Iodine status in children with intestinal failure.

OBJECTIVES: Children on long-term parenteral nutrition (PN) are at high risk of iodine deficiency (ID). However, most available information comes from cross-sectional studies. We investigated the iodine status, associated factors, and prevalence of hypothyroidism in children with intestinal failure (IF) who were followed up longitudinally. METHODS: This was a cohort study of children with IF monitored for urine iodine concentration (UIC), iodine intake, serum selenium concentration, and thyroid function in an intestinal rehabilitation program. The outcome variable ID was defined as a UIC value < 100 µg/L. Adjusted generalized estimating equations were used to assess the effects of the exposure variables on the UIC. RESULTS: Twenty-four patients aged 62.7 (39.1; 79.7) months who received PN for 46.5 (21.5) months were included. The average energy supply was 81.2 kcal/kg/day, 77.6% of which was provided by PN. An average of 5.2 UIC measurements per patient were performed. ID prevalence decreased from baseline (83.3%) to the last assessment (45.8%). Three patients had hypothyroidism secondary to iodine and selenium combined severe deficiency. Iodine intake from enteral or oral nutritional formulas was positively associated with UIC (ß = 0.71 [0.35, 1.07]; p < 0.001). Meeting approximately 80% of the estimated average requirement for iodine from nutritional formulas resulted in a greater probability of normal UIC values. CONCLUSION: ID is highly prevalent in children with IF who receive long-term PN and its frequency decreases with iodine intake from nutritional formulas. Severe combined iodine and selenium deficiencies are associated with the development of hypothyroidism in these patients.

Humor in Life Scale (EHV): Development and Evidence of Validity and Reliability in Spanish Speakers / Escala de Humor ante la Vida (EHV): Desarrollo, Evidencias de Validez y Confiabilidad en Hispanohablantes

Abstract Despite the growing attention given to the study of humor, a measure in the Spanish language of people's experience and enjoyment of humor in everyday life is still missing. The present study describes the development and validation of the EHV (from the Spanish Escala de Humor ante la Vida, Humor in Life Scale). In phase I, the items were developed using focus groups and interviews; their content validity was assessed through expert judgment. In phase II, the EHV was answered by two Mexican samples (N=1380), women and men, from 18 to 66 years; it was administered together with the Numeric Rating Scale of Humor and the Positive and Negative Affect Schedule, in both printed and electronic formats. The exploratory factor analysis supported the single factor structure and the confirmatory factor analysis showed adequate fit indices for the final eight-item scale; the factor structure was partially invariant between sexes. Reliability indices were satisfactory. Convergent and discriminant validity tests showed that the EHV is related to a global measure of humor and positive and negative affect. In sum, the results indicate that the EHV is a brief, valid and reliable measure to assess humor in life in Spanish speakers.

Resumen A pesar de la creciente atención que se ha otorgado al estudio del sentido del humor, no se contaba con un instrumento en español que evaluara la experiencia y el disfrute del humor en la vida diaria. La investigación constó de dos fases. En la primera, a partir de grupos focales y entrevistas, se elaboraron 30 reactivos potenciales. Para evaluar su validez de contenido, se obtuvieron índices V de Aiken de los juicios de cuatro expertos. Los 14 reactivos que alcanzaron el criterio fueron sometidos a un piloteo, después del cual la escala quedó conformada por 11 reactivos, con siete opciones de respuesta. En la segunda fase, la EHV se aplicó, junto con la Escala de Evaluación Numérica del Humor (una medida global de humor) y la Escala de Afecto Positivo y Negativo (PANAS), a dos muestras de población general (n1=1380 y n1=550), hombres y mujeres con edades de 18 a 66 años. La aplicación de la batería se efectuó tanto en línea como en formato impreso en diversos lugares públicos. El análisis factorial exploratorio mostró una estructura unidimensional que explicó el 55.97% de la varianza total; dos reactivos fueron eliminados. Se obtuvieron índices de ajuste adecuados al someter a análisis factorial confirmatorio a la EHV, los cuales mejoraron con la eliminación de un reactivo, por lo que la escala quedó finalmente conformada por ocho reactivos. El AVE fue >.50 y los índices de confiabilidad alfa y omega resultaron >.91. Al evaluar la invarianza de la estructura factorial de la EHV por sexo, ésta resultó parcial, ya que se obtuvieron índices dentro de los criterios señalados para el modelo métrico y para el fuerte, pero no todos para el estricto. La correlación entre la EHV y la Escala de Evaluación Numérica del Humor resultó, como se esperaba, alta y positiva, así como con el factor de afecto positivo del PANAS, en tanto que lo opuesto se observó con el factor de afecto negativo. Tener creencias espirituales o religiosas y tener una pareja no mostraron relación con los puntajes de la EHV. Estos resultaron apoyaron la validez convergente y discriminante de la EHV. En conclusión, la EHV es un instrumento breve y unidimensional, con evidencias de validez y confiabilidad, que evalúa la experiencia y disfrute del humor en la vida cotidiana en personas de habla hispana.

Encouraging outcomes of using a small-donor single graft in pediatric kidney transplantation.

BACKGROUND: The use of small pediatric kidneys as single grafts for transplantation is controversial, due to the potential risk for graft thrombosis and insufficient nephron mass. METHODS: Aiming to test the benefits of transplanting these kidneys, 375 children who underwent kidney transplantation in a single center were evaluated: 49 (13.1%) received a single graft from a small pediatric donor (≤ 15 kg, SPD group), 244 (65.1%) from a bigger pediatric donor (> 15 kg, BPD group), and 82 (21.9%) from adult living donors (group ALD). RESULTS: Groups had similar baseline main characteristics. After 5 years of follow-up, children from the SPD group were comparable to children from BPD and ALD in patient survival (94%, 96%, and 98%, respectively, p = 0.423); graft survival (89%, 88%, and 93%, respectively, p = 0.426); the frequency of acute rejection (p = 0.998); the incidence of post-transplant lymphoproliferative disease (p = 0.671); the odds ratio for severely increased proteinuria (p = 0.357); the rates of vascular thrombosis (p = 0.846); and the necessity for post-transplant surgical intervention prior to discharge (p = 0.905). The longitudinal evolution of eGFR was not uniform among groups. The three groups presented a decrease in eGFR, but the slope of the curve was steeper in ALD children. At 5 years, the eGFR of the ALD group was 10 ml/min/1.73m2 inferior to the others. At that time, the eGFR from the SPD group was statistically similar to the BPD group (p = 0.952). CONCLUSION: In a specialized transplant center, the use of a single small pediatric donor kidney for transplantation is as successful as bigger pediatric or adult living donors, after 5 years of follow-up. A higher resolution version of the Graphical abstract is available as Supplementary information.

Access to treatment for chronic kidney disease by children and adolescents in Brazil.

BACKGROUND: This study aimed to identify main factors associated with child and adolescent access to chronic kidney disease (CKD) treatment in Brazil. METHODS: Multi-center cross-sectional study conducted in eight pediatric nephrology centers across all Brazilian geographic regions. Information was collected on characteristics associated with referral and treatment of patients with CKD. The following outcomes were analyzed as follows: (1) age at first consultation, and (2) time elapsed between referral and treatment at the specialized service. RESULTS: Three hundred thirty-five children were assessed. Variables associated with age at first consultation were as follows: CAKUT (HR=1.7; 95%CI 1.3-2.2, p<0.01); private health plan (HR=1.54; 95%CI 1.06-2.23, p=0.02); modified Medical Outcomes Study Social Support Survey mMOS-SS score (HR=1.02; 95%CI 1.00-1.03, p=0.024); maternal age (HR=0.96; 95%CI 0.95-0.97, p<0.01); and number of siblings in the household (HR=0.86; 95%CI 0.79-0.83, p<0.01). Significant variables associated with time elapsed between referral and treatment at the specialized service were as follows: each additional occupant sharing the household (HR=0.94; 95%CI:0.89-0.99, p=0.02), residing in the Northeast (HR=0.81; 95%CI:0.67-0.98, p=0.03) and having someone to take them to the physician (HR=1.36; 95%CI 1.07-1.74, p=0.01). The median time interval between patient referral and treatment by the service was 11 days (IQR 10-31). CONCLUSION: There are potentially modifiable factors hampering access of children with CKD to specialized treatment. The importance of the role of social support for the two outcomes should serve as an alert for health managers and professionals to consider this aspect throughout all steps of the care process of children with CKD.

Copper Deficiency in Children With Intestinal Failure: Risk Factors and Influence on Hematological Cytopenias.

BACKGROUND: We investigated to what extent serum copper influences hemoglobin (Hb), neutrophil, and platelet counts and the factors associated with serum copper in children with intestinal failure (IF) who have their micronutrient status monitored according to a standard protocol. METHODS: Children with IF admitted to a pediatric intestinal rehabilitation program and receiving home parenteral nutrition (PN) were followed up prospectively. Patients received vitamins and multi-trace elements (TEs) as part of the PN. Copper, iron, zinc, vitamin A, and cobalamin serum levels were routinely monitored at 3-month intervals or monthly when a deficiency was detected. Complete blood counts were performed biweekly. Repeated-measures analyses were used to estimate the effect of explanatory variables on the outcomes. RESULTS: Thirteen children with a median time receiving PN of 16.6 months were included. An average of 7 copper measurements per patient were performed; 53.8% of patients had a low serum level at least twice during the follow-up. Eight patients with cholestasis had TEs of PN discontinued. In the multivariable analysis, copper was not associated with Hb levels; an increase of 10 µg/dL in serum copper resulted in an increase of 240/mm3 (95% confidence interval [CI], 18.0-30.1) neutrophils and of 8429/mm3 (95% CI, 466-1219) platelets (P < .001). Time of PN without copper, direct bilirubin levels, and ostomy were associated with lower serum copper. CONCLUSION: The increase in serum copper was associated with significant increases in neutrophil and platelet counts. Time without copper in PN, cholestasis, and having an ostomy negatively influenced copper status.

Is Preoperative Preparation Time a Barrier to Small Children Being Ready for Kidney Transplantation?

BACKGROUND: Small children are less frequently transplanted when compared with older. The objective of the present study was to compare the preparation time for transplantation in children of different weights and to identify factors associated with a delay in the workup of small children. METHODS: We report on a retrospective cohort comprising all children referred for renal transplantation (RTx) workup between 2009 and 2017. The main outcome was transplantation workup time, defined as the time elapsed between the first consultation and when the child became ready for the surgery. RESULTS: A total of 389 children (63.5% males) were selected, with a median weight of 18 kg (interquartile range, 11-32). Patients were categorized into 2 groups: group A (study group): ≤15 kg (n = 165) and group B (control group): >15 kg (n = 224). The probability of being ready for RTx was comparable between groups A and B. The cumulative incidence rate difference between groups is -0.05 (95% confidence interval, -0.03 to 0.02). The median time for RTx workup was 5.4 (2.4-9.4) in group A and 4.3 (2.2-9.0) months in group B (P = 0.451). Moreover, the presence of urinary tract malformation was associated with the need for longer transplantation workup time (P < 0.001). CONCLUSIONS: In children >7 kg, the workup time for transplantation is not related to body weight. In a specialized center, children weighing 7-15 kg became ready within the same timeframe as children weighing >15 kg, despite the smaller children had greater difficulty being nourished, dialyzed, and a greater need for surgical correction of the urinary tract pretransplant.

EMOTIONAL REPERCUSSIONS AND QUALITY OF LIFE IN CHILDREN AND ADOLESCENTS UNDERGOING HEMODIALYSIS OR AFTER KIDNEY TRANSPLANTATION.

OBJECTIVE: To investigate the emotional repercussions and quality of life (QOL) associated with end-stage kidney disease (ESKD) in children and adolescents undergoing hemodialysis or a kidney transplant (TX). METHODS: We conducted a quantitative-qualitative study. 48 children and adolescents with ESKD were interviewed; half of them underwent hemodialysis treatment, and the other half had a kidney transplantation. Their respective 48 caregivers also participated in the study. The questionnaire involved both the Pediatric Quality of Life Inventory and a thematic story-drawing tool. An analysis of the QOL questionnaire's results was done by comparing the sum of points between groups and the theme-based story-drawing consisted of interpreting the data contained in the material using Freudian and Lacanian theories. RESULTS: In the QOL questionnaires, the total score was higher in the transplanted patients and in their caregivers, suggesting a perception of better QOL after kidney transplantation. In the specific aspects of the questionnaire, physical capacity was considered superior by children who underwent transplants and their caregivers. There were no differences between the groups in the emotional, social and school aspects. However, the caregivers of the patients who had a transplant perceived a significant difference in QOL in the school aspect. In the thematic story-drawings, emotional suffering in the two analyzed groups was evidenced regardless of the treatment. CONCLUSIONS: Despite the questionnaire results suggesting that transplantation does improve some aspects of QOL, there were no differences observed between kidney replacement therapies regarding the emotional repercussion of chronic kidney disease.

Emotional repercussions and quality of life in children and adolescents undergoing hemodialysis or after kidney transplantation / Repercussões emocionais e qualidade de vida das crianças e adolescentes em hemodiálise ou após transplante renal

ABSTRACT Objective: To investigate the emotional repercussions and quality of life (QOL) associated with end-stage kidney disease (ESKD) in children and adolescents undergoing hemodialysis or a kidney transplant (TX). Methods: We conducted a quantitative-qualitative study. 48 children and adolescents with ESKD were interviewed; half of them underwent hemodialysis treatment, and the other half had a kidney transplantation. Their respective 48 caregivers also participated in the study. The questionnaire involved both the Pediatric Quality of Life Inventory and a thematic story-drawing tool. An analysis of the QOL questionnaire's results was done by comparing the sum of points between groups and the theme-based story-drawing consisted of interpreting the data contained in the material using Freudian and Lacanian theories. Results: In the QOL questionnaires, the total score was higher in the transplanted patients and in their caregivers, suggesting a perception of better QOL after kidney transplantation. In the specific aspects of the questionnaire, physical capacity was considered superior by children who underwent transplants and their caregivers. There were no differences between the groups in the emotional, social and school aspects. However, the caregivers of the patients who had a transplant perceived a significant difference in QOL in the school aspect. In the thematic story-drawings, emotional suffering in the two analyzed groups was evidenced regardless of the treatment. Conclusions: Despite the questionnaire results suggesting that transplantation does improve some aspects of QOL, there were no differences observed between kidney replacement therapies regarding the emotional repercussion of chronic kidney disease.

RESUMO Objetivo: Investigar as repercussões emocionais e a qualidade de vida (QV) associadas à doença renal crônica em crianças e adolescentes submetidos à hemodiálise ou ao transplante renal. Métodos: Foram entrevistadas 48 crianças e adolescentes com doença renal crônica, metade dos quais submetidos ao tratamento hemodialítico, e a outra metade, ao transplante renal. Os 48 respectivos cuidadores também participaram da pesquisa. Utilizou-se o questionário de QV Pediatric Quality of Life Inventory e o instrumento de desenho-estória com tema. A análise dos resultados do questionário de QV foi feita pela somatória dos pontos e a dos desenhos-estórias com tema consistiu na interpretação dos dados do material, utilizando as teorias freudiana e lacaniana. Resultados: Nos questionários de QV, a pontuação total foi superior na opinião dos pacientes transplantados e seus cuidadores, indicando percepção de melhor QV após o transplante renal. Nos aspectos específicos, a capacidade física também foi considerada superior por esse grupo. Não houve diferenças entre os grupos de pacientes nos aspectos emocional, social e escolar, porém os cuidadores dos pacientes transplantados notaram diferença na QV no aspecto escolar. Nos desenhos-estórias com tema, observou-se que o sofrimento emocional foi evidenciado nos dois grupos estudados. Conclusões: Não foram percebidas diferenças entre as terapêuticas renais substitutivas quanto às repercussões emocionais da doença renal crônica. Por outro lado, verificou-se que o transplante melhora a QV no aspecto geral.

Collaborative Brazilian pediatric renal transplant registry (CoBrazPed-RTx): A report from 2004 to 2018.

The Brazilian collaborative registry for pediatric renal transplantation began in 2004 as a multicenter initiative aimed at analyzing, reporting, and disseminating the results of pediatric renal transplantation in Brazil. Data from all pediatric renal transplants performed from January 2004 to May 2018 at the 13 participating centers were analyzed. A total of 2744 pediatric renal transplants were performed in the thirteen participating centers. The median age at transplantation was 12.2 years, with the majority being male recipients (56%). The main underlying diseases were CAKUT (40.5%) and glomerulopathy (28%). 1981 (72%) of the grafts were from deceased donors (DD). Graft survival at one year (censored by death) was 94% in the live donor group (LD) and 91% in the DD group (log-rank test P < 0.01). The patient's survival at one and 5 years was 97% and 95% for the LD group and 96% and 93% for the DD group (log-rank test P = 0.02). The graft loss rate was 19% (n = 517), more frequently caused by vascular thrombosis (n = 102) and chronic graft nephropathy (n = 90). DD recipients had 1.6 (1.0-2.2) times greater chance of death and 1.5 (1.2-1.8) times greater chance of graft loss compared to LD recipients. The mortality rate was 5.4% (n = 148), mainly due to infection (n = 69) and cardiovascular disease (n = 28). The results of this collaborative pediatric renal transplant record are comparable to other international registries, although we still have a high infection rate as a cause of death.

The transition between a poroma and a porocarcinoma evidenced by the dermoscopy.

Eccrine porocarcinoma (EPC) is a rare malignant skin tumor. The dermoscopy of invasive EPC reveals focal presence of whitish-pink, structureless areas surrounded by pinkish-white halos. In an eccrine poroma (EP), such areas present diffuse distribution in the "frog- eggs" pattern. We reported an EPC in situ that presents a transitional dermoscopy pattern between EP and invasive EPC. We found a diffuse distribution; whitish-pink, structureless areas surrounded by pinkish-white halos; a central exulceration and a polymorphic vascular pattern.

The transition between a poroma and a porocarcinoma evidenced by the dermoscopy

Abstract Eccrine porocarcinoma (EPC) is a rare malignant skin tumor. The dermoscopy of invasive EPC reveals focal presence of whitish-pink, structureless areas surrounded by pinkish-white halos. In an eccrine poroma (EP), such areas present diffuse distribution in the "frog- eggs" pattern. We reported an EPC in situ that presents a transitional dermoscopy pattern between EP and invasive EPC. We found a diffuse distribution; whitish-pink, structureless areas surrounded by pinkish-white halos; a central exulceration and a polymorphic vascular pattern.

Publisher Correction: PTEN regulates glioblastoma oncogenesis through chromatin-associated complexes of DAXX and histone H3.3.

This corrects the article DOI: 10.1038/ncomms15223.

Cost analysis of substitutive renal therapies in children / Análise de custos de terapias renais substitutivas em crianças

Abstract Objective: End-stage renal disease is a health problem that consumes public and private resources. This study aimed to identify the cost of hemodialysis (either daily or conventional hemodialysis) and transplantation in children and adolescents. Methods: This was a retrospective cohort of pediatric patients with End-stage renal disease who underwent hemodialysis followed by kidney transplant. All costs incurred in the treatment were collected and the monthly total cost was calculated per patient and for each renal therapy. Subsequently, a dynamic panel data model was estimated. Results: The study included 30 children who underwent hemodialysis (16 conventional/14 daily hemodialysis) followed by renal transplantation. The mean monthly outlay for hemodialysis was USD 3500 and USD 1900 for transplant. Hemodialysis costs added up to over USD 87,000 in 40 months for conventional dialysis patients and USD 131,000 in 50 months for daily dialysis patients. In turn, transplant costs in 50 months reached USD 48,000 and USD 70,000, for conventional and daily dialysis patients, respectively. For conventional dialysis patients, transplant is less costly when therapy exceeds 16 months, whereas for daily dialysis patients, the threshold is around 13 months. Conclusion: Transplantation is less expensive than dialysis in children, and the estimated thresholds indicate that renal transplant should be the preferred treatment for pediatric patients.

Resumo Objetivo: A Doença Renal em Estágio Final é um problema de saúde que consome recursos públicos e privados. Nosso objetivo é identificar o custo da hemodiálise (hemodiálise diarias ou convencional) e transplante em crianças e adolescentes. Métodos: Uma coorte retrospectiva de pacientes pediátricos com Doença Renal em Estágio Final (DREF) submetidos à hemodiálise após transplante de rim. Todos os custos incorridos no tratamento foram cobrados e o custo total mensal foi calculado por paciente e por cada terapia renal. Então, foi estimado um modelo dinâmico com dados em painel. Resultados: Estudamos 30 crianças submetidas à hemodiálise (16 hemodiálises convencionais/14 hemodiálises diárias) após transplante renal. O gasto médio mensal para hemodiálise foi US$3,5 mil e US$1,9 mil para transplante. Os custos de hemodiálise somam mais de US$87 mil em 40 meses para pacientes submetidos a hemodiálise convencional (HC) e US$131 mil em 50 meses para pacientes submetidos a hemodiálise diária (HD). Por outro lado, os custos de transplante em 50 meses atingem US$48 e US$70 mil, para pacientes submetidos a HC e HD, respectivamente. Para pacientes submetidos à hemodiálise convencional, o transplante é menos oneroso quando a terapia ultrapassa 16 meses, ao passo que para pacientes submetidos a hemodiálise diária o limiar é cerca de 13 meses. Conclusão: O transplante é menos caro que a diálise em crianças e os limiares estimados indicam que o transplante renal deve ser o tratamento preferencial para pacientes pediátricos.

Cost analysis of substitutive renal therapies in children.

OBJECTIVE: End-stage renal disease is a health problem that consumes public and private resources. This study aimed to identify the cost of hemodialysis (either daily or conventional hemodialysis) and transplantation in children and adolescents. METHODS: This was a retrospective cohort of pediatric patients with End-stage renal disease who underwent hemodialysis followed by kidney transplant. All costs incurred in the treatment were collected and the monthly total cost was calculated per patient and for each renal therapy. Subsequently, a dynamic panel data model was estimated. RESULTS: The study included 30 children who underwent hemodialysis (16 conventional/14 daily hemodialysis) followed by renal transplantation. The mean monthly outlay for hemodialysis was USD 3500 and USD 1900 for transplant. Hemodialysis costs added up to over USD 87,000 in 40 months for conventional dialysis patients and USD 131,000 in 50 months for daily dialysis patients. In turn, transplant costs in 50 months reached USD 48,000 and USD 70,000, for conventional and daily dialysis patients, respectively. For conventional dialysis patients, transplant is less costly when therapy exceeds 16 months, whereas for daily dialysis patients, the threshold is around 13 months. CONCLUSION: Transplantation is less expensive than dialysis in children, and the estimated thresholds indicate that renal transplant should be the preferred treatment for pediatric patients.