A Hypopituitary Patient who Attained Tall Stature without Growth Hormone.

“…It has been shown that patients with PSIS form a distinct anatomical and endocrinological syndrome–presenting with signs and symptoms of either isolated growth hormone deficiency or multiple anterior pituitary hormone deficiency. Posterior pituitary function is normal in these patients [3]–[5].…”

Section: Introductionmentioning

confidence: 81%

Pituitary Stalk Interruption Syndrome in Chinese People: Clinical Characteristic Analysis of 55 Cases

Guo

Yang

et al. 2013

PLoS ONE

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ObjectivePituitary stalk interruption syndrome (PSIS) is characterized by the absence of pituitary stalk, pituitary hypoplasia, and ectopic posterior pituitary. Due to the rarity of PSIS, clinical data are limited, especially in Chinese people. Herein, we analyzed the clinical characteristics of patients diagnosed with PSIS from our center over 10 years.Patients and MethodsWe retrospectively analyzed the clinical manifestations and laboratory and MRI findings in 55 patients with PSIS.ResultsOf the 55 patients with PSIS, 48 (87.3%) were male. The average age was 19.7±6.7 years and there was no familial case. A history of breech delivery was documented in 40 of 45 patients (88.9%) and 19 of 55 patients (34.5%) had a history of dystocia. Short stature was found in 47 of 55 patients (85.5%) and bone age delayed 7.26±5.37 years. Secondary sex characteristics were poor or undeveloped in most patients. The prevalence of deficiencies in growth hormone, gonadotropins, corticotropin, and thyrotropin were 100%, 95.8%, 81.8%, 76.3%, respectively. Hyperprolactinemia was found in 36.4% of patients. Three or more pituitary hormone deficiencies were found in 92.7% of the patients. All patients had normal posterior pituitary function and absent pituitary stalk on imaging. The average height of anterior pituitary was 28 mm, documented anterior pituitary hypoplasia. Midline abnormalities were presented in 9.1% of patients.ConclusionsThe clinical features of our Chinese PSIS patients seem to be different from other reported patients in regarding to the higher degree of hypopituitarism and lower prevalence of midline defects. In addition, our patients were older at the time of case detection and the bone age was markedly delayed. We also had no cases of familial PSIS.

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“…In patients with thyroid hormone deficiency, severe growth failure and arrest occurs. Although it is well knownthat GHplays a critical role in normal growth, some patients with GHdeficiency or hypopituitarism are reported to attain normalor even tall stature (2,3). This mechanism remains to be fully elucidated.…”

Section: Introductionmentioning

confidence: 99%

A Patient of Hypogonadotropic Hypogonadism Accompanied by Growth Hormone Deficiency and Decreased Bone Mineral Density Who Attained Normal Growth.

et al. 2000

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Wepresent here a rare case of hypopituitarism accompanied by growth hormone (GH) deficiency and hypogonadotropic hypogonadism, in which the patient attained normal height but was of eunuchoid appearance. A 23-year-old man whohad not reached puberty was referred to Saitama Medical School for hormonal evaluation. Basal hormonal data and hormone-stimulating tests revealed impaired secretion of GH,gonadotropins and adrenocorticotropic hormone (ACTH).Serum levels of testosterone, estrone, estradiol and estriol were all below the detectable ranges. The patient's plasma ACTHresponded to corticotropin releasing hormone, but not to insulin-induced hypoglycemia. Serum GHshowed a minimal response to GHreleasing hormone, but was unresponsive to insulin-induced hypoglycemia. Serum luteinizing hormone and follicle stimulating hormone did not respond to luteinizing hormone-releasing hormone. The results were compatible with a diagnosis of hypothalamic hypopituitarism. Magnetic resonance images of the brain showeda small anterior pituitary, an ectopic posterior lobe and transection of the pituitary stalk. Although the patient showed signs of hypopituitarism, he finally attained normal height, possibly because of failed epiphyseal maturation. His bone mineral density was markedly reduced to 0.647 g/cm2 in the lumbar spine; this level was 61.7 % of the average level of healthy youngmales. Our findings were compatible with a recently advocated view that estrogen is important in promoting epiphyseal fusion and in determining bone density in males as well as females. (Internal Medicine 39: 641-645, 2000)

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A Hypopituitary Patient who Attained Tall Stature without Growth Hormone.

Cited by 7 publications

References 10 publications

A prismatic case: A 31-year old man who did not miss his pituitary

A prismatic case: A 31-year old man who did not miss his pituitary

Pituitary Stalk Interruption Syndrome in Chinese People: Clinical Characteristic Analysis of 55 Cases

A Patient of Hypogonadotropic Hypogonadism Accompanied by Growth Hormone Deficiency and Decreased Bone Mineral Density Who Attained Normal Growth.

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