{"frequencyBands":{"HP:0040280":[100,100],"HP:0040281":[80,99],"HP:0040282":[30,79],"HP:0040283":[5,29],"HP:0040284":[1,4],"HP:0040285":[0,0]},"page":{"columns":[{"gene":"","key":"MONDO:0013187/OMIM:613225","label":"Factor XIII, A subunit, deficiency of","mondoId":"MONDO:0013187","onsetQualifier":null,"recordKeys":["0d6b01d13533858e842ff1b91b579db301cdc123f88900f33795dac215fc971c","126931bee357a55376369414f1a9c718a5a65e9439a7c7f544ca3c66595ee481","203765b17590d1731b6188b8c8c33fee295c7ee23a81e42a6ad2f0521356eab6","275e322d49fee6e5426edc0c8448fabf5c50b2e1f5097b0128d0c0f2117cd9b0","3d3371d9ddad0aa0b3663b58b598d29b1ebb3c2b8cd86528f3361798100755a8","5e0887f0675162b851b55c7f1023fe03b2c0d7400a940cf24b6283970290af39","7c0ff7a6defaf8f5bf4bf7e812f8757f744342803eb4cc4da11c0f4bc2126fc4","7f8888362089d9a3552d461ea2f56ae007ebe935cd90ba7bd58f7e25ee725386","951f3ea3b008521329ecc53fe43e9f2672446fce365017d5e34b1abd48221e59","9f4660f4d08348de1440bc2b06bd9deb04cf28862c029a1f7c0377c2129bbc72","d56cda7ab0944e4a975763cb42f19b9c3fadac7fe6ea951f4d19d7d28d405ab9","dbb069b335e878aa56e06e35ba7fdf63744dd0412368bf15835c2c363d0420e8"],"recordQualifiers":{},"scopeLabel":"OMIM:613225","sourceIds":["OMIM:613225"],"sourceScope":"This column retains the native source entry. It is not a pooled estimate for the umbrella condition."},{"gene":"","key":"MONDO:0013190/OMIM:613235","label":"Factor XIIIB deficiency","mondoId":"MONDO:0013190","onsetQualifier":null,"recordKeys":["3bf07b98e8b01c230c3171e41755355375395696b12b662aed3d06f7d8111a73","5246bcab92be55312fe55e9d62f54765f4c22897defaa9b5e1f3324e1b4f6a27","601323832a383b7eb9d8829bdbb0ec8df2118cfe0978ee44fd012b9abdc7d1f2","613031b172f0da36f49ee130bc704cc0e4a540f7a11a5ec458a074e5951858a6","978a16e2f153882af944f0ded1f8cde6873bcc5149d3039b207e9dbfd7b6b95c","ed71d82aa64f57cc36148d1b39c65a6cee0756ced9d4a18ef0f63450c93b00e3","f5693d315faeb1a60c7de247e47e2c96dac96577aa5dd2df05c9f68c725be633"],"recordQualifiers":{},"scopeLabel":"OMIM:613235","sourceIds":["OMIM:613235"],"sourceScope":"This column retains the native source entry. It is not a pooled estimate for the umbrella condition."},{"gene":"","key":"MONDO:0018029/ORPHA:331","label":"Congenital factor XIII deficiency","mondoId":"MONDO:0018029","onsetQualifier":null,"recordKeys":["15f89d521b30d05941243a507a747141427256718d25be58aa09f478dfe4cfd1","2b71e219438a2503f4e169b166c65d66c3fc935c583700a908a4abd66c8dd899","36e28468306cbb3a3ed729f70a1ce30ac18ecffab1248f2aa54f08b31398738e","3ba821fc5232db4b9433bad34bb660e69e7723110c33c797faa15d8f55536aac","3f57a13c21dd58d1ca24e9f00d56b3ef2219bc9eef8bb7c70846e42b9ae7b561","4dd4be1b8c4bc377970e4cf4c1a0468703976fbaa09008349b6abfd38d418c1c","4e42d0c793c09b19a98260451e0efc8cadac0427cae2543911a16bc03f9c63a1","5191054196b15128a3b79b7e792413404b3dc79313783356c2f77b4679d5cc80","6402a2a941b512d08164fe1ab9284d821e7b76d0be76975102709ff1e2c74792","66f94eb5a784ad072109468fee52fed72ee64e54b332d0e94925deb75274c551","71277169903ce8c27baedb96c4b4770ce4d5b4ed15f450e3a8b21a2dd0e82458","7480c17ede7c06e34c7b565824bf8c8792fb50fbf10bece3c05a3f7cf9bc3daf","88af35fc4c6b3b325f3c412eee5b9aa041ec74d491fdf71ae9950f64c6cc4c6b","8e8770d0b030f5f65b3b4394f441e47441284c550a39b9ffac3f712ba4177d17","90a12defcaef0cb15deeb4531bf0c4a6cff33b08471e68b1b4a0b0f06c05dec7","92b6cadece07245ee90bc70d266752b918d7318c8ee9f4c05ba4391ff2390e0c","9e1e378bb3ea56f9fe45a05a260c21b71341b834b9169b545eef058debf91d43","a18bc9e57ce05a5c5fb2f61e0ddb145a76779f67f64a600fbd667529b96a7f27","b67561cb3338b04ae287d57e6883a56014c8001ac1779a6ca7f960fe8b381840","bb4598a916e6ead9291458bf88848391f6fb9519d1efa4a4d030de34caa5dcb6","c580361b2315450af8469b84edfaed12baed1f3eb8a7e9c4313de3e0f74f25b6","db4f9a59b7e8056700fac65328a5e1ad8ebeeb057c9512a8ee0f2c862b604aa5","eff29313f11a3d7f1762b6ee725e046aaa001c0f40a4623209900fb3db48184e","f81104430cc19b25e7a77b4da359f7b2701dc1b2317c020d6b6cae8796ff5d9f","fa3eec6cf0455862d0f5a28c65516455594ecf243beeabe2d4925ee3eecc1ae9"],"recordQualifiers":{},"scopeLabel":"ORPHA:331","sourceIds":["ORPHA:331"],"sourceScope":"This column retains the native source entry. It is not a pooled estimate for the umbrella condition."}],"comparison":{"collapseColumnsLabel":"Show initial comparisons","defaultColumnKeys":["MONDO:0013187/OMIM:613225","MONDO:0013190/OMIM:613235","MONDO:0018029/ORPHA:331"],"expandColumnsLabel":"Compare all source entries","intro":"Compare the original source entries. Different entries may describe different patient groups.","primaryFeatureIds":[],"scopeNoteHtml":"

Each column keeps its native condition scope. Initial features are displayed alphabetically, not ranked by clinical importance. Counts remain separate, and an empty cell does not mean a feature cannot occur.

"},"coverage":{"countRecords":14,"emptyColumnKeys":[],"hpoRelease":"2026-06-23","narrativeSourceChanges":[],"records":44,"sourceGroups":{"omim":5,"orphanet":25,"published":14}},"features":[{"hpoId":"HP:0001892","label":"Abnormal bleeding","primary":false},{"hpoId":"HP:0011884","label":"Abnormal umbilical stump bleeding","primary":false},{"hpoId":"HP:0011889","label":"Bleeding with minor or no trauma","primary":false},{"hpoId":"HP:0000978","label":"Bruising susceptibility","primary":false},{"hpoId":"HP:0001342","label":"Cerebral hemorrhage","primary":false},{"hpoId":"HP:0040232","label":"Delayed onset bleeding","primary":false},{"hpoId":"HP:0031364","label":"Ecchymosis","primary":false},{"hpoId":"HP:0000421","label":"Epistaxis","primary":false},{"hpoId":"HP:0040233","label":"Factor XIII subunit A deficiency","primary":false},{"hpoId":"HP:0040234","label":"Factor XIII subunit B deficiency","primary":false},{"hpoId":"HP:0000225","label":"Gingival bleeding","primary":false},{"hpoId":"HP:0001399","label":"Hepatic failure","primary":false},{"hpoId":"HP:0002037","label":"Inflammation of the large intestine","primary":false},{"hpoId":"HP:0002170","label":"Intracranial hemorrhage","primary":false},{"hpoId":"HP:0012233","label":"Intramuscular hematoma","primary":false},{"hpoId":"HP:0005261","label":"Joint hemorrhage","primary":false},{"hpoId":"HP:0000132","label":"Menorrhagia","primary":false},{"hpoId":"HP:0012324","label":"Myeloid leukemia","primary":false},{"hpoId":"HP:0030140","label":"Oral cavity bleeding","primary":false},{"hpoId":"HP:0001934","label":"Persistent bleeding after trauma","primary":false},{"hpoId":"HP:0001058","label":"Poor wound healing","primary":false},{"hpoId":"HP:0011891","label":"Post-partum hemorrhage","primary":false},{"hpoId":"HP:0006298","label":"Prolonged bleeding after dental extraction","primary":false},{"hpoId":"HP:0004846","label":"Prolonged bleeding after surgery","primary":false},{"hpoId":"HP:0030137","label":"Prolonged bleeding following circumcision","primary":false},{"hpoId":"HP:0008357","label":"Reduced factor XIII activity","primary":false},{"hpoId":"HP:0007420","label":"Spontaneous hematomas","primary":false},{"hpoId":"HP:0001933","label":"Subcutaneous hemorrhage","primary":false},{"hpoId":"HP:0030657","label":"Umbilical cord hematoma","primary":false}],"ledeHtml":"Factor XIII deficiency is a rare bleeding disorder. Researchers have identified an inherited form and a less severe form that is acquired during a person's lifetime.","mondoId":"MONDO:0002241","records":[{"frequencyKind":"reported","key":"0d6b01d13533858e842ff1b91b579db301cdc123f88900f33795dac215fc971c","raw":{"aspect":"P","body_system":"blood","disease_id":"OMIM:613225","disease_name":"Factor XIII, A subunit, deficiency of","evidence":"TAS","frequency_raw":null,"hpo_id":"HP:0000421","is_excluded":false,"label":"Epistaxis","onset_id":null,"reference":"OMIM:613225","source_release":"2026-06-23"},"sourceGroups":["omim"]},{"frequencyKind":"count","key":"126931bee357a55376369414f1a9c718a5a65e9439a7c7f544ca3c66595ee481","raw":{"aspect":"P","body_system":"blood","disease_id":"OMIM:613225","disease_name":"Factor XIII, A subunit, deficiency of","evidence":"PCS","frequency_raw":"1/2","hpo_id":"HP:0001934","is_excluded":false,"label":"Persistent bleeding after trauma","onset_id":null,"reference":"PMID:7727776","source_release":"2026-06-23"},"sourceGroups":["published"]},{"frequencyKind":"range","key":"15f89d521b30d05941243a507a747141427256718d25be58aa09f478dfe4cfd1","raw":{"aspect":"P","body_system":"blood","disease_id":"ORPHA:331","disease_name":"Congenital factor XIII deficiency","evidence":"TAS","frequency_raw":"HP:0040282","hpo_id":"HP:0005261","is_excluded":false,"label":"Joint hemorrhage","onset_id":null,"reference":"ORPHA:331","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"count","key":"203765b17590d1731b6188b8c8c33fee295c7ee23a81e42a6ad2f0521356eab6","raw":{"aspect":"P","body_system":"blood","disease_id":"OMIM:613225","disease_name":"Factor XIII, A subunit, deficiency of","evidence":"PCS","frequency_raw":"2/2","hpo_id":"HP:0031364","is_excluded":false,"label":"Ecchymosis","onset_id":null,"reference":"PMID:7727776","source_release":"2026-06-23"},"sourceGroups":["published"]},{"frequencyKind":"count","key":"275e322d49fee6e5426edc0c8448fabf5c50b2e1f5097b0128d0c0f2117cd9b0","raw":{"aspect":"P","body_system":"blood","disease_id":"OMIM:613225","disease_name":"Factor XIII, A subunit, deficiency of","evidence":"PCS","frequency_raw":"2/2","hpo_id":"HP:0001892","is_excluded":false,"label":"Abnormal bleeding","onset_id":null,"reference":"PMID:7727776","source_release":"2026-06-23"},"sourceGroups":["published"]},{"frequencyKind":"range","key":"2b71e219438a2503f4e169b166c65d66c3fc935c583700a908a4abd66c8dd899","raw":{"aspect":"P","body_system":"blood","disease_id":"ORPHA:331","disease_name":"Congenital factor XIII deficiency","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0011891","is_excluded":false,"label":"Post-partum hemorrhage","onset_id":null,"reference":"ORPHA:331","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"36e28468306cbb3a3ed729f70a1ce30ac18ecffab1248f2aa54f08b31398738e","raw":{"aspect":"P","body_system":"blood","disease_id":"ORPHA:331","disease_name":"Congenital factor XIII deficiency","evidence":"TAS","frequency_raw":"HP:0040281","hpo_id":"HP:0011884","is_excluded":false,"label":"Abnormal umbilical stump bleeding","onset_id":null,"reference":"ORPHA:331","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"3ba821fc5232db4b9433bad34bb660e69e7723110c33c797faa15d8f55536aac","raw":{"aspect":"P","body_system":"musculoskeletal","disease_id":"ORPHA:331","disease_name":"Congenital factor XIII deficiency","evidence":"TAS","frequency_raw":"HP:0040282","hpo_id":"HP:0012233","is_excluded":false,"label":"Intramuscular hematoma","onset_id":null,"reference":"ORPHA:331","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"count","key":"3bf07b98e8b01c230c3171e41755355375395696b12b662aed3d06f7d8111a73","raw":{"aspect":"P","body_system":"blood","disease_id":"OMIM:613235","disease_name":"Factor XIIIB deficiency","evidence":"PCS","frequency_raw":"2/2","hpo_id":"HP:0040234","is_excluded":false,"label":"Factor XIII subunit B deficiency","onset_id":null,"reference":"PMID:11313256","source_release":"2026-06-23"},"sourceGroups":["published"]},{"frequencyKind":"count","key":"3d3371d9ddad0aa0b3663b58b598d29b1ebb3c2b8cd86528f3361798100755a8","raw":{"aspect":"P","body_system":"blood","disease_id":"OMIM:613225","disease_name":"Factor XIII, A subunit, deficiency of","evidence":"PCS","frequency_raw":"2/2","hpo_id":"HP:0040233","is_excluded":false,"label":"Factor XIII subunit A deficiency","onset_id":null,"reference":"PMID:7727776","source_release":"2026-06-23"},"sourceGroups":["published"]},{"frequencyKind":"range","key":"3f57a13c21dd58d1ca24e9f00d56b3ef2219bc9eef8bb7c70846e42b9ae7b561","raw":{"aspect":"P","body_system":"blood","disease_id":"ORPHA:331","disease_name":"Congenital factor XIII deficiency","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0004846","is_excluded":false,"label":"Prolonged bleeding after surgery","onset_id":null,"reference":"ORPHA:331","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"4dd4be1b8c4bc377970e4cf4c1a0468703976fbaa09008349b6abfd38d418c1c","raw":{"aspect":"P","body_system":"blood","disease_id":"ORPHA:331","disease_name":"Congenital factor XIII deficiency","evidence":"TAS","frequency_raw":"HP:0040281","hpo_id":"HP:0008357","is_excluded":false,"label":"Reduced factor XIII activity","onset_id":null,"reference":"ORPHA:331","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"4e42d0c793c09b19a98260451e0efc8cadac0427cae2543911a16bc03f9c63a1","raw":{"aspect":"P","body_system":"digestive","disease_id":"ORPHA:331","disease_name":"Congenital factor XIII deficiency","evidence":"TAS","frequency_raw":"HP:0040284","hpo_id":"HP:0002037","is_excluded":false,"label":"Inflammation of the large intestine","onset_id":null,"reference":"ORPHA:331","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"5191054196b15128a3b79b7e792413404b3dc79313783356c2f77b4679d5cc80","raw":{"aspect":"P","body_system":"blood","disease_id":"ORPHA:331","disease_name":"Congenital factor XIII deficiency","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0000225","is_excluded":false,"label":"Gingival bleeding","onset_id":null,"reference":"ORPHA:331","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"reported","key":"5246bcab92be55312fe55e9d62f54765f4c22897defaa9b5e1f3324e1b4f6a27","raw":{"aspect":"P","body_system":"blood","disease_id":"OMIM:613235","disease_name":"Factor XIIIB deficiency","evidence":"IEA","frequency_raw":null,"hpo_id":"HP:0031364","is_excluded":false,"label":"Ecchymosis","onset_id":null,"reference":"OMIM:613235","source_release":"2026-06-23"},"sourceGroups":["omim"]},{"frequencyKind":"reported","key":"5e0887f0675162b851b55c7f1023fe03b2c0d7400a940cf24b6283970290af39","raw":{"aspect":"P","body_system":"blood","disease_id":"OMIM:613225","disease_name":"Factor XIII, A subunit, deficiency of","evidence":"TAS","frequency_raw":null,"hpo_id":"HP:0002170","is_excluded":false,"label":"Intracranial hemorrhage","onset_id":null,"reference":"OMIM:613225","source_release":"2026-06-23"},"sourceGroups":["omim"]},{"frequencyKind":"count","key":"601323832a383b7eb9d8829bdbb0ec8df2118cfe0978ee44fd012b9abdc7d1f2","raw":{"aspect":"P","body_system":"blood","disease_id":"OMIM:613235","disease_name":"Factor XIIIB deficiency","evidence":"PCS","frequency_raw":"1/2","hpo_id":"HP:0004846","is_excluded":false,"label":"Prolonged bleeding after surgery","onset_id":null,"reference":"PMID:11313256","source_release":"2026-06-23"},"sourceGroups":["published"]},{"frequencyKind":"count","key":"613031b172f0da36f49ee130bc704cc0e4a540f7a11a5ec458a074e5951858a6","raw":{"aspect":"P","body_system":"blood","disease_id":"OMIM:613235","disease_name":"Factor XIIIB deficiency","evidence":"PCS","frequency_raw":"1/2","hpo_id":"HP:0011884","is_excluded":false,"label":"Abnormal umbilical stump bleeding","onset_id":"HP:0003623","reference":"PMID:11313256","source_release":"2026-06-23"},"sourceGroups":["published"]},{"frequencyKind":"range","key":"6402a2a941b512d08164fe1ab9284d821e7b76d0be76975102709ff1e2c74792","raw":{"aspect":"P","body_system":"blood","disease_id":"ORPHA:331","disease_name":"Congenital factor XIII deficiency","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0006298","is_excluded":false,"label":"Prolonged bleeding after dental extraction","onset_id":null,"reference":"ORPHA:331","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"66f94eb5a784ad072109468fee52fed72ee64e54b332d0e94925deb75274c551","raw":{"aspect":"P","body_system":"blood","disease_id":"ORPHA:331","disease_name":"Congenital factor XIII deficiency","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0030137","is_excluded":false,"label":"Prolonged bleeding following circumcision","onset_id":null,"reference":"ORPHA:331","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"71277169903ce8c27baedb96c4b4770ce4d5b4ed15f450e3a8b21a2dd0e82458","raw":{"aspect":"P","body_system":"blood","disease_id":"ORPHA:331","disease_name":"Congenital factor XIII deficiency","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0001934","is_excluded":false,"label":"Persistent bleeding after trauma","onset_id":null,"reference":"ORPHA:331","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"7480c17ede7c06e34c7b565824bf8c8792fb50fbf10bece3c05a3f7cf9bc3daf","raw":{"aspect":"P","body_system":"blood","disease_id":"ORPHA:331","disease_name":"Congenital factor XIII deficiency","evidence":"TAS","frequency_raw":"HP:0040282","hpo_id":"HP:0000978","is_excluded":false,"label":"Bruising susceptibility","onset_id":null,"reference":"ORPHA:331","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"count","key":"7c0ff7a6defaf8f5bf4bf7e812f8757f744342803eb4cc4da11c0f4bc2126fc4","raw":{"aspect":"P","body_system":"blood","disease_id":"OMIM:613225","disease_name":"Factor XIII, A subunit, deficiency of","evidence":"PCS","frequency_raw":"2/2","hpo_id":"HP:0000978","is_excluded":false,"label":"Bruising susceptibility","onset_id":null,"reference":"PMID:7727776","source_release":"2026-06-23"},"sourceGroups":["published"]},{"frequencyKind":"reported","key":"7f8888362089d9a3552d461ea2f56ae007ebe935cd90ba7bd58f7e25ee725386","raw":{"aspect":"P","body_system":"blood","disease_id":"OMIM:613225","disease_name":"Factor XIII, A subunit, deficiency of","evidence":"TAS","frequency_raw":null,"hpo_id":"HP:0005261","is_excluded":false,"label":"Joint hemorrhage","onset_id":null,"reference":"OMIM:613225","source_release":"2026-06-23"},"sourceGroups":["omim"]},{"frequencyKind":"range","key":"88af35fc4c6b3b325f3c412eee5b9aa041ec74d491fdf71ae9950f64c6cc4c6b","raw":{"aspect":"P","body_system":"blood","disease_id":"ORPHA:331","disease_name":"Congenital factor XIII deficiency","evidence":"TAS","frequency_raw":"HP:0040282","hpo_id":"HP:0001342","is_excluded":false,"label":"Cerebral hemorrhage","onset_id":null,"reference":"ORPHA:331","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"8e8770d0b030f5f65b3b4394f441e47441284c550a39b9ffac3f712ba4177d17","raw":{"aspect":"P","body_system":"skin","disease_id":"ORPHA:331","disease_name":"Congenital factor XIII deficiency","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0001058","is_excluded":false,"label":"Poor wound healing","onset_id":null,"reference":"ORPHA:331","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"90a12defcaef0cb15deeb4531bf0c4a6cff33b08471e68b1b4a0b0f06c05dec7","raw":{"aspect":"P","body_system":"blood","disease_id":"ORPHA:331","disease_name":"Congenital factor XIII deficiency","evidence":"TAS","frequency_raw":"HP:0040282","hpo_id":"HP:0030140","is_excluded":false,"label":"Oral cavity bleeding","onset_id":null,"reference":"ORPHA:331","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"92b6cadece07245ee90bc70d266752b918d7318c8ee9f4c05ba4391ff2390e0c","raw":{"aspect":"P","body_system":"blood","disease_id":"ORPHA:331","disease_name":"Congenital factor XIII deficiency","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0000132","is_excluded":false,"label":"Menorrhagia","onset_id":null,"reference":"ORPHA:331","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"count","key":"951f3ea3b008521329ecc53fe43e9f2672446fce365017d5e34b1abd48221e59","raw":{"aspect":"P","body_system":"blood","disease_id":"OMIM:613225","disease_name":"Factor XIII, A subunit, deficiency of","evidence":"PCS","frequency_raw":"2/2","hpo_id":"HP:0000225","is_excluded":false,"label":"Gingival bleeding","onset_id":null,"reference":"PMID:7727776","source_release":"2026-06-23"},"sourceGroups":["published"]},{"frequencyKind":"count","key":"978a16e2f153882af944f0ded1f8cde6873bcc5149d3039b207e9dbfd7b6b95c","raw":{"aspect":"P","body_system":"blood","disease_id":"OMIM:613235","disease_name":"Factor XIIIB deficiency","evidence":"PCS","frequency_raw":"1/1","hpo_id":"HP:0001892","is_excluded":false,"label":"Abnormal bleeding","onset_id":null,"reference":"PMID:8324218","source_release":"2026-06-23"},"sourceGroups":["published"]},{"frequencyKind":"range","key":"9e1e378bb3ea56f9fe45a05a260c21b71341b834b9169b545eef058debf91d43","raw":{"aspect":"P","body_system":"digestive","disease_id":"ORPHA:331","disease_name":"Congenital factor XIII deficiency","evidence":"TAS","frequency_raw":"HP:0040284","hpo_id":"HP:0001399","is_excluded":false,"label":"Hepatic failure","onset_id":null,"reference":"ORPHA:331","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"count","key":"9f4660f4d08348de1440bc2b06bd9deb04cf28862c029a1f7c0377c2129bbc72","raw":{"aspect":"P","body_system":"blood","disease_id":"OMIM:613225","disease_name":"Factor XIII, A subunit, deficiency of","evidence":"PCS","frequency_raw":"2/2","hpo_id":"HP:0011884","is_excluded":false,"label":"Abnormal umbilical stump bleeding","onset_id":null,"reference":"PMID:7727776","source_release":"2026-06-23"},"sourceGroups":["published"]},{"frequencyKind":"range","key":"a18bc9e57ce05a5c5fb2f61e0ddb145a76779f67f64a600fbd667529b96a7f27","raw":{"aspect":"P","body_system":"prenatal","disease_id":"ORPHA:331","disease_name":"Congenital factor XIII deficiency","evidence":"TAS","frequency_raw":"HP:0040281","hpo_id":"HP:0030657","is_excluded":false,"label":"Umbilical cord hematoma","onset_id":null,"reference":"ORPHA:331","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"b67561cb3338b04ae287d57e6883a56014c8001ac1779a6ca7f960fe8b381840","raw":{"aspect":"P","body_system":"blood","disease_id":"ORPHA:331","disease_name":"Congenital factor XIII deficiency","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0040232","is_excluded":false,"label":"Delayed onset bleeding","onset_id":null,"reference":"ORPHA:331","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"bb4598a916e6ead9291458bf88848391f6fb9519d1efa4a4d030de34caa5dcb6","raw":{"aspect":"P","body_system":"blood","disease_id":"ORPHA:331","disease_name":"Congenital factor XIII deficiency","evidence":"TAS","frequency_raw":"HP:0040282","hpo_id":"HP:0007420","is_excluded":false,"label":"Spontaneous hematomas","onset_id":null,"reference":"ORPHA:331","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"c580361b2315450af8469b84edfaed12baed1f3eb8a7e9c4313de3e0f74f25b6","raw":{"aspect":"P","body_system":"blood","disease_id":"ORPHA:331","disease_name":"Congenital factor XIII deficiency","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0000421","is_excluded":false,"label":"Epistaxis","onset_id":null,"reference":"ORPHA:331","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"count","key":"d56cda7ab0944e4a975763cb42f19b9c3fadac7fe6ea951f4d19d7d28d405ab9","raw":{"aspect":"P","body_system":"blood","disease_id":"OMIM:613225","disease_name":"Factor XIII, A subunit, deficiency of","evidence":"PCS","frequency_raw":"2/2","hpo_id":"HP:0008357","is_excluded":false,"label":"Reduced factor XIII activity","onset_id":null,"reference":"PMID:7727776","source_release":"2026-06-23"},"sourceGroups":["published"]},{"frequencyKind":"range","key":"db4f9a59b7e8056700fac65328a5e1ad8ebeeb057c9512a8ee0f2c862b604aa5","raw":{"aspect":"P","body_system":"blood","disease_id":"ORPHA:331","disease_name":"Congenital factor XIII deficiency","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0011889","is_excluded":false,"label":"Bleeding with minor or no trauma","onset_id":null,"reference":"ORPHA:331","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"count","key":"dbb069b335e878aa56e06e35ba7fdf63744dd0412368bf15835c2c363d0420e8","raw":{"aspect":"P","body_system":"blood","disease_id":"OMIM:613225","disease_name":"Factor XIII, A subunit, deficiency of","evidence":"PCS","frequency_raw":"1/2","hpo_id":"HP:0007420","is_excluded":false,"label":"Spontaneous hematomas","onset_id":null,"reference":"PMID:7727776","source_release":"2026-06-23"},"sourceGroups":["published"]},{"frequencyKind":"reported","key":"ed71d82aa64f57cc36148d1b39c65a6cee0756ced9d4a18ef0f63450c93b00e3","raw":{"aspect":"P","body_system":"blood","disease_id":"OMIM:613235","disease_name":"Factor XIIIB deficiency","evidence":"TAS","frequency_raw":null,"hpo_id":"HP:0000978","is_excluded":false,"label":"Bruising susceptibility","onset_id":null,"reference":"OMIM:613235","source_release":"2026-06-23"},"sourceGroups":["omim"]},{"frequencyKind":"range","key":"eff29313f11a3d7f1762b6ee725e046aaa001c0f40a4623209900fb3db48184e","raw":{"aspect":"P","body_system":"blood","disease_id":"ORPHA:331","disease_name":"Congenital factor XIII deficiency","evidence":"TAS","frequency_raw":"HP:0040284","hpo_id":"HP:0012324","is_excluded":false,"label":"Myeloid leukemia","onset_id":null,"reference":"ORPHA:331","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"count","key":"f5693d315faeb1a60c7de247e47e2c96dac96577aa5dd2df05c9f68c725be633","raw":{"aspect":"P","body_system":"blood","disease_id":"OMIM:613235","disease_name":"Factor XIIIB deficiency","evidence":"PCS","frequency_raw":"3/3","hpo_id":"HP:0008357","is_excluded":false,"label":"Reduced factor XIII activity","onset_id":null,"reference":"PMID:8324218;PMID:11313256","source_release":"2026-06-23"},"sourceGroups":["published"]},{"frequencyKind":"range","key":"f81104430cc19b25e7a77b4da359f7b2701dc1b2317c020d6b6cae8796ff5d9f","raw":{"aspect":"P","body_system":"blood","disease_id":"ORPHA:331","disease_name":"Congenital factor XIII deficiency","evidence":"TAS","frequency_raw":"HP:0040283","hpo_id":"HP:0031364","is_excluded":false,"label":"Ecchymosis","onset_id":null,"reference":"ORPHA:331","source_release":"2026-06-23"},"sourceGroups":["orphanet"]},{"frequencyKind":"range","key":"fa3eec6cf0455862d0f5a28c65516455594ecf243beeabe2d4925ee3eecc1ae9","raw":{"aspect":"P","body_system":"blood","disease_id":"ORPHA:331","disease_name":"Congenital factor XIII deficiency","evidence":"TAS","frequency_raw":"HP:0040282","hpo_id":"HP:0001933","is_excluded":false,"label":"Subcutaneous hemorrhage","onset_id":null,"reference":"ORPHA:331","source_release":"2026-06-23"},"sourceGroups":["orphanet"]}],"referenceLabels":{},"references":{"causeHtml":"","identityNote":"MONDO cross-references identify native condition entries. Ancestor membership does not transfer phenotype values between groups.","importantHtml":""},"sections":[{"bodyHtml":"

Inherited factor XIII deficiency results from mutations in the F13A1 gene or, less commonly, the F13B gene. These genes provide instructions for making the two parts (subunits) of a protein called factor XIII. This protein plays a critical role in the coagulation cascade, which is a series of chemical reactions that forms blood clots in response to injury. After an injury, clots seal off blood vessels to stop bleeding and trigger blood vessel repair. Factor XIII acts at the end of the cascade to strengthen and stabilize newly formed clots, preventing further blood loss.

Mutations in the F13A1 or F13B gene significantly reduce the amount of functional factor XIII available to participate in blood clotting. In most people with the inherited form of the condition, factor XIII levels in the bloodstream are less than 5 percent of normal. A loss of this protein's activity weakens blood clots, preventing the clots from stopping blood loss effectively.

The acquired form of factor XIII deficiency results when the production of factor XIII is reduced or when the body uses factor XIII faster than cells can replace it. Acquired factor XIII deficiency is generally mild because levels of factor XIII in the bloodstream are 20 to 70 percent of normal; levels above 10 percent of normal are usually adequate to prevent spontaneous bleeding episodes.

Acquired factor XIII deficiency can be caused by disorders including an inflammatory disease of the liver called hepatitis, scarring of the liver (cirrhosis), inflammatory bowel disease, overwhelming bacterial infections (sepsis), and several types of cancer. Acquired factor XIII deficiency can also be caused by abnormal activation of the immune system, which produces specialized proteins called autoantibodies that attack and disable the factor XIII protein. The production of autoantibodies against factor XIII is sometimes associated with immune system diseases such as systemic lupus erythematosus and rheumatoid arthritis. In other cases, the trigger for autoantibody production is unknown.

MedlinePlus Genetics: Causes

","key":"forms","title":"What causes Factor XIII deficiency?"},{"bodyHtml":"

Signs and symptoms of inherited factor XIII deficiency begin soon after birth, usually with abnormal bleeding from the umbilical cord stump. If the condition is not treated, affected individuals may have episodes of excessive and prolonged bleeding that can be life-threatening. Abnormal bleeding can occur after surgery or minor trauma. The condition can also cause spontaneous bleeding into the joints or muscles, leading to pain and disability. Women with inherited factor XIII deficiency tend to have heavy or prolonged menstrual bleeding (menorrhagia) and may experience recurrent pregnancy losses (miscarriages). Other signs and symptoms of inherited factor XIII deficiency include nosebleeds, bleeding of the gums, easy bruising, problems with wound healing, bleeding after surgery, and abnormal scar formation. Inherited factor XIII deficiency also increases the risk of spontaneous bleeding inside the skull (intracranial hemorrhage), which is the leading cause of death in people with this condition.

Acquired factor XIII deficiency becomes apparent later in life. People with the acquired form are less likely to have severe or life-threatening episodes of abnormal bleeding than those with the inherited form.

Source: MedlinePlus Genetics

","key":"overview","title":"Condition overview"}],"sharedSources":[],"slug":"factor-xiii-deficiency","sourceDocuments":[{"name":"Factor XIII deficiency","reviewed":"2015-08","sections":{"causes":{"fetchedAt":"2026-10-20T20:21:19.232550+00:00","label":"Causes","pageSha256":"71afd753dad37a3208ca00ce7d19a9ecd7fb39fbbe108cc12fef375d20d88556","sourceUrl":"https://medlineplus.gov/genetics/condition/factor-xiii-deficiency/#causes","status":"available"}},"source_release":"2026-10-01","url":"https://medlineplus.gov/genetics/condition/factor-xiii-deficiency"}],"sourceNote":"MedlinePlus descriptions and Causes; original HPO annotations.","takeawayHtml":"Cause explanations are from MedlinePlus Genetics. Feature values below retain their original sources and patient groups.","title":"Factor XIII deficiency"},"schemaVersion":1}