Sheehan's Syndrome

Sheehan's syndrome occurs as a result of ischemic pituitary necrosis due to severe postpartum hemorrhage. It may be rarely seen without massive bleeding or after normal delivery. Improvement in obstetric care and availability of rapid blood transfusion coincided with a remarkable reduction in the frequency of Sheehan's syndrome particularly in western society. But it has recently been reported more often from well-developed countries. It is one of the most common causes of hypopituitarism in underdeveloped or developing countries. Enlargement of pituitary gland, small sella size, disseminated intravascular coagulation and autoimmunity have been suggested to play a role in the pathogenesis of Sheehan's syndrome in women who suffer from severe postpartum hemorrhage. The patients may seek medical advice because of various presentations ranging from non-specific symptoms to coma and the clinical manifestation may change from one patient to another. Failure of postpartum lactation and failure to resume menses after delivery are the most common presenting symptoms. Although a small percentage of patients with Sheehan's syndrome may cause abrupt onset severe hypopituitarism immediately after delivery, most patients have a mild disease and go undiagnosed and untreated for a long time. It may result in partial or panhypopituitarism and GH is one of the hormones lost earliest. The great majority of the patients has empty sella on CT or MRI. Lymphocytic hypophysitis should be kept in mind in differential diagnosis. In this review, the old and recent data regarding Sheehan's syndrome are presented.     

Sheehan's syndrome: clinical profile, pituitary hormone responses and computed sellar tomography

Twenty patients with the typical clinical presentation of Sheehan's syndrome were studied. All had a severe degree of hypopituitarism. The circulating mean basal levels of thyroid hormones, Cortisol and prolactin were significantly lower (p< 0.05 to <0.02) compared to those in 50 age matched controls. The provoked pituitary responses to combined pituitary stimulation in 13 patients were markedly lower (p <0.02 to <0.001) than those in ten age matched control subjects. Sellar computed tomography revealed an empty sella in all the patients; partial in five and complete in the remaining. A secondary empty sella is considered a characteristic finding in the classical form of Sheehan's syndrome. (Aust NZ J Med 1993; 23: 26–31.)     

54例希恩综合征患者临床特征分析及文献复习

回顾性分析本院内分泌科2008年8月至2011年8月期间收治的54例住院希恩综合征患者病史及临床资料.47例患者诊断为垂体危象,19例患者(35.19％)在本次住院前曾长期被误诊,从发生产科事件至确诊的平均年限为( 12.36±1.28)年.静滴氢化可的松抢救垂体危象患者时,入院后第一个24 h平均剂量为( 83.93±7.73)mg,第2个24 h平均剂量为(42.19±5.13)mg.12例患者常规剂量糖皮质激素 口服替代治疗后发生一过性精神障碍.     

Case Report: Interferon induced coma in Sheehan's syndrome

A 54-year-old woman who was being treated with 10 million units (mu) of natural interferon (IFN)-α per day for chronic active hepatitis C at a local clinic, developed coma on the fourth day of treatment. On admission to Yamagata University Hospital, she was still in a state of semicoma with severe hyponatraemia (122 mEq/L) and hypochloraemia (89 mEq/L). After the administration of electrolytes, her condition improved remarkably. Endocrinological loading tests showed a hypofunction of the anterior pituitary gland. In consideration of these results, and her past experiences of haemorrhage during childbirth and subsequent amenorrhoea, we diagnosed her illness as a coma as a result of Sheehan's syndrome which had become overt during IFN therapy. She recovered completely after treatment with hydrocortisone and 1-thyroxine.     

Hypertriglyceridemic acute pancreatitis in a patient with Sheehan''''s syndrome

BACKGROUND: There is no report on case of severe acute hyperlipidemic pancreatitis after treatment of Sheehan's syndrome. METHODS: A 32-year-old female patient was diagnosed as having acute hyperlipidemic pancreatitis after treatment of Sheehan's syndrome, and treated with diet and lipidlowering agents in early stage. RESULTS: Abdominal pain and fever of the patient resolved within a few days. She was subjected to diet and oral lipid-lowering therapy on the 4th day after admission. The disease did not recur during the follow-up for more than one year. CONCLUSIONS: Estrogen replacement therapy should be prescribed for Sheehan's syndrome. The serum level of triglyceride should be monitored and treatment should be given to prevent severe acute pancreatitis. Lipid-lowering therapy in early stage is the key step towards a complete recovery.     

Xanthomatous Pituitary Lesions: A Report of Two Cases and Review of the Literature

We describe two young men with cystic pituitary enlargement on magnetic resonance imaging (MRI) causing hypopituitarism. The first patient presented acutely unwell with headache and vomiting associated with anterior and posterior pituitary dysfunction. The second patient presented with hypopituitarism after a long history of hypogonadism. In both cases yellow/brown fluid was found at surgery and histological examination revealed inflammatory infiltrate with foamy histiocytes, lymphocytes and multinucleated giant cells containing cholesterol clefts. Full recovery of pituitary function occurred after surgery in the first but not the second patient. The first case is the first documented case of xanthomatous hypophysitis with recovery of pituitary function following surgery. The cases differed in duration of disease, as indicated by the long history of symptoms, the histological finding of marked fibrosis and the lack of recovery of pituitary function in the second. Xanthomatous pituitary lesions categorized in the literature as xanthomatous hypophysitis, xanthogranulomatous hypophysitis and xanthogranuloma of the sellar region have overlapping histological features. Our two cases revealed histological features that do not fit completely into any of the categories but share features of all three. These findings suggest that the various xanthomatous lesions of the sellar region may be a spectrum of a common inflammatory process rather than distinct pathological entities.     

Pituitary Apoplexy in a Patient with Acute Myeloid Leukemia and Thrombocytopenia

We describe a 72-year-old woman with a history of acute myeloid leukemia who developed pituitary apoplexy associated with thrombocytopenia secondary to chemotherapy. She presented with new onset severe headache, nausea, vomiting and blurred vision. Initial physical examination was unremarkable. CT scan of the head was initially negative. Upon admission for further work up, She developed a high-grade fever, hypotension and obtundation. Subsequent physical examination revealed bitemporal visual fields defects and decreased visual acuity. Repeat imaging of head revealed a hemorrhagic pituitary mass compressing the optic chiasm. Laboratory results were compatible with the diagnosis of pan-hypopituitary syndrome. She received high dose steroids and was transferred for transnasal sphenoidotomy decompression surgery. The visual defects improved postoperatively. A literature review of Pituitary apoplexy is presented. Pituitary apoplexy secondary to thrombocytopenia has never been reported.     

Lymphocytic hypophysitis: Disease spectrum and approach to diagnosis and therapy

Lymphocytic hypophysitis (LYH) is a neuroendocrine disorder characterized by autoimmune inflammation of the pituitary gland with various degrees of pituitary dysfunction. The histopathology consists of an initial monoclonal lymphocytic infiltrate, which can heal with minimal sequela or progress to fibrosis and result in permanent hypopituitarism. Coexistence of other autoimmune conditions is reported in 25–50% of cases and pituitary autoantibodies have been detected in up to 70% of biopsy-proven cases. The clinical presentation varies depending on the pituitary segment that is more severely affected. In lymphocytic adenohypophysitis (LAH) an early destruction of the ACTH-producing cells is characteristic. Other anterior pituitary hormones can also be affected but posterior pituitary involvement is absent or minimum. Lymphocytic Infundibuloneurohypophysitis (LINH) typically presents as acute onset diabetes insipidus (DI) with intracranial mass-effect symptoms. A combination of extensive anterior pituitary involvement and DI characterizes lymphocytic Infudibulopanhypophysitis (LIPH). The diagnosis can be challenging in many cases, because distinction from pituitary adenomas and other sellar masses is not obvious. Significant efforts have been made to identify specific serum markers, but it would seem unlikely that this approach will ever have the specificity to replace histopathological examination of a surgical specimen. Diagnostic criteria have been proposed to help in the decision-making process and to avoid, whenever possible, unnecessary invasive procedures. The therapeutic approach is controversial and, although transsphenoidal surgery is often performed, a conservative medical management is justified in many cases, given the self-limited nature of the inflammatory process. This paper reviews the etiology, epidemiology, clinical and radiological findings, diagnosis and management of LYH.     

Sheehan's syndrome: experience in Africa

Ischemic necrosis of the antehypophysis is still observed in developing countries. Its prevalence is unknown, but a significant number of the patients are young women. Traditions of home delivery and lack of obstetrical facilities are the main risk factors. The clinical manifestations are often quite typical but atypical signs including neuropsychiatric ore water metabolism disorders are observed. The delay to onset of overt disease can be very long. Ischemic necrosis of the antehypophysis should be entertained as a possible diagnois in women with hypopituitarism.     

Pituitary Apoplexy: A Review of Clinical Presentation, Management and Outcome in 45 Cases

Objective: To review clinical presentation, management and outcomes following different therapies in patients with pituitary apoplexy. Methods: Retrospective analysis of case-records of patients with classical pituitary apoplexy treated in our hospitals between 1983–2004. Results: Forty-five patients (28 men; mean age 49 years, range 16–72 years) were identified. Only 8 (18%) were known to have pituitary adenomas at presentation. Thirty-four (81%) patients had hypopituitarism at presentation. CT and MRI identified pituitary apoplexy in 28% and 91% cases, respectively. Twenty-seven (60%) patients underwent surgical decompression, whilst 18 (40%) were managed conservatively. Median time from presentation to surgery was 6 days (range 1–121 days). Patients with visual field defects were more likely than those without these signs to be managed surgically (p = 0.01). Complete or near-complete resolution occurred in 93% (13/14), 94% (15/16) and 93% (13/14) of the surgically treated patients with reduced visual acuity, visual field deficit and ocular palsy, respectively. All patients with reduced visual acuity (4/4), visual field deficit (4/4) and ocular palsy (8/8) in the conservative group had complete or near-complete recovery. Only 5 (19%) patients in the surgical group and 2 (11%) in the conservative group had normal pituitary function at follow up. One (4%) patient in the surgical group and 4 (22%) in the conservative group had a recurrence of pituitary adenoma. Conclusions: This large series suggests that the patients with classical pituitary apoplexy, who are without neuro-ophthalmic signs or exhibit mild and non-progressive signs, can be managed conservatively in the acute stage.     

Hypopituitarism as a late complication of hemorrhagic fever

We report three patients who developed hypopituitarism as a late complication of hemorrhagic fever with renal syndrome (HFRS). Their past history, physical examination, and endocrine investigation confirmed hypopituitarism. Magnetic resonance imaging of the pituitary revealed atrophic pituitary gland with an empty sella. Hemorrhagic fever is endemic in certain regions of the Balkans, and this preliminary report suggests the importance of investigating the endocrine status in every patient who survived HFRS.     

Serum thyrotropin concentrations in patients with Sheehan's syndrome: reevaluation of the usefulness in the diagnosis

Serum concentrations of thyroid-stimulating hormone (TSH) were determined, using a highly sensitive immunoradiometric assay (IRMA), in 10 patients with Sheehan's syndrome. Serum TSH levels in these patients were from 2.3 to 9.0 microU/ml, with the mean of 6.4 +/- 2.3(SD) microU/ml, and the data were similar to those measured by a conventional RIA method. The levels of serum TSH in these patients were normal or even higher than those of healthy women (1.8 +/- 1.3 microU/ml). After supplement therapy by cortisol, serum TSH levels decreased, but remained within the detectable range that was greater than 0.15 microU/ml. After supplement therapy by 1-thyroxine (T4), serum TSH levels moderately decreased in all patients, and excessive 1-T4 administration resulted in a fall of serum TSH levels to lower than the detectable limit. Thyroidal 123 I uptake was low in 3 out of 6 patients examined, which supports a recent hypothesis of reduced biological activity of the patient's TSH. While, the remaining 3 patients had normal thyroidal 123 I uptake, and administration of perchlorate had no effects on thyroidal radioactivity. Thus, it may be possible that in the former group of patients the TSH has a reduced biological activity, and in the latter group of patients, iodine trapping is intact but further synthesis and secretion of thyroid hormone from the gland are impaired. When the response of serum TSH to TRH was examined, the peak serum TSH levels increased in all patients. The peak TSH levels were 4.8 to 10.2 microU/ml with the mean of 7.5 microU/ml, but the data overlapped with those of normal subjects (peak level ranging 5.9 approximately 27.7 microU/ml).(ABSTRACT TRUNCATED AT 250 WORDS)     

Unusual manifestations of adrenal insufficiency: A case report of hypopituitarism and Well's syndrome after apoplexy of a silent pituitary gonadotropic adenoma

Rationale:Pituitary apoplexy occurs in about 8% of those with nonfunctioning pituitary adenoma. Subsequent hormone deficiency, especially corticotropic deficiency, is the most common finding. We describe the unusual manifestations of adrenal insufficiency that are usually overlooked in such cases, with the aim of raising awareness of this disease.Patient concerns:A 53-year-old male with a history of hyponatremia came to our hospital with intermittent fever and generalized pruritic skin rash. He also reported general weakness, abdominal pain, poor appetite, and severe retroorbital headache.Diagnoses:Laboratory data revealed hypereosinophilia, hypotonic hyponatremia, and hypopituitarism, including secondary adrenal insufficiency. Sellar magnetic resonance imaging revealed a pituitary macroadenoma, 2 cm in height, with mild displacement of the optic chiasm. Pathologic report and immunohistochemical stains of surgical specimen showed pituitary gonadotropic adenoma with apoplexy.Interventions:Transsphenoidal removal of the pituitary adenoma was performed. The patient received intravenous hydrocortisone then oral form cortisone acetate regularly.Outcomes:His symptoms and laboratory data recovered after the operation and medical treatment.Lessons:This case highlights that eosinophilia, pruritic skin rash and fever can be manifestations of adrenal insufficiency, and that they may initially be regarded as cellulitis.     

肌钙蛋白诊断老年急性冠脉综合征的意义

心肌肌钙蛋白Ｔ（cTnt）是调节心脏肌肉收缩的肌钙蛋白复合体组成之一,健康人血液中含量很低,在心肌损伤时释放入血,具有在血中出现时间早、灵敏度高、诊断窗口时间长等优点,被认为是心肌损伤的特异性标志物.     

Circulating autoantibodies as serological markers in the differential diagnosis of pulmonary renal syndrome

Abstract. Objectives . Pulmonary renal syndrome (lung haemorrhage and glomerulonephritis) is a fulminant condition that warrants a rapid diagnosis and treatment to prevent mortality and preserve renal functions. However, the patients frequently present with non-specific pulmonary symptoms in the early phase of the syndrome and the diagnosis is often missed. Recently, several autoantibodies have been described in association with various forms of glomerulonephritis. We evaluated the association as well as the diagnostic and the prognostic significance of these antibodies in pulmonary renal syndrome. Design . Retrospective clinical study. Setting . University Hospital. Subjects . Forty consecutive patients with biopsy verified glomerulonephritis and overt haemoptysis or pulmonary infiltrates compatible with lung haemorrhage. Interventions . Analysis of proteinase 3 antineutrophil cytoplasm antibodies (PR3-ANCA), myeloperoxidase (MPO)-ANCA, antiglomerular basement membrane (GBM) and anti-entactin antibodies. Results . Thirty-six (90%) patients possessed one or more autoantibodies. Twenty-seven (70%) patients had ANCA (PR3-ANCA, MPO-ANCA or both). The remaining positive patients (n = 9) had anti-GBM antibodies. Only two patients had anti-entactin antibodies, suggesting a poor association of these antibodies with PRS. The majority of patients with anti-GBM antibodies had a very poor clinical outcome (five irreversible renal failure; three deaths). On the other hand, despite no significant difference in clinical features or renal morphology from patients with anti-GBM antibodies, 19 patients (70%) with ANCA recovered completely following treatment. Conclusions . Our study demonstrated that the presence of autoantibodies is a predominant feature of PRS and that the type of immunologic injury is of paramount importance in determining the course of illness in this syndrome. Analysis of the aforementioned antibodies can help in an early differential diagnosis and thus, in better management of PRS.     

Cost-effectiveness and accuracy of the tests used in the differential diagnosis of Cushing's syndrome.

Aim: Establish the minimal biochemical and radiological examinations necessary and their cost-effectiveness to accurately diagnose the etiology of Cushing's syndrome (CS).Material and Methods: In 71 patients with CS followed between 1982 and 1997 biochemical studies (basal ACTH, 8mg dexamethasone suppression test -HDST-, metyrapone stimulation test -MST-, or inferior petrosal sinus catheterization sinus catheterization -IPSC-) and radiological investigations (abdominal CT scan, pituitary CT scan or MRI) were performed. Once pathology confirmed the diagnosis (48 pituitary Cushing's disease-CD, 17 adrenal neoplasms, 2 bilateral macronodular hyperplasia -BMH-, and 4 ectopic ACTH syndrome -ES-), the sensitivity, specificity, positive and negative predictive value of the different studies was calculated to establish the most accurate and cost-effective diagnostic protocol.Results: In ACTH-independent CS (ACTH 9 pg/ml; normal 9 to 54) a unilateral tumor was identified on abdominal CT scanning in 17, and BMH in 1; the other BMH had detectable ACTH (43.2 pg/ml). In ACTH-dependent CS, ACTH was > 9 pg/ml and IPSC (performed in 22) correctly identified 20 patients with CD and differentiated them from 2 with an ES (100 % specificity and sensitivity). Pituitary MRI or CT did not disclose an adenoma in 41.7% of patients with CD, and was reported to exhibit a microadenoma in 2 of the 4 patients with ES. HDST and MST were of no additional use in the differentiation between CD and ES.Conclusions: Once CS is diagnosed low ACTH and an abdominal CT scan correctly identified all patients of adrenal origin. In ACTH-dependent CS IPSC was the best predictive test to differentiate CD from ES. BMH may behave as ACTH-dependent or independent. The other biochemical and radiological studies performed are not cost-effective and may even be misleading, and should not be routinely performed.     

Clinical evaluations of pituitary apoplexy in incidental nonfunctional pituitary adenomas

Pituitary apoplexy is an uncommon syndrome that often results in spontaneous hemorrhage or infarction of pituitary tumors or glands. We previously reported pituitary apoplexy occurred most frequently in nonfunctional pituitary adenomas among all types of pituitary incidentalomas. In the present study, we aimed to investigate the characteristics of pituitary apoplexy in patients with incidental nonfunctional pituitary adenomas. 65 patients with pituitary incidentaloma were enrolled. All patients underwent clinical/endocrinological/pathological investigations. As a result, 33 patients were diagnosed with nonfunctional pituitary adenomas. Of these, 12.1% of patients had pituitary apoplexy. There was no difference in tumor diameter, age, or sex between the apoplexy and the non-apoplexy groups. However, the liver enzymes aspartate transaminase and alanine aminotransferase were significantly higher, and plasma sodium and chloride levels were significantly lower in the apoplexy group than in the non-apoplexy group (each P < .05). In addition, low-density lipoprotein-cholesterol was significantly higher in the apoplexy group than in the non-apoplexy group (P < .05). Besides, thyroid-stimulating hormone, luteinizing hormone, follicle-stimulating hormone, and prolactin deficiencies were significantly more frequent in the apoplexy group than in the non-apoplexy group (each P < .05), and growth hormone and adrenocorticotropic hormone deficiencies were more frequent in the apoplexy group than in the non-apoplexy group (P = .09 and.08, respectively). Furthermore, tumor diameter was not associated with pituitary apoplexy, whereas thyroid-stimulating hormone, luteinizing hormone, and follicle-stimulating hormone deficiencies were significantly associated with the apoplexy group (each P < .05). Hence, the present study indicated that pituitary apoplexy could not be related to tumor diameter. Moreover, hormonal deficiencies, hepatic dysfunction, hyponatremia or hypochloremia, and dyslipidemia might be indicators of pituitary apoplexy. There could be the possibility the treatment for dyslipidemia prevents pituitary apoplexy.     

缺血修饰白蛋白在急性冠状动脉综合征诊断中的意义

<正>急性冠状动脉综合征(acute coronary syndromes,ACS)在临床上十分常见,是心源性猝死的重要原因之一,死亡风险在10%左右。急性心肌梗死(acute myocardial infarction,AMI)是缺血时间过长引起的心肌细胞死亡。目前,生化标志物检测组合仍是ACS风险预测的首选[1]。通常选用肌红蛋白、肌钙蛋白I(cTnI)以及肌酸激酶同工酶(CK-MB)作为诊断ACS的标志物[2],然而,这些标志物仅仅在心肌细胞坏死之后释放入血液中才能被检测为阳性,不可作为判断心肌