Wilson's disease: an analysis of 28 Brazilian children

OBJECTIVES: Clinical-laboratory and evolutionary analysis of twenty-eight patients with Wilson's disease. METHODS: Twenty-eight children (twelve females and sixteen males) with Wilson's disease were evaluated retrospectively between 1987 and 2009, with a follow-up of 72 months (1 -240 months). The clinical, laboratory, and histologic features at diagnosis were recorded at the end of the study. RESULTS: The median age at diagnosis was 11 years (2 -18 years). Twelve patients were asymptomatic, seven had hepatitis symptoms, five had raised aminotransferase levels, three had hepatomegaly associated with neurological disorders, one had fulminant hepatitis with hemolytic anemia, and six patients presented with a Kayser-Fleischer ring. A histological analysis revealed that six children had chronic hepatitis, seven had cirrhosis, two had steatosis, one had portal fibrosis, and one had massive necrosis. The treatment consisted of D-penicillamine associated with pyridoxine for 26 patients. Adverse effects were observed in the other two patients: one presented with uncontrollable vomiting and the other demonstrated elastosis perforans serpiginosa. At the end of the study, all 26 treated patients were asymptomatic. Twenty-four of the patients were treated with D-penicillamine and pyridoxine, and two were treated with trientine and zinc sulfate. A liver transplant was performed in one patient with fulminant hepatitis, but the final patient died 48 hours after admission to the intensive care unit. CONCLUSIONS: Family screenings associated with early treatment are important in preventing Wilson's disease symptoms and potentially fatal disease progression. The study suggests that Wilson's disease must be ruled out in children older than two years presenting with abnormal levels of hepatic enzymes because of the heterogeneity of symptoms and the encouraging treatment results obtained so far.

Weight and neuro-psycho-motor development in children discharged from neonatal intensive care unit

Background: Low birth weight affects child growth and development, requiring the intensive use of health services. There are conversely proportional associations between prematurity and academic performance around the world. In this study we evaluated factors involved in weight and neuropsychomotor profile in one and two years old discharged from Intensive Care Units (ICU). Methods/Design: We investigated 203 children from the ICU who were followed for 24 +/- 4 months. The research was conducted by collecting data from medical records of patients in a Follow-up program. We investigated the following variables: inadequate weight at one year old; inadequate weight at two years old and a severe neurological disorder at two years old. Results: We observed increase of almost 20% in the proportion of children which weighted between the 10th and 90th percentiles and decrease of around 40% of children below the 15th percentile, from one to two years old. In almost 60% of the cases neuropsychomotor development was normal at 2 years old, less than 15% of children presented abnormal development. Variables that remained influential for clinical outcome at 1 and 2 years old were related to birth weight and gestational age, except for hypoglycemia. Neurological examination was the most influential variable for severe neurological disturbance. Conclusion: Hypoglycemia was considered a new fact to explain inadequate weight. The results, new in Brazil and difficult in terms of comparison, could be used to identify risk factors and for a better approach of newborn discharged from ICUs.

Incidence of gastric extubation of users in a home care program of a university hospital

This quantitative, prospective study, aimed to characterize the profile of users and caregivers and to measure the incidence of gastric extubation, identifying the type and the reasons for the extubation of these users in a Home Care Program of a university hospital. The population consisted of 37 subjects and the date were collected from April to August 2010. For the analysis, descriptive statistics, test of significance of 5% and calculation of indicators were adopted. It was found that 51.4% of the users were female, 67.5% in the age group >= 60 years and 67.6% presented neurological diseases. Regarding the caregivers 89.2% were female and their mean age was 50.6 years. The incidence of extubation, considering 100 days of intubation, corresponded to 1.08, with 0.26 planned and 0.82 unplanned (p=0.009). These results allowed the rates to be calculated of the extubation of patients with gastric intubation for nutritional support in domicile care, providing support in establishing care and management goals for the continuous improvement of quality.

Laparoscopic Adrenalectomy in Children for Neuroblastoma: Report of Case Series

Background: Neuroblastoma is one of the most common solid tumors in the pediatric population and the adrenal gland is the main abdominal site of this tumor. The laparoscopic approach has become the standard of care for most benign adrenal tumors in adults, but the role of laparoscopic adrenalectomy in children for malignant tumor is still a point of controversy. However, there is a growing experience with laparoscopic neuroblastoma resection of small lesions and the use of minimally invasive techniques for the initial management of infiltrative neuroblastoma in the last years. The aim of this study is to describe our initial experience with laparoscopic adrenalectomy for neuroblastoma in children, based on surgical outcomes. Methods: A retrospective review of 7 laparoscopic adrenalectomies performed in a single institution between October 2008 and October 2009. We focused our analysis on early surgical outcomes. Results: The mean tumoral size was 2.8 +/- 0.9 cm, the average surgical time was 38.6 +/- 65.5 minutes, and the mean hospital stay was 2.9 +/- 1.6 days. One stage IV patient was submitted to conversion due to bleeding and needed blood transfusion. There were no late complications or deaths and the mean follow-up time was 18.8 +/- 6.1 months. Conclusions: The laparoscopic approach for adrenal neuroblastoma resection is feasible in children with good outcomes, but should be reserved to patients with small, well-circumscribed adrenal lesions, without invasive or infiltrative disease.

Qualifying instrument for evaluation of food and nutritional care in hospital

Establishing criteria for hospital nutrition care ensures that quality care is delivered to patients. The responsibility of the Hospital Food and Nutrition Service (HFNS) is not always well defined, despite efforts to establish guidelines for patient clinical nutrition practice. This study describes the elaboration of an Instrument for Evaluation of Food and Nutritional Care (IEFNC) aimed at directing the actions of the Hospital Food and Nutrition Service. This instrument was qualified by means of a comparative analysis of the categories related to hospital food and nutritional care, published in the literature. Elaboration of the IEFNC comprised the following stages: (a) a survey of databases and documents for selection of the categories to be used in nutrition care evaluation, (b) a study of the institutional procedures for nutrition practice at two Brazilian hospitals, in order to provide a description of the sequence of actions that should be taken by the HFNS as well as other services participating in nutrition care, (c) design of the IEFNC based on the categories published in the literature, adapted to the sequence of actions observed in the routines of the hospitals under study, (d) application of the questionnaire at two different hospitals that was mentioned in the item (b), in order to assess the time spent on its application, the difficulties in phrasing the questions, and the coverage of the instrument, and (e) finalization of the instrument. The IEFNC consists of 50 open and closed questions on two areas of food and nutritional care in hospital: inpatient nutritional care and food service quality. It deals with the characterization and structure of hospitals and their HFNS, the actions concerning the patients' nutritional evaluation and monitoring, the meal production system, and the hospital diets. "This questionnaire is a tool that can be seen as a portrait of the structure and characteristics of the HFNS and its performance in clinical and meal management dietitian activities." (Nutr Hosp. 2012;27:1170-1177) DOI:10.3305/nh.2012.27.4.5868

Failure to reduce C-reactive protein levels more than 25% in the last 24 hours before intensive care unit discharge predicts higher in-hospital mortality: A cohort study

Purpose: To discharge a patient from the intensive care unit (ICU) is a complex decision-making process because in-hospital mortality after critical illness may be as high as up to 27%. Static C-reactive protein (CRP) values have been previously evaluated as a predictor of post-ICU mortality with conflicting results. Therefore, we evaluated the CRP ratio in the last 24 hours before ICU discharge as a predictor of in-hospital outcomes. Methods: A retrospective cohort study was performed in 409 patients from a 6-bed ICU of a university hospital. Data were prospectively collected during a 4-year period. Only patients discharged alive from the ICU with at least 72 hours of ICU length of stay were evaluated. Results: In-hospital mortality was 18.3% (75/409). Patients with reduction less than 25% in CRP concentrations at 24 hours as compared with 48 hours before ICU discharge had a worse prognosis, with increased mortality (23% vs 11%, P = .002) and post-ICU length of stay (26 [7-43] vs 11 [5-27] days, P = .036). Moreover, among hospital survivors (n = 334), patients with CRP reduction less than 25% were discharged later (hazard ratio, 0.750; 95% confidence interval, 0.602-0.935; P = .011). Conclusions: In this large cohort of critically ill patients, failure to reduce CRP values more than 25% in the last 24 hours of ICU stay is a strong predictor of worse in-hospital outcomes. (C) 2012 Elsevier Inc. All rights reserved.

Human bocavirus infection diagnosed serologically among children admitted to hospital with community-acquired pneumonia in a tropical region

Human bocavirus (HBoV) is a human virus associated with respiratory disease in children. Limited information is available on acute infection with HBoV among children admitted to hospital with community-acquired pneumonia in tropical regions and the current diagnosis is inadequate. The aims were to diagnose and describe acute HBoV infections among children hospitalized for community-acquired pneumonia. In Salvador, Brazil, 277 children with community-acquired pneumonia were prospectively enrolled. Paired serum samples were tested by IgG, IgM, and IgG-avidity enzyme immunoassays (EIAs) using recombinant HBoV VP2. HBoV DNA was detected in nasopharyngeal aspirates and serum by a quantitative polymerase-chain reaction (PCR). HBoV DNA was detected in nasopharyngeal aspirates of 62/268 (23%) children and 156/273 (57%) were seropositive. Acute primary HBoV infection was reliably diagnosed (bearing at least two acute markers: Positive IgM, a fourfold increase/conversion of IgG, low IgG avidity or viremia) in 21 (8%) of 273 patients, 90% of 20 had HBoV DNA in nasopharyngeal aspirates, 83% with a high DNA load. The median age of infection with HBoV was 16 months, range 5-36.Community-acquired pneumonia was confirmed radiographically in 85% of 20 patients with acute HBoV infection diagnosed serologically. HBoV DNA was found in nasopharyngeal aspirates of 42/246(17%) children without an acute primary HBoV infection and available nasopharyngeal aspirate. Four children with HBoV secondary immune responses were detected, lacking both IgM and viremia. HBoV infection was diagnosed accurately in children aged 5-36 months with community-acquired pneumonia confirmed radiographically. PCR of nasopharyngeal aspirates is not a reliable marker of acute HBoV infection. J. Med. Virol. 84:253-258, 2012. (C) 2011 Wiley Periodicals, Inc.

Catch-up growth in children after repair of Tetralogy of Fallot

Purpose: To evaluate the growth of children after repair of Tetralogy of Fallot, as well as the influence of residual lesions and socio-economic status. Methods: A total of 17 children, including 10 boys with a median age of 16 months at surgery, were enrolled in a retrospective cohort, in a tertiary care university hospital. Anthropometric (as z-scores), clinical, nutritional, and social data were collected. Results: Weight-for-age and weight-for-height z-scores decreased pre-operatively and recovered post-operatively in almost all patients, most markedly weight for age. Weight-for-height z-scores improved, but were still lower than birth values in the long term. Long-term height-for-age z-scores were higher than those at birth, surgery, and 3 months post-operatively. Most patients showed catch-up growth for height for age (70%), weight for age (82%), and weight for height (70%). Post-operative residual lesions (76%) influenced weight-for-age z-scores. Despite the fact that most patients (70%) were from low-income families, energy intake was above the estimated requirement for age and gender in all but one patient. There was no influence of socio-economic status on pre- and post-operative growth. Bone age was delayed and long-term-predicted height was within mid-parental height limits in 16 children (93%). Conclusion: Children submitted to Tetralogy of Fallot repair had pre-operative acute growth restriction and showed post-operative catch-up growth for weight and height. Acute growth restriction could still be present in the long term.

Risk factors associated with death in Brazilian children with severe dengue: a case-control study

Objective: The purpose of this case-control study was to evaluate risk factors associated with death in children with severe dengue. Methods: The clinical condition of hospitalized patients with severe dengue who died (cases, n = 18) was compared with that of hospitalized patients with severe dengue who survived (controls, n = 77). The inclusion criteria for this study were age under 13 years; hospital admission in São Luis, northeastern Brazil; and laboratory-confirmed diagnosis of dengue. Results: Severe bleeding (hemoptysis), a defining criterion for dengue severity, was the factor most strongly associated with death in our study. We also found that epistaxis and persistent vomiting, both included as warning signs in the World Health Organization (WHO) classification of dengue, were strongly associated with death. No significant association was observed between any of the laboratory findings and death. Conclusions: The finding that epistaxis and persistent vomiting were also associated with death in children with severe dengue was unexpected and deserves to be explored in future studies. Because intensive care units are often limited in resource-poor settings, any information that can help to distinguish patients with severe dengue with a higher risk to progress to death may be crucial.