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Sounding out intracranial pressure with a hearing test

Heidary ear ICP measurement croppedBrain tumors, traumatic head injury and a number of brain and nervous system conditions can cause pressure to build up inside the skull. As intracranial pressure (ICP) rises, it can compress the brain and result in swelling of the optic nerves, damaging brain tissue and causing irreversible vision loss.

That’s what nearly happened to a 13-year-old boy who had three weeks of uncontrolled headaches and sudden double vision. His neuro-ophthalmologist at Boston Children’s Hospital, Gena Heidary, MD, PhD, found reduced vision in the right eye, along with poor peripheral vision, an enlarged blind spot and swelling of both optic nerves.

As Heidary suspected, he had idiopathic intracranial hypertension, a condition that can raise ICP both in children and adults. Heidary performed an operation around the optic nerve to relieve the pressure, and vision in the boy’s right eye gradually improved, though not completely. Heidary has had to monitor his ICP ever since to protect his visual system from further irreversible damage.

Unfortunately, such monitoring currently is pretty invasive.

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Proteomics provides new leads into nerve regeneration

Nerve regeneration. From Santiago Ramón y Cajal’s “Estudios sobre la degeneración y regeneración del sistema nervioso” (1913-14). Via Scholarpedia.

nerve regeneration proteomicsResearchers have tried for a century to get injured nerves in the brain and spinal cord to regenerate. Various combinations of growth-promoting and growth-inhibiting molecules have been found helpful, but results have often been hard to replicate. There have been some notable glimmers of hope in recent years, but the goal of regenerating a nerve fiber enough to wire up properly in the brain and actually function again has been largely elusive.

“The majority of axons still cannot regenerate,” says Zhigang He, PhD, a member of the F.M. Kirby Neurobiology Center at Boston Children’s Hospital. “This suggests we need to find additional molecules, additional mechanisms.”

Microarray analyses—which show what genes are transcribed (turned on) in injured nerves—have helped to some extent, but the plentiful leads they turn up are hard to analyze and often don’t pan out. The problem, says Judith Steen, PhD, who runs a proteomics lab at the Kirby Center, is that even when the genes are transcribed, the cell may not actually build the proteins they encode.

That’s where proteomics comes in. “By measuring proteins, you get a more direct, downstream readout of the system,” Steen says.

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What we’ve been reading: Week of April 27, 2015

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Crowdfunded science is here. But is it legit science? (Wired)
More and more scientists are turning to crowdfunding to pay for research. Is it the end of science as we know it or a meaningful and realistic evolution?

The world’s top 10 most innovative companies of 2015 in robotics (Fast Company)
Medical applications are well-represented in this compendium of companies working on the world of tomorrow today.

What Uber drivers can teach health care (KevinMD.com)
Doctors can learn from enterprising and fiercely independent Uber drivers.

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The silk scaffold: A promising road to hollow organ reconstruction

Silk photo_black backgroundSilk production and global interest in the lustrous fiber date back to prehistoric times. Today, the natural protein is solidifying itself as a biomaterials alternative in the world of regenerative medicine.

A recent study conducted by Boston Children’s Hospital urologist Carlos Estrada, MD and bioengineer Joshua Mauney, PhD, shows two-layer, biodegradable silk scaffolds to be a promising cell-free, “off-the-shelf” alternative to traditional implants for the reconstruction of hollow gastrointestinal structures such as the esophagus.

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RNSafe: Remote video checks of bedside drug dosing

RNSafe-Bunker nurse view Screenshot_2015-04-16-10-31-32When a nurse gives a complex medication at the bedside, a second nurse must come in to observe and verify the dose. But flagging down a nurse on a busy hospital floor can be pretty challenging, especially when the nurse has to “suit up” because of infection control precautions in the patient’s room. During a Nursing Morbidity and Mortality (M&M) Conference at Boston Children’s Hospital, a group of nurses expressed concern that this arrangement could potentially jeopardize safety. “We thought we should be able to do better,” says project co-developer Jennifer Taylor, MSEd, BSN, RN-BC, CPN.

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Early neglect and deprivation change the body’s stress response systems

Photo: Angela Catlin/Wikimedia Commons
Photo: Angela Catlin/Wikimedia Commons

Severe social and emotional deprivation in early life is written into our biochemical stress responses. That’s the latest learning from the long-running Bucharest Early Intervention Project (BEIP), which began in 2000 and has been tracking severely neglected Romanian children in orphanages. Some of these children were randomly picked to be placed with carefully screened foster care families, and they’ve been compared with those left behind ever since.

While studies in rodents have linked early-life adversity with hyper-reactivity of the sympathetic nervous system and the hypothalamicpituitaryadrenal (HPA) axis, the relationship has been harder to pin down in humans. BEIP’s study, involving almost 140 children around the age of 12, had children perform potentially stressful tasks, including delivering a speech before teachers, receiving social feedback from other children and playing a computer game that malfunctioned partway through.

Unlike the rodents, the institutionalized children had blunted responses in the sympathetic nervous system, which is associated with the “fight or flight” response, and in the HPA axis, which regulates production of the stress hormone cortisol. The researchers note that this dulled physiologic response has been linked to health problems, including chronic fatigue, pain syndrome and auto-immune conditions, as well as aggression and behavioral problems.

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Pediatric innovators showcase highlights inventions

Innovators Showcase Boston Children's HospitalSome great inventions were on view this week at the second annual Boston Children’s Hospital Innovators Showcase. Hosted by the hospital’s Innovation Acceleration Program and Technology & Innovation Development Office, the event featured everything from virtual reality goggles with gesture control to biomedical technologies. Below are a few new projects that caught Vector’s eye (expect to hear more about them in the coming months), a kid-friendly interview about the SimLab and list of inventions kids themselves would like to see. (Photos by Katherine Cohen except as noted)

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BabySee: Mobile app lets you see through an infant’s eyes

David Hunter, MD, PhD, chief of Ophthalmology at Boston Children’s Hospital, gets a lot of questions from parents, but the number one question is: “What can my baby see?”

That depends. How old is the baby?

Five days after birth, she might see something like the image at left; at 3 months, the image at right:

BabySee 5 days and 3 mos

At 6 months and 9 months, there’s increasing color and resolution:

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Advances in SCID (“bubble boy” disease): A Q&A with a child hematologist/oncologist

David Williams, Luigi Notarangelo and Sun-Yung PaiSung-Yun Pai, MD, a pediatric hematologist/oncologist at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, was lead author on two recent articles on severe combined immune deficiency (SCID) in The New England Journal of Medicine. The first reviewed outcomes after bone marrow transplantation; the second reported the first results of a new international gene therapy trial for X-linked SCID. Here, she discusses what’s known to date about these therapies.

Q: What is SCID?

A: SCID is a group of disorders that compromise the blood’s T cells, a key component of the immune system that helps the body fight common viral infections, other opportunistic infections and fungal infections. T-cells are also important for the development of antibody responses to bacteria and other microorganisms. A baby born with SCID appears healthy at birth, but once the maternal antibodies that the baby is born with start to wane, the infant is at risk for life-threatening infections. Unless diagnosed and treated—with a stem cell transplant from a healthy donor or a more experimental therapy like gene therapy—babies with SCID typically die before their first birthday.

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On the clock: Circadian genes may regulate brain plasticity

brain circadian rhythmsFirst in a two-part series on circadian biology and disease. Read part 2.

It’s long been known that a master clock in the hypothalamus, deep in the center of our brain, governs our bodily functions on a 24-hour cycle. It keeps time through the oscillatory activity of timekeeper molecules, much of which is controlled by a gene fittingly named Clock.

It’s also been known that the timekeeper molecules and their regulators live outside this master clock, but what exactly they do there remains mysterious. A new study reveals one surprising function: they appear to regulate the timing of brain plasticity—the ability of the brain to learn from and change in response to experiences.

“We found that a cell-intrinsic Clock may control the normal trajectory of brain development,” says Takao Hensch, PhD, a professor in the Departments of Molecular and Cellular Biology and Neurology at Harvard University and a member of the F.M. Kirby Neurobiology Center at Boston Children’s Hospital.

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