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COMPLEXITY OF PEDIATRIC CHRONIC DISEASE: CROSS-SECTIONAL STUDY WITH 16,237 PATIENTS FOLLOWED BY MULTIPLE MEDICAL SPECIALTIES

ABSTRACT

Objective:

To assess demographic data and characteristics of children and adolescents with pediatric chronic diseases (PCD), according to the number of specialties/patient.

Methods:

We performed a cross-sectional study with 16,237 PCD patients at outpatient clinics in one year. Data were analyzed by an electronic data system, according to the number of physician appointments for PCD. This study assessed: demographic data, follow-up characteristics, types of medical specialty, diagnosis (International Statistical Classification of Diseases and Related Health Problems - ICD-10), number of day hospital clinic visits, and acute complications.

Results:

Patients followed by ≥3 specialties simultaneously showed a significantly higher duration of follow-up compared to those followed by ≤2 specialties [2.1 (0.4-16.4) vs. 1.4 (0.1-16.2) years; p<0.001] and a higher number of appointments in all specialties. The most prevalent medical areas in patients followed by ≥3 specialties were: Psychiatry (Odds Ratio - OR=8.0; confidence interval of 95% - 95%CI 6-10.7; p<0.001), Palliative/Pain Care (OR=7.4; 95%CI 5.7-9.7; p<0.001), Infectious Disease (OR=7.0; 95%CI 6.4-7.8; p<0.001) and Nutrology (OR=6.9; 95%CI 5.6-8.4; p<0.001). Logistic regressions demonstrated that PCD patients followed by ≥3 specialties were associated with high risk for: number of appointments/patient (OR=9.2; 95%CI 8.0-10.5; p<0.001), day hospital clinic visits (OR=4.8; 95%CI 3.8-5.9; p<0.001), emergency department visits (OR=3.2; 95%CI 2.9-3.5; p<0.001), hospitalizations (OR=3.0; 95%CI 2.7-3.3; p<0.001), intensive care admissions (OR=2.5; 95%CI 2.1-3.0; p<0.001), and deaths (OR=2.8; 95%CI 1.9-4.0; p<0.001). The diagnosis of asthma, obesity, chronic pain, and transplant was significantly higher in patients followed by ≥3 specialties.

Conclusions:

The present study showed that PCD patients who required simultaneous care from multiple medical specialties had complex and severe diseases, with specific diagnoses.

Keywords:
Chronic disease; Child; Adolescent; Emergency; Intensive care unit; Hospitalization

RESUMO

Objetivo:

Avaliar dados demográficos e características de crianças e adolescentes com doenças crônicas pediátricas, de acordo com o número de especialidades/paciente.

Métodos:

Realizou-se um estudo transversal com 16.237 pacientes com doenças crônicas pediátricas durante um ano. A análise foi feita em um sistema eletrônico, de acordo com número de consultas médicas para doenças crônicas pediátricas. Este estudo avaliou dados demográficos, características do seguimento, tipos de especialidades médicas, diagnóstico (10ª Revisão da Classificação Estatística Internacional de Doenças e Problemas Relacionados com a Saúde - CID-10), número de visitas e complicações agudas.

Resultados:

Os pacientes acompanhados por três ou mais especialidades simultaneamente tiveram seguimento de maior duração comparados com aqueles seguidos por ≤2 especialidades [2,1 (0,4-16,4) vs. 1,4 (0,1-16,2) anos; p<0,001], bem como maior número de consultas em todas as especialidades. As áreas médicas mais comuns em pacientes acompanhados por ≥3 especialidades foram: psiquiatria (Odds Ratio - OR=8,0; intervalo de confiança de 95% - IC95% 6-10,7; p<0,001); dor/cuidados paliativos (OR=7,4; IC95% 5,7-9,7; p<0,001); doenças infecciosas (OR=7,0; IC95% 6,4-7,8; p<0,001); nutrologia (OR=6,9; IC95% 5,6-8,4; p<0,001). As regressões logísticas mostraram que os pacientes com doenças crônicas pediátricas seguidos por ≥3 especialidades tinham alto risco para: maior número de consultas/paciente (OR=9,2; IC95% 8,0-10,5; p<0,001); atendimentos em hospital-dia (OR=4,8; 95%IC3,8-5,9; p<0,001); atendimentos em pronto-socorro (OR=3,2; IC95% 2,9-3,5; p<0,001); hospitalizações (OR=3,0; IC95%2,7-3,3; p<0,001); internação em terapia intensiva (OR=2,5; IC95% 2,1-3,0; p<0,001); óbitos (OR=2,8; IC95%1,9-4,0; p<0,001). Os diagnósticos de asma, obesidade, dor crônica, transplante e infecção do trato urinário foram mais frequentes nos pacientes seguidos por três ou mais especialidades.

Conclusões:

O presente estudo mostrou que pacientes com doenças crônicas pediátricas que necessitaram de múltiplas especialidades médicas simultaneamente apresentavam doenças complexas e graves, com diagnósticos específicos.

Palavras-chave:
Doença crônica; Criança; Adolescente; Emergência; Unidade de cuidados intensivos; Hospitalização

INTRODUCTION

The prevalence of pediatric chronic diseases (PCD) has increased worldwide over the last years. This fact is due to the advancements in medical diagnosis, with new technologies and specific treatments for different illnesses and comorbidities.11. Alveno RA, Miranda CV, Passone CG, Waetge AR, Hojo ES, Farhat SC, et al. Pediatric chronic patients at outpatient clinics: a study in a Latin American University Hospital. J Pediatr (Rio J). 2018;94:539-45. http://dx.doi.org/10.1016/j.jped.2017.07.014
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Of note, child and adolescent populations with chronic health disabilities requiring highly complex medical care have been increasingly followed-up in tertiary centers. A French study with individuals younger than 14 years old showed that 3.3% of patients had long-term conditions, and 1.4% had complex chronic diseases.1212. Rachas A, Tuppin P, Meyer L, Falissard B, Faye A, Mahlaoui N, et al. Excess mortality and hospitalizations in transitional-age youths with a long-term disease: a national population-based cohort study. PLoS One. 2018;13:e0193729. https://doi.org/10.1371/journal.pone.0193729
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The outcomes are distinct for these patients and need assessment of multiple specialties.1313. Solé D, Camelo-Nunes IC, Wandalsen GF, Mallozi MC. Asthma in children and adolescents in Brazil: contribution of the International Study of Asthma and Allergies in Childhood (ISAAC). Rev Paul Pediatr. 2014;32:114-25. https://doi.org/10.1590/S0103-05822014000100018
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,1414. Della Manna T, Setian N, Savoldelli RD, Guedes DR, Kuperman H, Menezes HC Filho , et al. Diabetes mellitus in childhood: an emerging condition in the 21st century. Rev Assoc Med Bras (1992). 2016;62:594-601. https://doi.org/10.1590/1806-9282.62.06.594
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,1515. Carneiro-Sampaio M, Moraes-Vasconcelos D, Kokron CM, Jacob CM, Toledo-Barros M, Dorna MB, et al. Primary immunodeficiency diseases in different age groups: a report on 1,008 cases from a single Brazilian reference center. J Clin Immunol. 2013;33:716-24. https://doi.org/10.1007/s10875-013-9865-6
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,1616. Gormezano NW, Otsuzi CI, Barros DL, Silva MA, Pereira RM, Campos LM, et al. Macrophage activation syndrome: a severe and frequent manifestation of acute pancreatitis in 362 childhood-onset compared to 1830 adult-onset systemic lupus erythematosus patients. Semin Arthritis Rheum. 2016;45:706-10. https://doi.org/10.1016/j.semarthrit.2015.10.015
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,1717. Tannuri AC, Lima F, Mello ES, Tanigawa RY, Tannuri U. Prognostic factors for the evolution and reversibility of chronic rejection in pediatric liver transplantation. Clinics (Sao Paulo). 2016;71:216-20. https://doi.org/10.6061/clinics/2016(04)07
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,1818. Brunelli JB, Schmidt AR, Sallum AM, Goldenstein-Schainberg C, Bonfá E, Silva CA , et al. High rate of serious infection in juvenile idiopathic arthritis patients under biologic therapy in a real-life setting. Mod Rheumatol. 2018;28:264-70. https://doi.org/10.1080/14397595.2017.1349059
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Recently, a study evaluated a large population of children and adolescents with PCDs followed in a Latin American tertiary hospital. The authors reported that patients required many appointments in multiple medical specialties and hospital admissions, especially in early adolescence.11. Alveno RA, Miranda CV, Passone CG, Waetge AR, Hojo ES, Farhat SC, et al. Pediatric chronic patients at outpatient clinics: a study in a Latin American University Hospital. J Pediatr (Rio J). 2018;94:539-45. http://dx.doi.org/10.1016/j.jped.2017.07.014
https://doi.org/http://dx.doi.org/10.101...
However, no systematic analysis based on PCD patients followed simultaneously by multiple specialties and to evaluate etiological diagnosis has been carried out.

Therefore, the objective of the present study was to describe and compare demographic data and characteristics (etiological diagnosis, type of medical specialty, day hospital visit, emergency and ward visit, and intensive care hospitalizations) of children and adolescents with PCDs followed in a University Hospital, according to the number of specialties/patient.

METHOD

We performed a cross-sectional study with all PCD patients at the outpatient clinics of the Children’s Institute, Hospital das Clínicas of the School of Medicine at Universidade de São Paulo, followed during 2015. Patients treated exclusively by the emergency department, pediatric intensive care unit, and other hospitalizations were excluded. The Ethics Committee of our University Hospital approved this study.

PCDs were classified according to the duration of the disease (over three months), and the diagnosis was established by the physician’s scientific knowledge, valid methods or tools based on professional standards, and/or diagnostic classification criteria.11. Alveno RA, Miranda CV, Passone CG, Waetge AR, Hojo ES, Farhat SC, et al. Pediatric chronic patients at outpatient clinics: a study in a Latin American University Hospital. J Pediatr (Rio J). 2018;94:539-45. http://dx.doi.org/10.1016/j.jped.2017.07.014
https://doi.org/http://dx.doi.org/10.101...
,66. Mokkink LB, van der Lee JH, Grootenhuis MA, Offringa M, Heymans HS, Dutch National Consensus Committee Chronic Diseases and Health Conditions in Childhood. Defining chronic diseases and health conditions in childhood (0-18 years of age): national consensus in the Netherlands. Eur J Pediatr. 2008;167:1441-7. https://doi.org/10.1007/s00431-008-0697-y
https://doi.org/https://doi.org/10.1007/...
The 10th Revision of the International Statistical Classification of Diseases and Related Health Problems (ICD-10) was also systematically evaluated to characterize the main PCD diagnoses.66. Mokkink LB, van der Lee JH, Grootenhuis MA, Offringa M, Heymans HS, Dutch National Consensus Committee Chronic Diseases and Health Conditions in Childhood. Defining chronic diseases and health conditions in childhood (0-18 years of age): national consensus in the Netherlands. Eur J Pediatr. 2008;167:1441-7. https://doi.org/10.1007/s00431-008-0697-y
https://doi.org/https://doi.org/10.1007/...
,2121. World Health Organization. International statistical classification of diseases and related health problems [Internet]. Geneva: WHO; 2011 [cited on February 2018]. Available from: Available from: http://www.who.int/classifications/icd/ICD10Volume2_en_2010.pdf .
http://www.who.int/classifications/icd/I...
We assessed the following 23 pediatric specialties, according to the electronic data system: Allergy & Immunology, Cardiology, Endocrinology, Gastroenterology, Genetics, Hematology, Hematopoietic Cell Transplantation, Hepatology, Infectious Diseases, Nephrology/Renal Transplantation, Neurology, Nutrology, Oncology, Orthopedics, Palliative and Pain Care, Pediatric Surgery/Liver Transplantation, Pulmonology, Psychiatry, Rheumatology, and others (Adolescent Care, Preterm Care, and Pediatric Teaching Clinic).

We conducted data analyses using the electronic data system of institution, according to the number of physician appointments for PCDs. This study assessed: demographic data (current age, gender, and local of residence); characteristics of follow-up (duration, number of physician’s appointments/patient, and number of specialties/patient), types of pediatric specialty, number of day hospital clinic visits, and acute complications (number of emergency department visits, hospitalizations, intensive care unit admissions, and deaths). The etiology of each diagnosis was established based on ICD-10.

We divided the PCD patients into two groups, according to the number of pediatric specialties/patient: ≥3 specialties and ≤2 specialties.

The sample size provided a power of 80% to find differences of less than 2% in the two groups: ≥3 specialties and ≤2 specialties (GraphPad StatMate 1.01, GraphPad Software, Inc., CA, USA). The IBM-SPSS-22 software performed the statistical analyses. We presented the results as median (range) or mean±standard deviation (SD) for continuous variables and number (%) for categorical variables. Mann-Whitney test or Student’s t-test compared the continuous variables between the two study groups (≥3 specialties and ≤2 specialties). For categorical variables, the differences were evaluated by Fisher’s exact test. The multivariate analysis was carried out using backward stepwise logistic regression. In the regression model, the dependent variable was the presence of ≥3 specialties, and the independent variables were those with less than 20% significance level in the univariate analysis. For all statistical tests, p<0.05 was considered significant.

RESULTS

From January to December 2015, 16,237 children and adolescents with PCDs were followed by 23 pediatric specialties in our Children’s Hospital.

Table 1 includes demographic data and characteristics of these children and adolescents with PCDs followed in a University Hospital, according to the number of specialties/patient. Patients followed by ≥3 specialties showed a significantly higher duration of follow-up compared to those followed by ≤2 specialties [2.1 (confidence interval of 95% - 95%CI 0.4-16.4) vs. 1.4 (95%CI 0.1-16.2) years; p<0.001], similar to the number of appointments in all specialties (≥3 specialties=24% vs. ≤2 specialties=3%; p<0.001). Frequencies of pediatric specialties, such as Infectious Disease (47 vs. 11%; p<0.001), Endocrinology (33 vs. 15%; p<0.001), Nephrology/Renal Transplantation (28 vs. 8%; p<0.001), and Pediatric Surgery/Liver Transplantation (28 vs. 14%; p<0.001), were significantly higher in the former group. We found no differences in patients followed by Oncology (Table 1). Patients followed by ≥3 specialties had significantly higher frequencies of day hospital clinic visits (7 vs. 2%; p<0.001), emergency department visits (45 vs. 21%; p<0.001), hospitalizations (35 vs. 15%; p<0.001), intensive care admissions (9 vs. 4%; p<0.001), and deaths (2 vs. 1%; p<0.001) (Table 2).

Table 1
Demographic data and characteristics of 16,237 children and adolescents with pediatric chronic diseases followed in a University Hospital, according to the number of specialties/patient.
Table 2
Characteristics of emergency department visits and hospitalization of 16,237 children and adolescents with pediatric chronic diseases followed in a University Hospital, according to the number of specialties/patient.

Table 3 illustrates the logistic regression analysis between the dependent variable (≥3 specialties) and the number of appointments, types of specialty, hospitalizations, intensive care unit admissions, and deaths. Psychiatry (Odds Ratio - OR=8.0; 95%CI 6-10.7; p<0.001), Palliative and Pain Care (OR=7.4; 95%CI 5.7-9.7; p<0.001), Infectious Disease (OR=7.0; 95%CI 6.4-7.8; p<0.001) and Nutrology (OR=6.9; 95%CI 5.6-8.4; p<0.001) were the medical areas with higher chances of being one of the three or more medical specialties. Logistic regression analysis demonstrated that PCD patients followed by ≥3 specialties were associated with higher risk of appointments/patient (>13) (OR=9.2; 95%CI 8.0-10.5; p<0.001), day hospital clinic visits (OR=4.8; 95%CI 3.8-5.9; p<0.001), emergency department visits (OR=3.2; 95%CI 2.9-3.5; p<0.001), hospitalizations (OR=3.0; 95%CI 2.7-3.3; p<0.001), intensive care admissions (OR=2.5; 95%CI 2.1-3.0; p<0.001), and deaths (OR=2.8; 95%CI 1.9-4.0; p<0.001) (Table 2).

Table 3
Multiple logistic regression analysis between the dependent variable (≥3 specialties) and the number of appointments, type of medical specialty, hospitalizations, intensive care unit admissions, and deaths.

Of note, we evaluated 106,437 appointments of 16,237 children and adolescents with PCD. The diagnosis of each PCD patient was only considered once, thus resulting in a total of 37.057 diagnoses. More than 2,500 ICD-10 were registered in PCD patients. The 20 most prevalent ICD-10 observed in PCD patients were: asthma (n=1538; 4.15%), short stature (n=1054; 2.84%), leukemia (n=501; 1.35%), obesity (n=435; 1.17%), malformation syndromes (n=429; 1.16%), transplant (n=420; 1.13%), urinary tract infection (n=384; 1.04%), epilepsy (n=306; 0.83%), hypothyroidism (n=286; 0.77%), pneumonia (n=282; 0.76%), chronic kidney disease (n=264; 0.71%), type 1 diabetes (n=248; 0.67%), chronic pain (n=243; 0.66%), diarrhea (n=244; 0.66%), malnutrition (n=214; 0.58%), constipation (n=214; 0.58%), juvenile idiopathic arthritis (n=186; 0.5%), high blood pressure (n=177; 0.48%), cystic fibrosis (n=174; 0.47%), and primary immunodeficiency (n=121; 0.33%).

Table 3 presents further comparisons of the 20 most prevalent associated diagnoses based on 37,057 ICD-10 descriptions, according to the number of specialties/patient. The diagnosis of asthma, obesity, chronic pain, transplant, urinary tract infection, pneumonia, chronic kidney disease, malnutrition, epilepsy, hypothyroidism, high blood pressure, diarrhea, constipation, and immunodeficiency were significantly higher in patients followed by ≥3 specialties compared to those followed by ≤2 specialties (Table 4).

Table 4
Comparison of the 20 most prevalent diagnosis based on 37,057 International Statistical Classification of Diseases and Related Health Problems descriptions, according to the number of specialties/patient.

DISCUSSION

The present study showed that PCD patients who required simultaneous care from multiple medical specialties had complex and severe diseases, with specific diagnoses.

The prevalence of PCDs has increased in the last half-century. Chronic diseases in pediatric populations that interfere with daily activities have increased more than 400% after 1960, resulting in greater medical complexity in clinical practice.2222. Cohen E, Berry JG, Sanders L, Schor EL, Wise PH. Status Complexicus? The emergence of pediatric complex care. Pediatrics. 2018;141 Suppl 3:S202-11. https://doi.org/10.1542/peds.2017-1284E
https://doi.org/https://doi.org/10.1542/...
Our study demonstrated that PCD patients followed by various medical specialties had high morbidity and mortality rates, with emergency department visits, hospitalizations, intensive care admissions, and deaths. These PCD patients needed frequent appointments at outpatient clinics, a fact that could lead to higher financial costs, requiring specific policies and interventions.11. Alveno RA, Miranda CV, Passone CG, Waetge AR, Hojo ES, Farhat SC, et al. Pediatric chronic patients at outpatient clinics: a study in a Latin American University Hospital. J Pediatr (Rio J). 2018;94:539-45. http://dx.doi.org/10.1016/j.jped.2017.07.014
https://doi.org/http://dx.doi.org/10.101...
,2222. Cohen E, Berry JG, Sanders L, Schor EL, Wise PH. Status Complexicus? The emergence of pediatric complex care. Pediatrics. 2018;141 Suppl 3:S202-11. https://doi.org/10.1542/peds.2017-1284E
https://doi.org/https://doi.org/10.1542/...

Moreover, PCD patients of the present study followed by various medical specialties showed heterogeneity of diagnosis and subspecialties. Infectious Disease was the most frequent specialty for these patients, probably due to recurrent and severe infections, requiring multiple hospitalizations in immunosuppressed patients.1515. Carneiro-Sampaio M, Moraes-Vasconcelos D, Kokron CM, Jacob CM, Toledo-Barros M, Dorna MB, et al. Primary immunodeficiency diseases in different age groups: a report on 1,008 cases from a single Brazilian reference center. J Clin Immunol. 2013;33:716-24. https://doi.org/10.1007/s10875-013-9865-6
https://doi.org/https://doi.org/10.1007/...
,1616. Gormezano NW, Otsuzi CI, Barros DL, Silva MA, Pereira RM, Campos LM, et al. Macrophage activation syndrome: a severe and frequent manifestation of acute pancreatitis in 362 childhood-onset compared to 1830 adult-onset systemic lupus erythematosus patients. Semin Arthritis Rheum. 2016;45:706-10. https://doi.org/10.1016/j.semarthrit.2015.10.015
https://doi.org/https://doi.org/10.1016/...
,1717. Tannuri AC, Lima F, Mello ES, Tanigawa RY, Tannuri U. Prognostic factors for the evolution and reversibility of chronic rejection in pediatric liver transplantation. Clinics (Sao Paulo). 2016;71:216-20. https://doi.org/10.6061/clinics/2016(04)07
https://doi.org/https://doi.org/10.6061/...
,1818. Brunelli JB, Schmidt AR, Sallum AM, Goldenstein-Schainberg C, Bonfá E, Silva CA , et al. High rate of serious infection in juvenile idiopathic arthritis patients under biologic therapy in a real-life setting. Mod Rheumatol. 2018;28:264-70. https://doi.org/10.1080/14397595.2017.1349059
https://doi.org/https://doi.org/10.1080/...
Despite the relevant hospital-acquired infection committee, vaccination programs, and specific antibiotic and antifungal treatments for different PCDs in our University Hospital, patients might have many contributing factors for infections. These factors may be related to the disease itself (disease duration, lymphopenia, leukopenia, neutropenia, disease activity, functional asplenia, and primary immunodeficiencies) and its treatment (glucocorticoid and immunosuppressant agents).1313. Solé D, Camelo-Nunes IC, Wandalsen GF, Mallozi MC. Asthma in children and adolescents in Brazil: contribution of the International Study of Asthma and Allergies in Childhood (ISAAC). Rev Paul Pediatr. 2014;32:114-25. https://doi.org/10.1590/S0103-05822014000100018
https://doi.org/https://doi.org/10.1590/...
,1515. Carneiro-Sampaio M, Moraes-Vasconcelos D, Kokron CM, Jacob CM, Toledo-Barros M, Dorna MB, et al. Primary immunodeficiency diseases in different age groups: a report on 1,008 cases from a single Brazilian reference center. J Clin Immunol. 2013;33:716-24. https://doi.org/10.1007/s10875-013-9865-6
https://doi.org/https://doi.org/10.1007/...
,1616. Gormezano NW, Otsuzi CI, Barros DL, Silva MA, Pereira RM, Campos LM, et al. Macrophage activation syndrome: a severe and frequent manifestation of acute pancreatitis in 362 childhood-onset compared to 1830 adult-onset systemic lupus erythematosus patients. Semin Arthritis Rheum. 2016;45:706-10. https://doi.org/10.1016/j.semarthrit.2015.10.015
https://doi.org/https://doi.org/10.1016/...
,1818. Brunelli JB, Schmidt AR, Sallum AM, Goldenstein-Schainberg C, Bonfá E, Silva CA , et al. High rate of serious infection in juvenile idiopathic arthritis patients under biologic therapy in a real-life setting. Mod Rheumatol. 2018;28:264-70. https://doi.org/10.1080/14397595.2017.1349059
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,2424. Silva CA , Aikawa NE, Pereira RM , Campos LM . Management considerations for childhood-onset systemic lupus erythematosus patients and implications on therapy. Expert Rev Clin Immunol. 2016;12:301-13. https://doi.org/10.1586/1744666X.2016.1123621
https://doi.org/https://doi.org/10.1586/...

Endocrinology, Nephrology, Renal Transplantation, Pediatric Surgery, and Liver Transplantation were also frequent subspecialties in patients followed by more than three concomitant specialties. This fact results from a specific situation of our tertiary hospital, which is a reference center for the most common and severe endocrine, kidney, liver, and congenital diseases in Brazil.1414. Della Manna T, Setian N, Savoldelli RD, Guedes DR, Kuperman H, Menezes HC Filho , et al. Diabetes mellitus in childhood: an emerging condition in the 21st century. Rev Assoc Med Bras (1992). 2016;62:594-601. https://doi.org/10.1590/1806-9282.62.06.594
https://doi.org/https://doi.org/10.1590/...
,1616. Gormezano NW, Otsuzi CI, Barros DL, Silva MA, Pereira RM, Campos LM, et al. Macrophage activation syndrome: a severe and frequent manifestation of acute pancreatitis in 362 childhood-onset compared to 1830 adult-onset systemic lupus erythematosus patients. Semin Arthritis Rheum. 2016;45:706-10. https://doi.org/10.1016/j.semarthrit.2015.10.015
https://doi.org/https://doi.org/10.1016/...
,2525. Tannuri AC , Porta G, Miura IK, Santos MM, Moreira DA, Rezende NM, et al. Pediatric acute liver failure in Brazil: Is living donor liver transplantation the best choice for treatment? Liver Transpl. 2016;22:1006-13. https://doi.org/10.1002/lt.24435
https://doi.org/https://doi.org/10.1002/...

Importantly, Psychiatry and Palliative and Pain Care were the medical areas in which the patient had a great chance of being followed by three or more medical specialties. This finding is related to the medical complexities in our critically ill patients,2525. Tannuri AC , Porta G, Miura IK, Santos MM, Moreira DA, Rezende NM, et al. Pediatric acute liver failure in Brazil: Is living donor liver transplantation the best choice for treatment? Liver Transpl. 2016;22:1006-13. https://doi.org/10.1002/lt.24435
https://doi.org/https://doi.org/10.1002/...
,2626. Nelson KE, Feinstein JA, Gerhardt CA, Rosenberg AR, Widger K, Faerber JA, et al. Emerging methodologies in pediatric palliative care research: six case studies. Children (Basel). 2018;5.pii:E32. https://doi.org/10.3390/children5030032
https://doi.org/https://doi.org/10.3390/...
who may also present several mental health conditions and chronic pain diseases.

Asthma was the main diagnosis associated with PCD patients followed by ≥3 specialties. The prevalence of this relevant diagnosis in the city of São Paulo ranges from 4.9 to 10.2%, similarly to our data.2727. Solé D , Yamada E, Vana AT, Werneck G, Solano de Freitas L, Sologuren MJ, et al. International Study of Asthma and Allergies in Childhood (ISAAC): prevalence of asthma and asthma-related symptoms among Brazilian schoolchildren. J Investig Allergol Clin Immunol. 2001;11:123-8. Additionally, 22% of adolescents reported wheezing symptoms.2828. Pastorino AC, Rimazza RD, Leone C, Castro AP, Solé D , Jacob CM . Risk factors for asthma in adolescents in a large urban region of Brazil. J Asthma. 2006;43:695-700. https://doi.org/10.1080/02770900600925544
https://doi.org/https://doi.org/10.1080/...
This chronic disease might lead to more frequent admission rates, requiring emergency department visits, hospitalizations, and intensive care admissions. Therapeutic optimization for PCD patients should be an important goal to attain.1313. Solé D, Camelo-Nunes IC, Wandalsen GF, Mallozi MC. Asthma in children and adolescents in Brazil: contribution of the International Study of Asthma and Allergies in Childhood (ISAAC). Rev Paul Pediatr. 2014;32:114-25. https://doi.org/10.1590/S0103-05822014000100018
https://doi.org/https://doi.org/10.1590/...
,2727. Solé D , Yamada E, Vana AT, Werneck G, Solano de Freitas L, Sologuren MJ, et al. International Study of Asthma and Allergies in Childhood (ISAAC): prevalence of asthma and asthma-related symptoms among Brazilian schoolchildren. J Investig Allergol Clin Immunol. 2001;11:123-8.

Short stature was also relevant herein, a multifactorial finding related to long disease duration, chronic inflammatory diseases, physical inactivity, and therapies (such as glucocorticoid). In addition, Endocrinology and Nutrology were significant specialties due to the increase in obesity in the general population, as well as in chronic disease patients.2929. Belle FN, Weiss A, Schindler M, Goutaki M, Bochud M, Zimmermann K, et al. Overweight in childhood cancer survivors: the Swiss childhood cancer survivor study. Am J Clin Nutr. 2018;107:3-11. https://doi.org/10.1093/ajcn/nqx006
https://doi.org/https://doi.org/10.1093/...

Pediatric care systems and multidisciplinary health teams should develop evidence-based solutions to the challenges of caring for and treating PCD patients with a medically complex disease.2323. Litvinov N, Silva MT, van der Heijden IM, Graça MG, Marques de Oliveira L, Fu L, et al. An outbreak of invasive fusariosis in a children’s cancer hospital. Clin Microbiol Infect. 2015;21:268.e1-7. https://doi.org/10.1016/j.cmi.2014.09.004
https://doi.org/https://doi.org/10.1016/...
Strategies should be devised focusing on reducing hospital admission rates, thus improving hospital care for these patients.

The strong point of this study was the large population with different PCDs defined by ICD-10 descriptions and followed in a tertiary and pediatric teaching hospital. Our university hospital is a Brazilian reference center for pediatric and complex specialties that follows PCDs. This center is well known for its high standard and humane care, has many health care providers, modern resources with clinical/laboratory research and drug trials, evaluating children and adolescents with medically complex conditions.11. Alveno RA, Miranda CV, Passone CG, Waetge AR, Hojo ES, Farhat SC, et al. Pediatric chronic patients at outpatient clinics: a study in a Latin American University Hospital. J Pediatr (Rio J). 2018;94:539-45. http://dx.doi.org/10.1016/j.jped.2017.07.014
https://doi.org/http://dx.doi.org/10.101...
,1515. Carneiro-Sampaio M, Moraes-Vasconcelos D, Kokron CM, Jacob CM, Toledo-Barros M, Dorna MB, et al. Primary immunodeficiency diseases in different age groups: a report on 1,008 cases from a single Brazilian reference center. J Clin Immunol. 2013;33:716-24. https://doi.org/10.1007/s10875-013-9865-6
https://doi.org/https://doi.org/10.1007/...
,1616. Gormezano NW, Otsuzi CI, Barros DL, Silva MA, Pereira RM, Campos LM, et al. Macrophage activation syndrome: a severe and frequent manifestation of acute pancreatitis in 362 childhood-onset compared to 1830 adult-onset systemic lupus erythematosus patients. Semin Arthritis Rheum. 2016;45:706-10. https://doi.org/10.1016/j.semarthrit.2015.10.015
https://doi.org/https://doi.org/10.1016/...
The main limitations of the present study were the short evaluating period and its cross-sectional design.

In conclusion, the present study showed that PCD patients who needed care from multiple medical specialties had complex and severe diseases. PCD patients presented specific diagnoses, particularly asthma, short stature, and leukemia

ACKNOWLEDGMENTS

We thank Thiago de Freitas Passone for the database and all physicians of pediatric specialties, multidisciplinary groups, and Informatics team of our university hospital

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Funding

  • This study was funded by grants from the Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq; 303422/2015-7 to CAS), Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP; 2015/03756-4 to CAS), Federico Foundation, Switzerland (to CAS) and by the Núcleo de Apoio à Pesquisa “Saúde da Criança e do Adolescente” (NAP-CriAd) from Universidade de São Paulo (to MCS and CAS).

Publication Dates

  • Publication in this collection
    25 Nov 2019
  • Date of issue
    2020

History

  • Received
    09 Apr 2018
  • Accepted
    30 Aug 2018
  • Published
    08 Nov 2019
Sociedade de Pediatria de São Paulo R. Maria Figueiredo, 595 - 10o andar, 04002-003 São Paulo - SP - Brasil, Tel./Fax: (11 55) 3284-0308; 3289-9809; 3284-0051 - São Paulo - SP - Brazil
E-mail: rpp@spsp.org.br