Pompe JoannesCassianus

Dutch pathologist-anatomist, who described the type 2 glycogen storage disease Morbus Pompe. He was the youngest child of the Catholic Cornelis Andreas Pompe (15 March 1874, Utrecht - 22 Oct 1944, Maartensdijk) and Johanna Maria Alijda Brom (1874- 28 March 1905, Utrecht). They married 24 November 1891 in Utrecht and got between 1993 and 1901 twelve children. After Marias death his father remarried on 27 September 1911 her sister Arnolda Catharina Maria Brom (1862 - 21 November 1925, Utrecht). Pompe studied medicine at the University of Amsterdam. On 27 December 1930 he did autopsy of a 7 months old child with an enlarged heart and also noted enlargement of other body organs. The girl was a patient of Prof. Isidore Snapper (5 January 1889 1 PM, Amsterdam - 30 June 1973, New York) , who also had published on Von Gierke disease, now known as glycogen storage disease type 1. He discussed the unknown disease on 18 November 1931 with other physicians in Amsterdam. Today Pompes disease is known as an autosomal recessive metabolic disorder, characterized by storage of glycogen in organs and tissues, and associated with deficiency of the lysosomal enzyme acid maltase or alpha-1,4 glucosidase. As the glycogen cannot be metabolised, it accumulates in the tissues. He dissertated on 11 August 1936 with "Cardiomegalia glycogenica" In Nijmegen. After a spell at the St. Canisius Hospital in Nijmegen, he was appointed as Pathologist at Onze Lieve Vrouwen Gasthuis (OLVG, Our Loved Woman Guesthouse) in Amsterdam, where he worked from June 1939 until his death. When the Germans invaded the Netherlands, Pompe was called to arms. During the war he became active in resistance. He kept persecuted people in his laboratory and there was also an illegal transmitter in the animal house to contact the UK. Tre location of the transmitter was detected by the Germans. On Sunday 25 February 1945, at 10 am, 40-50 members of the SD entered the hospital. The wireless operator, Pierre Antoine Coronel (26 December 1914, Ouder-Amstel - 25 February 1945, Amsterdam), was broadcasting at the time and tried to resist. He was executed in the courtyard of the hospital. Pompe was warned and decided to hide, but was caught before he could leave. On April 14, 1945, the resistance blew up a railway bridge near St Pancras, destroying an army train in the process. As a reprisal, 20 Dutch prisoners, including Pompe and the animal keeper Louis Andreas Berben (26 Jan 1903, Amsterdam - 15 April 1945, Sint Pancras) were shot the next day at around 9 PM. The bodies were buried in a mass grave in dunes of Overveen. Personal On 1 October 1930 he married Augusta Maria Constantia Stordiau (1912, Antwerpen) in Amsterdam. Pompe was described as a Catholic polymath - a man of both science and the arts. His brother Willem Petrus Joseph Pompe (10 Match 1893 2 AM, Utrecht - 26 july 1968, Utrecht) became a noted criminologist. Link to Wikipedia Read less