Hypopituitarism Presenting as Osteoporotic Fracture after Cured Tuberculous Meningitis

Tuberculous meningitis is a well-known cause of hypothalamic pituitary dysfunction. However, deficiencies of anterior pituitary hormones may only become evident years after recovery because symptoms are of insidious-onset and nonspecific. Pituitary hormones are essential for normal growth and sexual development in childhood, and for maintenance of healthy body composition. In addition, pituitary hormones and vitamin D are important for cardiovascular and bone health. Although evidence of the relationship between hypovitaminosis D and hypopituitarism is limited, some studies suggested that the incidence of vitamin D deficiency increased in hypopituitarism. We describe herein an unusual case of hypopituitarism and severe hypovitaminosis D presenting as osteoporotic fracture after cured tuberculous meningitis.

Solitary Pituitary Metastasis of Advanced Breast Cancer Treated with Anti-Human Epidermal Growth Factor Receptor 2 Drug

Metastasis to the pituitary gland from systemic cancer is a rare condition. The breast and lung are the most common sites of primary tumor metastasis. Most often, they occur in the setting of widespread metastatic disease, which most frequently occurs in elderly patients. However, an increase in the incidence of solitary pituitary metastasis of breast cancer as the first recurrence has been reported. Diabetes insipidus is the most frequent symptom at presentation, and visual field defects or cranial nerve deficits are common symptoms of pituitary metastasis. Unlikely these symptoms, deficiencies of anterior pituitary hormones may only become evident in critical situation because symptoms are of an insidious onset and sometimes nonspecific. We report here on a rare case of solitary pituitary metastasis from breast cancer presenting as hyponatremia without other symptoms.

Electrolyte Imbalance in Patients with Sheehan's Syndrome

BACKGROUND: We investigated the prevalence of electrolyte imbalance and the relationship between serum electrolyte and anterior pituitary hormone levels in patients with Sheehan's syndrome. METHODS: In a retrospective study, we investigated 78 patients with Sheehan's syndrome. We also included 95 normal control subjects who underwent a combined anterior pituitary hormone stimulation test and showed normal hormonal responses. RESULTS: In patients with Sheehan's syndrome, the serum levels of sodium, potassium, ionized calcium, magnesium, and inorganic phosphate were significantly lower than those in control subjects. The prevalence of hyponatremia, hypokalemia, hypocalcemia, hypomagnesemia, and hypophosphatemia in patients with Sheehan's syndrome was 59.0% (n=46), 26.9% (n=21), 35.9% (n=28), 47.4% (n=37), and 23.1% (n=18), respectively. Levels of sodium and ionized calcium in serum were positively correlated with levels of all anterior pituitary hormones (all P<0.05). Levels of potassium in serum were positively correlated with adrenocorticotrophic hormone (ACTH) and growth hormone (GH) levels (all P<0.05). Levels of inorganic phosphate in serum were positively correlated with levels of thyroid-stimulating hormone, prolactin, and GH (all P<0.05), and levels of magnesium in serum were positively correlated with delta ACTH (P<0.01). CONCLUSION: Electrolyte imbalance was common in patients with Sheehan's syndrome. Furthermore, the degree of anterior pituitary hormone deficiency relates to the degree of electrolyte disturbance in patients with this disease.

Neonatal cholestasis in congenital pituitary hormone deficiency and isolated hypocortisolism: characterization of liver dysfunction and follow-up / Colestase neonatal na deficiência congênita de hormônio hipofisário e hipocortisolismo isolado: caracterização da disfunção hepática e acompanhamento

INTRODUCTION: Neonatal cholestasis due to endocrine diseases is infrequent and poorly reco-gnized. Referral to the pediatric endocrinologist is delayed. OBJECTIVE: We characterized cholestasis in infants with congenital pituitary hormone deficiencies (CPHD), and its resolution after hormone replacement therapy (HRT). SUBJECTS AND METHODS: Sixteen patients (12 males) were included; eleven with CPHD, and five with isolated central hypocortisolism. RESULTS: Onset of cholestasis occurred at a median age of 18 days of life (range 2-120). Ten and nine patients had elevated transaminases and γGT, respectively. Referral to the endocrinologist occurred at 32 days (range 1 - 72). Remission of cholestasis occurred at a median age of 65 days, whereas liver enzymes occurred at 90 days. In our cohort isolated, hypocortisolism was a transient disorder. CONCLUSION: Cholestasis due to hormonal deficiencies completely resolved upon introduction of HRT. Isolated hypocortisolism may be a transient cause of cholestasis that needs to be re-evaluated after remission of cholestasis.

INTRODUÇÃO: A colestase neonatal causada por doenças endócrinas é pouco frequente e reconhecida. Existe um atraso no encaminhamento dos pacientes a um endocrinologista pediátrico. OBJETIVO: Caracterizamos a colestase em recém-nascidos com deficiências congênitas de hormônio hipofisário (DCHH) e sua resolução após a terapia de reposição hormonal (TRH). SUJEITOS E MÉTODOS: Dezesseis pacientes (12 do sexo masculino) foram incluídos; sete com DCHH, e cinco com hipocortisolismo central isolado. RESULTADOS: O início da colestase ocorreu aos 18 dias de vida (variação 2-120). Dez e nove pacientes apresentaram elevação das transaminases e γGT, respectivamente. A consulta com um endocrinologista aconteceu aos 32 dias (variação 1-72). A remissão da colestase ocorreu em uma idade mediana de 65 dias, enquanto a remissão das enzimas hepáticas aconteceu aos 90 dias. Na coorte isolada, o hipocortisolismo foi uma desordem transitória. CONCLUSÃO: A colestase causada por deficiências hormonais foi completamente resolvida após a introdução da TRH. O hipocortisolismo pode ser uma causa transitória da colestase e precisa ser reavaliado após a remissão da colestase.

A Case of Pituitary Adenoma with Simultaneous Secretion of TSH and GH

Thyrotropin (TSH)-secreting pituitary adenoma is a very rare disease. In one-quarter of patients suffering from this disease, the pituitary tumor secretes other anterior pituitary hormones. Herein, we report a case of pituitary adenoma with simultaneous secretion of TSH and growth hormone (GH). A 34-year-old female visitied local hospital complaining of sweating, intermittent palpitation, and weight loss of 8 kg within 1 year. The patient had undergone trans-sphenoidal surgery 3 years prior for resolution of a TSH and GH co-secreting pituitary adenoma. She had been administered somatostatin analogue prior to visiting our hospital. The patient's GH levels were suppressed to below 1 ng/mL on the 75 g oral glucose tolerance test, and her basal insulin-like growth factor-I (IGF-I) level was within normal range. Thyroid function tests demonstrated increased levels of both free thyroxine and TSH. Sella-MRI revealed pituitary adenoma at the floor of the pituitary fossa, approximately 2 cm in height. Therefore, she was diagnosed with residual TSH-secreting pituitary adenoma. The patient again underwent trans-sphenoidal surgery and entered complete remission, based on hormone levels and MRI findings.

Anterior pituitary hormones in systemic lupus erythematosus patients

To evaluate the function of anterior pituitary gland in SLE patients and its association with different disease manifestations and disease activity index. The study was conducted on 20 female SLE patients, and 12 healthy age matched female controls. The mean age of SLE patients was 28.95 +/- 5.216 years. All the patients were subjected to full history taking, general examination, locomotor system examination, and laboratory investigations including complete blood count, ESR, urine analysis, liver function tests, serum creatinine, ANA and anti n-DNA antibodies. Disease activity was assessed on the day of endocrinological investigations using SLEDAI. Basal serum levels of anterior pituitary hormones: adrenocorticotropic hormone [ACTH], prolactin [PRL], follicle stimulating hormone [FSH], luiteinizing hormone [LH], growth hormone [GH], and thyroid stimulating hormone [TSH] were measured in all individuals by ELISA. SLE patients had statistically significant higher basal levels of FSH [mean 11.245 +/- 2.76 SD P=0.0001], LH [mean 2.358 +/- 3.6 SD P=0.001] and PRL [mean 19.34 +/- 7.79SD P=0.033].PRL serum levels was significantly positively correlated with disease activity measured by SLEDAI [r=0.683, P=0.001] as well as ESR [r=0.571, P=0.009]. Patients with lupus nephritis had higher levels of PRL [mean 22.9 +/- 7.089 SD compared to patients without nephritis, with P=0.02.The patients had lower levels of ACTH compared to control subjects but the difference was not statistically significant [P=0.07]. Higher than normal levels of prolactin in SLE patients appear to constitute immunostimulatory hormone environments in this disease. The presence of pituitary hormones abnormalities suggest that predisposing or even modulatory relationships exist between lupus disease and LH, FSH and PRL

Silent Adenomas of Pituitary Gland: It's Immunohistochemical Features and Clinical Characteristics

OBJECTIVE: The aim of the study was to review the clinical and radiological findings of those non-functioning adenomas(NFAs) with positive immnoreactivity for anterior pituitary hormones. METHODS: Sixty patients with pituitary adenoma were treated at the author's institution between January 2000 and July 2005. All consecutive patients were underwent transsphenoidal surgery by same operator. In addition to the routine histopathological examination, surgical specimen was examined by immunohistochemical staining against adenohypophyseal cells. And clinical analysis was performed by retrospective review of medical records, neuroimaging examinations and immunohistochemical technique. We classified these pituitary adenomas into functioning adenomas (group F), immunopositive NFAs (group S, so-called silent adenoma) and immuno-negative NFAs (group N), and compared clinical and radiological differences between group F, N, and S. RESULTS: Of the 60 cases, group F was 25, group S was 25, and group N was 10. Among the group S, 5 cases showed reactivity against PRL, 1 against GH, 1 against both PRL and GH, 1 against TSH and GH, 2 against ACTH, 11 against FSH and 4 against both LH and FSH. Radiologically, invasiveness was noted in 8 in group S, compared to 3 in group N and 1 in group F (p=0.02). Intratumoral bleeding was noted in 7 of group S, 2 of group N and 2 of group F (p>0.05). CONCLUSION: Silent adenomas were thought to behave more aggressive than other subgroups of pituitary adenomas. And so we suggest the immunohistochemical study against adenohypophyseal cells may be helpful for evaluating clinical course of pituitary adenoma, expecially for, NFAs.

A Case of Sheehan's Syndrome Presenting Central Diabetes Insipidus / 대한내분비학회지

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.

A Case of Sheehan's Syndrome Presenting Central Diabetes Insipidus / 대한내분비학회지

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.

Growth hormone treatment in a girl with Prader Willi syndrome.

Prader Willi syndrome (PWS) is a rare endocrine-metabolic disorder that is characterised by neonatal hypotonia, hyperphagia, marked obesity, short stature, hypogonadism and behavioural problems. 7-20% percent of these children develop diabetes mellitus. A large number of individuals with PWS show growth hormone (GH) deficiency. Recent studies indicate beneficial effects of GH replacement therapy not only for their linear growth but also for correction of metabolic dysfunction. In the present communication this article details about the therapeutic outcome in a girl with PWS who received recombinant growth hormone (rGH), Genotropin. Some carry-over therapeutic benefits have been observed even after discontinuation of rGH.

A Case of Anterior Pituitary Agenesis in an Adult Woman / 대한내분비학회지

Dear Author, You have used abbreviations that will need to be defined in the main paper, i.e. PIT1, PROP1 and MRI. This is just for your advice. Pituitary agenesis is an uncommon cause of panhypopituitarism. It has been proposed that breech delivery, or birth trauma, is a major factor causing pituitary agenesis. Recent studies have suggested that genetic defects in the PIT1 or PROP1 gene might be involved in the pathogenesis of pituitary agenesis. In this case we report on the diagnosis of a 33-years old female patient with-growth retardation and sexual infantilism. We diagnosed anterior pituitary hormones deficiencies, with the exception of adrenocorticotropic hormone, by a combined pituitary stimulation test. We observed pituitary agenesis using sella MRI. Involvement of the PIT1 or PROP1 genes in this case remains to be determined. Here we report a case of pituitary agenesis found in an adult woman together with a brief review about this disease entity.

The Combined Pituitary Stimulation Test in Patients Suffered from Massive Postpartum Hemorrhage / 대한내분비학회지

BACKGROUND: ackground: Sheehan's syndrome secondary to severe postpartum hemorrhage is one of the major causes of pituitary insufficiency in Korea. Most of these patients do not manifest symptoms or signs of gross endocrinopathies. Earlier detection of pituitary insufficiency is of clinical importance. The combined pituitary stimulation test that uses the four hypothalamic releasing hormones is a rapid, safe, and effective way to evaluate anterior pituitary function. However, the criteria for a normal response has not been established in Korea. METHODS: Combined anterior pituitary stimulation tests were performed on fourteen healthy women who had no history of endocrine disease. Combined tests of anterior pituitary reserve were done no forty-five patients who suffered from massive postpartum hemorrhage which required transfusing, along with subsequent shock or changing consciousness and in thirty-nine patients who experienced mild postpartum hemorrhage. RESULTS: 1) In the severe hemorrhage group, thirty-three of forty-five women (73.3%) showed blunted responses in more than one of the anterior pituitary hormones in the combined pituitary stimulation tests. However, in the mild hemorrhage group, only eighteen of thirty-nine women (46.2%) demonstrated blunted responses of more than one of the anterior pituitary hormones. 2) In the severe hemorrhage group, the TSH response was blunted in twenty-five patients (55.6%), prolactin in eleven patients (24.4%), ACTH in ten patients (22.2%), LH in ten patients (22.2%), GH in nine patients (20%), and FSH in five patients (11.1%). 3) The results of combined pituitary stimulation tests in the normal control group were different from the results of other studies. CONCLUSION: It is recommended that the women who experienced a severe postpartum hemorrhage should be evaluated by using the combined pituitary stimulation test. Moreover, criteria for a normal response to the combined pituitary stimulation test should be established in Korea.

The Effect of reconstituted basement membrane on the expression of the anterior pituitary hormones of the rat / 대한해부학회지

This study was performed to investigate the effect of Matrigel, a reconstituted basement membrane, on the expression of the anterior pituitary hormones in culture. Rat pituitary cells cultured for 6 days on Matrigel showed 3-dimensional, lobular structures with connecting cells while those on plastic showed flat, polygonal cells forming a monolayer. Western blot analysis showed that prolactin (PRL) content in the anterior pituitary cells was higher compared to those cultured on plastic. In comparison, TSH expression was not increased in cultures on Matrigel. The total cell number and the proportion of fibroblasts was decreased. These results suggested that Matrigel is a useful culture substrate for the enhanced expression of PRL but not for TSH. Further studies are needed in order to find a useful culture substrate for TSH cells.

A Case of Acromegaly Caused by Mixed Gangliocytoma-Adenoma of the Pituitary Gland / 대한내분비학회지

The tumors containing ganglion cells are rare in the pituitary gland. These tumors are divided into two groups. The first group is the cases of mixed gangliocytoma-adenoma that contained both gangliocytoma and pituitary adenoma elements in the same tumor and the second group is those of gangliocytoma that contained only gangliocytoma element. Since the first description by Kiyono in 1926, 45 cases have been reported. The most common presentation of these tumors is acromegaly and the cases of Cushings disease and galactorrhea-amenorrhea also have been reported. In immunohistochemical study, hypothalamic-releasing hormones are stained in gangliocytoma cells and adenohypophyseal hormones are stained in adenoma cells. The releasing hormones stained in gangliocytoma element and the pituitary hormones stained in adenoma elements are usually closely related but unrelated cases also have been reported. We report a case of a 41 year-old lady with acromegaly which was diagnosed as mixed gangliocytoma-adenoma after surgical removal of the pituitary tumor.

Pseudohermafroditismo masculino por deficiencia enzimática de 17 alfahidroxilasa; primer caso reportado en Puerto Rico / Male pseudohermaphroditism caused by enzymatic deficiency of 17-alpha-hydroxylase. 1st case reported in Puerto Rico

A 36 year old white female came to our service after having been evaluated on repetitive occasions in the past for a workup of gigantism and acromegalic features. Since childhood she had developed tall stature, frontal bossing, prominence of zygomatic bones, separated teeth, large hands and size 14 shoes. Human growth hormone and somatomedin serum levels had been normal on all occasions tested. Her past history was significant for primary amenorrhea and a 12 year history of hypertension. On physical examination BP was 140/100, height 6' 2[quot ], weight 2571 bs. Her phenotype was truly acromegalic. There was absence of axillary and pubic hair with no breast development. External genitalia was of female appearance. Laboratory evaluation showed increased FSH of 88 mlU/ml, increase LH of 65.6 mlU/ml and decreased E2 of 12.6 pg/ml. Other findings were low serum cortisol of 0.2 mg/dl, high ACTH of 344 pg/ml, low 17-Ketosteroids, high pregnenolone levels of 595 mg/dl, low 17-hydroxypregnenolone less than 10 ng/dl, very high aldosterone of 31 ng/dl and suppressed PRA of less than 0.1 ng/ml. A pelvic sonogram showed a right ovoid structure which could represent a gonad and failed to identify the uterus and left gonad. A bone densitometry showed a decrease bone mineral density compatible with osteoporosis. Chromosome study showed a karyotype of 46-XY. A diagnosis was made of congenital adrenal hyperplasia secondary to 17-alpha-hydroxylase deficiency in a genotypic male. Our patient was referred to the department of gynecology for surgical removal of the gonads. It is amazing how a patient with severe adrenal insufficiency can withstand 36 years of her life undiagnosed without going into an adrenal crisis. Her tall stature and acromegalic features were the striking signs confusing all physicians and delaying the correct diagnosis and appropriate treatment. There has been reported worldwide, nearly 120 cases with documented severe 17-alpha-hydroxylase deficiency. To our knowledge this is the first case identified in Puerto Rico of male pseudohermaphroditism secondary to 17-alpha-hydroxylase enzyme deficiency

A Case of Pituitary Metastasis of Lung Presenting as Diabetes Insipidus / 대한내분비학회지

This is a case report of a 61 years old man with suprasellar and pituitary metastasis from bronchogenic adenocarcinoma who developed polyuria and polydipsia. The clinical diagnosis has been made by bronchoscopy with washing cytology, biopsy of cervical lymph node, and brain MRI scan. Brain MRI scan showed metastatic tumor in the suprasellar area and pituitary gland with invasion of pituitary stalk. Light microscopic findings revealed adenocarcinoma in bronchoscopic washing cytology, and metastatic adenocarcinoma in the cervical lymph node. The combined pituitary stimulation test showed decreased reserve capacity of anterior pituitary hormones. And the water deprivation test also showed complete central diabetes inspidus. We report a case of suprasellar and pituitary metastasis from bronchogenic adenocarcinoma with a review of the literature.

A Case of Isolated ACTH Deficiency Accompanied by hyperprolactinemia / 대한내분비학회지

Isolated ACTH deficiency is a uncommon disorder causing secondary adrenocortical insufficiency. Less than 200 cases have been reported in the world. The major clinical manifestations are hypoglycemia, weight loss, hypotension, anemia, weakness, nausea, inability to excrete water load, and hyponatremia. A 56-year-old male was admitted because of weakness, fatigue, nausea and vomiting. He was pallor and not associated with hyperpigmentation. The basal plasma ACTH and cortisol levels were 8.30 pg/ml and 0.6 ug/dl. The serum cortisol did not response to rapid ACTH stimulation test. On combined pituitary stimulation test, the cortisol did not response to insulin-induced hypoglycemia. Other anterior pituitary hormones showed normal responses except elevated prolactin level. Six months after glucocorticoid replacement therapy, the elevated basal prolactin level returned to normal. Brain MRI did not show any anatomic abnormalities of the sellar and suprasella area. We report a case of isolated ACTH deficiency accompanied by hyperprolactineia, which respond to glucocorticoid replacement therapy.

The adenohypophysis of the swamp eel, Synbranchus marmoratus, an immunocytochemical analysis

The adenohypophyseal cell types of the protogynous fish Synbranchus marmoratus were studied by histochemical and immunocytochemical staining with antisera raised against piscine and human pituitary hormones to ascertain their distribution. The prolactin (PRL) cells were distributed in the rostral pars distalis and showed specific binding to antisera to carp and chum salmon prolactin. No reaction was observed with antiserum to human prolactin. The corticotrops showed strong immunoreactivity with anti-human ACTH, these cells bordered the neurohypophysis and islets between PRL cells in the rostral pars distalis. Growth hormone (GH) cells were densely distributed and associated with the neurohypophysis only in pars distalis proximal. They reacted with antisera to piscine GH but not with antisera to human growth hormone. The thyrotrops were scattered in the proximal pars distalis and showed strong immunoreactivity to the human thyrotropin Beta subunit antiserum. Gonadotrops were located in the central area of the proximal pars distalis and in the external border of the pars intermedia. These cells were alcian blue and PAS positive, and reacted with anti-croaker GTH and anti-coho GTH I and GTH II. The PAS positive cells from the pars intermedia bound specifically to anti-chum somatolactin.

Effect of opioids and opioid receptors antagonists on the anterior pituitary hormone secretions in man

The effects of morphine and nafloxone [Opioid receptor antagonist] on secretion of LH, FSH, GH and prolactin hormone were studied in twenty male subjects aged 35-45 years. The results revealed that morphine injection increased FSH, GH and prolactin hormone secretion without a significant effect on LH release, while naloxone injection increased LH and decreased prolactin hormone release without a significant change on FSH and GH release. These data show that both opioids and opioid receptors antagonists exert a rariety of effects on the anterior pituitary secretions in man and this is due to the multiplicity of opiate receptors in pituitary gland cells

Macroprolactinoma aos 6 anos de idade: dificuldades diagnósticas / Macroprolactinoma at 6 years of age: diagnostic difficulties

Prolactinoma é raro na infância e adolescência. O diagnóstico mais precoce conhecido na literatura ocorreu aos 8 anos de idade. Aqui é relatado o achado de tumor selar em menina com 6 anos, embora só fosse procurado auxílio médico tardiamente, quando a paciente já apresentava atrofia parcial de papila. Nessa ocasiäo, foi constatada reseva diminuída de hormônio do crescimento e de cortisol, bem como hiperprolactinemia. Devido à rápida deterioraçäo visual, a paciente foi submetida a craniotomia fronto-temporal com a hipótese diagnóstica de craniofaringioma. O exame imuno-histoquímico do tecido tumoral foi positivo para prolactina. Conclui-se que o prolactinoma deve ser considerado no diagnóstico diferencial de tumores selares na infância, por possibilitar terapêutica menos agressiva