No abstract

No abstract

No abstract

Langerhans cell histiocytosis (LCH) is a rare disease in which the most common endocrine manifestation is diabetes insipidus (DI). Data on anterior pituitary function in patients with LCH are limited. Thus, the present study investigated anterior pituitary function in LCH patients with DI via the evaluation of clinical and radiological findings at disease onset and during follow-up. The present study retrospectively evaluated nine patients with LCH (five males and four females). All diagnoses of LCH were made following histological and/or immunophenotypic analyses of tissue biopsies, bronchoalveolar lavage, or cerebrospinal fluid (CSF). Basal and, if necessary, dynamic pituitary function tests were used to assess anterior pituitary function, and magnetic resonance imaging (MRI) scans were used to image the pituitary. The LCH treatment modality was based on organ involvement. The mean age at onset of DI was 27.6 years (range 15-60 years). One patient (11%) exhibited single organ involvement, while eight patients (89%) displayed multisystem organ involvement. On admittance, one patient had hypogonadotropic hypogonadism, one patient exhibited panhypopituitarism [hypogonadotropic hypogonadism, central hypothyroidism, hypocortisolism, and growth hormone (GH) deficiency], and four patients (44%) displayed hyperprolactinemia. The MRI data revealed infundibular enlargement in seven patients (78%), a thalamic mass in one patient (11%), and the absence of the bright spot in all patients. A single patient (11%) showed a mass in the pons that had a partially empty sella. The patients were treated with radiation therapy (RT), chemotherapy (CT), or a combination of both (RT+CT) and were followed up for a median of 91.8 months (range 2-318 months). Seven patients were assessed during the follow-up period, of whom four patients (57.1%) developed anterior pituitary hormone deficiency, three (43%) were diagnosed with GH deficiency, and one (14%) exhibited gonadotropin deficiency. The gonadotropin deficiency in the patient, which was diagnosed on admittance, was resolved during the follow-up period. DI persisted in all patients, and the conditions of the seven patients who have remained on follow-up are stable. In the present study, patients with LCH exhibited altered function in the anterior pituitary as well as the posterior pituitary, which may be due to the natural course of the disease or the effects of treatment. The present findings indicate that anterior pituitary function should be assessed in LCH patients on admittance and during follow-up, especially in LCH patients with multisystem organ involvement.

Metformin causes an increase in (18)F-FDG uptake in the bowel and stopping metformin before PET/CT study significantly decreased this unwanted uptake, especially in the colon, facilitating the interpretation of images obtained from the abdomen and preventing the obliteration of lesions.

GAPO syndrome is a very rare genetic disorder with the cardinal manifestations of: growth retardation with delayed bone age (G), alopecia (A), pseudoanodontia (P), and optic atrophy (O) [Orbak et al., 2002].We present for the first time a patient with GAPO syndrome associated with dilated cardiomyopathy. We also discussed the possible explanation for this association. The purpose of this report is to investigate the importance of cardiovascular and endocrinologic investigations in GAPO syndrome.A 31-year-old male patient who was followed up as GAPO syndrome, was admitted to our hospital due to dyspnea complaint. His parents were consanguineous parents (first-degree cousins). His physical examination showed that he had normal mental status and typical clinical features of GAPO syndrome such as frontal bossing, prematurely aged face, glaucoma, buphthalmos, micrognatia, total alopecia, some body hair, having short stature, disproportional body build, failure of tooth eruption (pseudoanodontia), absent eyebrows and eyelashes (Figs. 1 and 2). His blood pressure was 130/80 mmHg and pulse rate was 110/min and rhythmic. He had pretibial pitting edema. His cardiovascular system examination showed that he had a systolic murmur at 2/6 intensity on mitral area. His respiratory system examination showed that he had crepitant rales on both basal areas of lungs and respiratory sounds at low intensity. Gastrointestinal system examination showed that he had a congestive hepatomegaly with free ascites and a liver size of 5 cm length and a smooth and soft surface. ECG examination revealed that he had normal sinus rhythm and normal axis. Telecardiography showed that his cardiothoracic index was increased and the right pleural sinus was closed. Hemogram was normal. Biochemical investigations were as follows: glucose 97 mg/dl, BUN 23 mg/dl, creatinine 1.1 mg/dl, albumin 3.7 g/dl, total cholesterol 132 mg/dl, triglyceride 55 mg/dl, HDL-cholesterol 38 mg/dl, LDL-cholesterol 83 mg/dl. Echocardiography investigation showed that his left cardiac cavities were increased moderately and the left ventricle had global hypokinesia and an ejection fraction as 30-35%. Echocardiography also indicated that he had grade 3 diastolic dysfunction on the left ventricle (deceleration time 105 msec, E/E 0 :18) and second degree mitral insufficiency. Systolic diameter of left ventricle and diastolic diameter of left ventricle were measured as 5.5 and 6.6 cm, respectively. His angiographic investigation showed normal coronary vessels.Endocrinologic investigations revealed that he had normal cortisol, insulin, TSH, FT4, FT3, FSH, LH, prolactin, free testosterone, and DHEA-S levels. While his growth hormone (GH) level was normal at 18 ng/ml (normal <7 ng/ml), insulin like growth factor-1 (IGF-1) level was low at 100 ng/ ml [normal 116-358 ng/ml]). Although his high sensitive C-reactive protein level and HbA1c levels were normal, brain natriuretic peptid (BNP) level was high as 1,020 pg/ ml (N < 100 pg/ml). The patient was diagnosed as dilated cardiomyopathy by...

We have studied the prevalence of fibromyalgia syndrome (FMS) in patients with type 1 and type 2 diabetes mellitus (DM). Relationship with disease control of DM and the presence of FMS was also evaluated. We have studied 93 consecutive patients with DM (85 with type 2 DM and 8 patients with type 1 DM) followed in Diabetes Center. Single researcher took the history and did physical examination including manual tender point examination according to Manual Tender Point Survey instructions. For the diagnosis of FMS, 1990 American College of Rheumatology Classification Criteria for FMS was used. We measured patients' fasting blood sugar levels and HbA1c levels around the same time period. Patients were excluded from the study if they had any other serious disease, and if there was any history of drug use that interferes with the symptoms of FMS. Patients with rheumatoid arthritis (RA) were accepted as controls. FMS was found in 18% of patients with DM type 2 (no patients with FMS in type 1 DM group), in 34% of patients with RA. Female patients with DM type 2 had significantly higher FMS rates. Mean fasting blood sugar levels and mean HbA1c levels were not significantly different between type 2 DM patients with FMS and DM patients without FMS. In RA group, in RA patients without FMS, the use of steroids was significantly higher. We have found an increased prevalence rate of FMS in patients with DM type 2 and RA. There was no correlation between the prevalence rate of FMS and good DM disease control.

IntroductionThere are still not enough data showing whether patients with idiopathic hirsutism (IH) also have insulin resistance. The association between polycystic ovary syndrome (PCOS) and insulin resistance is well documented in the literature, but the Rotterdam Consensus has concluded that principally obese women with PCOS should be screened for the metabolic syndrome. We intended to investigate the presence/absence of insulin resistance in non-obese women with hirsutism.Material and methodsTwenty-eight women with PCOS (14 non-obese and 14 obese), 12 non-obese with IH, and 16 non-obese healthy women were included in the study. The presence of insulin resistance was investigated by using basal insulin levels and the homeostasis model assessment (HOMA) score in the study group.ResultsPatients with obese and nonobese PCOS had significantly (p < 0.05) higher basal insulin levels and HOMA scores than IH and control subjects. Insulin levels and HOMA scores did not differ between obese and non-obese PCOS patients. Patients with IH did not show any difference from the control group.ConclusionsInsulin resistance exists in non-obese women with PCOS as well as obese women with PCOS. The PCOS is associated with insulin resistance independent of obesity. Insulin resistance should be assessed in all hirsute women with PCOS regardless of their body mass index. More studies in larger numbers of patients should be performed to investigate the role of insulin resistance in women with IH.