Stem-cell transplantation for children with primary immune deficiencies: A retrospective study of 19 patients from one centre in Mexico.

INTRODUCTION: For many patients with primary immune deficiency (PID), stem-cell transplantation (SCT) may be life-saving. OBJECTIVE: To review our experience of 11 years transplanting children with PID in Mexico. METHODS: Chart review of patients who underwent SCT from 2008 to 2018, to describe their diagnoses, time to transplant, conditioning regime, survival rate and outcomes. All patients received post-transplant cyclophosphamide as graft-versus-host-disease (GVHD) prophylaxis. RESULTS: 19 patients with combined, phagocytic or syndromic PID from 5 states. Twelve of them were male (58%) and 14 survive (79%). Mean age at HSCT was 41.9 months; mean time from diagnosis was 31.2 months. Seven grafts were umbilical cord and 12 haploidentical. The conditioning regime was myeloablative, with five primary graft failures. Two patients had partial and 10 full chimerism. Five patients died within 2 months after transplant. Immune reconstitution was complete in 11 of 19 patients. We found a prevalence of 21% GVHD. DISCUSSION: We describe 19 patients from Mexico with 8 PID diagnoses who underwent allogenic HSCT over a period of 11 years. Survival rate and other outcomes compare well with industrialized countries. We recommend the use of post-transplant cyclophosphamide to prevent GVHD in scenarios of resource scarcity and a lack of HLA-identical donors.

Conversion factor for size specific dose estimation of head CT scans based on age, for individuals from 0 up to 18 years old.

Assessing the radiation doses received by patients in computed tomography is still challenging. To overcome this, the American Association of Physicists in Medicine has introduced the concept of the size specific dose estimate (SSDE). However, the calculation of SSDE for head CT scans requires the knowledge of attenuation characteristics of the volume scanned, making its implementation in the daily clinical workflow cumbersome. In this study, we defined conversion coefficients from CTDIvol,16cmto SSDE for head CT scans based solely on the age of the patient. Using the head circumference-for-age from the child growth standards of the World Health Organization (WHO), the effective diameter-for-age was calculated for male and female individuals from 0 to 60 months-old. The effective diameter was converted into a water equivalent diameter-for-age, using a correlation established from the measurements of both quantities in 295 exams of male and female patients, from 0 to 18 years-old. WHO-estimated water equivalent diameter-for-age was validated against the measured water equivalent diameter-for-age. The head circumference-for-age from WHO was extrapolated for male and females individuals up to 18 years-old and their respective water equivalent diameter were estimated. Finally, the SSDE was calculated for all the CT head scans performed in a 9-years period in patients aged from 0 to 18 years old. Typical values of CTDIvol,16cmand DLP were also defined. SSDE varied from 0.80 up to 1.16 of the CTDIvol,16cm, depending on sex and age of the patient. WHO-estimated water equivalent diameter-for-age differed less than 20% from the measured water equivalent diameter-for-age. Typical values of SSDE varied from 28.5 up to 38.9 mGy, while typical values ranged from 30.9 up to 47.6 mGy for the CTDIvol,16cmand from 417.6 up to 861.1 mGy*cm for the DLP. SSDE can be directly calculated for head CT scans once the age of the patient is known.

Resultados visuales y sobrevida de injerto obtenidos en una población pediátrica sometida a queratoplastia / Visual outcomes and graft survival in pediatric patients underwent penetrating keratoplasty

Objetivo: Describir resultados visuales y complicaciones de una serie de casos de pacientes pediátricos a los que se les realizó queratoplastia penetrante en el Hospital de San José, en Bogotá, Colombia. Diseño: Estudio observacional descriptivo, retrospectivo, serie de casos. Método: Se evaluaron 14 ojos de 12 pacientes menores de 16 años a quienes se les realizó queratoplastia penetrante entre los años 1999-2014. Se hizo una revisión de la literatura sobre etiología, manejo quirúrgico, seguimiento y sobrevida de injerto. Resultados: De los 14 ojos estudiados 8 ojos (57,1%) tenían patología congénita con injerto claro en 6 ojos (75%) entre los 3 y 9 meses, 3 ojos (21,4%) tenían etiología traumática, de estos, todos los injertos permanecieron claros en un rango de 3 meses a 11 años y 3 ojos (21,4%) tenían leucomas, queratitis o inminencia de perforación, de estos el injerto permaneció claro entre los 3 y 18 meses. Se encontró una mejoría de visión en 8 de los ojos evaluados (57,1%). Las complicaciones encontradas en estos pacientes fueron: glaucoma en 5 ojos (35.7%), falla de injerto en 6 ojos (42.8%), rechazo endotelial en 7 ojos (50%) y desprendimiento de retina en 1 ojo (7.14%). Conclusión: En el estudio, la mejoría visual fue ligeramente menor a la reportada en la literatura. Las complicaciones presentadas fueron principalmente rechazo endotelial y falla de injerto, lo que difiere de la literatura donde la catarata es la principal complicación.

Purpose: To describe visual results and complications in a case series of paediatric patients who underwent penetrating keratoplasty at Hospital de San José in Bogotá, Colombia. Design: Observational, descriptive and retrospective study, series of cases. Method: 14 eyes of 12 patients under 16 years (2 months - 16 years) who underwent penetrating keratoplasty in Hospital de San Jose from 1999 to 2014 were evaluated. A literature review of etiology, surgical management, monitoring and graft survival of pediatric patients was done. Results: Of the 14 eyes studied, 8 eyes (57.1%) had congenital pathology with clear graft in six eyes between 3 and 9 months, 3 eyes (21.4%) had traumatic aetiology, of these, all graft s remained clear in a range of 3 months to 11 years and 3 eyes (21.4%) had leucoma, keratitis or impending perforation, of these, the graft remained clear between 3 and 18 months. An improvement in vision was found in eight of the evaluated eyes (57.1%). The complications found in these patients were: glaucoma in 5 eyes (35.7%), graft failure in 6 eyes (42.8%), endothelial rejection in 7 eyes (50%) and retinal detachment in 1 eye (7.14%). Conclusion: In our study, the visual improvement was slightly lower than that reported in the literature. The complications presented were mainly endothelial rejection and graft failure, which differs from the literature where cataract is the main complication.

Caracterización clínica del dengue y variables predictoras de gravedad en pacientes pediátricos en un hospital de segundo nivel en Chilpancingo, Guerrero, México: serie de casos / Clinical profile of dengue and predictive severity variables among children at a secondary care hospital of Chilpancingo, Guerrero, Mexico: case series

Resumen: Introducción: El dengue sigue en incremento a nivel mundial y actualmente la mayor incidencia de primera infección ocurre en población pediátrica. El dengue grave es potencialmente letal en menores de edad. Este estudio caracteriza el perfil clínico de pacientes pediátricos con dengue atendidos en un hospital de segundo nivel en Chilpancingo, Guerrero, México. Métodos: Serie de casos conformada por 133 pacientes pediátricos hospitalizados con diagnóstico de dengue no grave y dengue grave, de acuerdo a criterios clínicos. Los resultados del análisis univariado de los signos y síntomas clínicos fueron expresados como promedios o porcentajes, y se consideró nivel de significancia estadística de 0.05. Mediante GLMM (Generalised Linear Mixed Models) se estimó la predicción de dengue grave con la presencia de signos y síntomas clínicos. Resultados: El 58% (77/133) de los pacientes fue clasificado como dengue grave. Hubo diferencias significativas entre los grupos de dengue en los signos y síntomas siguientes: fiebre, dolor abdominal, epistaxis y cuenta plaquetaria. El dengue grave se presentó en mayor proporción en los pacientes mayores de cuatro años de edad (p<0.05). El GLMM identificó un conjunto de cuatro signos y síntomas clínicos (fiebre ≥39 °C, mialgias, artralgias y dolor abdominal) como predictores de la gravedad del dengue. Conclusiones: Los resultados de este estudio exploratorio sugieren cambios en la frecuencia de síntomas y signos clínicos del dengue en la población pediátrica. Pacientes pediátricos con diagnóstico presuntivo de dengue que presenten fiebre ≥39 °C, mialgias, artralgias y dolor abdominal deben considerarse como potenciales casos de dengue grave.

Abstract: Background: Dengue continues to increase globally. Currently, the highest incidence of first infection occurs in paediatric population, where severe dengue fever is potentially lethal. This study characterizes the clinical profile of paediatric patients with dengue fever in the South of Mexico. Methods: We undertook a series case study of 133 paediatric inpatients who presented clinical diagnosis of non-severe dengue and severe dengue fever. We described univariate analysis as means or percentages, using 0.05 as significance level. We estimated the prediction of severe dengue considering clinical signs and symptoms only using GLMM (Generalised Linear Mixed Models). Results: 58% (77/133) patients had severe dengue. There were significant differences among the dengue groups, in the following signs and symptoms: Fever, abdominal pain, epistaxis and platelet count. Children older than four years old had a higher proportion of severe dengue (p<0.05). GLMM identified a group of four clinical signs and symptoms (fever ≥39 °C, myalgia, arthralgia and abdominal pain) as predictors of severe dengue. Conclusions: The results of this exploratory study suggest changes in the frequency of clinical signs and symptoms among paediatric inpatients. Paediatric patients with a presumptive diagnosis of dengue, showing fever of ≥39° C, myalgia, arthralgia and abdominal pain should be considered as potential cases of severe dengue.

[Clinical profile of dengue and predictive severity variables among children at a secondary care hospital of Chilpancingo, Guerrero, Mexico: case series]. / Caracterización clínica del dengue y variables predictoras de gravedad en pacientes pediátricos en un hospital de segundo nivel en Chilpancingo, Guerrero, México: serie de casos.

BACKGROUND: Dengue continues to increase globally. Currently, the highest incidence of first infection occurs in paediatric population, where severe dengue fever is potentially lethal. This study characterizes the clinical profile of paediatric patients with dengue fever in the South of Mexico. METHODS: We undertook a series case study of 133 paediatric inpatients who presented clinical diagnosis of non-severe dengue and severe dengue fever. We described univariate analysis as means or percentages, using 0.05 as significance level. We estimated the prediction of severe dengue considering clinical signs and symptoms only using GLMM (Generalised Linear Mixed Models). RESULTS: 58% (77/133) patients had severe dengue. There were significant differences among the dengue groups, in the following signs and symptoms: Fever, abdominal pain, epistaxis and platelet count. Children older than four years old had a higher proportion of severe dengue (p<0.05). GLMM identified a group of four clinical signs and symptoms (fever ≥39°C, myalgia, arthralgia and abdominal pain) as predictors of severe dengue. CONCLUSIONS: The results of this exploratory study suggest changes in the frequency of clinical signs and symptoms among paediatric inpatients. Paediatric patients with a presumptive diagnosis of dengue, showing fever of ≥39° C, myalgia, arthralgia and abdominal pain should be considered as potential cases of severe dengue.