Cases reported "Dwarfism, Pituitary"

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1/32. Laron dwarfism: growth and immunoreactive insulin following treatment with human growth hormone.

    A 13 1/2-year-old boy with features of growth hormone deficiency had elevated fasting plasma GH levels (5.7 to 66 ng/ml). serum somatomedin values remained low despite treatment with human growth hormone. plasma GH values were suppressed following oral administration of glucose and increased following insulin-induced hypoglycemia, L-dopa, and arginine. chlorpromazine suppressed GH, both fasting and during IIH. These results suggest that the neuroendocrine mechanisms mediating GH secretion seemed to be intact. Peak plasma insulin levels increased in response to glucose administration after HGH suggesting that GH has a direct effect on the pancreatic beta cell which is not mediated by Sm. plasma testosterone values increased to adult male levels, but there was inadequate secondary sexual response. Growth was enhanced by HGH and may have been due to testosterone and/or insulin. Although Laron dwarfism may result from a receptor defect, an abnormality in GH structure is also possible.
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2/32. An unusual MR presentation of the neurohypophyseal "bright spot" in pituitary dwarfism.

    Abnormalities of the hypothalamo-hypophyseal axis are frequently associated with pituitary deficiency. We describe a previously unknown morphological presentation of the ectopic posterior neurohypophyseal "bright spot."
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3/32. Systemic growth hormone corrects sleep disturbance in smith-magenis syndrome.

    smith-magenis syndrome (SMS) is a multiple congenital anomaly syndrome characterized by an interstitial deletion of chromosome 17p11.2. Sleep problems such as nocturnal awakening and abnormality in the percentage of rapid eye movement (REM) sleep are frequently observed in patients with SMS, and several medications have been administered to improve the sleep disorders. Here we present a female case of SMS showing early waking and reduction of REM sleep, which were corrected by human growth hormone (GH) replacement for her dwarfism. Also, we report changes in the sleep-wakefulness circadian rhythm and polysomnographical data before and after the start of human GH replacement. It is speculated that GH deficiency could be involved in sleep disturbance in SMS.
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4/32. pregnancy complicated by multiple pituitary hormone deficiencies.

    We report a case of pituitary dwarfism and diabetes insipidus due to pituitary stalk transection in a pregnant Japanese woman, 138 cm in height, born by breech delivery with no evidence of ante- or intrapartum asphyxia. The patient had no central nervous disturbance, was diagnosed with pituitary dwarfism during childhood and was treated at another hospital with growth hormone supplement from 5 to 14 years of age. This patient was referred to our department at 17 weeks' gestation due to a change of residence. At 30 weeks' gestation, she was hospitalized for assessment of hydronephrosis and polyuria (15-20 L/day). Analysis of a 24-h urine sample showed creatinine clearance of 157 mL/min and urine osmolality of 38 mOsm/L. The patient's urine output decreased after receiving a test dose of 0.75 g of 1-desamino-8-D-arginine vasopressin (DDAVP). Cranial magnetic resonance imaging showed transection of the pituitary stalk. Subsequently, the patient's urine output was well controlled by a maintenance dose of 0.275 mL/day intranasal DDAVP. A cesarean section was performed at 37 weeks, as the patient height was 138 cm, and a pelvic X-ray showed cephalopelvic disproportion. She delivered a female baby weighing 2302 g, and both 1- and 5-min Apgar scores were 9. The patient was followed up after 4 months and showed no visual deterioration or polyuria while on DDAVP therapy, while the neonate grew favorably.
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5/32. An Indian family of hereditary pituitary dwarfism.

    Three girls and one boy out of six siblings born to parents of consanguineous marriage presented with pituitary dwarfism. Their parents were of normal height. All the affected children had features of classical isolated growth hormone deficiency. No hypoglycaemic attacks were noted. Three of them attained puberty at the age of 16 years.
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6/32. Spontaneous pregnancies in two women with Laron-type dwarfism: are growth hormone and circulating insulin-like growth factor mandatory for induction of ovulation?

    It has recently been claimed that growth hormone (GH) and insulin-like growth factors have a role in follicular development; different mechanisms of action have been proposed. Of late, many investigators have been led by these findings to use GH and growth hormone-releasing hormone (GHRH) for induction of ovulation, in combination with human menopausal gonadotrophins. It is, however, still doubtful whether or not growth hormones and/or insulin-like growth factors are mandatory for follicular development and fertility. In this study we describe two women with Laron-type dwarfism who lacked insulin-like growth factors and who had spontaneous pregnancies. We also discuss different natural defects in the production and metabolism of growth hormone and insulin-like growth factors in humans, and the fertility performance of the affected females. It is our assumption that GH and systemic insulin-like growth factors may modulate follicular development, but that they are not necessarily mandatory for ovarian follicular development.
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7/32. Encephalopathy with intracerebral calcification, white matter lesions, growth hormone deficiency, microcephaly, and retinal degeneration: two sibs confirming a probably distinct entity.

    Two sibs with an encephalopathy, including intracerebral calcification and white matter lesions, dwarfism owing to growth hormone deficiency, and retinal degeneration are reported. The onset of the disease in both patients occurred with retardation of motor development during the first year of life. Later, dwarfism, mental retardation, spasticity, ataxia, and retinal degeneration became apparent. These cases probably represent some form of connatal leucodystrophy. The differential diagnosis is discussed.
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8/32. High-affinity serum growth-hormone-binding protein, absent in Laron-type dwarfism, is diminished in heterozygous parents.

    Human serum high-affinity growth-hormone-binding protein (GHBP), as determined by incubation with 125I-GH followed by chromatography on AcA 44 gel minicolumns, is lacking in patients with Laron-type dwarfism (LTD). We found that the specific binding of 125I-GH to high-affinity GHBP in normal human serum (m /- SD) was 11.5 /- 1.8% in 10 children 2-3 years old, 15.3 /- 2.2% in 10 children 5-8 years old, and 19.3 /- 2.9% in 15 adults 20-40 years old. It was 0.3% in a 2-year-old child with LTD, and 10.6 /- 11.3% in his parents. It was 0.1% in another child with LTD, 7 years old, and 14.4 and 14.8% in his parents. The mean value in the heterozygous parents (12.8 /- 2.1%) was significantly lower (p less than 0.001) than control values. A void volume peak (VVP) of radioactivity, corresponding to the so-called low-affinity GHBP which eluted at the void volume in chromatographs of normal sera remained unchanged with sera of patients with LTD or of their parents and appeared even after incubations of the tracer without serum. This study (1) shows that high-affinity GHBP is diminished in heterozygotes with LTD; (2) confirms that high-affinity GHBP and VVP are independently regulated, and (3) suggests that a part of the VVP may not be related to GH binding to some serum components.
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9/32. A case of severe pituitary dwarfism associated with prolactin and thyroid stimulating hormone deficiencies.

    An extreme dwarf 10-year-old boy was described. His clinical features resemble those of isolated GH deficiency type 1A, but the Southern blot analysis showed no gross deletion in the GH structural gene. Endocrinological evaluations showed severe GH and PRL deficiencies, and mild TSH deficiency. The simultaneous deficiencies of anterior pituitary hormones in our patient resemble those of the Snell and Ames dwarf mice and suggest a common etiology. The evolutionary and embryological similarities between GH and PRL imply that mutations at a gene which controls GH and PRL production in somatotropes and lactotropes or at a gene of which product affects the embryological development from a common ancestral cell in the anterior pituitary gland may be a primary defect in our patient.
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10/32. Reversible somatotropin deficiency (psychosocial dwarfism) presenting as conduct disorder and growth hormone deficiency.

    The syndrome of reversible somatotropin deficiency (psychosocial dwarfism) is characterized by environment-related impairment of stature and mental growth and growth-hormone secretion. The syndrome is well illustrated by this case report of an eight-year-old boy with psychosocial dwarfism since the third year of life. It was undetected for four years, despite his having been in regular contact with a number of health workers. After he was removed from the deleterious environment, both his growth and behaviour improved markedly, and at follow-up he had maintained this progress.
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