Hepatic iron stores and markers of iron overload in alcoholics and patients with idiopathic hemochromatosis

Liver iron concentrations were determined in 60 alcoholics with liver disease of varying severity, 15 patients with untreated idiopathic hemochromatosis, and 16 control subjects with biliary tract disease. Mean liver iron concentrations (g/100 mg dry weight) were significantly greater in the alcoholics (156.4±7.8 (sem);P<0.05) and in patients with idiopathic hemochromatosis (2094.5±230.7;P<0.01) than in control subjects (53.0±7.0). Liver iron concentrations of >140 g/100 mg were found in 17 alcoholics (29%) and in all 15 patients with idiopathic hemochromatosis. Liver iron concentrations >1000 g/100 mg were found in all patients with idiopathic hemochromatosis but in none of the alcoholics. In the alcoholics no relationship existed between liver iron concentrations and the amount of alcohol consumed daily, the length of the drinking history, the amount of beverage iron consumed daily, or the severity of the liver disease. Serum ferritin concentrations reflected iron stores in patients with hemochromatosis and in alcoholics with minimal liver disease. However, in alcoholics with significant liver disease serum ferritin concentrations did not reflect iron stores accurately, although with normal values iron overload is unlikely. Serum iron concentration and percentage saturation of total iron-binding capacity were of little value in assessing iron status in either alcoholics or patients with hemochromatosis. Measurement of the liver iron concentration clearly differentiates between alcoholics with significant siderosis and patients with idiopathic hemochromatosis.R. W. Chapman was Watson-Smith Fellow of the Royal College of Physicians of London.     

The role of cranial and thoracic electromyography within diagnostic criteria for amyotrophic lateral sclerosis

Introduction: The contribution of cranial and thoracic region electromyography (EMG) to diagnostic criteria for amyotrophic lateral sclerosis (ALS) has not been evaluated. Methods: Clinical and EMG data from each craniospinal region were retrospectively assessed in 470 patients; 214 had ALS. Changes to diagnostic classification in Awaji‐Shima and revised El Escorial criteria after withdrawal of cranial/thoracic EMG data were ascertained. Results: Sensitivity for lower motor neuron involvement in ALS was highest in the cervical/lumbar regions; specificity was highest in cranial/thoracic regions. Cranial EMG contributed to definite/probable Awaji‐Shima categorization in 1.4% of patients. Thoracic EMG made no contribution. For revised El Escorial criteria, cranial and thoracic data reclassified 1% and 5% of patients, respectively. Conclusion: Cranial EMG data make small contributions to both criteria, whereas thoracic data contribute only to the revised El Escorial criteria. However, cranial and thoracic region abnormalities are specific in ALS. Consideration should be given to allowing greater diagnostic contribution from thoracic EMG. Muscle Nerve 54 : 378–385, 2016     

Clinical interventions for venous leg ulcers: Proposals to improve the quality of clinical leg ulcer research

The present status of clinical leg ulcer healing research was reviewed by 25 experts over 2 days on September 28 and 29, 2015. Multiple clinical effectiveness reviews were presented suggesting that published clinical wound healing research often does not meet present (2015) evidence based standards. Specific areas requiring remediation were highlighted and approaches to overcoming existing challenges were proposed. Participants using anonymous voting technology developed an action plan to resolve perceived deficiencies. Statements were accepted if 75% of participants agreed. Older patients with a high frequency of comorbid conditions posed particular difficulties in designing clinical research protocols and better diagnostic categorization is necessary A standardized model template for collecting information about diagnosis and evaluation of the effect of interventions on healing of all types of leg ulcers was considered a high priority. Such a model template could be modified depending on the specific etiology of the leg ulcers. Generally agreed on quantifiable standards to establish degree of morbidity was considered a high priority. There was universal agreement that sources of funding and conflicts of interest needed to be disclosed in presentations and all publications. All clinical research studies should be registered with appropriate authorities. There was substantial enthusiasm for a clinical research network with quality standards for membership and an advisory research core available to investigators. Such a network should be funded and actively managed to insure long‐term viability. The governance of such an entity needs to be established by the wound care community. The present trend to integrate patients into the clinical research process was endorsed and there was enthusiasm to develop patient advocacy for wound healing research.     

A non-transferrin-bound serum iron in idiopathic hemochromatosis

The nature of iron in the serum of patients with idiopathic hemochromatosis has been studied utilizing an isotope labeling method and results have been compared with those from normal individuals and patients with other forms of liver disease. Between 2 and 4% of a tracer dose of⁵⁹Fe added to normal serum was retained by DEAE Sephadex and has been designated non-transferrin-bound. Alcoholic liver disease, chronic active hepatitis, and iron deficiency have no effect on this fraction. In idiopathic hemochromatosis 34.6±3.9% of the added iron was not bound to transferrin at diagnosis, representing approximately 700 μg Fe/liter serum. Treatment lowers this fraction before serum iron concentration falls to normal. The majority of the non-transferrin-bound iron is of low molecular weight and is not bound to albumin. The presence of this fraction may contribute significantly to the development of tissue siderosis.