Increased Prevalence of Left Lobe Atrophy in Long-standing Extrahepatic Portal Vein Obstruction.

OBJECTIVES: The aim of this study was to describe long-term changes in standard blood tests and ultrasound (US) findings in pediatric patients with Extrahepatic Portal Vein Obstruction (EHPVO) who have not undergone Meso-Rex Bypass (MRB) surgery. METHODS: US, laboratory, and endoscopic data of 77 patients were analyzed and compared at 2 different points in time: at initial workup and at most recent follow-up. Differences were assessed using McNemar and Wilcoxon tests, while correlations were evaluated using generalized estimating equations. RESULTS: Upper gastrointestinal bleeding was the most frequent initial manifestation of EHPVO, in 45.4% of subjects. The mean age at diagnosis was 4.3 years. Seventy-four percentage had a previous history of umbilical catheterization and/or neonatal sepsis. Over time, there was a significant increase in the prevalence of leukopenia, thrombocytopenia, and, interestingly, of left lobe atrophy (LLA), even though the number of esophageal varices and the need for endoscopic interventions reduced. A significant correlation was found between history of umbilical catheterization and LLA, splenomegaly and LLA, and gallbladder wall thickening and LLA. Overall, the number of patients with cholelithiasis at initial workup was low (and therefore, not analyzed with inferential statistics); however, we observed a relative increase over time. CONCLUSIONS: In this cohort of patients with EHPVO followed over more than 8 years without treatment with MRB, we report the novel finding of a significant increase in the prevalence of LLA over time. LLA correlated with past medical history of umbilical catheterization, and findings of splenomegaly and gallbladder wall thickening. We propose that that LLA should be further explored as a marker of portal hypertension, particularly in the context of a history of umbilical catheterization.

Major Complications of Pediatric Percutaneous Liver Biopsy Do Not Differ Among Physicians With Different Degrees of Training.

INTRODUCTION: The objective was to compare safety of pediatric percutaneous liver biopsy (PLB) performed by fellows or staff physicians. METHODS: Outcomes of 212 PLB completed by first-year pediatric gastroenterology fellows or by staff physicians over 8 years were analyzed and compared. RESULTS: Approximately 81.5% of the biopsies were completed by trainees. No significant differences were found between groups (fellows vs staff) regarding number of punctures (median of 1.7 for both), nonrepresentative biopsies (4.2% vs 2.6%), and hemoglobin drop (median of 0.7 vs 0.5 g/L). DISCUSSION: Complications of pediatric PLB are uncommon and did not differ among physicians with different training levels.

Hepatic involvement in paediatric patients with paracoccidioidomycosis: a histological study.

AIM: Paracoccidioidomycosis is a systemic mycosis that is endemic to certain countries in Latin America. This study aimed to describe the histological features of liver involvement in patients with paracoccidioidomycosis aged <16 years of age who were treated between 1980 and 2010, with a diagnosis that was confirmed by detection of the fungus by pathological examination. METHODS AND RESULTS: Liver tissue was obtained from one necropsy and 12 biopsies. Throughout 2007, biopsies were taken from patients with persistent jaundice or portal hypertension, after which biopsies became indicated due to elevated aminotransferase and low albumin levels. Using haematoxylin and eosin (H&E), Masson's trichrome and immunohistochemical (CK7 and CK19) staining, we noted degenerative alterations in bile duct cells and inflammatory injury to the bile ducts in 10 biopsies. Using immunohistochemistry for CK7 and CK19, we observed ductal proliferation in all 12 samples. CONCLUSIONS: Bile duct injuries by inflammatory cells might explain the predominant increase in canalicular enzymes; immunohistochemistry is more sensitive in demonstrating ductular reactions and might show changes that are not apparent on H&E staining.

Ultrasonographic predictors of esophageal varices.

OBJECTIVE: The aim of this study was to identify ultrasonographic predictors of esophageal varices (EVs) in children and adolescents with chronic liver disease (CLD) and extrahepatic portal venous obstruction (EHPVO). METHODS: This study evaluates 53 patients younger than 20 years with CLD or EHPVO and no history of bleeding or prophylactic EVs treatment. They were divided into 2 groups: group I (35 with CLD) and group II (18 with EHPVO). Splenorenal shunt (SS), gallbladder wall varices, gallbladder wall thickening (GT), and lesser omental thickness (LOT) were compared with the presence of EVs, gastric varices, and portal hypertensive gastropathy (PHG). Univariate (χ² test, Fisher exact test, and Wilcoxon signed rank test) and multivariate (logistic regression) analyses were performed. The area under the receiver operating curve was calculated. RESULTS: EVs were observed in 48.5% of patients with CLD and in 83.3% of patients with EHPVO. SS (P = 0.0329) and LOT (P = 0.0151) predicted EV among patients with CLD. A median of 5.3 mm of LOT was considered a predictor of EVs among these patients. Multivariate analysis showed SS as an independent predictor of EVs in patients with EHPVO (odds ratio 15). Gallbladder varices (P = 0.0245) and GT (P = 0.0289) predicted EVs among patients with EHPVO. PHG occurred more often among patients with CLD who had SS (P = 0.0384) and greater LOT (P = 0.0226). CONCLUSIONS: SS and a greater LOT were indicative of EV among children and adolescents with CLD. Gallbladder varices and GT were indicative of EVs among patients with EHPVO. SS and a greater LOT were indicative of PHG among patients with CLD.

Hepatic involvement in pediatric patients with paracoccidioidomycosis: a clinical and laboratory study.

The liver is one of the organs most affected by paracoccidioidomycosis, a systemic mycosis endemic in some Latin American countries. The majority of articles focused on adult populations and failed to describe any detailed experience of liver abnormalities in pediatric patients. Therefore, the aim of this study was to describe the frequency and characteristics of liver involvement in children with paracoccidioidomycosis. This study comprised 102 patients less than 16 years of age (median 104.3 months) diagnosed with paracoccidioidomycosis from 1980 to 2010. Diagnosis was established by the identification of fungus. Forty-one patients had liver involvement. The main clinical features were generalized lymph node enlargement (39/41), weight loss (34/41) and fever 32/41). Approximately, one-third of the patients had jaundice. Patients with hepatic involvement were younger. A predominant elevation of canalicular enzymes occurred. There was a statistically significant difference in albumin (p < 0.001) and hemoglobin (p = 0.002) values between patients with and without liver involvement, and the lowest values were found in the former group. Cutoff levels of albumin (<3.05 g/dL) and hemoglobin (<9.2 g/dL) can be used to infer hepatic involvement. Hypoalbuminemia (median 2.4 g/dl) is more severe in patients with hepatic involvement and may indicate a worse liver function or complication of the disease (intestinal lymphangiectasia). Deaths (6) occurred only among patients with liver involvement. Particular clinical and laboratory characteristics are present in pediatric patients with hepatic involvement. Younger patients and those with severe hypoalbuminemia are more likely to present liver involvement by Paracoccidioides brasiliensis.

Pediatric Acute Liver Failure as Presentation of Autoimmune Hepatitis: Learning from a Fatal Case.

Severe acute liver failure (SALF) is a rare condition in children. Up to 50% of the cryptogenic causes of SALF are associated with autoimmune hepatitis (AIH). This report presents a 5-year-old girl with progressive jaundice for 10 days. Her 1999 AIH diagnostic score totaled 11 points, compatible with probable AIH. She fulfilled the SALF criteria and the King's College criteria for liver transplantation, despite treatment with corticosteroids, and underwent the transplant, but died in the immediate postoperative period due to massive bleeding. Subsequently, the liver-kidney microsome type 1 result was 1:80, increasing the AIH score to 13 points. The final diagnosis was probable AIH type 2, associated with SALF. The biopsy of the explanted liver was compatible with fulminant hepatitis. This report highlights the difficult diagnosis of AIH in SALF, limitations of the diagnostic criteria for SALF in indications for emergency transplantation, and the uncertain therapeutic response produced by corticosteroids.

ANTHROPOMETRIC AND DIETARY ASSESSMENT OF PATIENTS WITH GLYCOGENOSIS TYPE I.

OBJECTIVE: To perform anthropometric and dietary evaluation of patients with glycogenosis type Ia and Ib. METHODS: This cross-sectional study is composed of a sample of 11 patients with glycogenosis divided into two subgroups according to the classification of glycogenosis (type Ia=5 and type Ib=6), aged between 4 and 20 years. The analyzed anthropometric variables were weight, height, body mass index, and measures of lean and fat body mass, which were compared with reference values. For dietary assessment, a food frequency questionnaire was used to calculate energy and macronutrients intake as well as the amount of raw cornstarch consumed. Mann-Whitney U test and Fisher's exact test were performed, considering a significance level of 5%. RESULTS: Patients ingested raw cornstarch in the amount of 0.49 to 1.34 g/kg/dose at a frequency of six times a day, which is lower than recommended (1.75-2.50 g/kg/dose, four times a day). The amount of energy intake was, on average, 50% higher than energy requirements; however, carbohydrate intake was below the adequacy percentage in 5/11 patients. Short stature was found in 4/10 patients; obesity, in 3/11; and muscle mass deficit, in 7/11. There were no statistical differences between the subgroups. CONCLUSIONS: In patients with glycogenosis type I, there was deficit in growth and muscle mass, but no differences were found between the subgroups (Ia and Ib). Although the diet did not exceed the adequacy of carbohydrates, about 1/3 of the patients presented obesity, probably due to higher energy intake.

SYMPTOMATIC CHOLELITHIASIS AS THE PRESENTATION OF PEDIATRIC PRIMARY SCLEROSING CHOLANGITIS - CASE SERIES AND LITERATURE REVIEW.

BACKGROUND: Primary sclerosing cholangitis (PSC) is a rare hepatobiliary disorder, whose etiology remains not fully elucidated. Given how rare PSC is in childhood, until the recent publication of a multicenter international collaboration, even data on its characteristics and natural history were scarce. Symptomatic cholelithiasis has not been previously reported as the presentation of PSC. OBJECTIVE: The aim of this study was the diagnosis of PSC following the initial unusual presentation with symptomatic cholelithiasis, that followed an atypical clinical course that could not be explained by cholelithiasis alone. A literature review was also conducted. METHODS: We conducted a retrospective chart review of three patients, who were diagnosed and/or followed at the Clinics Hospital, University of Campinas - Sao Paulo/ Brazil, between 2014 and 2020. Data analyzed included gender, age of presentation, past medical history, imaging findings, laboratory results, endoscopic evaluation, response to medical therapy and follow-up. RESULTS: Age at time of presentation with cholelithiasis varied from 10 to 12 years. In two of the cases reported, a more subacute onset of symptoms preceded the episode of cholelithiasis. Two patients were managed with cholecystectomy, not followed by any surgical complications, one patient was managed conservatively. Percutaneous liver biopsy was performed in all three cases, showing histological findings compatible with PSC. Associated inflammatory bowel disease (IBD) was not seen in any of the patients. The patients have been followed for a mean time of 3.4 years. CONCLUSION: PSC and cholelithiasis are both rare in the pediatric population. This study reports on symptomatic cholelithiasis as a presentation of PSC and raises the importance of suspecting an underlying hepatobiliary disorder in children with cholelithiasis without any known predisposing factors and/or that follow an atypical clinical course for cholelithiasis alone.

PREVALENCE OF HEPATIC STEATOSIS AMONG CHILDREN AND ADOLESCENTS WITH CYSTIC FIBROSIS AND ITS ASSOCIATION WITH NUTRITIONAL STATUS.

OBJECTIVE: To determine the prevalence of hepatic steatosis (HS) in children and adolescents with cystic fibrosis (CF) and associate it with nutritional status. METHODS: Cross-sectional study with children and adolescents with CF diagnosis. Weight and height were used to calculate the body mass index (BMI) and subsequent classification of the nutritional status. The midarm circumference (MAC), triceps skinfold thickness (TSF) and midarm muscle circumference (MAMC) were used to evaluate body composition. Abdominal ultrasonography was performed for diagnosis of HS. The statistical tests used were Student's t test, Mann-Whitney test and chi-square test with significance level of 5%. RESULTS: 50 patients with CF were evaluated, 18 (36%) were diagnosed with HS (Group A) and 32 (64%) without HS (Group B). The mean age of Group A was 13,2±4,9 years old and Group B 11,7±4,9; for BMI, the value for Group A was 18,0±4,1 and Group B was 15,7±3,8; the TSF of Group A was 8,4±3,5 mm and Group B was 7,0±2,5 mm. For these variables, there was no significant difference between the groups. The mean of MAC and MAMC differed significantly between the groups, being higher in the HS group, with p values of 0,047 and 0,043. CONCLUSIONS: The frequency of HS in patients with CF is high and it is not related to malnutrition, according to the parameters of BMI, TSF and MAMC. The values of MAC and MAMC indicated a greater reserve of muscle mass in patients with HS.

PRE AND POST-OPERATIVE OTORHINOLARYNGOLOGY SURGERY CARE IN PATIENTS WITH GLYCOGEN STORAGE DISEASE TYPE 1.

OBJECTIVE: To discuss aspects of pre and post-operative otorhinolaryngology surgery in patients with glycogen storage disease type 1b. CASE DESCRIPTION: Description of three clinical cases with probable glycogen storage disease type 1b who underwent otorhinolaryngology surgery, showing the importance of multidisciplinary interaction to avoid episodes of hypoglycemia. COMMENTS: Patients with glycogen storage disease type 1b present recurrent infections, including the otorhinolaryngology affections. When there is an indication for surgical treatment, the caloric intake should be carefully followed in order to prevent hypoglycemia. The way to ensure this is to perform the pre and postoperative period in the hospital ward. In the postoperative period, it is important to make a slow transition between the intravenous and oral routes and not suspend the infusion of glucose during the surgical procedure. The cases illustrate the need for the interaction of the otorhinolaryngologic surgeon with the anesthesiologist, the pediatrician and the gastro-pediatrician in the management of these patients, avoiding hypoglycemic episodes.

DEFINITION OF VITAMIN D DEFICIENCY IN SCHOOLCHILDREN: SYSTEMATIC REVIEW WITH META-ANALYSIS.

BACKGROUND: Vitamin D deficiency is being recognized as a pandemic due to the volume of people affected by the deficiency and the number of illnesses generated or stimulated by the deficiency. There is a lack of consensus in the literature on what is considered vitamin D deficiency [25(OH)D]. OBJECTIVE: This review brings together the most common levels of 25(OH)D found in healthy schoolchildren and what is considered deficient. METHODS: This systematic review was based on the literature accessed from the electronic databases: MEDLINE, EMBASE, LILACS, SCOPUS and WEB OF SCIENCE. The following descriptors were used in English, Portuguese and Spanish: "Vitamin D"; "Vitamin D deficiency"; "Nutritional Supplements" as well as all their synonyms. The meta-analysis was performed considering the random model. Inclusion criteria: healthy children aged 6 to 12 years, studies that had vitamin D levels, defined vitamin D deficiency. RESULTS: Of the 191 potentially eligible articles, only six articles were included, with 2618 students in total. The mean value of 25(OH)D was estimated at 18.11 ng/mL with 95% confidence interval. Among the articles found, three were considered deficiency levels below 20 ng/mL, one considered below 18 ng/mL, another below 15 ng/mL, and the latter below 11 ng/ mL. The prevalence of vitamin D deficiency among the articles was 48.6%, 7%, 98%, 64.63%, 19.5%, 28.4%, according to each classification used by the same. CONCLUSION: The most common definition in the literature of 25(OH)D deficiency in schoolchildren was at levels below 20 ng/mL. No side effects have been reported in studies that used fortification and/or vitamin D supplementation. Daily supplementation is more effective than seasonal supplementation. However, more studies are needed to define what can be considered as optimal levels of 25(OH)D in children.

Inflammatory process of patients receiving parenteral nutrition is not exclusively responsible for low selenium and glutathione peroxidase levels.

OBJECTIVE: The aim of this study was to verify if the selenium status of patients residing in locations with selenium-poor soil who receive parenteral nutrition (PN) without selenium supplementation is associated with the inflammatory process. METHODS: This was a prospective cohort study with hospitalized patients who started PN. The analyzed biochemical tests were plasma selenium, glutathione peroxidase (GPx), C-reactive protein, prealbumin, albumin, creatinine, lymphocytes, total cholesterol, high-density lipoprotein, and triglycerides. RESULTS: Seventy-seven patients with a mean age of 56.2 ± 15.7 y were studied. Most of them used PN as a result of clinical issues (70.1%) such as, gastric, renal, or hematologic neoplasia; gastrointestinal dysfunction; pancreatitis; sepsis; trauma without surgical needs; chylothorax; and fistula not related to surgical procedure. There were low levels of plasma selenium (98.7%) and GPx (60%) and elevated C-reactive protein (98.5%) in most cases. At the beginning of PN there was no correlation between selenium and laboratory tests (P > 0.05). At the second evaluation (seventh day of PN), there was a positive correlation of selenium levels with lymphocyte levels (r = 0.36; P = 0.04). After 2 wk of PN, there was a statistically significant correlation between selenium and GPx (r = 0.70; P = 0.02). CONCLUSIONS: Very low values of selenium and GPx from the beginning of PN were identified. The correlation of selenium levels with GPx in only 14 d of PN, regardless of inflammation, may reflect a critical selenium status, mainly because the correlation was verified after the acute phase. Therefore it is important to emphasize that supplementation should be started from the beginning of PN, especially in regions with selenium-deficient soil.

Autoimmune hepatitis in 828 Brazilian children and adolescents: clinical and laboratory findings, histological profile, treatments, and outcomes.

OBJECTIVE: This large study with a long-term follow-up aimed to evaluate the clinical presentation, laboratory findings, histological profile, treatments, and outcomes of children and adolescents with autoimmune hepatitis. METHODS: The medical records of 828 children and adolescents with autoimmune hepatitis were reviewed. A questionnaire was used to collect anonymous data on clinical presentation, biochemical and histological findings, and treatments. RESULTS: Of all patients, 89.6% had autoimmune hepatitis-1 and 10.4% had autoimmune hepatitis-2. The female sex was predominant in both groups. The median age at symptom onset was 111.5 (6; 210) and 53.5 (8; 165) months in the patients with autoimmune hepatitis 1 and autoimmune hepatitis-2, respectively. Acute clinical onset was observed in 56.1% and 58.8% and insidious symptoms in 43.9% and 41.2% of the patients with autoimmune hepatitis-1 and autoimmune hepatitis-2, respectively. The risk of hepatic failure was 1.6-fold higher for autoimmune hepatitis-2. Fulminant hepatic failure occurred in 3.6% and 10.6% of the patients with autoimmune hepatitis-1 and autoimmune hepatitis-2, respectively; the risk was 3.1-fold higher for autoimmune hepatitis-2. The gamma globulin and immunoglobulin G levels were significantly higher in autoimmune hepatitis-1, while the immunoglobulin A and C3 levels were lower in autoimmune hepatitis-2. Cirrhosis was observed in 22.4% of the patients; biochemical remission was achieved in 76.2%. The actuarial survival rate was 93.0%. A total of 4.6% underwent liver transplantation, and 6.9% died (autoimmune hepatitis-1: 7.5%; autoimmune hepatitis-2: 2.4%). CONCLUSIONS: In this large clinical series of Brazilian children and adolescents, autoimmune hepatitis-1 was more frequent, and patients with autoimmune hepatitis-2 exhibited higher disease remission rates with earlier response to treatment. Patients with autoimmune hepatitis-1 had a higher risk of death.

Clinical and laboratory evaluation of 101 patients with intrahepatic neonatal cholestasis.

BACKGROUND: Intrahepatic neonatal cholestasis can be the initial manifestation of a very heterogeneous group of illnesses of different etiologies. AIM: To evaluate and compare clinical and laboratory data among intrahepatic neonatal cholestasis groups of infectious, genetic-endocrine-metabolic and idiopathic etiologies. METHODS: The study evaluated retrospectively clinical and laboratory data of 101 infants, from March 1982 to December 2005, 84 from the State University of Campinas Teaching Hospital, Campinas, SP, Brazil, and 17 from the Child's Institute of the University of São Paulo, SP, Brazil. The inclusion criteria consisted of: jaundice beginning at up to 3 months of age and hepatic biopsy during the 1st year of life. It had been evaluated: clinical findings (gender, age, birth weight, weight during the first medical visit, stature at birth, jaundice, acholia/hipocholia, choluria, hepatomegaly and splenomegaly) and laboratorial (ALT, AST, FA, GGT, INR). RESULTS: According to diagnosis, patients were classified into three groups: group 1 (infectious) n = 24, group 2 (genetic-endocrine-metabolic) n = 21 and group 3 (idiopathic) n = 56. There were no significant differences in relation to the variables: age, gender, stature at birth, jaundice, acholia/hipocholia, choluria, hepatomegaly, splenomegaly, AST, ALT, ALP, GGT, DB and albumin. Significant differences were observed in relation to the following variables: birth weight and weight during the first medical visit. Birth weight of group 1 was lower in relation group 2 and 3. Weight during the first medical visit followed the same pattern. There was a statistically significant difference in relation to the INR, as the patients of the group 2 presented higher values in relation to groups 2 and 3, despite the median was still pointing out normal values. CONCLUSIONS: There were no significant differences in relation to age, gender, stature at birth, jaundice, acholia/hipocholia, choluria, hepatomegaly, splenomegaly, AST, ALT, ALP, GGT, BD and albumin. Birth weight and the weight during the first medical visit were lower in the group with infectious etiology. In addition, a significant difference in INR reflected impaired coagulation of patients of the group of the genetic-endocrine-metabolic disease.

Antibody titers and response to vaccination against hepatitis A and B in pediatric patients with portal hypertension.

In Brazil, approximately 130 new cases of hepatitis A per 100,000 inhabitants occur annually and 15% of the population has been in contact with hepatitis B virus. Portal hypertension causes hypersplenism and reduces T cell production, which may lead to less effective response to hepatitis vaccination. The objective of the study was to evaluate the response to hepatitis A and B vaccination in patients with portal hypertension secondary to chronic liver disease or portal vein thrombosis. Twenty-three patients (2 to 18 years) with portal hypertension seen at the Pediatric Hepatology Service of Hospital das Clínicas, Universidade Estadual de Campinas, between 1994 and 2006 were studied. Hepatitis A and B serology was tested in all patients. Patients who had not been vaccinated before their visits received the vaccines during the study period. Patients who had been vaccinated before but had negative anti-HB antibodies received a booster dose, and their serology was repeated Blood counts were performed in each patient to assess for immunosuppression. Eighteen patients received hepatitis A vaccine and all became positive for anti-HAV antibodies. All patients had received hepatitis B vaccine and 17 (73.9%) were anti-HBs positive at the time of the study The other 6 received a booster dose and became anti-HBs positive afterward. The anti-HBs-positive and -negative patients did not differ significantly in age, leukocytes, lymphocytes, or duration between the vaccination and positive serology. In this study, hepatitis A vaccines elicited a 100% response and hepatitis B vaccine conferred protection and induced an anamnestic response in pediatric patients with portal hypertension.

Selenium supplementation in pediatric patients using parenteral nutrition: Is it time to do something?

OBJECTIVE: To analyze the nutritional status of selenium and verify the effect of its supplementation in pediatric patients during 14 days of parenteral nutrition (PN). METHOD: This is a series of cases with patients followed for two weeks while using PN. Data collection was performed at the beginning (T0), in the 7th (T1) and 14th days of PN (T2). The supplemented group received 2 µg/kg/day of selenous acid. Weight and height were measured for nutritional status assessment. Tests requested: plasma selenium, albumin, pre-albumin, C-reactive protein (CRP), total cholesterol and HDL-cholesterol. RESULTS: Fourteen (14) patients with inflammatory process and with low or very low weight for their ages were evaluated. In both groups (with and without supplementation), all patients had low selenium levels. Median plasma selenium concentrations were 17.4 µg/L (T0), 23.0 µg/L (T1) and 20.7 µg/L (T2). Increase and reduction of selenium occurred both in patients with high CRP and in those presenting normalization of this parameter. CONCLUSION: Lower plasma selenium levels have been detected since the start of the research and supplementation (2 µg/kg/day of selenous acid) was not to enough to approach the reference values.

Transthyretin levels: Potential biomarker for monitoring nutritional support efficacy and clinical complications risk in patients receiving parenteral nutrition.

Nutritional support is an effective strategy to restore or maintain nutritional status, to reduce clinical complications, hospitalization period and the morbidity/mortality risk of hospitalized patients. So, a good marker is important to evaluate the nutritional support. OBJECTIVES: This study aims to evaluate the evolution of transthyretin levels in patients receiving parenteral nutrition (PN) during 14 days. DESIGN & METHODS: Longitudinal study of 88 hospitalized patients. The assessments and samples were taken during the first 72 h (T0), on the 7th day (T7) and 14th day (T14) of PN. This study was approved by the Ethics Committee of the School of Medical Sciences at UNICAMP (No 538/2011). RESULTS: The C-reactive protein (CRP) levels were high and albumin and transthyretin levels were low at baseline. From T0 to T14, only transthyretin increased (p = 0.03). According to the receiver operation characteristic (ROC) curve, we found that the transthyretin had some improvement when the CRP levels were less than 10.4 mg/dl (T7). According to the CRP/albumin ratio, all patients classified as without risk for complications were discharged from the hospital. In addition, we observed that patients with transthyretin reduction had a concomitant higher risk for complications according to their ratio CRP/albumin (p = 0.03). CONCLUSIONS: CRP/albumin ratio was associated with the evolution of transthyretin levels. Transthyretin values showed significant improvement in the 14 days of PN. Especially, less inflamed patients (ie CRP less than 10.4 mg/dl) improved their transthyretin levels. So, CRP value at day 7 that predicts the transthyretin and transthyretin is a good biomarker for classification of nutritional support and clinical complications risk in patients receiving PN.

IMPORTANCE OF LIVER BIOPSY IN THE DIAGNOSIS OF LYSOSOMAL ACID LIPASE DEFICIENCY: A CASE REPORT. / IMPORTÂNCIA DA BIÓPSIA HEPÁTICA NO DIAGNÓSTICO DA DEFICIÊNCIA DE LIPASE ÁCIDA LISOSSOMAL: RELATO DE CASO.

OBJECTIVE: To describe a case of cholesteryl ester storage disease (CESD) and discuss the importance of liver biopsy for diagnosis. CASE DESCRIPTION: A female patient, aged two years and ten months, presented with an increased abdominal volume following hepatomegaly for four months. Abdominal ultrasound demonstrated hepatomegaly and hepatic steatosis. Laboratory tests showed elevated liver serum enzymes and dyslipidemia. Liver biopsy was consistent with CESD. COMMENTS: Although measuring enzyme activity is the gold standard for CESD diagnosis, liver biopsy is very helpful when investigating suspected cases of CESD, particularly upon other differential diagnoses to be considered.

OBJETIVO: Descrever a doença de depósito de ésteres de colesterol (DDEC) e a importância da biópsia hepática na realização do diagnóstico. DESCRIçÃO DO CASO: Paciente feminina, dois anos e dez meses de idade, com queixa de aumento do volume abdominal secundário à hepatomegalia há quatro meses. Ultrassonografia abdominal demonstrou hepatomegalia e esteatose hepática. Exames laboratoriais mostraram aumento de enzimas hepáticas e dislipidemia. A biópsia hepática foi compatível com DDEC. COMENTÁRIOS: Embora a medida da atividade enzimática seja o padrão-ouro para o diagnóstico de DDEC, a biópsia hepática é muito útil na investigação de casos suspeitos, particularmente quando há outros diagnósticos diferenciais a serem considerados.

[Is plasma selenium correlated to transthyretin levels in critically ill patients?]

BACKGROUND: Selenium is an essential trace element, but critically ill patients using total parenteral nutrition (PN) do not receive selenium because this mineral is not commonly offered. Threfore, the eval uation of plasma selenium levels is very important for treating or preventing this deficiency. Recent studies have shown that transthyretin may reflect the selenium intake and could be considered a biomarker. However, this issue is still little explored in the literature. OBJECTIVE: This study aims to investigate the correlation of transthyretin with the plasma selenium of critically ill patients receiving PN. METHOD: This was a prospective cohort study with 44 patients using PN without selenium. Blood samples were carried out in 3 stages: initial, 7th and 14th day of PN. In order to evaluate the clinical condition and the inflammatory process, albumin, C-reactive protein (CRP), transthyretin, creatinine and HDL cholesterol levels were observed. To assess the selenium status, plasma selenium and glutathione peroxidase (GPx) in whole blood were measured. Descriptive analyses were performed and the ANOVA, Mann-Whitney and Spearman's coefficient tests were conducted; we assumed a significance level of 5%. RESULTS: A positive correlation of selenium with the GPx levels (r = 0.46; p = 0.03) was identified. During two weeks, there was a positive correlation of transthyretin with plasma selenium (r = 0.71; p = 0.05) regardless of the CRP values. CONCLUSION: Transthyretin may have reflected plasma selenium, mainly because the correlation was verified after the acute phase.

Influence of selenium supplementation on patients with inflammation: A pilot double blind randomized study.

OBJECTIVE: The aim of the study was to analyze the effect of selenium supplementation on patients with inflammation receiving PN. METHODS: This double-blind randomized study included 20 hospitalized patients experiencing an inflammatory process while being fed by PN, who were monitored in three stages: first 72 h (0), day 7 (1), and day 14 (2) of PN. The supplemented patients group (G+S) received 60 µg/d (0.75 µmol) of selenium as selenious acid which was added to the PN bag. The nonsupplemented group (G-S) did not receive selenium. The concentration range of 84 to 100 µg/L (1.07-1.27 µmol/L) was used as a reference of plasma selenium. RESULTS: The study included 20 patients (8 G+S and 12 G-S) mainly diagnosed with cancer and/or sepsis. Most of them were hospitalized in the intensive care unit and were receiving PN for clinical reasons. Plasma selenium was greater in the G+S than in the G-S (P = 0.05) in two stages (0 and 1). Since the start of assessment, C-reactive protein (CRP) levels were elevated; however, there was no statistical difference in CRP values between groups (P > 0.05). There was no significant change of glutathione peroxidase over time or between groups (P > 0.05). CONCLUSION: The selenium concentration was greater in the G+S than in the G-S, acting independently from CRP behavior. However, supplementation was not enough to reach the reference values.