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  • article 0 Citação(ões) na Scopus
    Patau and Edwards Syndromes in a University Hospital: beyond palliative care
    (2024) MARCOLA, Ligia; ZOBOLI, Ivete; POLASTRINI, Rita Tiziana Verardo; BARBOSA, Silvia Maria de Macedo; FALCAO, Mario Cicero; GAIOLLA, Paula de Vicenzi
    Objective:To describe the newborn population with Patau (T13) and Edwards Syndrome (T18) with congenital heart diseases that stayed in the Intensive Care Unit (ICU) of a quaternary care hospital complex, regarding surgical and non -surgical medical procedures, palliative care, and outcomes. Methods: Descriptive case series conducted from January/2014 to December/2018 through analysis of records of patients with positive karyotype for T13 or T18 who stayed in the ICU of a quaternary hospital. Descriptive statistics analysis was applied. Results: 33 records of eligible patients were identified: 27 with T18 (82%), and 6 T13 (18%); 64% female and 36% male. Eight were preterm infants with gestational age between 30-36 weeks (24%), and only 4 among the 33 infants had a birth weight >2500 g (12%). Four patients underwent heart surgery and one of them died. Intrahospital mortality was 83% for T13, and 59% for T18. The majority had other malformations and underwent other surgical procedures. Palliative care was offered to 54% of the patients. The median hospitalization time for T18 and T13 was 29 days (range: 2-304) and 25 days (13-58), respectively. Conclusions: Patients with T13 and T18 have high morbidity and mortality, and long hospital and ICU stays. Multicentric studies are needed to allow the analysis of important aspects for creating protocols that, seeking therapeutic proportionality, may bring better quality of life for patients and their families.
  • article 0 Citação(ões) na Scopus
    Postorotracheal intubation dysphagia in patients with COVID-19: A retrospective study
    (2024) SACONATO, Mariana; MASELLI-SCHOUERI, Jean Henri; MALAQUE, Ceila Maria Sant'Ana; MARCUSSO, Rosa Maria; OLIVEIRA, Augusto Cesar Penalva de; BATISTA, Lucio Antonio Nascimento; ULTRAMARI, Graziela; LINDOSO, Jose Angelo Lauletta; GONCALVES, Maria Ines Rebelo; SZTAJNBOK, Jaques
    BACKGROUND: The cause of oropharyngeal dysphagia in patients with coronavirus disease (COVID-19) can be multifactorial and may underly limitations in swallowing rehabilitation. OBJECTIVE: Analyze the factors related to dysphagia in patients with COVID-19 immediately after orotracheal extubation and the factors that influence swallowing rehabilitation. DESIGN AND SETTING: A retrospective study. METHODS: The presence of dysphagia was evaluated using the American Speech-Language Hearing Association National Outcome Measurement System (ASHA NOMS) scale and variables that influenced swallowing rehabilitation in 140 adult patients who required invasive mechanical ventilation for >48 h. RESULTS: In total, 46.43% of the patients scored 1 or 2 on the ASHA NOMS (severe dysphagia) and 39.29% scored 4 (single consistency delivered orally) or 5 (exclusive oral diet with adaptations). Both the length of mechanical ventilation and the presence of neurological disorders were associated with lower ASHA NOMS scores (odds ratio [OR]: 0.80, 95% confidence interval [CI]: 0.74-0.87 P < 0.05; and OR: 0.13, 95% CI: 0.61-0.29; P < 0.05, respectively). Age and the presence of tracheostomy were negatively associated with speech rehabilitation (OR: 0.92; 95% CI: 0.87--0.96; OR: 0.24; 95% CI: 0.80--0.75), and acute post-COVID-19 kidney injury requiring dialysis and lower scores on the ASHA NOMS were associated with longer time for speech therapy outcomes ( beta : 1.62, 95% CI, 0.70-3.17, P < 0.001; beta : -1.24, 95% CI: -1.55--0.92; P < 0.001). CONCLUSION: Prolonged orotracheal intubation and post-COVID-19 neurological alterations increase the probability of dysphagia immediately after extubation. Increased age and tracheostomy limited rehabilitation.
  • article 0 Citação(ões) na Scopus
    Health-related quality of life for pediatric patients with end-stage kidney disease: A systematic review and meta-analysis of the Pediatric Quality of Life Inventory (PedsQL)
    (2024) DOSHI, Kush; RAINA, Rupesh; NG, Kar Hui; KOCH, Vera; BHATT, Girish C.; NADA, Arwa; FORESI, Brian; KAMALAKKANNAN, Sibee Sambandam; MCCULLOCH, Mignon; SETHI, Sidharth; FERRIS, Maria Diaz-Gonzalez de
    IntroductionHealth-related quality of life (HRQoL) studies demonstrate the impact of end-stage renal disease (ESRD) on the physical and psychosocial development of children. While several instruments are used to measure HRQoL, few have standardized domains specific to pediatric ESRD. This review examines current evidence on self and proxy-reported HRQoL among pediatric patients with ESRD, based on the Pediatric Quality of Life Inventory (PedsQL) questionnaires.MethodsFollowing PRISMA guidelines, we conducted a systematic review and meta-analysis on HRQoL using the PedsQL 4.0 Generic Core Scale (GCS) and the PedsQL 3.0 ESRD Module among 5- to 18-year-old patients. We queried PubMed, Embase, Web of Science, CINAHL, and Cochrane databases. Retrospective, case-controlled, and cross-sectional studies using PedsQL were included.FindingsOf 435 identified studies, 14 met inclusion criteria administered in several countries. Meta-analysis demonstrated a significantly higher total HRQoL for healthy patients over those with ESRD (SMD:1.44 [95% CI: 0.78-2.09]) across all dimensional scores. In addition, kidney transplant patients reported a significantly higher HRQoL than those on dialysis (PedsQL GCS, SMD: 0.33 [95% CI: 0.14-0.53]) and (PedsQL ESRD, SMD: 0.65 [95% CI: 0.39-0.90]) concordant with parent-proxy reports.DiscussionPatients with ESRD reported lower HRQoL in physical and psychosocial domains compared with healthy controls, while transplant and peritoneal dialysis patients reported better HRQoL than those on hemodialysis. This analysis demonstrates the need to identify dimensions of impaired functioning and produce congruent clinical interventions. Further research on the impact of individual comorbidities in HRQoL is necessary for developing comprehensive, integrated, and holistic treatment programs.
  • article 0 Citação(ões) na Scopus
    Pediatric hypertension as an early manifestation of cardiovascular disease in children
    (2024) KOCH, Vera Hermina Kalika; FURUSAWA, Erika Arai
    In adults, cardiovascular events associated with arterial hypertension (AH) have a major impact on morbidity and mortality. In light of recent findings, AH in children has been interpreted as early cardiovascular disease (CVD), while exposure to CV risk factors in children proves to be a predictor of subclinical CVD in adults. The American College of Cardiology/American Heart Association has recently updated the classifications for measuring blood pressure (BP) in adults and children. Primary AH in children is generally asymptomatic, and it is associated with a family history of AH, overweight/obesity, and normal morphofunctional characteristics of the urinary system. The younger the child and the higher the BP, the greater the likelihood of secondary AH. The investigation into the etiology of AH begins with a detailed anamnesis, which should include clinical information and details on the use of medication, smoking, and alcohol consumption from the perinatal period to the time of consultation. Modifying risk factors by reducing weight, decreasing alcohol consumption and increasing vegetable intake from childhood to adulthood has been associated with the resolution of AH in the childhoodadulthood transition, and with the reversal of cardiometabolic adverse effects in non-obese adult individuals. Pharmacological therapy should be initiated in cases of symptomatic AH, AH secondary to chronic kidney disease or diabetes mellitus, presence of target organ lesions, stage 2 AH with no modifiable cause and resistant AH unresponsive to lifestyle changes.
  • article 2 Citação(ões) na Scopus
    Feeding difficulties in children with food allergies: An EAACI Task Force Report
    (2024) HILL, Sarah-Anne; NURMATOV, Ulugbek; DUNNGALVIN, Audrey; REESE, Imke; VIEIRA, Mario C.; ROMMEL, Natalie; DUPONT, Christophe; VENTER, Carina; CIANFERONI, Antonella; WALSH, Joanne; YONAMINE, Glauce; BEAUREGARD, Alexia; MEYER, Rosan; VAZQUEZ-ORTIZ, Marta
    The term ""feeding difficulties"" refers to a spectrum of phenotypes characterized by suboptimal intake of food and/or lack of age-appropriate eating habits. While it is evident that feeding difficulties are prevalent within healthy children, no consensus has been reached for those with food allergies. The aim of this study was to systematically review all the available literature reporting the prevalence of feeding difficulties within food allergic children. We searched eight international electronic databases for all published studies until June 2022. International experts in the field were also contacted for unpublished and ongoing studies. All publications were screened against pre-defined eligibility criteria and critically appraised by established instruments. The substantial heterogeneity of included studies precluded meta-analyses, so narrative synthesis of quantitative data was performed. A total of 2059 abstracts were assessed, out of which 21 underwent full-text screening and 10 studies met the study criteria. In these, 12 different terms to define feeding difficulties and 11 diagnostic tools were used. Five papers included data of feeding difficulty prevalence in children with food allergies, ranging from 13.6% to 40%. Higher prevalence was associated with multiple food allergies. The current literature suggests that feeding difficulties are prevalent within food allergic children, particularly those with multiple food allergies. However, the heterogeneity of terminologies and diagnostic tools makes drawing conclusions challenging. Consensus guidelines for the diagnosis and management of feeding difficulties within food allergic children and further research on the development and perpetuation of feeding difficulties are needed to appropriately manage such patients.
  • article 0 Citação(ões) na Scopus
    Epidemiological Assessment of a Pediatric Palliative Care Clinic at a Brazilian Quaternary Hospital: 20 Years of Experience
    (2024) SPOLADOR, Gustavo Marquezani; BASTOS, Fernanda; POLASTRINI, Rita Tiziana Verardo; ZOBOLI, Ivete; HENRIQUE, Ana Cristina; VIEIRA FILHO, Joaquim Pinheiro; SILVEIRA, Mariana Ribeiro Marcondes da; FREITAS, Elaine; NASCIMENTO, Andrea Gislene do; BARBOSA, Silvia Maria de Macedo
    Background: The pediatric palliative care (PPC) sets up an interdisciplinary approach of chronic complex diseases throughout birth to adolescence. It encompasses countless contrasts in development and diagnosis scopes, which make this area a challenge to nonpediatric practitioners. Objective: We sought to assess the most prevalent diseases in follow-up of the PPC team. Methods: We analyzed the medical records of PPC clinic during the years 2001 and 2021 and the diagnosis of outpatients. We established a parallel with the world scientific literature concerning the epidemiology of PPC. Results: The most prevalent diseases were epidermolysis bullosa (36.9%), followed by neurological Inherited Errors of Metabolism (IEM) diseases (19.0%), IEM diseases (14.3%), dysmorphological and chromosomal disorders (8.5%), skeletal disorders mainly osteogenesis imperfecta (6.9%), and liver transplantation conditions (5.5%) (p < 0.001). The less frequent conditions were external causes, such as neonatal insults or traffic accidents (2.8%), cancer (1.7%), congenital cardiopathies (1.4%), congenital infectious diseases (1.1%), gastrointestinal and hepatic conditions (0.8%), and rheumatological conditions (0.3%). The patients were older at diagnosis (6.9 years) and at PPC referral (13.2 years) than patients with epidermolysis bullosa and skeletal disorders and dysmorphological and chromosomal disorders were younger on referral. Conclusion: There are a lot of complex chronic conditions which could benefit from palliative care in pediatric setting. However, epidemiological and symptomatological assessment of the health service is necessary to provide an appropriate care to the country's reality.
  • article 0 Citação(ões) na Scopus
    Autoinflammatory diseases: a Latin American multicenter to and sex
    (2024) PIOTTO, Daniela Gerent Petry; KOZU, Katia; AIKAWA, Nadia Emi; CARNEIRO, Pedro Lopes; KATSICAS, Maria Martha; OLIVEIRA, Sheila Knupp Feitosa de; FERNANDES, Taciana de Albuquerque Pedrosa; MAGALHAES, Claudia Saad; CUNHA, Ana Luiza Garcia; BICA, Blanca Elena Rios Gomes; RABELO JUNIOR, Carlos Nobre; BATTAGLIOTTI, Cristina; MATOS, Erica Naomi Naka; SANTOS, Flavia Patricia Sena Teixeira; SZTAJNBOK, Flavio Roberto; BEZRODNIK, Liliana; BANDEIRA, Marcia; RODRIGUES, Marta Cristine Felix; MUNITTIS, Pablo Garcia; APPENZELLER, Simone; ROBAZZI, Teresa Cristina Martins; CLEMENTE, Gleice; SILVA, Clovis Artur; TERRERI, Maria Teresa
    Objective: To evaluate autoinflammatory diseases (AID) according to age at diagnosis and sex, and response to therapy in a large population. Methods: This is a cross-sectional observational study of a Latin American registry using a designed web system for data storage, collected between 2015 and 2018. Any altered findings during follow-up were recorded. The forms were translated into Portuguese and Spanish, including demographic, clinical, laboratory, genetic and treatment characteristics. Results: We included 152 patients, 51.3% male and 75% Caucasian. The median age at disease onset was 2.1 years (0-15.6 years) and median age at diagnosis 6.9 years (0-21.9 years); 111 (73%) were children (0-9 years old), and 41 (27%) were adolescents and young adults (AYA) (10-21 years old). Periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome (PFAPA) occurred in 46/152 (30%), chronic non-bacterial osteomyelitis (CNO) in 32/152 (21%), and familial Mediterranean fever (FMF) in 24/152 (15.7%). PFAPA was significantly higher in young children than in AYA (38.7% vs. 7.3%, p<0.001), while CNO were lower (13.5% vs. 41.5%, p<0.001). The frequency of females was significantly higher in CNO (28.4% vs. 14.1%, p=0.031) and lower in FMF (8.1% vs. 23.1%, p=0.011). The most used drugs were glucocorticoids, non -steroidal anti-inflammatory drugs (NSAID), and colchicine. Glucocorticoids and colchicine treatment were used in all AID with good to moderate response. However, cryopyrinassociated periodic syndromes (CAPS) seemed unresponsive to glucocorticoids. NSAIDs and methotrexate were the main medications used to treat CNO. Conclusions: Differences among AID patients were observed in the LA population regarding sex and age at disease diagnosis.
  • article
    Speckle-Tracking: Incremental Role in Diastolic Assessment of Pediatric Patients with Chronic Kidney Disease
    (2024) PENACHIO, Flora Maciel; DINIZ, Maria de Fatima Rodrigues; LAURINO, Rosana Sbruzzi Prado; WATANABE, Andreia; SAWAMURA, Karen Saori Shiraishi; LIANZA, Alessandro Cavalcanti; MENEZES, Carolina Rocha Brito; SILVA, Isabela de Sousa Lobo; LEAL, Gabriela Nunes
    Background: Cardiovascular complications are the leading cause of mortality in pediatric patients with chronic kidney disease (CKD). Echocardiographic assessment of diastolic function in CKD has been limited to spectral and tissue Doppler imaging, known to be less reliable techniques in pediatrics. Two-dimensional Speckle tracking echocardiography (2DST) derived left atrial (LA) strain has recently been confirmed as a robust measure of diastolic function. Objectives: To investigate LA strain role in diastolic assessment of children at different stages of CKD. Methods: From February 2019 to July 2022, 55 CKD patients without cardiovascular symptoms and 55 controls were evaluated by standard and 2DST echocardiograms. The level of significance was set at 5% (p<0.05). Results: Patients and controls had similar age [9.78 (0.89 - 17.54) vs. 10.72 (1.03 -18,44) years; p = 0.41] and gender (36M:19F vs. 34M:21F; p=0.84). There were 25 non-dialysis patients and 30 dialysis patients. Left ventricular ejection fraction was >= 55% in all of them. Comparing CKD and controls, LA reservoir strain was lower (48.22 +/- 10.62% vs. 58.52 +/- 10.70%) and LA stiffness index was higher [0.14 (0.08-0.48)% -1 vs. 0.11 (0.06-0.23) % -1 ]; p<0.0001. LV hypertrophy was associated with lower LA reservoir strain (42.05 +/- 8.74% vs. 52.99 +/- 9.52%), higher LA stiffness [0.23(0.11 - 0.48)% -1 vs. 0.13 (0.08-0.23) % -1 and filling indexes (2.39 +/- 0.63 cm/s x % -1 vs. 1.74 +/- 0.47 cm/s x % (-1) ; p<0.0001. Uncontrolled hypertension was associated with lower LA reservoir strain (41.9 +/- 10.6% vs. 50.6 +/- 9.7; p=0.005). Conclusions: LA strain proved to be a feasible tool in the assessment of pediatric CKD patients and was associated with known cardiovascular risk factors.
  • article 1 Citação(ões) na Scopus
    Imaging Features and Complications of Facial Cosmetic Procedures
    (2023) SOUSA, Andrea Meneses Soares de; DUARTE, Angelo Chelotti; DECNOP, Marcos; GUIMARAES, Daniel de Faria; COELHO NETO, Carlos Alberto Ferreira; SARPI, Maira de Oliveira; DUARTE, Luis Gustavo Palhiari; SOUZA, Soraia Ale; SEGATO, Larissa Freire; ZAVARIZ, Julia Diva; MUKHERJI, Suresh K.; GARCIA, Marcio Ricardo Taveira
    Facial aesthetic procedures have become increasingly popular and complex, making knowledge of facial anatomy crucial for achiev-ing desired outcomes without complications. Some of the most common procedures include blepharoplasty, bichectomy, face-lifts, facial implants, thread lifting, and fillers. Blepharoplasty and bi-chectomy are surgical procedures that respectively aim to restore youthful contours to the periorbita and create a slimmer lower face by removing Bichat fat from the maxillofacial region. Facial im-plants are used for aesthetic augmentation of the skeletal structure and restoration of facial contour by using biomaterials or autoge-nous bone grafts. Face-lift surgeries involve incisions and removal of excess skin, and thread lifts involve less invasive procedures performed by inserting threads beneath the skin, with the aim to lift the skin and thus reduce wrinkles and sagging. Fillers improve wrinkles and loss of facial volume, with biologic types made from animal, human, or bacterial sources (such as hyaluronic acid), while synthetic fillers include sub-stances such as paraffin, silicone, calcium hydroxyapatite, polymethylmethacrylate microspheres, polyacrylamide hydrogel, hydroxyethyl-ethyl methacrylate, and poly-l-lactic acid. Synthetic fillers can be classified as rapidly resorbable (<12 months), slowly resorbable (<24 months), or permanent. Imaging modalities such as US, CT, and MRI can help identify and analyze each type of facial aesthetic procedure or filler, as well as their possible complications such as foreign-body granuloma, noninflammatory nodule, late intermittent persistent edema, filler migration, infec-tion, or complications after removal of the buccal fat pad. (c) RSNA, 2023 center dot
  • article 0 Citação(ões) na Scopus
    Sleep bruxism and associated physiological events in children with obstructive sleep apnea: a polysomnographic study
    (2024) BONACINA, Carlos F.; SOSTER, Leticia M. S. F. A.; BUENO, Clarissa; DINIZ, Joaquina S.; BOZZINI, Maria F.; FRANCESCO, Renata C. Di; OLEGARIO, Isabel Cristina; LIRA, Adriana de Oliveira
    Study Objectives: The aim of this study was to evaluate the physiological events associated with sleep bruxism (Sleep Bruxism [SB]; presence of mandibular movement activity) and the control window (4 minutes prior to SB event, where no mandibular movement activity was detected) in a polysomnography study in children with mild sleep apnea. Methods: Polysomnography data from children aged 4 to 9 years old diagnosed with mild sleep apnea were analyzed by 2 trained examiners. The mandibular movement activity (bruxism event; SB) was classified into phasic and tonic. The control window was selected 4 minutes prior to the SB event. All physiological events were recorded in both bruxism and control windows, including sleep phase (N1, N2, N3, and rapid eye movement), arousal, leg movements, tachycardia, bradycardia, oxygen desaturation, and number of obstructive and central sleep apnea events. The moment in which those phenomena occurred when associated with SB was also analyzed (before/after). Data were analyzed using 95% confidence intervals (alpha = 5%). Results: A total of 661 mandibular movements were analyzed and classified as tonic (n = 372) or phasic (n = 289). The mean apnea-hypopnea index was 1.99 (SD = 1.27) events/h. The frequency of leg movements, microarousal, and tachycardia was increased in SB events when compared with the control window (P < .05). There was an increase in bradycardia frequency in the control window when compared with SB (in both tonic and phasic events). The frequency of obstructive and central apnea during SB was lower when compared with the other physiological phenomena. Conclusions: There is a difference in the physiological parameters evaluated in children with mild sleep apnea when comparing the 2 windows (SB and control). Sleep bruxism is associated with other physiological phenomena, such as leg movements, tachycardia, and microarousal. The use of a control window (where no mandibular activity was detected) was representative since it did not show activation of the sympathetic nervous system.
  • article 0 Citação(ões) na Scopus
    Physical rehabilitation in Brazilian pediatric intensive care units: a multicenter point prevalence study
    (2023) REDIVO, Juliana; KANNAN, Harini; SOUZA, Andreia Aparecida Freitas; COLLETI JUNIOR, José; KUDCHADKAR, Sapna Ravi; HORIGOSHI, Nelson Kazunobu; COSTA, Graziela de Araújo; CASTILHO, Taísa Roberta Ramos de; PERON, Paula Peres Domingues; SCARANTO, Walter Perez; MEDEIROS, Daniela Nasu Monteiro; MATSUMOTO, Toshio; ALMEIDA, Carlos Gustavo de; OLIVEIRA, Felipe Rezende Caino de; BRANDÃO, Marcelo Barciela; LIMA-SETTA, Fernanda; PRATA-BARBOSA, Arnaldo; XAVIER, Glaciele Nascimento; ANDRADE, Livia Barbosa de; AGUIAR, Agda Ultra de; COUTINHO, Marcos Paulo Galdino; CASTRO, Roberta Esteves Viera de; LANDY, Glazia André; BALANIUC, Suzana Lopes Bonfim; YAMAGUCHI, Ricardo Silveira
    ABSTRACT Objective: To determine the prevalence and factors associated with the physical rehabilitation of critically ill children in Brazilian pediatric intensive care units. Methods: A 2-day, cross-sectional, multicenter point prevalence study comprising 27 pediatric intensive care units (out of 738) was conducted in Brazil in April and June 2019. This Brazilian study was part of a large multinational study called Prevalence of Acute Rehabilitation for Kids in the PICU (PARK-PICU). The primary outcome was the prevalence of mobility provided by physical therapy or occupational therapy. Clinical data on patient mobility, potential mobility safety events, and mobilization barriers were prospectively collected in patients admitted for ≥ 72 hours. Results: Children under the age of 3 years comprised 68% of the patient population. The prevalence of therapist-provided mobility was 74%, or 277 out of the 375 patient-days. Out-of-bed mobility was most positively associated with family presence (adjusted odds ratios 3.31;95%CI 1.70 - 6.43) and most negatively associated with arterial lines (adjusted odds ratios 0.16; 95%CI 0.05 - 0.57). Barriers to mobilization were reported on 27% of patient-days, the most common being lack of physician order (n = 18). Potential safety events occurred in 3% of all mobilization events. Conclusion: Therapist-provided mobility in Brazilian pediatric intensive care units is frequent. Family presence was high and positively associated with out-of-bed mobility. The presence of physiotherapists 24 hours a day in Brazilian pediatric intensive care units may have a substantial impact on the mobilization of critically ill children.
  • article 0 Citação(ões) na Scopus
    Performance and labor conditions of physiotherapists in Brazilian intensive care units during the COVID-19 pandemic. What did we learn?
    (2023) RESENDE, Andressa Sabrina de Oliveira; BACCI, Suzi Laine Longo dos Santos; PAULA, Ítalo Ribeiro; PEREIRA, Leandro Alves; JOHNSTON, Cíntia; LUSZCZYNSKI, Valéria Cabral Neves; AZEVEDO, Vívian Mara Gonçalves de Oliveira
    ABSTRACT Objective: To describe the role of physiotherapists in assisting patients suspected to have or diagnosed with COVID-19 hospitalized in intensive care units in Brazil regarding technical training, working time, care practice, labor conditions and remuneration. Methods: An analytical cross-sectional survey was carried out through an electronic questionnaire distributed to physiotherapists who worked in the care of patients with COVID-19 in Brazilian intensive care units. Results: A total of 657 questionnaires were completed by physiotherapists from the five regions of the country, with 85.3% working in adult, 5.4% in neonatal, 5.3% in pediatric and 3.8% in mixed intensive care units (pediatric and neonatal). In intensive care units with a physiotherapists available 24 hours/day, physiotherapists worked more frequently (90.6%) in the assembly, titration, and monitoring of noninvasive ventilation (p = 0.001). Most intensive care units with 12-hour/day physiotherapists (25.8%) did not apply any protocol compared to intensive care units with 18-hour/day physiotherapy (9.9%) versus 24 hours/day (10.2%) (p = 0.032). Most of the respondents (51.0%) received remuneration 2 or 3 times the minimum wage, and only 25.1% received an additional payment for working with patients suspected to have or diagnosed with COVID-19; 85.7% of them did not experience a lack of personal protective equipment. Conclusion: Intensive care units with 24-hour/day physiotherapists had higher percentages of protocols and noninvasive ventilation for patients with COVID-19. The use of specific resources varied between the types of intensive care units and hospitals and in relation to the physiotherapists’ labor conditions. This study showed that most professionals had little experience in intensive care and low wages.
  • article 0 Citação(ões) na Scopus
    (2023) MAFFEI, Helga Verena L; VIDOLIN, Eliana; REIS, Joceara Neves dos; FREITAS, Marcia de; CABRAL, Beatriz Helena; TRIGO-ROCHA, Flavio
    ABSTRACT Background: Functional constipation and enuresis frequently coexist. Constipation treatment often results in resolution or improvement of the enuresis. However, besides the classical presentation, patients can present with occult constipation (OC) diagnosed in complementary evaluation; in addition, semi-occult constipation (SOC) can be detected by means of a detailed questionnaire. Objective: To quantify OC and SOC frequency in children with monosymptomatic or non monosymptomatic enuresis (MNE or NMNE). Methods: Otherwise healthy children/adolescents, with enuresis refractory to behavioral therapy and denying constipation after simple questions, answered a structured bowel habit questionnaire and were submitted to a plain abdominal radiological exam. Constipation was classified considering the Boston diagnostic criteria (to allow diagnosis at initial stages), and fecal loading in the X-ray quantified ≥10 by the Barr score. Children with constipation received a standardized treatment (except 26 “pilot” children). Results: Out of 81 children, 80 aged 9.34±2.07 years, 52.5% male, were diagnosed with constipation: 30 OC, 50 SOC; 63.75% had MNE, 36.25% NMNE (six NMNE without behavioral therapy). Demographic data and the Barr score were similar for OC and SOC, but SOC children experienced significantly more constipation complications (retentive fecal incontinence and/or recurrent abdominal pain). Not showing the Bristol Stool Scale (BSS) to 24 “pilot” children, or absence of constipation symptoms accompanying BSS predominantly type 3, in 13 children, did not significantly impact the detection of constipation by the Barr score. Children identifying BSS 3 or ≤2 had similar results. Twenty-eight children, with adequate follow-up after treatment, improved or recovered from constipation at 44 of their 52 follow-up visits. Conclusion: In patients with MNE or NMNE refractory to behavioral therapy, and who initially denied constipation after simple questions, a detailed questionnaire based on the Boston diagnostic criteria detected SOC in 61.7%, and the radiological Barr score revealed fecal loading (OC) in 37.0% of them.
  • article 7 Citação(ões) na Scopus
    Haemodynamic support for paediatric septic shock: a global perspective
    (2023) RANJIT, Suchitra; KISSOON, Niranjan; ARGENT, Andrew; INWALD, David; VENTURA, Andrea Maria Cordeiro; JABORINSKY, Roberto; SANKAR, Jhuma; SOUZA, Daniela Carla de; NATRAJ, Rajeswari; OLIVEIRA, Claudio Flauzino De; SAMRANSAMRUAJKIT, Rujipat; JAYASHREE, Muralidharan; SCHLAPBACH, Luregn J.
    Septic shock is a leading cause of hospitalisation, morbidity, and mortality for children worldwide. In 2020, the paediatric Surviving Sepsis Campaign (SSC) issued evidence-based recommendations for clinicians caring for children with septic shock and sepsis-associated organ dysfunction based on the evidence available at the time. There are now more trials from multiple settings, including low-income and middle-income countries (LMICs), addressing optimal fluid choice and amount, selection and timing of vasoactive infusions, and optimal monitoring and therapeutic endpoints. In response to developments in adult critical care to trial personalised haemodynamic management algorithms, it is timely to critically reassess the current state of applying SSC guidelines in LMIC settings. In this Viewpoint, we briefly outline the challenges to improve sepsis care in LMICs and then discuss three key concepts that are relevant to management of children with septic shock around the world, especially in LMICs. These concepts include uncertainties surrounding the early recognition of paediatric septic shock, choices for initial haemodynamic support, and titration of ongoing resuscitation to therapeutic endpoints. Specifically, given the evolving understanding of clinical phenotypes, we focus on the controversies surrounding the concepts of early fluid resuscitation and vasoactive agent use, including insights gained from experience in LMICs and high-income countries. We outline the key components of sepsis management that are both globally relevant and translatable to low-resource settings, with a view to open the conversation to the large variety of treatment pathways, especially in LMICs. We emphasise the role of simple and easily available monitoring tools to apply the SSC guidelines and to tailor individualised support to the patient's cardiovascular physiology.
  • article 1 Citação(ões) na Scopus
    Exploring the Brazilian pediatric palliative care network: a quantitative analysis of a survey data
    (2023) FERREIRA, Esther Angelica Luiz; VALETE, Cristina Ortiz Sobrinho; BARBOSA, Silvia Maria de Macedo; COSTA, Graziela de Araujo; MOLINARI, Poliana Cristina Carmona; IGLESIAS, Simone Brasil de Oliveira; CASTRO, Ana Cristina Pugliese de
    Objective: The aim of this study was to identify the characteristics of services in Brazil that compound the Brazilian Pediatric Palliative Care (PPC) Network. Methods: An online survey was conducted among representatives from PPC services. A total of 90 services from Brazil completed the online survey and answered a questionnaire about the services characterization, health professionals working in PPC, access to opioid prescription and education, and research in PPC.Results: In total, 80 services (88.9%) were created after 2010, 52 (57.9%) were in the southeast region, 56 (62.2%) were in public hospitals, 63 (70%) had up to 100 beds, and 57 (63.3%) were at the tertiary level. Notably, 88 (97.8%) had a physician in the team and 68 (75.5%) dedicated part-time to PPC. Also, 33 (36.7%) revealed concern with the care of health professionals and 36 (40%) reported difficulty or no access to opioid prescription. Research studies were reported to be conducted in 29 (32.2%) services.Conclusions: This mapping points out to a concentration of PPC services in the southeast region, with part-time professional dedication, and the need to improve professionals' care. Difficulty in opioid access was reported. It is necessary to extend PPC participation to other Brazilian regions, increase time dedicated to PPC, improve professionals' care and improve access to opioid prescription.
  • article 5 Citação(ões) na Scopus
    POLR1A variants underlie phenotypic heterogeneity in craniofacial, neural, and cardiac anomalies
    (2023) SMALLWOOD, Kelly; WATT, Kristin E. N.; IDE, Satoru; BALTRUNAITE, Kristina; BRUNSWICK, Chad; INSKEEP, Katherine; CAPANNARI, Corrine; ADAM, Margaret P.; BEGTRUP, Amber; BERTOLA, Debora R.; DEMMER, Laurie; DEMO, Erin; DEVINSKY, Orrin; GALLAGHER, Emily R.; SACOTO, Maria J. Guillen; JECH, Robert; KEREN, Boris; KUSSMANN, Jennifer; LADDA, Roger; LANSDON, Lisa A.; LUNKE, Sebastian; MARDY, Anne; MCWALTERS, Kirsty; PERSON, Richard; RAITI, Laura; SAITOH, Noriko; SAUNDERS, Carol J.; SCHNUR, Rhonda; SKORVANEK, Matej; SELL, Susan L.; SLAVOTINEK, Anne; SULLIVAN, Bonnie R.; STARK, Zornitza; SYMONDS, Joseph D.; WENGER, Tara; WEBER, Sacha; WHALEN, Sandra; WHITE, Susan M.; WINKELMANN, Juliane; ZECH, Michael; ZEIDLER, Shimriet; MAESHIMA, Kazuhiro; STOTTMANN, Rolf W.; TRAINOR, Paul A.; WEAVER, K. Nicole
    Heterozygous pathogenic variants in POLR1A, which encodes the largest subunit of RNA Polymerase I, were previously identified as the cause of acrofacial dysostosis, Cincinnati-type. The predominant phenotypes observed in the cohort of 3 individuals were craniofacial anomalies reminiscent of Treacher Collins syndrome. We subsequently identified 17 additional individuals with 12 unique heterozygous variants in POLR1A and observed numerous additional phenotypes including neurodevelopmental abnormalities and structural cardiac defects, in combination with highly prevalent craniofacial anomalies and variable limb defects. To understand the pathogenesis of this pleiotropy, we modeled an allelic series of POLR1A variants in vitro and in vivo. In vitro assessments demonstrate variable effects of indi-vidual pathogenic variants on ribosomal RNA synthesis and nucleolar morphology, which supports the possibility of variant-specific phenotypic effects in affected individuals. To further explore variant-specific effects in vivo, we used CRISPR-Cas9 gene editing to reca-pitulate two human variants in mice. Additionally, spatiotemporal requirements for Polr1a in developmental lineages contributing to congenital anomalies in affected individuals were examined via conditional mutagenesis in neural crest cells (face and heart), the second heart field (cardiac outflow tract and right ventricle), and forebrain precursors in mice. Consistent with its ubiquitous role in the essential function of ribosome biogenesis, we observed that loss of Polr1a in any of these lineages causes cell-autonomous apoptosis resulting in embryonic malformations. Altogether, our work greatly expands the phenotype of human POLR1A-related disorders and demonstrates variant-specific effects that provide insights into the underlying pathogenesis of ribosomopathies.
  • article 1 Citação(ões) na Scopus
    Small and large bowel anatomy is associated with enteral autonomy in infants with short bowel syndrome: A retrospective cohort study
    (2024) SANDY, Natascha S.; ROBERTS, Amin J.; WALES, Paul W.; TOMA, Ricardo K.; BELZA, Christina; DOGRA, Harween; EVANS, Helen M.; GATTINI, Daniela; HIND, Jonathan; MERCER, David; POVONDRA, Jill M.; TURNER, Justine; YAP, Jason; WONG, Theodoric; AVITZUR, Yaron
    Background: Achievement of enteral autonomy (EA) is the ultimate treatment goal in pediatric intestinal failure (IF). We aimed to assess predictors of EA in pediatric short bowel syndrome (SBS) and explore the impact of residual small bowel (SB) and large bowel (LB) length on EA.Methods: A retrospective cohort study was performed on infants aged <12 months (n = 367, six centers) with SBS referred between 2010 and 2015. The cohort was stratified based on the achievement of EA. Statistical testing was completed using t-test, chi-square, Cox proportional hazards regression model, and Kaplan-Meier analysis.Results: EA was achieved in 229 patients. In the multivariable analysis, the percentage of residual LB (hazard ratio [HR] = 1.02; 95% CI = 1.01-1.02) and SB (HR = 1.01; 95% CI = 1.01-1.02) length, presence of the ileocecal valve (HR = 2.02; 95% CI=1.41-2.88), and not coming from a high-volume transplantation center (HR = 2.42; 95% CI = 1.68-3.49) were positively associated with EA, whereas a negative association was seen with the presence of stoma at the time when shortest remnant was documented (HR = 0.72; 95% CI = 0.52-1.00). EA achievement was significantly different between the anatomical subgroups (log-rank test P < 0.001) with an EA rate of 80.4% in infants with >= 50% SB and LB (median time 209 days); 62.5% with >= 50% SB and <50% LB (397 days); 58.3% with <50% SB and >= 50% LB (1192 days), and 25.9% with <50% SB and LB. Necrotizing enterocolitis (NEC) was not associated with a better achievement of EA (NEC vs other etiologies: log-rank test P = 0.33).Conclusions: Overall, 62% of infants with IF secondary to SBS achieved EA over a mean time of follow-up of 2.3 years. A colon length of >50% can compensate for the loss of small bowel (<50%) and account for similar EA rates as those in children with residual SB > 50%.
  • article 0 Citação(ões) na Scopus
    Effect of manual hyperinflation with versus without positive end-expiratory pressure on dynamic compliance in pediatric patients following congenital heart surgery: A randomized controlled trial
    (2023) GUIMARAES, Jessica Camara; SILVA, Thalis Henrique da; ARAGON, Davi Casale; JOHNSTON, Cintia; GASTALDI, Ada Clarice; CARLOTTI, Ana P. C. P.
    Background: We aimed to compare the effect of manual hyperinflation with versus without positive end-expiratory pressure (PEEP) on dynamic compliance of the respiratory system in pediatric patients undergoing congenital heart surgery; to assess the safety of the technique in this population.Methods: This was a randomized controlled trial conducted at the pediatric intensive care unit (PICU) of a tertiary-care hospital. Patients admitted to the PICU following cardiac surgery and receiving postoperative mechanical ventilation were randomized to the experimental or control group. Patients in the experimental group (n = 14) underwent manual hyperinflation with a PEEP valve set at 5 cm H2O, once daily, during the first 48 hours after surgery. Patients allocated to the control group (n = 16) underwent manual hyperinflation without PEEP, at the same time points. Lung mechanics was assessed before (T0) and 5 minutes (T5) after manual hyperinflation. The primary endpoint was dynamic compliance. Secondary outcomes included oxygen saturation index, duration of mechanical ventilation, length of stay, 28-day mortality and safety.Results: Demographic and clinical characteristics were comparable in both groups. There was no significant difference in dynamic compliance between times in each group (Day 1: (mean) 0.78 vs 0.81 and 0.70 vs 0.77; Day 2: 0.85 vs 0.78 and 0.67 vs 0.68 mL/kg/cm H2O, in experimental and control groups, respectively; P > .05). Mean deltas of dynamic compliance were not significantly different between groups. The proportion of patients extubated <72 hours after surgery was similar in experimental and control groups (43% vs 50%, respectively; P = .73). Oxygen saturation index, length of stay, and 28-day mortality were not significantly different between groups. None of the patients had hemodynamic instability.Conclusions: Manual hyperinflation was safe and well tolerated in pediatric patients following surgery for congenital heart disease. No significant change in dynamic compliance of the respiratory system or in oxygenation was observed with the use of manual hyperinflation with or without PEEP in this population.
  • article 1 Citação(ões) na Scopus
    Malnutrition and Nutrition Support in Latin American PICUs: The Nutrition in PICU (NutriPIC) Study
    (2023) CAMPOS-MINO, Santiago; FIGUEIREDO-DELGADO, Artur; ZARATE, Patricia; ZAMBERLAN, Patricia; MUNOZ-BENAVIDES, Eliana; COSS-BU, Jorge A.
    OBJECTIVES: To characterize the nutritional status of children admitted to Latin American (LA) PICUs and to describe the adequacy of nutrition support in reference to contemporary international recommendations. DESIGN: The Nutrition in PICU (NutriPIC) study was a combined point-prevalence study of malnutrition carried out on 1 day in 2021 (Monday 8 November) and a retrospective cohort study of adequacy of nutritional support in the week preceding. SETTING: Four-one PICUs in 13 LA countries. PATIENTS: Patients already admitted to the PICU of 1 month to 18 years old on the study day were included in the point-prevalence study. For the retrospective arm, we included patients receiving nutritional support on the study day and with a PICU length of stay (LOS) greater than or equal to 72 hours. Exclusion criteria were being a neonate, conditions that precluded accurate anthropometric measurements, and PICU LOS greater than 14 days. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Of 316 patients screened, 5 did not meet age criteria. There were 191 of 311 patients who were included in the point-prevalence study and underwent anthropometric evaluation. Underweight and length for age less than -2 z scores were present in 42 of 88 children (47.7%) and 41 of 88 children (46.6%) less than 24 months old, and 14 of 103 (13.6%) and (23/103) 22.3% of 103 children greater than or equal to 24 months, respectively. Evidence of obesity (body mass index > 2 z score) was present in 7 of 88 children (5.7%) less than 24 months old and 13 of 103 children (12.6%) greater than or equal to 24 months. In the 115 of 311 patients meeting criteria for the retrospective arm, a total of 98 patients reported complete nutritional data. The 7-day median (interquartile range) adequacy for delivered versus recommended enteral energy and protein requirement was 114% (75, 154) and 99% (60, 133), respectively. CONCLUSIONS: The NutriPIC study found that in 2021 malnutrition was highly prevalent especially in PICU admissions of less than 24 months old. Retrospectively, the 7-day median nutritional support appears to meet both energy and protein requirements.
  • article 0 Citação(ões) na Scopus
    Real-world data of Brazilian adults with X-linked hypophosphatemia (XLH) treated with burosumab and comparison with other worldwide cohorts
    (2024) VAISBICH, Maria Helena; CILLO, Antonio Cesar Paulillo de; SILVA, Barbara Campolina C.; DALVA, Catarina Brasil; CARVALHO, Erico Higino de; ALMEIDA, Juliana M. C. M. de; MARQUES, Larissa L. M.; RIBEIRO, Marcia; SILVA, Mauro Borghi M. da; MEDEIROS, Paula Frassinetti V. de; MENDES, Pedro Henrique
    Background: Disease-related variants in PHEX cause XLH by an increase of fibroblast growth factor 23 (FGF23) circulating levels, resulting in hypophosphatemia and 1,25(OH)(2) vitamin D deficiency. XLH manifests in early life with rickets and persists in adulthood with osseous and extraosseous manifestations. Conventional therapy (oral phosphate and calcitriol) improves some symptoms, but evidence show that it is not completely effective, and it can lead to nephrocalcinosis (NC) and hyperparathyroidism (HPT). Burosumab (anti-FGF23 antibody) has shown to be effective and safety in the clinical trials. Methods: The current real-world collaborative study evaluated genetic, clinical and laboratory data of XLH Brazilian adult patients treated with burosumab. Results: Nineteen unrelated patients were studied. Patients reported pain, limb deformities and claudication, before burosumab initiation. 78% of them were previously treated with conventional therapy. The severity of the disease was moderate to severe (15 patients with score >5). At the baseline, 3 patients presented NC (16.7%) and 12 HPT (63%). After 16 +/- 8.4 months under burosumab, we observed a significant: increase in stature (p = 0.02), in serum phosphate from 1.90 +/- 0.43 to 2.67 +/- 0.52 mg/dL (p = 0.02); in TmP/GFR from 1.30 +/- 0.46 to 2.27 +/- 0.64 mg/dL (p = 0.0001), in 1,25 (OH)(2) D from 50.5 +/- 23.3 to 71.1 +/- 19.1 pg/mL (p = 0.03), and a decrease in iPTH from 86.8 +/- 37.4 pg/mL to 66.5 +/- 31.1 (p = 0.002). Nineteen variants were found (10 novel). HPT tended to develop in patients with truncated PHEX variants (p = 0.06). Conclusions: This study confirms the efficacy and safety of burosumab on XLH adult patients observed in clinical trials. Additionally, we observed a decrease in iPTH levels in patients with moderate to severe HPT at the baseline.