DIETARY TREATMENT OF PROTEIN LOSS IN INTESTINAL LYMPHANGIECTASIA

PEDIATRICS ◽  
1964 ◽  
Vol 34 (5) ◽  
pp. 629-635
Author(s):  
Peter R. Holt
Keyword(s):  
Thoracic Duct ◽  
Marked Reduction ◽  
Medium Chain Triglyceride ◽  
Dietary Treatment ◽  
Intestinal Lymphangiectasia ◽  
Protein Loss ◽  
Lymphatic Flow ◽  
Formula Diet ◽  
Intestinal Protein ◽  
Long Chain

A 2-year-old child with exudative enteropathy due to intestinal lymphangiectasia and an associated congenital lymphangiomatous malformation of the arm is presented. Treatment with medium chain triglyceride formula diet resulted in marked reduction in intestinal protein losses and disappearance of all symptoms. It is suggested that the enhanced lymphatic flow resulting from the thoracic duct transport of absorbed long chain fat results in increased leakage of lymph into the gut in intestinal lymphangiectasia.

Intestinal Lymphangiectasia: A Reappraisal

PEDIATRICS ◽  
1975 ◽  
Vol 55 (6) ◽  
pp. 842-851 ◽  
Author(s):  
Peter A. Vardy ◽  
Emanuel Lebenthal ◽  
Harry Shwachman
Keyword(s):  
Lamina Propria ◽  
Clinical Laboratory ◽  
Specific Treatment ◽  
Medium Chain Triglyceride ◽  
Intestinal Lymphangiectasia ◽  
Protein Loss ◽  
Intravenous Hyperalimentation ◽  
Low Fat Diet ◽  
History Of ◽  
Severity Of The Disease

Intestinal lymphangiectasia (IL) may vary widely in its manifestations and severity. Fifteen children seen between 1960 and 1974 with histologically proven IL are analyzed by clinical, laboratory, radiologic, and histologic criteria. Remissions occurred in most patients and none died. Exacerbations occurred in five children. Diarrhea was present in 14 patients and in 13 appeared before the age of 3 years. Vomiting occurred in nine patients and growth retardation in seven. Four children had associated peripheral lymphedema and two of these had a family history of lymphedema, both had affected fathers and one had affected siblings and paternal cousins. Seven had hypoproteinemic edema, and, of these, four suffered from hypocalcemic seizures. Chylous effusions were present in five. Hypoproteinemia was present in 12 although five had no hypoalbuminemic edema. Six had lymphopenia which was related to the severity of the disease and was the last abnormality to disappear after clinical remission. Lymphopenia may first appear years after the protein loss begins. Upper gastrointestinal tract series were performed in 13 children and had diagnostic supportive value in seven. Six children had two or more small-intestinal biopsies done. They all showed great variation from one examination to the other, ranging from a normal appearance to severe changes. Lymphatic block may occur at different sites-in the lamina propria only, generalized (lamina propria, submucosa, serosa, and mesentery), or conversely in the mesentery alone with minimal changes in the lamina propria. In three patients intravenous hyperalimentation was necessary. Specific treatment with a high-protein, low-fat diet with added medium-chain triglyceride (MCT) is valuable. Surgical resection was of benefit in one patient, and anastomosis of mesenteric to para-aortic lymph nodes in another.

scholarly journals Congenital Chylothorax in Preterm Infants – A New Approach in Dietary Treatment with Skimmed Breast Milk

2020 ◽  
Author(s):  
Michaela Höck ◽  
Alexander Höller ◽  
Marlene Hammerl ◽  
Karina Wechselberger ◽  
Jakob Krösslhuber ◽  
...  
Keyword(s):  
Breast Milk ◽  
Dietary Intervention ◽  
Medium Chain Triglyceride ◽  
Dietary Treatment ◽  
Favourable Outcome ◽  
University Hospital ◽  
Early Introduction ◽  
Congenital Chylothorax ◽  
Long Chain ◽  
Milk Feeding

Abstract BackgroundCongenital chylothorax (CC) is a rare but potentially life-threatening condition in newborns. It is defined as an accumulation of chyle in the pleural cavity. The few publications regarding medical management and therapeutic dietary intervention motivated us to share our experience.MethodsNeonates diagnosed with congenital chylothorax and treated at Innsbruck Medical University Hospital between 2013 and 2019 (n = 5, gestational age: 36 3/7, 32 5/7, 36 4/7, 35 0/7, 35 4/7) were eligible for this report. The cornerstones of treatment for chylothorax conventionally consist of chest tube drainage (CTD), respiratory support, dietary restriction of long-chain triglycerides (LCT) or total parenteral nutrition (TPN). In further course the introduction of a medium-chain triglyceride (MCT)-based formula followed by an overlapping switch to a formula with low LCT and high MCT, containing the essential long-chain fatty acids (LCFA), is attempted. As soon as possible, the change is made to breast milk feeding or breastfeeding. In two patients we used fat-modified (skimmed) breast milk to avoid discontinuation of breast milk feeding.ResultsThe early introduction of LCFA in the form of breast milk after resolution of chylothorax was associated with favourable outcome (no recurrence of pleural effusion and adequate weight gain).ConclusionThe first-line therapy of chylothorax is a combination of respiratory stabilization and dietary modification. The purpose of this report is to point out the feasibility of a fast change from LCT fat-free nutrition to full-fat nutrition once the chylothorax has dissolved, especially the early introduction of breast milk feeding / breastfeeding in infants with chylothorax.

scholarly journals Dietary and Endogenous Long-Chain Fatty Acids in the Intestine of Sheep, with an Appendix on Their Estimation in Feeds, Bile, and Faeces

1969 ◽  
Vol 22 (4) ◽  
pp. 1015 ◽  
Author(s):  
TJ Heath ◽  
L N Hill
Keyword(s):  
Fatty Acids ◽  
Bile Duct ◽  
Palmitic Acid ◽  
Thoracic Duct ◽  
Quantitative Information ◽  
Significant Alteration ◽  
Long Chain Fatty Acids ◽  
Gain Access ◽  
Long Chain ◽  
Chain Fatty Acids

Normal sheep and sheep with fistulae of the bile duct or the thoracic duct were used to provide quantitative information on the movement of fatty acids into and out of the intestine. The operation used to gain access to the thoracic duct did not cause any significant alteration in the absorption of either [14C]tripalmitin injected into the rumen or [14C]palmitic acid injected into the duodenum. Normal sheep absorbed the major fatty acids oleic (92'1�1'3%), palmitic (87�3�5�0%), and stearic acids (93' 3� 1� 4%) with almost equal efficiency, and the absorption of labelled tri-palmitin injected into the rumen did not alter as the intake of fatty acids increased from 12g/day (90�1�2�3%) to 44g/day (90�1�1�3%).

Gastric Lipolysis and Fat Absorption in Preterm Infants: Effect of Medium-Chain Triglyceride or Long-Chain Triglyceride-Containing Formulas

PEDIATRICS ◽  
1989 ◽  
Vol 83 (1) ◽  
pp. 86-92 ◽  
Author(s):  
Margit Hamosh ◽  
Joel Bitman ◽  
Teresa H. Liao ◽  
N. R. Mehta ◽  
R. J. Buczek ◽  
...  
Keyword(s):  
Fatty Acids ◽  
Fatty Acid ◽  
Weight Gain ◽  
Preterm Infants ◽  
Lipase Activity ◽  
Medium Chain Triglyceride ◽  
Fat Absorption ◽  
Medium Chain ◽  
Long Chain ◽  
Long Chain Triglyceride

The extent of gastric lipolysis, fat absorption, and infant weight gain was studied in 12 preterm infants (gestational age 28.75 ± 0.50 weeks, postnatal age 6.08 ± 0.81 weeks) fed medium-chain triglyceride or long-chain triglyceride formula for 1 week in a crossover design. The former formula contained 42% of 8:0 and 10:0 and 19% of 12:0, 14:0, and 16:0; the latter formula contained only 7% of 8:0 and 10:0 and 46% of 12:0, 14:0, and 16:0. Gastric aspirates were obtained on the second and third day of formula feeding for quantitation of lipase activity and of the extent of gastric lipolysis. Fat balance studies were conducted during the last three days of each feeding regimen. The study showed that (1) there was marked hydrolysis of formula fat in the stomach during feeding of either medium-chain triglyceride formula or long-chain triglyceride formula (20% and 16%, respectively); (2) lipase activity in the gastric aspirates was less during feeding of medium-chain triglyceride formula than before the meal, which suggested stimulation of lipase secretion by long-chain fatty acid released from long-chain triglyceride formula fat or more rapid binding of lipase to ingested lipid in the medium-chain triglyceride formula; (3) fatty acid distribution in glycerides and free fatty acids showed preferential release of medium-chain (8:0, 10:0) and long-chain unsaturated (18:1, 18:2) fatty acids in the stomach. The low content of 8:0 and 10:0 in gastric triglyceride and free fatty acids suggested that medium-chain fatty acids were absorbed directly in the stomach. (4) fat balance studies showed almost identical absorption rates (84.6% ± 3.1% and 82.8% ± 4.0%) and weight gain (23.0 ± 1.5 g/d and 20.8 ± 1.8 g/d) during feeding of either medium-chain triglyceride or long-chain triglyceride formula. In this study, in which each infant was fed either formula alternately, it was shown that although the extent of fat digestion varied among infants, medium-chain and long-chain triglyceride were absorbed to the same extent by most infants.

scholarly journals Diaphragmatic fenestration an effective treatment for refractory postoperative chylothorax: a case report

2020 ◽  
Vol 8 (2) ◽  
pp. 767
Author(s):  
Oluwaseun R. Akanbi ◽  
Swaminathan Vaidyanathan ◽  
Prakash Agarwal ◽  
Janeel Musthafa ◽  
Neville A. G. Solomon
Keyword(s):  
Thoracic Duct ◽  
Medium Chain Triglyceride ◽  
Cystic Lymphangioma ◽  
Satisfactory Outcome ◽  
Thoracic Duct Ligation ◽  
Risk Of Mortality ◽  
Duct Ligation ◽  
Surgical Options ◽  
Polytetrafluoroethylene Patch ◽  
Substantial Morbidity

Postoperative chylothorax remains a clinical challenge to the surgeon with substantial morbidity and risk of mortality. Though an uncommon complication, it is known to complicate cardiac and non-cardiac thoracic surgeries. Conservative measures are first employed in managing this. Surgical options are adopted when the effusion is protracted, most recent of which includes diaphragmatic fenestration. A 9-year-old girl is presented who developed recurrent right chylothorax following thoracoscopic excision of a cystic lymphangioma. Following failed conservative therapy, she had thoracic duct ligation and right diaphragmatic fenestration (using fenestrated polytetrafluoroethylene patch) with satisfactory outcome. Aetio-pathologic mechanisms implicated in postoperative chylothorax have been classified into traumatic (iatrogenic injury to the thoracic duct or its branches) and non-traumatic. With initial conservative measures (repeated pleural aspirations and intercostal drainage, medium chain triglyceride/ low fat feeds or alternatively, fasting and total parenteral nutrition) spontaneous closure remains unpredictable. Diaphragmatic fenestration when employed resulted in faster resolution of effusion and earlier commencement of enteral feeding with no significant complication. Diaphragmatic fenestration is effective and safe for treating refractory post-operative chylothorax.

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