RESUMEN
PURPOSE OF REVIEW: Provide the most recent updates on the epidemiology, pathogenesis, and treatment advances in Kawasaki disease. RECENT FINDINGS: Treatment advances in complex, IVIG-refractory cases of Kawasaki disease. Multisystem inflammatory syndrome, a newly reported inflammatory condition with Kawasaki-like features and an association with the 2019 Coronavirus (COVID-19). Kawasaki disease (KD) is a rare systemic inflammatory disease that predominately affects children less than 5 years of age. Pathogenesis of KD remains unknown; the leading theory is that an unknown stimulus triggers an immune-mediated inflammatory cascade in a genetically susceptible child. Classic KD is a clinical diagnosis based on set criteria and excluding other similar clinical entities. Patients who do not fulfill complete diagnostic criteria for KD are often referred to as atypical (or incomplete) KD. The most feared complication of KD is coronary artery abnormality development, and patients with atypical KD are also at risk. Administration of intravenous immunoglobulin (IVIG) and aspirin has greatly reduced the incidence of coronary lesions in affected children. Several other immune-modulating therapies have recently been utilized in complex or refractory cases.
Asunto(s)
Betacoronavirus , Infecciones por Coronavirus/complicaciones , Diagnóstico por Imagen/métodos , Factores Inmunológicos/uso terapéutico , Síndrome Mucocutáneo Linfonodular/complicaciones , Pandemias , Neumonía Viral/complicaciones , COVID-19 , Infecciones por Coronavirus/epidemiología , Humanos , Síndrome Mucocutáneo Linfonodular/diagnóstico , Síndrome Mucocutáneo Linfonodular/terapia , Neumonía Viral/epidemiología , SARS-CoV-2Asunto(s)
Estenosis Carotídea/diagnóstico por imagen , Hipertensión/diagnóstico , Enfermedad de Moyamoya/diagnóstico , Convulsiones/diagnóstico , Accidente Cerebrovascular/diagnóstico , Negro o Afroamericano , Antihipertensivos/uso terapéutico , Determinación de la Presión Sanguínea/métodos , Estenosis Carotídea/patología , Niño , Angiografía por Tomografía Computarizada/métodos , Diagnóstico Diferencial , Humanos , Hipertensión/complicaciones , Hipertensión/tratamiento farmacológico , Angiografía por Resonancia Magnética/métodos , Masculino , Monitoreo Fisiológico/métodos , Enfermedad de Moyamoya/diagnóstico por imagen , Enfermedad de Moyamoya/etiología , Enfermedades Raras , Convulsiones/diagnóstico por imagen , Convulsiones/etiología , Índice de Severidad de la Enfermedad , Accidente Cerebrovascular/diagnóstico por imagen , Accidente Cerebrovascular/etiología , Factores de TiempoAsunto(s)
Ganglios Linfáticos/patología , Pelvis/patología , Sarcoidosis/diagnóstico , Dolor Abdominal/etiología , Dolor de Espalda/etiología , Femenino , Glucocorticoides/uso terapéutico , Humanos , Pelvis/diagnóstico por imagen , Poliuria/etiología , Prednisona/uso terapéutico , Sarcoidosis/tratamiento farmacológico , Tomografía Computarizada por Rayos X , Adulto JovenRESUMEN
A retrospective chart review was conducted to detect patients with sarcoidosis seen by pediatric rheumatology service from the period of 1992 to 2013 at Children's hospital of New Orleans. Twenty-seven patients were identified. The average duration of symptoms before diagnosis was 5 (range 1-120) months. Five patients had onset before the age of 5 years and were diagnosed with early-onset sarcoidosis. The most common manifestations at presentation were constitutional symptoms (62 %) followed by ocular (38 %). During the course of illness, 19/27 (70 %) had multiorgan involvement. Common manifestations included uveitis/iritis (77 %), fever (50 %), hilar adenopathy (42 %), arthritis (31 %), peripheral lympadenopathy (31 %), hepatosplenomegaly (31 %), parenchymal lung disease (27 %), and skin rash (19 %). Unusual manifestations included granulomatous bone marrow disease (3 cases), hypertension (2), abdominal aortic aneurysm (large vessel vasculitis; 1), granulomatous hepatitis (1), nephrocalcinosis (1), membranous nephropathy (1), refractory granulomatous interstitial nephritis with recurrence in transplanted kidney (1), CNS involvement (2), parotid gland enlargement (1), and sensorineural hearing loss (1). Biopsy specimen was obtained in 21/27 (77 %) patients, and demonstration of noncaseating granuloma associated with negative stains for mycobacteria and fungi was seen in 18 patients. Elevated angiotensin-converting enzyme level was seen in 74 % of patients. Treatment with oral prednisone was initiated in symptomatic patients with significant clinical improvement. Low-dose methotrexate (MTX) 10-15 mg/m(2)/week orally, as steroid-sparing agent, was administered in 14 patients. Other immunomodulators included cyclophosphamide (2 patients), etanercept (2), infliximab (2), mycophenolate mofetil (1), and tacrolimus (1). Childhood sarcoidosis is prevalent in Louisiana. Most of the affected children present with a multisystem disease associated with manifestations similar to those of adult patients. Low-dose MTX seems to be effective, steroid sparing, and safe adjunct to treat sarcoidosis with multiorgan involvement. Early-onset disease is less common and associated with increased morbidity, flares, and poor prognosis.
Asunto(s)
Artritis/tratamiento farmacológico , Artritis/epidemiología , Metotrexato/uso terapéutico , Prednisona/uso terapéutico , Sinovitis/tratamiento farmacológico , Sinovitis/epidemiología , Uveítis/tratamiento farmacológico , Uveítis/epidemiología , Adolescente , Artritis/patología , Niño , Preescolar , Ciclofosfamida/uso terapéutico , Femenino , Humanos , Lactante , Infliximab/uso terapéutico , Louisiana , Masculino , Estudios Retrospectivos , Sarcoidosis , Sinovitis/patología , Uveítis/patología , Adulto JovenRESUMEN
To compare disability and emotional health in individuals with irritable bowel syndrome (IBS), fibromyalgia, or both, patients completed the Questionnaire on Pediatric Gastrointestinal Symptoms-Rome III, childhood Functional Disability Inventory (FDI), and the Behavior Assessment System for Children, Second Edition. Patients' (age range 8-18 years, 19 IBS, 12 fibromyalgia, and 12 both) FDI scores showed greater disability than scores from historically healthy patients. Fibromyalgia (FDI 22.5â±â12.7, Pâ=â0.018) and patients with both (FDI 26.2â±â13.8, Pâ=â0.001) averaged greater disability than those with IBS (FDI 10.6â±â7.9). Disability was correlated with anxiety and depression symptoms. Disability and psychological symptoms are important when evaluating individuals with fibromyalgia and IBS.
Asunto(s)
Niños con Discapacidad , Fibromialgia/complicaciones , Síndrome del Colon Irritable/complicaciones , Calidad de Vida , Adolescente , Ansiedad/complicaciones , Niño , Depresión/complicaciones , Evaluación de la Discapacidad , Niños con Discapacidad/psicología , Femenino , Humanos , Masculino , Encuestas y CuestionariosAsunto(s)
Enfermedad de Hashimoto/complicaciones , Hipotiroidismo/complicaciones , Dolor Musculoesquelético/complicaciones , Adolescente , Diagnóstico Diferencial , Femenino , Enfermedad de Hashimoto/diagnóstico , Enfermedad de Hashimoto/tratamiento farmacológico , Humanos , Hipotiroidismo/diagnóstico , Hipotiroidismo/tratamiento farmacológico , Tiroxina/uso terapéuticoRESUMEN
Kawasaki Disease, a systemic vasculitis of unknown origin with specific predilection for the coronary arteries, is the most common cause of childhood-acquired heart disease in western countries. Despite its world-wide incidence, the pathophysiology of this enigmatic disease is still under investigation. Diagnosis is made on a clinical basis, with supportive laboratory evidence and imaging. Once identified, timely initiation of treatment is imperative in order to quell the inflammatory response and decrease the incidence of long-term sequelae, specifically coronary artery aneurysms. Finally, longitudinal follow-up should be implemented based on risk stratification and individualized to each patient.
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Síndrome Mucocutáneo Linfonodular/diagnóstico , Síndrome Mucocutáneo Linfonodular/tratamiento farmacológico , Aneurisma Coronario/etiología , Glucocorticoides/uso terapéutico , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Síndrome Mucocutáneo Linfonodular/etiología , Síndrome Mucocutáneo Linfonodular/fisiopatología , Factores de Riesgo , Factor de Necrosis Tumoral alfa/antagonistas & inhibidoresAsunto(s)
Lupus Eritematoso Sistémico/diagnóstico , Linfadenitis/etiología , Adolescente , Negro o Afroamericano , Femenino , Humanos , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/etnología , Ganglios Linfáticos/patología , Linfadenitis/etnología , Linfadenitis/patología , Cuello , NecrosisAsunto(s)
Neoplasias Óseas/diagnóstico , Neoplasias Femorales/diagnóstico , Osteoma Osteoide/diagnóstico , Neoplasias Óseas/cirugía , Niño , Diagnóstico Diferencial , Femenino , Neoplasias Femorales/cirugía , Cuello Femoral/diagnóstico por imagen , Cuello Femoral/patología , Cadera/diagnóstico por imagen , Humanos , Imagen por Resonancia Magnética , Osteoma Osteoide/cirugía , Dolor/etiología , Tomografía Computarizada por Rayos XAsunto(s)
Enfermedad de Hashimoto/diagnóstico , Deficiencia de IgA/diagnóstico , Lupus Eritematoso Sistémico/diagnóstico , Ventrículos Cerebrales/anomalías , Niño , Deleción Cromosómica , Trastornos de los Cromosomas/complicaciones , Trastornos de los Cromosomas/diagnóstico , Cromosomas Humanos Par 18 , Discapacidades del Desarrollo/diagnóstico , Oído Externo/anomalías , Cara/anomalías , Femenino , Enfermedad de Hashimoto/complicaciones , Enfermedad de Hashimoto/congénito , Humanos , Deficiencia de IgA/complicaciones , Deficiencia de IgA/congénito , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/congénitoAsunto(s)
Dolor Abdominal/etiología , Arteritis de Takayasu/complicaciones , Arteritis de Takayasu/diagnóstico , Dolor Abdominal/diagnóstico , Adolescente , Diagnóstico Diferencial , Femenino , Humanos , Imagen por Resonancia Magnética , Índice de Severidad de la Enfermedad , Arteritis de Takayasu/patologíaRESUMEN
A 64-year-old Egyptian man who resides in the United States presented to the rheumatology clinic with 6 months history of episodic recurrent pauci--arthritis along with constitutional symptoms. His Mediterranean ancestry, anti-cyclic citrullinated peptide negativity, and cyclical palindromic rheumatism prompted an investigation for familial Mediterranean fever gene mutation. He was found to have heterozygous 694I gene mutation during MEFV analysis. He also met Liveneh 1 major and 1 minor criteria for the diagnosis of familial Mediterranean fever.
Asunto(s)
Anticuerpos Antiidiotipos/metabolismo , Fiebre Mediterránea Familiar/diagnóstico , Péptidos Cíclicos/inmunología , Artritis Reumatoide/diagnóstico , Artritis Reumatoide/epidemiología , Artritis Reumatoide/inmunología , Comorbilidad , Proteínas del Citoesqueleto/genética , Diagnóstico Diferencial , Fiebre Mediterránea Familiar/epidemiología , Fiebre Mediterránea Familiar/genética , Humanos , Masculino , Persona de Mediana Edad , Mutación/genética , PirinaRESUMEN
This report describes the case of a 29-day-old infant with Kawasaki's disease who presented in extremis with multiple coronary aneurysms and coronary thromboses, myocardial ischemia, and congestive heart failure. The infant successfully underwent emergent coronary thrombolysis with tissue plasminogen activator.
Asunto(s)
Fibrinolíticos/uso terapéutico , Síndrome Mucocutáneo Linfonodular/tratamiento farmacológico , Terapia Trombolítica/métodos , Activador de Tejido Plasminógeno/uso terapéutico , Aspirina/uso terapéutico , Angiografía Coronaria , Trombosis Coronaria/tratamiento farmacológico , Trombosis Coronaria/etiología , Ecocardiografía , Electrocardiografía , Tratamiento de Urgencia , Aneurisma Cardíaco/tratamiento farmacológico , Aneurisma Cardíaco/etiología , Insuficiencia Cardíaca/tratamiento farmacológico , Insuficiencia Cardíaca/etiología , Humanos , Recién Nacido , Masculino , Síndrome Mucocutáneo Linfonodular/complicaciones , Isquemia Miocárdica/tratamiento farmacológico , Isquemia Miocárdica/etiologíaRESUMEN
Sarcoidosis is a multisystemic disease of unknown etiology. Minor renal involvement is not rare but kidney failure is uncommon and only rare cases of recurrent disease in a kidney transplant have been published. We report a patient who at age 10 yr developed ESRD secondary to renal sarcoidosis with GIN. Her disease subsequently recurred in the transplanted kidney despite standard immunosuppression with prednisone, tacrolimus, and mycophenolate mofetil. The recurrent disease appeared to respond to increased immunosuppression, which included infliximab. However, the patient died of disseminated histoplasmosis three yr post-transplant.
Asunto(s)
Trasplante de Riñón , Nefritis Intersticial/cirugía , Sarcoidosis/complicaciones , Adolescente , Resultado Fatal , Femenino , Humanos , Inmunosupresores/uso terapéutico , Fallo Renal Crónico , Nefritis Intersticial/tratamiento farmacológico , Nefritis Intersticial/etiología , Nefritis Intersticial/patología , RecurrenciaAsunto(s)
Enfermedades del Aparato Lagrimal/diagnóstico , Aparato Lagrimal/patología , Neumonía/diagnóstico por imagen , Sarcoidosis/diagnóstico , Biopsia , Niño , Humanos , Enfermedades del Aparato Lagrimal/patología , Masculino , Neumonía/patología , Radiografía , Sarcoidosis/diagnóstico por imagen , Sarcoidosis/patologíaAsunto(s)
Artralgia/diagnóstico , Médula Ósea/patología , Leucemia-Linfoma Linfoblástico de Células Precursoras/diagnóstico , Artralgia/etiología , Biopsia con Aguja , Análisis Químico de la Sangre , Niño , Diagnóstico Diferencial , Femenino , Humanos , Inmunohistoquímica , Dimensión del Dolor , Leucemia-Linfoma Linfoblástico de Células Precursoras/patología , Cintigrafía/métodos , Medición de Riesgo , Índice de Severidad de la EnfermedadRESUMEN
Systemic vasculitis is a group of disorders with multiorgan involvement. These disorders have diverse clinical manifestations associated with significant morbidity and mortality. The most common vasculitides in children--Henoch-Schönlein purpura and Kawasaki disease--are self-limiting conditions. The lifelong and chronic vasculitides (eg, giant cell arteritis, Wegener's granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome, polyarteritis nodosa, and Takayasu arteritis) are rarely seen in children. Therefore, the outcome in general is more favorable in children. This article offers an overview of the epidemiologic, etiologic, pathophysiologic, and clinical features of vasculitis in children, with emphasis on common conditions.