Legend
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

 Phenotype ID
|

 Phenotype details
|

 Diagnosis/Initial
|

 Diagnosis/Definite
|

 Inheritance
|

 Age/Examination
|

 Age/Diagnosis
|

 Age/Onset
|

 Phenotype/Onset
|

 Protein
|

 Owner
|

 Individual ID
|
0000180469 |
Probands presenting with a HAE of the intermediate type, where low antigenic C1-INH is associated with a co-expression of both variant and wt alleles |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00240405 |
0000184420 |
Severe HAE phenotype |
- |
- |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Christian Drouet |
00244447 |
0000184468 |
HAE-I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00244502 |
0000184837 |
Three independent pedigrees are carrying the variant, Denmark, France
Probands presenting with a HAE of the intermediate type, where low antigenic C1 Inhibitor is associated with a co-expression of both variant and wt alleles. |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00244873 |
0000184838 |
HAE type II |
- |
HAE with C1 Inhibitor deficiency |
Unknown |
- |
- |
- |
- |
- |
Christian Drouet |
00244874 |
0000185024 |
7 pedigrees with heterozygous individuals presenting with a HAE type II. |
- |
HAE with C1 Inhibitor deficiency |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Christian Drouet |
00245063 |
0000185030 |
Proband presenting with SLE-like syndrome, with polyarthritis, pleuropericarditis, Raynaud's phenomenon and alopecia (Zahedi 1997).
The proband had partial deficiency of complement C4: Unusual hereditary C4 deficiency, with a normal C4 gene. |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245070 |
0000185048 |
HAE-I with heterozygous C1 inhibitor deficiency. |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245071 |
0000185176 |
Probands presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245199 |
0000185177 |
Probands presenting with a HAE of intermediate type, with a low antigenic C1Inh and an co-expression of both variant and wild-type alleles. |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245207 |
0000185179 |
Probands presenting with a HAE type I phenotype, in line with in vitro investigations.
French proband is sporadic, Iranian and Russian probands are familial cases |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245210 |
0000185185 |
Probands presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245216 |
0000185293 |
Numerous pedigrees carrying a c.550G>A variant.
Probands presenting with a HAE type I.
A proband is presenting with a de novo mutation, Pappalardo 2000. |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245344 |
0000185298 |
Probands presenting with a HAE type I. |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245349 |
0000185343 |
Proband and his brother suffering from a HAE type I phenotype. |
- |
- |
Complex |
- |
- |
- |
- |
- |
Christian Drouet |
00245397 |
0000185363 |
Proband presenting with a HAE type I.
Asymptomatic parents. |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Christian Drouet |
00245419 |
0000185364 |
Probands presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245420 |
0000185365 |
Probands from 3 pedigrees presenting with a HAE type I.
A sporadic proband with a de novo mutation has been reported. |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245422 |
0000185366 |
Probands presenting with a HAE type I phenotype |
- |
HAE-1 |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245423 |
0000185367 |
Probands presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245424 |
0000185368 |
Probants presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245425 |
0000185369 |
Probands presenting with a HAE type I phenotype. |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245426 |
0000185370 |
Probands presenting with a HAE type I phenotype. |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245427 |
0000185372 |
Proband presenting with a HAE type II phenotype |
- |
- |
Familial |
- |
47y |
- |
- |
- |
Christian Drouet |
00245429 |
0000185373 |
Probants presenting with a HAE type II phenotype. |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245430 |
0000185374 |
Probands presenting with a HAE type II phenotype. |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245431 |
0000185401 |
probands presenting with HAE of the intermediate type: low antigenic C1 Inhibitor and detectable variant product p.(Val473Gly) |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245432 |
0000185402 |
Proband presenting with a HAE type I phenotype of medium severity. |
- |
- |
Unknown |
- |
- |
- |
- |
Low antigenic C1 Inhibitor |
Christian Drouet |
00245433 |
0000185612 |
Variant associated with a HAE type I phenotype, but probably not causative |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245717 |
0000185613 |
Probands from French pedigrees have been identified with a HAE of the intermediate type, where low antigenic C1-INH is associated with a co-expression of both variant p.(Arg494*) and wt alleles. |
HAE-I |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245718 |
0000185614 |
Homozygous proband presenting with a mild HAE type II phenotype, heterozygotes are asymptomatic; c.[-21T>C];[-21T>C] might be a condition responsible for the disease |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Christian Drouet |
00245745 |
0000185615 |
Proband and his uncle presenting with a severe HAE type I phenotype, with a normal antigenic C4. |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245746 |
0000185616 |
Homozygous probands presenting with a HAE type I phenotype |
- |
- |
Familial, autosomal recessive |
- |
53y |
- |
- |
- |
Christian Drouet |
00245747 |
0000185617 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245748 |
0000185618 |
Probands presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245749 |
0000185619 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245750 |
0000185620 |
Proband presenting with a HAE phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245752 |
0000185621 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245753 |
0000185622 |
Probant presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245754 |
0000185623 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245755 |
0000185626 |
Proband presenting with a HAE phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245758 |
0000185627 |
Proband presenting with a HAE phenotype.
Initially identified as a type I HAE based on levels of functional and antigenic C1 Inhibitor (17% both) and low C4 levels.
Variant provisionnally attributed to a HAE of the intermediate type.
Upon analysis of plasma by immunoblotting, low level of an aberrant, low molecular mass form of C1 Inhibitor of about 70 kDa has been detected.
This species displays a smaller MW than the intact and cleaved forms C1 Inhibitor of 104 kDa and 97 kDa, respectively, naturally occuring in human plasma. |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245759 |
0000185630 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245762 |
0000185631 |
Probands presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245763 |
0000185632 |
Probands presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245764 |
0000185633 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245765 |
0000185634 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245767 |
0000185635 |
Probands presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245773 |
0000185660 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Christian Drouet |
00245797 |
0000185662 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245799 |
0000185663 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245800 |
0000185664 |
Probant presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245801 |
0000185665 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245802 |
0000185669 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245806 |
0000185670 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245807 |
0000185671 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00229843 |
0000185672 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00229875 |
0000185673 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00229816 |
0000185674 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245808 |
0000185675 |
Probands presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245809 |
0000185676 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245810 |
0000185687 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245849 |
0000185688 |
Probands presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245850 |
0000185689 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245851 |
0000185690 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245852 |
0000185691 |
Proband presenting with a HAE type I phenotype (Mete Gökmen 2019) |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245853 |
0000185692 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245854 |
0000185693 |
Probands presenting with a HAE type I phenotype. |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245855 |
0000185694 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245856 |
0000185695 |
Proband without specified phenotype |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Christian Drouet |
00245857 |
0000185696 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245858 |
0000185697 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245859 |
0000185698 |
Proband with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245860 |
0000185699 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245861 |
0000185700 |
Proband likely presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245862 |
0000185701 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245863 |
0000185702 |
Proband presenting with an unidentified phenotype |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Christian Drouet |
00245864 |
0000185703 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245865 |
0000185704 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245866 |
0000185705 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245868 |
0000185706 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245869 |
0000185707 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245870 |
0000185708 |
Probands likely presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245871 |
0000185709 |
Probands in many independent pedigrees presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245872 |
0000185710 |
Probands in 3 independent pedigrees presenting with a HAE type I phenotype. |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245873 |
0000185711 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245874 |
0000185712 |
Proband likely presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245875 |
0000185713 |
Probands from 2 independent pedigrees presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245876 |
0000185714 |
proband likely presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245877 |
0000185715 |
Probands likely presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245878 |
0000185716 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245879 |
0000185717 |
proband presenting with a HAE type I phenotype |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Christian Drouet |
00245880 |
0000185718 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245881 |
0000185738 |
Probands presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245903 |
0000185739 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245904 |
0000185740 |
Probands presenting with a HAE type II phenotype
A de novo condition has been recorded in a pedigree by Lopez-Lera 2011 |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245905 |
0000185741 |
Proband likely presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245906 |
0000185742 |
Proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245907 |
0000185743 |
proband presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245908 |
0000185744 |
Probands presenting with a HAE type I phenotype |
- |
- |
Familial |
- |
- |
- |
- |
- |
Christian Drouet |
00245909 |