divya ramadoss - Academia.edu (original) (raw)

Papers by divya ramadoss

Research paper thumbnail of Standardized nailfold capillaroscopy in children with rheumatic diseases: a worldwide study

Rheumatology

Objectives To standardly assess and describe nailfold videocapillaroscopy (NVC) assessment in chi... more Objectives To standardly assess and describe nailfold videocapillaroscopy (NVC) assessment in children and adolescents with juvenile rheumatic and musculoskeletal diseases (jRMD) vs healthy controls (HCs). Material and methods In consecutive jRMD children and matched HCs from 13 centres worldwide, 16 NVC images per patient were acquired locally and read centrally per international consensus standard evaluation of the EULAR Study Group on Microcirculation in Rheumatic Diseases. A total of 95 patients with JIA, 22 with JDM, 20 with childhood-onset SLE (cSLE), 13 with juvenile SSc (jSSc), 21 with localized scleroderma (lSc), 18 with MCTD and 20 with primary RP (PRP) were included. NVC differences between juvenile subgroups and HCs were calculated through multivariable regression analysis. Results A total of 6474 images were assessed from 413 subjects (mean age 12.1 years, 70.9% female). The quantitative NVC characteristics were significantly lower or higher in the following subgroups c...

Research paper thumbnail of Deficiency of Adenosine Deaminase 2 (DADA2): One Disease, Several Faces

Indian Journal of Pediatrics

Research paper thumbnail of Angiokeratomas—A Mimic to Remember

JCR: Journal of Clinical Rheumatology, 2020

FIGURE. Arrowheads pointing to red pinpoint lesions suggestive of angiokeratomas. Color online-fi... more FIGURE. Arrowheads pointing to red pinpoint lesions suggestive of angiokeratomas. Color online-figure is available at http://www. jclinrheum.com. A 20-year-old male patient with a marfanoid habitus was on a diagnostic odyssey for 10 years for intermittent incapacitating pain and burning sensation of his palms and soles. Recent-onset palpitations had prompted a cardiology consult. On echocardiography, trivial mitral and tricuspid regurgitations were noted. He was referred to us in the pediatric rheumatology clinic to correlate his features because several investigations had not yielded a diagnosis. We additionally observed early claw hand deformity and digital clubbing, but the physical sign that clinched the diagnosis was the presence of raised reddish nodular lesions over the buttocks and pinpoint reddish lesions over his fingers suggesting angiokeratomas. Slit-lamp examination showed right faint subepithelial stromal corneal scars, and his renal profile and sonography were normal. Leukocyte α-galactosidase enzyme activity was absent, corroborating the suspicion of Fabry disease (FD). Genetic studies are pending for cost constraints. Angiokeratomas are benign vascular lesions, characterized by ectatic blood vessels in the upper third of the dermis. In FD, deficiency of α-galactosidase enzyme leads to the accumulation of globotriaosylceramide (Gb3) in the dermal vessel endothelium, which causes ectasia. They may be the earliest sign, appearing between the ages of 5 and 15 years. The involvement of soles, chin, ears, axillae, and mucosa has been reported. Although not pathognomonic of FD, their presence in a given clinical setting of chronic acroparesthesia and acral limb pains without localization must flag the suspicion of FD. Fabry disease is awell-knownmimic in an adult and pediatric rheumatology clinic, and enzyme replacement therapy, although expensive, is now available and highly effective in preventing multisystem damage. Angiokeratomas are also found in hereditary hemorrhagic telangiectasia, Fordyce disease, Schindler disease, fucosidosis, and sialidosis. Differential diagnosis of these lesions includes trauma-induced hemorrhage, hemorrhagic Darier disease, infective endocarditis (splinter hemorrhages), and dermatitis herpetiformis.

Research paper thumbnail of Standardized nailfold capillaroscopy in children with rheumatic diseases: a worldwide study

Rheumatology

Objectives To standardly assess and describe nailfold videocapillaroscopy (NVC) assessment in chi... more Objectives To standardly assess and describe nailfold videocapillaroscopy (NVC) assessment in children and adolescents with juvenile rheumatic and musculoskeletal diseases (jRMD) vs healthy controls (HCs). Material and methods In consecutive jRMD children and matched HCs from 13 centres worldwide, 16 NVC images per patient were acquired locally and read centrally per international consensus standard evaluation of the EULAR Study Group on Microcirculation in Rheumatic Diseases. A total of 95 patients with JIA, 22 with JDM, 20 with childhood-onset SLE (cSLE), 13 with juvenile SSc (jSSc), 21 with localized scleroderma (lSc), 18 with MCTD and 20 with primary RP (PRP) were included. NVC differences between juvenile subgroups and HCs were calculated through multivariable regression analysis. Results A total of 6474 images were assessed from 413 subjects (mean age 12.1 years, 70.9% female). The quantitative NVC characteristics were significantly lower or higher in the following subgroups c...

Research paper thumbnail of Deficiency of Adenosine Deaminase 2 (DADA2): One Disease, Several Faces

Indian Journal of Pediatrics

Research paper thumbnail of Angiokeratomas—A Mimic to Remember

JCR: Journal of Clinical Rheumatology, 2020

FIGURE. Arrowheads pointing to red pinpoint lesions suggestive of angiokeratomas. Color online-fi... more FIGURE. Arrowheads pointing to red pinpoint lesions suggestive of angiokeratomas. Color online-figure is available at http://www. jclinrheum.com. A 20-year-old male patient with a marfanoid habitus was on a diagnostic odyssey for 10 years for intermittent incapacitating pain and burning sensation of his palms and soles. Recent-onset palpitations had prompted a cardiology consult. On echocardiography, trivial mitral and tricuspid regurgitations were noted. He was referred to us in the pediatric rheumatology clinic to correlate his features because several investigations had not yielded a diagnosis. We additionally observed early claw hand deformity and digital clubbing, but the physical sign that clinched the diagnosis was the presence of raised reddish nodular lesions over the buttocks and pinpoint reddish lesions over his fingers suggesting angiokeratomas. Slit-lamp examination showed right faint subepithelial stromal corneal scars, and his renal profile and sonography were normal. Leukocyte α-galactosidase enzyme activity was absent, corroborating the suspicion of Fabry disease (FD). Genetic studies are pending for cost constraints. Angiokeratomas are benign vascular lesions, characterized by ectatic blood vessels in the upper third of the dermis. In FD, deficiency of α-galactosidase enzyme leads to the accumulation of globotriaosylceramide (Gb3) in the dermal vessel endothelium, which causes ectasia. They may be the earliest sign, appearing between the ages of 5 and 15 years. The involvement of soles, chin, ears, axillae, and mucosa has been reported. Although not pathognomonic of FD, their presence in a given clinical setting of chronic acroparesthesia and acral limb pains without localization must flag the suspicion of FD. Fabry disease is awell-knownmimic in an adult and pediatric rheumatology clinic, and enzyme replacement therapy, although expensive, is now available and highly effective in preventing multisystem damage. Angiokeratomas are also found in hereditary hemorrhagic telangiectasia, Fordyce disease, Schindler disease, fucosidosis, and sialidosis. Differential diagnosis of these lesions includes trauma-induced hemorrhage, hemorrhagic Darier disease, infective endocarditis (splinter hemorrhages), and dermatitis herpetiformis.