DEFICIENCY OF AN EXTRA-THYROID ENZYME IN SPORADIC CRETINISM

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    tative voice, and we are grateful to you, Sir, for the leadyou have given in your columns.

    I regret that I feel obliged to sign myself as yet ANOTHER SENIOR REGISTRAR.

    1. Querido, A., Stanbury, J. B., Kassenaar, A. A., Meijer, J. WJ. clin. Endocrin. 1956, 16, 1096.

    DEFICIENCY OF AN EXTRA-THYROID ENZYMEIN SPORADIC CRETINISM

    A. QUERIDO.University Hospital,Leiden, Holland.

    SIR,-I was interested in the article by Dr. Hutchisonand his colleagues (Aug. 17). A valid clinical observationshowing that hypothyroidism may be unresponsive tothyroid powder and react favourably to treatment withtriiodothyronine would provide important informationupon a controversial issue.The step from there to a conclusion that there is a

    deficiency of extrathyroidal enzymes, however, requiresmore direct evidence. An explanation would be neededfor the low pretreatment serum level of protein-bound 127 Iif it is to be assumed that thyroxine is producednormally. Further, one would like to be informed aboutthe serum-p.B.I.127 level obtained in the course of dry-thyroid therapy, for proper assessment of the efficacy ofsuch therapy in terms of intestinal absorption. It seemscurious that large doses of thyroid powder, which wouldpresumably contain traces of triiodothyronine, should beineffective.

    Lastly, it is my belief that absence of enzyme activity,let alone absence of the enzyme itself, may be postulatedwith some confidence only if its presence cannot bedemonstrated in tissues where it can be shown to bepresent in normal people. In this department it hasbeen possible to do so for dehalogenase, first in one patientand recently again (unpublished) in a member of anotherfamily, so that we now feel confident about the validityof the postulated nature of this particular form of hypo-thyroidism.

    SiR,The account by Dr. Hutchison and his col-leagues of an example of cretinism relieved by triiodo-thyronine but not by thyroxine prompts me to record aparallel case which I have been able to observe closelywithout the opportunity for proper clinical investigation.An unmarried woman in her late thirties had suffered for

    many years from a variety of symptoms, most of them vagueand transitory. Ubiquitous aches and pains, sensations ofpressure or constriction especially around the throat, weaknessaffecting various parts of the body, and transient inability toread were complaints at different times and were described ina highly coloured and fanciful manner. She mentioned alsothat she had once had a complete paralysis of her legs for somedays. It was not difficult to diagnose hysteria.I A few further signs, however, were observed. She was thinwith a poor appetite, constipated, very intolerant of coldweather, and had perpetually cold hands ; she was slow inaction and thought, her skin was dry, and her hair tended tofall out ; menstruation was irregular.A trial of dry thyroid (gr. 1/2 daily), far from improving her

    condition, made her, within a few weeks, distinctly worse.Headache, depression, and weakness were the predominantcomplaints and she spent longer in bed each day. A completebreakdown seemed imminent.A switch was then made to triiodothyronine starting with

    20 g. daily and increasing to 80 [Lg. Improvement was rapidand dramatic and has been maintained on the latter dose foralmost a year. Not only are the somatic elements of hercondition relieved but her whole personality has changed.She is now alert, industrious, and cheerful. Her weight hasincreased and her periods are regular. She seldom has anycomplaints but those she has-an occasional headache, forexample-she now describes in an objective and rationalmanner, totally unlike her previous exaggerations and fan-tasies.

    It is of interest that her mother is stated to be lackingthyroid and a brother is mentally abnormal, but two

    sisters are apparently normal. The condition suggeatsa Fdefect, possibly familial, in the deiodination of thyroxine, tless severe than in the case described by Hutchison etal,,

    I

    but compatible, perhaps, with the syndrome of "M.myxoedematous hypometabolism

    " described by Kmb et al.2 It is a pity that circumstances prevent a fullinveatiaa.tion of the metabolic ahnormalitv in this case,

    H. E. M. KAY.Royal Marsden Hospital,London, S.W.3.

    2. Kurland, G. S., Hamolsky, M. W., Freedberg, A. S. Ibid.1955, 15, 1354.

    * The Payer Hut is named after Julius von Payer, who in 1865.together with Dr. von Mojsisovic.-;, ascended to the summit ofthe Ortler by a new way.

    HEXYLRESORCINOL IN THE TREATMENT OFARTHRITIS

    S. HILLMAN.

    SiR,I was glad to see Dr. Lipkins work on thissubject published in your journal of Aug. 24. 1 wafprivileged to see some of the results of his treatmentand*they were undoubtedly impressive.

    I have followed this regime in a number of my puttedof whom there are too few as yet to report, but I feelextensive trial of this treatment is merited.

    HYPOGLYCMIC ATTACKS IN NORMALINDIVIDUALS

    Sm,-I think I should have enlarged the title of thisletter to " hypoglycsemic attacks in normal individiialsinduced by unusual (if not actually abnormal) beha.viour." The following is an example :

    In 1925, I was having a holiday with my wife at Sulden(Soldano). One morning I arranged to cross the Suldenvalley and mount the ridge which separates it from theTrafoi valley. This ridge forms part of the base of theOrtler mountain and leads on to the left to the Payer Hut,through which nearly everyone passed on their way to theOrtler summit. When I reached the ridge, it was only a short,almost level, walk to the hut, but I suddenly felt almostpowerless, and had to lie flat on my back hoping that someonewould pass that way. But no-one came, and after a time Icrawled along the path, with my legs feeling like lead, to thehut, where I knew I could get provisions. Hot tea withmilk, alcohol, and plenty of sugar made me in a few minutesa different being. I felt in such good spirits that I descendedon the other side of the ridge to Trafoi and was able to sharea motor-car with a German lady and her son around the baseof the mountain, back to Sulden, which I reached about8 r.r.

    Recently, after going over these data with my friend,Dr. Hugo Rast, I came to the conclusion that the attackjust before reaching the Payer Hut was almost entirelyof hypoglycaemic origin, and could have been preventedhad I had sandwiches, a pocket cognac flask, sugar orglucose with me-or had I made use of an excellentrestaurant-hut which I passed on the way.The experience gained by this incident did not prevent

    me, under pressure of circumstances, from makingmistakes in the arrangement of excursions, leading todistressing results, which I now regard as of more or lesshypoglycaemic origin. Indeed I consider myself as anexpert in arranging conditions which lead to attacksalmost certainly partly of hypoglycaemic origin, thoughless typical than the example which I have just described,but it would be tedious to enter into the details of eachcase as thoroughly as would be necessary.My conversation with the German boy and his mother on

    the way back to Sulden, made me almost as enthusiastic asthe boy himself to get to the summit of the Ortler on thenext really fine morning. Two or three days later I wasfortunate enough to obtain the service of the last availableguide at the Payer Hut (no guide was allowed to take morethan one person). He rushed me to the summit, and likemost others I was so breathless that I could not really close