An 18-month-old girl with AIDS related complex following congenital HIV infection suffered since the first days of life from severe neurological disturbances. The extensive congenital brain atrophy and the diffuse intracranial calcification which were found were strongly reminiscent of intrauterine infection. Since the agents usually responsible for congenital infections were excluded an HIV embryopathy appears likely.
We describe deep vein thrombosis associated with lupus anticoagulant and anticardiolipin antibodies in three children aged 10 to 14 years. One of them also had arterial thromboses. None of the patients had systemic lupus erythematosus when the thrombosis first occurred, but one fulfilled the criteria for systemic lupus erythematosus 3 years later. At presentation all had symptoms suggestive of pulmonary embolism and evidence of an autoimmune disease: Addison's disease in one, anti-DNA or antinuclear antibodies in all three, and a positive Coombs' test in two. Two of the three gave a false-positive test for syphilis. In the patient with systemic lupus erythematosus recurrent thrombocytopenia and severe haemolytic anaemia necessitated splenectomy. A child should be tested for lupus anticoagulant or anticardiolipin antibody if venous or arterial occlusion occurs without a known predisposing cause, or if there is pulmonary embolism or symptoms or laboratory findings suggestive of a connective tissue disease.
The aim of the present study was to describe linear growth of infants with celiac disease, using the "ICP-growth model". Supine length during the first three years of life was studied longitudinally in 63 infants with diagnosed celiac disease. "Undisturbed" linear growth was seen during the first six postnatal months followed by reduced growth during the second half of the first year. After 1.5 years of age a pattern of catch-up growth was observed, leading to an average attained length at 3 years of age similar to that of the controls. According to the "ICP-growth model", normal linear growth can mathematically be represented during the first 3 years of life by an Infancy component with the addition of a Childhood component, the latter acting from the second half of the first postnatal year. The onset of the Childhood component (assumed to represent the age at which growth hormone begins to influence linear growth significantly) was delayed by about an average of 3 months, which is in agreement with the observed reduction in gain during the second half of the first year of life. Children suffering from celiac disease and with "late" onset of the Childhood component were shorter at 1, 2 and 3 years of age than those with "normal" onset. The results of this investigation show that ICP-based growth charts are helpful in detecting and monitoring growth for children with celiac disease, and indicate a possible mechanism whereby malabsorption (and perhaps secondary malnutrition) leads to reduced growth velocity.
Plasma lipids and apoproteins A-I, A-II and B levels were measured in 125 children whose fathers had coronary atherosclerosis (paternal coronary heart disease group), in 172 children with blood pressure lability and in 154 children selected at random from a representative subsample (reference). The paternal coronary heart disease group had, compared with the reference group, lower levels of high density lipoprotein cholesterol and apoprotein A-I, higher levels of low density lipoprotein cholesterol, triglycerides and apoprotein B, and also a higher ratio of apoprotein B to apoprotein A-I. The paternal coronary heart disease group subjects with blood pressure lability had the most pronounced changes in the lipoprotein spectrum, which were similar to atherogenic changes in the lipoprotein profile, characteristic of patients with coronary atherosclerosis.
Three children with cystic fibrosis and localized bronchiectasis were treated by surgical removal of the affected lobe, after attempts to clear the obstructed bronchus by medical therapy and bronchoscopy with suction and lavage had been unsuccessful. Three to six years after surgery, the results are excellent. Pulmonary disease is mild in all patients, shown by chest radiographs and pulmonary function tests. A review of 102 cases reported in the literature demonstrates that pulmonary surgery is effective and can be performed safely in selected patients.