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Biomedical subjects

B Childs

Publications and source records attributed to B Childs.

At least 55 records · Page 3Linked to original sources
PubMed1987

Quality of care for life-supported children who require prolonged mechanical ventilation at home.

Home care is now possible for ventilator-dependent children. It is crucial, however, to ensure that the children's conditions are medically stable and that their home care is carefully planned before they are discharged. The Ventilator-Dependent Discharge Program at Children's Memorial Hospital in Chicago uses a comprehensive, multidisciplinary approach to discharge planning. The program includes training for parent caregivers, organization of community resources, and arrangement for funding.

Chicago↗
PubMed1985

The effect of Mendelian disease on human health: a measurement.

We describe an attempt to measure effects of Mendelian phenotypes on human health (homeostasis) in man. We used the McKusick Catalogs as the source for descriptions of autosomal dominant, recessive, and X-linked phenotypes. Three hundred and fifty one entries (76% of the initial sample) were disadaptive causing an impairment, disability, or handicap. (Terms used are in accordance with World Health Organization (WHO) definitions.) Phenotypic effects were scored to measure impact on life-span, reproductive capability, and psychosocial characteristics. We found 1) 25% of the disadaptive Mendelian phenotypes were apparent at birth and over 90% by the end of puberty; age at onset is unimodal in distribution for autosomal recessive and X-linked diseases, and trimodal for autosomal dominant (with modes during morphogenesis, infancy, and early adult life); 2) 58% of phenotypes involved more than one anatomical or functional system; autosomal dominants were more likely to involve only a single system; 3) life-span was reduced in 57%, particularly in those with onset in pre- or intra-reproductive life, and more often in recessive and X-linked diseases (data corrected for genetic lethals); prognosis varied with system involvement; 4) reproductive capability was impaired in 69% of phenotypes; and 5) most phenotypes compatible with life beyond infancy caused psychosocial handicap and limited the access to schooling and work. These findings have implications for medical care.

Adaptation, Psychological↗
PubMed1985

The effect of Mendelian disease on human health. II: Response to treatment.

We describe an attempt to measure efficacy of treatment in the Mendelian diseases of man. We used the McKusick Catalogs to identify 351 single gene diseases. We scored the impact of each disease in seven phenotypic categories: lifespan, reproductive capability, somatic growth, intellectual development, learning ability, capacity to work, and cosmetic effect. We then scored the success of treatment in ameliorating each of these component manifestations separately and together. The response to treatment was slight in the whole sample (n = 351): lifespan was increased in 15%, reproductive capability in 11%, and social adaptation in 6%. We observed that the mutant gene product was known in only 15% of the conditions comprising our sample. Since the mutant polypeptide is known in most inborn errors of metabolism, the diseases of this type (n = 65) in our sample of Mendelian traits were studied separately. In each of the seven categories of phenotypic impact, only a few of the hereditary metabolic diseases responded in any degree to specific treatment: the treatment gave complete relief in 12%, there was a partial response in 40%, and none in the remaining 48%. These findings have implications for prognosis, genetic counseling, and medical care of patients with Mendelian disease.

Adaptation, Psychological↗
PubMed1984

Crystalline fibril structure of type II collagen in lamprey notochord sheath.

We report here the existence of a crystalline molecular packing of type II collagen in the fibrils of the lamprey notochord sheath. This is the first finding of a crystalline structure in any collagen other than type I. The lamprey notochord sheath has a composition similar to that of cartilage, with type II collagen, a minor collagen component with 1 alpha, 2 alpha and 3 alpha chains, and cartilage-like proteoglycan. The high degree of orientation of fibrils in the notochord makes it possible to use X-ray diffraction to determine collagen fibril organization in this type II-containing tissue. The low angle equatorial scattering shows the fibrils are all about 17 nm in diameter and have an average center-to-center separation of 31 nm. These results are supported by electron microscope observations. A set of broad equatorial diffraction maxima at higher angles represents the sampling of the collagen molecular transform by a limited crystalline lattice, extending over a lateral dimension close to the diameter of one fibril. This indicates that each 17 nm fibril contains a crystalline array of molecules and, although a unit cell is difficult to determine because of the broad overlapping reflections, it is clear that the quasi-hexagonal triclinic unit cell of type I collagen in rat tail tendon is not consistent with the data. The meridional diffraction pattern showed 26 orders with the characteristic 67 nm periodicity found for tendon. However, the intensities of these reflections differ markedly from those found for tendon and cannot be explained by an unmodified gap/overlap model within each 67 nm period. Both X-ray diffraction and electron microscope data indicate a low degree of contrast along the fibril axis and are consistent with a periodic binding of a non-collagenous component in such a way as to obscure the gap region.

Animals↗
PubMed1984

Derivation and validation of a quantitative definition of specific reading disability for adults.

One hundred and fifty-four adult relatives of children with specific reading disability, and a group of 90 adults matched for age, sex, educational level and IQ, were given a battery of intelligence, reading and spelling tests and a questionnaire on their reading history, habits and attitudes. Data obtained from the latter group were used to derive multiple regression equations for the prediction of reading and spelling scores. An index was derived for each adult, expressing actual scores relative to those predicted. A definition of severe and borderline reading disability was validated, using the scores from the questionnaire.

Achievement↗
PubMed1983

Classification of spelling errors and their relationship to reading ability, sex, grade placement, and intelligence.

A simple scheme for the classification of spelling errors was applied to the errors of four groups of children, totaling 483 subjects, in grades 3 to 12. The subjects in two of the groups, Group I and Group IV, consisted of individuals who attended special schools for children with dyslexia or specific reading disability (SRD). Group II included school age siblings of subjects in Group I, and Group III included subjects drawn from regular school programs. It was shown that (a) type of spelling error is independent of sex, (b) there are no consistent effects of IQ or grade level on type of spelling error, and (c) disabled readers as a group are more likely to produce dysphonetic errors than are normal readers. Although the type of spelling error produced by children who had a spelling disability only was shown to be similar to that of normal readers and to differ from that of disabled readers as a group, disabled readers were shown to differ among themselves, lending strong support to the use of spelling error type as a characteristic for identifying subgroups.

Adolescent↗
PubMed1983

Risk factors in schizophrenia: season birth in Maryland, USA.

The association between the risk for schizophrenia and season of birth was studied using a standard actuarial life table method. This method of analysis eliminates the possibility of the previously described statistical artifacts from the data. An increased risk for schizophrenia for winter born individuals was demonstrated. The analysis also supported the hypothesis that season of birth is associated with an increased risk for a subtype of schizophrenia.

Actuarial Analysis↗