ACID HYPERPHOSPHATASIA IN THREE FAMILIES WITH OSTEOGENESIS IMPERFECTA

1084 in serum-cholesterol was greatest, on the first exhibition but was seen again second course. Insulin by itself failed to this level, although when’ carbutamide was given as well there was a drop. It will be noted that ketonuria was absent during this third course. Incidentally, and, this may be of significance, carbutamide was not wholly without effect on the diabetes as judged by the the abolition of ketonuria, and urinary-sugar the blood-glucose determinations. Moreover, it seemed to have a slight insulin-sparing action. The

drop

during the

drug’s

modify

output,

In two normal subjects given carbutamide in the same dosage no similar change in serumcholesterol was found. In the few diabetics studied for it specifically, the change was not produced by giving insulin alone. Furthermore, there was no change in one diabetic with mild glycosuria and a low-normal serum-cholesterol who responded well to carbutamide; nor again in another patient with moderate glycosuria and an elevated serum-cholesterol who showed no response to the drug. Does this change in the serum-cholesterol reflect, then, some fundamental difference in the types of diabetes studied!Or is it evidence that, compared with insulin, carbutamide has a different (or-at least morewidespread) effect on intermediarv, metabolism? We have to thank Dr. Ian Murray, under whose these patients were admitted to hospital, for helpful criticism and advice. One of us (D. M.) received a, grant from the British Insulin Manufacturers. care

at the age-of 56, a year before diabetic symptoms appeared. The diagnosis was not made, however, until immediately before the carbutamide trial, and he had had no treatment of any sort. On admission, the fasting blood-glucose level was 212 mg. per 100 ml. Slight ketonuria (1+) was present. He was free of detectable diabetic or vascular complications. Fig. 2 depicts the response to varying amounts of carbutamide. was

ACID HYPERPHOSPHATASIA IN THREE FAMILIES WITH OSTEOGENESIS IMPERFECTA

THERE do not seem to be (so far as I am aware) any observations on the acid-phosphatase level in patients with either osteogenesis imperfecta or osteopsathyrosis, which are usually considered, in English-speaking countries, to be different forms of one disease. I have investigated three families with the clinical and radiological picture of osteogenesis imperfecta : Icase 1, a man aged 40, and his two daughters, aged 5 years and case 3 aged 7 years. Family IIcase 4, a woman aged 35, and her two daughters, case 5 aged 2 years and case 6 aged 10 years. Family -TI-I : case 7, a woman aged 40, and her son, case 8 aged Iyears. In all 8 cases the alkaline-phosphatase and acid-

Family

case

2

levels in the blood-serum and- in -the whole blood were estimated by Bodansky’s modified method.! The alkaline-phosphatase levels (optimal activity at pH 84-100) were within normal limits (children, 5=15 Bodansky units in serum and 5-5 Roberts units in the whole blood; adults, 2-4 Bodansky units in serum and 5-5 Roberts units in the whole blood 2). The acid-phosphatase levels activity at pH 45-50) were much higher than normal in all the

phosphatase

(optimal

patients (see table). These cannot be fortuitous findings, because the results were controlled and confirmed. Presumably the raised acid-phosphatase level is due to abnormal osteoclastic activity. I base my hypothesis on the following facts : .

Fractures and wounds of heal well and quickly, blastic activitv is normal.

(1)

operations)

bone (after orthopaedic indicating that the osteo-

1. Levinson, S. A., MacFate, R. P. Clinical Laboratory Diagnosis Philadelphia, 1946. 2. Roberts, W. M. Brit. J. exp. Path. 1930, 11, 90.

I. B. MUNRO M.B. Department of Metabolic Diseases, The Victoria Infirmary,

Glasg.,

F.R.F.P.S.

D. MURRAY B.Sc.

Glasgow

Glasg.

(2) Radiography shows unusual enlargement of the spongy bone and thinness of the cortex. (3) The calcium and phosphorus levels are normal or even raised. (4) The acid-phosphatase level in the serum is raised in cases of carcinoma of the prostate metastasising to bones. New growths were excluded in all 8 observed cases. (5) In osteoblastic dysplasia the alkaline-phosphatase level falls.3 (6) The alkaline-phosphatase level in the serum is raised. in active rickets. In healing rickets it returns slowly to a normal value. In rickets the cartilage cells do not complete their normal cycle of proliferation and degeneration and do not undergo their normal degenerative change along the epiphysealmetaphyseal line.4 ..

3. 4.

Schlesinger, B., Luder, J., Bodian, M. Arch. Dis. Childh. 1955, 30, 265. Nelson, W. E. Textbook of Pediatrics. Philadelphia and London, 1954.

ACID-PHOSPHATASE LEVELS IN HEALTH -

INIPERFECTA

AND *

IN OSTEOGENESIS

1085 .

I hope that my remarks will stimulate other workers to elucidate the role of the raised acid-phosphatase level in the

pathogenesis

of

osteogenesis imperfecta. A. GEBALA

Pædiatric Clinic, Cracow, Poland

M.D.

Jagiellonian

Medical Societies ROYAL SOCIETY OF MEDICINE Treatment of Morbid

as also were the newer benactyzine and hydroxycompounds and the meprobamates. At St. Thomas’s Hospital, some patients had benefited from intravenous : acetylcholine and methedrine injections. In summary, Dr. Shorvon emphasised the variety of possibilities in tackling the problem of anxiety and the importance of knowing from what illness it stemmed. He thought that the development of effective treatment for anxiety in all its settings was still open to the psychiatrist of the future : indeed, psychiatry was still largely in tlie position of general medicine thirty years ago before the development of specific treatments. on

trial,

zine

.

,

Anxiety

THE section of psychiatry met on Nov. 13 to discuss the treatment of morbid anxiety. Dr. W. W. SARGANT, the president, was in the chair. Dr. E. A. BENNET defined psychotherapy as a coöperative psychological procedure in which one person sought to influence the health of another, and in which the phenomenon termed transference was of central importance. It was noteworthy that despite the thought and effort devoted to every form of psychotherapy over the past fifty or sixty years, no generally accepted method of treatment for morbid anxiety had been devised. Psychotherapy had in fact become more complicated, yet no objective techniques had yet been established, and the work of psychotherapists remained obstinately subjective. Most psychiatrists now opposed a frankly suggestive therapy because it approached the doctor-patient relation on the assumption that the doctor was more important than the patient. The coexistence of depressive symptoms with anxiety should be no deterrent to psychotherapy. Indeed, the familiar division of neuroses into hysteria, anxiety states, and obsessional states was inexact and deceptively simple. Hysteria might be said to occupy one end of a scale, obsessional states the other, and anxiety states an intermediate position. The hysteric met the present as though it were the past, the obsessional as though it were the future, while the patient with an anxiety state was rooted in the present and could not see either past or future in true perspective. The aim of psychological treatment should be the enlargement of the patient’s consciousness so that his morbid anxiety became comprehensible and he acquired a respect for his mind, conscious and unconscious. Dr. Bennet gave chief place in the treatment of morbid anxiety to dream analysis and the use of drawings and paintings, as well as the products of active imagination. With such treatment, interviews could be spaced out often weeks apart. Dr. H. J. SHORVON also gave pride of place to psychotherapy in the treatment of acute and chronic morbid anxiety. But the number of patients who clamoured for relief was much greater than the available means for psychotherapy could deal with. Therefore attempts were being made to develop a variety of physical- approaches. To decide which physical method was called for, it was essential to assess the patient’s previous personality, the intensity of symptoms, and whether his morbid anxiety was simply a manifestation of a so-called anxiety state or secondary to some other psychiatric disorder. Bromides might be of great value although they were liable to aggravate depression ; and while short-acting barbiturates had no superior in this sphere of sedation, their prolonged use might engender or exacerbate anxiety instead of relieving it. Chlorpromazine had not fulfilled the claims of its manufacturers : the anxious patient carrying on at work often disliked the drug because it slowed up his motor and intellectual functions without necessarily easing tension, and there was the .danger of complications, such as jaundice. A useful feature was its tendency to increase appetite. Other tranquillisers, such as mephazine and reserpine,,were still

New Inventions A NEW ACETABULAR REAMER FOR ARTHROPLASTY AFTER any

arthroplasty of

the

for

hip-joint

osteo-

arthritis, complications arise which can be caused by failure in the operative technique. Postoperative pain is common and can be related to inadequate clearing of diseased cartilage and bone, with insufficient smoothing of theirregular surface of the acetabulum. The prosthesis which subluxes and dislocates during the early postoperative stage of physiotherapy after the period of immobilisation, although it can be caused by a valgus deformity of the femor&l neck, can also be caused by inadequate deepening and restoration of an anatomically shaped acetabulum. To clear and remodel this area. an instrument must have the following qualities :. (1) An adequate and strong cutting edge which cuts to the full depth of the acetabulum and not around the margins

only. (2)

A

sharp projecting point

surface to maintain the

reamer

at the apex of the cutting it is being

in position while

rotated. (3) Some means of self-clearing of the cutting edge from the cartilaginous and bony debris, without the necessity for frequent removal of the instrument. (4) A means of collecting and removing all the debris without scatter or spread into the surrounding soft tissues, where heterotopic bone might be formed.

This new reamer satisfies these criteria more fully than other types. The bit or head fits into a standard brace

cutting, and the shaft is long enough to prevent any obstruction to the rotation of the brace handle. In

for

where there is much eburnation with sclerosis of the acetabular surface it is advisable to break through and to remove this with a gouge before reaming. The reamer is now being used in the orthopaedic departcases

ment of the

Royal Infirmary of Edinburgh.

It is manufactured

by Zimmer Manufacturing Co., Warsaw, Ind., U.S.A. Department of Orthopædic Surgery, ROBERT B. DUTHIE University of Edinburgh

CH.M.

Edin.,

F.R.C.S.E.