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Biomedical subjects

D P Wilson

Publications and source records attributed to D P Wilson.

At least 19 recordsLinked to original sources

Hypothyroxinemia of prematurity: rite of passage or therapeutic necessity?

Hypothyroxinemia is a common finding in premature infants, presumably resulting from an immature hypothalamic-pituitary-thyroid axis. Because dynamic studies of thyroid function in premature infants are normal and the condition resolves spontaneously, HOP has been considered physiologic rather than pathologic. Thus, thyroid hormone supplementation has been assumed to be not required in premature infants. True hypothyroidism of hypothalamic pituitary or thyroid origin, however, does occur in premature as well as in term infants and should be investigated aggressively and treated appropriately. Current studies in premature infants with hypothyroxinemia suggest the following: infants with more than 27 weeks of gestation do not appear to benefit and may, in fact, be harmed by thyroid hormone supplementation; and short-term thyroid hormone supplementation in infants born before 27 weeks of gestation may be important to diminish morbidity and to improve neurodevelopmental outcome.

Deficiency Diseases↗

Angiotensin II receptor antagonists prevent neointimal proliferation in a porcine coronary artery organ culture model.

OBJECTIVES: Angiotensin II (AngII) generation in response to vascular injury has long been assumed to influence neointimal proliferation contributing to restenosis. This concept has been supported by evidence that ACE inhibition and AT1 receptor blockade effectively limits restenosis in the rat. On the other hand, ACE inhibition has proven ineffective in clinical trails. The present study examines the response of the porcine coronary artery after angioplasty in vitro and compares the actions of an ACE inhibitor to AngII receptor antagonists. METHODS AND RESULTS: Captopril, an ACE inhibitor, and the AngII receptor antagonists, losartan and PD123319, were evaluated for their ability to attenuate neointimal proliferation in a porcine organ culture model of coronary restenosis. The neointima was significantly increased by 300% after angioplasty compared to non-angioplasty controls. The AT1 receptor antagonist, losartan, produced a significant reduction in neointimal index at 10(-5) mol/l, while its in vivo metabolite, EXP3174, reduced neointimal proliferation at 10(-6) mol/l. PD123319, a selective antagonist of the AT2 receptor, also restricted neointimal proliferation at 10(-5) mol/l. Treatment with captopril (10(-6) mol/l) increased the neointimal proliferation by approximately 200% after angioplasty. CONCLUSIONS: Direct blockade of AngII receptors effectively inhibits cell proliferation and restenosis post-angioplasty in vitro. ACE inhibition, exclusive of flow, does not attenuate proliferative restenosis. These data suggest that AngII contributes to neointimal proliferation and validates the concept that receptor antagonists could contribute to the therapeutic management of restenosis.

Analysis of Variance↗

Growth hormone improves weight velocity and height velocity in prepubertal children with cystic fibrosis.

We undertook this study to determine if growth hormone treatment of prepubertal children with cystic fibrosis could improve their height and weight. Nine prepubertal children with cystic fibrosis were treated with human recombinant growth hormone for one year. Results obtained during this year were compared to similar measurements made for each patient for the one year prior to the treatment year. Anthropometric data including: height, height velocity, weight, weight velocity and skin fold thickness were measured at three month intervals. Pulmonary function and skeletal muscle strength were measured at three month intervals. Glucose tolerance was evaluated by HbAlc and by fasting blood glucose and insulin levels every three months. Our results demonstrate that growth hormone treatment resulted in significant improvement in height velocity and height Z scores. Weight increased in all subjects, with a significant increase in weight velocity (year prior to treatment = 1.7+/-1.0 kg/yr, treatment year = 3.8+/-1.6 kg/yr; p=0.03). Measurements of skin fold thickness suggests that lean body mass improved with growth hormone treatment. Pulmonary function improved in all but two patients, whose pulmonary function remained the same and muscle strength improved in all subjects. These results suggest that growth hormone used in prepubertal children with cystic fibrosis can improve height and weight and may improve lean body mass.

Autoantibodies↗

FH Tulsa-1 and -2: two unique alleles for familial hypercholesterolemia presenting in an affected two-year-old African-American male.

A two-year-old African American boy presented with cutaneous xanthomata and extreme hypercholesterolemia. Subsequent studies revealed that the LDL-cholesterol was 1,001 mg/dl and apoB 507 mg/dl. LDL-receptor activity was almost undetectable, which is compatible with the finding of two newly described defective alleles on exon 4 of the LDL-receptor gene coding for part of the ligand-binding domain. One allele contained a 21 base-pair insertion from codon 200 to 207 whereas the other had a point mutation at codon 207. The rarity of genes for FH reported in individuals of African ancestry is discussed.

Alleles↗

How short is too short?

A review of growth parameters should be a routine part of pediatric care. Children and parents need to be helped in understanding normal growth and development. Variations in growth should be explained, and concerns of parents and children openly discussed. Age appropriate educational materials are often helpful. Children with abnormal growth should be referred promptly for definitive diagnosis, selection of treatable candidates, and monitoring.

Adolescent↗

Lipids and apolipoproteins in growth hormone-deficient children during treatment.

The role of growth hormone (GH) in regulating the transport of plasma lipoproteins has not been clearly defined, but past studies suggest that GH may influence cholesterol levels. This protocol was designed to evaluate possible changes in lipid and apolipoprotein status in GH-deficient children and children with neurosecretory dysfunction (NS) before GH therapy and at intervals after GH therapy was started. Twenty children with classic GH deficiency were evaluated, and 28% were hyperlipidemic at the onset of the study. Seven children were evaluated in the NS group, and only one (14%) showed an elevated total cholesterol (TC) greater than 200 mg/dL. The mean TC for all the GH-deficient children was elevated above the normal range, but not for the NS group. The mean apolipoprotein (apo) C-III level and its heparin-precipitated fraction (HP) were also elevated in the GH-deficient group, but only the apo C-III HP was elevated in the NS group. A standard replacement dose of recombinant methionyl GH was used, and therapy had no significant effect on TC or triglyceride (TG) levels. Apo C-III HP, a marker of hypertriglyceridemia, increased after the start of therapy, but no other lipoprotein levels changed significantly in the GH-deficient group. No changes were seen with treatment in the NS group. The longitudinal design of this study allowed demonstration of the later changes in the apolipoproteins and the presence of a distinct subset of patients with both GH deficiency and hypercholesterolemia. This study supports the role of GH in modulating lipid metabolism.

Adolescent↗

Human growth hormone and Creutzfeldt-Jakob disease.

For more than 20 years cadaver-derived human growth hormone (HGH) was used successfully to enhance linear growth in short children. In 1985 the US Food and Drug Administration (FDA) stopped use of the hormone in response to reported deaths due to Creutzfeldt-Jakob (CJD) agent in 3 former HGH recipients. To date, a total of 9 patients have been identified who both received HGH and became infected with CJD agent (7 in the United States, 1 in Britain, and 1 in New Zealand). Circumstances make it likely that HGH contaminated with a slow growing, viral-like particle may have been responsible for these fatalities. In Oklahoma at least 60 children and adolescents previously received HGH and are potentially at risk of developing CJD. It is important that health care providers responsible for the care of these individuals be aware of this fatal illness and remain informed of new developments in the field.

Adolescent↗

Growth hormone: past, present, and future.

Recent progress in the diagnosis and therapy of IGHD illustrates the ever-increasing influence of recombinant DNA research. Recombinant DNA technology has already resulted in the synthesis of therapeutic growth hormone that is both relatively easy to produce and safe. Recombinant DNA probes have also been developed that can clearly diagnose one form of growth hormone deficiency (IGHD-1A) and are approaching identification of other forms. Finally, since they function by direct interaction with the genes, probes will also detect the precise molecular defect(s) causing IGHD. This knowledge potentially could lead to a means of correcting the mutations themselves.

DNA Probes↗

Partial deletion 21: case report with biochemical studies and review.

An unbalanced translocation of a portion of the long arm of chromosome 21 to the short arm of chromosome 4 resulted in a partial deletion of chromosome 21 (pter----q21.05) and in the loss of the telomere of 4p. The phenotype of the child included asymmetrical facies, microcephaly, short stature, hypotonia, and psychomotor retardation associated with frequent infections. Normal SOD-1 activity in red blood cells and fibroblasts and normal cystathionine beta synthase activity in fibroblasts suggest that these gene loci are distal to 21q21.05.

Abnormalities, Multiple↗

Umbilical artery catheterization: a potential cause of refractory hypoglycemia.

Two infants with persistent, symptomatic hypoglycemia are reported. Both were thought to have iatrogenic hyperinsulinism due to a malpositioned umbilical artery catheter. Repositioning of the catheter to avoid direct infusion into the arterial blood supply to the pancreas resulted in prompt cessation of hypoglycemia.

Adult↗

Microcomputer assisted growth assessment.

A microcomputer program is described which facilitates analysis of normal and abnormal growth patterns in children. The program compares individual growth data with established normals, calculates indices and provides both printed summaries as well as graphic displays.

Age Determination by Skeleton↗

Compliance with blood glucose monitoring in children with type 1 diabetes mellitus.

Compliance with blood glucose monitoring was studied in 18 children with type 1 diabetes. Using specially equipped blood glucose reflectance meters, children monitored their blood glucose levels, with and without knowledge of the meter's memory capability. Poor compliance, as exhibited by fabricated test results (40%) and failure to record test results (18%), occurred in a significant proportion of children. Further research is needed to explore reasons for such behavior and to suggest strategies for improving compliance.

Adolescent↗

Newborn hypothyroid screening. The private sector.

Newborn hypothyroid screening in four private hospitals was prospectively evaluated and compared with a state screening program. During 1982 the hospitals screened a total of 10,786 infants compared with 47,525 by the state. Eight cases of primary hypothyroidism were confirmed by the state, for an incidence of one in 5,941. No cases were detected by the hospital programs. The cost for all infants screened in the private hospitals was 51.5 times more than the total cost of the state program, yet the state screened 4.4 times more infants.

Cost-Benefit Analysis↗

Increased levels of HDL-cholesterol and apolipoprotein A-I after intensified insulin therapy for diabetes.

Sixteen subjects with insulin-dependent diabetes mellitus were studied to determine whether changes in plasma lipids and apolipoproteins follow intensified control using preprandial doses of regular insulin with an additional dose of NPH insulin before bedtime. The mean total dialy dose of insulin was increased from 1.03 +/- 0.09 to 1.17 +/- 0.44 units/kg throughout the six-month period. Levels of HDL-cholesterol and apolipoprotein A-I increased without significant changes in hemoglobin A1 (HbA1), triglyceride, or cholesterol. These findings suggest that increases in HDL-cholesterol and apolipoprotein A-I were a result of the intensified insulin delivery.

Adolescent↗