Maria Odete Esteves Hilário

EULAR/PRINTO/PRES criteria for Henoch–Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria

Objectives To validate the previously proposed classification criteria for Henoch–Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA). Methods Step 1: retrospective/prospective web-data collection for children with HSP, c-PAN, c-WG and c-TA with age at diagnosis ≤18 years. Step 2: blinded classification by consensus panel of a representative sample of 280 cases. Step 3: statistical (sensitivity, specificity, area under the curve and κ-agreement) and nominal group technique consensus evaluations. Results 827 patients with HSP, 150 with c-PAN, 60 with c-WG, 87 with c-TA and 52 with c-other were compared with each other. A patient was classified as HSP in the presence of purpura or petechiae (mandatory) with lower limb predominance plus one of four criteria: (1) abdominal pain; (2) histopathology (IgA); (3) arthritis or arthralgia; (4) renal involvement. Classification of c-PAN required a systemic inflammatory disease with evidence of necrotising vasculitis OR angiographic abnormalities of medium-/small-sized arteries (mandatory criterion) plus one of five criteria: (1) skin involvement; (2) myalgia/muscle tenderness; (3) hypertension; (4) peripheral neuropathy; (5) renal involvement. Classification of c-WG required three of six criteria: (1) histopathological evidence of granulomatous inflammation; (2) upper airway involvement; (3) laryngo-tracheo-bronchial involvement; (4) pulmonary involvement (x-ray/CT); (5) antineutrophilic cytoplasmic antibody positivity; (6) renal involvement. Classification of c-TA required typical angiographic abnormalities of the aorta or its main branches and pulmonary arteries (mandatory criterion) plus one of five criteria: (1) pulse deficit or claudication; (2) blood pressure discrepancy in any limb; (3) bruits; (4) hypertension; (5) elevated acute phase reactant. Conclusion European League Against Rheumatism/Paediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society propose validated classification criteria for HSP, c-PAN, c-WG and c-TA with high sensitivity/specificity.

Percepção e satisfação corporal em adolescentes e a relação com seu estado nutricional

Background: The self-perception of and the satisfaction with their body image are determinant factors for the self-acceptance of the adolescents, and may generate inadequate attitudes that impair their growth and development. Objective: To investigate the association between nutritional state in adolescents and the self-perception of the satisfaction with body image. Methods: We obtained body mass index (BMI) through anthropometric data in order to classify the nutritional state of the adolescents. We also used standardized silhouettes to evaluate their self-perception of their body image, and a self-completing questionnaire to determine their satisfaction with their body image. Results: Most of the population was eutrophic. The girls, however, did not regard themselves that way. Even Perception and satisfaction with body image in adolescents and correlations with nutrition status

Quality of life of children and adolescents from São Paulo: reliability and validity of the Brazilian version of the Pediatric Quality of Life InventoryTM version 4.0 Generic Core Scales

OBJECTIVES To evaluate the reliability and validity of the Brazilian version of the Pediatric Quality of Life Inventory (PedsQL 4.0) Generic Core Scales and measure the quality of life of healthy children and adolescents and patients with rheumatic diseases. METHODS We followed the translation methodology proposed by the developer of the original English version of the PedsQL 4.0. The instrument was administered by interviews in two groups: 240 apparently healthy children and adolescents from São Paulo (SP, Brazil) and 105 patients with chronic rheumatic diseases matched by age, as well as their respective parents or caregivers. The parent proxy-report was administered to the childrens parents or caregivers separately on the same day. RESULTS Cronbachs alpha values were between 0.6 and 0.9 for all dimensions, demonstrating adequate internal consistency. Patients with rheumatic diseases reported significantly lower PedsQL scores on all dimensions when compared to the healthy control group (p < 0.0001). Construct validity of the Brazilian Portuguese version of the PedsQL 4.0 was also confirmed. Parent proxy-report of patients with rheumatic diseases highly correlated with child self-report for physical functioning (r = 0.77, p < 0.001) and school functioning (r = 0.73, p < 0.001). Lower correlations were observed for emotional and social functioning (r = 0.40 and 0.59, respectively, p < 0.001). CONCLUSIONS The tool demonstrated reliability, validity, and the administration was fast and easy. Quality of life in patients with rheumatic diseases was significantly lower than in the healthy control group, supporting the necessity of a comprehensive approach to rheumatic disease management, focused on the psychosocial dimensions.

Qualidade de vida de crianças e adolescentes de São Paulo: confiabilidade e validade da versão brasileira do questionário genérico Pediatric Quality of Life InventoryTM versão 4.0

OBJECTIVES: To evaluate the reliability and validity of the Brazilian version of the Pediatric Quality of Life InventoryTM (PedsQLTM 4.0) Generic Core Scales and measure the quality of life of healthy children and adolescents and patients with rheumatic diseases. METHODS: We followed the translation methodology proposed by the developer of the original English version of the PedsQLTM 4.0. The instrument was administered by interviews in two groups: 240 apparently healthy children and adolescents from Sao Paulo (SP, Brazil) and 105 patients with chronic rheumatic diseases, matched by age, as well as to their respective parents or caregivers. The parent proxy-report was administered to the childrens parents or caregivers separately on the same day. RESULTS: Cronbachs alpha values were between 0.6 and 0.9 for all dimensions, demonstrating adequate internal consistency. Patients with rheumatic diseases reported significantly lower PedsQLTM scores on all dimensions when compared to the healthy control group (p < 0.0001). Construct validity of the Brazilian Portuguese version of the PedsQLTM 4.0 was also confirmed. Parent proxy-report of patients with rheumatic diseases highly correlated with child self-report for physical functioning (r = 0.77, p < 0.001) and school functioning (r = 0.73, p < 0.001). Lower correlations were observed for emotional and social functioning (r = 0.40 and 0.59, respectively, p < 0.001). CONCLUSIONS: The tool demonstrated reliability, validity and the administration was fast and easy. Quality of life of patients with rheumatic diseases was significantly lower than the healthy control group, supporting the necessity of a comprehensive approach to rheumatic disease management, focused on the psychosocial dimensions.

Symptomatic cardiac involvement in juvenile rheumatoid arthritis

In a retrospective study of 172 patients with juvenile rheumatoid arthritis, symptomatic cardiac involvement occurred in 13 (7.6%) patients (11 systemic and 2 polyarticular). There was predominance of the male sex and in most patients the involvement occurred in the initial years of the disease. Pericarditis occurred in seven patients; perimyocarditis in four and myocarditis in two patients. In the follow-up, one of the patients with pericarditis died of an arrhythmia during pericardiocentesis for cardiac tamponade. Among the patients with myocarditis, three died of septicemia during active disease. One of these three patients had myocarditis associated with cardiac tamponade. Among the 172 patients with juvenile rheumatoid arthritis, five children died; four belonged to the symptomatic cardiac involvement group (P less than 0.001). Cardiac involvement, in particular myocarditis and cardiac tamponade, can be regarded as a factor of worse prognosis.

Frequency of Antinuclear Antibodies in Healthy Children and Adolescents

Sera from 214 healthy children and adolescents (108 females [50.4%]) aged 6 months to 20 years (mean 8.7 years) and from 116 patients with rheumatic diseases were assayed for antinuclear antibody (ANA) by indirect immunofluorescence (IIF) by using HEp-2 cells as substrate. Twenty-seven healthy children (12.6%) presented a positive ANA test; there was no difference between genders, and we observed a trend for higher frequency of ANA ≥1/80 among children between 5 years and 10 years. Eight of the 27 healthy children with positive ANA test were reevaluated 36 months later, and none of them had developed any rheumatic disease, though the sera remained positive in 2 of them. ANA was present in 42/116 patients (36.2%). In daily medical practice ANA determination should be required only in individuals with clinical signs and symptoms suggestive of autoimmune disease.

Musculoskeletal manifestations as the onset of acute leukemias in childhood

OBJECTIVE To study the frequency, the clinical features and laboratory exams of patients with musculoskeletal symptoms at the onset of acute leukemia. METHODS Retrospective, descriptive study including patients diagnosed with acute leukemia, and treated at the Institute of Pediatric Oncology of UNIFESP, carried out from November 1999 to February 2000. The data on musculoskeletal complaints were obtained from a questionnaire. The medical records were revised in order to get data on the clinical examination and laboratory tests at the beginning of the illness. RESULTS Sixty-one children were included in this trial, 93% with acute lymphoid leukemia, and 7% with acute myeloid leukemia. Thirty-eight patients (62%) had musculoskeletal pain at the onset. Arthritis was observed in eight patients. The mean number of involved joints was 2.5 (1-6). The most frequently involved joints were the knees, ankles and elbows. Three patients (4.9%) had normal blood count. Low hemoglobin was reported in 54 patients (88%) (in six patients it was the only hematological abnormality), leukopenia in 14 (22%), leukocytosis in 26 (42%) and low platelet count in 46 (75%). CONCLUSIONS The musculoskeletal symptoms are common at the onset of acute leukemia so, malignancy should always be ruled out in patients presenting chronic or acute arthritis or benign limb pain. The laboratory tests may be normal at the onset of the illness, making differential diagnosis more difficult.

Nonsteroidal anti-inflammatory drugs: cyclooxygenase 2 inhibitors

OBJECTIVES To analyze selective COX 2 inhibitor nonsteroidal anti-inflammatory drugs (NSAID) in terms of their mechanism of action, principal indications, posology and most common adverse effects. SOURCES MEDLINE and LILACS databases and Food and Drug Administration (FDA) and National Agency for Sanitary Vigilance (ANVISA - Agência Nacional de Vigilância Sanitária) websites. The most important articles were selected and preference was given to articles published within the last 5 years. SUMMARY OF THE FINDINGS The principal indications for NSAID are for control of pain and acute and chronic inflammation. There is no overwhelming evidence that demonstrates the superiority of one NSAID over another in terms of effectiveness. To date none of the COX 2 inhibitors has been liberated for use in the pediatric age group. Only meloxicam and etoricoxib can be prescribed for adolescents (13 and 16 years, respectively). Selective COX 2 inhibitors are indicated for patients with adverse effects that have proven to be associated with nonselective NSAID use. Selective COX 2 inhibitors can be prescribed in some cases of allergy to aspirin, but they must be used with care. Principal adverse effects include cardiovascular events and thrombotic phenomena. CONCLUSIONS Selective COX 2 inhibitors are medicines that have been used in certain well-defined clinical situations and which may offer certain advantages over nonselective NSAID. Nevertheless, taking into consideration the higher cost involved and the potential for adverse cardiovascular effects, they should be employed only in accordance with strict criteria.

Diagnosis of malignancies in children with musculoskeletal complaints

CONTEXT Musculoskeletal complaints may be associated with neoplasias as an initial manifestation of the disease. When these symptoms predominate at the onset of the disease, the differential diagnosis includes several rheumatic diseases. OBJECTIVE To assess the frequency, clinical features and types of cancer manifested in children presenting with musculoskeletal complaints over a seven-year period. TYPE OF STUDY Retrospective. SETTING Discipline of Allergy, Clinical Immunology and Rheumatology, Universidade Federal de São Paulo-Escola Paulista de Medicina. METHODS The medical records of patients with musculoskeletal complaints and final diagnosis of malignant disease were reviewed. The data collected were: age when symptoms initially presented, age at diagnosis, clinical features presented, laboratory findings, and the initial and final diagnoses. RESULTS A final diagnosis of cancer was found in nine out of 3,528 patients (0.25%) whose initial symptom was musculoskeletal pain. The mean time between disease onset and final diagnosis was five months. The most common features presented were pauciarticular arthritis or arthralgia involving the large joints. Juvenile rheumatoid arthritis was the most frequent initial diagnosis, in four out of nine patients. Anemia was the most frequent initial hematological change. Six out of eight patients had an increased erythrocyte sedimentation rate. The lactate dehydrogenase level was raised in five out of eight patients. The malignancies found included acute lymphocytic leukemia, acute myeloid leukemia, lymphoma, neuroblastoma and Ewings sarcoma. DISCUSSION The frequency of neoplasia in patients with musculoskeletal pain resembled reports in the literature. Consumptive symptoms were not the warning signal in most of our patients. In subsidiary tests, progressive anemia was the most common finding, although the peripheral blood cell count may continue to be normal for weeks or months after symptom onset. CONCLUSION Malignancy always needs to be ruled out in cases of children with musculoskeletal complaints. Uncharacteristic clinical manifestations and nonspecific laboratory tests may cause difficulty in the final diagnosis, and rigorous investigation should be performed.

Juvenile idiopathic inflammatory myopathies: the value of magnetic resonance imaging in the detection of muscle involvement

CONTEXT One of the major current challenges related to juvenile idiopathic inflammatory myopathy is the search for highly sensitive and specific non-invasive methods for diagnosis as well as for follow-up. OBJECTIVES The aim of our study was to describe typical magnetic resonance imaging findings and to investigate the usefulness of this method in detecting active muscle disease in juvenile dermatomyositis and juvenile systemic lupus erythematosus patients. DESIGN Transverse study, blinded assessment. SETTING University referral unit (Pediatric Rheumatology section, Department of Pediatrics, Universidade Federal de São Paulo / Escola Paulista de Medicina). SAMPLE Thirteen patients (9 girls) with dermatomyositis, as well as 13 patients (12 girls) with juvenile systemic lupus erythematosus and 10 normal children (5 girls), were enrolled in the study. MAIN MEASUREMENTS Qualitative and quantitative analyses of gluteus maximus, quadriceps, adductors and flexors were performed and evaluated by two radiologists, blinded to all clinical information. Spin-echo in T1, DP, T2 and IR was used in all MRI images. RESULTS The different muscle groups presented non-uniform involvement in the patients. The patients with dermatomyositis presented acute and chronic muscular alterations, while those with lupus presented only chronic myopathy, especially atrophy. In the dermatomyositis group, the major alterations were found in the gluteus and flexor regions (signal intensity and fat replacement). The signal intensity was increased in all acute myopathies. CONCLUSION The qualitative and quantitative resonance analyses are useful in detecting clinically active disease in patients with dermatomyositis.