Primary intestinal lymphangiectasia (PIL) is a rare disease of intestinal lymphatics presenting with hypoproteinemia, bilateral lower limb edema, ascites, and protein losing enteropathy. [ncbi.nlm.nih.gov]

The case presented is not unusual in its clinical features. [saudijgastro.com]

• Intravenous Administration

  Methods evaluating enteric protein loss include quantitation of protein loss by determining fecal clearance of α 1 -antitrypsin or measurement of the magnitude of protein loss by counting fecal excretion of radioactivity following intravenous administration [kjim.org]

  In earlier studies, both tests have detected abnormal enteric loss of proteins. 6,7 Differently, radioactive methods, including intravenous administration of 51 Cr albumin or 51 Cr chloride, are cumbersome and expensive, and are rarely used because radioactive [lymphedemapeople.com]

• Localized Edema

  Lymphedema is less pitting than edema due to hypoproteinemia, and is localized to the lower limbs (foot, ankle, calf, rarely thigh) and predominantly bilateral. [lymphedemapeople.com]

• Crying

  During the last admission, the patient presented with weakness being unable to sit or stand, wasted, frequent crying associated with abdominal colic and distension, loose motions and vomiting with carpopedal spasms (tetany). [saudijgastro.com]

• Acutely Ill Patient

  After 4 months, the patient was transferred to Gifu University Hospital to establish a definitive diagnosis and undergo additional therapy. Physical examination on admission revealed an acutely ill patient with a puffy face and generalized edema. [pediatrics.aappublications.org]

• Sputum

  The AFB sputum examination by direct microscopy, the tuberculin skin test, the 24h proteinuria and the immunological tests were negative. [panafrican-med-journal.com]

• Intestinal Disease

  Depending on the cause of the disease, it can be classified into primary intestinal lymphangiectasia (PIL) or secondary intestinal lymphangiectasia, so the diagnosis of PIL must first exclude the possibility of secondary IL, e.g. other intestinal diseases [oapublishinglondon.com]

  Whipple’s disease from Tropheryma whipplei ) or inflammatory disorders (e.g. Crohn’s disease) may also present with profound changes in intestinal permeability and protein-losing enteropathy that also require exclusion. [wjgnet.com]

• Periumbilical Pain

  We report a case of primary intestinal lymphangiectasia, of the diffuse type, presenting with abdominal pain and voluminous diarrhea in a previously healthy 8-year-old boy. He had periumbilical pain for 3 months before presentation. [ncbi.nlm.nih.gov]

• Systolic Murmur

  The cardiovascular exam indicated a pericardial rub with a systolic murmur at the mitral area. The abdominal exam found a shifting dullness with a smoothed umbilicus. The skin exam showed a thin skin with no dehydration signs, skin lesions or edema. [panafrican-med-journal.com]

Duration of the octreotide treatment changed between 3 and 37 months. Stool frequency decreased in all of the patients after starting octreotide treatment. Serum albumin could be maintained at normal levels in 3 patients. [ncbi.nlm.nih.gov]

There is no consensus on the treatment of this condition. Treatment is generally symptomatic and may include nutritional therapy and replacement therapy. [pediatrics.aappublications.org]

Although the patient had a partial remission following the use of a slow release formula of octreotide, his prognosis, clinical course, and future treatment challenges are yet to be determined. [ncbi.nlm.nih.gov]

Treatment and prognosis Treatment has usually comprised of a low fat diet along with a medium-chain triglyceride oral supplements. History and etymology It was first described by TA Waldmann in 1961 1. [radiopaedia.org]

Prognosis - Waldmann disease Not supplied. Treatment - Waldmann disease The main treatment for PIL is a low-fat diet with medium-chain triglyceride (MCT) supplements. [checkorphan.org]

Prognosis The clinical course of intestinal lymphangiectasia is highly variable with about 23% of patients showing improvement and 64% remaining unchanged; the mortality rate is 13%. [emedicine.medscape.com]

Etiological work-up reveals pleural and pericardial tuberculosis. The clinical aggravation of an enteropathy, particularly in adulthood, requires a search for a secondary etiology. Tuberculosis should be sought systematically. [ncbi.nlm.nih.gov]

It can occur from a number etiologies which include. primary/congenital form : primary idiopathic intestinal lymphangiectasia / Waldmann disease 5 secondary form : resulting from a tumor, fibrosis, or another cause of lymphatic obstruction. [radiopaedia.org]

Epidemiology Because the condition is so multifactorial, the prevalence rate is not known. [patient.info]

[…] also be affected. [4] Etiology The following conditions can cause intestinal lymphangiectasia: Abdominal or retroperitoneal carcinoma Mesenteric tuberculosis Retroperitoneal fibrosis Intestinal endometriosis Sclerosing mesenteritis Lymphenteric fistula Epidemiology [emedicine.medscape.com]

Epidemiology The prevalence of clinically overt PIL is unknown. However, PIL can be asymptomatic; it primarily affects children (generally diagnosed before 3 years of age) and young adults but may be diagnosed later in adults[ 2, 3 ]. [lymphedemapeople.com]

Lower Digestive Tract, 2nd Edition, part 2 in the 3-book Digestive System volume, covers the small bowel and colon, and provides a concise and highly visual approach from normal anatomy and physiology through pathophysiology, diagnostics and treatment [books.google.com]

Since such condition is extremely rare, no precise classification and pathophysiological explanation are so far available. We observed a case of duodenal lymphangiectasia associated to a lymphangioma of the 4th finger of the left hand. [ricerca.univaq.it]

Gastrointestinal Disease: Pathophysiology, Diagnosis, Management. Vol. 2. 5th ed. Philadelphia: WB Saunders Co; 1993:1379-1393. [consultant360.com]

[…] include diarrhea, nausea, swelling of the legs, protein-losing enteropathy, immunodeficiency and loss of lymphatic fluid into the intestines. [2] [4] It is usually diagnosed before the patient is 3 years old, but it is sometimes diagnosed in adults. [3] Pathophysiology [en.wikipedia.org]

Morbidity/mortality Morbidity is related to the pathophysiology of this disease. [emedicine.medscape.com]

The absence of fat in the diet prevents chyle engorgement of the intestinal lymphatic vessels thereby preventing their rupture with its ensuing lymph loss. [ncbi.nlm.nih.gov]

Can it be prevented? As doctors do not know what causes PIL, there is no way it can be prevented. When should you seek medical help? A child should be taken to the doctor if parents notice any swelling in the legs. [gosh.nhs.uk]

It is likely that the absence of fat in the diet prevents engorgement of the intestinal lymphatics with chyle, thereby preventing their rupture that results in protein and T-cell loss. [2] Thus, a low-fat diet with supplemental MCT forms the cornerstone [jdeonline.in]

Prevention - Waldmann disease Not supplied. Diagnosis - Waldmann disease Diagnosis of PIL is often made in childhood, usually before age 3. The presence of PIL is suggested by edema of the legs. [checkorphan.org]

This prevented expression of the CD55 protein, which contributes to immune regulation by inhibiting complement activation. [healio.com]