1143 INTRAVENOUS COPPER IN MENKES’ KINKY-HAIR SYNDROME

BILE CANALICULAR ANTIBODIES IN DISEASES OF KIDNEY AND URINARY TRACT

SiR—The letter by Dr Dekaban and Dr Steusing1 prompts us to report our experience with parenteral

immunofluorescence staining of double lines along the bile canaliculi is associated with various diseases (unpublished observation). The immunofluorescence pattern of the bile canaliculi in Linder et al.’s and Ablin et al.’s investigations is not recorded. A different staining pattern of the bile canaliculi, however, need not be the only reason for the difference between these findings. Another could be the use of liver from different species as antigen. Linder et al. and Ablin et al. used rabbit liver and I used ox liver. Of various livers, we found ox liver to be best for the demonstration of B.C.A. in patients with chronic liver disease. Another reason for the discrepancy could lie in the evaluation of the reactions. Many sera give a pericellular staining of hepatocytes, and this staining could be taken for staining of bile canaliculi.ó An exchange between interested laboratories of B.C.A.positive sera from patients with chronic liver disease and other diseases as controls in the investigation for B.C.A. would be one way to get comparable results between investigations in different laboratories. Blood Bank and Medical Department M,

University Hospital, DK 5000 Odense, Denmark.

HANS DIEDERICHSEN.

DECREASED FIBRINOLYTIC ACTIVITY AND

copper in a child with Menkes’ kinky-hair syndrome. The diagnosis was made in 1971 at the age of 11 months, when the boy, who had developed normally for the first 3-6 months, showed progressive cerebral deterioration, which ended in severe spastic tetraplegia. The scalp hair showed the typical " steely woolappearance, and pili torti were demonstrated- microscopically. At 32 months, serum-copper levels, determined for the first time, were between 38 and 50 (Lg. per 100 ml. (bathocuproine method, normal range 65-165). Serum-casruloplasmin was 13-15 mg. per 100 ml. (p-phenylenediamine-oxidase assay, normal range 30-60). No rise in copper levels was noted after 20 mg. and 200 mg. of copper sulphate were given by mouth, the latter dose being followed by vomiting after 60 minutes. 1 mg. of copper sulphate in 250 ml. of 5 % glucose solution was infused over 3 hours. Serum-copper levels were 38 (Lg. per 100 ml. immediately before and immediately after the infusion and 72 p.g. per 100 ml. 72 hours later. 0°4 ml. of a commercially available copper-sulphate solution in distilled water (250 (Lg. copper per ml.) was injected subcutaneously 1-3 times weekly without local irritation and without effect on serum-copper levels. In February, 1974, at age 3! years, the child was readmitted after a cerebral convulsion. The clinical state remained unchanged. Sharp-wave foci had developed in the electroencephalogram. Serum-copper was 56 (Lg. per 100 ml. and serumcaeruloplasmin 17 mg. per 100 ml. A sterile solution of copper sulphate in physiological saline was prepared, so that 1 ml. contained 1 mg. copper (stock solution). 1 ml. and 2 ml. of this solution were added to 250 ml. of 5% glucose solution and the infusions were repeated. The results Maximum copper and are shown in the accompanying figure. cxruloplasmin levels were reached only after a lag of 72-96 hours after the infusions. This may mean a reflux of copper bound to newly synthesised caeruloplasmin (and possibly other coppercontaining proteins) from the liver into the plasma. Similar results have been reported.2.3 From then on we tried to treat the child with subcutaneous in-

MALARIA

SIR,-Fibrinolytic activity measured by different tests, has been found to be consistently low in malaria/-1O but it was not known if this is due to decreased blood-fibrinolytic components or increased fibrinolytic inactivators. Plasminogen levels (determined by an immunological technique unaffected by plasmin inactivator 11) was studied in 89 specimens from 39 patients with acute falciparum malaria, of whom 3 had cerebral or pulmonary complications. The plasminogen levels in all our specimens were found to be normal, indicating that there is no increase in plasmin inactivator in malaria. Prolonged euglobulin lysis time 7-10 and low or absent fibrinolysis on fibrin plate by euglobulin 8-10 must be due to decreased bloodplasminogen-activator as euglobulin is freed from inactivator,12 and the present study indicates no increase in plasmin inactivators. Vascular endothelium is the most likely source of blood-plasminogen-activator, 12 and vasculitis leads to impaired fibrinolysis.13 In the light of other studies,7-10 our data reinforce the concept that vascular injury is an important pathological component of malaria.14 Department of Pathology, Faculty of Medicine, BENCHA PETCHCLAI Ramathibodi Hospital, UBOLRATANA PRATUMVINIT. Bangkok. Phrabudhabat Hospital, WISITH BENJAPONGES. Bangkok, Thailand. Eichelberger, J. W., Inman, M. M., Conrad, M. E. Blood, 1967, 29, 713. Borochovitz, D., Crosley, A. L., Metz, J. Br. med. J. 1972, ii, 710. Reid, H. A., Sucharit, P. Lancet, 1972, ii, 1110. Sucharit, P., Reid, H. A. S.E. Asian J. trop. Med. publ. Hlth, 1973, 4, 15. Pratumvinit, U., Petchclai, B. Am. J. clin. Path. 1974, 61, 458. Astrup, T., Thorsen, S. Med. Clins. N. Am. 1972, 56, 153. Cunliffe, W. J., Menon, I. S. Br. J. Derm. 1971, 84, 99. Maegraith, B., Fletcher, A. Adv. Parasit. 1972, 10, 49.

Dekaban, A. S., Steusing, J. K. Lancet, 1974, ii, 1523. Danks, D. M., Stevens, B. J., Campbell, P. E., Gillespie, J. M., Walker-Smith, J., Blomfield, J., Turner, B. ibid. 1972, i, 1100. 3. Bucknall, W. E., Haslam, R. H. A., Holtzman, N. A. Pediatrics, Springfield, 1973, 52, 653.

1. 2.

7. Dennis, L. H., 8. 9. 10. 11. 12. 13. 14.

Effect of

two copper

infusions

on serum

caeruloplasmin levels.

copper and

Letter: decreased fibrinolytic activity and malaria.

1143 INTRAVENOUS COPPER IN MENKES’ KINKY-HAIR SYNDROME BILE CANALICULAR ANTIBODIES IN DISEASES OF KIDNEY AND URINARY TRACT SiR—The letter by D...
133KB Sizes 0 Downloads 0 Views