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At least 1,117 records · Page 62Linked to original sources
PubMed1994

Predominance of the adrenomyeloneuropathy phenotype of X-linked adrenoleukodystrophy in The Netherlands: a survey of 30 kindreds.

X-linked adrenoleukodystrophy (X-ALD) is an inherited disorder of peroxisomal beta-oxidation associated with accumulation of saturated very long-chain fatty acids, which results in central and peripheral demyelination and in impaired function of adrenal cortex and testes. The phenotypic expression is highly variable, childhood cerebral ALD (CCALD) and adrenomyeloneuropathy (AMN) being the main variants. We explored the 30 Dutch kindreds well known to the Dutch X-ALD/AMN Study Group and phenotyped 77 male patients: 35 (46%) had AMN and 24 (31%) CCALD or adolescent cerebral ALD (AdolCALD). These percentages differ significantly from previous reports, in which 25 to 28% of the patients developed AMN and 53 to 57% CCALD or AdolCALD. Our findings indicate that--at least in the Netherlands--AMN may be the most frequent phenotype of X-ALD.

Adolescent↗
PubMed1997

[Study on effect of reinforcing kidney and invigorating spleen principle on severe asthma].

OBJECTIVE: To explore on prevention and treatment of severe asthma with reinforcing the Kidney and invigoration the Spleen Principle (RKISP) and its mechanism. METHODS: Patients were treated with steroid aerosol (as control group), and both steroid aerosol and RKISP (as test group) respectively. RESULTS: The markedly effective rate of the test group was higher than that of the control group, the doses of beta-analeptic and the side-effect rates of the test group were lower than that of the control group, the pulmonary function of the test group was more remarkably improved. After treatment, the T cell hyperplasia response to specific allergen, the basophilic cell releasing capacity, the serum IgE and eosinophil of the test group were all reduced more obviously than those of the control group. In the control group, the neutrophil increased and the neutrophil phagocytic capability as well as the function of adrenal cortex were declined. While in the test group, the neutrophil phagocytic capability was raised, the neutrophil and the function of adrenal cortex had no significant changes. CONCLUSIONS: RKISP cooperated with steroid in relieving attack of asthma, and it was more important for it to display an obvious modulation action on immune and neuroendocrine system.

Administration, Inhalation↗