1/50. 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.- - - - - - - - - - ranking = 1keywords = deficiency (Clic here for more details about this article) |
2/50. Primary central nervous system lymphoma in childhood presenting as progressive panhypopituitarism.We report a 15-year-old boy who had isolated central diabetes insipidus initially diagnosed at age 11 years. A brain magnetic resonance imaging (MRI) was normal at the time. At age 12 years, growth hormone (GH) testing was performed because of a decline in linear growth rate and demonstrated GH deficiency. After a repeat normal brain MRI, GH therapy was begun. Three years later, hormonal testing revealed prepubertal gonadotropins and low testosterone levels, free thyroxine index, and morning cortisol levels. Repeat brain MRI demonstrated a 9-mm enhancing lesion in the region of the pituitary stalk. The pathologic diagnosis was that of a high-grade malignant B-cell lymphoma, suggestive of burkitt lymphoma. Growth hormone therapy has not been associated with an increased incidence of lymphoma. This report underscores the need for vigilance in follow-up brain imaging and hormonal evaluation in children with diabetes insipidus, especially those with evolving anterior hormone deficiencies.- - - - - - - - - - ranking = 1keywords = deficiency (Clic here for more details about this article) |
3/50. 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."- - - - - - - - - - ranking = 1keywords = deficiency (Clic here for more details about this article) |
4/50. Progressive nodular histiocytosis in a child with a hypothalamic tumor.We report a 13-year-old girl with multiple cutaneous histiocytic lesions, precocious puberty, growth hormone deficiency and a hypothalamic tumour. We conclude that she has progressive nodular histiocytosis, but this case illustrates the difficulty in differentiating the type II histiocytoses.- - - - - - - - - - ranking = 1keywords = deficiency (Clic here for more details about this article) |
5/50. megestrol acetate to correct the nutritional status in an adolescent with growth hormone deficiency: Increase of appetite and body weight but only by increase of body water and fat mass followed by profound cortisol and testosterone depletion.megestrol acetate (MA) is a synthetic, orally active derivative of the naturally occurring hormone progesterone. MA is increasingly used to correct loss of appetite and improve the nutritional status. We used MA in an adolescent with growth hormone (GH) deficiency due to former irradiation therapy in order to evaluate if MA can improve the nutritional status. In fact, MA increased appetite and weight dose-dependent. The energy expenditure measured by indirect calorimetry changed from hypo- to normometabolism. However, weight gain was first primarily due to an increase in body water and then in fat mass. The gain of fat mass was much more prominent than the gain of fat free mass. As important side-effect, MA lead to rapid and profound cortisol and testosterone depletion after only 10 days with a long-lasting effect on testosterone depletion. Therefore, MA as a single therapy cannot be recommended to improve the nutritional status. If MA is given, cortisol and testosterone levels have to be monitored and supplemented as needed.- - - - - - - - - - ranking = 5keywords = deficiency (Clic here for more details about this article) |
6/50. Solitary median maxillary central incisor, duane retraction syndrome, growth hormone deficiency and duplicated thumb phalanx: a case report.A 4-year-old Italian child with the association of a solitary median maxillary central incisor, growth hormone deficiency, duane retraction syndrome and a duplicated thumb phalanx is described.- - - - - - - - - - ranking = 5keywords = deficiency (Clic here for more details about this article) |
7/50. 18p- syndrome and hypopituitarism.A patient is described with 18p- syndrome and hypopituitarism. This is the first patient with this syndrome who has been shown to benefit from growth hormone therapy. patients with this syndrome who have growth deficiency should be considered for evaluation for hypopituitarism, if the quality of their lives would improve with an increase in stature.- - - - - - - - - - ranking = 1keywords = deficiency (Clic here for more details about this article) |
8/50. Isolated atlantal stenosis in a patient with idiopathic growth hormone deficiency, and Klippel-Feil and Duane's syndromes.OBJECTIVE AND IMPORTANCE: Isolated atlantal stenosis is very rare with less than ten cases reported, mostly in adult Asians; however, this pathology should be recognized by the neurosurgeon. An unusual case of this rare clinical entity is reported in conjunction with multiple other anomalies. CLINICAL PRESENTATION: We report a Caucasian girl with symptomatic stenosis of the first cervical vertebrae who presented with episodes of loss of tone with subsequent falling, facial cyanosis, urinary incontinence, hand weakness, and difficulties with swallowing. This patient also had the diagnoses of Duane's and Klippel-Feil syndromes, and idiopathic growth hormone deficiency. In addition, this patient was found to have retroflexion of the odontoid process, which further compromised the spinal canal. Flexion-extension radiographs failed to identify cervical spine instability. INTERVENTION: Following suboccipital craniectomy and the removal of the posterior arch of the atlas, the patient's symptoms were resolved and her urinary incontinence improved. CONCLUSIONS: We theorize that our case represents a variant of Wildervanck syndrome. Further case reports are necessary to verify that isolated atlantal stenosis is a component of this constellation of findings. Furthermore, our case demonstrates that degenerative changes of the spine are not necessary for the production of symptoms in cases of isolated stenosis of the atlas.- - - - - - - - - - ranking = 5keywords = deficiency (Clic here for more details about this article) |
9/50. Gingival fibromatosis and growth hormone deficiency syndrome--report of a rare case and review of literature.Oikarinen et al in 1989 reported a syndrome associated with generalized gingival fibromatosis and growth hormone deficiency. This is a case report of a 15-year-old female patient who presented to the government Dental College, Chennai with generalized gingival fibromatosis and growth hormone deficiency. Interestingly, the histopathology of the excised gingival overgrowth showed dense collagenous connective tissue in which were strewn calcified structures that resembled cementum. This syndrome is being reported for the second time after its first case report in 1989 by Oikarinen et al. We are herewith reporting this case for its rarity with a brief review of literature of syndromes associated with generalized gingival fibromatosis.- - - - - - - - - - ranking = 6keywords = deficiency (Clic here for more details about this article) |
10/50. insulin tolerance test causes hypokalaemia and can provoke cardiac arrhythmias.We report the observation and analysis of a new adverse event during the insulin tolerance test (ITT) and propose additional safety procedures. An 8-year-old girl with growth hormone insufficiency had a cardiac arrest due to ventricular flutter when she was tested for growth hormone deficiency by the ITT. Severe hypokalaemia (K 2.6 mmol/l) was observed after resuscitation. ergometry ECG revealed catecholaminergic polymorphic ventricular tachycardia, a hereditary arrhythmogenic disease. Consecutive measurements of serum potassium during ITT in 29 short children (21 boys) with growth failure revealed a mean decrease of serum potassium by 1.1 /- 0.4 mmol/l with the nadir at 30 min after the insulin bolus. Hypokalaemia (serum potassium < 3.5 mmol/l) occurred in all but one child; severe hypokalaemia (serum potassium < 2.9 mmol/l) was measured in every third child. This observation indicates that acute hypokalaemia which is induced by insulin and catecholamine excess occurs frequently in ITT. The case shows that the combination of acute hypokalaemia and the adrenergic counterregulation in ITT is a strong trigger of cardiac arrhythmias, which can become life-threatening if the child has an arrhythmogenic disease. Therefore, we recommend ECG monitoring during ITT to enhance the detection of cardiac arrhythmias. In addition, in the case of a comatose child during ITT the determination of the glucose and potassium level as well as adequate treatment are necessary.- - - - - - - - - - ranking = 1keywords = deficiency (Clic here for more details about this article) |
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