Donor immune reconstitution after liver–small bowel transplantation for multiple intestinal atresia with immunodeficiency

Our aim was to analyze the clinical course and outcome of patients with graft vs. host disease (GVHD) after intestinal transplantation (ITx). All patients receiving ITx between May, 1990 and December, 2003 were retrospectively reviewed for evidence of GVHD. Two hundred and fifty patients underwent ITx during the study period. Graft vs. host disease was suspected clinically in 23 patients on the clinical basis of presentation such as skin rash, ulceration of oral mucosa, diarrhea, lymphadenopathy, or native liver dysfunction. Fourteen (eight children and six adults) patients (5.6% of total patient population) had GVHD confirmed by histopathological criteria including keratinocyte necrosis (n = = 9), epithelial apoptosis of the native gastrointestinal tract (n = = 4), and epithelial cell necrosis of oral mucosa (n = = 1). Donor-cell tissue infiltration or extensive peripheral blood donor-cell chimerism was documented on seven occasions. The majority of cases of GVHD resolved with steroid administration and optimization of tacrolimus immunosuppression.The incidence of histologically proven GVHD after clinical intestinal transplantation is 6.5% (8/122) in children and 4.7% (6/128) in adults. Successful clinical management requires a high index of suspicion to minimize morbidity and mortality. Diagnostic and treatment strategies based on this experience are proposed.

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“…Immune system dysfunction may predispose to GVHD (34,35). Immune deficiency was present in one of the confirmed cases in this series.…”

Section: Discussionsupporting

confidence: 54%

Graft Versus Host Disease in Intestinal Transplantation

Mazariegos¹,

Abu‐Elmagd²,

Jaffe³

et al. 2004

American Journal of Transplantation

140

119

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“…The outcome of combined HMIA and immunodeficiency is bleak. Two of our reviewed cases had undergone liver-small bowel transplantation; one developed graft-versus-host disease21 and the second died at the age of 13 months from parainfluenza virus infection 16. BMT is an established successful mode of treatment for SCID 22.…”

Section: Discussionmentioning

confidence: 99%

Hereditary multiple intestinal atresia (HMIA) with severe combined immunodeficiency (SCID): a case report of two siblings and review of the literature on MIA, HMIA and HMIA with immunodeficiency over the last 50 years

Ali

Rahman²,

Bhat

et al. 2011

Case Reports

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Hereditary multiple intestinal atresia (HMIA), a presumed autosomal recessive disorder, is an unusual and rare form of recurrent intestinal atresia which can be associated with severe combined immunodeficiency (SCID). The combination of HMIA and SCID is invariably lethal. The authors describe this fatal association in two siblings. The parents are consanguineous and have three other normal healthy children. Both index cases had abnormal antenatal ultrasounds and were symptomatic after birth. The final diagnosis of HMIA with SCID was confirmed in both siblings. They were never able to receive enteral feeds, remained totally dependent on parenteral nutrition, had repeated episodes of sepsis and died after a very difficult neonatal intensive care course. In this article we have reviewed the clinical course and outcome of both cases. The existing literature on multiple intestinal atresia, HMIA and HMIA with immunodeficiency is also reviewed.

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“…[8][9][10][11] There is no current screening program for SCID, which is caused by diverse mutations in any of at least 11 known genes and others not presently known, as summarized in Table I. [12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28] Screening newborns for treatable genetic disorders allows early intervention. 29,30 After the introduction by Guthrie of filter paper spotted with heel-stick blood to screen for phenylketonuria (PKU), 31 all states in the United States test newborns for PKU, hypothyroidism, and galactosemia.…”

mentioning

confidence: 99%