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Biomedical subjects

K Zuppinger

Publications and source records attributed to K Zuppinger.

At least 37 records · Page 2Linked to original sources
PubMed1985

Utilization of galactose in cultured brain cells of neonatal mice.

Metabolism of galactose was examined in dissociated brain cells from neonatal mice after 10-13 days in culture. Consumption of galactose at levels up to 26 mM was much less than consumption of glucose at corresponding concentrations. Lactate was consumed from the media at all galactose levels, in contrast to experiments with glucose in which lactate was formed and released into the media. Generation of CO2 from 4 mM glucose was 9-fold greater than from an equimolar level of galactose. Relatively low concentrations of glucose could reduce uptake of galactose, whereas galactose at levels up to 11.6 mM failed to inhibit consumption of glucose or formation of lactate. In glucose-deficient states, galactose supplementation of the media led to a marked increase in sulfatide synthesis by oligodendrocytes in the culture with a maximum effect at 2.3 mM. Under these conditions, [1-14C]galactose was incorporated directly into the carbohydrate portion of sulfatide, although most of the label was found in phospholipids and in the nonlipid fraction of the cellular homogenate. These data suggest that galactose is poorly metabolized by brain cells, but does not exhibit toxic effects.

Animals↗
PubMed1985

[Effect of two years of deferoxamine therapy on iron balance, ferritin, liver and heart in patients with thalassemia major].

The iron balance, the urinary excretion of iron between transfusions, the serum ferritin, the liver density, the size of the heart, the ECG and the echocardiogram of 14 children with thalassaemia major were assessed before and during or after 2 years of deferoxamine therapy (DF, 1 or 2 g/kg body weight/day according to age, by subcutaneous infusion on 5 days per week, 11 months per year). The mean iron balance decreased significantly (p less than 0.001) from 15.4 +/- 4 mg/kg body weight/month before DF to -7.1 +/- 9.4 mg/kg body weight/month in the first year of DF and increased to -2.5 +/- 6.5 mg/kg body weight/month during the second year of DF therapy. Despite administration of a constant dose of DF the urinary excretion of iron during the last days before transfusion was twice as high as during the first days after transfusion. The mean serum ferritin level fell from 6380 +/- 2600 ng/ml before DF to 5074 +/- 1600 ng/ml during the first and 4346 +/- 1900 ng/ml during the second year of DF therapy (p less than 0.05). There was no significant change in liver density or cardiac parameters.

Adolescent↗
PubMed1984

Oxandrolone in girls with Turner's syndrome. A pair-matched controlled study up to final height.

Twenty-six one-year treatment periods on oxandrolone (0.1 mg/kg/day) were studied in 20 patients with Turner's syndrome. Control patients with Turner's syndrome were matched by using the following criteria: difference in bone age being not greater than 0.5 years and difference in the Bayley-Pinneau height prediction not greater than 3 cm. Height and height velocity were compared with standards of girls with Turner's syndrome (10) and expressed in standard deviation scores (SDS). On oxandrolone height velocity increased significantly from -0.3 SDS to + 3.0 SDS. The increase in height velocity was negatively correlated to the bone age at onset of treatment (r = -0.62, p less than 0.01). Height SDS improved by 0.45 SDS in the treated patients whereas it did not change in the control patients. The bone age velocity during the treatment period (including a six-month period after treatment) was 0.75 year/year in the treated, compared to 0.66 year/year in the control patients (NS). 15 of the 20 patients have reached final height. The difference in final height minus predicted height (Bayley-Pinneau) at onset of treatment was taken as a measure of "gain in final height". Seven of those (mean bone age 12.1 years at onset of treatment) were treated for one year only and had--compared to the matched controls--a mean net gain in final height of 2.5 cm (NS). Eight patients (mean bone age 10.1 years at onset of treatment) were treated for two one-year periods and had a significant mean net gain in final height of 5.2 cm. Height predictions calculated by the method of Lenko (14) gave an identical mean net gain in final height (5.1 cm).

Adolescent↗
PubMed1984

Hypergonadotropic hypogonadism in two sisters with galactosaemia.

Two sisters with transferase deficiency galactosaemia presented with hypergonadotropic hypogonadism. In the younger girl galactosaemia was documented first at 9 months of age, although she had never been exposed to exogenous galactose in utero or after birth.

Child, Preschool↗
PubMed1983

Disorders of the endocrine pancreas.

Hyperinsulinemic hypoglycemia in the neonate or in early infancy may be caused by an islet cell adenoma or a diffuse organic lesion of the endocrine pancreas such as nesidioblastosis. The 2 disorders cannot be distinguished by biochemical means but only by immunohistochemical analysis after subtotal pancreatectomy. An aggressive therapeutic approach is mandatory to avoid permanent neurological damage. In nesidioblastosis, 20% of patients are healed by subtotal pancreatectomy and the remainder may be controlled by postoperative treatment with diazoxide and hydrochlorothiazide. Some patients may need total pancreatectomy which leads to permanent insulin-dependent diabetes in most of them. When hyperinsulinemic hypoglycemia first manifests after 1 year of age, it is always caused by an islet cell adenoma, which is cured by enucleation.

Adenoma, Islet Cell↗
PubMed1979

Hypodipsia-hypernatremia syndrome.

The pathogenesis of the rare hypernatremia, usually described in the literature as "neurogenic" or "essential" hypernatremia, consists of defective thirst mechanism either alone or in combination with impaired osmoregulation of ADH release. As etiology, disturbances of the neoplastic, vascular and degenerative type and malformations in the hypothalamic area are known. In patients with the hypodipsia-hypernatremia syndrome, dysfunction of the anterior pituitary lobe, obesity, abnormal regulation of body temperature, psychomotor retardation and episodic muscular weakness are frequently encountered as additional abnormalities. A 6-year-old patient is described with hypodipsia-hypernatremia syndrome manifest for 3 years. Besides hypernatremia, hypodipsia and the relative insensitivity of the osmoreceptors regulating ADH release, elevated body temperature, polyphagia and obesity, partial hypothalamic-hypophyseal dysfunction, lethargy and psychomotor retardation are the principal findings. An inflammatory lesion or one occupying an intracranial space was not demonstrable until now. Under forced water intake and hypocaloric diet the patient has progressed well with nearly complete normalization of the hypernatremia, body temperature and obesity.

Child↗
PubMed1979

Increased risk of diabetes mellitus in beta- thalassemia major due to iron overload.

Frequent transfusions improve the general well being in patients with beta-thalassemia major but carry the risk of iron intoxication including the development of diabetes mellitus. Of 22 patients with beta-thalassemia major (age 3-17 years) only 3 had a normal oral glucose tolerance. The remainder had either borderline or moderately pathological glucose curves. The mean glucose concentration was increased, and the mean insulin concentration and insulin/glucose ratio were diminished. In contrast to the oral test, the i.v. glucose tolerance test gave pathological results in only 2 of 16 patients tested. The i.v. glucose test thus may be less selective than the oral test. The mean insulin concentration was lower also after intravenous glucose, but the early insulin peak was preserved. Arginine infusion led to a normal insulin and growth hormone release. This moderate impairment of insulin release found in most of the patients leaves the hope that an efficient chelating therapy scheme might reverse beta-cell dysfunction.

Adolescent↗
PubMed1978

[Experimental coronary ligation in swine: reduction of myocardial infarct and hemodynamic and metabolic changes by means of the calcium antagonist RO 11-1781].

In a comparative study the effect of the new calcium antagonist Ro 11-1781 on experimental infarct size and left ventricular function in the pig has been investigated. The calcium antagonist was administered 30 min prior to ligation of the left anterior descending coronary artery and twice daily on the following 4 days. For morphometric assessment of infarct size the ventricular myocardium was cut into slices and stained with nitro-benztoluene. There was a significant reduction in infarct size of about 25% in the calcium antagonist-treated group in comparison with the control group. After coronary occlusion left ventricular function is equally depressed in both groups, as is myocardial lactate extraction, which becomes negative.

Animals↗
PubMed1978

Cyproteroneacetate and ACTH adrenal function.

Cyproteroneacetate, an antiandrogenic and gonadotropin-inhibiting steroid, has a marked ACTH suppressive effect. In rats, adrenal atrophy and severe impairment of ACTH and corticosterone responses to stress are induced by a 10-day treatment with 3-0.75 mg/100 g BW cyproteroneacetate/day. Two weeks after cessation of treatment, the ACTH adrenal system has not yet recovered. The ACTH suppression is evident 6 h after a single dose. In 25 human volunteers, a single dose of 200 mg cyproteroneacetate impaired their ACTH and 11-deoxycorticosteroid response to 1 g metyrapone. A similar impairment was seen in 12 women on sequential treatment with cyproteroneacetate and ethinyl estradiol. In 4 out of 11 children treated for precocious puberty, random plasma ACTH and cortisol measurements, cortisol responses to ACTH, and ACTH and cortisol responses to insulin-induced hypoglycemia revealed severely impaired ACTH adrenal function. Questionable impairment was found in 2 out of 11 and normal function in 5 out of 11 children. In 10 patients with endogenous elevated plasma ACTH, 10 days of treatment with cyproteroneacetate, in addition to the steroid substitution, diminished the morning plasma ACTH levels. It is concluded that cyproteroneactate has a pronounced ACTH-suppressive effect. The individual susceptibility of treated patients varies and the effect is dose dependent. A cortisol-like effect must be assumed, because cyproteroneacetate-treated animals and patients under therapy can withstand stress situations without signs of adrenal insufficiency. ACTH adrenal function must, however, be closely watched in treated patients and steroid cover must be considered in conditions of stress. Great care has to be taken when the drug, with its own "stress-protective" effect, is withdrawn. The recovery of ACTH adrenal function may take several months.

Adrenal Cortex↗
PubMed1977

Hyperprolactinemia as a cause of delayed puberty: successful treatment with bromocriptine.

An 18-year-old male patient was referred because of galactorrhea and delayed puberty. There was no gynecomastia, but a white milky secretion could easily be expressed from each breast. The chest and skull X-rays were normal. The plasma prolactin was increased to 58 ng/ml and rose to 97 ng/ml after 200 microgram TRF iv. The patient was treated for one year with testosterone; his voice deepened, body hair developed, libido and sexual function became overt, and bone age advanced from 14 1/2 to 17 years, but the galactorrhea increased. After a satisfactory stage of pubertal development was reached, the testosterone was stopped. tthe galactorrhea then decreased to its pretreatment intensity; however, sexual potency diminished, sexual hair growth decreased, and the plasma prolactin levels rose to 246 ng/ml. After a 5-month interval without treatment, bromocriptine was given and brought about an impressive improvement. Virilization and general well being were superior to that during testosterone treatment, the galactorrhea vanished, plasma prolactin decreased, testosterone rose to normal values, and a normal semen analysis was recorded.

Adolescent↗