Disease Information for Congenital Central Lymphatics Obstruction/Dysplasia

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Clinical Manifestations
Signs & Symptoms
Delayed/poor wound healing
Intracutaneous edema signs
Abdominal bloating
Abdominal Bloating in Children
Abdominal Distension
Abdominal Distension in Children
Abdominal distension/protrusion/sign
Abdominal Distention
Abdominal fluid wave on exam
Anorexia in Infant
Diarrhea
Diarrhea, chronic
Failure to Thrive
Failure to Thrive Child
Failure to thrive/infant sign
Feeding/Apetite Problems Child
Increased abdominal girth/Potbelly
Protruberant Abdomen
Shifting dullness/abdomen
Hypertrophic lymphatics/subcutaneous/signs
Edema of Lower Extremities
Laboratory Tests
Abnormal Lab Findings - Decreased
Albumin, serum (Lab)
Lymphocytes (Lab)
Abnormal Lab Findings - Increased
ASCITIC Leukocytes
ASCITIC Lymphocytes
Associated Diseases & Rule outs
Associated Disease & Complications
Abdominal lymphatics obstruction/Cysterna ch.
Anasarca/Generalized Edema
Ascites
Ascites in Children
Chylous ascites
Chylous pleural effusion
Edema
Hypoalbuminemia Hypoproteinemia
Peritoneal Effusion
Pleural Effusion
Protein losing enteropathy
Disease Mechanism & Classification
Class
CLASS/Cysterna Chyli/main lymphatic channels disorder (ex)
Process
PROCESS/Congenital/developmental (category)
PROCESS/Structural/anatomic/foreign body (category)
PROCESS/Anomalies/Deformities/Malformations (EX)
PROCESS/Dysplasia disorder/Syndromes (ex)
Definition

chylous ascites presents with diarrhea and failure to thrive in kids; acquired or congenital; paracentesis and medium chain fat diet will occasionally give spontaneous cure in kids; Lymphectasia congenital defect with milder dysplasia in onset as adults presents as protein losing enteropathy; Hypogammaglobulinemia, hypoambuminemia, and lymphopenia occur; Ascitic fluid has lymphocytes but may not be milky if not postprandial; No real treatment in kids; Surgical venous drainage connection more successful in adults; Cause of acquired disease are blocks at central lymphatics, cysterna chyle, and thoracic duct; infections, inflammatory,neoplastic malignancy, and trauma with hematoma scar retroperitoneal sepsis; may be complication of hemorrhagic necrotizing pancreatitis; If thoracic duct involved pleural chylous effusion predates the ascites; Often the fluid wave, shifting dullness, and distended abdomen are present; Retroperitoneal edema shows on imaging; One or both lower extremities are edematous; Often this is fatal if untreated, especially with kids; Complicating infection occurs; Some causes in adults are tuberculosis, schistosomiasis, retroperitoneal hematoma, carcinoma retroperitoneal, section at surgery, transection with gunshot etc Usually not discovered and idiopatic acquired---------Pediatric current DXRX Hay Sondheimer 14th 1999]--------------------------------

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External Links Related to Congenital Central Lymphatics Obstruction/Dysplasia
Google
Wikipedia
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PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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