In this glycogen storage disease, the enzyme deficiency causes the body to form glycogen molecules that have an abnormal structure. This abnormal structure also prevents the glycogen from being broken down into free glucose.
Synonyms:
Limit Dextrinosis
Debrancher Deficiency
Cori Disease
Forbes Disease
There have been observed a variety of subtypes of this disorder, and there appears to be considerable variation in the tissues affected by the defect viz.such as white blood cells, muscle, liver, and so forth. In GSD Type IIIa, the disease involves both liver and muscle tissues. In Type IIIb only the liver is involved.
ABDOMEN – LIVER and region of liver; complaints of
abies-c.;1 _ abrot.;1 _ acon.;3 _ aesc.;2 _ agar.;2 _ all-c.;1 _ aloe;2 _ alum.;2 _ am-m.;2 _ ant-t.;1 _ apoc.;2 _ arg-n.;2 _ arn.;2 _ ars.;2 _ ars-i.;1 _ asaf.;1 _ aur.;2 _ aur-ar.;1 _ aur-i.;1 _ aur-m.;1 _ bapt.;2 _ bar-c.;1 _ bell.;3 _ benz-ac.;1 _ berb.;3 _ bry.;3 _ bufo;3 _ cadm-s.;1 _ calc.;3 _ calc-f.;2 _ calc-p.;3 _ calc-sil.;1 _ camph.;1 _ carb-v.;2 _ carbn-s.;3 _ card-m.;3 _ cham.;2 _ chel.;3 _ chin.;2 _ chrystl.;1 _ cimx.;2 _ cinnb.;1 _ clem.;1 _ cocc.;2 _ colch.;2 _ coll.;2 _ coloc.;2 _ combr-r.;1 _ con.;2 _ corn.;3 _ croc.;2 _ crot-c.;2 _ crot-h.;2 _ cupr.;2 _ dig.;1 _ dros.;1 _ dulc.;1 _ ferr.;1 _ ferr-ar.;1 _ ferr-p.;1 _ fl-ac.;2 _ gels.;1 _ graph.;2 _ grin.;1 _ haru-ma.;1 _ hep.;2 _ hydr.;2 _ hypoes-t.;1 _ iod.;3 _ iris;2 _ kali-bi.;2 _ kali-c.;3 _ kali-i.;1 _ kali-s.;1 _ lac-d.;1 _ lach.;3 _ laur.;2 _ lept.;3 _ lyc.;3 _ mag-c.;1 _ mag-m.;3 _ mang.;1 _ merc.;3 _ merc-c.;1 _ microg-p.;1 _ mur-ac.;1 _ nat-ar.;1 _ nat-c.;1 _ nat-m.;2 _ nat-p.;12 _ nat-s.;3 _ nit-ac.;3 _ nux-m.;3 _ nux-v.;3 _ oci-su.;1 _ petr.;1 _ ph-ac.;2 _ phos.;3 _ plat.;1 _ plb.;2 _ podo.;3 _ prun.;2 _ psor.;2 _ puls.;1 _ ran-b.;1 _ ran-s.;1 _ rhus-t.;1 _ ruta;1 _ sabad.;1 _ sang.;2 _ sel.;1 _ sep.;3 _ sil.;1 _ spig.;1 _ sul-ac.;1 _ sul-i.;1 _ sulph.;3 _ tab.;1 _ ther.;1 _ verat.;1 _ zinc.;2
Children with GSD III are often first diagnosed because they have been noticed to have a swollen abdomen due to a very large liver. Some children have problems with low blood sugars on fasting but this is not as common as in GSD I. Growth may be delayed during childhood but most reach a normal adult height. Muscle weakness is commonly present in childhood and can, at times, be severe. Often the liver returns to a normal size at puberty, although the enzyme defect persists.
ABDOMEN – ENLARGED – Liver
aconin.;1 _ agar.;1 _ anis.;1 _ ant-t.;1 _ ars.;2 _ ars-i.;1 _ aur.;1 _ aur-ar.;1 _ aur-i.;1 _ aur-m.;2 _ aur-s.;1 _ bar-m.;1 _ boerh-d.;1 _ bry.;2 _ bufo;1 _ calc.;2 _ calc-ar.;2 _ calc-sil.;1 _ carb-v.;2 _ carc.;1;fb _ card-m.;1 _ chel.;2 _ chin.;3 _ chinin-ar.;1 _ chion.;2 _ cocc.;2 _ con.;2 _ con.;2 _ dig.;2 _ dig.;2 _ eup-per.;1 _ ferr.;2 _ ferr-ar.;1 _ ferr-i.;1 _ ferr-i.;1 _ ferr-p.;1 _ fl-ac.;2 _ hep.;2 _ hippoz.;2 _ hippoz.;2 _ hydr.;1 _ iod.;2 _ kali-c.;2 _ kali-s.;1 _ lach.;1 _ lact.;1 _ laur.;2 _ luf-b.;1 _ lyc.;3 _ mag-m.;3 _ merc.;2 _ merc-i-r.;1 _ mur-ac.;1 _ nat-m.;2 _ nat-s.;3 _ nat-s.;3 _ nit-ac.;2 _ nux-m.;2 _ nux-v.;3 _ phos.;2 _ pin-s.;1 _ plb.;1 _ podo.;2 _ pop-c.;1 _ ptel.;1 _ sec.;1 _ sec.;1 _ sel.;1 _ sep.;1 _ sil.;1 _ sul-i.;1 _ sulph.;2 _ tab.;1 _ thuj.;1 _ thuj.;1 _ tub.;2 _ urt-u.;1 _ vip.;1 _ zinc.;2 _ zinc-p.;1
Chemical analysis of the blood usually shows low blood sugar, elevated glycogen content in red blood cells, and elevated levels of fat. Uric acid and lactic acid levels are usually normal. Biopsy of the liver shows inflammatory changes, with great elevations of abnormal-structured glycogen content, and a deficiency of the debrancher enzyme. Biopsy of muscle shows an accumulation of abnormal-structured glycogen in Type IIIa.
Treatment for Type III glycogen storage disease consists of frequent feedings and a high protein diet. Continuous nasogastric feedings and the starch regimes similar to those used for Type I GSD are useful.
Persons with debrancher deficiency have lived well into late adulthood. Muscle disorders seem to be an increasing problem with age in those persons with Type IIIa. The heart can be mildly enlarged, but the function is usually normal.
Dr. Rajneesh Kumar Sharma
Homoeo Cure & Research Centre P. Ltd.
NH 74, Moradabad Road, Kashipur (Uttaranchal) 244713 India
Ph- 05947- 275535, 260327, 274338, 277418
Fax- 274338, 275535; Cells- 98 976 18594, 98 976 21896
drrajneeshhom@hotmail.com, drrajneshhom@yahoo.co.in