Secondary intestinal lymphangiectasia
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adult, Elderly
- Status
- active
Secondary intestinal lymphangiectasia is an acquired from of intestinal lymphangiectasia manifesting as a protein-losing enteropathy due to another disorder such as Crohns disease, congestive heart failure, sarcoidosis, Turner syndrome and often in patients who have undergone a Fontan operation. It is characterized by malabsorption, diarrhea, edema due hypoproteinemia, steatorrhea and serosal effusions.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Secondary intestinal lymphangiectasia | |
| fr | Lymphangiectasie intestinale secondaire | |
| de | Lymphangiektasie, intestinale sekundäre | |
| es | Linfangiectasia intestinal secundaria | |
| it | Linfangiectasia intestinale secondaria | |
| nl | Secundaire intestinale lymfangiëctasie | |
| pt | Linfangiectasia intestinal secundária | |
| pl | Wtórna limfangiektazja jelitowa | |
| cs | Sekundární střevní lymfangiektázie | |
| uk | Вторинна кишкова лімфангіектазія | |
| zh | 继发性小肠淋巴管扩张 | |
| tr | Sekonder bağırsak lenfanjiyektazi |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | I89.0 | NTBT | attributed | validated |
| ICD-11 | BD92.0 | NTBT | index | validated |
| MONDO | 0019574 | E | validated | |
| UMLS | C4273969 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:36204Intestinal lymphangiectasia
rare gastroenterological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
40 annotations · validated 2021-11-19| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0031686 | Increased stool alpha1-antitrypsin concentration | |
| HP:0001004 | Lymphedema | |
| Frequent (79-30%) | ||
| HP:0002028 | Chronic diarrhea | |
| HP:0000969 | Edema | |
| HP:0012378 | Fatigue | |
| HP:0003146 | Hypocholesterolemia | |
| HP:0004788 | Intestinal lymphedema | |
| HP:0001888 | Lymphopenia | |
| HP:0002024 | Malabsorption | |
| HP:0011741 | Secondary hyperaldosteronism | |
| Occasional (29-5%) | ||
| HP:0011848 | Abdominal colic | |
| HP:0012050 | Anasarca | |
| HP:0002960 | Autoimmunity | |
| HP:0002608 | Celiac disease | |
| HP:0012281 | Chylous ascites | |
| HP:0001394 | Cirrhosis | |
| HP:0002563 | Constrictive pericarditis | |
| HP:0002720 | Decreased circulating IgA level | |
| HP:0032136 | Decreased circulating IgG1 level | |
| HP:0004313 | Decreased circulating antibody level | |
| HP:0031085 | Decreased circulating prealbumin concentration | |
| HP:0002850 | Decreased circulating total IgM | |
| HP:0001945 | Fever | |
| HP:0012537 | Food intolerance | |
| HP:0032064 | Gastrointestinal eosinophilia | |
| HP:0003073 | Hypoalbuminemia | |
| HP:0011900 | Hypofibrinogenemia | |
| HP:0002665 | Lymphoma | |
| HP:0002202 | Pleural effusion | |
| HP:0032387 | Reduced transferrin level | |
| HP:0100324 | Scleroderma | |
| HP:0002013 | Vomiting | |
| Very rare (<4-1%) | ||
| HP:0012191 | B-cell lymphoma | |
| HP:0002584 | Intestinal bleeding | |
| HP:0005214 | Intestinal obstruction | |
| HP:0002576 | Intussusception | |
| HP:0002719 | Recurrent infections | |
| HP:0001708 | Right ventricular failure | |
| HP:0001907 | Thromboembolism | |
| HP:0002580 | Volvulus | |
Run the differential on the 2 obligate / very frequent terms
Annotation source: 25618488[PMID]_33431540[PMID]_26837404[PMID]_30322285[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/90363
- This record as JSON: /data/d/90363.json