Publications by authors named "Cal Matsumoto"

Intestinal failure (IF) remains as a life-threatening medical condition worldwide, but the disparity on the type and quality of medical care available, together with the different limitations to access among individual countries or regions, turned IF assessment and therapy into a difficult matter, which becomes a major hazard for the developing world. This article aims to provide an update regarding definitions used, the current general worldwide data, the developments, achievements, and the different access alternatives in Latin-America, Middle East, and Asia to exemplify what can be done to help patients with IF.

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Objectives: To review recent evaluations of pediatric patients with intestinal failure (IF) for intestinal transplantation (ITx), waiting list decisions, and outcomes of patients listed and not listed for ITx at our center.

Methods: Retrospective chart review of 97 patients evaluated for ITx from January 2014 to December 2021 including data from referring institutions and protocol laboratory testing, body imaging, endoscopy, and liver biopsy in selected cases. Survival analysis used Kaplan-Meier estimates and Cox proportional hazards regression.

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Background: Intestinal transplant (ITx) rejection is associated with memory T helper type 17 cell (Th17) infiltration of grafted tissues. Modulation of Th17 effector cell response is facilitated by T regulatory (Treg) cells, but a phenotypic characterization of this process is lacking in the context of allograft rejection.

Methods: Flow cytometry was performed to examine the expression of surface receptors, cytokines, and transcription factors in Th17 and Treg cells in ITx control (n = 34) and rejection patients (n = 23).

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Background: Idiopathic ileal ulceration after intestinal transplantation (ITx) has been discussed infrequently and has an uncertain natural history and relation to graft rejection. Herein, we review our experience with this pathology.

Methods: We retrospectively reviewed 225 ITx in 217 patients with minimum 1 y graft survival.

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Background: Unlike other solid organs, no standardized treatment algorithms exist for intestinal transplantation (ITx). We established a consortium of American ITx centers to evaluate current practices.

Methods: All American centers performing ITx during the past 3 y were invited to participate.

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Article Synopsis
  • Planning for future SARS-CoV-2 outbreaks is critical for healthcare providers treating immunocompromised children, especially those with solid organ transplants.
  • A study analyzed 77 children with different types of transplants diagnosed with COVID-19, focusing on factors that influence the severity of their illness.
  • Results indicated that a higher lymphocyte count at diagnosis reduced the risk of severe disease, while a higher monocyte count in the months before infection increased the risk; steroid use and immunosuppressive medication levels did not significantly impact severity outcomes.
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We analyzed the fecal microbiome by deep sequencing of the 16S ribosomal genes and the metabolomic profiles of 43 intestinal transplant recipients to identify biomarkers of graft function. Stool samples were collected from 23 patients with stable graft function five years or longer after transplant, 15 stable recipients one-year post-transplant and four recipients with refractory rejection and graft loss within one-year post-transplant. and species were predominant in patients with stable graft function both in the short and long term, with a microbiome profile consistent with the general population.

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Objectives: This is a descriptive study to characterize rates of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in pediatric solid organ transplant (SOT) recipients during the early days of the pandemic. We hypothesized that asymptomatic infection may represent a large proportion of SARS-CoV-2 infection in pediatric SOT recipients.

Methods: We queried Organ Transplant Tracking Record (OTTR) for all pediatric SOT recipients followed at our center and reviewed medical records to identify patients tested for SARS-CoV-2 between March 15, 2020 and June 30, 2021.

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Short bowel syndrome is the most common etiology of intestinal failure, resulting from either resections of different intestinal segments or a congenital condition. Due to the absence or considerable reduction of intestinal loops in the abdominal cavity, patients with short bowel syndrome present with atrophy and muscle retraction of the abdominal wall, which leads to loss of abdominal domain and elasticity. This complication is an aggravating factor of intestinal transplantation since it can prevent the primary closure of the abdominal wall.

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Article Synopsis
  • On February 4, 2020, liver allocation changed from Donor Service Areas to acuity circles, but the effects on waitlist outcomes for combined liver-intestine transplantation remain unclear.
  • An analysis of data from the Organ Procurement and Transplantation Network revealed that while the 90-day waitlist mortality in adults was similar before and after the change, their chances of receiving a transplant decreased significantly post-acuity circles.
  • For pediatric patients, there was no significant difference in waitlist outcomes, but both adult and pediatric patients saw a reduced proportion of transplants granted with exception points after the change, indicating a potential need for improved organ allocation strategies.
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Background: Graft versus host disease (GVHD) is an uncommon but highly morbid complication of intestinal transplantation (ITx). In this study, we reviewed our 17-y experience with GVHD focusing on factors predicting GVHD occurrence and survival.

Methods: Retrospective review of 271 patients who received 1 or more ITx since program inception in 2003 with survival analysis using Cox proportional hazard modeling.

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Background: Warm-antibody AIHA is known to complicate solid organ (SOT) and HSCT, the disease maybe refractory to standard therapy. Immunosuppressive therapies as well as IVIG, and rituximab have been the main stay of treatment. Over the past decade, B-lymphocyte targeted, anti-CD-20 antibody has been recognized in the treatment of autoimmune diseases and utilized in AIHA.

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Purpose Of Review: It has been well established that antibody to donor HLA pretransplant and the development of anti-human leukocyte antigen (HLA) antibodies posttransplant contribute to inferior graft survival outcomes. This article serves to review the current status of the management of pretransplant sensitized intestinal transplant candidate as well as to review posttransplant care of patients that harbor antidonor HLA antibodies.

Recent Findings: The intestinal transplant candidate oftentimes presents for transplant listing with high levels of anti-HLA antibodies that necessitate a careful preoperative strategy to avoid a donor-recipient pair that would result in a positive crossmatch.

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Graft-versus-host disease (GvHD) is a common, morbid complication after intestinal transplantation (ITx) with poorly understood pathophysiology. Resident memory T cells (T ) are a recently described CD69+ memory T cell subset localizing to peripheral tissue. We observed that T effector memory cells (T ) in the blood increase during GvHD and hypothesized that they derive from donor graft CD69+T migrating into host blood and tissue.

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Article Synopsis
  • Intestinal transplantation is a potential solution for children with permanent intestinal failure, and a study was conducted to analyze long-term survival rates of grafts in pediatric patients from 2003 to 2013.
  • The study found that 5-year and 10-year graft survival rates were 71% and 65%, with better outcomes seen in patients with anatomic intestinal failure compared to those with functional failure.
  • Key factors associated with better graft survival included fewer complications like graft-versus-host disease and lower donor-recipient weight ratios, indicating that avoiding severe complications is crucial for post-transplant success.
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Acute graft-versus-host disease (GvHD) has been a clinical problem in solid organ transplant that includes intestine due to the donor lymphoid tissue mass which accompanies the intestinal component of the graft. We report a case that demonstrated the efficacy and feasibility of ruxolitinib a JAK 1/2 inhibitor in the treatment of chronic steroid-refractory GVHD (SR-GVHD). The child developed SR-GVHD following a composite intestine transplant (small bowel, colon, liver, and pancreas).

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Intestinal transplantation (ITx) can be life-saving for patients with advanced intestinal failure experiencing complications of parenteral nutrition. New surgical techniques and conventional immunosuppression have enabled some success, but outcomes post-ITx remain disappointing. Refractory cellular immune responses, immunosuppression-linked infections, and posttransplant malignancies have precluded widespread ITx application.

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De novo HCC following transplantation in a child is a rare occurrence. Even within the adult liver transplantation population, there are a limited number of published cases. In this report, we present a case of de novo HCC found in a child, post-multivisceral transplantation.

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By presenting the first case report of true operational tolerance in an intestinal transplant patient, we aim to demonstrate that tolerance is possible in a field that has been hampered by suboptimal outcomes. Although operational tolerance has been achieved in liver and kidney transplantation, and some intestinal transplant patients have been able to decrease immunosuppression, this is the first instance of true operational tolerance after complete cessation of immunosuppression. A patient received a deceased-donor small intestinal and colon allograft with standard immunosuppressive treatment, achieving excellent graft function after overcoming a graft-versus-host-disease episode 5 months posttransplant.

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Background: Despite improved outcomes in the modern era of targeted immunotherapy, intestinal failure and chronic parenteral nutrition remains a significant burden for patients with Crohn's disease (CD) worldwide. Transplantation is a key component of management when a patient with CD suffers from life-threatening complications of parenteral nutrition. Nucleotide-binding oligomerization domain 2 (NOD2) mutation is a risk factor for both development of CD and intestinal allograft rejection.

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Although innate lymphoid cells (ILCs) play fundamental roles in mucosal barrier functionality and tissue homeostasis, ILC-related mechanisms underlying intestinal barrier function, homeostatic regulation, and graft rejection in intestinal transplantation (ITx) patients have yet to be thoroughly defined. We found protective type 3 NKp44 ILCs (ILC3s) to be significantly diminished in newly transplanted allografts, compared to allografts at 6 months, whereas proinflammatory type 1 NKp44 ILCs (ILC1s) were higher. Moreover, serial immunomonitoring revealed that in healthy allografts, protective ILC3s repopulate by 2-4 weeks postoperatively, but in rejecting allografts they remain diminished.

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Combining HSCT with SOT is an unusual and challenging undertaking given the complexities of immune modulation, the need to balance comorbidities, and the cumulative potential for complications. Early life-threatening complications include infections and related effects, graft rejection, and GVHD can be expected to be increased especially if the HSCT is indicated for high-risk cases such as individuals with severe combined immune deficiency and SOT that includes an intestine graft. Herein, we report such a case.

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