Cutis marmorata telangiectatica congenita and neonatal ascites

Six children with Prader-Willi syndrome were studied in order to assess their plasma immunoreactive insulin and growth hormone levels in responses to oral glucose, protein-glucose meal, and intravenous l-arginine. Their responses were almost identical to those observed in children with long-standing obesity: marked hyperinsulinemia, blunted growth hormone responses, normal plasma glucose levels, and high fasting levels of free fatty acids. The plasma decrements in free fatty acids during administration of the different stimuli were, in general, similar to those of normal or obese children. It is suggested that the described hormonal changes are related to the obesity of children with Prader-Willi syndrome and by no means represent a characteristic hormonal profile of the syndrome, per se. Six children with Prader-Willi syndrome were studied in order to assess their plasma immunoreactive insulin and growth hormone levels in responses to oral glucose, protein-glucose meal, and intravenous l-arginine. Their responses were almost identical to those observed in children with long-standing obesity: marked hyperinsulinemia, blunted growth hormone responses, normal plasma glucose levels, and high fasting levels of free fatty acids. The plasma decrements in free fatty acids during administration of the different stimuli were, in general, similar to those of normal or obese children. It is suggested that the described hormonal changes are related to the obesity of children with Prader-Willi syndrome and by no means represent a characteristic hormonal profile of the syndrome, per se.

Extract: Levels of plasma insulin, growth hormone (HGH), glucose, and free fatty acids (FFA) were investigated in 17 obese, nondiabetic children or adolescents after administration of intravenous arginine, oral glucose, and a protein-glucose meal. These results were compared with those obtained in eight normal adolescents. Four of the obese patients had a family history of diabetes but had hormonal responses identical with those observed in obese subjects with a negative family history of diabetes. Subjects were arbitrarily separated into two groups depending on duration of obesity (Table I) (age of onset was different: infancy or early childhood for group B, 5–8 years for group A). Subjects in group B (longstanding obesity) all had a strong family history of obesity with marked increases in body weight, body fat, and excess fat. Fasting levels of insulin were greater than normal (P < 0.001 (Table II)). Patients in group A (short duration), with lesser increments in body weight and body fat, had normal fasting levels of insulin and normal response to intravenous arginine (Fig. 4). All obese subjects had hyperinsulinemia in response to other stimuli, but patients in group B achieved the highest insulin levels (Figs. 2, 3, and 4). All obese patients had significantly decreased levels of HGH during tests and elevated fasting levels of FFA. Patients in group B exhibited a reduced mean decrement in FFA during the oral glucose tolerance test. Whether or not responses seen in patients in group A represent an early stage of the conditions observed in patients in group B cannot be determined by this study, but the biochemical changes reported in established obesity of childhood mimic those found in the adult. Speculation: Obesity in children is associated with excess lipogenesis and hyperinsulinemia, which are interdependent and initiated through maximal nutritional intake in infancy. Eventually muscle becomes resistant to insulin with decreased transport of glucose into the cell and decreased protein synthesis relative to DNA. The reduced levels of growth hormone in the circulation may indicate excessive uptake by muscle, which is followed, in some instances, by an associated proliferation of nuclei.

Extract: In 14 obese females and 9 obese males, body length was used as a base line for the detection of excess growth of lean and adipose tissue.Eighteen patients had increased lean body mass based on body water determinations (fig.1), and 40K was found to be an unreliable diagnostic agent for the prediction of lean body mass. A similar number of patients had excessive amounts of intracellular mass (fig.2). Males had an increment in total protein content within the adipose tissue mass (table IV), whereas half of the females (and all those possessing advanced bone age) had increments in muscle mass (fig.3) and in the DNA content of their musculature (fig.4). A sufficient number of female patients were studied to document two classes of obesity—one group with, and one group without advanced maturation, excess nuclei (fig.4), and reduced protein/DNA (fig.5) in the musculature.Studies of noncollagen protein in adipose tissue mass of male subjects indicated an increase in the number of adipocytes compared with those found in normal or obese females (table IV). In addition, protein and water concentrations of adipose tissue were reduced in all the obese patients studied (table III). The extracellular volume was found to be constant at 17% of body weight in obese adolescent males.Speculation: Obesity in adolescents is the result of genetic, environmental, nutritional, and hormonal factors. Over-nutrition in infancy influences hormonal secretion and produces advanced maturation and excessive cell growth in muscle (as found in this study in one group of obese females). Also, in one group of obese females, estrogens, which retard cell number increase (in the normal pubertal female), are possibly suppressed by androgens and thus allow androgen and growth hormone to exert maximal effects at the muscle level (increase of cell number).In the obese male, the superimposition of high levels of circulating insulin on androgen secretion enhances the growth of collagen and of adipocytes in the adipose tissue mass. Thus, the particular changes in body composition occurring in obesity are dependent on the sex of the individual.

The clinical characteristics and predicted body composition of ten obese nondiabetic and six normal adolescents were compared in regard to plasma insulin, growth hormone, glucose, free fatty acids and alpha-amino nitrogen in response to different stimuli. Analysis of the data suggested the presence of two groups of patients. In obese Group A there was no family history of obesity, the youngsters first became obese between seven to twelve years of age, had a normal height for age, moderate increase in body weight and estimated total body fat, and normal or less than normal fasting levels of insulin. In obese Group B there was a strongly positive family history of obesity, the subjects became obese in infancy or early childhood, they were tall for their age, had marked increase in total body weight and total body fat as well as significantly higher than normal fasting insulin levels. Hyperinsulinemia was present in Group A during the oral glucose tolerance test and protein glucose meal. Normal insulin responses were observed during the protein meal and the arginine tolerance test. On the other hand, Group B manifested hyperinsulinemia in response to all stimuli. Both obese groups had undetectable plasma growth hormone levels during both the protein and the protein-glucose meal, but normal growth hormone responses during the oral glucose and the arginine tolerance tests. The differences in plasma levels of glucose and free fatty acids were minimal. Significant differences in plasma alpha-amino nitrogen values were observed during the protein-glucose meal. Differences in the hormonal responses of obese adolescents and adults are discussed. Possible explanations for heterogeneous responses of obese adolescents are presented.

Journal Article Influence of Epinephrine-Propranolol Infusions on Growth Hormone Release in Normal and Hypopituitary Subjects Get access A. PARRA, A. PARRA 1The Department of Pediatrics, The Johns Hopkins University School of Medicine, The Johns Hopkins Hospital Baltimore, Maryland 21205 Search for other works by this author on: Oxford Academic Google Scholar R. B. SCHULTZ, R. B. SCHULTZ 1The Department of Pediatrics, The Johns Hopkins University School of Medicine, The Johns Hopkins Hospital Baltimore, Maryland 21205 Search for other works by this author on: Oxford Academic Google Scholar T. P. FOLEY, JR., T. P. FOLEY, JR. 1The Department of Pediatrics, The Johns Hopkins University School of Medicine, The Johns Hopkins Hospital Baltimore, Maryland 21205 Search for other works by this author on: Oxford Academic Google Scholar R. M. BLIZZARD R. M. BLIZZARD 1The Department of Pediatrics, The Johns Hopkins University School of Medicine, The Johns Hopkins Hospital Baltimore, Maryland 21205 Search for other works by this author on: Oxford Academic Google Scholar The Journal of Clinical Endocrinology & Metabolism, Volume 30, Issue 1, 1 January 1970, Pages 134–137, https://doi.org/10.1210/jcem-30-1-134 Published: 01 January 1970 Article history Received: 14 May 1969 Published: 01 January 1970

Eighteen studies were performed in 5 grossly obese adolescents in order to determine whether or not epinephrine and/or Propranolol are capable of decreasing the hyperinsulinemia characteristic of obese subjects and to study the metabolic response during the administration of these drugs. When epinephrine (6 μg./min.) was infused during a protein-glucose meal, there was a 50 per cent (or greater) decrease in the release of immunoreactive insulin as compared with the response to protein-glucose alone. However, the basal level of immunoreactive insulin was unchanged. There were also a marked hyperglycemia as well as a concomitant rise in the plasma free fatty acids. Plasma level of immunoreactive human growth hormone were undetectable. Epinephrine (10 μg./min.) in combination with Propranolol (80 μg./min.) resulted in a marked decrease in both the basal level and the subsequent release of immunoreactive insulin. Hyperglycemia was present and the plasma free fatty acids showed a precipitous fall. One patient had a brisk immunoreactive growth hormone response of 7 mμg./ml. after cessation of the infusion. When epinephrine (2 μg./min.) was simultaneously infused with Propranolol (80 μg./min.) only a slight decrease in the basal level of immunoreactive insulin was observed. The inhibition of the subsequent insulin release was mild and transitory. Again hyperglycemia occurred and the plasma levels of immunoreactive growth hormone were almost undetectable. Propranolol alone (80 μg./min.) did not affect the basal level or the subsequent release of immunoreactive insulin. One subject had a peak plasma immunoreactive growth hormone value of 5.9 mμg./ml. shortly after the infusion began. It is concluded that Propranolol and epinephrine in specific dose combinations have a synergistic action on the inhibition of insulin release in obese adolescents.

Placental lactogen (1), a hormone with somatotropic, luteotropic and lactogenic activities and which is secreted by the human placenta, has been reported to have growth hormone-like activity (2). Elsewhere in this Journal a study comparing the effects of animal prolactins with human growth hormone is reported (3). Similar studies were undertaken to determine the metabolic effects of placental lactogen as compared to human growth hormone.