Whole-body magnetic resonance imaging in the assessment of muscular involvement in juvenile dermatomyositis/polymyositis patients.

OBJECTIVES: Our aim was to demonstrate the benefit of whole-body magnetic resonance imaging (WBMRI) as a diagnostic modality in the detection of muscle activity in juvenile dermatomyositis (JDM)/polymyositis (JPM) patients and to correlate these findings with clinical evaluation, laboratory examinations, nailfold capillaroscopy (NFC), and muscle biopsy. METHOD: Thirty-four patients aged 5.5 to 18.9 years with a diagnosis of JDM/JPM were prospectively evaluated using clinical examination, muscle enzyme determination, the Childhood Myositis Assessment Scale (CMAS), Disease Activity Score (DAS), Manual Muscle Testing (MMT), NFC, and WBMRI. An open muscle biopsy was performed if muscle disease activity was detected on WBMRI. RESULTS: Disease activity was detected in WBMRI in four (11.7%) patients and confirmed by muscle biopsy. All four patients had elevation of at least one muscle enzyme and NFC showed scleroderma patterns in these patients. CONCLUSIONS: WBMRI allows us to evaluate the extent and symmetry of muscle disease and inflammatory activity. NFC is an important additional examination to assess disease activity.

Low serum concentrations of 25-hydroxyvitamin D in children and adolescents with systemic lupus erythematosus

We evaluated the concentrations of 25-hydroxyvitamin D [25(OH)D] in children and adolescents with juvenile systemic lupus erythematosus (JSLE) and associated them with disease duration and activity, use of medication (chloroquine and glucocorticoids), vitamin D intake, calcium and alkaline phosphatase levels, and bone mineral density. Thirty patients with JSLE were evaluated and compared to 30 healthy individuals, who were age and gender matched. Assessment was performed of clinical status, disease activity, anthropometry, laboratory markers, and bone mineral density. The 30 patients included 25 (83.3%) females and 16 (53.3%) Caucasians, with a mean age of 13.7 years. The mean age at diagnosis was 10.5 years and mean disease duration was 3.4 years. Mean levels of calcium, albumin, and alkaline phosphatase were significantly lower in patients with JSLE compared with controls (P<0.001, P=0.006, and P<0.001, respectively). Twenty-nine patients (97%) and 23 controls (77%) had 25(OH)D concentrations lower than 32 ng/mL, with significant differences between them (P<0.001). Fifteen patients (50%) had vitamin D levels <20 ng/mL and 14 had vitamin D levels between 20 and 32 ng/mL. However, these values were not associated with greater disease activity, higher levels of parathormone, medication intake, or bone mineral density. Vitamin D concentrations were similar with regard to ethnic group, body mass index, height for age, and pubertal stage. Significantly more frequently than in controls, we observed insufficient serum concentrations of 25(OH)D in patients with JSLE; however, we did not observe any association with disease activity, higher levels of parathormone, lower levels of alkaline phosphatase, use of medications, or bone mineral density alterations.

Low serum concentrations of 25-hydroxyvitamin D in children and adolescents with systemic lupus erythematosus.

We evaluated the concentrations of 25-hydroxyvitamin D [25(OH)D] in children and adolescents with juvenile systemic lupus erythematosus (JSLE) and associated them with disease duration and activity, use of medication (chloroquine and glucocorticoids), vitamin D intake, calcium and alkaline phosphatase levels, and bone mineral density. Thirty patients with JSLE were evaluated and compared to 30 healthy individuals, who were age and gender matched. Assessment was performed of clinical status, disease activity, anthropometry, laboratory markers, and bone mineral density. The 30 patients included 25 (83.3%) females and 16 (53.3%) Caucasians, with a mean age of 13.7 years. The mean age at diagnosis was 10.5 years and mean disease duration was 3.4 years. Mean levels of calcium, albumin, and alkaline phosphatase were significantly lower in patients with JSLE compared with controls (P<0.001, P=0.006, and P<0.001, respectively). Twenty-nine patients (97%) and 23 controls (77%) had 25(OH)D concentrations lower than 32 ng/mL, with significant differences between them (P<0.001). Fifteen patients (50%) had vitamin D levels <20 ng/mL and 14 had vitamin D levels between 20 and 32 ng/mL. However, these values were not associated with greater disease activity, higher levels of parathormone, medication intake, or bone mineral density. Vitamin D concentrations were similar with regard to ethnic group, body mass index, height for age, and pubertal stage. Significantly more frequently than in controls, we observed insufficient serum concentrations of 25(OH)D in patients with JSLE; however, we did not observe any association with disease activity, higher levels of parathormone, lower levels of alkaline phosphatase, use of medications, or bone mineral density alterations.

Serum 25-hydroxyvitamin D and biochemical markers of bone metabolism in patients with juvenile idiopathic arthritis.

Our objective was to evaluate the concentrations of serum 25-hydroxyvitamin D [25(OH)D], serum calcium, serum phosphorus, alkaline phosphatase, and parathormone (PTH) in patients with polyarticular juvenile idiopathic arthritis (JIA) and to associate them with disease duration and activity, bone mineral density and use of medications. In a cross-sectional and controlled study, 30 patients with polyarticular JIA were evaluated and compared to 30 healthy individuals matched for age and gender. Clinical status, anthropometry, laboratory markers in both patients and controls, and bone mineral density, only in the patients, were measured. Of the 30 patients included in the study, 23 (76.7%) were female and 16 (53.3%) non-Caucasian; mean age was 14 years (range = 4 to 20 years). Mean disease duration was 5 years (range = 1 to 12 years). The mean concentrations of serum albumin-corrected calcium (9.04 ± 0.41 mg/dL) and alkaline phosphatase (153.3 ± 100.1 IU) were significantly lower in patients with JIA than in controls (P < 0.0001 and P = 0.001, respectively). No differences in 25(OH)D, PTH or serum phosphorus were observed between JIA and control subjects. Regarding 25(OH)D concentration, 8 patients (26.7%) and 5 controls (16.7%) had 25(OH)D concentrations compatible with deficiency (lower than 20 ng/mL) and 14 patients (46.7%) and 18 controls (60%) had concentrations compatible with insufficiency (20-32 ng/mL). These values were not associated with disease activity, use of medications or bone mineral density. We observed a high frequency of 25(OH)D insufficiency and deficiency in the study sample. The compromised bone metabolism emphasizes the importance of follow-up of JIA patients.

Serum 25-hydroxyvitamin D and biochemical markers of bone metabolism in patients with juvenile idiopathic arthritis

Our objective was to evaluate the concentrations of serum 25-hydroxyvitamin D [25(OH)D], serum calcium, serum phosphorus, alkaline phosphatase, and parathormone (PTH) in patients with polyarticular juvenile idiopathic arthritis (JIA) and to associate them with disease duration and activity, bone mineral density and use of medications. In a cross-sectional and controlled study, 30 patients with polyarticular JIA were evaluated and compared to 30 healthy individuals matched for age and gender. Clinical status, anthropometry, laboratory markers in both patients and controls, and bone mineral density, only in the patients, were measured. Of the 30 patients included in the study, 23 (76.7%) were female and 16 (53.3%) non-Caucasian; mean age was 14 years (range = 4 to 20 years). Mean disease duration was 5 years (range = 1 to 12 years). The mean concentrations of serum albumin-corrected calcium (9.04 ± 0.41 mg/dL) and alkaline phosphatase (153.3 ± 100.1 IU) were significantly lower in patients with JIA than in controls (P < 0.0001 and P = 0.001, respectively). No differences in 25(OH)D, PTH or serum phosphorus were observed between JIA and control subjects. Regarding 25(OH)D concentration, 8 patients (26.7%) and 5 controls (16.7%) had 25(OH)D concentrations compatible with deficiency (lower than 20 ng/mL) and 14 patients (46.7%) and 18 controls (60%) had concentrations compatible with insufficiency (20-32 ng/mL). These values were not associated with disease activity, use of medications or bone mineral density. We observed a high frequency of 25(OH)D insufficiency and deficiency in the study sample. The compromised bone metabolism emphasizes the importance of follow-up of JIA patients.

Bone mineral density of Brazilian girls with juvenile dermatomyositis.

We measured bone mineral density (BMD) in girls with juvenile dermatomyositis (JDM) considering multiple factors in order to determine if it could be used as a predictor of reduction in bone mass. A cross-sectional study of lumbar spine BMD (L2-L4) was conducted on 10 girls aged 7-16 years with JDM. A group of 20 age-matched healthy girls was used as control. Lumbar spine BMD was measured by dual-energy X-ray absorptiometry. Weight, height and pubertal Tanner stage were determined in all patients and controls. Duration of disease and mean daily and cumulative steroid doses were calculated for all patients on the basis of their medical charts. JDM activity was determined on the basis of the presence of muscle weakness, cutaneous vasculitis and/or elevation of serum concentration of one or more skeletal muscle enzymes. Seven patients demonstrated osteopenia or osteoporosis. Lumbar BMD was significantly lower in the JDM patients than the age-matched healthy control girls (0.712 vs 0.878, respectively; Student t-test, P = 0.041). No significant correlation between BMD and age, height, Tanner stage, disease duration, corticosteroid use, or disease activity was observed in JDM girls, but a correlation was observed between BMD and weight (Pearson's correlation coefficient, r = 0.802). Patients with JDM may be at risk for a significant reduction in BMD that might contribute to further skeletal fragility. Our results suggest that reduced bone mass in JDM may be related to other intrinsic mechanisms in addition to steroid treatment and some aspects of the disease itself may contribute to this condition.

Bone mineral density of Brazilian girls with juvenile dermatomyositis

We measured bone mineral density (BMD) in girls with juvenile dermatomyositis (JDM) considering multiple factors in order to determine if it could be used as a predictor of reduction in bone mass. A cross-sectional study of lumbar spine BMD (L2-L4) was conducted on 10 girls aged 7-16 years with JDM. A group of 20 age-matched healthy girls was used as control. Lumbar spine BMD was measured by dual-energy X-ray absorptiometry. Weight, height and pubertal Tanner stage were determined in all patients and controls. Duration of disease and mean daily and cumulative steroid doses were calculated for all patients on the basis of their medical charts. JDM activity was determined on the basis of the presence of muscle weakness, cutaneous vasculitis and/or elevation of serum concentration of one or more skeletal muscle enzymes. Seven patients demonstrated osteopenia or osteoporosis. Lumbar BMD was significantly lower in the JDM patients than the age-matched healthy control girls (0.712 vs 0.878, respectively; Student t-test, P = 0.041). No significant correlation between BMD and age, height, Tanner stage, disease duration, corticosteroid use, or disease activity was observed in JDM girls, but a correlation was observed between BMD and weight (Pearson's correlation coefficient, r = 0.802). Patients with JDM may be at risk for a significant reduction in BMD that might contribute to further skeletal fragility. Our results suggest that reduced bone mass in JDM may be related to other intrinsic mechanisms in addition to steroid treatment and some aspects of the disease itself may contribute to this condition.

Bone mineral density in juvenile systemic lupus erythematosus.

We evaluated spine bone mineral density (BMD) in Brazilian children with juvenile systemic lupus erythematosus (JSLE) in order to detect potential predictors of reduction in bone mass. A cross-sectional study of BMD at the lumbar spine level (L2-L4) was conducted on 16 female JSLE patients aged 6-17 years. Thirty-two age-matched healthy girls were used as control. BMD at the lumbar spine was measured by dual-energy X-ray absorptiometry. Weight, height and pubertal Tanner stage were determined in patients and controls. Disease duration, mean daily steroid doses, mean cumulative steroid doses and JSLE activity measured by the systemic lupus erythematosus disease activity index (SLEDAI) were determined for all JSLE patients based on their medical charts. All parameters were used as potential determinant factors for bone loss. Lumbar BMD tended to be lower in the JSLE patients, however, this difference was not statistically significant (P = 0.10). No significant correlation was observed in JSLE girls between BMD and age, height, Tanner stage, disease duration, corticosteroid use or disease activity. We found a weak correlation between BMD and weight (r = 0.672). In the JSLE group we found no significant parameters to correlate with reduced bone mass. Disease activity and mean cumulative steroid doses were not related to BMD values. We did not observe reduced bone mass in female JSLE.

Bone mineral density in juvenile systemic lupus erythematosus

We evaluated spine bone mineral density (BMD) in Brazilian children with juvenile systemic lupus erythematosus (JSLE) in order to detect potential predictors of reduction in bone mass. A cross-sectional study of BMD at the lumbar spine level (L2-L4) was conducted on 16 female JSLE patients aged 6-17 years. Thirty-two age-matched healthy girls were used as control. BMD at the lumbar spine was measured by dual-energy X-ray absorptiometry. Weight, height and pubertal Tanner stage were determined in patients and controls. Disease duration, mean daily steroid doses, mean cumulative steroid doses and JSLE activity measured by the systemic lupus erythematosus disease activity index (SLEDAI) were determined for all JSLE patients based on their medical charts. All parameters were used as potential determinant factors for bone loss. Lumbar BMD tended to be lower in the JSLE patients, however, this difference was not statistically significant (P = 0.10). No significant correlation was observed in JSLE girls between BMD and age, height, Tanner stage, disease duration, corticosteroid use or disease activity. We found a weak correlation between BMD and weight (r = 0.672). In the JSLE group we found no significant parameters to correlate with reduced bone mass. Disease activity and mean cumulative steroid doses were not related to BMD values. We did not observe reduced bone mass in female JSLE

Use of single photon emission computed tomography and magnetic resonance to evaluate central nervous system involvement in patients with juvenile systemic lupus erythematosus.

The objective of the present study was to identify the single photon emission computed tomography (SPECT) and magnetic resonance (MR) findings in juvenile systemic lupus erythematosus (JSLE) patients with CNS involvement and to try to correlate them with neurological clinical history data and neurological clinical examination. Nineteen patients with JSLE (16 girls and 3 boys, mean age at onset 9.2 years) were submitted to neurological examination, electroencephalography, cerebrospinal fluid analysis, SPECT and MR. All the evaluations were made separately within a period of 15 days. SPECT and MR findings were analyzed independently by two radiologists. Electroencephalography and cerebrospinal fluid analysis revealed no relevant alterations. Ten of 19 patients (53%) presented neurological abnormalities including present or past neurological clinical history (8/19, 42%), abnormal neurological clinical examination (5/19, 26%), and abnormal SPECT or MR (8/19, 42% and 3/19, 16%, respectively). The most common changes in SPECT were cerebral hypoperfusion and heterogeneous distribution of blood flow. The most common abnormalities in MR were leukomalacia and diffuse alterations of white matter. There was a correlation between SPECT and MR (P<0.05). We conclude that SPECT and MR are complementary and useful exams in the evaluation of neurological involvement of lupus.

Use of single photon emission computed tomography and magnetic resonance to evaluate central nervous system involvement in patients with juvenile systemic lupus erythematosus

The objective of the present study was to identify the single photon emission computed tomography (SPECT) and magnetic resonance (MR) findings in juvenile systemic lupus erythematosus (JSLE) patients with CNS involvement and to try to correlate them with neurological clinical history data and neurological clinical examination. Nineteen patients with JSLE (16 girls and 3 boys, mean age at onset 9.2 years) were submitted to neurological examination, electroencephalography, cerebrospinal fluid analysis, SPECT and MR. All the evaluations were made separately within a period of 15 days. SPECT and MR findings were analyzed independently by two radiologists. Electroencephalography and cerebrospinal fluid analysis revealed no relevant alterations. Ten of 19 patients (53 percent) presented neurological abnormalities including present or past neurological clinical history (8/19, 42 percent), abnormal neurological clinical examination (5/19, 26 percent), and abnormal SPECT or MR (8/19, 42 percent and 3/19, 16 percent, respectively). The most common changes in SPECT were cerebral hypoperfusion and heterogeneous distribution of blood flow. The most common abnormalities in MR were leukomalacia and diffuse alterations of white matter. There was a correlation between SPECT and MR (P<0.05). We conclude that SPECT and MR are complementary and useful exams in the evaluation of neurological involvement of lupus

Características clínicas das espondiloartropatias na infância: análise de 26 pacientes / Clinical features of spondyloarthropaties in childhood: analysis of 26 patients

OBJETIVO: Avaliar retrospectivamente as características clínicas dos pacientes com diagnóstico de espondiloartropatia. MÉTODOS: Foram analisados os prontuários de todos os pacientes com diagnóstico de espondiloartropatia seguidos no ambulatório de reumatologia pediátrica da UNIFESP-EPM no período de junho de 1982 a abril de 2000. Foram avaliados a idade de início e tempo de evoluçäo da doença, manifestaçöes clínicas, dados laboratoriais, achados radiológicos, tratamento e evoluçäo. RESULTADOS: Dos 26 pacientes estudados, 10 (38,4 por cento) apresentavam SEA, um (3,8 por cento) espondiloartropatia indiferenciada (EAI), 10 (38,4 por cento) EAJ, dois (7,7 por cento) artropatia da DIIC, dois (7,7 por cento) síndrome de Reiter e um (3,8 por cento) artrite psoriásica. O início da doença variou de 1 a 15 anos (média de 9,2 anos). Os pacientes com síndrome de Reiter tiveram menor idade de início (média de 6,5 anos). Houve predomínio do sexo masculino (25 pacientes) e da raça caucasóide (15 pacientes). A maioria dos pacientes apresentou artrite periférica em membros inferiores (96,1 por cento), entesite (61,5 por cento) e HLA B27 positivo (14/23 - 60,9 por cento). Dez pacientes (38,4 por cento) apresentaram comprometimento axial. Quinze pacientes receberam diagnóstico de ARJ ou FR no início do quadro. CONCLUSÄO: Embora menos freqüentes do que a ARJ, as espondiloartropatias devem ser consideradas no diagnóstico diferencial das crianças e adolescentes, principalmente do sexo masculino, com artrite crônica

Bone mineral density of the lumbar spine of Brazilian children and adolescents aged 6 to 14 years

The authors performed a study of bone mass in eutrophic Brazilian children and adolescents using dual-energy X-ray absorptiometry (DXA) in order to obtain curves for bone mineral content (BMC) and bone mineral density (BMD) by chronological age and correlate these values with weight and height. Healthy Caucasian children and adolescents, 120 boys and 135 girls, 6 to 14 years of age, residents of São Paulo, Brazil, were selected from the Pediatric Department outpatient clinic of Hospital São Paulo (Universidade Federal de São Paulo). BMC, BMD and the area of the vertebral body of the L2-L4 segment were obtained by DXA. BMC and BMD for the lumbar spine (L2-L4) presented a progressive increase between 6 and 14 years of age in both sexes, with a distribution that fitted an exponential curve. We identified an increase of mineral content in female patients older than 11 years which was maintained until 13 years of age, when a new decrease in the velocity of bone mineralization occurred. Male patients presented a period of accelerated bone mass gain after 11 years of age that was maintained until 14 years of age. At 14 years of age the mean BMD values for boys and girls were 0.984 and 1.017 g/cm², respectively. A stepwise multiple regression analysis of paired variables showed that the "vertebral area-age" pair was the most significant in the determination of BMD values and the introduction of a third variable (weight or height) did not significantly increase the correlation coefficient

Febre reumática: manifestaçöes articulares atípicas / Rheumatic fever: atypical articular manifestations

O diagnóstico de febre reumática (FR) continua sendo um dos mais difíceis em pediatria, devido ao polimorfismo do seu quadro e à falta de exames laboratoriais específicos ou patognomônicos da doença. Este trabalho aborda o critério diagnóstico mais freqüente e menos específico, a artrite. Foram estudadas 93 crianças (117 surtos) acompanhadas no setor de Reumatologia Pediátrica do Departamento de Pediatria da Escolar Paulista de Medicina, sendo critério de inclusäo a presença de artrite. O diagnóstico foi baseado na história clínica, exame físico e exames complementares, tendo sido considerados os critérios de Jones modificados. Em 45// observamos artrite e cardite, em 7// artrite e coréia e em 4// artrite, cardite e coréia. A artrite foi migratória em 75 surtos (64// e aditiva em 42 (32//), poliarticular em 64// e pauciarticular em 36//. Monoartrite foi encontrada em 3//. Em 5// foi observada artrite de coxofemoral como manifestaçäo inicial da doença. O tempo de duraçäo da artrite foi igual ou maior a seis semanas em 5// dos surtos. O diagnóstico definitivo de FR nos casos com manifestaçöes articulares atípicas só foi possível com a presença de cardite ou coréia. Os autores chamam a atençäo para o diagnóstico diferencial da FR com outras doenças, principalmente quando a artrite é o único critério maior encontrado

Effects of nutritional copper deficiency on adult non-pregnant and pregnant rats and their newborns

Se estudiaron los efectos de la deficiencia nutricional de cobre sobre la ganancia de peso, ingesta de alimento y fertilidad de ratas Wistar adultas, preñadas y sin preñar, y peso de sus recién nascidos. Los animales adultos fueron sumetidos a dietas con diferentes contenidos de cobre (control - 5 ppm; deficiencia moderada - 1.2 ppm; deficiencia severa - 0.3 ppm) durante seis semanas (no preñadas) y nueve semanas (preñadas). No se observó ningún efecto sobre la ganancia ponderal ni sobre el apetito del grupo de animales sujetos a deficiencia de cobre. Ni la fertilidad, evaluada por el número de conceptus y nascidos vivos de las ratas preñadas, ni el peso de sus recién nascidos, sufrieron ningún trastorno. Estos datos revelan que no sólo es el grado de deficiencia, sino también el período durante el cual es impuesto, al igual que su duración, los factores que condicionan la aparición de alteraciones propias de esta deficiencia

Estudo clínico multicêntrico de naproxeno suspensäo vs ácido acetilsalicílico no tratamento da artrite reumatóide juvenil / Multicenter clinical trial with naproxen suspension vs acetylcalicylic acid in the treatment of juvenile rheumatoid arthritis

Os autores relatam os resultados obtidos num ensaio clínico multicêntrico, simples-cego, de naproxeno suspensäo vs. ácido acetilsalicílico no tratamento de 52 crianças com artrite reumatóide juvenil. Os pacientes foram divididos em dois grupos de 26 cada um, aos quais foram administrados naproxeno suspensäo (25 mg/ml, na dose de 10 mg/Kg/dia, divididos em duas administraçöes diárias, ou ácido acetilsalicílico, em comprimidos de 500 mg, na dose de 100 mg/Kg/dia divididos em quatro administraçöes diárias. A duraçäo do estudo foi de 60 dias, para ambos os grupos, e a idade das crianças variou de dois a 14 anos. A avaliaçäo final feita pelos investigadores apresentou os seguintes resultados: no grupo naproxeno (69,2%), regular em 5(19,2%) e má em 3(11,5%). No grupo ácido acetilsalicílico os resultados foram bons em 16 pacientes (61,4%), regulares em 5(19,2%) e maus em 5(19,2%). Ao final, 80,7% dos pais, 57,6% das crianças e 69,2% dos investigadores consideraram os resultados do uso do naproxeno entre muito bons ou bons, comparados respectivamente, a 61,4%, 49,9% e 61,4% com o uso do ASS. Quanto aos efeitos colaterais apesar de mais freqüentes no grupo com AAS (vômitos, náuseas, dor epigástrica, cefaléia) do que no grupo com naproxeno (náuseas, diarréia, epistaxe), em geral foram leves e transitórios, com exceçäo de uma criança do grupo naproxeno, que apresentou hematúria macroscópica e foi retirada do estudo, observando-se regressäo completa da lateraçäo em 15 dias. A colaboraçäo das crianças ao esquema terapêutico com o naproxeno suspensäo foi mais facilmente obtida, näo só por necessitar de apenas duas administraçöes diárias, comparadas as quatro do ácido acetilsalicílico, como pelo fato de ser formulaçäo líquida e ter sabor agradável. Teve influência, também, o aspecto cultural negativo que grande parte dos pais tem quanto ao ácido acetilsalicílico, por considerarem-no uma medicaçäo de uso comum, pouco adequada a uma doença täo séria como a artrite reumatóide juvenil e, eventualmente, por ter sido usada anteriormente em algumas crianças...