Half a century ago, a doctor who built a long career taking care of children with hormone deficiencies was just getting his scientific start.
In a brief column appearing online this week, “50 years ago in The Journal of Pediatrics,” three Stanford physicians highlight the first scientific publication of Raymond Hintz, MD, who founded Stanford’s Division Pediatric Endocrinology & Diabetes. The January 1968 paper, written when Hintz was still completing his training in Ohio, described important consequences of a congenital brain defect called holoprosencephaly. The disease affects the pituitary gland, a pea-sized lump inside the brain that serves as the master regulator of the body’s hormonal communication system:
In 1968, Hintz et al described 2 cases of holoprosencephaly occurring in siblings. The older sibling died on the first day of life, and autopsy revealed an absent pituitary and adrenal hypoplasia. Testing on the younger sibling diagnosed endocrinopathies involving both anterior and posterior pituitary hormones. The authors were the first to report a thorough endocrine evaluation of an infant with holoprosencephaly. Their recommendations for a careful search for neuroendocrine deficiencies in similar patients remains pertinent advice for contemporary pediatric care.
Hintz came to Stanford in 1975 and became a prolific scientist, eventually publishing more than 350 papers. He died in 2014 at age 75.
Hintz and his wife, Carol, had four children, one of whom followed him into academic pediatrics. Today, Susan Hintz, MD, is medical director of the Fetal and Pregnancy Health Program at Lucile Packard Children’s Hospital Stanford, where she works with many families who receive difficult prenatal diagnoses, including holoprosencephaly. She spoke recently with me about her dad’s influence on her work.
"We were very, very lucky to be able to actually work side-by-side on many cases," Susan Hintz said. "It was very special to have that connection, incredibly valuable to me as both a daughter and as a physician."
Her father loved to talk with her about the benefits of the scientific advances he had seen during his career. Case in point: Holoprosencephaly can now often be diagnosed during pregnancy with an MRI scan of the fetal brain. "That was not possible when he was in training; there was barely even fetal imaging of any kind," Hintz said. Prenatal diagnoses allow physicians to quickly evaluate and give hormones and other treatments from birth, averting the endocrine crises that devastated babies when Raymond Hintz was beginning his career.
Susan Hintz also felt fortunate to observe her dad's compassionate approach to the children in his care. His example helped her learn how to deliver difficult news, and still shapes how she gives hard news to families today, she said. "Our responsibility is not only to share information in words they can understand, but to also be able to comfort them, be empathetic and help them through what may be the biggest crisis of their lives."
Previously: What history can teach us about medicine: A Q&A with a Stanford medical student, Stanford-led study suggests changes to brain scanning guidelines for preemies and Panel of Stanford Medicine leaders discusses compassion in health care
Photo of Raymond Hintz courtesy of Susan Hintz