IgA deficiency and autoimmune comorbidities in Juvenile Idiopathic Arthritis.

OBJECTIVES: (1) To describe the prevalence of IgA deficiency (IgAD), uveitis, coeliac disease (CD) and thyroid disorders in a multicentric cohort of patients diagnosed with JIA and, (2) to evaluate whether patients with JIA and IgAD present other autoimmune diseases more frequently than patients with normal serum levels of IgA. METHODS: Retrospective chart review of a cohort of patients diagnosed with JIA followed at the paediatric rheumatology units of two hospitals in Madrid, Spain. RESULTS: A total of 193 patients were included. Of them, 123 were females (64%). Median age at disease onset was 5.6 years (IQR 2.5-9.7) and the median time of follow-up was 5.1 years (IQR 2.2-8.1). The three most common ILAR categories were oligoarticular (53%), polyarticular RF negative (20%) and enthesitis related arthritis (10%). Serum IgA levels were available in 172/193 (89%); 25/172 (15%) had selective (<7mg/dl, n=8) or partial (7-69mg/dl, n=17) IgAD. All the patients had periodic eye exams. Eighteen children (9%) had anterior uveitis, 15/18 chronic and 3/18 acute. Serum anti transglutaminase IgA, or IgG in IgAD were obtained in 135/193 (70%). Four children (3%) were diagnosed with CD either by intestinal biopsy (n=3) or by the combination of characteristic clinical, serological and genetic features (n=1); two of them had IgAD (p=0.12; OR=6.4; 95% CI 0.9-47.6). Only 1/153 (0.7%) patient had hyperthyrotropinemia with positive anti-thyroid antibodies and required replacement therapy. CONCLUSION: Patients with JIA frequently present autoimmune comorbidities. IgAD does not seem to increase their prevalence, with the possible exception of CD.

[Clinical and epidemiological features of SARS-CoV-2 infection in children under 16 years of age]. / Caracterización clínica y epidemiológica de la infección por SARS-CoV-2 en menores de 16 años.

INTRODUCTION: During the SARS-CoV-2 pandemic, efforts have focused on trying to identify the routes of transmis sion of the virus, characterize its symptoms and signs, and investigate the best diagnostic and thera peutic methods. There are fewer published data and series in the pediatric population than in adults. OBJECTIVE: To analyze the clinical and epidemiological characteristics in children under 16 years of age diagnosed with SARS-CoV-2. PATIENTS AND METHOD: Descriptive study carried out on children who underwent SARS-CoV-2 RNA testing due to compatible symptoms, close contact, or requiring hospitalization or surgery, in the Emergency Department of a hospital in Madrid, Spain. 30 variables were collected including epidemiological data, symptoms, and signs of infection. RESULTS: Out of 1378 patients, 12% were positive (165). There was a higher proportion of patients of North African origin in the positive group than in the negative one (p < 0.01). Of all patients, 35.6% reported close contact with a confirmed case, which was more frequent in the positive group. 75.8% of the positive patients had some symptoms, most frequently fever, runny nose, and cough, followed by digesti ve symptoms. There was one case of COVID-19 pneumonia and two patients with MIS-C, one of which had SARS-CoV-2 infection. Eight of the positive patients (4.8%) required hospitalization due to SARS-CoV-2 infection. CONCLUSION: Although SARS-CoV-2 infection is milder in the pediatric population, almost 5% will require hospitalization. No close contact was identified in a high percen tage of patients (61%). Further studies are needed at all levels of care to characterize the infection in children and adolescents.

[Hot-water epilepsy: case report in an infant]. / Epilepsia refleja por agua caliente: a propósito de un caso en un lactante.

Hot-water epilepsy is a rare type of seizure whose pathophysiology is unknown. These reflex seizures usually begin a few seconds after contact with water, commonly at temperatures between 37-48 ºC. Automations and complex partial crises, with or without secondary generalization, are the main manifestation mode of this type of reflex epilepsies. Neurological examination and intercritical electroencephalography are usually normal, which may condition a delay in diagnosis, and the clinical suspicion is fundamental. Antiepileptic treatment is initiated when associated with another type of epilepsy or when certain simple measures, such as lowering the water temperature in the bath, do not control crises. Spontaneous disappearance is possible; when pharmacological therapy is necessary, there is usually a good response. We present the case of an infant diagnosed with hot-water epilepsy. Key words: seizures, reflex epilepsies, hot water-induced reflex epilepsy.

La epilepsia refleja por agua caliente es un tipo de convulsión poco frecuente cuya fisiopatología se desconoce. Estas crisis reflejas suelen iniciarse pocos segundos tras el contacto con el agua y, comúnmente, a temperaturas de 37-48 ºC. Los automatismos y las crisis parciales complejas, con o sin generalización secundaria, son el modo de manifestación principal. La exploración neurológica y el electroencefalograma intercrítico no suelen presentar alteraciones, lo que puede condicionar un retraso en el diagnóstico, por lo que es fundamental la sospecha clínica. El tratamiento antiepiléptico se inicia cuando se asocia a otro tipo de epilepsia o cuando ciertas medidas sencillas, como el descenso de la temperatura del agua en el baño, no controlan las crisis. Es posible la desaparición espontánea. Cuando es necesaria la terapéutica farmacológica, existe, normalmente, buena respuesta. Se presenta el caso de un lactante con diagnóstico de epilepsia refleja por agua caliente.

[Myelosupression induced by linezolid: a pediatric case]. / Mielosupresión inducida por linezolid: un caso pediátrico.

Linezolid is an antibiotic of oxazolidinones family that inhibits proteical synthesis. It is used in several Gram-positive multirresistent infections. Its more frequent side effects are gastrointestinal, followed by peripheral neuropathy and myelosuppression. We report the case of a 12-year-old boy diagnosed with septic osteoarthritis of the hip and femoral osteomyelitis, following treatment with linezolid, who complained about digestive intolerance and weight loss. He showed severe normocytic anemia and mild leukopenia and thrombocytopenia with data of hematopoiesis disorder in the blood smear that suggested drug toxicity. These findings reverted when the treatment was discontinued. Reversible myelosuppression associated with linezolid is related to long treatments (more than 28 days). So it is necessary to check the blood count during long treatments.

El linezolid es un antibiótico de la familia de las oxazolidinonas, que actúa inhibiendo la síntesis proteica. Se emplea en infecciones graves por cocos Gram-positivos multirresistentes. Sus principales efectos secundarios son los gastrointestinales y, con menor frecuencia, la neuropatía periférica, la acidosis láctica y la mielosupresión. Se presenta el caso clínico de un niño de 12 años con diagnóstico de osteoartritis séptica de cadera derecha con osteomielitis femoral en tratamiento con linezolid, que presentó un cuadro de intolerancia digestiva, asociado a astenia y pérdida de peso. Presentaba, además, anemia normocítica, junto con leucopenia y trombopenia leves, con datos sugestivos de alteración de la hematopoyesis en el frotis sanguíneo, sugerente de toxicidad por fármacos. El cuadro se resolvió con la interrupción de la administración del fármaco. La mielosupresión reversible asociada a linezolid se relaciona con tratamientos prolongados (> 28 días), por lo que son necesarios los controles hematológicos periódicos durante ellos.

Anaerobio osteomyelitis. A rare condition in children / Osteomielitis por anaerobios. Una entidad poco frecuente en pediatría

ABSTRACT Osteoarticular infections due to anaerobes are very rare in children, with the Fusobacterium genus being the most frequently isolated. The course is usually subacute and, although there are predisposing factors described, most patients do not present with them. Generally, joint fluid cultures are sterile since these microorganisms are very sensitive to contact with oxygen, so they require specific culture media. All of the above causes the diagnosis to be delayed, increasing the risk of long-term sequelae. However, the prognosis improves when treatment is started early. The case is presented of a 10-year-old patient who was admitted for 30 days due septic arthritis of the right hip caused by Fusobacterium nucleatum During the admission, he required three surgical interventions, and completed 6 weeks of effective antibiotic therapy, with a good outcome and remaining asymptomatic at the current time.

RESUMEN Las infecciones osteoarticulares por anaerobios son muy raras en los niños, siendo el género Fusobacterium el que se aisla con más frecuencia. El curso suele ser subagudo y, aunque hay factores predisponentes descritos, la mayoría de los pacientes no los presenta. Generalmente, los cultivos de liquido articular son estériles ya que estos microorganismos son muy sensibles al contacto con el oxigeno, por lo que precisan medios de cultivo específicos. Todo lo anterior hace que el diagnóstico se retrase y que el riesgo de secuelas a largo plazo aumente. Sin embargo, el pronóstico mejora cuando el tratamiento se inicia de modo precoz. Por todo ello, presentamos el caso de un paciente de 10 anos con una artritis séptica de cadera derecha por Fusobacterium nucleatum que permaneció ingresado 30 dias. Durante el ingreso precisó 3 intervenciones quirúrgicas y cumplió 6 semanas de antibioterapia efectiva, con buena evolución; permanece asintomático en el momento actual.

Epilepsia refleja por agua caliente: a propósito de un caso en un lactante / Hot-water epilepsy: case report in an infant

La epilepsia refleja por agua caliente es un tipo de convulsión poco frecuente cuya fisiopatología se desconoce. Estas crisis reflejas suelen iniciarse pocos segundos tras el contacto con el agua y, comúnmente, a temperaturas de 37-48 ºC. Los automatismos y las crisis parciales complejas, con o sin generalización secundaria, son el modo de manifestación principal.La exploración neurológica y el electroencefalograma intercrítico no suelen presentar alteraciones, lo que puede condicionar un retraso en el diagnóstico, por lo que es fundamental la sospecha clínica.El tratamiento antiepiléptico se inicia cuando se asocia a otro tipo de epilepsia o cuando ciertas medidas sencillas, como el descenso de la temperatura del agua en el baño, no controlan las crisis. Es posible la desaparición espontánea. Cuando es necesaria la terapéutica farmacológica, existe, normalmente, buena respuesta.Se presenta el caso de un lactante con diagnóstico de epilepsia refleja por agua caliente.

Hot-water epilepsy is a rare type of seizure whose pathophysiology is unknown. These reflex seizures usually begin a few seconds after contact with water, commonly at temperatures between 37-48 ºC. Automations and complex partial crises, with or without secondary generalization, are the main manifestation mode of this type of reflex epilepsies.Neurological examination and intercritical electroencephalography are usually normal, which may condition a delay in diagnosis, and the clinical suspicion is fundamental.Antiepileptic treatment is initiated when associated with another type of epilepsy or when certain simple measures, such as lowering the water temperature in the bath, do not control crises. Spontaneous disappearance is possible; when pharmacological therapy is necessary, there is usually a good response.We present the case of an infant diagnosed with hot-water epilepsy.

Mielosupresión inducida por linezolid: un caso pediátrico / Myelosupression induced by linezolid: a pediatric case

El linezolid es un antibiótico de la familia de las oxazolidinonas, que actúa inhibiendo la síntesis proteica. Se emplea en infecciones graves por cocos Gram-positivos multirresistentes. Sus principales efectos secundarios son los gastrointestinales y, con menor frecuencia, la neuropatía periférica, la acidosis láctica y la mielosupresión. Se presenta el caso clínico de un niño de 12 años con diagnóstico de osteoartritis séptica de cadera derecha con osteomielitis femoral en tratamiento con linezolid, que presentó un cuadro de intolerancia digestiva, asociado a astenia y pérdida de peso. Presentaba, además, anemia normocítica, junto con leucopenia y trombopenia leves, con datos sugestivos de alteración de la hematopoyesis en el frotis sanguíneo, sugerente de toxicidad por fármacos. El cuadro se resolvió con la interrupción de la administración del fármaco. La mielosupresión reversible asociada a linezolid se relaciona con tratamientos prolongados (> 28 días), por lo que son necesarios los controles hematológicos periódicos durante ellos.

Linezolid is an antibiotic of oxazolidinones family that inhibits proteical synthesis. It is used in several Gram-positive multirresistent infections. Its more frequent side effects are gastrointestinal, followed by peripheral neuropathy and myelosuppression. We report the case of a 12-year-old boy diagnosed with septic osteoarthritis of the hip and femoral osteomyelitis, following treatment with linezolid, who complained about digestive intolerance and weight loss. He showed severe normocytic anemia and mild leukopenia and thrombocytopenia with data of hematopoiesis disorder in the blood smear that suggested drug toxicity. These findings reverted when the treatment was discontinued. Reversible myelosuppression associated with linezolid is related to long treatments (more than 28 days). So it is necessary to check the blood count during long treatments.