Hirosato Kikuchi

DSC_2282_8.jpg

Professor Hirosato Kikuchi graduated from Hiroshima University School of Medicine in 1968. He continued his academic path at Hiroshima University, where in 1974, together with his mentor Prof. Michio Morio and his colleague Osafumi Yuge, helped to establish a list of clinical diagnostic criteria for malignant hyperthermia emphasizing on hypermetabolic signs other than the rapid rate of temperature rise.

He completed graduate studies at Yale University in 1978 and went back to Hiroshima. He moved to Tokyo in 1990, where he became Professor and Chair of the Anesthesia Department at Toho University School of Medicine.

Prof. Kikuchi championed the calcium-induced calcium release test for diagnosis of MH susceptibility in Japan, using the method of chemically permeabilized single muscle fibers developed by Makoto Endo. He established research and diagnostic laboratories for MH at Toho University, finally moving to Saitama Medical University in the early 2000's, where he remained working until the time of his death.

He introduced me to anesthesia and mentored me wisely until the present day, shedding light in my path as a human being, a clinician and MH researcher.

May his memory be blessed.

Carlos Ibarra

Ron Litman

image.jpeg

We were greatly saddened to learn that Ron Litman, Professor of Anesthesiology and Pediatrics, Perelman School of Medicine at the University of Pennsylvania and Anesthesiologist at The Children's Hospital of Philadelphia, Philadelphia, PA recently passed away. Ron was a paediatric anaesthetist by heart and a driving force for improved safety in anaesthesia, with a major interest in MH. He was heavily involved in MHAUS and he engaged constructively with the EMHG on numerous occasions. We have known Ron for many years and appreciated him as an open minded, extremely knowledgeable and loyal colleague. He will be greatly missed.

read more

Neil Eastwood Street

image0.jpeg

Dr Neil Eastwood Street AM MB BS (Hons II) M App Sc FANZCA 

Neil Street trained at Royal Prince Alfred Hospital and accepted a Fellowship in paediatric anaesthesia at the Children’s Hospital at Camperdown in 1985 before undertaking further paediatric anaesthetic training in the UK at Great Ormond Street. In 1987 he returned to consultant positions at the Children’s Hospital at Camperdown and Westmead Hospital, before taking on a full-time consultant position in 1995 at the Children’s Hospital after it moved to Westmead. He was Deputy Head of the Anaesthetic Department from 2005 to 2015 and Head from 2015-2019. 

In 2001, on a very limited budget, Neil developed the Malignant Hyperthermia testing facility at the Children’s Hospital, eventually providing from 2003 a testing service for NSW and Queensland patients and their families. Together with the CHW biomed team, Neil designed and built his own testing laboratory and continued to use it until recently, trouble-shooting its aging components and repairing it as only a true scientist and outstanding technician could. He was internationally recognized in the MH field and became one of the elders to whom others could turn to get advice and information. He gave many, many hours, much of his own personal time, to managing the MH unit, fielding questions and offering advice to the numerous anaesthetists (and indeed physicians and others) who contacted him with their concerns.

Neil was the epitome of an anaesthetist to which all could aspire. He was kind and respectful to patients, their families and staff at all levels. He was a superb technician with superlative skills. He could and did manage every case that came his way and was free with his advice, which was constantly sought. His special interests included anaesthesia for paediatric cardiac surgery, liver transplantation and spinal surgery, but there was no paediatric sub-specialty that he couldn’t manage with dexterity, knowledge and expertise. He was in demand by surgeons who recognized his calm experience, his exceptional skills and his wonderful sense of humour that could defuse the most tense moment. Neil understood the importance of caring for all patients to the highest standard, be they the sickest pre-term neonate with complex cardiac disease or a robust child with a broken arm.

 In 2006 Neil joined the volunteers of Open Heart International and over the years made many trips to Papua New Guinea (and one to Myanmar) to provide cardiac services to sick children who would otherwise have remained untreated. He not only helped provide anaesthesia in circumstances which would stress anyone, but at the highest level and with an attitude that drew instant respect from those with whom he worked. He was a much-loved regular team member on these annual trips, and he was recognized for his contribution to the training and development of the local anaesthetists and staff, who beautifully acknowledged this in a letter sent to his colleagues and his family after his passing. 

Neil was made a Member of the Order of Australia in 2015 for his significant contribution to paediatric anaesthesia, to malignant hyperthermia and to the people of the Asia-Pacific region though medical aid programs. He was almost -no, actually- embarrassed by this recognition and felt that he had always just been doing his job and no more – a manifest understatement of his contribution, as usual for Neil.

Neil, the person, was a wonderful man, friendly, funny, helpful, wise and perhaps above all, humble. His life reflected the values of irreverence and an understanding that every single person has equal value, instilled in him by his father, Fred Street, a paediatric surgeon, and his mother Gillie. He has been appropriately described as a polymath, equally at ease repairing a motor vehicle, welding up a grass catcher or installing electrical switches in his new caravan, or preparing a baby for complex cardiac surgery. He was a family man to the hilt with a wonderful love for his wife Cathy who had to deal with his huge commitment to his work over so many years. He was enormously proud of his children Philippa, Doug and Alice, their partners and grandson Freddie, and loved them unconditionally, not to mention his close and extended family. It is a great sadness to all that he became sick and eventually passed away at a time when he was planning to spend more time with his family and friends.

With Neil’s passing, there has been an outpouring of both love and grief from all whose lives he touched. There is such a huge sense of loss which can only be countered by the recognition of the goodness and care Neil brought to the world. Those who knew him will remember him as the wonderful man he was, and he will live on in our hearts.

Vale my great friend.

 

David Baines AM FANZCA

Frank Lehmann-Horn

lehmann_horn.png

Open channels – The life of Prof. Dr. Dr. h.c. Dipl. Ing. Frank Lehmann-Horn

Prof. Dr. Dr. h.c. Dipl. Ing. Frank Lehmann-Horn, director of the department of applied physiology at Ulm university Germany from 1992 to 2010, passed away on May 8th, 2018 after suffering a severe disease for many years. Prof. Lehmann-Horn leaves behind his wife Christa, three sons and four grand-children.

The scientist Prof. Frank Lehmann-Horn did not only investigate the opening of ion channels in cells, first and foremost he dedicated his life to create open channels for patients with rare diseases of skeletal muscle for whom no diagnosis and treatment were known in the past. Through his passionate scientific and clinical work, Lehmann-Horn opened new horizons for this group of patients. He always had an open ear for his patients and his open-mindedness for new ideas let him develop novel concepts for disease pathogenesis, diagnostic imaging, and choice of treatment. Furthermore he opened many channels for financial support of the research and established several successful co-operations and research networks around the world.

Lehmann-Horn had three professional degrees - mechanical engineer, specialist for neurology and with great enthusiasm and personal dedication specialist for physiology. Having this diversification, he was predestinated to measure and clinically interpret the miniature currents conducted by defective ion channels in tissue. Among others his mentors and later companions were the neurologists Prof. Albrecht Struppler and Prof. Kenneth Ricker as well as the physiologist Prof. Reinhardt Rüdel, director of the department of general physiology at Ulm University from 1979 to 2004. These scientists inspired Lehmann-Horn in the 1970ies as a young researcher of the technical university Munich and guided him towards the field of muscle disorders. One of Lehmann-Horn´s virtues was successful team-work and the team published the first ever functional description of an ion channel disease in human muscle [1] and in 1982 he was awarded the Duchenne-Erb-award together with Prof. Ricker and Prof. Rüdel. In 1990 Rüdel and Lehmann-Horn organized the VIIth International Congress on Neuromuscular Diseases in Munich, Germany, where the term “channelopathies” was coined.

From 1992 to 2010, Prof. Lehmann-Horn was directing professor for physiology at Ulm University, Germany. He was very skilled to form a successful team and was a respected personality with enthusiasm and a visionary leadership. At Ulm university, Frank and his team researched the field of ion channelopathies, here not only in terms of electrophysiological measurements, but also using most advanced methods of molecular genetics at that time.

Prof. Lehmann-Horn was a pioneer of research on Malignant Hyperthermia, a life-threatening pharmacogenetic disorder of skeletal muscle metabolism. His contributions helped to understand the pathophysiological basis of Malignant Hyperthermia being a dysregulation of calcium in skeletal muscle and that genetic alterations of the genes encoding calcium conducting proteins of skeletal muscle are causative [2]. He introduced the diagnostic in-vitro contracture test in Germany. In due course, he could identify many individuals carrying the trait for Malignant Hyperthermia thereby saving their life from suffering severe metabolic crises.

By systematic description of other rare muscle disorders, the interpretation of functional aspects and discovering genetic alterations, Lehmann-Horn contributed substantially to the current understanding of physiology and pathophysiology of ion channels.  Furthermore he implemented innovative treatment options and could help some patients, who were wheelchair-bound due to their muscle disorders, to regain enough strength to walk [3]. 

In 2008 he was awarded Senior Professor for Neuroscience and he was funded by the independent non-profit Hertie Foundation. He headed the Division of Neurophysiology of Ulm University from 2010 to 2016, the Interdisciplinary Center for Clinical Research 1995-2002, coordinated two EU Networks for Muscle Disease 1996-2006, and founded the Center of Rare Diseases in Ulm 2010. For his work, he received numerous awards, e.g. the Science Award of the city of Ulm 1995 and the Gaetano Conte Award 2004. In 2003, he received an honorary doctorate from Debrecen University for his achievements and was initiated into the Heidelberg Academy of Science in 2009. In 2016 Lehmann-Horn was awarded “honorary member” of the European Malignant Hyperthermia Group (emhg.org). 

Thanks to the life-time achievement of Lehmann-Horn, channelopathies are now found in every medical textbook and many patients benefit from his findings. Representative for his many scholars, collaborators, colleagues and everybody else, who valued his “open channels”, we bow to Prof. Dr. Dr. h.c. Dipl. Ing. Frank Lehmann-Horn. 

Prof. Dr. Werner Klingler
Director German Malignant Hyperthermia Hotline
Chair, Department of Anaesthesiology, Academic SRH hospitals Sigmaringen, Germany

Dr. med Karin Jurkat-Rott, MD, PhD
Vize-Director, Division of Neurophysiology 2010-2016, Ulm University, Germany

Prof. Dr. Holger Lerche
Chair, Department of Neurology/Epilepsy, Tübingen University, Germany

1 Lehmann-Horn F, Rüdel R, Dengler R, Lorković H, Haass A, Ricker K. Membrane defects in paramyotonia congenita with and without myotonia in a warm environment. Muscle Nerve. 1981 Sep-Oct;4(5):396-406.

2 McCarthy TV, Healy JM, Heffron JJ, Lehane M, Deufel T, Lehmann-Horn F, Farrall M, Johnson K. Localization of the malignant hyperthermia susceptibility locus to human chromosome 19q12-13.2. Nature. 1990 Feb 8;343(6258):562-4.

3 Jurkat-Rott K, Weber MA, Fauler M, Guo XH, Holzherr BD, Paczulla A, Nordsborg N, Joechle W, Lehmann-Horn F. K+-dependent paradoxical membrane depolarization and Na+ overload, major and reversible contributors to weakness by ion channel leaks. Proc Natl Acad Sci 2009 Mar 10;106(10):4036-41.

Yves Nivoche

Professor Yves Nivoche passed away on the 31th May, 2015 at the age of 64. He was in charge of the MH center in Paris Hopital Robert Debré. 

He had two professional passions: Pediatric Anesthesiology and Intensive Care and Malignant Hyperthermia. In both areas, he participated in the development of knowledge since the early days in the 70s up to the maturity of these two important sectors of Anesthesiology. He was for instance the co-author of a paper published in the British J of Anaesthesia in 1988 which is still regularly cited these days: Complications related to anesthesia in infants and children. 1988; 61: 263-9. The conclusions of this article helped to the obligation of recovery rooms and to the improvement of the safety and quality of pediatric anesthesia. He was, for a long time, Head of Department of Anesthesiology Department at the Robert Debré Hospital, one of the first french hospital for mother and children and he started there the in vitro diagnosis of MH. He was an important member of the French MH Group and organized in Paris with Renée Krivosic one of the meeting of the EMHG. 

Yves had remarkable qualities of intelligence, encyclopedic knowledge and was very emotionnal. He was an excellent teacher and showed lot of interest for his students. He participated to many training missions, especially in Viet Nam and North Africa. 

The sickness that took him away, hit him in his maturity. His courage and determination were flawless. He will remain in the memory of all who had the pleasure to work with him. May he rest in peace now. 

Professeur Renée KRIVOSIC-HORBER,
renee.krivosic@chru-lille.fr

Michael A. Denborough

image.png

Michael A. Denborough AM
M.D., M.B., Ch.B., D.Phil., D.Sc., F.R.A.C.P., F.R.C.P., Hon F.A.N.Z.C.A.
July 11,1929 – February 8, 2014

Michael Antony Denborough AM was born in Rhodesia in July 1929.

From early teenage years, he envisioned his future as a doctor and the persistence he showed in pursuing his medical education was a strong personality trait that was reflected in his later achievements. Michael studied at University of Cape Town and was granted a Rhodes scholarship to study at Oxford.

His clinical years began as resident medical officer at National Heart Hospital in London in 1958 and it was while he was studying and working in London he met his Australian born wife, Erica. Michael and Erica settled in Melbourne in 1960 where he commenced work as First Assistant (research fellow) at the University of Melbourne and Royal Melbourne Hospital.

It was at the Royal Melbourne that Michael was charged (because of his interest in genetics) by his supervising Professor Richard Robert Haynes Lovell, to investigate a family who were remarkable for their sad history of anaesthetic deaths.

Michael led an investigation into this family and coined the term malignant hyperpyrexia (MH) in a letter published in the Lancet in 1960. This letter and the follow up article two years later entitled “ Anaesthetic deaths in a family” in the British Journal of Anaesthesia were the first publications describing MH as a familial condition. For Michael, they were the start of a lifetime's investigation.

After 14 years at the RMH, Michael moved to the John Curtin School of Medical Research in Canberra where he was a Professorial Fellow from 1974 to 1991 and continued his remarkable research expanding to include muscle disorders with a relationship to MH. From 1992 to 1994 he was Professor at the John Curtin School of Medical Research at the Australian National University, retiring in 1995 to the role of Emeritus Professor.

Michael's achievements have been formally honoured over the years. In 1972 he was awarded the Eric Susman Prize from the Royal Australian College of Physicians and in 1982 he was honoured with a gold medal at the Fifth International Congress on Neuromuscular Diseases in Marseilles. In 1999 Michael became a member of the order of Australia for services to medicine.

Outside of Medicine, Michael was a passionate anti-nuclear campaigner. He was absolute in his opposition to war and the development and sale of weapons. He, Erica, and others founded the Nuclear Disarmament Party in 1984 and he also maintained a solo vigil outside parliament house in Canberra for 52 days campaigning to stop the war in Iraq. In his children’s words, he charged himself and others to “do something positive to save the world each day”.

Michael was a gracious gentleman. He was supporter of the individual – his actions reflect his belief that one man could make a difference – and he not only lived this dogma but also translated it to patient care, research and politics. As a teacher, Michael has been described as a gentleman physician most memorable to young students for showing a keen interest in their practical education and focusing on the doctor patient relationship. His legacy lives on in the lives of MH families and in all those who practice anaesthesia.

Michael died on February 8 2014 and his memorial service was held in Melbourne on Valentines Day. His children’s reflections at his memorial service described a father who loved unconditionally and was immensely proud of his family. He celebrated their achievements and encouraged them in their endeavours. Michael is survived by his wife Erica, his children Paul, Liz, David and Kate and his many grandchildren.

Vale Michael Antony Denborough – your contribution will not be forgotten.

Robyn Gillies MBBS (Hons) FANZCA
Department of Anaesthesia and Pain Management
Malignant Hyperthermia Diagnostic Unit
Royal Melbourne Hospital

Mary Lehane

Dr Mary Lehane, who died recently aged 69, was a founder member of the European Malignant Hyperthermia Group at its inaugural meeting in the University of Leeds in 1983.

Mary studied medicine in University College Cork and graduated with MB, BAO , BCh  degrees in 1970.  After anaesthetic training in Cork she was elected to Fellowship of the Faculty of Anaesthetists of the Royal College of Surgeons in Ireland. She was subsequently appointed senior registrar in the Birmingham group of hospitals and returned to Cork in 1978 to take up the position of Consultant Anaesthetist in Cork University Hospital where she played a key role in establishing a new intensive care unit. This was followed by setting up a collaboration on the in vitro diagnosis of malignant hyperthermia with Jim Heffron  of Cork University’s  Department of Biochemistry where  the pig was in use as a research model for MH.  After the initial EMHG meeting in Leeds a formal MH centre for Ireland was established in Department of Biochemistry. Mary was active in the in vitro diagnosis of MH until her retirement in 2009 and she attended many of the regular meetings of the EMHG in Austria,  Belgium, Denmark, France, Germany, Holland, Iceland, Italy, Sweden, Switzerland and the United Kingdom. Members of the MH units will remember Mary’s quiet and unassuming disposition combined with her clinical and research excellence; her research publications on MH in collaboration with Tommy McCarthy and Jim Heffron and numerous members of the EMHG have appeared in the leading anaesthesia and genetics journals and Nature. Mary was a valued member of Cork University’s Medical School where she also lectured in the Department of Pharmacology.  She was honoured by the College of Anaesthetists in Ireland in 2007 receiving the President's Award for her major contributions to anaesthesia and patient care and her role in education and MH research.

Outside of anaesthesia Mary had an intense interest in gardening and was a primary mover in developing Cork University Hospital’s gardens. She was an avid follower of rugby union, in particular, attending Munster’s many successes in Europe notably in the Heineken Cup. She bore her final illness with great courage and bravery and kept in contact with her university colleagues to the end.  Her sister and five brothers survive her.