Thaís Costa Nascentes Queiroz

Nutritional evaluation of children with chronic cholestatic disease

OBJECTIVE To evaluate the nutritional status of children with persistent cholestasis and to compare the anthropometric indices between children with and without liver cirrhosis and children with and without jaundice. METHODS Children with persistent cholestasis, i.e. increased direct bilirrubin or changes in the canalicular enzyme gamma-glutamyl transferase (GGT), were included. The anthropometric measures were weight (W), height or length (H), arm circumference (AC), triceps skinfold thickness (TST), arm muscle circumference (AMC), and body mass index (BMI). RESULTS Ninety-one children with cholestasis, with current median age of 12 months, were evaluated. W/age (A) and H/A indices below -2 Z-scores were observed in 33% and 30.8% of patients, respectively. Concerning the W/H index and BMI, only 12% and 16% of patients, respectively, were below -2 Z-scores. Regarding AC, 43.8% of 89 evaluated patients had some depletion. Observing the TST, 64% of patients had depletion, and 71.1% of the 45 evaluated patients had some degree of depletion regarding the ACM index. CONCLUSION Evaluation using weight in patients with chronic liver diseases may overestimate the nutritional status due to visceromegaly, subclinical edema, or ascites. Indices that correlate weight and height, such as W/H and BMI, may also not show depletion because of the chronic condition in which there are depletion of both weight and height. TST, AC, and ACM are parameters that better estimate nutritional status and should be part of the management of patients with liver diseases and cholestasis.

PRIMARY SCLEROSING CHOLANGITIS IN CHILDREN AND ADOLESCENTS

BACKGROUND Primary sclerosing cholangitis is a rare disease, but its prevalence has been underestimated in children and adolescents due to broad variation in clinical presentation as well as diagnostic challenges in this life period. OBJECTIVE To evaluate children and adolescents with primary sclerosing cholangitis and to describe their clinical, laboratorial, histopathological, and cholangiography conditions. METHODS This is an observational descriptive research that took place from 2005 to 2016 and included all the patients seen in the Outpatient Unit for Pediatric Hepatology of Hospital das Clinicas of UFMG who had been diagnosed with primary sclerosing cholangitis before the age of 18. Diagnosis was established through clinical, laboratory, radiographic and/or histopathologic criteria. Other chronic liver diseases were excluded, as well as secondary causes of cholangitis. Data analysis used statistic resources in SPSS software. Variables were expressed as averages, standard deviation, absolute frequency, and percentage. RESULTS Twenty-one patients fulfilled criteria to be included in the research sample. Male patients predominated (3.2:1) and average age at diagnosis was 6.7±3.9 years. Five (23.8%) patients had associated inflammatory bowel disease, four had ulcerative colitis and one indeterminate colitis. Signs and symptoms vary and are usually discrete at presentation. The most frequent symptom was abdominal pain (47.6%) followed less frequently by jaundice (28.6%) and itching (14.3%). The reason for medical investigation was asymptomatic or oligosymptomatic enzyme alterations in 33.3% of patients. All patients presented increased hepatic enzymes: aminotransferases, gamma glutamyl transferase, and alkaline phosphatase. Twenty patients had alterations compatible to primary sclerosing cholangitis in their cholangiography exam; one patient had no alterations at magnetic resonance cholangiography, but presented histopathologic alterations that were compatible to small duct cholangitis. Hepatic fibrosis was present in 60% of 15 patients who were biopsied upon admission; cirrhosis being present in four (26.7%) patients. A total of 28.5% of patients had unfavorable outcomes, including two (9.5%) deaths and four (19%) transplants. CONCLUSION Primary sclerosing cholangitis is a rare disease in childhood and adolescence and its initial diagnosis may be delayed or overlooked due to asymptomatic or unspecific clinical manifestations. The association with inflammatory bowel disease is common. Prognosis may be unfavorable as the disease progresses and hepatic transplant is the definitive treatment.

Telemonitoring of Neonatal Intensive Care Units: Preliminary Experience in the State of Minas Gerais, Brazil

Objective This article describes the implementation and functioning of a telemonitoring center of neonatal intensive care units (NICUs) organized by the Telehealth Center of the Medical School of the Federal University of Minas Gerais who signed an agreement with the Minas Gerais State Department of Health (SES/MG, in Portuguese), Brazil. Study Design This is a descriptive retrospective study from December 2012 to June 2014 of a NICU telemonitoring project. Results Twelve Web conferences have been performed. Six thousand, eight hundred and thirty nine (6,839) online teleconsultations of pediatricians and nurses with experience in neonatology and intensive care have been performed, of which 174 were with subspecialists, with an average response time of 6 hours. Discussion The project has demonstrated to be satisfactory, with fast response in relation to the demands of subspecialists, promoting care, and management support to the NICU. The qualification via Web conferences prevents the displacement of the professionals to big centers for continuous medical education. Conclusion The telemonitoring of NICU can contribute in the process of management, regulation of beds, reduction of displacements and transferences, adhesion to best practices, care support, and qualification of the involved professionals.

Portal vein thrombosis after umbilical vein catheterization: review of the epidemiology, prophylaxis, diagnostic and treatment

The extrahepatic obstruction of the portal vein is one of the most important causes of upper gastrointestinal bleeding in children. Studies about its physiopathology reveals that umbilical vein catheterization during the neonatal period is one of the most relevant risk factor. There are few data on the incidence, prevalence, risk factors and the effect of treatment of portal vein thrombosis in the newborn. It was conducted a search in the database of the MEDLINE, PUBMED, Elsevier, Web of Science, Scielo e Lilacs and included articles in Portuguese and English. The incidence of portal vein thrombosis after umbilical vein catheterization varies from 1% to 43% depending on the population, the moment of the ultrasound and the design of the study. The main risk factors are sepsis and longtime of catheterization. It is still not clear if the treatment with anticoagulation, either heparin or tissue plasminogen activator, changes the prognosis. If chooses to treat, it should be done with heparin; the thrombolysis reserved for the cases where there is limb, organ, or life threatening thromboses. Spontaneous regression of neonatal portal vein thrombosis is common but about 3% of these patients progress to extrahepatic obstruction of the portal vein and portal hypertension.

Autoimmune liver disease in children and adolescents

The autoimmune liver diseases are a group of immunomediated disordes that targets the hepatocytes and bile ducts. Those diseases includes the autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC) and the association between these two conditions called overlap syndrome or autoimmune sclerosing cholangitis. AIH is characterized by elevated transaminase levels, autoantibodies and immunoglobulin G. According to the antibody identified, two types of AIH can be recognized: type I and type II. Positivity to antinuclear antibody and smooth muscle antibody defines type I AIH, which can affect both adults and children, while antibodies to liver-kidney microsome define type II, most common in younger children. Both types are greatly responsive to immunosuppressive therapy with corticoid and azathioprine. PSC, although, is a chronic cholestatic liver disease, of unknown etiology, characterized by progressive inflammation, fibrosis and stricture of intra and extra hepatic bile ducts. The diagnosis established by cholangiography, which revels areas of focal dilatations and stricture. Until this moment, there are no effective treatments beside the liver transplantation. Autoimmune liver disease in children and adolescents Eduardo Ramos Santos1, Eleonora Druve Tavares Fagundes2, Alexandre Rodrigues Ferreira2, Thaís Costa Nascentes Queiroz3, Caroline Caldeira Hosken4 Doenças hepáticas autoimunes em crianças e adolescentes DOI: 10.5935/2238-3182.20170029

Diagnosis and management of ascites secondary to cirrhosis in pediatrics

The most important cause of ascites in pediatric population is cirrhosis with portal hypertension. The presence of ascites is one of the evidences of advanced stages of liver diseases and poorer prognosis. The first sign of ascitis can be inappropriate weight gain and increased abdominal girth. Although, it can be asymptomatic at the beginning. Ultrasound is a sensitive imaging technique for the detection of ascites and abdominal paracentesis is a way to confirm the presence of ascetic fluid and detect bacterial peritonitis. The treatment of ascites aims, primarily, patient comfort. Small amounts of ascitic fluid that do not produce symptoms may require little or no treatment. Tense ascites causing respiratory compromise, should be treated promptly, and may need therapeutic paracentesis. Furosemide and Spironolactone are the most common diuretics prescribed for ascitis management. Spontaneous bacterial peritonitis (SBP) is a complication of ascites that commonly occurs in cirrotic patients with portal hypertention. It can be favored by small intestine bacterial overgrowth, damaged enteric motility and permeability. It is important to SBP be quickly diagnosed by paracentesis or culture, so antibiotic treatment can be promptly initiated. It presents with heterogeneous clinical features. Thus, it is recommended that diagnostic paracentesis be Diagnosis and management of ascites secondary to cirrhosis in pediatrics Thaís Costa Nascentes Queiroz1, José Ricardo Borém Lopes2, Bárbara Fonseca Gazzinelli2, Flávia Pípolo3, Ana Paula Pereira de Oliveira4, Eleonora Druve Tavares Fagundes5, Alexandre Rodrigues Ferreira6, Regiane Urbano7 Diagnóstico e manejo da ascite secundária à cirrose em pediatria DOI: 10.5935/2238-3182.20160058

Intestinal Inflamatory disease in childood

The incidence of inflammatory bowel disease in the Western countries has increased in recent years. Classically it is divided into Crohn’s Disease and Ulcerative Colitis. The most common clinical manifestations are abdominal pain, diarrhea, weight loss and blood in the stool. The intestinal inflammation reduces the absorption of nutrients, which increases susceptibility to micronutrient deficiency. extra-intestinal manifestations may be present. Primary immunodeficiency research in children diagnosed with IBD before two years of age should be performed. Laboratory tests should include screening of acute and/or chronic, evaluation of anemia and nutritional status. Endoscopic examinations and feces are needed, especially before the start of treatment. Outpatient follow-up and complementary tests should be judicious. Treatment is difficult because it is a chronic disease with phases of relapse and remission. The drugs have adverse effects sometimes severe. The most current view of IBD therapy is based on the modification of the natural history of the disease, with the goal of achieving mucosal healing, reducing complications of the disease and improve the patient’s quality of life. It is essential to monitor the linear growth, muscle development, skeletal and pubertal. The general pediatrician should be aware of the diagnosis, requesting additional examinations and evaluating the need for referral to the pediatric gastroenterologist.

Acute liver failure in children and adolescents

The Acute Liver Failure (ALF) is a rare but devastating condition that can lead to multiple organ failure and death as its natural course. Intensive therapy and liver transplantation have changed its natural history and raised survival rates. ALF usually presents in a previously healthy child who initiates with unspecific symptoms of variable duration and then evolve with jaundice, vomiting, hypoglycemia and seizures, when the clinical syndrome becomes evident. The etiologic diagnosis is important because some diseases have specific treatments. However in 50% of the cases a specific diagnosis is not estabilished. The criteria for diagnosing ALF is biochemical evidence of acute hepatic failure without signs of chronic liver disease associated with coagulopathy non responsive to vitamin K (INR>1,5 with encephalopathy or INR>2 regardless the presence of encephalopathy signs). The time between the initial symptoms and the development of encephalopathy must be less than 8 weeks. Recognizing and referring the patient for a reference center must be a priority. The need for liver transplantation should be periodically assessed. Intensive multidisciplinary care is essential for survival.

BILIARY ATRESIA: evaluation on two distinct periods at a reference pediatric service

CONTEXT Biliary atresia is a progressive, idiopathic, fibro-obliterative disease of the extrahepatic biliary tree that presents with biliary obstruction exclusively in the neonatal period. OBJECTIVES To assess the differences regarding age at referral, age at surgery, duration of propaedeutics and waiting time for surgery between two groups of infants in different periods. METHODS Retrospective study of infants diagnosed with biliary atresia on two periods: 1983-1993 and 1998-2011. RESULTS Biliary atresia was diagnosed in 129 infants, being 48 in casuistic I and 81 in casuistic II. The median age at admission was 94 and 60 days, respectively (P = 0.0001). On evaluating patients who had undergone portoenterostomy before 120 days of age, no difference was observed regarding the duration of propaedeutics or waiting time for surgery (P = 0.15), but difference was found when comparing the age at surgery (P = 0.002). Among those infants with no biliary flow and without liver transplantation or death after 18 post-operative months, the estimated probability of survival was 44.6% and 38.7% in casuistics I and II, respectively. In casuistic I, all infants who showed biliary flow were alive during the observation period and, in casuistic II, 80.3% were alive after 7 years of follow-up. CONCLUSIONS Even though patients were admitted and treated earlier, it is clear that surgery could be done sooner. Delay in referral and timely propaedeutics were the main contributors.

Yellow alert: icterus after two weeks of life is equal of medical evaluation

Neonatal cholestasis manifests clinically with jaundice, dark urine, fecal acholia or hipocholia. It is of great importance, since it requires urgent and precise propaedeutics to define diagnosis and treatment. Before every newborn whose jaundice persists beyond 14 days of life, it is necessary the dosage of bilirubin (total and fraction) and the color observation of feces and urine. Once confirmed cholestasis, it is required urgent transfer to a referral center. To investigate the cause of cholestasis, the use of systematic protocol is useful to accelerate the diagnosis. Biliary Atresia (BA), a major cause of cholestatic jaundice, requires surgical correction before 60 days of life. This procedure is successful in 70-80% of patients operated before this age.The late referral of patients with suspected BA is one of the main factors for the failure of surgical correction. Therefore, disclosure of the importance of analyzing the color of feces and the determination of bilirubin through campaigns such as yellow alert is required and must reach not only parents, but also health professionals.