breastfeeding (EBF) and 133 (57%) mothers were using exclusive infant formula feeding (EIF). Solid and semi-solid foods were introduced between 4 to 6 months of life in 89% of the infants. On logistic regression analysis, mothers who were in paid employment [OR 0.25, 95% CI 0.15, 0.42] and not breast feeding at 6 weeks [OR 0.32, 95% CI 0.19, 0.54] had decreased odds of EBF. Antenatal plans to breastfeed, breast-feeding difficulties, ethnicity, level of parental education, parental ages, fathers' income, primigravida status and infants' gender were not significant co-variates. In comparison, EIF was more likely in mothers who worked, practised mixed feedings at 6 weeks and of Chinese descent. There were no significant differences in the rates of upper respiratory tract infections (URTI) or diarrhoeal illnesses between the infants who were or were not being breast-fed.
CONCLUSIONS: Most mothers were unable to breastfeed their infants exclusively in the recommended first 4 to 6 months of life. Complementary changes outside the hospital and maternity services are essential in improving breastfeeding rates. Breastfeeding does not appear to confer significant protection to either URTI or gastrointestinal tract infections.
Study site: Postnatal clinic, University Malaya Medical Centre (UMMC), Kuala Lumpur, Malaysia
While it is not difficult to recognise the classical clinical features of congenital syphilis in most cases, some of them may present with unusual manifestations which can defy early diagnosis. We report our experience with 13 cases of early congenital syphilis over a period of 10 years from 1980 to 1989. Twelve of the thirteen patients were less than 3 months at presentation. There were two infants born prematurely and six of the babies were born with a low birthweight (less than 2.5 kg). All but four patients survived following treatment. Skin lesions either in the form of typical vesiculobullous eruption over the palms and soles or a maculopapular skin rash over the body were the most common presentation and was seen in 10 patients. Splenomegaly with or without hepatomegaly was the most consistent physical sign. Radiological changes in the form of periostitis and/or metaphysitis were seen in all cases where an X-ray of the long bones was performed. An elevated serum immunoglobulin M, though non-specific for the disease, was found to be a useful screening test for recent infection.
Cholestatic disorders of infancy (viz neonatal hepatitis and biliary atresia) have not been well studied in Malaysia. In a retrospective study in the Department of Paediatrics, University Hospital, Kuala Lumpur from January 1982 through December 1991, a total of ninety-three infants with such conditions were identified: 35 (38%) had biliary atresia, 58 (62%) neonatal hepatitis. There was a statistically significant male preponderance in the neonatal hepatitis group (P = 0.020). There was no significant difference in the racial distribution and in the proportions of low birthweight infants between the two groups of disorders. When the biliary atresia group was compared with the neonatal hepatitis group, significant differences were observed in the age of presentation (mean +/- SD) 9.8 +/- 6.8 VS 20 +/- 17.3 weeks (P < 0.001), proportion of infants with prolonged jaundice (> seven weeks) 28/35 (80%) VS 20/58 (34.5%) (P < 0.00001), occurrence of alcoholic stools 26/35 (74.3%) VS 27/58 (46.6%) (P = 0.020), liver size (mean +/- SD): 4.3 (1.6 cm VS 3.3 +/- 1.8 cm (P < 0.01) and splenic size: 2.5 (1.8 cm VS 1.4 (1.2 cm (P < 0.001). There was however considerable overlap between the two groups in these features at presentation, making clinical differentiation between the two conditions difficult. Infants with cholestasis tended to present late, compromising the chance of survival. In order to improve the medical care of these patients, these conditions must be emphasised during the training of medical practitioners, and efforts to increase public awareness of these conditions must be created.
Oral sodium phosphate (OSP), an effective bowel purgative, is available over the counter (OTC) and requires a substantially lower volume than polyethylene glycol-based preparative agents. Rarely, OSP consumption has been associated with acute hypocalcemia and hyperphosphatemia. We describe a case of chronic kidney disease patient developing symptomatic hypocalcemia following OTC OSP.