Referral Hospital In Southern Brazil Research Articles

Are there regional variations in the presentation of childhood leukemia?

Introduction: Treatment of childhood acute lymphoblastic leukemia (ALL) is based on risk stratification. This study aimed to assess the agreement between risk group classifications in the different childhood ALL treatment protocols used in a referral hospital in southern Brazil. Methods: We retrospectively reviewed the medical records of patients aged 1 to 18 years with B-cell ALL treated at a hospital from January 2013 to April 2017. Agreement between risk classifications was assessed by the kappa coefficient. Results: Seventy-five patients were analyzed. There was poor agreement between risk stratification by GBTLI 2009 and BFM 95 protocols (kappa=0.22; p = 0.003) and by GBTLI 2009 and IC-BFM 2002 protocols (kappa=0.24; p = 0.002). Risk group distribution was 13.3% for low risk, 32.0% for intermediate risk, and 54.7% for high risk based on stratification by the GBTLI 2009 protocol, and 28.0% for low risk, 42.7% for intermediate risk, and 29.3% for high risk based on stratification by the IC-BFM 2002 protocol. Overall survival was 68.6%. Conclusion: This study provides numerous points to ponder about the treatment of leukemia in Brazil. The percentage of patients classified as high risk in our sample was higher than that reported in the international literature. This difference, however, had no impact on overall survival, which was shorter than that reported in the international literature.

Epidemiological characteristics and causes of death after Aneurysmal Subarachnoid Hemorrhage – SAH

This study analyzed the epidemiological characteristics and causes of deaths after SAH in patients admitted to a referral hospital in southern Brazil. A hospital database was used, comprising the years 2006-2018, composed of 148 patients. Cross-tabulations and Logistic Regression were performed to determine the odds of death of epidemiological characteristics. Ruptured aneurysms had a higher incidence in women, aged between 41 and 60 years. Headache is the predominant symptom in 95% patients. Hypertension (61%) and smoking (47%) are the prevalent risk factors. The age group with the highest frequency of deaths is 71 to 80 years old. The causes of death are: external ventricular shunt (34%), ischemic injury (9%), bleeding (7%), cerebral edema (8%), sepsis (28%) and pneumonia (24.3%). Knowing the risk factors and complications involved provide important insights that help in understanding their genesis, treatment and prevention.

Hospitalization perceived by children and adolescents undergoing cancer treatment.

To know the perception of children and adolescents undergoing cancer treatment about hospitalization. Qualitative research conducted with 13 children and adolescents hospitalized for cancer treatment at a referral hospital in southern Brazil. Semi-structured interviews mediated by the drawing technique were conducted from May to November 2018. Data was submitted to inductive thematic analysis based on the theoretical framework of Brazil's National Humanization Policy. Disturbances were detected in the professionals' communication with the hospitalized children and adolescents. The participants felt socially isolated and highlighted the importance of recreational activities during hospitalization. Infant cancer is complex and needs multidisciplinary care during the treatment, enabling the organization of healthy and welcoming spaces that favor humanization of care.

Association between dietary pattern and metabolic disorders in children and adolescents with urolithiasis

ObjectiveTo describe the dietary patterns and occurrence of metabolic disorders in children and adolescents with urolithiasis treatment at a referral hospital in southern Brazil in order to learn the features of urolithiasis in this population to better develop preventive actions. MethodsDescriptive study conducted between 2016 and 2017 in a tertiary care referral hospital. Fourty patients aged 2–19 years old with urolithiasis proven by imaging were included. Clinical and dietary data were obtained through interviews and medical records. For statistical analyses, the chi‐squared test was performed. Results40 individuals were analyzed. Mean age at diagnosis was 7.2±4 years. 25% were overweight or obese. 95% had metabolic disorders, hypocitraturia being the predominant type. Protein intake was adequate in all participants and carbohydrate intake, in 70% of them; 37.5% had lipid intake above recommended and 65% had low fiber intake. The mean daily sodium intake was 2.64g (±1.74), with 55% of participants ingesting more than the recommended amount. A total of 52.5% had low potassium intake, with a mean of 4.79g/day (±2.49). Calcium intake was adequate in 27.5%. No significant differences were identified in relation to mean daily consumption among participants with or without the various metabolic disorders. ConclusionPediatric urolithiasis is often accompanied by metabolic disorders; therefore, metabolic evaluation should be part of the diagnostic process and subsequent analysis of these patients’ dietary patterns, helping to optimize treatment and prevent recurrences and complications.

Influence of minimal residual disease by multiparametric flow cytometry at day 15 of induction in risk stratification of children with B-cell acute lymphoblastic leukemia treated at a referral hospital in southern Brazil.

BackgroundThe minimal residual disease (MRD) is the most important prognostic factor for acute lymphoblastic leukemia (ALL) in children. This study aimed to investigate the influence of detecting the MRD by the multiparametric flow cytometry (MFC) at day 15 (D15) of the induction on the analysis of the risk group classifications of the different childhood ALL treatment protocols used in a referral hospital in southern Brazil.MethodWe retrospectively reviewed the medical records of patients with B-cell ALL, aged 1 to 18 years, treated at a hospital from January 2013 to April 2017.Main resultsSeventy-five patients were analyzed. Regarding the MRD by the MFC at D15, the analyses showed statistical significance when the MRD was grouped into three categories, < 0.1%, 0.1–10%, and > 10%, with the following distribution: 30.7%, 52.0%, and 17.3%, respectively. There was a significant association between D15 MRD-MFC < 0.1% and the likelihood of dying or relapsing and between D15 MRD-MFC > 10% and the likelihood of dying or relapsing. The cumulative hazard ratio for the relapse of patients with D15 MRD-MFC < 0.1%, 0.1–10%, and > 10% was 19.2%, 59.8%, and 80.1%, respectively.ConclusionOur analysis suggests D15 MRD-MFC < 0.1% as a cut-off point for patients with more favorable outcomes and that the MRD at D15 in risk classifications is particularly useful for the stratification of patients with a more favorable prognosis.

Follow-Up of Women with Cervical Cytological Abnormalities: Progression and Regression Events

Section TitleAbnormalities in the cervix, when identified early by Pap smear, can be treated in the early stages or in the precursor stages of the neoplasia, which may increase the chances of regression of the lesion. The aim to verify the rate of cervical abnormalities and to evaluate the risk of progression or regression associated with age and cytological diagnosis. Methods:The study was conducted in a referral hospital in Southern Brazil, based on the results of pathology and cytopathology laboratory tests of uterine cervix. The historical cohort included patients with an abnormal cytology diagnosis in the period from January 2010 to December 2014, followed until July 2016. Results:A total of 42,389 cervical smears were analyzed, 4,427 of which were eligible for analysis of the evolution of cervical abnormalities. In progression and regression events analysis, we observed that patients with a cytological diagnosis of atypical glandular cells presented a higher risk of cervical abnormality progression (Hazard Ratio: 2.0 and 95% confidence intervals 1.36–3.48). We also observed that patients younger than 25 years old were more likely to regress the cervical lesions (Hazard Ratio:1.4 and 95% confidence intervals 1.20–1.74). Conclusions:The associations found between the events (progression and regression), age and cytological diagnosis, highlights the importance of cytological screening in populations at risk of precursor of cervical cancer lesions, especially in women older than 25 years.

Association between dietary pattern and metabolic disorders in children and adolescents with urolithiasis

ObjectiveTo describe the dietary patterns and occurrence of metabolic disorders in children and adolescents with urolithiasis treatment at a referral hospital in southern Brazil in order to learn the features of urolithiasis in this population to better develop preventive actions. MethodsDescriptive study conducted between 2016 and 2017 in a tertiary care referral hospital. Fourty patients aged 2–19 years old with urolithiasis proven by imaging were included. Clinical and dietary data were obtained through interviews and medical records. For statistical analyses, the chi-squared test was performed. Results40 individuals were analyzed. Mean age at diagnosis was 7.2±4 years. 25% were overweight or obese. 95% had metabolic disorders, hypocitraturia being the predominant type. Protein intake was adequate in all participants and carbohydrate intake, in 70% of them; 37.5% had lipid intake above recommended and 65% had low fiber intake. The mean daily sodium intake was 2.64g (±1.74), with 55% of participants ingesting more than the recommended amount. A total of 52.5% had low potassium intake, with a mean of 4.79g/day (±2.49). Calcium intake was adequate in 27.5%. No significant differences were identified in relation to mean daily consumption among participants with or without the various metabolic disorders. ConclusionPediatric urolithiasis is often accompanied by metabolic disorders; therefore, metabolic evaluation should be part of the diagnostic process and subsequent analysis of these patients’ dietary patterns, helping to optimize treatment and prevent recurrences and complications.

Prevalence of Peripheral Neuropathy and associated factors in HIV-infected patients

The progress on HIV infection treatment has allowed a longer survival for HIV-infected patients. However, chronic comorbidities are emerging. Peripheral Neuropathy (PN) represents one of the most prevalent neurologic disorders among these patients, and comprehensive studies may contribute to a reduction in the morbidity of this condition. This is a cross-sectional analytic study conducted in a tertiary referral hospital in southern Brazil. This study investigates the prevalence of PN among HIV-infected patients and associated demographic, clinical and laboratory variables. A number of 150 HIV-infected patients admitted between January and May 2016 were interviewed, submitted to physical and neurological examination, and data from their medical records were obtained. The prevalence of PN was 31.3%. It was increased among older patients (p=0.02), patients with higher CD4 lymphocytes levels (p=0.02), and smokers (OR=3.4; 95% CI 1.6–6.9; p<0.01). The research identified a high prevalence of PN in HIV-infected patients. Older age and higher CD4 levels have been associated with PN. To the best of our knowledge, this was one of the first studies reporting an association between tobacco use and PN among HIV-infected patients. Further studies are necessary to elucidate the pathological mechanisms linking PN and tobacco.

Pacientes com Príon: Medidas de Bloqueio Epidemiológico

Objetivo: Apresentar as medidas de bloqueio epidemiológico realizadas no atendimento hospitalar a pacientes com Doença de Creutzfeldt-Jacob. Metodologia: Série de casos de caráter retrospectivo com quatro pacientes internados em um hospital de referência no sul do Brasil, de junho de 2012 a junho de 2015. Resultados: Perfil epidemiológico: sexo feminino, faixa etária entre 56 e 65anos, apenas uma com comorbidade prévia, todos em cuidados paliativos, um óbito, uma alta e duas transferências para hospitais de origem. Medidas de bloqueio epidemiológico adotadas: registro de notificação compulsória da DCJ; precauções padrão, identificação e acondicionamento dos materiais por risco de transmissibilidade; desinfecção dos materiais precedendo a esterilização; e garantia de manuseio e local de descarte adequado dos resíduos. Conclusões: As medidas de controle epidemiológico e resoluções quanto ao manuseio desses pacientes são recentes. É importante que a equipe de saúde se aproprie da temática afim de prestar a assistência correta e de qualidade.

Characteristics of fetuses evaluated due to suspected anencephaly: a population-based cohort study in southern Brazil.

Anencephaly is considered to be the most common type of neural tube defect. Our aim was to assess the clinical and gestational features of a cohort of fetuses with suspected anencephaly. Population-based retrospective cohort study in a referral hospital in southern Brazil. The sample consisted of fetuses referred due to suspected anencephaly, to the Fetal Medicine Service of Hospital Materno Infantil Presidente Vargas, between January 2005 and September 2013. Clinical, radiological, pathological and survival data were gathered. Our sample was composed of 29 fetuses. The diagnosis of suspected anencephaly was made on average at 21.3 weeks of gestation. Seven fetuses had malformations that affected other organs, and these included oral clefts (n = 4) and congenital heart defects (n = 2). In 16 cases, there was termination of pregnancy (n = 12) or intrauterine death (n = 4). Regarding those who were born alive (n = 13), all of them died in the first week of life. After postnatal evaluation, the diagnosis of anencephaly was confirmed in 22 cases (75.9%). Other conditions included amniotic band disruption complex (6.9%), microhydranencephaly (6.9%), merocrania (3.4%) and holoprosencephaly (3.4%). Different conditions involving the cranial vault may be confused with anencephaly, as seen in our sample. However, these conditions also seem to have a poor prognosis. It seems that folic acid supplementation is not being properly performed.

Clinical, Virologic and Immunological Outcomes in a Cohort of Long-Term Non-Progressor HIV Infected Patients, Southern Brazil

Objectives: We evaluated the HIV-1 subtype diversity, clinical, genetic and epidemiological profiles of a cohort of long-term non-progressor (LTNPs) followed-up at a referral hospital in southern Brazil. Methods: This prospective study included patients who had more than eight years of HIV-1 diagnosis without antiretroviral therapy (ART). Clinical and epidemiological profiles of LTNPs were obtained from interviews and medical records. Periodic blood draws were taken to determine HIV-1 genetic variability and host genetic patterns. Results: The study included 22 LTNPs, corresponding to 1.57% of patients followed-up at the Infectious Diseases Division. The gender distribution was nearly homogeneous, median age was 45 years; 18% were elite controllers, 23% were viremic controllers and 59% were non-viremic controllers. Three out of 22 patients were heterozygous for the CCR5Δ32 genotype. In most study patients, receptor use was consistent with an R5 phenotype. HIV-1 genotyping showed subtype C in 50% (11/22) of patients, subtype B in 32% (7/22), and the recombinant forms BF and BC in 14% (3/22) and 4% (1/22), respectively. There was a significant association of subtype C with female patients, and LTNPs patients infected with subtype C had lower viral loads compared with those infected with subtype B. Conclusions: The HIV/AIDS epidemic in Brazil is complex, and there are variations in the subtype distribution. This is the first study of LTNPs in the southern region of Brazil, and the data obtained will help to characterize this group and aid in determining the probable mechanisms associated with delayed clinical progression.

Factors associated with hospital length of stay among cancer patients with febrile neutropenia.

PurposeThis study sought to evaluate factors associated with hospital length of stay in cancer patients with febrile neutropenia.MethodsA prospective cohort study was performed at a single tertiary referral hospital in southern Brazil from October 2009 to August 2011. All adult cancer patients with febrile neutropenia admitted to the hematology ward were evaluated. Stepwise random-effects negative binomial regression was performed to identify risk factors for prolonged length of hospital stay.ResultsIn total, 307 cases of febrile neutropenia were evaluated. The overall median length of hospital stay was 16 days (interquartile range 18 days). According to multiple negative binomial regression analysis, hematologic neoplasms (P = 0.003), high-dose chemotherapy regimens (P<0.001), duration of neutropenia (P<0.001), and bloodstream infection involving Gram-negative multi-drug-resistant bacteria (P = 0.003) were positively associated with prolonged hospital length of stay in patients with febrile neutropenia. The condition index showed no evidence of multi-collinearity effect among the independent variables.ConclusionsHematologic neoplasms, high-dose chemotherapy regimens, prolonged periods of neutropenia, and bloodstream infection with Gram-negative multi-drug-resistant bacteria are predictors of prolonged length hospital of stay among adult cancer patients with febrile neutropenia.

45,X/46,XY mosaicism: report on 14 patients from a Brazilian hospital. A retrospective study

CONTEXT AND OBJECTIVE: 45,X/46,XY mosaicism, or mixed gonadal dysgenesis, is considered to be a rare disorder of sex development. The aim of our study was to investigate the clinical and cytogenetic characteristics of patients with this mosaicism.DESIGN AND SETTING: A retrospective study in a referral hospital in southern Brazil.METHODS: Our sample consisted of patients diagnosed at the clinical genetics service of a referral hospital in southern Brazil, from 1975 to 2012. Clinical and cytogenetic data were collected from the medical records.RESULTS: Fourteen patients were included in the sample, with ages at the first evaluation ranging from 2 days to 38 years. Nine of them had female sex of rearing and five, male. Regarding the external genitalia, most were ambiguous (n = 10). One patient presented male phenotype and was treated for a history of azoospermia, while three patients presented female phenotype, of whom two had findings of Turner syndrome and one presented secondary amenorrhea alone. Some findings of Turner syndrome were observed even among patients with ambiguous genitalia. None presented gonadal malignancy. One patient underwent surgical correction for genital ambiguity and subsequent exchange of sex of rearing. Regarding cytogenetics, we did not observe any direct correlation between percentages of cell lines and phenotype.CONCLUSIONS: 45,X/46,XY mosaicism can present with a wide variety of phenotypes resulting from the involvement of different aspects of the individual. All these observations have important implications for early recognition of these patients and their appropriate management.

Wilms tumor: Experience of a hospital in southern Brazil

Wilms tumor (WT) is the most common renal malignancy of childhood. The aim of this study was to verify the epidemiological profile and prognosis of a sample of patients from Brazil and compare them to similar data from other Latin American studies. The sample consisted of consecutive patients diagnosed with WT in an oncohematology service of a referral hospital in Southern Brazil, between 1989 and 2009. Clinical, radiological, pathological and survival data were collected from the medical records. Analysis was done using Excel and SPSS version 18.0. The significance level was set at P < 0.05. The final sample consisted of 45 patients. The male/female ratio was 1.25:1. Mean age at diagnosis was 43.9 months and all patients were of European descent. Thirty-three patients (73.3%) had both signs/symptoms of abdominal mass and hypertension. Malformation was observed in nine patients (20%) and there was one case of Fanconi's anemia (2.2%). Three patients had bilateral disease (6.7%). The majority of patients had stage III and IV (62.2%). Patients with malformation had an earlier age at diagnosis (P = 0.018) and a higher prevalence of bilateral disease (P = 0.044). Overall survival was 75%. Age at diagnosis was the only significant independent predictor associated with death. Death is closely related to late diagnosis in WT. Oncologic services should also be concerned about morbidity caused by therapeutic options in cases of late diagnosis, and the consequences for quality of life.

Cohort Study of the Impact of Time to Antibiotic Administration on Mortality in Patients with Febrile Neutropenia

The time to antibiotic administration (TTA) has been proposed as a quality-of-care measure in febrile neutropenia (FN); however, few data regarding the impact of the TTA on the mortality of adult cancer patients with FN are available. The objective of this study was to determine whether the TTA is a predictor of mortality in adult cancer patients with FN. A prospective cohort study of all consecutive cases of FN, evaluated from October 2009 to August 2011, at a single tertiary referral hospital in southern Brazil was performed. The TTA was assessed as a predictive factor for mortality within 28 days of FN onset using the Cox proportional hazards model. Kaplan-Meier curves were used for an assessment of the mortality rates according to different TTAs; the log-rank test was used for between-group comparisons. In total, 307 cases of FN (169 subjects) were evaluated. During the study period, there were 29 deaths. In a Cox regression analysis, the TTA was independently associated with mortality within 28 days (hazard ratio [HR], 1.18; 95% confidence interval [CI], 1.10 to 1.26); each increase of 1 h in the TTA raised the risk of mortality within 28 days by 18%. Patients with FN episodes with a TTA of ≤ 30 min had lower 28-day mortality rates than those with a TTA of between 31 min and 60 min (3.0% versus 18.1%; log-rank P = 0.0002). Early antibiotic administration was associated with higher survival rates in the context of FN. Efforts should be made to ensure that FN patients receive effective antibiotic therapy as soon as possible. A target of 30 min to the TTA should be adopted for cancer patients with FN.

Screening for 22q11 deletion syndrome among patients with congenital heart defects

Our aim was to evaluate the effectiveness of different screening methods for 22q11DS in patients with CHD. Our study evaluated a consecutive sample of patients with CHD hospitalized for the first time in a pediatric and cardiac intensive care unit of a referral hospital in southern Brazil. All of them underwent the examination through fluorescent in situ hybridization for 22q11DS. These patients were part of the study by Rosa et al.

Gestational, perinatal and family findings of patients with Patau syndrome

OBJECTIVE:To describe gestational, perinatal and family findings of patients with Patau syndrome (PS). METHODS: The study enrolled patients with PS consecutively evaluated during 38 years in a Clinical Genetics Service of a pediatric referral hospital in Southern Brazil. The clinical data and the results of cytogenetic analysis were collected from the medical records. For statistical analysis, the two-tailed Fisher's exact test and the chi-square test with Yates' correction were used, being significant p<0.05. RESULTS: The sample was composed of 27 patients, 63% were male, with a median age of nine days at the first evaluation. Full trisomy of chromosome 13 was the main cytogenetic finding (74%). Only six patients were submitted to obstetric ultrasound and none had prenatal diagnosis of PS. The patients' demographic characteristics, compared to born alive infants in the same Brazilian state showed a higher frequency of: mothers with 35 years old or more (37.5%); multiparous mothers (92.6%); vaginal delivery (77%); preterm birth (34.6%); birth weight <2500g (33.3%), and Apgar scores <7 in the 1st (75%) and in the 5th minute (42.9%). About half of them (53%) died during the first month of life. CONCLUSIONS: The understanding of the PS patients' gestational, perinatal and family findings has important implications, especially on the decision about the actions to be taken in relation to the management of these patients.

Trisomy 18 and Neural Tube Defects

Trisomy 18 or Edwards syndrome is a chromosomal abnormality characterized by a broad clinical picture and a limited survival. More than 130 different abnormalities have been described in these patients-among them are neural tube defects. We verified the frequency and types of major neural tube defects observed among patients with trisomy 18. Our sample consisted of consecutive patients evaluated by a clinical genetics service of a referral hospital in southern Brazil between 1975 and 2008. Fisher's exact test (two-tailed) and chi-square test with Yates' correction were used to compare frequencies (P < 0.05 values were considered as significant). During the period of evaluation, we identified 50 patients with trisomy 18; 33 (66%) were female and age at the first evaluation ranged from 1 day to 16 years (median 14 days). One cell line with full trisomy 18 was the predominant cytogenetic finding (90%). Three patients (6%) had major neural tube defects, all females. These were two patients (4%) with encephaloceles and one (2%) with myelomeningocele. This last patient undergo to correction surgery on her first day of life. Our data, in accordance with the literature, support the idea that the presence of neural tube defects among patients with trisomy 18 is not coincidental (i.e., these defects are actually part of the spectrum of abnormalities presented in trisomy 18). Thus, the diagnosis of trisomy 18 should be considered in children with major neural tube defects, especially in the presence of other abnormalities or dysmorphisms.

Chromosomal Abnormalities in Patients with Congenital Heart Disease

BackgroundChromosomal abnormalities (CAs) are an important cause of congenital heart disease (CHD).ObjectiveDetermine the frequency, types and clinical characteristics of CAs identified in a sample of prospective and consecutive patients with CHD.MethodOur sample consisted of patients with CHD evaluated during their first hospitalization in a cardiac intensive care unit of a pediatric referral hospital in Southern Brazil. All patients underwent clinical and cytogenetic assessment through high-resolution karyotype. CHDs were classified according to Botto et al. Chi-square, Fisher exact test and odds ratio were used in the statistical analysis (p < 0.05).ResultsOur sample consisted of 298 patients, 53.4% males, with age ranging from 1 day to 14 years. CAs were observed in 50 patients (16.8%), and 49 of them were syndromic. As for the CAs, 44 (88%) were numeric (40 patients with +21, 2 with +18, 1 with triple X and one with 45,X) and 6 (12%) structural [2 patients with der(14,21), +21, 1 with i(21q), 1 with dup(17p), 1 with del(6p) and 1 with add(18p)]. The group of CHDs more often associated with CAs was atrioventricular septal defect.ConclusionsCAs detected through karyotyping are frequent in patients with CHD. Thus, professionals, especially those working in Pediatric Cardiology Services, must be aware of the implications that performing the karyotype can bring to the diagnosis, treatment and prognosis and for genetic counseling of patients and families.

Trisomy 18: Experience of a reference hospital from the south of Brazil

Trisomy 18 is a chromosomal syndrome characterized by a broad clinical picture, as well as a very reserved prognosis. The aim of our study was to verify the clinical characteristics and survival of patients diagnosed in a referral hospital in southern Brazil. Our sample consisted of 31 patients, 22 were female (71%), ages ranging from 1 to 1,395 days (median 14 days). The majority had a single cell lineage with full trisomy of chromosome 18 (94%). Concerning pregnancy complications, pre-eclampsia was the main abnormality described (17%). Fetal ultrasound was performed in 23 cases, and the most frequent abnormalities were polyhydramnios (41%) and intrauterine growth retardation (27%). There were no reports of prenatal identification of the syndrome. Most patients were born by cesarean due to pregnancy and fetal complications and about half of the cases were premature. Congenital heart defects represented the main major malformation observed (94%). Thirty patients (97%) progressed to death (survival ranged from 2 to 780 days, and 87% died within the first 6 months of life). Trisomy 18 is a serious chromosomal disorder with limited survival. Abnormalities of pregnancy appear to be frequent, which can lead to complications for both fetus and mother. The prenatal identification of these patients in our country is still inadequate, resulting in important implications for genetic counseling and management of these patients and their families. And this makes the possibility of interruption of pregnancy, regardless of ethical factors involved, an unlikely option.