SOCIETY FOR THE STUDY
OF INBORN ERRORS OF METABOLISM

The aim of the society is to foster the study of inherited
metabolic disorders and related topics.


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SAD PASSING OF PROF Louis woolf

Prof L Woolf

Professor Emeritus, Department of Psychiatry, University of British Columbia

Born 24 April 1919 in London
BSc, University College London, 1940
PhD, University College London, 1943
Died 7 February 2021, heart condition, Vancouver

Professor Louis Woolf died aged 101 from a heart condition on Sunday 7 February 2021 in Vancouver, British Columbia.  He is best known for his pioneering work developing his concept of a dietary treatment for phenylketonuria, PKU, based on a phenylalanine - depleted acid hydrolysate of the milk protein casein.

He was born in London, UK and brought up in Hackney, the third child of Jewish immigrant parents from northern Romania.  He attended the then prestigious grammar school, Hackney Downs School, where he became inspired to study science.  He gained scholarships to study for a degree in chemistry at University College London, UCL, achieving First Class Honours, and subsequently for a PhD.  In 1939, at the outbreak of World War 2, he along with the rest of UCL, was evacuated to Aberystwyth in Wales and it is there he met his future wife, Frances Mary Richards, on a blind date.  They married in Welshpool in 1943. 

His first appointment in 1943 was as a lecturer for a pre-university chemistry course in Brighton College of Technology.  After 1 year he left for a research post at pharmaceutical and special dietary products manufacturer Allen and Hanbury based in Ware, north of London.  One of the food products they manufactured - considered easy to assimilate for babies and invalids - was casein hydrolysate.  During those years it was produced to feed the post - World War 2 starving in Europe.  Across the site in their research laboratory Louis developed pharmaceuticals notably a new antacid, dihydroxyaluminiumaminoacetate, and also attempted to purify the company’s proprietary penicillin.  In 1947 he secured an Imperial Chemical Industries (ICI) funded post as Research Fellow at Great Ormond Street Hospital, GOSH, initially to study tyrosine metabolism in premature babies.  He was provided with a laboratory on the 4th floor entirely for his own use.  He introduced and developed the new technique known only since the mid 1940s of two dimensional paper chromatography for his studies of urine amino acids.  His laboratory thereby became one of the few in the UK to offer this.  At that time the general view was that PKU was untreatable.  In what could now be considered translational research, a bridge between science and clinical practice, Louis had a ‘light bulb’ moment at a biochemistry meeting in which a more economical growth medium for a bacteriological test for amino acids was suggested during a discussion. Individual, pure amino acids were expensive at that time whereas amino acids produced from protein hydrolysates prepared by treating protein with acid were much less so.  Selective removal of phenylalanine and some other amino acids from a hydrolysate by treatment with charcoal was described at the meeting.  Louis thought that by adding other essential nutrients this could form a suitable liquid protein substitute food for PKU patients.  Moreover he was aware of a source of a protein, casein, hydrolysate from Allen and Hanbury’s.  By potentially lowering the phenylalanine in the blood of PKU patients this artificial diet could test the hypothesis that the main cause of disability was their abnormally high phenylalanine.  Louis was unsuccessful in persuading paediatricians at GOSH to trial this.

Louis had been visited several times since 1949 by the German doctor, Horst Bickel, who had asked to spend a number of weeks as laboratory observer when he was en route from Zürich Kinderspital in Switzerland to a new post at Birmingham Children’s Hospital, UK.  On one return visit Bickel enquired whether Louis could think of a way to treat PKU.  Louis described his concept and whilst allegedly sceptical of Louis’ idea, Bickel in fact successfully treated a 2 year old girl with PKU in Birmingham this way.  This was a world first with a lasting legacy.

Less well known is Louis’ conviction since his time at GOSH that newborn screening would likely improve PKU outcomes by allowing earlier treatment.  Urine testing appeared a simple and non-invasive way to do this.  In 1958 he had moved with his family to Oxford to continue PKU research.  At a chance meeting back in London he discussed the idea with Senior Medical Officer in Public Health, Dr Nancy Gibbs, from Wales.  This conversation led to the first community based urine screening programme of the general newborn population.  It was aimed at detecting all cases of PKU in a cohort of babies in Cardiff from 1958 to 1959.  This was another world first and soon to be followed by similar urine screening programmes in cities in England, Scotland and Northern Ireland.  Louis’ knowledge and experience was becoming increasingly recognised and accordingly he was invited to join 2 working groups in 1963 and 1968 reporting to the UK’s Medical Research Council on detection and treatment of PKU.  From evaluation of these screening schemes it became apparent that urine testing was not sufficiently reliable.  The conclusion and recommendation of the expert groups in 1968 was that blood analysis with the heel prick test we are now so familiar with was more satisfactory.

Louis’ published work had become noticed internationally and in 1968 Dr William Gibson from the University of British Columbia, UBC, offered him a post as Associate Professor at UBC which he took up, with promotion to Professor following 6 years later in 1974.  He published extensively with over 120 papers to his name, a book on renal tubular dysfunction and he contributed to books, conferences and supervised research students, notably Savio L-C Woo on the isolation and properties of human liver phenylalanine hydroxylase.  He retired in 1984 after a 16 year teaching and research career at UBC.

A shy and modest man he was devoted to his family.  His wife Frances died in 1991 and Louis is survived by his only daughter Lesley, grandsons Benjamin and Oliver and his youngest brother, Henry.  He was beautifully spoken in a refined, old-fashioned British way with an astonishing memory to the end.  Generous to people who criticised him he quietly stood his ground, recognising that valid debate is integral to scientific discovery.  And - like many another innovative scientist - it is in this way that he triumphed over sceptical adversity to leave a legacy in pioneering the successful treatment of PKU.

Louis asked that there should be no funeral and in lieu of flowers that people might make a donation to CARE, Canada.

  • 23 April 2021

    ISIMD Spring Conference
    Maple Syrup Urine Disease & Branched-Chain Amino Acids: Above and Beyond

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  • 6 May 2021

    Epnet Webinar - Diagnosing and managing porphyria cutanea tarda: the basics 

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  • 12 May 2021

    WEBINAR: “Childhood Hypoglycaemia – why worry? ….”

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  • 14 - 16 May 2021

    XVth International Congress of Paediatric Laboratory Medicine

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  • 27 - 29 May 2021

    NEUROMETABOLIC DISEASES "it's all in the brain"

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  • 23 - 24 June 2021

    International Scientific CDG Symposium 2021 (Virtual)

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