1136

examination was normal. The patient’s mother had had a chronic paraparesis for several years but no cause had been established. All other family members were healthy and none of them had any of the recognised risk factors for HTLV-I infection. None of the remaining 39 Iraqi patients had serological evidence of HTLV-1 infection. Simultaneous onset of lymphoma and HTLV-I associated myelopathy (HAM) is unusual, as is familial HAM.4,5 This is to our knowledge the first report of HTLV-I infection affecting several members of an Arab family. Department of Oncology, Ibn AI-Bitar Hospital, Baghdad, Iraq

Virology Division, Department of Medical Microbiology, University College and Middlesex School of Medicine,

London W1P 7PN, UK

SRDJAN DENIC PETER NOLAN JOHN DOHERTY

JEREMY GARSON PHILIP TUKE RICHARD TEDDER

1. El-Farrash MA, Badr MF, Hawas SA, et al. Sporadic carriers of human T-lymphotropic virus type I in northern Egypt. Microbiol Immunol 1988; 32: 981-84. 2. Sidi Y, Meytes D, Shohat B, et al. Adult T-cell lymphoma in patients of Iranian origin Cancer 1990; 65: 590-93. 3. Lee H, Swanson P, Shorty VS, et al. High rate of HTLV-II infection in seropositive IV drug abusers in New Orleans. Science 1989; 244: 471-75. 4. Kawai H, Nishida Y, Takagi M, et al. HTLV-I associated myelopathy with adult T-cell leukemia. Neurology 1989; 39: 1129-31. 5. Mori M, Ban N, Kinoshita K. Familial occurrence of HTLV-I associated myelopathy. Ann Neural 1988; 23: 100.

Neutrophil mobility was examined when the neutrophil count had been raised by rhG-CSF. Defective neutrophil mobility was demonstrated in vitro in a Boyden chamber but not in vivo in the Rebuck skin window assay. The patient had steatorrhoea, very low serum pancreatic amylase, lipase, and trypsin activities, and very few chymotrypsin activity in stools. Electrolyte concentrations in sweat were normal. His bone age was 8 months. The fever and bronchopneumonia responded to fosfomycin 150 mg/kg daily by intravenous infusion and rhG-CSF (Kirin Brewery Co, Tokyo) at a daily dose of 100 Jlg/m2 by subcutaneous injection. rhG-CSF raised the neutrophil count to 45 300/ul in 7 days. The platelet count also rose (figure). Bone marrow aspiration showed myeloid hyperplasia with a nuclear cell count of 362 000/p.l, and mature neutrophils occupied about 25% of these nuclear cells. Absolute counts of erythroid and megakaryocyte lineage cells also increased. This fmding suggests that rhG-CSF can enhance the growth of multipotent progenitor cells in the presence of other factors, as reported in vitro.s Although the neutrophil count falls to pre-treatment level in 2 weeks, periodic administration of rhG-CSF has fully protected this boy from infection.

Department of Paediatrics, Kumamoto University Medical School, Kumamoto 860, Japan

NAOTO ADACHI HIROYUKI TSUCHIYA HIROYUKI NUNOI SHIGENORI HIGUCHI IZUMI AKABOSHI SHOUJI CHIKAZAWA

H, Diamond LK, Oski FA, et al. The syndrome of pancreatic insufficiency and bone marrow dysfunction.J Pediatr 1964; 65: 645-63. Shmerling DH, Prader A, Hitzig WH, et al. The syndrome of exocrine pancreatic insufficiency, neutropenia, metaphyseal dysostosis, and dwarfism. Helv Paediatr

1. Shwachman

rhG-CSF for Shwachman’s syndrome

2.

SIR,-Shwachman’s syndrome is characterised by pancreatic exocrine insufficiency and bone marrow dysfunction,1 and the leading cause of death is infection, usually because of the neutropenia and defective neutrophil mobility.’ We describe here the use of recombinant human granulocyte colony-stimulating factor (rhG-CSF) to raise the neutrophil count in a patient with this

syndrome. A 1 -1-year-old boy was admitted in March, 1990, with high fever, bronchopneumonia, and severe neutropenia. He had a history of frequent lower-respiratory-tract infections and failure to thrive since the age of 6 months. There was no contributory family history. Leucocyte count was 6900/)il with a neutropenia of 552/111 (differential stab 7%, segment 1%, lymphocyte 87%, monocyte 5%) and he had slight anaemia and thrombocytopenia. Bone marrow aspiration revealed hypoplastic marrow with a nuclear cell count of 64 000/)il, and mature neutrophils were rare.

Acta 1969; 24: 547-75.

Aggett PJ, Harris JT, Harvey BAM, et al. An inherited defect of neutrophil mobility in Shwachman’s syndrome. J Pediatr 1979; 94: 391-94. 4. Ruutu P, Savilahti E, Repo H, et al. Constant defect m neutrophil locomotion but with age decreasing susceptilibity to infection in Shwachman syndrome. Clin Exp Immunol 1984; 57: 249-55 5. Ikebuchi K, Clark SC, Ihle JN, et al. Granulocyte colony-stimulating factor enhances interleukin-3-dependent proliferation of multipotential hemopoietic progenitors. Proc Natl Acad Sci USA 1988; 85: 3445-49. 3.

Rhabdomyolysis and a "greenhouse effect" SIR,-Non-traumatic rhabdomyolysis may result from toxins or drugs, electrolyte disturbances, enzyme deficiencies, and hyperpyrexia.1,2 We describe a case of acute renal failure due to rhabdomyolysis resulting from direct heating of muscles during the exceptionally hot summer of 1990 in the UK. A previously fit 37-year-old glazier was admitted with a 4-day history of vomiting, sweats, dizziness, sore throat, and generalised weakness. He was heavily built, peripherally shut down, and appeared ill, but there were no specific abnormalities on examination. He was dehydrated with a central venous pressure of 5 cm HO; despite resuscitation he remained oliguric, passing only 80 ml urine in the first 24 hours after admission. Abnormal investigations included white cell count 39 600/1, sodium 126, potassium 5-7, urea 30-8, calcium 1-15, and phosphate 4-75 mmol/l, -

creatinine 686 kinase 423 000

Haematological changes after rhG-CSF injections Shwachman’s syndrome.

in

boy

with

WBC=white blood cell count, Neut=neutrophil count, Plt=platelet count

umol/1, aspartate transaminase 1480 IU/1, creatine IU/I (normal below 60). Renal ultrasound and virus

antibody titres were normal. 4 days after admission painful swelling of the upper arms developed and a computed tomographic scan indicated low density areas in the arm muscles consistent with the rhabdomyolysis, fmding being supported by electrophysiological tests. The acute renal failure was complicated by hypercalcaemia (3-11 mmol/1), which resulted in the "acute red eye of uraemia". 4 weeks later, a technetium-99m pyrophosphate scan showed diffuse calcification in the upper arm muscle but a plain X-ray was normal. He required haemodialysis for 3 weeks, after which there was complete recovery. History and investigations excluded the usual causes of acute rhabdomyolysis and revealed no explanation for the unusual distribution of involved muscles. However, on the day before the

1137

of symptoms he had been fitting shop windows, working on the inside of the glass in direct sunlight, wearing a singlet. That day was the hottest in the UK since meteorological records began, the outside temperature in Leeds rising to 34-4°C. We suggest that this man’s rhabdomyolysis and the consequent acute renal failure resulted from direct thermal trauma to exposed areas from direct sunlight on an unusually hot day, exacerbated by working under onset

glass. J.A.COOK J. M. HILL J. H. TURNEY

Regional Renal Unit, General Infirmary

at

Leeds,

Leeds LS1 3EX, UK

RA, Hamilton RW, Morse BM, Penn AS, Goldberg M. Nontraumatic rhabdomyolysis and acute renal failure. N Engl J Med 1974; 291: 807-11. 2. Dubrow A, Flamenbaum W. Acute renal failure associated with myoglobmuria and haemoglobinuria. In: Brenner BM, Lazarus JM, eds. Acute renal failure, 2nd ed. Edinburgh: Churchill Livingstone, 1988: 279-93. 3. Frimoyer PA, Giammarco R, Farrar FM, Schroeder ET. Technetium Tc99m medronate bone scanning in rhabdomyolysis. Arch Intern Med 1985; 145: 1991-95. 1 Grossman

Radiation exposure and cardiac catheterisation SIR,-Dr Pipilis and colleagues report (Sept 1,

2-3-fold increase in radiation exposure of staff when cardiac catheterisation is done via the brachial rather than femoral approach. It should be a matter of considerable concern that staff may be subjected to even greater variations in radiation exposure when performing comparable procedures with similar equipment at two different cardiac centres. This hospital uses Siemens X-ray equipment similar to that used by Pipilis and colleagues in Oxford, including a ’Pandoros Optimatic 12’ pulse DC generator, as part of a ’Cardoskop U’ system. Angiography is usually done with 35 mm cinefilm at 25 &ames/s. However, our equipment does not include either a mobile waist-high screen or the swivelling leaded glass and leaded rubber screen used by Pipilis et al. For two years I have monitored the radiation dose received at various parts of my body during cardiac catheterisation. I used a ’Pendix’ personal dosimeter (GST, Eppelheim/Heidelberg) to record exposure when acting as operator and when assisting a junior doctor under training. One site was identical to that studied by Pipilis and colleagues (ie, at the level of the neck above the lead apron). Other sites were the left axilla and left thigh beneath the lead apron. The left side is nearest the radiation source when the femoral approach is used. Only data from diagnostic left-heart catheterisations via the femoral approach are reported here and data are restricted to those procedures when operator and assistant did not change places during the catheterisation. At St Thomas’ Hospital operators and assistants receive doses of radiation at the neck (above the lead apron) 8-10 times higher than those received by staff doing procedures with longer screening times in Oxford (table). This difference is at least partly the result of lack of additional radiation screening measures at St Thomas’. p

567)

a

RADIATION DOSES TO STAFF DURING CARDIAC CATHETERISATION

Data

as mean

(SD). 0=operator, A=assistant

The split-sided, wrap-around lead aprons used at St Thomas’ allow radiation exposures to the left axilla and thigh comparable with those detected above the apron at the neck. Most staff here carry just one film badge, which is worn on the front of the abdomen beneath the lead apron. The highest dose I have ever recorded at that site during cardiac catheterisation was 13 }iSv but doses over 800 Sv have been recorded at other sites during complex cases. In some hospitals the monitoring of radiation exposure seems to be too insensitive to reflect the exposure. Furthermore algorithms to calculate how many cardiac catheterisations, pacemaker implantations, and angioplasties may be done in a year before a cardiologist need be classified under UK radiological protection legislation’ are worthless since they take no account of variations in radiation exposure during orocedures at different cardiac centres. Department of Cardiology, St Thomas’ Hospital, London SE1 7EH, UK

PETER WILMSHURST

HG, Faulkner K. Comparison of radiation risks with other nsks in cardiac radiological procedures. In: Faulkner K, Wall BF, eds. Are X-rays safe enough? Patient doses and nsks in diagnostic radiology. (IPSM Rep no 55). York. Institute of Physical Sciences in Medicine, 1988: 64-71.

1. Love

in a blood donor after administration of desmopressin

Myocardial infarction

SIR,- The capacity of desmopressin to raise plasma levels of coagulation factors has led to its use in patients with congenital and acquired bleeding tendencies,1 and in blood donors to augment yields of factor VIII and von Willebrand factor.2-4 Although desmopressin had an excellent safety record,s there have been reports of myocardial and cerebral infarctions after its administration to patients 6 I report an acute myocardial infarction in a blood donor shortly after infusion of this agent. A 47-year-old man in a plasma exchange donation programme to provide cryoprecipitate for his haemophilic son had donated 143 times (88 with desmopressin stimulation) since 19837 He met all medical criteria for blood donation and seemed in excellent health; however, he had risk factors for coronary artery disease, including a family history of premature heart disease, a 52 pack-year history of cigarette smoking before 1984, and raised cholesterol (300-340 mg/dl) and triglyceride (475 mg/dl) levels. On Jan 6, 1990, he received desmopressin 0-3 p.g/kg intravenously over 30 min, as usual. Donation then began, but after another 30 min he complained of chest pain. An electrocardiogram indicated subtle ST segment changes in the inferior leads. Creatine kinase was initially 103 IU/1 (normal below 145), but 6 h later it had risen to 549 lUll with 14% in the MB fraction. The patient recovered uneventfully without thrombolytic therapy. For the preceding 75 donations, his factor VIII level immediately after demospressin infusion had averaged 331% (range 100-690%); on Jan 6 it was 1040%. Angiography on day 5 showed a 10 mm hypokinetic area in the left ventricle. There were 50% narrowings in the left anterior descending and right coronary arteries, but no occlusion of even a small terminal branch could be identified. Previous reports of myocardial infarction associated with desmopressin have been confined to patients with antecedent vascular disease, and some investigators have suggested that the stimulation of coagulation causes thrombosis of a diseased coronary artery. This man’s factor VIII level was unusually high at the time of the incident, but angiography did not conclusively demonstrate a thrombotic occlusion. Paradoxical vasospasm might also account for these observations. Vascular events after desmopressin are uncommon. A recent survey found only ten reports for the years 1985-88, during which 433 000 doses were sold by the manufacturer. Such a low frequency in a population with independent risk factors might be labelled as fortuitous. Indeed, in controlled studies of patients undergoing open heart surgery, there was no significant difference between the frequencies of thromboembolism in desmopressin recipients and controls.6 The incident in the blood donor is more disturbing because he had been in excellent health and had no prior indication of vascular disease; furthermore angiography, though not