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.

FACTORS ASSOCIATED WITH A HIGHER NUMBER OF ESOPHAGEAL DILATIONS IN CHILDREN WITH A HISTORY OF ALKALINE INGESTION.

BACKGROUND: Children who experience alkaline injury are at risk for the development of esophageal strictures and the need for esophageal dilations. OBJECTIVE: We aimed to assess predictors for a higher number of esophageal dilatations in children following alkali ingestion. METHODS: Single-center retrospective cohort study including children who underwent esophagogastroduodenoscopy (EGD) after alkali ingestion. Possible predictive factors for the need for esophageal dilatations were evaluated. RESULTS: A total of 34 patients were included, and 19 were female (55.9%). The median age at the time of the accidents was 20.6 months (IQR 15-30.7). All alkali ingestions were accidental, in all cases involving liquid products, and most (24/34; 70%) occurred at the child's home. Homemade liquid soap was the agent in half of the cases. The most frequently reported symptom at presentation was vomiting (22/34, 64.7%). The median follow-up time was 3.2 years (IQR 1.1-7.4). On follow-up, the median number of esophageal dilatations required for these patients was 12.5 (IQR 0-34). Among demographic factors, male gender (P=0.04), ingestion of homemade products (P<0.01), and accidents happening outside of the household environment (P=0.02) were associated with a greater number of esophageal dilations on follow-up. An endoscopic classification Zargar of 2B or higher (P=0.03), the presence of stricture at the time of the second EGD (P=0.01), and gastroesophageal reflux disease (GERD) as a late complication (P=0.01) were also associated with a greater number of esophageal dilations on long term follow-up. CONCLUSION: Beyond the endoscopic classification severity - a well-known risk factor for the strictures after alkali ingestions, we found that male gender, accidents with homemade products, and accidents occurring outside the household environment were significantly associated with a greater number of esophageal dilatations in the long-term follow-up of children following alkali ingestion.

Outcomes and factors associated with tolerance in infants with non-IgE-mediated cow's milk allergy with gastrointestinal manifestations.

OBJECTIVES: To evaluate outcomes of oral food challenge (OFC) test to assess tolerance in infants with non-IgE-mediated cow's milk allergy (CMA) with gastrointestinal manifestations and explore clinical data predictive of these outcomes. METHODS: Single-center retrospective study including infants (age < 12 months) who were referred for CMA between 2000 and 2018 and underwent OFC on follow-up. A univariate logistic regression test was performed to evaluate variables associated with the outcomes of the follow-up OFC test. RESULTS: Eighty-two patients were included, 50% were male. Eighteen patients had a positive OFC test (22%). Most patients had presented with hematochezia (77%). The median age of symptom onset was 30 days. Two-thirds of the patients were on appropriate infant formula (extensively hydrolyzed or amino acid-based formula), exclusively or in association with breastfeeding. The median time on an elimination diet before the OFC test was 8 months (Q1 6 - Q3 11 months). All cases with positive follow-up OFC tests (n = 18) had been exposed to cow's milk-based formula before the first clinical manifestation of CMA. Five out of eight cases with Food Protein-Induced Enterocolitis Syndrome (FPIES) had positive OFC tests. Exposure to cow's milk-based formula before diagnosis, a history of other food allergies, hematochezia and diarrhea were predictors of a positive OFC test. CONCLUSIONS: In infants with non-IgE-mediated CMPA with gastrointestinal manifestations, the use of cow's milk-based formula, a history of other food allergies, and hematochezia and diarrhea upon initial presentation were associated factors for the later achievement of tolerance.

FUNCTIONAL ABDOMINAL PAIN IS THE MAIN ETIOLOGY AMONG CHILDREN REFERRED TO TERTIARY CARE LEVEL FOR CHRONIC ABDOMINAL PAIN.

BACKGROUND: Chronic abdominal pain (CAP) carries a significant burden of disease. The last edition of the Rome Criteria (Rome IV) allows the diagnosis of functional gastrointestinal disorders (FGIDs) according to symptoms-based criteria; however, patients continue to experience a delay in their diagnosis and to be submitted to different interventions before the establishment of a positive diagnosis. OBJECTIVE: We aimed to characterize etiology, clinical features, and interventions in a pediatric cohort of patients with CAP secondary to FGIDs, who were referred to our tertiary care university-affiliated hospital, in Brazil. METHODS: A retrospective descriptive study of children and adolescents (aged 20 years and younger) referred to our institution, from January/2013 to December/2018, for CAP, and who fulfilled criteria for FGIDs classified according to Rome IV criteria. RESULTS: Three hundred twenty-eight patients with CAP were screened, of which 67.9% (223 patients) fulfilled the criteria for FGIDs and were included in the study. Sixty percent were female, with a mean age of 8.3 years. At the time of referral, the mean duration of symptoms was 2.8 years. Length/height for age and weight for age mean z-scores were -0.08±1.87 and -0.38±1.62, respectively. Functional abdominal pain not otherwise specified was overall the most common diagnosis (70.4%). Before establishing the diagnosis of FGIDs, multiple pharmacological interventions were described, while after, the mainstay of therapy was education/reassurance and dietary interventions. Thirty-two percent of patients did not further require specialized follow-up. CONCLUSION: Even at the tertiary care level, FGIDs were still the most common etiology of chronic abdominal pain, particularly functional abdominal pain not otherwise specified. Despite the relatively long duration of symptoms at referral, cessation of specialized care follow-up was possible in approximately a third of the cases.

Management of children with functional constipation referred to tertiary care.

OBJECTIVES: To describe the management, to compare treatment at initial referral vs. during specialized follow-up, and to describe outcomes of children with functional constipation (FC) referred to a Brazilian tertiary care center. METHODS: Retrospective study, including children (4-18 years) with FC followed at a single center from 2006 to 2019. Demographics, treatments, time of follow-up, and outcomes were analyzed. The management of FC followed an institutional protocol. RESULTS: 104 patients were identified, 79 were eligible and included in the analysis: 59% male, mean age at referral was 6.4 years, and mean duration of symptoms was 4.4 years. There were significant changes in the therapy(ies) used at the time of referral compared to during follow-up, with a noticeable increase in the frequency of the use of polyethylene glycol, enemas, magnesium hydroxide, and bisacodyl; 5.1% received trans-anal irrigation, and 3.8% underwent surgery. Outcomes were favorable in more than half of the cases: 31% improved; 19.5% had complete resolution and 2.5% were transferred back to primary care. Symptoms remained unchanged in 30.4%, and no patients experienced worsening of symptoms. The mean duration of follow-up was 2.8 years. When comparing patients with favorable vs. unfavorable outcomes, the authors did not identify significant differences in gender, age, therapies used, duration of symptoms, or length of follow-up. CONCLUSIONS: Children with FC are often referred to specialized care not receiving optimal therapy. Many patients whose FC was labeled "refractory" may be treated successfully with a well-established plan of care, and do not truly present intractable constipation.

INDUCED PROCTOCOLITIS - ORAL FOOD CHALLENGE SHOULD BE DONE TO CONFIRM THE DIAGNOSIS OF COW'S MILK ALLERGY IN NEONATES?

BACKGROUND: Suspicion of food protein-induced proctocolitis based on empirical understanding of rectal bleeding can lead to misdiagnosis. OBJECTIVE: to verify clinical and evaluative characteristics of patients who presented neonatal rectal bleeding and were on a restricted cow's milk diet. METHODS: A cross-sectional retrospective study included patients followed up in a tertiary care center, who presented rectal bleeding in the neonatal period. The analyzed data included gender, gestational age, type of delivery, use of antibiotics during the last trimester of pregnancy, use of parenteral nutrition before the first manifestation, use of mechanical ventilation, initial clinical manifestations associated with rectal bleeding, diet before the first manifestation, period of elimination diet, oral food challenge (OFC) results and symptoms presented in cases of positive OFC. Fisher's exact test and Mann-Whitney test were used to analyze the data. The level of significance was set to 5%. RESULTS: Forty-two patients were selected: 30 preterm infants, 34 cesarean deliveries, 10 exclusively breastfed patients before rectal bleeding. Median age at OFC was 6.3 months old. Median of length of the elimination period before OFC was 5.9 months. OFC was negative in 33/42 (79%) patients and positive in 9/42 (21%). There was no association between OFC results and the evaluated data. The main symptom observed in patients with positive OFC was blood in stools. CONCLUSION: OFC was negative in most cases of suspected cow's milk allergy due to rectal bleeding in neonates, most of them with a history of prematurity.

Patients with treated autoimmune hepatitis and persistent suppression of plasmacytoid dendritic cells: A different point of view.

Objectives: Plasmacytoid dendritic cells (pDCs) have been shown to have a role in autoimmune diseases, but their role in Autoimmune Hepatitis (AIH) is not completely clear. In the present study, we assessed the frequency of pDCs in peripheral blood of AIH patients under long-term standard immunosuppressive therapy. Methods: This cross-sectional analysis enrolled 27 AIH patients and 27 healthy controls. We analyzed and compared their proportion of pDCs, CD4+, CD8+, γδ T cells, CD25+ regulatory T (Treg) cells, FoxP3+, Foxp3+CD39+ Treg cells, total B (CD19+) cells, and plasma cells (CD38+) in peripheral blood using flow cytometry immunophenotyping. Results: AIH patients had a lower percentage of pDCs (median frequencies of 0.2% vs. 0.4%; p = .002) and higher expression of CD8 T cells (32.5% vs 28.6%; p = 0.008) in peripheral blood, when compared to healthy controls. We did not find statistically significant differences between the groups regarding the other cell subtypes.Conclusion: Our data suggest a persistent suppression of pDCs in AIH patients, along with increased CD8 T cell activity, years after AIH diagnosis and despite of good clinical response to treatment, thus pointing to a role of pDCs in the AIH pathogenesis.

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.

GASTROESOPHAGEAL REFLUX DISEASE IN INFANTS WHO PRESENTED BRIEF RESOLVED UNEXPLAINED EVENT (BRUE).

BACKGROUND: The term brief resolved unexplained events (BRUE) is a description of the acute event occurring in infants less than 1-year-old that includes at least one of the following characteristics: cyanosis or pallor; absent, decreased, or irregular breathing; marked change in tone or altered level of responsiveness. An investigative proceeding is required to identify the triggering phenomenon in those who are at high risk of complications. Prolonged esophageal pHmetry has been used as a tool in searching for gastroesophageal reflux disease (GERD) as one of the underlying etiologies. OBJECTIVE: The study aims to verify the frequency of GERD in infants up to 1-year-old, when pHmetry has been performed for investigating high-risk BRUE (HR-BRUE) and to analyze if clinical characteristics or any particular symptom related by caregivers during BRUE could be correlated to GERD. METHODS: It was performed a cross-sectional study. The data was collected retrospectively of patients less than 1-year-old, who had performed pHmetry in a tertiary hospital for investigating HR-BRUE between October 2008 and January 2018. For the analysis of medical records, a data collection protocol included: gender, age at the first HR-BRUE episode, age at the time of the pHmetry, gestational age, type of delivery (normal or caesarean) and birth weight and symptoms associated to HR-BRUE related by caregivers. Relation between variables were assessed using Fisher's exact test and Mann-Whitney test. The significance level was set at 0.05. RESULTS: A total of 54 infants were included (preterm 25, term 29), 62.9% males, median age at the HR-BRUE was 36 days, 53.7% HR-BRUE episodes had occurred during or right after feeding. According to pHmetry results: nine pHmetry results were considered inconclusive, physiological reflux (n=30) and GERD (n=15). The frequency of GERD diagnosed by pHmetry was 33%. GERD was not statistically related to gender (P-value=0.757), age at first HR-BRUE episode (P-value=0.960), age at the time of the pHmetry (P-value=0.720), prematurity (P-value=0.120) or type of delivery (P-value=0.738). GERD was statistically related to low birth weight (P-value=0.023). There was no association between symptoms reported by caregivers during HR-BRUE and GERD. CONCLUSION: GERD diagnosed by the pHmetry was found in one third of infants that experiencing a HR-BRUE, showing the importance of properly investigation. In half of infants BRUE occurred during or right after feeding. Besides low birth weight, it was not possible to select other data from the clinical history that suggest that these patients would be more likely to have GERD.

PEUTZ-JEGHERS SYNDROME IN RESOURCE-LIMITED SCENARIO.

BACKGROUND: Peutz-Jeghers Syndrome (PJS) is a rare, autosomal dominant disease, caused by deletions in the chromosome 19p33.3/ gene LKB1/STK11. These mutations inactivate a serine/threonine kinase and predispose to carcinogenesis. In PJS, tumors of the gastrointestinal, testicles, pulmonary, breast, pancreas, uterus and ovaries can be found. OBJECTIVE: To evaluate demographics, clinical presentation and complication/outcomes of pediatric patients presenting with Peutz-Jeghers syndrome (PJS), as well as to present and discuss management in the context of limited resources. METHODS: We conducted a retrospective chart review of a cohort of six patients, who were diagnosed and/or followed at the Clinics Hospital, University of Campinas - Sao Paulo/Brazil, between 2000 and 2018. Data analyzed included gender, age of presentation, age of diagnosis, family history, PJS complications. RESULTS: Median age of diagnosis of 6.7 years, with a mean time of follow-up of 8.1 years. Mucocutaneous pigmentation was universally present. Half of the patients had a known family history at the time of diagnosis. On follow up, intestinal intussusception was documented in four out of six patients, in most (three), in different locations and in multiple occasions. The active investigation of siblings and parents of the index case led to the diagnosis of three first-degree relatives in the present case series. CONCLUSION: In this first pediatric PJS Brazilian case series, we report a wide spectrum of PJS manifestations and complications. In a resource limited scenario, despite limitations for the surveillance of complications, the relative frequency of complications was not higher than historically reported.

QUALITY OF LIFE IN CAREGIVERS OF PEDIATRIC PATIENTS WITH CEREBRAL PALSY AND GASTROSTOMY TUBE FEEDING.

BACKGROUND: Cerebral palsy is the most common cause of physical disability in childhood. Caregivers of patients presenting tetraparesis cerebral palsy (TCP) and gastrostomy tube feeding (GTF) were selected for this study because both conditions represent a great demand for their caregivers. OBJECTIVE: To describe the quality of life related to the state of health of caregivers of patients with TCP who were fed by gastrostomy, to assess the results linked to the mental health of these caregivers, to compare our data with data from other studies on children with cerebral palsy without gastrostomy and to evaluate the possible interference of gastrostomy in the quality of life. METHODS: A number of 30 major caregivers were interviewed and assessed. Quality of life and mental health tools applied and analyzed only for caregivers were: Medical Outcomes Study (MOS) 36-item Short Form Health Survey (SF-36), WHOQOL-BREF and Beck scales. Other information (age, gender, marital status, number of residents per household and psychological support) was evaluated. The Spearman's rank correlation coefficient was used to analyze. A 5% significance level was adopted. RESULTS: Results obtained through questionnaires are as follows: moderate hopelessness in 20% of caregivers (the higher the number of residents per household the higher the level of caregiver's hopelessness); moderate and severe anxiety in 33.33% of the sample studied; moderate and severe depression identified in 46.67% of interviewed caregivers; health-related quality of life of caregivers of patients with TCP were found to be below world averages; no significant figures for suicide potential were noted for the population under this study. CONCLUSION: The HRQOL of caregivers of TCP patients who were fed by gastrostomy is below the average of the general population. Our results are very similar to those found in other studies that evaluated caregivers of patients with cerebral palsy with different degrees of neurological impairment and no report of using GTF, suggesting that the presence of gastrostomy did not negatively interfere with the caregiver's HRQOL.

CASE-CONTROL STUDY ON VITAMIN D STATUS IN CHILDREN AND ADOLESCENTS WITH EOSINOPHILIC ESOPHAGITIS.

BACKGROUND: Vitamin D is an essential fat-soluble steroid hormone and vitamin D deficiency is a global public health problem especially among children and adolescents. Factors such as the low intake of vitamin D-rich food sources, poor absorption and less exposure to the sun influence this outcome. Vitamin D has an anti-inflammatory effect in the body by promoting regulatory T cell differentiation as well as recovering T helper 17 cell response and secretion of anti-inflammatory cytokines. Eosinophilic esophagitis (EoE) is a chronic disease, histologically characterized by predominantly eosinophilic inflammation. The most common therapeutic approaches are allergen-eliminating diets, such as excluding cow's milk, egg, soy, wheat, peanuts and seafood, or more specific dietary restrictions. OBJECTIVE: To verify the serum levels of vitamin D in children and adolescents with eosinophilic esophagitis on a restricted food diet and to analyze their association with nutritional status, consumption of different food sources, exposure to the sun and skin color. METHODS: Case-control study conducted in the city of Campinas-SP, Brazil, in which included patients were aged 2 to 18 years old, and those diagnosed with eosinophilic esophagitis was referred to as the case group (n=15), meanwhile a control group (n=17) was also formed. Epidemiological data, nutritional status, data on vitamin D intake (24-hour recall - performed only by EoE patients - and self-reported intake of vitamin D food sources: milk and dairy products, canned tuna and sardines, Bull's liver, chicken eggs - applied in both groups), and daily time of sun exposure (≥30 min or ≤30 min) were recorded. The samples were collected for serum levels of 25-hydroxy-vitamin D, where sufficiency levels >30 ng/mL were considered, insufficiency 21 to 30 ng/mL, deficiency <20 ng/mL. RESULTS: There was a higher frequency of vitamin D insufficiency/ deficiency in the Eosinophilic Esophagitis group (P=0.035), even with longer sun exposure (P= 0.035). Skin color was not associated with lower levels of vitamin D in both groups studied. No difference was found in nutritional status between the groups. CONCLUSION: The present study demonstrated a higher frequency of inadequate/ deficient levels of vitamin D in children and adolescents with EoE on a restricted diet. When necessary, serum levels should be investigated and correct exposure to the sun should be encouraged, with special attention to the recommended guidelines, time spent in the sun and the appropriate clothing for correct absorption. Since exposure for more than 30 minutes in the sun does not appear to have provided a protective effect in the EoE group, even in a region with high levels of solar radiation. There was a significant difference only in the consumption of cow's milk between the case and control groups, demonstrating the low adherence to the restriction diet by the case group. No association was found between serum 25 hydroxyvitamin D levels and nutritional status. Moreover, no association regarding the adequate or inadequate status of 25 hydroxyvitamin D and the consumption vitamin D-rich foods was identified. Multicentered studies with a larger number of cases should be performed to assess serum 25 hydroxyvitamin D levels and associated factors in pediatric patients with EoE.

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.

Outcomes and factors associated with tolerance in infants with non-IgE-mediated cow's milk allergy with gastrointestinal manifestations

Abstract Objectives To evaluate outcomes of oral food challenge (OFC) test to assess tolerance in infants with non-IgE-mediated cow's milk allergy (CMA) with gastrointestinal manifestations and explore clinical data predictive of these outcomes. Methods Single-center retrospective study including infants (age < 12 months) who were referred for CMA between 2000 and 2018 and underwent OFC on follow-up. A univariate logistic regression test was performed to evaluate variables associated with the outcomes of the follow-up OFC test. Results Eighty-two patients were included, 50% were male. Eighteen patients had a positive OFC test (22%). Most patients had presented with hematochezia (77%). The median age of symptom onset was 30 days. Two-thirds of the patients were on appropriate infant formula (extensively hydrolyzed or amino acid-based formula), exclusively or in association with breastfeeding. The median time on an elimination diet before the OFC test was 8 months (Q1 6 - Q3 11 months). All cases with positive follow-up OFC tests (n= 18) had been exposed to cow's milk-based formula before the first clinical manifestation of CMA. Five out of eight cases with Food Protein-Induced Enterocolitis Syndrome (FPIES) had positive OFC tests. Exposure to cow's milk-based formula before diagnosis, a history of other food allergies, hematochezia and diarrhea were predictors of a positive OFC test. Conclusions In infants with non-IgE-mediated CMPA with gastrointestinal manifestations, the use of cow's milk-based formula, a history of other food allergies, and hematochezia and diarrhea upon initial presentation were associated factors for the later achievement of tolerance.

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.

Pegylated interferon for treating severe recurrent respiratory papillomatosis in a child: case report.

CONTEXT: Recurrent respiratory papillomatosis (RRP) is the most common laryngeal tumor. During childhood, it may present in extremely severe forms defined by the need for frequent surgical procedures to relieve respiratory distress and/or involvement of extralaryngeal sites such as lung involvement. Adjuvant therapies are indicated in these cases and interferon is one of the options. Pegylated interferon is more effective than conventional alpha interferon and, given its reported results in relation to treating hepatitis C over the past decade, we hypothesized that this might be more effective than conventional interferon also for treating respiratory papillomatosis. Use of a treatment strategy that eliminates the need for general anesthesia is particularly appealing, yet obtaining approval for use of medications that are not currently used for this purpose is challenging. CASE REPORT: We report the case of a child with severe RRP that had been followed for the preceding six years, who was treated with pegylated interferon after failure of other adjuvant therapies. There was noticeable improvement in the frequency of surgical procedures, which was regarded very receptively, considering the child's history and previous response to other therapies. CONCLUSION: Pegylated interferon may be a good option for diminishing the need for surgical intervention in severe cases of recurrent respiratory papillomatosis.

Comparative study on gastrostomy and orally nutrition of children and adolescents with tetraparesis cerebral palsy.

BACKGROUND: Gastrostomy tube feeding (GTF) is indicated for children with feeding difficulties due to tetraspastic cerebral palsy, although there are no definitive conclusions about the benefits of GTF. OBJECTIVE: To compare nutritional status and diet of pediatric patients with tetraparesis cerebral palsy who are fed by GTF with those fed orally (PO). METHODS: A transversal and descriptive study on 54 patients with spastic tetraparesis was held. The referred parameters were: weight, knee height and estimated height, cutaneous folds and circumferences. The Frisancho reference was used to compare the skin folds and body circumferences. The Brooks et al. curve was adopted as a reference for weight, height, and BMI. Food inquiry was performed using the Habitual Dietary Recall method. The total energetic value (TEV) of macronutrients and fibers was performed by Avanutri® version 4.0, a nutrition software program. The differences of nutritional parameters between the GTF and the PO groups were calculated by chi-squared and Fisher's exact tests, and the comparison between the groups for variable numbers was performed using the Mann-Whitney test. The significance level adopted was 5%. RESULTS: The PO group presented more individuals in the malnourished range (24.14%) and high levels of adipose and thin mass depletion. The ingestion of lipids was larger in the GTF group, even though the proteins and fibers were higher in the PO group. The comparison between the diets in the GTF group indicated that the mixed diet (industrialized and artisanal) supplied a greater contribution of proteins and fibers. CONCLUSION: Comparing the groups, the tetraparesis cerebral palsy patients fed orally have a greater impairment of their nutritional status, even though they have higher intakes of protein and fiber than those patients fed by gastrostomy, demonstrating a consistent argument for the use of gastrostomy.

ASSESSMENT OF NUTRITIONAL STATUS OF CHILDREN AND ADOLESCENTS WITH SPASTIC QUADRIPLEGIC CEREBRAL PALSY.

BACKGROUND:: Due to several factors, such as gastrointestinal's diseases and difficulty in feeding, children with Spastic Quadriplegic Cerebral Palsy tend to present nutritional deficits. OBJECTIVE:: To assess the nutritional status of pediatric patients with Spastic Quadriplegic Cerebral Palsy according to reference curves for this population and with the measures of folds and circumferences, obtained by the upper arm circumference and triceps skin fold. METHODS:: The data were obtained from: knee-height, estimated height, weight, upper arm circumference, and triceps skin fold. Values of folds and circumferences were compared with Frisancho, and specific curves for these patients were used as reference. The relationship between the values in the growth curve for healthy children, Z-Score, and comparison with the reference curve were verified by Fisher's exact test. We adopted the significance level of 5%. RESULTS:: We evaluated 54 patients. The mean age was 10.2 years, and 34 were male, 25 fed by gastrostomy and 29, orally. The frequency of low weight by the reference curve was 22.22%. More than half of the patients presented the parameters indicating lean mass below the 5th percentile. The height of all patients was classified as adequate for the age by the reference curve. CONCLUSION:: Low weight was found in 22% of patients, and there is a greater tendency to present reduced muscle mass and increased fat mass, showing the need for evaluation and appropriate interventions for patients with Spastic Quadriplegic Cerebral Palsy.

Dor abdominal funcional é a principal etiologia em crianças encaminhadas ao nível de atenção terciária por dor abdominal crônica / Functional abdominal pain is the main etiology among children referred to tertiary care level for chronic abdominal pain

ABSTRACT Background Chronic abdominal pain (CAP) carries a significant burden of disease. The last edition of the Rome Criteria (Rome IV) allows the diagnosis of functional gastrointestinal disorders (FGIDs) according to symptoms-based criteria; however, patients continue to experience a delay in their diagnosis and to be submitted to different interventions before the establishment of a positive diagnosis. Objective We aimed to characterize etiology, clinical features, and interventions in a pediatric cohort of patients with CAP secondary to FGIDs, who were referred to our tertiary care university-affiliated hospital, in Brazil. Methods A retrospective descriptive study of children and adolescents (aged 20 years and younger) referred to our institution, from January/2013 to December/2018, for CAP, and who fulfilled criteria for FGIDs classified according to Rome IV criteria. Results Three hundred twenty-eight patients with CAP were screened, of which 67.9% (223 patients) fulfilled the criteria for FGIDs and were included in the study. Sixty percent were female, with a mean age of 8.3 years. At the time of referral, the mean duration of symptoms was 2.8 years. Length/height for age and weight for age mean z-scores were -0.08±1.87 and -0.38±1.62, respectively. Functional abdominal pain not otherwise specified was overall the most common diagnosis (70.4%). Before establishing the diagnosis of FGIDs, multiple pharmacological interventions were described, while after, the mainstay of therapy was education/reassurance and dietary interventions. Thirty-two percent of patients did not further require specialized follow-up. Conclusion Even at the tertiary care level, FGIDs were still the most common etiology of chronic abdominal pain, particularly functional abdominal pain not otherwise specified. Despite the relatively long duration of symptoms at referral, cessation of specialized care follow-up was possible in approximately a third of the cases.

RESUMO Contexto A dor abdominal crônica (DAC) pode acarretar importante morbidade. A última edição dos Critérios de Roma (Roma IV) permite o diagnóstico de distúrbios gastrointestinais funcionais (DGIFs) de acordo com critérios baseados em sintomas; no entanto, esses pacientes continuam a apresentar atraso no diagnóstico e a serem submetidos a diferentes intervenções antes do estabelecimento de um diagnóstico. Objetivo Caracterizar a etiologia, características clínicas e intervenções de crianças com DAC que não são mantidas na atenção primária e que foram encaminhadas ao nosso hospital universitário de nível terciário, no Brasil. Métodos Estudo retrospectivo descritivo de crianças e adolescentes (com idade igual ou inferior a 20 anos) encaminhados a nossa instituição, entre janeiro/2013 e dezembro/2018, por DAC e que preenchiam os critérios para DGIFs conforme o consenso de Roma IV. Resultados Trezentos e vinte e oito pacientes com DAC foram triados, 67,9% (223 pacientes) preencheram os critérios para DGIFs e foram analisados. Sessenta por cento do sexo feminino, com idade média de 8,3 anos. A duração média dos sintomas no encaminhamento era de 2,8 anos. Os escores z médios de estatura para idade e peso para idade foram -0,08±1,87 e -0,38±1,62, respectivamente. Dor abdominal funcional sem outra especificação foi o diagnóstico mais comum (70,4%). Antes do diagnóstico de DGIFs, múltiplas intervenções farmacológicas foram descritas, enquanto depois, a base da terapia foi a educação, passar segurança à família e intervenções dietéticas. Trinta e dois por cento dos pacientes apresentaram resolução dos sintomas e receberam alta do acompanhamento especializado. Conclusão Mesmo no nível terciário, as desordens gastrointestinais funcionais ainda constituem a etiologia mais comum da DAC, particularmente a dor abdominal funcional não especificada. Apesar da duração longa dos sintomas, alta do serviço especializados foi possível em aproximadamente um terço dos casos.