Intestinal enterokinase deficiency in 2 sibs in described. A boy failed to gain weight and had vomiting, diarrhoea, oedema, hypoproteinaemia, and anaemia in early infancy. His duodenal juice contained very low or absent proteolytic enzyme activity, which increased markedly after addition of enterokinase. He was treated with pancreatic extract and gained weight rapidly. At 44 months of age he is normal, apart from some development delay, and no longer needs pancreatic extract. His older sister, who had had similar symptoms in early infancy but then grew normally, had the same abnormality in her duodenal juice when seen at 4 years of age. Enterokinase activity was virtually absent in the duodenal mucosa of both patients. Mucosal morphology was normal. The findings suggest that enterokinase deficiency is an inherited congenital defect and not the result of mucosal damage. Affected patients may show spontaneous improvement and normal growth after the age of 6 to 12 months. This phenomenon may be related to the decreasing growth volocity during the first 2 years of life and the concimitant decrease in protein requirements per unit bodyweight.
Twenty-two infants in whom hypoglycemia (blood sugar less than 20 mg./100 ml.) was noted during the first few days of life were followed up when eight to 30 months of age. In eight such symptoms as muscular tremors, cyanosis, apneic spells and convulsions were associated with the hypoglycemia; five of these had abnormal central nervous system signs and retarded development. One other had possible impairment of development and another had a recurrence of hypoglycemia after having been well for four years. Fourteen of the 22 infants had no symptoms associated with the hypoglycemia, and on follow-up only two of these showed possible impairment. The rest were normal.
This preliminary study suggests that hypoglycemia associated with neurological symptoms in the newborn period carries a poor prognosis with respect to permanent neurological damage. Asymptomatic hypoglycemia may have a relatively good prognosis.
A retrospective study was undertaken at two isolated Manitoba Indian communities to determine whether the type of infant feeding was related to infection during the first year of life. Of 158 infants 28 were fully breast-fed, 58 initially breast-fed and then changed to bottle-feeding and 72 fully bottle-fed. Fully bottle-fed infants were hospitalized with infectious diseases 10 times more often and spent 10 times more days in hospital during the first year of life than fully breast-fed infants. Diagnoses were mainly lower respiratory tract infection and gastroenteritis. Gastroenteritis occurred in only one breast-fed infant. Breast-feeding was strongly protective against severe infection requiring hospital admission and also against minor infection. The protective effect, which lasted even after breast-feeding was discontinued, was independent of family size, overcrowding in the home, family income and education of the parents. Measures to achieve breast-feeding for virtually all infants, particularly in northern communities, should be given high priority.
The newborn screening program for hereditary metabolic disorders in Manitoba is reviewed. In 1965, screening was begun on infants born in Metropolitan Winnipeg, and since January 1966 screening has been provincewide. Bloods from 85,868 infants have been screened so far. For the past two-and-a-half years 98.5% of live-born infants surviving the first seven days of life have been screened.
The Guthrie bacterial inhibition test was used initially. In 1966 an evaluation was undertaken of one-dimensional amino-acid paper chromatography, and in 1969 this method replaced the Guthrie test. Five cases of phenylketonuria have been identified, and incidence of 1:17, 174. Screening for abnormal sugars in the blood has disclosed two cases of galactosemia. The incidence of galactosemia in the province is 1 in 16,069 live births.
Three children with hypophosphatasia also had muscle pains, stiffness, and symptoms of proximal lower limb muscle weakness that occurred early in the disorder and were the presenting features in two. A non-progressive myopathy may be an important sign of hypophosphatasia.
Vitamin-D-deficient rickets still exists in children in Manitoba and adjacent areas. Between 1972 and 1984, 48 cases were documented at Winnipeg Children's Hospital. The patients ranged in age from 1 to 49 months; 40 were Canadian natives (38 Indians and 2 Inuit), most of whom lived in the Island Lake area of northern Manitoba. Of the 48, 16 had clinical signs of rickets, 12 had tetany due to hypocalcemia and 38 had radiologic evidence of rickets. Hypocalcemia was found in 27, and hypophosphatemia in 19; hyperaminoaciduria was found in 7 of 20. All 48 had elevated serum alkaline phosphatase levels. In addition to rickets, 16 patients aged 12 months or more had evidence of malnutrition. Climate and lifestyle in northern areas of the Canadian midwest result in little or no biosynthesis of vitamin D by solar radiation; therefore, adequate dietary vitamin D intake is essential to prevent deficiency. The diets of pregnant women and infants in these areas are deficient in vitamin D. The authors recommend vitamin D supplements for all pregnant women and infants in areas of risk to eradicate this preventable disease.
By sequential random numbering 10 schools in greater Winnipeg were selected for a nutrition survey. Interviews were conducted with 201 grade 3 children and 182 grade 6 children for whom parental consent was obtained. Of these, 48 in grade 3 and 51 in grade 6 were studied in further detail. There were no differences in descriptive data between the general and detailed groups or among the 10 schools. Most fathers were skilled or unskilled labourers and about 50% of the mothers were homemakers without outside employment; parental occupation did not influence eating patterns. Breakfast was the meal most often missed; 8% of the 383 children had come to school without breakfast. Since many children in grade 3 had prepared their own breakfast and since there was a relative lack of physical activity, school health programs should incorporate more than nutritional supplements and nutrition education. On the basis of body weight and height the nutritional status of the 99 children studied in detail was judged to be generally satisfactory; according to the Boston standards the boys were heavy and tall, and the girls were normal in weight but short.
The total dietary intake of energy and of individual nutrients of 99 grades 3 and 6 children from 10 greater Winnipeg shcools were generally comparable to those reported by Nutrition Canada for the Manitoba and national samples, although the percentile distributions of total caloric intake and dietary intake of vitamin A for the Winnipeg children tended to be lower. The median daily intake of protein was 212% of the Canadian Dietary Standard and most came from animal sources. Dietary fat was largely from animal sources as well. Hemoglobin concentrations were marginally low in four children, and urinary riboflavin:creatinine ratios were low in six children. There was no biochemical evidence of thiamin deficiency. The results suggest a need for change in dietary patterns and for education in nutrition, including relative nutrient/cost benefits. A well planned school snack program with an education component is a medium by which change could be introduced. This should preferable be part of a total school health program.