Pulmonary atresia with ventricular septal defect in adult patients.

Hemodynamic data and angiograms of 15 adult patients with pulmonary atresia and ventricular septal defect were reviewed to assess the pulmonary circulation and other associated features. The most common variety of pulmonary atresia was that of pulmonary valve, main pulmonary artery, and the confluence of pulmonary arteries (6 cases, 40%). The collateral vessels to the lungs were well developed in all cases; selective injections into the collateral vessels were of great value in their delineation. The left ventricle was well developed in 11 cases (73.3%). Congestive heart failure was seen in 8 (53.3%), tricuspid regurgitation in 10 (66.7%), and aortic regurgitation in 7 (46.7%) cases. The long survival in these patients was related to the favorable anatomy of central pulmonary arteries (12 cases, 80%) and adequate pulmonary collateral circulation.

Child abuse and neglect as seen in General Hospital, Kuala Lumpur--a two year study.

Eighty-six children diagnosed as child abuse and/or neglect were admitted to the Paediatric wards of the General Hospital, Kuala Lumpur during 1985 and 1986. Of these cases, 62 were of physical abuse, six of sexual abuse, one case of both physical and sexual abuse and 17 of neglect. There were 25 boys and 61 girls. Thirty-four of these children were Malays, 16 Chinese, 26 Indians, three mixed and seven illegal immigrants. Twenty-one were below the age of one year, 24 from one to four years, 25 from five to nine years and 16 were ten years and above. The abusers were mainly close members of the family. Of these children, 24 were sent back to their parents and 11 to their relatives home. Twenty-seven were taken into care by the Ministry of Social Welfare and the remaining seven children who were illegal immigrants, were deported with their parents. Only one child was successfully fostered. Eleven children were taken away from the hospital by their parents or guardians without the knowledge of the health staff. There were five deaths in the series.

Familial nesidioblastosis: more evidence for autosomal recessive inheritance.

Severe neonatal hypoglycaemia in five Saudi Arab infants of both sexes belonging to two families of consanguineous parents is reported. All of these infants needed sub-total pancreatectomy to maintain normoglycaemia. Histopathological studies with immunohistochemistry proved diffuse nesidioblastosis of the pancreas in all five infants. Data obtained from these families and other families reported in the literature strongly suggest that familial nesidioblastosis is an autosomal recessive disorder. Knowledge of the inheritance pattern is important both for genetic counselling and for making a prompt diagnosis in subsequently affected siblings, as delay in appropriate treatment may have serious consequences.