Landscape of congenital adrenal hyperplasia cases in adult endocrinology clinics of Türkiye-a nation-wide multicentre study.

BACKGROUND AND AIMS: Congenital adrenal hyperplasia (CAH) is a group of disorders that affect the production of steroids in the adrenal gland and are inherited in an autosomal recessive pattern. The clinical and biochemical manifestations of the disorder are diverse, ranging from varying degrees of anomalies of the external genitalia to life-threatening adrenal insufficiency. This multicenter study aimed to determine the demographics, biochemical, clinical, and genetic characteristics besides the current status of adult patients with CAH nationwide. METHODS: The medical records of 223 patients with all forms of CAH were evaluated in the study, which included 19 adult endocrinology clinics. A form inquiring about demographical, etiological, and genetic (where available) data of all forms of CAH patients was filled out and returned by the centers. RESULTS: Among 223 cases 181 (81.16%) patients had 21-hydroxylase deficiency (21OHD), 27 (12.10%) had 11-beta-hydroxylase deficiency (110HD), 13 (5.82%) had 17-hydroxylase deficiency (17OHD) and 2 (0.89%) had 3-beta-hydroxysteroid-dehydrogenase deficiency. 21OHD was the most prevalent CAH form in our national series. There were 102 (56.4%) classical and 79 (43.6%) non-classical 210HD cases in our cohort. The age of the patients was 24.9 ± 6.1 (minimum-maximum: 17-44) for classical CAH patients and 30.2 ± 11.2 (minimum-maximum: 17-67). More patients in the nonclassical CAH group were married and had children. Reconstructive genital surgery was performed in 54 (78.3%) of classical CAH females and 42 (77.8%) of them had no children. Thirty-two (50.8%) NCAH cases had homogenous and 31 (49.2%) had heterogeneous CYP21A2 gene mutations. V281L pathological variation was the most prevalent mutation, it was detected in 35 (55.6%) of 21OHD NCAH patients. CONCLUSION: Our findings are compatible with the current literature except for the higher frequency of 110HD and 17OHD, which may be attributed to unidentified genetic causes. A new classification for CAH cases rather than classical and non-classical may be helpful as the disease exhibits a large clinical and biochemical continuum. Affected cases should be informed of the possible complications they may face. The study concludes that a better understanding of the clinical characteristics of patients with CAH can improve the management of the disorder in daily practice.

An extremely rare case of hypoglycemia with a novel mutation and review of the literature: fructose-1,6 bisphosphatase deficiency in an adult man.

Hypoglycemia is an uncommon clinical problem among non-diabetic patients. It requires systematic evaluation to determine the etiology. It may be related to critical illness, hepatic insufficiency, renal insufficiency, cardiac insufficiency, drugs, alcohol, cortisol insufficiency, growth hormone insufficiency, insulinoma, gastric bypass surgery, and paraneoplastic (insulin-like growth factor-2-related) immune-mediated or inherited metabolic disorders. We aimed to summarize the literature and present a case who suffered from hypoglycemia throughout his life and was diagnosed with fructose-1, 6 bisphosphatase deficiency in adulthood to attract attention to the rare causes of hypoglycemia in adulthood.

Diagnostic value of thyroglobulin washout in fine-needle aspiration samples for diagnosis and follow-up of differentiated thyroid cancer.

Our goal was to assess the effectiveness of fine-needle aspiration thyroglobulin (FNA-Tg) in detecting malignant lymph nodes (LNs) in patients with differentiated thyroid cancer (DTC). We also aimed to determine the factors that affect the accuracy of FNA-Tg. We conducted a retrospective cohort study using the laboratory, ultrasonographic, histopathological, FNA cytology (FNA-C), and FNA-Tg results of 176 DTC patients. We used receiver operating characteristic analysis to identify the cutoff value of FNA-Tg, and binary regression analysis to compare FNA-Tg with other diagnostic parameters. Spearman correlation was utilized to identify factors that influence FNA-Tg. Our study revealed that a cutoff value of 3.14 ng/mL for FNA-Tg had a sensitivity of 91.8% and a specificity of 96.6% in detecting malignant LNs in the entire group. In the subgroup with thyroid tissue, the optimal cutoff value for FNA-Tg was determined to be 15.5 ng/mL. Additionally, FNA-C had a sensitivity of 82.4% and a specificity of 99.4% for the entire group. The combined use of FNA-Tg and FNA-C yielded a sensitivity of 100% and a specificity of 96%, which was found to be more effective than using either test alone. Serum Tg positivity and serum thyroid-stimulating hormone were positively correlated with FNA-Tg levels in detecting malignant LNs. Our study demonstrated that FNA-Tg is a reliable method for detecting LN metastases in DTC patients, with a 3.14 ng/mL cutoff value. However, each center should take into account factors such as serum thyroid-stimulating hormone, serum Tg, and the presence of thyroid tissue when interpreting FNA-Tg results and determining the appropriate cutoff level.

Comparative evaluation of clinical outcomes of dapagliflozin and empagliflozin in type-2 diabetes mellitus.

BACKGROUND: Sodium-glucose transporter 2 (SGLT-2) inhibitors provide additional benefits besides glycemic control. AIM: This study aims to compare the clinical outcomes of dapagliflozin and empagliflozin. METHODS: This retrospective study evaluated data retrieved from medical records of patients who were under follow-up with the diagnosis of type 2 diabetes mellitus (T2DM) and were started on dapagliflozin or empagliflozin treatment between January 1, 2017, and June 1, 2020. Demographic features, comorbidities, clinical features, duration of diabetes, baseline, and follow-up laboratory test results were recorded. The significance level was set at p < 0.05. RESULTS: This study comprised 342 patients who are on the treatment with dapagliflozin (n = 228) or empagliflozin (n = 114). The glycosylated hemoglobin a1c (HBA1C) level was significantly decreased in both the dapagliflozin (8.18-7.59, p < 0.001) and empagliflozin (8.35-7.58, p < 0.001) groups. The urine albumin-to-creatinine ratio (ACR) was also decreased in both groups. A decrease in urine ACR was observed independent of using a renin-angiotensin-aldosterone system (RAAS) blocker both in the whole group and in patients with diabetic nephropathy. The time to addition of a new anti-diabetic agent to the treatment was shorter in the dapagliflozin group (14.4 months vs 17.7 months, p = 0.041, respectively). CONCLUSION: Dapagliflozin and empagliflozin are the drugs to choose for renoprotection in diabetics independent of the use of a RAAS blocker. Even the time to addition of a new anti-diabetic agent is longer in the empagliflozin group, head-to-head comparative trials are needed to asess the potential differences in this regard.

Identifying Clinical Characteristics of Hypoparathyroidism in Turkey: HIPOPARATURK-NET Study.

Hypoparathyroidism is an orphan disease with ill-defined epidemiology that is subject to geographic variability. We conducted this study to assess the demographics, etiologic distribution, treatment patterns and complication frequency of patients with chronic hypoparathyroidism in Turkey. This is a retrospective, cross-sectional database study, with collaboration of 30 endocrinology centers located in 20 cities across seven geographical regions of Turkey. A total of 830 adults (mean age 49.6 ± 13.5 years; female 81.2%) with hypoparathyroidism (mean duration 9.7 ± 9.0 years) were included in the final analysis. Hypoparathyroidism was predominantly surgery-induced (n = 686, 82.6%). The insulting surgeries was carried out mostly due to benign causes in postsurgical group (SG) (n = 504, 73.5%) while patients in nonsurgical group (NSG) was most frequently classified as idiopathic (n = 103, 71.5%). The treatment was highly dependent on calcium salts (n = 771, 92.9%), calcitriol (n = 786, 94.7%) and to a lower extent cholecalciferol use (n = 635, 76.5%) while the rate of parathyroid hormone (n = 2, 0.2%) use was low. Serum calcium levels were most frequently kept in the normal range (sCa 8.5-10.5 mg/dL, n = 383, 46.1%) which might be higher than desired for this patient group. NSG had a lower mean plasma PTH concentration (6.42 ± 5.53 vs. 9.09 ± 7.08 ng/l, p < 0.0001), higher daily intake of elementary calcium (2038 ± 1214 vs. 1846 ± 1355 mg/day, p = 0.0193) and calcitriol (0.78 ± 0.39 vs. 0.69 ± 0.38 mcg/day, p = 0.0057), a higher rate of chronic renal disease (9.7% vs. 3.6%, p = 0.0017), epilepsy (6.3% vs. 1.6%, p = 0.0009), intracranial calcifications (11.8% vs. 7.3%, p < 0.0001) and cataracts (22.2% vs. 13.7%, p = 0.0096) compared to SG. In conclusion, postsurgical hypoparathyroidism is the dominant etiology of hypoparathyroidism in Turkey while the nonsurgical patients have a higher disease burden with greater need for medications and increased risk of complications than the postsurgical patients.

Clinical Characteristics and Follow-Up Results of Adrenal Incidentaloma.

It is recommended that adrenal incidentaloma patients should be monitored for radiological changes, increase in size and new functionality that may occur in the future, even if they are benign and nonfunctional at the initial evaluation. Our aim is to evaluate the key clinical characteristics of adrenal incidentaloma patients focusing on changes during follow-up and associated clinical outcomes. A total of 755 patients (median age: 56 years), with an adrenal incidentaloma > 1 cm and underwent functionality tests, were included in the study. Clinical characteristics, functionality status and follow-up durations were recorded. During the course of follow-up, any changes in size and development of new functionality, and clinical consequences thereof were evaluated. In 71.8% of patients, incidentalomas were non-functional. Most frequent functionality (15.8%, n=119) was subclinical hypercortisolemia (SH) [10.9% (n=82) possible autonomous cortisol secretion (PACS) and 4.9% (n=37) autonomous cortisol secretion (ACS)] of all incidentalomas. Frequencies of Cushing's syndrome (CS), pheochromacytoma and primary hyperaldosteronism were 4.9% (n=37), 3.8% (n=29) and 3.7% (n=28), respectively. Adrenocortical carcinoma frequency was 1.5% (n=11). Of 755 patients, 43% (n=325) were followed up regularly more than 6 months. Median follow-up duration was 24 months (6-120). A total of 17 (5.2%) patients, which had non-functional incidentalomas at baseline had developed new functionality during follow-up, of which 15 (4.6%) were SH [13 patients (4%) PACS and 2 patients (0.6%) ACS] and 2 (0.6%) were CS. During follow-up, 24% (n=78) of the patients had an increase in mass size between 5-9 mm, while 11.7% (n=38) of the patients had an increase of ≥10 mm. During follow-up, 4% (n=13) of the patients developed a new lesion with a diameter ≥10 mm on the opposite side. In patients with a follow-up duration of more than 2 years, frequencies of size increase and new lesion emerging at the opposite adrenal gland were higher. 14 patients (4.3% of the patients with regular follow-up) underwent surgery due to increase in size or development of new functionality during follow-up. Our study demonstrated that a necessity for surgery may arise due to increase in size and development of functionality during follow-up period in adrenal incidentaloma patients, and thus continuing patient follow-up, even with wider intervals, will be appropriate.

Cytopathology-histopathology correlation and the effect of nodule diameter on diagnostic performance in patients undergoing thyroid fine-needle aspiration biopsy.

INTRODUCTION: Although thyroid fine-needle aspiration biopsy (FNAB) is established to have a good overall sensitivity and specificity, various outcomes have been reported on its performance in large nodules. The aim of the study was to evaluate the diagnostic performance of FNAB and the effect of the nodule diameter on its diagnostic performance. MATERIALS AND METHODS: The outcomes of a total of 7319 patients who underwent FNAB over the course of 5 years were analyzed retrospectively and 648 patients who had undergone post-FNAB thyroidectomy or lobectomy were included in the study. FNAB results were classified according to the Bethesda system. After evaluating the compatibility between cytology and pathology results, all-nodules and diameter-based (<4 cm and ≥4 cm) sensitivity, specificity, false positivity, false negativity, and accuracy rates of FNAB were calculated. RESULTS: Sensitivity of FNAB was 85.4% for all nodules, 88.3% for nodules <4 cm, and 75.8% for nodules >4 cm (P < 0.001). Specificity was 58.4% for all nodules, 49.3% for nodules <4 cm, and 75.1% for nodules >4cm (P < 0.001). While false positivity was 41.6% for all nodules, it was 50.7% for nodules smaller than 4 cm and was 24.9% for nodules larger than 4 cm (P < 0.001). False negativity was 14.6% for all nodules and was 11.7% for nodules smaller than 4 cm and 24.2% for nodules larger than 4 cm (P < 0.001). Finally, among the entire set of nodules, the accuracy was 64.4%, which was 59.2% in nodules smaller than 4 cm, and 75.2% in nodules larger than 4 cm (P < 0.001). CONCLUSION: Despite a higher rate of false negativity, FNAB has higher specificity and accuracy in large nodules than those in the small nodules. Nodule diameter should not be used alone as a criterion to recommend thyroidectomy to the patient.

Effects of Different Immunosuppressive Drugs on Incretins in Renal Transplant Patients.

BACKGROUND: Immunosuppressive drugs used in transplantation patients, may contribute to the development of post-transplant diabetes mellitus through their possible adverse effects on incretins. We aimed to compare the effects of different immunosuppressive drugs used in renal transplantation patients on glucagon-like peptide-1 (GLP-1) and glucose-dependent insulinotropic polypeptide (GIP) levels. PATIENTS AND METHODS: Forty five subjects were enrolled in the study (cyclosporine-treated 15 and tacrolimus-treated renal transplant patients 15, and healthy volunteers as a control group 15). Oral glucose tolerance test with 75 gr glucose was performed. GLP-1 and GIP levels were measured at 0 (baseline), 30, 60, 90, 120 min using ELISA method. RESULTS: A statistically significant level of difference was detected in GLP-1 levels at the baseline, 30th and 120th minutes among all three groups (p < 0,001, p = 0,026 and p = 0,022, respectively). Baseline GLP-1 levels in cyclosporine-treated renal transplant patients were higher than in both tacrolimus-treated renal transplant patients (p = 0,016) and control groups (p < 0,001). GLP-1 levels at the 30th minute were higher in tacrolimus-treated renal transplant patients when compared to the cyclosporine-treated renal transplant patients (p = 0,024). GLP-1 levels at the 120th minute were higher in tacrolimus-treated renal transplant patients than the control group (p = 0,024). The areas under the curve of GLP-1 was higher in tacrolimus-treated renal transplant patients when compared to the control group (p = 0,018). GIP levels at 120th was lower in cyclosporine-treated renal transplant patients when compared to control group (p = 0,003). CONCLUSION: These findings showed a temporally affected incretin hormones in renal transplant patients, a preserved GLP-1 response to an oral glucose load in renal transplant patients on cyclosporine and increased GLP -1 response to an oral glucose load in those on tacrolimus.

Diagnostic value of the late-night salivary cortisol in the diagnosis of clinical and subclinical Cushing's syndrome: results of a single-center 7-year experience.

Late-night salivary cortisol (LNSaC) is an easy-to-use test reflecting the free cortisol level in the serum and does not require hospitalization. Controlled studies reported that LNSaC has a high sensitivity and specificity, but have not set a clearly defined cut-off value to be used in the diagnosis of Cushing's syndrome. In this study, we aimed to evaluate the diagnostic performance of LNSaC in patients with clinical Cushing's syndrome (CCS) and subclinical Cushing's syndrome (SCS). The data of 543 patients, whose LNSaC levels were assessed using electrochemiluminescence immunoassay method, were retrospectively evaluated. The study included a total of 324 patients: 58 patients with CCS, 53 patients with SCS, and 213 patients without Cushing's syndrome (NoCS). The cause of the Cushing's syndrome was hypophyseal in 26 patients (45%), adrenal in 24 patients (41%), and ectopic in 8 patients (14%) in the CCS group. Median LNSaC levels were 0.724 (0.107-33) µg/dL in CCS group, 0.398 (0.16-1.02) µg/dL in SCS group, and 0.18 (0.043-0.481) µg/dL in NoCS group (p=0.001). Accordingly, LNSaC had 89.6% sensitivity and 81.6% specificity at a cut-off value of 0.288 µg/dL in the diagnosis of CCS; and had 80.7% sensitivity and 85.1% specificity at a cut-off value of 0.273 µg/dL in the diagnosis of SCS. In the present study, a lower sensitivity and specificity than previously reported was found for LNSaC in the diagnosis of CCS. Moreover, the diagnostic performance of LNSaC in patients with SCS was close to its diagnostic performance in patients with CCS. Each center should determine its own cut-off value based on the method adopted for LNSaC measurement, and apply that cut-off value in the diagnosis of Cushing's syndrome.

Evaluation of Incidental Thyroid Nodules in Cancer Patients.

OBJECTIVE: Frequency of thyroid cancer in incidental thyroid nodules identified by imaging techniques in cancer patients is higher than that in the normal population. In the retrospective study, we have both investigated the incidence of thyroid cancer in incidentally identified nodules and compared the imaging techniques to determine whether there is any difference between them in detection of malign nodules. METHODS: A total of 7319 patients who underwent thyroid fine-needle aspiration biopsy (FNAB) were included in the study. The data of 174 patients who had previously been diagnosed with a hematologic or solid malignancy prior to the FNAB procedure and had incidentally identified thyroid nodules were evaluated retrospectively. RESULTS: Eighty-six (49.5%) of the incidental nodules were identified with ultrasonography (USG), 62 (35.6%) with positron emission tomography (PET) or PET/computed tomography (PET/CT), and 26 (14.9%) with CT. As a result of thyroidectomy, papillary carcinoma was identified in 8 (4.6%) patients, and metastasis to the thyroid of a primary cancer was found in 3 (1.7%) patients. While the papillary carcinoma proportion in the nodules identified by USG was 3.4%, PET/CT was 8.9%. A cut-off maximal standardized uptake value of 11.6 in PET/CT indicated malignancy achieving a sensitivity of 83.3% and a specificity of 91.1%. CONCLUSION: Whether the nodule in the incidental thyroid nodules of cancer patients is identified using USG or PET/CT, the risk of thyroid cancer is similar. However, cancer risk is higher in the event of a higher focal uptake in the nodules identified by PET/CT.

GLP-1 and GIP Levels in Patients With Hyperthyroidism: The Effect of Antithyroid Treatment.

BACKGROUND: Incretin hormones (glucagon-like peptide-1 [GLP-1] and gastric inhibitory polypeptide [GIP]) may play a role in the development of glucose intolerance and hyperglycemia in patients with hyperthyroidism. OBJECTIVE: We aimed to assess both incretin levels and treatment-induced changes in incretin levels in those with hyperthyroidism. METHODS: A total of 24 subjects (12 with hyperthyroidism and 12 healthy) were enrolled in the study. Oral glucose tolerance test was performed and serum glucose, insulin GLP1, and GIP levels were evaluated at 0 (baseline), 30, 60, 90, and 120 minutes using ELISA. Measurements were repeated after euthyroidism was reached in subjects with hyperthyroidism. RESULTS: The baseline glucose level was higher in those with hyperthyroidism compared with controls ( P = 0.03). GLP-1 and GIP responses to oral glucose load did not differ significantly between those with hyperthyroidism and controls. Peak GLP-1 and GIP levels were reached in both groups at 60 and 90 minutes, respectively. Areas under the curve (AUCs) for GLP1 and GIP were similar in those with hyperthyroidism and controls. Although GLP-1 and GIP levels did not change before and after antithyroid treatment in subjects with hyperthyroidism, time to peak GLP-1 and GIP levels were reached at 30 minutes after euthyroid state was achieved. Reversal of hyperthyroid to euthyroid status did not induce significant changes in AUCs for incretins. CONCLUSION: The findings of the present study suggest that the total incretin response to oral glucose load is preserved in patients with hypertyhroidism, but peak incretin responses may change after achieving euthyroid state.

ASSESSMENT OF DIASTOLIC DYSFUNCTION, ARTERIAL STIFFNESS, AND CAROTID INTIMA-MEDIA THICKNESS IN PATIENTS WITH ACROMEGALY.

OBJECTIVE: Early diagnosis and treatment of cardiovascular diseases, the most frequent cause of morbidity and mortality in acromegaly, may be an efficient approach to extending the lifespan of affected patients. Therefore, it is crucial to determine any cardiovascular diseases in the subclinical period. The study objectives were to determine markers of subclinical atherosclerosis and asses heart structure and function. METHODS: This was a cross-sectional, single-center study of 53 patients with acromegaly and 22 age- and sex-matched healthy individuals. Carotid intima-media thickness (CIMT), pulse-wave velocity (PWV), and echocardiographic data were compared between these groups. RESULTS: CIMT and PWV were higher in the acromegaly group than in the healthy group (P = .008 and P = .002, respectively). Echocardiography showed that left ventricular diastolic dysfunction was present in 11.3% of patients. Left ventricular mass index and left atrial volume index were higher in the patients (P = .016 and P<.001, respectively). No differences in the CIMT, PWV, or echocardiographic measurements were identified between the patients with biochemically controlled and uncontrolled acromegaly and the control group. CONCLUSION: Our results showed that subclinical atherosclerosis (i.e., CIMT and PWV markers) and heart structure and function were worse in patients with acromegaly than in healthy individuals. Because there were no differences in these parameters between patients with controlled and uncontrolled acromegaly, our results suggest that the structural and functional changes do not reverse with biochemical control. ABBREVIATIONS: AA = active acromegaly BSA = body surface area CA = biochemically controlled acromegaly CH = concentric hypertrophy CIMT = carotid intima-media thickness DBP = diastolic blood pressure DM = diabetes mellitus ECHO = echocardiography EDV = enddiastolic volume EF = ejection fraction ESV = endsystolic volume GH = growth hormone HC = healthy control HL = hyperlipidemia HT = hypertension IGF-1 = insulin-like growth factor 1 LA = left atrial LAV = left atrial volume LAVI = left atrial volume index LV = left ventricular LVDD = left ventricular diastolic dysfunction LVEF = left ventricular ejection fraction LVH = left ventricular hypertrophy LVMI = left ventricular mass index PWV = pulse-wave velocity RWT = relative wall thickness.

Markers of Subclinical Cardiovascular Disease in Nonfunctional Adrenal Incidentaloma Patients without Traditional Cardiovascular Risk Factors.

Introduction: Data regarding cardiovascular risk in patients with non-functional adrenal incidentaloma (NFAI) are limited. The objectives of this study are to investigate markers of subclinical cardiovascular disease like carotid intima media thickness (CIMT), pulse wave velocity (PWV), augmentation index (AIx), soluble CD40 ligand (sCD40L) and leptin levels in NFAI patients without traditional cardiovascular risk factors and healthy control group. Methods: This study involved 35 patients with NFAI (11 males, 24 females; mean age, 52.4±7.7 years) and 35 healthy subjects as control group (11 males, 24 females; mean age, 51.8±7.2 years). CIMT was evaluated by ultrasonographical methods. PWV and AIx were measured with TensioClinic arteriograph system. Serum leptin and sCD40L levels were measured by ELISA. Results: In NFAI patients group; CIMT (p<0.001), PWV (p<0.001), AIx brachial (p<0.001) and AIx aorta (p=0.008) were found higher than the control group. Cortisol levels after 1mg dexamethasone suppression test (DST) were higher (p=0.006) and DHEASO4 levels were lower (p=0.008) in NFAI patients than control group. We found that CIMT had positive correlation with age (r=0.484, p<0.005), triglycerides (r=0.378, p<0.005) and cortisol level after 1 mg DST (r=0.346, p<0.005); PWV had positive correlation with total cholesterol (r=0.338, p<0.005) triglycerides (r=0.335, p<0.05) and insulin levels (r=0.426, p<0.005); AIx brachial had a positive correlation with triglycerides (r=0414, p<0.05) and negative correlation with DHEASO4 (r=-0.380, p<0.005); leptin levels had a positive correlation with body mass index (r=0.541, p<0.001) and HOMA-IR index. Conclusion: We showed that subjects with NFAI without traditional cardiovascular risk factors featured several disturbances (CIMT, PWV and AIx) compared to the control group that could be attributable to increased cardiovascular risk.

Parathyroidectomy in asymptomatic primary hyperparathyroidism reduces carotid intima-media thickness and arterial stiffness.

OBJECTIVE: Although an International Workshop has suggested that cardiovascular assessment in asymptomatic primary hyperparathyroidism (PHPT) patients is not necessary, improvements in risk factors of subclinical atherosclerosis have been shown following parathyroidectomy. The objectives of this study were to determine whether parathyroidectomy in asymptomatic PHPT patients causes any change in carotid intima-media thickness (CIMT), arterial stiffness [pulse wave velocity (PWV)] and soluble CD40 ligand (sCD40L) levels. DESIGN: Prospective study evaluating female patients diagnosed with asymptomatic PHPT in a single centre over a 6-month period. PATIENTS: A total of 48 subjects were included: 17 hypercalcaemic (HC, mean age: 51 ± 8 years, Ca: 2·73 ± 0·17 mmol/l) and 16 normocalcaemic (NC, mean age: 58 ± 7 years, Ca: 2·30 ± 0·10 mmol/l) PHPT patients, and 15 healthy controls (mean age: 52 ± 4 years, Ca: 2·27 ± 0·07 mmol/l). MEASUREMENTS: Biochemical tests, CIMT, PWV and sCD40L levels were compared at baseline and 6 months after parathyroidectomy (PTx). RESULTS: At baseline, CIMT and PWV values in the HC and NC patients were higher than in the control group. While there was a significant reduction in CIMT (601 ± 91 µm vs 541 ± 65 µm, P = 0·006) and PWV (9·6 ± 1·8 vs 8·4 ± 1·5 m/s, P = 0·000) in the hypercalcaemic group at the end of the 6th month after PTx, no change was observed in normocalcaemic group (P = 0·686 and P = 0·196 respectively). No differences were observed in sCD40L levels between patient and control groups or between baseline and 6 months in patients undergoing parathyroidectomy. CONCLUSION: Parathyroidectomy leads to an improvement in the structural and functional impairment associated with atherosclerosis in the vascular wall in asymptomatic hypercalcaemic PHPT patients.

Acquired partial lipodystrophy is associated with increased risk for developing metabolic abnormalities.

OBJECTIVE: Acquired partial lipodystrophy (APL) is a rare disorder characterized by progressive selective fat loss. In previous studies, metabolic abnormalities were reported to be relatively rare in APL, whilst they were quite common in other types of lipodystrophy syndromes. METHODS: In this nationwide cohort study, we evaluated 21 Turkish patients with APL who were enrolled in a prospective follow-up protocol. Subjects were investigated for metabolic abnormalities. Fat distribution was assessed by whole body MRI. Hepatic steatosis was evaluated by ultrasound, MRI and MR spectroscopy. Patients with diabetes underwent a mix meal stimulated C-peptide/insulin test to investigate pancreatic beta cell functions. Leptin and adiponectin levels were measured. RESULTS: Fifteen individuals (71.4%) had at least one metabolic abnormality. Six patients (28.6%) had diabetes, 12 (57.1%) hypertrigylceridemia, 10 (47.6%) low HDL cholesterol, and 11 (52.4%) hepatic steatosis. Steatohepatitis was further confirmed in 2 patients with liver biopsy. Anti-GAD was negative in all APL patients with diabetes. APL patients with diabetes had lower leptin and adiponectin levels compared to patients with type 2 diabetes and healthy controls. However, contrary to what we observed in patients with congenital generalized lipodystrophy (CGL), we did not detect consistently very low leptin levels in APL patients. The mix meal test suggested that APL patients with diabetes had a significant amount of functional pancreatic beta cells, and their diabetes was apparently associated with insulin resistance. CONCLUSIONS: Our results show that APL is associated with increased risk for developing metabolic abnormalities. We suggest that close long-term follow-up is required to identify and manage metabolic abnormalities in APL.

Subcutaneous NPH Insulin for Severe Hypertriglyceridemia in a Pregnant Patient with Type V Hyperlipoproteinemia: a Case Report.

An increase in triglyceride levels in familial hyperlipidemia during pregnancy has been reported. Severe hypertriglyceridemia can lead to complications such as acute pancreatitis, preeclampsia, maternal and fetal complications. Because of the teratogenic effects associated with fibrate therapy in pregnancy, alternative treatment strategies such as insulin as a rapid and potent activator of lipoprotein lipase are required during pregnancy. We report a case of hypertriglyceridemia in a 33-year-old pregnant woman in whom treatment with merely single one time administration of Neutral Protamine Hagedorn insulin was accompanied by a reduction in the serum triglyceride level; to the best of our knowledge, this has never been reported in the literature. Her triglyceride level was 3616 mg/dL before insulin treatment and 1246 mg/dL after insulin treatment. Although this regimen was used safely and effectively in our patient, comprehensive studies are required to evaluate the effectiveness and safety of subcutaneously intermediate-acting Neutral Protamine Hagedorn insulin for the treatment of severe hypertriglyceridemia in non-diabetic pregnant women.

Diagnostic yield of routine duodenal biopsies in iron deficiency anaemia: a study from Western Anatolia.

OBJECTIVE: The role of routine endoscopic duodenal biopsies obtained during the evaluation of iron deficiency anaemia is being increasingly emphasized, but insufficiently applied. Diagnostic yield of this practice, mainly identification of coeliac disease, differs in different populations and geographic regions. The aim of this study is to assess the usefulness of routine duodenal biopsies during upper endoscopy in patients presenting with iron deficiency anaemia in Western Anatolia. METHODS: Routine duodenal biopsies were evaluated over a 12-month period in 100 consecutive adult patients with iron deficiency anaemia undergoing upper endoscopy. All potential bleeding lesions were identified and gastric as well as duodenal biopsies were taken for histopathologic investigation. RESULTS: A bleeding lesion is identified in 44% of cases. Duodenal biopsy gives an additional 5% diagnostic yield and revealed three patients with coeliac disease and two patients with giardiasis. One of the patients diagnosed with coeliac disease had a second bleeding lesion at the upper endoscopic examination. Appearance of the duodenal mucosa was normal in all patients including those with diagnostic duodenal biopsy. CONCLUSIONS: Routine duodenal sampling during the upper endoscopic examination gives an additional 5% diagnostic benefit and this practice should be included in the diagnostic work-up of patients with iron deficiency anaemia. As one of the patients who was found to have coeliac disease had a second bleeding lesion that may otherwise explain iron deficiency anaemia, finding a source for bleeding at the upper endoscopy should not preclude duodenal biopsy. Moreover, performing duodenal biopsy is still necessary even though the endoscopic appearance of the mucosa is normal. Aside from coeliac disease, Giardia infestation could be identified as a contributory factor for iron deficiency anaemia, in endemic regions.

[The pathogens and their antibiotic sensitivities in hospital-acquired pneumonia cases]. / Hastane kökenli pnömoni olgularinda etkenler ve antibiyotik duyarliliklari.

Dynamic precautions in the early diagnosis and treatment of hospital acquired pneumonias are necessary because of their high mortality. In these patients, invasive diagnostic approaches may be needed since clinical and radiological findings and other non-invasive approaches frequently fail to establish the diagnosis. Thirty eight patients were prospectively included in the study. Average age of patients was 45.5 +/- 16.4 years; 31 were males (81.6%) and 7 (18.4%) were females. Pneumonia was detected in 9 (23.7%) cases during the first five days and in 29 (76.3%) cases after the fifth day of admission to the hospital. Bronchoscopic interventions diagnostic purpose were carried out in 25 (65.8%) patients. The culture results were negative in 7 (18.4%) cases. While more than one pathogen was determined on the cultures of 16 (42.1%) patients only one pathogen was isolated in the cultures of 15 (39.4%) cases. The frequently isolated pathogen on cultures was Staphylococci (45.4%). Other pathogens were Enterobacter spp., Pseudomonas spp., Escherichia coli, Serratia and Streptococcus pneumoniae according to their frequency on cultures. High resistance rates to the third generation cephalosporins were determined. Eleven of 17 deaths in 38 pneumonia cases were attributable to pneumonia. As a conclusion, isolation of pathogen and antibiotic resistance should be determined in the cases with hospital acquired pneumonia. Invasive diagnostic interventions were not avoided when necessary. Although pro-BAL and PSB methods were expensive, their use in selected cases may prevent unnecessary antibiotic use and contribute to a decrease in mortality rate.