Sheffield Diagnostic Genetics Service 

 

Address

Sheffield Children’s NHS Foundation Trust

Western Bank

Sheffield

S10 2TH, UK

Contact 1

Dr Ann Dalton

Contact 2

Mrs Joanne Martindale

Contact 3

Dr Anne Goodeve

Telephone 1

+44 (0)114 271 7004

Telephone 2

+44 (0)114 271 7003

Telephone 3

+44 (0)114 271 7005

Fax:

 +44 (0)114 275 0629

Email 1

Molecular.Genetics@sch.nhs.uk

Email 2

 

Email 3

 

Home page

 

 

Services offered: 19/10/09 MP update

Disease

OMIM

UKGTN service*

(Approved, Pending, N/A)

Acute Lymphoblastic Leukaemia (ALL)/BCR-ABL

151410, 189980

 N/A

 

Acute Myeloid Leukaemia /AML/ AML-M2

601626, 133435

 N/A

 

Acute Promyelocytic Leukaemia (APL)/AML M3/AML-15/MRD monitoring

102578, 180240

N/A

 

Amyotrophic Lateral Sclerosis 1 (SOD1)

105400

N/A

Adrenoleukodystrophy, X-linked

 (X-ALD)

300100

N/A

Alpha-thalassaemia

604131, 141800, 141850

N/A

Androgen Insensitivity Syndrome (CAIS, PAIS, MAIS, androgen receptor, AR)

 

300068

 

Approved

 

Antithrombin deficiency (antithrombin III deficiency, SERPINC1)

 

107300

 

N/A

ApoE (E2 allele for Hyperlipidemia/Hyperlipoproteinaemia Type III only)

 

 107741

 

Approved

 

Ataxia telengiectasia (ATM)

208900

N/A

BCR-ABL

 

151410,189980,#608232

 

N/A

 

BCR-ABLQ (Quantitative)

 

 

151410,189980,#608232

 

N/A

 

Beta-thalassaemia

604131, 141900

N/A

BRAF testing as HNPCC pre-screen

N/A

 

N/A

 

BRAF testing for response to anti-EGFR treatment (p.Val600Glu mutation)

 

 N/A

N/A

Carnitine Acylcarnitine Translocase (CACT) deficiency

212138

 

Approved

 

c-KIT (D816V) mutation

 N/A

 

 N/A

 

Carnitine Palmitoyl Transferase Type II deficiency (CPT2 deficiency)

 

255110, 600649, 608836

 

Approved

 

CBCL/CTCL / Skin lymphoma/ mycosis fungoides (IgH or T cell gene rearrangements)

 N/A

 

 N/A

 

Cerebral AD Arteriopathy with Subcortical Infarcts & Leukoencephalopathy  (CADASIL)

125310

 

 N/A

 

Cerebellar Ataxias, DRPLA, Friedreich ataxia, SCA1, SCA2, SCA3, SCA6, SCA7

 

125370, 229300, 164400, 183090, 109150, 183086, 164500

 

Approved

 

Chimerism/pre or post bone marrow/stem cell transplant (BMT/SCT)/donor for BMT/SCT/matched unrelated donor (MUD)

 N/A

N/A

Chronic Myeloid Leukaemia (CML)/BCR-ABL

608232, 151410, 189980

N/A

CPEO

 

157640

 

Approved

 

Crigler-Najjar Syndrome (CNI, CNII, UGT1A1)

 

218800, 606785

 

N/A

 

Cystic Fibrosis (CF,CFTR)

 

219700

 

Approved

 

Diamond Blackfan Anaemia

105650

 

Dopa-responsive dystonia (Segawa syndrome), dominant

GCH1 deficient

233910

 

Approved

 

Dopa-responsive dystonia, recessive

Tyrosine hydroxylase deficient

605407

 

Approved

 

Dystonia 1 or Idiopathic Torsion Dystonia, dominant

128100

 

Approved

 

EGFR gene analysis

 

 

Ehlers Danlos syndrome type I (COL5A1 and COL5A2)

130000,130010

N/A

Ehlers-Danlos syndrome, Type IV

 

130050

 

N/A

 

Ehlers Danlos syndrome type VI (kyphoscoliotic PLOD1)

225400

N/A

Episodic ataxia type 1

160120

 

Episodic ataxia type 2

108500

 

Familial Adenomatous Polyposis, Gardner syndrome, Turcots syndrome, Hereditary Desmoid disease (APC)

 

175100, 175100, 276300, 135290

 

Approved

 

Familial hemiplegic migraine type 1

141500

 

Familial motor neurone disease / amyotrophic lateral sclerosis (ALS)

SOD1 and TARDBP genes

147450, 605078

 

Fanconi Anaemia

(FANCC gene)

227650,227645

N/A

Fanconi Anaemia

(FANCA gene)

227650, 607139

N/A

Fanconi Anaemia

(FANCD1 gene)

227650, 605724

N/A

Fragile X syndrome

 

309550

 

Approved

 

Fructose-1,6-Bisphosphatase deficiency (FBP1)

 

229700

 

N/A

 

Fumarate Hydratase deficiency (FH; Fumarase deficiency)

 

 

606812

 

 

Approved

 

 

Gilbert Syndrome (UGT1A1, hyperbilirubinaemia)

 

 

143500

 

 

Approved

 

Glanzmann Thrombasthenia

(GP2B and GP3A genes)

273800, 187800

N/A

Glutaric Acidaemia Type I (GA1, glutaric aciduria type 1, GCDH)

 

231670

 

Approved

 

Glycogen Storage Disease Type 0 (GSD0, GYS2, glycogen synthase deficiency)

 

240600

 

N/A

 

Glycogen Storage Disease Type Ia (GSD1a, G6PC, glucose-6-phophatase deficiency, Von Gierke Disease)

 

232200

 

Approved

 

Glycogen Storage Disease Type I non-a (GSD1b, SLC37A4, G6PT, glucose-6-phosphate transporter deficiency)

 

602671

 

Approved

 

Glycogen Storage Disease Type II (GSD2, GAA, acid maltase deficiency, acid alpha-glucosidase deficiency, Pompe Disease)

 

232300

 

N/A

 

Glycogen Storage Disease Type III (GSD3, AGL, glycogen debrancher deficiency, Forbes/Cori Disease)

 

232400

 

N/A

 

Glycogen Storage Disease Type IV (GSD4, GBE1, glycogen brancher deficiency, Anderson Disease, adult polyglucosan body disease)

 

232500 & 263570

 

N/A

 

Glycogen Storage Disease Type V (GSD5, PYGM, muscle glycogen phosphorylase deficiency, McArdle Disease)

 

232600

 

N/A

 

Glycogen Storage Disease Type VI (GSD6, PYGL, liver glycogen phosphorylase deficiency, Hers Disease)

 

232700

 

N/A

 

Glycogen Storage Disease Type VII (GSD7, PFKM, phosphofructokinase deficiency, Tarui Disease)

 

232800

 

N/A

 

Glycogen Storage Disease Type IX (GSD9, PHKA2, PHKB, PHKG2, glycogen phosphorylase kinase deficiency)

 

306000, 172490, 172470

 

N/A

 

Haemochromatosis (HFE)

 

235200

 

N/A

 

Haemophilia A (F8)

 

306700

 

Approved

 

Haemophilia B (F9)

 

306900

 

Approved

 

(Haemophilia C) Factor XI deficiency (F11)

 

264900

 

N/A

 

Hereditary Leiomyomatosis with renal cell carcinoma (HLRCC/MCUL)

(FH gene)

605839

 

Pending

 

Hereditary Non Polyposis Carcinoma of Colon (HNPCC) MLH1/MSH2/MSH6 genes

120435, 609310

 

Approved

 

Hereditary Spastic Paraplegia 3 (ATL1)

 

182600

 

N/A

 

Hereditary Spastic Paraplegia 4 (SPAST)

 

182601

 

Approved

 

Huntington Disease

 

143100

 

Approved

 

Immunoglobulin heavy chain gene rearrangement (IgH)/B cell lymphocytosis/B cell gene rearrangement

147100, 186970, 186000

 

N/A

 

JAK2 (V617F mutation)

147796

N/A

 

JAK2 Exon 12 mutation screening

147796

N/A

 

Kearns-Sayre syndrome

 

530000

 

Approved

 

KRAS ( v-Ki-ras2 Kirsten rat sarcoma viral oncogene homolog )

codons 12, 13 and 61 only

N/A

 

N/A

 

Leber Hereditary Optic Neuropathy (LHON)

535000

 

Approved

 

Leigh syndrome

 

256000

 

Approved

 

Long Chain Hydroxyl-CoA-Dehydrogenase deficiency (LCHAD deficiency, HADHA)

 

609016

 

Approved

 

Medium Chain Acyl-CoA-Dehydrogenase deficiency (MCAD deficiency, ACADM)

 

201450

 

Approved

 

MELAS

 

540000

 

Approved

 

MERRF

 

545000

 

Approved

 

Metabolic Myopathy screen (CPT2, PYGM common mutations)

255110, 232600

N/A

Methylentetrahydrofolate Reductase deficiency (MTHFR deficiency, thermolabile variant)

 

236250

 

Approved

 

Microsatellite Instability Analysis (MSI)

 

 

Multiple Colorectal Adenomas (MYH gene)

 

 

Multiple Endocrine Neoplasia Type 1

(MEN1 gene)

131100

 

Approved

Multiple Endocrine Neoplasia Type 2 and Familial Medullary Tyroid Carcinoma (RET gene)

171400, 162300, 155240

 

Approved

Multiple Cutaneous & Uterine Myomatoma (MCUL)

150800

 

Pending

 

Myeloproliferative disorder/essential thrombocythaemia(ET)/polycythaemia rubra vera (PRV)/ myelofibrosis (MF) - JAK2

263300, 254450, 187950

N/A

Myoclonus-dystonia syndrome (SGCE)

604149

 

Myotonic dystrophy 1

 

160900

 

Approved

 

Osteogenesis imperfecta type I

 

166200

 

N/A

 

Osteogenesis imperfecta type II

 

166210

 

Approved

 

Osteogenesis imperfecta type III

 

259420

 

Approved

 

Osteogenesis imperfecta type IV

 

Osteogenesis imperfecta type IV

 

Approved

 

Osteogenesis Imperfecta Autosomal Recessive (CRTAP)

610854, 610682

 

N/A

 

Osteogenesis Imperfecta Autosomal Recessive (LEPRE1)

 

610915

 

N/A

 

Osteogenesis Imperfecta

autosomal recessive

Peptidylprolyl Isomerase B (PPIB)

N/A

N/A

Osteoporosis/osteoporosis pseudoglioma (OPPG) -LRP5 gene

259770

N/A

Peroxisome Biogenesis Disorders (PBD, Zellweger Syndrome spectrum, ZSS, Infantile Refsum Disease, neonatal adrenoleukodystrophy, NALD, PEX1, PEX6, PEX12, PEX26)

 

214100

 

N/A

 

PIK3CA testing for response to anti-EGFR treatment

 

N/A

 

N/A

 

Polycystic Kidney Disease, Autosomal dominant

173900, 601313, 173910

Approved

Protein C deficiency (PROC)

 

176860

 

N/A

 

Protein S deficiency (PROS1)

 

176880

 

N/A

 

Prothrombin (3' non 20210G>A prothrombin variants)

176930

N/A

Pseudoxanthoma Elasticum (PXE)

 

264800

 

N/A

 

Quantitative BCR-ABL (MRD)

151410, 189980

N/A

 

Sickle cell disease

603903

Approved

Spinal and Bulbar Muscular Atrophy, (Kennedy disease), X-linked

313200

 

Approved

 

Spinal Muscular Atrophy, 5q-linked

253300

 

Approved

 

T cell lymphocytosis / T cell receptor gene rearrangements (TCR)

N/A

 

N/A

 

Trimethylaminuria (TMAU, FMO3, flavin-containing mono-oxygenase 3 deficiency, fish odour syndrome)

 

602079

 

N/A

 

UKALL2003/UKALLR3 MRD TRIALS

N/A

 

N/A

 

Urea Cycle Disorders

(OTC, CPS1, NAGS, ASL, ASS)

311250, 237300, 237310, 207900, 215700

N/A

Wilms Tumour,Denys Drash syndrome, Frasier syndrome,Mesangial Sclerosis (WT1)

 

194070,194080, 256370, 256370

 

Approved

 

Very Long Chain Acyl-CoA Dehydrogenase deficiency (VLCAD deficiency, ACADVL)

 

201475

 

N/A

 

von Willebrand Disease types 1, 2A, 2B, 2M, 2N and 3

193400,  #277480

 

N/A

Platelet-type (pseudo) von Willebrand disease (GP1BA)

177820

 

Warfarin resistance

(VKORC1 gene analysis)

122700, 608547

 

Wilson Disease (ATP7B)

 

277900

 

Approved

 

*Services offered outside main commissioning area geographical

boundaries under the UK specialist Genetic Testing Network - enquire to the laboratory on costs.