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Monday, July 13, 2009

Lenz-Majewski Hyperostosis Syndrome

General:
Look like progeria patients

Airway:
Choanal stenosis/atresia
Abnormal enamel
Cleft palate
Micrognathia

Respiratory:
Recurrent pneumonia
Restrictive lung disease

Gastrointestinal:
Inguinal hernias

Neurological:
Mental retardation
Cerebral atrophy
Agenesis of corpus callosum
Hydrocephalus

Musculoskeletal:
Flexion contractures
Hyperostosis
hyperextensible joints

Other:
Cutis laxa

Considerations:
Respiratory insufficiency
Chronic lung disease
Positioning challenge

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