Porto‐sinusoidal vascular disorder in a pediatric patient with prolidase deficiency: A case report
Abstract Prolidase deficiency (PD) is a rare autosomal recessive disorder affecting collagen turnover, leading to diverse clinical manifestations including dermatologic lesions, hepatosplenomegaly, and vascular anomalies. Liver involvement in PD is poorly understood, with few reported cases.
Melissa Castro +5 more
wiley +1 more source
Intramural ureteric calcification in dermatomyositis mimicking pelviureteric junction obstruction
Juvenile dermatomyositis classically manifests as necrotising vasculitis in multiple organs. It mainly involves the vessels of skin and muscle and is associated with subcutaneous deposits of calcium.
Shah Amar, Thakur Rakesh, Parashar Karan
doaj
Cricoarytenoid joint arthritis: a possible complication of dermatomyositis
Cricoarytenoid joint arthritis is most frequently reported in Rheumatoid Arthritis and in other systemic diseases such as Sjogren´s syndrome, Systemic Lupus Erythematosus, Ankylosing Arthritis, Juvenile Chronic Arthritis, and autoimmune hepatitis but it ...
Chadi Farah +4 more
doaj +1 more source
Biomarkers for muscle disease activity are needed for trials in Becker muscular dystrophy (BMD). We investigated magnesium (Mg2+), phosphodiesters (PDE) and pH from 31P‐MRS; and membrane permeability from random permeable barrier model (RBPM) diffusion as candidates, studying ‘preserved’ and ‘progressing’ muscles in patients with BMD versus controls ...
Esther J. Schrama +5 more
wiley +1 more source
Dermatomyositis: analysis of 109 patients surveyed at the Hospital das Clínicas (HCFMUSP), São Paulo, Brazil [PDF]
BACKGROUND: Dermatomyositis affects striated muscles, skin and other organs. OBJECTIVE: To characterize the disease from January 1992 to December 2002, assessing its classification, cutaneous and systemic manifestations, and also laboratory results ...
Luciena Cegatto Martins Ortigosa +1 more
doaj +1 more source
The Aetiology of Elevated Total Serum Immunoglobulin E in Children
ABSTRACT Aim The paucity of real‐world data on the aetiology of elevated total serum immunoglobulin E (TS‐IgE) in children afflicts families and health care systems with diagnostic uncertainty. We explored a cohort of children with TS‐IgE levels over 1000 kU/L, focusing on the prevalence of atopic and non‐atopic conditions, the aetiology of extremely ...
Aliisa Ärölä +2 more
wiley +1 more source
Reproductive health aspects in men with idiopathic inflammatory myopathy: a multicenter study [PDF]
OBJECTIVE: To evaluate reproductive health of males with idiopathic inflammatory myopathies (IIM), and comparing them with a control group. METHODS: Demographic data, urologic evaluation (including pubertal parameters and sexual/erectile function ...
Bonfá, Eloisa Silva Dutra de Oliveira +10 more
core +2 more sources
The EuroMyositis registry: an international collaborative tool to facilitate myositis research [PDF]
Aims: The EuroMyositis Registry facilitates collaboration across the idiopathic inflammatory myopathy (IIM) research community. This inaugural report examines pooled Registry data.
Andersson, Helena +27 more
core +7 more sources
Coexistence of Localized and Systemic Juvenile Scleroderma: A Case Report and Review of Literature
ABSTRACT Juvenile scleroderma (JS) is a rare chronic connective tissue disorder characterized by progressive fibrosis of the skin and soft tissues with/without internal organ involvements. Scleroderma manifests itself in both systemic (SSc) and localized (LS) forms.
Aye Miremarati +4 more
wiley +1 more source

