RESUMO
This study sought to determine the appropriate starting dose of colchicine in children aged 2 to 4 years with familial Mediterranean fever (FMF) based on steady-state pharmacokinetics in pediatric patients with FMF from 2 to less than 16 years and adult patients with FMF from 16 to 65 years. Outpatients received colchicine for 90 days starting with a fixed dose for 14 days (blood sampling days 14 and 15). After starting doses of colchicine (0.6 mg/day [2 to less than 4 years], 0.9 mg/day [from 4 to less than 6 years], 0.9 mg/day [from 6 to less than 12 years], 1.2 mg/day [from 12 to less than 16 years], and 1.2 mg/day [from 16 to less than 65 years]), the observed steady-state pharmacokinetic parameters were comparable across age groups, despite the higher doses of colchicine on a mg/kg/day basis in the younger age groups. An exception occurred with once-daily colchicine, whereby mean Cmax for colchicine was higher in patients 4 to less than 6 years (9.4 ng/mL) compared with the younger and older age groups (6.1-6.7 ng/mL). Mean AUC0?24h values in children 2 to less than 4, 6 to less than 12, and 12 to less than 16 years were similar to those in adults. However, mean AUC0?24h values in children 4 to less than 6 years were 25 percent higher than those observed in adults. The results show that the recommended starting dose for children 2-4 years and 4-6 years should be 0.6 mg/day (half the US adult dose). Children aged 6 to less than 12 years should receive 0.9 mg/day (i.e. three-quarters of the US adult dose). The safety of colchicine in children 2 to less than 4 years was comparable to that in older children and adults.
Assuntos
Colchicina/farmacocinética , Febre Familiar do Mediterrâneo/tratamento farmacológico , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Colchicina/administração & dosagem , Colchicina/efeitos adversos , Feminino , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
OBJECTIVE: To assess whether the removal of aids/devices and/or help from another person in the Childhood Health Assessment Questionnaire (C-HAQ) leads to a significant change in the disability index (DI) score and responsiveness in juvenile idiopathic arthritis (JIA). METHODS: Changes in the C-HAQ DI score in a cross-sectional sample of 2663 children with JIA and in 530 active patients with JIA in a trial of methotrexate (MTX) were compared. RESULTS: Patients in the MTX trial had higher disease activity and disability than the cross-sectional sample. The frequency of aids/devices (range 1.2-10.2%) was similar between the two samples, while help (range 5.3-38.1%) was more frequently used in the MTX group. Correlation between disease severity variables and the two different C-HAQ DI scoring methods did not change substantially. There was a decrease in the C-HAQ DI score for both the cross-sectional (mean score from 0.64 with the original method to 0.54 without aids/devices and help, p<0.0001) and the MTX sample (mean score from 1.23 to 1.07, p<0.0001). A linear regression analysis of the original C-HAQ DI score versus the score without aids/devices and help demonstrated the substantial overlap of the different scoring methods. Responsiveness in the responders to MTX treatment did not change with the different C-HAQ DI scoring methods (range 0.86-0.82). CONCLUSION: The removal of aids/devices and help from the C-HAQ does not alter the interpretation of disability at a group level. The simplified C-HAQ is a more feasible and valid alternative for the evaluation of disability in patients with JIA.
Assuntos
Artrite Juvenil/reabilitação , Avaliação da Deficiência , Tecnologia Assistiva , Atividades Cotidianas , Antirreumáticos/uso terapêutico , Artrite Juvenil/tratamento farmacológico , Artrite Juvenil/fisiopatologia , Criança , Pré-Escolar , Estudos Transversais , Feminino , Humanos , Masculino , Metotrexato/uso terapêutico , Reprodutibilidade dos Testes , Índice de Gravidade de Doença , Resultado do TratamentoRESUMO
Evaluating the impact of urban development on natural ecosystem processes has become an increasingly complex task for planners, environmental scientists, and engineers. As the built environment continues to grow, unregulated nonpoint pollutants from increased human activity and large-scale development severely stress urban streams and lakes resulting in their currently impaired or degraded state. In response, integrated water quality management programs have been adopted to address these unregulated nonpoint pollutants by utilizing best management practices (BMPs) that treat runoff as close to the source as possible. Knowing where to install effective BMPs is no trivial task, considering budget constraints and the spatially extensive nature of nonpoint stormwater runoff. Accordingly, this paper presents an initial, straightforward and cost-effective methodology to identify critical nonpoint pollutant source watersheds through correlation of water quality with land use. Through an illustrative application to metropolitan Denver, Colorado, it is shown how this method can be used to aid stormwater professionals to evaluate and specify retrofit locations in need of water quality treatment features reduce, capture and treat stormwater runoff prior to entering receiving waters.
Assuntos
Chuva , Urbanização , Movimentos da Água , Cidades , Colorado , Poluição da ÁguaRESUMO
OBJECTIVES: To present an analysis of patients with protracted febrile myalgia (PFM), a rarely reported manifestation of familial Mediterranean fever (FMF), and propose clinical criteria for working diagnosis. METHODS: A multicenter retrospective cohort study of children with PFM was performed. Clinical and laboratory data were obtained by medical record review. RESULTS: The study group included 15 patients with PFM. PFM occurred as the presenting sign of FMF in 33%. FMF was diagnosed clinically in all and by genetic analysis in 93%. M694V allelic involvement was noted in 93% of the patients. PFM occurred at a mean age of 9 +/- 3.4 years and was characterized by severe generalized muscle pain in all patients and fever in 71%. Mean duration up to diagnosis was 15.5 +/- 6 days. Mean erythrocyte sedimentation rate was 104 +/- 26 mm/h; mean C-reactive protein was 15.4 +/- 6.3 mg%. Creatine kinase was normal. Treatment included corticosteroids (4 patients) and nonsteroidal anti-inflammatory drugs (NSAIDs) (9 patients) with a symptomatic relief achieved at a mean of 7.7 +/- 4.3 days and 5 +/- 3.8 days, respectively (p = 0.14) (mean severity score 3 and 2.2, respectively, p = 0.075). Symptomatic relief in 2 untreated patients was achieved at a mean of 45.5 days. CONCLUSION: Based on our data, we propose criteria for working diagnosis including: severe disabling myalgia of at least 5 days in a young patient with FMF, associated with fever, elevated levels of inflammatory markers and presence of at least one M694V mutation.
Assuntos
Febre Familiar do Mediterrâneo/complicações , Febre Familiar do Mediterrâneo/diagnóstico , Febre/complicações , Debilidade Muscular/complicações , Doenças Musculares/diagnóstico , Adolescente , Adulto , Criança , Estudos de Coortes , Proteínas do Citoesqueleto/genética , Febre Familiar do Mediterrâneo/genética , Feminino , Humanos , Masculino , Doenças Musculares/imunologia , Dor , Polimorfismo de Nucleotídeo Único , Pirina , Estudos Retrospectivos , SíndromeRESUMO
FMF is widely distributed in populations inhabiting the Mediterranean basin. It is mainly attributed to five founder mutations (M680I, M694V, M694I, V726A, E148Q) in the MEFV gene. The frequencies and distribution of these mutations in 146 FMF patients, of Arab and Jewish descent, were compared to that observed in 1173 healthy individuals of pertinent ethnic groups. Five mutations accounted for 91% of FMF chromosomes in our patients. Mutation M694V, predominant in North African Jews, was observed in all patients other than Ashkenazi Jews; mutation V726A was prevalent among all patients other than North African Jews; mutations M694I and M680I were mainly confined to Arab patients. Overall carrier rates, for four mutations (M680I, M694V, V726A, E148Q), were extremely high in our healthy cohort composed of Ashkenazi (n=407); Moroccan (n=243); Iraqi Jews (n=205); and Muslim Arabs (n=318); calculated at 1 : 4.5; 1 : 4.7; 1 : 3.5 and 1 : 4.3 respectively. The V726A allele prevalent among Ashkenazi and Iraqi Jews and Muslim Arabs (carrier rates: 7.4, 12.8 and 7.3%, respectively) was not found among Moroccan Jews. The M694V allele detected among Moroccan and Iraqi Jews and Muslim Arabs (carrier rates 11.1, 2.9 and 0.6%, respectively) was not observed among Ashkenazim. The overall frequency of mutations V726A and E148Q in Ashkenazim, Iraqi Jews and Arabs indicates that the bulk of individuals that comply with the genetic definition of FMF remain asymptomatic.
Assuntos
Febre Familiar do Mediterrâneo/epidemiologia , Febre Familiar do Mediterrâneo/genética , Frequência do Gene/genética , Penetrância , Árabes/genética , Árabes/estatística & dados numéricos , Febre Familiar do Mediterrâneo/etnologia , Humanos , Islamismo , Israel/etnologia , Judeus/genética , Judeus/estatística & dados numéricos , Mutação/genéticaRESUMO
Spatial summation of thermal pain crosses dermatomal boundaries. In this study we examined whether a vibrational stimulus applied to adjacent or remote dermatomes affects thermal pain perception to the volar forearm. Contact heat at 2 degrees C above thermal pain threshold was applied, and a Visual Analog Scale (VAS) was used for pain assessment. We found a significant decrease in mean VAS rating when simultaneous vibratory stimuli were given to the dermatome adjacent to that receiving thermal stimulation, or to the same dermatome on the contralateral side. There was no change in VAS rating when vibration was given two or more dermatomes away. Vibration within the same dermatome also did not yield a significant change in VAS rating, possibly due to difficulty in magnitude assessment of stimuli given simultaneously within a single dermatome. The finding that vibration can reduce pain across dermatomes may allow for more flexible design of stimulation therapy for pain.
Assuntos
Dor/fisiopatologia , Vibração , Adolescente , Adulto , Feminino , Lateralidade Funcional/fisiologia , Temperatura Alta , Humanos , Masculino , Neurônios Aferentes/fisiologia , Manejo da Dor , Medição da Dor , Estimulação FísicaRESUMO
We report on a baby girl with congenital muscular dystrophy (CMD) with neurological abnormalities ("CMD Plus" condition), who also had Hirschsprung disease. This association may indicate a category of congenital muscular dystrophy with involvement of the visceral nervous system. We propose that Hirschsprung disease be added to the list of anomalies pertaining to the "CMD Plus" array, and that CMD should be considered when Hirschsprung disease occurs with central nervous system anomalies.
Assuntos
Doença de Hirschsprung/complicações , Distrofias Musculares/congênito , Distrofias Musculares/complicações , Encéfalo/anormalidades , Feminino , Humanos , Lactente , Músculos/ultraestruturaRESUMO
A 12-year-old girl presented with arthritis, myalgia, anemia and positive ANA. Subsequently, she developed recurrent episodes of pulmonary hemorrhage, thrombocytopenia, CNS abnormalities, skin ulcers and diffuse calcinosis. This was followed by secondary antiphospholipid syndrome. Despite vigorous immunosuppression, the patient became bedridden. A peripheral blood stem cell autograft was offered when she developed pulmonary hypertension and digital ischemia at the age of 16 years. The post-transplantation course was uneventful. Liquefaction of calcinosis nodules with improvement of mobility occurred gradually. She is now 24 months post-transplant with no sign of disease activity and total disappearance of calcinosis nodules.
Assuntos
Doenças Autoimunes/terapia , Calcinose/terapia , Transplante de Células-Tronco de Sangue Periférico , Anemia , Artrite , Calcinose/diagnóstico por imagem , Calcinose/etiologia , Criança , Feminino , Fibromialgia , Humanos , Cintilografia , Indução de Remissão/métodos , Transplante AutólogoRESUMO
BACKGROUND & AIMS: Children with cerebral palsy (CP) have a high prevalence of pathologic fractures. Bone quantitative ultrasonography (QUS) has emerged as a radiation-free method for the assessment of bone quality and fracture risk. In this study, we applied QUS technique in order to investigate bone status in handicapped institutionalized children and adolescents. METHODS: This cross-sectional study included 87 handicapped institutionalized patients. Measurements of the velocity of ultrasound wave, speed of sound (SOS), at distal radius and midshaft tibia, were performed using Omnisense 7000S analyser (Sunlight Ltd., Tel Aviv, Israel). In addition, all the participants had a thorough evaluation of nutritional status, demographic and clinical characteristics. RESULTS: Forty-five of patients had either radius or tibia bone SOS lower than -1 SD, and 21% had either radius or tibia bone SOS lower than -2.5 SD. Using step-wise regression analysis, female gender (P=0.003) and stature (P=0.008) were correlated with radius SOS. Age (P=0.03) and fracture history (P=0.04) were negatively correlated with tibia SOS. CONCLUSION: In this group of children and adolescents with CP one-fifth had poor bone status as suggested by low tibia/radius SOS assessed by QUS. Female gender, stature, age and fracture history were significantly correlated with poor bone status.
Assuntos
Densidade Óssea , Osso e Ossos/diagnóstico por imagem , Paralisia Cerebral/diagnóstico por imagem , Paralisia Cerebral/fisiopatologia , Estado Nutricional , Adolescente , Adulto , Antropometria , Criança , Pré-Escolar , Estudos Transversais , Feminino , Fraturas Ósseas , Humanos , Lactente , Institucionalização , Masculino , Rádio (Anatomia)/diagnóstico por imagem , Análise de Regressão , Fatores Sexuais , Som , Tíbia/diagnóstico por imagem , Ultrassonografia/instrumentação , Ultrassonografia/métodosRESUMO
Previous studies have strongly suggested an association between rheumatoid factors (RF's), particularly IgA-RF, and the presence of erosions in rheumatoid arthritis (RA). The present study was aimed at studying this association in seronegative erosive arthritides. Forty-eight patients with seronegative arthritis were evaluated for the presence of IgM- and IgA-RFs using an enzyme linked immunosorbent assay (ELISA). Twenty-nine had seronegative RA and nineteen had psoriatic arthritis (PA). Twelve (41%) seronegative RA patients were found to be seropositive for IgM- or IgA-RF. Only 1 (7%) patient with PSA was positive for IgA-RF alone. Fifteen (51%) of the RA patients and eight (42%) of the PSA patients had erosive disease. A significant correlation between IgA-RF alone and erosive disease was found only in the seronegative RA patient (p less than 0.02). We conclude that in PSA patients there appears to be no need to define isotype specific RFs. On the other hand, our findings indicate that an early detection of IgA-RF can have clinical importance in seronegative rheumatoid arthritis, as it may constitute an indication for the timely institution of disease-modifying drugs in these patients.
Assuntos
Artrite Reumatoide/imunologia , Artrite/imunologia , Imunoglobulina A/análise , Imunoglobulina M/análise , Psoríase/imunologia , Fator Reumatoide/análise , Adulto , Idoso , Artrite/etiologia , Artrite/patologia , Artrite Reumatoide/patologia , Ensaio de Imunoadsorção Enzimática , Estudos de Avaliação como Assunto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Psoríase/complicações , Psoríase/patologiaRESUMO
OBJECTIVE: We have previously shown that the administration of D-penicillamine (D-PEN) to patients with rheumatoid arthritis induces circulating insulin autoantibodies (INSAAB). In order to gain further insight into such immune responses, we measured a battery of circulating autoantibodies in 4 strains of mice receiving D-PEN: C57BL/KsJ, BALB/c, C3H/HeJ, and C57BL/6. These rodents groups differ in their degree of susceptibility to streptozotocin (STZ)-induced immune diabetes (SIMD), which is high in the first 2 strains, and mild and nil in the third and fourth, respectively. METHODS: Randomly assigned animals from each group were given a weekly subcutaneous (SC) injection of either D-PEN 1 mg, D-PEN 3 mg, or solvent (PBS) for a period of 4 weeks. Serum levels of antibodies to insulin, single stranded DNA (ssDNA), thyroglobulin, and cardiolipin were measured weekly. RESULTS: Only the C57BL/KsJ and C3H/HeJ mice reacted to D-PEN administration. When compared to the pre-treated and solvent-treated mice, D-PEN 1 mg, and to a lesser degree D-PEN 3 mg, induced elevation of antibodies to insulin and to ssDNA in C57/KsJ mice (p < 0.001), while only ssDNA antibodies were detected in the C3H/HeJ mice (p < 0.0001 for D-PEN 1 mg; p < 0.05 for D-PEN 3 mg). D-PEN had no effect on the level of antibodies to cardiolipin or to thyroglobulin in any of the mice. CONCLUSIONS: This study showed that D-PEN induces an antigen(s)-specific humoral response only in mice already inherently prone to autoimmunity. This model suggests that the activation of autoimmunity by environmental factors is probably facilitated by genetic background, and might partly explain the diversity of autoimmune manifestations in D-PEN-treated patients.
Assuntos
Autoanticorpos/análise , Penicilamina/imunologia , Animais , Anticorpos Anticardiolipina/análise , Anticorpos Antinucleares/análise , DNA de Cadeia Simples/imunologia , Diabetes Mellitus Experimental/genética , Diabetes Mellitus Experimental/imunologia , Feminino , Predisposição Genética para Doença , Insulina/imunologia , Camundongos , Camundongos Endogâmicos BALB C , Camundongos Endogâmicos C3H , Camundongos Endogâmicos C57BL , Tireoglobulina/imunologiaRESUMO
We report herein the results of the cross-cultural adaptation and validation into the Hebrew language of the parent's version of two health related quality of life instruments. The Childhood Health Assessment Questionnaire (CHAQ) is a disease specific health instrument that measures functional ability in daily living activities in children with juvenile idiopathic arthritis (JIA). The Child Health Questionnaire (CHQ) is a generic health instrument designed to capture the physical and psychosocial well-being of children independently from the underlying disease. The Hebrew CHAQ-CHQ were fully developed with 3 forward and 3 backward translations. A total of 144 subjects were enrolled: 80 patients with JIA (12% systemic onset, 34% polyarticular onset, 23% extended oligoarticular subtype, and 31% persistent oligoarticular subtype) and 64 healthy children. The CHAQ clinically discriminated between healthy subjects and JIA patients, with the systemic, polyarticular and extended oligoarticular subtypes having a higher degree of disability, pain, and a lower overall well-being when compared to their healthy peers. Also the CHQ clinically discriminated between healthy subjects and JIA patients, with the JIA patients having a lower physical and psychosocial well-being when compared to their healthy peers. In conclusion the Hebrew version of the CHAQ-CHQ is a reliable, and valid tool for the functional, physical and psychosocial assessment of children with JIA.
Assuntos
Artrite Juvenil/diagnóstico , Comparação Transcultural , Nível de Saúde , Inquéritos e Questionários , Adolescente , Criança , Características Culturais , Avaliação da Deficiência , Feminino , Humanos , Israel , Idioma , Masculino , Psicometria , Qualidade de Vida , Reprodutibilidade dos TestesRESUMO
OBJECTIVE: Behçet's disease (BD) is a vasculitis mainly observed in young adult males. Juvenile BD is rare and only small series of pediatric cases have been reported. The objective of this study was to define the epidemiology and clinical features of BD among Israeli children. METHODS: A questionnaire was sent to 8 pediatric rheumatology units in Israel and 30 cases of BD diagnosed before the age of 16 years were identified. RESULTS: Fifteen patients fulfilled the International Study Group Criteria for BD, while 15 had an incomplete form of BD. Among the patients with complete BD, stomatitis and skin involvement were the most common manifestations. Other symptoms included genital ulcers, uveitis, CNS involvement, arthritis, and gastrointestinal involvement. A positive family history was elicited in 3 patients. HLA B5 was found in 7 of 12 patients (58%). The 15 patients with incomplete BD all had recurrent stomatitis; other manifestations included uveitis, arthritis, and genital ulcers. HLA B5 was found in 94% of this group. CONCLUSION: Juvenile BD in Israel is not uncommon, and is frequently associated with HLA B5 positivity. This could indicate a genetic susceptibility in our region. Half of the patients in our series had an incomplete form of BD, which may represent a less severe variant of the disease. In any case, careful follow-up is required, since their condition could eventually evolve into complete BD.
Assuntos
Síndrome de Behçet/epidemiologia , Adolescente , Síndrome de Behçet/imunologia , Síndrome de Behçet/patologia , Criança , Pré-Escolar , Feminino , Seguimentos , Antígenos HLA-B/sangue , Humanos , Israel/epidemiologia , Masculino , Inquéritos e QuestionáriosRESUMO
Scleroderma-like syndrome (SLS) may represent the earliest apparent diabetes complication in insulin-dependent diabetic (IDDM) patients. To evaluate the frequency of SLS and its association with other diabetes-related pathology in our diabetic population, we studied 153 (127 Jewish and 26 Arab) IDDM patients and 45 healthy age- and gender-matched controls (25 Jewish, 20 Arab). The mean age and diabetes duration of the patients were 14.09 +/- 5.1 years and 51 +/- 45 months, respectively. While no diabetes-related pathology was found in the controls, SLS was detected in 47% of all patients (skin, 31.4%; arthropathy, 37.9%; both, 22%), and nephropathy, neuropathy, and retinopathy were present in 10.5%, 5.2%, and 4.6%, respectively. Independent of age, SLS directly correlated with diabetes duration (p < 0.01) and with the presence of either nephropathy or neuropathy (p < 0.009 and p < 0.005, respectively). One or more features of systemic diabetic involvement were present in 22% of patients with SLS, compared to only 7.2% in patients without SLS (p < 0.009). When patients were analyzed according to ethnicity, the frequency of skin involvement and neuropathy were found to be higher among Arab patients, particularly males (p < 0.002 and p < 0.005, respectively), and detection of one was significantly associated with the presence of the other (p < 0.001). In conclusion, our results suggest that SLS is the most common diabetic complication among Jewish and Arab IDDM patients, and its presence may reflect an inherited tendency to develop other serious diabetic complications. Ethnicity (Arab) by itself, particularly when associated with male gender, seems to accelerate neurological and dermatological diabetic involvement.
Assuntos
Diabetes Mellitus Tipo 1/complicações , Escleroderma Sistêmico/epidemiologia , Adolescente , Adulto , Árabes/estatística & dados numéricos , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Israel/epidemiologia , Judeus/estatística & dados numéricos , Masculino , Prevalência , Escleroderma Sistêmico/etnologiaRESUMO
Twenty-six patients with Behçet's disease were studied for serum levels of anticardiolipin (ACL) antibody isotypes IgG and IgM. The frequency of elevated levels of ACL antibody isotype IgM was found to be significantly increased in these patients. This increased frequency, however, did not correlate statistically with a thrombotic tendency, colchicine intake, antinuclear antibodies, or a positive VDRL test.
Assuntos
Autoanticorpos/sangue , Síndrome de Behçet/imunologia , Cardiolipinas/imunologia , Adulto , Idoso , Síndrome de Behçet/complicações , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Trombose/etiologiaRESUMO
The mean time from initiation of methotrexate (MTX) treatment of juvenile rheumatoid arthritis (JRA) to partial remission of clinical symptoms and total clinical remission was assessed. 9 girls and 8 boys, from 3 to 18 years of age (mean 11.4 +/- 5.4) with active JRA by American College of Rheumatology (ACR) criteria (5 systemic, 8 polyarticular and 4 pauciarticular disease onset), who failed to respond to adequate courses of non-steroidal anti-inflammatory drugs (NSAID), steroids or disease-modifying drugs were studied. Clinic visits were scheduled at monthly intervals for physical and laboratory assessment of disease activity and drug safety. Partial response to MTX was defined a 25% reduction of the active joint count and/or articular severity score. Total clinical remission was defined as in adult rheumatoid arthritis. The duration of disease activity until enrollment ranged from 6 months to 14 years (4.5 +/- 3.7 yr); duration of therapy was 3 months to 3 years (14.6 +/- 9.3 mo) and dosage ranged from 5 to 15 mg/m2/week. Prednisone in doses below 10 mg/day and NSAID were permitted. 14 of 17 patients (82%) had a 25% reduction in joint activity after 6 weeks to 4 months (9.2 +/- 3.2 weeks); 10 (59%) went into full clinical remission after 5 to 26 months (14.3 +/- 9 months); 3 relapsed after an initial response to treatment, and 4 (23%) did not respond to MTX. The non-responders were males who required higher doses of prednisone (p < 0.0001). MTX appears to be effective therapy for children with JRA. An initial response can be expected in most patients after 9 weeks of treatment, and full clinical remission occurs after a mean of 14 months.
Assuntos
Antirreumáticos/uso terapêutico , Artrite Juvenil/tratamento farmacológico , Metotrexato/uso terapêutico , Adolescente , Adulto , Idade de Início , Anti-Inflamatórios não Esteroides/uso terapêutico , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Estudos Retrospectivos , Resultado do TratamentoRESUMO
Hepatic involvement in juvenile rheumatoid arthritis of the systemic type (JRA) is a diagnostic challenge because of the varied clinical picture it presents. We describe a 21-year-old woman in whom JRA had started at the age of 4 years as an illness resembling familial Mediterranean fever. This long-standing illness was complicated by liver damage and needle biopsy showed massive hepatic deposition of amyloid. It is possible that this patient may have developed JRA as a complication of familial Mediterranean fever.
Assuntos
Amiloidose/etiologia , Artrite Juvenil/complicações , Hepatopatias/etiologia , Adulto , Artrite Juvenil/diagnóstico , Artrite Juvenil/etiologia , Febre Familiar do Mediterrâneo/complicações , Febre Familiar do Mediterrâneo/diagnóstico , Feminino , HumanosRESUMO
Patients with Type I diabetes may develop a scleroderma-like syndrome, including limitation of joint mobility. This syndrome, cherioarthropathy, is considered a complication of diabetes, but its cause is unknown. We examined 30 Jewish and 13 Arab patients in our juvenile diabetes clinic for skin and joint involvement. Signs of cherioarthropathy in both hands were found in 13 children (30.2%); all had skin changes and 6 (13.5%) also had articular involvement of the hands. There was no correlation between the presence of cherioarthropathy and the patient's age or the duration of diabetes. The syndrome was significantly more frequent among Arabs (8/15, 61.5%) than Jewish children (5/30, 16.6%), p less than 0.01. There was a indirect correlation between incidence of cherioarthropathy and adequacy of glycemic control, but no difference in glycemic control between Arab and Jewish children. This may indicate a genetic factor in the development of cherioarthropathy in juvenile diabetes.
Assuntos
Diabetes Mellitus Tipo 1/complicações , Dermatoses da Mão/complicações , Artropatias/complicações , Esclerodermia Localizada/complicações , Criança , Diabetes Mellitus Tipo 1/etnologia , Mãos , Dermatoses da Mão/etnologia , Humanos , Judeus , Esclerodermia Localizada/etnologia , SíndromeRESUMO
Systemic lupus erythematosus (SLE) is a rare disease in children that might possibly be modulated by genetic and environmental factors. In order to delineate the characteristic features of SLE among Israeli children, we reviewed the medical records of 38 cases from 8 pediatric rheumatology clinics. All fulfilled the 1982 American Rheumatism Association revised criteria for SLE. The illness became apparent at the age of 16 years or younger and the mean age of onset was 11.9 +/- 2.4 y (range 7-16) and the mean duration of follow-up 4.0 +/- 4.8 y (range 0.5-15). The female to male ratio was 2.8:1; 28 were Jewish and 10 Arabs. Systemic complaints, such as fever, malaise and weight loss, were noted in 90%, malar rash in 65%, and other skin manifestations in 40%. Arthritis was noted in 57% and additional musculoskeletal complaints in 70%; 90% had hematological abnormalities. Major organ system involvement included: renal disease in 50% pulmonary involvement 28% and CNS involvement 28%. 2 patients are currently on renal dialysis and 1 died from hypertensive crisis. We conclude that the features of SLE in children in Israel are not influenced by ethnic or geographic factors, and are similar to those reported worldwide.
Assuntos
Lúpus Eritematoso Sistêmico , Adolescente , Idade de Início , Criança , Feminino , Humanos , Israel , Lúpus Eritematoso Sistêmico/etnologia , Lúpus Eritematoso Sistêmico/patologia , Masculino , Razão de MasculinidadeRESUMO
In the wake of a community outbreak of bronchiolitis in northern Israel from December 1993 to March 1994, we conducted a retrospective study of 108 infants aged 2 weeks to 14 months with proven respiratory syncytial virus (RSV) infection (diagnosed by a rapid RSV antigen test in nasopharyngeal secretions). 47% of the infants were less than 8 weeks old. Mean hospital stay was 6.6 days (range 1-60). The characteristic clinical findings were: cough in all patients, dyspnea in 96%, rhinitis in 95% and fever in 55%. In those younger than 8 weeks, or in those with underlying diseases, hospitalization was longer, the disease was more serious and complications more frequent (p < 0.002). 4 children (3%) died, 3 of whom had severe congenital heart defects. All children were treated with oxygen and beta-agonist inhalations. The 33% who also received corticosteroids were older and most had a history of pulmonary diseases, such as asthma or bronchopulmonary dysplasia. There was no difference between those who did or did not receive corticosteroids with regard to severity of disease or rate of complications. Ribavirin was used to treat 19 (17.5%), most of whom had underlying lung disease. The others were otherwise healthy infants younger than 8 weeks. 13 were cared for in the intensive care unit, 11 of whom required mechanical ventilation. A rapid test for detection of RSV infection enabled prompt isolation of infected patients so that the risk of nosocomial infection was reduced and Ribavirin therapy could be started early, if required.