RESUMOIntrodução: a infância é um período de grande importância na formação do ser humano, logo, um episódio de doença pode interromper esse processo, além de interferir na estrutura familiar e gerar ônus ao sistema de saúde. A epidemiologia e a morbidade atuam como importantes instrumentos, pois promovem o conhecimento em saúde e auxiliam na distribuição dos pediatria. Metodologia: estudo realizado na enfermaria de pediatria do Hospital Universitário Walter Cantídio (HUWC) com amostra de 119 registros, referentes aos meses de agosto a dezembro de 2015. Resultados: a idade média é de 8,6 anos, sendo que no sexo feminino houve predomínio na faixa da adolescência. Os 3 sistemas mais acometidos foram: sistema digestivo, sistema 48,1% dos registros, sendo o Diabetes Mellitus a mais comum. 25,2% dos casos possuíam internamentos prévios, em sua grande maioria portadores de patologias crônicas. A grande maioria das altas ocorreram para domicílio. Discussão e Conclusão: o estudo demonstra particularidades da enfermaria de pediatria do HUWC, sendo possível guiar o ensino de residentes e estudantes, além de auxiliar na formação de políticas, melhoria do atendimento e preparo da equipe ligada aos cuidados com o paciente.Palavras-chave: Epidemiologia. Pediatria. Internação hospitalar. ABSTRACTIntroduction: childhood is a very important period in the formation of the human being, thus, an episode of illness can interrupt this process, in addition to interfering in family structure and generate cost to the health system. The epidemiology and morbidity act as important tools for promoting health knowledge and assist in the distribution of resources, reaching the various administrative
BACKGROUNDJuvenile localized scleroderma (JLS), also known as morphea, is a spectrum of rare inflammatory pediatric disorders associated with skin and underlying tissue thickening and sclerosis. Clinical manifestations range from skin lesions to extracutaneous involvement that often vary with its subtype. Early recognition is important to reduce disease-related functional impact. METHODSReview of medical records of children and adolescents with diagnosis of JLS from 2015 to May 2022 and regularly followed up in the Pediatric Rheumatology Unit of a tertiary hospital in the state of Ceará was performed. Data included demographics, clinical manifestations, treatment and disease course. RESULTSTwenty patients were included and 13 (65%) were female. The median age at onset, age at diagnosis and time elapsed from symptom onset to diagnosis were, respectively, 6.60 (2-16.4), 9.05 (3.2-17) and 2.45 (0-10.5) years. The most prevalent subtype was mixed morphea (35%), followed by linear form (25%). Involvement was predominantly appendicular, distributing in upper and/or lower limbs in 75%. Five patients had disease restricted to the face, two with en coup de sabre variant and three with Parry-Romberg syndrome. Most of the patients (75%) already had characteristics of long-standing skin disease at diagnosis. Seventeen patients (85%) had extracutaneous manifestations, with a predominance of joint involvement (arthritis/arthralgia of small joints of the hands, wrists and knees) in 13 (65%) of them, followed by gastrointestinal tract (20%). Autoantibodies were positive in 9 (45%) patients, of which ANA (78%) and RF (22%) were the most prevalent. Eighteen patients used corticosteroids at diagnosis, with an average of 8.5 months treatment, and a partial response in 15 (83.3%) of them. Methotrexate was associated in 19 cases, allowing 12 (63.1%) patients to achieve remission. In refractory cases (35%), six received mycophenolate mofetil and two cyclophosphamide. After approximately 3 years of follow-up, 85% of patients had good disease control. CONCLUSIONThe female-to-male ratio (1.85) and the age at onset of symptoms (6.60) are in line with literature (1.7:1 to 3.7:1 and 6.1 to 8.1, respectively). Our study differs in the higher prevalence of mixed morphea subtype in relation to the linear one. Time until diagnosis was long, with most patients presenting already with chronic skin changes, warning about the lack of knowledge about the disease. Immunosuppressive standard treatment, mainly with methotrexate and corticosteroids, shows promising results in controlling the disease progression.
BACKGROUNDLeprosy, a chronic infectious disease caused by Mycobacterium leprae, affects the skin, peripheral nerves, upper respiratory tract, musculoskeletal system and eyes. As it presents a wide spectrum of clinical manifestations, it can be a diagnostic challenge, especially in early stages of disease. Some of these manifestations resemble pictures of rheumatic diseases that affect adults and children. Among the musculoskeletal manifestations of childhood leprosy are inflammatory chronic arthritis, mimicking juvenile idiopathic arthritis or spondyloarthritis, inflammatory swelling of the hands and feet, neuropathic arthritis, septic arthritis, arthralgias/ myalgias, soft tissue rheumatism and multisystem involvement similar to collagenases, including vasculitis and myositis. CASE REPORTWe report the case of a previously healthy 8-year-old boy admitted to the pediatric rheumatology unity due to the presence of puffy hands and fingers and hard and shiny edema of the lower limbs, suggesting systemic scleroderma diagnosis. He had an 11-month history of recurrent episodes of intermittent fever, plaque-like, erythematous, nonpruritic facial skin lesions and joint pain in the knees and ankles. On physical examination, he presented, in addition to the aforementioned findings, purpuric lesions on the toes, hepatosplenomegaly, arthralgias (wrist, small joints of the hands, knees and ankles) and erythematous plaques infiltrated in the bilateral malar region, nasal region and auricular pavilion. Complementary tests showed normocytic and normochromic anemia, leukocytosis, neutrophilia, thrombocytosis, elevation of inflammatory tests and negativity of ANA, RF, ANCA, anti-RNP and anti-Scl70. Due to the infiltrated skin lesions, lymph smear testing was performed, which was strongly positive, and a diagnosis of Virchowian leprosy associated with mixed leprosy reaction was made. There was clinical and laboratory improvement after initiation of multidrug therapy and systemic corticosteroid therapy. CONCLUSIONLeprosy is known as a great mimic of rheumatic diseases, often fulfilling diagnostic criteria for many of them. The multibacillary forms present greater musculoskeletal involvement. It should be considered in differential diagnosis of children with musculoskeletal symptoms, autoantibody positivity and cutaneous and/or neurological involvement.
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