Comparison of Pheochromocytoma-Specific Morbidity and Mortality among Adults with Bilateral Pheochromocytomas Undergoing Total Adrenalectomy vs Cortical-Sparing Adrenalectomy

Importance: Large studies investigating long-term outcomes of patients with bilateral pheochromocytomas treated with either total or cortical-sparing adrenalectomies are needed to inform clinical management. Objective: To determine the association of total vs cortical-sparing adrenalectomy with pheochromocytoma-specific mortality, the burden of primary adrenal insufficiency after bilateral adrenalectomy, and the risk of pheochromocytoma recurrence. Design, Setting, and Participants: This cohort study used data from a multicenter consortium-based registry for 625 patients treated for bilateral pheochromocytomas between 1950 and 2018. Data were analyzed from September 1, 2018, to June 1, 2019. Exposures: Total or cortical-sparing adrenalectomy. Main Outcomes and Measures: Primary adrenal insufficiency, recurrent pheochromocytoma, and mortality. Results: Of 625 patients (300 [48%] female) with a median (interquartile range [IQR]) age of 30 (22-40) years at diagnosis, 401 (64%) were diagnosed with synchronous bilateral pheochromocytomas and 224 (36%) were diagnosed with metachronous pheochromocytomas (median [IQR] interval to second adrenalectomy, 6 [1-13] years). In 505 of 526 tested patients (96%), germline mutations were detected in the genes RET (282 patients [54%]), VHL (184 patients [35%]), and other genes (39 patients [7%]). Of 849 adrenalectomies performed in 625 patients, 324 (52%) were planned as cortical sparing and were successful in 248 of 324 patients (76.5%). Primary adrenal insufficiency occurred in all patients treated with total adrenalectomy but only in 23.5% of patients treated with attempted cortical-sparing adrenalectomy. A third of patients with adrenal insufficiency developed complications, such as adrenal crisis or iatrogenic Cushing syndrome. Of 377 patients who became steroid dependent, 67 (18%) developed at least 1 adrenal crisis and 50 (13%) developed iatrogenic Cushing syndrome during median (IQR) follow-up of 8 (3-25) years. Two patients developed recurrent pheochromocytoma in the adrenal bed despite total adrenalectomy. In contrast, 33 patients (13%) treated with successful cortical-sparing adrenalectomy developed another pheochromocytoma within the remnant adrenal after a median (IQR) of 8 (4-13) years, all of which were successfully treated with another surgery. Cortical-sparing surgery was not associated with survival. Overall survival was associated with comorbidities unrelated to pheochromocytoma: of 63 patients who died, only 3 (5%) died of metastatic pheochromocytoma. Conclusions and Relevance: Patients undergoing cortical-sparing adrenalectomy did not demonstrate decreased survival, despite development of recurrent pheochromocytoma in 13%. Cortical-sparing adrenalectomy should be considered in all patients with hereditary pheochromocytoma

Preparation of unsymmetrical ketones from tosylhydrazones and aromatic aldehydes via formyl C–H bond insertion

Preparation of ketones by insertion of diazo compounds into the formyl C−H bond of an aldehyde is an attractive procedure, but use of structurally diverse diazo compounds is hampered by preparation and safety issues. A convenient procedure for the synthesis of unsymmetrical ketones from bench-stable tosylhydrazones and aryl aldehydes is reported. The procedure can be performed in one pot from the parent carbonyl compound and needs only a base, with no additional promoters being required

H6-TYPE IGBT CONFIGURATION FOR SINGLE PHASE GRID CONNECTED TRANSFORMERLESS PHOTOVOLTAIC APPLICATIONS

All active switches is presented for a proposed high-efficiency photovoltaic inverter, no isolated, grid connected photovoltaic applications. The proposed H6-type configuration features high efficiency over a wide load range, low ground leakage current, no need for split capacitors, and low-output ac-current distortion. The detailed SPWM scheme, photovoltaic power supply and the power stage of operating principle are described. In this paper not only IGBT, but also MOSFET switch inverter topology explained. The proposed H6 type transformerless inverter topology can be able to reduce strong ground leakage current. The single phase IGBT H6-type transformerless grid connected PV system is simulated using MATLAB/SIMULINK

H6-type Igbt Configuration for Single Phase Grid Connected Transformerless Photovoltaic Applications

All active switches is presented for a proposed high-efficiency photovoltaic inverter, no isolated, grid connected photovoltaic applications. The proposed H6-type configuration features high efficiency over a wide load range, low ground leakage current, no need for split capacitors, and low-output ac-current distortion. The detailed SPWM scheme, photovoltaic power supply and the power stage of operating principle are described. In this paper not only IGBT, but also MOSFET switch inverter topology explained. The proposed H6 type transformerless inverter topology can be able to reduce strong ground leakage current. The single phase IGBT H6-type transformerless grid connected PV system is simulated using MATLAB/SIMULINK

Characterization of adult onset growth hormone deficiency syndrome in patients with hypothalamopituitary diseases: Asian Indian data

<b>Background:</b> Hardly any data is available on Adult onset growth hormone deficiency (AOGHD) in Patients with hypothalamopituitary diseases in India. <b>Aims:</b> To characterize Asian Indian AOGHD syndrome in hypothalamopituitary diseases. <b>Settings and Design:</b> Cross-sectional analysis of data from a tertiary care hospital. <b>Materials and Methods:</b> Thirty patients with AOGHD were compared with 30 age-, sex-, body mass index-matched controls with respect to endocrine evaluation, biochemistry, body composition (BC), bone mineral density (BMD), cardiovascular risk profile and quality of life (QoL). <b>Statistical Analysis Used:</b> Comparisons were performed using two-tailed Student&#x2032;s test (SPSS Software version 10.0). <b>Results:</b> Most of the patients had abnormal BC with central obesity [Truncal FM (&#x0025;): males {33.9&#x00B1;4.4 (patient) vs. 29.31&#x00B1;6.2 (control); <i> P</i> -0.027}; females {39.87&#x00B1;5.93 (patient) vs. 35.76&#x00B1;3.16 (control); <i> P</i> - 0.025}] and poor QoL. Patients aged over 45 years did not show low bone mass or lipid abnormalities as compared to controls. Low BMD and abnormal lipid profile {Triglycerides [mg/dl]:170.55&#x00B1;72.5 (patient) vs101.24&#x00B1;31.0 (control); <i> P</i> -0.038}; {very low density lipoprotein cholesterol [mg/dl]: 33.54&#x00B1;14.9 (patient) vs. 20.25&#x00B1;6.18 (control); <i> P</i> - 0.05} was seen in female patients less than 45 years of age. <b>Conclusions:</b> Male and female (more than 45 years) AOGHD patients have increased cardiovascular risk factors and poor QoL while BMD is unaffected. Females less than 45 years of age have the major characteristics of AOGHD and would be the group to benefit maximally with recombinant human Growth Hormone treatment, which is similar to that in the western literature

Accuracy of 3D Printed Model Acquired from Different Types of Intra Oral Scanners and 3D Printers

ObjectiveThe aim of this study was to verify the influence of different types of intraoral scanners and 3D printers on the accuracy of printed models in comparison to plaster models obtained from conventional impressions. Material and MethodsA dental study model was used as the reference model and was molded with polyvinyl siloxane to produce the plaster models which were scanned by a reference scanner. Two types of intraoral scanners and digital files were printed by two types of 3D printers. To measure the accuracy (trueness and precision) amongst the groups, the datasets were superimposed via a best-fit alignment method utilizing a 3D analysis program (Geomagic Verify; 3D Systems). The trueness of the complete arch was evaluated by superimposing the STL file data of the reference model with STL file data obtained from other scanners. The precision of the complete arch was evaluated by superimposing the scan data within each group. The quantitative values were automatically calculated by the 3D analysis program based on the root mean square (RMS). ResultsIt was observed that all the tested combinations of the scanner and 3D printer showed variation from reference which was nonsignificant. However, Trios 4 intraoral scanner and Formlabs 3D printer was the combination that showed the best trueness and precision values. ConclusionsIt was concluded that the accuracy of printed and plaster models was impaired due to the trueness of the models. The type of printer influenced the accuracy of the printed models, while the type of scanner did not. The standardization of the method of obtaining printed models must be carried out to provide the production of quality models. However, there will be differences between the technologies

The value of an acute octreotide suppression test in predicting response to long-term somatostatin analogue therapy in patients with acromegaly

Context: The usefulness of the acute octreotide test in the selection of patients with acromegaly for chronic somatostatin depot analogues treatment is controversial. Aims: To determine the efficacy of acute octreotide suppression test (OST) in predicting response to long-term somatostatin analogue (Octreotide-long-acting repeatable, OCT-LAR) therapy in patients with acromegaly. Settings and Design: Prospective study (2006-2007) conducted at a tertiary healthcare centre in western India. Materials and Methods: Sixteen drug-naive patients with active acromegaly (postoperative±post radiotherapy) underwent 50 µg subcutaneous OST. Ten patients were treated with OCT-LAR for one year. Remission was defined as a nadir growth hormone (GH) < 1 ng/ml during 75 g oral glucose tolerance test (OGTT) (0, 10, 30, 60, 120, 180 min) and normal age, sex-matched insulin-like growth factor 1 (IGF1) levels. Statistical Analysis: SPSS Software Version 11 was used for data analysis. Results: Using GH cutoff < 1 ng/ml, four patients (40%) achieved control at 12 months, while five patients (50%) achieved normal IGF1 values. The mean basal GH levels in 10 responsive, four controlled and six uncontrolled patients were 34.7±61.14, 4.5±1.3 and 54.8±74.2 ng/ml respectively which suppressed to mean nadir GH of 3.75±4.03, 0.66±0.15 and 5.8±4 ng/ml respectively. Sensitivity, specificity, negative and positive predictive value for nadir GH < 1 ng/ml reached after an OST was 100% each in predicting remission in our cohort. Odds for control increased if the baseline GH was low (< 5 ng/ml in our cases). Conclusions: Nadir GH < 1 ng/ml following an OST is a useful predictive marker of achieving disease remission with long-term OCT-LAR therapy