A rare form of a devastating disease which causes low blood sugar levels in babies and infants may now be recognised earlier thanks to a new test developed by researchers from The University of Manchester.
Congenital hyperinsulinism starves a baby's brain of blood sugar and can lead to lifelong brain damage or permanent disability according to previous research carried out by the Manchester team.
The condition occurs when specialised cells in the pancreas release too much insulin which causes frequent low sugar episodes - the clinical opposite of diabetes. Treatment includes drugs to reduce insulin release but in the most serious cases the pancreas is removed.
For some infants with this disease, the release of excess insulin is due to mutations in genes which govern the way our bodies control insulin release. But for more than two thirds of child patients the genetic causes are not yet known.
Genes and hormones were analysed in 13 children with congenital hyperinsulisnism at the Manchester Children's Hospital and the findings have been published in The Journal of Pediatrics.
Dr Karen Cosgrove from the Faculty of Life Sciences led the research: "We have discovered a new clinical test which can identify congenital hyperinsulinism in some patients with no known genetic cause of the disease. This is the first step to understanding what causes the disease in these particular patients. In future the test may influence how these children are treated medically, perhaps even avoiding the need to have their pancreas removed."
The new test measures a pair of hormones called incretins which are released by specialised cells in the gut when food is passing through. The hormones normally tell the cells in the pancreas to release more insulin to regulate sugar levels in our blood. If the child's body releases too much incretin hormones, the pancreas will release too much insulin causing dangerous low blood sugar levels.
"Although we are the first researchers to report high incretin hormone levels in patients with congenital hyperinsulinism, further studies are needed to see if our test works on a larger group of patients" says Dr Cosgrove.
Researchers from The University of Manchester along with consultants from the Manchester Children's Hospital, part of Central Manchester University Hospitals NHS Foundation Trust, teamed up for the study. Royal Manchester Children's Hospital is the base for the Northern Congenital Hyperinsulinism (NorCHI) service, a national centre for treatment of this disease.
Doctor Indi Banerjee, Consultant in Paediatric Endocrinology at Royal Manchester Children's Hospital and clinical lead for NorCHI says: "Our new results are timely since clinical trials of a new incretin-blocking treatment for congenital hyperinsulinism have recently started. We anticipate that our clinical test will help to identify the patients who are likely to benefit from this new treatment the most."
Julie Raskin, Executive Director of Congenital Hyperinsulinism International is impressed with the research: "A new diagnostic test for this devastating disease is welcome news to the international hyperinsulinism patient community because timely diagnosis is key to reducing the chance of brain damage and death, and the research also suggests a path to treatment other than sub-total pancreatectomy, which almost always leads to diabetes."
Morwenna Grills | Eurek Alert!
A promising target for kidney fibrosis
21.04.2017 | Brigham and Women's Hospital
Stem cell transplants: activating signal paths may protect from graft-versus-host disease
20.04.2017 | Technische Universität München
The nearby, giant radio galaxy M87 hosts a supermassive black hole (BH) and is well-known for its bright jet dominating the spectrum over ten orders of magnitude in frequency. Due to its proximity, jet prominence, and the large black hole mass, M87 is the best laboratory for investigating the formation, acceleration, and collimation of relativistic jets. A research team led by Silke Britzen from the Max Planck Institute for Radio Astronomy in Bonn, Germany, has found strong indication for turbulent processes connecting the accretion disk and the jet of that galaxy providing insights into the longstanding problem of the origin of astrophysical jets.
Supermassive black holes form some of the most enigmatic phenomena in astrophysics. Their enormous energy output is supposed to be generated by the...
The probability to find a certain number of photons inside a laser pulse usually corresponds to a classical distribution of independent events, the so-called...
Microprocessors based on atomically thin materials hold the promise of the evolution of traditional processors as well as new applications in the field of flexible electronics. Now, a TU Wien research team led by Thomas Müller has made a breakthrough in this field as part of an ongoing research project.
Two-dimensional materials, or 2D materials for short, are extremely versatile, although – or often more precisely because – they are made up of just one or a...
Two researchers at Heidelberg University have developed a model system that enables a better understanding of the processes in a quantum-physical experiment...
Glaciers might seem rather inhospitable environments. However, they are home to a diverse and vibrant microbial community. It’s becoming increasingly clear that they play a bigger role in the carbon cycle than previously thought.
A new study, now published in the journal Nature Geoscience, shows how microbial communities in melting glaciers contribute to the Earth’s carbon cycle, a...
20.04.2017 | Event News
18.04.2017 | Event News
03.04.2017 | Event News
21.04.2017 | Physics and Astronomy
21.04.2017 | Health and Medicine
21.04.2017 | Physics and Astronomy