DERMATOGLYPHICS IN ECTODERMAL DYSPLASIA
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1093 and paromomycin (’ Humatin ’), 500 mg. every 4 hours, through a Ryle’s tube, together with 15% glucose solution by intravenous drip transfusion, and parenteral vitamins. After 48 hours on this regimen the depth of coma was unchanged. At this stage (i.e., 60 hours after the onset of deep coma, and 108 hours after the onset of drowsiness) an exchange transfusion was performed with 10 pints of fresh heparinised blood. While making the skin incision and doing the dissection to identify the long saphenous vein, no local anxsthetic was needed because of deep coma; but as the transfusion was coming to an end, the patient seemed to have some awareness of pain in the groin. Twelve hours after the exchange transfusion the patient, who had been lying absolutely still, became extremely restless and was restrained in bed with great difficulty. Plantar response remained extensor on both sides. Later the depth of coma gradually lessened and 30 hours after the transfusion the patient complained of headache. At that time he was still confused and not fully orientated. His confused state gradually improved and 38 hours after the transfusion he was able to obey simple commands and answer simple questions. At this stage the plantar responses became flexor. Improvement was rapid during the next few hours and 50 hours after transfusion he was mentally alert and answered all questions rationally. There- after his recovery was uneventful and he is now back at work, after a period of convalescence. Our experience is so far limited to this one case, but we feel that this form of treatment offers some hope in an otherwise hnmalPCe situation NAZIR A. CHAUDHARY NASEER AHMAD SHAIKH MUSHTAQ HASAN. Dow Medical College and Civil Hospital, Karachi, Pakistan. TRAINING IN MEDICAL LABORATORY TECHNOLOGY SIR The response in your correspondence columns to the statement from the Institute of Medical Laboratory Technology (Oct. 28, p. 935) reflects a widespread endeavour to maintain high standards of technology and efficiency. There is general agreement on a system of coordinated training conducted by experts and based on day or block release; and there should be close liaison between training institutes and working laboratories. However, laboratories must continue to function at all times as efficient working units, and the problem of providing full training facilities is linked with that of main- taining adequate work output of a high technical standard. Whether or not day release is made compulsory, there is little doubt that it is here to stay: it is also clear that the constraints imposed by regular staff absences can be overcome only by increases in technical establishments; but, because of present financial restrictions and shortage of labour, it is unlikely that early increases in senior establishments will come about or that, in any case, these would solve the immediate problem. In the recent correspondence, no account has been taken of those who are willing and capable of giving useful service in laboratories but who, for various reasons, have no wish to undergo training in depth or to acquire technological qualifica- tions. Much of the work of medical laboratories-clinical, academic, or reference departments-is elementary or repeti- tive, and much of this can be carried out by people with comparatively little training. Is it not time, as a matter of national policy, to encourage employment of a quota of semi- skilled labour in laboratories in order to permit greater flexibility in educational arrangements for those most suitable for advanced training ? Recruitment of such semiskilled labour should be based on recognised salary scales proportional to, but slightly less than, those of technicians in training; and increments could be based on age, length of service, and, possibly, degree of local responsi- bility. This suggestion in no way implies a lowering of technical standards. Most experienced laboratory workers are acquainted with technicians who are excellent at their daily work but pass examinations only with great difficulty, if at all. Surely, employment and recognition of such workers would plug some of the obvious leaks in technical establishments, because many who are now deterred by academic handicaps would be encouraged to enter laboratory services. Con- temporary proposals for technicians’ training are to be com- mended for their far-sighted aims and their optimism. But this optimism must be modified by the realities of the present situation. Only by paying due regard to the pressures on the lower echelons of laboratory workers will a future elite body of technologists be created. G. D. WASLEY R. FRASER WILLIAMS. Department of Medical Microbiology, St. Thomas’s Hospital Medical School, London S.E.1. DERMATOGLYPHICS IN ECTODERMAL DYSPLASIA SIR,—I wish to report briefly my dermatoglyphic analyses of two families with hereditary anhydrotic ectodermal dysplasia, since there seem to be findings common to affected individuals and carriers. In this X-linked disorder there are decreased numbers of sweat-glands in the skin, hypotrichosis, and anodontia. The carrier female usually has symptoms. Since the skin is involved, one might expect to find characteristic dermatoglyphic changes, as in this short series. In family 1 the father was clinically normal; the mother had thin hair, decreased sweating, and poor teeth; three sons were all severely affected. In family 2 the father was clinically normal; the mother had decreased sweating; three sons were severely involved and one son was normal. The dermato- glyphics of all these people were analysed; the affected ones had striking ridge disruption in all areas of palms and soles. There was also a tendency for the patterns to be vestigial, particularly in the third and fourth interdigital and plantar hallucal areas. The c and d digital triradii tended to be absent, vestigial, or displaced, and the C and D palmar lines were impossible to trace or stopped without going anywhere. The hypothenar patterns were usually too complex to describe by a single pattern name, and in addition the palmar axial triradius tended to be distal. The carriers (mothers) had some but not all of these patterns. The complex hypothenar patterns were present in both mothers. The normal people in this series had none of these findings. Thus dermatoglyphics may prove to be another clinical measure for diagnosis of patients with anhydrotic ectodermal dysplasia and of the carrier or less affected female. This work was aided by a grant from the National Foundation. JEAN H. PRIEST. Departments of Pathology and Pediatrics, University of Colorado Medical Center, Denver, Colorado 80220. INSULIN AND GLUCOSE RESPONSE TO GLUCAGON IN DOWN’S SYNDROME SIR,-Abnormal glucose tolerance has been reported 1-3 in patients with Down’s syndrome (D.S.). In children with D.S., other mentally retarded children, and normal children, we have found that the mean rise in plasma-immunoreactive- insulin level after oral glucose is only about half that observed in adults.4 Since glucagon has been shown to promote insulin secretion in adults,5 6 we decided to measure the response to it of these children. 16 children (aged 20 months to 6 years 4 months) were studied: 6 had D.s., 5 were mentally retarded from other causes, and 5 were normal. All were taking a high-carbohydrate diet containing 1000-2000 calories per day. After an overnight fast 0-5 mg. glucagon was given intravenously during 1-2 1. O’Leary, W. D. Am. J. Dis. Child. 1931, 41, 544. 2. Runge, G. H. Am. J. ment. Defic. 1959, 63, 822. 3. Benda, C. E. The Child with Mongolism; p. 175. New York, 1960. 4. Milunsky, A., Rubenstein, A. H., Lowy, C., Wright, A. D. Devl Med. Child Neurol (in the press). 5. Yalow, R. S., Black, H., Villazon, M., Berson, A. A. Diabetes, 1960, 9, 356. 6. Samols, E., Marri, G., Marks, V. Lancet, 1965, ii, 415.
Transcript of DERMATOGLYPHICS IN ECTODERMAL DYSPLASIA
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and paromomycin (’ Humatin ’), 500 mg. every 4 hours,through a Ryle’s tube, together with 15% glucose solution byintravenous drip transfusion, and parenteral vitamins. After48 hours on this regimen the depth of coma was unchanged.At this stage (i.e., 60 hours after the onset of deep coma, and108 hours after the onset of drowsiness) an exchange transfusionwas performed with 10 pints of fresh heparinised blood. Whilemaking the skin incision and doing the dissection to identify thelong saphenous vein, no local anxsthetic was needed because ofdeep coma; but as the transfusion was coming to an end, thepatient seemed to have some awareness of pain in the groin.Twelve hours after the exchange transfusion the patient, whohad been lying absolutely still, became extremely restless andwas restrained in bed with great difficulty. Plantar responseremained extensor on both sides. Later the depth of comagradually lessened and 30 hours after the transfusion the patientcomplained of headache. At that time he was still confused andnot fully orientated. His confused state gradually improved and38 hours after the transfusion he was able to obey simplecommands and answer simple questions. At this stage theplantar responses became flexor. Improvement was rapidduring the next few hours and 50 hours after transfusion he wasmentally alert and answered all questions rationally. There-after his recovery was uneventful and he is now back at work,after a period of convalescence.Our experience is so far limited to this one case, but we feel
that this form of treatment offers some hope in an otherwisehnmalPCe situation
NAZIR A. CHAUDHARYNASEER AHMAD SHAIKH
MUSHTAQ HASAN.
Dow Medical Collegeand Civil Hospital,Karachi, Pakistan.
TRAINING IN MEDICAL LABORATORY
TECHNOLOGY
SIR The response in your correspondence columnsto the statement from the Institute of Medical LaboratoryTechnology (Oct. 28, p. 935) reflects a widespread endeavour tomaintain high standards of technology and efficiency. Thereis general agreement on a system of coordinated trainingconducted by experts and based on day or block release; andthere should be close liaison between training institutes andworking laboratories. However, laboratories must continue tofunction at all times as efficient working units, and the problemof providing full training facilities is linked with that of main-taining adequate work output of a high technical standard.Whether or not day release is made compulsory, there is littledoubt that it is here to stay: it is also clear that the constraints
imposed by regular staff absences can be overcome only byincreases in technical establishments; but, because of presentfinancial restrictions and shortage of labour, it is unlikely thatearly increases in senior establishments will come about orthat, in any case, these would solve the immediate problem.
In the recent correspondence, no account has been taken ofthose who are willing and capable of giving useful service inlaboratories but who, for various reasons, have no wish toundergo training in depth or to acquire technological qualifica-tions. Much of the work of medical laboratories-clinical,academic, or reference departments-is elementary or repeti-tive, and much of this can be carried out by people withcomparatively little training. Is it not time, as a matter ofnational policy, to encourage employment of a quota of semi-skilled labour in laboratories in order to permit greaterflexibility in educational arrangements for those most suitablefor advanced training ?Recruitment of such semiskilled labour should be based on
recognised salary scales proportional to, but slightly less than,those of technicians in training; and increments could be basedon age, length of service, and, possibly, degree of local responsi-bility. This suggestion in no way implies a lowering oftechnical standards. Most experienced laboratory workersare acquainted with technicians who are excellent at theirdaily work but pass examinations only with great difficulty,if at all.
Surely, employment and recognition of such workers wouldplug some of the obvious leaks in technical establishments,because many who are now deterred by academic handicapswould be encouraged to enter laboratory services. Con-temporary proposals for technicians’ training are to be com-mended for their far-sighted aims and their optimism. Butthis optimism must be modified by the realities of the presentsituation. Only by paying due regard to the pressures on thelower echelons of laboratory workers will a future elite body oftechnologists be created.
G. D. WASLEYR. FRASER WILLIAMS.
Department of Medical Microbiology,St. Thomas’s Hospital Medical School,
London S.E.1.
DERMATOGLYPHICS IN ECTODERMALDYSPLASIA
SIR,—I wish to report briefly my dermatoglyphic analyses oftwo families with hereditary anhydrotic ectodermal dysplasia,since there seem to be findings common to affected individualsand carriers. In this X-linked disorder there are decreasednumbers of sweat-glands in the skin, hypotrichosis, andanodontia. The carrier female usually has symptoms. Sincethe skin is involved, one might expect to find characteristicdermatoglyphic changes, as in this short series.
In family 1 the father was clinically normal; the mother hadthin hair, decreased sweating, and poor teeth; three sons wereall severely affected. In family 2 the father was clinicallynormal; the mother had decreased sweating; three sons wereseverely involved and one son was normal. The dermato-
glyphics of all these people were analysed; the affected ones hadstriking ridge disruption in all areas of palms and soles. Therewas also a tendency for the patterns to be vestigial, particularlyin the third and fourth interdigital and plantar hallucal areas.The c and d digital triradii tended to be absent, vestigial, ordisplaced, and the C and D palmar lines were impossible totrace or stopped without going anywhere. The hypothenarpatterns were usually too complex to describe by a singlepattern name, and in addition the palmar axial triradius tendedto be distal. The carriers (mothers) had some but not all ofthese patterns. The complex hypothenar patterns were presentin both mothers. The normal people in this series had none ofthese findings.Thus dermatoglyphics may prove to be another clinical
measure for diagnosis of patients with anhydrotic ectodermaldysplasia and of the carrier or less affected female.
This work was aided by a grant from the National Foundation.
JEAN H. PRIEST.
Departments of Pathology and Pediatrics,University of Colorado Medical Center,
Denver, Colorado 80220.
INSULIN AND GLUCOSE RESPONSE TO
GLUCAGON IN DOWN’S SYNDROME
SIR,-Abnormal glucose tolerance has been reported 1-3in patients with Down’s syndrome (D.S.). In children with
D.S., other mentally retarded children, and normal children,we have found that the mean rise in plasma-immunoreactive-insulin level after oral glucose is only about half that observedin adults.4 Since glucagon has been shown to promote insulinsecretion in adults,5 6 we decided to measure the response toit of these children.
16 children (aged 20 months to 6 years 4 months) werestudied: 6 had D.s., 5 were mentally retarded from othercauses, and 5 were normal. All were taking a high-carbohydratediet containing 1000-2000 calories per day. After an overnightfast 0-5 mg. glucagon was given intravenously during 1-2
1. O’Leary, W. D. Am. J. Dis. Child. 1931, 41, 544.2. Runge, G. H. Am. J. ment. Defic. 1959, 63, 822.3. Benda, C. E. The Child with Mongolism; p. 175. New York, 1960.4. Milunsky, A., Rubenstein, A. H., Lowy, C., Wright, A. D. Devl Med.
Child Neurol (in the press).5. Yalow, R. S., Black, H., Villazon, M., Berson, A. A. Diabetes, 1960,
9, 356.6. Samols, E., Marri, G., Marks, V. Lancet, 1965, ii, 415.
