Primary intestinal lymphangiectasia: twenty years of experience at a Mexican tertiary care hospital

Valdovinos-Oregón, D.; Ramírez-Mayans, J.A.; Cervantes-Bustamante, Roberto; Monjaráz, Erick Manuel Toro; Cazares-Méndez, M.; Cadena-León, José Francisco; Zárate-Mondragón, Flora; Montijo-Barrios, Ericka

doi:10.1016/j.rgmxen.2014.05.002

Cited by 4 publications

(5 citation statements)

References 27 publications

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“…Hypoalbuminemia, hypocalcaemia, lymphocytopenia, and reduced immunoglobulin levels (IgM, IgA and IgG) are the main laboratory features described previously in the literature. 2,4 In our study all 4 patients presented with anaemia, diarrhoea, and ascites/pedal oedema (Table 1). Investigations in all of our patients revealed anaemia, lymphopenia, and hypoalbuminemia.…”

Section: Discussionmentioning

confidence: 54%

“…Diarrhoea, edema, and ascites are common symptoms of PIL patients, other clinical features include obstructive ileus, iron deficiency anaemia, weight loss, inability to gain weight hypoproteinaemia, and lymphocytopenia. 4,11 Hypogammaglobulinemia may also be present due to lymph leakage from the ruptured lymph vessels. 2 All except one in our series presented before the age of three years.…”

Section: Discussionmentioning

confidence: 99%

“…1 Other therapies with varying degrees of effectiveness have been mentioned in the literature, such as antiplasmin therapy, octreotide, corticosteroids, small bowel resection, albumin infusions, everolimus, and intestinal transplant. 1,4,6,16 All these mentioned treatment modalities can be used after or in combination with a low-fat diet along with MCT supplementation. Their efficacy is variable and insufficiently evaluated.…”

Section: Discussionmentioning

confidence: 99%

“…PIL is usually diagnosed before 3 years of age. 4 Hypoproteinaemia, hypoalbuminemia, lymphocytopenia, hypogammaglobulinemia, and reduced levels of transferrin and fibrinogen are the typical laboratory features. 5 Diagnosis is by radiologic and endoscopic findings, confirmed by Four patients were diagnosed to have IL, between July 2015 to August 2018.…”

Section: Introductionmentioning

confidence: 99%

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Lymphopenia: a clue to diagnose primary intestinal lymphangiectasia

Nayak

Gopalkrishnan²,

Ramakrishna³

2022

Int J Contemp Pediatr

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Primary intestinal lymphangiectasia (PIL) is a rare protein-losing gastroenteropathy characterized by dilatation of the intestinal lymphatics and loss of lymph fluid into the gastrointestinal tract, leading to the development of hypoproteinaemia, oedema, lymphocytopenia, hypogammaglobinaemia, and immunologic abnormalities. We report a series of 4 children from Bangalore, India presenting with anasarca, diarrhoea, and hypoproteinaemia. All four patients were confirmed to have of PIL on histopathology. We retrospectively reviewed the case records of children diagnosed with primary intestinal lymphangiectasia presenting to department of pediatric gastroenterology within the time frame of July 2015 to September 2018. Four patients were diagnosed with primary intestinal lymphangiectasia. All of them presented with features of protein losing enteropathy (generalized oedema, ascites and low albumin), chronic diarrhoea, lymphopenia, hypercalcemia, and hypogammaglobulinemia. Endoscopically mucosa was normal in two patients and showed Snowflake appearance in other 2 patients. Intestinal biopsies were characteristic of lymphangiectasia in all four patients.

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Section: Discussionmentioning

confidence: 54%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Lymphopenia: a clue to diagnose primary intestinal lymphangiectasia

Nayak

Gopalkrishnan²,

Ramakrishna³

2022

Int J Contemp Pediatr

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“…e pathological dilation of lymphatic vessels in the intestinal wall, especially in the submucosa, causes chronic loss of lymph containing immunoglobulins and lymphocytes (predominantly naïve CD4 and CD8 T cells) and severe hypoalbuminemia. is state of secondary immunodeficiency is of difficult diagnosis and poses a high risk for opportunistic fungal infections such as cryptococcosis, as in our patient [13,14].…”

Section: Discussionmentioning

confidence: 77%

Gastrointestinal Cryptococcosis Associated with Intestinal Lymphangiectasia

Naranjo-Saltos¹,

Hallo

Hallo³

et al. 2020

Case Reports in Medicine

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Intestinal lymphangiectasia is a pathological dilation of enteric lymphatic vessels resulting in lymph leakage to the intestinal lumen. This chronic lymph leakage leads to a state of immunosuppression secondary to the loss of humoral and cellular components of the immune system and represents a potential risk factor for opportunistic infections. We report a case of protein-losing enteropathy in a seemingly immunocompetent patient. An intestinal histopathological study revealed the unusual association of lymphangiectasia and intestinal cryptococcosis. Although cryptococcal infection is common in immunocompromised patients, intestinal involvement is rarely reported. We found no reports on the association of intestinal cryptococcosis in patients with lymphangiectasia. This case report is the first to describe intestinal cryptococcosis associated with intestinal lymphangiectasia.

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Progress in Diagnosis and Treatment of Intestinal Lymphangiectasia

曾¹

2022

ACM

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Primary intestinal lymphangiectasia: twenty years of experience at a Mexican tertiary care hospital

Cited by 4 publications

References 27 publications

Lymphopenia: a clue to diagnose primary intestinal lymphangiectasia

Lymphopenia: a clue to diagnose primary intestinal lymphangiectasia

Gastrointestinal Cryptococcosis Associated with Intestinal Lymphangiectasia

Progress in Diagnosis and Treatment of Intestinal Lymphangiectasia

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