Hemorrhagic cystitis following allogeneic hematopoietic stem cell transplantation: experience in a pediatric oncological institution. / Cistitis hemorrágica tras trasplante alogénico de progenitores hematopoyéticos: experiencia en un centro oncológico pediátrico.

OBJECTIVE: To analyze the risk factors associated with hemorrhagic cystitis (HC) severity and the treatment strategies available in HC patients following allogeneic hematopoietic stem cell transplantation (AHSCT). MATERIALS AND METHODS: A retrospective study of medical records was carried out. Patients with HC following AHSCT treated from 2017 to 2021 were divided into two groups according to severity -mild and severe. Demographic data, disease-specific characteristics, urological sequelae, and overall mortality were compared between both groups. The hospital's protocol was used for patient management. RESULTS: 33 episodes of HC were collected in 27 patients, 72.7% of whom were male. HC incidence following AHSCT was 23.4% (33/141). 51.5% of HCs were severe (grades III-IV). Severe graft host disease (GHD) (grades III-IV) and thrombopenia at HC onset were associated with severe HC (p= 0.043 and p= 0.039, respectively). This group had longer hematuria times (p< 0.001) and required more platelet transfusions (p= 0.003). In addition, 70.6% required bladder catheterization, but only 1 case needed percutaneous cystostomy. None of the patients with mild HC required catheterization. No differences were found in terms of urological sequelae or overall mortality. CONCLUSIONS: Severe HC could be predicted thanks to the presence of severe GHD or thrombopenia at HC onset. Severe HC can be managed with bladder catheterization in most of these patients. A standardized protocol may help reduce the need for invasive procedures in patients with mild HC.

OBJETIVO: Analizar factores de riesgo asociados a la gravedad de la cistitis hemorrágica (CH) y estrategias de tratamiento en pacientes con CH tras trasplante alogénico de progenitores hematopoyéticos (TAPH). MATERIAL Y METODOS: Estudio retrospectivo de historias clínicas. Los pacientes con CH tras TAPH tratados entre 2017 y 2021 se dividieron en dos grupos según la gravedad del cuadro (leve y grave). Se compararon datos demográficos, características específicas de la enfermedad, secuelas urológicas y mortalidad global entre ambos grupos. Se utilizó el protocolo del hospital para el manejo de los pacientes. RESULTADOS: Se recogieron 33 episodios de CH en 27 pacientes, de los cuales el 72,7% fueron varones. La incidencia de CH tras TAPH fue del 23,4% (33/141). El 51,5% de las CH fueron graves (grados III-IV). La enfermedad de injerto contra huésped (EICH) grave (grados III-IV) y la trombopenia al inicio se asociaron a CH grave (p= 0,043 y p= 0,039, respectivamente). Este grupo tuvo mayor tiempo de hematuria (p< 0,001) y necesitó más transfusiones de plaquetas (p= 0,003). Además, el 70,6% precisó sondaje vesical, pero solo un caso cistostomía percutánea. Ningún paciente con CH leve precisó sondaje. No hubo diferencias en las secuelas urológicas ni en la mortalidad global. CONCLUSIONES: Una CH más grave podría predecirse por la presencia de EICH grave o trombopenia al inicio del cuadro. La CH grave puede manejarse con sondaje vesical en la mayoría de estos pacientes. Seguir un protocolo estandarizado puede reducir la necesidad de procedimientos invasivos en pacientes con CH leve.

Pediatric perianal Crohn's disease behavior in the era of biologic therapy. / Comportamiento de la afectación perianal en pacientes pediátricos con enfermedad de Crohn en la era de la terapia biológica.

AIM OF THE STUDY: To describe perianal Crohn's disease behavior and the role of biological therapy in a sample of pediatric patients. METHODS: A retrospective study of pediatric patients with Crohn's disease (CD) treated in our institution from 2017 to 2021, with a minimum follow up period of 6 months, was conducted. Patients were divided whether they had perianal disease (PD) or not. Baseline characteristics, extension of disease, growth failure rate, aggressive pattern rate, use of biological therapy and need for surgery, among other variables, were compared between both groups. Clinical and/or radiological improvement in the last 6 months of follow up was considered good control of PD. RESULTS: Seventy eight pediatric patients with CD were included. Median age at diagnosis was 10.5 years, and median follow up time was 3.8 years. 64.1% patients were male. Of all, 15 (19.2%) had perianal disease, of which 10 had fistulizing findings and 5 had non fistulizing findings. PD was presented at diagnosis in 8 patients, and the rest developed it in a median time of 1 year from diagnosis. PD was associated with growth failure (p = 0.003), use of biological therapies (p = 0.005), and need for second line of biologics (p = 0.005). Most patients (12/15, 80%) had good control of PD with the treatment received. CONCLUSIONS: CD patients with PD seem to need a more aggressive treatment, with biological therapies playing a key role for its handling nowadays. These patients require close nutritional evaluation that ensures proper development and growth.

OBJETIVO DEL ESTUDIO: Describir el comportamiento de la enfermedad de Crohn perianal y el papel de la terapia biológica en una muestra de pacientes pediátricos. METODOS: Estudio retrospectivo de pacientes pediátricos con enfermedad de Crohn (EC) tratados en nuestro centro entre 2017 y 2021, con un seguimiento mínimo de seis meses. Los pacientes se dividieron en función de si tenían enfermedad perianal (EP) o no. Se compararon entre ambos grupos las características iniciales, la extensión de la enfermedad, el índice de retraso en el crecimiento, el índice de patrón agresivo, el empleo de terapia biológica y la necesidad de cirugía, entre otras variables. Se consideró un buen control de la EP una mejoría clínica o radiológica en los 6 últimos meses de seguimiento. RESULTADOS: Se incluyeron 78 pacientes pediátricos con EC. La edad mediana en el momento del diagnóstico fue de 10,5 años, y el tiempo mediano de seguimiento fue de 3,8 años. El 64,1% de los pacientes eran varones. Del total, 15 (19,2%) tenían enfermedad perianal, de los cuales 10 presentaban hallazgos fistulizantes y 5 no fistulizantes. La EP estaba presente en el momento del diagnóstico en 8 pacientes, y el resto la desarrolló en una mediana de 1 año desde el diagnóstico. La EP se asoció con retraso en el crecimiento (p = 0,003), empleo de terapias biológicas (p = 0,005) y necesidad de una segunda línea de terapia biológica (p = 0,005). La mayoría de los pacientes (12/15, 80%) tuvieron un buen control de la EP con el tratamiento recibido. CONCLUSIONES: Los pacientes de EC con EP parecen necesitar un tratamiento más agresivo, en el que las terapias biológicas desempeñan hoy en día un papel fundamental. Estos pacientes precisan de una estrecha evaluación nutricional que garantice su correcto crecimiento y desarrollo.

Fisioterapia deportiva / Sports Physical therapy

Se ha realizado un estudio para conocer la opinión de los deportistas amateur acerca de la labor del fisioterapeuta en el ámbito deportivo. Una vez más se ha demostrado que hay una confusión en los conceptos de fisioterapeuta y masajista, así como se detecta la falta de los mismos dentro de los clubes o asociaciones con recursos económicos reducidos (AU)

Renal tubular acidosis in the Silver-Russell syndrome.

Several patients with the Silver-Russell syndrome (SRS) attending our Genetics Clinic were diagnosed as having persistent metabolic acidosis. Since this abnormality has not been reported previously in the SRS, we reexamined 33 SRS patients to evaluate the frequency and type of metabolic acidosis, the clinical and laboratory findings, and the growth pattern in SRS patients with and without metabolic acidosis. Among them, 14 had a consistent decrease in HCO3- levels. Renal studies in acidotic patients showed urine pH of 5.8 and 24 h urine calcium of < 2.4 mg/kg/24 h; serum creatinine, excretion of glucose, and amino acids were normal, as were renal ultrasound and excretory urography findings. These data supported the diagnosis of renal tubular acidosis, probably type II; the patients were treated with oral bicarbonate and acidosis was corrected successfully. Clinical manifestations were similar in acidotic and non-acidotic patients. The nutritional indices at diagnosis and at last evaluation (at least 8 months after diagnosis) were abnormally low in all patients; however, acidotic patients, treated with bicarbonate, showed an improvement of nutritional status particularly in the weight/height index, although the difference between groups after follow-up did not reach statistical significance. We suggest that metabolic acidosis due to renal tubular acidosis, probably type II, may occur in children with the SRS and should be looked for and treated in all patients.

[Nephropathy in Schönlein-Henoch purpura]. / Nefropatía en la púrpura de Henoch Schonlein.

Over one hundred patients diagnosed with vascular purpura at the National Institute of Pediatrics between 1979 and 1988 were retrospectively studied. Frequency was similar for both sexes. 82% were between the ages of six months and 11 years old. The extrarenal clinical manifestations were: petechias in 98%; abdominal pain 78% arthralgias 45%; melena 39%; and arthritis in 19% of the cases, 49 patients had nephropathy of different degrees and they did differently, 32 had hematuria with or without proteinuria within a nephrotic range, seven suffered from a nephrotic syndrome, one with a nephritic syndrome and nine others had a combination of two or three syndromes (nephritic/nephrotic/renal failure) with an unfavorable evolution towards terminal renal failure in eight of them; on the other hand, this was not seen in the remaining patients. Thirteen renal biopsies were taken from patients with more severe clinical manifestations, finding in them mesangial proliferation or endo- and extracapillary proliferation. These findings suggest that the initial clinical presentation of the illness allows for the prediction of the future.