In recent investigations, the specificity of the human intestinal disaccharidases has been studied and their relation to different forms of hereditary disaccharide intolerance discussed (1, 2). The results have indicated isomaltose and sucrose to be hydrolyzed by two different enzymes in the human intestine.In inborn errors of metabolism, as far as we now know, only a single enzyme is missing. In several patients with sucrose intolerance, a certain intolerance for starch and maltose-dextrin mixtures has also been described (3). After the administration to such patients of a 1,6-a-oligosaccharide preparation containing chiefly isomaltose, severe intolerance symptoms occurred (4). This was taken to indicate that the patients had intolerance for both sucrose and isomaltose, and thus should lack two different enzyme activities. The patients also had intolerance for palatinose (isomaltulose), a sugar with a close structural resemblance to isomaltose (3, 4).The aim of the present investigation was to study further the relation between the isomaltase and invertase (sucrase) activities of human small intestinal mucosal preparations, and also the importance of these enzymes for the hydrolysis of palatinose and the 1,6-a-oligosaccharide prepara-* This work was supported in part by: Association for the Aid of Crippled Children, New York, N. Y.; Consiglio Nazionale delle Ricerche, Rome, Italy; Schweizerische Nationalfond zur F6rderung der wissenschaftliche Forschung, Stiftung fur wissenschaftliche Forschung an

Menstrual patterns were studied longitudinally in 140 adolescent Swiss girls. The mean cycle length was 32.2 (S.D. 11.24) d in the first year after menarche, and 29.9 (S.D. 7.12) d in the sixth year after menarche. The values of the mean cycle length became essentially constant from the sixth post-menarcheal year, the chronological age of 20 y and the 36th post-menarcheal cycle. No association was found between cycle length and age at menarche. The menarcheal age of mothers and that of their daughters were significantly correlated with each other (r = 0.29, P less than 0.001). The most frequent cycles of 21-27 and 28-34 days constituted 64-81% of all cycles depending on post-menarcheal age. Short cycles (14-20 d), representing 2-6% of all cycles, were present in 14-31% of the girls; long cycles (35-41 d), constituting 10-16% of all cycles, occurred in 46-68% of the girls. The frequency of the long cycles tended to decrease at higher post-menarcheal ages. There were minimal changes in the duration of menstrual flow throughout the first six years after menarche. The mean duration was 5.4 (S.D. 1.72) d for the first year, and 5.1 (S.D. 1.33) d for the sixth year. The most frequent flow duration was 3-7 d, present in 88-94% of cycles. Prolonged bleeding tended to decrease with rising post-menarcheal and chronological age. The intensity of menstrual flow was mild in 11-16%, moderate in 62-78% and severe in 11-25% of the girls. The frequency of these three categories remained essentially unchanged during the first five post-menarcheal years.

In a consecutive series of 15 male adolescents and young adults with congenital adrenal hyperplasia (CAH), the size, shape, firmness and echostructure of the testes were assessed. The latter was abnormal in 7 patients under long standing treatment with glucocorticoids (group I). In 8, 5 under and 3 off treatment for several years, ultrasonography (US) was normal (group II). On the basis of the US findings the patients were placed in two groups. In group I, the testes had a heterogeneous ultrasonographic pattern; clinically, most felt hard and irregular, although their volume was normal. Sperm count in 3 patients was 5.0 to 14.4 x 10(6)/ml. Five patients have 21-hydroxylase deficiency that was diagnosed in early infancy and had salt-wasting; two have 11-beta-hydroxylase deficiency that was diagnosed late and had no salt-wasting. In group II, testicular volume, shape, firmness and echostructure were normal. All have 21-hydroxylase deficiency, no history of salt-wasting and were diagnosed late. Sperm counts in 3 patients off treatment were 10.5 to 66.0 x 10(6)/ml. In severe cases with a history of salt loss, TALT with deficient spermiogenesis seems likely despite treatment. In mild cases, TALT is absent and spermiogenesis may be normal even without treatment. US is much more accurate in assessing the testes than palpation.

ExtractCeliac disease provides a model for investigation of one form of infantile malnutrition occurring under fully controlled circumstances. Thirteen patients aged 9-15 months were followed for a period of at least 3 years while they were on a strict gluten-free diet.Observed values for height, weight, bone age, metacarpal diameter, and cortical thickness are compared with the normal range based on local standards, and the results are expressed as standard scores. The mean curves of these data are presented in Figures 1 and 2. At the time of diagnosis and the beginning of diet (zero time), all variables were retarded, and standard scores were significantly below normal in height (P < 0.01), weight (P < 0.001), bone age (P < 0.01), metacarpal cortex (P < 0.05), and diameter (P < 0.001). Weight was significantly more depressed than height (P < 0.01), bone age (P < 0.001), metacarpal cortex (P < 0.001), and diameter (P < 0.001). Height was significantly more depressed than bone age (P < 0.05). As the patients recovered, weight caught up faster than the other measurements; weight was not significantly below normal after the patients had been on a gluten-free diet for 6 months and it reached normal values between 6 months and 1 year after initiation of diet. Height and bone age did not reach normal levels until after 2 years of treatment, although the values were no longer significantly depressed after 1 year on the diet. Cortical thickness was no longer significantly depressed after 6 months on the diet; it reached normal values by 6 months to 1 year after initiation of diet, and then showed an overshooting type of catch-up with values remaining significantly elevated at 2 years (P < 0.05) and 3 years (P < 0.05) after the onset of diet. Metacarpal diameter showed a slow steady catch-up growth which remained below normal throughout the follow-up period although the values were no longer significantly below normal 3 years after patients had begun diet.The study provides evidence that children aged 1 year (±3 months) who suffer from malnutrition caused by celiac disease catch up completely in weight, height, bone age, and metacarpal cortical thickness during a dietary treatment period of 3 years. SpeculationPoor physical growth resulting from this type and this degree of malnutrition in the second half of the 1st year of life is completely remediable. Permanent somatic sequelae from a similar severity of late infantile malnutrition in underdeveloped countries, therefore, might not be inevitable, provided that the therapeutic program is optimal and adverse factors do not continue to operate.

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