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Complications during home parenteral nutrition in children affected by congenital microvillous atrophy
ELSEVIER
Complications by Congenital M. Gambarara,
During Home Parenteral Microvillous Atrophy
F. Ferretti, 6. Papadatou,
A. Diamanti,
A
MONG the heterogeneous group of intractable diarrheas in infancy is a now better-identified new clinical entity, intestinal epithelial dysplasia (IED).’ These forms, together with autoimmune enteropathies, enteropathies described in immunological deficits,2 and those associated to some rare inborn errors of metabolism and/or in some genetical syndromes represent the most severe microvillous atrophies.“.4 Intestinal epithelial dysplasias reveal themselves with intractable diarrhea starting in the neonatal period: the diarrhea is severe, watery, with electrolitic losses and without extradigestive manifestations. It is resistent to all the most common dietetic and pharmacological treatments and also to total bowel rest. An histological picture of severe microvillous atrophy with normal or hyperplastic cryptae is always present but is only sometimes associated with microvillous inclusions.4 These conditions that represent the more typical example of irreversible intestinal failure may be considered today to be one of the more frequent indications to small bowel transplant (Sb-Tx) in childhood. Parenteral nutrition (PN) has completely modified prognosis of this disease assuring to the patients not only survival but also normal growth while waiting for Sb-Tx. In these cases PN is started soon after birth and has a prolonged duration with home parenteral nutrition (HPN) programs. It is a total PN that assures the whole nutritional need and that has to cover the persistent severe fecal losses. In these patients, PN dependence and its complications in particular influence Sb-Tx timing. The aim of this study is to evaluate the complications we observed during HPN in three cases of congenital microvillous atrophy with epithelial dysplasia waiting for Sb-Tx. METHODS Three children (two boys and one girl), 7 years, 46 and 11 months old, respectively, affected by intractable diarrhoea caused by IED, were treated by PN since their first days of life. Diarrhea began in neonatal age and there was an histological evidence of total atrophy of intestinal mucosa, with microvillous inclusions in two cases. A HPN program was started at the age of 13, 12 and 11 months respectively. The three children are still on HPN; they take a minimal oral feeding and receive PN for 12 to 18 hours nightly through a central vein catheter (CVC). HPN provided, up to now, more than 80% of total nutritional needs: nonnitrogen caloric
Nutrition in Children Affected
V. Lucidi,
S. Bella, P. Rosati, and M. Castro
intake is 75 to 90 Cal/kg/d, nitrogen intake is 380 to 510 mg N/kg/d, water intake is 120 to 210 mL/kg/d. All three children have a good somatic growth. Total duration of PN till now is 4650 days for the 3 patients, and HPN duration is 3570 days (range 80 to 2280). During HPN we used 10 catheters and the mean duration of each catheter
was 1 year. All catheters
were flushed
daily with heparin
(200 units).
RESULTS
We observed 14 septic complications most of whom were during hospital PN (l/y), while during HPN the complications incidence was of 0.3/y. In half of the cases the septic complications were due to intestinal bacterial translocation and most of them were successfully treated by antibiotic therapy; just three cases needed catheter removal. Mechanical complications were more frequent and serious; we observed eight occlusions that required catheter replacement in five cases because of urokinase treatment failure in restoring catheter patency. Of two deplacements, one was complicated with a late pericardial effusion of parenteral solution. This was caused by a minimal lesion of ventricular wall due to the catheter dislocation, although the catheter had been placed 2 months before. This complication was treated successfully with a pericardiocentesis. Moreover we observed two caval thromboses in two different patients. One of these was particularly severe with a complete superior caval syndrome successfully treated with caval dilatation through cardiac catheterism. The incidence of caval thrombosis has been, to date, 1 on 5 years of PN, which is greater than that observed in a previous report.’ All three patients showed mild hepatic abnormalities and moderate osteoporosis. One patient is affected by biliary and renal lithiasis. Finally, we report one case of acute medullary macrophagic activation receded after reduction of lipid intake.
From the Department of Pediatric Gastroenterology, Ospedale Pediatric0 Bambino Gesti, Rome, Italy. Address reprint requests to M. Gambarara, Divisione di Gastroenterologia, Ospedale Bambino Gesti, Piazza S Onofrio, 4, 00165 Roma, Italy.
0041-1315/97/$17.00 PII SO041 -1345(97)00104-8
0 1997 by Elsevier Science Inc. 655 Avenue of the Americas, New York, NY 10010
1870
Transplantation
Proceedings,
29, 1870-l 871 (1997)
COMPLICATIONS
1871
DURING HOME PN
DISCUSSION
The greater incidence of septic complications during hospital PN compared with home PN period (l/y vs 0.3/year) may be due to the worse clinical condition in the early stages of the disease, to the younger age, and to the intensive care that these patients often need before reaching a better stabilization. During HPN, a greater incidence of mechanical and thrombotic complications was observed compared with that experienced in patients affected by other diseases.’ This is probably due to the specific parenteral intakes that have to cover persistent intestinal losses that often require higher doses of macro and micronutrients and higher flow rate during infusion. Although we did not observe any major metabolic complication, the PN dependence of these patients represents
by itself a serious risk factor. In conclusion, we confirm the importance of prolonged PN programs in patients affected by intractable diarrhea due to epithelial dysplasia, but also the necessity of precocious intestinal transplantation, because of the greater incidence of complications compared with other diseases that require prolonged PN. REFERENCES 1. Goulet 0: Arch PCdiatr 3:324, 1996 2. Cuenod B, Brousse N, Goulet 0, et al: Gastroenterol99:1037,
1990 3. Girault D, Goulet 0, Ledeist F, et al: J Pediatr 125:36, 1994 4. Goulet 0, Kedinger M, Brousse N, et al: J Pediatr 127:212, 1995 5. Pharaon I, Despres C, Aigrain Y, et al: Transplant Proc 261442, 1994
Complications by Congenital M. Gambarara,
During Home Parenteral Microvillous Atrophy
F. Ferretti, 6. Papadatou,
A. Diamanti,
A
MONG the heterogeneous group of intractable diarrheas in infancy is a now better-identified new clinical entity, intestinal epithelial dysplasia (IED).’ These forms, together with autoimmune enteropathies, enteropathies described in immunological deficits,2 and those associated to some rare inborn errors of metabolism and/or in some genetical syndromes represent the most severe microvillous atrophies.“.4 Intestinal epithelial dysplasias reveal themselves with intractable diarrhea starting in the neonatal period: the diarrhea is severe, watery, with electrolitic losses and without extradigestive manifestations. It is resistent to all the most common dietetic and pharmacological treatments and also to total bowel rest. An histological picture of severe microvillous atrophy with normal or hyperplastic cryptae is always present but is only sometimes associated with microvillous inclusions.4 These conditions that represent the more typical example of irreversible intestinal failure may be considered today to be one of the more frequent indications to small bowel transplant (Sb-Tx) in childhood. Parenteral nutrition (PN) has completely modified prognosis of this disease assuring to the patients not only survival but also normal growth while waiting for Sb-Tx. In these cases PN is started soon after birth and has a prolonged duration with home parenteral nutrition (HPN) programs. It is a total PN that assures the whole nutritional need and that has to cover the persistent severe fecal losses. In these patients, PN dependence and its complications in particular influence Sb-Tx timing. The aim of this study is to evaluate the complications we observed during HPN in three cases of congenital microvillous atrophy with epithelial dysplasia waiting for Sb-Tx. METHODS Three children (two boys and one girl), 7 years, 46 and 11 months old, respectively, affected by intractable diarrhoea caused by IED, were treated by PN since their first days of life. Diarrhea began in neonatal age and there was an histological evidence of total atrophy of intestinal mucosa, with microvillous inclusions in two cases. A HPN program was started at the age of 13, 12 and 11 months respectively. The three children are still on HPN; they take a minimal oral feeding and receive PN for 12 to 18 hours nightly through a central vein catheter (CVC). HPN provided, up to now, more than 80% of total nutritional needs: nonnitrogen caloric
Nutrition in Children Affected
V. Lucidi,
S. Bella, P. Rosati, and M. Castro
intake is 75 to 90 Cal/kg/d, nitrogen intake is 380 to 510 mg N/kg/d, water intake is 120 to 210 mL/kg/d. All three children have a good somatic growth. Total duration of PN till now is 4650 days for the 3 patients, and HPN duration is 3570 days (range 80 to 2280). During HPN we used 10 catheters and the mean duration of each catheter
was 1 year. All catheters
were flushed
daily with heparin
(200 units).
RESULTS
We observed 14 septic complications most of whom were during hospital PN (l/y), while during HPN the complications incidence was of 0.3/y. In half of the cases the septic complications were due to intestinal bacterial translocation and most of them were successfully treated by antibiotic therapy; just three cases needed catheter removal. Mechanical complications were more frequent and serious; we observed eight occlusions that required catheter replacement in five cases because of urokinase treatment failure in restoring catheter patency. Of two deplacements, one was complicated with a late pericardial effusion of parenteral solution. This was caused by a minimal lesion of ventricular wall due to the catheter dislocation, although the catheter had been placed 2 months before. This complication was treated successfully with a pericardiocentesis. Moreover we observed two caval thromboses in two different patients. One of these was particularly severe with a complete superior caval syndrome successfully treated with caval dilatation through cardiac catheterism. The incidence of caval thrombosis has been, to date, 1 on 5 years of PN, which is greater than that observed in a previous report.’ All three patients showed mild hepatic abnormalities and moderate osteoporosis. One patient is affected by biliary and renal lithiasis. Finally, we report one case of acute medullary macrophagic activation receded after reduction of lipid intake.
From the Department of Pediatric Gastroenterology, Ospedale Pediatric0 Bambino Gesti, Rome, Italy. Address reprint requests to M. Gambarara, Divisione di Gastroenterologia, Ospedale Bambino Gesti, Piazza S Onofrio, 4, 00165 Roma, Italy.
0041-1315/97/$17.00 PII SO041 -1345(97)00104-8
0 1997 by Elsevier Science Inc. 655 Avenue of the Americas, New York, NY 10010
1870
Transplantation
Proceedings,
29, 1870-l 871 (1997)
COMPLICATIONS
1871
DURING HOME PN
DISCUSSION
The greater incidence of septic complications during hospital PN compared with home PN period (l/y vs 0.3/year) may be due to the worse clinical condition in the early stages of the disease, to the younger age, and to the intensive care that these patients often need before reaching a better stabilization. During HPN, a greater incidence of mechanical and thrombotic complications was observed compared with that experienced in patients affected by other diseases.’ This is probably due to the specific parenteral intakes that have to cover persistent intestinal losses that often require higher doses of macro and micronutrients and higher flow rate during infusion. Although we did not observe any major metabolic complication, the PN dependence of these patients represents
by itself a serious risk factor. In conclusion, we confirm the importance of prolonged PN programs in patients affected by intractable diarrhea due to epithelial dysplasia, but also the necessity of precocious intestinal transplantation, because of the greater incidence of complications compared with other diseases that require prolonged PN. REFERENCES 1. Goulet 0: Arch PCdiatr 3:324, 1996 2. Cuenod B, Brousse N, Goulet 0, et al: Gastroenterol99:1037,
1990 3. Girault D, Goulet 0, Ledeist F, et al: J Pediatr 125:36, 1994 4. Goulet 0, Kedinger M, Brousse N, et al: J Pediatr 127:212, 1995 5. Pharaon I, Despres C, Aigrain Y, et al: Transplant Proc 261442, 1994