TY - CHAP M1 - Book, Section TI - Wiedemann-Rautenstrauch Syndrome A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Engelhardt, Thomas PY - 2019 T2 - Syndromes: Rapid Recognition and Perioperative Implications, 2e AB - This is a medical condition characterized by an aged appearance at birth, prenatal and postnatal growth retardation, and subcutaneous lipoatrophy with abnormal deposits of fat around the buttocks, the anogenital area, and the flanks. Other clinical features include craniofacial abnormalities (macrocephaly due to frontal and parietal bossing) that lead to a pseudohydrocephalus aspect. Coronary and cerebrovascular atherosclerosis raise significant concerns in such children. Also, these infants have sparse hair, prominent scalp veins, entropion, malar hypoplasia, hypotrichosis, beaked nose, and mild to severe mental retardation. Those affected have been prone to recurrent respiratory infections that have resulted in life-threatening complications. The life expectancy is significantly decreased and death is usually encountered during childhood. There are only few reported individuals who have reached adolescence and even in their 20s. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/10/07 UR - accesspediatrics.mhmedical.com/content.aspx?aid=1164088103 ER -