DEVELOPMENTAL MEDICINE AND CHILD NEUROLOGY. 1962, 4

4. Yerganian, G., Leonard, M. J. (1961) ‘Maintenance of normal in-situ chromosomal features in long- term tissue cultures.’ Science, 133, 1600.

5. Taylor, J. H. (1960) ‘Asynchronous duplication of chromosomes in cultured cells of Chinese hamster.’ J. biophys. biochem. Cytol., 7,455.

6. Morishima, A., Grumbach, M. M., Taylor J. H. (1962) ‘Asynchronous duplication of human chromo- somes and the origin of sex chromatin.’ Proc. nut. Acad. Sci. (Wash.), 48,756.

7. German, J. L. I11 (1962) ‘Synthesis of deoxyribonucleic acid during interphase.’ Lancet, i, 744. 8. Gilbert, C. W., Muldal, S., Lajtha, L. G., Rowley, J. (1962) ‘Time-sequence of human chromosome

duplication.’ Nature (Lond.), 195, 869. 9. Lyon, M. F. (1961) ‘Gene action in the X-chromosome of the mouse (Mus musculus L.)’ Nature (Lond.),

190,372. 10. - 1962) ‘Sex chromatin and gene action in the mammalian X-chromosome.’ Amer. J. hum. Genet.,

14, 135. 11. Edwards, J. H., Harnden, D. G., Cameron, A. H., Crosse, V. M., Wolff, 0. H. (1960) ‘A new trisomic

syndrome.’ Lancet, i, 787. 12. Patau, K., Smith, D. W., Therman, E., Inhorn, S. L., Wagner, H. P. (1960) ‘Multiple congenital anomaly

caused by an extra autosome.’ Zbid, p. 790. 13. Grumbach, M. M., Marks, P. A., Morishima, A. (1962) ‘Erythrocyte glucose-6-phosphate dehydro-

genase activity and X-chromosome polysomy.’ Ibid, p. 1330. 14. Beutler, E., Yoh, M., Fairbanks, V. F. (1962) ‘The normal human female as a mosaic of X-chromosome

activity: studies using the gene for G-6-P. D.-deficiency as a marker.’ Proc. nut. Acad. Sci. (Wash.), 48, 9.

15. Stem, C. (1960) ‘The Fifth Huskins Memorial Lecture. Dosage compensation-development of a concept and new facts.’ Canad. J. Genet. Cytol., ii, 105.

16. Mann, J. D., Cahan, A., Gelb, A. G., Fisher, N., Hamper, J. Tippett, P., Sanger, R., Race, R. R. (1962) ‘A sex- l ied blood group.’ Lancet, i, 8.

17. Kalmus, H. (1962) ‘Distance and sequence of the loci for protan and deutan defects and for glucose-6- phosphate dehydrogenase deficiency.’ Nature. (Lond.), 194,215.

18. Adam, A. Sheba, C., Race, R. R., Sanger, R., Tippet, P., Hamper, J., Gavin, J. (1962) ‘Linkage relations of the X-borne genes responsible for glucose-6-phosphate dehydrogenase and for the Xg blood-groups. Lancet, i, 1188.

19. O’Brien, J. R. Harris, J. B., Race, R. R., Sanger, R., Tippett, P., Hamper, J., Gavin, J. (1962) Haemophilia (WI) and the Xg blood-groups.’ Zbid, p. 1026.

CHILDREN IN HOSPITAL WITH MOTHERS ‘ALL who have to look after sick children in hospital should learn not only how to deal with the child’s ailment but also how to meet his emotional and other needs.’ The Platt Report on the Welfare of Children in Hospital, from which this quotation comes (Para. 137), recommended that visiting in children’s wards should be unrestricted, and that mothers should be admitted with their young children to share in their care and prevent the distress of separation. The Ministry of Health accepted the Report and recommended it to all hospitals, but it has largely been disregarded and there are comparatively few children’s wards where either of these recommendations is practi~ed.~

The admission of the mother is especially desirable for children under the age of five years. There is good evidence that such children are commonly distressed while in hospital and disturbed in behaviour for weeks or months afterwards.* Bringing the mother in with the child is good for her too; it enables her to help him at a time when she knows he wants- and needs-her help; and she gains confidence in her capacity to help him in sickness as well as in health.

Some older children, especially those handicapped by mental subnormality, cerebral palsy and other defects, are emotionally infantile and show it by their need for their mothers when in hospital. In such cases the mother can often give the nurses valuable guidance on the best ways of caring for this child and for similarly handicapped children.

The experiences of DERMOD MACCARTHY and his colleagues1 with 1,350 mothers admitted with their children during 11 years to the children’s wards of two hospitals in Buckinghamshire are therefore a welcome addition to our knowledge in this field. They outline what the mothers do, how their families fare at home, how the sick child reacts,

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and the benefits accruing to mother, child and hospital staff. A section on difficult mothers highlights many of the anxieties and tensions we see in parents and how they can be relieved. It is encouraging to read that the nurse’s attitude can be not only to help and teach the mother but to learn from her too.

The experiment of admitting mothers of young children, begun by the late Prof. Sir JAMES SPENCE in Newcastle in 1925 and ever since an accepted part of paediatric practice in his Unit there, was slow to spread, and it is only in the last 10 to 15 years that a few other wards in the British Isles have begun the practice. One hopes the idea will spread widely and ultimately become universal, but care is needed if the custom is not to fall into disrepute through lack of insight into the details involved. The study groups of the ‘MCCH’ (Mother Care for Children in Hospital), now springing up all over the country4, will remind hospital authorities and doctors of what they can do to further this highly desirable end.

Considerable thought is needed to make the mother-and-child admission a satisfying one. Among other points to be settled are how, when and by whom the mother’s admission is suggested, how much she should be influenced in her decision, the details of the reception of child and mother on their arrival, the arrangements for the mother to receive visitors, do her washing, telephone, talk to other mothers, smoke, and take time off. The welfare of the family at home needs daily review. When distance permits, the mother should visit her home while the patient is asleep or when another relative or friend can sit with him. If the distance is too great, the father may bring the other children to visit, and sometimes the mother and the patient may go home for the week-ends.

The sister in charge of the ward is the key figure in the scheme, for on her attitudes and judgments those of the other members of the hospital staff will depend. She should enjoy caring for the child and his mother as a unit, recognising without sentimentality their mutual depzndence. She will constantly boost the mother’s morale, encourage her to play a part in caring for the child and increase her compztence, to prevent her feeling that ‘the experts know best’ about ordinary care and mothering. At the same time, by engender- ing absolute confidence in the medical and nursing skills used in diagnosis and treatment, she and her colleagues can do much to support the parents in their fears and anxieties.

JAMES ROBERTSON’S two films,5 and his book, Young Children in Hospital,6 published four years ago, did much by setting out good evidence of the children’s need to have their mothers with them. His new book, Hospitals and Children: A Parent’s Eye View,’ is a valuable summary of some hundreds of letters by parents about their children’s stay in hospital, followed by comments. It reveals striking contrasts in patient care in different parts of the country, and even in different wards of the same hospital.

Let us hope that by the end of this decade the admission of mothers with their young and/or handicapped children will be normal practice throughout the country. DONALD GARROW’? figures of the numbers of mothers admitted to the Victoria Hospital, Tite Street, with their children since October 1958 show that, given the will, the act can be achieved:

Years 1958 1959 1960 1961 Mothers Admitted with Children 14 76 134 148

CHRISTINE COOPER REFERENCES

I . MacCarthy, D., Lindsay, M., Morris, I. (1962) ‘Children in hospital with mothers.’ Lancet, i, 603-607. 2. Garrow, D. (1962) Letter on above. Zbid, pp. 857-858. 3. Kidd, H. B. (1962) ditto. Ibid, p. 1023.

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4. Further information on the M.C.C.H. from the Organising Group Secretary, Mrs. Valerie Elder, 12 Albany Mansions, Albert Bridge Road, London, S.W.11.

5. Robertson, J. (1952) Film: A Two-Year-Old Goes to Hospital. 40 mins., sound. London: Tavistock Clinic, (1958) Film: Going to Hospital With Mother. 40 mins., sound. London: Tavistock Clinic,

6. - Robertson, J. (1958) Young Children in Hospital. London: Tavistock Publications. 7. - (1962) Hospitals and Children: A Parent's Eye View. London: Gollancz. 8. Vaughn, G. F. (1957) 'Children in hospital.' (Letter.) Lancet, i, 1117-1120.

WILSON'S DISEASE DURING the past few years considerable attention has been focused on the condition described by WILSON~~ in 1912 and named after him. At that time the clinical picture described was largely one of neurological disorder in late adolescence with an early fatal termination. Since then there have been many changes in thought, and as WALSHE~~ has recently shown, almost as many cases present with symptoms of liver disease as with neurological signs, and the liver manifestations are commonly seen in young children. This was suggested by CHALMERS, IBER and UZMAN~ in the U.S.A. and was also noted in England, at The Hospital for Sick Children, Great Ormond Street, and The National Hospital, Queen Square, and by SASS-KORTSAK et al.13 in Canada, all of whom found examples of Wilson's disease in children with cirrhosis of the liver. There now seems little doubt that a number of children with cirrhosis do have unrecognised Wilson's disease.

How can this condition be recognised? From the clinical aspect a slit-lamp examination should be made, for in most if not all cases this will demonstrate a Kayser-Fleischer ring. The case described recently by WARREN and BROUGHTON~~, the cases at Great Ormond Street, and those reported by CUMINGS~ all showed this abnormality, and SILVERBERG and GELLIS" have lately reviewed the clinical and biochemical findings in 12 children seen in Boston, U.S.A., of whom 8, between the ages of 7 and 14 years, presented with hepatic manifestations, while at some stage of the disease Kayser-Fleischer rings were present in all. However, a very few patients out of the several hundreds on record have not shown this most valuable sign, perhaps because adequate use was not made of the slit- lamp technique or because they were examined before the ring had developed. Other rarely occurring features, consisting of osteochondritis dissecans and renal tubular reabsorption defects warranting a diagnosis of the Fanconi syndrome, have been described recentlys.

Most of the recent work on this disease has been biochemical (see WALSHE and CUMINGS~~). Of the investigating procedures that have been described (for details see BOUDIN and EPIN~ and CUMINGS~) three methods could be used for diagnosis, all of which depend on the fact that this disease is characterised by an abnormality of copper metabolism. The blood copper is reduced from the normal figure of around 100 pg. to between 40 and 60 pg. per 1 0 0 mL7, the caeruloplasmin level of the blood is almost zeroI4, and there is an increased excretion of urinary copperlo. Most cases demonstrate all three abnormalities, but in a few patients some or all of them are absent. Normal levels of blood copper have been found" and a normal caeruloplasmin content has been re- corded7. 11, 12, 15, though in some of these patients both these biochemical features became abnormal in the course of the disease. The urinary copper excretion has always been increased, except in one recorded case4 where this abnormality was not found until later. OSBORN and WALSHE~ have suggested that abnormality in the liver uptake of 64Cu will be useful in the diagnosis of doubtful cases, and STERNLIEB, MORELL and SCHEINBERG~' have found a rather similar technique valuable. This type of examination may be of considerable use in resolving one of the very difficult problems connected with this disease.

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