Molecular analysis of mutations by NGS in genes that cause congenital metabolic disorders.
The neonatal screening programs implemented in Spain cover the screening of a variable number of genetic diseases, ranging from 3 to more than 20 depending on the autonomic community where the test is performed. On July 23, 2013, the Interterritorial Council of the National Health System approved the creation of a common portfolio of neonatal screening that includes seven tests for early detection of the following diseases in newborns in Spain: congenital hypothyroidism, phenylketonuria, cystic fibrosis, medium chain acyl dehydrogenated coenzyme A deficiency (MCADD), long chain 3-hydroxyl acyl-CoA dehydrogenase deficiency (LCHADD), glutaric acidemia type 1 and sickle-cell anemia.
These programs are intended to identify congenital metabolic disorders before they manifest with clinical symptoms, thus avoiding or minimizing their possible consequences (mental retardation, developmental delay or even premature death of the neonate). In case of a positive results the first biochemical heel test, the neonate will be referred to the specialist for additional tests and, if necessary, a genetic study is requested for definite confirmation of the diagnosis.
One single comprehensive genetic test
The qSeq Easy® Neonatal service is based on Next Generation Sequencing (NGS) and developed by qGenomics in collaboration with researchers from different reference centers in genomics and the specific diseases. In a single experiment, the totality of coding regions (exons), intron / exon boundaries, and deep intron regions of more than 70 genes that are known to associated with this type of diseases are analyzed. This approach allows to have one sole procedure for the analysis of mutations in all these genes, reducing the cost per analysis and response time.
This assay allows the identification of any type of mutation present at any location of the gene with high reliability.
Turn-around time: 30 working days from sample reception.
Gene
Associated phenotype
OMIM dissease ID
ACADM
Acyl-CoA dehydrogenase, medium chain deficiency of
201450
ACADS
Short-chain acyl-CoA dehydrogenase deficiency
201470
ACADVL
VLCAD deficiency
201475
ACAT1
Alpha-methylacetoacetic aciduria
203750
AHCY
Hypermethioninemia
613752
ALDH4A1
Hyperprolinemia
239510
ARG1
Arginase deficiency
207800
ASL
Argininosuccinate Lyase Deficiency
207900
ASS1
Citrullinemia
215700
BCKDHA
Maple syrup urine disease type Ia
248600
BCKDHB
Maple syrup urine disease type Ib
248600
BTD
Biotinidase deficiency
253260
CBS
Homocystinuria B6-responsive and nonresponsive types
236200
CFTR
Cystic Fibrosis
219700
CPT1A
CPT I deficiency, hepatic
255120
CPT2
CPT II deficiency, lethal neonatal
608836
CTH
Cystathioninuria
219500
CYP21A2
Adrenal hyperplasia due to 21-hydroxylase deficiency
201910
DBT
Maple syrup urine disease type II
248600
DLD
Maple syrup urine disease type III
248600
DUOX2
Thryoid dyshormonogenesis 6
607200
DUOXA2
Thyroid dyshormonogenesis 5
274900
ETFA
Glutaric acidemia IIA
231680
ETFB
Glutaric acidemia IIB
231680
ETFDH
Glutaric acidemia IIC
231680
FAH
Tyrosinemia type I
276700
G6PD
Glucose-6-phosphate dehydrogenase deficiency
305900
GALE
Galactose epimerase deficiency
230350
GALK1
Galactokinase deficiency with cataracts
230200
GALT
Galactosemia
230400
GCDH
Glutaric aciduria, type I
231670
GJB2
Deafness Autosomal Dominant 3A
601544
GLIS3
Diabetes mellitus, neonatal, with congenital hypothyroidism
610199
GNAS
Pseudopseudohypoparathyroidism
612463
GNMT
Hypermethioninemia
606664
HADHA
LCHAD deficiency
609016
HAL
Histidinemia
235800
HBA1,HBA2
Alpha Thalassemias
604131
HBB
Beta Thalassemias
613985
HGD
Alkaptonuria
203500
HMGCL
HMG-CoA lyase deficiency
246450
IVD
Isovaleric acidemia
243500
IYD
Thyroid dyshormonogenesis 4
274800
MAT1A
Hypermethioninemia
250850
MCCC1
3-methylcrotonyl-CoA carboxylase deficiency
210200
MCCC2
3-methylcrotonyl-CoA carboxylase deficiency
210210
MCEE
Vitamin B12-unresponsive methylmalonic acidemia
251120
MMAA
Methylmalonic aciduria, vitamin B12-responsive
251100
MMAB
Methylmalonic aciduria, vitamin B12-responsive, due to defect in synthesis of adenosylcobalamin, cblB complementation type
251110
MMACHC
Methylmalonic aciduria and homocystinuria, cblC type
277400
MMADHC
Methylmalonic aciduria and homocystinuria, cblC type
277410
MUT
Methylmalonic aciduria, mut(0) type
251000
MVK
Mevalonate kinase deficiency
260920
NKX2-1
Choreoathetosis, hypothyroidism, and neonatal respiratory distress
610978
NKX2-5
Hypothyroidism, congenital nongoitrous 5
225250
PAH
Phenylketonuria
261600
PAX8
Hypothyroidism, congenital, due to thyroid dysgenesis or hypoplasia
Identify the adequate test from our service catalogue.
Prepare the test application form and fill in all the information available both for the sample and the clinical record to guarantee highest quality of test results and shortest response time.
It is crucial to correctly identify the sample with patient name or identifier and date of birth.
Print the completed application form and sign it.
Collect the required sample quantity.
Properly prepare the sample shipment along with the application form.
Alternatively, the order can be processed conveniently via our online order platform.
Call us at +34 932.301.270 and we will take care of a smooth sample pick-up and shipping.
How to prepare your sample for shipping?
Blood-EDTA, purified DNA, saliva, blood on paper, cord blood, biopsy, … are stable at room temperature for 3 or 4 days. Samples can be sent, adequately protected, by ordinary mail or courier.
Amniotic fluid (a minimum of 10 ml collected in a sterile falcon test tube). Can be sent at room temperature and low temperatures should be avoided. Samples should reach our laboratory in no more than 24 hours.
Chorionic villi samples must travel in the same way, in a tube with conical bottom FILLED TO CAPACITY with PBS or culture medium.
Cultured cells should be shipped at room temperature, in a culture flask filled to capacity with culture medium.
Blood for cfDNA analysis: must be shipped at room temperature.
Para solicitar un test, simplemente tiene que rellenar el formulario de petición de servicio que se puede descargar en formato PDF editable en esta página, y hacérnoslo llegar junto a la muestra a analizar:
Una vez cumplimentados, háznoslo llegar a la dirección:
Quantitative Genomic Medicine Laboratories, SL. C/. Joan XXIII, 10, Planta 208950 Esplugues de Llobregat. Barcelona - España
No obstante, si tiene dudas sobre la prueba que más se ajusta a sus necesidades o sobre el procedimiento, póngase en contacto con nosotros a través del siguiente número de teléfono: