Tarsitis as an initial manifestation of juvenile spondyloarthropathy.

OBJECTIVE: The aim of this study was to determine the frequency of tarsitis as one of the first symptoms of juvenile spondyloarthropathy (JSpA) and to analyze whether patients with tarsitis at onset differ from those without it. METHODS: A retrospective chart review was performed, from January 1996 to September 2007, at a paediatric rheumatology unit of a tertiary university hospital. RESULTS: Tarsitis was detected in one-third of the children diagnosed with JSpA. They had fever and received antibiotics due to a suspected infection more frequently than those without tarsitis. Inflammatory low back pain was extremely unusual among these patients. CONCLUSION: There were some differences between children diagnosed with JSpA initially affected with tarsitis and those without it. Patients with tarsitis as one of the first symptoms were often misdiagnosed as soft tissue infections.

[Spondylodiscitis in the autonomus community of Madrid (Spain)]. / Espondilodiscitis en la Comunidad de Madrid.

INTRODUCTION: Spondylodiscitis is a relatively uncommon entity in infancy and childhood, with typical, although non-specific symptoms. The aim of this study was to describe the clinical features at presentation and follow-up in patients diagnosed with spondylodiscitis in hospitals in the Autonomous Community of Madrid. PATIENTS AND METHODS: All cases of spondylodiscitis diagnosed in children in the hospitals of La Paz, Niño Jesús, Gregorio Marañón, Severo Ochoa, Doce de Octubre and Getafe in Madrid were reviewed. Their clinical features, diagnostic tests, treatment and follow-up were analyzed. RESULTS: Twenty children with a mean age of 37 months were studied. The level of disc involvement was L5-S1 in six patients, L2-L3 in five, L3-L4 in four, C6-C7 in two, and D12-L1 in one. The mean time before diagnosis was 20 +/- 16 days. The most frequent symptoms were gait disturbances, limping, or inability to remain seated. Eleven patients had low grade fever (< 38.5 degrees C). Other less specific symptoms were irritability, constipation and abdominal pain. All patients presented moderate leukocytosis without neutrophilia. The mean erythrocyte sedimentation rate was 60 +/- 26. The most frequently used diagnostic tests were conventional spine radiographs, technetium-99m bone scan and magnetic resonance imaging. All patients received antibiotics; three received oral antibiotics only and the remaining patients received intravenous and oral antibiotics. The most frequently prescribed antibiotics were cefuroxime, cloxacillin and amoxicillin-clavulanate. The duration of treatment ranged between 3 and 8 weeks. All patients had a favorable outcome, although in eight, radiological sequelae were observed. CONCLUSIONS: Spondylodiscitis is not exceptional in childhood and awareness of this entity among pediatricians should be increased.

Lymphoproliferative disorders in paediatric rheumatic diseases. A report of two cases.

Lymphoproliferative disorders (LPD) are reported with a much lower frequency in children with rheumatic diseases than in their adult counterparts. We describe 2 patients who developed a lymphoma during the course of the disease. The first is a 16-year-old girl diagnosed with systemic juvenile idiopathic arthritis 6 years before who developed a mucosa-associated lymphoid tissue (MALT) lymphoma. The second report involves a boy diagnosed with systemic lupus erythematosus at 9 years of age who developed a Hodgkin's lymphoma 9 years after the disease onset. In spite of the low frequency of LPD in children with rheumatic diseases, these processes do occur.

Relapsing focal myositis: a case report.

We present a 9 year old boy with a focal myositis presenting as a pseudotumor on the right calf. Other smaller masses were present in the thigh and pretibial area on the same leg. The principal lesion recurred after two surgical excisions. Corticosteroid treatment was unsuccessful. However, methotrexate therapy resulted in a progressive regression of the lesions.

The European Spanish version of the Childhood Health Assessment Questionnaire (CHAQ) and the Child Health Questionnaire (CHQ).

We report herein the results of the cross-cultural adaptation and validation of 2 health related quality of life instruments into the European Spanish language. 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 an underlying disease. The Spanish CHQ was fully validated with 3 forward and 3 backward translations, while the Spanish CHAQ, already published, was revalidated. A total of 149 subjects were enrolled: 80 patients with JIA (28% systemic arthritis, 34% polyarthritis, 17% extended oligoarthritis, and 21% persistent oligoarthritis) and 69 healthy children. The CHAQ appropriately distinguished healthy subjects from JIA patients, with those classified in the systemic arthritis, polyarthritis and extended oligoarthritis categories having a higher degree of disability and pain, as well as a lower overall well-being than their healthy peers. The CHQ was also able to discriminate healthy subjects from JIA patients, with those allocated in the systemic arthritis, polyarthritis and extended oligoarthritis categories having a lower physical and psychosocial well-being than their healthy counterparts. In conclusion, the European Spanish version of the CHAQ-CHQ is a reliable and valid tool for the functional, physical and psychosocial assessment of children with JIA.

Systemic onset juvenile chronic arthritis, polyarticular pattern and hip involvement as markers for a bad prognosis.

OBJECTIVE: To explore all the common clinical and biological variables that are characteristic of Systemic onset Juvenile Chronic Arthritis (SoJCA) in order to determine which of them are suitable as predictors of a bad articular outcome (persistence of inflammatory symptoms and/or established limitation of the range of motion (ROM). MATERIAL AND METHODS: Clinical charts for 124 SoJCA patients were retrospectively reviewed. From them, 91 were finally included in the study because they had all of the clinical and biological data at disease onset properly recorded. All have been followed for at least 3 years since the beginning of the disease. Data collected at onset, and after 3 and 6 months of the disease included: 1) systemic symptoms; 2) joint involvement, using both the usual articular count and the value of an articular index (Helsinki Index = HI) which intentionally excludes those joints that are not uniformly recorded in clinical charts; and 3) biological data. HI was used to separate the patients into two groups. When applied 3 years after the disease onset, HI > or = 10 represented a bad articular outcome while HI < 10 meant a good prognosis. SPSS for Windows 6.1 was used for both the univariate and multivariate analyses. RESULTS: From the multivariate logistic regression analysis, two different "clusters" of clinical data were found to be the best predictors of a bad articular outcome. A bad prognosis was linked at onset with the presence of generalized lymphadenopathies, age < 8 years and an HI > 6; at six months a bad outcome was linked with the presence of a polyarticular pattern plus hip involvement. CONCLUSION: Clinical parameters at the beginning of the disease were shown to be extremely useful in predicting the articular outcome of SoJCA. Therefore, they could constitute a good instrument to help clinicians tailor the best therapy for their patients.

[Clinical characteristics of children with chronic pain in a pediatric pain unit: oncologic pain versus non-oncologic pain]. / Análisis de las características clínicas de los pacientes con dolor crónico tratados por la unidad de dolor infantil: dolor oncológico frente a no oncológico.

BACKGROUND: Despite undoubted scientific advances in the field of chronic pain in children, there is no evidence of clinical application of this knowledge. OBJECTIVE: To describe the experience of a pediatric pain unit (PPU) specifically dedicated to the treatment of chronic pain in children. MATERIAL AND METHODS: We performed an analytic, observational, retrospective, cohort study of the clinical features of the first 42 patients treated for chronic pain in the PPU during a two-year period. The patients were assigned to two groups: an oncologic group and a non-oncologic group. ANOVA was used to analyze quantitative variables and the Chi-square test was used to analyze qualitative variables. RESULTS: No significant differences were found between the two groups in the demographic variables studied (age and sex). Concerning the type of treatment used, no significant differences were found in effectiveness or compliance. However, treatment duration was significantly longer in the non-oncologic group than in the oncologic group (74.2 days vs 37.5 days, p(0.008). The duration of non-oncologic chronic pain before attending the PPU (mean: 557 days) influenced the effectiveness (r 5 0.781; p 5 0.0001) and duration of treatment (r 5 0.61; p 5 0.0051). However, the duration of previous chronic oncologic pain was significantly shorter (mean: 34 days) and showed no influence on treatment effectiveness or duration. CONCLUSIONS: The pediatric population presents chronic pain syndromes that can be appropriately treated in a PPU with conventional, easy to manage analgesics. We recommend the establishment of pediatric pain units similar to those for adults, using a multidisciplinary approach to mitigate children's suffering.

[Involvement of betamimetics in increased survival during fetal surgery in sheep]. / Implicación de los betamiméticos en el aumento de la supervivencia tras procedimientos quirúrgicos en el feto del cordero común.

INTRODUCTION: Fetal surgery is a therapeutic reality. Available only in a few centres worldwide. Experimental animal models are needed to continue research in this field. The target problem being the control of preterm labour. OBJECTIVES: To find a tocolytic drug regimen in order to reduce fetal loss. MATERIAL AND METHODS: Pregnant sheep were divided randomly in two groups. At a gestation age of seventy days sheep were operated in order to create a myelomeningocel model in the fetus. Follow-up with weekly amniocentesis guided by ultrasound scan. Group A (n = 6) were treated pre and post-op with indomethacine and with magnesium sulphate during surgery and in the following 24 hours. Group B (n = 8) were treated with diclofenac pre-op, and post-op with ritodrine until the end of pregnancy. RESULTS: Group A we find a survival rate 50% (n = 3) being 87% (n = 7) in group B. Fetal weight being higher in group A. We find an increase heart rate and a weight loss in both the fetus and mother in group B. Urea and creatinine amniotic fluid levels were increase in group B. CONCLUSIONS: The use of ritodrine as a tocolytic agent reduces fetal loss significantly. Ritodrine increases fetal and mother cardiac output causing weight loss.

[Priapism associated with Fabry's disease]. / Priapismo asociado a enfermedad de Fabry.

We present a rare case of priapism in a child, ten years old, in association with Fabry's disease. The child had a history of disseminated nodular enlargement, crises of fever, intermittent pain in the extremities and ten hours persistent painful erection of the penis. We don't obtain pain or erection relief with sedation, epidural block and irrigation of the corporal bodies. A saphenous-cavernous shunt, in the Grayhack fashion made, being results satisfactory. In the follow-up, the child had sporadic pain in the extremities and no erection of the penis. The cavernosography showed the shunt open. Fabry's disease was confirmed by nodular biopsy and the demonstration of deficient alpha-galactosidase.

[Differentiation between transient synovitis and septic arthritis of the hip with clinical and ultrasound criteria]. / Diferenciación de sinovitis transitoria y artritis séptica de cadera con criterios clínicos y ecográficos.

INTRODUCTION: Ultrasound does not distinguish between different types of synovitis. The aim of this study was to evaluate its contribution, together with several clinical data, in the diagnosis of septic arthritis (SA) and transient synovitis (TS) of the hip. METHODS: Prospective study of patients diagnosed with unilateral SA or TS of the hip carried out between December 2006 and July 2009. A set of clinical variables and ultrasound measurements were analysed. The ultrasound examinations were performed using a standardised procedure. RESULTS: The sample included 26 children, 22 diagnosed with TS and 4 with SA. A difference was found in the history of fever (P=0.002). On the other hand, no differences were detected in the age of the children, although mean and median in the TS group were 6 years vs a mean of 4.3 with a median of 2.3 years in the SA group. There were no differences in the ultrasound measurements either. The positive predictive value of the criterion "older than 4 years of age and no history of fever" for the diagnosis of transient synovitis was 100%, while "younger than 4 years and history of fever" for the diagnosis of septic arthritis was 75%, once radiology had excluded orthopaedic processes and ultrasound showed an effusion. CONCLUSIONS: In spite of the study limitations (sample size and low prevalence) the combination of age and history of fever appears to be useful in distinguishing transient synovitis from septic arthritis. The contribution of ultrasound was to confirm the presence of joint effusion.

Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study.

OBJECTIVE: Juvenile localized scleroderma (JLS) includes a number of conditions often grouped together. With the long-term goal of developing uniform classification criteria, we studied the epidemiological, clinical and immunological features of children with JLS followed by paediatric rheumatology and dermatology centres. METHODS: A large, multicentre, multinational study was conducted by collecting information on the demographics, family history, triggering environmental factors, clinical and laboratory features, and treatment of patients with JLS. RESULTS: Seven hundred and fifty patients with JLS from 70 centres were enrolled into the study. The disease duration at diagnosis was 18 months. Linear scleroderma (LS) was the most frequent subtype (65%), followed by plaque morphea (PM) (26%), generalized morphea (GM) (7%) and deep morphea (DM) (2%). As many as 15% of patients had a mixed subtype. Ninety-one patients (12%) had a positive family history for rheumatic or autoimmune diseases; 100 (13.3%) reported environmental events as possible trigger. ANA was positive in 42.3% of the patients, with a higher prevalence in the LS-DM subtype than in the PM-GM subtype. Scl70 was detected in the sera of 3% of the patients, anticentromere antibody in 2%, anti-double-stranded DNA in 4%, anti-cardiolipin antibody in 13% and rheumatoid factor in 16%. Methotrexate was the drug most frequently used, especially during the last 5 yr. CONCLUSION: This study represents the largest collection of patients with JLS ever reported. The insidious onset of the disease, the delay in diagnosis, the recognition of mixed subtype and the better definition of the other subtypes should influence our efforts in educating trainees and practitioners and help in developing a comprehensive classification system for this syndrome.

TNF-alpha promoter gene polymorphisms in Spanish children with persistent oligoarticular and systemic-onset juvenile idiopathic arthritis.

OBJECTIVE: To explore the possible association/s of the first reported tumour necrosis factor (TNF-alphaTNF-) alpha promoter gene polymorphisms -308, -238, -376 and -163 (G-->A) with systemic (SoJIA) and oligoarticular subtypes of juvenile idiopathic arthritis (JIA); and to test the association between these polymorphisms and the class I/class II HLA alleles in our population. METHODS: The patient group comprised 29 oligoarticular and 26 systemic Caucasian Spanish children with JIA; 68 healthy volunteers from the same ethnic group and geographical region served as controls. HLA alleles were determined using low-resolution polymerase chain reaction (PCR). TNF-alpha promoter gene polymorphisms were screened using PCR denaturing gradient gel electrophoresis (PCR-DGGE), followed, if positive, by restriction fragment length polymorphism (RFLP) analysis for identification. RESULTS: No statistical association was found between the four polymorphisms studied and JIA. However, the -308 G-->A polymorphism (TNF A2) tended to be more frequent in patients with SoJIA than in the oligoarticular group. TNF A2 was strongly associated with the extended haplotype A1B8DR3 (p = 0.003), and the tandem polymorphism -238/-376 in the presence of B18 and DR3. CONCLUSION: The TNF A2 allele was more frequent in SoJIA than in the oligoarticular group. TNF A2 can help to create a more inflammatory milieu in this JIA subtype, in combination with other polymorphisms involved in regulatory sequences of key molecules in the inflammatory response. The association of the -308 and -238/-376 polymorphisms with specific alleles of the HLA is reconfirmed.

Systemic lupus erythematosus: a case report with unusual manifestations and favourable outcome after plasmapheresis.

We report a case of systemic lupus erythematosus (SLE) in a 15-year-old girl with severe neurological disease, platelet function disorder and pulmonary haemorrhage, which remitted after plasmapheresis. The patient developed protein-losing enteropathy shrinking lung, and acute pancreatitis with pseudocyst formation. These infrequent complication of SLE are discussed.

Evaluation of ILAR classification criteria for juvenile idiopathic arthritis in Spanish children.

OBJECTIVE: To evaluate the proposed International League of Associations for Rheumatology (ILAR) classification criteria for juvenile idiopathic arthritis in a cohort of Spanish children. METHODS: One hundred twenty-five patients with chronic arthritis were categorized according to one of the traditional classifications and the proposed ILAR classification system after at least 6 months of disease. The traditional classifications included the European League Against Rheumatism (EULAR) criteria for pauciarticular, polyarticular rheumatoid factor (RF) negative, and systemic juvenile chronic arthritis (JCA), as well as for RF+ polyarthritis; the Vancouver criteria for juvenile psoriatic arthritis (JPsA); and the European Spondylarthropathy Study Group (ESSG) preliminary criteria for juvenile spondyloarthropathy (JSpA). RESULTS: The ILAR criteria classified 106/125 patients (84.8%). All patients with systemic and polyarticular JCA, RF+ polyarthritis, and definite juvenile psoriatic arthritis were reclassified in the corresponding ILAR category. In contrast, only 80% of pauciarticular JCA and 47% of JSpA patients could be allocated to the ILAR oligoarthritis (47/59 patients, 35 persistent and 12 extended) and enthesitis related arthritis (ErA. 8/17 patients) categories. Two children with probable PsA were reclassified in the RF- polyarthritis category. Nineteen patients (15.2%) were allocated to the ILAR "other arthritis" group, 13/19 because they did not fulfill criteria for any of the other categories (12 due to family history of psoriasis and one because of family history of HLA-B27 associated disease). The remaining 6 patients met criteria for 2 categories, RF- polyarthritis and either ErA (n = 5) or PsA (n = 1). No differences other than family history of psoriasis were found in any of the variables studied between pauciarticular JCA patients classified in the oligoarthritis (n = 47) and those in the "other arthritis" (n = 11) ILAR categories. CONCLUSION: The proposed ILAR criteria allocated 84.8% of the patients classified by traditional criteria. Family history of psoriasis (n = 12) and polyarticular onset of disease in patients with ErA (n = 5) were responsible for most of the exclusions from other ILAR categories.

Histiocytic cytophagic panniculitis: report of a case in a 12-year-old girl.

We report a 12-year-old girl with nodular skin lesions, fever, pancytopenia, coagulation defects and a fatal outcome within 4 months. Histopathology revealed proliferation of histiocytes with haemophagocytosis in skin, perivisceral fat and haemopoietic organs. The symptoms were most consistent with cytophagic histiocytic panniculitis.

Difficulty walking. A presentation of idiopathic juvenile osteoporosis.

Idiopathic juvenile osteoporosis is a rare condition that may present as difficulty walking. Difficulty walking represents a challenging differential diagnosis in pediatrics. Diagnostic studies are usually oriented to rule out neuromuscular etiologies or, once organic causes have been reasonably discarded, conversion disorders. We describe 2 patients who presented with difficulty or inability to walk due to idiopathic juvenile osteoporosis, a rare disease unfamiliar to most physicians. However, early diagnosis in patients can avoid extensive testing and prolonged hospitalizations.

Priapism and Fabry disease: a case report.

A 10-year-old boy presented with priapism of 10 h duration which after unsuccessful conservative measures, was relieved by a saphenocorporeal shunt. A 4-year history of intermittent vague aching of fingers and toes accompanied by low-grade fever was reported. Fabry disease was confirmed by the lack alpha-galactosidase activity in serum and leucocytes. Other characteristic features of Fabry disease were not present. The condition was also diagnosed in his 14-year-old brother and suspected in his maternal grandfather who died at the age of 49 from renal failure of unknown aetiology.

[Dermatomyositis in children: clinical course of 9 cases]. / Dermatomiositis infantil: evolución clínica de nueve casos.

Eight cases of dermatomyositis and one of polymyositis were studies among five male and four female patients. All had criteria definitive of the disease. All nine patients were give prednisone; six of them responded favorably to treatment and after discontinuing still remain asymptomatic. Three male patients have had a worse response to therapy, two of them deceased and the third still remains symptomatic, but very slowly improving after fourteen months with steroids and cyclophosphamide. These three patients have had chronic cutaneous vasculitic ulcers and this is a sign of bad prognosis. Both deceased patients exhibited important visceral complications (digestive and pulmonary). A more aggressive therapy has been suggested in these forms of worse clinical evolutions, including use of immunosuppressors.

[Coxalgia in adolescents: apropos of three different cases: chondrolysis, transient osteoporosis and epiphyseolysis]. / Coxalgia en adolescentes: a propósito de tres procesos diferentes: condrolisis, osteoporosis transitoria y epifisiolisis.

We report 3 adolescents presenting with hip pain. Idiopathic chondrolysis, transient osteoporosis and slipped epiphisis were the final diagnosis. Initial diagnosis may be difficult despite adequate investigation. In such cases long-term follow-up is essential. This paper includes a differential diagnosis of hip problems in childhood.