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Nutritional therapy and effect assessment of infants with primary intestinal lymphangiectasia: Case reports
Suyun Li1, Xiaoqian Liu, Yuan He
1Department of Nutrition, Beijing Shijitan Hospital, Capital Medical University Department of Lymphatic Surgery, Beijing Shijitan Hospital, Capital Medical University Department of Nuclear Medicine, Beijing Shijitan Hospital, Capital Medical University, Beijing, China.
Insights
Nutritional therapy effectively treated primary intestinal lymphangiectasia (PIL) in infants, improving chylous reflux disorder symptoms and promoting growth. All patients showed significant recovery in nutritional markers and overall health.
Area of Science:
- Pediatric Gastroenterology
- Rare Diseases
- Nutritional Science
Background:
- Primary intestinal lymphangiectasia (PIL) is a rare enteropathy causing intestinal lymphatic channel expansion and rupture.
- It is exceptionally uncommon in infants, presenting diagnostic and therapeutic challenges.
- Chylous reflux disorder, including chylothorax and chylic abdomen, is a severe manifestation of PIL.
Purpose of the Study:
- To investigate the efficacy of nutritional therapy in infants diagnosed with primary intestinal lymphangiectasia (PIL).
- To assess the impact of nutritional interventions on chylous reflux disorder secondary to PIL in infants.
- To evaluate the recovery and prognosis of infants with PIL following tailored nutritional management.
Main Methods:
- Retrospective analysis of 9 infants diagnosed with chylous reflux syndrome caused by PIL.
- Individualized nutritional therapy administered to all participating patients.
- Assessment of clinical outcomes, nutritional markers, and anthropometric data post-intervention.
Main Results:
- All 9 infants survived and demonstrated significant improvements in serum total protein, albumin, and hemoglobin levels.
- Comprehensive nutritional therapy successfully controlled diarrhea in all patients, leading to normalized stool consistency and frequency.
- Post-treatment, all patients exhibited increased height and weight within normal WHO ranges, with a mean serum albumin level of 41.3 g/L.
Conclusions:
- Nutritional therapy is an effective treatment for primary intestinal lymphangiectasia in infants.
- This therapeutic approach can significantly improve clinical symptoms and promote recovery in children with PIL.
- Early and comprehensive nutritional management is crucial for managing chylous reflux disorder in infants with PIL.
Rationale:
Intestinal lymphangiectasia (IL) is a rare enteropathy involving the expansion and rupture of intestinal lymphatic channels. Although several reports have studied cases of primary IL (PIL), this condition is very rare, and is even less commonly encountered in infants. This study aimed to investigate the nutritional therapy and effect assessment of chylous reflux disorder caused by PIL in infants.
Patient Concerns:
Infantile patients were enrolled in the Affiliated Beijing Shijitan Hospital of the Capital Medical University between January 2012 and March 2014. The minimum age of onset was 4 months and the maximum age of onset was 16 months, with an average age of 4.9 months.
Diagnoses:
All children were inpatient who had been diagnosed with chylous reflux syndrome (chylothorax and/or chylic abdomen) caused by PIL.
Interventions:
Retrospective analysis and individualized nutrition therapy of these cases were carried out. Finally, nutritional therapy and prognosis of PIL were assessed and summarized.
Outcomes:
All the children survived, showed improvement in the serum total protein, albumin, and HGB levels after nutritional therapy. After comprehensive nutritional therapy, we were able to achieve diarrhea control for all the 9 patients, and after treatment, the children passed soft, yellow stools 1 to 2 times/d. After treatment, the height and weight of all patients increased to within the normal ranges of the World Health Organization standard chart. The mean serum albumin level reached 41.3 g/L. All nutrition-related indicators were found to have significant improvement compared with the baseline levels.
Lessons:
The results revealed that nutritional therapy for the 9 children with PIL was effective, and it may be able to improve the clinical syndromes and symptoms of children with PIL and promote recovery.
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