Clinical features of hematological disorders caused by copper deficiency during long-term enteral nutrition.

OBJECTIVE: Copper deficiency has been reported to cause hematological disorders. However, its clinical and hematological characteristics are not fully understood. Therefore, we investigated bedridden patients suffering from copper deficiency and tried to clarify the clinical features of hematological disorders caused by this condition. PATIENTS AND METHODS: Five patients with typical copper deficiency who had been dependent upon enteral nutrition for a long period of time due to various diseases were investigated. We measured hematological parameters and observed the response to copper supplementation therapy and the recovery process of hematological disorders. RESULTS: Their mean age was 82.6+/-10.4 years and the mean duration of enteral nutrition was 16.4+/-5.2 months. Their serum copper concentration was extremely decreased (range, 3 to 8 microg/dl). All five patients had anemia and neutropenia. On the other hand, platelet count remained within the normal range. After copper supplementation therapy, hemoglobin concentration increased from 6.8+/-0.7 g/dl to 9.9+/-0.7 g/dl within a few months (p<0.01). Neutrophil count also increased from 750+/-370/microl to 3,690+/-1,210/microl in a few weeks (p<0.01). Mean corpuscular volume (MCV) decreased from 94.3+/-7.3 fl to 86.0+/-4.8 fl (p<0.05). Elevated serum ferritin and erythropoietin (EPO) levels were normalized after the improvement of anemia. CONCLUSION: Bicytopenia (anemia and neutropenia) with normal platelet count is a feature of hematological disorders caused by copper deficiency. MCV tends to indicate macrocytic anemia. Serum ferritin and EPO levels are elevated. These hematological abnormalities are improved within a few months after copper supplementation therapy.

Establishment of an assay method for human mast cell chymase.

Chymase secreted by mast cells found in fibroblast-containing interstitial connective tissue has been implicated in collagen fiber formation and extracellular matrix production. We established a method for determination of human chymase activity, and applied this technique to measurements in serum and liver tissue. The mean chymase concentration in liver biopsy specimens from 26 patients with chronic hepatitis was 5.23+/-5.98 ng/mg (ranges 0.32-21.4). The serum chymase concentration was below the limit of detection, in both chronic hepatitis patients and healthy individuals. No significant relationship was seen between chymase activity in liver tissue and severity of liver fibrosis, but further investigation in larger numbers of patients is warranted.