Growth after pediatric kidney transplantation: a 25-year study in a pediatric kidney transplant center.

OBJECTIVES: Growth failure is one of the major complications of pediatric chronic kidney disease. Even after a kidney transplant (KT), up to 50 % of patients fail to achieve the expected final height. This study aimed to assess longitudinal growth after KT and identify factors influencing it. METHODS: A retrospective observational study was performed. We reviewed the clinical records of all patients who underwent KT for 25 years in a single center (n=149) and performed telephone interviews. Height-for-age and body mass index (BMI)-for-age were examined at KT, 3 months, 6 months, 1 year, and 5 years post-transplant and at the transition to adult care. We evaluated target height, disease duration before KT, need and type of dialysis, recombinant human growth hormone pretransplant use, nutritional support, glomerular filtration rate (GFR), and cumulative corticosteroid dose. RESULTS: At transplant, the average height z-score was -1.38, and height z-scores showed catch-up growth at 6 months (z-score -1.26, p=0.006), 1 year (z-score -1.15, p<0.001), 5 years after KT (z-score -1.08, p<0.001), and on transition to adult care (z-score -1.22, p=0.012). Regarding BMI z-scores, a significant increase was also detected at all time points (p<0.001). After KT, GFR was significantly associated with height z-score (p=0.006) and BMI z-score (p=0.006). The height in transition to adult care was -1.28 SD compared to the target height. CONCLUSIONS: Despite the encouraging results regarding catch-up growth after KT in this cohort, results remain far from optimum, with a lower-than-expected height at the time of transition.

Vaccination practices in pediatric transplantation: A survey among member centers of the European reference network TransplantChild.

BACKGROUND: There is considerable variation in vaccination practices between pediatric transplant centers. This study aims to evaluate active immunization attitudes and practices among ERN-TransplantChild centers and identify potential areas of improvement that could be addressed by shared evidence-based protocols. METHODS: A cross-sectional questionnaire of attitudes and practices toward immunization of pediatric SOT and HSCT candidates and recipients was sent to a representative member of multidisciplinary teams from 27 European centers belonging to the ERN-TransplantChild. RESULTS: A total of 28/62 SOT programs and 6/12 HSCT programs across 21 European centers participated. A quarter of centers did not have an on-site protocol for the immunizations. At the time of transplantation, pediatric candidates were fully immunized (80%-100%) in 57% and 33% of the SOT and HSCT programs. Variations in the time between vaccine administration and admission to the waiting list were reported between the centers, with 2 weeks for inactivated vaccines and variable time (2-4 weeks) for live-attenuated vaccines (LAVs). Almost all sites recommended immunization in the post-transplant period, with a time window of 4-8 months for the inactivated vaccines and 16-24 months for MMR and Varicella vaccines. Only five sites administer LAVs after transplantation, with seroconversion evaluated in 80% of cases. CONCLUSIONS: The immunization coverage of European pediatric transplant recipients is still inconsistent and far from adequate. This survey is a starting point for developing shared evidence-based immunization protocols for safe vaccination among pediatric transplant centers and generating new research studies.

Hemolytic-uremic syndrome: 24 years' experience of a pediatric nephrology unit / Síndrome hemolítico-urêmica: 24 anos de experiência de uma unidade de nefrologia pediátrica

Abstract Introduction: A better understanding of hemolytic-uremic syndrome (HUS) pathophysiology significantly changed its treatment and prognosis. The aim of this study is to characterize the clinical features, severity, management, and outcomes of HUS patients. Materials and Methods: Retrospective study of HUS patients admitted to a Pediatric Nephrology Unit between 1996 and 2020. Demographic and clinical data regarding etiology, severity, treatment strategies, and patient outcome were collected. Results: Twenty-nine patients with HUS were admitted to our unit, but four were excluded. Median age at diagnosis was two years (2 months - 17 years). Clinical manifestations included diarrhea, vomiting, oliguria, hypertension, and fever. During the acute phase, 14 patients (56%) required renal replacement therapy. Infectious etiology was identified in seven patients (five Escherichia coli and two Streptococcus pneumoniae). Since 2015, 2/7 patients were diagnosed with complement pathway dysregulation HUS and there were no cases of infectious etiology detected. Six of these patients received eculizumab. The global median follow-up was 6.5 years [3 months-19.8 years]. One patient died, seven had chronic kidney disease, four of whom underwent kidney transplantation, one relapsed, and seven had no sequelae. Conclusion: These results reflect the lack of infectious outbreaks in Portugal and the improvement on etiological identification since genetic testing was introduced. The majority of patients developed sequels and mortality was similar to that of other countries. HUS patients should be managed in centers with intensive care and pediatric nephrology with capacity for diagnosis, etiological investigation, and adequate treatment. Long-term follow-up is essential.

Resumo Introdução: Um melhor entendimento da fisiopatologia da síndrome hemolítico-urêmica (SHU) mudou significativamente seu tratamento e prognóstico. Este estudo teve como objetivo caracterizar condições clínicas, gravidade, manejo e desfechos de pacientes com SHU. Materiais e Métodos: Estudo retrospectivo de pacientes com SHU admitidos numa Unidade de Nefrologia Pediátrica entre 1996-2020. Foram coletados dados demográficos e clínicos sobre etiologia, gravidade, estratégias de tratamento, desfechos de pacientes. Resultados: 29 pacientes com SHU foram admitidos em nossa unidade, mas quatro foram excluídos. A idade mediana ao diagnóstico foi dois anos (2 meses-17 anos). Manifestações clínicas incluíram diarreia, vômitos, oligúria, hipertensão e febre. Durante a fase aguda, 14 pacientes (56%) necessitaram de terapia renal substitutiva. Identificou-se a etiologia infecciosa em sete pacientes (cinco Escherichia coli; dois Streptococcus pneumoniae). Desde 2015, 2/7 pacientes foram diagnosticados com SHU por desregulação da via do complemento e não foram detectados casos de etiologia infecciosa. Seis desses pacientes receberam eculizumab. A mediana global de acompanhamento foi 6,5 anos [3 meses-19,8 anos]. Um paciente faleceu, sete apresentaram doença renal crônica, sendo quatro submetidos a transplante renal, uma recidiva e sete sem sequelas. Conclusão: Estes resultados refletem a ausência de surtos infecciosos em Portugal e a melhoria na identificação etiológica desde que os testes genéticos foram introduzidos. A maioria dos pacientes desenvolveu sequelas e a mortalidade foi semelhante à de outros países. Pacientes com SHU devem ser manejados em centros com cuidados intensivos e nefrologia pediátrica com capacidade para diagnóstico, investigação etiológica e tratamento adequado. O acompanhamento alongo prazo é essencial.

Hemolytic-uremic syndrome: 24 years' experience of a pediatric nephrology unit.

INTRODUCTION: A better understanding of hemolytic-uremic syndrome (HUS) pathophysiology significantly changed its treatment and prognosis. The aim of this study is to characterize the clinical features, severity, management, and outcomes of HUS patients. MATERIALS AND METHODS: Retrospective study of HUS patients admitted to a Pediatric Nephrology Unit between 1996 and 2020. Demographic and clinical data regarding etiology, severity, treatment strategies, and patient outcome were collected. RESULTS: Twenty-nine patients with HUS were admitted to our unit, but four were excluded. Median age at diagnosis was two years (2 months - 17 years). Clinical manifestations included diarrhea, vomiting, oliguria, hypertension, and fever. During the acute phase, 14 patients (56%) required renal replacement therapy. Infectious etiology was identified in seven patients (five Escherichia coli and two Streptococcus pneumoniae). Since 2015, 2/7 patients were diagnosed with complement pathway dysregulation HUS and there were no cases of infectious etiology detected. Six of these patients received eculizumab. The global median follow-up was 6.5 years [3 months-19.8 years]. One patient died, seven had chronic kidney disease, four of whom underwent kidney transplantation, one relapsed, and seven had no sequelae. CONCLUSION: These results reflect the lack of infectious outbreaks in Portugal and the improvement on etiological identification since genetic testing was introduced. The majority of patients developed sequels and mortality was similar to that of other countries. HUS patients should be managed in centers with intensive care and pediatric nephrology with capacity for diagnosis, etiological investigation, and adequate treatment. Long-term follow-up is essential.

Growth Patterns After Kidney Transplantation in European Children Over the Past 25 Years: An ESPN/ERA-EDTA Registry Study.

BACKGROUND: Improved management of growth impairment might have resulted in less growth retardation after pediatric kidney transplantation (KT) over time. We aimed to analyze recent longitudinal growth data after KT in comparison to previous eras, its determinants, and the association with transplant outcome in a large cohort of transplanted children using data from the European Society for Paediatric Nephrology/European Renal Association and European Dialysis and Transplant Association Registry. METHODS: A total of 3492 patients transplanted before 18 years from 1990 to 2012 were included. Height SD scores (SDS) were calculated using recent national or European growth charts. We used generalized equation models to estimate the prevalence of growth deficit and linear mixed models to calculate adjusted mean height SDS. RESULTS: Mean adjusted height post-KT was -1.77 SDS. Height SDS was within normal range in 55%, whereas 28% showed moderate, and 17% severe growth deficit. Girls were significantly shorter than boys, but catch-up growth by 5 years post-KT was observed in both boys and girls. Children <6 years were shortest at KT and showed the greatest increase in height, whereas there was no catch-up growth in children transplanted >12. CONCLUSIONS: Catch-up growth post-KT remains limited, height SDS did not improve over time, resulting in short stature in nearly half of transplanted children in Europe.

Vitamin D-dependent rickets: a resurgence of the rachitic lung in the 21st century.

Respiratory complications of rickets may be life-threatening particularly in developing countries. A 7-month-old boy presented with recurrent infections, seizures, failure to thrive, wheezing and respiratory distress progressing to global respiratory failure. Several antimicrobial regimens, bronchodilators and corticosteroids resulted in only short-term improvement. He was transferred from Cape Verde to a third-care hospital in Portugal. He was hypotonic and undernourished, with respiratory anguish and classical skeletal signs of rickets, despite vitamin D supplementation. Hypocalcaemia, normal phosphate levels and normal vitamin D status 25(OH)D3 and 1.25(OH)2D3) pointed to vitamin D-dependent rickets type II. Treatment with high doses of calcium and calcitriol allowed progressive respiratory, musculoskeletal and neurological recovery. Although respiratory manifestations of rickets were described many years ago, the present case raises relevant issues about the level of diagnostic support, the risk of complications and how they should be assessed and monitored.

[Complications of pediatric renal transplantation]. / Complicações da transplantação renal em idade pediátrica.

BACKGROUND: Renal transplantation is the treatment of choice for children with end stage renal disease with positive impact on survival and quality of life. However, some complications affect morbidity and mortality. This study examines the renal transplantation complications profile in pediatric patients (<18 y). MATERIAL AND METHODS: Retrospective analysis of clinical files from renal transplantation patients followed in Pediatric Nephrology Unit from September 1995 to August 2010. Collection of data regarding: demography, end stage renal disease etiology, previous renal replacement therapy, graft loss and death, complications: surgical, infectious and non-infectious (acute and chronic rejection, primary disease recurrence, metabolic and cardiovascular risk factors). Descriptive statistical analysis was performed. RESULTS: 78 children (male: 48.7%), mean age at renal transplantation: 11.7 4.1 years. Previous peritoneal dialysis in 49 (62.6%). Five patients (6.4%) with preemptive renal transplantation. Median follow up: 37.5 months (1-169). Main end stage renal disease etiologies were urologic conditions in 41% and glomerular disease in 28.2%. Infectious complications occurred in 74%: viral: 56.4% (cytomegalovirus in 39.7%); bacterial in 53.8% (mainly urinary tract infections). Non-infectious complications were: 1) cardiovascular risk factors: hypertension in 85.9%, hyperlipidemia in 16.7% and new onset diabetes post transplantation in 7.7%; 2) acute graft dysfunction in 32.1%; graft chronic nephropathy 17.9%; 3) surgical complications 16.7%. In the first month after renal transplantation, surgical complications (11.5%) and bacterial infections were the most prevalent complications. Between the 1st and the 6th month there were more bacterial (34.6% patients) and viral (17.9% patients) infections. From 6th month on, cardiovascular risk factors (89.7% patients) became the more prevalent. There was one death. CONCLUSIONS: The most frequent infections were viral, mainly CMV. Acute graft dysfunction was frequent after the 6th month, probably associated with poor compliance. New morbidities, namely cardiovascular risk factors, are emerging with the evolution of new diagnostic, prophylactic and therapeutic strategies for renal transplantation.

Introdução: A transplantação renal é a terapêutica de eleição na criança com doença renal crónica terminal, evidenciando impacto positivo na sobrevida e qualidade de vida dos doentes. Não é, no entanto, isenta de complicações, algumas com importante morbilidade. Os autores pretendem caracterizar o perfil de complicações pós transplantação renal em doentes pediátricos (até 18 anos).Material e Métodos: Análise retrospectiva dos doentes submetidos a transplantação renal e seguidos na Unidade de Nefrologia Pediátrica entre Setembro de 1995 e Agosto de 2010. Dados obtidos dos processos clínicos: características demográficas, etiologia da doença renal crónica terminal, terapêutica de substituição renal, mortalidade e perda de enxertos, complicações cirúrgicas, infecciosas e não infecciosas (rejeição aguda e crónica, recidiva da doença de base, alterações metabólicas e factores de risco cardiovascular). Análise estatística descritiva simples.Resultados: Foram incluídas 78 crianças transplantadas (48,7% sexo masculino), com idade mediana à data da transplantaçãorenal de 12 anos (2 - 18). A maioria fez previamente diálise peritoneal: 49 (62,6%). Cinco doentes (6,4%) foram transplantados sem diálise prévia. A mediana do tempo de seguimento após transplante foi 37,5 meses (1 - 169). As principais etiologias de doença renal crónica terminal foram: uronefropatias (41%) e glomerulopatias (28,2%). As complicações infecciosas ocorreram em 74,4%; infecçõesvirais em 56,4%, sendo a mais prevalente a infecção citomegalovírus (39,7%); infecções bacterianas em 53,8% (na maioria infecções urinárias em doentes urológicos). Outras complicações: 1) factores de risco para doença cardiovascular: hipertensão arterial em 85,9%; dislipidémia em 16,7% e diabetes de novo em 7,7%; 2) episódios de rejeição aguda em 32,1% e nefropatia crónica do enxerto em 17,9%; 3) complicações relacionáveis com a cirurgia em 16,7%. No primeiro mês após transplantação renal as complicações maisfrequentes foram as infecções bacterianas que ocorreram em 15,4% dos doentes e as complicações associadas à cirurgia que ocorreram em 11,5%; entre 1º e 6º mês prevaleceram as infecções bacterianas (em 34,6%) e virais (em 17,9%) e a partir do sexto mês os factores de risco cardiovascular (hipertensão arterial em 85,9% e dislipidémia em 16,7%). Registou-se um óbito.Conclusões: As infecções mais frequentes foram as virais, nomeadamente a infecção citomegalovírus. A disfunção aguda do enxerto permanece uma complicação frequente. Com a evolução de novas estratégias diagnósticas, profilácticas e terapêuticas, assistiu-se a emergência de novas morbilidades, nomeadamente os factores de risco cardiovascular.

Severe immune haemolytic anaemia due to ceftriaxone in a patient with congenital nephrotic syndrome.

AIM: To describe the first case of ceftriaxone-related haemolysis in a patient with congenital nephrotic syndrome (CNS). BACKGROUND: Severe haemolysis caused by an immune reaction to ceftriaxone has mostly been described in patients with underlying haematological or immune dysfunction. CASE REPORT: The authors present a 20-month-old boy with CNS of the Finnish type with several previous severe infections treated with ceftriaxone, admitted for suspected sepsis. Following ceftriaxone administration he developed shock secondary to an acute haemolytic reaction, with severe anaemia. Hypersensitivity to ceftriaxone was documented through positive agglutination tests. CONCLUSION: Onset of haemolysis following ceftriaxone administration, particularly in a patient previously exposed to the drug, must raise the suspicion of a possible immune reaction.

[Breastfeeding: prevalence and determinant factors]. / Aleitamento materno: prevalência e factores condicionantes.

Breastfeeding is the best way of feeding the baby for the first six months of life. However, in Portugal the abandonment rate of breastfeeding is very high during the baby first's months of life. The aim of this study was to assess prevalence of breastfeeding and to identify related factors during the six months after delivery, as socio demographic variables and life styles. We conducted a cohort study at the Maternity of the Hospital Santa Maria. A standard questionnaire was applied to 475 women after delivery, at three and six months postpartum. We studied socio demographics aspects, life styles and the way of feeding during the six months after delivery. Multivariate analysis was performed. The women studied (mean age of 29.8 +/- 5,4 years), 52.2% were primiparous, 86.1% were Caucasian, 40% had a high school degree and 33% had a University degree. Four hundred and sixty (96.8%) received prenatal care. The mean gestational age was 38.8 +/- 2 weeks and the birth weight was 3198.3 +/- 545.3 g. At the discharge 91% were breastfeeding (77% exclusively), 54.7% at third month and 34.1% at sixth month. The main causes pointed for abandoning breastfeeding were insufficient milk production, bad sucking and return to work. The milk formula introduction was in 68.6% cases by medical recommendation. The decision in maintenance breastfeeding at third and sixth months was correlated with a previous positive breastfeed experience, high educational level, healthy lifestyles, as non-smoking, regular physical activity, and information about advantage of breastfeed for mother health. Information about breastfeeding was received by media, friends, family and only 9% by health professionals. Fifty (13%) women had no information about breastfeeding. Although breastfeeding rate at discharge was high, there was an important rate of abandonment at third and sixth month. Healthy lifestyles, high educational level, a previous positive breastfeed experience had a positive influence in breastfeeding. Understanding attitudes towards pregnancy and breastfeeding can lead to new strategies for its promotion and maintenance.

[Pregnancy and physical exercise: myths, evidence and recommendations]. / Gravidez e exercício físico: mitos, evidências e recomendações.

In recent years there has been a great increase of scientific research regarding physical exercise during pregnancy. Nevertheless, many doubts persist leading to a decrease of its practice. We aimed to evaluate the main factors that influence the practice of physical activity during pregnancy and mothers' knowledge regarding its effects. Physical activity has decreased significantly, although it was higher in nulliparous mothers, with higher educational level, employment and between 25 and 34 years old. Mothers who practiced exercise during pregnancy breastfed longer. No correlation was found concerning birth weight, gestational age or obstetric pathology. Although most mothers understand the benefits of physical activity in pregnancy, that doesn't seem to translate in an increasing practice. We believe there should be an increased awareness of health professionals in order to promote controlled physical exercise in pregnant woman.

[Pregnancy and smoking: an opportunity to change behaviours]. / Gravidez e tabagismo: uma oportunidade para mudar comportamentos.

INTRODUCTION: Maternal smoking during pregnancy is related not only to perinatal adverse events but also to important postnatal problems. Smoking is very prevalent in women with several socio-demographic factors playing an important role. AIMS: To assess the frequency of smoking as well as mothers' change in behaviours during pregnancy. To identify socio-demographic factors associated to smoking and to assess women's knowledge and sources of information about adverse effects of smoking during pregnancy. METHODS: We started a cohort study between March and October 2003 at the Maternity, Hospital Santa Maria. After an informed consent the mothers in the post natal ward were asked to fill in a questionnaire. RESULTS: Four hundred and seventy five mothers responded to the questionnaire and among them 30% were smokers before pregnancy. Thirty five percent stopped smoking during pregnancy and those who continued significantly reduced the number of cigarettes per day. Smoking was less prevalent in married women, with higher educational level and with stable employment (statistically significant correlations). Forty five percent of the mothers were misinformed or had no information about the adverse effects of smoking during pregnancy. Their main source of information were the media. DISCUSSION: Smoking prevalence was high (30%) among the respondents but the pregnancy lowered the numbers of cigarettes per day. The percentage of misinformed mothers concerned about the adverse effects of smoking during pregnancy was high (45%), which also showed that doctors' role as a source of information was surpassed by the media. The study also brought to light the need for the health professionals to improve their competences in their role on prevention of smoking during pregnancy and the postnatal period.

Orbital cysticercosis: diagnosis and treatment controversies.

Orbital cysticercosis is a rare condition and its management is controversial. We report 2 cases of orbital cellulitis associated with cysticercosis in which the treatment with antihelminthics was withheld. The 2 children had good evolution with spontaneous progressive resolution. The current literature is reviewed.