Factors related to the readiness of Brazilian chronic pediatric patients to transition to care in adult clinics

Abstract Objective Advances in medicine have increased the life expectancy of pediatric patients with chronic illnesses, and challenges with the guided transition of adolescents and young adults from pediatric clinics to adult clinics have grown. The aim of this study was to better understand readiness and factors related to this transition process in Brazil. Method In this cross-sectional study of 308 patients aged from 16 to 21 years under follow-up in pediatric specialties, the degree of readiness for transition was assessed using the Transition Readiness Assessment Questionnaire (TRAQ) and its domains. Associations with demographic data, clinical data, socio-economic level, medication adherence, family functionality, and parental satisfaction with health care were evaluated. Results The median TRAQ score was 3.7 (3.2 - 4.2). Better readiness was associated with female patients, socio-economic class A-B, current active employment, higher level of education, not failing any school year, attending medical appointments alone, functional family, and a good knowledge of disease and medications. A low correlation was observed between TRAQ and age. TRAQ presented good internal consistency (alpha-Cronbach 0.86). In the multiple linear regression, TRAQ score showed a significant association with female gender, advanced age, socio-economic class A-B, better knowledge of disease and medications, and independence to attend appointments alone. Conclusion TRAQ instrument can guide healthcare professionals to identify specific areas of approach, in order to support adolescents with chronic disease to set goals for their own personal development and improve their readiness to enter into the adult healthcare system. In this study, some factors were related to better TRAQ scores.

Factors related to the readiness of Brazilian chronic pediatric patients to transition to care in adult clinics.

OBJECTIVE: Advances in medicine have increased the life expectancy of pediatric patients with chronic illnesses, and challenges with the guided transition of adolescents and young adults from pediatric clinics to adult clinics have grown. The aim of this study was to better understand readiness and factors related to this transition process in Brazil. METHOD: In this cross-sectional study of 308 patients aged from 16 to 21 years under follow-up in pediatric specialties, the degree of readiness for transition was assessed using the Transition Readiness Assessment Questionnaire (TRAQ) and its domains. Associations with demographic data, clinical data, socio-economic level, medication adherence, family functionality, and parental satisfaction with health care were evaluated. RESULTS: The median TRAQ score was 3.7 (3.2 - 4.2). Better readiness was associated with female patients, socio-economic class A-B, current active employment, higher level of education, not failing any school year, attending medical appointments alone, functional family, and a good knowledge of disease and medications. A low correlation was observed between TRAQ and age. TRAQ presented good internal consistency (alpha-Cronbach 0.86). In the multiple linear regression, TRAQ score showed a significant association with female gender, advanced age, socio-economic class A-B, better knowledge of disease and medications, and independence to attend appointments alone. CONCLUSION: TRAQ instrument can guide healthcare professionals to identify specific areas of approach, in order to support adolescents with chronic disease to set goals for their own personal development and improve their readiness to enter into the adult healthcare system. In this study, some factors were related to better TRAQ scores.

Evaluation of pulse wave velocity and central systolic blood pressure in children and adolescents with chronic kidney disease.

OBJECTIVE: Investigate pulse wave velocity and central systolic blood pressure among pediatric population with chronic kidney disease. METHODS: In this cross-sectional study, 57 patients (61.4% male) aged 6.2 to 17.5 years old, 44 with nondialysis chronic kidney disease and 13 on chronic dialysis, were included in the analysis. The pulse wave velocity and the central systolic blood pressure were measured with an oscillometric device with an inbuilt ARC SolverⓇ algorithm and were compared with previously established percentiles. RESULTS: The prevalence of elevated pulse wave velocity was 21.1% (95%Cl: 11.4-33.9) and elevated central systolic blood pressure was 28.1% (95%CI: 17.0-41.5). According to the generalized linear model, there was a higher risk of elevated pulse wave velocity in patients undergoing chronic dialysis treatment than nondialysis chronic kidney disease patients (adjPR=4.24, 95%CI: 1.97-9.13, p=<0.001). Hypertensive patients (stage 2) had a higher risk of elevated pulse wave velocity than normotensive ones (adjPR=2.70, 95%CI: 1.05-6.95, p=0.040), as did patients younger than 12 years than the older patients (adjPR=2.95, 95%CI: 1.05-8.40, p=0.041). Hypertensive patients had a higher risk of elevated central systolic blood pressure than normotensives (adjPR=3.29, 95%Cl: 1.36-7.94), as did patients undergoing chronic dialysis treatment when comparing to nondialysis chronic kidney disease patients (adjPR=2.08, 95%Cl: 1.07-4.02). CONCLUSION: Younger age, dialysis, and hypertension in children are independently associated with higher pulse wave velocity. Hypertension and dialysis are independently associated with higher central systolic blood pressure.

Evaluation of pulse wave velocity and central systolic blood pressure in children and adolescents with chronic kidney disease

ABSTRACT Objective Investigate pulse wave velocity and central systolic blood pressure among pediatric population with chronic kidney disease. Methods In this cross-sectional study, 57 patients (61.4% male) aged 6.2 to 17.5 years old, 44 with nondialysis chronic kidney disease and 13 on chronic dialysis, were included in the analysis. The pulse wave velocity and the central systolic blood pressure were measured with an oscillometric device with an inbuilt ARC SolverⓇ algorithm and were compared with previously established percentiles. Results The prevalence of elevated pulse wave velocity was 21.1% (95%Cl: 11.4-33.9) and elevated central systolic blood pressure was 28.1% (95%CI: 17.0-41.5). According to the generalized linear model, there was a higher risk of elevated pulse wave velocity in patients undergoing chronic dialysis treatment than nondialysis chronic kidney disease patients (adjPR=4.24, 95%CI: 1.97-9.13, p=<0.001). Hypertensive patients (stage 2) had a higher risk of elevated pulse wave velocity than normotensive ones (adjPR=2.70, 95%CI: 1.05-6.95, p=0.040), as did patients younger than 12 years than the older patients (adjPR=2.95, 95%CI: 1.05-8.40, p=0.041). Hypertensive patients had a higher risk of elevated central systolic blood pressure than normotensives (adjPR=3.29, 95%Cl: 1.36-7.94), as did patients undergoing chronic dialysis treatment when comparing to nondialysis chronic kidney disease patients (adjPR=2.08, 95%Cl: 1.07-4.02). Conclusion Younger age, dialysis, and hypertension in children are independently associated with higher pulse wave velocity. Hypertension and dialysis are independently associated with higher central systolic blood pressure.

Evaluation of carotid intima-media thickness and factors associated with cardiovascular disease in children and adolescents with chronic kidney disease / Avaliação da espessura médio-intimal da carótida e fatores associados à doença cardiovascular em crianças e adolescentes com doença renal crônica

ABSTRACT Objective: To assess the carotid intima-media thickness and factors associated with cardiovascular disease in children and adolescents with chronic kidney disease. Material and methods: Observational, cross-sectional study carried out at the Universidade Federal de São Paulo (chronic kidney disease outpatient clinics) with 55 patients (60% males) with a median age of 11.9 years (I25-I75: 9.2-14.8 years). Of the 55 patients, 43 were on conservative treatment and 12 were on dialysis. Serum laboratory parameters (creatinine, uric acid, C-reactive protein, total cholesterol and fractions, and triglycerides), nutritional status (z-score of body mass index, z-score of height/age), body fat (fat percentage and waist circumference), and blood pressure levels were evaluated. The carotid intima-media thickness measure was evaluated by a single ultrasonographer and compared with percentiles established according to gender and height. Data collection was performed between May 2015 and March 2016. Results: Of the children and adolescents with chronic kidney disease, 74.5% (95% CI: 61.0; 85.3) showed an increase (>P95) in carotid intima-media thickness. In patients with stages I and II hypertension, 90.9% had increased carotid intima-media thickness. Nutritional status, body fat and laboratory tests were not associated with increased carotid intima-media thickness. After multivariate adjustment, only puberty (PR = 1.30, p = 0.037) and stages I and II arterial hypertension (PR = 1.42, p = 0.011) were independently associated with carotid intima-media thickness alterations. Conclusion: The prevalence of increased carotid thickness was high in children and adolescents with chronic kidney disease. Puberty and arterial hypertension were independently associated with increased carotid intima-media thickness.

RESUMO Objetivo: Avaliar a espessura médio-intimal da carótida e os fatores associados à doença cardiovascular em crianças e adolescentes com doença renal crônica. Material e métodos: Estudo observacional transversal feito na Universidade Federal de São Paulo (ambulatórios de doença renal crônica) com 55 pacientes (60% do sexo masculino) com mediana de 11,9 anos (I25-I75: 9,2-14,8). Dos 55 pacientes, 43 estavam em tratamento conservador e 12 em terapia dialítica. Foram avaliados os parâmetros laboratoriais séricos (creatinina, ácido úrico, proteína C-reativa, colesterol total e frações e triglicérides), estado nutricional (escore z de índice de massa corpórea, escore z de estatura/idade), gordura corporal (percentual de gordura e circunferência abdominal) e pressão arterial. A medida da espessura médio-intimal da carótida foi avaliada por um único ultrassonografista e comparada com percentis estabelecidos de acordo com o sexo e a estatura. A coleta de dados foi feita entre maio de 2015 e março de 2016. Resultados: Das crianças e adolescentes com doença renal crônica, 74,5% (IC 95%: 61,0; 85,3) apresentaram aumento (> P95) da espessura médio-intimal da carótida. Nos pacientes com hipertensão arterial estágios I e II, 90,9% apresentaram aumento da espessura médio-intimal da carótida. O estado nutricional, a gordura corporal e os exames laboratoriais não apresentaram associação com o aumento da espessura médio-intimal da carótida. Após ajuste multivariado, apenas a puberdade (RP = 1,30; p = 0,037) e a hipertensão arterial estágios I e II (RP = 1,42; p = 0,011) mostraram-se independentemente associados à alteração da espessura médio-intimal da carótida. Conclusão: A prevalência do aumento da espessura da carótida foi elevada em crianças e adolescentes com doença renal crônica. A puberdade e a hipertensão arterial mostraram-se independentemente associadas ao aumento da espessura médio-intimal da carótida.

Evaluation of carotid intima-media thickness and factors associated with cardiovascular disease in children and adolescents with chronic kidney disease.

OBJECTIVE: To assess the carotid intima-media thickness and factors associated with cardiovascular disease in children and adolescents with chronic kidney disease. MATERIAL AND METHODS: Observational, cross-sectional study carried out at the Universidade Federal de São Paulo (chronic kidney disease outpatient clinics) with 55 patients (60% males) with a median age of 11.9 years (I25-I75: 9.2-14.8 years). Of the 55 patients, 43 were on conservative treatment and 12 were on dialysis. Serum laboratory parameters (creatinine, uric acid, C-reactive protein, total cholesterol and fractions, and triglycerides), nutritional status (z-score of body mass index, z-score of height/age), body fat (fat percentage and waist circumference), and blood pressure levels were evaluated. The carotid intima-media thickness measure was evaluated by a single ultrasonographer and compared with percentiles established according to gender and height. Data collection was performed between May 2015 and March 2016. RESULTS: Of the children and adolescents with chronic kidney disease, 74.5% (95% CI: 61.0; 85.3) showed an increase (>P95) in carotid intima-media thickness. In patients with stages I and II hypertension, 90.9% had increased carotid intima-media thickness. Nutritional status, body fat and laboratory tests were not associated with increased carotid intima-media thickness. After multivariate adjustment, only puberty (PR=1.30, p=0.037) and stages I and II arterial hypertension (PR=1.42, p=0.011) were independently associated with carotid intima-media thickness alterations. CONCLUSION: The prevalence of increased carotid thickness was high in children and adolescents with chronic kidney disease. Puberty and arterial hypertension were independently associated with increased carotid intima-media thickness.

Raquitismo hipofosfatêmico: relato de caso / Hypophosphatemic rickets: case report

RESUMO Objetivo: O raquitismo hipofosfatêmico precisa ser precocemente diagnosticado porque seu tratamento previne sequelas incapacitantes. Este relato alerta para a doença. Relato de caso: Relato de perfil metabólico, depuração de creatinina, estado nutricional e desenvolvimento pôndero-estatural de paciente com características clínico-laboratoriais de raquitismo hipofosfatêmico, atendido em ambulatório de tubulopatias por período de 12 meses. Chegou ao serviço após tempo prolongado acamado, dependente de ventilação mecânica e com perfil metabólico ósseo alterado. Terapêutica consistiu na administração de fósforo (inicial: 65 mg/kg/dia, final: 24,2 mg/kg/dia), cálcio (inicial: 127 mg/kg/dia, final: 48,4 mg/kg/dia) e calcitriol (inicial: 0,06 mcg/kg/dia, final: 0,03 mcg/kg/dia), e a análise constou da descrição das consultas, utilizando-se mediana de exames laboratoriais e dados antropométricos. Observou-se nítida melhora inicial do padrão respiratório do paciente, que evoluiu com ventilação espontânea e deambulação autônoma; com exames laboratoriais: cálcio (mg/dL) inicial 7,1, final 10,1; fósforo (mg/dL) inicial 1,7, final 3,2; magnésio (mg/dL) inicial 1,5, final 2,1; paratormônio (pg/L) inicial 85,8, final 52,7; fosfatase alcalina (UI/L) inicial 12660, final 938; e melhora do desenvolvimento pôndero-estatural (escore Z: E/I inicial: -6,05, final -3,64; P/I: inicial -2,92, final -1,57) com presença de litíase transitória. A depuração de creatinina (mL/min/1,73 m2sc) foi constante durante o seguimento. O tratamento propiciou benefícios clínicos, bioquímicos e nutricionais, mas, apesar da boa resposta inicial, a família abandonou o seguimento por dois anos, apresentando o paciente piora da deambulação e das deformidades esqueléticas. Comentários: Não apenas diagnóstico precoce é necessário, como também a adesão ao tratamento é fundamental para o sucesso na condução dessa patologia.

ABSTRACT Objective: Early diagnosis and immediate treatment of hypophosphatemic rickets is of utmost importance as it may prevent subsequent sequelae. This report aims at warning pediatricians to consider the presence of the disease. Case description: Description of the metabolic profile, creatinine clearance, nutritional status, weight and body structure of a patient who presented the clinical-laboratorial characteristics of hypophosphatemic rickets and was followed in an outpatient clinic for tubulopathies over the period of 12 months. The patient had been bedridden for some time, was dependent on mechanical ventilation and presented an altered metabolic bone condition. Treatment was phosphate (initial: 65 mg/kg/day and final: 24,2 mg/kg/day), calcium (initial: 127 mg/kg/day, final: 48,4 mg/kg/day) and calcitriol (initial: 0.06 mcg/kg/day, final: 0.03 mcg/kg/day). The patient improved, evolving into spontaneous breathing and walking unaided. Laboratory results: calcium (mg/dL) initial 7.1, final 10.1; phosphate (mg/dL) initial 1.7 final 3.2; magnesium (mg/dL) initial 1.5 final 2.1, parathyroid hormone (pg/l) initial 85.8, final 52.7, alkaline phosphatase (UI/l) initial 12660, final 938; there was also improvement in weight/structural development (Z score: H/A initial: -6.05, final -3.64; W/A: initial -2.92, final -1.57) with presence of transitory gallstones. Creatinine clearance (mL/min/1.73m2bsa) was constant. The medication improved his laboratory results and nutritional status, but the patient did not return for two years for follow-up and, during this period, his condition has noticeably deteriorated. Comments: Early diagnosis and follow-up are essential in dealing with this pathology.

HYPOPHOSPHATEMIC RICKETS: CASE REPORT. / RAQUITISMO HIPOFOSFATÊMICO: RELATO DE CASO.

OBJECTIVE: Early diagnosis and immediate treatment of hypophosphatemic rickets is of utmost importance as it may prevent subsequent sequelae. This report aims at warning pediatricians to consider the presence of the disease. CASE DESCRIPTION: Description of the metabolic profile, creatinine clearance, nutritional status, weight and body structure of a patient who presented the clinical-laboratorial characteristics of hypophosphatemic rickets and was followed in an outpatient clinic for tubulopathies over the period of 12 months. The patient had been bedridden for some time, was dependent on mechanical ventilation and presented an altered metabolic bone condition. Treatment was phosphate (initial: 65 mg/kg/day and final: 24,2 mg/kg/day), calcium (initial: 127 mg/kg/day, final: 48,4 mg/kg/day) and calcitriol (initial: 0.06 mcg/kg/day, final: 0.03 mcg/kg/day). The patient improved, evolving into spontaneous breathing and walking unaided. Laboratory results: calcium (mg/dL) initial 7.1, final 10.1; phosphate (mg/dL) initial 1.7 final 3.2; magnesium (mg/dL) initial 1.5 final 2.1, parathyroid hormone (pg/l) initial 85.8, final 52.7, alkaline phosphatase (UI/l) initial 12660, final 938; there was also improvement in weight/structural development (Z score: H/A initial: -6.05, final -3.64; W/A: initial -2.92, final -1.57) with presence of transitory gallstones. Creatinine clearance (mL/min/1.73m2bsa) was constant. The medication improved his laboratory results and nutritional status, but the patient did not return for two years for follow-up and, during this period, his condition has noticeably deteriorated. COMMENTS: Early diagnosis and follow-up are essential in dealing with this pathology.

OBJETIVO: O raquitismo hipofosfatêmico precisa ser precocemente diagnosticado porque seu tratamento previne sequelas incapacitantes. Este relato alerta para a doença. RELATO DE CASO: Relato de perfil metabólico, depuração de creatinina, estado nutricional e desenvolvimento pôndero-estatural de paciente com características clínico-laboratoriais de raquitismo hipofosfatêmico, atendido em ambulatório de tubulopatias por período de 12 meses. Chegou ao serviço após tempo prolongado acamado, dependente de ventilação mecânica e com perfil metabólico ósseo alterado. Terapêutica consistiu na administração de fósforo (inicial: 65 mg/kg/dia, final: 24,2 mg/kg/dia), cálcio (inicial: 127 mg/kg/dia, final: 48,4 mg/kg/dia) e calcitriol (inicial: 0,06 mcg/kg/dia, final: 0,03 mcg/kg/dia), e a análise constou da descrição das consultas, utilizando-se mediana de exames laboratoriais e dados antropométricos. Observou-se nítida melhora inicial do padrão respiratório do paciente, que evoluiu com ventilação espontânea e deambulação autônoma; com exames laboratoriais: cálcio (mg/dL) inicial 7,1, final 10,1; fósforo (mg/dL) inicial 1,7, final 3,2; magnésio (mg/dL) inicial 1,5, final 2,1; paratormônio (pg/L) inicial 85,8, final 52,7; fosfatase alcalina (UI/L) inicial 12660, final 938; e melhora do desenvolvimento pôndero-estatural (escore Z: E/I inicial: -6,05, final -3,64; P/I: inicial -2,92, final -1,57) com presença de litíase transitória. A depuração de creatinina (mL/min/1,73 m2sc) foi constante durante o seguimento. O tratamento propiciou benefícios clínicos, bioquímicos e nutricionais, mas, apesar da boa resposta inicial, a família abandonou o seguimento por dois anos, apresentando o paciente piora da deambulação e das deformidades esqueléticas. COMENTÁRIOS: Não apenas diagnóstico precoce é necessário, como também a adesão ao tratamento é fundamental para o sucesso na condução dessa patologia.