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Biomedical subjects

B Y Whitman

Publications and source records attributed to B Y Whitman.

At least 19 recordsLinked to original sources

Leptin concentrations in Prader-Willi syndrome before and after growth hormone replacement.

OBJECTIVE: This study explored leptin concentrations in Prader-Willi syndrome (PWS), a genetic disorder characterized by significant obesity and presumed hypothalamic dysfunction. The potential interaction of leptin metabolism with the growth hormone (GH) axis was also studied. STUDY DESIGN: Plasma leptin concentrations and percent body fat were determined by radioimmunoassay and dual energy x-ray absorptionmetry, respectively, in 23 children with Prader-Willi syndrome and 23 children with exogenous obesity. RESULTS: Log plasma leptin concentrations were positively correlated with percentage body fat in PWS (r = 0.844) and exogenous obesity (r = 0.869). When the regression lines for the two groups were compared, there were no differences in their slopes (P = 0.737) or intercepts (P = 0.701). Administration of recombinant human growth hormone to PWS children for 12 months significantly reduced both percentage body fat and plasma leptin concentrations, but the relationship of log plasma leptin to percentage body fat was unchanged. CONCLUSION: Prader-Willi syndrome is not accompanied by deranged leptin concentrations and there was no evidence of an interaction of the GH axis with leptin metabolism in these GH-deficient children.

Body Composition↗

Sustained benefit after 2 years of growth hormone on body composition, fat utilization, physical strength and agility, and growth in Prader-Willi syndrome.

BACKGROUND: Obesity and hypotonia in children with Prader-Willi syndrome (PWS) are accompanied by abnormal body composition resembling a growth hormone (GH)-deficient state. Hypothalamic dysfunction in PWS includes decreased GH secretion, suggesting a possible therapeutic role for GH treatment. Although recent studies have demonstrated short-term benefits of treatment with GH, a critical question is whether beneficial changes persist or wane with prolonged therapy. OBJECTIVES AND METHODS: Effects of 24 months of GH treatment (1 mg/m(2)/d) on growth, body composition, strength and agility, pulmonary function, resting energy expenditure, and fat utilization were assessed in 35 children with PWS. Percent body fat, lean muscle mass, and bone mineral density were measured by dual-energy x-ray absorptiometry. Indirect calorimetry was used to determine resting energy expenditure and to calculate the respiratory quotient. RESULTS: Compared with baseline evaluations, increased height velocity (SD score -1.1 +/- 2.5 to 2.2 +/- 2.3; P <. 001), reduced percent body fat (46.4% +/- 8.4% to 40.3% +/- 10.0%, P <.001), and improved respiratory muscle function and physical strength and agility (sit-ups, weight-lifts, running speed, and broad jump; P <.01) were observed after 24 months of GH treatment. A decline in respiratory quotient (0.81 +/- 0.07 to 0.75 +/- 0.06; P <. 01) and a trend toward increased resting energy expenditure were also observed. Changes in response to GH occurred predominantly during the initial 12 months of GH therapy. CONCLUSIONS: Children with PWS had sustained increases in lean body mass, decreases in percent body fat, improvements in physical strength and agility, and increased fat oxidation after 24 months of GH therapy. However, between 12 and 24 months, the growth rate slowed. Consequently, encouraging initial results require even more prolonged study to draw conclusions regarding the long-term value of GH therapy in changing body composition in children with PWS.

Adipose Tissue↗

Growth hormone improves body composition, fat utilization, physical strength and agility, and growth in Prader-Willi syndrome: A controlled study.

BACKGROUND: Obesity and hypotonia in children with Prader-Willi syndrome (PWS) are accompanied by abnormal body composition and diminished energy expenditure resembling a growth hormone deficient state. Hypothalamic dysfunction in PWS often includes decreased growth hormone (GH) secretion, suggesting a possible therapeutic role for exogenous GH treatment. OBJECTIVES AND METHODS: After 6 months of observation to determine baseline growth rate, and with the use of a 12-month randomized controlled study design, the effects of GH treatment (1 mg/m2/d) on growth, body composition, strength and agility, pulmonary function, resting energy expenditure (REE), and fat utilization were assessed in 54 children with PWS (n = 35 treatment and n = 19 control). Percent body fat and bone mineral density were measured by dual x-ray absorptiometry. Indirect calorimetry was used to determine REE and to calculate respiratory quotients. RESULTS: Stimulated levels of GH in response to clonidine testing were low in all patients (peak, 2.0 ng/mL). After 12 months, GH-treated subjects showed significantly increased height velocity Z scores (mean, 1.0 1.7 to 4.6 2.9; P <.001), decreased percent body fat (mean, 46.3% 8.4% to 38.3% 10.7%; P <.001), and improved respiratory muscle function, physical strength, and agility (sit-ups, weight-lifts, running speed, and coordination). A significant decline in respiratory quotients occurred during GH therapy (0.81 to 0.77, P <.001), but total REE did not change. CONCLUSIONS: GH treatment of children with PWS accelerated growth, decreased percent body fat, and increased fat oxidation but did not significantly increase total REE. Improvements in respiratory muscle strength, physical strength, and agility also occurred, suggesting that GH treatment may have value in reducing some physical disabilities experienced by children with PWS.

Adipose Tissue↗

Physical effects of growth hormone treatment in children with Prader-Willi syndrome.

A randomized, controlled study of 54 children (age, 4-16 years) with Prader-Willi syndrome was conducted to assess the potential beneficial effects of growth hormone (GH) treatment. After observation for 6 months, the children were randomized to receive GH at a dose of 3 IU/m2/day (1 mg/m2/day) (n = 35) or no intervention (n = 19). The effects of GH treatment on linear growth, body composition, muscle strength, pulmonary function and resting energy expenditure were assessed. The levels of GH secreted in response to clonidine stimulation were universally low, and mean (+/- SD) insulin-like growth factor I SDS was -1.2 +/- 0.8 pretreatment. In children treated for 1 year, mean height velocity SDS significantly increased from -1.0 +/- 2.5 to 4.6 +/- 2.9 (p < 0.0001), mean percentage body fat decreased from 46.3 +/- 8.4% to 38.4 +/- 10.7% (p < 0.001), mean lean body mass increased from 20.5 +/- 6.3 kg to 25.6 +/- 4.3 kg (p < 0.01) and respiratory muscle function and physical strength improved. Mean respiratory quotients significantly decreased from 0.81 to 0.77 (p < 0.001); however, resting energy expenditure did not change. Therefore, GH therapy appears to reduce some of the physical disabilities experienced by children with Prader-Willi syndrome.

Adolescent↗

Early ear problems and developmental problems at school age.

Retrospective history of middle ear disease was compared with developmental diagnosis in 507 consecutively referred school-age children. History of major ear problems was positively associated with discrepancies between the performance and verbal IQ on the WISC-R. History of major ear problems was positively associated with the presence of articulation disorders for children in the low social class, hyperactivity in the middle social class, and language problems in the high social class. A history of significant middle ear disease in early childhood should raise concerns for articulation difficulties and possible language problems in children presenting to clinicians with school problems.

Adolescent↗

Maternally reported fetal activity levels and developmental diagnoses.

Retrospective maternal report of fetal activity level was compared with developmental diagnosis in 608 consecutively referred children. Maternal history of fetal activity level was also obtained from 140 unmatched well children in a general pediatric clinic. Fetal hyperactivity was positively associated with a diagnosis of child hyperactivity, and fetal hypoactivity was positively associated with a diagnosis of mental retardation in the children. Maternal histories of fetal activity level in the control group weakened the strength of the association between fetal hyperactivity and child hyperactivity but did not affect the association between fetal hypoactivity and mental retardation in children.

Apgar Score↗

Prader-Willi syndrome: consensus diagnostic criteria.

The diagnosis of Prader-Willi syndrome (PWS) is based on clinical findings that change with age. Hypotonia is prominent in infancy. Obesity, mild mental retardation or learning disability, and behavior problems, especially in association with food and eating, result in a debilitating physical and developmental disability in adolescence and adulthood. No consistent biological marker is yet available for PWS in spite of recent research activity in cytogenetics and molecular genetics. Diagnostic criteria for PWS were developed by consensus of seven clinicians experienced with the syndrome in consultation with national and international experts. Two scoring systems are provided: one for children aged 0 to 36 months and another one for children aged 3 years to adults. These criteria will aid in recognition of the syndrome in hypotonic infants and in obese, mildly retarded, behaviorally disturbed adolescents and adults. They will also ensure uniform diagnosis for future clinical and laboratory research in PWS.

Adolescent↗

Minor malformations, hyperactivity, and learning disabilities.

Standardized minor malformation scores have been reported to predict and identify children with attentional problems and hyperactivity. The reason why this marker works for only a subset of children with attention deficit hyperactivity disorder remains unclear. The dysmorphology scores on all children presenting for a multidisciplinary team evaluation of developmental disorders were examined for diagnostic correlations after children with chromosomal disorders and recognized dysmorphic syndromes were excluded. For 1233 subjects, the mean minor malformation score was 2.94 (SD = 2.05). A significant association between minor malformation scores and IQ (mean = 80.95, SD = 23.67) was accounted for by the group with IQs greater than 100 exhibiting the higher dysmorphology scores. An analysis of variance revealed no significant association between minor malformation scores and hyperactivity or attention deficit disorder. Indeed, the presence of an attention deficit disorder yielded lower mean dysmorphology scores. When the minor malformation scores were compared for those subgroups of children with and without specific learning disabilities, the learning-disabled subjects had significantly higher dysmorphology scores. Minor dysmorphic features do not relate to the presence or absence of attentional problems or hyperactivity in referred children. Rather they appear to characterize that subpopulation of children with attention deficit disorder and learning disabilities as well as a group of learning-disabled children without attentional disorders.

Adolescent↗

Multiple personality disorder: a risk indicator, diagnostic marker and psychiatric outcome for severe child abuse.

Recent studies suggest that multiple personality disorder (MPD) is not as rare as previously believed. Indeed, it may represent a relatively common (as many as 25% of cases) outcome of severe physical and sexual abuse of children. Because diagnosis and treatment offer the best prognosis, it is important for the pediatric clinician to become familiar with both the at risk situations (such as cult practices and multigenerational sexual abuse) as well as the more unusual behavioral presentations of this disorder.

Adolescent↗

Autistic disorder in Sotos syndrome: a case report.

A case study of a child with Sotos syndrome, normal intelligence, and autistic disorder is presented. Initial descriptions of Sotos syndrome included severe to mild mental retardation. More recent studies indicate language and learning disabilities with normal intelligence. Our patient met criteria for a diagnosis of autistic disorder. Sotos syndrome is another genetic and neurodevelopmental syndrome that can be associated with autistic as well as communication and language disorders.

Autistic Disorder↗

Homelessness and cognitive performance in children: a possible link.

The developmental impact of homelessness on children was assessed by using a screening battery to measure cognitive and language delays as well as emotional status. The results of testing 88 children in a family shelter program indicate a possible significant impact of homelessness on both the cognitive and language development of these children, with language more severely affected. Emotional pathology, however, was not detected with the screening instruments used. Most service providers who deal with homeless children fail to recognize their special programming needs. It is important for the social worker to advocate with the educational system for these children's special needs.

Child↗

Disorders of attention and activity level in a referral population.

Of 614 children referred for an evaluation of hyperactivity and inattention, the 422 (68.7%) who qualified for a diagnosis of attention deficit disorder (ADD) were compared with the 192 (31.3%) who did not. The children with ADD had significantly higher full-scale IQ scores, verbal scores, and parental educational levels. They also had a significantly higher incidence of specific learning disabilities (73.7%). The absence of hyperactivity in children with ADD further exaggerated the differences in learning disability between the two groups. Cognitive limitation, severe parental psychopathology, and child neglect/abuse were significantly higher in the group without ADD. The two groups did not differ in the incidence of parental or child depressive symptomatology, motor diagnoses, language disorders, conduct disorders, and psychosomatic complaints. That only 29 children in whom ADD was absent (4.7% of all referrals) had been given a trial of stimulant medication does not support the presence of a major abuse of such drugs in the community.

Adolescent↗

Emotional symptoms in Prader-Willi syndrome adolescents.

Clinical observations and parental reports on the behavior of Prader-Willi syndrome (PWS) patients suggest the development of a wide variety of psychiatric disorders as the PWS child enters adolescence. Documentation of these emotional disorders remains unsystematic. Here we describe the results of administering the Survey Diagnostic Instrument (SDI) to the parents of 35 PWS adolescents. The questionnaire data were supplemented by additional selected demographic and clinical data. The SDI is a 134 item questionnaire filled out by one parent. It screens for the DSM-III criterion-based diagnostic categories of neurosis (dysphoric, compulsive, anxious), somatization, conduct disorder (antisocial, violent), and hyperactivity. The following diagnostic pattern resulted: neurosis, dysphoric, (1 probable); neurosis, compulsive, 3 (6 probable); neurosis, anxious, 4 (and 10 probable); somatization, 0; conduct disorder, violent 0; conduct disorder, antisocial, 0; hyperactivity, 1 (and 1 probable). An odds ratio algorithm is used to uncover possible predisposing factors, and the results are discussed in the context of organic versus psychiatric causes.

Adolescent↗

Mentally retarded parents in the community: identification method and needs assessment survey.

Mentally retarded parents in the community represent a relatively new and increasing population. Due to the parents' limitations, the children are at significant risk for medical, emotional, and cognitive delays and neglect. An estimate of the size of this population that comes to agency attention is needed in order to program supportive services. The methodology and results of a community-based survey to determine the size of this population receiving agency services in a large metropolitan area are described as are the results of a survey interview with a subset of these parents and their children to determine services needed from the parents' perspective.

Child↗

Association of alcoholism and antisocial personality in a narcotic-dependent population: the Lexington addicts.

Men with antisocial personality have a higher prevalence of alcoholism than men without. This relationship was examined in a narcotic-dependent sample, and it was found that narcotic-dependent men with antisocial personality had a rate of alcoholism comparable to that of narcotic-dependent men without antisocial personality. The methodological and clinical explanations for this finding are discussed along with the neurophysiological implications.

Adult↗

The use of epidemiologic methods as the bridge between prevention and social work practice.

The concepts, scope and methods of epidemiology as well as the application of these tools to preventive social work practice for both policy setting and case management are described. Using data concerning lead poisoning, two areas are discussed. These are: 1) the stages of prevention and its implication for practice, and 2) definitions of the epidemiologic concepts of population-at-risk, prevalence, incidence, case rate, relative risk, attributable risk, and epidemiologic causal models. Finally, suggestions for using epidemiologic knowledge and methods in formulating policy for, and programs pertaining to, prevention in social work practice are set forth.

Child↗