IDEA Group Consensus Statement on Medical Management of Adult Gender Incongruent Individuals Seeking Gender Affirmation as Male.

Gender-affirming hormone therapy (GAHT) is the most frequent treatment offered to gender-incongruent individuals, which reduces dysphoria. The goal of therapy among gender-incongruent individuals seeking gender affirmation as male is to change their secondary sex characteristics to affect masculine physical appearances. GAHT greatly improves mental health and quality of life among gender incongruent individuals. India-specific guideline for appropriate care for gender-incongruent individuals is almost absent. This document is intended to assist endocrinologists and other healthcare professionals interested in gender incongruity for individuals seeking gender affirmation as male. A safe and effective GAHT regimen aims to effect masculinising physical features without adverse effects. In this document, we offer suggestions based on an in-depth review of national and international guidelines, recently available evidence and collegial meetings with expert Indian clinicians working in this field. Clinicians represented in our expert panel have developed expertise due to the volume of gender incongruent individuals they manage. This consensus statement provides protocols for the hormone prescribing physicians relating to diagnosis, baseline evaluation and counselling, prescription planning for masculinising hormone therapy, choice of therapy, targets for monitoring masculinising hormone therapy, clinical and biochemical monitoring, recommending sex affirmation surgery and peri-operative hormone therapy. The recommendations made in this document are not rigid guidelines, and the hormone-prescribing physicians are encouraged to modify the suggested protocol to address emerging issues.

Hypophysitis - A Review of Fourteen Cases.

Background: Primary hypophysitis is a rare condition, usually diagnosed retrospectively after surgery for a suspected pituitary adenoma. Improved awareness of the condition and better imaging have resulted in more patients being diagnosed without surgery. Objective: A retrospective chart analysis study of hypophysitis from a single secondary endocrine and neurosurgical referral center in eastern India was conducted between 1999 and 2021 to assess the diagnostic and therapeutic challenges posed by these patients. Methods and Material: Fourteen patients presented to the center between 1999 and 2021. All patients had an MRI of the head with contrast and a full clinical workup. Twelve patients had headache, of whom one had progressive visual impairment. One patient had severe weakness, attributed later to hypoadrenalism and one had sixth nerve palsy. Results: Six patients had glucocorticoids as their primary treatment, four refused treatment, and one was on glucocorticoid replacement. One patient had decompressive surgery because of progressive visual loss, and two had surgery because of a presumptive diagnosis of pituitary adenoma. There was no difference between the patients who had glucocorticoids and those who did not. Conclusions: Our data suggest that it is possible to identify most patients with hypophysitis on clinical and radiological grounds. In the largest published series on this subject and in ours, glucocorticoid treatment did not alter the outcome.

Non-conventional Genetic Basis of Congenital Adrenal Hypoplasia in South Asia.

Congenital adrenal hypoplasia or adrenal hypoplasia congenita (AHC) is a rare disorder ascribed to mutations in three genes, namely, the dosage-sensitive sex reversal-adrenal hypoplasia congenita critical region on the X chromosome, gene 1 (DAX-1/NROB1 gene), steroidogenic factor-1 gene (SF-1/NR5A1 gene), and Achalasia-Addisonianism-Alacrima syndrome gene (AAAS gene). Five cases of AHC of local South Asian origin are described here. Golden Helix VarSeq 2.2.0 (Golden Helix Inc., Bozeman, MT, United States), the clinical genomics interpretation and reporting platform, was used for genetic study. No subject had congenital adrenal hyperplasia (CAH). Four male neonates presented with hypoglycemia, and one older female child presented with hyperpigmentation. This girl had a recognized mutation in the AAAS gene, while none of the four male neonates had any of the recognized mutations associated with AHC. Further, none were salt-losing, which is the conventional Western phenotype. Thus, additional, yet unknown, gene(s) must be operative in AHC among South Asian subsets.

Clinical Profile of Long-Term Survivors and Nonsurvivors with Type 1 Diabetes in India.

Objective: To compare the clinical profile of long-term survivors and nonsurvivors with type 1 diabetes (T1D) in India. Research Design and Methods: This is a retrospective study of 76 individuals with T1D who had survived for at least 40 years ("survivors") and 51 individuals with T1D who had died with shorter duration of diabetes ("non-survivors"), from diabetes clinics in different cities of India. Prevalence of complications in both groups and causes of death of the nonsurvivors were analyzed. Retinopathy was diagnosed by retinal photography; chronic kidney disease (CKD) by urinary albumin excretion (micro-or macroalbuminuria) and estimated glomerular filtration rate; peripheral vascular disease (PVD) by doppler measurement of ankle-brachial pressure index; coronary artery disease (CAD) based on history of myocardial infarction or coronary revascularization, and neuropathy by biothesiometry. Results: Mean glycated hemoglobin (8.4% ± 1.5% vs. 10.7% ± 2.2%, P < 0.001), serum low-density lipoprotein cholesterol (91 ± 29 mg/dL vs. 107 ± 22 mg/dL, P = 0.004), and systolic blood pressure (135 ± 16 mmHg vs. 153 ± 37 mmHg, P = 0.003) were lower, and high-density lipoprotein cholesterol (51 ± 11 mg/dL vs. 43 ± 15 mg/dL, P = 0.002) higher, among survivors compared to nonsurvivors. Diabetic retinopathy, CKD, neuropathy, PVD, and CAD were more frequent among nonsurvivors. CAD [25.5%] and renal failure [23.5%] were the most frequent causes of death. Conclusions: In this first report of long-term survivors with T1D from India, we report that survivors had better glycemic and blood pressure control, more favorable lipid profiles and lower prevalence of complications compared to nonsurvivors. However, there could be other protective factors as well, which merit further studies.

Exploring Cross-Sectional Predictors of Suicide Ideation, Attempt, and Risk in Gender Incongruent Adults in India.

INTRODUCTION: Suicide rates and suicidal tendencies among gender incongruent persons are higher compared to the general population. Yet little is known about the factors that are relevant for suicide-related outcomes among Indian gender incongruent individuals. MATERIALS AND METHODS: Within a large sample of gender incongruent adults (n=120), we examined the contribution of demographic (age, assigned sex, gender identity, relationship, and addiction status) and socio-economic variables (education, profession, income, social support) in the prediction of three suicide-related outcomes: past-year suicidal thought, history of suicidal attempt and a composite measure of the two. RESULTS: Of the entire sample, 25.8% (n=31) reported a past suicide attempt, with 18.3% (n = 22) reporting one attempt, 2.5% (n = 3) reporting two attempts, 1.7% (n = 2) reporting three attempts and 2.5% (n=3) reporting four to six attempts. The age at which the first suicide attempt occurs is mostly between 16 to 18 years. 19.26% (n = 21) reported that although they had not attempted suicide, they had given serious thought to killing themselves in the last year. A Chi-square test was conducted to ascertain each demographic variable and socio-economic marker. However, none of these proposed predictors correlated with suicide-related outcomes in our cohort. CONCLUSIONS: The gender incongruent community is highly susceptible to suicidal behavior. Gender identity may be the risk factor for that behavior. Further study with larger population needs to identify other relevant risk factors, including gender-related victimization and mental health conditions as risk factors.

Impact of oral anticholinergic on insulin response to oral glucose load in patients with impaired glucose tolerance.

BACKGROUND: Preliminary data indicates there is a cholinergic basis to insulin secretion. AIMS & OBJECTIVE: To investigate the impact of oral anticholinergics on insulin secretion in subjects with impaired glucose tolerance (IGT), in comparison with volunteers having normal glucose tolerance (NGT). Material & Methods: This prospective observational study recruited 10 IGT and 10 NGT subjects. An oral glucose tolerance test (OGTT) was conducted twice in the absence and presence of hyoscine butyl-bromide (HBB). The plasma glucose (PG) and insulin levels were serially estimated at 30-min increments for 2 h after the OGTT. Early (ΔI30/ΔPG30) & late (insulin/PGAUC 60-120) phase insulin activity were assessed subsequently. RESULTS: The study constituted of 10 IGT (4M/6F, BMI: 28.80 ± 2.30) and 10 NGT (5M/5F, BMI: 23.00 ± 0.80) subjects. In the NGT group, the pre-HBB mean glucose levels (0-120 min) were comparable with those recorded after HBB intake. However, after HBBB, the mean insulin levels decreased significantly at t = 90 and 120min, confirmed by attenuated late phase insulin activity in IGT (P = 0.023) & NGT (P = 0.006) group. On the other hand, in the IGT group, however, HBB did not impact on the mean PG and insulin levels (0-120 min). CONCLUSIONS: Our study findings indicate that insulin secretion is influenced by cholinergic system and that oral anticholinergics may attenuate the late phase insulin activity in varying degrees of glycemic status.

Excellent Outcome of Protocol-Based Treatment of Motivated Type 2 Diabetes Mellitus (T2DM) Patients: A Real World Follow-Up for 11 Years.

Introduction and aim To evaluate the real-world clinical outcome of guideline-based treatment among adherent and committed type 2 diabetes mellitus (T2DM) patients. Methods The study reports the outcomes of an 11-year clinic-based standard care regime, based on the American Diabetes Association (ADA) guidelines and implemented in the authors' practices. Records of 145 T2DM patients, who were regularly followed up, were reviewed. Descriptive and inferential statistical analysis was carried out with the Statistical Analysis System (SAS) (SAS Institute Inc., Cary, NC, USA) and with Statistical Package for Social Sciences (SPSS) (IBM Corp., Armonk, NY, USA), with Microsoft Word and Excel to generate graphs and tables. Results Apart from a significant increase of body weight (but not of body mass index, BMI) and a significant decrease of diastolic blood pressure (DBP), there were insignificant changes in all major biochemical parameters, fasting plasma glucose (FPG), postprandial plasma glucose (PPG), glycated hemoglobin (HbA1c), low-density lipoprotein cholesterol (LDL-C), creatinine, estimated glomerular filtration rate (eGFR) and urine albumin creatinine ratio (ACR), over the 11 years of follow-up. Conclusion ADA guideline-based management effectively maintained treatment goals among treatment adherent and committed T2DM patients over 11 years. Glycemic parameters (FPG, PPG, and HbA1c) and renal parameters (serum creatinine, eGFR, and ACR levels) remained stable. Our outcomes data were better than those recorded in the landmark United Kingdom Prospective Diabetes Study (UKPDS) and Action in Diabetes and Vascular Disease: Preterax and Diamicron Modified Release Controlled Evaluation (ADVANCE).

In the Spotlight: Role of Gla-100 in the Glycemic Management of Hospitalized people with diabetes.

Both hyperglycemia and hypoglycemia in hospitalized patients represent a major concern as they are associated with adverse outcomes-including increased rates of infection, longer hospital stay, and even death. Insulin therapy is the mainstay in the management of inpatient hyperglycemia. The traditional approach of sliding scale insulin (SSI) therapy for the temporary management of blood glucose levels in hospitalized patients, has now given way to basal-bolus insulin (BBI) therapy. This is owing to the BBI affording a better glycemic control in non-critical hospital settings as observed in multiple clinical studies using insulin glargine 100 U/mL (Gla-100) as the basal component. Furthermore, a string of clinical studies has also attested to Gla-100 being used effectively even in patients on corticosteroids, enteral or parenteral nutrition, and in perioperative settings. Hence, overall, the existing evidence would point to the growing role of BBI regimens centering around basal insulin like Gla-100 as an effective option with low safety concerns for insulin therapy in both hospitalized and out-patient settings in the treatment of patients with type 2 diabetes mellitus (T2DM).

IDEA Group Consensus Statement on Medical Management of Adult Gender Incongruent Individuals Seeking Gender Reaffirmation as Female.

Cross sex hormone therapy (CSHT) is a strongly desired medical intervention for gender incongruent individuals. The goal is to change secondary sex characteristics to facilitate gender presentation that is consistent with the desired sex. When appropriately prescribed CSHT can greatly improve mental health and quality of life for gender incongruent individuals. Appropriate care for gender incongruent individuals in India is almost absent due to lack of country specific guideline and lack of training amongst the medical professionals. This document is intended to assist endocrinologists and physicians whose adult gender incongruent client is seeking gender reaffirmation as female (transfeminine). These individuals require a safe and effective CSHT regimen that will suppress endogenous male hormone secretion and maintain physiologic levels of female sex hormone. In this document, we offer suggestions based on an in-depth review of Guidelines of Endocrine Society, The World Professional Association for Transgender Health guidelines, the Sappho Good Practice Guide of India and collegial meetings with expert Indian clinicians working in this field. Clinicians represented in our expert panel are not gender specialists by training but have developed expertise due to the volume of gender incongruent individuals they manage. This consensus statement on medical management provides protocols for the prescribing clinician relating to diagnosis, baseline evaluation and counselling, prescription planning for feminizing hormone therapy and anti-androgen therapy, targets for monitoring hormone therapy, choice of therapy, clinical and biochemical monitoring, recommending sex reaffirmation surgery and peri-operative hormone therapy. The recommendations made in this document should not be perceived as a rigid set of guidelines and the treating clinicians are encouraged to modify our suggested protocols to address emerging issues.

Lives of Gender Incongruent Community: An Indian Subset Chants "All is Well".

CONTEXT: Gender incongruent individuals are exposed to unique stressors as a result of their minority social position. Poor social support has a further adverse impact on the lives and wellbeing of gender incongruent individuals. There is a paucity of scientific data from India on the socioeconomic status (SES) of gender incongruent community. AIMS: Aim of the study is to understand and estimate the social support, wellbeing, and SES of gender incongruent individuals in Eastern India. SUBJECTS AND METHODS: Data of 120 gender incongruent patients from the endocrinology outpatient department of a tertiary care hospital in eastern India were collected. We looked at demographic characteristics, social support, underlying psychiatric comorbidities, and SES. SES was calculated by the Kuppuswamy's socioeconomic status (KSS) scale based on occupation, education, and income. STATISTICAL ANALYSIS USED: Microsoft Word and Excel were used to generate tables. RESULTS: Most of the gender incongruent individuals were transfeminine. Almost half of them had no history of addiction. Most of them had good support from family and friends and very few (only 3%) had mental health problems. Calculation by KSS scale showed most of the study population lay in the upper middle or lower middle socioeconomic class. CONCLUSIONS: Strong support from friends and family appears a key factor for protection against psychiatric comorbidities and an all-round impact on the lives and wellbeing of the study population.

Consensus Recommendations on GLP-1 RA Use in the Management of Type 2 Diabetes Mellitus: South Asian Task Force.

The advent of incretin mimetics such as glucagon-like peptide-1 receptor agonists (GLP-1 RAs) has enriched the armamentarium for diabetes management owing to their glycaemic as well as extra-glycaemic benefits. The approval status and availability of this class of drugs vary widely across the globe. Being a relatively newer class of drug with numerous benefits, several national and international guidelines are working towards addressing clinical questions pertaining to the optimal use of GLP-1 RAs for the management of diabetes. Although the newer class of drugs are associated with significant benefits such as patient-centric approach, these drugs demand the providers to be vigilant and knowledgeable about the medication. The South Asian population is at higher risk of type 2 diabetes mellitus (T2DM) because of their genetic predisposition and lifestyle changes. Hence, prevention and management of T2DM and its associated complications in this population are of paramount importance. The current report aims to present an overview of current knowledge on GLP-1 RAs based on pragmatic review of the available clinical evidence. In addition, this report is a consensus of expert endocrinologists representing South Asian countries including India, Pakistan, Bangladesh, Nepal, Sri Lanka, Afghanistan and the Maldives on essential recommendations related to the use of GLP-1 RAs in a real-world scenario.

Advanced Diabetic Nephropathy with "Clean" Eyes: An Extreme Phenotype.

INTRODUCTION: It is generally accepted that renal and eye changes in diabetes are concordant. There are however a fair number of patients with diabetes who have end-stage renal disease (ESRD) without any of the typical eye changes. The present study highlights the discordance between retinopathy and nephropathy and describes a series of patients of long-standing diabetes undergoing renal transplant who had little or no evidence of retinopathy. METHODS: All patients with ESRD undergoing renal transplants underwent comprehensive fundus evaluation including dilated indirect ophthalmoscopy, slit lamp biomicroscopy, and fundus photography. The patients' age, gender, physical parameters (body mass index and blood pressure), duration of diabetes, glycosylated hemoglobin (HbA1c), albumin creatinine ratio, and presence of diabetic peripheral neuropathy (DPN) were determined. Renal histopathology was reviewed, if available. RESULTS: Five patients with diabetic nephropathy (DN) underwent renal transplant and had no evidence of diabetic retinopathy (DR) or up to two microaneurysms per fundus. All the patients were between 50 and 65 (mean ± standard deviation - 58.6 ± 4.67) years of age. The mean duration of diabetes was 16 ± 2.91 years. All had poor glycemic control with a mean HbA1c of 9.2 ± 0.837%. All had hypertension, macroalbuminuria, and DPN. CONCLUSION: There is a well-recognized association between retinopathy and nephropathy, in which nephropathy without retinopathy is rare but retinopathy without nephropathy is common. We have identified a subset of patients with kidney disease of sufficient severity to warrant renal transplant but who are protected from retinopathy. It is possible that there is an extreme phenotype of DN patients with unaffected eyes who carry genes protecting against DR.

Centralised versus local measurement of glycated haemoglobin in clinical trial settings: a comment on Arch et al., Trials. 2016.

Arch and colleagues in their 24 October 2016 paper in Trials focus on the issue of centralised versus local measurement of glycated haemoglobin (HbA1c) in clinical trial settings. Resolution of the debate is important: while local HbA1c measurement is less costly, and would thereby ease the stretched funding situations for clinical trials worldwide, it cannot be implemented at the expense of clinically unacceptable disparities between centralised and localised measurements. Arch and colleagues favour centralised measurement in their paper's conclusion. However, critical questions regarding the methods require a closer look. In this letter, we discuss some of the issues that the authors could clarify in order that the reader can agree (or disagree) to their inference with greater confidence.

Thyroid profile and autoantibodies in Type 1 diabetes subjects: A perspective from Eastern India.

CONTEXT: There has been a rise in the incidence of type 1 diabetes mellitus (T1DM) in India. The prevalence of thyroid autoantibodies and thyroid dysfunction is common in T1DM. AIMS: The aim of this study is to determine the incidence of thyroid dysfunction and thyroid autoantibodies in T1DM subjects, without any history of thyroid disease, and the prevalence of glutamic acid decarboxylase (GAD) antibody, Islet antigen-2 antibody (IA2), thyroid peroxidase (TPO), and thyroglobulin autoantibodies (Tg-AB) in T1DM subjects. SETTINGS AND DESIGN: This was a cross-sectional clinical-based study. SUBJECTS AND METHODS: Fifty subjects (29 males, 31 females) with T1DM and without any history of thyroid dysfunction were included in the study. All subjects were tested for GAD antibody, IA2 antibody, TPO antibody, thyroglobulin antibody, free thyroxine, and thyroid-stimulating hormone. STATISTICAL ANALYSIS USED: A Chi-square/pooled Chi-square test was used to assess the trends in the prevalence of hypothyroidism. A two-tailed P < 0.05 was considered statistically significant. RESULTS: The mean age of the subjects was 23.50 years. 9.8% of subjects were below the age of 12 years, 27.45% of subjects were of age 12-18 years, 37.25% of subjects were of age 19-30 years, and 25.49% of subjects were above 30 years. 78% were positive autoantibody for GAD, 30% for IA-2, 24% for TPO, and 16% were positive for Tg-AB. A total of 6.0% of T1DM subjects had evidence of clinical hypothyroidism, but the prevalence of subclinical hyperthyroidism (SCH) varied from 32% to 68.0% for we considered different definitions of SCH as advocated by different guidelines. All subjects with overt hypothyroidism had positive GAD and thyroid autoantibodies. One (2%) subject had clinical hyperthyroidism with strongly positive GAD, TPO, and Tg-AB. CONCLUSIONS: We found a high prevalence of GAD, IA2, TPO, and Tg-AB in our T1DM subjects. A substantial proportion of our subjects had undiagnosed thyroid dysfunction with a preponderance of subclinical hypothyroidism. All T1DM subjects with overt hypothyroidism or hyperthyroidism had positive GAD and thyroid autoantibodies. The high prevalence of undiagnosed thyroid dysfunction highlights the importance of regular thyroid screening in T1DM subjects.

Effect of pioglitazone on nerve conduction velocity of the median nerve in the carpal tunnel in type 2 diabetes patients.

AIM: To evaluate the impact of pioglitazone pharmacotherapy in median nerve electrophysiology in the carpal tunnel among type 2 diabetes patients. METHODS: The study was executed in patients with type 2 diabetes, treated with oral drugs, categorized under pioglitazone or non-pioglitazone group (14 in each group), and who received electrophysiological evaluation by nerve conduction velocity at baseline and 3 mo. RESULTS: At 3 mo, pioglitazone-category had inferior amplitude in sensory median nerve [8.5 interquartile range (IQR) = 6.5 to 11.5) vs non-pioglitazone 14.5 (IQR 10.5 to 18.75)] (P = 0.002). Non-pioglitazone category displayed amelioration in amplitude in the sensory median nerve [baseline 13 (IQR = 9 to 16.25) vs 3 mo 8.5 (IQR = 6.5 to 11.5)] (P = 0.01) and amplitude in motor median nerve [baseline 9 (IQR = 4.75 to 11) vs 3 mo 6.75 (IQR = 4.75 to 10.25)] (P = 0.049); and deterioration of terminal latency of in motor ulnar nerve [baseline 2.07 (IQR = 1.92 to 2.25) vs 3 mo 2.16 (IQR = 1.97 to 2.325)] (P = 0.043). There was amelioration of terminal latency in sensory ulnar nerve [baseline 2.45 (IQR = 2.315 to 2.88) vs 3 mo 2.37 (IQR = 2.275 to 2.445) for pioglitazone group (P = 0.038). CONCLUSION: Treatment with pioglitazone accentuates probability of compressive neuropathy. In spite of comparable glycemic control over 3 mo, patients treated with pioglitazone showed superior electrophysiological parameters for the ulnar nerve. Pioglitazone has favourable outcome in nerve electrophysiology which was repealed when the nerve was subjected to compressive neuropathy.

Control of evanescent field using a dynamic waveguide composed of gelatin-coated few-layer fiber.

We report here the results of our studies on dynamic refractive-index (RI) profile few-layer fibers in view of controlling the mode-field profile, in particular the evanescent tails under varying structural configuration. We experimentally fabricate dynamic RI profile few-layer fibers using thin gelatin coating on selectively etched fibers and illustrate how the excitation of various modes and the evanescent field at the interface can be controlled with changing humidity parameter. As a technology outcome of this research, we demonstrate through an optimized structural configuration a well performing fiber-optic high (70%-98%) relative humidity (RH) sensor with sensitivity as high as -1.07 dBm/%RH.

Single all-optical platform for measurement of twist and transverse stress using polarization modulation in distinct dual-mode fiber placed in a Sagnac loop.

We report here the experimental demonstration of measurement of both twist and transverse stress using polarization modulation in a single all-fiber circuit consisting of a single-mode fiber (SMF)/dual-mode fiber (DMF) in a Sagnac interferometer (SI) loop. The SMF-SI prototype setup is seen to be suitable for precise measurement of twist over a broad range of ±50° and transverse stress up to 5 N with a sensitivity as high as 2.85×10(6) pW/° and 2.08×10(7) pW/N, respectively. It is envisaged that nearly ideal operation for twist measurement can be achieved by appropriately selecting the operating domain (pretwisted Sagnac loop for practical realization of the device) and required magnitude of applied transverse stress (weight yielding maximum sensitivity). Unlike SMF-SI, a DMF assisted SI exhibits asymmetric transmittance yielding a peak shift (∼45°) in addition to falling/rising peak amplitude of effective power(∼20 µW). This key characteristic is further utilized for tunable measurement of torsion (unidirectional from -70° to 40°) while keeping the sensitivity fixed. This research problem is then analyzed on the avenue of theoretical consideration and using classical polarization optics; we have derived the Jones birefringence matrix that accurately describes the transmission behavior of the configured fiber circuit (SMF-SI and DMF-SI) for each of the three cases, namely, transverse stress, twist, and both twist and transverse stress. Series of experimental measurements for various conditions of induced birefringence (linear/circular) were performed at length, and the results were compared with those determined theoretically towards configuring a twist and stress measuring device. The study provides an understanding of the underlying physics of dual-mode interference in a Sagnac configuration experiencing linear and circular birefringence.

Copper coordinated ligand thioether-S and NO2(-) oxidation: relevance to the CuM site of hydroxylases.

In order to gain insight into the coordination site and oxidative activity of the CuM site of hydroxylases such as peptidylglycine α-hydroxylating monooxygenase (PHM), dopamine ß-monooxygenase (DßM), and tyramine ß-monooxygenase (TßM), we have synthesized, characterized and studied the oxidation chemistry of copper complexes chelated by tridentate N2Sthioether, N2Osulfoxide or N2Osulfone donor sets. The ligands are those of N-2-methylthiophenyl-2'-pyridinecarboxamide (HL1), and the oxidized variants, N-2-methylsulfenatophenyl-2'-pyridinecarboxamide (HL1(SO)), and N-2-methylsulfinatophenyl-2'-pyridinecarboxamide (HL1(SO2)). Our studies afforded the complexes [(L1)Cu(II)(H2O)](ClO4)·H2O (1·H2O), {[(L1(SO))Cu(II)(CH3CN)](ClO4)}n (2), [(L1)Cu(II)(ONO)] (3), [(L1(SO))Cu(II)(ONO)]n (4), [(L1)Cu(II)(NO3)]n (5), [(L1(SO))Cu(II)(NO3)]n (6) and [(L1(SO2))Cu(II)(NO3)] (7). Complexes 1 and 3 were described in a previous publication (Inorg. Chem., 2013, 52, 11084). The X-ray crystal structures revealed either distorted octahedral (in 2, 4-6) or square-pyramidal (in 1, 3) coordination geometry around Cu(II) ions of the complexes. In the presence of H2O2, conversion of 1→2, 3-5→6 and 6→7 occurs quantitatively via oxidation of thioether-S and/or Cu(ii) coordinated NO2(-) ions. Thioether-S oxidation of L1 also occurs when [L1](-) is reacted with [Cu(I)(CH3CN)4](ClO4) in DMF under O2, albeit low in yield (20%). Oxidations of thioether-S and NO2(-) were monitored by UV-Vis spectroscopy. Recovery of the sulfur oxidized ligands from their metal complexes allowed for their characterization by elemental analysis, (1)H NMR, FTIR and mass spectrometry.

Nifedipine in Congenital Hyperinsulinism - A Case Report.

Congenital hyperinsulinism (CHI) is the commonest cause of persistent hypoglycemia in neonates. Diazoxide is the first-line drug in its treatment, but the more severe cases are usually diazoxide-resistant. Recessive ABCC8 and KCNJ11 mutations are responsible for most (82%) of the severe diazoxide-unresponsive CHI. Oral nifedipine has been effective in isolated cases of CHI. Successful treatment of diazoxide-unresponsive CHI with a combination of octreotide and nifedipine has been reported in a single isolated case so far. We report here a case of diazoxide-resistant CHI due to homozygous ABCC8 nonsense mutation. In this case, hypoglycaemia uncontrolled by pancreatectomy and octreotide alone showed a good response to a combination of nifedipine and octreotide. Octreotide was tapered off by one year age and thereafter the child is euglycaemic on oral nifedipine alone. Continuous glucose monitoring sensor was used as an aid to monitor glycaemic control and was found to be a safe and reliable option reducing the number of needle-pricks in small children.