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Biomedical subjects

Patricia I Dickson

Publications and source records attributed to Patricia I Dickson.

2 recordsLinked to original sources

Costello syndrome with pancreatic islet cell hyperplasia.

A 3-month-old boy with Costello syndrome (CS) developed respiratory distress and fatal arrhythmias. An autopsy showed cardiac hypertrophy, mild coarctation of the aorta, and pancreatic islet cell hyperplasia. To our knowledge, this is the first report of a pancreatic abnormality in CS. Islet cell hyperplasia has also been observed in leprechaunism, Beckwith-Wiedemann (BWS), and Simpson-Golabi-Behmel syndromes. The syndromes are thought to involve abnormal insulin or insulin-like growth factor (IGF) pathways. Clinical similarities among these disorders and CS, together with the finding of islet cell hyperplasia, suggest that they may be related.

Abnormalities, Multiple↗

Normal right and left ventricular mass development during early infancy.

The average newborn's right ventricular mass, indexed to body surface area, is approximately 20% above the adult level, consistent with in utero physiologic hypertrophy. In contrast, the left ventricular mass index is underdeveloped by about 30% at birth, resulting in a ratio of right to left ventricular mass that is nearly 75% higher than at maturity. Left ventricular mass index increases rapidly during the first 2 weeks of life and then more slowly thereafter, whereas the right ventricular mass index decreases steadily during the first 4 months of life.

Adult↗