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PubMedDate not supplied

MR imaging of pituitary dwarfism.

The purpose of this study was to determine whether the MR findings of idiopathic pituitary dwarfism correlated with a clinical history of perinatal abnormalities and abnormal levels of pituitary hormones. MR examinations of 18 patients with pituitary dwarfism were performed; these patients were divided into two groups: those with ectopic posterior lobes (group 1) and those with normal posterior lobes (group 2). Among the seven patients in group 1, MR showed hyperintense signal at the median eminence of the hypothalamus, which was regarded as the ectopic posterior lobe of the pituitary gland. The mean anteroposterior length and height of the pituitary gland in group 1 patients were significantly smaller than those dimensions in the normal control group; the pituitary stalk was not detected in three of seven patients. Six of seven patients were products of breech presentation with perinatal asphyxia. The peak serum growth hormone level was less than 5 ng/ml when assessed by insulin-induced hypoglycemia or the clonidine test. MR findings in the 11 patients with pituitary dwarfism and normal posterior lobes were normal except that the mean size of the pituitary gland was slightly smaller than that of normal controls. The clinical history of these patients was normal except for perinatal asphyxia in one case. Our findings in patients with pituitary dwarfism, with or without an ectopic posterior pituitary lobe suggest that the ectopic lobe, visualized as a bright spot at the median eminence of the hypothalamus, may be common when pituitary dwarfism follows perinatal anoxic/ischemic episodes.

Adolescent↗
PubMed1980

[Pituitary dwarfism in a German shepherd dog (author's transl)].

Pituitary dwarfism has been described in more than 20 German shepherd dogs. Some cases probably were caused by persistent cysts of Rathke's pouch. This is the first histopathological, immunohistochemical and endocrinological study. A 13-month-old, 7-kg, dwarf purebred German shepherd bitch with alopecia and hyperpigmentation was admitted to the clinic for euthanasia. Retardation of growth was noticed when the dog was about two months old. No littermates had this condition. Two subsequent breedings from the same parents produced normal offspring. The clinical parameters in our dog (hematology, function of liver and kidney) were normal. Grossly, the pituitary gland had small and large multiple cysts, which light microscopy showed to be exclusively within the adenohypophysis. The latter had pressure atrophy, and immunohistochemically showed only remnants of the hormone-producing cells (growth hormone-GH; prolactin-PRL; thyrotropin-TSH; luteinizing hormone-LH; adrenocorticotrophin-ACTH/MSH). The thyroid was relatively small, and histologically showed focally resting follicles without lumen. Endocrinological studies showed a surprisingly high value for serum growth hormone (cGH 4.1 ng/ml; normal range 1.8-3.8 ng/ml as determined by a specific homologous radioimmunoassay) and a pathologically low serum somatomedin (SM 0.132; normal value more than 0.50 unit/ml, determined by 35S incorporation in piglet rib cartilage). Hypothyroidism was verified by a low T4 binding value (T4 0.9 micrograms/100 ml; normal 4.1 +/- 0.9 micrograms/100 ml) and a low thyroid binding index (TBI 0.54; normal 0.61 +/- 0.05). While ACTH was lowered (ACTH less than 10 pg/ml; normal 74-210 pg/ml), cortisol was normal (0.81 micrograms/100 ml; normal 0.5-2.5 micrograms/100 ml). Pituitary dwarfism in the 13-month-old bitch can be ascribed to the persistence of one end of the ductus craniopharyngeus, Rathke's pouch. Pressure atrophy of the adenohypophysis led to the loss of most of the hormone-producing tissue. An increase in growth hormone with lowered somatomedin raises questions. We have no conclusive explanation for this, due to the present lack of knowledge of how growth is regulated. High growth hormone and low somatomedin values are found in Laron's syndrome in infants. The literature indicates that pituitary dwarfism in German shepherd dogs may be a hereditary autosomal recessive trait.

Adrenocorticotropic Hormone↗
PubMed2002

Use of proligestone in the management of three German shepherd dogs with pituitary dwarfism.

Three German shepherd dogs were diagnosed with pituitary dwarfism and subsequently treated with proligestone. Treatment resulted in development of an adult hair coat, increased bodyweight and elevated insulin-like growth factor-1 concentration. Two dogs received thyroid supplementation during proligestone therapy. Adverse effects (cystic endometrial hyperplasia and acromegaly) were reported in two cases. No side effects were reported in the remaining case. This is the first report of the use of proligestone in the management of pituitary dwarfism.

Acromegaly↗
PubMed1984

Long term effects of human growth hormone on 1,959 patients with pituitary dwarfism throughout Japan.

The results of 6 months to 8 years of treatment of pituitary dwarfism in 1,959 patients in Japan were summarized. The data were based on the reports of the physicians treating the patients. Among the patients, 1,720 cases suffered from idiopathic pituitary dwarfism, 227 cases from secondary pituitary dwarfism, and 12 cases from unknown causes. Their initial age ranged from 9 months to 36 years old and their initial bone age ranged from 3 months to 15 years old. In most cases, a definite effect was observed. The effect was most remarkable during the initial 6 months and then declined gradually up to the fourth year. Males responded better than females. In younger and more immature patients, the effect was more remarkable. There were almost no side effects other than a few cases of impairment of capital femoral epiphysis.

Adolescent↗
PubMed2002

Measurement of zinc, copper, manganese, and iron concentrations in hair of pituitary dwarfism patients using flameless atomic absorption spectrophotometry.

Pituitary dwarfism (hGHD) is known to be associated with trace element deficiency, which causes improper functioning of the involved endocrine system. Previously, we reported on the head hair concentrations of zinc, copper, manganese, and iron from a total of 418 normal subjects (154 male and 264 female). In this report, we analyzed the head hair concentrations of the same four trace metals of 103 hGHD children (60 male and 43 female) under treatment with human growth hormone (hGH). These subjects ranged in age from 5 to 18 yr. The results were compared with 338 age-matched normal subjects (120 male and 218 female). Both male and female hGHD showed approx 1.7 times higher zinc concentrations than normal subjects. Cheruvanky et al. reported a similar trend but with a slightly lower difference between hGHD and normal subjects. The average copper content in the hair of both male and female subjects also showed higher values for the hGHD than for the normal subjects, a trend similar to the values reported by Teraoka et al. In the case of manganese, the concentrations in hair of the hGHD were approx 50% of the values in the normal subjects. Head hair concentrations of iron in the hGHD were commensurate with the normal subjects. Because the content of trace elements in hair varies with the age of subjects, as a control, we investigated the head hair concentration of zinc from 20 healthy girls ranging in age from 10 to 18 yr. The average zinc concentration decreased from 10 to 12 yr, but no clear relation to age was observed from 13 yr and older. These trends were similar to our previous report. The zinc concentration in hair and body weight gain over a year was negatively correlated. The age variation in the content of zinc, copper, manganese, and iron in hair was measured comparing hGHD with the normal subjects in various ages. Concerning the zinc-level variation of hGHD and normal subjects, there were conspicuous differences between hGHD and normal subjects. For copper, the variations in concentration with age were similar to zinc. Regarding the age variations for manganese, hGHD had lower concentrations in hair compared to the normal subjects throughout adolescence (11-18 yr). We have studied the effects between the hair and these trace element concentrations in hGHD before and after hGH administration. These results suggest that hGH affects the metabolism of these trace elements.

Adolescent↗
PubMed1989

Ectopic posterior lobe of the pituitary gland and intractable nocturnal enuresis in a case with pituitary dwarfism.

An 11 5/12-year-old girl with pituitary dwarfism had intractable nocturnal enuresis. Magnetic resonance imaging revealed pituitary stalk transection and the formation of an ectopic posterior lobe. The results of responses to dehydration, infusion of hyperosmolar NaCl solution, and 1-desamino-8-D-arginine vasopressin showed that the nocturnal enuresis in the present case was due to a partial vasopressin deficiency. We suggest that the ectopic posterior lobe is one cause of nocturnal enuresis.

Child↗
PubMed1988

Secretion of growth hormone-releasing hormone in patients with idiopathic pituitary dwarfism and acromegaly.

The plasma levels of immunoreactive-GHRH in patients with idiopathic pituitary dwarfism and acromegaly were studied in the basal state and during various tests by a sensitive and specific RIA. The fasting plasma GHRH level in 22 patients with idiopathic pituitary dwarfism was 6.3 +/- 2.3 ng/l (mean +/- SD), which was significantly lower than that in normal children (9.8 +/- 2.8 ng/l, N = 21), and eight of them had undetectable concentrations (less than 4.0 ng/l). Little or no response of plasma GHRH to oral administration of L-dopa was observed in 7 of 10 pituitary dwarfs, and 3 of the 7 patients showed a response of plasma GH to iv administration of GHRH (1 microgram/kg). These findings suggest that one of the causes of idiopathic pituitary dwarfism is insufficient GHRH release from the hypothalamus. The fasting plasma GHRH level in 14 patients with acromegaly and one patient with gigantism was 8.0 +/- 3.9 ng/l, which was slightly lower than that in normal adults (10.4 +/- 4.1 ng/l, N = 72). One acromegalic patient with multiple endocrine neoplasia type I had a high level of plasma GHRH (270 ng/l) with no change in response to L-dopa and TRH test. In 3 untreated patients with acromegaly L-dopa did not induce any response of plasma GHRH in spite of inconsistent GH release, and in 4 patients with acromegaly, TRH evoked no response of plasma GHRH in spite of a marked GH release, suggesting that the GH responses are not mediated by hypothalamic GHRH.(ABSTRACT TRUNCATED AT 250 WORDS)

Acromegaly↗
PubMed1979

Serum levels of somatomedin A and growth during long-term treatment of patients with pituitary dwarfism with human growth hormone.

Eighteen patients with pituitary dwarfism were treated for 1 7/12 to 6 years with human growth hormone (hGH) at a dose of 0.19--0.62 unit (U) per kg of body weight per week. The mean increment in height was 2.0 +/- 0.4 and 8.8 +/- 0.5 cm/year before and the first year after treatment of hGH, respectively. A significant positive correlation was observed between serum levels of somatomedin A and growth rate, espcially in children with bone age below 10 and a duration of treatment of less than one year (r = 0.66, P less than 0.005). Long-term treatment with hGH was accompanied by a decreasing response. However, the serum levels of somatomedin A did not decrease significantly. Therefore, decreased growth increment in these situations was not due to decreased serum levels of somatomedin A.

Adolescent↗
PubMed1983

Treatment of idiopathic pituitary dwarfism with methionyl human growth hormone.

Ten patients with idiopathic pituitary dwarfism were treated with methionyl human growth hormone (met-hGH) produced by recombinant DNA technology. They were given 0.5 IU/kg/week of met-hGH for three months. There were no significant changes in physical, blood, urine examinations. Their height increased between 1.5 and 2.7 cm during the 3 months of treatment, which was calculated to be equivalent 6.0 and 10.8 cm per year with a mean increase of 8.7 cm. Anti-hGH antibody was observed in all patients between one and two months of treatment with a titer between 10(1)X and 10(4)X. In spite of the formation of antibodies, the growth rate did not decrease in 7 of the 10 cases.

Adolescent↗
PubMed1975

[Reactivity of peripheral blood lymphocytes in children with pituitary dwarfism treated with human growth hormone (author's transl)].

The study has aimed at investigating the blastic transformation of peripheral blood lymphocytes in patients with pituitary dwarfism before, during and after the treatment with human growth hormone. The reactivity of lymphocytes in vitro was studied in cultures stimulated with phytohemagglutinin-M of the Difco Company (PHA), in cultures stimulated with human growth hormone (HGH), and in cultures non-stimulated in vitro. The investigations were carried out in 34 patients with pituitary dwarfism at the age of 7 to 19 years. The control group was made up of 16 healthy children, 8 to 15 years old who had never been administered HGH. The patients were divided into groups according to the circumstance whether it was the first or the subsequent contact with HGH, and according to the periods at which the blood samples were collected during after therapy. It was found that the capability of lymphocytes to blastic transformation after stimulation with a specific stimulus (HGH) in patients with pituitary dwarfism in comparison with the reaction of healthy children is reduced (Fig. 2). The ability of the transformation of lymphocytes in this case shows selectivity towards the stimulus because the inhibiting of the response was noted only after the stimulation with HGH, but not after stimulation with PHA. During the treatment with HGH, allergization of the body appears; this is evidenced by the increase in the percentage of blastic cells in autotransformation (Fig. 1).

Adolescent↗