Low serum PON1 activity: an independent risk factor for coronary artery disease in North-West Indian type 2 diabetics.

The paraoxonase (PON1) gene polymorphisms are known to affect the PON1 activity and coronary artery disease (CAD) risk. Studies done so far have given conflicting results. In the present study, we determined the role of PON1 genetic variants and PON1 activity in the development of CAD in North-West Indian Punjabis, a distinct ethnic group, having high incidence of both CAD and type 2 diabetes.

Renal cortical necrosis, peripheral gangrene, perinephric and retroperitoneal haematoma in a patient with a viper bite.

Snakebites are estimated to affect more than 2.5 million people annually, of whom more than 100,000 die. Viper bites cause various systemic symptoms such as: coagulopathy; haemolysis; acute renal failure; a generalized increase in capillary permeability; rhabdomyolysis; and neurotoxicity.

Dietary adequacy of HIV infected individuals in north India--a cross-sectional analysis.

Background & Objectives: Dietary inadequacy is common in developing countries and so is in immune-deficient HIV infected individuals. Hence, an assessment of dietary patterns was done among a group of HIV infected individuals and compared with recommended dietary allowances.

Methods: One hundred consecutive HIV infected individuals were interviewed from the Immunodeficiency Clinic of a tertiary care center at Chandigarh.

Primary immunodeficiencies in India: a perspective.

Although primary immunodeficiency diseases (PIDs) were first reported in India in the 1970s, those diagnoses were based predominantly on clinical presentations-very limited immunological analyses were performed. Therefore, the validity of many early reports of PIDs may be questionable. However, in the last 10-15 years, diagnoses of PIDs have been based on flow cytometric analysis and, in a few cases, by mutational analysis.

Antinuclear antibody positive autoimmune disorders in North India: an appraisal.

The autoimmune disorders (AID) have since long been considered to be commoner in Western world as compared to Asian countries. This, however, may not be true as in developing countries, there are incomplete epidemiological data and lack of advanced diagnostic facilities leading to under diagnosis in many cases. In this study, we performed an 11-year retrospective analysis of medical records of all clinically suspected and immunofluorescence antinuclear antibody test (IF-ANA)-positive cases.

Symptomatic myocarditis in Kawasaki disease.

Kawasaki disease (KD) is an acute febrile illness of infants and young children that is characterized by a medium vessel vasculitis, most commonly involving the coronary arteries. Though subclinical myocarditis is rather common in KD, symptomatic congestive heart failure is extremely uncommon. The authors report a 9-y-old boy who developed heart failure (ejection fraction 28%) in the acute phase of KD.

Atypical trichinellosis without eosinophilia associated with osteomyelitis.

Human trichinellosis is an important food-borne zoonosis caused by a nematode worm, Trichinella. The symptoms of the disease vary widely depending on the infection load, stage of infection and host immunity and include nausea, vomiting, abdominal pain, fever, facial edema and muscle pain. The disease is usually characterized by moderate to high eosinophilia.

Correlation of defects in regional cerebral blood flow determined by 99mTc SPECT with residual neurocognitive testing abnormalities during and 3 months post exposure in acutely poisoned patients with organophosphates.

Introduction: Following acute organophosphate exposure, morphological changes in certain regions of the brain have been reported to develop within a few hours and involve neuronal degeneration. Single photon emission computed tomography (SPECT) has been used to determine changes in the regional cerebral blood flow and attempts have been made to correlate these changes with long-term neurological sequelae.

Purpose Of Study: The aim of the study was to determine changes in the regional cerebral blood flow by 99mTc-ECD SPECT following acute organophosphate poisoning and to correlate these defects with abnormalities in neurocognitive testing carried out during admission and at 3 months post exposure, in order to determine whether any changes in the cerebral blood flow could help in predicting future development of neurocognitive deficit.

Anasarca as the initial presentation of juvenile polymyositis: an uncommon occurrence.

Polymyositis is uncommon in childhood. Unlike dermatomyositis, which is common in pediatric age group, skin involvement is lacking in polymyositis. We report an 8-year-old boy who presented with anasarca as the initial complaint.

Serum paraoxonase-1 (PON1) activities (PONase/AREase) and polymorphisms in patients with type 2 diabetes mellitus in a North-West Indian population.

Objective: Paraoxonase-1 (PON1), an HDL-C associated enzyme, protects lipoproteins from oxidation. There is evidence that PON1 enzyme activity is reduced in the patients with type 2 diabetes mellitus (T2DM). North-West Indian Punjabis, a distinct ethnic group has high incidence of T2DM.

Paraoxonase 1 (PON1) polymorphisms, haplotypes and activity in predicting cad risk in North-West Indian Punjabis.

Background: Human serum paraoxonase-1 (PON1) prevents oxidation of low density lipoprotein cholesterol (LDL-C) and hydrolyzes the oxidized form, therefore preventing the development of atherosclerosis. The polymorphisms of PON1 gene are known to affect the PON1 activity and thereby coronary artery disease (CAD) risk. As studies are lacking in North-West Indian Punjabi's, a distinct ethnic group with high incidence of CAD, we determined PON1 activity, genotypes and haplotypes in this population and correlated them with the risk of CAD.

A decade long experience of anti-neutrophil cytoplasmic antibody testing in a tertiary care referral center in North India: perspective from a developing country.

Background: In a developing, tropical country like India, discontinuous power supply, high temperatures during summer, and lack of consistent cold chain and funds provide a challenging atmosphere for anti-neutrophil cytoplasmic antibody (ANCA) testing and reporting. However, a simple in-house test and testing algorithm are described here, which have been developed and tested over time.

Materials And Methods: An analysis of a decade of testing and reporting of ANCA in the Department of Immunopathology in a tertiary referral health care center was performed to highlight the importance of testing for ANCA in proposed 1999 guideline recommended indications.

Amyloidosis in a child with leucocyte adhesion deficiency type-1: an unusual association.

Leucocyte adhesion deficiency type I (LAD I) is a rare autosomal recessive disorder of leucocyte function, characterized by delayed separation of the umblical cord, recurrent bacterial and fungal infections, defective wound healing and impressive constant blood neutrophilia. The authors report a child with genetic diagnosis of leucocyte adhesion deficiency (LAD) type I, who succumbed to his illness and developed amyloidosis preterminally. To the best of author's knowledge this is the first case of leucocyte adhesion deficiency type I associated with amyloidosis among the human LAD cases worldwide.

Peripheral gangrene in a breast fed neonate--is hypernatremic dehydration the cause?

Hypernatremic dehydration in breast fed neonates is a rare but increasingly recognized problem. If not identified early, it may lead to potentially life threatening complications. The authors describe a neonate who developed thrombosis and lower limb ischemia secondary to hypernatremic dehydration.

Wiskott-Aldrich syndrome presenting with early onset recurrent acute hemorrhagic edema and hyperostosis.

We report two unusual presenting manifestations of Wiskott-Aldrich syndrome (WAS), recurrent acute hemorrhagic edema of infancy (AHEI); a form of cutaneous vasculitis and hyperostosis of the tibia. Though cutaneous vasculitis is known to occur in WAS, presentation in early infancy and as AHEI is extremely uncommon. Hyperostosis is not a well-recognized association in WAS; only three patients with this association have been previously reported.

Polymorphism in the promoter of the CCL5 gene (CCL5G-403A) in a cohort of North Indian children with Kawasaki disease. A preliminary study.

Objectives: Kawasaki disease (KD) is a common vasculitic disorder and a leading cause of acquired heart disease in children. However, there is a paucity of information on KD from developing countries. The clinical phenotype of KD in India is different from that in the West.

Macrophage activation syndrome in children with systemic onset juvenile idiopathic arthritis: clinical experience from northwest India.

The objective of this study is to describe the clinical and laboratory features of macrophage activation syndrome (MAS) in systemic onset juvenile idiopathic arthritis (SOJIA) at a tertiary care center in northwest India. Review of medical records of all children with SOJIA admitted during the period January 1995-December 2008 in Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, was done. Six patients (5 boys and 1 girl) with SOJIA and MAS were identified.

Exercise myocardial perfusion imaging to evaluate inducible ischaemia in children with Kawasaki disease.

Objective: Kawasaki disease (KD) is an acute multi-system vasculitis of unknown aetiology, which occurs predominantly in infants and young children. Coronary artery abnormalities may occur in 15-25% of patients who are not treated in the acute phase of the disease with a high dose of intravenous immunoglobulin. Myocardial perfusion imaging is used as a modality to monitor the cardiovascular effects of the disease.

Spinal cord compression caused by anaplastic large cell lymphoma in an HIV infected individual.

Lymphomas occur with an increased frequency in patients with Human Immunodeficiency Virus (HIV) infection. These are usually high-grade immunoblastic lymphomas and primary central nervous system lymphomas. Anaplastic large cell lymphoma (ALCL) is a distinct type of non-Hodgkin's lymphoma.

Lupus anticoagulant in a child with celiac disease: a rare association.

Celiac disease or gluten-sensitive enteropathy is characterized by malabsorption, chronic inflammation of the small intestine mucosa, villous atrophy and crypt hyperplasia. Association of auto-immunity has been reported to be almost threefold higher in patients with celiac disease. While there are several reports of celiac disease in association with autoimmune diseases, there is paucity of literature on its association with antiphospholipid antibodies.

Is Kawasaki disease incidence rising in Chandigarh, North India?

Objective: To estimate incidence of Kawasaki disease (KD) over time among children in the city of Chandigarh, North India.

Patients And Methods: We analysed records of all children with KD below 15 years of age at the Pediatric Allergy Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, from January 1994 to December 2008. Diagnosis and treatment of KD were based on American Heart Association criteria.