dwarfism associated with dystrophy of bones and muscles

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Page 1: DWARFISM ASSOCIATED WITH DYSTROPHY OF BONES AND MUSCLES

551

distance of 40 years we find the pathetically faithful servant,now become conservator, able to sketch from memory atdead of night-his tiny pretty plan being dated 2 A.M.,Nov. lst, 1832-every little detail in the once-adored

museum house. Thus he has set down such delightfultrifling matters as the situation of Mrs. Hunter’s sedan-

chair, of Mr. Hunter’s "afternoon bedroom or sofa bed,"" great red japanned army chest" between the windows ofthe "House pupils’ room," pictures such as "Harlot’s

and Rake’s Progress," drawbridge, yard for washing whaleand carriage, and books, prints, and "dry preps. patho-logical." The plan gives a good idea of an eighteenthcentury museum, and we are struck by certain very moderntouches. Thus, Hunter’s desk was of the slide-top kind,and the theatre and other rooms were warmed-very badly,says Clift-by hot air from two of Jackson and Moser’s

stoves. Of the backyard it is recorded that no grassgrew," and that it was called the garden"; there werealso deep areas, as there might be now. Clift has left

one of his careful MSS., containing minute details as

to the expense of shifting the Hunterian Museum in

wagons from Leicester-square to Lincoln’s Inn Fields.Another of his small sketches represents the Museum in1810. This was admirably reproduced on a large scale toillustrate the Hunterian Oration this year, and though it

shows dignified vistas of vaulted roofing it is hard to discoverwhere the specimens were shown. We know, however, thatClift devoted his laborious life to the setting out of the fineHunterian specimens, contained in red-stoppered bottles, ofwhich there remains Clift’s MS. list. The same indefatigableworker alludes, in his curious unpublished year books of

work done, to a forgotten function of the College, thefitting out of the scientific side of several famous Arctic

expeditions. Of these expeditions only printed records

remain in the College library, but at the presenttime it would be of great public interest if anyrelics of Ross, Franklin, and others could be unearthed

and exhibited. Captain Cook’s little MS. log-books, for

instance, now form part of the priceless Marsden Libraryat King’s College, London. In these appears the list ofthe names of the crew of Cook’s ship according to theOtahitian pronunciation. The Captain himself figures as"Tute," and by this name recent travellers tell us he is

still known by the descendants of the men who murderedhim because they loved him so well that they wished toretain his divine ghost in their midst. Museums and

libraries, in fact, are romantic through the associations oftheir contents, among which lurk unconsidered details

often strangely confirmatory of fact observed at the ends

of the earth. __

UNUSUAL ILLNESS AMONG WEAVERS OF

COTTON CLOTH.1

CASES of primary aspergillosis are very rare, and in anaccount of illness affecting weavers in four sheds in

Lancashire Dr. E. L. Collis suggests that this was the

most likely cause. The symptoms developed very acutelywith febrile disturbance, asthmatic attacks, and charac-

teristic cough. The process common to all the weavingsheds in question differed from the usual manufacture ofcotton cloth in that addition of the antiseptic zinc chloride(usually added to prevent occurrence of mildew on thecloth at a later stage) was omitted, as dyers specify thatno chlorides shall be present in the cloth. Obviouslycotton threads passed through size and quickly wound, while Istill containing moisture, on to the warp beam, furnish

1 Report on Unusual Illness among Weavers of Cotton Cloth. ByE. L. Collis, M.B. Oxon., H.M. Medical Inspector of Factories. London:Darling and Sons, Limited. 1913.

conditions favourable to the development of moulds-Under such circumstances mildew is not infrequently seen,but in these cases more attention seems to have been giveto the unpleasant odour given off from some of the warps,suggesting the possibility of the liberation of gas, than-to the isolation of the fungi present. (Di-etbyl-arsine-liberated when penicillium brevicaule grows on arsenical

wall-papers is an example quite to the point.) Neither-the symptoms among the weavers nor chemical analysis,however, lent support to the view that injurious gases.were a cause of the trouble. No unusual mould was.

found in the sputum, so that it is only by inference

that the theory of aspergillosis was arrived at. The remedyfor the condition which has proved effective-addition of’formaldehyde to the size-also favours it. Primary pnenmo-mycosis is noted by Rolleston and Latham, in accordance-with the description of Chantemesse and Widal, as an

occupational disease among two very small classes of

persons in Paris : (1) those making a bolus of seeds in themouth as a preliminary to artificially feeding pigeons ; and(2) hair sorters who inhale the flour of rye used to enable,them to separate the hairs more readily. Both aspergillns.fumigatus and aspergillus niger are parasitic and believed to-produce morbid changes in the human body. In this con-

nexion a remarkable account is given in the December issue’of the Joacrnal of Hygiene by G. Massee, of the Jodrell Labora--tory, Kew Gardens, of a fungus, cladosporium herbarum,which produces black spots on the surface of chilled meat.In its adaptation to this unusual substratum the fungus has.so far departed from the normal type of species that thetypical form of reproduction by spores has been bandonedand has been replaced by a secondary and lower form oi.

reproductive bodies-chlamydospores-immersed in the sub--stratum produced from specialised cells of the vegetative-hyphas of the fungus.

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DWARFISM ASSOCIATED WITH DYSTROPHY OFBONES AND MUSCLES.

ABOUT a year ago M. Hutinel, of Paris-, gave a de--

scription of a peculiar form of retardation and perversionof growth which he had been able to separate from the-crowd of indefinite cases of malnutrition attending hishospital clinic. Since that time-indeed, within a few

months-Dr. Leon Tixier and Dr. Carle Roecaerer have’

observed four examples of this form of dwarfism, which:are briefly recorded, with a summary of the chief features>of the condition, in La Pre$se ff-r4dicale of Feb. lsto-Three of their four patients were members of one-

family--indeed, of the same generation of that family;.their mother showed traces of bony abnormalities similar-to those exhibited in grosser form by her children. The

most obvious of these abnormalities is inadequate stature,the patients falling short of the average height by 10 or-20 per cent. This is partly due to curvature of the long-bones and of the spine, partly to retardation of the ossificatory-process by the abnormal state of the diaphyseal eairtilage.The first symptom noticed, however, is difficulty in learn-ing to walk ; this is accompanied by an antero-esternaYcurvature of the tibise. In the arms a similar deformity-is present, though it is less pronounced. The femora and.

humeri are also curved outwards, and there is marked,

genu valgum. Scoliosis is a constant and striking:feature ; with it there is a lordotic curve in the-

lumbar region, and the usual secondary changes in,

the thorax and pelvic and pectoral girdles. In these

as well as in other respects the bony changes closely-resemble those of rickets ; as in the commoner disorder,-there are epiphyseal thickenings, and the frontal bones are-prominent. Radiographic examination, however, reveals-

Page 2: DWARFISM ASSOCIATED WITH DYSTROPHY OF BONES AND MUSCLES

552

several features which seem to hint at a different type ofmorbid process being responsible. There are evidences of

.attempts at repair, the concavities of some of the curves

’being reinforced by thickening of the bone. The medullary’canal of the long bones is large, as in rickets ; but its out-llin-e (as seen in the radiograph) is irregular, as in syphiliticosteitis. In the cases recorded by Dr. Tixier and Dr. Rosderer’the Wassermann reaction was negative, and there was noreason to suspect a syphilitic basis. In one importantrespect the skiagraphic appearances recall those of osteo-

’malacia ; the shafts of the long bones cast a stippled shadow,apparently due to a disorderly commixture of non-calcifiedwith normal areas. The muscles are generally weak andexhibit a diffuse dystrophy; this is too definite to be

regarded as a result of the child’s immobility, and must belooked upon as a primary feature of the disorder. The

intellect is often feeble, and the evolution of the mental í,powers retarded ; in one case there was obesity, in others I

the children were wasted. In one instance sexual develop- 1

ment was delayed. Apart from the possibility of the

’hereditary factor alluded to above, nothing is clear as to thecausation of this singular affection ; neither has there beenany appreciable benefit from any particular line of treat-,ment. It is possible, of course, that other critics may findthe separation of this condition from rickets an arbitrary.a,nd unjustifiable one; yet in its later development and in:some other features its claim to individuality does appearireasonably well founded.

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A FLANNELETTE BILL.

A BILL has been introduced into the House of Commons;and read for the first time, entitled ° ° A Bill to deal with

Misdescription of Fabrics," the object of which is to prohibitthe sale of flannelette or any other textile fabric describeda,s being non-inflammable or safe unless it conforms to suchstandard of non-inflammability as may be prescribed byTegulations to be made by the Secretary of State. It

may be remembered that in 1910 a report was issued

by a Home Office Departmental Committee upon the

inquiry into the question of the danger arising from the saleof flannelette for articles of clothing, and legislation wasrecommended making it penal to describe as uninflammablematerial which will not stand certain prescribed tests. The

Bill is based upon this finding, although the DepartmentalCommittee expressed the opinion that the question of mis-representation could be dealt with under the provisions ofthe Merchandise Marks Act, 1887. It has since been decided,’however, that a separate Bill was necessary, as there weredifficulties in the way of including a special matter such asthat of the inflammability of fabrics in any existing Act.’The question as to whether or not a fabric is safe or non-

inflammable can, of course, only be decided by test, and thismeans the setting up of a standard, and here there will

be some trouble in regard to getting a general endorsementfrom all concerned as to its efficiency or fairness. Weourselves made an attempt to lay down a standardmethod of testing which we considered would, if

adopted, afford sufficient protection. It is required to know,,in our opinion, what is safe and not what is non-

inflammable, and that consideration led us to employ atest which may have passed inflammable materials, but

which readily discriminated the reasonably safe from theobviously dangerous. The ordinary linen or calico nightgarments are, of course, inflammable, but their liability tocatch fire and the spread of the flame is not of the alarmingaiature experienced with the common forms of flannelette.But there are superior flannelettes which are as reasonablysafe as linen or calico. The Departmental Committeereferred to were adverse to certain suggestions which had

been made in favour of precipitate and drastic legislation,for they held that " legislation of the kind suggested isthe less justifiable in view of the fact that matters may incourse of time be expected to right themselves." Again,they reported that " great improvement has already beenmade in the manufacture of flannelette, and further

improvements may be anticipated"; and finally, theyexpressed the opinion that there is also the possi-bility that new and cheaper methods of diminishingthe inflammability of the material may be discovered.’’It is, we think, unquestionably the case that the cheap andclearly unsafe flannelette has been responsible for bringingdisaster to many helpless little ones, and the fact thatsuch flimsy material can be sharply discriminated from thereasonably safe fabric should render the adoption of a pro-tective test conducted under practical conditions feasible,workable, and fair. With a standard imposed, we thinkthat good will come of the Bill, for it is obvious that themanufacturer who is not honestly able to stamp his goodsas "safe" " will eventually have to make room for thosewho can.

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NON-MALIGNANT CANCER OF THE BREAST.

THAT cancer is a progressive disease leading to death,except when it has been completely extirpated by opera-tion, is so generally true that the exceptions are overlooked.Many well-authenticated cases of spontaneous recovery fromcancer are now on record, and the possibility of this

occurring is forgotten by those who claim recovery fromsome medicinal treatment. Another and even less re-

cognised exception to the rule that cancer is a fatal diseaseis the condition called" non-malignant cancer," an expres-sion which at first may seen a contradiction in terms, butthe meaning of which will be plain if it is rememberedthat it is the structure of a growth and not its destructiveeffects which determines whether it is to be classed as a

cancer or not. Some years ago at the Pathological Societyof London Dr. S. Bontor reported, under the title of Non-malignant Cancer of the Breast," the case of a woman whodied at the age of 77 years, and had a growthin the left breast for 23 years.1 It never ulcerated

or caused pain, but latterly there had been some in-

convenience from a sense of constriction of the chest.

There were several secondary deposits in the rightbreast and in the skin of the abdomen, but there were no

enlarged glands or signs of involvement of distant organs.The cancerous nature of the growth was proved by bothmacroscopic and microscopic appearances. In the dis-

cussion which followed Sir Watson Cheyne referred to a

case in which a similar condition had lasted for 14 years andthen a sudden outburst took place. Mr. C. A. Ballance

mentioned the case of a woman who at the age of 70

developed carcinoma in both breasts. The disease had no

effect on her general health and she did not die until the ageof 82. He pointed out that carcinoma supervening in theaged is of slow growth. In the A2cstrctlian Medical Journalof Dec. 28th, 1912, Dr. M. Crivelli and Dr. A. J. Trinca havereported a case of carcinoma of the breast of 47 years’ dura-tion. The patient was a married woman who since thebeginning of the disease has been under medical observation.At one time the growth was declared inoperable and she wastold she had only a short time to live. She came under Dr.Crivelli’s care 20 years ago. She stated that she firstnoticed a lump in the right breast 27 years before. It com-menced in the region of the nipple as a hard nodule whichirew very slowly for about 10 years and never became veryprominent. During the next 35 years it remained more or

1 THE LANCET, Dec. 8th, 1900, p. 1652.