A Case Report of Larsen's Syndrome, Antiphospholipid Syndrome, Diaphragmatic Hernia, and a Colon Polyp: A Hidden Association or a Mere Coincidence.

Larsen's syndrome is an exceedingly rare and debilitating skeletal disorder characterized by multiple bony deformities and joint contractures. A 47-year-old female grappled with its severe manifestations, including functional quadriplegia, limb atrophy, and various orthopedic surgeries. This is the only reported case of the concurrent presence of antiphospholipid antibody syndrome (APLA) and Larsen's syndrome.

A Case of Hepatocellular Carcinoma after Treatment for Chronic Myeloid Leukemia in a Patient without Liver Disease.

Introduction: Chronic myeloid leukemia (CML) is a myeloproliferative disease caused by the reciprocal translocation of chromosomes 9 and 22, which leads to a chimeric gene product known as BCR-ABL. Some studies have shown a higher incidence of secondary malignancies than the one seen in the general population in patients with CML. Hepatocellular carcinoma (HCC) is rarely reported in association with CML and/or CML-related treatment.

Factors Affecting the Availability and Utilization of Essential Medicines in India: A Systematic Review.

Essential medicines or drugs are recognized as highly cost-effective components within contemporary healthcare, demonstrating significant potential for improving health outcomes. The provision of essential medicines directly impacts the functioning of healthcare facilities, resulting in financial hardship. This review aims to fill knowledge gaps by examining obstacles hindering access and utilization of essential medicines in India.

Clinical Profile and Outcome of Critically-ill Hospitalized COVID-19 Patients Aged 18-50 Years: A Limited Resource Setting Perspective.

Objectives: The coronavirus disease of 2019 (COVID-19) pandemic started by affecting the older age groups with comorbidities but gradually advanced to severely affect even young adults. This study attempts to clinically evaluate critically ill young and middle-aged adults hospitalized with COVID-19 and study the association of risk factors and the markers of inflammation and coagulation with their outcome.

Materials And Methods: A prospective observational study on 146 patients was conducted in a tertiary care hospital in Western India.

Characteristics and determinants of pulmonary long COVID.

BACKGROUNDPersistent cough and dyspnea are prominent features of postacute sequelae of SARS-CoV-2 (also termed "long COVID"); however, physiologic measures and clinical features associated with these pulmonary symptoms remain poorly defined. Using longitudinal pulmonary function testing (PFT) and CT imaging, this study aimed to identify the characteristics and determinants of pulmonary long COVID.METHODSThis single-center retrospective study included 1,097 patients with clinically defined long COVID characterized by persistent pulmonary symptoms (dyspnea, cough, and chest discomfort) lasting for 1 or more months after resolution of primary COVID infection.

Gujarati translation, validity and reliability of Walking Impairment Questionnaire in people with intermittent claudication due to peripheral artery disease.

Introduction: The Walking Impairment Questionnaire (WIQ) is a common and easy-to-use assessment of walking incapacity in people with claudication due to peripheral artery disease (PAD). It has four subscales: pain severity, walking distance, walking speed, and ability to climb stairs. It has not been translated into Gujarati, which limits its use in Indian subjects.

A Study of Clinical Profile and Treatment in Adult Hemophilia Patients with Special Reference to the Inhibitor Levels.

Introduction Hemophilia is an uncommon, X-linked recessive bleeding condition characterized by a lack of either factor VIII or factor IX. It is more prevalent in men. Due to the substantial impact inhibitor development has on patient prognosis, the primary treatment for hemophilia is the transfusion of recombinant factors.

Half a Century in Hiding: A Unique Case of Tuberculoid Leprosy with an Unprecedented Incubation Period.

BACKGROUND Leprosy, also known as Hansen's disease, is a neglected tropical disease with low prevalence in the United States. The disease's long incubation period can cause delayed presentation, and most affected individuals have a history of travel or work in leprosy-endemic regions. The immune response to Mycobacterium leprae determines the clinical characteristics of leprosy, with tuberculoid leprosy being characterized by well-defined granulomas and involvement of peripheral nerves.

Towards using bacterial microcompartments as a platform for spatial metabolic engineering in the industrially important and metabolically versatile .

Advances in synthetic biology have enabled the incorporation of novel biochemical pathways for the production of high-value products into industrially important bacterial hosts. However, attempts to redirect metabolic fluxes towards desired products often lead to the buildup of toxic or undesirable intermediates or, more generally, unwanted metabolic cross-talk. The use of shells derived from self-assembling protein-based prokaryotic organelles, referred to as bacterial microcompartments (BMCs), as a scaffold for metabolic enzymes represents a sophisticated approach that can both insulate and integrate the incorporation of challenging metabolic pathways into industrially important bacterial hosts.

A rare case of scedosporium apiospermum osteomyelitis in an immunocompetent patient.

Scedosporium, a widespread filamentous fungus found in diverse environments, has experienced a rise in cases due to escalating malignancies and chronic immunosuppression. Clinical manifestations span mycetoma, airway involvement, and various infections, with osteomyelitis being a notable complication. We present a case of a 77-year-old female initially displaying cutaneous Scedosporium signs, which progressed to osteomyelitis.

Counseling intervention improves treatment adherence among newly diagnosed drug sensitive Tuberculosis patients of Ahmedabad city - A mixed methods approach.

Introduction: Counselling sessions based on the health behaviour module for patients on TB treatment may help improve the level of understanding of tuberculosis and its treatment, importance of adherence to therapy, possible side effects, dietary habits and to address any misconceptions; leading to better adherence and improved outcome. Hence, study attempted to assess the impact of brief counselling intervention through treatment adherence supportive activist (TASA) in improving adherence to treatment among drug sensitive TB patients.

Methods: An explanatory mixed methods design (QUAN-Qual) was carried out.

Iron homeostasis and post-hemorrhagic hydrocephalus: a review.

Iron physiology is regulated by a complex interplay of extracellular transport systems, coordinated transcriptional responses, and iron efflux mechanisms. Dysregulation of iron metabolism can result in defects in myelination, neurotransmitter synthesis, and neuronal maturation. In neonates, germinal matrix-intraventricular hemorrhage (GMH-IVH) causes iron overload as a result of blood breakdown in the ventricles and brain parenchyma which can lead to post-hemorrhagic hydrocephalus (PHH).

Late Onset Systemic Lupus Erythematosus - Clinical and Autoantibody Profile and its Comparison with Young Onset Systemic Lupus Erythematosus.

Objectives: The aim of the study was to determine the clinical features & autoantibody profile of patients having late onset Systemic Lupus Erythematosus (SLE) and to compare with young onset SLE due to its scarce data from India.

Methods: All patients who fulfilled the 1997 ACR criteria for SLE were included. Late onset patients were >50 years of age and young onset were <50 years >18 years at the time of first SLE-related symptom.

Cost-effectiveness analysis of parenteral iron therapy compared to oral iron supplements in managing iron deficiency anemia among pregnant women.

Objective: This study compared the clinical efficacy and cost-effectiveness of parenteral iron, using intravenous iron sucrose (IVIS) therapy against the standard regimen of oral iron (OI) therapy for managing iron-deficiency anemia (IDA) among pregnant women in a natural primary care setting in Gujarat.

Design: A prospective cost-effectiveness study was conducted in natural programme setting wherein 188 pregnant women in their 14 to 18 weeks with moderate and severe anemia women enrolled from two districts of Gujarat, and 142 were followed up until the post-partum phase. The intervention group comprised of 82 participants who were administered IVIS, while the comparison group comprised of 106 participants who were put on OI therapy.

A systemic review of case reports up to october 2023, featuring our unique case study.

Background: Mycobacterium nebraskense is a rare, slow growing nontuberculous mycobacterium species with limited documented cases. This systematic review aims to comprehensively analyze the clinical characteristics, presentation, and management of M. nebraskense infections by analyzing the available literature, including a newly reported case that we present in this article.

Sfrp4 is required to maintain Ctsk-lineage periosteal stem cell niche function.

We have previously reported that the cortical bone thinning seen in mice lacking the Wnt signaling antagonist is due in part to impaired periosteal apposition. The periosteum contains cells which function as a reservoir of stem cells and contribute to cortical bone expansion, homeostasis, and repair. However, the local or paracrine factors that govern stem cells within the periosteal niche remain elusive.

The resuscitation-promoting factor (Rpf) from and its putative reaction product 1,6-anhydro-MurNAc increase culturability of environmental bacteria.

Dormant bacterial cells do not divide and are not immediately culturable, but they persist in a state of low metabolic activity, a physiological state having clinical relevance, for instance in latent tuberculosis. Resuscitation-promoting factors (Rpfs) are proteins that act as signalling molecules mediating growth and replication. In this study we aimed to test the effect of Rpfs from on the number and diversity of cultured bacteria using insect and soil samples, and to examine if the increase in culturability could be reproduced with the putative reaction product of Rpf, 1,6-anhydro--acetylmuramic acid (1,6-anhydro-MurNAc).

larvae are tolerant to oral infection with the bacterial pathogen .

The fruit fly is an excellent model for dissecting the molecular and functional bases of bacterial pathogenicity and host antibacterial immune response. The Gram-negative bacterium is an insect-specific pathogen that forms a mutualistic relationship with the entomopathogenic nematode . Here we find that oral infection of larvae with moderately reduces their survival ability while the bacteria replicate efficiently in the infected insects.

Clinical Profile of Adult Hemophilia Patients with Special Reference to FISH and WFHPE Score: An Observational Cross-sectional Study.

Background And Objectives: Hemophilia is an X-linked recessive inherited disease affecting the coagulation pathway due to congenital deficiencies in either factor VIII (hemophilia A) or factor IX (hemophilia B). The clinical assessment of a patient's functional ability and the state of joint conditions is carried out by the clinicians by administering questionnaires namely the Gilbert or the World Federation of Hemophilia Physical Examination (WFH-PE) score for joint condition and Functional Independence Score in Hemophilia (FISH) for joint function. Here, we have studied the clinical profile of adult hemophilia patients with the short- and long-term complications of the disease.

Monosodium Glutamate (MSG) Symptom Complex (Chinese Restaurant Syndrome): Nightmare of Chinese Food Lovers!

Chinese food, containing the ingredient monosodium glutamate (MSG) as the main additive agent, results in a variety of symptoms in susceptible individuals. The spectrum of symptoms ranges from headache, sweating, abdominal pain, and urticaria to angioedema in severe cases. This group of symptoms is known as MSG symptom complex or Chinese restaurant syndrome (CRS).

Liver Involvement in Individuals with Obesity: A Cross-sectional Study from Western India.

Background: Obesity is a largely neglected health problem in developing countries which leads to additional morbidities including nonalcoholic fatty liver disease (NAFLD), one of the most important causes of chronic liver disease. Central obesity is intricately related to the pathogenesis of the NAFLD, which over time could result in a fiogenic response and end-stage liver disease. We have attempted to study the association of various risk factors and laboratory investigations with the incidence of liver involvement in obese individuals.

Imd signaling interacts with insulin signaling and alters feeding rate upon parasitic nematode infection.

Significant progress has been made in recent years on exploring immunometabolism, a field that integrates two processes essential for maintaining tissue and organismal homeostasis, immunity and metabolism. The nematode parasite , its mutualistic bacteria , and the fruit fly constitute a unique system to investigate the molecular basis of host immunometabolic response to nematode-bacterial complexes. In this study, we explored the contribution of the two major immune signaling pathways, Toll and Imd, to sugar metabolism in larvae during infection with nematodes.