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Aagenaes syndrome

A syndrome characterised by congenital hypoplasia of lymph vessels, which causes lymphedema of the legs and recurrent cholestasis in infancy, and slow progress to hepatic cirrhosis and giant-cell hepatitis with fibrosis of the portal tracts1. more...

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Aagenaes syndrome
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The genetic cause is unknown, but it is autosomal recessively inherited and the gene is located to chromosome 15q1,2. A common feature of the condition is a generalised lymphatic anomaly, which may be indicative of the defect being lymphangiogenetic in origin1. The condition is particularly frequent in southern Norway, where more than half the cases are reported from, but is found in patients in other parts of Europe and the US2. It is named after Oystein Aagenaes, a Norwegian paediatrician.

Also called cholestasis-lymphedema syndrome (CLS).

Read more at Wikipedia.org


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Ailing quadruplet dies in his mother's arms
From Spokesman Review, The (Spokane), 3/10/00 by Kelly McBride Staff writer

Caleb Haglund never did cry.

The 6-1/2-month-old boy died Wednesday at Sacred Heart Medical Center, where he spent his entire life.

Caleb, the only boy among quadruplets born to Randy and Lisa Haglund Aug. 20, had been in critical condition most of his life.

"He gave it a good fight," Lisa Haglund said. "We all did."

The Haglund babies were the fifth set of quadruplets born at Sacred Heart over the past two decades. They were born 15 weeks premature, a few days after Caleb's amniotic sac ruptured. All four babies started life on ventilators. Caleb's sisters - Grace, Emma and Rebecca - all breathed on their own by the time they made the trip to their Hillyard home last November and December. Caleb was never well enough to have the ventilator removed. The babies have an older brother, 6-year-old Josh.

The quads were conceived via in vitro fertilization, in which a doctor unites the sperm and eggs in a laboratory and then inserts them into the woman's uterus.

Dr. Edwin Robins of Spokane transferred four fertilized eggs, but did not expect any of the embryos to survive. When they all did, he suggested Lisa Haglund undergo a selective reduction, where one or two of the embryos are destroyed, making room for the others to grow.

The Haglunds declined, on moral grounds.

"It was a hard decision. But we really put things in God's hands," Randy Haglund said. The babies weighed between 1 pound 6 ounces and 1 pound 11 ounces at birth. They are among the smallest babies to survive an extended period of time in Spokane.

The doctors treating Caleb were increasingly frustrated by his failing lymphatic system, which caused him to retain excessive fluid in his body. Known as Aagenaes syndrome, the condition is hereditary but exacerbated in premature babies.

After diagnostic tests earlier this week showed that Caleb's body was pooling fluid in every possible location, that portions of his brain had atrophied and that he was suffering from a pulmonary hemorrhage, his parents decided to withhold further treatment.

"I got to hold him for the first time in a long time on Wednesday.

His heart rate started to drop," said Randy Haglund. "Then I passed him to Lisa and his heart rate continued to go down."

He died in his mother's arms.

Caleb's sisters are doing well at home, their parents said. Emma has no lasting effects from her prematurity. Rebecca is blind and has liver and gall bladder problems that may be caused by the same syndrome from which Caleb suffered. Grace has had her gall bladder removed and has a brain injury that may cause cerebral palsy.

Grace and Rebecca both get therapy from the Spokane Guild School.

The family asks that memorial donations be made to the Guild School, 2118 W. Garland, Spokane, WA 99205; or to Hillyard Baptist Church, 2121 E. Wabash, Spokane, WA 99207.

Copyright 2000 Cowles Publishing Company
Provided by ProQuest Information and Learning Company. All rights Reserved.

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