We experienced one case of severe pituitary dwarfism in a 16 years old male boy that magnetic resonance image(MRI) revealed transection of the pituitary stalk with the ectopic location of posterior pituitary gland and hypoplastic anterior pituitary gland. The serum growth horrnone(GH) response to clonidine and L-dopa revealed severe GH deficiency. The patient revealed normal response in TSH, prolactin and LH, FSH to TRH and LHRH respectively. So far, the primary cause of idiopathic pituitary dwarfism in many patients was due to hypoxic injury to hypothalmus by perinatal insults. In this patients, there was no history of perinatal insults and postnatal head trauma. We report a case of severe dwarfism due to hypogenesis of anterior pituitary gland.
Clonidine ; Craniocerebral Trauma ; Dwarfism ; Dwarfism, Pituitary ; Gonadotropin-Releasing Hormone ; Humans ; Levodopa ; Male ; Pituitary Gland ; Pituitary Gland, Anterior ; Pituitary Gland, Posterior ; Prolactin

Hypopituitarism is not a common cause of delayed puberty, however it should always be considered, especially if there are such signs as severe dwarfism, dollish face, truncal obesity, small hands and feet, and microgenitalia. Either congenital or acquired, hypopituitarism can be resulted from hypothalamic and hypophyseal lesions. The clinical feature can be diverse depending on age of the patients, rate of progression, degree of hormone deficiency and characteristics of the lesion. The recent high interest in delayed puberty and the improved detection of hypothalamic hypophyseal lesions using combined pituitary fuction stimulation test, brain CT and MRJ, has made the differential diagnosis of hypopituitarism possible as the cause of delayed puberty. MRI has shown hypophyeal hypoplasia accompanied by anterior pituitary hypoplasia and ectopic neurohypophysis in some of the patients with hypopituitasm, and although the anatomical abnormality around the hypophysis in these patients is considered the reason for hypopituitarism, the pathogensis of which has not yet to be known. We, here, report a case of delayed puberty by hypopituitarism due to hypoplasia of anterior pituitary gland, pituitary stalk agenesis and ectopic neurohypophysis with brief review of the litereature.
Brain ; Diagnosis, Differential ; Dwarfism ; Foot ; Hand ; Humans ; Hypopituitarism ; Magnetic Resonance Imaging ; Obesity ; Pituitary Gland* ; Pituitary Gland, Anterior* ; Pituitary Gland, Posterior* ; Puberty, Delayed*

Pituitary stalk interruption syndrome (PSIS) involves the occurrence of a thin or absent pituitary stalk, hypoplasia of the adenohypophysis, and ectopic neurohypophysis. Diagnosis is confirmed using magnetic resonance imaging. Patients with PSIS have a variable degree of pituitary hormone deficiency and a wide spectrum of clinical manifestations. The clinical course of the disease in our patient is similar to that of a syndrome of inappropriate antidiuretic hormone secretion. This is thought to be caused by failure in the suppression of vasopressin secretion due to hypocortisolism. To the best of our knowledge, there is no case report of a patient with PSIS presenting with hyponatremia as the first symptom in Korean children. Herein, we report a patient with PSIS presenting severe recurrent hyponatremia as the first symptom, during adolescence and explain the pathophysiology of hyponatremia with secondary adrenal insufficiency.
Adolescent ; Adrenal Insufficiency* ; Child ; Delayed Diagnosis* ; Diagnosis ; Humans ; Hyponatremia* ; Hypopituitarism ; Inappropriate ADH Syndrome ; Magnetic Resonance Imaging ; Pituitary Gland* ; Pituitary Gland, Anterior ; Pituitary Gland, Posterior ; Vasopressins

Sheehan's syndrome occurs as a result of ischemic pituitary necrosis due to severe postpartum hemorrhage. The manifestations of this clinical syndrome are most often caused by a deficiency in the hormones produced by the anterior pituitary gland, whereas the neurohypophysis is usually preserved but can be involved in severe cases that manifest as diabetes insipidus. This is a report of Sheehan's syndrome that manifested with diabetes insipidus as presenting symptom 2 month's after delivery. The patient suffered massive bleeding, so received a blood transfusion. A combined pituitary stimulation and water deprivation test revealed deficiencies of not only anterior pituitary hormones, such as growth hormone and prolactin, but also of anti-diuretic hormone. We report this case, with a review of the literature.
Blood Transfusion ; Diabetes Insipidus ; Diabetes Insipidus, Neurogenic* ; Growth Hormone ; Hemorrhage ; Humans ; Hypopituitarism* ; Necrosis ; Pituitary Gland, Anterior ; Pituitary Gland, Posterior ; Pituitary Hormones, Anterior ; Postpartum Hemorrhage ; Prolactin ; Water Deprivation

Sheehan's syndrome occurs as a result of ischemic pituitary necrosis due to severe postpartum hemorrhage. The manifestations of this clinical syndrome are most often caused by a deficiency in the hormones produced by the anterior pituitary gland, whereas the neurohypophysis is usually preserved but can be involved in severe cases that manifest as diabetes insipidus. This is a report of Sheehan's syndrome that manifested with diabetes insipidus as presenting symptom 2 month's after delivery. The patient suffered massive bleeding, so received a blood transfusion. A combined pituitary stimulation and water deprivation test revealed deficiencies of not only anterior pituitary hormones, such as growth hormone and prolactin, but also of anti-diuretic hormone. We report this case, with a review of the literature.
Blood Transfusion ; Diabetes Insipidus ; Diabetes Insipidus, Neurogenic* ; Growth Hormone ; Hemorrhage ; Humans ; Hypopituitarism* ; Necrosis ; Pituitary Gland, Anterior ; Pituitary Gland, Posterior ; Pituitary Hormones, Anterior ; Postpartum Hemorrhage ; Prolactin ; Water Deprivation

Aging ; genetics ; physiology ; Diagnosis, Differential ; Humans ; Hypothalamo-Hypophyseal System ; physiology ; Kidney ; physiology ; Medicine, Chinese Traditional ; Neuroimmunomodulation ; physiology ; Pituitary-Adrenal System ; physiology ; Thymus Gland ; physiology ; Yang Deficiency ; physiopathology

Acute encephalopathies can be defined as an acute central nervous system (CNS) insult, due to an underlying pathology. The clinical symptoms almost always include an acute state of confusion and cognitive impairment. Toxic encephalopathies can occur acutely or chronically depending on the toxic drugs and other substances as well as the individual metabolism of the drug. The organs acutely affected include the heart, lung and kidneys. However, the brain, spinal cord and sympathetic nerves can be affected chronically. If the toxic substance passes through the bloodbrain barrier into the hypothalamus and the posterior pituitary gland, the result can be diabetes insipidus. If the substance affects the anterior pituitary gland, the result can include hormone dysfunction, impaired immune function and altered cognition or personality. We report a patient that developed acute toxic encephalopathy after the prescribed dose of oxycodone was exceeded.
Brain ; Central Nervous System ; Cognition ; Diabetes Insipidus ; Heart ; Humans ; Hypothalamus ; Kidney ; Lung ; Neurotoxicity Syndromes ; Oxycodone ; Pituitary Gland, Anterior ; Pituitary Gland, Posterior ; Spinal Cord

We experienced one case of severe pituitary dwarfism in a 10 years old female girl. Magnetic resonance image (MRI) revealed transection of the pituitary stalk stalk with the formation of high intensity ectopic posterior lobe located at the median eminence and agenesis of an anterior lobe of pituitary gland. The serum growth Hormone (GH) response to clonidine and L-dopa revealed severe GH deficiency. The patient had responses to TRH, normal TSH and partial prolactin response, respectively. There was not response LH and FSH to GnRH. The morning cortisol concentration and serum T4 concentration were decreased below the normal range. These findings and no hyperprolactinemia suggested the presence of a vascular connection between the pituitary gland and hypothalamus, which is not visible on MRI. Sofar, the primary cause of idiopathic pituitary dwarfism in many patients is injury to hypothalamus by perinatal insults. In this patient, there was no history of perinatal insults and postnatal head trauma but transection of the pituitary stalk. We report a case of severe pituitary dwarfism due to agenesis with brief review of related litereature.
Child ; Clonidine ; Craniocerebral Trauma ; Dwarfism, Pituitary* ; Female ; Gonadotropin-Releasing Hormone ; Growth Hormone ; Humans ; Hydrocortisone ; Hyperprolactinemia ; Hypothalamus ; Levodopa ; Magnetic Resonance Imaging ; Median Eminence ; Pituitary Gland* ; Pituitary Gland, Anterior* ; Prolactin ; Reference Values

The pituitary gland develops from two different parts of the brain. The anterior pituitary gland originates from the Rathke pouch and the posterior one from the infundibulum. Therefore, the pathologic findings of congenital hypopituitarism can be different in each case. Congenital hypopituitarism is a rare disorder. The characteristic clinical features of the affected newborns are prolonged jaundice, persistent or recurrent hypoglycemia without hyperinsulinism and microphallus. Their genitalia are usually underdeveloped and sexual maturation may be delayed or absent. In adulthood, patients retain childish feature, short stature with normal body proportion. We experienced a case of congenital hypopituitarism in a 12-year-old female patient with short stature and delayed sexual maturation(Tanner stageI). The endocrinological studies revealed growth hormone, FSH, LH and TSH deficiencies. Magnetic resonance imaging indicated a hypoplastic anterior pituitary and an ectopic posterior pituitary gland located within the tuber cinereum of the hypothalamus.
Brain ; Child ; Female ; Genitalia ; Growth Hormone ; Humans ; Hyperinsulinism ; Hypoglycemia ; Hypopituitarism* ; Hypothalamus ; Infant, Newborn ; Jaundice ; Magnetic Resonance Imaging ; Pituitary Gland ; Pituitary Gland, Anterior ; Pituitary Gland, Posterior* ; Sexual Maturation ; Tuber Cinereum

The pituitary gland develops from two different parts of the brain. The anterior pituitary gland originates from the Rathke pouch and the posterior one from the infundibulum. Therefore, the pathologic findings of congenital hypopituitarism can be different in each case. Congenital hypopituitarism is a rare disorder. The characteristic clinical features of the affected newborns are prolonged jaundice, persistent or recurrent hypoglycemia without hyperinsulinism and microphallus. Their genitalia are usually underdeveloped and sexual maturation may be delayed or absent. In adulthood, patients retain childish feature, short stature with normal body proportion. We experienced a case of congenital hypopituitarism in a 12-year-old female patient with short stature and delayed sexual maturation(Tanner stageI). The endocrinological studies revealed growth hormone, FSH, LH and TSH deficiencies. Magnetic resonance imaging indicated a hypoplastic anterior pituitary and an ectopic posterior pituitary gland located within the tuber cinereum of the hypothalamus.
Brain ; Child ; Female ; Genitalia ; Growth Hormone ; Humans ; Hyperinsulinism ; Hypoglycemia ; Hypopituitarism* ; Hypothalamus ; Infant, Newborn ; Jaundice ; Magnetic Resonance Imaging ; Pituitary Gland ; Pituitary Gland, Anterior ; Pituitary Gland, Posterior* ; Sexual Maturation ; Tuber Cinereum