Good growth response to growth hormone treatment in the ring chromosome 15 syndrome.

Ring chromosome 15 syndrome is a rare condition in which severe growth retardation is a major finding. We report a 4 year old boy with the karyotype 46,XY, r(15)(p11.2q26.2) whom we have treated with recombinant human growth hormone (GH) for two years. During the first year of treatment, the insulin-like growth factor I increased from subnormal 4.2 nmol/l to normal 13.8 nmol/l and the insulin-like growth factor binding protein 3 levels increased from 2.6 to 3.8 mg/l, whereas high binding protein 1 concentrations normalised from 52.0 to 16.7 micrograms/l. During the two years of treatment his relative height improved from -6.2 SD to -4.4 SD and the predicted adult height from 159.6 cm to 163.5 cm. Owing to the good growth response, we have decided to continue GH treatment.

Consequences of caustic ingestions in children.

A retrospective analysis of 98 patients, less than 15 years of age, treated for caustic ingestion during 1976-1990 was performed to evaluate the modern consequences of caustic ingestion in children and to set indications for esophagoscopies and radiographic and laboratory examinations. Dishwasher detergents were ingested by 56 children. There were no lye ingestions, since lye has not been freely available in Finland since 1969. Household acetic acid (vinegar) was the most commonly (12/23) ingested acid. Primary esophagoscopy was performed in 79 of the 98 cases (80.6%). Esophageal burns were found in 20 patients. Acids caused burns more often than alkalies (9/23 (39.1%) versus 11/75 (14.7%); p = 0.011; 95% confidence intervals (CI) for the difference 5.6-43.3%) and acid burns more often developed into scars (7.4% versus 4%; p = 0.029; 95% CI for the difference 1.4-25.4%). The only esophageal stricture developed after ingestion of a Clinitest tablet. The mean time for hospitalization as a result of acid ingestion was significantly longer than after alkaline ingestion (3.2 (SD 3.5) days, n = 23 versus 1.5 (1.6) days, n = 75; p < 0.05; 95% CI for the difference 0.7-2.8 days). Prolonged drooling and dysphagia (12-24 h) predicted esophageal scar formation with 100% sensitivity and 90.1% specificity, but signs and symptoms did not predict esophageal burns after primary esophagoscopy. Radiographic examinations and leukocyte counts were of no value in predicting esophageal burns and scars. The panorama of caustic ingestion appears to have changed, probably due in part to the law banning sale of lye products since 1969. This type of law should be encouraged elsewhere. Acids cause even more caustic burns than alkalies. Vinegar should be regarded as a potent caustic substance and distributed in baby-safe bottles with appropriate information on its caustic nature. As severe esophageal lesions after accidental ingestion of caustic substances are now rare in children, primary esophagoscopies and hospitalization of patients are not indicated routinely. The decision on esophagoscopy can be made on the basis of drooling and dysphagia during follow-up.

[Fever--friend or foe?]. / Feber--vän eller fiende?

Since the turn of the century intensive research has been carried out to ascertain whether fever is associated with the organism's defence against microbial agents, and evidence is now available concerning the beneficial impact of fever under certain circumstances. However, fever also often gives rise to risks and inimical sequelae. Antipyretics should be used in the correct manner, and when the fever constitutes a hazard to the patient or causes suffering.

Accidental astemizole overdose in young children.

6 cases of accidental astemizole poisoning in children have been reported to the Finnish Poison Information Center. The children were aged 1 year 7 months to 3 years 4 months and had taken doses of 2.5 to 16.7 mg/kg, at least twelve times that recommended. In 2 children the overdose was verified by measurement of drug concentrations. Despite measures to prevent drug absorption prolonged QTc-interval, a sign of cardiac toxicity, was found in all 5 children on whom an electrocardiogram was available for analysis, and severe ventricular arrhythmias developed in 1. Young children with accidental astemizole overdose should be carefully monitored in hospital. Similar precautions may be justified in cases of overdose with other selective H1-histamine receptor antagonists.

Ultrasonic features of the congenital nephrotic syndrome of the Finnish type.

Ultrasonic features of the kidneys of seven infants with congenital nephrotic syndrome of the Finnish type under active conservative treatment are described. In all cases the kidney were enlarged an the cortex more echogenic than the liver and spleen parenchyma. The pyramids were small with hazy borders resulting in an indistinct or lost corticomedullary border. At follow-in the echogenity of the renal cortex increased still further in four infants and the pyramids became invisible. The differential diagnosis is discussed.

Finnish approach to the treatment of acute otitis media. Report of the Finnish Consensus Conference.

The theme of the first consensus conference to be held in Finland was the treatment of acute otitis media. The statements and recommendations accepted by the conference, which was organized according to the National Institutes of Health model, are presented in this report. On the basis of scientific knowledge, clinical experience, and conditions in Finland, the conference delegates concluded that penicillin V, in large doses, is still the drug of first choice in this disease. The importance of surgical drainage of the middle ear was stressed, as was the necessity of careful follow-up of the patient until the condition is completely healed. Decongestants were considered rather useless. In the prevention of recurrent attacks, adenoidectomy but not tonsillectomy was regarded as being of help, tympanostomy of probable benefit, antimicrobial (sulfonamide) prophylaxis worth considering in selected cases, but the effect of pneumococcal vaccination poor. The conference delegates agreed that uncomplicated acute otitis media should, as before, usually be treated by physicians taking care of children at the primary health care level.

Intrapair similarity of immunoglobulin levels in twins.

Levels of immunoglobulins IgG, IgA, IgM and IgE were determined in 8 MZ and 14 DZ twin pairs at the ages of 6-11 years, 12-17 years and 15-20 years. Intrapair similarity in immunoglobulin levels was found to be higher in the MZ than in the DZ twins, especially in the case of immunoglobulins IgA and IgM.

Levels of some purine metabolizing enzymes in lymphocytes from patients with Down's syndrome.

The activities of a number of purine metabolizing enzymes of erythrocytes and lymphocytes were determined in 18 subjects with Down's syndrome and in 18 age- and sex-matched control subjects. An increase of adenosine deaminase activity (adenosine or deoxyadenosine as substrates) was found in erythrocytes (P less than 0.001) as well as in lymphocytes (P less than 0.001) of Down's syndrome subjects compared to controls. The purine nucleoside phosphorylase activities in lymphocytes and plasma urate concentrations were also significantly higher in Down's syndrome subjects than in controls (P less than 0.001 and less than 0.02, respectively). Adenine phosphoribosyltransferase activities and hypoxanthine-guanine phosphoribosyltransferase activities in lymphocytes were identical in the two groups. In all subjects studied there were positive correlations between the erythrocyte adenosine deaminase activities, lymphocyte adenosine deaminase or deoxyadenosine activities, and plasma urate concentrations (P less than 0.05 in all cases), and between lymphocyte nucleoside phosphorylase and lymphocyte adenosine deaminase or deoxyadenosine deaminase activities (P less than 0.01 and less than 0.05, respectively). The results suggest that increased activities of some purine metabolizing enzymes found in both erythrocytes and lymphocytes may contribute to increased purine degradation and hyperuricemia in subjects with Down's syndrome. In addition, the increased adenosine deaminase and nucleoside phosphorylase activities may be related to the immunological dysfunction found in subjects with Down's syndrome.

Insulinotropic activity in the serum of obese and nonobese infants and children.

The ability of a serum fraction, mol wt 1000-5000, to stimulate insulin release in vitro was studied in 123 obese and normal weight children aged 0-17 yr. The sera were fractionated by serial molecular filtration after treatment with urea. Stimulation of insulin release was determined with a bioassay using isolated rat islets in perifusion. The islet-stimulating activity was found in all obese children less than the age of 10 yr and in the majority of the obese children older than 10 yr of age. In normal weight children the activity was also found in the majority of infants, but was infrequent in older children. The serum islet-stimulating activity was positively correlated with the duration and degree of obesity and with linear growth rate. The molecular structure and origin of the insulinogenic activity in the serum is still unknown. In high-performance liquid chromatography it has the same elution characteristics as the hypothalamic insulin-glucagon liberin. The present results suggest a role for the serum islet-stimulating activity in the pathogenesis of obesity.

Serum insulin-releasing activity and the Prader-Willi syndrome.

Pathogenesis and etiology of the various clinical features of the Prader-Willi syndrome (PWS) are not completely understood. There is evidence suggesting a hypothalamic abnormality leading to hypogonadism in this syndrome. To test the possible hypothalamic involvement in the pathogenesis of obesity in these patients we studied the sera of 5 patients with PWS for insulin-releasing activity of possible hypothalamic origin. In addition 4 patients with a PWS-like syndrome and 6 obese patients with signs of central nervous system damage were studied. All five patients with PWS showed significant insulin-releasing activity in their sera. Of the four patients with PWS-like symptoms three showed similar activity while three of the obese patients with central nervous system damage had this activity in their sera. Serum insulin-releasing activity has been shown earlier to be associated with childhood obesity and it could play a role in the pathogenesis of obesity in PWS.

Group A streptococcal peritonitis in infancy.

Primary peritonitis in infancy is a rare condition. It is usually associated with a severe pathological process, such as nephrotic syndrome. When it affects previously healthy infants it causes diagnostic and therapeutic difficulties. Pneumococci, streptococci and Haemophilus influenzae are possible causative agents, other causes are rare. The clinical course of two infants with group A streptococcal peritonitis is described.