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1.
Pediatr Transplant ; 28(1): e14659, 2024 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-38012111

RESUMEN

BACKGROUND: POLG is one of several nuclear genes associated with mitochondrial DNA maintenance defects and is a group of diseases caused by mitochondrial DNA deficiency that results in impaired adenosine triphosphate production and organ dysfunction. Myocerebrohepatopathy spectrum (MCHS) is the most severe and earliest presentation of POLG mutations, and liver transplantation (LT) for MCHS has never been reported. CASE PRESENTATION: The patient was a 3-month-old boy with acute liver failure and no neurological manifestations (e.g., seizures). We performed a living donor LT using a left lateral segment graft from his father. The postoperative course was uneventful. Subsequently, a homozygous POLG mutation (c.2890C>T, p. R964C) was identified by multigene analysis of neonatal/infantile intrahepatic cholestasis. Moreover, respiratory chain complex I, II, and III enzyme activities and the ratio of mtDNA to nuclear DNA in the liver were reduced. Therefore, we considered that these clinical manifestations and examination findings met the definition for MCHS. During meticulous follow-up, the patient had shown satisfactory physical growth and mental development until the time of writing this report. CONCLUSION: We presumed that the absence of remarkable neurologic manifestations prior to LT in patients with MCHS is a good indication for LT and contributes to a better prognosis in the present case.


Asunto(s)
Fallo Hepático Agudo , Trasplante de Hígado , Masculino , Humanos , Recién Nacido , Lactante , ADN Polimerasa Dirigida por ADN/genética , ADN Polimerasa gamma/genética , Donadores Vivos , Mutación , ADN Mitocondrial/genética
2.
Pediatr Cardiol ; 2023 Feb 08.
Artículo en Inglés | MEDLINE | ID: mdl-36754885

RESUMEN

Portopulmonary hypertension is an intractable form of pulmonary hypertension. Although liver transplantation is recommended for patients who respond poorly to treatments, the mechanisms by which liver transplantation improves pulmonary hypertension remain unclear. The present study investigated these mechanisms by retrospectively evaluating patients' data. This study retrospectively evaluated echocardiography and catheterization data before and after liver transplantation in 12 patients who underwent liver transplantation from 2001 to 2019. The 12 patients included one male and 11 females, of median age at liver transplantation of 10 years, 2 months. Nine patients underwent liver transplantation for congenital biliary atresia and three for portal vein aplasia or hypoplasia. Mean pulmonary arterial pressure was 44.1 ± 8.1 mmHg at the first cardiac catheter examination, 35.3 ± 7.8 mmHg before liver transplantation, and 29.5 ± 9.3 mmHg 6 months after liver transplantation. Pulmonary artery pressure was reduced by treatments of pulmonary hypertension and by liver transplantation. Pulmonary vascular resistance did not differ before and after liver transplantation, whereas the cardiac index decreased significantly, indicating that the significant reduction in mean pulmonary artery pressure was due to a decrease in cardiac index. Decreased cardiac index was thought to result from improvements in hyperdynamic conditions due to increased (normalized) systemic vascular resistance. Liver transplantation likely suppresses shear stress on pulmonary arteries, preventing further damage by hyper-circulation. A longer-term evaluation is required to determine the effect of improving pulmonary artery remodeling.

3.
Surg Today ; 53(1): 130-134, 2023 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-35771302

RESUMEN

PURPOSE: Many patients with coronavirus disease 2019 require mechanical ventilation and tracheostomy. However, the timing and indications for tracheostomy are controversial. This study assessed 11 patients with coronavirus disease 2019 who underwent tracheostomy with clinical information and retrospective analyses. METHODS: A single-center retrospective observational study was performed on patients with coronavirus disease 2019 who underwent tracheostomy between 2020 and 2021. RESULTS: Failure to wean was the most common indication for tracheostomy, followed by extracorporeal membrane oxygenation decannulation and the need for secretion management. After tracheostomy, six patients (54.5%) were liberated from the ventilator. The time from intubation to tracheostomy (21.1 ± 9.14 days) was correlated with the duration of ventilator dependency (36.83 ± 20.45 days, r2 = 0.792, p = 0.018). The mean Acute Physiological and Chronic Health Evaluation II score was significantly lower in the ventilator-liberated group (23 ± 2.77) than in the non-ventilator-liberated group (31 ± 6.13, p = 0.0292). Furthermore, patients with Acute Physiological and Chronic Health Evaluation II scores of < 27 points achieved ventilator liberation and a long-term survival (p = 0.0006). CONCLUSIONS: This study describes the outcomes of a cohort of patients who underwent tracheostomy after intubation for coronavirus disease 2019. The Acute Physiological and Chronic Health Evaluation II score predicted whether or not the patient could achieve ventilator liberation.


Asunto(s)
COVID-19 , Desconexión del Ventilador , Humanos , Traqueostomía , Estudios Retrospectivos , Japón
4.
Liver Transpl ; 28(10): 1588-1602, 2022 10.
Artículo en Inglés | MEDLINE | ID: mdl-35603526

RESUMEN

Donor-recipient human leukocyte antigen (HLA) compatibility has not been considered to significantly affect liver transplantation (LT) outcomes; however, its significance in living-donor LT (LDLT), which is mostly performed between blood relatives, remains unclear. This retrospective cohort study included 1954 LDLTs at our institution (1990-2020). The primary and secondary endpoints were recipient survival and the incidence of T cell-mediated rejection (TCMR) after LDLT, respectively, according to the number of HLA mismatches at all five loci: HLA-A, HLA-B, HLA-C, HLA-DR, and HLA-DQ. Subgroup analyses were also performed in between-siblings that characteristically have widely distributed 0-10 HLA mismatches. A total of 1304 cases of primary LDLTs were finally enrolled, including 631 adults (recipient age at LT ≥18 years) and 673 children (<18 years). In adult-to-adult LDLT, the more HLA mismatches at each locus, the significantly worse the recipient survival was (p = 0.03, 0.01, 0.03, 0.001, and <0.001 for HLA-A, HLA-B, HLA-C, HLA-DR, and HLA-DQ, respectively). This trend was more pronounced when multiple loci were combined (all p < 0.001 for A + B + DR, A + B + C, DR + DQ, and A + B + C + DR + DQ). Notably, a total of three or more HLA-B + DR mismatches was an independent risk factor for both TCMR (hazard ratio [HR] 2.66, 95% confidence interval [CI] 1.21-5.87; p = 0.02) and recipient survival (HR 2.44, 95% CI 1.11-5.35; p = 0.03) in between-siblings. By contrast, HLA mismatch did not affect pediatric LDLT outcomes at any locus or in any combinations; however, it should be noted that all donor-recipient relationships are parent-to-child that characteristically possesses one or less HLA mismatch at each locus and maximally five or less mismatches in total. In conclusion, HLA mismatch significantly affects not only TCMR development but also recipient survival in adult LDLT, but not in children.


Asunto(s)
Trasplante de Hígado , Donadores Vivos , Adulto , Niño , Rechazo de Injerto/epidemiología , Supervivencia de Injerto , Antígenos HLA , Antígenos HLA-A , Antígenos HLA-B , Antígenos HLA-C , Antígenos HLA-DQ , Antígenos HLA-DR , Prueba de Histocompatibilidad , Humanos , Trasplante de Hígado/efectos adversos , Estudios Retrospectivos
5.
Liver Transpl ; 28(4): 647-658, 2022 04.
Artículo en Inglés | MEDLINE | ID: mdl-34655506

RESUMEN

Programmed death 1 (PD1)/its ligand PD-L1 concomitant with T cell immunoglobulin and mucin domain-containing protein 3 (TIM-3)/its ligand galectin 9 (Gal-9) and the forkhead box P3 (FOXP3) might be involved in tolerance after liver transplantation (LT). Liver biopsies from 38 tolerant, 19 nontolerant (including 16 samples that triggered reintroduction of immunosuppression [IS] and 19 samples after IS reintroduction), and 38 control LT patients were studied. The expressions of PD1, PD-L1, Gal-9, and FOXP3 were determined by immunohistochemical and immunofluorescence (IF) staining. The success period of IS withdrawal was calculated using Kaplan-Meier curve analysis. Tolerant and control patients exhibited higher PD-L1, Gal-9, and FOXP3 levels than nontolerant patients at the moment of triggering IS reintroduction. High expressions of PD-L1 and Gal-9 were associated with prolonged success of tolerance (83.3% versus 36.7% [P < 0.01] and 73.1% versus 42.9% [P = 0.03]). A strong correlation between PD-L1 and Gal-9 expression levels was detected (Spearman r = 0.73; P ≤ 0.001), and IF demonstrated colocalization of PD-L1 and Gal-9 in the cytoplasm of hepatocytes. In conclusion, the present study demonstrated that increased expressions of PD-L1 and Gal-9 were associated with sustained tolerance after IS withdrawal in pediatric liver transplantation.


Asunto(s)
Antígeno B7-H1 , Trasplante de Hígado , Antígeno B7-H1/análisis , Antígeno B7-H1/metabolismo , Niño , Factores de Transcripción Forkhead/análisis , Galectinas/metabolismo , Humanos , Terapia de Inmunosupresión/efectos adversos , Ligandos , Trasplante de Hígado/efectos adversos , Receptores de Trasplantes
6.
Cancer Sci ; 112(11): 4617-4626, 2021 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-34464480

RESUMEN

Neuroblastoma, the most common extracranial solid tumor of childhood, is thought to arise from neural crest-derived immature cells. The prognosis of patients with high-risk or recurrent/refractory neuroblastoma remains quite poor despite intensive multimodality therapy; therefore, novel therapeutic interventions are required. We examined the expression of a cell adhesion molecule CD146 (melanoma cell adhesion molecule [MCAM]) by neuroblastoma cell lines and in clinical samples and investigated the anti-tumor effects of CD146-targeting treatment for neuroblastoma cells both in vitro and in vivo. CD146 is expressed by 4 cell lines and by most of primary tumors at any stage. Short hairpin RNA-mediated knockdown of CD146, or treatment with an anti-CD146 polyclonal antibody, effectively inhibited growth of neuroblastoma cells both in vitro and in vivo, principally due to increased apoptosis via the focal adhesion kinase and/or nuclear factor-kappa B signaling pathway. Furthermore, the anti-CD146 polyclonal antibody markedly inhibited tumor growth in immunodeficient mice inoculated with primary neuroblastoma cells. In conclusion, CD146 represents a promising therapeutic target for neuroblastoma.


Asunto(s)
Anticuerpos/uso terapéutico , Antígeno CD146/antagonistas & inhibidores , Terapia Molecular Dirigida/métodos , Neuroblastoma/terapia , ARN Interferente Pequeño/uso terapéutico , Animales , Apoptosis , Antígeno CD146/metabolismo , Línea Celular Tumoral , Supervivencia Celular , Quinasa 1 de Adhesión Focal/metabolismo , Proteína-Tirosina Quinasas de Adhesión Focal/metabolismo , Técnicas de Silenciamiento del Gen , Xenoinjertos , Humanos , Ratones , FN-kappa B/metabolismo , Recurrencia Local de Neoplasia , Trasplante de Neoplasias , Neuroblastoma/metabolismo , Neuroblastoma/patología , Pronóstico , Transducción de Señal , Esferoides Celulares , Transducción Genética/métodos
7.
Ann Surg ; 274(6): e599-e604, 2021 12 01.
Artículo en Inglés | MEDLINE | ID: mdl-31977513

RESUMEN

OBJECTIVE: The aim of the study was to investigate the effect of abdominal drainage at appendectomy for complicated appendicitis in children. SUMMARY OF BACKGROUND DATA: Although an abdominal drain placement at appendectomy is an option for reducing or preventing postoperative infectious complication, there is controversy regarding its effect for complicated appendicitis. METHOD: The study used the data on appendectomies for complicated appendicitis in children (≤15 years old) that were operated in 2015 and registered in the National Clinical Database, a nationwide surgical database in Japan. One-to-two propensity score matching was performed to compare postoperative outcomes between patients with and without drainage at appendectomy. RESULT: The study included 1762 pediatric appendectomies for complicated appendicitis, 458 of which underwent abdominal drainage at appendectomy. In the propensity-matched analysis, the drainage group showed a significant increase in wound dehiscence [drain (-) vs drain (+); 0.3% vs 2.4%, P = 0.001], and postoperative hospital stay (median: 7 days vs 9 days, P < 0.001). There were no significant differences in the incidence of any complications, organ space surgical site infection, re-admission, and reoperation.Subgroup analyses in perforated appendicitis and perforated appendicitis with abscess, and open and laparoscopic appendectomy all demonstrated that drain placement was not associated with a reduction in any complication or organ space surgical site infection. However, it was significantly associated with longer hospital stays. CONCLUSION: This study suggested that an abdominal drain placement at appendectomy for complicated appendicitis among children has no advantage and can be harmful for preventing postoperative complications.


Asunto(s)
Absceso Abdominal/prevención & control , Apendicectomía , Apendicitis/complicaciones , Apendicitis/cirugía , Drenaje , Infección de la Herida Quirúrgica/prevención & control , Adolescente , Apendicectomía/efectos adversos , Niño , Preescolar , Drenaje/efectos adversos , Femenino , Humanos , Tiempo de Internación , Masculino , Puntaje de Propensión , Dehiscencia de la Herida Operatoria/etiología
8.
Liver Transpl ; 27(8): 1165-1180, 2021 08.
Artículo en Inglés | MEDLINE | ID: mdl-33655645

RESUMEN

Post-transplant lymphoproliferative disorders (PTLDs) are life-threatening neoplasms after organ transplantation. Because of their rarity and multiple grades of malignancy, the incidence, outcomes, and clinicopathological features affecting patient survival after liver transplantation (LT) remain unclear. We reviewed 1954 LTs in 1849 recipients (1990-2020), including 886 pediatric (<18 years of age) and 963 adult recipients. The following clinicopathological factors were studied: age, sex, liver etiologies, malignancy grades, Epstein-Barr virus status, performance status (PS), Ann Arbor stage, international prognostic index, and histopathological diagnosis. Of 1849 recipients, 79 PTLD lesions (4.3%) were identified in 70 patients (3.8%). After excluding 3 autopsy cases incidentally found, 67 (45 pediatric [5.1%] and 22 adult [2.3%]) patients were finally enrolled. Comorbid PTLDs significantly worsened recipient survival compared with non-complicated cases (P < 0.001). The 3-year, 5-year, and 10-year overall survival rates after PTLD diagnosis were 74%, 66%, and 58%, respectively. The incidence of PTLDs after LT (LT-PTLDs) was significantly higher (P < 0.001) with earlier onset (P = 0.002) in children, whereas patient survival was significantly worse in adults (P = 0.002). Univariate and multivariate analyses identified the following 3 prognostic factors: age at PTLD diagnosis ≥18 years (hazard ratio [HR], 11.2; 95% confidence interval [CI], 2.63-47.4; P = 0.001), PS ≥2 at diagnosis (HR, 6.77; 95% CI, 1.56-29.3; P = 0.01), and monomorphic type (HR, 6.78; 95% CI, 1.40-32.9; P = 0.02). A prognostic index, the "LT-PTLD score," that consists of these 3 factors effectively stratified patient survival and progression-free survival (P = 0.003 and <0.001, respectively). In conclusion, comorbid PTLDs significantly worsened patient survival after LT. Age ≥18 years and PS ≥2 at PTLD diagnosis, and monomorphic type are independent prognostic factors, and the LT-PTLD score that consists of these 3 factors may distinguish high-risk cases and guide adequate interventions.


Asunto(s)
Infecciones por Virus de Epstein-Barr , Trasplante de Hígado , Trastornos Linfoproliferativos , Adolescente , Adulto , Niño , Infecciones por Virus de Epstein-Barr/diagnóstico , Infecciones por Virus de Epstein-Barr/epidemiología , Herpesvirus Humano 4 , Humanos , Trasplante de Hígado/efectos adversos , Trastornos Linfoproliferativos/diagnóstico , Trastornos Linfoproliferativos/epidemiología , Trastornos Linfoproliferativos/etiología , Complicaciones Posoperatorias/epidemiología , Estudios Retrospectivos , Factores de Riesgo
9.
J Pediatr Gastroenterol Nutr ; 72(3): 425-429, 2021 03 01.
Artículo en Inglés | MEDLINE | ID: mdl-33264179

RESUMEN

OBJECTIVES: Progressive familial intrahepatic cholestasis type 1 (PFIC-1), an autosomal recessive disorder, is characterized by cholestasis, jaundice, and refractory pruritus. In some patients with PFIC-1, liver cirrhosis and end-stage liver disease develop and lead to liver transplantation (LT). In this observational study, we sought to clarify the long-term outcomes of LT for PFIC-1 and predictors of favorable outcomes. METHODS: The study cohort constituted 12 patients with PFIC-1 who had undergone living donor liver transplantation (LDLT) during the previous 3 decades (1990-2019). We compared the clinical manifestations and type of ATP8B1 mutations between patients in whom LDLT had been successful and those in whom it had been unsuccessful. RESULTS: LDLT failed in 5 of the 12 patients and the 25-year survival rate was 58%. Comparison of physical growth after LDLT revealed significant retardation of stature in patients in whom LDLT had been unsuccessful; these patients developed severe and persistent diarrhea. ATP8B1 genotypic analysis revealed that frameshifting, splicing, and large deletion mutations occurred more commonly in successful cases, whereas missense mutations occurred more frequently in unsuccessful cases. No mutations were identical in the 2 groups. CONCLUSIONS: These results suggest an association between post-LT outcomes and extrahepatic manifestations, especially intestinal function. Further investigation of correlations between ATP8B1 genotypes and intestinal function could help to identify patients with PFIC-1 who will achieve favorable post-LT outcomes.


Asunto(s)
Colestasis Intrahepática , Colestasis , Trasplante de Hígado , Adenosina Trifosfatasas , Colestasis Intrahepática/genética , Colestasis Intrahepática/cirugía , Progresión de la Enfermedad , Humanos , Donadores Vivos
10.
J Infect Chemother ; 27(7): 1043-1050, 2021 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-33865699

RESUMEN

INTRODUCTION: Most of the currently used prognostic models for COVID-19 are based on Western cohorts, but it is unknown whether any are applicable to patients with COVID-19 in Japan. METHODS: This retrospective cohort study included 160 patients with COVID-19 who were admitted to the National Center for Global Health and Medicine between January 26, 2020 and July 25, 2020. We searched PubMed for prognostic models for COVID-19. The predicted outcome was initiation of respiratory support or death. Performance of the candidate models was evaluated according to discrimination and calibration. We recalibrated the intercept of each model with our data. We also updated each model by adding ß2-microglobulin (ß2MG) to the model and recalculating the intercept and the coefficient of ß2MG. RESULTS: Mean patient age was 49.8 years, 68% were male, 88.7% were Japanese. The study outcomes occurred in 15 patients, including two deaths. Two-hundred sixty-nine papers were screened, and four candidate prognostic models were assessed. The model of Bartoletti et al. had the highest area under receiver operating characteristic curve (AUC) (0.88; 95% confidence interval 0.81-0.96). All four models overestimated the probability of occurrence of the outcome. None of the four models showed statistically significant improvement in AUCs by adding ß2MG. CONCLUSIONS: Our results suggest that the existing prediction models for COVID-19 overestimate the probability of occurrence of unfavorable outcomes in a Japanese cohort. When applying a prediction model to a different cohort, it is desirable to evaluate its performance according to the prevalent health situation in that region.


Asunto(s)
COVID-19 , Humanos , Japón/epidemiología , Masculino , Persona de Mediana Edad , Pronóstico , Curva ROC , Estudios Retrospectivos , SARS-CoV-2
11.
J Clin Apher ; 36(3): 313-321, 2021 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-33325084

RESUMEN

OBJECTIVE: To evaluate the efficacy and safety of direct hemoperfusion using a polymyxin B-immobilized polystyrene column (PMX-DHP) in severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-positive pneumonia patients. METHODS: This study was a case series conducted at a designated infectious diseases hospital. Twelve SARS-CoV-2-positive patients with partial pressure of arterial oxygen/percentage of inspired oxygen (P/F) ratio < 300 were treated with PMX-DHP on two consecutive days each during hospitalization. We defined day 1 as the first day when PMX-DHP was performed. PMX-DHP efficacy was assessed on days 7 and 14 after the first treatment based on eight categories. Subsequently, improvement in P/F ratio and urinary biomarkers on days 4 and 8, malfunctions, and ventilator and extracorporeal membrane oxygenation avoidance rates were also evaluated. RESULTS: On day 14 after the first treatment, disease severity decreased in 58.3% of the patients. P/F ratio increased while urine ß2-microglobulin decreased on days 4 and 8. Cytokine measurement pre- and post-PMX-DHP revealed decreased levels of interleukin-6 and the factors involved in vascular endothelial injury, including vascular endothelial growth factor. Twenty-two PMX-DHPs were performed, of which seven and five PMX-DHPs led to increased inlet pressure and membrane coagulation, respectively. When the membranes coagulated, the circuitry needed to be reconfigured. Circuit problems were usually observed when D-dimer and fibrin degradation product levels were high before PMX-DHP. CONCLUSIONS: Future studies are expected to determine the therapeutic effect of PMX-DHP on COVID-19. Because of the relatively high risk of circuit coagulation, coagulation capacity should be assessed beforehand.


Asunto(s)
COVID-19/terapia , Hemoperfusión/instrumentación , Hemoperfusión/métodos , Polimixina B/química , Poliestirenos/química , Adulto , Anciano , Anciano de 80 o más Años , Arterias/metabolismo , Biomarcadores/orina , Análisis de los Gases de la Sangre , Citocinas/sangre , Endotelio Vascular/metabolismo , Femenino , Hospitalización , Humanos , Masculino , Persona de Mediana Edad , Oxígeno/metabolismo , Respiración Artificial , Estudios Retrospectivos , Riesgo , Microglobulina beta-2/orina
12.
Pediatr Transplant ; 24(3): e13675, 2020 05.
Artículo en Inglés | MEDLINE | ID: mdl-32068328

RESUMEN

We utilized patient-specific 3D liver models based on preoperative computed tomography images as intraoperative navigation and describe our experience. A 1-year and 10-month-old girl with situs inversus totalis underwent living donor liver transplantation for biliary atresia. Information on the hepatic artery, portal vein, inferior vena cava, and liver parenchyma was extracted and segmented from computed tomography images using liver analysis software. Laser lithography produced each 3D part of the liver from these data. The 3D models of each part of the liver were molded from polyurethane resin using different colors for each part and combined together, resulting in a patient-specific liver model. The industrial computed tomography scan of the patient-specific 3D liver model revealed that the gaps between the liver model and the original data were <0.4 mm in the 90% area, <0.8 mm in the 98% area, and 1.53 mm at the maximum. The 3D liver model was brought into the operative field and used as intraoperative navigation for total liver resection. The procedure was finished successfully without any major intraoperative complications. In conclusion, the 3D model facilitates the identification of vessels during operations; it is possible to promptly share patients' anatomy with the operative team.


Asunto(s)
Anomalías Múltiples/cirugía , Atresia Biliar/cirugía , Trasplante de Hígado/métodos , Donadores Vivos , Modelos Anatómicos , Impresión Tridimensional , Situs Inversus/cirugía , Femenino , Humanos , Lactante , Hígado/anomalías , Hígado/diagnóstico por imagen , Hígado/cirugía , Trasplante de Hígado/instrumentación , Tomografía Computarizada por Rayos X
13.
Pediatr Transplant ; 24(8): e13813, 2020 12.
Artículo en Inglés | MEDLINE | ID: mdl-33099865

RESUMEN

Severe post-transplant hypoxemia, which is defined as <50 mm Hg of the partial pressure of oxygen in arterial blood/fraction of inspired oxygen (P/F) ratio, is a major post-operative complication with high mortality rates in patients with hepatopulmonary syndrome (HPS). Non-invasive positive pressure ventilation therapy and mechanical ventilation are options for respiratory support of patients with severe post-transplant hypoxemia. However, these therapies are associated with several problems, such as compliance, ventilator-associated pneumonia, and lung injury. We here firstly described two children with HPS who developed severe post-transplant hypoxemia (lowest post-operative P/F ratio, 49.7 and 34.0 mm Hg, respectively) that was successfully managed with high-flow nasal cannula (HFNC) oxygen therapy and vasodilation drugs without adverse complications or necessity of reintubation. We consider that HFNC oxygen therapy could become a safe alternative respiratory therapy or be added to the other such as inhaled nitric oxide (iNO), methylene blue (MB), inhaled epoprostenol, embolization of abnormal pulmonary vessels, and combination of iNO and MB for severe post-transplant hypoxemia in children with HPS.


Asunto(s)
Síndrome Hepatopulmonar/cirugía , Hipoxia/terapia , Trasplante de Hígado , Terapia por Inhalación de Oxígeno/métodos , Complicaciones Posoperatorias/terapia , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino
14.
Gan To Kagaku Ryoho ; 47(13): 2269-2271, 2020 Dec.
Artículo en Japonés | MEDLINE | ID: mdl-33468930

RESUMEN

We present a 46-year-old female patient who underwent resection of a retroperitoneal tumor, which was found by medical check-up. The tumor which was elastic hard and had good mobility displaced the duodenum to her abdominal wall. Since her right ovarian vein adhered to the tumor, we removed the tumor with the ligated vein. Pathological findings showed the tumor consisted of spindle-shaped cells with pleomorphic nucleus and it presented the fascicular growth pattern. Additional immunostaining showed positive for HHF35, h-caldesmon. Because the leiomyosarcoma connected with the smooth muscle of the right ovarian vein, we considered the vascular smooth muscle was the origin of the tumor. It is 2 years after the operation, there has been no local recurrence or metastasis.


Asunto(s)
Leiomiosarcoma , Neoplasias Retroperitoneales , Femenino , Humanos , Leiomiosarcoma/cirugía , Persona de Mediana Edad , Recurrencia Local de Neoplasia , Venas , Vena Cava Inferior
15.
Gan To Kagaku Ryoho ; 47(13): 2335-2337, 2020 Dec.
Artículo en Japonés | MEDLINE | ID: mdl-33468952

RESUMEN

An 80-year-old woman who visited our hospital with chief complaints of decreased appetite and diarrhea. Lower gastrointestinal endoscopy showed a type 2 tumor in the rectal Ra, and biopsy revealed a well-differentiated adenocarcinoma. The patient had locally advanced rectal cancer with widespread contact with the sacrum, and preoperative radiochemotherapy (S-1 100 mg/day plus radiotherapy 50 Gy/25 Fr)was performed. After the treatment was completed, the tumor was remarkably reduced, but the surgery was strongly rejected, and therefore chemotherapy became the policy. XELOX plus bevacizumab therapy was started, but in the second course was performed, obstructive symptoms appeared, so a semi-urgent lower anterior resection and ileostomy were performed. Postoperative pathological findings showed only a small amount of tumor cells in the mucosa, suggesting that preoperative treatment was effective.


Asunto(s)
Adenocarcinoma , Neoplasias del Recto , Adenocarcinoma/terapia , Anciano de 80 o más Años , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Quimioradioterapia , Femenino , Fluorouracilo/uso terapéutico , Humanos , Estadificación de Neoplasias , Neoplasias del Recto/patología
17.
Gan To Kagaku Ryoho ; 46(1): 127-129, 2019 Jan.
Artículo en Japonés | MEDLINE | ID: mdl-30765663

RESUMEN

A 79-year-old man was diagnosed with Stage ⅢB(T4N2M0)adenocarcinoma of the lung, administered. He suddenly developed abdominal pain with muscle guarding and rebound tenderness. An abdominal computed tomography scan revealed a thickened small bowel wall and mesenteric mass, as well as massive ascites and free air. He underwent an emergency laparotomy following a diagnosis of pan-peritonitis due to intestinalperforation. A partialresection of the smallintestine and abdominal drainage were performed. The resected specimen included an ulcerative lesion on the mucosal surface. The pathological diagnosis was a metastasis of lung cancer. The patient died in hospice 29 days postoperatively. In the present case, however, surgery improved the patient's quality of life. Although lung cancer metastasis to the small bowel is associated with a poor prognosis, palliative surgery is indicated in otherwise fatal circumstances.


Asunto(s)
Adenocarcinoma del Pulmón , Perforación Intestinal , Neoplasias Pulmonares , Adenocarcinoma del Pulmón/patología , Anciano , Humanos , Perforación Intestinal/etiología , Neoplasias Pulmonares/patología , Masculino , Metástasis de la Neoplasia , Calidad de Vida
18.
Gan To Kagaku Ryoho ; 46(13): 2303-2305, 2019 Dec.
Artículo en Japonés | MEDLINE | ID: mdl-32156912

RESUMEN

Laparoscopy and endoscopy cooperative surgery(LECS)is a surgical technique to resect a tumor with minimal invasion, using both a laparoscope and endoscope. Twenty-eight surgeries for gastric submucosal tumors(SMT)were performed between 2009 and 2019. Seven of those cases were performed using LECS. Two male and 5 female patients underwent LECS; their mean age was 53 years. The tumors were located at the anterior wall of the fornix in 1 case, anterior wall of the subcardia in 2 cases, anterior wall of the upper gastric body in 3 cases, and anterior wall of the lower gastric body in 1 case. Two cases were intraductal growing types, and 5 cases were intramural growing types. No postoperative complications have occurred. The mean size of the tumors was 21.1 mm. In pathological findings, 5 cases were gastrointestinal stromal tumor (GIST); 1 case was high risk, 2 cases were low risk, and 1 case was very low risk as classified using the modified-Fletcher's classification. Imatinib was administered to the high risk case, and there have been no recurrences in any cases.


Asunto(s)
Neoplasias Gastrointestinales , Tumores del Estroma Gastrointestinal , Laparoscopía , Femenino , Neoplasias Gastrointestinales/cirugía , Tumores del Estroma Gastrointestinal/cirugía , Humanos , Masculino , Persona de Mediana Edad , Recurrencia Local de Neoplasia , Complicaciones Posoperatorias , Resultado del Tratamiento
19.
Gan To Kagaku Ryoho ; 46(13): 2312-2314, 2019 Dec.
Artículo en Japonés | MEDLINE | ID: mdl-32156915

RESUMEN

A 55-year-old man was admitted to our hospital for jaundice. Magnetic resonance cholangiopancreatography showed a mass in the pancreatic head as well as biliary obstruction. We strongly suspected invasive ductal carcinoma of the pancreas. We performed pancreaticoduodenectomy with partial resection of the portal vein. The histopathological diagnosis was small cell carcinoma of the pancreas. We detected metastasis of the right hilar lymph node in PET-CT scan performed 2 months after the surgery and started chemotherapy with cisplatin(CDDP)plus irinotecan(CPT-11). However, we observed recurrent metastasis of the right hilar lymph node 12 months after the surgery. We started second-line chemotherapy with amrubicin( AMR)and radiotherapy. Unfortunately, the patient died from multiple metastases of the left adrenal gland and brain 26 months after the surgery. The prognosis of small cell carcinoma of the pancreas is extremely poor. Multimodal treatment such as chemotherapy, radiotherapy, and curative operation are required for long-term survival.


Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Carcinoma de Células Pequeñas , Neoplasias Pancreáticas , Carcinoma de Células Pequeñas/diagnóstico por imagen , Carcinoma de Células Pequeñas/tratamiento farmacológico , Cisplatino , Humanos , Irinotecán , Masculino , Persona de Mediana Edad , Neoplasias Pancreáticas/diagnóstico por imagen , Neoplasias Pancreáticas/tratamiento farmacológico , Tomografía Computarizada por Tomografía de Emisión de Positrones
20.
Gan To Kagaku Ryoho ; 46(13): 2005-2007, 2019 Dec.
Artículo en Japonés | MEDLINE | ID: mdl-32157041

RESUMEN

A63 -year-old man complaining of anal pain visited our hospital. Three years 6 months previously, the patient underwent endoscopic submucosal dissection(ESD)for early-stage rectal cancer. Based on the pathological findings, adenocarcinoma with invasion to the submucosal layer(2,000 mm)and lymphovascular invasion were diagnosed. Abdominal computed tomography( CT)revealed a solid tumor 50mm in diameter and hematoma measuring approximately 90mm in length adjoining the tumor in the mesorectum. We performed exploratory laparoscopy. Ahematoma was confirmed in the mesentery from the sigmoid colon and rectum. After the surgery, endoscopic ultrasound-guided fine needle aspiration(EUS-FNA)revealed well-differentiated adenocarcinoma. We diagnosed a hematoma associated with mesenteric recurrence following ESD for rectal cancer. The patient received chemotherapy first because of the large size of the recurrent cancer. Four courses of mFOLFOX6(5-FU: bolus 400mg/m / / / 2,2,400mg/m2,oxaliplatin 85 mg/m2) and panitumumab(6 mg/kg)were administered. Based on the CT findings following chemotherapy, the hematoma had disappeared, and the size of the recurrent cancer in the mesorectum reduced to 28 mm. The patient underwent laparoscopic lower anterior resection with D3 lymph node dissection and ileostomy. The postoperative course was uneventful. Currently, the patient has no recurrence.


Asunto(s)
Hematoma/cirugía , Recurrencia Local de Neoplasia , Neoplasias del Recto , Terapia Combinada , Humanos , Masculino , Mesenterio , Persona de Mediana Edad , Neoplasias del Recto/tratamiento farmacológico , Neoplasias del Recto/cirugía , Recto
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