Unusual presentation of Sjogren's syndrome.

Sjogren's syndrome is a known cause of renal tubular acidosis (RTA). However, osteomalacia associated with Sjogren's syndrome is rare and seldom reported in literature. We report a case of pseudofractures of both femora due to osteomalacia as a result of RTA secondary to Sjogren's syndrome, which was initially misdiagnosed as a stress fracture. A man in his 30s presented with hip pain and was initially misdiagnosed to have stress fractures because of the 'through and through' extension of the 'fracture' lines at the neck of both femora. The patient had a normal serum biochemistry profile except for elevated alkaline phosphatase levels. On further evaluation, he was found to have distal RTA secondary to Sjogren's syndrome. The patient responded to sodium bicarbonate therapy with clinical, biochemical and radiological improvement. A high index of suspicion for RTA should be kept in a patient with osteomalacia with a normal calcium profile and vitamin D level.

Vibrationally-resolved RIXS reveals OH-group formation in oxygen redox active Li-ion battery cathodes.

Vibrationally-resolved resonant inelastic X-ray scattering (VR-RIXS) at the O K-edge is emerging as a powerful tool for identifying embedded molecules in lithium-ion battery cathodes. Here, we investigate two known oxygen redox-active cathode materials: the commercial LixNi0.90Co0.05Al0.05O2 (NCA) used in electric vehicles and the high-capacity cathode material Li1.2Ni0.13Co0.13Mn0.54O2 (LRNMC) for next-generation Li-ion batteries. We report the detection of a novel vibrational RIXS signature for Li-ion battery cathodes appearing in the O K pre-peak above 533 eV that we attribute to OH-groups. We discuss likely locations and pathways for OH-group formation and accumulation throughout the active cathode material. Initial-cycle behaviour for LRNMC shows that OH-signal strength correlates with the cathodes state of charge, though reversibility is incomplete. The OH-group RIXS signal strength in long-term cycled NCA is retained. Thus, VR-RIXS offers a path for gaining new insights to oxygen reactions in battery materials.

Electronic Structure and Surface Chemistry of BaZrS3 Perovskite Powder and Sputtered Thin Film.

Chalcogenide perovskites exhibit optoelectronic properties that position them as potential materials in the field of photovoltaics. We report a detailed investigation into the electronic structure and chemical properties of polycrystalline BaZrS3 perovskite powder by X-ray photoelectron spectroscopy, complemented by an analysis of its long- and short-range geometric structures using X-ray diffraction and X-ray absorption spectroscopy. The results obtained for the powdered BaZrS3 are compared to similar measurements on a sputtered polycrystalline BaZrS3 thin film prepared through rapid thermal processing. While bulk characterization confirms the good quality of the powder, depth-profiling achieved by photoelectron spectroscopy utilizing Al Kα (1.487 keV) and Ga Kα (9.25 keV) radiations shows that, regardless of the fabrication method, the oxidation effects extend beyond 10 nm from the sample surface, with zirconium oxides specifically distributing deeper than the oxidized sulfur species. A hard X-ray photoelectron spectroscopy study on the powder and thin film detects signals with minimal contamination contributions and allows for the determination of the valence band maximum position with respect to the Fermi level. Based on these measurements, we establish a correlation between the experimental valence band spectra and the theoretical density of states derived from density functional theory calculations, thereby discerning the orbital constituents involved. Our analysis provides an improved understanding of the electronic structure of BaZrS3 developed through different synthesis protocols by linking it to material geometry, surface chemistry, and the nature of doping. This methodology can thus be adapted for describing electronic structures of chalcogenide perovskite semiconductors in general, a knowledge that is significant for interface engineering and, consequently, for device integration.

Hypogonadotropic hypogonadism with Zinner syndrome: a coincidence or a consequence?

This case report describes a man in his 20s presenting with bilateral crypto-orchidism, micropenis and underdeveloped secondary sexual characteristics. The patient also exhibited hyposmia, eunuchoid stature and gynecomastia. Biochemical investigations revealed low levels of testosterone, luteinising hormone and follicle-stimulating hormone. Hence, he was diagnosed with Kallmann syndrome. Imaging studies showed an absent right kidney and cystic dilatation of the distal ureteric bud, seminal vesicle and absent/hypoplastic ejaculatory duct. The association of hypogonadotropic hypogonadism with Zinner syndrome, a rare condition characterised by renal agenesis, seminal vesicle cyst and ejaculatory duct obstruction, was noted.

Impact of short-term application of continuous glucose monitoring system(CGMS) on long-term glycemic profile in adolescents and adults with type 1 diabetes mellitus: An open-label randomized control cross over study.

AIMS: The use of Continuous Glucose Monitoring System (CGMS) improves glycemic parameters in Type 1 Diabetes Mellitus (T1D), but the cost is prohibitive. Here, we investigated the effect of short-term application of real-time and intermittently-scanned CGMS (rt and is-CGMS) in T1D individuals on change in HbA1c at the end of 3 months. METHODS: T1D individuals were randomized into three groups in a ratio of 1:1:2 - Group A (rt-CGMS for 2 weeks initially, followed by is-CGMS for 2 weeks at 3 months), Group B (is-CGMS for 2 weeks initially followed by rt-CGMS for 2 weeks at 3 months) and Group C (only self-monitoring of blood glucose), respectively. HbA1c at baseline, 3, and 6 months were compared. RESULTS: Out of a total 68 T1D patients, HbA1c decreased significantly in groups A and B at 6 months compared to the baseline, but not in group C. HbA1c was significantly lower in Group A compared to Group C at 3 and 6 months. Fructosamine levels significantly decreased in Group B before and after cross-over. Glycemic variability indices improved significantly after cross-over from is-CGMS to rt-CGMS. CONCLUSION: Intermittent application of CGMS for 2 weeks improves short- and long-term blood glucose control in T1D.

The role of oxygen in automotive grade lithium-ion battery cathodes: an atomistic survey of ageing.

The rising demand for high-performance lithium-ion batteries, pivotal to electric transportation, hinges on key materials like the Ni-rich layered oxide LiNixCoyAlzO2 (NCA) used in cathodes. The present study investigates the redox mechanisms, with particular focus on the role of oxygen in commercial NCA electrodes, both fresh and aged under various conditions (aged cells have performed >900 cycles until a cathode capacity retention of ∼80%). Our findings reveal that oxygen participates in charge compensation during NCA delithiation, both through changes in transition metal (TM)-O bond hybridization and formation of partially reversible O2, the latter occurs already below 3.8 V vs. Li/Li+. Aged NCA material undergoes more significant changes in TM-O bond hybridization when cycling above 50% SoC, while reversible O2 formation is maintained. Nickel is found to be redox active throughout the entire delithiation and shows a more classical oxidation state change during cycling with smaller changes in the Ni-O hybridization. By contrast, Co redox activity relies on a stronger change in Co-O hybridization, with only smaller Co oxidation state changes. The Ni-O bond displays an almost twice as large change in its bond length on cycling as the Co-O bond. The Ni-O6 octahedra are similar in size to the Co-O6 octahedra in the delithiated state, but are larger in the lithiated state, a size difference that increases with battery ageing. These contrasting redox activities are reflected directly in structural changes. The NCA material exhibits the formation of nanopores upon ageing, and a possible connection to oxygen redox activity is discussed. The difference in interaction of Ni and Co with oxygen provides a key understanding of the mechanism and the electrochemical instability of Ni-rich layered transition metal oxide electrodes. Our research specifically highlights the significance of the role of oxygen in the electrochemical performance of electric-vehicle-grade NCA electrodes, offering important insights for the creation of next-generation long-lived lithium-ion batteries.

An audit of medullary thyroid carcinoma from a tertiary care hospital in northwest India.

Introduction: Medullary thyroid carcinoma (MTC) is a rare thyroid malignancy originating from parafollicular C cells. It accounts for 5%-10% of all thyroid malignancies. Methods: An ambispective analysis of pathologically proven MTC presented in a tertiary care hospital in northwest India was performed after considering demography, clinical manifestation, RET mutation status, management, and outcome as denominators. Results: Among 2,735 thyroid malignancy cases who presented to our institute in the last 10 years (2012-2022), 78 (3%) had MTC with a mean age of presentation of 43 ± 11 years; 60% of them were female. The median duration of symptoms was 23 months (IQR 12-36 months). The most common presenting complaint was goiter with lymphadenopathy (80.8%). Among the atypical presentations, one each had ectopic Cushing's syndrome, hypertensive crisis in pregnancy due to pheochromocytoma, synchronous chondrosarcoma, and Von Hippel-Lindau disease spectrum. Median calcitonin and carcinoembryonic antigen (CEA) levels at presentation were 1,274 pg/mL (n = 64) and 149 ng/mL (n = 39), respectively. Twenty-two patients were germline RET mutation-positive, and they presented at a younger age. Majority of the patients presented with stage IV disease. Surgery was the primary modality of therapy. Twenty-nine patients received radiotherapy and 25 patients received tyrosine kinase inhibitors (TKIs). Nine patients received peptide receptor radiotherapy (PRRT) with Lu-177 with neoadjuvant capecitabine. Median progression-free survival (PFS) was 60 months. Patients without structurally and biochemically residual disease and stable disease after the first modality of therapy (Log-rank 11.4; p = 0.004) had a better PFS. Female patients (Log-rank: 9.5; p = 0.002) had a better PFS than male patients. Conclusion: This study showed that MTC comprises 3% of thyroid malignancies with a female preponderance. RET mutation-positive patients had a younger age at presentation. Surgery was the first-line therapy. Radiotherapy, TKI, and PRRT were given as a part of second-line or third-line therapy due to persistent disease and/or disease recurrence. The median PFS was better in female patients and in patients who had no residual lesions and stable disease after the primary modality of therapy.

The Role of Genetic Analysis in Demystifying the Diagnosis in a Middle-Aged Male Presenting With Proximal Muscle Weakness and Sclerotic-Lytic Skeletal Lesions.

Paget's disease of bone (PDB) usually presents with bone pain and deformities. Herein, we describe a case of PDB who presented with gradually progressive quadriparesis. A man in his forties presented with gradually progressive proximal muscle weakness involving all four limbs. The patient had an elevated serum alkaline phosphatase level and osteosclerosis at various skeletal sites in a radiological skeletal survey. 18F-fluorodeoxyglucose (FDG) PET-CT showed FDG-avid sclerotic-lytic lesions at multiple skeletal sites. Histopathology evaluation of bone and muscle biopsy specimens revealed PDB and inclusion body myopathy (IBM) with neurogenic atrophy, respectively. A diagnosis of IBM associated with PDB without frontotemporal dementia (IBMPFD) was suspected and confirmed by exome sequencing, which revealed a heterozygous mutation in the VCP gene. The bone disease responded to zoledronate administration. A high index of suspicion for IBMPFD should be kept in mind in any patient with PDB presenting with proximal muscle weakness.