INTERTROCHANTERIC HIP FRACTURE TREATED BY IMMEDIATE MOBILISATION IN A SPLINT

301

f ’

INTERTROCHANTERIC HIP FRACTURE TREATED BY IMMEDIATE MOBILISATION IN A SPLINT

its primary mechanism of action in our patient’s pedigree, all members heterozygous for LDL-receptor deficiency should have responded similarly. Instead only those with the apo-E3-D phenotype responded dramatically, despite the unchanged E3/E2 ratio in the grandfather. Hence, clofibrate may preferentially facilitate clearance of remnant

this

were

A

Case-report

’

lipoproteins lacking apo-E 3We thank Joan Benderson for skilled technical assistance and Janis Heggins and Camille Granger for preparing the manuscript. This work was supported by grants from the American Egg Board and the National Institutes of Health (NIHV-12157A, HL 22607, and HL 23474). W. R. H. has been an investigator of the Howard Hughes Medical Institute. J. J. A. is an established investigator of the American Heart Association.

Requests for reprints should be addressed to W. R. H. REFERENCES 1. Hazzard WR.

III

Type hyperlipoproteinemia. In: Rifkind BM, Levy RI, eds. Hyperlipidemia: diagnosis and therapy. New York: Grune & Stratton, 1977:

137-75 2. Chait A, Brunzell JD, Albers JJ, Hazzard WR. Type III hyperlipoproteinaemia ("remnant removal disease"): Insight into the pathogenetic mechanism. Lancet 1977; i: 1176-78. 3. Hazzard WR, Chait A, Brunzell J, et al. Type III hyperlipoproteinaemia: Pathophysiological implications of its paradoxical hypolipidaemic response to oestrogen. In Peeters H, ed. Protides of the biological fluids. Oxford and New York:

Pergamon Press, 1978: 183-87. 4. Utermann G, Vogelberg KH, Steinmetz A, et al. Polymorphism of apolipoprotein E II. Genetics of hyperlipoproteinemia type III. Clin Genet 1979; 15: 37-62. 5. Manual of laboratory operations, lipid research clinics program. DHEW publication no. (NIH) 75-628, Washington D.C., U.S. Government Printing Office, vol. 1, 1974. 6. Noble RP. Electrophoretic separation of plasma lipoproteins in agarose gel. J Lipid Res 1968; 9: 693. 7. Lindgren FT, Jensen LC, Hatch FT. The isolation and quantitative analysis of serum lipoproteins. In: Nelson GJ, ed. Blood lipids and lipoproteins. New York: Wiley & Sons, 1972: 181-274. C, Albers JJ, Hazzard WR. A gel isoelectric focusing method for specific diagnosis of familial hyperlipoproteinemia type III. Clin Chem 1979; 25:

8. Warnick GR, Mayfield

279 9. Bilheimer DW, Ho YK, Brown MS, Anderson AW, Goldstein JL. Genetics of the low density lipoprotein receptor. Diminished receptor activity in lymphocytes from heterozygotes with familial hypercholesterolaemia. J Clin Invest 1978; 61: 678-96. 10. Utermann G, Jaeschke J, Menzel J. Familial hyperlipoproteinemia type III: deficiency of a specific apolipoprotein (apo E-III) in the very-low-density lipoproteins. FEBS Letts 1975; 56: 352-55. 11. Utermann G, Canzler H, Hees M, et al. Genetics of broad-&bgr; disease (hyperlipoproteinaemia type III). In: Peeters H, ed Protides of the biological fluids, vol. 25. Oxford and New York: Pergamon Press, 1978: 277-84. 12. Utermann G, Hees M, Steinmetz A. Polymorphism of apolipoprotein E and occurrence of dysbetalipoproteinaemia in man. Nature 1977; 269: 604-07. 13 Goldstein JL, Hazzard WR, Schrott HG, Bierman EL, Motulsky AG. Hyperlipidemia in coronary heart disease. II. Genetic analysis of lipid levels in 176 families and delineation of a new inherited disorder, combined hyperlipidemia. J Clin Invest

1973; 52: 1544-68. 14. Boman H, Hazzard WR, Albers JJ, Cooper MN, Motulsky AG. Frequency of monogenic forms of hyperlipidemia in a normal population. Am J Hum Genet 1975; 27: 19A. 15. Chait A, Albers JJ, Brunzell JD. Very low density lipoprotein overproduction in genetic forms of hypertriglyceridaemia. Eur J Clin Invest 1980; 10: 17-22. 16. Marien KJ, Huismanns AA, van Gent CM. On a family with co-existence of phenotypes II and III hyperlipoproteinaemia. Acta Med Scand 1974; 169: 149-53. 17 Lasser NL, Katz S. The occurrence of type III and type II hyperlipoproteinemia in a single kindred. Clin Res 1972; 20: 549. 18 Vessby B, Hedstrand H, Lundm L-G, Olsson U On the inheritance of type III hyper-

JOHN H. PATRICK Robert Jones and Agnes Hunt Orthopaedic Hospital, Oswestry, Department of Orthopaedic and Accident Surgery University of Liverpool

and

A 64-year-old man sustained an intertrochanteric hip fracture after a fall. He was un-

Summary

suited for general inflatable hip spica

spinal anaesthesia so a lightweight fitted for daytime wear. The splint permitted weightbearing and allowed mobility from the day of admission. The patient stopped wearing the spica after 4 weeks and was sent home with a clinically united fracture. or

was

Introduction Britain will have a million more people over the age of 65 in the year 2000 than there are today. This increase, together with the incidence of hip fracture (an average of 5 - 0 1000 elderly persons in a recent Newcastle survey’) suggest that these fractures will take up more and more hospital beds. Early postoperative mobilisation for the patient with hip fracture has been standard practice in Britain for the past 30 years. Orthopaedic surgeons have stressed the need to avoid the complications of lying in bed, noted by Asher,2 and implants and techniques have been designed to achieve a quick return to the upright stance. All orthopaedic surgeons acknowledge that this aim is achievable and worthwhile. Treatment of the intertfochanteric fracture by internal fixation after reduction at formal operation has stood the test of time; the available alternatives (hip spica plaster casts after reduction or traction, usually on a splint), have immense problems, which, historically, internal fixation has eliminated. This lack of an alternative has allowed us to overlook the problems of surgery, notably infection and implant migration, which may arise in 20% of cases. There is also the difficulty, for surgeon and anaesthetist, of operating on patients who are unfit when they come into hospital. This prompted us to look for a technique that would avoid the need for surgery whilst retaining the essential ingredient of immediate movement. Our solution is a light-weight hip spica that can be fitted in the accident department. This allows immediate mobilisation in the orthopaedic ward, in a rehabilitation unit, or even in the patient’s home (fig. 1).

cases per

lipoproteinemia. Lipoprotein patterns in first degree relatives Metabolism 1977; 26:

Case-report

225-54. 19 Brown MS, Goldstein

JL. Receptor-mediated control of cholesterol metabolism. Science 1976; 191: 150-54. 20 Godolphin WJ, Conradi G, Campbell DJ. Type-III hyperlipoproteinaemia in a child. Lancet 1972; i: 209-10. 21 Brown MS, Goldstein JL. Familial hypercholesterolemia: genetic, biochemical, and

pathophysiologic considerations. Adv Intern Med 1975; 20: 273-96. 22. Utermann G, Pruin N, Steinmetz A. Polymorphism ofapolipoprotein E III. Effect of a single polymorphic gene locus on plasma lipid levels in man. Clin Genet 1979; 15: 63-72. 23. Langer T, Strober W, Levy RI. The metabolism of low density lipoprotein in familial type II hyperlipoproteinemia. J Clin Invest 1972; 51: 1528-36. 24. Pitas RE, Innerarity TL, Arnold KS, Mahley RW. Rate and equilibrium constants for binding of apo-E HDLC (a cholesterol-induced lipoprotein) and low density lipoproteins to human fibroblasts: evidence for multiple receptor binding of apo-E HDLC Proc Nat Acad Sci 1979; 76: 2311-15. 25 Gianturco SH, Gotto AM Jr, Jackson RO, et al. Control of 3-hydroxy-3-methylglutarylCoA reductase activity in cultured human fibroblasts by very low density lipoproteins of subjects with hypertriglyceridemia. J Clin Invest 1978; 61: 320-28. 26. Levy RI, Fredrickson DS, Shulman R, et al. Dietary and drug treatment of primary hypertriglyceridemia Ann Intern Med 1972; 77: 267-94.

On Feb. 1, 1980, a 64-year-old retired widowed docker who had had severe chronic bronchitis for 13 years fell in his flat while alone, and it was 31/2 days before he was found by a friend who happened to call. He had not been able to attract the attention of his neighbours. He was immediately admitted to hospital, and, while not hypothermic, he was very ill with bronchopneumonia and cardiac failure. He had an intertrochanteric fracture of his left hip (fig. 2). His haemoglobin was 11’2g/dl (haematocrit 33%) and blood urea was 4.2mmol/1 (25 mg/dl). His general condition ruled out general anaesthesia, and a spinal anaesthetic could not be given for technical reasons.

It was decided to treat his fracture in an inflatable splint (fig. 3) providing immediate mobilisation. This greatly assisted recovery from his cardiorespiratory problems, it stabilised the fracture, and no

anaesthetic

was

needed.

302

Fig. 3-Splint and accessories. Fig. 1-Patient standing in splint.

Simple analgesics were prescribed regularly and by Feb. 15 (2 weeks post fracture) he could walk unassisted using a Zimmer frame. The fracture had become completely pain free by Feb. 23, and he could maintain and initiate a straight leg raise on the injured side by Feb. 28, exactly 4 weeks from the fracture date, when the splint was dis carded. He was discharged home on the 27th day after admission. A follow up X-ray (Sept. 29) is shown in fig. 4. On this day the patient’s only complaints were about the prominent greater

Fig. Fig. 2-X-ray of left hip

on

The splint provides three-point fixation of the fracture, and supports the injured limb with a weightbearing lateral beam. This is inflated by a portable mains electric pump to two atmospheres of pressure. The thin nylon material of the spica is carefully adjusted to the shape of the patient’s trunk and thigh by tightening its many adjustable lacings. The spica then tightly encloses the leg, thigh, and pelvis and the patient can be stood up, full weightbearing on the fracture, under the guidance of doctor or physiotherapist (fig. 1). The spica is donned daily and walking begins from the day of the fracture. It is usually pain free, if the spica has been properly ad-

justed, although regular simple analgesics (e.g., paracetamol

4-X-ray 34

weeks

post-fracture showing evidence

of sound

union, with shortening and varus deformity.

admission.

or

’Distalgesic’ [paracetamol plus dextropropoxyphene]) can be prescribed. A ’Tubigrip’ undergarment is worn for comfort and skin protection. The patient found the spica painful to put on and the hip unpleasant to flex or extend when rising from or getting into a chair.

trochanter, and

an inability to tie his shoelaces. The patient can shop for himself and walk at least a mile. He has no fixed deformity of the hip, but a range of flexion of 0-90°. Abduction is 300 (R=45°). Adduction is 45and equal to the opposite limb. Thereis no internal rotation possible, and external rotation of the hip is only 10°. His left knee is stiff with a range of motion, 0-90° only, and this limb is 1 - 5 cm truly short. This is due to a varus deformity (fig. 4) which occurred within a few days of starting weightbearing. This shortening does not upset the patient, who has a heel raise.

Other

Experience

Other elderly patients have been treated by this method, and we have discovered that care has to be taken to wear the spica properly to avoid the limb "rolling-out" at the fracture site, causing disabling external rotation.

I

303

"Unstable" fractures3 collapse into varus within a few days of weightbearing, but this can easily be treated with a heel raise, and the malunion is accepted. An abductor muscle inefficiency is seen but patients tolerate this. Sacral pressure points need relief at night by removal of the splint. Both legs are then nursed in a polyurethane foam gutter backslab, and this is quite comfortable. The appearance of the splint may worry an elderly patient, but this can be overcome by explanation and encouragement. The splint usually has to be used for only a month, until clinical union is achieved. Discussion There are several advantages to this form of treatment. Rehabilitation can begin as soon as the diagnosis has been made. An orthopaedic bed is vacated. Immediate mobilisation can be achieved without an operation, and this could be life-saving in unfit patients. Postoperative hypoxia and other anaesthetic problems are eliminated, so the mental and

Hypothesis IS WILSON’S DISEASE CAUSED BY A CONTROLLER GENE MUTATION RESULTING IN PERPETUATION OF THE FETAL MODE OF COPPER METABOLISM INTO CHILDHOOD?

O. EPSTEIN

S. SHERLOCK

Department of Medicine, Royal Free Hospital, Hampstead, London NW3 2QG

Pond Street,

Wilson’s disease is an inborn error o: copper metabolism, characterised by raised liver-copper concentrations and low serum levels of copper and caeruloplasmin. The autosomal recessive mode of in heritance strongly suggests that mutation of a single gent causes the impairment of both caeruloplasmin synthesis and biliary copper excretion. The normal infant is born with the biochemical features of Wilson’s disease (very high livercopper levels and low serum copper and caeruloplasmin). In duction of normal copper metabolism after birth results in a fall in liver-copper concentrations and rise in serum caeruloplasmin. The repression of normal copper metabolism in the fetus and its induction after birth is probably regulated by a controller gene. It is suggested that mutation of a controller rather than a structural gene underlies the pathogenesis of Wilson’s disease and that the disease results from failure tc switch from the positive copper balance of the fetus to the normal copper balance of the child.

Summary

INTRODUCTION

WiLSON’s disease is an inborn error of copper metabolism caused by an autosomal recessive genetic defect.’ The autosomal recessive mode of inheritance strongly suggests that a single gene defect is responsible for the increased livercopper concentration and reduced total serum copper and caeruloplasmin levels which characterise the disease.’ There is evidence that the copper accumulation is due to impairment of caeruloplasmin synthesis and biliary copper excretion,’ and attempts to explain the pathophysiology of

physical impairment met with in some hip fracture patients managed surgically will be avoided. The psychological upset, for patient and family, of an operation is also avoided. Simultaneous treatment of other medical problems can be continued while the fracture repairs. The splint is reusable after simple washing and can be used in other circumstances (e.g., fracture of femoral shaft). Further research and development of this splinting technique is under way in the Robert Jones and Agnes Hunt Hospital, Oswestry, and at the University of Liverpool group of hospitals. Requests for reprints should be addressed to: J. H. P. Orthotic Research and Locomotor Assessment Unit, Robert Jones and Agnes Hunt Orthopaedic Hospital, Owestry, Salop SY10 7AG. REFERENCES 1.

Grimley Evans J, Prudham DE, Wandless I. A prospective study offractured proximal

femur: Incidence and outcome. Publ Hlth, Lond 1979; 93: 235-41. 2. Asher R. On the danger of going to bed. Br Med J 1947; ii: 967. 3. Evans EM. The treatment of trochanteric fractures of the femur. J Bone 31B: 190.

Jt Surg 1949;

Wilson’s disease have assumed that a single structural gene mutation causes both these biochemical defects.’ It has been suggested that a hypothetical liver-cell carrier protein presents copper both for incorporation into caeruloplasmin and for excretion into bile. 1,2 A structural gene mutation resulting in an abnormal carrier protein might then account for both impaired export of copper from the parenchymal liver cells and accumulation of copper in the liver. This hypothesis fails to account for evidence that caeruloplasmin synthesis and biliary copper excretion are independent pathways,’ deriving their copper from separate pools.3 Furthermore there is no positive evidence to support the concept of a common carrier protein. An alternative hypothesis is that Wilson’s disease is caused by a lysosomal defect, resulting in impaired excretion of lysosomal copper into bilge.4 This hypothesis does not account for the impaired caeruloplasmin synthesis which characterises the disease. Finally, the observation that oestrogen administration may increase caeruloplasmin levels in Wilson’s disease suggests that the structural gene responsible for caeruloplasmin synthesis is intact and may be stimulated by hormonal manipulation.5 COPPER METABOLISM IN THE HUMAN FETUS AND NEONATE

The liver is responsible for maintaining normal copper balance. Dietary copper is transported in portal blood to the liver, where it is incorporated into copper-containing proteins (especially caeruloplasmin), and excess copper is excreted in bile. In patients with chronic cholestasis (e.g., primary biliary cirrhosis) interruption of biliary copper excretion is complicated by retention of copper in the liver, associated with raised serum copper and caeruloplasmin levels.6 The normal human neonate also has very high livercopper concentrations; 7-9 serum copper and caeruloplasmin levels, however, are greatly reduced" (see figure). The biochemical copper profile of the normal human neonate is indistinguishable from that of a patient with homozygous Wilson’s disease. In both, liver-copper concentrations are greater than 250 pg/g dry weight (normal adult, less than 55 g/g) and serum copper and caeruloplasmin levels are low’, 10 (see figure). As in Wilson’s disease,’ the amount of copper in neonatal serum is out of proportion to the amount of caerulo-