An Atypical Electrophoretic Peak in Serum of Patients with Familial Primary Systemic Amyloidosis

Block, Walter D.; Rukavina, John G.; Curtis, Arthur C.

doi:10.3181/00379727-89-21748

Cited by 18 publications

(3 citation statements)

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“…-It was made at least once in 16 patients, resulting normal in 4 cases only. The most frequent changes were disorders of repolarization (11), ven tricular overload (6), first degree atrioventricular block (5), extrasystolia (4), and complete block (2).…”

Section: Electrocardiogrammentioning

confidence: 99%

Type I primary neuropathic amyloidosis (Andrade, Portuguese): a clinical and laboratory study of 21 cases

Azevedo

Scaff

Canelas

et al. 1975

Arq. Neuro-Psiquiatr.

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The authors present a review of 21 cases with the diagnosis of type I amyloid neuropathy based on epidemiological data, clinical evolution and histopathological findings. They call attention to the possibility of cranial nerves involvement (hyposmia, diplopia, masseterian hypotrophy, peripheral facial paralysis, hypoacusis, dysphonia, laryngeal paralysis, dysphagia, and trapezium muscle hypotrophy), to the severeness of the digestive symptoms, to the precocity of the autonomic disorders, and to the rather high incidence (6 cases) of heart involvement. The electromyography showed anterior horn involvement in 3 cases. The electrocardiography showed repolarization disorders in 11 cases, left ventricular overload in 6 cases and atrioventricular block in 5 cases. The serum proteins electrophoresis showed frequent abnormalities, but no typical curve could be obtained. The barium-contrasted X-rays of the gastrointestinal tract showed no anatomical lesions, but functional abnormalities (hypo or hypermotility) were found in 14 examination. The Schilling test showed impairment of vitamin B12 absorption in 50% of the cases. However, with the concomitant administration of intrinsic factor (3 cases) there was improvement of it absorption. This proves that the gastric mucosa plays an important role in the disease malabsorption. The test with labeled-triolein showed slow absorption in 2 cases and steatorrhea in 3 (6 tests). For the confirmation of the amyloid deposits, the best histopathological procedure was nerve biopsy. In men, when the nerve biopsy was negative, testicular biopsy has shown to be a good option.

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Section: Electrocardiogrammentioning

confidence: 99%

Type I primary neuropathic amyloidosis (Andrade, Portuguese): a clinical and laboratory study of 21 cases

Azevedo

Scaff

Canelas

et al. 1975

Arq. Neuro-Psiquiatr.

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show abstract

“…This is sometimes called a "curtain effect". I t is possible that this is caused by the presence of an extra fraction as described for example, by Block et al (1956). They called this fraction a,l-globulin.…”

Section: Clinical Manifestationsmentioning

confidence: 99%

Amyloidosis of the Heart

Buchem

Mandema

Arends

1962

Journal of Internal Medicine

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“…The predominant sign in this type is vitreous opacities made up of amyloid de posits [15]. With some frequency, a peak in the region corresponding to an a,-globulin can be demonstrated in sera from these patients [5]. Histo logically, the amyloid deposits are predominantly found in the retinacu lum flexorum manus [16], in the fascia of the calf and in the vascular walls.…”

Section: Introductionmentioning

confidence: 99%