116

need to be revised, and the effect of humic acid on blood coagulation needs to be studied further. Department of Biochemistry, College of Medicine, National Taiwan University,

F. J. Lu

Taipei, Taiwan

S, How SW. A pathological study on the blackfoot disease in Taiwan. Rep Inst Pathol Natl Univ Taiwan 1963; 14: 25-73. 2. Tseng WP, Chen WY, Sung JL, Chen JS. A clinical study of blackfoot disease. Mem Coll Med Natl Univ Taiwan 1961; i: 1-8. 3. Chen CJ, Kuo TL, Wu MM. Arsenic and cancers. Lancet 1988; i: 414. 4. Lu FJ, Yang CK, Lin KH. Physico-chemical characteristics of drinking water in blackfoot endemic areas in Chia-I and Taiwan Hsiens. J Formosan Med Assoc 1975; 74: 596-605. 5. Lu FJ, Yamamuma Y, Yamauchi H. Studies on fluorescent compounds in water of a well in blackfoot disease endemic areas in Taiwan: humic substances. J Formosan Med Assoc 1988; 87: 66-75. 6. Lu FJ, Liu TM. Fluorescent compounds in drinking water of blackfoot disease endemic areas: animal experimental model. Formosan Med Assoc 1986; 85: J 352-58. 7. Lu FJ, Shih SR, Liu TM, Shown SH. The effect of fluorescent humic substances existing in the well water of blackfoot disease endemic areas in Taiwan on prothrombin time and activated partial thromboplastin time in vitro. Thromb Res 1990; 57: 747-53. 8. Lu FJ. Arsenic as a promoter in the effect of humic substances on plasma prothrombin time in vitro. Thromb Res (in press). 1. Yeh

Mean creatinine concentrations during intravenous gammaglobulin (IVIG).

treatment

with

Numbers of patients shown in parentheses. Vertical bars=SD.

dramatic response. Because of improvement steroid doses could be reduced in nine patients. Minor side-effects (nausea, vomiting, dizziness, and confusion) were noted in three patients and disappeared spontaneously. IVIG seems to be an effective new treatment for PM and DM, and could be used to replace or reduce steroid and immunosuppressive drug treatment.

a

Intravenous

immunoglobulin for dermatomyositis

polymyositis and

SiR,—Polymyosids (PM) and dermatomyositis (DM) are inflammatory immunological diseases of striated muscle. Various treatments have been used—cordcosteroids, immunosuppressive drugs, and, more recently, plasmapheresis, cyclosporin, and total body irradiation. However, these treatments have serious sideeffects.! Intravenous gammaglobulin (IVIG) has been used successfully in the management of some autoimmune diseases.2 Roifman et aP described the dramatic response to IVIG in a young girl with PM who had not responded to corticosteroid and

immunosuppressive drugs. Between August, 1987, and September, 1989, thirteen female and two male caucasian patients (thirteen with PM and two with DM) were treated by high doses of IVIG because of the failure of traditional treatments: prednisolone (fourteen), methotrexate (eight), azathioprine (five), cyclosphosphamide (three), plasmapheresis (six), lymphopheresis (one), and total body irradiation (one). In one patient positive for picornavirus, IVIG was the first treatment choice. The mean age of the patients was 44 years (SD 14), and the mean duration of the disease before IVIG was 39 years (range 8 months to 14 years). IVIG was given with prednisolone in eleven, with methotrexate in five, and with plasmapheresis in one; in some patients IVIG was given with more than one treatment. There were no treatment changes in the 2 months before the introduction of IVIG and no increases in dose during this treatment. We used preparations of polyvalent human IVIG with increased intact IgG. Ten patients received 1 g/kg daily for 2 days each month and five received 0-4 g/kg daily for 5 days each month. The mean course of treatment was 4 months. Patients were evaluated before each treatment period. Proximal muscle power was measured on a scale of 0-5 (Daniels and Worthington).4 Treatment was judged successful if a clinical improvement of 20% or greater was obtained. Mean serum creatine kinase concentrations were measured after each treatment period and were compared by the paired t-test. Clinical improvement was noted in thirteen patients and serum creatine kinase concentrations improved in the two remaining patients. A total of thirteen patients showed improvement in serum creatine kinase, and two with normal initial concentrations improved clinically. One subject had a moderate (30-50%) improvement in serum creatine kinase and twelve had major (over 50%) improvement. In general, improvement occurred during the first two perfusions. The mean creatine kinase concentrations improved significantly during IVIG therapy (figure). The creatine kinase concentrations continued to fall until the fourth perfusion. The patient with acute PM first treated by gammaglobulins showed

Service of Internal Medicine,

Hôpital Pitié, Paris, France Service of Internal Medicine, Hôpital Salpétrière, Paris

P. CHERIN* S. HERSON B. WECHSLER

Service of Internal Medicine, Hôpital Pitié, Paris

O. BLETRY C. DEGENNES

J. C. PIETTE J. M. ZIZA P. GODEAU

*Present address: Service of Internal Medicine, Hôpital Antoine Beclere, 92141 C1amart, France. 1. Plotz PH, Dalakas M, Leff RL, Love LA, Miller FW, Cronin ME. Current concepts in the idiopathic inflammatory myopathies: polymyositis, dermatomyositis, and related disorders. Ann Intern Med 1989; 111: 143-57. 2. Stiehm ER, Ashida E, Kim KS, Winston DJ, Haas A, Gale RP. Intravenous immunoglobulins as therapeutic agents. Ann Intern Med 1987; 107: 367-82. 3. Roifman CM, Schaffer FM, Wachsmuth SE, Murphy G, Gelfand EW. Reversal of chronic polymyositis following intravenous immune serum globulin therapy. JAMA 1987; 258: 513-15. 4. British Medical Research Council. Aids to the investigation of peripheral nerve injuries. 2nd ed. London: HM Stationery Office, 1943.

Potassium and sudden unexplained nocturnal death SIR,-Sudden unexpected nocturnal death (SUND) amongst Asians has been given much publicity recently (May 12, p 1154) one reason being the deaths in Thai construction workers in Singapore. Most of the victims came from north-east Thailand. Deaths usually occur at night in previously healthy men. The cause of death is not known but ventricular fibrillation is the most cited immediate cause. Dietary factors (especially thiamine deficiency), stress, and strenuous exercise are the most cited hypotheses. We have recently reported a clinical syndrome, prevalent in north-east Thailand, which is termed endemic primary distal tubular acidosis.l We described 103 patients admitted because of hypokalaemic paralysis and metabolic acidosis. The condition occurs in a homogeneous (Lao-Thai) population and has a high familial tendency. We encountered a pattern of death similar to that described in SUND in the relatives of patients with the renal disease. 2 male siblings of one of the propositi died during sleep, in a setting not dissimilar to SUND. They were muscular men aged 47 and 33; both deaths occurred at night. Serum potassium concentrations measured during our survey before the deaths were 3-5 and 3-1 mmol/1, respectively.

Intravenous immunoglobulin for polymyositis and dermatomyositis.

116 need to be revised, and the effect of humic acid on blood coagulation needs to be studied further. Department of Biochemistry, College of Medicin...
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