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1.
Int J Mol Sci ; 25(16)2024 Aug 08.
Article in English | MEDLINE | ID: mdl-39201333

ABSTRACT

Lysosomal acid lipase deficiency (LALD) varies from a severe infantile-onset form (Wolman disease) to a late-onset form known as cholesteryl ester storage disease (CESD), both of which are autosomal recessive disorders caused by biallelic LIPA pathogenic variants. We evaluated seventy-three patients enlisted for liver transplant (LT) at Instituto da Criança (HCFMUSP-Brazil) who were subjected to LAL activity measurement and LIPA Sanger sequencing analysis, resulting in a positive LALD diagnosis for only one of these individuals. This LALD patient presented recurrent diarrhea, failure to thrive, hepatomegaly, and dyslipidemia at the age of 4 months and liver failure by the age of 13 years. The LALD diagnosis confirmation was conducted at 24 years old due to low levels of LAL enzyme activity. The causal homozygous variant LIPA(NM_000235.4):c.266T>C(p.Leu89Pro) was identified, but the patient had already undergone his first LT at 18 years with several rejection episodes. Despite beginning treatment with sebelipase alfa at 26 years old (total of five infusions), this patient died at 28 years from complications after his second liver transplant. LALD is an important differential diagnosis in cases presenting with hepatomegaly, elevated liver enzymes, and dyslipidemia. Detecting low/absent LAL activity and identifying the LIPA causal variant are essential for diagnosis and specific treatment, as well as for appropriate genetic counseling. Early diagnosis, along with sebelipase alfa therapy, may improve the prognosis of affected patients.


Subject(s)
Liver Transplantation , Sterol Esterase , Wolman Disease , Humans , Wolman Disease/genetics , Wolman Disease/diagnosis , Male , Sterol Esterase/genetics , Sterol Esterase/deficiency , Female , Adolescent , Infant , Adult , Child, Preschool , Child , Young Adult
2.
Fetal Diagn Ther ; 50(4): 259-268, 2023.
Article in English | MEDLINE | ID: mdl-37379809

ABSTRACT

INTRODUCTION: Abdominal wall defects (AWDs) interfere with postnatal respiratory parameters. We aimed to evaluate lung volume (LV) in fetuses with AWD using three-dimensional (3D) ultrasound (US) and to correlate AWD with the type (omphalocele and gastroschisis) and size of the defect and neonatal morbidity and mortality. METHODS: This prospective observational study included 72 pregnant women with fetuses with AWD and a gestational age <25 weeks. The data on abdominal volume, 3D US LV, and herniated volume were acquired every 4 weeks up to 33 weeks. LV was compared with normal reference curves and correlated with abdominal and herniated volumes. RESULTS: Omphalocele (p < 0.001) and gastroschisis (p < 0.001) fetuses had smaller LV than normal fetuses. LV was positively correlated with abdominal volume (omphalocele, r = 0.86; gastroschisis, r = 0.88), whereas LV was negatively correlated with omphalocele-herniated volume/abdominal volume (p < 0.001, r = -0.51). LV was smaller in omphalocele fetuses that died (p = 0.002), were intubated (p = 0.02), or had secondary closure (p < 0.001). In gastroschisis, a smaller LV was observed in fetuses discharged using oxygen (p = 0.002). CONCLUSION: Fetuses with AWD had smaller 3D LV than normal fetuses. Fetal abdominal volume was inversely correlated with LV. In omphalocele fetuses, a smaller LV was associated with neonatal mortality and morbidity.


Subject(s)
Abdominal Wall , Digestive System Abnormalities , Gastroschisis , Hernia, Umbilical , Infant, Newborn , Pregnancy , Humans , Female , Infant , Gastroschisis/diagnostic imaging , Gastroschisis/complications , Hernia, Umbilical/complications , Abdominal Wall/diagnostic imaging , Fetus/diagnostic imaging
3.
Pediatr Transplant ; 26(4): e14226, 2022 06.
Article in English | MEDLINE | ID: mdl-35037358

ABSTRACT

BACKGROUND: PTLD is a clinical condition with high mortality. Monitoring EBV replication can be a useful tool to avoid the development of PTLD. MATERIALS AND METHODS: This was a retrospective analysis of 428 pediatric patients who underwent liver transplantation between 1989 and 2016. The patients were divided into 2 groups (transplanted before 2006, when PCR-EBV was not monitored, and after 2006, when PCR-EBV monitoring was started). Patients with continuous PCR measurements for EBV were evaluated for the impact of a reduction in immunosuppression or a change in immunosuppressants on the number of viral copies. A logistic regression model was applied to evaluate factors related to PTLD. RESULTS: The prevalence of PTLD was 4.2%. After monitoring patients with PCR for EBV levels, a predominance of the most severe, monomorphic form of lymphoproliferative disorder was observed (p = .009). The PTLD mortality was 5%. There was a change in the PCR level after tacrolimus reduction (p = .002) and after tacrolimus exchange for mTOR (p = .008). The number of EBV copies was significantly higher (p = .029) in patients who developed PTLD. In the multiple regression model, seropositivity for CMV was an independent protective factor for lymphoproliferative disorder (OR=0.09; 95% CI 0.02-0.42), reducing the chance of having PTLD adjusted by serology for EBV by 91%. CONCLUSIONS: Monitoring the EBV viral load by PCR seems to prevent the emergence of milder forms of lymphoproliferative disorder. Pretransplant seropositivity for CMV is a protective factor for PTLD.


Subject(s)
Cytomegalovirus Infections , Epstein-Barr Virus Infections , Liver Transplantation , Lymphoproliferative Disorders , Child , Cytomegalovirus/genetics , Cytomegalovirus Infections/complications , Cytomegalovirus Infections/diagnosis , DNA, Viral , Epstein-Barr Virus Infections/complications , Epstein-Barr Virus Infections/diagnosis , Herpesvirus 4, Human/genetics , Humans , Lymphoproliferative Disorders/diagnosis , Lymphoproliferative Disorders/epidemiology , Lymphoproliferative Disorders/etiology , Polymerase Chain Reaction , Protective Factors , Retrospective Studies , Tacrolimus/therapeutic use , Viral Load
4.
J Vasc Bras ; 21: e20210221, 2022.
Article in English | MEDLINE | ID: mdl-35911477

ABSTRACT

The aim of this study was to report on use of ethanol lock in long-term catheters in newborns with severe intestinal dysfunction, dependent on total and prolonged parenteral nutrition, in a Neonatal Intensive Care Center (tertiary level), between 2015 and 2020. Six infants (0.65%) out of the 914 admitted during the period met the inclusion criteria. The median age at catheter placement was 121.5 days. Two Powerpicc (PICC Power Sinergy™, São Paulo), one Groshong (Groshong™ Central Venous Catheter BD, São Paulo), and three silicone catheters were used, all tunneled, and the median dwell duration was 182.5 days. Four patients had at least one episode of infection related to the central venous catheter, and Gram-positive, Gram-negative, and fungal agents were isolated. The median length of hospital stay was 555 days and mortality was 33.3%. The ethanol lock did not cause any side effects and was relatively effective in preventing infections related to the central venous catheter.

5.
Medicina (Kaunas) ; 56(3)2020 Mar 05.
Article in English | MEDLINE | ID: mdl-32151106

ABSTRACT

. Background and Objectives: Cirrhosis is a liver disease that causes about one million deaths annually worldwide. The estimated cirrhosis prevalence ranges from 4.5-9.5% in the general population. Up to 40% of cirrhotic patients are asymptomatic and may be diagnosed late. Studies have described the importance of the functions of the liver and autonomic nervous system (ANS) and their relationship. There is limited information available on non-alcoholic cirrhosis and heart rate variability (HRV), which is a measure of the ANS. This study aimed to evaluate cardiac autonomic modulation through HRV in non-alcoholic cirrhosis individuals reported in previous observational and clinical trial studies. Materials and Methods: We performed a systematic review according to the Preferred Reporting Items for Systematic Review and Meta-Analyses (PRISMA) statement using the Medline, Scopus, and Web of Science electronic databases. Five studies were identified and reviewed. Results: HRV was decreased in patients with non-alcoholic cirrhosis, even in the first stage. Conclusions: HRV could be used as a complementary method to improve both the diagnosis and prognosis of non-alcoholic cirrhosis.


Subject(s)
Autonomic Nervous System/physiopathology , Heart Rate , Heart/innervation , Heart/physiopathology , Liver Cirrhosis/physiopathology , Female , Humans , Male
6.
Dig Dis Sci ; 64(11): 3192-3202, 2019 11.
Article in English | MEDLINE | ID: mdl-31076984

ABSTRACT

BACKGROUND: Biliary cirrhosis is associated with hepatopulmonary syndrome (HPS), which is related to increased posttransplant morbidity and mortality. AIMS: This study aims to analyze the pathophysiology of biliary cirrhosis and the onset of HPS. METHODS: Twenty-one-day-old Wistar rats were subjected to common bile duct ligation and were allocated to two groups: group A (killed 2, 3, 4, 5, or 6 weeks after biliary obstruction) and group B (subjected to biliodigestive anastomosis 2, 3, 4, 5, or 6 weeks after the first procedure and killed 3 weeks later). At the killing, arterial blood was collected for the analyses, and samples from the liver and lungs were collected for histologic and molecular analyses. The gasometric parameters as well as the expression levels of ET-1, eNOS, and NOS genes in the lung tissue were evaluated. RESULTS: From a total of 42 blood samples, 15 showed hypoxemia (pO2 < 85 mmHg) and 17 showed an increased oxygen gradient [p (A-a) O2 > 18 mmHg]. The liver histology revealed increased ductular proliferation after common bile duct ligation, and reconstruction of bile flow promoted decreased ductular proliferation 5 and 6 weeks post-common bile duct ligation. Pulmonary alterations consisted of decreased parenchymal airspace and increased medial wall thickness. Biliary desobstruction promoted transitory improvements 5 weeks after biliary obstruction (increased parenchymal airspace and decreased MWT-p = 0.003 and p = 0.004, respectively) as well as increased endothelin expression levels (p = 0.009). CONCLUSIONS: The present model showed lung tissue alterations promoted by biliary obstruction. The biliodigestive anastomosis had no clear direct effects on these alterations.


Subject(s)
Bile Ducts/pathology , Disease Models, Animal , Hepatopulmonary Syndrome/pathology , Liver Cirrhosis, Biliary/pathology , Anastomosis, Surgical/methods , Animals , Bile Ducts/surgery , Female , Hepatopulmonary Syndrome/blood , Ligation , Liver Cirrhosis, Biliary/blood , Lung/pathology , Male , Rats , Rats, Wistar
7.
Acta Obstet Gynecol Scand ; 98(2): 154-161, 2019 02.
Article in English | MEDLINE | ID: mdl-30264855

ABSTRACT

INTRODUCTION: The present study aims to investigate the association of spontaneous labor onset with neonatal outcomes in pregnancies with fetal gastroschisis. The secondary goal was to evaluate the association between ultrasound markers and labor onset in pregnancies with fetal gastroschisis. MATERIAL AND METHODS: A retrospective analysis was performed for 207 pregnancies with fetal gastroschisis between 2005 and 2017. Patients were followed according to a strict protocol involving ultrasound scans and routine evaluations of fetal biophysical profile parameters and intra- and extra-abdominal bowel diameters. Labor, delivery and neonatal records were reviewed. The neonatal outcomes were complex gastroschisis, staged surgical closure of the abdominal defect, short bowel syndrome, time on mechanical ventilation, time to full enteral feeding and necrotizing enterocolitis. RESULTS: Spontaneous labor onset was associated with complex gastroschisis (30.7% vs 6.8%, P = 0.002) and short bowel syndrome (13.2% vs none, P = 0.013) in cases with spontaneous preterm delivery compared with the indicated preterm deliveries. Spontaneous labor onset was associated with a longer time on mechanical ventilation for term and preterm newborns. Associations were not observed between ultrasound markers and spontaneous labor onset. CONCLUSIONS: Spontaneous labor onset in pregnancies with fetal gastroschisis was associated with adverse neonatal outcomes, particularly in those born preterm.


Subject(s)
Correlation of Data , Delivery, Obstetric , Gastroschisis , Infant, Newborn, Diseases , Intestines , Labor Onset , Premature Birth , Adult , Brazil/epidemiology , Delivery, Obstetric/adverse effects , Delivery, Obstetric/methods , Female , Fetus/diagnostic imaging , Gastroschisis/diagnosis , Gastroschisis/epidemiology , Gestational Age , Humans , Infant, Newborn , Infant, Newborn, Diseases/diagnosis , Infant, Newborn, Diseases/epidemiology , Intestines/injuries , Intestines/physiopathology , Male , Pregnancy , Pregnancy Outcome/epidemiology , Premature Birth/diagnosis , Premature Birth/epidemiology , Premature Birth/etiology , Premature Birth/physiopathology , Prenatal Care/methods , Retrospective Studies , Ultrasonography, Prenatal/methods
8.
Pediatr Surg Int ; 34(11): 1215-1224, 2018 Nov.
Article in English | MEDLINE | ID: mdl-30121781

ABSTRACT

PURPOSE: To evaluate the role of maintenance of the ileocecal valve (ICV) in intestinal adaptation mechanisms, in a weaning rat experimental model of short bowel. METHODS: Forty animals were operated on to produce short bowel syndrome. They were divided into five groups: maintenance (MV) or resection of ICV (RV), kill after 4 days (MV4 and RV4) or 21 days (MV21 and RV21), and a control group (21-day-old rats). Body weights, small bowel and colon lengths and diameters, villus heights, crypt depths, lamina propria and muscle layer thickness, as well as the apoptosis index of villi and crypts and expression of pro- and anti-apoptotic genes, were studied. RESULTS: Preservation of the ICV promoted increased weight gain (p = 0.0001) and intestinal villus height after 21 days; crypt depth was higher in comparison to controls. It was verified a higher expression of Ki-67 in bowel villi and crypts (p = 0.018 and p = 0.015, respectively) in RV4 group and a higher expression in bowel villi of MV4 group animals (p = 0.03). The maintenance of ICV promoted late increased expression of the anti-apoptotic gene Bcl-XL in the colon (p = 0.043, p = 0.002, p = 0.01). CONCLUSION: The maintenance of the ICV led to positive changes in this model.


Subject(s)
Adaptation, Physiological , Ileocecal Valve , Organ Sparing Treatments , Short Bowel Syndrome/physiopathology , Animals , Colon/metabolism , Disease Models, Animal , Ileocecal Valve/surgery , Intestine, Small/metabolism , Ki-67 Antigen/metabolism , Mucous Membrane/pathology , RNA, Messenger/metabolism , Rats, Sprague-Dawley , Weight Gain , bcl-X Protein/genetics , bcl-X Protein/metabolism
9.
Pediatr Transplant ; 21(5)2017 Aug.
Article in English | MEDLINE | ID: mdl-28436075

ABSTRACT

In pediatric liver transplantations with LFS grafts, higher incidences of graft dysfunction probably occur due to IRI. It was postulated that increasing the blood supply to the graft by means of a meso-caval shunt could ameliorate the IRI. Eleven pigs underwent liver transplantation and were divided into two groups: LFS and LFS+SHUNT group. A series of flowmetric, metabolic, histologic, and molecular studies were performed. No significant metabolic differences were observed between the groups. One hour after reperfusion, portal flow was significantly lower in the recipients than in the donors, proving that the graft was maintained in low portal blood flow, although the shunt could promote a transient increase in the portal blood flow and a decrease in the arterial flow. Finally, it was verified that the shunt promoted a decrease in inflammation and steatosis scores and a decrease in the expression of the eNOS gene (responsible for the generation of nitric oxide in the vascular endothelium) and an increase in the expression of the proapoptotic gene BAX. The meso-caval shunt was responsible for some positive effects, although other deleterious flowmetric and molecular alterations also occurred.


Subject(s)
Body Weight , Liver Transplantation/methods , Liver/anatomy & histology , Portasystemic Shunt, Surgical/methods , Reperfusion Injury/prevention & control , Animals , Biomarkers/metabolism , Liver/blood supply , Liver/metabolism , Organ Size , Random Allocation , Reperfusion Injury/diagnosis , Reperfusion Injury/etiology , Reperfusion Injury/metabolism , Swine , Treatment Outcome
10.
Liver Transpl ; 22(7): 1006-13, 2016 07.
Article in English | MEDLINE | ID: mdl-26946330

ABSTRACT

Acute liver failure (ALF) in children is a life-threatening condition that often leads to urgent liver transplantation (LT). The aim of the present investigation was to describe the experience in Brazil in treating pediatric ALF, with an emphasis on the role of living donor liver transplantation (LDLT) in treating this condition. All children with ALF who fulfilled the criteria for an urgent LT were admitted to the intensive care unit. Patients were divided into 2 groups based on the moment of admission: before and after June 2007, when the LDLT program for ALF was started. Statistical analyses were performed to identify prognostic factors of patients with ALF. For the study, 115 children with ALF were admitted. All patients had some degree of encephalopathy. Among the patients, 26% of them required intracranial pressure monitoring (IPM), 12.8% of the patients required hemodialysis, and 79 patients underwent transplantation (50 deceased donors and 29 living donors) corresponding to 12.4% of all pediatric LTs. Only 9 children recovered without LT. The need for IPM and nonperformance of LT were related to a higher mortality. The mortality rate of patients who underwent LT was significantly lower than that of children with ALF who did not undergo a LT (48.1% versus 75%; P = 0.02). The incidences of primary nonfunction and mortality were statistically higher among deceased donor liver transplantations than LDLTs. Finally, it was verified that the overall survival rate of transplanted patients was increased after the introduction of LDLT (P = 0.02). In conclusion, ALF in children continues to be a severe and devastating condition, and a LT should be performed promptly. The introduction of LDLT could increase the survival rate of patients in Brazil. Liver Transplantation 22 1006-1013 2016 AASLD.


Subject(s)
Hepatic Encephalopathy/epidemiology , Liver Failure, Acute/mortality , Liver Failure, Acute/therapy , Liver Transplantation/methods , Living Donors , Adolescent , Brazil/epidemiology , Child , Child, Preschool , Clinical Decision-Making , Delayed Graft Function/epidemiology , Female , Graft Rejection/epidemiology , Humans , Incidence , Infant , Infant, Newborn , Intracranial Pressure , Liver Failure, Acute/etiology , Male , Prognosis , Renal Dialysis , Survival Rate , Time Factors , Treatment Outcome
11.
Pediatr Surg Int ; 30(4): 423-9, 2014 Apr.
Article in English | MEDLINE | ID: mdl-24468714

ABSTRACT

The aim of the present investigation was to describe a new model of liver regeneration in growing rats with reduced portal flow. In addition, it was studied whether tacrolimus and insulin could have any pro-regenerative effect under such conditions. Ninety-five rats were divided into five groups: Group 1 (sham), abdominal incision without intervention; Group 2, 70% hepatectomy; Group 3, 70% hepatectomy + PV stenosis; Group 4, 70% hepatectomy + portal vein stenosis + insulin; and Group 5, 70% hepatectomy + portal vein stenosis + tacrolimus. The remnant liver lobes were harvested for analyses. The liver weight decreased in the PV stenosis group and it increased with the use of insulin and tacrolimus. The mitotic activity was higher in the hepatectomy, insulin and tacrolimus groups and this parameter was reduced by portal stenosis. Levels of interleukin 6 (IL-6) were higher in the hepatectomy group compared to the sham and PV stenosis groups. The expression of IL-6 and Ki67 was significantly increased in the insulin and tacrolimus groups compared to the portal stenosis group. A highly reproducible model was standardized to study liver regeneration with portal blood inflow reduction in weaning rats. It was demonstrated that insulin or tacrolimus administration may partially reverse the harmful effects of PV stenosis.


Subject(s)
Calcineurin Inhibitors/pharmacology , Insulin/pharmacology , Liver Regeneration/drug effects , Portal Vein/pathology , Tacrolimus/pharmacology , Animals , Constriction, Pathologic , Immunohistochemistry , Interleukin-6/genetics , Liver/blood supply , Male , Models, Animal , Polymerase Chain Reaction , RNA/analysis , Rats , Rats, Wistar , Regional Blood Flow
12.
Rev Paul Pediatr ; 42: e2022152, 2023.
Article in English | MEDLINE | ID: mdl-37436246

ABSTRACT

OBJECTIVE: To describe the incidence and to analyze risk factors associated with cholestasis in neonates with gastroschisis. METHODS: This is a retrospective cohort study in a tertiary single center analyzing 181 newborns with gastroschisis between 2009 and 2020. The following risk factors associated with cholestasis were analyzed: gestational age, birth weight, type of gastroschisis, silo closure or immediate closure, days of parenteral nutrition, type of lipid emulsion, days of fasting, days to reach a full diet, days with central venous catheter, presence of infections, and outcomes. RESULTS: Among the 176 patients evaluated, 41 (23.3%) evolved with cholestasis. In the univariate analysis, low birth weight (p=0.023), prematurity (p<0.001), lipid emulsion with medium-chain triglycerides and long-chain triglycerides (p=0.001) and death (p<0.001) were associated with cholestasis. In the multivariate analysis, patients who received lipid emulsion with fish oil instead of medium chain triglycerides/long chain triglycerides (MCT/LCT) emulsion had a lower risk of cholestasis. CONCLUSIONS: Our study shows that lipid emulsion with fish oil is associated with a lower risk of cholestasis in neonates with gastroschisis. However, this is a retrospective study and a prospective study should be performed to confirm the results.


Subject(s)
Cholestasis , Gastroschisis , Humans , Infant, Newborn , Birth Weight , Retrospective Studies , Emulsions , Gastroschisis/epidemiology , Gastroschisis/chemically induced , Gastroschisis/complications , Prospective Studies , Soybean Oil/adverse effects , Fish Oils , Cholestasis/etiology , Cholestasis/chemically induced , Triglycerides
13.
Acta Cir Bras ; 38: e381323, 2023.
Article in English | MEDLINE | ID: mdl-37283357

ABSTRACT

PURPOSE: Acute mesenteric ischemia (AMI) is a condition in pediatric surgery that ranges from intestine necrosis to death. Ischemic postconditioning (IPoC) methods were developed to reduce the damage caused by revascularization. This study aimed to evaluate the efficacy of these methods in an experimental weaning rat model. METHODS: Thirty-two 21-day-old Wistar rats were allocated into four groups according to the surgical procedure performed: control, ischemia-reperfusion injury (IRI), local (LIPoC) and remote IPoC (RIPoC). At euthanasia, fragments of the intestine, liver, lungs, and kidneys were submitted to histological, histomorphometric, and molecular analyses. RESULTS: In the duodenum, intestines, and kidneys histological alterations promoted by IRI were reversed by remote postconditioning method. In the distal ileum, the histomorphometric alterations could be reversed by the postconditioning methods with more evident effects promoted by the remote method. The molecular analysis found that the levels of expression of Bax (proapoptotic) and Bcl-XL (antiapoptotic) genes in the intestine were increased by IRI. These alterations were equally reversed by the postconditioning methods, with more evident effects of the remote method. CONCLUSIONS: IPoC methods positively reduced the damage caused by IRI in weaning rats.


Subject(s)
Ischemic Postconditioning , Mesenteric Ischemia , Reperfusion Injury , Rats , Animals , Ischemic Postconditioning/methods , Rats, Wistar , Models, Animal , Reperfusion Injury/prevention & control , Reperfusion Injury/pathology
14.
Clinics (Sao Paulo) ; 78: 100198, 2023.
Article in English | MEDLINE | ID: mdl-37075514

ABSTRACT

BACKGROUND: Hirschsprung's Disease (HD) is characterized by intestinal sub-occlusion and the absence of enteric ganglion cells. A rectal biopsy examination is performed to confirm the diagnosis. In a recent study, we demonstrated that the analysis of 60 sections of rectal mucosa and submucosa stained by H&E may ensure a 90% diagnostic accuracy. Although the need to analyze so many sections makes the process of reading the slides more time-consuming, this encouraged us to study their distribution in the healthy rectal submucosa, to simplify the diagnosis. OBJECTIVES: To develop a method that facilitates HD diagnosis by studying the distribution of ganglion cells in the submucosal plexus. METHODS: Using the calretinin technique, we studied the distribution of plexuses in 60 fragments of rectal submucosa from 19 cadavers. After the study, the reading method created was used for diagnosis in 47 cases of suspected HD, using H&E staining. The accuracy was verified by comparing the results obtained with H&E to those obtained with the acetylcholinesterase technique, the golden standard in our laboratory. RESULTS: The study of submucosal plexus distribution showed that just by examining the submucosal region every 20 µm, approximately, it is possible to locate a ganglionic plexus, and we have already been able to diagnose HD with 93% accuracy. CONCLUSION: The study of ganglion cell distribution enabled the creation of a simplified method for reading the slides. The method applied achieved good accuracy and it can be used as an alternative method in HD diagnosis.


Subject(s)
Hirschsprung Disease , Humans , Infant , Hirschsprung Disease/diagnosis , Hirschsprung Disease/pathology , Acetylcholinesterase/analysis , Rectum/chemistry , Rectum/pathology , Biopsy/methods
15.
Surg Oncol ; 46: 101896, 2023 Feb.
Article in English | MEDLINE | ID: mdl-36571933

ABSTRACT

BACKGROUND: To identify prognostic factors for overall survival through the analysis of 132 patients with Wilms tumor followed at a single center, with emphasis on the inferior vena cava/right atrium extension. METHODS: Retrospective analysis of overall survival using logistic regression models and including age, sex, clinical features, associated syndromes, comorbidities, tumor size before chemotherapy, stage, presence of metastatic disease and its site, invasion of adjacent structures, inferior vena cava/right atrium extension, laterality, tumor histology, chemotherapy protocol, and radiotherapy as potential risk factors. RESULTS: From January 2000 through November 2021, 132 patients met the inclusion criteria, 64 females and 68 males; 15 (11.4%) patients presented with tumoral extension to inferior vena cava/right atrium and 44 had metastatic disease (33.3%). Based on logistic regression, the factors correlating to a fatal outcome were male sex (p = 0.046), high risk histology (p = 0.036), and the presence of metastatic disease (p = 0.003). None of the patients presenting inferior vena cava/right atrium extension died (p = 0.992). In a specific analysis of metastatic sites, hepatic metastasis alone showed correlation with a fatal outcome (p = 0.001). CONCLUSION: These results underline the importance of identifying and treating metastatic disease and high-risk tumors. The female gender as a potential driver for a less aggressive disease is a new finding that deserves further investigation. The accurate identification of inferior vena cava/right atrium extension, subsequent preoperative chemotherapy, and resection with a skilled team promoted survival rates of all patients. LEVEL OF EVIDENCE: II.


Subject(s)
Kidney Neoplasms , Wilms Tumor , Humans , Male , Child , Female , Vena Cava, Inferior/surgery , Vena Cava, Inferior/pathology , Prognosis , Retrospective Studies , Wilms Tumor/surgery , Wilms Tumor/pathology , Heart Atria/surgery , Heart Atria/pathology , Kidney Neoplasms/surgery , Kidney Neoplasms/pathology , Multivariate Analysis
16.
Transplant Proc ; 55(9): 2121-2125, 2023 Nov.
Article in English | MEDLINE | ID: mdl-37758563

ABSTRACT

A new outbreak of hepatitis of unknown origin raised awareness in the international community. A few reports have attempted to associate new cases with adenovirus infection and the immunologic effects of previous SARS-CoV-2 infections through a superantigen mechanism. Moreover, according to a case series, viral isolates were identified in 7 of 10 cases of pediatric patients with hepatitis of unknown origin and acute liver failure. Adenovirus was detected by respiratory secretion polymerase chain reaction in 2 patients, with neither presenting with SARS-CoV-2 acute infection. Clinical and laboratory descriptions and cross-referencing epidemiologic and pathophysiological data can help identify possible disease etiologies.


Subject(s)
COVID-19 , Hepatitis , Liver Failure, Acute , Child , Humans , SARS-CoV-2 , COVID-19/complications , Polymerase Chain Reaction , Liver Failure, Acute/diagnosis , Liver Failure, Acute/etiology
17.
J Pediatr (Rio J) ; 98(6): 607-613, 2022.
Article in English | MEDLINE | ID: mdl-35405144

ABSTRACT

OBJECTIVE: Pediatric acute liver failure (PALF) is a heterogeneous, rare, and severe condition, which outcome is survival due to liver spontaneous recovery or death. The patients who do not recover may be allocated to liver transplantation, which is the standard treatment. This study aimed to build a prognostic model to support the clinical decision to indicate liver transplantation for patients with PALF in a Brazilian center. METHODS: The authors retrospectively analyzed the clinical variables of 120 patients in the liver transplantation program of the 'Children's Institute of the University of São Paulo, Brazil. The authors conducted a univariate analysis of variables associated with survival in PALF. Logistic multivariate analysis was performed to find a prognostic model for the outcome of patients with pediatric acute liver failure. RESULTS: Risk factors were analyzed using univariate analysis. Two prognostic models were built using multiple logistic regression, which resulted in 2 models: model 1(INR/ALT) and model 2 (INR/Total bilirubin). Both models showed a high sensitivity (97.9%/96.9%), good positive predictive value (89.5%/90.4%), and accuracy (88.4%/88.5%), respectively. The receiver operating characteristic was calculated for both models, and the area under the curve was 0.87 for model 1 and 0.88 for model 2. The Hosmer-Lemeshow test showed that model 1 was good. CONCLUSION: The authors built a prognostic model for PALF using INR and ALT that can contribute to the clinical decision to allocate patients to liver transplantation.


Subject(s)
Liver Failure, Acute , Liver Transplantation , Child , Humans , Prognosis , Brazil , Retrospective Studies , Liver Failure, Acute/surgery , Liver Transplantation/methods , ROC Curve
18.
J Invest Surg ; 35(4): 793-800, 2022 Apr.
Article in English | MEDLINE | ID: mdl-34583613

ABSTRACT

PURPOSE: To compare the adaptation processes of the remaining jejunum or ileum after extensive intestinal resection in a growing animal model. MATERIALS AND METHODS: Forty 21-day old rats were divided into four groups: JG: remaining jejunum group - ileal enterectomy; IG: ileum remaining group - jejunum enterectomy; SHAM: sham-operated group - open-and-close laparotomy; and NO: non-operated animals. RESULTS: After 3 weeks, JG and IG animals had decreased weights comparing to SHAM and NO animals (p = 0.017 and p = 0.005, respectively). The histomorphometric analysis showed that in JG animals the villi were higher than in SHAM, NO, and IG animals (p = 0.007, p = 0.008, and p = 0.01), the depth of crypts in JG and IG animals was greater than in NO and SHAM animals (p = 0.03, p = 0.002, and p = 0.003 respectively), and muscle layer thickness of the jejunum of JG animals had values greater than SHAM and NO animals (p = 0.01 and p = 0.02, respectively). The Ki-67 expression in the ileum was higher in comparison with the jejunum (p = 0.014). The pro-apoptotic gene (Bax) expression was decreased in JG animals compared to IG, SHAM, and NO animals (p = 0.013, p = 0.024, and p = 0.021). The anti-apoptotic gene (Bcl-XL) expression was decreased in JG animals in comparison to IG and NO animals (p = 0.002 and p = 0.046) although it was increased in the colon of IG animals in comparison to JG, SHAM, and NO animals (p = 0.002, p = 0.001, and p = 0.001, respectively). The Bax/Bcl-XL ratio was higher in JG than in IG animals (p = 0.011). CONCLUSION: Adaptive responses seemed to be more effective in the ileum than in the jejunum.


Subject(s)
Ileum , Jejunum , Adaptation, Physiological/physiology , Animals , Ileum/metabolism , Ileum/surgery , Intestinal Mucosa , Intestines , Jejunum/surgery , Rats
19.
Acta Cir Bras ; 37(6): e370604, 2022.
Article in English | MEDLINE | ID: mdl-36074397

ABSTRACT

PURPOSE: To evaluate the caliber of an arterial micro-anastomosis in the young growing animal using a continuous suture technique. Additionally, late morphological changes and blood flows distal to the anastomosis were evaluated. METHODS: Seventy-four Wistar rats were submitted to laparotomy to access the aorta for blood flow measurement. The aorta was sectioned using microsurgery technique and an end-to-end anastomosis with continuous suture. After a period of six months to one year, the anastomosis was checked. RESULTS: Regarding the size of the aortas, comparing the pre- and postoperative values, there was an increase of 13.33% in adult animals and 25% in young animals, without any difference in the blood flows. CONCLUSIONS: The arteries of young rats show signs of growth at the site of the anastomosis performed with continuous suture.


Subject(s)
Arteries , Microsurgery , Anastomosis, Surgical/methods , Animals , Arteries/surgery , Microsurgery/methods , Rats , Rats, Wistar , Suture Techniques , Vascular Surgical Procedures
20.
J Invest Surg ; 35(6): 1197-1207, 2022 Jun.
Article in English | MEDLINE | ID: mdl-34965813

ABSTRACT

BACKGROUND: In pediatric liver transplantation, the optimal size of the transplanted liver ranges between 0.8% and 4.0% of the recipient's weight. Sometimes, the graft weight exceeds this upper limit, characterizing the large-for-size condition potentially associated with reduced blood flow and worsening of ischemia-reperfusion injury. Therefore, it would be beneficial to increase the portal flow through arterialization of the portal vein. Materials and methods: Fifteen pigs underwent large-for-size liver transplants. They were divided into two groups: control (CTRL 6 animals - conventional technique) and arterialization - a shunt was established between the portal vein and the splenic artery (ART 9 animals). Hemodynamic, biochemical, histological, and molecular variables were compared. Results: Arterialization resulted in a significant increase in portal vein pressure but no changes in other hemodynamic variables, as shown in the analysis of variance. It was observed lower ALT values (p = 0.007), with no differences regarding the values of blood pH and lactate (p = 0.54 and p = 0.699 respectively) or histological variables (edema, steatosis, inflammation, necrosis, and IRI - p = 1.0, p = 0.943, p = 0.174, p = 0.832, p = 0.662, respectively). The molecular studies showed significantly increased expression of IL6 after 3 hours of reperfusion (p = 0.048) and decreased expression of ICAM immediately after reperfusion (p = 0.03). The regression analysis suggested a positive influence of portal flow and pressure on biochemical parameters. Conclusion: Arterialization of the portal vein showed no histological, biochemical, or molecular benefits in large-for-size transplantation.


Subject(s)
Liver Transplantation , Portal Vein , Animals , Hemodynamics , Humans , Liver/pathology , Liver Transplantation/adverse effects , Liver Transplantation/methods , Portal Vein/physiology , Portal Vein/surgery , Reperfusion , Swine
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