Addison’S Disease in Evolution: An Illustrative Case and Literature Review

ObjectiveTo present a case of symptomatic autoimmune adrenal insufficiency with initially normal serum cortisol and to caution about limitations of the current diagnostic algorithm for adrenal insufficiency, which does not reflect the pathophysiology of early disease.MethodsWe describe the clinical presentation and relevant investigations of a patient ultimately found to have Addison’s disease, which is followed by a focused review of the literature.ResultsA 41-year-old Caucasian woman with autoimmune hypothyroidism, premature ovarian failure, and microscopic colitis presented with nausea, salt craving, increased skin pigmentation, and postural hypotension. Initial bloodwork revealed a normal morning cortisol of level of 19.2 μg/dL (normal, 7.2 to 25 μg/dL) but an adrenocorticotropic hormone (ACTH) level 10 times normal, at 513.6 pg/mL (normal, < 52.5 pg/mL). Her potassium was normal, but her aldosterone level was 4.12 ng/dL (normal, 12.3 to 62.5 ng/dL) and her renin activity was increased (23.0 mg/dL/hour; normal, < 6.0 mg/dL/hour). Six weeks after initial presentation, she was found to have anti-adrenal antibodies. It was not until 10 weeks after her initial symptomatic presentation that her morning cortisol level was found to be subnormal and a formal diagnosis of adrenal insufficiency was made.ConclusionThe present case and literature review reveal that common diagnostic approaches will miss patients with (possibly symptomatic) early adrenal insufficiency. We suggest that serum ACTH level testing or tests of mineralocorticoid function be included in the initial step of investigation for suspected primary adrenal insufficiency. (Endocr Pract. 2014;20:e176-e179)     

Sellar Masses That Present With Severe Hyponatremia

ObjectiveHyponatremia is a known but underrecognized presentation of sellar lesions. Herein, we present a series of patients who presented with single or multiple episodes of hyponatremia.MethodsOver 5 years, patients undergoing endonasal surgery for a de novo sellar mass with hyponatremia as an initial presentation were included. Pathology, sodium levels, pituitary hormonal status, and treatment course were documented.ResultsOf 282 patients, 16 (5.7%) (9 males, 7 females, age 32 to 84 years) presented with severe hyponatremia, with a mean serum sodium level of 115 ± 6 mmol/L (range, 101 to 125 mmol/L), and 3 patients had 2 or more episodes. Severe hyponatremia was a presenting sign in 0, 4.1, 14.3, and 37.5% of patients with craniopharyngiomas (n = 10), pituitary adenomas (n = 243), Rathke’s cleft cysts (RCCs) (n = 21), and sellar arachnoid cysts (n = 8), respectively (P < .01). Half of the patients presenting with hyponatremia, including 6 of 10 patients with adenomas and 2 of 3 patients with RCCs, had pituitary apoplexy or cyst rupture. All patients had anterior pituitary gland dysfunction, including 81% with hypoadrenalism and 69% with hypothyroidism. Following surgery, hormonal status was unchanged or improved in 15 patients (median follow-up, 14 months). No patient had tumor/cyst recurrence or recurrent hyponatremia.ConclusionSevere hyponatremia was a presenting sign in 5.7% of patients with sellar pathology, most frequently in patients with arachnoid cysts, RCCs, and pituitary apoplexy. Patients with new-onset severe hyponatremia and no obvious pharmacologic or systemic cause should undergo pituitary hormonal evaluation and brain imaging. Surgical resection and correction of hormonal deficiencies are associated with resolution of recurrent hyponatremic episodes. (Endocr Pract. 2014;20:1178-1186)     

Pituitary Dysfunction in Patients with Intracranial Germ Cell Tumors Treated with Radiotherapy

Objective:To evaluate the endocrine abnormalities in intracranial germ cell tumors (iGCTs) treated with radio-therapy (RT), and to discuss the effects of RT on pituitary functions.Methods: Seventy-seven patients diagnosed with iGCTs who had received RT and endocrine follow-up in Huashan Hospital between January 2010 and July 2017 were retrospectively analyzed, consisting of 49 germinomas and 28 NGGCTs. The median follow-up period was 50.0 months. Fifty-one patients had radiologically proved suprasellar/sellar lesions.Results: The male to female ratio was 62/15. The median endocrine follow-up period was 19 (4, 42) months. The median age at the last endocrine visit was 18 (16, 20) years old. The 5-year overall and recurrence-free survival were both 98.7%. The overall prevalence of central adrenal insufficiency (CAI), central hypothyroidism (CHT), central hypogonadism (CHG), hyperprolactinemia, and central diabetes insipidus (CDI) was 57.3%, 56%, 56.6%, 35.3%, and 52.1%, respectively, after RT. Patients having suprasellar/sellar lesions showed significantly higher post-therapeutic prevalence of hypopituitarism than those who didn’t. Compared to that before RT, CAI, CHT, and CHG weren’t significantly improved while the levels of prolactin and the prevalence of CDI declined significantly (P =.03 and.001). The radiation doses to pituitary and hypothalamus between those with and without CAI, CHT, and CHG weren’t significantly different.Conclusion: The prevalence of hypopituitarism was high in iGCTs, especially in those with suprasellar/sellar involvement. The levels of prolactin and the prevalence of CDI declined significantly after RT. The hypopituitarism in iGCTs was mainly induced by tumor effects, and RT showed no additional damage to pituitary functions in our study.Abbreviations: AFP = alpha-fetoprotein; CAI = central adrenal insufficiency; CDI = central diabetes insipidus; CHG = central hypogonadism; CHT = central hypothyroidism; CT = computed tomography; DA = dopamine; GH = growth hormone; βHCG = beta-human chorionic gonadotropin; HPA = hypothalamus-pituitary-adrenal; HPG = hypothalamus-pituitary-gonadal; HPL = hyperprolactinemia; HPT = hypothalamus-pituitary-thyroid; iGCT = intracranial germ cell tumor; IGF-1 = insulin-like growth factor 1; NGGCT = nongerminomatous germ cell tumors; OS = overall survival; PFS = progression-free survival; PRL = hypothalamus-pituitary-prolactin; RT = radiotherapy     

Overt and Subclinical Adrenal Insufficiency in Pulmonary Tuberculosis

BackgroundTuberculosis of the adrenal glands may cause overt or subclinical adrenal insufficiency. An algorithm-based approach including assessment of paired basal cortisol and plasma adrenocorticotropic hormone (ACTH), short Synacthen, and plasma renin activity assays could be useful to diagnose all forms of adrenal insufficiency.MethodsThis cross-sectional study included consecutive, treatment-naive subjects diagnosed with pulmonary tuberculosis. Tuberculosis severity was classified by radiological criteria. Baseline parameters plus morning (8 am) serum cortisol and paired plasma ACTH were measured in all patients. Synacthen stimulation tests and plasma renin activity assays were performed as required.ResultsEighty-four treatment-naive consecutive cases of pulmonary tuberculosis were evaluated for adrenal insufficiency. Twenty-seven (32.14%) subjects had normal adrenocortical function and 8 (9.5%), 7 (8.3%), 40 (47.6%), and 2 (2.4%) subjects had stage 1, stage 2, stage 3, and stage 4 adrenal insufficiency, respectively. Serum cortisol was negatively correlated with radiological severity (P = .01) and duration of illness (P = .001). Adrenal dysfunction was present in 27.3%, 82.5%, and 80% of those with radiologically minimal, moderately advanced, and far-advanced disease, respectively. Mean cortisol was 19.74 ± 5.52, 17.42 ± 8.53, and 15.71 ± 7.14 (μg/dL) in the 3 groups, respectively (P = .042). Hyponatremia was present in 83.3% of the patients. Serum sodium was negatively correlated with severity but not with the duration of disease.ConclusionThe prevalence of overt and subclinical adrenal dysfunction in pulmonary tuberculosis was high and was correlated with disease severity and duration. An algorithmic approach may be useful to detect the same and may have important clinical implications.     

Sheehan syndrome: clinical and laboratory evaluation of 20 cases

Sheehan syndrome (SS) or post-partum pituitary necrosis is a pituitary insufficiency secondary to excessive post-partum blood losses. SS is a very significant cause of maternal morbidity and mortality in developing countries although it is a rarity in developed countries in which obstetrical care has been improved. In this study, we reviewed 20 cases retrospectively who were diagnosed as SS in our clinic. The patients aged 40 to 65 years with a mean age of 51.12 +/- 9.44 years (mean +/- SD). Time to make a definitive diagnosis of the disease ranged between 5 and 25 years with a mean of 16.35 +/- 4.74 years. Three of our patient (15%) had a previous diagnosis of SS. Three patients (15%) were referred to emergency service for hypoglycemia, three patients (15%) for hypothyroidism and one patient (5%) for hyponatremia. Dynamic examination of the pituitary revealed GH, Prolactin, FSH, TSH and ACTH insufficiency in all of the patients. One of our patients had a sufficient LH response to LHRH challenge. All of the patients were imaged with pituitary MRI. Eleven patients had empty sella and 9 patients had partial empty sella. SS is still a common problem in our country, especially in rural areas. Considering the duration of disease, important delays occur in diagnosis and treatment of the disease.     

Sheehan Syndrome in Costa Rica: Clinical Experience With 60 Cases

ObjectiveTo describe the clinical and hormonal characteristics of patients with Sheehan syndrome.MethodsWe performed a 20-year cohort study (1969 to 1989) of patients with Sheehan syndrome who were cared for in the endocrinology unit of the México Hospital in San José, Costa Rica. Medical history, physical examination findings, and laboratory test results were documented and reviewed.ResultsSixty patients with a diagnosis of Sheehan syndrome were identified. Mean age at diagnosis was 45.8 ± 10.6 years. The average time between the previous obstetric event and Sheehan syndrome diagnosis was 13 years. Thirteen patients (22%) had a home birth. The median parity was 7. The most frequent obstetric antecedents were history of obstetric hemorrhage in 49 (82%), shock in 28 (47%), blood transfusion in 26 (43%), and peripartum hysterectomy in 4 (7%). The most common symptoms included asthenia and adynamia in 51 (85%), amenorrhea in 44 (73%), loss of axillary or pubic hair in 40 (67%), and agalactia in 40 (67%). The most common findings on physical examination were absence of axillary hair in 56 (93%) and pubic hair in 56 (93%), dry skin in 49 (82%), and pallor in 42 (70%). When a combined pituitary test was performed, 38 of 38 patients (100%) had human growth hormone deficiency, 29 of 30 patients (97%) had adrenal insufficiency, 8 of 10 patients (80%) had hypothyroidism, 8 of 12 patients (67%) had hypogonadism, and 9 of 13 patients (69%) had prolactin deficiency. Despite early onset of symptoms, Sheehan syndrome was diagnosed relatively late in these 60 patients. Hemorrhage during the last delivery, especially if severe and/or associated with shock or transfusion, was the most important obstetric antecedent event. Absence of amenorrhea or presence of postpartum lactation did not exclude the diagnosis. Abnormal responses of human growth hormone to insulin tolerance testing and of prolactin to thyrotropin-releasing hormone confirm the diagnosis.ConclusionsIdentification of patients affected with Sheehan syndrome is critical to avoid delays in diagnosis and treatment. Appropriate replacement therapy is capable of yielding complete remission of symptoms. (Endocr Pract. 2011;17:337-344)     

Association of ipilimumab therapy for advanced melanoma with secondary adrenal insufficiency: a case series

ObjectiveTo present a case series of ipilimumabrelated secondary adrenal insufficiency.MethodsIn this cases series, we review the presentation, evaluation, diagnosis, and management of patients with advanced melanoma who received ipilimumab and were referred to our endocrinology clinic for evaluation of hormonal abnormalities.ResultsSeven patients presented with symptoms, signs, or biochemical evidence of adrenal insufficiency 6 to 12 weeks after starting ipilimumab therapy. Ipilimumab is a cytotoxic T-lymphocyte antigen 4 (CTLA-4) monoclonal antibody that is approved for the treatment of metastatic melanoma and has widespread use for this disease. All 7 patients had biochemical evidence of profound secondary adrenal insufficiency. Thyroid function abnormalities, central hypogonadism, and low insulinlike growth factor 1 levels were seen in a subset of patients. Only 2 patients had abnormal findings on pituitary magnetic resonance imaging. Posterior pituitary function remained normal.ConclusionsOur findings suggest that the enhanced immune response associated with ipilimumab therapy may have a predilection for corticotroph and possibly thyrotroph cells. We recommend periodic hypothalamic-pituitary-adrenal axis monitoring for patients on this therapy.(Endocr Pract. 2012;18:351-355)     

Autoimmune Hyperthyroidism due to Secondary Adrenal Insufficiency: Resolution with Glucocorticoids

ObjectiveTo describe the course of autoimmune hyperthyroid disease in a patient with corticotropin (ACTH) deficiency treated with glucocorticoids.MethodsWe report the clinical presentation, laboratory data, imaging studies, and management of a patient with weight loss, fatigue, apathy, hallucinations, and arthritis.ResultsAutoimmune hyperthyroidism (positive thyroperoxidase and thyroglobulin antibodies and borderline positive thyrotropin receptor antibody) was diagnosed in a 71-year-old woman. New psychotic symptoms prompted brain magnetic resonance imaging, which revealed a partially empty sella. Undetectable morning cortisol, undetectable ACTH, and failure to stimulate cortisol with synthetic ACTH (cosyntropin 250 mcg) secured the diagnosis of long-standing secondary adrenal insufficiency. Hydrocortisone replacement improved the patient’s symptoms, resolved the thyroid disease, and decreased thyroid antibody titers. In retrospect, the patient recalled severe postpartum hemorrhage requiring blood transfusion at age 38 years. A Sheehan event probably occurred 33 years before the patient presented with corticotropin deficiency. Hyperthyroidism accelerated cortisol metabolism and provoked symptoms of adrenal insufficiency.ConclusionsThe hypocortisolemic state may precipitate hyperimmunity and autoimmune thyroid disease. Rapid resolution of hyperthyroidism and decreased thyroid antibody titers with glucocorticoid treatment support this hypothesis. (Endocr Pract. 2011;17:85-90)     

Adrenocorticotropic Hormone Stimulation Test During High-Dose Glucocorticoid Therapy

ObjectiveTo define the effective time frame of adequate serum cortisol response to the short standard 250-μg adrenocorticotropic hormone stimulation test (ACTH-ST) after initiation of high-dose glucocorticoid therapy in order to assist in the diagnosis of adrenal insufficiency.MethodsWe performed an ACTH-ST at 4 different times in hospitalized patients, who had a documented intact hypothalamic-pituitary-adrenal axis and were receiving high-dose dexamethasone therapy for nonendocrine diseases, to determine the time until the serum cortisol response is compromised. The ACTH-ST was performed at the following time intervals after initiation of dexamethasone therapy—24, 48, 72, and 96 hours. The outcome measures were cortisol levels measured at 0, 30, and 60 minutes after administration of 250 μg of cosyntropin.ResultsOf the overall group of 11 study patients, all those (n = 8) tested within the first 72 hours after initiation of dexamethasone therapy had an adequate response (serum cortisol level at 60 minutes, ≥ 18 μg/dL). Two of the 3 patients tested at 96 hours after initiation of dexamethasone therapy had a suppressed cortisol response at 60 minutes. At 30 minutes, all cortisol levels except 1 were inadequate (< 18 μg/dL).ConclusionThis study suggests indirectly that the ACTH-ST may be used for the diagnosis of suspected adrenal insufficiency within the first 3 days after initiation of empiric glucocorticoid therapy. An inadequate response of serum cortisol at 60 minutes to the ACTH-ST may be indicative of adrenal insufficiency in this setting. Additional studies with more patients in each therapy time point are needed to confirm these initial results. (Endocr Pract. 2009;15:122-127)     

Biochemical Diagnosis of Adrenal Insufficiency: the Added Value of Dehydroepiandrosterone Sulfate Measurements

ObjectiveTo review biochemical tests used in establishing the challenging diagnosis of adrenal insufficiency.MethodsWe reviewed the relevant literature, including our own data, on various biochemical tests used to determine adrenal function. The advantages and limitations of each approach are discussed.ResultsBaseline measurements of serum cortisol are helpful only when they are very low (≤ 5 μg/dL) or clearly elevated, whereas baseline plasma adrenocorticotropic hormone levels are helpful only when primary adrenal insufficiency is suspected. Measurements of baseline serum dehydroepiandrosterone sulfate (DHEA-S) levels are valuable in patients suspected of having adrenal insufficiency. Although serum DHEA-S levels are low in patients with primary or central adrenal insufficiency, a low level of this steroid is not sufficient by itself for establishing the diagnosis. A normal age- and sex-adjusted serum DHEA-S level, however, practically rules out the diagnosis of adrenal insufficiency. Many patients require dynamic biochemical studies, such as the 1-μg cosyntropin test, to assess adrenal function.ConclusionIn establishing the diagnosis of central adrenal insufficiency, we recommend measurements of baseline serum cortisol and DHEA-S levels. In addition to these, determination of plasma levels of aldosterone, adrenocorticotropic hormone, and renin activity is necessary when primary adrenal insufficiency is suspected. With a random serum cortisol level of ≥ 12 μg/dL in the ambulatory setting or a normal age- and sex-adjusted DHEA-S level (or both), the diagnosis of adrenal insufficiency is extremely unlikely. When serum DHEA-S levels are low or equivocal, however, dynamic testing will be necessary to determine hypothalamic-pituitary-adrenal axis function. (Endocr Pract. 2011;17:261-270)     

Diagnosing Adrenal Insufficiency: Which Test is Best—The 1-μG or the 250-μG Cosyntropin Stimulation Test?

ObjectiveTo review the available literature on the low-dose cosyntropin stimulation test (CST) for the diagnosis of primary and secondary adrenal insufficiency in both nonstressed and acutely ill patients.MethodsWe performed a MEDLINE search of all English-language literature, published between 1965 and 2007, in which the 1-μg and the 250-μg CSTs were compared in patients with primary and secondary adrenal insufficiency.ResultsThe majority of published evidence suggests that the 1-μg CST is more sensitive than the 250-μg CST for the diagnosis of secondary adrenal insufficiency in nonstressed patients. In patients with primary adrenal insufficiency, the low-dose CST is unlikely to add any diagnostic sensitivity to the high-dose CST. In critically ill patients, the 1-μg test is also likely to be more sensitive than the 250-μg test when an appropriate cutoff value is used (25 μg/dL).ConclusionThe 1-μg CST with a cortisol level determined at 30 minutes after stimulation, with use of a cutoff level of 18 to 20 μg/dL in nonstressed patients and less than 25 μg/dL or an increment of less than 9 μg/dL from baseline in critically ill patients, is the best test that is currently available for establishing the diagnosis of secondary adrenal insufficiency. (Endocr Pract. 2008;14: 233-238)     

Repairing a "broken heart" with hormone replacement therapy: case report of cardiogenic shock due to undiagnosed pituitary insufficiency

ObjectivesTo indicate cardiogenic shock as a very rare but serious clinical consequence of untreated panhypopituitarism attributable to Sheehan syndrome; to emphasize the importance of eliciting a detailed endocrine and obstetric history in women presenting with idiopathic heart failure; to highlight the diagnostic shortcomings of screening for thyroid dysfunction solely with thyroidstimulating hormone determinations; and to report the reversibility of severe heart failure induced by longterm pituitary insufficiency.MethodsDescribed is a case report of a 35yearold woman who presented with severe congestive heart failure, hypotension, and confusion. Her 2dimensional echocardiogram revealed appreciable systolic and diastolic dysfunction. In screening for possible endocrine causes of heart failure, a normal thyroidstimulating hormone level of 0.72 mIU/L (reference range, 0.35 to 5.5) was unremarkable; however, a profoundly low free thyroxine level of 0.12 ng/dL (reference range, 0.9 to 1.8) led clinicians to pursue a workup of central hypothyroidism.ResultsEndocrine testing confirmed the presence of panhypopituitarism and adrenal insufficiency. Magnetic resonance imaging of the brain revealed empty sella syndrome. Further questioning of the patient revealed a history of extensive postpartum bleeding 15 years earlier, failure to lactate, and secondary amenorrhea—all consistent with undiagnosed Sheehan syndrome. In the hospital, the patient was treated with intravenously administered corticosteroids and levothyroxine. Her mental status and symptomatic heart failure improved dramatically. After 9 months of oral levothyroxine and glucocorticoid therapy, the patient remained asymptomatic, and repeated echocardiography indicated completely normalized cardiac function.ConclusionSevere heart failure and cardiogenic shock can be a very rare (but fortunately reversible) complication of longstanding panhypopituitarism resulting from undiagnosed Sheehan syndrome. (Endocr Pract. 2012;18:e26e31)     

Autoimmune thyroid disease in patients with vitiligo: prevalence study in India

ObjectiveTo identify the prevalence of autoimmune thyroid disease (AITD) in Asian Indian patients with vitiligo and to compare the clinical profile between thyroid peroxidase (TPO) antibody-positive and TPO antibodynegative groups.MethodsIn this cross-sectional, case-controlled study, 50 patients with vitiligo (29 women and 21 men) were included. Patients with previous disorders, irradiation, or surgical procedures involving the thyroid were excluded from the study. All participants underwent a complete physical examination, and a single fasting blood sample was analyzed for thyroid function (triiodothyronine, thyroxine, thyroid-stimulating hormone, and TPO and thyroglobulin antibodies), inflammatory and immunologic markers (erythrocyte sedimentation rate, C-reactive protein, and rheumatoid factor), and serum calcium, phosphorus, and alkaline phosphatase concentrations. All patients underwent thyroid ultrasonography, and the data were analyzed by appropriate statistical methods.ResultsThe mean age of the study participants was 42.7 ± 17 years, and 14 of 50 patients (28%) had TPO antibody positivity. A goiter was present in 11 of 50 patients, and the thyroid volume by ultrasonography was similar between the 2 groups. Subclinical hypothyroidism was found in 14 of 50 patients (28%) but more frequently in the TPO antibody-positive group (8 of 14 or 57%) than in the TPO antibody-negative group (6 of 36 or 17%). The prevalence of AITD was 20 of 50 patients (40%) when the TPO antibody-positive group and those with subclinical hypothyroidism were considered collectively. None of the patients had overt hypothyroidism or hyperthyroidism. All other clinical, biochemical, and inflammatory variables did not differ significantly between the TPO antibody-positive and antibody-negative groups.ConclusionOur data showed a 40% prevalence of thyroid disease in patients with vitiligo in India. The risk is exacerbated in patients with thyroid autoimmunity; thus, regular screening of patients with vitiligo for AITD is needed. (Endocr Pract. 2012;18:194-199)     

A 30-Year,Single-Center Experience of Unilateral Adrenalectomy for Primary Bilateral Macronodular Adrenal Hyperplasia

ObjectiveThe aim was to assess the short- and long-term outcomes of unilateral adrenalectomy (UA) in patients with primary bilateral macronodular adrenal hyperplasia (PBMAH).MethodsWe conducted a retrospective study of 124 patients with PBMAH who underwent UA.ResultsOne hundred sixteen patients were available for follow-up (median, 28.5 months). Cushingoid features remitted in 43 of 65 patients (70.8%) with overt Cushing syndrome (CS). Hypertension and diabetes mellitus improved in 79 of 96 (82.3%) and 29 of 42 patients (69.0%), respectively. Glucocorticoid insufficiency developed in 7 of 116 patients (6.0%) after the surgery, and it resolved in all the patients during follow-up. The mean 24-hour urinary free cortisol level decreased gradually from 456.02 ± 422.33 mg/24 h at baseline to 84.47 ± 70.06 mg/24 h within 3 months and then increased progressively in some patients. Sixty-four of the 116 patients (55.2%) had biochemical recurrence and 43 patients (67.2%) underwent contralateral adrenalectomy. The median time interval between the second operation and the first UA was 24 months. Patients with overt CS had a larger surgical-side or contralateral adrenal volume than patients without overt CS. Patients with a contralateral adrenal volume of >33.54 mL or with a preoperative urinary free cortisol level of >216.08 mg/24 h were more likely to have recurrence.ConclusionThe efficiency of UA is transient for the majority of patients, and the indications should be strictly limited to those with subclinical or milder CS. Patients who undergo successful UA still require close life-time follow-up for the recurrence of hypercortisolism.     

Residual dysphasia after severe hypoglycemia in a patient with immune-mediated primary Adrenal insufficiency and type 1 diabetes mellitus: Case report and systematic review of the literature

ObjectiveTo describe a 24-year-old patient with immune-mediated primary adrenal insufficiency and type 1 diabetes mellitus (T1DM) receiving intensive diabetes management who was comatose at presentation attributable to severe hypoglycemia and had residual dysphasia after recovery and to summarize the related literature.MethodsWe present a case report and the findings on systematic review of the pertinent literature to identify the cumulative incidence of severe hypoglycemia with use of intensive insulin therapy in patients with primary adrenal insufficiency and T1DM and to determine the incidence of dysphasia after severe hypoglycemia.ResultsAfter 5 days of mechanical ventilation, our patient was revived. He had severe dysphasia after recovery of consciousness. Magnetic resonance imaging of the brain revealed encephalomalacia in the left temporal, frontal, and parietal lobes. After 6 years of follow-up, he continues to have residual deficits of expressive dysphasia and difficult-to-control seizures but no other neurologic disorders. Systematic review of the literature revealed that studies from the 1950s reported mortality due to hypoglycemia in such a cohort, but no recent studies have described the cumulative incidence of severe hypoglycemia in a cohort of patients with primary adrenal insufficiency and T1DM. To the best of our knowledge, we report the first findings on magnetic resonance imaging of the head in such a patient.ConclusionFortunately, residual dysphasia is an infrequent outcome after severe hypoglycemia. (Endocr Pract. 2007;13:384-388)     

Diagnosis and Management of Pheochromocytoma in an Academic Hospital 3 Years After Formation of A Pheochromocytoma Interest Group

ObjectiveTo examine whether establishment of a pheochromocytoma interest group improves diagnosis and management of pheochromocytoma in an academic hospital.MethodsThe medical records of patients who had preoperative or pathologic diagnosis of pheochromocytoma at a large academic hospital from July 2007 to July 2010 were retrieved and pertinent information was gathered. Quality measures for diagnosis and management of pheochromocytoma before and after establishment of a pheochromocytoma interest group, and by group and nongroup physicians, were compared.ResultsBetween 2007 and 2010, 16 patients were confirmed to harbor pheochromocytoma. The rates of overdiagnosis and underdiagnosis were similar before and after establishment of the pheochromocytoma interest group (23%-25% vs 17%, respectively); however, after interest group formation, pheochromocytoma was excluded in 9 patients for whom other physicians recommended adrenalectomy. Compared with nonmembers, members of the pheochromocytoma interest group more frequently performed pheochromocytoma testing before adrenal biopsy or adrenalectomy in patients with adrenal masses (71% vs 13%), including those with suspected malignancy (50% vs 7%). After interest group formation, many more patients were optimally prepared preoperatively and advised on follow-up plan and genetic testing.ConclusionsFormation of a pheochromocytoma interest group significantly enhances the quality of diagnosis and management of pheochromocytoma. The key to the group’s success is its incorporation of members’ formal or informal opinions into the care of patients with suspected pheochromocytoma. This model may be applied to other rare endocrine diseases. (Endocr Pract. 2011;17:356-362)     

Adrenal Insufficiency Attributable to Adrenal Hemorrhage: Long-Term Follow-Up with Reference to Glucocorticoid and Mineralocorticoid Function and Replacement

ObjectiveTo describe the long-term follow-up of acute adrenal insufficiency attributable to bilateral adrenal hemorrhage.MethodsWe performed a retrospective review of medical records of four patients who underwent follow-up for 6½ to 19 years.ResultsDespite published reports of more than 500 patients with bilateral massive adrenal hemorrhage through 2001, no long-term data assessing the continuing requirements for glucocorticoid and mineralocorticoid replacement are available. After follow-up of four patients with acute bilateral adrenal hemorrhage and glucocorticoid insufficiency for 6½ to 19 years, we document the absence of need for long-term mineralocorticoid replacement on the basis of no occurrence of postural hypotension, normal electrolytes, normal baseline or cosyntropin-stimulated serum aldosterone levels, and generally, though not invariably, normal plasma renin activity levels. We further document the improvement in either or both baseline and cosyntropin-stimulated serum cortisol levels in three of the four patients and the ability of one patient to function normally without cortisol replacement for 4 years. Adrenal histologic findings in this last-mentioned patient revealed previously undescribed changes consistent with regeneration and myelolipoma. Finally, we confirm bilateral atrophic adrenal glands by computed tomography 5½ to 11½ years after bilateral adrenal hemorrhage in three of the four patients.ConclusionLong-term follow-up of patients with acute adrenal insufficiency attributable to adrenal hemorrhage demonstrates, for the first time, absence of need for prolonged mineralocorticoid replacement and some improvement in endogenous glucocorticoid function in at least some of these patients. (Endocr Pract. 2004;10: 55-61)     

Anticuerpos antihipófisis en pacientes con sospecha de hipofisitis autoimmune

IntroductionDefinitive diagnosis of autoimmune hypophysitis (AH) is histological. However, a presumptive diagnosis can be made through clinical, biochemical and imaging data.ObjectiveThe objective of this study was to review the presence of antipituitary antibodies (APA) and antithyroid antibodies (ATA) in patients with suspected AH in order to determinate the utility of APA in the diagnosis of AH.Material and methodsWe studied 36 patients divided into seven groups according to the data suggesting AH (isolated corticotropin deficiency, other idiopathic pituitary deficiencies, idiopathic hyperprolactinemia, empty sella, sellar mass with thickened stalk, proven histological hypophysitis) or because of previous autoimmune endocrine diseases. Twenty-four controls without endocrinological disease were also included. In all subjects, APA were determined by immunofluorescence over primate pituitary gland and ATA by an agglutination technique.ResultsNone of the controls and only 9 patients, all of them women, were APA-positive. Of the 9 APA-positive patients, 43% belonged to the group of patients with isolated ACTH deficiency. In 8 patients, APA were determined again during the follow-up; 6 remained APA-negative, but one patient became APA-positive and another became APA-negative. ATA were positive in 12 patients, 22.2% of whom were also APA-positive, and in one control.ConclusionsThe prevalence of APA positivity among the study patients was low. These antibodies were found only in women, mostly with isolated corticotropin deficiency. APA and ATA coexisted in only 22% of the patients studied.     

Clinical Profile of Ectopic Thyroid in Asian Indians: a Single-Center Experience

ObjectiveTo assess clinical characteristics of patients with ectopic thyroid seen at a single tertiary care center in India.MethodsIn this case series, we retrospectively reviewed the data of patients who presented with ectopic thyroid between January 1995 and March 2008. Clinical presentation, nuclear imaging findings, endocrine profile, and clinical management were analyzed.ResultsRecords of 22 female patients and 14 male patients were reviewed. Ectopic thyroid was more common in female patients. Mean age of presentation was 14.3 years (median, 14 years; range, 5 months to 40 years). Seventeen patients (47%) presented with lingual thyroid, detected incidentally or because of dysphagia and bleeding while eating, and 19 patients (53%) had sublingual thyroid, which mainly presented as an anterior neck swelling. Thirty patients (83%) had hypothyroidism (overt or subclinical). In 29 patients (81%), ectopic thyroid either in the neck or in the lingual area was the only functional thyroid tissue. Thirty-one patients (86%) were treated medically, and surgery was performed in only 5 patients (14%) who had either recurrent bleeding or dysphagia.ConclusionsEctopic thyroid should be considered during the evaluation of a midline neck mass or hypothyroidism. Careful clinical examination, thyroid function tests, and radionuclide imaging help establish the diagnosis and localize ectopic thyroid. Appropriate treatment should be decided on an individual basis. (Endocr Pract. 2009;15:322-325)