Sheffield Diagnostic Genetics Service 

 

Address

Sheffield Children’s NHS Foundation Trust

Western Bank

Sheffield

S10 2TH, UK

Contact 1

Mrs Joanne Martindale (Neurodegenerative disorders)

Contact 2

Mr Richard Kirk (Inborn Errors of Metabolism)

Contact 3

Dr Anne Goodeve (Haematology)

Contact 4

Ms Rebecca Pollitt (Connective Tissue Disorders)

Contact 5

Dr Gill Wilson (Oncology)

Telephone

+44 (0)114 271 7003

Fax:

+44 (0)114 275 0629

Email

SDGS@sch.nhs.uk

Home page

http://www.sheffieldchildrens.nhs.uk/SDGS.htm

 

 

 

 

 

Services offered: 27/10/2011 NJB 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

Bruck Syndrome (PLOD2)

609220, 601865

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, SCA17

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 (Classical COL5A1 and COL5A2)

130000, 130010

N/A

Ehlers Danlos syndrome type I (COL5A1 - Null allele testing)

130000, 130010

N/A

Ehlers-Danlos syndrome, Type IV

(Vascular COL3A1)

130050

N/A

Ehlers Danlos syndrome type VI (kyphoscoliotic PLOD1)

225400

N/A

Ehlers Danlos syndrome type VII (Athrochalasic COL1A1, COL1A2)

130060

N/A

Episodic ataxia type 1

160120

 

Episodic ataxia type 2

108500

 

Factor XIII Deficiency (F13A1, F13B)

613225, 134570, 613235, 134570

N/A

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

Fanconi Anaemia

(FANCG gene)

602956, 614082

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

 

Hereditary Spastic Paraplegia 31 (REEP1)

610250

 

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

 

166220

Approved

 

Osteogenesis Imperfecta Autosomal Recessive type IIB (CRTAP)

610854, 610682

 

N/A

 

Osteogenesis Imperfecta Autosomal Recessive type XI (LEPRE1)

 

610339, 610915

 

N/A

 

Osteogenesis Imperfecta

autosomal recessive type VII

Peptidylprolyl Isomerase B (PPIB)

123841, 259440

N/A

Osteogenesis Imperfecta Autosomal Recessive type X (SERPINH1)

600943, 613848

N/A

Osteogenesis Imperfecta Autosomal Recessive type XII (SERPINF1)

172860, 613982

N/A

Osteogenesis Imperfecta Autosomal Recessive type VI (FKBP10)

607063, 610698

N/A

Osteogenesis Imperfecta Autosomal Recessive type XI (SP7)

606633, 613849

N/A

Osteoporosis/osteoporosis pseudoglioma (OPPG) -LRP5 gene

603506

N/A

Peroxisome Biogenesis Disorders (PBD, Zellweger Syndrome spectrum, ZSS, Infantile Refsum Disease, neonatal adrenoleukodystrophy, NALD, PEX1, PEX6, PEX10, PEX12, PEX26)

214100

 

N/A

 

PIK3CA testing for response to anti-EGFR treatment

 

N/A

 

N/A

 

Polycystic Kidney Disease, Autosomal dominant (PKD1,  PKD2)

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

Sickle cell disease (newborn screening - transfused babies)

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,  ASS1, ARG1)

311250, 237300, 237310, 207900, 215700, 207800

Approved

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

N/A

Warfarin resistance

(VKORC1 gene analysis)

122700, 608547

N/A

Wilms Tumour, Frasier syndrome, Denys Drash syndrome, (Wilms Tumour Suppressor)

194080, 607102

N/A

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.