Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0017638 (glioma)
 30,880 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cerebrospinal fluid (CSF) concentration and urinary excretion of cyclic adenosine-3',5'-monophosphate(cAMP) were measured in children aged from 3 days to 15 years by the protein-binding method of Gilman (1970). The mean CSF cAMP concentration (22.4 plus or minus 0.6 (S.E.) nmol/l) of 24 "healthy" children tended to be lower (P less then 0.2) than that of adult patients who revealed no pathological findings on clinical examination. No difference in the results was foung between the sexes. High cAMP concentrations were found in CSF of children suffering from cerebellar glioma, hypothalamic precocious puberty, bacterial meningitis, or Cushing's disease. The urinary excretion of cAMP varied from 0.2 to 5.3 in "healthy" and from 1.3 to 7.6 mumol/24 hrs in diseased children. Two children with pheochromocytoma showed a striking decrease in the rate of urinary excretion of the nucleotide after surgical treatment.
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PMID:Cerebrospinal fluid concentration and urinary excretion of cyclic adenosine-3', 5'-monophosphate in various diseases of children. 16 45

Hypothalamo-pituitary function in children with optic glioma may be impaired by the tumour itself and by the high cranial radiation doses used in treatment. This study evaluates the effect of optic glioma and its treatment on patient growth and pubertal development. Twenty-one patients (13 boys, 8 girls), treated for optic glioma by cranial irradiation (45-55 Grays) at a mean age of 5.4 years, were evaluated before (n = 10) and/or after (n = 21) irradiation. Growth hormone (GH) deficiency was present in only 1 patient tested before irradiation and in all patients after irradiation. Precocious puberty occurred in 7/21 cases, before irradiation in 5 patients and after irradiation in 2 patients. The cumulative height loss during the 2 years after irradiation was 0.2 +/- 0.2 SD (m +/- SEM) in 7 patients with precocious puberty and 1.1 +/- 0.2 SD in 14 prepubertal patients (P less than 0.01). The corresponding bone age advance over chronological age, evaluated 1-3 years after irradiation, was 1.1 +/- 0.5 and -0.7 +/- 0.3 year in the two groups (P less than 0.01). The mean height loss between time of irradiation and the final height was 2.3 +/- 0.6 SD (n = 6). Primary amenorrhoea, associated with low oestradiol levels, occurred in two of the three girls of pubertal age. These data indicate that the high dose of cranial radiation used to treat optic glioma invariably results in GH deficiency within 2 years and that hGH therapy is required when GH deficiency is documented.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Growth and endocrine disorders in optic glioma. 222 66

A 1-year 11-month-old girl was admitted for losing her weight and gait disturbance. At 4 months of age, she began to become emaciated inspite of normal food intake. Physical and neurological examinations were normal except for a marked lack of subcutaneous fat, irritability and nystagmus. CT scans demonstrated a large tumor occupied in the third ventricle and marked dilatation of the lateral ventricles. Endocrinological studies revealed high levels of plasma growth hormone (GH) in contrast with normal levels of somatomedin-C. The basal value of GH returned to normal with a subnormal response to insulin subsequently after VP-shunt. Then, a pilocytic astrocytoma was partially resected with transcallosal approach. Postoperative course was uneventful and her growth rate returned to normal range. CT scans after radiation therapy of 49 Gy showed marked decrease in size of the tumor. At 3 years and 6 months of age, enlargement of her breast was pointed out although MRI indicated no enlargement of the tumor. Basal value of LH, FSH, E 1 and E 2 elevated and LHRH test showed over-response of LH and FSH. Other hypothalamic-pituitary functions were partially preserved. Case of precocious puberty following diencephalic syndrome associated to the hypothalamic and/or optochiasmatic glioma is quite rare in the previous literature. Mechanisms of diencephalic syndrome and following puberty are unclear. However, endocrinological and radiological findings observed in the present case suggest that hormonal disfunction might be due to the failure of inhibition on GH and LHRH secretion mechanism in the anterior hypothalamus.
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PMID:[Hypothalamic glioma with diencephalic syndrome and following precocious puberty--a case report]. 251 56

External cranial radiation for the treatment of malignant diseases has become a frequent cause of growth hormone deficiency (GHD). The timing of occurrence and the frequency of GHD were related to the hypothalamic-pituitary radiation dose. Frequency varied from 50% in leukemia (2400 cGy) to 75% in face and neck tumors or medulloblastoma (2500-4500 cGy) and up to 100% in optic glioma (greater than 4500 cGy). The significantly more severe growth deficit in patients with GHD given higher radiation doses suggests different levels of residual GH secretion according to radiation dosage. The minimum harmful radiation dose is probably close to 1800-2000 cGy. Our data show that stimulation tests remain a useful means of defining GHD and predicting growth. A fair agreement between GH secretion and growth was found in most cases, regardless of the radiation dose. The only exception was a group of leukemic children (2400 cGy) who achieved normal prepubertal growth despite a low GH response. The 24-h spontaneous plasma GH profiles and IGF-I measurements may add information if growth is retarded despite a normal GH response. We showed that growth retardation occurring after some schedules of total body irradiation was not due to GH deficiency but rather to radiation-induced skeletal lesions. Early or true precocious puberty, generally associated with GHD, was another cause of height loss. As the role of GH deficiency in the final height reduction was demonstrated in all groups of patients after cranial radiation, we suggest that hGH therapy should be considered in any child with proven GH deficiency and significant growth retardation after such radiation.
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PMID:Growth and endocrine disorders secondary to cranial irradiation. 266 28

Tumors are rare, but well-documented causes of precocious puberty in both sexes. The therapeutic and prognostic implications of a diagnosis of cancer require that the presence of a neoplastic process be ruled out in any case of precocious puberty. Granulosa-cell tumor of the ovary and Leydig-cell tumor of the testis are the most frequent gonadal tumors inducing precocious pseudopuberty in the two sexes. Adrenal tumors sustain a variety of endocrine syndromes, the most frequent one being virilization with or without hypercortisolism. Pure feminizing adrenal neoplasms have been described. For reasons not yet well understood, hypothalamochiasmatic glioma (beta-HCG) secreting tumors have almost never been described in association with female precocious puberty. Among these neoplasia, pineal germ-cell tumor inducing sexual maturation must be included. Hypothalamochiasmatic glioma and craniopharyngioma are the two cerebral tumors capable of inducing true precocious puberty. Even if equally distributed between both sexes, these tumors interfere with sexual maturation less frequently in girls than in boys. Hypothalamic hamartoma is considered a benign tumor, since it does represent a space-occupying mass. It more correctly could be called a malformation if its histologic characteristics are recalled. This cerebral lesion is now frequently described in children with true precocious puberty, probably because of improved diagnostic imaging methods.
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PMID:Oncologic causes of precocious puberty. 270 2

This paper reviews the clinical findings, pituitary gonadotrophin reserve and plasma oestradiol, neurological findings and pelvic ultrasound appearance in 47 girls with precocious puberty starting before the age of 7 years. Of the 39 girls who had air encephalograms or cranial CT scans, 19 showed intracranial abnormalities (hamartomas 11; hydrocephalus 5; optic glioma 2; arachnoid cyst 1). There was no significant difference in the peak serum luteinizing hormone and follicle-stimulating hormone responses to intravenous gonadotrophin stimulating hormone in girls with and without intracranial lesions. Pelvic ultrasound examination showed development of the ovaries, uterus, and vagina similar to that seen in normal puberty. Treatment with cyproterone acetate (50-100 mg/day) in 26 girls resulted in arrest of breast development and suppression of menstruation, but a definite effect on growth was not documented.
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PMID:Isosexual precocious puberty in girls. 293 58

We recorded brain stem auditory evoked potentials, pattern reversal visual evoked potentials and electroencephalograms in 19 children, 2-8 years old. Sixteen had precocious puberty and three had precocious adrenarche. Two of the children with precocious puberty had hypothalamic hamartomas, one had an optic glioma, and one had a history of hydrocephalus. The EEG was abnormal in 7 patients: three had diffuse slowing, and four showed possible paroxysmal features. BAEPs and VEPs were normal in all patients. This study showed no abnormalities in two conduction pathways of the central nervous system and no evidence of a deleterious effect of hormonal aberrations in patients with precocious puberty. Abnormal EEG in these patients is more likely to reflect occult diencephalic disease than widespread neurophysiological dysfunction.
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PMID:EEG and evoked potentials in precocious puberty. 618 4

Monozygotic twins with neurofibromatosis both showed accelerated growth in association with optic glioma. Endocrine abnormalities were similar, but only one of the two had precocious puberty. This identical presentation in twins supports the view that the site of tumour formation is determined during intra-uterine development.
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PMID:Optic gliomata affecting twins with neurofibromatosis. 640 98

The occurence is reported of optic chiasma gliomas in a pair of identical twins, both with neurofibromatosis. One twin developed precocious puberty in the sixth year of life and was shown by cisternal computerized tomography to have a supersellar tumor, which proved to be a malignant glioma. The second twin had an abnormal growth spurt but not overt precocious puberty. Cisternal computerized tomography of the second twin revealed evidence of a similar, smaller tumor at the same site. The literature is reviewed and some possible genetic explanations discussed. This report also emphasises the importance of careful investigation of the second twin when a malignancy is found in one, irrespective of the site or type of the tumor.
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PMID:Concordant optic glioma in a pair of monozygotic twins with neurofibromatosis. 680 49

The present review is based on the retrospective study of 124 children with precocious puberty, 92 girls and 32 boys. In girls, the analysis of the clinical initial presentation has shown that premature pubarche (n = 18), premature adrenarche (n = 2) or isolated menstruations (n = 3) must be ruled out, as these symptoms can remain isolated for more than a year. However, in most cases the presence of growth acceleration and vaginal estrogenisation was of major diagnostic value. Bone maturation, although generally accelerated, can be normal in recently developed puberty. Precocious puberty may proceed by steps, with complete disappearance of physical signs in the intervals. Organic causes were found in 31% of the girls, and 44% of the boys with some characteristic features as rapid progression, and elevated LH response to LRF stimulation. Main causes were glioma of the optic chiasma (n = 11), 3rd ventricule invasive tumors, hamartoma (n = 8). The latter should be looked for by a non invasive procedure as the CT scan. In girls, precocious puberty with very high circulating estrogen levels was observed as part of a McCune-Albright syndrome. As the effect of precocious puberty on the final adult height is variable, the evaluation of therapeutic results remains uncertain. Medroxyprogesterone as well cyproterone acetate have not been fully efficient in controlling bone maturation. More recently, and still controversial, the treatment with long acting LRF analogues might provide a more satisfactory statural prognosis.
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PMID:[Precocious puberty. Comment on the diagnostic conditions and etiological aspects]. 718 38


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