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Biomedical subjects

W Andler

Publications and source records attributed to W Andler.

At least 55 records · Page 3Linked to original sources
PubMed1980

[Familial partial pyruvic dehydrogenase deficiency (author's transl)].

Pyruvic dehydrogenase deficiency was found in two living children of a Turkish family, whereas lactate acidosis was found in a dead child. Two further children died with a pattern of a therapy-refractory metabolic acidosis without any recognisable cause. The prerequisites for the creation of lactate acidosis are demonstrated by the example of pyruvic dehydrogenase deficiency. The extent of remaining activity in case of peruvic dehydrogenase deficiency determines the pattern of clinical signs and the course of the disease which can be influenced to a varying degree by additional complications and different attempts at treatment.

Acidosis↗
PubMed1979

Pre- and postoperative evaluation of hypothalamo- pituitary function in children with craniopharyngiomas.

Pre- and postoperative evaluation of hypothalamic-pituitary function was performed in six children, aged 5.5 to 13.3 years with craniopharyngiomas. Before surgery growth hormone deficiency (GHD) was documented in four, hypothalamic hypothyroidism in three, and secondary ACTH-deficiency and hyperprolactinaemia in one patient. Diabetes insipidus was absent in all patients. After neurosurgical treatment GHD was present in all, hypothyroidism in five, ACTH-deficiency in three, hyperprolactinaemia in three, and diabetes insipidus in four children. The study shows that all endocrine functions tested may be defective even before surgery, although diabetes insipidus seems to be a rare preoperative complaint. Surgical intervention, however, often leads to additional endocrine disorders. From the data presented here one may suggest that TRH stimulation tests, evaluation of serum prolactin, and lysin-vasopressin stimulation tests are the most useful investigations to distinguish between hypothalamic and primary pituitary disorders.

Adolescent↗
PubMed1979

[Hypothalamic hyperosmolarity in childhood (author's transl)].

Hypothalamic lesions occasionally lead to excessive hypernatraemia and hyperosmolarity which cannot be explained by defective ADH secretion alone. As osmoregulation is a complex system the clinical features differ widely from one patient to another. In general central dysregulation of osmolarity is due to diffuse hypothalamic lesions, e.g. inflammatory inflammatory infiltration by histiocytosis X or by large suprasellar tumours. We report on a ten-year-old girl suffering from a suprasellar spongioblastoma and a twelve-year-old-girl, who had been operated for a large craniopharyngioma. Polyuria and polydipsia were not present. Whereas one patient presented hypernatraemic crises and showed normal osmolarity at the intervals, the other patient suffered from sustained hypernatraemia and hyperosmolarity. In the first patient water loading led promptly to clinical and laboratory normalisation. In the other case water loading failed to decrease hyperosmolarity but led to oedema. In the first patient hypernatraemic crises were combined with decreased serum potassium levels and elevated urinary aldosterone excretion. Therefore acute and long-term trials of spironolactone treatment were successful. Exogenous ADH-derivatives failed to normalize hyperosmolarity. In the other patient, however, DDAVP decreased the serum sodium level seen with small doses.

Astrocytoma↗
PubMed1979

[Endocrine disorders in children with neurofibromatosis von Recklinghausen (author's transl)].

Endocrine disturbances have been studied in 4 children with neurofibromatosis von Recklinghausen, aged 5.0-10.5 years. Hypothalamic precocious puberty was seen in three boys; growth hormone deficiency was diagnosed in a girl. After TRH stimulation one boy and the girl showed a diminished TSH-response. Another boy showed a relatively high basal level of TSH and an elevated TSH-response to TRH. All children were euthyroid. Two boys had a hyperprolactinemia even under basal conditions; the one with elevated TSH-response showed an excessive response of prolactin as well. In all our patients a suprasellar tumor caused the endocrine disorders described.

Astrocytoma↗
PubMed1979

[Traumatic pancreatic pseudocysts in childhood (author's transl)].

Two children had extensive resection of the pancreas due to a posttraumatic pseudocyst. Function of the endocrine and exocrine pancreas was reinvestigated following surgery and found to be normal. Based upon these observations pathogenesis, clinical and therapeutical aspects of pancreatic pseudocysts in childhood are reviewed.

Blood Glucose↗
PubMed1978

Thyroid function in children with growth hormone deficiency, either idiopathic or caused by diseases of the central nervous system.

Thyroid function was assessed in thirty two patients with growth hormone deficiency (GHD) by clinical examination and by measurement of T4-levels, free T4-indices, basal TSH values and TSH responses to TRH (100 mu/m2). Sixteen patients (50%) were hypothyroid. In thirteen patients, the endocrine disorders were considered to be of hypothalamic origin. Ten of them showed prolonged responses to TRH and in the other three the responses were exaggerated. In three patients hypothyroidism was due to a primary pituitary disorder. Sixteen patients were euthyroid although three of them showed impaired TSH responses. In the cases with idiopathic hypopituitarism (n = 20) there a high incidence of abnormal births in the children with additional hypothalamic hypothyroidism, but not in the euthyroid patients. It is concluded that in patients with previous breech or vacuum extraction delivery, growth hormone deficiency when combined with hypothalamic hypothyroidism may be due to birth trauma.

Adolescent↗
PubMed1978

Endotoxin stimulated nitroblue-tetrazolium (NBT)-test in patients with hypoparathyroidism, pseudohypoparathyroidism and other forms of hypocalcemia.

Endotoxin-stimulated NBT-tests were carried out in 15 patients with hypocalcemia of varying etiology and in 14 normocalcemic children free of infection. In the control group the formazan cell percentage (FCP) was 73.8 +/- 1.6% (range 63% to 83%). In 5 patients with hypoparathyroidism or pseudohypoparathyroidism the FCP before treatment was lower than normal. Vitamin D3 therapy produced a rapid increase of serum calcium but normalisation of NBT-test was only achieved after a latent period of one or more months. Patients with hypocalcemic rickets and children with an acute relapse of the nephrotic syndrome also showed abnormal results. The clinical significance of the NBT-test in hypocalcemic conditions is discussed.

Adolescent↗
PubMed1978

[Virilizing adrenal cortical tumors in childhood (author's transl)].

We report on four patients, aged seven months to seven years, with virilizing adrenal cortical tumors. Diagnosis was made by clinical and laboratory data. Tumor localisation was achieved preoperatively in three patients. In two patients both androgen and glucocorticoid excretion were elevated. In three patients histological examination showed a carcinoma, in one patient an adenoma was suggested. There were no metastases. In two patients surgical removal was followed by cytostatic therapy, in one of them additional irradiation treatment was carried out. This child died 10 1/2 years later from a metastasizing renal cell carcinoma which originated from the kidney being situated in the irradiated area.

17-Ketosteroids↗
PubMed1978

Endocrine dysfunction in the diencephalic syndrome of emaciation in infancy.

A 10-month-old boy with the clinical features of the diencephalic syndrome of emaciation due to a suprasellar spongioblastoma is described. The patient showed high basal levels of growth hormone (GH greater than 80 muU/ml on several occasions). In addition, elevated concentration of plasma testosterone (125.5 ng/100ml) was combined with a relatively high LH-increase to LHRH (45.6 mU/ml). After completion of irradiation basal GH-levels had been normalized, and GH responses to insulin induced hypoglycemia (IIH) and propranolol-glucagon (PG) were adequate. Complete clinical remission of emaciation occurred soon after radiation therapy and went parallel with the normalization of GH-regulation.

Astrocytoma↗
PubMed1977

[Cogans's syndrome in childhood (author's transl)].

A case--report is given of a patient with Cogan's syndrome in childhood. This syndrome consists of non-syphilitic interstitial keratitis and vestibuloauditory symptoms. Visual loss is rare but hearing loss is rapidly progressive. A relation to autoimmune diseases especially to panarteriitis nodosa has been suggested by a number of authors.

Autoimmune Diseases↗
PubMed1977

[Central nervous manifestations of neurofibromatosis in children (author's transl)].

Serious central nervous manifestation of neurofibromatosis have been reported in 11 children aged 1,5-12 years. According to the present literature we conclude: 1. Central nervous lesions of neurofibromatosis are common even in childhood. 2. About two thirds of these patients suffer from brain tumors. 3. The tumors involve the suprasellar region in many cases. 4. By this localisation endocrine manifestations, especially precocious puberty, are frequent.

Astrocytoma↗
PubMed1975

A comparative study of serum growth hormone and plasma cortisol levels in stimulation tests with insulin and propranolol-glucagon.

Insulin and propranolol-glucagon stimulation tests were carried out on 28 children and 5 adolescents and the results of their growth hormone and plasma cortisol estimations were compared. Twenty-nine subjects with normal growth hormone reserves showed a mean maximum rise of 17.4 muU/ml of serum growth hormone in the insulin test whereas the intramuscular injection of glucagon after oral premedication with propranolol produced a rise of 38.5 muU/ml. Five subjects with normal growth hormone reserves showed a reduced hormone output in the insulin stimulation tests but normal response in the propranolol-glucagon stimulation tests. Only one subject showed a poor response in the propranolol-glucagon but normal response in the insulin stimulation test. In 30 subjects with normal adrenocortical function the mean maximum increase of plasma cortisol was 15.6 muU/ml in the insulin - and 14.9 muU/ml in the propranolol-glucagon stimulation tests, respectively. Both methods are suitable for studying the pituitary-adrenocortical interrelationships. The mechanism of the release of glucagon-induced growth hormone is not clear but the fall in blood glucose does not seem to play a major role in the process. A stress-like mechanism is equally unlikely because vegetative symptoms occurred only i a small number of subjects after intramuscular glucagon administration. It is possible that glucagon possesses a releasing-like mechanism which operates in the pituitary itself.

Adolescent↗