CEREBRAL GIGANTISM IN CHILDHOOD

PEDIATRICS ◽  
1967 ◽  
Vol 40 (3) ◽  
pp. 395-402
Author(s):  
Aubrey Milunsky ◽  
Valerie A. Cowie ◽  
Elaine C. Donoghue
Keyword(s):  
Growth Hormone ◽  
Mental Retardation ◽  
Neurological Disorder ◽  
Pathogenic Mechanism ◽  
Plasma Growth Hormone ◽  
High Arched Palate ◽  
Cerebral Gigantism ◽  
Hypothalamic Pituitary Axis ◽  
Neurological Lesion ◽  
Mechanism Operative

Two cases of cerebral gigantism in childhood are reported, and 14 earlier cases are reviewed. The major manifestations of this non-progressive neurological disorder included gigantism, macrocrania, dolichocephaly, mental retardation, characteristic facies, high arched palate, and ataxia or clumsiness. Pneumoencephalography in 10 out of 11 cases revealed a dilated ventricular system. Normal fasting plasma growth hormone levels were found in our patients, but both showed evidence of impaired function of the hypothalamic-pituitary axis in that these levels failed to rise following marked hypoglycemia. Abnormal dermatoglyphic patterns are reported and their value as an aid to diagnosis is mooted. Both the cause and the nature of the neurological lesion remain obscure. The evidence favors a pathogenic mechanism operative in utero.

Jansky-Bielschowsky syndrome, a lysosomal disease: First diagnosed cash in Oman

1992 ◽  
Vol 50 (1) ◽  
pp. 632-633
Author(s):  
Line Buhl ◽  
David Muirhead
Keyword(s):  
Mental Retardation ◽  
Clinical Picture ◽  
Neurological Disorder ◽  
Clinical Presentation ◽  
Neuronal Ceroid Lipofuscinosis ◽  
Infantile Form ◽  
Lysosomal Disease ◽  
Juvenile Form ◽  
Progressive Encephalopathy ◽  
Lysosomal Diseases

There are four lysosomal diseases of which the neuronal ceroid lipofuscinosis is the rarest. The clinical presentation and their characteric abnormal ultrastructure subdivide them into four types. These are known as the Infantile form (Santavuori-Haltia), Late infantile form (Jansky-Bielschowsky), Juvenile form (Batten-Spielmeyer-Voght) and the Adult form (Kuph's).An 8 year old Omani girl presented wth myclonic jerks since the age of 4 years, with progressive encephalopathy, mental retardation, ataxia and loss of vision. An ophthalmoscopy was performed followed by rectal suction biopsies (fig. 1). A previous sibling had died of an undiagnosed neurological disorder with a similar clinical picture.

Single and repeated testing of growth hormone secretory capacity in hypopituitarism using growth hormone releasing factor

1986 ◽  
Vol 113 (4_Suppl) ◽  
pp. S118-S122 ◽  
Author(s):  
O. BUTENANDT ◽  
M. EMMLINGER ◽  
H. DOERR
Keyword(s):  
Growth Hormone ◽  
Body Weight ◽  
Pituitary Gland ◽  
Bolus Injection ◽  
Hormone Deficiency ◽  
Test Results ◽  
Repeated Testing ◽  
Plasma Growth Hormone ◽  
Once Daily ◽  
Secretory Capacity

Abstract 38 patients with proven growth hormone deficiency (GHD) and 19 children with familial short stature received an iv GRF-bolus injection of 1 ug/kg body weight. Whereas in all control children plasma growth hormone rose significantly (mean of maximal values 36 ng/ml), only 7 out of 38 patients with GHD reached peak values of 8 ng/ml or more. GRF-priming by 1 ug GRF/kg BW given once daily s.c. for 5 days in 19 patients improved the response of the pituitary gland in 11. Thus, following the first GRF test, only 21 % of patients demonstrated function of the pituitary gland whereas 45 % did so when all test results are combined. To evaluate the pituitary function in patients with GHD correctly, GRF tests following a GRF priming period seems to be necessary to reactivate atrophic somatotropic cells of the pituitary gland.

Effect of Growth Hormone-Releasing Factor on Plasma Growth Hormone, Prolactin and Somatomedin C in Hypopituitary and Short Normal Children

1985 ◽  
Vol 22 (1-2) ◽  
pp. 32-45 ◽  
Author(s):  
Guy Van Vliet ◽  
Danièle Bosson ◽  
Claude Robyn ◽  
Margareta Craen ◽  
Paul Malvaux ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Plasma Growth Hormone ◽  
Normal Children ◽  
Somatomedin C ◽  
Growth Hormone Releasing Factor

CHANGES IN PLASMA GROWTH HORMONE LEVELS IN RETARDED CHILDREN

2008 ◽  
Vol 12 (3-4) ◽  
pp. 294-306
Author(s):  
J. HILLMAN ◽  
J. HAMMOND ◽  
J. SOKOLA ◽  
M. REISS
Keyword(s):  
Growth Hormone ◽  
Plasma Growth Hormone ◽  
Hormone Levels

Variation in plasma growth hormone during first parity in lactating cows1

2007 ◽  
Vol 85 (2) ◽  
pp. 388-394 ◽  
Author(s):  
P. Theilgaard ◽  
K. L. Ingvartsen ◽  
P. Løvendahl
Keyword(s):  
Growth Hormone ◽  
Plasma Growth Hormone

PITUITARY FUNCTION AFTER YTTRIUM IMPLANTS AS MEASURED BY PLASMA GROWTH HORMONE LEVELS

1971 ◽  
Vol 50 (1) ◽  
pp. 41-50 ◽  
Author(s):  
HELEN J. STEWART ◽  
E. A. BENSON ◽  
M. MAUREEN ROBERTS ◽  
A. P. M. FORREST ◽  
F. C. GREENWOOD
Keyword(s):  
Breast Cancer ◽  
Growth Hormone ◽  
Advanced Breast Cancer ◽  
Adrenal Function ◽  
Advanced Breast ◽  
Pituitary Function ◽  
Post Mortem ◽  
Plasma Growth Hormone ◽  
Fasting Level ◽  
Plasma Gh

SUMMARY Plasma growth hormone (GH) levels during insulin hypoglycaemia were measured in 30 women with implants of 90Y in the pituitary for advanced breast cancer. There was evidence of continued pituitary activity in six patients (20%), the rise in plasma GH level being greater than 4 ng/ml during hypoglycaemia. Thirteen patients (43%) were regarded as having complete ablations because they had no GH response and a fasting level of less than 4 ng/ml. In the remaining 11 patients (37%) there was no rise in the GH level during hypoglycaemia, but there were significant fasting levels. From the post-mortem evidence it was concluded that these patients also had adequate ablations. This test is shown to be of more value in estimating residual pituitary function than routine tests of thyroid or adrenal function.

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