Global Variome shared LOVD
POLR1C (polymerase (RNA) I polypeptide C, 30kDa)
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Phenotypes for disease #03138 (PKKD (prekallikrein (Fletcher factor) deficiency), OMIM:612423)
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Please note that a short description of a certain column can be displayed when you move your mouse cursor over the column's header and hold it still. Below, a more detailed description is shown per column.
Phenotype details
: additional information on the phenotype of the individual, preferably use HPO terms only (http://www.human-phenotype-ontology.org/)
Diagnosis/Initial
: initial diagnosis, before molecular testing
Diagnosis/Definite
: phenotype individual after molecular testing (OMIM abbreviation)
Inheritance
: Indicates the inheritance of the phenotype in the family; unknown, familial (autosomal/X-linked, dominant/ recessive), paternal (Y-linked), maternal (mitochondrial), isolated (sporadic) or complex
All options:
Unknown
Familial
Familial, autosomal dominant
Familial, autosomal recessive
Familial, X-linked
Familial, X-linked dominant
Familial, X-linked dominant, male sparing
Familial, X-linked recessive
Paternal, Y-linked
Maternal, mitochondrial
Isolated (sporadic)
Di-genic
Complex
- = Not applicable
Age/Examination
: age at which the individual was examined.
35y = 35 years
04y08m = 4 years and 8 months
00y00m01d12h = 1 day and 12 hours
18y? = around 18 years
30y-40y = between 30 and 40 years
>54y = older than 54
? = unknown
Age/Diagnosis
: age diagnosis was confirmed
35y = 35 years
04y08m = 4 years and 8 months
00y00m01d12h = 1 day and 12 hours
18y? = around 18 years
30y-40y = between 30 and 40 years
>54y = older than 54
? = unknown
Age/Onset
: Age first symptoms disease appeared in individual:
35y = 35 years
04y08m = 4 years and 8 months
00y00m01d12h = 1 day and 12 hours
18y? = around 18 years
30y-40y = between 30 and 40 years
>54y = older than 54
? = unknown
Phenotype/Onset
: individual's phenotype at Age/Onset described using HPO
Protein
: result from protein staining
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17 entries on 1 page. Showing entries 1 - 17.
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How to query
Phenotype ID
Phenotype details
Diagnosis/Initial
Diagnosis/Definite
Inheritance
Age/Examination
Age/Diagnosis
Age/Onset
Phenotype/Onset
Protein
Owner
Individual ID
0000304472
Abnormal bleeding presented with prolongation of the activated partial thromboplastin time (aPTT)
-
-
Familial
-
-
-
-
-
Christian Drouet
00412470
0000304473
Proband presenting with an extremely prolonged K-APTT in a complete coagulation evaluation for hemostasis screening before surgery for a skin tumour
-
-
Familial
53y
-
-
-
-
Christian Drouet
00412471
0000304497
Prolonged coagulation time during hemostasis screening before surgery for sinusitis
-
-
Familial
47y
-
-
-
-
Christian Drouet
00412495
0000304580
A routine coagulation testing displayed aPTT prolongation, normal activity of coagulation factors, absence of heparinemia and lupus anticoagulant, quasi-normalization of aPTT with increasing pre-incubation time and PK:C and PK:Ag<1% each
-
-
Unknown
68y
-
-
-
-
Christian Drouet
00412586
0000304585
Isolated aPTT prolongation, normal coagulation factor activities were with no heparinemia and lupus anticoagulant. PK:C <1%, PK:Ag 10%, normal HK:C and HK:Ag
-
-
Familial
17y
-
-
-
-
Christian Drouet
00412591
0000304586
In a outine coagulation testing diagnosis of a severe PK deficiency based on aPTT prolongation, normal activity of coagulation factors except for FXII:C (39%-50%), neither heparinemia nor lupus anticoagulant, decreasing aPTT with increasing pre-incubation time, and <1% for PK:C and PK:Ag
-
-
Unknown
26y
-
-
-
-
Christian Drouet
00412593
0000304587
Combined PK deficiency and Bernard Soulier, proband presenting with recurrent epistaxis
-
-
Familial
03y
-
-
-
-
Christian Drouet
00412594
0000304588
Prolonged aPTT, normal PT and TT. Prolonged aPTT fully corrected by adding normal plasma, serum, plasma deficient in FXI, FXII, or HMWK
-
-
Familial
40y
-
-
-
-
Christian Drouet
00412596
0000304779
Proband presenting with a decreased aPTT and normal activity of coagulation factors
-
PK deficiency
Familial
13y
-
-
-
-
Christian Drouet
00412789
0000304781
Proband presenting with prolonged aPTT, aPTT shortening after prolonged pre-incubation, PK:C 5%, and PK:Ag 2%
-
-
Familial
71y
-
-
-
-
Christian Drouet
00412790
0000304887
Prolonged aPTT without any history of bleeding symptoms: 96 s (reference range: 24–36 s); the family history negative for bleeding or thrombotic events. Normal prothrombin time and thrombin time.
-
-
Familial
14y
-
-
-
-
Christian Drouet
00412901
0000304888
Proband presenting with a severe bleeding diathesis, with a low level of plasma prekallikrein activity; severity and recurrence of mucosal bleeding appeared late in life.
-
-
Unknown
-
75y
-
-
-
Christian Drouet
00412902
0000304915
Proband presenting with a iatrogenic oropharyngeal angioedema associated with an ACE inhibitor with complete absence of plasma prekallikrein
-
-
Unknown
67y
-
-
-
-
Christian Drouet
00412929
0000304936
proband presenting with a markedly prolonged aPTT (159 s ; control 27–34 s), with normal prothrombin time, thrombin time, fibrinogen level and platelet count.
-
-
Familial
-
60y
-
-
-
Christian Drouet
00412950
0000304937
Proband presenting with a second-trimester pregnancy loss, with a prolonged aPTT.
-
-
Familial
-
38y
-
-
-
Christian Drouet
00412951
0000325454
Proband presenting with a decreased aPTT and normal activity of coagulation factors
-
4y
Familial
-
-
-
-
-
Christian Drouet
00435257
0000325455
see paper; ...
prekallikrein deficiency
PKKD
Familial, autosomal recessive
14y
-
-
-
-
Johan den Dunnen
00435258
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