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Wisconsin Newborn Screening Laboratory Newsletter: 2005 Testing Summary

Wisconsin Newborn Screening Laboratory Newsletter:

2005 Testing Summary

Newborn Screening Newsletter               No. 62

 

May 2006               

Printable Version (PDF)

2005 Testing Summary   

This newsletter summarizes the newborn screening findings for the year 2005. The number of newborns screened (which reflects the birth rate) increased slightly (~1,000) over 2004. The distribution of specimen collection ages has remained constant since 2000. The number of repeat specimens has leveled off -- possibly due to full implementation of the low birth weight re-testing guidelines. Although the number of hypothyroid (43) babies detected in 2005 decreased substantially from 2004 (57), the 2005 number was still the second highest since screening began in 1978. This number includes 9 NICU babies that were identified on subsequent repeat screens after the initial screen was normal. Two very rare disorders were identified in 2005: Argininosuccinic acidemia (ASA) and beta-ketothiolase deficiency ( b -KT). One of the two Maple Syrup Urine Disease cases detected was done by mutation analysis since it was known that both parents were carriers. The baby was identified as having MSUD within 10 hours of birth.

 

Cumulative and 2005 Newborn Screening Statistics

 

GENERAL STATISTICS (Jan - Dec 2005)

 

Number of infants screened:     69,566

Number of repeat screens:     5,306

Total number of samples screened:   74,872

 

AGE AT TIME OF SAMPLE COLLECTION

 

Age (in Hours) at time

of initial Collection

Number of infants

Jan - Dec 2005

% of total Cumulative %

0 - 11

12 - 23

24 - 35

36 - 47

48 - 71

72 - 95

96+

Unknown

413

1,219

28,205

24,748

10,856

1,814

2,182

39

0.6% 0.6%

1.8.% 2.4%

40.6% 43.0%

35.6% 78.6%

15.6 % 94.2%

2.6% 96.8%

3.1% 99.9%

0.1% 100.0%

Mean collection age: 39.2 hrs (39.0 in 2004) Median collection age: 37.5 hrs (37.2 hrs in 2004)

 

ENDOCRINE SCREENING

 

Infants positive for:

Jan - dec 2005

May 1978-dec 2005

 

Hypothyroidism

Congenital Adrenal Hyperplasia *

43

4

639 (23/yr)

59 (5/yr)

* Screening began in March 1993

CYSTIC FIBROSIS SCREENING

 

Abnormal Results Reported

Jan - Dec 2005

July 1994 - Dec 2005

Number of Abnormals Reported *

With One mutant allele

With Two mutant alleles

With IRT = 170 ng/mL (no alleles)

Confirmed Positive (i.e. with CF) by sweat test

With One mutant allele

With Two mutant alleles

With No mutant alleles

IRT = 170 ng/mL

IRT < 170 ng/mL

Double Heterozygotes - Non-classical CF

D F508/R117H

D F508/I148T

R117H/R117H

R117H/I148T

2789+5G>A/R17OH

R117H/N1303K

D F508/I506V

192

12

25

 

9

12

 

0

0

 

5

0

0

0

0

1

1

1,928 (175/yr)

111 (10/yr)

402 (36/yr)

 

95 (8/yr)

92 (8/yr)

 

5

5

 

20

1

1

1

1

1

1

  * Screening for 25 mutant alleles began on March 1, 2002

 

METABOLIC SCREENING

 

Infants positive for:

Jan - dec 2005

May 1978-Dec 2005

Phenylketonuria (PKU)

Galactosemia

Biotinidase Deficiency *

Fatty Acid Oxidation **

MCAD

SCAD

VLCAD

GA-II

CPTII

Organic Acidemia **

MMA

PA

3-MCC

IVA

GA-I

2MBCD

b -KT

Aminoacidopathies***

Citrulinemia (type II)

Tyrosinemia (type II)

Homocystinuria

Maple Syrup Urine Disease

Argininosuccinic Acidemia

5

2

0

 

1

0

0

0

0

0

1

1

1

0

4

1

0

0

0

2

1

174 (6/yr)

35 (1.5/yr)

9 (1/yr)

 

17 (4/yr)

17 (4/yr)

1

2

1

9 (7 mild/benign)

3

15 (3/yr)

3

2

26 (4/yr)

1

1

1

1

2

1

* Screening began in February 1991

** Screening began in April, 2000

*** Screening began in February 2003

HEMOGLOBINOPATHY SCREENING

 

Infants positive for:

Jan - Dec 2005

nov 1988 - Dec 2005

Sickle Cell Disease (FS)

Sickle Hemoglobin C Disease (FSC)

Hemoglobin C Disease (FC)

Sickle Cell Trait (FAS)

Hemoglobin C Trait (FAC)

S-beta Thalassemia (FSA) *

C-beta Thalassemia (FCA) *

Hemoglobin E Disease

Hemoglobin CE Disease

20

1

2

697

203

2

1

2

0

203 (12/yr)

110 (6/yr)

21 (1/yr)

9,285 (572/yr)

2,759 (170/yr)

31 (2/yr)

10 (<1/yr)

56 (3/yr )

1

* Reporting of S-beta & C-beta Thalassemia began in 1992

 

Other Issues

 

•  The US Department of Health and Human Services (HHS) has finalized the recommended 29 core disorders that should be included in all state screening programs. In addition to the core panel, there is another list of 25 secondary disorders that HHS recommends that state programs "consider" reporting if the technology used can identify them. The Wisconsin program screens for all 29 core disorders and 19 of the secondary disorders. The states newborn screening advisory group has not recommended the additional 6 secondary disorders due to the timing of specimen collection and treatment issues. Therefore the current number of disorders screened for in Wisconsin is 48. A list of the disorders screened for can be seen by accessing our website: www.slh.wisc.edu/newborn

 

•  The increase in the number of disorders screened for includes several that can be very life-threatening in the first few days of life. We ask each of you to review your shipping process to minimize the time specimens are kept in your facility. Items that should be addressed include time of courier pickup, staff coverage for illness and vacations, use of private courier vs UPS (state financed), etc. Minimizing transportation delays may result in improved outcomes for babies with life-threatening disorders.

 

•  The newborn screening laboratory receives many inquires (phone and fax) for results. To expedite the retrieval of the information, the following demographic information is required:


•  Mother's Name           •  Birth date

•  Baby's name                •  Hospital of birth

In addition to requests for results, requests are made by the specimen submitter to correct information after the report from our lab has been received. Below are some guidelines for requesting corrections. Please share this information with all hospital staff that may make requests for corrections.

 

•  All correction requests must be in writing (fax is ok). Be sure to include the information noted above.

•  Indicate if a corrected report is needed and if so should it be faxed or mailed.

•  Acceptable corrections

•  Obvious typographical errors
•  Addition of mother's first name
•  Correction to mother's first or last name
•  Correction of birth day and/or time*
•  Correction of specimen collection day and/or time*
•  Correction of birth weight*

•  Corrections that will NOT be made

•  Addition of baby's first name
•  Change the baby's last name
•  Change or addition of baby's ID number

 

*Changing date/time/weight may result in a different interpretation of the results.

 

Newborn Screening in Wisconsin - Thanks to YOU it's working!!

 

Also, best wishes and a special thank you to all of our Wisconsin colleagues from all of us in the Newborn Screening Program (Wisconsin Division of Public Health and the State Laboratory of Hygiene). At the end of the day, every day, the babies in Wisconsin benefit from all of our hard work. Certainly no one deserves more credit than YOU--the staff in the newborn units of every hospital, the NICUs and the physicians and nurses who receive the reports and follow-up with all of the babies. Special thanks to the midwives of Wisconsin for their efforts. Because of the dedication of all of you, in 2005 almost 100 babies (and their parents) have a very good chance to look forward to a nearly normal life as opposed to one of mental retardation, neurological impairment, or even death. Don't ever think that your efforts are not worth it -- just ask one of 100 sets of parents.

 

Cost of Newborn Screening - the best efforts from all of us;

Favorable newborn screening outcomes - PRICELESS!

 

Sincerely,

Ronald H. Laessig, PhD.

Director, State Laboratory of Hygiene

Professor Population Health Sciences

Professor Pathology and Laboratory Medicine

Murray L. Katcher, MD, PhD.

Chief Medical Officer for Family and   

    Community Health

State Maternal and Child Health Director

Wisconsin Division of Public Health