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1.
BMC Pediatr ; 24(1): 454, 2024 Jul 15.
Artículo en Inglés | MEDLINE | ID: mdl-39009961

RESUMEN

PURPOSE: This study analyzed the efficacy of web excision combined with a pre-membranous incision on the dilated proximal segment for congenital intestinal atresia with type I and stenosis (CIA-I/S). PATIENTS AND METHODS: Twenty-six patients underwent surgery for CIA-I/S from January 1990 to June 2022. Patients were categorized into 3 groups according to the surgical procedure: Group A, web excision with pre-membranous incision of the dilated intestine (n = 14); Group B, enteroplasty with a trans-membranous vertical incision (n = 7) and Group C, diamond-shaped anastomosis (n = 5). To minimize the impact of obstruction location on outcomes, we specifically examined 17 cases of duodenal atresia/stenosis: Group D-A, (n = 6); Group D-B, (n = 6) and Group D-C, (n = 5). We retrospectively compared the operative and postoperative parameters among the three groups. RESULTS: No patient experienced anastomotic leakage or obstruction. There were no significant differences in operative duration or blood loss among the 3 Groups. The median time to feeding initiation was 4, 6.5, and 5 days in Groups A, B, and C, respectively (p = 0.04) and was 4, 6.5, and 5 days in Groups D-A, D-B, and D-C, respectively (p = 0.04). CONCLUSION: Web excision, when compared to enteroplasty and diamond-shaped anastomosis, showed comparable results in terms of the operative duration and postoperative complications. However, it may allow for an earlier initiation of enteral nutrition.


Asunto(s)
Atresia Intestinal , Humanos , Atresia Intestinal/cirugía , Femenino , Masculino , Estudios Retrospectivos , Recién Nacido , Anastomosis Quirúrgica/métodos , Resultado del Tratamiento , Constricción Patológica/cirugía , Obstrucción Duodenal/cirugía , Obstrucción Duodenal/congénito , Lactante , Complicaciones Posoperatorias
2.
Pediatr Surg Int ; 40(1): 226, 2024 Aug 15.
Artículo en Inglés | MEDLINE | ID: mdl-39145817

RESUMEN

PURPOSE: Patients with intestinal failure (IF) require long-term parenteral nutrition using central venous catheters (CVCs), which often require replacement. We adopted a less fallible guidewire replacement (GWR) method and verified its effectiveness and validity. METHODS: We enrolled 108 cases that underwent a CVC replacement with "GWR" method with IF at our department between 2013 and 2023. We retrospectively reviewed patients' clinical details with tunneled CVC (Hickman/Broviac catheter). For the analysis, we compared for the same time period the catheter exchange method "Primary placement"; newly inserted catheter by venipuncture. RESULTS: The success rate of catheter replacement using GWR was 94.4%. There were six unsuccessful cases. A log-rank test showed no significant difference in catheter survival between primary placement and the GWR, and the time to first infection was significantly longer in the GWR (p = 0.001). Furthermore, no significant differences were observed between the two methods until the first infection, when the exchange indication was limited to infections. In the same way, when the indication was restricted to catheter-related bloodstream infection, there was no significant difference in catheter survival between the two approaches. CONCLUSION: Our GWR procedure was easy to perform and stable, with a high success rate and almost no complications. Moreover, using a guidewire did not increase the frequency of catheter replacement and the infection rate.


Asunto(s)
Cateterismo Venoso Central , Catéteres Venosos Centrales , Insuficiencia Intestinal , Humanos , Estudios Retrospectivos , Masculino , Femenino , Cateterismo Venoso Central/métodos , Insuficiencia Intestinal/terapia , Lactante , Preescolar , Niño , Infecciones Relacionadas con Catéteres , Nutrición Parenteral/métodos , Resultado del Tratamiento , Remoción de Dispositivos/métodos , Adolescente
3.
J Pediatr Gastroenterol Nutr ; 77(3): 339-346, 2023 09 01.
Artículo en Inglés | MEDLINE | ID: mdl-37364133

RESUMEN

OBJECTIVES: Our objective was to evaluate the short- and long-term safety and efficacy of teduglutide treatment in infants and children with short bowel syndrome with intestinal failure (SBS-IF). METHODS: Two open-label phase 3 studies and 1 extension study investigated the short- and long-term safety and efficacy of teduglutide (0.05 mg/kg/day) in infants and children with SBS-IF: NCT03571516, 24-week study of infants who were randomized to receive teduglutide or standard of care (SoC); NCT02980666, 24-week study of infants and children who all received teduglutide; and NCT03268811, 24-week extension study of patients who completed NCT02980666 (patients could receive up to 48 weeks of total treatment). RESULTS: Twelve infants and 8 children enrolled in the core studies, and 2 infants and 7 children in the extension study. After 24 weeks of treatment, parenteral support (PS) requirements reduced by ≥20% from baseline for 4 infants (57.1%) and 4 children (66.7%) receiving teduglutide and for 2 infants receiving SoC (50.0%). One infant (50.0%) and 4 children (80.0%) receiving teduglutide maintained the ≥20% reduction in PS at 48 weeks of treatment. Two children receiving teduglutide achieved enteral autonomy, after 12 weeks and 28 weeks of treatment, respectively. All adverse events (AEs) were in line with known impacts of SBS-IF and adverse reactions to teduglutide. Only one serious AE (abdominal pain) was considered related to teduglutide. CONCLUSIONS: Short- and long-term treatment with teduglutide resulted in clinically meaningful reductions in PS requirements for infants and children with SBS-IF. Teduglutide was well tolerated, and efficacy improved with longer-term treatment.


Asunto(s)
Síndrome del Intestino Corto , Humanos , Lactante , Niño , Síndrome del Intestino Corto/tratamiento farmacológico , Nutrición Parenteral/métodos , Intestino Delgado , Péptidos/efectos adversos , Fármacos Gastrointestinales/efectos adversos
4.
Surg Today ; 53(3): 347-359, 2023 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-36201060

RESUMEN

PURPOSE: The short- and long-term efficacy, safety, and pharmacokinetics of teduglutide were analyzed in adult Japanese patients with short bowel syndrome and intestinal failure (SBS-IF). METHODS: Patients received teduglutide 0.05 mg/kg/day in clinical trials (TED-C14-004, SHP633-306, and extension SHP633-307). Data were analyzed at 24 weeks and an interim data cut-off of 4.5 years. RESULTS: The parenteral support (PS) volume decreased by ≥ 20% for 9/18 patients at 24 weeks and in all 11 patients by data cut-off in SHP633-307. The mean (standard deviation) PS volume decreased from baseline at 24 weeks in TED-C14-004 (-30.1 ± 25.9%) and SHP633-306 (-25.6 ± 25.5%), and at data cut-off in SHP633-307 (-57.08 ± 28.49%). Teduglutide was absorbed quickly. The adverse events were consistent with the underlying disease and known adverse drug reactions. Anti-teduglutide antibody titers declined with long-term treatment. CONCLUSIONS: In Japanese adults with SBS-IF, teduglutide treatment was associated with clinically meaningful reductions in PS requirements, similar to findings in prior international studies. No new safety concerns specific to the Japanese SBS-IF patient population were identified with short- or long-term teduglutide treatment. Anti-teduglutide antibody titers disappeared in most Japanese adults with long-term treatment. These results constitute the longest evaluation of teduglutide treatment within clinical trials reported to date.


Asunto(s)
Fármacos Gastrointestinales , Insuficiencia Intestinal , Síndrome del Intestino Corto , Adulto , Humanos , Pueblos del Este de Asia , Fármacos Gastrointestinales/farmacocinética , Fármacos Gastrointestinales/uso terapéutico , Nutrición Parenteral/métodos , Síndrome del Intestino Corto/tratamiento farmacológico
5.
Pediatr Surg Int ; 39(1): 276, 2023 Sep 27.
Artículo en Inglés | MEDLINE | ID: mdl-37755555

RESUMEN

INTRODUCTION: Intestinal transplantation (ITx) is the ultimate treatment for intestinal failure (IF). In Japan, most cases of IF are a result of pediatric disease, including secondary or congenital intestinal disease or allied disorders of Hirschsprung's disease. Here, we report the results of the Japanese ITx registry. METHODS: A web-based survey form was completed. We investigated the number, age, sex, indication, surgical procedure, immunosuppressants, postoperative course, and the effects of transplantation in patients who underwent cadaveric or living-donor ITx. RESULTS: By the end of 2022, 42 cases of ITx have been performed in 38 patients in Japan. The donor sources included cadavers (29 cases) and living donors (13 cases). The surgical method was isolated ITx (N = 40) and combined liver and ITx (n = 2). Survival rates were 92%, 73%, and 59% at 1 year, 5 years, and 10 years, respectively. Ninety percent of patients completely discontinued parenteral nutrition. Approximately 80% of the patients had a performance status of 1 or less, indicating that the QOL of patients after ITx was extremely good. CONCLUSION: The results of ITx are acceptable to treat IF patients and the QOL after transplantation is also good.


Asunto(s)
Enfermedades Intestinales , Síndrome del Intestino Corto , Niño , Humanos , Japón , Calidad de Vida , Síndrome del Intestino Corto/cirugía , Estudios Retrospectivos , Intestinos , Enfermedades Intestinales/cirugía , Donadores Vivos
6.
Pediatr Surg Int ; 39(1): 283, 2023 Oct 17.
Artículo en Inglés | MEDLINE | ID: mdl-37847289

RESUMEN

PURPOSE: The incidence and risk factors of catheter-related bloodstream infections (CRBSI) in patients with intestinal failure (IF) have not been established, partly because catheter management methods vary from different facilities. This study aimed to identify the risk factors and incidence rate of CRBSIs in patients with IF who were given prophylactic treatment. METHODS: Sixteen patients with IF who required home parenteral nutrition were enrolled in this study. Prophylactic management of CRBSI included monthly ethanol lock therapy and standardized infection prevention education. The outcomes included the incidence and risk factors of CRBSI. RESULTS: The median incidence rate of CRBSI was 1.2 per 1000 catheter days. Univariate analysis showed that the risk of developing CRBSI was significantly associated with short bowel syndrome (< 30 cm) (p = 0.016). Other relevant findings included a significant negative correlation between serum albumin and CRBSI rate (r = - 0.505, p = 0.046), and past history of mixed bacterial infections was significantly associated with increased CRBSI rate (p = 0.013). CONCLUSION: CRBSIs can still develop despite undergoing prophylactic management. Risk factors for CRBSI include the residual intestinal length, nutritional status, and susceptibility to certain microorganisms.


Asunto(s)
Bacteriemia , Infecciones Relacionadas con Catéteres , Catéteres Venosos Centrales , Insuficiencia Intestinal , Nutrición Parenteral en el Domicilio , Humanos , Catéteres Venosos Centrales/efectos adversos , Infecciones Relacionadas con Catéteres/prevención & control , Bacteriemia/epidemiología , Bacteriemia/etiología , Bacteriemia/prevención & control , Nutrición Parenteral en el Domicilio/efectos adversos , Nutrición Parenteral en el Domicilio/métodos , Factores de Riesgo , Estudios Retrospectivos
7.
Curr Opin Organ Transplant ; 28(3): 237-241, 2023 06 01.
Artículo en Inglés | MEDLINE | ID: mdl-37053076

RESUMEN

PURPOSE OF REVIEW: The intestinal rehabilitation program (IRP) is a comprehensive treatment strategy that employs various approaches implemented by multidisciplinary teams to treat intestinal failure in children. This program has shown promising results, such as reducing complications and improving prognosis and quality of life (QOL). In this review, we discuss the current status of this program and relevant topics. RECENT FINDINGS: IRP includes the prevention and treatment of various complications such as intestinal failure associated liver disease, catheter-related bloodstream infection or sepsis, and venous thromboembolism. In addition, treatment strategies such as glucagon-like peptide-2 analogs, surgical interventions, and intestinal transplantation have evolved over time and have contributed to improved outcomes. In addition, the scope and regions for IRP activities have expanded. SUMMARY: IRP improves the prognosis and QOL of children with intestinal failure. The development of new drugs, surgical methods, and treatment strategies is expected to improve the current and future status of pediatric patients with intestinal failure. Furthermore, international institutions must collaborate, share knowledge, conduct joint research, and establish patient registries to advance IRP progress.


Asunto(s)
Enfermedades Intestinales , Insuficiencia Intestinal , Fallo Hepático , Síndrome del Intestino Corto , Niño , Humanos , Calidad de Vida , Síndrome del Intestino Corto/rehabilitación , Síndrome del Intestino Corto/cirugía , Intestinos , Enfermedades Intestinales/terapia
8.
Med Mol Morphol ; 55(2): 131-145, 2022 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-35238992

RESUMEN

Biliary atresia (BA) is a cholestatic disease with extrahepatic bile duct obstruction that requires early surgical intervention and occasionally liver transplantation (LT). Accumulation of toxic bile acids induces oxidative stress that results in cell damage, such as cell senescence, mitochondrial dysfunction and others. However, details of their reciprocal association and clinical significance are unexplored. Therefore, we used immuno-localization of markers for cell senescence (p16 and p21), nuclear double-strand DNA damage (γH2AX), autophagy (p62), and mtDNA damage (mtDNA copy number) in patients with BA who underwent Kasai portoenterostomy (KP) and LT. We studied liver biopsy specimens from 54 patients with BA, 14 who underwent LT and 11 from the livers of neonates and infants obtained at autopsy. In hepatocytes, p21 expression was significantly increased in KP. In cholangiocytes, p16 expression was significantly increased in LT, and p21 expression was significantly increased in KP. p62 expression was significantly increased in the KP hepatocytes and LT cholangiocytes. Furthermore, mtDNA copy number significantly decreased in KP and LT compared with the control. Cell senescence and mitochondrial DNA damage progression were dependent on the BA clinical stages and could possibly serve as the markers of indication of LT.


Asunto(s)
Atresia Biliar , Trasplante de Hígado , Atresia Biliar/genética , Atresia Biliar/metabolismo , Atresia Biliar/cirugía , Biomarcadores/metabolismo , Senescencia Celular , Daño del ADN , ADN Mitocondrial/genética , ADN Mitocondrial/metabolismo , Humanos , Lactante , Recién Nacido , Hígado/patología , Trasplante de Hígado/efectos adversos , Trasplante de Hígado/métodos , Mitocondrias/genética , Mitocondrias/patología , Portoenterostomía Hepática
9.
Kyobu Geka ; 75(10): 864-871, 2022 Sep.
Artículo en Japonés | MEDLINE | ID: mdl-36155584

RESUMEN

Bochdalek hernia is the most common type of congenital diaphragmatic hernia( CDH). Patients with CDH often develop cardiopulmonary dysfunction secondary to pulmonary hypoplasia and pulmonary hypertension, which necessitates emergency surgery. Advanced cardiopulmonary management is essential in such cases;therefore, patients should be treated at a well-facility that is familiar with the treatment of CDH. Respiratory management using gentle ventilation and control of pulmonary hypertension using nitric oxide are necessary for preoperative stabilization of patients. Intraoperatively, the hernia should be cautiously reduced and contents should be returned into the abdominal cavity. Surgery is performed to secure the circumference of the rim and ensure firm closure of the defect. Thoracoscopic surgery may be considered for late-onset cases with a stable general condition.


Asunto(s)
Hernias Diafragmáticas Congénitas , Hipertensión Pulmonar , Hernias Diafragmáticas Congénitas/complicaciones , Hernias Diafragmáticas Congénitas/cirugía , Humanos , Hipertensión Pulmonar/complicaciones , Hipertensión Pulmonar/terapia , Pulmón , Óxido Nítrico , Toracoscopía
10.
FASEB J ; 34(11): 14820-14831, 2020 11.
Artículo en Inglés | MEDLINE | ID: mdl-32910505

RESUMEN

Glucocorticoid-induced TNFR family related gene (GITR) is a member of the TNFR superfamily that is expressed on cells of the immune system. Although the protective and pathogenic roles of GITR in T cell immunity are well characterized, the role of GITR in innate immunity in the intestinal tissues has not been well clarified. In this study, using a dextran sulfate sodium (DSS)-induced colitis model in mice, we found that GITR-deficiency rendered mice more susceptible to acute intestinal inflammation and that a significantly higher number of activated natural killer (NK) cells was accumulated in the colonic lamina propria of Gitr-/- mice as compared to wild-type mice. Additionally, Rag2-/- Gitr-/- mice, which lack T cells but have NK cells, also displayed more severe colonic inflammation than Rag2-/- mice. In contrast, an anti-GITR agonistic antibody significantly alleviated colitis in Rag2-/- mice. Engagement of GITR inhibited IL-15-mediated activating signaling events in NK cells, which include cell activation and proliferation, and production of cytokines and cytotoxic granules. Taken together, our results provide the first evidence that GITR negatively controls intestinal inflammation through NK cell functions.


Asunto(s)
Colitis Ulcerosa/inmunología , Proteína Relacionada con TNFR Inducida por Glucocorticoide/metabolismo , Mucosa Intestinal/inmunología , Células Asesinas Naturales/inmunología , Animales , Células Cultivadas , Colitis Ulcerosa/genética , Proteínas de Unión al ADN/genética , Proteínas de Unión al ADN/metabolismo , Proteína Relacionada con TNFR Inducida por Glucocorticoide/genética , Interleucina-15/metabolismo , Activación de Linfocitos , Ratones , Ratones Endogámicos C57BL
11.
Pediatr Surg Int ; 35(2): 203-207, 2019 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-30390138

RESUMEN

PURPOSE: Our institution employs rectoplasty with a posterior triangular colonic flap (RPTCF) for classic-type Hirschsprung's disease. Recently, we employed a modified Soave procedure: transanal endorectal pull-through with rectoanal myotomy (TEPTRAM). In both procedures, the internal sphincter muscle is completely divided vertically at the 6 o'clock position. Unlike RPTCF, TEPTRAM does not require abdominal manipulation. We aimed to verify the usefulness of TEPTRAM. METHODS: 64 patients with classic-type Hirschsprung's disease who underwent surgery between 1970 and 2017 were divided into group R (RPTCF, 47 cases) and group T (TEPTRAM, 17 cases). We compared the defecation function of the groups. RESULTS: No patient showed fecal incontinence (R: 0/41, T: 0/10, ns). Three patients (6%) in group R and four (24%) in group T developed postoperative enterocolitis (P = 0.16). Three patients (10%) in group R and one (20%) in group T needed an enema (P = 0.36). CONCLUSION: There was no adverse effect of rectoanal myotomy; incontinence was not observed in either procedure. Although there was no significant difference, the incidences of enterocolitis and constipation were slightly higher in group T, perhaps because of the residual muscle cuff; therefore, it is necessary to provide best care with attention to constipation immediately after surgery.


Asunto(s)
Procedimientos Quirúrgicos del Sistema Digestivo/métodos , Enfermedad de Hirschsprung/cirugía , Miotomía , Niño , Preescolar , Estreñimiento/etiología , Enterocolitis/etiología , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Masculino , Complicaciones Posoperatorias , Estudios Retrospectivos
12.
Pediatr Surg Int ; 33(1): 33-41, 2017 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-27696212

RESUMEN

PURPOSE: The purpose of this study is to identify the current clinical features of neonatal gastrointestinal perforation in Japan. METHODS: A questionnaire about cases of neonatal gastrointestinal perforation treated in recent 5 years was sent to participating institutions of the Japanese Society of Pediatric Surgeons (JSPS). RESULTS: Five hundred and thirty-six neonates with gastrointestinal perforation were treated. They consisted of 42 patients with gastric rupture/perforation (GR), 33 patients with intestinal atresia/stenosis (IA), 3 patients with malrotation (ML), 118 patients with necrotizing enterocolitis (NEC), 160 patients with focal intestinal perforation (FIP), 46 patients with meconium-related ileus (MRI), 77 patients with meconium peritonitis (MP), and 57 patients with other conditions. The total mortality rate was 20.5 %. The mortality rates of the patients with GR, IA, ML, NEC, FIP, MRI, and MP were 9.5, 9.1, 0, 33.1, 20.6, 28.2, and 9.1 %, respectively. In 263 cases involving extremely low-birth-weight neonates (ELBW), 108 died (mortality rate 41.1 %). The mortality rates for ELBW with GR, NEC, FIP, MRI, MP, and other conditions were 27.3 % (3/11), 58.5 % (48/82), 21.6 % (24/111), 70.6 % (24/34), 57.1 % (4/7), and 27.8 % (5/18), respectively. CONCLUSIONS: The mortality rates for ELBW decreased from 62.8 % in the previous survey to 41.1 % by the time of this survey.


Asunto(s)
Perforación Intestinal/epidemiología , Vigilancia de la Población , Enterocolitis Necrotizante/complicaciones , Femenino , Humanos , Recién Nacido , Perforación Intestinal/etiología , Japón/epidemiología , Masculino
13.
Pediatr Surg Int ; 32(9): 839-43, 2016 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-27464487

RESUMEN

PURPOSE: Long-term survivors of biliary atresia (BA) sometimes experience liver dysfunction. We evaluated the prognostic factors for long-term native liver survival (NLS) in BA patients after the Kasai procedure. METHODS: This study included 67 patients with jaundice disappearance after the Kasai procedure performed between 1972 and 1995, and NLS for over 10 years. We retrospectively evaluated the clinical parameters, including the type of BA, age at the Kasai procedure, medical conditions, and treatments. The adjusted odds ratios (aOR) were obtained for 20-year NLS using logistic regression analysis. RESULTS: The median age of the patients at the Kasai procedure was 63 days. Of the 67 study patients, 62 patients (92.5 %) had jaundice-free NLS at the age of 20 years, 4 patients died before the age of 20 years from liver failure, and 1 patient underwent living related liver transplantation. The presence of gastro-esophageal varices requiring endoscopic injection sclerotherapy was a significant factor (aOR 33.8; p = 0.0033), while hypersplenism and cholangitis were not identified as significant factors. CONCLUSIONS: The existence of symptomatic portal hypertension would influence long-term NLS in BA patients after the Kasai procedure. In such patients, accurate evaluation of hepatic function and adequate treatment for sequelae are needed.


Asunto(s)
Atresia Biliar/mortalidad , Atresia Biliar/cirugía , Fallo Hepático/mortalidad , Sobrevivientes , Várices Esofágicas y Gástricas/terapia , Femenino , Estudios de Seguimiento , Humanos , Lactante , Japón/epidemiología , Trasplante de Hígado , Masculino , Pronóstico , Estudios Retrospectivos , Escleroterapia , Adulto Joven
14.
Eur J Immunol ; 44(10): 3015-25, 2014 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-25103720

RESUMEN

T-cell homeostasis preserves the numbers, the diversity and functional competence of different T-cell subsets that are required for adaptive immunity. Naïve CD4(+) T (TN ) cells are maintained in the periphery via the common γ-chain family cytokine IL-7 and weak antigenic signals. However, it is not clear how memory CD4(+) T-cell subsets are maintained in the periphery and which factors are responsible for the maintenance. To examine the homeostatic mechanisms, CFSE-labeled CD4(+) CD44(high) CD62L(low) effector memory T (TEM ) cells were transferred into sublethally-irradiated syngeneic C57BL/6 mice, and the systemic cell proliferative responses, which can be divided distinctively into fast and slow proliferations, were assessed by CFSE dye dilution. We found that the fast homeostatic proliferation of TEM cells was strictly regulated by both antigen and OX40 costimulatory signals and that the slow proliferation was dependent on IL-7. The simultaneous blockade of both OX40 and IL-7 signaling completely inhibited the both fast and slow proliferation. The antigen- and OX40-dependent fast proliferation preferentially expanded IL-17-producing helper T cells (Th17 cells). Thus, OX40 and IL-7 play synergistic, but distinct roles in the homeostatic proliferation of CD4(+) TEM cells.


Asunto(s)
Linfocitos T CD4-Positivos/inmunología , Proliferación Celular , Memoria Inmunológica/inmunología , Interleucina-7/inmunología , Receptores OX40/inmunología , Subgrupos de Linfocitos T/inmunología , Traslado Adoptivo , Animales , Linfocitos T CD4-Positivos/metabolismo , Proliferación Celular/fisiología , Citometría de Flujo , Homeostasis/inmunología , Interleucina-7/metabolismo , Ratones , Ratones Endogámicos C57BL , Ratones Transgénicos , Receptores OX40/metabolismo , Subgrupos de Linfocitos T/metabolismo
15.
Surg Today ; 45(7): 813-8, 2015 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-25212566

RESUMEN

The efficacy of early Kasai portoenterostomy has been repeatedly reported. However, the optimal age for performing this procedure remains controversial. This article reviews the literature on the age of patients at the time of Kasai portoenterostomy and its utility as a prognostic indicator. The age at the time of surgery is a known predictor of outcome; however, its exact predictive value in this context is unclear. Multicenter studies involving large volumes of data have tended to show advantages of early Kasai portoenterostomy, and there is no clear evidence to recommend any delay in the timing of surgery. At present, a reasonable strategy would be to perform a Kasai portoenterostomy as early as possible. The stool color card system has recently been implemented in Japan as part of a nationwide screening program, and it is expected to work well based on the early reports. However, efforts to identify an optimal screening system for ensuring the earliest diagnosis of biliary atresia should continue. An early diagnosis of biliary atresia is difficult, and global efforts are required to improve the early diagnosis rates.


Asunto(s)
Atresia Biliar/cirugía , Portoenterostomía Hepática , Factores de Edad , Atresia Biliar/diagnóstico , Diagnóstico Precoz , Humanos , Trasplante de Hígado , Pronóstico , Reoperación , Resultado del Tratamiento
16.
Pediatr Surg Int ; 31(10): 931-6, 2015 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-26272074

RESUMEN

PURPOSE: Some patients with cystic biliary atresia (BA) achieve exceptionally good postoperative courses. Early differentiation of pediatric patients with the favorable-type disease can lead to beneficial long-term postoperative management. We examined whether the hepatic hilum morphology affects long-term prognosis in type I/I cyst BA, atresia of the common bile duct with/without a cyst. METHODS: Of 253 BA patients identified since 1972, 40 were classified as having type I/I cysts and were divided into two subtypes according to hepatic duct size (subtype α: n = 18; duct diameter, ≥ 1 mm and subtype ß: n = 22; duct diameter, <1 mm) to compare postoperative clinical courses. RESULTS: In subtypes α and ß, jaundice disappeared in 16 (89 %) and 19 (86 %) patients, respectively (p = 0.81), and 13 (72 %) and 12 (55 %) survived with native livers (p = 0.18) at a mean age of 23.3 and 25.5 years, respectively (p = 0.42). CONCLUSIONS: There were no significant differences in long-term outcomes between subtypes α and ß, although approximately 40 % developed liver failure during the postoperative course. Thus, close long-term follow-up is essential in type I/I cysts, regardless of the hepatic hilum morphology.


Asunto(s)
Atresia Biliar/cirugía , Colangiografía , Quistes/complicaciones , Complicaciones Posoperatorias/diagnóstico por imagen , Enfermedades de los Conductos Biliares/complicaciones , Enfermedades de los Conductos Biliares/cirugía , Conductos Biliares , Atresia Biliar/complicaciones , Quistes/cirugía , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Masculino , Pronóstico , Análisis de Supervivencia
17.
Pediatr Surg Int ; 30(9): 871-5, 2014 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-25064225

RESUMEN

PURPOSE: This study aimed to assess outcomes of liver transplantation (LTx) in patients with biliary atresia (BA). METHODS: The Kasai procedure was performed for 358 patients at Tohoku University Hospital between January 1955 and December 2013; 64 (17.9%) required LTx. These 64 patients were divided into 4 groups according to their age at the time of transplantation: Group 1, aged <2 years (n = 27); Group 2, aged 2-9 years (n = 16); Group 3, aged 10-19 years (n = 11); and Group 4, aged ≥20 years (n = 10). Clinical parameters were evaluated retrospectively. RESULTS: Both living-donor (n = 57) and deceased-donor (n = 7) LTx were performed. Indications were irreversible jaundice (n = 53), intractable cholangitis (n = 3), hepatopulmonary syndrome (n = 6), portopulmonary hypertension (n = 1), and intestinal bleeding (n = 1). Jaundice occurred more frequently in Groups 1 and 2 than in Groups 3 and 4 (p = 0.031). Survival rates were 81.5, 100, 90.9, and 80% in Groups 1, 2, 3, and 4, respectively. CONCLUSION: Although the overall LTx survival rate was satisfactory, some adult recipients experienced LTx-related difficulty. Close follow-up, meticulous assessment of physical and social conditions, presence of a multidisciplinary support system, and appropriate time course for LTx are all essential factors in the treatment of BA.


Asunto(s)
Atresia Biliar/cirugía , Trasplante de Hígado/métodos , Portoenterostomía Hepática/métodos , Adolescente , Adulto , Factores de Edad , Niño , Preescolar , Femenino , Humanos , Lactante , Japón , Estimación de Kaplan-Meier , Masculino , Complicaciones Posoperatorias , Estudios Retrospectivos , Tasa de Supervivencia , Resultado del Tratamiento , Adulto Joven
18.
Gastroenterol Clin North Am ; 53(3): 481-491, 2024 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-39068009

RESUMEN

This comprehensive review focuses on advances in surgical techniques and in vivo animal models for treating short bowel syndrome (SBS) with intestinal organoids. Notably, this review discusses a novel method involving the replacement of the epithelium of large intestinal tissue with small intestinal organoids, which improves function and prognosis when grafted back into the small intestine. This study not only underscores the importance of integrating organoid technology and surgical techniques to improve the outcomes of patients with SBS but also acknowledges the challenges that lie ahead, including achieving functional organoids with peristaltic movement and vascularization.


Asunto(s)
Organoides , Síndrome del Intestino Corto , Síndrome del Intestino Corto/cirugía , Humanos , Organoides/trasplante , Animales , Colon/cirugía , Modelos Animales de Enfermedad , Intestino Delgado/trasplante , Mucosa Intestinal/trasplante
19.
J Pediatr Surg ; 59(9): 1791-1797, 2024 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-38839469

RESUMEN

BACKGROUND: Although congenital portosystemic shunts (CPSSs) are increasingly being recognized, the optimal treatment strategies and natural prognosis remain unclear, as individual CPSSs show different phenotypes. METHODS: The medical records of 122 patients who were diagnosed with CPSSs at 15 participating hospitals in Japan between 2000 and 2019 were collected for a retrospective analysis based on the state of portal vein (PV) visualization on imaging. RESULTS: Among the 122 patients, 75 (61.5%) showed PV on imaging. The median age at the diagnosis was 5 months. The main complications related to CPSS were hyperammonemia (85.2%), liver masses (25.4%), hepatopulmonary shunts (13.9%), and pulmonary hypertension (11.5%). The prevalence of complications was significantly higher in patients without PV visualization than in those with PV visualization (P < 0.001). Overall, 91 patients (74.6%) received treatment, including shunt closure by surgery or interventional radiology (n = 82) and liver transplantation (LT) or liver resection (n = 9). Over the past 20 years, there has been a decrease in the number of patients undergoing LT. Although most patients showed improvement or reduced progression of symptoms, liver masses and pulmonary hypertension were less likely to improve after shunt closure. Complications related to shunt closure were more likely to occur in patients without PV visualization (P = 0.001). In 25 patients (20.5%) without treatment, those without PV visualization were significantly more likely to develop complications related to CPSS than those with PV visualization (P = 0.011). CONCLUSION: Patients without PV visualization develop CPSS-related complications and, early treatment using prophylactic approaches should be considered, even if they are asymptomatic. LEVEL OF EVIDENCE: Level III.


Asunto(s)
Vena Porta , Malformaciones Vasculares , Humanos , Japón/epidemiología , Vena Porta/anomalías , Estudios Retrospectivos , Lactante , Pronóstico , Masculino , Femenino , Preescolar , Malformaciones Vasculares/epidemiología , Malformaciones Vasculares/cirugía , Niño , Recién Nacido , Trasplante de Hígado
20.
Pediatr Surg Int ; 29(10): 1065-70, 2013 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-23982390

RESUMEN

INTRODUCTION: We assessed the impact of intestinal transplantation on Japanese pediatric patients with intestinal failure with data from the Japanese intestinal transplant registry. METHODS: Standardized forms were sent to all known intestinal transplantation programs, requesting information on transplants performed between 1996 and June 30, 2012. Patients younger than 18 years were analyzed. Patient and graft survival estimates were obtained using the Kaplan-Meier method. RESULTS: Of the 14 intestinal transplants, 4 were deceased and 10 were living donor transplants. The primary indications were: short gut syndrome (n = 7), intestinal functional disorder (n = 6), and re-transplantation (n = 1). The overall 1- and 5-year patient survival rates were 77 and 57%, respectively. In transplants performed after 2006 (n = 6), the one-year patient survival rate was 83%, and the 5-year survival rate was 83%. Graft one- and 5-year survival rates were 83 and 83%, respectively. The living-related transplant survival rate was 80% at 1 year and 68% at 2 years, compared to 67 and 67% for cadaveric transplant recipients. There were no statistically significant differences in patient (p = 0.88) and graft (p = 0.76) survival rates between living donor and cadaveric transplant recipients. All current survivors discontinued PN. CONCLUSION: Intestinal transplantation has become an effective therapy for patients with intestinal failure who cannot tolerate PN.


Asunto(s)
Rechazo de Injerto/epidemiología , Supervivencia de Injerto , Enfermedades Intestinales/cirugía , Intestinos/trasplante , Sistema de Registros , Donantes de Tejidos/provisión & distribución , Obtención de Tejidos y Órganos/métodos , Adolescente , Niño , Preescolar , Femenino , Humanos , Incidencia , Lactante , Japón/epidemiología , Masculino , Tasa de Supervivencia/tendencias , Resultado del Tratamiento
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