A woman with normotensive primary hyperaldosteronism

Normotensive hyperaldosteronism is a rare disorder. It is usually diagnosed with hypokalemia or an adrenal mass. Our patient was a 27-year-old female presented with weakness. She had normal blood pressure, hypokalemia, high plasma aldosterone level and suppressed plasma renin activity. After the saline load, test aldosterone didn�t show suppression. Adrenal computed tomography revealed a left adrenal mass. The patient was treated with spironolactone and potassium supplement. Surgical adrenalectomy was done. Final pathologic diagnosis was benign adrenocortical adenoma without capsular invasion. In postoperative course serum, potassium was normal. © 2015 Tehran University of Medical Sciences. All rights reserved

Ectopic Cushing's syndrome secondary to lung and mediastinal tumours - Report from a tertiary care centre in Iran

Introduction: Ectopic Cushing's syndrome (ECS) secondary to neuroendocrine tumours (NETs) of the lung and mediastinum are rarely encountered. In this study, we present our experience in Iran on 15 patients with ECS secondary to lung and mediastinal tumours over a period of 27 years. Material and methods: Since 1985, 15 patients with ECS secondary to lung and mediastinal tumours have been diagnosed and prospectively followed by the endocrinology team of Taleghani Hospital, Tehran, Iran. The clinical signs and symptoms, laboratory findings, radiological features, immunohistochemical characteristics, management strategies and outcome data are here presented. Results: There were six women and nine men, aged 26-70 years, all presenting with typical features of Cushing's syndrome. Based on histopathologic evaluations, four patients had small cell lung cancer (SCLC) and seven patients had pulmonary NETs, one patient had a thymic NET, and one case was diagnosed as a lung tumourlet. The mean ± SD serum cortisol, 24h urine free cortisol and plasma ACTH were 47.2 ± 20.5 μg/dL, 2,702 ± 5,439 μg/day, and 220 ± 147 pg/mL, respectively. Pulmonary lesions ranged in diameter from 1.1 to 4 cm (mean 1.9 ± 1.1 cm). One patient had a 10 cm mediastinal mass. The duration of follow up in these cases was between one month and seven years (mean 29.9 ± 27.5 months). The four patients with SCLC died within three months of diagnosis. Conclusion: Our data demonstrates the protean clinical and laboratory manifestations of ECS secondary to lung and mediastinal tumours, the problems encountered in diagnosis, and the need for a multidisciplinary approach. This study confirms other series from Western Europe and North America that, unlike the SCLC patients who show a poor outlook, ECS secondary to lung carcinoids has a more favourable prognosis

Ektopowy zespół Cushinga u pacjentów z nowotworem śródpiersia lub płuc — doniesienie z ośrodka trzeciego stopnia referencyjności w Iranie

Introduction: Ectopic Cushing`s syndrome (ECS) secondary to neuroendocrine tumours (NETs) of the lung and mediastinum are rarely encountered. In this study, we present our experience in Iran on 15 patients with ECS secondary to lung and mediastinal tumours over a period of 27 years. Material and methods: Since 1985, 15 patients with ECS secondary to lung and mediastinal tumours have been diagnosed and prospectively followed by the endocrinology team of Taleghani Hospital, Tehran, Iran. The clinical signs and symptoms, laboratory findings, radiological features, immunohistochemical characteristics, management strategies and outcome data are here presented. Results: There were six women and nine men, aged 26–70 years, all presenting with typical features of Cushing’s syndrome. Based on histopathologic evaluations, four patients had small cell lung cancer (SCLC) and seven patients had pulmonary NETs, one patient had a thymic NET, and one case was diagnosed as a lung tumourlet. The mean ± SD serum cortisol, 24h urine free cortisol and plasma ACTH were 47.2 ± 20.5 μg/dL, 2,702 ± 5,439 μg/day, and 220 ± 147 pg/mL, respectively. Pulmonary lesions ranged in diameter from 1.1 to 4 cm (mean 1.9 ± 1.1 cm). One patient had a 10 cm mediastinal mass. The duration of follow up in these cases was between one month and seven years (mean 29.9 ± 27.5 months). The four patients with SCLC died within three months of diagnosis. Conclusion: Our data demonstrates the protean clinical and laboratory manifestations of ECS secondary to lung and mediastinal tumours, the problems encountered in diagnosis, and the need for a multidisciplinary approach. This study confirms other series from Western Europe and North America that, unlike the SCLC patients who show a poor outlook, ECS secondary to lung carcinoids has a more favourable prognosis. Wstep: Ektopowy zespół Cushinga (ectopic Cushing`s syndrome, ECS), który rozwija się u pacjentów mających nowotwory neuroendokrynne (neuroendocrine tumours, NET) śródpiersia lub płuc, jest zaburzeniem rzadko spotykanym. Niniejsza publikacja przedstawia doświadczenia autorów z ośrodka w Iranie, którzy w okresie 27 lat prowadzili 15 pacjentów z zespołem Cushinga wtórnym do nowotworów śródpiersia lub płuc. Materiał i metody: W okresie od 1985 zespół endokrynologów ze szpitala Taleghani Hospital w Teheranie zidentyfikował i prowadził w sposób prospektywny 15 pacjentów z objawami ECS uwarunkowanymi obecnością nowotworów śródpiersia lub płuc. Niniejsza publikacja przedstawia obraz kliniczny i objawy podmiotowe, wyniki badań laboratoryjnych i radiologicznych oraz badań immunohistochemicznych materiału tkankowegoz guzów nowotworowych, a także informacje na temat wdrożonego leczenia i jego wyników. Wyniki: W badanej grupie znalazło się 6 kobiet i 9 mężczyzn, w wieku 26–70 lat. U wszystkich pacjentów stwierdzono typowe cechy kliniczne zespołu Cushinga. Na podstawie badań histopatologicznych wycinków z guzów nowotworowych postawiono rozpoznanie drobnokomórkowego raka płuca (SCLC, small cell lung cancer) u 4 pacjentów, neuroendokrynnego nowotworu płuca (NET) bliżej nieokreślonego typu u 7 pacjentów, NET grasicy u jednej osoby oraz rozpoznanie zmiany typu tumorlet w płucu u jednej osoby. Średnie stężenie kortyzolu w surowicy ( ± odchylenie standardowe) wyniosło 47,2 ± 20,5 μg/dl, stężenie wolnego kortyzolu w moczu ze zbiórki dobowej 2.702 ± 5.439 μg/dobę, a stężenie ACTH w osoczu 220 ± 147 pg/ml. Zmiany nowotworowe w płucach miały wielkość od 1,1 do 4 cm (średni wymiar guza 1,9 ± 1,1 cm). Guz śródpiersia (u jednej osoby) miał średnicę 10 cm. Czas obserwacji wyniósł od 1 miesiąca do 7 lat (średnia 29,9 ± 27,5 miesięcy). Czterech chorych na SCLC zmarło w okresie do 3 miesięcy od postawienia rozpoznania. Wnioski: Przedstawione dane wskazują na różnorodność obrazu klinicznego i danych laboratoryjnych u pacjentów z objawami ECS w przebiegu choroby nowotworowej śródpiersia lub płuc. Konstelacja ta wymaga więc wielospecjalistycznej opieki już od momentu wdrożenia postępowania diagnostycznego. Przedstawione wyniki są zbieżne z danymi z publikacji pochodzących z Europy Zachodniej i Stanów Zjednoczonych i wskazują na stosunkowo korzystne rokowanie u pacjentów mających objawy ECS w związku z rakowiakiem płuca, w przeciwieństwie do pacjentów mających zmiany typu SCLC

N-acetylcysteine does not prevent contrast-induced nephropathy after cardiac catheterization in patients with diabetes mellitus and chronic kidney disease: a randomized clinical trial

<p>Abstract</p> <p>Background</p> <p>Patients with diabetes mellitus (DM) and chronic kidney disease (CKD) constitute to be a high-risk population for the development of contrast-induced nephropathy (CIN), in which the incidence of CIN is estimated to be as high as 50%. We performed this trial to assess the efficacy of <it>N</it>-acetylcysteine (NAC) in the prevention of this complication.</p> <p>Methods</p> <p>In a prospective, double-blind, placebo controlled, randomized clinical trial, we studied 90 patients undergoing elective diagnostic coronary angiography with DM and CKD (serum creatinine ≥ 1.5 mg/dL for men and ≥ 1.4 mg/dL for women). The patients were randomly assigned to receive either oral NAC (600 mg BID, starting 24 h before the procedure) or placebo, in adjunct to hydration. Serum creatinine was measured prior to and 48 h after coronary angiography. The primary end-point was the occurrence of CIN, defined as an increase in serum creatinine ≥ 0.5 mg/dL (44.2 μmol/L) or ≥ 25% above baseline at 48 h after exposure to contrast medium.</p> <p>Results</p> <p>Complete data on the outcomes were available on 87 patients, 45 of whom had received NAC. There were no significant differences between the NAC and placebo groups in baseline characteristics, amount of hydration, or type and volume of contrast used, except in gender (male/female, 20/25 and 34/11, respectively; P = 0.005) and the use of statins (62.2% and 37.8%, respectively; P = 0.034). CIN occurred in 5 out of 45 (11.1%) patients in the NAC group and 6 out of 42 (14.3%) patients in the placebo group (P = 0.656).</p> <p>Conclusion</p> <p>There was no detectable benefit for the prophylactic administration of oral NAC over an aggressive hydration protocol in patients with DM and CKD.</p> <p>Trial registration</p> <p>NCT00808795</p

Treatment of Invasive Silent Somatotroph Pituitary Adenoma with Temozolomide. Report of a Case and Review of the Literature

Improved imaging techniques have contributed to increased diagnosis of pituitary tumors. These tumor types can be microadenomas or macroadenomas and can either be functional or non-functional. Atypical or aggressive pituitary adenomas are tumors that rapidly increase in size and may invade into the suprasellar or parasellar regions. They are characterized by a Ki-67 nuclear labeling index greater than 10 . Management of these tumors is difficult, and many recur after surgery. Temozolomide, a second generation alkylating agent, has been showing promising results in the treatment of these tumors. The patient was a 39-year-old male diagnosed with an invasive silent somatotroph pituitary macroadenoma treated with temozolomide after surgery. We present the case along with the review of the literature of the therapeutic effects of temozolomide in somatotroph macroadenomas. © 2015, Springer Science+Business Media New York

A survey of clinical practice patterns in diagnosis and management of Cushing's disease in Iran

Background: Cushing's disease is the most prevalent cause of endogenous adrenocorticotrophic hormone hypersecretion. The aim of this study was to document the current clinical practice pattern in the management of Cushing's disease by Iranian Endocrinologists to determine their opinions and compare them with the current clinical practice guidelines. Methods: An eight-item questionnaire dealing with diagnosis, treatment and follow up of patients with Cushing's disease was developed, piloted, and sent to the members of Iranian Endocrinology Society. Results: Among 90 endocrinologists invited to participate in the survey, 76 replied. Most respondents selected overnight dexamethasone suppression test (ONDST) and assessment of 24-hour urinary free cortisol (UFC) as the best screening tests followed by midnight serum cortisol and midnight salivary cortisol. Classic high dose dexamethasone suppression test and measurement of serum ACTH were selected for localization of the primary lesion by 64.5. The primary choice of treatment was trans-sphenoidal pituitary surgery (86.8). For the recurrence of Cushing's disease, the preferred treatment modality was medical therapy followed by bilateral adrenalectomy, and pituitary re-surgery. In case of treatment failure after the first pituitary surgery and ketoconazole treatment, 51 chose bilateral adrenalectomy, while36.8 selected pasireotide only. Conclusion: ONDST and UFC are two most common tests used to screen an index case with signs and symptoms of hypercortisolism. The primary choice of treatment in Cushing's disease is pituitary surgery. However, medical treatment by ketokonazol is preferred for the recurrences. Pasireotide is the second alternative after bilateral adrenalectomy in case of treatment failure after pituitary surgery and ketoconazole

A survey of clinical practice patterns in diagnosis and management of Cushing's disease in Iran

Background: Cushing's disease is the most prevalent cause of endogenous adrenocorticotrophic hormone hypersecretion. The aim of this study was to document the current clinical practice pattern in the management of Cushing's disease by Iranian Endocrinologists to determine their opinions and compare them with the current clinical practice guidelines. Methods: An eight-item questionnaire dealing with diagnosis, treatment and follow up of patients with Cushing's disease was developed, piloted, and sent to the members of Iranian Endocrinology Society. Results: Among 90 endocrinologists invited to participate in the survey, 76 replied. Most respondents selected overnight dexamethasone suppression test (ONDST) and assessment of 24-hour urinary free cortisol (UFC) as the best screening tests followed by midnight serum cortisol and midnight salivary cortisol. Classic high dose dexamethasone suppression test and measurement of serum ACTH were selected for localization of the primary lesion by 64.5. The primary choice of treatment was trans-sphenoidal pituitary surgery (86.8). For the recurrence of Cushing's disease, the preferred treatment modality was medical therapy followed by bilateral adrenalectomy, and pituitary re-surgery. In case of treatment failure after the first pituitary surgery and ketoconazole treatment, 51 chose bilateral adrenalectomy, while36.8 selected pasireotide only. Conclusion: ONDST and UFC are two most common tests used to screen an index case with signs and symptoms of hypercortisolism. The primary choice of treatment in Cushing's disease is pituitary surgery. However, medical treatment by ketokonazol is preferred for the recurrences. Pasireotide is the second alternative after bilateral adrenalectomy in case of treatment failure after pituitary surgery and ketoconazole

Cushing syndrome secondary to a thymic carcinoid tumor due to multiple endocrine neoplasia type 1.

OBJECTIVE: To present an Iranian patient with a nonclassic form of multiple endocrine neoplasia type 1 (MEN 1) who presented with ectopic Cushing syndrome (CS) secondary to a corticotropin (ACTH)-producing thymic neuroendocrine tumor (NET), recurrent renal stones, and a giant cell granuloma of the jaw due to primary hyperparathyroidism (PHPT) without involvement of the pituitary or pancreas. METHODS: Relevant imaging and hormonal evaluations were performed. The patient was operated on 2 occasions for a thymic NET and on 3 occasions for PHPT. DNA from a peripheral blood sample was extracted for sequencing of the MEN1 gene. RESULT: Histopathologic evaluation of the thymic tumor removed during the first surgery showed an atypical carcinoid tumor with a Ki-67 labeling index of 5%. Evaluation after the second surgery revealed an invasive carcinoid tumor with a Ki-67 labeling index of 30%. Parathyroid pathology was suggestive of glandular hyperplasia. Menin gene sequencing revealed a novel frameshift mutation c.1642_1648dup in exon 10. CONCLUSION: This case of MEN 1 is unusual because most thymic NETs in MEN 1 are nonfunctional, and secretion of ACTH or other ectopic hormones rarely occurs. In patients presenting with thymic NETs, the possibility of MEN 1 should be considered, especially in the presence of hyperparathyroidism. This case also demonstrates that the behavior of thymic NETs can change over time from slow-growing tumors to highly invasive neoplasia, and that ectopic ACTH can be produced by these tumors in the context of MEN 1