A possible connection between circulating 25-hydroxy-vitamin D and molecular response in chronic myeloid leukemia.

OBJECTIVE: In this study we aimed to evaluate whether there is a link between circulating 25-OH-D levels and molecular response in chronic myeloid leukemia (CML). MATERIAL AND METHOD: A total of 61 patients with CML (31 women, 30 men) were recruited in this cross-sectional study. RESULTS: Binary logistic regression analysis demonstrated that increased vitamin D levels were independently associated with molecular response in subjects with CML. CONCLUSION: Our results indicated for the first time in the literature that severe deficiency of vitamin D was independently associated with molecular unresponsiveness in subjects with CML. 25-OH-D may be contributing to molecular response in the patients (Tab. 3, Ref. 24).

Etiology of hypopituitarism in tertiary care institutions in Turkish population: analysis of 773 patients from Pituitary Study Group database.

Hypopituitarism in adult life is commonly acquired and the main causes are known as pituitary tumors and/or their treatments. Since there are new insights into the etiology of hypopituitarism and presence of differences in various populations, more studies regarding causes of hypopituitarism are needed to be done in different ethnic groups with sufficient number of patients. Therefore, we performed a multi-center database study in Turkish population investigating the etiology of hypopituitarism in 773 patients in tertiary care institutions. The study was designed and coordinated by the Pituitary Study Group of SEMT (The Society of Endocrinology and Metabolism of Turkey). Nineteen tertiary reference centers (14 university hospitals and 5 training hospitals) from the different regions of Turkey participated in the study. It is a cross-sectional database study, and the data were recorded for 18 months. We mainly classified the causes of hypopituitarism as pituitary tumors (due to direct effects of the pituitary tumors and/or their treatments), extra-pituitary tumors and non-tumoral causes. Mean age of 773 patients (49.8 % male, 50.2 % female) was 43.9 ± 16.1 years (range 16-84 years). The most common etiology of pituitary dysfunction was due to non-tumoral causes (49.2 %) among all patients. However, when we analyze the causes according to gender, the most common etiology in males was pituitary tumors, but the most common etiology in females was non-tumoral causes. According to the subgroup analysis of the causes of hypopituitarism in all patients, the most common four causes of hypopituitarism which have frequencies over 10 % were as follows: non-secretory pituitary adenomas, Sheehan's syndrome, lactotroph adenomas and idiopathic. With regard to the type of hormonal deficiencies; FSH/LH deficiency was the most common hormonal deficit (84.9 % of the patients). In 33.8 % of the patients, 4 anterior pituitary hormone deficiencies (FSH/LH, ACTH, TSH, and GH) were present. Among all patients, the most frequent cause of hypopituitarism was non-secretory pituitary adenomas. However, in female patients, present study clearly demonstrates that Sheehan's syndrome is still one of the most important causes of hypopituitarism in Turkish population. Further, population-based prospective studies need to be done to understand the prevalence and incidence of the causes of hypopituitarism in different countries.

Hand measurements in the follow-up of acromegaly.

Acromegaly is a chronic progressive disease that originates from the increased secretion of the insulin-like growth-hormone (IGF-1) secondary to the hypersecretion of the growth hormone (GH). The enlargement of the minor hand and foot bones represents an early finding in this disease. Kleinberg et al. used the sesamoid index (SI) values for diagnosing the disease. The present trial was designed to investigate whether there was a difference between the control patients and the treated acromegalic patients in the SI, the terminal tuft width, the joint space and the metacarpal thickness. 34 patients were diagnosed and treated for acromegaly at the Rheumatology and Endocrinology Outpatient Clinics and 26 control patients presenting to the Rheumatology Outpatient Clinic, who were not detected to have an inflammatory rheumatologic pathology were enrolled. The hand radiographs of the patients that followed up for acromegaly and the control group were retrospectively evaluated. The SI, the tuft width, the joint space and the metacarpal thickness were measured. There was a statistically significant difference in the other parameters between the acromegalic patients and the control patients except the mean metacarpal thickness.

A case series of Bardet-Biedl syndrome in a large Turkish family and review of the literature.

BACKGROUND AND AIM: Bardet-Biedl syndrome (BBS) is a rare autosomal recessive genetic disorder. We aimed to report a case series of Bardet-Biedl syndrome in a Turkish family and review the literature. PATIENTS AND METHODS: This family had 3 females and 4 males, totally 7 alive; 2 children (1 female and 1 male) had died. Parents were consanguineous. The first was a birth of twins and female sibling of these twins had congenital anal atresia and died when she was three-month old. Third (30 yr), 4th (28 yr) and 9th (19 yr) alive siblings were obese, blind and diabetic. We detected truncal obesity, postaxial polydactyly, cognitive impairment and hypogonadism. Rod-cone dystrophy was detected in ophthalmic examination. With these typical clinical findings, BBS was diagnosed. There was also a male member of the family which shared the same features of his affected brothers but he had died while he was eight months old. Marked glycosuria was determined and urine density was 1021 g/cm3. There was not any further endocrinological abnormality. Fasting blood glucose levels were changing between 290 and 452 mg/dl and the last glycated hemoglobine levels (A1c) were 9.3%, 11.2% and 12.8%, respectively. Diabetes mellitus and obesity were treated with diet, exercise, multiple daily insulin injections and metformine at the dose of 2000 mg/d. CONCLUSIONS: Although it is an infrequent condition due to autosomal recessive transmission, consanguineous marriage may increase the risk of emergence of BBS. Genetic counseling is a very important issue in the family of patients with BBS, in order to prevent new cases.

Minimally invasive focused parathyroidectomy without using intraoperative parathyroid hormone monitoring or gamma probe.

BACKGROUND: Minimally invasive parathyroidectomy (MIP) is widely used worldwide for the treatment of primary hyperparathyroidism (pHPT). It is usually combined with a perioperative adjunct for high success rate. AIM: To demonstrate that MIP can be successfully performed in a selected group of patients with presumabally solitary adenoma as the cause of pHPT without using any perioperative adjuncts. SETTINGS AND DESIGN: A prospective data analysis of two surgeons' series from a teaching hospital in Turkey. MATERIALS AND METHODS: Of the 47 patients referred with a diagnosis of pHPT during January 2004-May 2008, 30(63%) patients with sporadic pHPT with presumed solitary adenoma were included for analysis. These patients underwent MIP via focused lateral (n=24) or anterior (n=6) approach. Preoperative localization was done using 99 mTc-labelled sestamibi scan and ultrasonography. Only patients with concordant tests for single adenoma were selected for MIP. Serum parathyroid hormone and calcium levels were measured postoperatively and at follow-up visits. STATISTICAL ANALYSIS: Parametric data presented were analyzed with Excel XP (Microsoft, Redmond, WA, USA). RESULTS: Barring one patient, all other patients were initially biochemically cured by MIP. One patient remained hypercalcemic, who was found to have a second adenoma at the second operation. During a mean follow-up of 16 (3-55) months, all patients were normocalcemic with a mean serum calcium level of 9.4 (8.9-10.2) mg/dl. Parathormone levels were persistantly elevated only in one patient (3.4%). No postoperative permanent complication was encountered. CONCLUSION: The results of MIP achieved in high-volume endocrine surgery centers can be replicated in low-volume center without any intraoperative adjuncts, in patients with overt clinical pHPT and concordant results of sestamibi and ultrasound.