Aagenaes Syndrome Research Paper

Aagenaes syndrome is a syndrome that portray by congenital hypoplasia of lymph vessels. This causes lymphedema of the legs and recurrent cholestasis in infancy. This also causes slow progress to hepatic cirrhosis and giant cell hepatitis with fibrosis of the portal tracks. The genetic cause is unknown, but it is inherited in some way in the chromosomes that it is located in. People who have this suffer severe neonatal cholestasis, but this usually lessons during child hood. After the childhood stage it usually becomes episodic, and they also develop chronic severe lymphedema. The cause is unknown. Signs of this disease may vary on an individual basis for each patient. Some clinical features are jaundice, normal appetite, but limited weight gain in addition to edema.

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The prognosis of this …show more content…
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This disease is characterized by growth defects. Some defects are brachycephalic, deformities of the humerus, radius and ulna, short and broad hands, hyponastic maxilla, and mental retardation. This is a cystic disease. It is not cirrhosis because it is not active with inflammatory cells. The disorder is probably proliferation and dilation of portal bile ducts. Some other features are retinal pigmentation anomalies, optic atrophy, strabismus, nystagmus, cleft lip and palate, cardiovascular anomalies, hernia, abnormal nipples, and fits.

This disease is named after Osteon Aagenaes, a Norwegian pediatrician. These are similar because they have common side effects. This seems to indicate the defect of lymphangiogeneis. The development of small lymphoid vessels is deficient around small biliary tracts and in general. People experience jaundice soon after they are born, and edema of the legs was caused by hypoplasia of the lymphatic's. Liver histology showed giant cells transformation in infancy, and some fibrosis or cirrhosis in later childhood.

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