𝔖 Bobbio Scriptorium
✦   LIBER   ✦

Muscle computed tomography in adult-onset acid maltase deficiency

✍ Scribed by Aeiko E.J. de Jager; Ton M. van der Vliet; Taco C. van der Ree; Bart J. Oosterink; M. Christa B. Loonen

Publisher
John Wiley and Sons
Year
1998
Tongue
English
Weight
85 KB
Volume
21
Category
Article
ISSN
0148-639X

No coin nor oath required. For personal study only.

✦ Synopsis

Nine patients with adult-onset acid maltase deficiency (Pompe's disease) were examined clinically and with computed tomography (CT). The CT scan showed early and severe involvement of the muscles of trunk and thighs, with selective sparing of the tensor fasciae latae, short head of biceps femoris, gracilis, and sartorius muscles. Shoulder and leg muscles were less affected. The disease spread over the years from trunk to extremities. Muscle strength and CT findings were positively correlated.

πŸ“œ SIMILAR VOLUMES

Respiratory improvement by muscle traini
Respiratory improvement by muscle training in adult-onset acid maltase deficiency
✍ Dr. Richard J. Martin; Dr. Robert L. Sufit; Dr. Steven P. Ringel; Dr. David W. H πŸ“‚ Article πŸ“… 1983 πŸ› John Wiley and Sons 🌐 English βš– 240 KB

## Abstract A patient with progressive involvement of respiratory function from adult‐onset acid maltase deficiency is reported. The decrement in objective and subjective waking and sleeping respiratory variables was significantly reversed by inspiratory muscle training. This training was accomplis

Genotype-phenotype correlation in adult-
Genotype-phenotype correlation in adult-onset acid maltase deficiency
✍ Dr John H. J. Wokke; Margreet G. E. M. Ausems; Marie-JosΓ© H. van den Boogaard; E πŸ“‚ Article πŸ“… 1995 πŸ› John Wiley and Sons 🌐 English βš– 508 KB

We performed a clinical, biochemical, and genetic study in 16 patients from 11 families with adult-onset acid maltase deficiency. All patients were compound heterozygotes and carried the IVS1( --13T+G) transversion on one alkele; the second allele harbored either a deletion of a T at position 525 in