Patient Demographics > Country of Treatment > Turkey

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Kursad Unluhizarci Department of Endocrinology

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Hulya Akgun Department of Pathology

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Bahadır Oz Department of Surgery, Erciyes University Medical School, Kayseri, Turkey

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Zuleyha Karaca Department of Endocrinology

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Fatih Tanriverdi Department of Endocrinology

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Fahrettin Kelestimur Department of Endocrinology

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Summary

Among various substances produced by C-cells, the most important one is calcitonin (CT) that is used for detection, post-operative follow-up and evaluation of individuals at risk of developing medullary thyroid carcinoma (MTC). However, the role of serum CT measurement in the evaluation of thyroid nodules has been widely discussed, and there is still no consensus about the role of CT in the initial evaluation of all thyroid nodules. Two patients with thyroid nodules whose fine-needle aspiration results were compatible with benign cytology besides having mildly elevated basal serum calcitonin levels were reported. Calcitonin responses (peak levels were 313 and 229 pg/mL, respectively) to calcium stimulation test were compatible with the possible diagnosis of MTC. However, the final diagnosis was papillary thyroid carcinoma of the thyroid gland. There are limited numbers of case reports showing such an increased serum calcitonin responses to calcium stimulation test associated with papillary or follicular thyroid carcinoma of the thyroid. We suggest to measure serum CT level once and in case of normal levels, no further CT measurement is necessary. Physicians should keep in mind that thyroid carcinomas other than MTCs may also be associated with high serum CT levels.

Learning points:

  • Although serum calcitonin is a valuable tumor marker for MTC, it is well known that mild elevations may be seen in some other diseases such as Hashimoto thyroiditis, neuroendocrine tumors or due to medications such as proton pump inhibitors, calcium salts, beta blockers and glucocorticoids.

  • Those two cases indicate that high calcitonin responses to calcium stimulation test, mimicking MTC, may also be seen in patients with papillary thyroid carcinoma although the mechanism is not clear.

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Gulay Simsek Bagir Departments of Endocrinology

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Soner Civi Departments of Endocrinology

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Ozgur Kardes Departments of Endocrinology

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Fazilet Kayaselcuk Departments of Endocrinology

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Melek Eda Ertorer Departments of Endocrinology

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Summary

Pituitary apoplexy (PA) may very rarely present with hiccups. A 32-year-old man with classical acromegaloid features was admitted with headache, nausea, vomiting and stubborn hiccups. Pituitary magnetic resonance imaging (MRI) demonstrated apoplexy of a macroadenoma with suprasellar extension abutting the optic chiasm. Plasma growth hormone (GH) levels exhibited suppression (below <1 ng/mL) at all time points during GH suppression test with 75 g oral glucose. After treatment with corticosteroid agents, he underwent transsphenoidal pituitary surgery and hiccups disappeared postoperatively. The GH secretion potential of the tumor was clearly demonstrated immunohistochemically. We conclude that stubborn hiccups in a patient with a pituitary macroadenoma may be a sign of massive apoplexy that may result in hormonal remission.

Learning points:

  • Patients with pituitary apoplexy may rarely present with hiccups.

  • Stubborn hiccupping may be a sign of generalized infarction of a large tumor irritating the midbrain.

  • Infarction can be so massive that it may cause cessation of hormonal overproduction and result in remission.

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Ozen Oz Gul Department of Endocrinology and Metabolism

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Pinar Sisman Department of Endocrinology and Metabolism

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Soner Cander Department of Endocrinology and Metabolism

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Erdem Gozden Department of Hematalogy

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Meral Kurt Department of Radiation Oncology

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Ozlem Saraydaroglu Department of Pathology

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Turkay Kirdak Department of Surgery, Uludağ University Medical School, Bursa, Turkey

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Canan Ersoy Department of Endocrinology and Metabolism

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Erdinc Erturk Department of Endocrinology and Metabolism

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Summary

Langerhans cell histiocytosis (LCH) is a rare sporadic disease characterized by histiocytic neoplastic infiltration of various organ systems and a wide spectrum of clinical manifestations, ranging from benign and self-limiting to lethal. Herein, we report a rare case of adult-onset multi-systemic LCH in a 36-year-old male patient with an initial perianal presentation and incidental finding of subsequent thyroid gland involvement in the follow-up period. The patient with a history of perianal LCH treated with surgical excision and local radiotherapy was referred to our Endocrinology Department upon detection of hypermetabolic nodular lesions in the left lateral lobe of thyroid gland on positron emission tomography–computed tomography (PET/CT) scan in the nineth month of follow-up. Current evaluation revealed euthyroid status, a hypoechoic solid lesion of 13 × 9 mm in size with irregular borders in the left thyroid lobe on thyroid USG and cytologic assessment of thyroid nodule. The patient was diagnosed with suspected, oncocytic lesion, Hashimoto thyroiditis or LCH. The patient underwent total thyroidectomy and pathological assessment confirmed the diagnosis of Langerhans cell histiocytosis. Assessments in the sixth month of postoperative follow-up revealed euthyroid status with no thyroid tissue remnants or pathological lymph node on thyroid USG. In view of the multifocal lesions indicating multi-system disease, a systemic chemotherapy protocol with combination of prednisone (PRED) and vinblastine (VBL) has been planned by the hematology department.

Learning points:

  • Langerhans cell histiocytosis (LCH) shows a wide clinical spectrum and prognosis that ranges from benign and self-limiting single-system disease (with single or multifocal lesions) to a potentially lethal multi-system disease with severe organ dysfunction and death in some cases.

  • It has been stated that the diagnosis is often delayed in perianal LCH unless LCH is specifically considered in the etiology, despite the fact that mucosal involvement may precede systemic involvement.

  • Our findings support the statement that most of patients with LCH were PET positive at the time of initial diagnosis, while also emphasize the inclusion of this imaging modality as a part of the diagnostic workflow as well as in the setting of treatment response evaluation among adult LCH patients.

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Bilal Katipoglu Department of Internal Medicine, Ankara Numune Training and Research Hospital, Ankara, Turkey

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Ihsan Ates Department of Internal Medicine, Ankara Numune Training and Research Hospital, Ankara, Turkey

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Fatih Acehan Department of Internal Medicine, Ankara Numune Training and Research Hospital, Ankara, Turkey

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Ayşenur Meteris Department of Internal Medicine, Ankara Numune Training and Research Hospital, Ankara, Turkey

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Nisbet Yılmaz Department of Internal Medicine, Ankara Numune Training and Research Hospital, Ankara, Turkey

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Summary

Hypothyroidism is a wide clinical spectrum disorder and only a few cases in literature show this. Rhabdomyolysis and acute renal impairment can be seen concurrently in a hypothyroid state. We report a case of severe hypothyroidism with poor drug compliance leading to rhabdomyolysis and acute kidney injury.

Learning points:

  • Hypothyroidism is a rare cause of acute kidney injury.

  • In this case report, we studied a rare occurrence of acute renal impairment due to hypothyroidism with poor drug compliance, which induced rhabdomyolysis.

  • Our report emphasized that thyroid status should be evaluated in patients with unexplained acute renal impairment or presenting with the symptoms of muscle involvement.

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Aysenur Ozderya Division of Endocrinology and Metabolic Diseases, Kartal Dr Lutfi Kirdar Training and Research Hospital, 34890, Istanbul, Turkey

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Sule Temizkan Division of Endocrinology and Metabolic Diseases, Kartal Dr Lutfi Kirdar Training and Research Hospital, 34890, Istanbul, Turkey

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Kadriye Aydin Tezcan Division of Endocrinology and Metabolic Diseases, Kartal Dr Lutfi Kirdar Training and Research Hospital, 34890, Istanbul, Turkey

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Feyza Yener Ozturk Division of Endocrinology and Metabolic Diseases, Sisli Etfal Training and Research Hospital, Istanbul, Turkey

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Yuksel Altuntas Division of Endocrinology and Metabolic Diseases, Sisli Etfal Training and Research Hospital, Istanbul, Turkey

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Summary

Madelung's disease is a rare fat metabolism disorder characterised by benign multiple symmetric, encapsulated lipomatosis. The exact cause of the disease is unknown; it may be associated with chronic alcoholism and mutations in mitochondrial DNA (A8344G), but there have been cases without these factors reported in the literature. A 29-year-old man with a 6-year history of diabetes mellitus was admitted to our hospital for poorly regulated diabetes and decreased libido. He was not an alcohol consumer. His family history was unremarkable. Physical examination revealed that he had a eunuchoid body shape. There was a symmetric excess fat accumulation in his submandibular, deltoid, nuchal, suprapubic and inguinal areas. He was diagnosed with Madelung's disease, and imaging studies supported the diagnosis. Hormonal evaluation revealed a hypergonadotropic hypogonadism. Karyotype analysis revealed a 47,XXY mutation. Genetic research showed no mitochondrial DNA mutation. Metabolic disorders, such as diabetes mellitus, hyperlipidaemia, hyperuricaemia and liver disease, endocrine gland diseases, such as hypothyroidism, and neurological diseases, such as polyneuropathy and cognitive disorders, may accompany Madelung's disease. The present study represents the first reported case of Madelung's disease accompanied by Klinefelter's syndrome.

Learning points

  • Madelung's disease is a rare fat metabolism disorder characterised by benign multiple symmetric and encapsulated lipid accumulation.

  • The exact cause of the disease is unknown.

  • Metabolic disorders, such as diabetes mellitus, hyperlipidaemia, hyperuricaemia and liver disease, endocrine gland diseases, such as hypothyroidism, and neurological diseases, such as polyneuropathy and cognitive disorders, may accompany Madelung's disease.

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