S. Similä

DEVELOPMENT OF BOWEL AND BLADDER CONTROL IN THE MENTALLY RETARDED

For the mentally retarded, bowel and bladder control are important prerequisites for an independent life. A study of these functions was made in a cohort of children born in Northern Finland in 1966. Relevant data up to the age of 20 years were obtained for 105 of the 132 children with mental retardation (IQ <70) who were alive at that age. 80 per cent had attained bowel control at a mean age of 4.2 years, but 30.5 per cent were still encopretic at seven years, and 19 per cent at the age of 20 years. Full bladder control had been achieved by 62.9 per cent at the age of seven and by 82.9 per cent at the age of 20. It is concluded that systematic, appropriate toilet training could improve these figures markedly.

Type 7 adenovirus pneumonia

Twenty-nine cases of severe type 7 adenovirus pneumonia in children ages 8 months to 6 4/12 years were observed in northern Finland in 1967 and 1968. Adenovirus type 7 was isolated from 17 patients. The other 12 had serologic evidence of adenovirus infection. The clinical picture and complications were compared with those of 17 children with pneumonia associated with bacterial or other viral infections hospitalized during the same period. The clinical course of the adenovirus type 7 infection was especially severe. Extrapulmonary manifestations (meningism, encephalitis, hepatomegaly, heart failure, and hemorrhagic tendency) were common especially in children less than 3 years of age. Three of the 29 patients died, and 4 of the survivors (14 per cent) had permanent pulmonary damage: 2 had bronchiectasis and 2 had residual lung fibrosis.

Achalasia sicca--juvenile Sjögren's syndrome with achalasia and gastric hyposecretion.

Sjögrens syndrome (SS) in its classical form, which includes kerato-conjunctivitis sicca, xerostomia and recurrent enlargement of the salivary glands, is associated with a connective tissue disease in at least half the patients.According to the present study of three patients with SS, achalasia and gastric hyposecretion seem to be either further manifestations of SS, or separate phenomena associated with SS. The gastric hyposecretion involves both the hydrochloric acid and the total volume of the secretion, but the gastric mucosa has a normal appearance on microscopy.Because of the simultaneous presence of achalasia, gastric hyposecretion and reduced salivation, we have called the combination “achalasia sicca”. The reduction in the secretions of the upper gastrointestinal tract might have a pathogenic association with achalasia.

Hypercalcemia and nephrocalcinosis in patients with congenital lactase deficiency

We describe 11 infants with congenital lactase deficiency, whose age at diagnosis varied from 6 to 88 days. At the time of admission, 7 of 10 infants had hypercalcemia. Five of the seven infants for whom renal ultrasonography was performed at the time of diagnosis had medullary nephrocalcinosis. Hypercalcemia ceased within a week of the start of a lactose-free diet. At the time of reevaluation, at the ages of 2 to 10 years, one of the patients still had hypercalciuria and nephrocalcinosis was still present in 3 of 11 patients. The mechanism of hypercalcemia is unclear but may be related to metabolic acidosis or may be promoted by the lactose effect (i.e., by nonhydrolyzed lactose that has a direct enhancing effect on calcium absorption in the ileum).

Oral Antipyretic Therapy: Evaluation of Ibuprofen

The capacity of ibuprofen to reduce fever in children was compared with that of aspirin, paracetamol, aminophenazone and indomethacin. The series of cases studied consisted of 79 patients in the age range 3 months to 13 years and with a rectal temperature above 38.5°C. Temperatures were recorded at 15 and 30 minutes, and 1,2, 4 and 6 hours after challenge with the drug. The antipyretic effect of ibuprofen with a dose of 6 mg/kg was optimal and twice that of aspirin or paracetamol and similar to that of aminophenazone. The antipyretic effect of indomethacin was about 12 times that of ibuprofen. This ratio is almost the same as what is said to occur between the antirheumatic effects between these drugs. Ibuprofen with a dose of 6 mg/kg would thus appear to be a useful antipyretic drug when both antipyretic and antirheumatic effects are needed.

Intracranial calcifications in cerebro-oculo-facio-skeletal (COFS) syndrome

Two brothers with typical clinical findings of the COFS-sundrome are described. In the cranial CT performed on the younger patient at the age of 2 years and 6 months, foci of intracranial calcification located symmetrically in the region of the lenticular nucleus and hemispheric white matter were noted. This finding is clearly distinguishable from the other known patterns of intracranial calcification and could be pathognomonic of the COFS-syndrome.

Gastrointestinal findings in atopic children.

Abstract36 children aged 0.13 to 13.05 years with severe manifestations of atopy were studied for circulating parietal cell antibodies (PCA), fasting serum gastrin and ferritin. Gastric acid secretion was measured using a pentagastrin test. In addition, 21 gastric and 28 jejunal biopsies were taken for evaluation of the mucosal morphology. In infants the gastric secretion studies were repeated after a three month hypoallergic diet.Maximal acid output (MAO) was significantly (P<0.01) diminished in atopic infants under one year of age. In this group one girl with transient PCA was achlorhydric and seven others were hypochlorhydric, as compared with controls of the same age. Acid secretion returned to normal in most children while on the elimination diet, and only two remained hypochlorhydric thereafter. The picture was more complex in the older children as some continued to exhibit hyposecretion in their second year of life, while others were even hyperchlorhydric. Epithelial degeneration, found in 13 out of the 21 samples, was the most striking feature in the gastric biopsies. Jejunal biopsies revealed increased eosinophilic infiltration of the lamina propria in 10 out of 28 diagnostic samples; two had slight and another two partial villous atrophy.It is concluded that in atopic children gastric hyposecretion and epithelial degeneration may promote the passage of unhandled food allergens through the jejunal mucosa, predisposing to more severe changes, as seen in cows milk intolerance. Slight villous atrophy with eosinophilic infiltration and oedema of the lamina propria may cause mild absorption defects, growth and nutritional deficiencies in these children. Atopy should be considered as a possible aetiological factor in prolonged gastrointestinal disorders in infants.

Cholic acid and chenodeoxycholic acid concentrations in serum during infancy and childhood.

Abstract. Heikura, S., Similä, S., Finni, K., Mäentausta, O. and Jänne, O. (Departments of Clinical Chemistry, Biochemistry and Paediatrics, University of Oulu, Oulu, Finland). Cholic acid and chenodeoxycholic acid concentrations in serum during infancy and childhood. Acta Paediatr Scand, 69: 659, 1980.—Concentrations of two primary bile acids (cholic and chenodeoxycholic acids) were determined by radioimmunoassay in the serum of infants and children at ages ranging from 1 hour to 15 years. The same bile acids were also measured in umbilical cord serum. Concentrations of the primary bile acids were significantly higher in the serum of 1‐hour old infants than those in the umbilical cord serum or the peripheral vein serum of adults. The levels of cholic and chenodeoxycholic acid remained high until the age of 6 months, being about 5‐fold higher than those in the sera of adults. Primary bile acid concentrations reached the adult level by the age of 1–2 years. These results indicate that developmental changes occur in the metabolism and excretion of bile acids in man. The relatively high concentrations of the primary bile acids in serum during the first 6 months of life suggest that up to this age, the mature ability of the liver to excrete the bile salts into the bile and/or to clear them from the circulation has not yet been reached.

Sternal abscess as a complication of BCG-revaccination

A sternal abscess appeared 14 months after BCG revaccination of a 14-year-old girl. Culture taken from the abscess was positive for Mycobacterium bovis, which was identified as a BCG strain.

Dipropylacetate and aminoaciduria

Dipropylacetate (DPA) is an anticonvulsant, which has been successfully used in the treatment of several types of epilepsy. The mode of action has not yet been definitely elucidated, although evidence of influence on gamma aminobutyric acid (GABA) turnover in brain has been presented. Several recent reports of the occurrence of hyperglycinemia in association with DPA-treatment indicate that this agent also influences other areas of amino acid metabolism. In the present study of 10 patients receiving DPA for epilepsy, high concentrations of glycine in plasma and CSF were observed, whereas the levels of all other amino acids remained virtually unchanged. The effect of DPA on urinary excretion of amino acids seems to be of considerable significance as marked elevation of urine concentrations of alanine, asparagine, cystine, glycine, histidine, isoleucine and leucine, phenylalanine and tyrosine were observed. This secondary hyperglycinemia could be due to suppression of glycine conjugation reactions, whereas DPA or its metabolites might interfere with tubular reabsorption of various amino acids, thereby causing hyperaminoaciduria.