Pandiarajan Vignesh
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View article: Fungal Infections – a Stealthy Enemy in Patients with Chronic Granulomatous Disease: a 28-years’ Experience from North India
Fungal Infections – a Stealthy Enemy in Patients with Chronic Granulomatous Disease: a 28-years’ Experience from North India Open
View article: Genetic Landscape of Kawasaki Disease: An Update
Genetic Landscape of Kawasaki Disease: An Update Open
Kawasaki disease (KD), first identified in 1967 by Dr. Tomisaku Kawasaki, is an acute, self-limited vasculitis and remains the leading cause of acquired heart disease in children worldwide, particularly affecting those under the age of fiv…
View article: Role of CT Coronary Angiography at Initial Presentation in Kawasaki Disease—Insights from a Tertiary Care Center in North India
Role of CT Coronary Angiography at Initial Presentation in Kawasaki Disease—Insights from a Tertiary Care Center in North India Open
Background: Kawasaki disease (KD) is a systemic vasculitis and the leading cause of acquired heart disease in children. Early identification of coronary artery abnormalities (CAAs) is crucial to guide treatment and improve outcomes. While …
View article: Cytokine signaling defects in primary atopic diseases—an updated review
Cytokine signaling defects in primary atopic diseases—an updated review Open
Primary atopic disorders (PADs) are monogenic conditions associated with severe, early-onset atopic diseases. Clinically, they often overlap with polygenic allergic conditions, making specialized laboratory testing necessary to distinguish…
View article: Clinicopathological and Immunogenetic Characterization in 8 Patients with Familial Hemophagocytic Lymphohistiocytosis Type 2: A Study from North India with Literature Review
Clinicopathological and Immunogenetic Characterization in 8 Patients with Familial Hemophagocytic Lymphohistiocytosis Type 2: A Study from North India with Literature Review Open
Familial hemophagocytic lymphohistiocytosis type 2 (FHL2) is the commonest cause of familial hemophagocytic lymphohistiocytosis (FHLH). In this retrospective study, we analyzed 8 patients with a genetic diagnosis of FHL2 and then examined …
View article: Successful coil occlusion of intracranial aneurysm in a child with STAT3 hyper IgE syndrome
Successful coil occlusion of intracranial aneurysm in a child with STAT3 hyper IgE syndrome Open
Hyperimmunoglobulin E (hyper IgE) syndrome (HIES) is a rare form of inborn errors of immunity. We report an unusual presentation of intracranial aneurysm in an adolescent girl with AD-HIES. The child was managed successfully by coil occlus…
View article: ABS0914 ANTIPHOSPHOLIPID ANTIBODY SYNDROME IN PEDIATRIC LUPUS FROM NORTH-WEST INDIA: CLINICO-LABORATORY FEATURES AND APPLICATION OF 2023 ACR/EULAR CRITERIA
ABS0914 ANTIPHOSPHOLIPID ANTIBODY SYNDROME IN PEDIATRIC LUPUS FROM NORTH-WEST INDIA: CLINICO-LABORATORY FEATURES AND APPLICATION OF 2023 ACR/EULAR CRITERIA Open
View article: POS1123 Myositis Community Proposes a Framework to Guide Clinical Trials and Ensure Patient Safety during Influx of CAR-Based Therapies
POS1123 Myositis Community Proposes a Framework to Guide Clinical Trials and Ensure Patient Safety during Influx of CAR-Based Therapies Open
View article: ABS0840 ROLE OF CT CORONARY ANGIOGRAPHY FOR CORONARY ARTERY ASSESSMENT IN KAWASAKI DISEASE DURING THE ACUTE PHASE – EXPERIENCE FROM CHANDIGARH, NORTH INDIA
ABS0840 ROLE OF CT CORONARY ANGIOGRAPHY FOR CORONARY ARTERY ASSESSMENT IN KAWASAKI DISEASE DURING THE ACUTE PHASE – EXPERIENCE FROM CHANDIGARH, NORTH INDIA Open
View article: Secondary Hypogammaglobulinemia: An Under-Recognized Clinical Entity in Children
Secondary Hypogammaglobulinemia: An Under-Recognized Clinical Entity in Children Open
View article: THE EVOLUTION OF SHIPPING TRANSPORT ININDIA: CHALLENGES, INNOVATIONS, AND FUTURE PROSPECTS
THE EVOLUTION OF SHIPPING TRANSPORT ININDIA: CHALLENGES, INNOVATIONS, AND FUTURE PROSPECTS Open
This paper explores the historical evolution, challenges, innovations, and future prospectsofIndia's shipping industry, offering insights into how the country can navigate the complexitiesof the modern maritime ecosystem. India’s shipping …
View article: Hybrid modeling approaches for predicting COVID-19 mortality: A comparative study across USA, France, and India
Hybrid modeling approaches for predicting COVID-19 mortality: A comparative study across USA, France, and India Open
View article: Markers of T Lymphocyte Activation in Children With Kawasaki Disease: An Experimental Study From North India
Markers of T Lymphocyte Activation in Children With Kawasaki Disease: An Experimental Study From North India Open
Background The exact pathogenesis of Kawasaki disease (KD) is unknown despite extensive research in the area. Several studies have also implicated CD8 + T lymphocytes in the pathogenesis of KD. However, studies on the activation status of …
View article: Quality of life in patients with hereditary angioedema correlates with angioedema control: Our experience at Chandigarh, India
Quality of life in patients with hereditary angioedema correlates with angioedema control: Our experience at Chandigarh, India Open
Background: Hereditary angioedema (HAE) is characterized by unpredictable acute attacks that impair the patient’s quality of life (QoL) not only due to the impact on functional abilities caused by edema but also due to pain and other sympt…
View article: Centralized rapid genetic diagnosis of combined immunodeficiency in Japan
Centralized rapid genetic diagnosis of combined immunodeficiency in Japan Open
Purpose Severe combined immunodeficiency (SCID) is a pediatric emergency, and rapid genetic diagnosis is necessary for proper patient management, leading to successful stem cell transplantation and gene therapy. Ataxia telangiectasia (AT) …
View article: Hodgkin lymphoma in a child with biallelic FASL variant (ALPS‐FASL): Case report and review of literature
Hodgkin lymphoma in a child with biallelic FASL variant (ALPS‐FASL): Case report and review of literature Open
View article: The value of CT coronary angiography for a comprehensive assessment of left circumflex artery in Kawasaki disease: 9 years of experience from a tertiary center
The value of CT coronary angiography for a comprehensive assessment of left circumflex artery in Kawasaki disease: 9 years of experience from a tertiary center Open
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View article: Epidermodysplasia Verruciformis in CADINS Disease: Expanding the Phenotype
Epidermodysplasia Verruciformis in CADINS Disease: Expanding the Phenotype Open
View article: Supplementary Material for: X-linked lymphoproliferative syndrome: a spectrum of clinical and immunological profile and novel pathogenic variants from Chandigarh, India
Supplementary Material for: X-linked lymphoproliferative syndrome: a spectrum of clinical and immunological profile and novel pathogenic variants from Chandigarh, India Open
Introduction: X-linked lymphoproliferative syndrome (XLP) is a rare primary immune deficiency. Two types of XLP have been described: XLP-1 and XLP-2. Methods: We found 7 patients with XLP (3 had XLP-1 and 4 had XLP-2) after reviewing the d…
View article: Haploidentical Transplant in Radiosensitive Severe Combined Immunodeficiency Disease
Haploidentical Transplant in Radiosensitive Severe Combined Immunodeficiency Disease Open
View article: Impact of COVID-19 Pandemic on Clinical Care of Patients and Psychosocial Health of Affected Families with Chronic Granulomatous Disease: an Observational Study from North India
Impact of COVID-19 Pandemic on Clinical Care of Patients and Psychosocial Health of Affected Families with Chronic Granulomatous Disease: an Observational Study from North India Open
View article: Disseminated Mycobacterium fortuitum infection in a young girl with IFN-γR1 defect masquerading as histiocytosis
Disseminated Mycobacterium fortuitum infection in a young girl with IFN-γR1 defect masquerading as histiocytosis Open
DisseminatedMycobacterium fortuitum infection in a young girl withIFN-γR1 defect masquerading as histiocytosisArchan Sila, Suprit Basua, Jhumki Dasa, Sunil Sethib, Debajyoti Chatterjee c, Pandiarajan Vignesha, Deepti Suria, Ankur Kumar Jin…
View article: Case report: Chronic granulomatous disease presenting with early-onset inflammatory bowel disease and normal oxidative burst testing
Case report: Chronic granulomatous disease presenting with early-onset inflammatory bowel disease and normal oxidative burst testing Open
Background Due to the lack of widespread availability of flow cytometry services for immunodeficiency, nitroblue tetrazolium test (NBT) is the commonly used screening modality to identify patients with chronic granulomatous disease (CGD) i…
View article: Psychosocial impact of SARS- CoV-2 outbreak on patients with pediatric-onset systemic lupus erythematosus and their caregivers
Psychosocial impact of SARS- CoV-2 outbreak on patients with pediatric-onset systemic lupus erythematosus and their caregivers Open
A BSTRACT Aim: To assess distress, insomnia, and psychosocial impact of SARS-CoV-2 outbreak on children with SLE and their caregivers. Methods: Patients with pSLE undergoing treatment in the Department of Pediatrics, PGIMER, Chandigarh, an…
View article: Case report: Corticosteroids as an adjunct treatment for the management of liver abscess in Papillon–Lefèvre syndrome: A report on two cases
Case report: Corticosteroids as an adjunct treatment for the management of liver abscess in Papillon–Lefèvre syndrome: A report on two cases Open
Papillon–Lefèvre syndrome (PLS) is a rare autosomal recessive disorder characterized clinically by palmoplantar keratoderma, periodontitis, and recurrent pyogenic infections. Liver abscess is rarely reported in patients. The use of cortico…
View article: Challenges in the diagnosis of periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome in developing countries—A decade of experience from North India
Challenges in the diagnosis of periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome in developing countries—A decade of experience from North India Open
Background Reports of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome from developing countries are sparse. Recognizing PFAPA is often challenging in these regions due to a higher incidence of infectious ill…
View article: Blau syndrome: Lessons learned in a tertiary care centre at Chandigarh, North India
Blau syndrome: Lessons learned in a tertiary care centre at Chandigarh, North India Open
Objectives Blau syndrome (BS) is a rare autoinflammatory disease characterized by arthritis, dermatitis, and granulomatous uveitis in early childhood. The study presents the clinical experience of patients with BS at a tertiary care centre…
View article: Role of Infectious Agents in the Etiology of Kawasaki Disease
Role of Infectious Agents in the Etiology of Kawasaki Disease Open
Kawasaki disease (KD) is a multisystemic childhood vasculitis that predominantly affects the coronary arteries. The constellation of clinical features in KD, such as, acute onset of fever, redness of lips and oral mucosa, rash, swelling ov…
View article: Healing With Complication: An Unusual Case of Nasal Tip Ulceration in Leukocyte Adhesion Deficiency Type 1
Healing With Complication: An Unusual Case of Nasal Tip Ulceration in Leukocyte Adhesion Deficiency Type 1 Open
View article: Targeted Gene Sanger Sequencing Should Remain the First-Tier Genetic Test for Children Suspected to Have the Five Common X-Linked Inborn Errors of Immunity
Targeted Gene Sanger Sequencing Should Remain the First-Tier Genetic Test for Children Suspected to Have the Five Common X-Linked Inborn Errors of Immunity Open
To address inborn errors of immunity (IEI) which were underdiagnosed in resource-limited regions, our centre developed and offered free genetic testing for the most common IEI by Sanger sequencing (SS) since 2001. With the establishment of…