Limosilactobacillus reuteri ATCC PTA 5289 and DSM 17938 as adjuvants to improve evolution of pharyngitis/tonsillitis in children: randomised controlled trial.

Pharyngitis and tonsillitis are the most common acute respiratory infections (ARIs) in children aged ≤5 years. The analysis of published data showed that some probiotics could decrease the frequency and number of days with ARIs. This study evaluated the safety and efficacy of Limosilactobacillus reuteri ATCC PTA 5289 and DSM 17938 to reduce the duration and severity of ARI symptoms. This randomised controlled trial included children aged from 6 months to 5 years, with pharyngitis or tonsillitis, who were randomised to receive a probiotic product containing L. reuteri ATCC PTA 5289 and L. reuteri DSM 17938 or placebo, as drops, ingested orally for 10 days as adjuvants to the use of non-steroidal anti-inflammatory drugs. The main outcomes were the duration and severity of ARI symptoms. The secondary outcomes were changes in salivary immunoglobulin A and inflammatory biomarkers. There was no fever on day 2 and subsequent days in the L. reuteri group (37.3 ±0.5 °C vs 38.6±0.3 °C, P<0.05). Beginning on day 3, the severity of sore throat (5±0.9 vs 8±1.2, P<0.05) was lower in the L. reuteri group. Significant differences in the days with runny nose, nasal congestion, days of non-programmed visits to the medical office or emergency department, levels in tumoral necrosis factor-alpha (TNF-alpha) and related costs of treatment were observed in the L. reuteri group. The frequency of adverse events was similar between the groups. Therefore, L. reuteri ATCC PTA 5289 combined with L. reuteri DSM 17938 is a safe and effective adjunct to reduce the symptoms of pharyngitis or tonsillitis in children.

When should we use partially hydrolysed formulae for frequent gastrointestinal symptoms and allergy prevention?

UNLABELLED: Experts reviewed the literature to determine whether partially whey hydrolysed formulas (HF) offer benefits in the dietary management of frequent gastrointestinal symptoms and allergy prevention. Compared with standard cow's milk-based formulas, partially whey HF confer a limited protective effect against allergic disease in high-risk infants, particularly atopic dermatitis, but not respiratory allergies. No randomised clinical trials have been published on partially whey HF in infants with colicky symptoms. The group did not find sufficient evidence to support the use of partially whey HF in regurgitation, although recent data suggest that a thickened partially whey HF may be more effective. Partially whey HF, fortified with prebiotics and/or probiotics, with high levels of sn-2 palmitate in the fat blend or without palm oil, provide some benefit in functional constipation. CONCLUSION: Overall, partially whey HF may offer a useful alternative to intact protein in the dietary management of common functional gastrointestinal symptoms.

Survival of Mexican patients with paediatric-onset systemic lupus erythematosus and abnormal electroencephalogram

Background: Over the past three decades, there has been a remarkable improvement in the outcome of children diagnosed with systemic lupus erythematosus (SLE). In general, paediatric-onset SLE has been associated with higher mortality rates and more disease damage than adults with SLE. The objective was to determinate the impact of clinical, laboratory, and electroencephalographic findings on survival amongst patients with paediatric-onset SLE. Methods: Charts of Mexican patients with paediatric-onset SLE diagnosed between 1970 and 2001 were analysed retrospectively; univariate and multivariate analyses were used for analysing associations between clinical and laboratory features and death; Kaplan–Meier tests were used to estimate survival curves. Results: 159 patients were included, 105 were female, with a median age of 12.7 years at diagnosis and a median duration of symptoms prior to diagnosis of 8.4 months. Univariate analysis showed that haematuria, leukocyturia, proteinuria, presence of urine cast, <60% glomerular filtration rate, haemolytic anaemia, and abnormal electroencephalogram, were all poor prognostic factors (p<0.05). Multivariate analysis showed that the presence of proteinuria and abnormal electroencephalograms (p<0.05) were independent factors associated with death. The overall survival rate was 82.9% at five years and 77.4% at ten years upon follow-up. Infection and high disease activity were the most common causes of death. Conclusions: Survival of paediatric-onset SLE patients was lower compared to that reported for patients in wealthier countries. Amongst the patients who died, the presence of proteinuria and abnormal electroencephalograms were found to be determinant for survival. Infection and activity were the most common causes of death(AU)

Survival of Mexican patients with paediatric-onset systemic lupus erythematosus and abnormal electroencephalogram.

BACKGROUND: Over the past three decades, there has been a remarkable improvement in the outcome of children diagnosed with systemic lupus erythematosus (SLE). In general, paediatric-onset SLE has been associated with higher mortality rates and more disease damage than adults with SLE. The objective was to determinate the impact of clinical, laboratory, and electroencephalographic findings on survival amongst patients with paediatric-onset SLE. METHODS: Charts of Mexican patients with paediatric-onset SLE diagnosed between 1970 and 2001 were analysed retrospectively; univariate and multivariate analyses were used for analysing associations between clinical and laboratory features and death; Kaplan-Meier tests were used to estimate survival curves. RESULTS: 159 patients were included, 105 were female, with a median age of 12.7 years at diagnosis and a median duration of symptoms prior to diagnosis of 8.4 months. Univariate analysis showed that haematuria, leukocyturia, proteinuria, presence of urine cast, <60% glomerular filtration rate, haemolytic anaemia, and abnormal electroencephalogram, were all poor prognostic factors (p<0.05). Multivariate analysis showed that the presence of proteinuria and abnormal electroencephalograms (p<0.05) were independent factors associated with death. The overall survival rate was 82.9% at five years and 77.4% at ten years upon follow-up. Infection and high disease activity were the most common causes of death. CONCLUSIONS: Survival of paediatric-onset SLE patients was lower compared to that reported for patients in wealthier countries. Amongst the patients who died, the presence of proteinuria and abnormal electroencephalograms were found to be determinant for survival. Infection and activity were the most common causes of death.

MTHFR C677T, FII G20210A, FV Leiden G1691A, NOS3 intron 4 VNTR, and APOE epsilon4 gene polymorphisms are not associated with spontaneous cervical artery dissection.

AIMS: The pathogenesis of spontaneous cervical artery dissection remains unknown. We examined the association between different polymorphisms frequently found in young patients with cryptogenic stroke [methylenetetrahydrofolate reductase (MTHFR) C677T, factor II (prothrombin) G20210A, factor V G1691A (Leiden), nitric oxide synthase 3 (NOS3) intron 4 VNTR, and apolipoprotein E (APOE) epsilon4 gene] in patients with a cerebral infarct caused by spontaneous cervical artery dissection. METHODS: Forty-eight patients (27 males) and 96 matching control subjects were recruited. Clinical history, including cardiovascular risk factors, was assessed in all subjects. Genotypes were determined by a polymerase chain reaction with and without a restriction fragment length polymorphism. The genotypes and allele frequencies of the five genetic variants studied were compared between spontaneous cervical artery dissection cases and controls. We also incorporated our data into a meta-analysis of the MTHFR/C677T variant. RESULTS: Of 48 patients with spontaneous cervical artery dissection (28 vertebral and 20 carotid), the mean age of the patients was 36.6 +/- SD 9.9 years. There were no significant associations between the alleles of the five genetic polymorphisms studied and spontaneous cervical artery dissection. In the meta-analysis of the MTHFR/C677T variant, a total of 564 individuals (231 cases and 333 controls) were analysed; no significant association was observed. CONCLUSIONS: The results from this exploratory case-control study show the lack of an association between MTHFR, factor II G20210A, factor V G1691A, NOS3, intron 4 VNTR, and APOE epsilon4 gene polymorphisms and the development of spontaneous cervical artery dissection. Our findings contribute towards a better understanding of the genetic risk factors associated with spontaneous cervical artery dissection.

Manejo de la gastroenteritis aguda en menores de 5 años: un enfoque basado en la evidencia / An evidence based Iberic-Latin American guideline for acute gastroenteritis management in infants and prescholars

La morbi-mortalidad en menores de 5 años por gastroenteritis aguda (GEA) en países en desarrollo sigue siendo elevada. Los autores han elaborado un documento que ayude a tomar decisiones en el tratamiento del menor de 5 años con GEA en el contexto Ibero-latinoamericano. Se realizó una revisión sistemática de la literatura (mayo 2008). La gradación de la evidencia se realizó siguiendo las guías Oxford y expertos latinoamericanos opinaron respecto a las recomendaciones. La rehidratación oral representa la piedra angular del tratamiento de la GEA en niños, asociándose a menos efectos adversos que la rehidratación intravenosa. La GEA no es contraindicación para la alimentación normal. Racecadotrilo, zinc y esmectita pueden coadyuvar al tratamiento, así como Lactobacillus GG y Saccharomyces boulardii. No se recomiendan otros fármacos. En el tratamiento de niños con GEA se recomienda la rehidratación oral junto con racecadotrilo, zinc o esmectita, y algunos probióticos (AU)

Acute gastroenteritis (AG) morbidity and mortality rates in infants and prescholars continue to be high in developing countries. Authors want to develop an evidence-based document that supports decision making regarding AG therapy in infants and children younger than 5 y/o. A systematic review of the literature was performed (May, 2008). Evidence grading was established according to Oxford guidelines and Latin American experts submitted their opinions on the recommendations generated. Oral rehydration solutions are the threatment's keystone for children with AG, showing lesser complications due to therapy than IV fluids. AG is no contraindication of a normal diet. Racecadotril, zinc and smectite can contribute to AG treatment, as well as Lactobacillus GG and Saccharomycces boulardii. No other drugs are recommended. It is recommended to treat children presenting AG with oral rehydration solutions among racecadotril, zinc or smectite as well as some probiotics (AU)

[An evidence based Iberic-Latin American guideline for acute gastroenteritis management in infants and prescholars]. / Manejo de la gastroenteritis aguda en menores de 5 años: un enfoque basado en la evidencia Guía de práctica clínica Ibero-Latinoamericana.

Acute gastroenteritis (AG) morbidity and mortality rates in infants and prescholars continue to be high in developing countries. Authors want to develop an evidence-based document that supports decision making regarding AG therapy in infants and children younger than 5 y/o. A systematic review of the literature was performed (May, 2008). Evidence grading was established according to Oxford guidelines and Latin American experts submitted their opinions on the recommendations generated. Oral rehydration solutions are the threatment's keystone for children with AG, showing lesser complications due to therapy than IV fluids. AG is no contraindication of a normal diet. Racecadotril, zinc and smectite can contribute to AG treatment, as well as Lactobacillus GG and Saccharomycces boulardii. No other drugs are recommended. It is recommended to treat children presenting AG with oral rehydration solutions among racecadotril, zinc or smectite as well as some probiotics.

[Increased risk of neoplasia among relatives of glioma patients]. / Incremento en el riesgo de neoplasia entre familiares de pacientes con glioma.

INTRODUCTION: Some previous studies have suggested familial aggregation of gliomas, although the results have not always been replicated. SUBJECTS AND METHODS: In the present study of a Mexican population, we compared 100 cases of glioma with 124 healthy unrelated controls, as well as their 1st, 2nd and 3rd degree relatives (n = 3,575 and 4,520 respectively). RESULTS: The relatives of the cases had a significantly higher risk of developing brain tumors than the relatives of controls (OR = 5.3; p < 0.05; 95% CI = 1.1-25.7), and their risk of developing any cancer was also increased (OR = 2; p < 0.05; 95% CI = 1.16-3.51), this risk was twofold for men when compared to females (OR = 2; p < 0.05; 95% CI = 1.15-3.37). CONCLUSION: The present study supports familial aggregation of brain tumors and warrants further research into their genetic etiology.

Incremento en el riesgo de neoplasia entre familiares de pacientes con glioma / Increased risk of neoplasia among relatives of gliomapatients

Introducción. Estudios previos han sugerido que existe agregación familiar de gliomas; sin embargo, los resultados no siempre han sido replicables. Sujetos y métodos. En el presente estudio de una población mexicana, comparamos 100 casos de glioma con 124 controles sanos no emparentados, así como sus familiares de primer, segundo y tercer grado (n = 3.575 y 4.520, respectivamente). Resultados. Los familiares de los casos tuvieron un riesgo significativamente mayor de desarrollar tumores cerebrales que los familiares de los controles (odds ratio, OR = 5,3; p < 0,05; intervalo de confianza al 95%, IC 95% = 1,1-25,7), su riesgo de desarrollar cualquier tipo de cáncer también fue mayor (OR = 2; p < 0,05; IC 95% = 1,16-3,51), y este riesgo fue el doble para varones que para mujeres (OR = 2; p < 0,05; IC 95% = 1,15-3,37). Conclusión. El presente estudio apoya la existencia de agregación familiar de neoplasias cerebrales y obliga a profundizar en el estudio de su etiología genética

Introduction. Some previous studies have suggested familial aggregation of gliomas, although the results have not always been replicated. Subjects and methods. In the present study of a Mexican population, we compared 100 cases of gliomawith 124 healthy unrelated controls, as well as their 1st, 2nd and 3rd degree relatives (n = 3,575 and 4,520 respectively).Results. The relatives of the cases had a significantly higher risk of developing brain tumors than the relatives of controls (OR = 5.3; p < 0.05; 95% CI = 1.1-25.7), and their risk of developing any cancer was also increased (OR = 2; p < 0.05; 95% CI = 1.16-3.51), this risk was twofold for men when compared to females (OR = 2; p < 0.05; 95% CI = 1.15-3.37). Conclusion. The present study supports familial aggregation of brain tumors and warrants further research into their genetic etiology

Systemic inflammation, endothelial dysfunction, and activation in clinically healthy children exposed to air pollutants.

Mexico City children are chronically exposed to significant concentrations of air pollutants and exhibit chronic respiratory-tract inflammation. Epidemiological, controlled human exposures, laboratory-based animal models, and in vitro/in vivo studies have shown that inflammatory, endothelial dysfunction, and endothelial damage mediators are upregulated upon exposure to particulate matter (PM). Endothelial dysfunction is a critical event in cardiovascular disease. The focus of this work was to investigate whether exposure to ambient air pollution including PM(2.5) produces systemic inflammation and endothelial injury in healthy children. We measured markers of endothelial activation, and inflammatory mediators in 52 children age 8.6+/-0.1 yr, residents of Mexico City (n: 28) or of Polotitlán (n: 24), a city with low levels of pollutants. Mexico City children had significant increases in inflammatory mediators and vasoconstrictors, including tumor necrosis factor (TNF)alpha, prostaglandin (PG) E2, C-reactive protein, interleukin-1beta, and endothelin-1. There was a significant anti-inflammatory response, and a downregulation of vascular adhesion molecule-1, intercellular adhesion molecule-1 and -2, and selectins sE and sL. Results from linear regression found TNF a positively associated with 24- and 48-h cumulative levels of PM(2.5), while the 7-d PM(2.5) value was negatively associated with the numbers of white blood cells in peripheral blood in highly exposed children. Systemic subclinical inflammation, increased endothelin- 1, and significant downregulation of soluble adhesion molecules are seen in Mexico City children. Children chronically exposed to fine PM above the standard could be at risk of developing cardiovascular diseases, atherosclerosis, stroke, and other systemic effects later in life.

Cutaneous mastocytosis in children: a clinical analysis of 71 cases.

OBJECTIVE: To characterize the clinical features, response to therapy, evolution and prognosis of cutaneous mastocytosis in children. BACKGROUND: Mastocytosis in children, instead of being induced by a potentially oncogenic c-kit mutation, is probably a clonal disease with benign prognosis. METHODS: The clinicopathological features, evolution and response to treatment were analysed in 71 children with mastocytosis. RESULTS: There were 53 (75%) cases of urticaria pigmentosa, 12 (17%) cases of mastocytoma, and six (8%) cases of diffuse cutaneous mastocytosis. In 92% of cases disease onset was in the first year of life. There was a male predominance 1.8 : 1. Treatment did not modify the disease evolution. Eighty per cent of patients improved or had spontaneous resolution of the disease. CONCLUSION: The most frequent clinical form of mastocytosis was urticaria pigmentosa followed by mastocytoma and diffuse cutaneous mastocytosis. Darier's sign was present in 94% of cases. A negative Darier's sign does not rule out mastocytosis. In contrast to adults, mastocytosis in children usually has a benign course making sophisticated or invasive diagnostic tests unnecessary. A classification of paediatric cutaneous mastocytosis is proposed.

Cisapride and QTc interval in children.

BACKGROUND: Recent reports about cisapride have raised some concerns about the safety and efficacy of this medication in children. The aim of this study was to identify electrocardiographic changes and a predisposition to develop arrhythmias in children. METHODS: Patients were divided in 2 groups: 1) 63 children (mean age: 29 months) who received cisapride (0.2 mg/kg/dose 3 times/day), and 2) 57 children (mean age: 27 months) who did not receive cisapride (they served as controls). Both groups did not have any associated disease. Electrocardiogram (EKG) was performed to children when they were included in the study. The QT interval was corrected using Bazett's formula. Twenty-four-hour Holter recording was performed in children with prolonged QT interval (PQTI). When PQTI was identified in group 1, cisapride was discontinued and a new EKG was performed. RESULTS: Five children from group 1 and 6 from group 2 had PQTI. In 3 children with PQTI, the QTc interval returned to normal values when cisapride was discontinued. In children under 4 months of age, a statistical difference was found, with QTc interval being longer in group 2 (without cisapride) than in group 1. Holter recordings were normal in all children with PQTI. CONCLUSION: PQTI can be found in normal children with or without cisapride. In our study PQTI was not associated with any life-threatening event.

[Anthelmintics as a risk factor in intestinal obstruction by Ascaris lumbricoides in children]. / Antihelmínticos como factor de riesgo en la obstrucción intestinal por Ascaris lumbricoides en niños.

In a retrospective study the authors analyzed the clinical records of 199 children ages one month to 16 years hospitalized, with the diagnosis of intestinal ascariasis, in the Instituto Nacional de Pediatria of Mexico from 1984 to 1999. The purpose of the study was to evaluate the use of anthelmintics drugs as a risk factor of intestinal obstruction by A. lumbricoides. Two groups were made for the study: Group A (n = 66) of children who presented intestinal obstruction, Group B (n = 133) children with no complications. A comparative analysis of clinical data of both groups was made by means of chi square with Yates correction and a stratified analysis by means of chi square. Possible confusing elements were overcrowding, age and the use of antiparasitic drugs. The calculus of risk factors for intestinal obstruction by A. lumbricoides was done by means of contingency tables of 2 x 2 and odds ratio with an IC of 95%. The significant risk factors were included in a model of logistics regression with an impact variable consting in the presence or absence of intestinal obstruction in order to establish a multivariate model of predictive risk at level of significance of p < 0.05. Twenty-seven patients (40.90%) in group A (n = 66) were given anthelmintics medications prior to the intestinal obstruction: mebendazol, 14 (51-85%); two, albedazol (7.4%); eight, a non-specified anthelmintic (29.6%). In addition, an anthelmintic medication without a specified time of ingestion: two with mebendazol and one with piperazine (11.3%). In the case of mebendazol, the drug most frequently associated with intestinal obstruction, seven patients received it on the same day of the obstruction; five patients received it between one and seven days prior to the obstruction; two received it seven days prior to the complication. In the control group, only 7% had taken the anthelmintic one to seven days before the diagnosis of uncomplicated intestinal ascariasis diagnosis was made. With the step by step (Backward) logistic regression conditioned by the treatment variable with an anthelmintic, an X2 = 38.15 gl, p < 0.000 was obtained for which reason it was considered by A. lumbricoides. Of the probable risk factors analyzed in this study, the only one capable of influencing and predicting the presentation of intestinal obstruction by A. lumbricoides in children, was the prior anthelmintic treatment particularly with mebendazol.

Are oxygen indices effective for predicting pathological intrapulmonary shunt in mechanically ventilated children?

BACKGROUND: This study was undertaken to evaluate whether oxygen indices accurately predict pathological intrapulmonary shunt (Qsp/Qt), and to evaluate the sensitivity and specificity of the FiO2-required formula to obtain a desired arterial oxygen tension (PaO2) in mechanically ventilated children. METHODS: A prospective, hospital-based, comparative study was conducted on 50 mechanically ventilated children at the Intensive Care Units of the National Institute of Pediatrics (INP) in Mexico City. Blood gas data were prospectively collected from 50 critically ill, mechanically ventilated children, 50 taken before and 40 after FiO2 change. Assessment of Qsp/Qt, P(A-a)O2, PaO2/FiO2, PaO2/PAO2, and P(A-a)O2/PaO2 was carried out before and after FiO2 change. RESULTS: In first blood gas data, 31 patients were hypoxemic (PaO2 < 90 Torr), 10 were normal, and 9 were hyperoxemic (PaO2 > 100 Torr). Forty patients required FiO2 modifications that were carried out according to Maxwell's formula. Five children showed persistent oxygen disturbance after FiO2 changes. P(A-a)O2, PaO2/FiO2, PaO2/PAO2, and P(A-a)O2/PaO2 had sensitivities of 0.66, 0.71, 0.98, and 0.93, respectively, and specificities of 0.79, 0.91, 0.29, and 0.64, respectively, to detect pathological Qsp/Qt. All oxygen indices changed significantly after FiO2 modifications compared from initials; Qsp/Qt also showed significant change after FiO2 change. Pearson product-moment showed lineal correlation between each index, and Qsp/Qt demonstrated their significant correlation (p < 0.01). Correlation of Qsp/Qt and PaO2/FiO2 and PaO2/PAO2 was significantly higher in younger children (< 13 years) p < 0.05. The FiO2-required formula to obtain a desired PaO2 had a sensitivity of 0.93 and a specificity of 0.75. CONCLUSIONS: The oxygen indices showed sufficient efficacy to detect pathological intrapulmonary shunt, and to have a statistically significant lineal correlation that permits its use during the clinical evaluation of oxygen transport data in most mechanically ventilated children, which is consistent with other reports on adult populations. However, one limitation for its use in clinical assessment, as reported in previous studies, would be that all indices in the present study are FiO2-dependent; therefore, when the FiO2 varies, the use is misleading. The FiO2-required formula is efficient for defining the appropriated FiO2 for the obtaining of the desired PaO2, but will always be merely a guide that should be confirmed through blood gas analysis.

[Plasma fibronectin in pediatric patients]. / Fibronectina plasmática en pacientes pediátricos.

OBJECTIVE: To determine the levels of plasma fibronectin (FBN) in pediatric patients and to correlate them with serum albumin (ALB). METHODOLOGY: The FBN was measured by nephelometry and ALB by enzymatic analysis in samples from 95 healthy Mexican pediatric patients seen in the ambulatory surgery department. RESULTS: The levels of FBN was similar in boy and girls (mean +/- SD) 273 +/- 90 and 268 +/- 106 micrograms/mL respectively. FBN was significantly lower in children less than 6 months old. We found seven patients with ALB < 3.0 g/dL who had significantly lower values of FBN than the normoalbuminemic patients. The correlation of FBN and ALB was low (r = 0.23). CONCLUSIONS: FBN in pediatric patients is significatively lower than in adults. The lowest concentrations were seen in children less than 6 months old. These lower FBN may be an additional factor for immunodeficiency in this group at risk.

PRISM score evaluation to predict outcome in pediatric patients on admission at an emergency department.

This study was undertaken in order to evaluate for the first time the usefulness of PRISM score to predict outcome in pediatric patients in the Intensive Care Area of the Emergency Department at the Instituto Nacional de Pediatría in Mexico City. A prolective evaluation of PRISM score was done using 100 consecutive pediatric patients admitted to INP-ED between July and November 1992 and considered critically ill by the attending pediatricians to calculate by a lineal logistic model the expected mortality and compare with the observed one. Using a cut-off of r = 0, we evaluated at the same time the sensitivity, specificity and efficiency of this score. Fifty-eight patients were male and 42 were female. The mean age was 51 months with a range of 3 days -192 months. PRISM score for survivors was in general 8.7 +/- 7.2 and 25.8 +/- 14 for nonsurvivors (p < 0.001). Based on the logistic regression coefficients defined by Pollack et al., our sample of 100 patients was estimated to expect 12.91 deaths whereas in fact 11 were observed. Inspection of the survival rates across the different categories of expected mortality showed agreement and consistency in relation to original reports (9). The sensitivity, specificity and efficiency in general were 1.0, 0.98 and 0.98, respectively. The PRISM is an objective and efficient method which helps physicians to predict patients' outcome and risk of mortality, providing the medical staff with an epidemiological criteria. Additionally, it may be helpful in decision-making for ICU admissions and correct identification of patients who can benefit from that level of care.

[Spondyloepiphyseal dysplasia tarda with progressive arthropathy: 2 siblings affected]. / Displasia espóndilo epifisiaria tarda con artropatía progresiva: 2 hermanos afectados.

We described a Mexican family whose parents were consanguineous. Therefore two children were affected by a progressive arthropathy with deformity in all finger joints, restricted joint mobility and broad major joints. This condition was diagnosed like atypical juvenile rheumatoid arthritis (JRA) because the test for serum rheumatoid factors and antibodies were negative and failed to respond to anti-rheumatoid treatment. However their radiographic studies showed the spine with universal platyspondyly, enlargement epiphyses of the hands, the absence of destructive and the presence of the dysplastic bone changes. These manifestations permit us to do the diagnosis of spondyloepiphyseal dysplasia tarda with progressive arthropathy. In this report we suggest that a complete radiologic study of the patient will allowed to diagnosis this hereditary autosomal recessive entity; likewise it will let us differ of JRA and others polyarticular conditions of childhood.