Symptoma

Primary Intestinal Lymphangiectasia

Intestinal Lymphatic Obstruction

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

  • 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

    - Mechanical ileus of the small intestine caused by localized edema leading to intestinal wall-thickening and lumen diminution. [lymphedemapeople.com]

  • Collapse

    Despite intensive management, the patient expired due to circulatory collapse most probably secondary to severe hypoalbuminaemia and clinical sepsis. The case presented is not unusual in its clinical features. [saudijgastro.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]

  • Apyrexia

    However, 6 months ago, the patient presented MRC grade 3 dyspnea with alternated fever and apyrexia. It appeared after she chose to stop, 8 months ago, the low fat diet that was prescribed by her pediatrician. [panafrican-med-journal.com]

  • 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]

    It is especially critical to ensure that changes of intestinal lymphangiectasia are not secondary to some other cause, particularly from a “downstream” obstruction, including another intestinal disease (e.g. [wjgnet.com]

  • Periumbilical Pain

    He had periumbilical pain for 3 months before presentation. He was managed by segmental bowel resections and end-to-end anastomoses. [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]

  • Hepatosplenomegaly

    We present two patients with biopsy proven primary intestinal lymphangiectasia in whom CT demonstrated diffuse nodular thickening of small bowel without adenopathy or hepatosplenomegaly. One patient had extensive ascites. [ncbi.nlm.nih.gov]

    There was no abdominal mass, ascites, lymphadenopathy, or hepatosplenomegaly. Her weight was 63 kg and height 161 cm. The family history was not contributory. Erythrocyte and blood cell counts were normal except for lymphopenia (400–600 per μl). [gut.bmj.com]

  • Thin Skin

    The skin exam showed a thin skin with no dehydration signs, skin lesions or edema. Suspected diagnoses were: Tuberculosis, type B Lymphoma, Disseminated Lupus erythematosus. Paraclinical tests showed a moderate inflammatory syndrome. [panafrican-med-journal.com]

  • Osteopenia

    We report a case of secondary hyperparathyroidism, osteopenia, monoclonal gammopathy and digital clubbing in a 57-year-old patient with a 12-year history of discontinuous diarrhea. [ncbi.nlm.nih.gov]

  • Carpopedal Spasm

    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]

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]

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]

Because the severity of symptoms in infants can vary, the infants healthcare providers are best able to provide specific information regarding a child's prognosis. [rarediseases.info.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]

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]

The etiology and prevalence of PIL remain unknown. Keywords: Capsule endoscopy, double balloon enteroscopy, intestinal lymphangiectasia How to cite this article: Gupta V, Ranjan P, Kumar M, Sachdeva M. [jdeonline.in]

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]

The pathophysiology includes: a decline of the oncotic pressure due to hypoprotidemia, responsible of edema with calcium, iron, copper, and lipoproteins circulating lymphocytes leakage; a rupture of the intestinal epithelial barrier with ulcers, inflammation [panafrican-med-journal.com]

The underlying pathophysiology and clinical features of this disease apparently overlaps with the physiological changes that happened during pregnancy (e.g., hormonal changes and the resulting fluid retention). [journals.lww.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]

In fact, the nonfat diet avoids chyle engorgement of the intestinal lymphatic vessels and prevents their rupture and lymph loss.Other inconsistently effective treatments have been proposed for PIL patients, such as antiplasmin, octreotide or corticosteroids [panafrican-med-journal.com]