The present case is the fourth report describing a response to operative resection. [ncbi.nlm.nih.gov]

Clinical presentation: The patient may be asymptomatic or present as vague abdominal pain, chronic diarrhea, steatorrhea, edema, chylous pleural effusion, ascites, hypoproteinemia, lymphocytopenia or protein-losing enteropathy. [histopathology-india.net]

• Lymphedema

  In one family, the association of cortical dysplasia and congenital lymphedema have been related to mutations in the RELN gene. [ncbi.nlm.nih.gov]

  Abstract Congenital lymphedema occurs with intestinal lymangiectasia in several syndromes, including those described by Turner, Noonan, and Hennekam. We report a 16 year-old Filipino female with an unusual presentation of congenital lymphedema. [nature.com]

• Disability

  Please enable JS and disable any ad blocker [patientcareonline.com]

• Cough

  There was no history of cough, wheeze, rash or vomiting. Clinical examination revealed mild pallor, pedal edema, facial puffiness with weight and height less than 3SD as per WHO growth chart. [omicsonline.org]

  About one month prior to this admission, the patient complained of a fever (the highest temperature being 39°C) accompanied by sore throat, cough, and runny nose, but no fear of cold or shivering. [jmedicalcasereports.com]

• Abdominal Pain

  Abdominal pain and diarrhea subsided postoperatively. The present case is the fourth report describing a response to operative resection. [ncbi.nlm.nih.gov]

  We report a case of a 9 years old boy who underwent Mustard's repair and presented diarrhoea, abdominal pain, hypoproteinemia and ascites. [moh-it.pure.elsevier.com]

• Borborygmi

  Adverse effects of MCT Oil include sedation, narcosis, hepatic encephalopathy in patients with cirrhosis ketosis, nausea, vomiting, abdominal pain, diarrhea, or borborygmi. [cancertherapyadvisor.com]

• Dyspepsia

  Finally, gastrointestinal symptoms that might arise include abdominal pain, nausea, diarrhea, flatulence, cholelithiasis, biliary sludge, constipation, vomiting, biliary duct dilatation, anorexia, tenesmus, dyspepsia, steatorrhea, feces discoloration. [cancertherapyadvisor.com]

• Skin Rash

  There was no history of skin rashes, oral ulcers, joints pain, tremor or history related to fat and water-soluble vitamin deficiency. [jdeonline.in]

Because IL is a rare disease, the symptoms, diagnostic workup, and treatment are unfamiliar to many clinicians. Current literature documents only a few reported cases of IL in a preterm infant. [ncbi.nlm.nih.gov]

There is no specific treatment although enteric protein loss may decrease with a low-fat diet. [ncbi.nlm.nih.gov]

Dietary treatment of protein loss in intestinal lymphangiectasia. Pediatrics 34:629. 1964. [kjim.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]

Prognosis The prognosis varies. PIL may be asymptomatic or mildly symptomatic in moderate forms of the disease or in patients who follow a low-fat diet. [orpha.net]

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]

Summary Epidemiology Prevalence is unknown. Less than 500 cases have been reported worldwide. Clinical description PIL is generally diagnosed before 3 years of age but may be diagnosed in older patients. [orpha.net]

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]

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]

This classification was useful in pathophysiological clustering and in predicting the therapeutic response. © 2014 S. Karger AG, Basel. [ncbi.nlm.nih.gov]

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

"Small bowel lymphangiectasia and angiodysplasia: a positive association; novel clinical marker or shared pathophysiology?". J Clin Gastroenterol. vol. 44. 2010. pp. 610-4. (Pathophysiology of intestinal lymphangiectasia.) [cancertherapyadvisor.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]

The absence of fat in the diet prevents chyle engorgement of the intestinal lymphatic vessels thereby preventing their rupture with ensuing lymph loss. [orpha.net]

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]