SHNKND
MCID: SCH030
MIFTS: 44

Schneckenbecken Dysplasia (SHNKND)

Categories: Bone diseases, Fetal diseases, Genetic diseases, Metabolic diseases, Rare diseases

Aliases & Classifications for Schneckenbecken Dysplasia

MalaCards integrated aliases for Schneckenbecken Dysplasia:

Name: Schneckenbecken Dysplasia 56 12 52 58 73 36 29 13 6 43 15 71
Chondrodysplasia, Lethal Neonatal, with Snail-Like Pelvis 56 73
Shnknd 56 73
Chondrodysplasia Lethal Neonatal with Snail Like Pelvis 52
Chondrodysplasia with Snail-Like Pelvis 58
Dysplasia, Schneckenbecken 39
Slc35d1-Cdg 58

Characteristics:

Orphanet epidemiological data:

58
schneckenbecken dysplasia
Inheritance: Autosomal recessive; Prevalence: <1/1000000 (Worldwide); Age of onset: Antenatal; Age of death: embryofetal,stillbirth;

OMIM:

56
Inheritance:
autosomal recessive

Miscellaneous:
stillborn or lethal in the neonatal period


HPO:

31
schneckenbecken dysplasia:
Clinical modifier stillbirth
Inheritance autosomal recessive inheritance


Classifications:

Orphanet: 58  
Rare bone diseases
Inborn errors of metabolism
Developmental anomalies during embryogenesis


External Ids:

Disease Ontology 12 DOID:0050775
OMIM 56 269250
KEGG 36 H00474
MESH via Orphanet 44 C536637
ICD10 via Orphanet 33 Q77.7
UMLS via Orphanet 72 C0432194
Orphanet 58 ORPHA3144
MedGen 41 C0432194
UMLS 71 C0432194

Summaries for Schneckenbecken Dysplasia

NIH Rare Diseases : 52 The following summary is from Orphanet , a European reference portal for information on rare diseases and orphan drugs. Orpha Number: 3144 Definition Schneckenbecken dysplasia (or chondrodysplasia with snail-like pelvis) is a prenatally lethal spondylodysplastic dysplasia. Epidemiology Less than 20 cases have been reported in the literature so far. Clinical description The typical radiographic finding is the snail-like configuration of the hypoplastic iliac bone. Additional features include flattened hypoplastic vertebral bodies, short ribs, short and wide fibulae, short and broad long bones with a dumbbell-like appearance, and precocious ossification of the tarsus. Etiology This syndrome is caused by loss-of-function mutations of the SLC35D1 gene (1p32-p31) Genetic counseling Schneckenbecken dysplasia is transmitted in an autosomal recessive manner. Visit the Orphanet disease page for more resources.

MalaCards based summary : Schneckenbecken Dysplasia, also known as chondrodysplasia, lethal neonatal, with snail-like pelvis, is related to fibrochondrogenesis and odontochondrodysplasia. An important gene associated with Schneckenbecken Dysplasia is SLC35D1 (Solute Carrier Family 35 Member D1), and among its related pathways/superpathways are Transport of glucose and other sugars, bile salts and organic acids, metal ions and amine compounds and Transport of vitamins, nucleosides, and related molecules. Affiliated tissues include bone, and related phenotypes are short neck and macrocephaly

Disease Ontology : 12 An osteochondrodysplasia that is characterized by hypoplastic iliac bones, hypoplastic vertebral bodies, short ribs and broad long bones, prenatally lethal, has material basis in homozygous or compound heterozygous mutation in the SLC35D1 gene on chromosome 1p31.

OMIM : 56 Schneckenbecken dysplasia is a perinatally lethal skeletal dysplasia. The German term 'Schneckenbecken' refers to the distinctive, snail-like appearance of the ilia that results from a medial bone projection from the inner iliac margin. Other hallmarks of the disorder include thoracic hypoplasia, severe flattening of the vertebral bodies, and short, thick long bones (summary by Hiraoka et al., 2007). (269250)

KEGG : 36 Schneckenbecken dysplasia is a severe skeletal dysplasia that leads to perinatal death. The characteristic is the snail-like configuration of the hypoplastic iliac bones. Patients also have limbs with extremely short long bones and flat vertebratal bodies. Loss-of-function mutations in SLC35D1 gene impair the transporter activity of nucleotide sugars in Schneckenbecken dysplasia. The disease is inherited as an autosomal recessive trait.

UniProtKB/Swiss-Prot : 73 Schneckenbecken dysplasia: A rare, lethal autosomal recessive skeletal dysplasia characterized by snail-like configuration of the hypoplastic iliac bone, short-limbed dwarfism, short ribs, and flattened, hypoplastic vertebral bodies. SHNKND is lethal in the neonatal period.

Related Diseases for Schneckenbecken Dysplasia

Diseases related to Schneckenbecken Dysplasia via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 48)
# Related Disease Score Top Affiliating Genes
1 fibrochondrogenesis 29.0 TRIP11 SLC35D1 COL2A1 COL11A2 COL11A1
2 odontochondrodysplasia 27.9 TRIP11 TRAPPC2B SLC35D1 SLC26A2 COL2A1 COL11A2
3 dwarfism 10.4
4 thanatophoric dysplasia, type i 10.3
5 atelosteogenesis 10.2 SLC35D1 SLC26A2
6 congenital disorder of glycosylation, type iim 10.2 SLC35A3 SLC35A1
7 spondyloepimetaphyseal dysplasia, strudwick type 10.2
8 spondylometaphyseal dysplasia, sedaghatian type 10.2
9 opsismodysplasia 10.2
10 skeletal dysplasias 10.2
11 lethal chondrodysplasia 10.2
12 congenital disorder of glycosylation, type iid 10.2 SLC35C1 SLC35A1
13 boomerang dysplasia 10.1 TRIP11 SLC35D1 SLC26A2
14 congenital disorder of glycosylation, type iib 10.1 SLC35C1 SLC35A1
15 congenital disorder of glycosylation, type iia 10.1 SLC35C1 SLC35A1
16 vitreoretinal degeneration 10.1 COL2A1 COL11A1
17 macroglossia 10.0 COL2A1 COL11A1
18 brachyolmia 10.0 SLC26A2 COL2A1
19 achondrogenesis, type ia 10.0 TRIP11 SLC26A2 COL2A1
20 legg-calve-perthes disease 10.0 SLC26A2 COL2A1
21 larsen syndrome 10.0 SLC35D1 SLC26A2 COL2A1
22 diastrophic dysplasia 9.9 SLC26A2 COL2A1
23 congenital disorder of glycosylation, type iif 9.9 SLC35D1 SLC35C1 SLC35A3 SLC35A1
24 congenital disorder of glycosylation, type in 9.8 SLC35D1 SLC35C1 SLC35A3 SLC35A1
25 fibrochondrogenesis 1 9.8 COL11A2 COL11A1
26 multiple epiphyseal dysplasia due to collagen 9 anomaly 9.7 SLC26A2 COL11A2
27 hypochondrogenesis 9.7 TRAPPC2B SLC26A2 COL2A1
28 achondrogenesis, type ii 9.7 COL2A1 COL11A2
29 exostosis 9.7 EXTL3 COL2A1
30 caffey disease 9.6 SLC35D1 COL2A1 COL11A2
31 kohler's disease 9.5 COL2A1 COL11A2 COL11A1
32 vitreous syneresis 9.5 COL2A1 COL11A2 COL11A1
33 retinal perforation 9.5 COL2A1 COL11A2 COL11A1
34 otospondylomegaepiphyseal dysplasia, autosomal dominant 9.5 COL2A1 COL11A2 COL11A1
35 marshall syndrome 9.5 COL2A1 COL11A2 COL11A1
36 otospondylomegaepiphyseal dysplasia, autosomal recessive 9.5 COL2A1 COL11A2 COL11A1
37 cleft soft palate 9.5 COL2A1 COL11A2 COL11A1
38 spinal stenosis 9.5 COL2A1 COL11A2 COL11A1
39 stickler syndrome 9.4 COL2A1 COL11A2 COL11A1
40 achondrogenesis 9.4 TRIP11 TRAPPC2B SLC35D1 SLC26A2 COL2A1
41 kniest dysplasia 9.3 SLC35D1 COL2A1 COL11A2 COL11A1
42 spondyloepiphyseal dysplasia congenita 9.3 TRAPPC2B COL2A1 COL11A2
43 intervertebral disc disease 9.3 COL11A2 COL11A1
44 multiple epiphyseal dysplasia 9.2 SLC26A2 COL2A1 COL11A2 COL11A1
45 orofacial cleft 9.2 COL2A1 COL11A2 COL11A1
46 bone development disease 9.1 TRAPPC2B SLC26A2 COL2A1 COL11A2
47 spondyloepiphyseal dysplasia with congenital joint dislocations 8.8 TRAPPC2B SLC26A2 COL2A1 COL11A2 COL11A1
48 spondyloepimetaphyseal dysplasia, matrilin-3 related 8.8 TRAPPC2B SLC26A2 COL2A1 COL11A2 COL11A1

Graphical network of the top 20 diseases related to Schneckenbecken Dysplasia:



Diseases related to Schneckenbecken Dysplasia

Symptoms & Phenotypes for Schneckenbecken Dysplasia

Human phenotypes related to Schneckenbecken Dysplasia:

58 31 (show all 35)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 short neck 58 31 hallmark (90%) Very frequent (99-80%) HP:0000470
2 macrocephaly 58 31 hallmark (90%) Very frequent (99-80%) HP:0000256
3 lymphedema 58 31 hallmark (90%) Very frequent (99-80%) HP:0001004
4 polyhydramnios 58 31 hallmark (90%) Very frequent (99-80%) HP:0001561
5 malar flattening 58 31 hallmark (90%) Very frequent (99-80%) HP:0000272
6 narrow chest 58 31 hallmark (90%) Very frequent (99-80%) HP:0000774
7 micromelia 58 31 hallmark (90%) Very frequent (99-80%) HP:0002983
8 disproportionate short-limb short stature 58 31 hallmark (90%) Very frequent (99-80%) HP:0008873
9 hypoplastic scapulae 58 31 hallmark (90%) Very frequent (99-80%) HP:0000882
10 hypoplastic vertebral bodies 58 31 hallmark (90%) Very frequent (99-80%) HP:0008479
11 fibular hypoplasia 58 31 hallmark (90%) Very frequent (99-80%) HP:0003038
12 short ribs 58 31 hallmark (90%) Very frequent (99-80%) HP:0000773
13 hypoplastic ilia 58 31 hallmark (90%) Very frequent (99-80%) HP:0000946
14 lateral clavicle hook 58 31 hallmark (90%) Very frequent (99-80%) HP:0000895
15 increased fibular diameter 58 31 hallmark (90%) Very frequent (99-80%) HP:0012107
16 cryptorchidism 58 31 frequent (33%) Frequent (79-30%) HP:0000028
17 hypoplastic toenails 58 31 frequent (33%) Frequent (79-30%) HP:0001800
18 dolichocephaly 58 31 frequent (33%) Frequent (79-30%) HP:0000268
19 abnormal fingernail morphology 31 frequent (33%) HP:0001231
20 cleft palate 58 31 occasional (7.5%) Occasional (29-5%) HP:0000175
21 diaphyseal thickening 58 31 occasional (7.5%) Occasional (29-5%) HP:0005019
22 accelerated skeletal maturation 58 31 occasional (7.5%) Occasional (29-5%) HP:0005616
23 dumbbell-shaped long bone 58 31 occasional (7.5%) Occasional (29-5%) HP:0000947
24 advanced tarsal ossification 58 31 occasional (7.5%) Occasional (29-5%) HP:0008108
25 abnormality of the metaphysis 58 Very frequent (99-80%)
26 ovoid vertebral bodies 31 HP:0003300
27 abnormal form of the vertebral bodies 58 Very frequent (99-80%)
28 abnormality of the fingernails 58 Frequent (79-30%)
29 brachydactyly 31 HP:0001156
30 midface retrusion 31 HP:0011800
31 anterior rib cupping 31 HP:0000907
32 flat acetabular roof 31 HP:0003180
33 metaphyseal irregularity 31 HP:0003025
34 advanced ossification of carpal bones 31 HP:0004233
35 snail-like ilia 31 HP:0031026

Symptoms via clinical synopsis from OMIM:

56
Head And Neck Neck:
short neck

Skeletal Hands:
brachydactyly
precociously ossified carpal bones

Chest External Features:
narrow chest

Head And Neck Face:
flat midface

Head And Neck Head:
large head

Skeletal Spine:
round vertebral bodies
sacral stenosis

Skeletal Feet:
precociously ossified tarsal bones

Head And Neck Mouth:
cleft palate

Prenatal Manifestations Amniotic Fluid:
polyhydramnios

Chest Ribs Sternum Clavicles And Scapulae:
hypoplastic scapulae
short, splayed ribs
hook-shaped clavicles

Skeletal Pelvis:
flat acetabular roofs
snail-shaped ilia

Growth Height:
dwarfism, micromelic

Skeletal Limbs:
dumbbell-shaped short long bones
metaphyseal irregularities

Prenatal Manifestations Delivery:
stillborn or lethal in the neonatal period

Clinical features from OMIM:

269250

GenomeRNAi Phenotypes related to Schneckenbecken Dysplasia according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Resistant to vaccinia virus (VACV-A4L) infection GR00351-A-1 9.17 C1orf141 COL11A1 COL2A1 SLC35A1 SLC35B3 SLC35C1

MGI Mouse Phenotypes related to Schneckenbecken Dysplasia:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 craniofacial MP:0005382 9.5 COL11A1 COL11A2 COL2A1 INPPL1 SLC26A2 SLC35D1
2 mortality/aging MP:0010768 9.32 COL11A1 COL2A1 EXTL3 INPPL1 PAM16 SLC26A2

Drugs & Therapeutics for Schneckenbecken Dysplasia

Search Clinical Trials , NIH Clinical Center for Schneckenbecken Dysplasia

Cochrane evidence based reviews: schneckenbecken dysplasia

Genetic Tests for Schneckenbecken Dysplasia

Genetic tests related to Schneckenbecken Dysplasia:

# Genetic test Affiliating Genes
1 Schneckenbecken Dysplasia 29 SLC35D1

Anatomical Context for Schneckenbecken Dysplasia

MalaCards organs/tissues related to Schneckenbecken Dysplasia:

40
Bone

Publications for Schneckenbecken Dysplasia

Articles related to Schneckenbecken Dysplasia:

(show all 19)
# Title Authors PMID Year
1
Identification of loss-of-function mutations of SLC35D1 in patients with Schneckenbecken dysplasia, but not with other severe spondylodysplastic dysplasias group diseases. 61 6 56
19508970 2009
2
Nucleotide-sugar transporter SLC35D1 is critical to chondroitin sulfate synthesis in cartilage and skeletal development in mouse and human. 6 56 61
17952091 2007
3
Schneckenbecken dysplasia, radiology, and histology. 56 61
11200994 2001
4
A distinct lethal neonatal chondrodysplasia with snail-like pelvis: Schneckenbecken dysplasia. 61 56
3799723 1986
5
"Snail-like pelvis" chondrodysplasia: a further case report. 56
1746621 1991
6
A new category of lethal short-limbed dwarfism. 56
3799722 1986
7
Family with probable achondrogenesis and lipid inclusions in fibroblasts. 56
4120916 1973
8
Familial recurring thanatophoric dwarfism. A case report. 56
4553206 1972
9
Familial thanatophoric dwarfism. 56
4103426 1971
10
A hypomorphic allele of SLC35D1 results in Schneckenbecken-like dysplasia. 61
31423530 2019
11
Molecular asymmetry in the 8-cell stage Xenopus tropicalis embryo described by single blastomere transcript sequencing. 61
26100918 2015
12
A second locus for Schneckenbecken dysplasia identified by a mutation in the gene encoding inositol polyphosphate phosphatase-like 1 (INPPL1). 61
25997753 2015
13
Congenital disorders of glycosylation. Part II. Defects of protein O-glycosylation. 61
24051442 2013
14
[Lethal osteo-chondro-dysplasia: feto-pathological study of 32 cases]. 61
19522446 2009
15
Schneckenbecken dysplasia in fetus: report of four cases. 61
19407457 2009
16
Perinatally lethal, short-limbed dwarfism with distinct features -- Schneckenbecken dysplasia. 61
15386610 2004
17
Metatropic dysplasia lethal variants. 61
14566438 2004
18
Case report 870. Schneckenbecken dysplasia, possibly a new variant. 61
7824994 1994
19
Case report 693: Schneckenbecken dysplasia. 61
1754916 1991

Variations for Schneckenbecken Dysplasia

ClinVar genetic disease variations for Schneckenbecken Dysplasia:

6 (show all 18) ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎
# Gene Name Type Significance ClinVarId dbSNP ID GRCh37 Pos GRCh38 Pos
1 SLC35D1 SLC35D1, 4.9-KB DEL, EX7deletion Pathogenic 1128
2 SLC35D1 SLC35D1, 1-BP DEL, 125Adeletion Pathogenic 1123
3 SLC35D1 NM_015139.3(SLC35D1):c.932G>A (p.Trp311Ter)SNV Pathogenic 1124 rs137853111 1:67474795-67474795 1:67009112-67009112
4 SLC35D1 NM_015139.3(SLC35D1):c.636+1G>TSNV Pathogenic 1125 1:67512947-67512947 1:67047264-67047264
5 SLC35D1 NM_015139.3(SLC35D1):c.319C>T (p.Arg107Ter)SNV Pathogenic 1126 rs267607063 1:67518459-67518459 1:67052776-67052776
6 SLC35D1 NM_015139.3(SLC35D1):c.392+3A>GSNV Pathogenic 1127 1:67517692-67517692 1:67052009-67052009
7 SLC35D1 NM_015139.3(SLC35D1):c.193A>C (p.Thr65Pro)SNV Pathogenic 1129 rs267607062 1:67519504-67519504 1:67053821-67053821
8 SLC35D1 NM_015139.3(SLC35D1):c.814G>A (p.Ala272Thr)SNV Conflicting interpretations of pathogenicity 440280 rs143218310 1:67486114-67486114 1:67020431-67020431
9 SLC35D1 NM_015139.3(SLC35D1):c.40G>A (p.Gly14Arg)SNV Uncertain significance 530927 rs1553268935 1:67519657-67519657 1:67053974-67053974
10 SLC35D1 NM_015139.3(SLC35D1):c.836A>G (p.Tyr279Cys)SNV Uncertain significance 662949 1:67486092-67486092 1:67020409-67020409
11 SLC35D1 NM_015139.3(SLC35D1):c.508A>G (p.Ile170Val)SNV Uncertain significance 661129 1:67515490-67515490 1:67049807-67049807
12 SLC35D1 NM_015139.3(SLC35D1):c.301C>T (p.Leu101Phe)SNV Uncertain significance 642827 1:67518477-67518477 1:67052794-67052794
13 SLC35D1 NM_015139.3(SLC35D1):c.712A>G (p.Thr238Ala)SNV Uncertain significance 856165 1:67507936-67507936 1:67042253-67042253
14 SLC35D1 NM_015139.3(SLC35D1):c.526G>T (p.Ala176Ser)SNV Uncertain significance 845566 1:67515472-67515472 1:67049789-67049789
15 SLC35D1 NM_015139.3(SLC35D1):c.813C>T (p.Tyr271=)SNV Likely benign 734963 1:67486115-67486115 1:67020432-67020432
16 SLC35D1 NM_015139.3(SLC35D1):c.159C>G (p.Ser53=)SNV Likely benign 752714 1:67519538-67519538 1:67053855-67053855
17 SLC35D1 NM_015139.3(SLC35D1):c.660C>G (p.Leu220=)SNV Likely benign 811263 1:67507988-67507988 1:67042305-67042305
18 SLC35D1 NM_015139.3(SLC35D1):c.730-8T>CSNV Benign 709549 1:67487293-67487293 1:67021610-67021610

Expression for Schneckenbecken Dysplasia

Search GEO for disease gene expression data for Schneckenbecken Dysplasia.

Pathways for Schneckenbecken Dysplasia

Pathways related to Schneckenbecken Dysplasia according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.56 SLC35D1 SLC35C1 SLC35B3 SLC35A3 SLC35A1 SLC26A2

GO Terms for Schneckenbecken Dysplasia

Cellular components related to Schneckenbecken Dysplasia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 Golgi membrane GO:0000139 9.72 TRIP11 SLC35C1 SLC35B3 SLC35A3 SLC35A1
2 collagen trimer GO:0005581 9.43 COL2A1 COL11A2 COL11A1
3 integral component of Golgi membrane GO:0030173 9.33 SLC35B3 SLC35A3 SLC35A1
4 Golgi apparatus GO:0005794 9.23 TRIP11 SLC35D1 SLC35C1 SLC35B3 SLC35A3 SLC35A1
5 collagen type XI trimer GO:0005592 8.96 COL11A2 COL11A1

Biological processes related to Schneckenbecken Dysplasia according to GeneCards Suite gene sharing:

(show all 14)
# Name GO ID Score Top Affiliating Genes
1 sensory perception of sound GO:0007605 9.7 COL2A1 COL11A2 COL11A1
2 collagen fibril organization GO:0030199 9.61 COL2A1 COL11A2 COL11A1
3 ossification GO:0001503 9.56 SLC26A2 PAM16 COL2A1 COL11A1
4 endochondral ossification GO:0001958 9.54 INPPL1 COL2A1
5 tissue homeostasis GO:0001894 9.51 COL2A1 COL11A2
6 skeletal system morphogenesis GO:0048705 9.5 COL2A1 COL11A2 COL11A1
7 cartilage condensation GO:0001502 9.49 COL2A1 COL11A1
8 proteoglycan metabolic process GO:0006029 9.48 COL2A1 COL11A1
9 3'-phosphoadenosine 5'-phosphosulfate biosynthetic process GO:0050428 9.46 SLC35B3 SLC26A2
10 cartilage development GO:0051216 9.46 TRIP11 COL2A1 COL11A2 COL11A1
11 UDP-N-acetylglucosamine transmembrane transport GO:1990569 9.4 SLC35D1 SLC35A3
12 UDP-galactose transmembrane transport GO:0072334 9.37 SLC35A3 SLC35A1
13 pyrimidine nucleotide-sugar transmembrane transport GO:0090481 9.13 SLC35D1 SLC35A3 SLC35A1
14 carbohydrate transport GO:0008643 8.92 SLC35D1 SLC35C1 SLC35A3 SLC35A1

Molecular functions related to Schneckenbecken Dysplasia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 transmembrane transporter activity GO:0022857 9.58 SLC35D1 SLC35C1 SLC35B3
2 extracellular matrix structural constituent GO:0005201 9.54 COL2A1 COL11A2 COL11A1
3 antiporter activity GO:0015297 9.5 SLC35D1 SLC35C1 SLC35A1
4 UDP-N-acetylglucosamine transmembrane transporter activity GO:0005462 9.26 SLC35D1 SLC35A3
5 UDP-galactose transmembrane transporter activity GO:0005459 9.16 SLC35A3 SLC35A1
6 extracellular matrix structural constituent conferring tensile strength GO:0030020 9.13 COL2A1 COL11A2 COL11A1
7 pyrimidine nucleotide-sugar transmembrane transporter activity GO:0015165 8.8 SLC35D1 SLC35A3 SLC35A1

Sources for Schneckenbecken Dysplasia

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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