Results 121 to 130 of about 346,856 (231)
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
Manual muscle testing and hand-held dynamometry in people with inflammatory myopathy : an intra- and interrater reliability and validity study [PDF]
Manual muscle testing (MMT) and hand-held dynamometry (HHD) are commonly used in people with inflammatory myopathy (IM), but their clinimetric properties have not yet been sufficiently studied.
Baschung Pfister, Pierrette +5 more
core +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
Juvenile Dermatomyositis in Pregnancy
Juvenile dermatomyositis has variable clinical presentations both in and outside of pregnancy. A literature review indicated that optimal maternal and fetal outcomes can be anticipated when the pregnancy is undertaken while the disease is in remission ...
Anthony Emeka Madu +3 more
doaj +1 more source
Fulminant systemic capillary leak syndrome due to C1 inhibitor deficiency complicating acute dermatomyositis: a case report [PDF]
INTRODUCTION: Dermatomyositis is a chronic inflammatory disorder characterized by muscular and dermatologic symptoms with variable internal organ involvement.
Andreas Brunauer +5 more
core +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
Update in juvenile myositis. [PDF]
This update on childhood idiopathic inflammatory myopathies (IIMs) reviews recent progress in the field of translational science and clinical research over the past 12-18 ...
Nistala, K, Wedderburn, LR
core +1 more source
Pharmacological management of dermatomyositis [PDF]
K
Dankó, Katalin +2 more
core +1 more source
A juvenilis és felnőttkori dermatomyositises betegek klinikai jellemzői [PDF]
Absztrakt Bevezetés: A juvenilis és felnőttkori dermatomyositisek szisztémás autoimmun megbetegedések, jellegzetességük a proximális végtagizomzat szimmetrikus gyengesége és típusos bőrtünetek ...
Dankó, Katalin +3 more
core +1 more source
The Vasculopathy of Juvenile Dermatomyositis
Juvenile dermatomyositis (JDM) is a rare autoimmune disease mainly characterized by muscle and skin involvement. Vasculopathy is considered central to the pathogenesis of the disease.
Charalampia Papadopoulou +2 more
doaj +1 more source

