Baseline Characteristics of a Patient Cohort and Predictors of In-hospital MORtality in CORonary Care Units (MORCOR-TURK) Trial in Türkiye.

OBJECTIVE: The MORtality in CORonary Care Units in Türkiye (MORCOR-TURK) trial is a national registry evaluating predictors and rates of in-hospital mortality in coronary care unit (CCU) patients in Türkiye. This report describes the baseline demographic characteristics of patients recruited for the MORCOR-TURK trial. METHODS: The study is a multicenter, cross-sectional, prospective national registry that included 50 centers capable of 24-hour CCU service, selected from all seven geographic regions of Türkiye. All consecutive patients admitted to CCUs with cardiovascular emergencies between September 1-30, 2022, were prospectively enrolled. Baseline demographic characteristics, admission diagnoses, laboratory data, and cardiovascular risk factors were recorded. RESULTS: A total of 3,157 patients with a mean age of 65 years (range: 56-73) and 2,087 (66.1%) males were included in the analysis. Patients with arterial hypertension [1,864 patients (59%)], diabetes mellitus (DM) [1,184 (37.5%)], hyperlipidemia [1,120 (35.5%)], and smoking [1,093 (34.6%)] were noted. Non-ST elevation myocardial infarction (NSTEMI) was the leading cause of admission [1,187 patients (37.6%)], followed by ST elevation myocardial infarction (STEMI) in 742 patients (23.5%). Other frequent diagnoses included decompensated heart failure (HF) [339 patients (10.7%)] and arrhythmia [272 patients (8.6%)], respectively. Atrial fibrillation (AF) was the most common pathological rhythm [442 patients (14%)], and chest pain was the most common primary complaint [2,173 patients (68.8%)]. CONCLUSION: The most common admission diagnosis was acute coronary syndrome (ACS), particularly NSTEMI. Hypertension and DM were found to be the two leading risk factors, and AF was the most commonly seen pathological rhythm in all hospitalized patients. These findings may be useful in understanding the characteristics of patients admitted to CCUs and thus in taking precautions to decrease CCU admissions.

The Effect of Aortic Angulation on Clinical Outcomes of Patients Undergoing Transcatheter Aortic Valve Replacement.

INTRODUCTION: The aim of this study was to assess the impact of aortic angulation (AA) on periprocedural and in-hospital complications as well as mortality of patients undergoing Evolut™ R valve implantation. METHODS: A retrospective study was conducted on 264 patients who underwent transfemoral-approach transcatheter aortic valve replacement with self-expandable valve at our hospital between August 2015 and August 2022. These patients underwent multislice computer tomography scans to evaluate AA. Transcatheter aortic valve replacement endpoints, device success, and clinical events were assessed according to the definitions provided by the Valve Academic Research Consortium-3. Cumulative events included paravalvular leak, permanent pacemaker implantation, new-onset stroke, and in-hospital mortality. Patients were divided into two groups, AA ≤ 48° and AA > 48°, based on the mean AA measurement (48.3±8.8) on multislice computer tomography. RESULTS: Multivariable logistic regression analysis was performed to identify predictors of cumulative events, utilizing variables with a P-value < 0.2 obtained from univariable logistic regression analysis, including AA, age, hypertension, chronic renal failure, and heart failure. AA (odds ratio [OR]: 1.73, 95% confidence interval [CI]: 0.89-3.38, P=0.104), age (OR: 1.04, 95% CI: 0.99-1.10, P=0.099), hypertension (OR: 1.66, 95% CI: 0.82-3.33, P=0.155), chronic renal failure (OR: 1.82, 95% CI: 0.92-3.61, P=0.084), and heart failure (OR: 0.57, 95% CI: 0.27-1.21, P=0.145) were not found to be significantly associated with cumulative events in the multivariable logistic regression analysis. CONCLUSION: This study demonstrated that increased AA does not have a significant impact on intraprocedural and periprocedural complications of patients with new generation self-expandable valves implanted.

Effects of the protective and therapeutic effect of yogurt enriched with Citrus sinensis essential oil against ibuprofen-induced gastric ulcer in elderly rats.

The aim of this study was to evaluate the effect of Citrus sinensis essential oil (EO) on the proximate composition of yogurt over a 28-day shelf life and to investigate the therapeutic and prophylactic effects of functional yogurt on ibuprofen-induced gastric ulcers in a rat model. It was observed that the yogurt group containing C. sinensis EO had higher acidity, total solids, and ash values. Histologic evaluation of the stomachs of rats with gastric ulcers revealed that rats fed with functional yogurt had fewer lesions compared to the control group. The treatment group had fewer lesions than the positive control (p > 0.05). Lesions in the glandular mucosa of the prophylactic group were significantly lower than those in the positive control group (p < 0.05). Yogurt with C. sinensis EO may be beneficial in reducing the severity of ulcers and improving overall health.

The effect of body mass index on complications in cardiac implantable electronic device surgery.

BACKGROUND: Cardiac implantable electronic device (CIED) procedures are prone to complications. In our study, we investigated the effect of body mass index (BMI) on CIED-related complications. METHODS: 1676 patients who had undergone CIED surgery (de novo implantation, system upgrade, generator change, pocket revision or lead replacement) at two heart centers in Turkey and met the study criteria were included in our study. For analysis of primary and secondary endpoints, patients were classified as non-obese (BMI < 25 kg/m2 ), overweight (25 ≤ BMI < 30 kg/m2 ), and obese (BMI ≥ 30 kg/m2 ). The primary endpoint was accepted as cumulative events, including the composite of clinically significant hematoma (CSH), pericardial effusion or tamponade, pneumothorax, and infection related to the device system. Secondary outcomes included each component of cumulative events. RESULTS: The rate of cumulative events, defined as primary outcome, was higher in the obese patient group, and we found a significant difference between the groups (3.0%, 4.3%, 8.9%, p = .001). CSH and pneumothorax rates were significantly higher in the obese patient group (0.3%, 0.9%, 1.9%, p = .04; 1.0%, 1.4%, 3.3%, p = .04, respectively). According to our multivariate model analysis; gender (OR:1.882, 95%CI:1.156-3.064, p = .01), hypertension (OR:4.768, 95%CI:2.470-9.204, p < .001), BMI (OR:1.069, 95%CI:1.012-1.129, p = .01) were independent predictors of cumulative events rates. CONCLUSIONS: Periprocedural complications associated with CIED (especially hematoma and pneumothorax) are more common in the group with high BMI.

The Effect of Aortic Angulation on Clinical Outcomes of Patients Undergoing Transcatheter Aortic Valve Replacement

ABSTRACT Introduction: The aim of this study was to assess the impact of aortic angulation (AA) on periprocedural and in-hospital complications as well as mortality of patients undergoing Evolut™ R valve implantation. Methods: A retrospective study was conducted on 264 patients who underwent transfemoral-approach transcatheter aortic valve replacement with self-expandable valve at our hospital between August 2015 and August 2022. These patients underwent multislice computer tomography scans to evaluate AA. Transcatheter aortic valve replacement endpoints, device success, and clinical events were assessed according to the definitions provided by the Valve Academic Research Consortium-3. Cumulative events included paravalvular leak, permanent pacemaker implantation, new-onset stroke, and in-hospital mortality. Patients were divided into two groups, AA ≤ 48° and AA > 48°, based on the mean AA measurement (48.3±8.8) on multislice computer tomography. Results: Multivariable logistic regression analysis was performed to identify predictors of cumulative events, utilizing variables with a P-value < 0.2 obtained from univariable logistic regression analysis, including AA, age, hypertension, chronic renal failure, and heart failure. AA (odds ratio [OR]: 1.73, 95% confidence interval [CI]: 0.89-3.38, P=0.104), age (OR: 1.04, 95% CI: 0.99-1.10, P=0.099), hypertension (OR: 1.66, 95% CI: 0.82-3.33, P=0.155), chronic renal failure (OR: 1.82, 95% CI: 0.92-3.61, P=0.084), and heart failure (OR: 0.57, 95% CI: 0.27-1.21, P=0.145) were not found to be significantly associated with cumulative events in the multivariable logistic regression analysis. Conclusion: This study demonstrated that increased AA does not have a significant impact on intraprocedural and periprocedural complications of patients with new generation self-expandable valves implanted.

Evaluation and management of neonatal onset hyperinsulinemic hypoglycemia: a single neonatal center experience.

OBJECTIVES: To evaluate the clinical characteristics and treatment options of neonates requiring prolonged hospitalization due to persistent hyperinsulinemic hypoglycemia (HH). METHODS: This retrospective cohort study included infants >34 weeks of gestation at birth who were born in our hospital between 2018 and 2021, diagnosed with HH, and required diazoxide within the first 28 days of life. The baseline clinical characteristics, age at the time of diagnosis and treatment options in diazoxide resistance cases were recorded. Genetic mutation analysis, if performed, was also included. RESULTS: A total of 32 infants diagnosed with neonatal HH were followed up. Among the cohort, 25 infants were classified as having transient form of HH and seven infants were classified as having congenital hyperinsulinemic hypoglycemia (CHI). Thirty-one percent of the infants had no risk factors. The median birth weight was significantly higher in the CHI group, whereas no differences were found in other baseline characteristics. Patients diagnosed with CHI required higher glucose infusion rate, higher doses, and longer duration of diazoxide treatment than those in the transient HH group. Eight patients were resistant to diazoxide, and six of them required treatment with octreotide and finally sirolimus. Sirolimus prevented the need of pancreatectomy in five of six patients without causing major side effects. Homozygous mutations in the ABCC8 gene were found in four patients with CHI. CONCLUSIONS: The risk of persistent neonatal hyperinsulinism should be considered in hypoglycemic neonates particularly located in regions with high rates of consanguinity. Our study demonstrated sirolimus as an effective treatment option in avoiding pancreatectomy in severe cases.

Unusual case report of malignant pheochromocytoma presenting with STEMI.

Background: Pheochromocytomas (PHEOs) are a group of tumours that leads to multiple symptoms and can induce hypercoagulability and promote thrombosis. Pheochromocytomas may also present without elevated serum and urinary markers. We aimed to provide tips and tricks for the diagnostic and therapeutic management of an unusual case of PHEOs. Case summary: Thirty-four-year-old woman with the unremarkable medical history presented with epigastric pain and dyspnoea. Electrocardiogram showed ST-segment elevation in the inferior limb leads. She underwent an emergency coronary angiogram, which showed a high thrombus burden in the distal right coronary artery. A subsequent echocardiogram demonstrated a 31 × 33 mm right atrial mass adhering to the inferior vena cava and abdominal computed tomography (CT) scan revealed a 113 × 85 mm necrotic mass in the left adrenal bed, with tumour thrombus extending proximally to the confluence of hepatic veins immediately inferior to the right atrium and distally to iliac vein bifurcation. Blood parameters, thrombophilia panel, vanillylmandelic acid, 5 hydroxy indole acetic acid, and homovanillic acid levels were normal. Tissue sampling confirmed the diagnosis of PHEOs. The surgical procedure was not planned due to the presence of metastatic foci on imaging, including positron emission tomography (PET)-CT. Anticoagulation with rivaroxaban and treatment with 177Lu-DOTATATE-based peptide receptor radionuclide therapy (PRRT) was initiated. Discussion: The coexistence of arterial and venous thrombosis is extremely rare in patients with PHEOs. Multidisciplinary approaches are required for the care of such patients. Catecholamines likely contributed to the development of thrombosis in our patient. Early recognition of PHEOs is the key point to ameliorate clinical outcomes.

Catastrophic embolism of a suddenly ruptured isolated cardiac cyst hydatid: An unusual case report.

Hydatid cysts can be located in any organ or tissue system. Cardiac hydatid cyst is a rare, but fatal pathology. A 21-year-old male Syrian refugee patient with no previous known medical conditions was admitted to the hospital for chest pain and shortness of breath. He had increasing leg pain for 12 hours. Hydatid cyst rupture was detected on echocardiography. The peripheral artery thrombus and hydatid cyst membrane were removed with the embolectomy. The patient had renal and cranial infarctions. He underwent fasciotomy due to compartment syndrome. In conclusion, delayed diagnosis and treatment of cardiac hydatid cysts may result in a poor prognosis associated with the risk of rupture and is responsible for the spread of infection throughout the body as a result of the rupture. Even if there is an early diagnosis, surgical treatment supported by medical treatment is recommended very early.

Clinical features of generalized lipodystrophy in Turkey: A cohort analysis.

AIM: To describe the Turkish generalized lipodystrophy (GL) cohort with the frequency of each complication and the death rate during the period of the follow-up. METHODS: This study reports on 72 patients with GL (47 families) registered at different centres in Turkey that cover all regions of the country. The mean ± SD follow-up was 86 ± 78 months. RESULTS: The Kaplan-Meier estimate of the median time to diagnosis of diabetes and/or prediabetes was 16 years. Hyperglycaemia was not controlled in 37 of 45 patients (82.2%) with diabetes. Hypertriglyceridaemia developed in 65 patients (90.3%). The Kaplan-Meier estimate of the median time to diagnosis of hypertriglyceridaemia was 14 years. Hypertriglyceridaemia was severe (≥ 500 mg/dl) in 38 patients (52.8%). Seven (9.7%) patients suffered from pancreatitis. The Kaplan-Meier estimate of the median time to diagnosis of hepatic steatosis was 15 years. Liver disease progressed to cirrhosis in nine patients (12.5%). Liver disease was more severe in congenital lipodystrophy type 2 (CGL2). Proteinuric chronic kidney disease (CKD) developed in 32 patients (44.4%) and cardiac disease in 23 patients (31.9%). Kaplan-Meier estimates of the median time to diagnosis of CKD and cardiac disease were 25 and 45 years, respectively. Females appeared to have a more severe metabolic disease, with an earlier onset of metabolic abnormalities. Ten patients died during the follow-up period. Causes of death were end-stage renal disease, sepsis (because of recurrent intestinal perforations, coronavirus disease, diabetic foot infection and following coronary artery bypass graft surgery), myocardial infarction, heart failure because of dilated cardiomyopathy, stroke, liver complications and angiosarcoma. CONCLUSIONS: Standard treatment approaches have only a limited impact and do not prevent the development of severe metabolic abnormalities and early onset of organ complications in GL.

Metreleptin Treatment in a Boy with Congenital Generalized Lipodystrophy due to Homozygous c.465_468delGACT (p.T156Rfs*8) Mutation in the BSCL2 Gene: Results From the First-year

Congenital generalized lipodystrophy (CGL) is a rare, autosomal recessive disorder characterized by an almost complete absence of body fat. In CGL, patients may have hyperphagia due to leptin deficiency. Recombinant human leptin (metreleptin) has been suggested as an effective treatment option. We present successful treatment with metreleptin in a boy with CGL and results from the first year of follow-up. An eight-month-old boy presented with excessive hair growth and a muscular appearance. On examination he had hypertrichosis, decreased subcutaneous adipose tissue over the whole body and hepatomegaly. Laboratory investigations revealed hypertriglyceridemia, hyperinsulinemia, elevated liver transaminases and low leptin levels. Molecular genetic analysis detected a homozygous, c.465_468delGACT (p.T156Rfs*8) mutation in the BSCL2 gene. A diagnosis of CGL type 2 was considered. Despite dietary intervention, exercise, and treatment with additional omega-3 and metformin, the hypertriglyceridemia, hyperinsulinemia, and elevated liver transaminase levels worsened. Metreleptin treatment was started and after one year hyperphagia had disappeared, and there was dramatic improvement in levels of insulin, hemoglobin A1c, triglycerides and liver transaminases. Hepatosteatosis was lessened and hepatosplenomegaly was much improved. Metreleptin appears to be an effective treatment option in children with CGL that remarkably improved metabolic complications in the presented case. Initiation of metreleptin treatment in the early period may decrease mortality and morbidity, and increase the quality of life in children with CGL.

The effect of nutritional scores on mortality in COVID-19 patients

SUMMARY OBJECTIVES: While studies on the treatment for the coronavirus disease 2019 (COVID-19) pandemic continue all over the world, factors that increase the risk of severe disease have also been the subject of research. Malnutrition has been considered an independent risk factor. Therefore, we aimed to investigate the clinical effect of dietary habits and evaluate the prognostic value of the Controlling Nutritional Status score in the COVID-19 patients we followed up. METHODS: A total of 2760 patients hospitalized for COVID-19 were examined. Patients were retrospectively screened from three different centers between September 1 and November 30, 2020. A total of 1488 (53.9%) patients who met the criteria were included in the study. Risk classifications were made according to the calculation methods of prognostic nutritional index and Controlling Nutritional Status scores and total scores. The primary outcome of the study was in-hospital mortality. RESULTS: The groups with severe Controlling Nutritional Status and prognostic nutritional index scores had a significantly higher mortality rate than those with mild scores. In the multivariable regression analysis performed to determine in-hospital mortality, the parameters, such as age (OR 1.04; 95%CI 1.02-1.06, p<0.001), admission oxygen saturation value (SaO2) (OR 0.85; 95%CI 0.83-0.87, p<0.001), and Controlling Nutritional Status score (OR 1.34; 95%CI 1.23-1.45, p<0.001), were independent predictors. The patient groups with a low Controlling Nutritional Status score had a higher rate of discharge with recovery (p<0.001). CONCLUSIONS: Higher Controlling Nutritional Status scores may be effective in determining in-hospital mortality in patients with COVID-19. Nutrition scores can be used as a useful and effective parameter to determine prognosis in patients with COVID-19.

Relationship between contrast-induced nephropathy and long-term mortality after percutaneous coronary intervention in patients with chronic coronary total occlusion

SUMMARY OBJECTIVE: Intervention in chronic total occlusion lesions involves long procedure time, a serious contrast load, and complex procedures. In this study, we aimed to investigate mortality rate of patients who had procedural coronary angiography done for chronic total occlusion lesions in coronary angiography series and who developed contrast-induced nephropathy. METHODS: A total of 218 patients with chronic total occlusion lesion in at least one coronary artery, from three different medical centers, who underwent procedural coronary angiography were recruited for the study. Patient population was divided into two groups: those who developed contrast-induced nephropathy and those who did not. Mortality due to all causes was investigated between both groups throughout a 100-month follow-up. RESULTS: Mean age of patients with incidence of contrast-induced nephropathy was 66.7±11.8, and 23.8% of them were comprised by female. We found a significantly higher mortality in long-term follow-up in the patient group with contrast-induced nephropathy (42.9 vs. 57.1%, p≤0.001). According to Kaplan-Meier analysis performed additionally, survival during follow-up was significantly shorter in this group and, in logistic regression analysis, it was an independent predictor of mortality (OR 3.02; 95%CI 1.41-6.45, p=0.004). CONCLUSION: We identified that the development of contrast-induced nephropathy is associated with long-term mortality. It might be possible to reduce adverse events with prophylactic approaches before the procedure and close follow-up of such patients after the procedure.

A comparison of postoperative complications following cardiac implantable electronic device procedures in patients treated with antithrombotic drugs.

BACKGROUND: The incidence of postoperative complications following cardiac implantable electronic device (CIED) procedures in patients treated with antithrombotic drugs has not been studied sufficiently. Here we present a comparison of complications after CIED implantations. METHODS: Using an observational study design, the study included 1807 patients with a taking antiplatelet drugs (n: 1601), nonvitamin K anticoagulants (NOAC) (n: 136), and warfarin (n: 70) undergoing CIED surgery. Primary endpoint was accepted as cumulative events including composite of clinically significant hematoma (CSH), pericardial effusion or tamponade, pneumothorax, and infection related to device system. Secondary outcomes included each compenent of cumulative events. Multivariable analysis was performed to identify predictors of cumulative events. RESULTS: The overall cumulative event rate was 3.7% (67 of 1807). Cumulative events occured 3.1% (50 of 1601) in the antiplatelet, 5.1% (7 of 136) NOAC, and 14.3% (10 of 70) warfarin groups (p < 0.001). CSH occurred in 2 of 70 patients (2.9%) in the warfarin group, as compared with 5 of 1601 (0.3%) in the antiplatelet group (p: 0.032). However, no significant differences were found between NOAC and warfarin groups in terms of CSH (0.7% vs. 2.9% respectively, p: 0.267). Warfarin treatment was an independent predictor of cumulative events and increased 2.9-fold the risk of cumulative events. Major surgical complications were rare and did not differ significantly between the study groups. CONCLUSIONS: The incidence and severity of complications may be lower in patients treated with periprocedurally antiplatelet or NOAC therapy when compared with warfarin therapy. Further randomized control studies are required to confirm our findings.

A novel predictor in patients with coronary chronic total occlusion: systemic immune-inflammation index: a single-center cross-sectional study

SUMMARY OBJECTIVE: Severe inflammation is reportedly associated with subsequent cardiovascular events, including in patients with coronary artery disease. This study aimed to examine the prognostic value of systemic immune-inflammation index and determine mortality and clinical outcomes in patients with chronic coronary total occlusion. METHODS: Our study evaluated 366 consecutive coronary total occlusion patients. The clinical end points were all-cause mortality and major adverse cardiovascular events, which include target vessel revascularization, myocardial infarction, and cerebrovascular events during 105 months follow-up. RESULTS: The study findings showed 59 (16.1%) all-cause death, 22 (6%) target vessel revascularization cases, 32 (8.7%) myocardial infarction cases, and 13 (3.6%) cerebrovascular events cases, with a median follow-up of 49 months (26-74). Multivariate logistic regression analysis showed that systemic immune-inflammation index was not associated with target vessel revascularization, myocardial infarction, and cerebrovascular events. Multivariate Cox regression analysis found systemic immune-inflammation index to be associated with all-cause death. Kaplan-Meier analysis showed a lower survival rate and myocardial infarction-free survival time in patients with higher systemic immune-inflammation index scores. CONCLUSION: Although systemic immune-inflammation index is a preferable tool for the detection of mortality, it failed to give adverse outcomes. Larger multicenter studies are thus warranted to investigate the effect of systemic immune-inflammation index on clinical outcomes.

Recommendations for Clinical Decision-making in Children with Type 1 Diabetes and Celiac Disease: Type 1 Diabetes and Celiac Disease Joint Working Group Report

It is well-known that in children with type 1 diabetes (T1D), the frequency of Celiac disease (CD) is increased due to mechanisms which are not fully elucidated but include autoimmune injury as well as shared genetic predisposition. Although histopathologic examination is the gold standard for diagnosis, avoiding unnecessary endoscopy is crucial. Therefore, for both clinicians and patients' families, the diagnosis of CD remains challenging. In light of this, a joint working group, the Type 1 Diabetes and Celiac Disease Joint Working Group, was convened, with the aim of reporting institutional data and reviewing current international guidelines, in order to provide a framework for clinicians. Several controversial issues were discussed: For CD screening in children with T1D, regardless of age, it is recommended to measure tissue transglutaminase-immunoglobulin A (tTG-IgA) and/or endomysial-IgA antibody due to their high sensitivity and specificity. However, the decision-making process based on tTG-IgA titer in children with T1D is still debated, since tTG-IgA titers may fluctuate in children with T1D. Moreover, seronegativity may occur spontaneously. The authors' own data showed that most of the cases who have biopsy-proven CD had tTG-IgA levels 7-10 times above the upper limit. The decision for endoscopy based solely on tTG-IgA levels should be avoided, except in cases where tTG-IgA levels are seven times and above the upper limit. A closer collaboration should be built between divisions of pediatric endocrinology and gastroenterology in terms of screening, diagnosis and follow-up of children with T1D and suspicious CD.

Frequency of Celiac Disease and Spontaneous Normalization Rate of Celiac Serology in Children and Adolescent Patients with Type 1 Diabetes

Objective: The prevalence of celiac disease (CD) varies between 1% and 10% in patients with type 1 diabetes mellitus (T1DM). This study aimed to determine the frequency of spontaneous recovery of celiac serology and the biopsy-proven CD (BPCD) frequency in patients with T1DM. Methods: The data of 668 patients with available celiac serology tests from a total of 779 patients who were followed for the last 10 years with the diagnosis of T1DM were retrospectively evaluated. Results: Positive serology was detected in 103 out of 668 (15.4%) patients. There was spontaneous normalization in 24 (23.3%), fluctuation in 11 (10.7%) and permanently positive serology in 68 (66%). In 46 out of 53 (86.8%) patients with positive serology and biopsy, CD diagnosis was confirmed by biopsy (BPCD). The frequency of BPCD was 6.9%, and the serology in 76.1% was positive at the time of diagnosis of T1DM. The weight, height and body mass index-standard deviation score at diagnosis were lower in patients with BPCD compared to the group without CD. An anti-tissue transglutaminase-IgA (anti-TTG-IgA) level of 11.8 times the upper limit of normal was the most sensitive (93%) and specific (90%) cut-off for BPCD (area under the curve: 0.95; 95% confidence interval: 0.912-1; p<0.001). Conclusion: In our cohort, the frequency of positive serology for CD was 15.4%, while the rate of BPCD was 6.9%. The majority (97.8%) of cases were diagnosed within the first five years of T1DM. In 23.3% of cases, positive anti-TTG-IgA spontaneously resolved without a gluten-free diet (GFD). Therefore, serological follow-up instead of immediate duodenal biopsy or GFD therapy, particularly for patients with asymptomatic and mild anti-TTG IgA level, is warranted.

Clinical Characteristics of 46,XX Males with Congenital Adrenal Hyperplasia

Objective: To retrospectively evaluate the follow-up data in patients with 46,XX congenital adrenal hyperplasia (CAH) who were raised male. Methods: A national database was created. The data of patients were asked to be recorded in the data form. Results: The median (range) age of diagnosis was three (0.1-18.3) years in 44 patients. Twenty nine cases were diagnosed after the age of two years. Most (95.4%) cases were stage 4-5 virilized. Hysterectomy and bilateral salpingoopherectomy, at a median age of 7.25 (2.4-25.3) years, was performed in 35 cases. Testicular prostheses were placed in 11 (25%) cases at a median age of 11.2 (2.8-17) years. The median final height was 149.2 (132.8-172) cms in 38 patients, including simple virilizing (n=18), salt-wasting (n=6), and 11-beta hydroxylase (n=12). Of the 16 patients above the age of eighteen, university education was completed in 25%. Conclusion: It was seen that most (65.9%) of the 46,XX CAH cases raised male were diagnosed after two years of age. In these cases, hysterectomy and bilateral salpingoopherectomy, genital corrective surgeries and testicular prosthesis operations were performed in a very wide age rage.

Color Doppler ultrasound for surveillance following EVAR as the primary tool.

OBJECTIVE: As aneurysm-related events and rupture is not eliminated, postoperative lifelong surveillance is mandatory after endovascular aneurysm repair (EVAR). For surveillance colored Doppler ultrasound (CDUS) is a standard method of noninvasive evaluation having the advantages of availability, cost-effectiveness, and lack of nephrotoxicity and radiation. We evaluated CDUS for primary surveillance tool after elective EVAR by comparing with computerized tomography. METHODS: Between January 2018 and March 2020, 84 consecutive post-EVAR patients were evaluated. First, CDUS was performed by two Doppler operators from the Radiology Department and then computed tomographic angiography (CTA) was performed. The operators were blind to CTA reports. A reporting protocol was organized for endoleak detection and largest aneurysm diameter. RESULTS: Among 84 patients, there were 11 detected endoleaks (13.1%) with CTA and seven of them was detected with CDUS (r = .884, p < .001). All Type I and III endoleaks were detected perfectly. There is an insufficiency in detecting low flow by CDUS. Eliminating this frailty, there was a strong correlation of aneurysm sac diameter measurement between CTA and CDUS (r = .777, p < .001). The sensitivity and specificity of CDUS was 63.6% and 100%, respectively. The accuracy was 95.2%. Positive and negative predictive values were 100% and 94.8%. Bland-Altman analysis and linear regression analysis showed no proportional bias (mean difference of 1.5 ± 2.2 mm, p = .233). CONCLUSIONS: For surveillance, CDUS promises accurate results without missing any potential complication requiring intervention as Type I or III endoleak. Lack of detecting Type II endoleaks may be negligible as sac enlargement was the key for reintervention in this situation and CDUS has a remarkably high correlation with CTA in sac diameter measurement. CDUS may be a primary surveillance tool for EVAR and CTA will be reserved in case of aneurysm sac enlargement, detection of an endoleak, inadequate CDUS, or in case of unexplained abdominal symptomatology. By this way we not only avoid ionizing radiation and nephrotoxic agents, but also achieve cost saving issue also.

A novel diagnostic tool for the evaluation of hypothalamic-pituitary region and diagnosis of growth hormone deficiency: pons ratio.

Backgrounds Limitations in the evaluation of the pituitary size and changes according to pubertal status make its validity questionable. Recently, in a small-scale study, pons ratio (PR) has been suggested as a more sensitive tool for diagnosis and etiological evaluation of growth hormone deficiency (GHD). The aim of the study is to evaluate the diagnostic value of PR in the diagnosis of GHD. Methods We retrospectively evaluated the pituitary magnetic resonance imaging (MRI) of 133 patients with a diagnosis of GHD. Primary axis (PA) was assigned as a line crossing the mid-sagittal dorsum sella and fourth ventricle. PR was defined as the pons height above the PA divided by total pons height. The PR of patients with GHD was compared to subjects without GHD. Results Study included 133 patients with GHD and 47 controls. In total, 121 (91%) patients had isolated GHD and 12 (9%) patients had multiple pituitary hormone deficiency. The PR of the patient group (mean: 0.32 ± 0.89; range: 0.14-0.63) was significantly higher than controls (mean: 0.26 ± 0.067; range 0.19-0.44) (p: 0.000). The optimal cut-off value of PR for GHD diagnosis was 0.27 (sensitivity 71% specificity 56%). There was a negative correlation between anterior pituitary height (APH)-SDS and PR (p: 0.002; r: -0.27). APH was increased, but PR remained unchanged in pubertal patients (p: 0.089). Conclusions PR measurement is a noninvasive, practical method with a cost-benefit clinical value. As it is not affected by pubertal status, PR is potentially a more sensitive tool for evaluation of pituitary gland in GHD patients compared to APH.

Unusual response to His-refractory atrial premature complex: What is the mechanism?

A 32-year-old woman underwent radiofrequency catheter ablation of narrow QRS tachycardia that was terminated with intravenous adenosine.