We're unable to sign you in at this time. Please try again in a few minutes.
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
Article |

Hereditary Tyrosinemia and Tyrosyluria in a French Canadian Geographic Isolate

Am J Dis Child. 1967;113(1):41-46. doi:10.1001/archpedi.1967.02090160091008.
Text Size: A A A
Published online


FOUR patients with "tyrosinosis" were brought to our attention and investigated during the past year. All of them came from the secluded Lac St Jean-Saguenay area of Quebec province (Fig 1), with a population of 500,000. The principle immigration to this region occurred 150 years ago, and relatively little internal intermingling from adjacent communities has occurred since. The ancestry of the majority of this population is reputed to trace back to 21 original French-Canadian settlers. During the past decade, 41 patients with "tyrosinosis" (including the present four patients) have been discovered in 29 families who live in the region. Five of the infants are still living and have proven "tyrosinosis;" 35 infants have died with its acute manifestations;1,2 and one has died of encephalitis. The current birth rate in this region is approximately 5%; the local incidence of tyrosinosis is therefore about one in 6,000 births, or at least


Sign in

Purchase Options

• Buy this article
• Subscribe to the journal
• Rent this article ?

First Page Preview

View Large
First page PDF preview





Also Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
Please click the checkbox indicating that you have read the full article in order to submit your answers.
Your answers have been saved for later.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.


Some tools below are only available to our subscribers or users with an online account.

0 Citations

Sign in

Purchase Options

• Buy this article
• Subscribe to the journal
• Rent this article ?

Related Content

Customize your page view by dragging & repositioning the boxes below.