Abstract
Orthotopic liver transplantation (OLT) has been proposed to treat patients with type IV glycogenosis because of early progressive cirrhosis. Reports have shown absence of disease progression in other organs after OLT and even regression of cardiac amylopectin infiltration in one case. We describe a 15-month-old child in whom a liver transplant was performed for type IV glycogenosis. There were no clinical signs of extrahepatic disease before OLT. Nine months later, the patient developed progressive cardiac insufficiency and died from cardiac failure. Because of massive amylopectin deposits, decreased myofibrils in cardiac cells, and exclusion of other causes of cardiac failure, death was attributed to amylopectinosis. Our observation contrasts with the Pittsburgh experience and suggests that cardiac amylopectionosis may progress after OLT.
| Original language | English |
|---|---|
| Pages (from-to) | 200-203 |
| Number of pages | 4 |
| Journal | European Journal of Pediatrics |
| Volume | 151 |
| Issue number | 3 |
| DOIs | |
| Publication status | Published - Mar 1992 |
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Keywords
- Inborn error metabolism
- Liver cirrhosis
- Metabolic diseases
- Orthotopic liver transplantation
- Type IV Glycogenosis
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
Cite this
Progressive cardiac failure following orthotopic liver transplantation for type IV glycogenosis. / Sokal, E. M.; Van Hoof, F.; Alberti, D.; de Ville de Goyet, J.; de Barsy, T.; Otte, J. B.
In: European Journal of Pediatrics, Vol. 151, No. 3, 03.1992, p. 200-203.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Progressive cardiac failure following orthotopic liver transplantation for type IV glycogenosis
AU - Sokal, E. M.
AU - Van Hoof, F.
AU - Alberti, D.
AU - de Ville de Goyet, J.
AU - de Barsy, T.
AU - Otte, J. B.
PY - 1992/3
Y1 - 1992/3
N2 - Orthotopic liver transplantation (OLT) has been proposed to treat patients with type IV glycogenosis because of early progressive cirrhosis. Reports have shown absence of disease progression in other organs after OLT and even regression of cardiac amylopectin infiltration in one case. We describe a 15-month-old child in whom a liver transplant was performed for type IV glycogenosis. There were no clinical signs of extrahepatic disease before OLT. Nine months later, the patient developed progressive cardiac insufficiency and died from cardiac failure. Because of massive amylopectin deposits, decreased myofibrils in cardiac cells, and exclusion of other causes of cardiac failure, death was attributed to amylopectinosis. Our observation contrasts with the Pittsburgh experience and suggests that cardiac amylopectionosis may progress after OLT.
AB - Orthotopic liver transplantation (OLT) has been proposed to treat patients with type IV glycogenosis because of early progressive cirrhosis. Reports have shown absence of disease progression in other organs after OLT and even regression of cardiac amylopectin infiltration in one case. We describe a 15-month-old child in whom a liver transplant was performed for type IV glycogenosis. There were no clinical signs of extrahepatic disease before OLT. Nine months later, the patient developed progressive cardiac insufficiency and died from cardiac failure. Because of massive amylopectin deposits, decreased myofibrils in cardiac cells, and exclusion of other causes of cardiac failure, death was attributed to amylopectinosis. Our observation contrasts with the Pittsburgh experience and suggests that cardiac amylopectionosis may progress after OLT.
KW - Inborn error metabolism
KW - Liver cirrhosis
KW - Metabolic diseases
KW - Orthotopic liver transplantation
KW - Type IV Glycogenosis
UR - http://www.scopus.com/inward/record.url?scp=0026560136&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0026560136&partnerID=8YFLogxK
U2 - 10.1007/BF01954384
DO - 10.1007/BF01954384
M3 - Article
C2 - 1601012
AN - SCOPUS:0026560136
VL - 151
SP - 200
EP - 203
JO - European Journal of Pediatrics
JF - European Journal of Pediatrics
SN - 0340-6199
IS - 3
ER -