ORPHA:95427

Secondary short bowel syndrome

Type: Disease
Group: Disorder
Inheritance: Not applicable
Age of onset: All ages
Status: active

Secondary short bowel syndrome is an intestinal failure caused by any condition that results in a functional small intestine of less than 200 cm in length and is characterized by diarrhea, nutrient malabsoption, bowel dilation and dysmobility.

Names in Orphadata languages

12 of 12 languages

Lang	Name	Synonyms
en	Secondary short bowel syndrome
fr	Syndrome du grêle court secondaire	SGC secondaire · Syndrome de l'intestin court secondaire
de	Kurzdarm-Syndrom, sekundäres	Kurzdarm-Syndrom durch nekrotisierende Enterokolitis · Kurzdarm-Syndrom durch operative Resektion · Kurzdarm-Syndrom durch totale oder subtotale Aganglionose · Kurzdarm-Syndrom durch Thrombose · Kurzdarm-Syndrom durch Trauma · Kurzdarm-Syndrom durch Volvulus
es	Síndrome secundario del intestino corto
it	Sindrome secondaria dell'intestino corto
nl	Secundair kortedarmsyndroom	Kortedarmsyndroom als gevolg van chirurgische resectie · Kortedarmsyndroom als gevolg van necrotiserende enterocolitis · Kortedarmsyndroom als gevolg van totale of subtotale aganglionose · Kortedarmsyndroom als gevolg van trauma · Kortedarmsyndroom als gevolg van trombose · Kortedarmsyndroom als gevolg van volvulus
pt	Síndrome do intestino curto secundário
pl	Wtórny zespół krótkiego jelita
cs	Sekundární syndrom krátkého střeva
uk	Вторинний синдром короткої кишки
zh	继发性短肠综合征
tr	Sekonder kısa bağırsak sendromu

Definition in:

Cross-references

qualifier = Orphanet mapping relation

Source	Code	Qualifier	ICD relation	Validation
ICD-10	K91.2	BTNT	attributed	validated
ICD-11	DA96.04	NTBT	index	validated
MONDO	0019802	E		validated
UMLS	C5681579	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):

ORPHA:97935Rare gastroenterologic disease

Parents in the classifications (1):

ORPHA:104008Short bowel syndrome
rare gastroenterological diseases; rare transplant-related disorders

No children.

Genes

0 direct

Orphanet records no gene directly on this code.

Prevalence and incidence

2 records

Type	Class	Value	Area	Qualification	Validation	Source
Annual incidence	Unknown	—	Worldwide	Class only	Validated	30725620[PMID]_ORPHANET
Point prevalence	Unknown	—	Worldwide	Class only	Validated	30725620[PMID]_ORPHANET

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

HPO phenotypes

32 annotations · validated 2019-06-17

HPO	Term (as annotated by Orphanet)	Diagnostic
Frequent (79-30%)
HP:0002244	Abnormality of the small intestine
HP:0100508	Abnormality of vitamin metabolism
HP:0002024	Malabsorption
HP:0004395	Malnutrition
HP:0001824	Weight loss
Occasional (29-5%)
HP:0003270	Abdominal distention
HP:0003111	Abnormal blood ion concentration
HP:0001977	Abnormal thrombosis
HP:0002621	Atherosclerosis
HP:0001396	Cholestasis
HP:0002019	Constipation
HP:0001944	Dehydration
HP:0002014	Diarrhea
HP:0004387	Enterocolitis
HP:0001508	Failure to thrive
HP:0001543	Gastroschisis
HP:0001510	Growth delay
HP:0003572	Low plasma citrulline
HP:0030248	Mesenteric venous thrombosis
HP:0002591	Polyphagia
HP:0000832	Primary hypothyroidism
HP:0012850	Small intestinal dysmotility
HP:0030247	Splanchnic vein thrombosis
HP:0002570	Steatorrhea
HP:0011473	Villous atrophy
HP:0002580	Volvulus
HP:0002013	Vomiting
Very rare (<4-1%)
HP:0002251	Aganglionic megacolon
HP:0011787	Central hypothyroidism
HP:0001265	Hyporeflexia
HP:0011100	Intestinal atresia
HP:0100806	Sepsis

Annotation source: 30037012[PMID]_30613802[PMID]_30624508[PMID]_30725620[PMID]_28394957[PMID]_28827911[PMID]

Sources for this card

Orphanet page: orpha.net/en/disease/detail/95427
This record as JSON: /data/d/95427.json