scholarly journals A case of juvenile form Pompe's disease manifested as chronic alveolar hypoventilation

1993 ◽  
Vol 8 (3) ◽  
pp. 221 ◽  
Author(s):  
Dong Gyu Kim ◽  
Kisuck Jung ◽  
Myung Koo Lee ◽  
In Gyu Hyun ◽  
Hwa Jung Lim ◽  
...  
Keyword(s):  
Juvenile Form ◽  
Pompe’S Disease ◽  
Pompe's Disease ◽  
Alveolar Hypoventilation

??-Glucosidase appears promising in Pompe's disease

2000 ◽  
Vol &NA; (1249) ◽  
pp. 8
Author(s):  
&NA;
Keyword(s):  
Pompe’S Disease ◽  
Pompe's Disease

Diagnosis of Pompe's disease using pyridylamino-maltooligosaccharides as substrates of α-1,4-glucosidase

1985 ◽  
Vol 147 (2) ◽  
pp. 97-102 ◽  
Author(s):  
Mitsuo Midorikawa ◽  
Shintaro Okada ◽  
Tomochika Kato ◽  
Tohru Yutaka ◽  
Hyakuji Yabuuchi
Keyword(s):  
Pompe’S Disease ◽  
Pompe's Disease

Administration of a Mixture of Fungal Glucosidases to a Patient with Type II Glycogenosis (Pompe's Disease)

PEDIATRICS ◽  
1968 ◽  
Vol 42 (4) ◽  
pp. 672-676
Author(s):  
Ronald M. Lauer ◽  
Thelma Mascarinas ◽  
Antonio S. Racela ◽  
Antoni M. Diehl ◽  
Barbara Illingworth Brown
Keyword(s):  
Skeletal Muscle ◽  
Enzyme Assay ◽  
Glycogen Content ◽  
Hydrolytic Enzymes ◽  
Type Ii ◽  
Electron Microscopic ◽  
Pompe’S Disease ◽  
Pompe's Disease ◽  
Type Ii Glycogenosis

A case of Type II glycogenosis (Pompe's disease) has been studied by histochemical, electron microscopic, and biochemical techniques. These studies have been made prior to and after the intramuscular administration for 1 week of a mixture of hydrolytic enzymes containing both α-1,4- and α-1,6-glucosidase activities. Electron photomicrographs of the liver before enzyme administration showed glycogen to be located both within and outside of membrane-limited vacuoles. No change in this distribution could be detected in tissue removed by biopsy after enzyme administration. This impression was confirmed by the determination of glycogen content which was shown to be unchanged. Nevertheless, the liver was found by enzyme assay to contain the administered enzyme. Leucocytes isolated from blood taken 4 hours after the last enzyme injection were also shown to contain the parenterally administered glucosidases. In skeletal muscle glycogen was present chiefly as extrasaccular deposits which were unchanged in appearance by enzyme administration. No glucosidase activity was demonstrable in the skeletal muscle after such a treatment. Myocardium sectioned after autopsy had major deposits of glycogen in extrasaccular areas.

The conduction system in Pompe's disease

1982 ◽  
Vol 2 (1) ◽  
pp. 25-32 ◽  
Author(s):  
Saroja Bharati ◽  
Maria Serratto ◽  
Ira DuBrow ◽  
Milton H. Paul ◽  
Steven Swiryn ◽  
...  
Keyword(s):  
Conduction System ◽  
Pompe’S Disease ◽  
Pompe's Disease

Cardiomyopathy in Pompe's disease

2008 ◽  
Vol 19 (1) ◽  
pp. 57-59 ◽  
Author(s):  
A. Fayssoil
Keyword(s):  
Pompe’S Disease ◽  
Pompe's Disease

G.P.8.03 Misleading muscle biopsies in late onset Pompe’s disease

2009 ◽  
Vol 19 (8-9) ◽  
pp. 591-592 ◽  
Author(s):  
A.L. Taratuto ◽  
A. Dubrovsky ◽  
J. Corderi
Keyword(s):  
Late Onset ◽  
Pompe’S Disease ◽  
Pompe's Disease ◽  
Muscle Biopsies
Sign in / Sign up

Export Citation Format

Share Document