Sandor Magony

Increased short-term beat-to-beat variability of QT interval in patients with acromegaly

Cardiovascular diseases, including ventricular arrhythmias are responsible for increased mortality in patients with acromegaly. Acromegaly may cause repolarization abnormalities such as QT prolongation and impairment of repolarization reserve enhancing liability to arrhythmia. The aim of this study was to determine the short-term beat-to-beat QT variability in patients with acromegaly. Thirty acromegalic patients (23 women and 7 men, mean age±SD: 55.7±10.4 years) were compared with age- and sex-matched volunteers (mean age 51.3±7.6 years). Cardiac repolarization parameters including frequency corrected QT interval, PQ and QRS intervals, duration of terminal part of T waves (Tpeak-Tend) and short-term variability of QT interval were evaluated. All acromegalic patients and controls underwent transthoracic echocardiographic examination. Autonomic function was assessed by means of five standard cardiovascular reflex tests. Comparison of the two groups revealed no significant differences in the conventional ECG parameters of repolarization (QT: 401.1±30.6 ms vs 389.3±16.5 ms, corrected QT interval: 430.1±18.6 ms vs 425.6±17.3 ms, QT dispersion: 38.2±13.2 ms vs 36.6±10.2 ms; acromegaly vs control, respectively). However, short-term beat-to-beat QT variability was significantly increased in acromegalic patients (4.23±1.03 ms vs 3.02±0.80, P<0.0001). There were significant differences between the two groups in the echocardiographic dimensions (left ventricular end diastolic diameter: 52.6±5.4 mm vs 48.0±3.9 mm, left ventricular end systolic diameter: 32.3±5.2 mm vs 29.1±4.4 mm, interventricular septum: 11.1±2.2 mm vs 8.8±0.7 mm, posterior wall of left ventricle: 10.8±1.4 mm vs 8.9±0.7 mm, P<0.05, respectively). Short-term beat-to-beat QT variability was elevated in patients with acromegaly in spite of unchanged conventional parameters of ventricular repolarization. This enhanced temporal QT variability may be an early indicator of increased liability to arrhythmia.

In the labyrinth of calcium metabolism

The authors present the case of a 27-year-old male patient. In 2010, he suffered from a bone fracture of the pelvis. As imaging techniques showed multiple osseal lytic lesions, diagnostic investigations were performed for multiple myeloma. Later, a mass lesion measuring 37 mm in size was removed from the left side of his mandible. Histology revealed a giant-cell tumour of the bone and oncologic therapy was considered. However, before this planned treatment a PET-CT was performed, which showed numerous distinct lesions with enhanced glucose metabolism in the skeleton as well as in soft tissue behind the right lobe of the thyroid. Hence, the patient was referred to endocrinologists. On the basis of severe hypercalcemia (serum calcium 3.66 mmol/l) and high serum parathyroid hormone level (162.5 pmol/l) the diagnosis of a right sided parathyroid tumour was established. After surgical excision of the parathyroid tumour, high levels of serum calcium and parathyroid hormone returned to normal. Histology failed to show malignancy and the patient recovered soon. This case report may shed some light on the importance of serum calcium measurements and the differential diagnostic significance of primary hyperparathyroidism.

Xanthomatous hypophysitis as a cause of cluster headache: a case report

Our case: We report a 23-year-old man with XH who presented with cluster type headache, diabetes insipidus and MRI-proven pituitary intrasellar mass. Since 2009 our patient suffered from recurrent headache. CT scan, ophthalmological, neurological investigations revealed no obvious cause of the symptoms. In April 2011, polyuria-polydipsia occurred and endrocrine investigations revealed diabetes insipidus. Anterior pituitary hormone levels were normal: TSH: 1.3 mIU/l, FSH: 2.4 IU/l, LH: 3.7 IU/l, PR L: 197 mIU/l, ACTH: 7.78 pm/l, cortisol 08 h: 444 nm/l. After initialization of ddAVP treatment, diuresis returned to normal. The pituitary MRI scan revealed a 14x10x17 mm inhomogenous lesion with the disappearance of the hyperintense signal of the neurohypophysis. In July 2011, transsphenoideal surgery was performed and histology proved xanthomatous hypophysitis. We could stop the glucocorticoid (GC) treatment without having any perioperative complication. The headache resolved but the diabetes insipidus persisted. The anterior pituitary function after the surgery was normal: serum cortisol 08 h: 404-445 nm/l, ACTH:6.49 pm/l, FSH:3.1 mIU/l, LH:4.2 mIU/l, TSH:1.61 mIU/l. 2 months later severe cluster type headache occurred. Endocrine investigations revealed hypadrenia, hypothyroidism and peripheral hypogonadism: serum cortisol 08 h: 96 nm/l, TSH: 1.32 mIU/l, ft4: 10.5 pm/l, testosterone: 3.44 nm/l, FSH: 3.3 mIU/l, LH: 2.8 mIU/l, ACTH: 3.38pm/l. LHRH test results: FSH: 0 min: 2.8, 30 min: 4.7, 60 min: 5.1 mIU/l, LH: 0 min: 2.9, 30 min:13.5, 60 min:13.8 mIU/l. The postopreative pituitary MRI scan proved the persistent presence of the inhomogenous mass. After initialization of glucocorticoid replacement the headache disappeared. With levothyroxin, testosterone supplementation and gradually lowered dosage of GC and all symptomps disappeared with the exception of diabetes insipidus. Despite of low IGF 1 (92 ng/ml,age matched reference rate:117-329 ng/ml) and hGH (0.08 ng/ml) levels GH therapy was not introduced. Autoimmune screen: ANA, antiCL, antib2GP, antitransglutaminase, antiTPO and antiparietal cell antibody was negative. Regularly performed sella MRI scans showed no change in tumor size and appearance after the surgery and after the introduction of hormone replacement therapy. The patient requires GC supplementation only in case of recurrent cluster type headache, but no persistent replacement is needed. Conclusion: Typical cluster type headache and diabetes insipidus were the two main syndromes of the XH In our case. The patient requires GC supplementation only in case of recurrent cluster type headache, but no persistent replacement is needed. The cause of the XH is still unknown, but the regular endocrine check-up can reveal disturbances in the pituitary function and, as in our case, glucocorticoid replacement seems to be effective in the treatment of the disease. ABSTRACT Introduction: Hypophysitis is an inflammatory disease of the pituitary gland that may mimic pituitary tumors clinically and radiologically. Primary hypophysitis has traditionally been classified as lymphocytic (LH), granulomatous (GH), and xanthomatous (XH). Case description: We report on a case of a xanthomatous hypophysitis initially diagnosed as pituitary adenoma. A 23-year-old men suffered from typical cluster type headache. Two years after the first symptoms, we confirmed diabetes insipidus. All the anterior pituitary hormone levels were normal. Sella MRI scan depicted a 14x10x17 mm inhomogenous mass. Transphenoidal surgery was performed; the removed tissue showed accumulation of foamy cells and xanthomatous epithelioid cells. Following an uneventful postoperative recovery, severe cluster type headache returned after stopping the hydrocortisone therapy. The endrocrine work-up revealed hypadrenia (morning cortisol: 96 nm/l, ACTH:3.38 pm/l), hypothyroidism ( ft4:10.5 pm/l), hypogonadism (testosterone: 3.44 nm/l) with normal FSH and LH (FSH:3,3 mIU/l,LH:2,8 mIU/l). We restarted hormonal therapy: hydrocortisone, levothyroxine and testosterone were stepwise reintroduced. During the followup period we could stop the hydrocortisone and levothyroxine supplementations, whereas the patient has permanently required desmopressin and testosterone substitution. Control sella MRI scans revealed no progression of the intitially seen pituitary mass. Conclusion: We describe an unusual case of xanthomatous hypophysitis causing cluster type headache and permanenty requiring ddAVP (desmopressine) and testosterone supplementation without requiring maintenance medication with hydrocortisone and levothyroxin.