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Biomedical subjects

B M Lippe

Publications and source records attributed to B M Lippe.

At least 37 records · Page 2Linked to original sources
PubMed1987

Hyperthyroidism in children treated with long term medical therapy: twenty-five percent remission every two years.

We use an antithyroid drug for the treatment of hyperthyroidism due to Graves' disease in children and adolescents for as long as the patients are willing to comply and/or tolerate the drug. In more than 60 patients treated since 1961, the remission rate was 25% in the first 2 yr. This report looks at these same patients again, followed for an additional 5 yr. Survival analysis methods applied to the follow-up data on 63 children confirm our original statistical findings and suggest a continuing remission rate of 25% every 2.1 +/- 0.4 (+/- SE) yr regardless of the duration of previous therapy. The median time to remission was 4.3 +/- 1.5 yr, and 75% of patients are predicted to be in remission in 10.9 +/- 2.3 yr. Of 36 patients who went into remission, defined by their being euthyroid for 1 yr after cessation of therapy, 1 relapsed, and 2 developed spontaneous hypothyroidism; the remainder are euthyroid 1-11.7 yr after therapy was discontinued. Of 14 who switched from medical therapy, 2 of 7 treated surgically and 4 of 7 treated with 131I are hypothyroid. Only 1 patient had a significant adverse reaction to both methimazole and propylthiouracil. While medical therapy may have some direct effect on the autoimmune response in hyperthyroidism, its role in affecting the time to ultimate remission is unknown. These data, however, describe the course of children so treated and allow us to present therapeutic options initially or during treatment based on statistically derived probabilities of outcome.

Child↗
PubMed1987

The role of immunotherapy in type I diabetes mellitus.

Type I diabetes mellitus appears to result from an insidious immunologic destruction of pancreatic beta-cells in genetically susceptible persons exposed to one or a series of environmental insults. This genetic susceptibility is related to alleles located on the sixth chromosome in the HLA-DR or an adjacent region. With superimposition of a viral or other environmental triggering event, cell-and antibody-mediated events are activated that lead to the specific autorejection of beta-cells and consequent insulin deficiency. Immunosuppressive strategies to impede or halt complete destruction of beta-cells, using cyclosporine, have already been initiated in both animals and humans with diabetes mellitus. Because of the potential toxicity of all current immunosuppressive regimens, such therapies cannot, at this time, be considered for wide-scale use in persons with type I diabetes. Reported inductions, however, of insulin independence in patients with newly diagnosed type I diabetes using cyclosporine or other agents underscore the role of the immune system in the pathogenesis of the disease and highlight the need to develop safer, more specific immunomodulation designed to avoid complete beta-cell destruction.

Animals↗
PubMed1986

Growth without growth hormone: evidence for a potent circulating human growth factor.

A potent growth factor was detected in the serum of a child with growth hormone (GH) deficiency and early growth delay whose growth velocity spontaneously increased to supranormal levels despite persistent GH deficiency by both radioimmunoassay (RIA) and radioreceptor assay. Thyroid function, prolactin, insulin response to oral glucose, glucose response to intravenous insulin, and computerised tomography of the head were all normal. Whilst somatomedin-C levels measured by RIA were low or low-normal, in vitro somatomedin bioactivity measured by bioassay was normal, suggesting the presence of a growth factor other than somatomedin-C. By way of confirmation, the patient's serum was incubated with erythroid progenitor cells from peripheral blood of a normal individual and a Laron dwarf. In this system, proliferation of normal erythroid progenitors was almost double that obtained with physiological concentrations of GH or control sera, and Laron erythroid progenitors, which were completely resistant to added GH, also responded strongly to the patient's serum. The patient's growth is therefore independent of GH and other known growth factors.

Child↗
PubMed1986

Aortic dilation, dissection, and rupture in patients with Turner syndrome.

We report two patients with Turner syndrome who had aortic dissection and rupture, one with prior repair of coarctation. We also note the high incidence (8.8%) of unrecognized aortic root dilation in a group of 57 patients with Turner syndrome whom we prospectively evaluated by echocardiography. Our analysis and review of previously reported cases suggests that multiple risk factors may exist for aortic dissection, including coarctation, bicuspid aortic valve, and systemic hypertension, but that these need not be present. Aortic root dilation may be an additional finding that suggests the patient with Turner syndrome is also at risk. When it is present, magnetic resonance imaging visualizes the entire aorta and allows quantification of the site and degree of dilation. In patients with dissection, the aorta often exhibits pathologic evidence of cystic medial necrosis similar to the finding in patients with Marfan syndrome. Therapeutic methods to decrease risk, such as those directed toward prevention of bacterial endocarditis, blood pressure control, and perhaps prophylactic beta blockade or surgical reconstruction, may need to be considered. Patients with Turner syndrome, their families, and the physicians who care for them should be aware of the significance of unexplained chest pain, dyspnea, or hypotension as potential manifestations of aortic dissection or rupture.

Adolescent↗
PubMed1985

Insulin resistance in total lipodystrophy: evidence for a pre-receptor defect in insulin action.

The cause of insulin resistance in lipodystrophic diabetes is unknown but has generally been ascribed to dysfunction at either the receptor or post receptor level. In a 14 year-old girl with total acquired lipodystrophy, subcutaneous and intravenous insulin requirements approximated 600 units daily. However, circulating total and free insulin levels were not increased, and during testing by the euglycemic clamp method, the glucose response to increasing free insulin concentrations was within the range found in eight subjects with insulin-dependent diabetes. Insulin clearance during the euglycemic clamp was 43, 98, 115, and 116 mL/kg/min at each of four insulin infusion rates compared to means of 13, 13, 12, and 11 in the control subjects with diabetes. No detectable degrading activity was present in serum, and serum inhibited insulin degradation normally. Binding of insulin to IgG, IgM, and IgE was not increased, insulin binding to monocytes and erythrocytes was not sufficiently abnormal to account for the the insulin resistance, and insulin receptor increased insulin clearance or accelerated degradation of insulin by tissues.

Adolescent↗
PubMed1985

Diminished in vitro responsiveness of circulating erythroid progenitor cells to insulin as an indicator of insulin resistance.

While insulin resistance is considered characteristic of extreme obesity, it may be more difficult to demonstrate in less severe forms of obesity. We studied five moderately obese individuals [mean body mass index (MBMI), 34.1 +/- 1.85 (+/- SE) kg/m2], one massively obese patient (BMI, 50.2 kg/m2), and seven age-matched normal subjects (MBMI, 22.4 +/- 0.93 kg/m2). While two of the obese patients had normal glucose tolerance, all had fasting hyperinsulinemia (P less than 0.02 vs. normal subjects) and exaggerated insulin responses after oral glucose challenge, as defined by area under the 3-h insulin response curve (P less than 0.01 vs. normal subjects). That this hyperinsulinemia represented in vivo insulin resistance was supported by the glucose and insulin responses in four individuals to an iv glucose bolus analyzed by the minimal modeling technique. Study of monocyte insulin receptors revealed no reduction in total insulin binding in the four obese patients tested. Since physiological concentrations of insulin stimulate the in vitro growth of normal human erythroid progenitor cells (EPC), we reasoned that this response might be blunted in cells from individuals with endogenous insulin resistance. The mean peak EPC proliferative response (26.7 +/- 9.11% above baseline) in the obese hyperinsulinemic group was significantly less than the corresponding mean value in the control group (92.6 +/- 5.24% above baseline, P less than 0.001). These results suggest that the minimal modeling technique is a sensitive method for the in vivo demonstration of insulin resistance in moderately obese individuals and that EPC responsiveness to physiological concentrations of insulin reflects in vivo insulin sensitivity and may be used as an in vitro indicator of insulin resistance.

Adolescent↗
PubMed1984

Insulin resistance in a young man with cystic fibrosis.

An 18-year-old man had cystic fibrosis (CF) and insulin-resistant carbohydrate intolerance characterized by (1) obesity, basal hyperinsulinemia, and hyperglucagonemia; (2) impaired oral glucose tolerance; (3) hyperinsulinemia in response to oral and intravenous (IV) administration of glucose and to IV administration of tolbutamide; (4) exaggerated gastric inhibitory polypeptide secretion following orally administered glucose; and (5) diminished sensitivity to insulin administered IV compared with other patients with CF. Both parents also demonstrate basal and stimulated hyperinsulinemia in response to orally administered glucose. The long-term outlook for patients with CF is improving, and more patients are surviving childhood. Thus, it should be recognized that an insulin-resistant form of carbohydrate intolerance may develop in patients with CF with obesity and/or genetic risk factors.

Adolescent↗
PubMed1984

Carbohydrate tolerance in cystic fibrosis is closely linked to pancreatic exocrine function.

We evaluated carbohydrate tolerance in nine thin cystic fibrosis (CF) patients and in six controls, measuring responsiveness to the following insulinotropic secretagogues: oral glucose, IV glucose, and IV tolbutamide. Glucose responses segregated patients into two groups: Group I with normal carbohydrate tolerance associated with normal to slightly increased insulin responses, and Group II with impaired carbohydrate tolerance associated with insulinopenia. This latter group included one patient with frank diabetes. The CF patients demonstrated a significant positive correlation between insulin secretion, in response to each secretagogue, and pancreatic exocrine function as measured by serum pancreatic amylase isoenzyme concentration. Pancreatic alpha-cell function, as reflected by basal plasma glucagon concentrations, also correlated well with exocrine function in the CF patients, excluding the diabetic individual. The enteroinsular axis of the CF group was intact as reflected by normal plasma gastric inhibitory polypeptide concentrations in Group I and by elevated levels, basally and in response to oral glucose, in the insulinopenic Group II patients. Furthermore, those patients with impaired tolerance demonstrated a greater magnitude of insulinopenia compared to controls following IV glucose and possibly IV tolbutamide, than following oral glucose. Thus, these data suggest that loss of carbohydrate tolerance in patients with CF, like that seen with classical chronic pancreatitis, 1) parallels the loss of exocrine function, 2) is associated with appropriate enteroinsular signaling, and 3) can be detected earlier or more easily following testing with direct IV secretagogues than following oral glucose stimulation.

Adult↗
PubMed1983

Self-monitoring of blood glucose levels and intensified insulin therapy. Acceptability and efficacy in childhood diabetes.

Prospective studies have shown that children and adolescents with diabetes have a high prevalence of serious complications and a sharp reduction in life expectancy. Recently, self-monitoring of blood glucose levels has become available and, for the first time, provides a method for determining the concentration of blood glucose with considerable accuracy. We have introduced this method of control assessment to our pediatric diabetic patient population in conjunction with a program of intensified insulin administration (two or more injections per day). This is a report of the ready acceptance of these methods by children and adolescents and their parents (53/63, or 84%). The effectiveness of this program is evidenced by a progressive and significant reduction in the percentage of glycosylated hemoglobin during a period of 18 months in a majority of the subjects. These observations suggest that improved glycemic control can be achieved in young diabetics by using multiple insulin injections and self-monitoring of blood glucose levels. Whether such control can lead to a better long-term outlook for diabetics remains to be seen.

Adolescent↗
PubMed1983

Precocious puberty and chronic renal failure.

A 7 1/2-year-old boy with chronic renal failure (CRF) experienced transient central isosexual precocious puberty. Simultaneously, he demonstrated hyperprolactinemia with galactorrhea and apparent secondary or tertiary hypothyroidism. These findings could have been a consequence of an unexplained hypothalamic dysregulation. During this period, marked linear growth ensued (8.7 cm during nine months) associated with rapid bone age advancement (four years during a 21-month period). Whereas most boys with CRF demonstrate delayed pubertal development and suppressed linear growth, our patient's transient condition demonstrated that both growth and puberty can occur in the presence of uremia.

Body Height↗
PubMed1983

Echocardiography reveals a high incidence of bicuspid aortic valve in Turner syndrome.

The most common cardiac defect in Turner syndrome has been described previously as coarctation of the aorta. We have evaluated 35 consecutive patients with Turner syndrome by clinical examination and by M-mode and two-dimensional echocardiography. Twelve patients (34%) had isolated, nonstenotic bicuspid aortic valve. A high correlation (82%) existed between the presence of a systolic ejection click and echocardiographic evidence of a bicuspid aortic valve. These data indicate that bicuspid aortic valve may be the most common cardiac anomaly in Turner syndrome.

Adolescent↗
PubMed1982

Urinary and serum steroid concentrations in the management of congenital adrenal hyperplasia. Lack of physiologic correlations.

Serum concentrations of 17-hydroxyprogesterone, dehydroepiandrosterone, dehydroepiandrosterone sulfate, progesterone, testosterone, and androstenedione and 24-hour excretion of 17-ketosteroids and pregnanetriol were measured serially in 18 children with congenital adrenal hyperplasia (21-hydroxylase deficiency) during a two-year period. Correlations were sought between results of measurements of these steroids and clinical progress assessed by physical examination and skeletal maturity to determine if measurement of concentration of these substances at a single point in time could be used to gauge the dose of corticosteroids for optimum treatment. We found that these measurements of steroids were generally not useful indicators of optimum control of the disease. Repeated careful clinical examination and assessment of changes in growth velocity and skeletal maturation seem to be the best criteria on which to base dosage of corticosteroids used for therapy.

17-Ketosteroids↗