Body Composition and Phase Angle: How to Improve Nutritional Evaluation in Juvenile Dermatomyositis Patients.

(1) Background: This study aimed to assess body composition (BC) using bioelectrical impedance and food intake in juvenile dermatomyositis (JDM) patients. Associations between BC and physical activity, disease activity/cumulative damage and health-related quality of life parameters were also evaluated; (2) Methods: This was a cross-sectional study with 30 consecutive JDM patients (18 female and 12 male) and 24 healthy volunteers (14 female and 10 male) of both sexes followed at our pediatric rheumatology unit. The gathering of anthropometric and dietary data, and the performance of physical activity and bioelectrical impedance were undertaken in face-to-face meetings and through questionnaires. Clinical and therapeutic data were collected from medical records according to information from routine medical appointments; (3) Results: The frequency of high/very high body fat was significantly higher in controls compared with JDM patients (66.7% vs. 91.7%; p = 0.046). The median phase angle was significantly lower in patients compared with controls (5.2 ± 1.3 vs. 6.1 ± 1.0; p = 0.016). Body fat and lean mass were positively correlated with disease duration (rs = +0.629, p < 0.001 and rs = +0.716, p < 0.001, respectively) and phase angle (PhA) (rs = +0.400, p = 0.029 and rs = +0.619, p < 0.001, respectively). JDM patients with PhA ≥ 5.5 presented higher lean mass when compared with patients with PhA < 5.5 (p = 0.001); (4) Conclusions: Bioelectrical impedance can be a useful auxiliary exam in the medical and nutritional follow-up of JDM patients, because it seems to impact functional ability. These findings may assist professionals when advising JDM patients about the importance of physical activity and healthy eating in the preservation of lean mass.

Extensive cervical lymphadenitis mimicking bacterial adenitis as the first presentation of Kawasaki disease / Extensa linfadenite cervical mimetizando adenite bacteriana como primeira manifestação da doença de Kawasaki

Cervical adenitis >1.5cm in diameter is the less frequently observed criteria in patients with Kawasaki disease and it is usually found in association with other symptoms during the acute phase. Moreover, the finding of fever and lymphadenitis with intense local signs of inflammation and phlegmon is rarely seen as the initial manifestation of Kawasaki disease. We report the case of a 7-year-old boy who had cervical lymphadenitis with adjacent cellulitis and phlegmon mimicking bacterial adenitis as the first presentation of Kawasaki disease. The patient had fever, cervical lymphadenitis with adjacent cellulitis, and severe headache. Cefadroxil was prescribed based on the clinical diagnosis of bacterial adenitis. Because he remained febrile and phlogistic signs worsened, after 1 day of hospitalization, antibiotics were administrated intravenously (ceftriaxone and oxacillin). The computed tomography of the neck showed primary infectious/inflammatory process. On the fourth day, the patient had dry and scaly lips, and treatment with oxacillin was replaced by clindamycin because the patient was still febrile. On the ninth day, he presented non-exudative bilateral conjunctival injection. On the tenth day of febrile disease, a rash appeared on his trunk, hands and feet. Patient’s symptoms resolved after intravenous administration of immunoglobulin (2g/kg/dose), and he was discharged 2 days later. On the 14th day, the patient had lamellar desquamation of fingers. Kawasaki disease should be considered as a differential diagnosis in children with febrile cervical lymphadenitis unresponsive to empiric antibiotics even if they have adjacent cellulitis and phlegmon.

Adenite cervical >1,5cm é o critério menos frequentemente observado em pacientes com doença de Kawasaki e manifesta-se habitualmente em associação com os demais sintomas da fase aguda. Entretanto, linfadenite febril isolada com intensos sinais flogísticos e flegmão é raramente observada como primeira manifestação da doença de Kawasaki. Assim, relatou-se aqui um caso de uma criança que apresentou linfadenite cervical com celulite adjacente e flegmão mimetizando adenite bacteriana como primeira manifestação da doença de Kawasaki. Paciente previamente hígido, 7 anos, masculino, iniciou quadro de febre, adenite cervical com celulite adjacente e cefaleia intensa, sendo prescrita cefadroxila devido ao diagnóstico clínico de linfadenite bacteriana. Por se manter febril e com piora dos sinais flogísticos, após 1 dia foi internado para receber antibioticoterapia endovenosa (oxacilina e ceftriaxona). Tomografia computadorizada da região cervical mostrou processo infeccioso/inflamatório primário. No quarto dia, apresentou lábios ressecados e descamativos, sendo a oxacilina substituída por clindamicina devido à persistência da febre e sinais flogísticos. No nono dia, iniciou hiperemia ocular não exsudativa. No décimo dia de febre, apresentou exantema em tronco, membros, mãos e pés. Recebeu gamaglobulina endovenosa (2g/kg/dose), evoluiu com resolução dos sintomas e, após 2 dias, recebeu alta hospitalar. No 14odia, apresentou descamação lamelar dos dedos das mãos. Portanto, doença de Kawasaki deve ser considerada no diagnóstico diferencial das linfadenites cervicais febris na infância não responsivas à antibioticoterapia empírica, mesmo que esteja presentes celulite adjacente e flegmão.

Extensive cervical lymphadenitis mimicking bacterial adenitis as the first presentation of Kawasaki disease.

Cervical adenitis >1.5cm in diameter is the less frequently observed criteria in patients with Kawasaki disease and it is usually found in association with other symptoms during the acute phase. Moreover, the finding of fever and lymphadenitis with intense local signs of inflammation and phlegmon is rarely seen as the initial manifestation of Kawasaki disease. We report the case of a 7-year-old boy who had cervical lymphadenitis with adjacent cellulitis and phlegmon mimicking bacterial adenitis as the first presentation of Kawasaki disease. The patient had fever, cervical lymphadenitis with adjacent cellulitis, and severe headache. Cefadroxil was prescribed based on the clinical diagnosis of bacterial adenitis. Because he remained febrile and phlogistic signs worsened, after 1 day of hospitalization, antibiotics were administrated intravenously (ceftriaxone and oxacillin). The computed tomography of the neck showed primary infectious/inflammatory process. On the fourth day, the patient had dry and scaly lips, and treatment with oxacillin was replaced by clindamycin because the patient was still febrile. On the ninth day, he presented non-exudative bilateral conjunctival injection. On the tenth day of febrile disease, a rash appeared on his trunk, hands and feet. Patient's symptoms resolved after intravenous administration of immunoglobulin (2g/kg/dose), and he was discharged 2 days later. On the 14th day, the patient had lamellar desquamation of fingers. Kawasaki disease should be considered as a differential diagnosis in children with febrile cervical lymphadenitis unresponsive to empiric antibiotics even if they have adjacent cellulitis and phlegmon.

Peripheral polineuropathy in patients with juvenile systemic lupus erythematosus / Polineuropatia periférica em pacientes com lúpus eritematoso sistêmico juvenil

Introdução: A polineuropatia periférica é uma das 19 síndromes neuropsiquiátricas do lúpus eritematoso sistêmico, segundo os critérios de classificação propostos pelo Colégio Americano de Reumatologia (ACR) para síndromes neuropsiquiátricas. No entanto, há descrições raras dessa manifestação, particularmente em populações de lúpus eritematoso sistêmico juvenil (LESJ). Métodos: De 1983 a 2007, 5.079 pacientes foram acompanhados na Unidade de Reumatologia Pediátrica do ICrûHCûFMUSP, e o diagnóstico de LESJ segundo os critérios do ACR foi estabelecido em 228 casos (4,5%). Polineuropatia periférica foi diagnosticada de acordo com as síndromes neuropsiquiátricas do ACR. Resultados: Dos 228 pacientes com LESJ, cinco (2,2%) desenvolveram polineuropatia periférica e foram retrospectivamente descritos. O diagnóstico foi confirmado por eletroneuromiografia, que evidenciou polineuropatia periférica distal, sensitiva e/ou motora, envolvendo quatro membros em dois pacientes e membros inferiores nos demais. Três eram pacientes do sexo feminino e todos tiveram a polineuropatia periférica após o diagnóstico de LESJ. A mediana de idade de início da doença foi de 14 anos, e a mediana de tempo entre o início de LESJ e o diagnóstico da polineuropatia periférica foi de 23 meses. As apresentações clínicas mais comuns foram fraqueza muscular e hiporreflexia. Todos os pacientes apresentavam anticorpos antifosfolípides. O tratamento foi realizado com corticosteroides em todos os pacientes, associado com ciclofosfamida endovenosa em três. Um paciente evoluiu com incapacitação funcional, presença de paresia de membros inferiores e necessidade de cadeira de rodas. Uma paciente faleceu por sepse grave. Conclusões: A polineuropatia periférica é uma manifestação rara no LESJ, grave, por vezes incapacitante e habitualmente associada a anticorpos antifosfolípides.

INTRODUCTION: Peripheral polyneuropathy is one of 19 neuropsychiatric syndromes seen in systemic lupus erythematosus, according to the classification criteria proposed by the American College of Rheumatology (ACR) for neuropsychiatric syndromes. However, this manifestation has not been reported very often, especially in patients with juvenile systemic lupus erythematosus (JSLE). PATIENTS AND METHODS: From 1983 to 2007, 5,079 patients were seen at the Pediatric Rheumatology Unit of the ICr-HC-FMUSP; 228 (4.5%) patients were diagnosed with JSLE according to the criteria of the ACR. Peripheral polyneuropathy was diagnosed according to the criteria for neuropsychiatric syndromes of the ACR. RESULTS: Five (2.2%) out of 228 patients with JSLE developed peripheral polyneuropathy and were described retrospectively. The diagnosis was confirmed by electroneuromyography, which showed the presence of distal peripheral polyneuropathy, sensorial and/or motor, involving all four limbs, in two patients, and the lower limbs, in three patients. Three of those patients were females, and peripheral neuropathy developed after the diagnosis of JSLE. The mean age of onset of the disease was 14 years, and the mean time between the onset of JSLE and the diagnosis of peripheral polyneuropathy was 23 months. The most common clinical presentations included muscular weakness and hyporeflexia. Antiphospholipid antibodies were present in all patients. Treatment consisted of corticosteroids in all patients, associated with intravenous cyclophosphamide in three patients. One patient evolved to functional disability and paresis of the lower limbs, requiring a wheelchair. One female patient died of severe sepsis. CONCLUSIONS: Peripheral polyneuropathy is a rare, severe, and occasionally incapacitating manifestation of JSLE, commonly associated with the presence of antiphospholipid antibodies.

[Musculoskeletal involvement as a first manifestation of neoplasm disease]. / Comprometimento musculoesquelético como primeira manifestação de neoplasias.

OBJECTIVE: To describe the clinical and laboratory findings that contributed for the diagnosis of neoplasia in patients with musculoskeletal symptoms at presentation. METHODS: Retrospective analysis of medical records from patients with final diagnosis of neoplasia attended at the "Unidade de Reumatologia do Instituto da Criança - FMUSP" between January 1983 and December 2006. Data on musculoskeletal complaints, clinical examination, laboratory tests, radiological studies and diagnostic procedures were obtained. RESULTS: From 4876 patients, 25 (0.5%) children were studied (52% with acute lymphoid leukemia and 24% with neuroblastoma). Twenty children (80%) presented arthritis and/or arthralgia at onset of the disease. All patients presented systemic symptoms, such as fever (22 cases - 88%). The initial blood cell count was abnormal in 16 patients (64%), showing anemia and thrombocytopenia (12 and 5 cases, respectively). Blast cells were present in only two patients and eleven patients developed blood cell count abnormalities during follow-up. X-ray studies showed abnormalities in 11/14 patients, ultrasound in 12/18, scintigraphy in 5/5, CT in 7/9 and MRI in 3/3. Bone marrow smear was abnormal in 18/22 patients, but in three of them the abnormalities were not detected by the first test. CONCLUSION: Musculoskeletal symptoms are common at the onset of neoplasia, especially for acute lymphoid leukemia, and this possibility should be considered in the differential diagnosis of rheumatic diseases. Laboratory tests may be normal at the onset of the disease, therefore serial exams should be performed. For a correct diagnosis. radiological studies and bone marrow aspiration have proven to be essential.

Comprometimento musculoesquelético como primeira manifestação de neoplasias / Musculoskeletal involvement as a first manifestation of neoplasm disease

OBJETIVO: Identificar pacientes portadores de neoplasias encaminhados ao ambulatório de reumatologia pediátrica por apresentarem sintomas musculoesqueléticos como manifestação inicial, bem como apontar os achados clínicos, laboratoriais e radiológicos que contribuíram para o esclarecimento diagnóstico. MÉTODOS: Foi realizada análise retrospectiva dos prontuários dos pacientes com diagnóstico final de neoplasia, que foram avaliados na Unidade de Reumatologia do Instituto da Criança - FMUSP entre janeiro de 1983 e dezembro de 2006. Foram registrados os dados referentes às queixas musculoesqueléticas, exame físico, provas laboratoriais e procedimentos radiológicos e invasivos realizados. RESULTADOS: Dos 4876 pacientes, 25 (0,5 por cento) crianças foram estudadas, sendo que 52 por cento apresentavam leucemia linfóide aguda - LLA e 24 por cento neuroblastoma. Vinte crianças (80 por cento) apresentaram artrite e/ou artralgia no início da doença. Todos os pacientes apresentavam sintomas constitucionais, sendo febre o mais prevalente (22 casos - 88 por cento). O hemograma inicial estava alterado em 16 pacientes (64 por cento), mas presença de blastos no sangue periférico foi observada em apenas dois pacientes. Onze pacientes demonstraram alterações evolutivas nos hemogramas seriados. Radiografias simples se mostraram alteradas em 11/14 pacientes, ultra-som em 12/18, cintilografia em 5/5, tomografia em 7/9 e ressonância em 3/3. O mielograma estava alterado em 18/22 pacientes, três deles apenas na repetição do exame. CONCLUSÃO: Queixas musculoesqueléticas são manifestações iniciais freqüentes das neoplasias, em especial da LLA, que devem ser consideradas no diagnóstico diferencial das doenças reumatológicas. Os hemogramas podem ser inicialmente normais, sendo necessário seu seguimento evolutivo. Exames de imagem e punção de medula óssea demonstraram ser fundamentais no diagnóstico.

OBJECTIVE: To describe the clinical and laboratory findings that contributed for the diagnosis of neoplasia in patients with musculoskeletal symptoms at presentation. METHODS: Retrospective analysis of medical records from patients with final diagnosis of neoplasia attended at the "Unidade de Reumatologia do Instituto da Criança - FMUSP" between January 1983 and December 2006. Data on musculoskeletal complaints, clinical examination, laboratory tests, radiological studies and diagnostic procedures were obtained. RESULTS: From 4876 patients, 25 (0.5 percent) children were studied (52 percent with acute lymphoid leukemia and 24 percent with neuroblastoma). Twenty children (80 percent) presented arthritis and/or arthralgia at onset of the disease. All patients presented systemic symptoms, such as fever (22 cases - 88 percent). The initial blood cell count was abnormal in 16 patients (64 percent), showing anemia and thrombocytopenia (12 and 5 cases, respectively). Blast cells were present in only two patients and eleven patients developed blood cell count abnormalities during follow-up. X-ray studies showed abnormalities in 11/14 patients, ultrasound in 12/18, scintigraphy in 5/5, CT in 7/9 and MRI in 3/3. Bone marrow smear was abnormal in 18/22 patients, but in three of them the abnormalities were not detected by the first test. CONCLUSION: Musculoskeletal symptoms are common at the onset of neoplasia, especially for acute lymphoid leukemia, and this possibility should be considered in the differential diagnosis of rheumatic diseases. Laboratory tests may be normal at the onset of the disease, therefore serial exams should be performed. For a correct diagnosis. radiological studies and bone marrow aspiration have proven to be essential.

Anticorpos antinucleossomo em pacientes com lúpus eritematoso sistêmico juvenil / Antinucleosome antibodies in patients with juvenile systemic lupus erythematosus

Este estudo teve por objetivos avaliar a positividade do anticorpo antinucleossomo (anti-Ncs) no LES juvenil, sua associação com manifestações e atividade da doença e compará-lo ao anticorpo anti-DNA. A pesquisa dos anticorpos anti-Ncs e anti-DNA foi realizada em 74 pacientes com LESJ e 64 controles. Os anti-Ncs e anti-DNA demonstraram sensibilidade / The aims of this study were to evaluate the positivity of antinucleosome antibodies (Anti-Ncs) in SLE children, their association to disease manifestations and activity, and to compare them to anti-DNA antibodies. Anti-Ncs and anti-DNA were tested on 74 JSLE patients and 64 controls...

Antiphospholipid antibodies in 57 children and adolescents with systemic lupus erythematosus.

OBJECTIVE: To investigate the frequencies and behavior of antiphospholipid antibodies in 57 children and adolescents with systemic lupus erythematosus. METHODS: Anticardiolipin antibodies were investigated by ELISA and lupus anticoagulant antibodies by the international tests recommended. The antiphospholipid antibodies analyses were performed in frozen samples (mean of 5.3 samples per patient obtained during a mean follow-up period of 3 years and 7 months) and on blood samples collected between January 1997 and November 1998 (mean of 2.5 samples per patient during a 2-year follow-up period). RESULTS: The frequencies of antiphospholipid antibodies (anticardiolipin and lupus anticoagulant) were similar in the samples collected prospectively and in the frozen samples (retrospective study): 63.2% and 75.4% respectively. Positivity for these antibodies fluctuated during the follow-up period and was not associated with any clinical or laboratory parameters of lupus erythematosus, including autoantibodies and also including disease activity and/or severity scores. CONCLUSIONS: The frequencies of antiphospholipid antibodies in children and adolescents with lupus erythematosus were similar to those observed in adults. The positivity fluctuated during the follow-up and was not correlated with clinical and/or laboratory disease parameters.

Antiphospholipid antibodies in 57 children and adolescents with systemic lupus erythematosus

OBJECTIVE: To investigate the frequencies and behavior of antiphospholipid antibodies in 57 children and adolescents with systemic lupus erythematosus. METHODS: Anticardiolipin antibodies were investigated by ELISA and lupus anticoagulant antibodies by the international tests recommended. The antiphospholipid antibodies analyses were performed in frozen samples (mean of 5.3 samples per patient obtained during a mean follow-up period of 3 years and 7 months) and on blood samples collected between January 1997 and November 1998 (mean of 2.5 samples per patient during a 2-year follow-up period). RESULTS: The frequencies of antiphospholipid antibodies (anticardiolipin and lupus anticoagulant) were similar in the samples collected prospectively and in the frozen samples (retrospective study): 63.2 percent and 75.4 percent respectively. Positivity for these antibodies fluctuated during the follow-up period and was not associated with any clinical or laboratory parameters of lupus erythematosus, including autoantibodies and also including disease activity and/or severity scores. CONCLUSIONS: The frequencies of antiphospholipid antibodies in children and adolescents with lupus erythematosus were similar to those observed in adults. The positivity fluctuated during the follow-up and was not correlated with clinical and/or laboratory disease parameters