CLINICAL, LABORATORIAL AND EVOLUTIONARY ASPECTS OF PEDIATRIC PATIENTS WITH LIVER DISEASE DUE TO ALPHA 1-ANTITRYPSIN DEFICIENCY.

BACKGROUND: Alpha 1-antitrypsin deficiency (AATD) is a hereditary codominant autosomal disease. This liver disease ranges from asymptomatic cases to terminal illness, which makes early recognition and diagnosis challenging. It is the main cause of pediatric liver transplantation after biliary atresia. OBJECTIVE: To describe the clinical characteristics, as well as those of histologic and laboratory tests, phenotypic and/or genetic evaluation and evolution of a cohort of pediatric patients with AATD. METHODS: This is a retrospective observational study of 39 patients with confirmed or probable AATD (without phenotyping or genotyping, but with suggestive clinical features, low serum alpha 1-antitrypsin (AAT) level and liver biopsy with PAS granules, resistant diastasis). Clinical, laboratory and histological varia-bles, presence of portal hypertension (PH) and survival with native liver have been analyzed. RESULTS: A total of 66.7% of 39 patients were male (26/39). The initial manifestation was cholestatic jaundice in 79.5% (31/39). Liver transplantation was performed in 28.2% (11/39) of patients. Diagnosis occurred at an average of 3.1 years old and liver transplantation at 4.1 years of age. 89.2% (25/28) of the patients with confirmed AATD were PI*ZZ or ZZ. The average AAT value on admission for PI*ZZ or ZZ patients was 41.6 mg/dL. All transplanted patients with phenotyping or genotyping were PI*ZZ (or ZZ). Those who were jaundiced on admission were earlier referred to the specialized service and had higher levels of GGT and platelets on admission. There was no significant difference in the survival curve when comparing cholestatic jaundiced to non-cholestatic jaundiced patients on admission. Comparing patients who did or did not progress to PH, higher levels of AST and APRI score at diagnosis (P=0.011 and P=0.026, respectively) were observed and in the survival curves patients with PH showed impairment, with 20.2% survival with native liver in 15 years. CONCLUSION: Jaundice is an important clinical sign that motivates referral to a specialist, but it does not seem to compromise survival with native liver. Patients progressing to PH had higher AST, APRi score on admission and significantly impaired survival with native liver. It is important to pay attention to these signs in the follow-up of patients with AATD.

Aspectos clínicos, laboratoriais e evolutivos de pacientes pediátricos com doença hepática por deficiência de alfa 1-antitripsina / Clinical, laboratorial and evolutionary aspects of pediatric patients with liver disease due to alpha 1-antitrypsin deficiency

ABSTRACT Background: Alpha 1-antitrypsin deficiency (AATD) is a hereditary codominant autosomal disease. This liver disease ranges from asymptomatic cases to terminal illness, which makes early recognition and diagnosis challenging. It is the main cause of pediatric liver transplantation after biliary atresia. Objective: To describe the clinical characteristics, as well as those of histologic and laboratory tests, phenotypic and/or genetic evaluation and evolution of a cohort of pediatric patients with AATD. Methods: This is a retrospective observational study of 39 patients with confirmed or probable AATD (without phenotyping or genotyping, but with suggestive clinical features, low serum alpha 1-antitrypsin (AAT) level and liver biopsy with PAS granules, resistant diastasis). Clinical, laboratory and histological varia­bles, presence of portal hypertension (PH) and survival with native liver have been analyzed. Results: A total of 66.7% of 39 patients were male (26/39). The initial manifestation was cholestatic jaundice in 79.5% (31/39). Liver transplantation was performed in 28.2% (11/39) of patients. Diagnosis occurred at an average of 3.1 years old and liver transplantation at 4.1 years of age. 89.2% (25/28) of the patients with confirmed AATD were PI*ZZ or ZZ. The average AAT value on admission for PI*ZZ or ZZ patients was 41.6 mg/dL. All transplanted patients with phenotyping or genotyping were PI*ZZ (or ZZ). Those who were jaundiced on admission were earlier referred to the specialized service and had higher levels of GGT and platelets on admission. There was no significant difference in the survival curve when comparing cholestatic jaundiced to non-cholestatic jaundiced patients on admission. Comparing patients who did or did not progress to PH, higher levels of AST and APRI score at diagnosis (P=0.011 and P=0.026, respectively) were observed and in the survival curves patients with PH showed impairment, with 20.2% survival with native liver in 15 years. Conclusion: Jaundice is an important clinical sign that motivates referral to a specialist, but it does not seem to compromise survival with native liver. Patients progressing to PH had higher AST, APRi score on admission and significantly impaired survival with native liver. It is important to pay attention to these signs in the follow-up of patients with AATD.

RESUMO Contexto: Deficiência de alfa 1-antitripsina (DAAT) é uma doença hereditária, de caráter autossômico codominante. A apresentação da doença hepática varia desde casos assintomáticos até doença terminal, o que dificulta reconhecimento e diagnóstico precoces. É a principal causa de transplante hepático pediátrico após atresia de vias biliares. Objetivo: Descrever as características clínicas, de exames laboratoriais, histológicos, avaliação fenotípica e/ou genética e sobrevida de uma coorte de pacientes pediátricos com DAAT. Métodos: Estudo observacional retrospectivo de 39 pacientes com diagnóstico de DAAT confirmada ou provável (sem fenotipagem ou genotipagem, mas com clínica sugestiva, baixo nível sérico de alfa 1-antitripsina (A1AT) e biópsia hepática com grânulos PAS, diástase resistentes). Variáveis clínicas, laboratoriais, histológicas, presença de hipertensão portal (HP) e sobrevida com fígado nativo foram analisadas. Resultados: Dos 39 pacientes, 66,7% eram do sexo masculino (26/39). A manifestação inicial foi icterícia colestática em 79,5% (31/39). Em 28,2% (11/39) houve necessidade de transplante hepático. O diagnóstico ocorreu com uma idade média de 3,1 anos e, o transplante hepático, 4,1 anos. Dos pacientes com DAAT confirmada, 89,2% (25/28) eram PI*ZZ ou ZZ. O valor médio de A1AT na admissão de pacientes PI*ZZ ou ZZ foi 41,6 mg/dL. Todos os transplantados com fenotipagem ou genotipagem eram PI*ZZ (ou ZZ). Os ictéricos à admissão foram referenciados mais cedo ao serviço especializado e apresentaram níveis mais elevados de GGT e plaquetas à admissão. Não houve diferença significativa na curva de sobrevida ao compararmos icterícia colestática ou não à admissão. Ao comparar os pacientes que progrediram ou não para HP, observou-se níveis mais elevados de AST e APRI escore ao diagnóstico (P=0,011 e P=0,026, respectivamente) e, nas curvas de sobrevida, pacientes com HP apresentaram comprometimento, com 20,2% de sobrevida com fígado nativo em 15 anos. Conclusão: Icterícia é um sinal clínico importante que motiva o encaminhamento ao especialista, mas parece não comprometer a sobrevida com fígado nativo. Pacientes com evolução para HP tiveram AST e escore APRi mais elevados à admissão e comprometimento significativo da sobrevida com fígado nativo. Importante atentar a esses sinais no seguimento de pacientes com DAAT.

PREDICTIVE FACTORS OF MORBIDITY ASSOCIATED WITH ESOPHAGEAL VARICEAL BLEEDING IN CHILDREN WITH PORTAL HYPERTENSION.

•Most data on the natural history of portal hypertension come from studies in adults. •The morbidity rate of upper gastrointestinal bleeding in children with portal hypertension tend to be underestimated. •This study showed the relevance of morbidity rates after variceal hemorrhage in pediatric patients, especially those with cirrhosis. •Patients with hemodynamic instability requiring blood transfusion or expansion on admission are at increased risk of complications secondary to upper gastrointestinal bleeding and should be closely monitored. Background - Most data on the natural history of portal hypertension come from studies in adults. The morbidity rate of upper gastrointestinal bleeding (UGIB) in children with portal hypertension has not been systematically characterized. Objective - To describe the morbidity and mortality of UGIB in pediatric patients with portal hypertension and identify predictive factors for the occurrence of its main complications. Methods - This retrospective study included pediatric patients with cirrhotic portal hypertension or with extrahepatic portal vein obstruction (EHPVO). Mortality and UGIB complications within a period of up to 6 weeks of the bleeding were investigated. To determine the predictive factors of morbidity, a multivariate analysis was performed using logistic regression; all results were considered significant at P<0.05. Results - A total of 86 patients (51.2% with EHPVO and 48.8% with cirrhosis) had 174 bleeding events. Ascites was the most common complication (43.1% of all cases), being more prevalent in patients with cirrhosis (P<0.001). Cirrhosis was a predictor of the occurrence of any morbidity (OR 20.3). The need for blood transfusion was predictor of at least one complication (OR 5.8), ascites (OR 7.2) and infections (OR 3.8) in the general group and at least one complication (OR 11.3) and ascites (OR 5.8) in cirrhotic patients. The need for expansion was a predictor of any morbidity (OR 4.6) and infections (OR 3.9) in the general group, in addition to being predictor of infection in cirrhotic patients (OR 5.4). There were no deaths from UGIB in the six weeks post-bleeding. Conclusion - The study showed the relevance of morbidity after UGIB in pediatric patients with portal hypertension, especially in those with cirrhosis. The patients with hemodynamic instability requiring blood transfusion or expansion on admission are at increased risk of complications related to upper gastrointestinal bleeding and should be closely monitored.

Fatores preditivos de morbidade associada à sangramento de varizes esofágicas em crianças com hipertensão porta / Predictive factors of morbidity associated with esophageal variceal bleeding in children with portal hypertension

ABSTRACT Background: Most data on the natural history of portal hypertension come from studies in adults. The morbidity rate of upper gastrointestinal bleeding (UGIB) in children with portal hypertension has not been systematically characterized. Objective: To describe the morbidity and mortality of UGIB in pediatric patients with portal hypertension and identify predictive factors for the occurrence of its main complications. Methods: This retrospective study included pediatric patients with cirrhotic portal hypertension or with extrahepatic portal vein obstruction (EHPVO). Mortality and UGIB complications within a period of up to 6 weeks of the bleeding were investigated. To determine the predictive factors of morbidity, a multivariate analysis was performed using logistic regression; all results were considered significant at P<0.05. Results: A total of 86 patients (51.2% with EHPVO and 48.8% with cirrhosis) had 174 bleeding events. Ascites was the most common complication (43.1% of all cases), being more prevalent in patients with cirrhosis (P<0.001). Cirrhosis was a predictor of the occurrence of any morbidity (OR 20.3). The need for blood transfusion was predictor of at least one complication (OR 5.8), ascites (OR 7.2) and infections (OR 3.8) in the general group and at least one complication (OR 11.3) and ascites (OR 5.8) in cirrhotic patients. The need for expansion was a predictor of any morbidity (OR 4.6) and infections (OR 3.9) in the general group, in addition to being predictor of infection in cirrhotic patients (OR 5.4). There were no deaths from UGIB in the six weeks post-bleeding. Conclusion: The study showed the relevance of morbidity after UGIB in pediatric patients with portal hypertension, especially in those with cirrhosis. The patients with hemodynamic instability requiring blood transfusion or expansion on admission are at increased risk of complications related to upper gastrointestinal bleeding and should be closely monitored.

RESUMO Contexto: A maioria dos dados sobre a história natural da hipertensão porta provém de estudos em adultos. A morbidade associada à hemorragia digestiva alta (HDA) em crianças com hipertensão porta ainda não foi sistematicamente estudada. Objetivo: Descrever a morbimortalidade da HDA em pacientes pediátricos com hipertensão porta e identificar fatores preditivos para a ocorrência de suas principais complicações. Métodos: Este estudo retrospectivo incluiu pacientes pediátricos com hipertensão porta cirrótica ou com obstrução extra-hepática da veia porta (OEHVP). A mortalidade e as complicações da HDA foram estudadas até seis semanas após o sangramento. Para determinar os fatores preditivos de morbidade, foi realizada análise multivariada por meio de regressão logística; todos os resultados foram considerados significativos com P<0,05. Resultados: Oitenta e seis pacientes (51,2% com OEHVP e 48,8% com cirrose) tiveram 174 eventos hemorrágicos. A ascite foi a complicação mais comum (43,1% de todos os casos), sendo mais prevalente em pacientes com cirrose (P<0,001). A cirrose foi preditor da ocorrência de pelo menos uma complicação (OR 20,3). A necessidade de transfusão sanguínea foi preditora de pelo menos uma complicação (OR 5,8), ascite (OR 7,2) e infecções (OR 3,8) no grupo geral e pelo menos uma complicação (OR 11,3) e ascite (OR 5,8) nos cirróticos. A necessidade de expansão foi preditor de qualquer morbidade (OR 4,6) e infecções (OR 3,9) no grupo geral, além de ser preditor de infecção em cirróticos (OR 5,4). Não houve óbitos por HDA nas 6 semanas pós-sangramento. Conclusão: O estudo mostrou a relevância da morbidade após HDA em pacientes pediátricos com hipertensão porta, principalmente naqueles com cirrose. Os pacientes com instabilidade hemodinâmica que necessitam de transfusão de sangue ou expansão na admissão têm risco aumentado de complicações relacionadas à hemorragia digestiva alta e devem ser monitorados de perto.

Abnormalities of Cardiac Situs and Heart Disease Diagnosed by Echocardiography in Patients with Biliary Atresia

Abstract Background: Left isomerism (LI) is a common finding in patients with biliary atresia (BA), and it can be identified by echocardiography. Several comorbidities may be present in patients with LI, including heart disease. Objective: To investigate the prevalence of LI and heart disease in children (< 18 years of age) with BA followed-up at Hospital das Clínicas, UFMG. Methods: This is a cross-sectional study involving patients diagnosed with BA between February 2016 and April 2020 who underwent transthoracic echocardiography and, in case of situs abnormalities, also electrocardiography. Results: Our study recruited 58 patients (mean age: 3.08 years; female/male ratio: 1.5:1). The general prevalence of situs abnormalities was 8.6% (5/58) and the most common one was LI (4/5 or 80%). One patient had situs inversus. Among patients with situs abnormalities, the general prevalence of heart disease was 80% (4/5), apart from anomalies of the inferior vena cava), with pulmonary valve stenosis (PVS) as the only change seen (75% of mild forms and 25% of moderate forms). Among patients with situs abnormalities, the prevalence of rhythm changes was 80% (4/5), and low atrial rhythm was the most common finding (3/4 or 75%). Conclusion: The prevalence of situs abnormalities in our sample was similar to that described in the literature. We observed an exclusive prevalence of PVS and a high prevalence of rhythm changes among patients with LI. Although the diagnosis of isomerism does not initially add much cardiovascular risk to the sample, possible late deterioration should be considered.

Mild Phenotype of Arthrogryposis, Renal Dysfunction, and Cholestasis Syndrome 1 Caused by a Novel VPS33B Variant.

The arthrogryposis, renal dysfunction, and cholestasis syndrome (ARCS) is an autosomal recessive multisystem disease caused by variants in VPS33B or VIPAS39. The classical presentation includes congenital joint contractures, renal tubular dysfunction, cholestasis, and early death. Additional features include ichthyosis, central nervous system malformations, platelet dysfunction, and severe failure to thrive. We studied three patients with cholestasis, increased aminotransferases, normal gamma-glutamyl transferase, and developmental and language delay. Whole exome sequencing analysis identified VPS33B variants in all patients: patients 1 and 2 presented a novel homozygous variant at position c.1148T>A. p.(Ile383Asn), and patient 3 was compound heterozygous for the same c.1148T>A. variant, in addition to the c.940-2A>G. variant. ARCS is compatible with the symptomatology presented by the studied patients. However, most patients that have been described in the literature with ARCS had severe failure to thrive and died in the first 6 months of life. The three patients studied here have a mild ARCS phenotype with prolonged survival. Consequently, we believe that the molecular analysis of the VPS33B and VIPAS39 should be considered in patients with normal gamma-glutamyl transferase cholestasis.

PERCUTANEOUS ENDOSCOPIC GASTROSTOMY IN CHILDREN AND ADOLESCENTS: 15-YEARS' EXPERIENCE OF A TERTIARY CENTER.

BACKGROUND: Percutaneous endoscopic gastrostomy (PEG) is an important option for enteral nutrition for both children and adults. It is considered a safe, effective, and advantageous technique in comparison to other complementary feeding routes. It allows continuous feeding, the feeding of patients with swallowing disorders due to neurological causes or others, and the administration of non-palatable diets or medications, all with low rates of complications and mortality. OBJECTIVE: This study aimed to evaluate the main indications and complications of PEG in pediatric patients. In addition, the impact on the nutritional status of patients undergoing PEG was also compared with weight, body mass index (BMI), and height according to references from the World Health Organization. METHODS: This observational and retrospective study included 152 children and adolescents who underwent PEG between January/2003 and December/2018. Patients up to 18 years of age at the time of the procedure were included. Complications related to the procedure were classified as minor or major. Patients with PEG indication for nutritional supplementation were evaluated for weight gain, height, and BMI, using the Z score at the day of the procedure and six months, 1 year, and 2 years after the procedure. RESULTS: Indications for PEG were: swallowing disorder of neurological cause (67.1%), need for nutritional supplementation (25%), swallowing disorder of mechanical origin (6.6%), and indication of gastric decompression (1.3%). Minor complications occurred in 57.8% of patients and major complications in 9.8% of patients. The traction technique corresponded to 92.1% and puncture to 7.9%. The death rate was 1.3%. Thirty-eight patients had an indication for nutritional supplementation. In these patients, there was a gradual increase in both BMI and weight, reaching statistically significant differences (P=0.0340 and P= 0.0105, respectively). These differences were more evident in chronic renal disease patients. Height did not vary significantly (P=0.543). CONCLUSION: PEG proved to be an advantageous option as an auxiliary feeding method in pediatric patients. Dysphagia of neurological origin was the main indication followed by the need for nutritional supplementation. PEG has low frequency of major complications and mortality. This study also showed the importance of PEG in patients who need nutritional supplementation, as it enabled patients to move from undernutrition to normal weight ranges.

Gastrostomia endoscópica percutânea em crianças e adolescentes: 15 anos de experiência em um hospital terciário / Percutaneous endoscopic gastrostomy in children and adolescents: 15-years' experience of a tertiary center

ABSTRACT BACKGROUND: Percutaneous endoscopic gastrostomy (PEG) is an important option for enteral nutrition for both children and adults. It is considered a safe, effective, and advantageous technique in comparison to other complementary feeding routes. It allows continuous feeding, the feeding of patients with swallowing disorders due to neurological causes or others, and the administration of non-palatable diets or medications, all with low rates of complications and mortality. OBJECTIVE: This study aimed to evaluate the main indications and complications of PEG in pediatric patients. In addition, the impact on the nutritional status of patients undergoing PEG was also compared with weight, body mass index (BMI), and height according to references from the World Health Organization. METHODS: This observational and retrospective study included 152 children and adolescents who underwent PEG between January/2003 and December/2018. Patients up to 18 years of age at the time of the procedure were included. Complications related to the procedure were classified as minor or major. Patients with PEG indication for nutritional supplementation were evaluated for weight gain, height, and BMI, using the Z score at the day of the procedure and six months, 1 year, and 2 years after the procedure. RESULTS: Indications for PEG were: swallowing disorder of neurological cause (67.1%), need for nutritional supplementation (25%), swallowing disorder of mechanical origin (6.6%), and indication of gastric decompression (1.3%). Minor complications occurred in 57.8% of patients and major complications in 9.8% of patients. The traction technique corresponded to 92.1% and puncture to 7.9%. The death rate was 1.3%. Thirty-eight patients had an indication for nutritional supplementation. In these patients, there was a gradual increase in both BMI and weight, reaching statistically significant differences (P=0.0340 and P= 0.0105, respectively). These differences were more evident in chronic renal disease patients. Height did not vary significantly (P=0.543). CONCLUSION: PEG proved to be an advantageous option as an auxiliary feeding method in pediatric patients. Dysphagia of neurological origin was the main indication followed by the need for nutritional supplementation. PEG has low frequency of major complications and mortality. This study also showed the importance of PEG in patients who need nutritional supplementation, as it enabled patients to move from undernutrition to normal weight ranges.

RESUMO CONTEXTO: A gastrostomia endoscópica percutânea (GEP) é uma importante opção de nutrição enteral para crianças e adultos, sendo considerada uma técnica segura, eficaz e vantajosa em comparação às outras vias de alimentação complementar. Permite a alimentação contínua, a alimentação em pacientes com distúrbios de deglutição de causa neurológica ou outros, a administração de dietas ou medicamentos não palatáveis, todos com baixos índices de complicações e mortalidade. OBJETIVO: Avaliar as principais indicações e complicações de pacientes pediátricos submetidos à GEP e o impacto no estado nutricional de pacientes submetidos à GEP para suplementação nutricional, comparando peso, índice de massa corporal (IMC) e estatura com referências da Organização Mundial de Saúde. MÉTODOS: Estudo observacional e retrospectivo de 152 crianças e adolescentes submetidos à GEP, no período de janeiro/2003 a dezembro/2018. Foram incluídos pacientes até 18 anos de idade na época do procedimento. As complicações relacionadas ao procedimento foram divididas em menores e maiores. Pacientes com indicação de GEP para suplementação nutricional foram avaliados quanto ao ganho de peso, altura e IMC, por meio do escore Z no dia do procedimento e 6 meses; 1 ano; e 2 anos após o procedimento. RESULTADOS: As indicações para GEP foram distúrbio de deglutição de causa neurológica (67,1%), necessidade de suplementação nutricional (25%), distúrbio de deglutição de origem mecânica (6,6%), e indicação de descompressão gástrica (1,3%). Complicações menores ocorreram em 57,8% dos pacientes e complicações maiores em 9,8%. A técnica de tração correspondeu a 92,1% e a punção, 7,9%. A taxa de mortalidade foi de 1,3%. Trinta e oito pacientes tinham indicação de suplementação nutricional. Nestes, houve aumento gradativo tanto do IMC quanto do peso, com variação estatisticamente significativa da mediana P=0,0340 e P=0,0105, respectivamente, mais evidente nos pacientes renais crônicos. A altura não variou significativamente (P=0,543). CONCLUSÃO: A GEP mostrou-se uma opção vantajosa como forma auxiliar de alimentação em pacientes pediátricos, tendo como principais indicações a disfagia de causa neurológica e a necessidade de suplementação nutricional, com baixa prevalência de complicações maiores e mortalidade. Este estudo também mostrou a importância da GEP em pacientes com necessidade de suplementação nutricional, possibilitando a passagem dos pacientes desnutridos para escores nutricionais de peso adequados à idade.

Non-invasive Predictors of Esophageal Varices With a High Risk of Bleeding in Pediatric Cirrhotic Patients.

OBJECTIVES: To evaluate non-invasive predictive factors of varices with a high risk of bleeding in pediatric cirrhotic patients. METHODS: This retrospective, cross-sectional study included data from 158 children with cirrhosis, median age of 5.38 years (interquartile [IQ] 2.08-11.52 years), and no history of upper gastrointestinal bleeding. Patients underwent an endoscopy to screen for esophageal varices. Varices with a high risk of bleeding were defined as those with a medium to large caliber, presence of red spots, or the presence of gastric varices and identified as high-risk varices (HRV). Laboratory and clinical factors were evaluated as possible predictors of HRV. RESULTS: HRV were detected in 30 children (19%) after the first endoscopy. In the multivariate analysis, only the risk score (RS), as described by Park et al, and the aspartate aminotransferase-to-platelet ratio index (APRi) were predictive of HRV. The best non-invasive predictor of HRV was the RS with an area under the receiver operating characteristic curve of 0.764. When used a cut-off point of -1.2, the sensitivity of the RS was 90% and specificity was 53%. The use of RS or APRi correctly identified 96% of children with HRV. CONCLUSIONS: The described predictors allow the correct identification of patients with HRV. The association of RS >-1.2 or APRi >1.4 has a good sensitivity to identify HRV and to prevent unnecessary endoscopy in about one-third of children with no HRV.

Endoscopic prophylaxis and factors associated with bleeding in children with extrahepatic portal vein obstruction.

OBJECTIVES: This study aimed to evaluate factors associated with upper digestive hemorrhage and primary and secondary endoscopic prophylaxis outcomes in children with extrahepatic portal vein obstruction. METHODS: This observational and prospective study included 72 children with extrahepatic portal vein obstruction who were followed from 2005 to 2017. Risk factors associated with upper digestive hemorrhage and the results of primary and secondary prophylaxis of these patients were evaluated. RESULTS: Fifty patients (69.4%) had one or more episodes of bleeding during follow-up, with a median age at first hemorrhage of 4.81 years. The multivariate analysis showed that medium- to large-caliber esophageal varices were associated with an 18-fold risk of upper digestive hemorrhage (95% CI: 4.33-74.76; p < 0.0001). Primary prophylaxis was administered to 14 patients, with eradication in 85.7%; however, 14.3% of these patients had hemorrhages during the follow-up period and 41.7% had a relapse of varices. Secondary prophylaxis was administered to 41 patients. Esophageal varices were eradicated in 90.2% of patients. There were relapse and re-bleeding of esophageal varices in 45.9% and 34.1% of the children, respectively. CONCLUSION: Primary and secondary endoscopic prophylaxes showed high rates of esophageal varix eradication, but with significant relapses. Eradication of esophageal varices cannot definitively prevent recurrent upper digestive hemorrhage, since bleeding from alternate sites can occur. Medium- and large-caliber esophageal varices were associated with upper digestive hemorrhage in patients with extrahepatic portal vein obstruction. To the best of the authors' knowledge, this study is the first to evaluate bleeding risk factors in children with extrahepatic portal vein obstruction.

MULTIVARIATE ANALYSIS OF BILIARY FLOW-RELATED FACTORS AND POST-KASAI SURVIVAL IN BILIARY ATRESIA PATIENTS.

BACKGROUND: Biliary atresia represents the most common surgically treatable cause of cholestasis in newborns. If not corrected, secondary biliary cirrhosis invariably results. OBJECTIVE: To evaluate, through multivariate analysis, the prognostic factors associated with the presence of biliary flow and survival with the native liver following Kasai portoenterostomy. METHODS: The study analyzed data from 117 biliary atresia patients who underwent portoenterostomy and had suitable histological material for evaluation. A logistic regression model was used to assess the presence of biliary flow. Survival was investigated through Kaplan-Meier curves and Cox-adjusted models. RESULTS: One third of patients achieved biliary flow and the median age at surgery was 81 days. Age at surgery, albumin, postoperative complications, biliary atresia structural malformation (BASM), liver architecture, larger duct diameter at porta hepatis, and cirrhosis (Ishak score) were the initial variables for the multivariate analysis. Age at surgery >90 days was the only variable associated with the absence of biliary drainage. Survival analysis revealed that the absence of biliary flow (P<0.0001), age at surgery >90 days (P=0.035), and the presence of BASM (P<0.0001), alone, could predict death or need for liver transplantation. Multivariate analysis demonstrated that the absence of biliary flow (P<0.0001 hazard ratio [HR] 6.25, 95% confidence interval [CI] 3.19-12.22) and the presence of BASM (P=0.014 HR 2.16, 95% CI 1.17-3.99) were associated with lowest survival with the native liver. CONCLUSION: Age at surgery >90 days was associated with absence of biliary flow. The presence of biliary drainage and the absence of structural malformations are cornerstone features for higher survival rates with the native liver.

Análise multivariada de variáveis relacionadas ao fluxo biliar e sobrevida pós-Kasai em pacientes com atresia biliar / Multivariate analysis of biliary flow-related factors and post-kasai survival in biliary atresia patients

ABSTRACT BACKGROUND: Biliary atresia represents the most common surgically treatable cause of cholestasis in newborns. If not corrected, secondary biliary cirrhosis invariably results. OBJECTIVE: To evaluate, through multivariate analysis, the prognostic factors associated with the presence of biliary flow and survival with the native liver following Kasai portoenterostomy. METHODS: The study analyzed data from 117 biliary atresia patients who underwent portoenterostomy and had suitable histological material for evaluation. A logistic regression model was used to assess the presence of biliary flow. Survival was investigated through Kaplan-Meier curves and Cox-adjusted models. RESULTS: One third of patients achieved biliary flow and the median age at surgery was 81 days. Age at surgery, albumin, postoperative complications, biliary atresia structural malformation (BASM), liver architecture, larger duct diameter at porta hepatis, and cirrhosis (Ishak score) were the initial variables for the multivariate analysis. Age at surgery >90 days was the only variable associated with the absence of biliary drainage. Survival analysis revealed that the absence of biliary flow (P<0.0001), age at surgery >90 days (P=0.035), and the presence of BASM (P<0.0001), alone, could predict death or need for liver transplantation. Multivariate analysis demonstrated that the absence of biliary flow (P<0.0001 hazard ratio [HR] 6.25, 95% confidence interval [CI] 3.19-12.22) and the presence of BASM (P=0.014 HR 2.16, 95% CI 1.17-3.99) were associated with lowest survival with the native liver. CONCLUSION: Age at surgery >90 days was associated with absence of biliary flow. The presence of biliary drainage and the absence of structural malformations are cornerstone features for higher survival rates with the native liver.

RESUMO CONTEXTO: A atresia biliar representa a principal causa de colestase tratada cirurgicamente durante o período neonatal. Se a criança não for operada, ela evolui invariavelmente para cirrose biliar secundária. OBJETIVO: Avaliar, através de análise multivariada, os fatores prognósticos associados à presença de fluxo biliar e à sobrevida com fígado nativo após a realização da portoenterostomia de Kasai. MÉTODOS: O estudo analisou 117 pacientes com atresia biliar submetidos à portoenterostomia e com material histológico adequado para avaliação. O modelo de regressão logística foi utilizado para avaliar a presença de fluxo biliar. Sobrevida foi estudada através das curvas Kaplan-Meier e ajuste do modelo de Cox. RESULTADOS: Um terço dos pacientes obteve fluxo biliar e a mediana de idade à cirurgia foi de 81 dias. Idade à cirurgia, albumina, complicação pós-operatória, BASM (do inglês, biliary atresia structural malformation), arquitetura hepática, diâmetro do maior canalículo no porta hepatis e cirrose, segundo o escore de Ishak, foram as variáveis iniciais da análise multivariada. Idade à cirurgia maior que 90 dias de vida foi a única variável associada à ausência de drenagem biliar. A análise de sobrevida mostrou que as variáveis: ausência de fluxo biliar (P<0,0001), idade à cirurgia maior que 90 dias (P=0,035) e presença de BASM (P<0,0001), isoladamente, predizem morte ou necessidade de transplante hepático. Na análise multivariada, ausência de fluxo biliar (P<0,0001 HR:6,25 [IC95% 3,19; 12,22]) e presença de BASM (P=0,014 HR:2,16 [IC95% 1,17; 3,99]) mostraram-se associadas, com significância estatística, a menor sobrevida com fígado nativo. CONCLUSÃO: Idade à cirurgia maior que 90 dias foi identificada como fator de risco independente para ausência de fluxo biliar. Além disso, a presença de drenagem biliar e a ausência de malformações estruturais da atresia biliar são variáveis fundamentais para a maior sobrevida com fígado nativo.

Colangite esclerosante primária em crianças e adolescentes / Primary sclerosing cholangitis in children and adolescents

ABSTRACT 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.

RESUMO CONTEXTO: A colangite esclerosante primária é uma doença rara, mas sua prevalência tem sido subestimada em crianças e adolescentes, tanto pela variedade de apresentação clínica quanto pela dificuldade diagnóstica neste período. OBJETIVO: Avaliar crianças e adolescentes com colangite esclerosante primária descrevendo seu quadro clínico, laboratorial, histopatológico e colangiográfico. MÉTODOS: Trata-se de um estudo descritivo observacional, de 2005 a 2016, de todos os pacientes atendidos no Ambulatório de Hepatologia Pediátrica do Hospital das Clínicas da UFMG, com o diagnóstico de colangite esclerosante primária até a idade de 18 anos. O diagnóstico foi estabelecido segundo os critérios clínicos, laboratoriais, radiológicos e/ou histopatológicos. Foi realizada investigação para exclusão de outras doenças hepáticas crônicas e causas secundárias de colangite. A análise dos dados foi efetuada com os recursos estatísticos do software SPSS. As variáveis foram expressas por meio de médias, desvio-padrão, frequência absoluta e porcentagem. RESULTADOS: Vinte e um pacientes preencheram os critérios de inclusão. Houve predomínio no sexo masculino (3,2:1). A média de idade ao diagnóstico foi 6,7±3,9 anos. Cinco (23,8%) pacientes apresentavam doença inflamatória intestinal associada, sendo quatro casos de colite ulcerativa e um de colite indeterminada. Na apresentação, os sinais e sintomas são variados, em geral discretos. O sintoma mais frequente foi dor abdominal (47,6%), seguido menos frequentemente por icterícia (28,6%) e prurido (14,3%). Em 33,3% dos pacientes, o motivo de início da investigação foi alteração de enzimas em pacientes assintomáticos ou oligossintomáticos. Todos os pacientes exibiam aumento das enzimas hepáticas: aminotransferases, gama glutamiltransferase e fosfatase alcalina. Vinte pacientes apresentavam alterações em exame colangiográfico, compatíveis com colangite esclerosante primária; um paciente não apresentava alterações na colangiorressonância, mas apresentava alterações histopatológicas compatíveis com colangite de pequenos ductos. Fibrose hepática já estava presente em 60% dos 15 pacientes com biópsia à admissão; com cirrose estabelecida em quatro pacientes. 28,5% dos pacientes tiveram evolução desfavorável, com dois (9,5%) óbitos e quatro (19%) pacientes transplantados. CONCLUSÃO: Colangite esclerosante primária é uma doença rara na criança e no adolescente, cujo diagnóstico inicial pode ser atrasado ou passar despercebido, principalmente por apresentar manifestações clínicas inespecíficas ou cursar de forma assintomática. É frequente a associação com doença inflamatória intestinal. O prognóstico pode ser reservado com o avançar da doença, sendo o transplante hepático o tratamento definitivo.

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.

Nutritional evaluation of children with chronic cholestatic disease / Avaliação nutricional de crianças com colestase crônica

Abstract 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.

Resumo Objetivo: Avaliar a situação nutricional de crianças com colestase persistente e comparar os índices antropométricos entre crianças com e sem cirrose hepática e crianças com e sem icterícia. Métodos: Foram incluídas crianças com colestase persistente, ou seja, aumento da bilirrubina direta ou alterações na enzima canalicular, gamaglutamiltransferase (GGT). As medidas antropométricas foram peso, estatura ou altura, circunferência do braço (CB), espessura da prega cutânea do tríceps (TST), circunferência muscular do braço (CMB) e índice de massa corporal (IMC). Resultados: Foram avaliadas 91 crianças com colestase, com idade média de 12 meses; 33% e 30,8% dos pacientes apresentaram índices P/I e A/I com escore Z abaixo de –2, respectivamente. Com relação ao índice P/A e IMC, somente 12% e 16% dos pacientes, respectivamente, apresentaram escore Z abaixo de –2. Com relação à CB, 43,8% de 89 pacientes avaliados apresentaram alguma depleção. Observando a TST, 64% dos pacientes que apresentaram depleção, 71,1% dos 45 pacientes avaliados apresentaram algum grau de depleção com relação ao índice de CMB. Conclusão: A avaliação do peso em pacientes com doenças hepáticas crônicas poderá superestimar a situação nutricional devido a visceromegalia, edema subclínico ou ascite. Os índices que correlacionam peso e altura, como P/A e IMC, também podem não mostrar depleção devido à doença crônica em que há depleção tanto do peso quanto da altura. A TST, BC e CMB são parâmetros que estimam melhor a situação nutricional e devem fazer parte de gestão de pacientes com doenças hepáticas e colestase.

Doença inflamatória intestinal na infância / Intestinal Inflamatory disease in childood

A incidência da doença inflamatória intestinal (DII) tem aumentado nos países ocidentais nos últimos anos. Classicamente ela é dividida em doença de Crohn e colite ulcerativa. As manifestações clínicas mais comuns são a dor abdominal, diarreia, perda de peso e sangue nas fezes. A inflamação intestinal reduz a absorção de nutrientes. Manifestações extraintestinais podem estar presentes. Deve ser realizada a pesquisa de imunodeficiência primária em crianças com diagnóstico de DII antes dos dois anos de idade. Os exames laboratoriais devem incluir triagem de inflamação aguda e/ou crônica, avaliação de anemia e estado nutricional. Exames endoscópicos e de fezes são necessários, principalmente antes do início do tratamento. O seguimento ambulatorial e a solicitação de exames complementares devem ser criteriosos. O tratamento é difícil por se tratar de uma doença crônica, com fases de recidiva e remissão. Os medicamentos apresentam efeitos adversos, algumas vezes graves. A visão mais atual da terapia da DII baseia-se na modificação da história natural da doença, com o objetivo de alcançar cicatrização da mucosa, redução das complicações da doença e melhora da qualidade de vida do paciente. É fundamental acompanhar o crescimento linear, o desenvolvimento muscular, esquelético e puberal. O pediatra geral deve estar atento para o diagnóstico, solicitando os exames complementares e avaliando a necessidade de encaminhamento para um gastroenterologista pediatra.(AU)

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.(AU)

Falência hepática aguda em crianças e adolescentes / Acute liver failure in children and adolescents

A halência hepática aguda (FHA) é uma condição rara, mas devastadora, que evolui para falência de múltiplos órgãos e óbito como seu curso natural. A assistência intensiva e o transplante hepático possibilitaram a modificação da história natural e aumento da sobrevida. A FHA, geralmente, se apresenta em uma criança ou adolescente previamente hígido que inicia com sintomas inespecíficos de duração variada e com a evolução do quadro surgem outros sintomas como icterícia, vômitos, hipoglicemia e convulsões, tornando a síndrome clínica evidente. O diagnóstico etiológico é importante, uma vez que algumas doenças de base possuem tratamentos específicos. Contudo, em até 50% dos casos, um diagnóstico específico não é estabelecido. Sua presença será estabelecida se há evidência bioquímica de falência hepática aguda, na ausência de doença hepática crônica e associada à coagulopatia de origem hepática não corrigível pela vitamina K (RNI > 1,5 em paciente com encefalopatia hepática ou RNI ? 2 na ausência de encefalopatia hepática). O tempo entre o início do quadro clínico e o desenvolvimento da encefalopatia deve ser inferior a oito semanas. O reconhecimento e o encaminhamento para centro especializado devem ser o mais precoce possível. A indicação de transplante hepático deve ser avaliada periodicamente. O tratamento intensivo e multidisciplinar é essencial para sobrevida.

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 importantbecause 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.

BILIARY ATRESIA: evaluation on two distinct periods at a reference pediatric service / Atresia biliar: avaliação em dois períodos distintos em um serviço de referência

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. .

Contexto A atresia biliar é uma doença progressiva, idiopática, fibro obliterativa, da vias bilares extra hepáticas que se apresenta com obstrução biliar exclusivamente no periodo neonatal. Objetivos Caracterizar duas épocas distintas visando avaliar se ocorreram alterações quanto a idade de encaminhamento das crianças, idade cirúrgica, duração do tempo entre internação e realização da cirurgia de Kasai, resultados cirúrgicos quanto a presença de fluxo biliar e sobrevida entre dois períodos. Métodos Estudo retrospectivo de crianças com diagnóstico de atresia biliar em dois períodos: 1983-1993 e 1998-2011. Resultados Foram admitidas 129 crianças com diagnóstico de AB, 48 (casuística I) e 81 (casuística II), com idade de admissão mediana de 94 e 60 dias, nas casuísticas I e II, respectivamente (P = 0,0001). Na avaliação dos pacientes submetidos à portoenterostomia com idade até 120 dias de vida, não houve diferença em relação ao tempo entre internação e realização da cirurgia de Kasai (P = 0,15), mas ocorreu ao comparar a idade à cirurgia entre os dois períodos (P = 0,002). A probabilidade estimada de sobrevida dos pacientes sem fluxo biliar livre de transplante ou óbito em 18 meses foi de 44,6%, 38,7% nas casuísticas I e II, respectivamente. Com fluxo biliar, todos estavam vivos ao longo do seguimento da casuística I e 80,3% em sete anos na casuística II. Conclusões Apesar dos pacientes chegarem mais cedo e serem submetidos à portoenterostomia, ainda se opera tarde. Encaminhamento tardio e a propedêutica prolongada são os principais contribuintes. Quanto maior a idade cirúrgica, menor a probabilidade de se obter fluxo biliar e consequentemente menor sobrevida. .

Alerta amarelo: icterícia após duas semanas de vida é uma urgência pediátrica / Yellow alert: icterus after two weeks of life is equal of medical evaluation

A colestase neonatal manifesta-se clinicamente com icterícia, colúria, hipocolia ou acolia fecal. Reveste-se de grande importância, uma vez que requer propedêutica urgente e precisa, com o propósito de definir o diagnóstico e tratamento. Diante de todo recém-nascido cuja icterícia persista além dos 14 dias de vida, impõe-se a dosagem das bilirrubinas (total e fração) e a observação da coloração das fezes e urina. Uma vez confirmada a colestase, é necessário o encaminhamento urgente para umcentro de referência. Para investigação da causa da colestase, o uso de protocolo sistemático é útil para agilizar o diagnóstico. Atresia biliar, uma das principais causas de icterícia colestática, necessita de correção cirúrgica antes dos 60 dias de vida. Tal procedimento mostra-se bem-sucedido em 70 a 80% dos pacientes operados antes dessa idade. O encaminhamento tardio dos pacientes com suspeita de AB constitui um dos principais fatores para o fracasso da correção cirúrgica. Por isso, a divulgação da importância da análise da cor das fezes e da dosagem de bilirrubinas, a partir de campanhas como alerta amarelo, é necessária e deve atingir não apenas os pais, mas também os profissionais da saúde...

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...