Showing papers in "Pediatrics in 1963"

A simple phenylalanine method for detecting phenylketonuria in large populations of newborn infants.

Abstract: A new method is described for rapid and economical screening of large numbers of hospital nursery infants for elevation in blood phenylalanine associated with phenylketonuria. Results are presented for 682 infants, 96% of whom were 4 days of age. None of the blood phenylalanine values were found to be as high as 4 mg/100 ml, and only 8% were above 2 mg/100 ml. These values appear to be in agreement with values obtained by other methods, and indicate that a very low rate of "false-positives" will be encountered during screening of the 10,000 or more infants that may be necessary to detect a case of phenylketonuria. It is recommended that any result of 6 mg/100 ml or above be considered positive, and require confirmation by phenylalanine determination of a second blood specimen.

Intrauterine growth as estimated from liveborn birth-weight data at 24 to 42 weeks of gestation.

Abstract: Percentile curves representing intrauterine growth of Caucasian infants in Colorado are constructed from the birthweights of liveborn infants at gestational ages ranging from 24 to 42 weeks. The median weights of Colorado babies were found to be lower at 40 weeks of gestation than the national median, and mean weights at 40 and 42 weeks were lower than those given by other authors. The use of the intrauterine growth chart in studying the nutrition of babies at birth and during their postnatal growth period has been described.

Exercising testing in adult normal subjects and cardiac patients.

Abstract: 1. Several approaches to assessment of physical work capacity in the upright posture in normal adults and cardiac patients have been reviewed critically. 2. Preliminary experience has been presented with a new multistage treadmill test of maximal exercise which permits appraisal of either a physically trained normal subject or an impaired but ambulatory cardiac patient. 3. The most effective means of differentiating cardiacs from normals has been an estimate of the total oxygen intake/Kg of body weight. 4. The need for monitoring the circulation with respect to arrhythmias, ischemia and/or hypotension has been emphasized. The authors wish to acknowledge their appreciation of many physicians, particularly Dr. Robert M. Levenson of the Seattle Cardiac Work Evaluation Clinic, and medical and graduate students who have assisted in the testing of patients and normals. The assistance of Mrs. Gladys Pettet is gratefully acknowledged also.

Milk allergy. i. oral challenge with milk and isolated milk proteins in allergic children.

Abstract: The diagnosis of milk allergy was confirmed in 89 children by oral challenge with milk and/or purified milk proteins. Most of these patients were diagnosed as allergic during the first 2 years of life. The symptoms of milk allergy usually began during the first few months of life. Eighteen of the total patients experienced spontaneous remission of milk hypersensitivity. Multiple symptoms were produced by oral milk challenge in 77% of the patients. Vomiting, diarrhea, abdominal pain, asthma, rhinitis, and atopic dermatitis were frequently presenting and challenge symptoms. Several patients had central nervous system symptoms, urticaria, or anaphylactic reactions following milk challenge. Seventy-eight per cent of the presenting symptoms were reproduced by oral milk challenges. Challenge feedings produced symptoms not found in the history in 18% of the patients. No consistent patterns of symptom combinations were found. The onset time of oral challenge reactions was usually within the first 12 to 24 hours. Some reactions occurred within a few minutes while others required 2 days or more before they were discernible. The duration of challenge reactions was usually between 12 to 24 hours. Allergic symptoms appeared much sooner after oral challenge in the patients who required less milk to elicit an allergic reaction. Forty-five patients were challenged with amounts of the purified milk proteins, casein, alpha-lactalbumin, beta-lactoglobulin, and bovine serum albumin, present in the challenge doses of 100 ml skim milk. Every patient had an allergic reaction to one or more milk proteins. The frequencies of reactions were casein, 57%; BSA, 51%; beta-lactoglobulin, 66%; and alpha-lactalbumin, 54%. The reactions from oral challenge to skim milk or purified milk protein in a given patient were very similar. No correlation was found between the symptoms produced and the specific protein which produced these reactions. By oral challenge tests few patients (13%) were allergic solely to the heat labile protein, BSA. Only two of these patients could be actually managed on a processed milk in which BSA was inactive by laboratory tests.

ROLE OF LINOLEIC ACID IN INFANT NUTRITION: Clinical and Chemical Study of 428 Infants Fed on Milk Mixtures Varying in Kind and Amount of Fat

Abstract: Four hundred and twenty-eight infants over a 4-year period were studied for approximately 4,000 patient-months of observation while receiving five different milk mixtures varying in linoleic acid content from less than 0.1 to 7.3% of the calories. It was found that linoleic acid is a required nutrient for the young human infant. The following pertinent observations were made. (1) Evidences of linoleic acid deficiency developed in young infants who received either a diet practically devoid of fat or one providing 42% of the calories as fat but extremely low in linoleic acid (less than 0.1% of the calories). (2) Manifestations of the deficiency state disappeared promptly when linoleic acid was given as the ester or triglyceride or in a milk mixture providing 1% or more of the calories as linoleic acid. (3) The most characteristic feature of the deficiency state was dryness of the skin with desquamation, thickening and later intertrigo. (4) The incidence of bacterial skin infections was the same in the different groups. It was noted that young infants receiving diets very low in linoleic acid seemed to react severely when outbreaks of staphylococcal infection developed in the hospital environment. (5) Records for rate of growth showed unsatisfactory progress for many of the infants on low linoleic acid intakes, whereas the course of events was satisfactory in almost all of the infants who received 1.3 to 7.3% of the calories as linoleic acid. (6) Blood serum levels for dienoic acid of 5.6 ± 1.8% of the total fatty acids were indicative of the deficiency state, whereas values of 12.9 ± 2.6% of the total fatty acids represented the minimal normal. (7) Histologic alterations in the skin of infants on diets low in linoleic acid showed the same characteristics as seen in experimental animals. (8) Infants given milk mixtures extremely low in linoleic acid had a gradual amelioration of fat deficiency manifestations as well as increasing levels of dienoic acid in the blood serum following the introduction of cereals to their diet.

Behavioral observations on parents anticipating the death of a child

Abstract: There are few tasks in the practice of medicine as difficult as trying to help the parents of a child afflicted with a disease which is invariably fatal. Since the physician cannot change the reality of the tragic situation, he frequently feels totally unable to lessen the parental suffering. However, understanding the nature of the stress as experienced by the parents, and appreciating that there are characteristic ways in which they cope with the situation, should enable the physician to offer helpful support in a majority of cases. Forty-six parents of children with neoplastic disease were involved at the National Institutes of Health (NIH) in a study of the adrenal cortical response under conditions of chronic psychological stress and this work has been reported elsewhere.1 The present paper is concerned with the clinical impressions gained over a 2-year period while this study was in progress and the implication of these findings to physicians caring for children with similar diseases, adding to what is presently in the litenature.2-8 SUBJECTS AND GENERAL METHOD OF STUDY The 46 subjects represented one or both parents of 27 children, all of whom had been referred for treatment with chemotherapeutic agents to the Medicine Branch of the National Cancer Institute. In all cases, the child had previously been hospitalized elsewhere for clinical evaluation, and the suggestion for referral was most frequently made by a physician at the time he communicated the diagnosis to the parents. In a minority of cases, the matter of referral was initiated at a time later in the child9s clinical course.

Low birth weight

Abstract: SINCE THE First World Health Assembly in 1948, the international definition of prematurity based on birth weight (≤ 2500 gm) has been used widely and successfully for purposes of vital statistics concerning newborn infants. This definition is objective and subject to standardization in contrast to a classification based on estimates of gestational age which are dependent upon the mother9s memory and menstrual regularity. On the other hand the taxonomic advantage of the birth weight criterion has tended to obscure the fact that fetal growth may be influenced by a number of factors; thus, size at birth is always an uncertain measure of the duration of gestation.

Reduction of mortality from respiratory distress syndrome of prematurity with early administration of intravenous glucose and sodium bicarbonate.

Abstract: In a controlled series evaluation of early administration of intravenous glucose and sodium bicarbonate, the mortality rate among 35 treated premature infants with respiratory distress syndrome was 17%, while among 35 controls it was 37% (p = 0.06). The mortality rate was similarly reduced among the total number of premature infants who have been treated by the author for this condition over the past five years: 24/118, or 20%, mortality with early intravenous therapy versus 38/94, or 42%, mortality with conventional therapy (p With this therapy for respiratory distress syndrome, the mortality rate among 736 premature infants consecutively delivered at two hospitals was exceptionally low—5.7% of infants weighing 1,001 to 2,500 gm. There was no evidence that the therapy operated to reduce mortality by lessening the respiratory distress process, which was equally severe among treated and untreated infants. The fluids administered to these infants did not produce heart failure or fluid overload. Sudden collapse which may have been due to reactive hypoglycemia sometimes resulted, however, when intravenous therapy was stopped before the infant was receiving adequate enteral feedings. It is concluded that early administration of intravenous glucose and sodium bicarbonate to premature infants with respiratory distress (hyaline membrane) syndrome can reduce the mortality rate by two-thirds among those who weigh more than 1,500 gm.

An electron microscope study of the fetal development of human lung

Abstract: Results of a study of lung ultrastructure in 13 human fetuses of about 1 to 6 months of gestation are reported. Light microscope controls were obtained in all cases. The columnar epithelial cells of young fetuses contained large deposits of glycogen which tended to become less abundant between the fourth and the sixth month. The fine structure of epithelial glycogen is described. A gradual transition between the columnar or cuboidal epithelium and the attenuated cytoplasmic lining of fully developed alveoli started during the fifth month, when the first contacts between capillaries and epithelium were established. Further differentiation of the epithelial cells in two morphologically different types, as observed in adult lung, took place during the sixth month. Both types appeared to have the same endodermal origin. Lamellar inclusions were first observed during the sixth month. Their significance is discussed in the light of recent work on alveolar surface activity.

The thymus and other lymphoid tissues in congenital agammaglobulinemia. i. thymic alymphoplasia and lymphocytic hypoplasia and their relation to infection.

Abstract: Patients with congenital agammaglobulinemia may be separated clinically and pathologically into two groups. One group manifests a persistent lymphopenia and is characterized clinically by an unremitting course marked by pneumonia, moniliasis, and other infections usually beginning in the first three months of life and, thus far, terminating fatally during the patient9s infancy. These patients have thymic alymphoplasia and lymphocytic hypoplasia of their tissues: the thymus is rudimentary, and the other lymphoid structures contain only sparse populations of small lymphocytes. The thymic alymphoplasia and general deficiency in small lymphocytes appear to be inherited characteristics and the evidence suggests that the primary defect is thymic alymphoplasia which results in generalized lymphocytic hypoplasia including lymphopenia. The second group of patients often has a later onset and a more intermittent course of bacterial infections, a variable leukocyte response marked only occasionally by transitory lymphopenia; their lymphoid organs including the thymus contain more nearly normal numbers of lymphocytes. Plasma cells and germinal follicles are absent from the tissues of both groups of patients, but the marked generalized paucity of small lymphocytes in these patients with thymic alymphoplasia results in an increased susceptibility to infection beyond that seen in nonlymphopenic agammaglobu-linemia. Whatever the exact contribution of the small lymphocyte to resistance to infection, it appears to be significant and its loss is not replaced by γ-globulin therapy.

Measures of body fat and hydration in adolescent boys.

Abstract: A study of total body fat by simultaneously performing a variety of measurements of adiposity on each of 66 adolescent boys is described. Estimates of total body fat by densitometry indicate a 50% loss of body fat9s contribution to total body weight from 12 to 18 years. Total body water, as measured by deuterium oxide, increases from 61% at age 12 years to 65% at age 18 years. Fat loss from this measurement closely parallels the fat changes estimated from densitometry. Lean body mass, hydration and adiposity appear to reach adult values at the sixteenth year. Subcutaneous fat measured by soft tissue x-rays films of the arm shows a similar fat loss, and of the same magnitude, when compared to densitometry and total body water estimates of fat. The triceps skinfold has a high correlation in estimating losses in fat during adolescence. The skinfold technique at this site provided a practical and accurate estimate of adiposity in adolescent boys.

Closure of the ductus arteriosus in the newborn infant.

Abstract: Hemodynamic observations were made in 71 normal newborn infants up to 60 hours of age. A right-to-left or bidirectional shunt through the ductus arteriosus was demonstrated in 8 of 63 infants. Evidence of a definite left-to-right shunt was found in 7 of 30 infants. The data indicate that a bidirectional shunt is present shortly after birth and may persist for as long as 6 hours. This eventually becomes entirely left-to-right, and by 15 hours of age the shunting of blood either becomes physiologically insignificant or ceases entirely.

The Relationship of Acute Bronchiolitis to Bronchial Asthma—A 4-to-14-year Follow-up

Abstract: Of 63 patients suffering a single attack of acute bronchiolitis in infancy, 16 (25.4%) were found to have bronchial asthma on follow-up 4 to 14 years later. The familial incidence of allergic manifestations was high (62%) in this group. Thirteen patients (20.6%) continued to have attacks of wheezing associated with respiratory infections. This tendency to wheeze gradually subsided after an average period of five years. The ultimate prognosis of these children is not known. The presence of a positive immediate family history of allergy in an infant with bronchiolitis considerably alters the usually excellent prognosis. The first attack of bronchiolitis may be a form of respiratory allergic manifestation in an already potentially asthmatic infant.

Spontaneous alveolar rupture at birth.

Abstract: Spontaneous pneumothorax in the newborn period was recognized in fifteen infants over a 6-year period. Seven of the 15 infants were symptomatic in the delivery room, which suggests that lung rupture occurred with the first few breaths. Three of the seven were meconium stained. The remaining eight infants were symptomatic within the first 24 hours. The unique features of the first breath of the newborn infant, namely, the serial opening of ventilatory units and high applied pressures, suggest that any foreign material in the airway would make rupture of opened units likely. Studies on the inflation of airless lungs demonstrate the opening phenomenon. Four of the 15 infants died, 2 from their pneumothoraces. This bad experience led us to examine principles of treatment of spontaneous pneumothorax with the recommendation that oxygen breathing in some term infants, and prompt aspiration in others would be useful. The majority, however, can be expected to resolve spontaneously. Measurements of the rate of absorption of air in the pleural cavity in the rabbit show a six-fold increase with 100% O2 breathing.

Familial erythrophagocytic lymphohistiocytosis.

Abstract: The clinical records and gross and histological findings are described in the case of two siblings who died at different times and at slightly different ages of an unusual, but apparently similar, disease complex. Letterer-Siwe9s disease was suspected clinically, but the autopsy findings characterized by hyperplasia of histiocytic cells, erythrophagocytosis, and an infiltration of lymphocytes and histiocytes into almost every organ in the body, including, in one of the children, the central nervous system, were distinctly different from the disease described by Letterer. Neither the etiology nor the nature of the disease is understood. Little or nothing is known about therapy though splenectomy in one of the two infants led to a temporary relief. Its similarity to other familial diseases of infancy designated by such terms as familial haemophagocytic reticulosis, familial nonlipid reticuloendotheliosis, familial generalized lympho-histiocytic infiltration, and possibly, Chediak-Higashi syndrome, is so striking as to suggest that these exceptional diseases may themselves comprise a distinct clinical and anatomical category. Until more is known about this group, the descriptive term "familial erythrophagocytic lympho-histiocytosis" is suggested.

NATAL AND NEONATAL TEETH Folklore and Fact

Abstract: The occurrence of natal and/or neonatal teeth is a rare anomaly, which for centuries has been associated with diverse superstitions among many different ethnic groups. The incidence of natal and/or neonatal teeth is far more frequent than previously supposed. Review of 359 recorded cases suggests an incidence of at least 1 in 3,000 births. Natal teeth are more frequent than neonatal teeth, the ratio being approximately 3 to 1. Rarely, a child will exhibit both natal and neonatal teeth. There appears to be no sex preference. Most frequently (ca. 85% of the cases), the natal or neonatal teeth are the deciduous mandibular incisors. In almost 90% of the cases, the teeth are of the normal deciduous complement. The rest are supernumerary. In about 60% of the cases, both of the natal and/or neonatal mandibular incisors erupt prematurely. Occasionally, a child may be born with a considerable number of his deciduous teeth erupted. Several such cases have been cited. About 70% of the natal and/or neonatal teeth are firmly fixed but a small number subsequently become loose. Similarly, a small fraction of those initially loose become fixed. Though the etiology is not known, approximately 15% had parents, siblings, or other near relatives with a history of natal and/or neonatal teeth. In several well-documented cases, the inheritance pattern has been that of an autosomal dominant trait. Three syndromes have been associated with natal teeth: ( a ) chondroectodermal dysplasia or Ellis-van Creveld syndrome, ( b ) oculo-mandibulo-dyscephaly with hypotrichosis or Hallermann-Streiff syndrome, and ( c ) pachyonychia congenita or Jadassohn-Lewandowski syndrome. Natal teeth may also be associated with cleft lip, cleft palate and cyclopia. Histologic investigation has revealed a failure of root formation despite eruption, a large vascular pulp, irregular genesis of dentin and a failure of cementum formation.

ANEURYSMAL MALFORMATION OF THE GREAT VEIN OF GALEN CAUSING HEART FAILURE IN EARLY INFANCY: Report of Five Cases

Abstract: Five cases of heart failure, resulting from a cerebral arteriovenous fistula and aneurysm of the great vein of Galen, are reported. In two infants the diagnosis was made roentgenographically; one improved after arterial ligation. In four cases, autopsy demonstrated the anomalous vessels and dilatation or hypertrophy of the heart. In three cases there was spongy necrosis of the brain. The pathogenesis of heart failure seems to be related to an enormous increase of the intake of the right atrium and ventricle. The cerebral damage seems to be due to ischemia caused by shunting of blood toward the veins bypassing the capillary network. Early diagnosis can be made by auscultation of the head and cerebral arteriography in infants who develop heart failure. Treatment is discussed.

Milk allergy. ii. skin testing of allergic and normal children with purified milk proteins.

Abstract: Skin testing separately and with equal concentrations of the purified milk proteins, casein, alpha-lactalbumin, beta-lactoglobulin, and BSA was done in 85 milk allergic children. For control purposes, intradermal tests with the same antigens were done in 102 normal children and 31 children allergic to non-milk substances. Six per cent of the normal children had weakly positive reactions. However, positive skin tests occurred in 68% of the children allergic, but not to milk, and in 59% of the children allergic to milk. In the milk allergic children, the incidence of positive reactions to each milk protein was similar. The incidence of positive skin tests in milk allergic children was higher than previously apprised. Positive skin tests in the milk allergic child were obtained long after the dietary elimination of milk antigens and after the disappearance of circulating milk antibodies as detected by a variety of techniques. In contrast to the control groups, strongly positive skin tests occurred only in milk allergic patients. Although no correlation was found between the results of all skin tests and the results of oral challenges with the same milk protein, a close correlation was found between the results of strongly positive skin tests and the results of oral challenge with the same milk protein. It appears that weakly positive skin tests with purified milk proteins have a little diagnostic value, but strongly positive skin tests have diagnostic significance.

Obstetric and pediatric complications of juvenile pregnancy

Abstract: This study analyzed the outcome of 636 pregnancies occurring in girls 14 years of age or younger delivered at the Johns Hopkins Hospital in the period 1936-60. 70 of these patients were white and 566 were nonwhite. Comparable data from the broader clinic population and for Baltimore City with regard to 15-19-year old nonwhite primiparous patients were collected as well. For the period 1951-60 when there was comparable reporting of perinatal deaths at the Johns Hopkins Hospital and the Baltimore City Health Department the perinatal mortality rates were 8.2% for nonwhites under age 15 at the hospital compared with 5.3% for all nonwhite deliveries in Baltimore City. When the under-15-year age group is compared with a 15-19-year age group of nonwhite primiparae the perinatal mortality rate is significantly higher for the younger patients. A higher incidence of toxemia contracted pelvis prematurity and immaturity was found in the study population than is contained in reports in the literature or in other clinical samples. For example the rate of infants with birthweights under 1000 gm was 5.1% in the study population compared with 2.3% for nonwhite patients at the hospital over 14 years of age. Such comparisons indicate that the higher rate of adverse outcomes of pregnancy recorded in the study population than in 15-19-year old nonwhite primiparous subjects is a result of age per se. It is concluded that very young primigravidas constitute a problem of obstetric and pediatric concern both with respect to their increasing numbers and the outcome of their pregnancies. The fact that this is one of the 1st studies to highlight this problem is attributed to the statistical dilution of very small series of young patients by statistics pertaining to outcomes in gestations of those in their later teens.

Staphylococcal antagonism to penicillin-G therapy of hemolytic streptococcal pharyngeal infection. Effect of oxacillin.

Abstract: Twelve pharyngeal specimens containing both group A hemolytic streptococci and coagulase-positive staphylococci were tested individually and together against penicillin-G and oxacillin. The hemolytic streptococci were always completely inhibited at very low concentrations of penicillin-G when tested individually. When combined with cultures of coagulase positive. penicillin-G destroying staphylococci, the inhibitory concentrations rose by a factor of 200. Similar tests with oxacillin showed no increase for inhibitory or bactericidal concentrations when the organisms were tested singly or in combination. Penicillin-G resistent staphylococci seemed to protect group A hemolytic streptococci from the action of penicillin-G. This was not the case when oxacillin was exhibited.

Antibodies to cow's milk proteins. Their presence and significance.

Abstract: A study was made of antibodies to cow9s milk proteins in the sera of 288 persons, mostly children. The sera were assayed for both precipitating and hemagglutinating antibody. Comparisons between the two systems are presented. The incidence of precipitins and/or elevated hemagglutinating titers was analyzed by age, nature of illness, and duration of disability. Chronically disabled children, as a group, had sigficantly higher titers than did normal or acutely ill children. Grouped patients with recurrent or chronic pneumonia, and Aldrich9s and Hurler9s syndromes, had higher titers than even the other chronically ill children. In the majority of cases it was not possible to attribute a pathogenetic role to the observed antibody. Whether or not these antibodies can be responsible for disease under special circumstances remains unknown.

Esophageal motility in infants and children.

Abstract: Patterns of esophageal motility are presented, and normal values are established in 47 normal infants and children between 2 days and 14 years of age The tone of the inferior esophageal sphincter mechanism is poor during the first 6 days of life, but increases significantly after the first week and through the first year of life In the majority of children the inferior esophageal sphincter mechanism is located at or above the level of the effective diaphragmatic hiatus, and an intra-abdominal segment cannot be demonstrated A progression of peristaltic waves from the esophagus into the fundus of the stomach is noted in children less than 2 years old This is not found in older children or in adults Inferior esophageal sphincter tone, relative to intragastric pressure, is poor in infants with regurgitation and with pyloric stenosis These occur rhythmically and measure 5 to 35 mm Hg in height

Hyperbilirubinemia in breast-fed infants.

Abstract: The case records of 11 infants with prolonged hyperbilirubinemia have been presented. The elevation of indirect reacting bilirubin in these infants may be due to interference with the normal conjugating mechanism by a substance present in breast milk. Introduction of cow9s milk formulas should be carried out as part of any extensive investigation of prolonged hyperbilirubinemia of the indirect type in otherwise well infants who have been wholly breast fed from birth.

Cortisol production rate in newborn babies, older infants, and children.

Abstract: The rates of cortisol production by 22 newborn infants were estimated. Vaginally delivered full-term and premature infants had rates, which when corrected for surface area, were comparable to those for adults. Babies delivered by cesarean section, when studied under 38 hours of age, had low rates. Two babies with salt-losing congenital adrenal hyperplasia had the lowest values. In the newborns the urinary 17-OHCS were low, and represented a small, variable fraction of the cortisol production. In a group of 14 older infants and children, the lowest production rates were observed in patients with adrenal calcification, moniliasis, hydrocephalus, and hypopituitarism. Administration of Wilhelmi growth hormone did not affect the production rates of the hypopituitary, or of the nonhypopituitary dwarfs.

Temperature regulation in exercise

Abstract: The central body temperature of a man rises gradually during the first half hour of a period of work to a higher level and this level is precisely maintained until the work is stopped; body temperature then slowly declines to the usual resting level. During prolonged work the temperature regulatory center in the hypothalamus appears to be reset at a level which is proportional to the intensity of the work and this setting is independent of environmental temperature changes ranging from cold to moderately warm. In hot environments the resistance to heat loss may be so great that all of the increased metabolic heat of work cannot be dissipated and the man9s central temperature will rise above the thermostatic setting. If this condition of imbalance is continued long enough heat stroke will ensue. We have found that in a 3 mile race lasting only 14 minutes on a hot summer day a runner9s rectal temperature may rise to 41.1°C., with heat stroke imminent. The physiological regulation of body temperature of men in warm environments and during the increased metabolic heat production of work is dependent on sweating to provide evaporative cooling of the skin, and on adjustments of cutaneous blood flow which determine the conductance of heat from the deeper tissues to the skin. The mechanisms of regulating these responses during work are complex and not entirely understood. Recent experiments carried out in this laboratory indicate that during work, sweating may be regulated by reflexes originating from thermal receptors in the veins draining warm blood from the muscles, summated with reflexes from the cutaneous thermal receptors, both acting through the hypothalamic center, the activity of which is increased in proportion to its own temperature. At the beginning of work the demand for blood flow to the muscles results in reflex vasoconstriction in the skin. As the body temperature rises the thermal demand predominates and the cutaneous vessels dilate, increasing heat conductance to the skin. Large increments in cardiac output and compensatory vasoconstriction in the abdominal viscera make these vascular adjustments in work possible without circulatory embarrassment.

Carcinoma of the colon in children.

Abstract: Two cases of primary adenocarcinoma of the colon are presented. Patient C.P. had a highly undifferentiated, signet ring type of adenocarcinoma and survived only 3 months. Patient P.G. had a well-differentiated adenocarcinoma with early, adequate resection of the lesion and has survived 8 years with no evidence of recurrence. The literature on carcinoma of the colon in children under 17 years of age is reviewed. There are twice as many boys with this disease as girls. Abdominal pain, vomiting, constipation, weight loss, blood in the stool, abdominal distension, and anorexia were common symptoms. The most common physical findings were the presence of an abdominal mass, abdominal distension and tenderness, anemia, and dehydration. These signs and symptoms are similar to those seen in adults with this disease. Approximately 50% of these children had a highly invasive, signet ring type of adenocarcinoma on microscopic examination. This type of carcinoma is rare in adults. Those patients with a signet ring type of adenocarcinoma have a very poor prognosis. However, the remaining 50% had well-dif-ferentiated adenocarcinoma where early diagnosis and adequate surgical resection could reduce the mortality.

The histidine requirement of the infant

Abstract: Histidine appears to be an essential amino acid for the young infant. Its omission from the diets of young infants gives rise to a depression of weight gain and of nitrogen retention. It also resulted in a dermatitis clinically and pathologically similar to infantile eczema, except for the absence of pruritus and atrophic changes in the sebaceous glands. Under the conditions of this study, the histidine requirement was less than 35 mg/kg/day in all six infants tested with this intake. Five infants were given a trial of 22 mg/kg/day; in three this figure was satisfactory, but in the remaining two there was some evidence of inadequacy. An intake of 16.6 mg/kg/day appeared to be adequate for one infant.

The vitamin B6 deficiency syndrome in human infancy: biochemical and clinical observations.

Abstract: A 14-month-old female infant with seizures, controlled by vitamin B6, was studied. Clinical and biochemical disturbances could be induced or corrected by manipulation of vitamin B6 intake. Seizures, hyperirritability, hyperacousis, and changes in growth rate comprised the important clinical manifestations. Biochemical disturbances affecting metabolism of tryptophan, cystathionine, and taurine were identified. Widespread impairment of PLP availability at apoenzyme sites is implied by the data. Elevated daily requirement for vitamin B6 were found, without excessive intestinal or renal loss of active vitamin, nor obvious vitamin antagonism. This suggested that a systemic abnormality of endogenous vitamin B6 metabolism, not restricted to brain, might account for the "deficiency" syndrome.

THE DIAGNOSIS OF MAPLE SYRUP URINE DISEASE (Branched-chain Ketoaciduria): BY THE IN VITRO STUDY OF THE PERIPHERAL LEUKOCYTE

Abstract: The metabolism of the three branched-chain amino acids has been investigated in vitro, using the peripheral leukocyte. The normal leukocyte can transaminate and decarboxylate the three amino acids. These functions are demonstrable at birth. Five cases of maple syrup urine disease (branched-chain ketoaciduria) were studied. The peripheral leukocyte could transaminate the three amino acids, but decarboxylation was greatly reduced or absent. This confirms the site of metabolic block in maple syrup urine disease, and suggests an early and specific approach to diagnosis. Oxidative-decarboxylation of the branched-chain ketoacids involves an enzyme common to all three ketoacids.

Sexual maturation of chinese girls in hong kong

Abstract: Three-thousand two-hundred seventy-eight school girls in Hong Kong, all of Southern Chinese origin, were carefully divided into high, middle, and low socioeconomic groups. The occurrence of menarche and the development of the breast, pubic hair, and axillary hair were investigated. The mean ages at which these traits of sexual maturation occurred were calculated by probit analysis. The various mean ages, in the order of high, middle, and low socioeconomic groups, are as follows: the first sign of breast development, 9.93, 10.22, and 10.65 years; the first appearance of the pubic hair, 11.63, 12.20, and 13.05 years; the menarche, 12.51, 12.75, 13.25 years; and the first appearance of axillary hair, 13.10, 13.45, and 13.74 years. The differences between these means of the three socioeconomic groups are all highly significant, as shown by the t-tests. Generally speaking, the breast develops 2½ years before menarche, the pubic hair appears half a year before menarche, and the axillary hair emerges half a year after menarche. The development of the breast and of the pubic hair were assessed according to the four-stage and the five-stage scores respectively, and the mean stages were calculated for both traits in each age group. Both the breast and the pubic hair showed steady advancement in the mean stages with age up to 19 or 20 years. When the girls of each age group were divided into groups according to whether they had or had not menstruated, the mean stages of both the breast and pubic hair development were always higher among girls who had menstruated. Comparison of the mean age of menarche with that in some other countries indicates that our girls are among the very earliest ones to mature. The possibility of the presence of a secular trend in the mean ages of attaining sexual maturation of Chinese girls is discussed.