Recurrent and Opportunistic Infections in Children With Primary Intestinal Lymphangiectasia

Dierselhuis, Miranda P.; Boelens, Jaap Jan; Versteegh, F. G. A.; Weemaes, C.M.R.; Wulffraat, Nico

doi:10.1097/01.mpg.0000233192.77521.2f

Cited by 31 publications

(13 citation statements)

References 11 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Patients suffering from PIL have a significantly higher likelihood of infections due to moderate or severe hypogammaglobulinemia and lymphopenia (e.g. Streptococcus G, meningitis, Cryptococcus, cytomegalovirus) [16, 17]. We saw no infections at 3 years follow-up in our 2 cases even with low levels of IgG.…”

Section: Discussionmentioning

confidence: 63%

Primary Intestinal Lymphangiectasia: Is It Always Bad Two Cases with Different Outcome

Xinias

Mavroudi

Sapountzi

et al. 2013

Case Rep Gastroenterol

View full text Add to dashboard Cite

Primary intestinal lymphangiectasia (PIL) or Waldmann's disease is a rare protein-losing gastroenteropathy of unknown etiology. Less than 200 cases have been reported globally. Patients may be asymptomatic or present edema, lymphedema, diarrhea, ascites and other manifestations. We report two pediatric cases with PIL with extremely different outcome in a 3-year follow-up period. The first patient presented with persistent diarrhea, hypoalbuminemia and failure to thrive, while the second patient presented with an abrupt eyelid edema. Hypoproteinemia was the common laboratory finding for the two patients and upper gastrointestinal endoscopy established the diagnosis. The first patient relapsed five times during the follow-up period after the diagnosis had been made and required intravenous albumin administration and micronutrient supplementation. The second patient revealed normal gastrointestinal endoscopy 4 months after the diagnosis had been established; he followed an unrestricted diet and remained asymptomatic throughout the follow-up period. PIL can be either severe, affecting the entire small bowel, leading to lifetime disease, or sometimes affects part of the small bowel, leading to transient disorder.

show abstract

Section: Discussionmentioning

confidence: 63%

Primary Intestinal Lymphangiectasia: Is It Always Bad Two Cases with Different Outcome

Xinias

Mavroudi

Sapountzi

et al. 2013

Case Rep Gastroenterol

View full text Add to dashboard Cite

show abstract

“…Congenital lymphedema is also associated with a selective deficit of naive CD4+ T lymphocytes. PIL with very low CD4+ counts and immunoglobulin G levels is related to recurrent and opportunistic infections and associated with increased morbidity and mortality [4] . The diagnosis of PIL is based on histological analysis of surgical specimens or endoscopic biopsies that reveal lacteal juice and dilated mucosal and submucosal lymphatic vessels.…”

Section: Case Reportmentioning

confidence: 99%

Primary intestinal lymphangiectasia diagnosed by capsule endoscopy and double balloon enteroscopy

Oh¹

2011

WJGE

View full text Add to dashboard Cite

Author contributions: Oh TG designed the case report as a main author; Chung JW and Kim HM contributed to provide background knowledge; Han SJ provided many informations about the patient; Lee JS provided genetic information and accessment; Park JY participated in endoscopic evaluation and accessment; Song SY supervised and checked up the manuscript finally as a corresponding author; all authors reviewed and approved the final manuscript. AbstractPrimary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated intestinal lymphatics and the development of protein-losing enteropathy. Patients with PIL develop hypoalbuminemia, hypocalcemia, lymphopenia and hypogammaglobulinemia, and present with bilateral lower limb edema, fatigue, abdominal pain and diarrhea. Endoscopy reveals diffusely elongated, circumferential and polypoid mucosae covered with whitish enlarged villi, all of which indicate intestinal lymphangiectasia. Diagnosis is confirmed by characteristic tissue pathology, which includes dilated intestinal lymphatics with diffusely swollen mucosa and enlarged villi. The prevalence of PIL has increased since the introduction of capsule endoscopy. The etiology and prevalence of PIL remain unknown. Some studies have reported that several genes and regulatory molecules for lymphangiogenesis are related to PIL. We report the case of a patient with PIL involving the entire small bowel that was confirmed by capsule endoscopy and double-balloon enteroscopy-guided tissue pathology who carried a deletion on chromosome 4q25. The relationship between this deletion on chromosome 4 and PIL remains to be investigated.

show abstract

“…1 However, opportunistic infection is uncommon in intestinal lymphangiectasia, and to the best of our knowledge, infection with PCP has not been reported. 1 Although both intestinal lymphangiectasia and SCID are associated with lymphopenia, 1,2 lymphocyte function following mitogen stimulation is preserved in the former 3 and significantly impaired in the latter. 2 Thus, opportunistic infection is more commonly reported in SCID than intestinal lymphangiectasia.…”

Section: Discussionmentioning

confidence: 87%

Beware the lymphopenia: A case of severe combined immunodeficiency

Mehr

Kakakios²,

Shaw

et al. 2010

J Paediatrics Child Health

View full text Add to dashboard Cite

We present a case of a 2-month-old boy with partially treated meningitis and suspected Pneumocystis carinii pneumonia. A full blood count revealed profound lymphopenia. The child was diagnosed with adenosine deaminase deficiency, a rare cause of severe combined immunodeficiency (SCID). SCID is an immunological emergency and must be considered in any lymphopaenic infant with opportunistic infection. We discuss adenosine deaminase-deficient SCID, which can involve multiple systems and in which other treatment options apart from bone marrow transplant are available.

show abstract

Recurrent and Opportunistic Infections in Children With Primary Intestinal Lymphangiectasia

Cited by 31 publications

References 11 publications

Primary Intestinal Lymphangiectasia: Is It Always Bad Two Cases with Different Outcome

Primary Intestinal Lymphangiectasia: Is It Always Bad Two Cases with Different Outcome

Primary intestinal lymphangiectasia diagnosed by capsule endoscopy and double balloon enteroscopy

Beware the lymphopenia: A case of severe combined immunodeficiency

Contact Info

Product

Resources

About