ORPHA:26790
Pseudomyxoma peritonei
Synonyms: Adenomucinosis · Gelatinous ascites · PMP
- Type
- Disease
- Group
- Disorder
- Inheritance
- Unknown
- Age of onset
- Adult
- Status
- active
Pseudomyxoma peritonei is characterized by disseminated intra-peritoneal mucinous tumors and mucinous ascites in the abdomen and pelvis.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Pseudomyxoma peritonei | Adenomucinosis · Gelatinous ascites · PMP |
| fr | Maladie gélatineuse du péritoine | Ascite gélatineuse · PMP · Pseudomyxome péritonéal |
| de | Pseudomyxoma peritonei | Aszites, gallertiger · PMP · Adenomuzinose |
| es | Pseudomixoma peritoneal | Adenomucinosis · Ascitis gelatinosa · PMP |
| it | Pseudomixoma del peritoneo | Adenomucinosi · Ascite gelatinosa · PMP |
| nl | Pseudomyxoma peritonei | Adenomucinosis · Gelatineuze ascites · PMP |
| pt | Pseudo-mixoma do peritoneu | Ascite gelatinosa |
| pl | Śluzak rzekomy otrzewnej | = en |
| cs | Pseudomyxom peritonea | Adenomucinóza · Želatinózní ascites · PMP |
| uk | Псевдоміксома очеревини | Аденомуциноз · Желатинозний асцит · PMP |
| zh | 腹膜假粘液瘤 | 明胶性腹水 |
| tr | Psödomiksoma peritonei | PMP · Adenomüsinoz · Jelatinimsi asit |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C78.6 | NTBT | index | validated |
| ICD-11 | 2D91 | NTBT | index | validated |
| MONDO | 0017048 | E | validated | |
| MeSH | D011553 | E | validated | |
| UMLS | C0033822 | E | validated | |
| GARD | 7488 | E | validated | |
| MedDRA | 10037138 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:168807Primary malignant peritoneal tumor
rare abdominal surgical diseases; rare neoplastic diseases; rare transplant-related disorders
No children.
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Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
4 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.1 / 100 000 | Worldwide | Value and class | Validated | 19838447[PMID]_17524597[PMID]_16678994[PMID]_[EXPERT] |
| Annual incidence | 1-9 / 1 000 000 | 0.2 / 100 000 | Netherlands | Value and class | Validated | 17524597[PMID] |
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | ORPHANET |
| Point prevalence | 1-9 / 100 000 | 2 / 100 000 | Europe | Value and class | Validated | European Medicines Agency 2018[OTHER] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
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HPO phenotypes
12 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0004298 | Abnormality of the abdominal wall | |
| HP:0002585 | Abnormality of the peritoneum | |
| HP:0001541 | Ascites | |
| Frequent (79-30%) | ||
| HP:0002037 | Inflammation of the large intestine | |
| Occasional (29-5%) | ||
| HP:0002027 | Abdominal pain | |
| HP:0002019 | Constipation | |
| HP:0100790 | Hernia | |
| HP:0005214 | Intestinal obstruction | |
| HP:0002716 | Lymphadenopathy | |
| HP:0002017 | Nausea and vomiting | |
| HP:0002093 | Respiratory insufficiency | |
| HP:0001824 | Weight loss | |
Run the differential on the 3 obligate / very frequent terms
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/26790
- This record as JSON: /data/d/26790.json