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LEYDIG-CELL HYPERPLASIA IN TESTICULAR-FEMINISATION SYNDROME
708 on
the results of surgery in these
cases
with concomitant
ulcers, but the evidence suggests that vagotomy and drainage by pyloroplasty is usually successful. Obviously pyloroplasty will reverse the effects of pyloric stenosis, but it will also permit greater reflux of duodenal contents into the stomach and might be expected to prevent healing of the gastric ulcer. I suggest, though, that as a result of a wide pylorus, stomach emptying is rapid and refluxed duodenal contents do not stay in the stomach long enough to maintain the abnormal physiological state necessary for the persistence of the gastric ulcer. Perth Radiological Clinic, 8 King’s Park Road, West Perth, Western Australia 6005.
ERIC M. BATESON.
TRAINING IN CLINICAL PHARMACOLOGY
LEYDIG-CELL HYPERPLASIA IN TESTICULAR-FEMINISATION SYNDROME SIR,-In a letter early this year, Dr. Faulds and Professor Lennoxreported that the number and volume of Leydig cells in the testis of a patient with testicular feminisation was not increased. This is not true of all cases, as the following case-report shows. The patient was 20 years of age and presented with the chief complaint of amenorrhcea. On examination she had a moderate amount of pubic and axillary hair. The clitoris was somewhat enlarged, the vagina short and narrow, the uterus small, and in the inguinal canals on both sides a retractile testis was palpable. The breasts were moderately well developed, and she had the psychosomatic habitus of a woman. Chromosomal analysis revealed a 46,XY pattern. One testis was removed together with the uterus (see figure) and the other was fixed in the
SiR,ŅIs it too much to hope that the rigorous expertise of the clinical pharmacologist outlined by Dr. Prichard and Dr. Turner (Sept. 18, p. 653) can be extended to include some knowledge of human psychology and behaviour ? Enormous quantities of drugs are, we all know, in daily use in attempts to relieve all manner of psychological disturbance and bodily symptoms of psychological origin (quite apart from the specialised use of psychoactive drugs in overt psychiatric illness). Few can doubt that personality variables and social and situational factors can profoundly influence treatment responses, and that our interest in these areas needs to go further than double-blind-trial design, important though this may be. Perhaps the psychologist and the sociologist merit a place in the team, along with the biochemists, biometricians, pharmacists, electronic engineers, and the like ? University College London, C. J. LUCAS. WC1E 6BT. INFECTIOUS HEPATITIS AND APLASTIC ANÆMLA—IN TWO SISTERS SiR,ŅTo the series of cases of infectious hepatitis and aplastic anaemia reported during the past few months, I should like to add an account of two sisters with the diseases. In 1958, two sisters aged 19 and 17, acquired infectious hepatitis and were treated in the county hospital of Ozd (under the care of Dr. Kirchknopf). In 1963, the older sister showed all the signs of aplastic anaemia, including a hypoplastic bone-marrow, and despite therapeutic efforts she died a few months later. The first signs of aplastic anaemia in the other sister appeared in 1966. The bone-marrow was repeatedly found to be hypoplastic, and she was treated with high doses of anabolic steroids and prednisolone. During the first year she needed bloodtransfusions every fourth or fifth week. In the second and third years, however, she improved strikingly, and no transfusion was necessary for 14 months. In April, 1970, she relapsed after a " common cold ". She was transfused, and her condition improved again. At present she is fairly well, with a haemoglobin of 8-9 g. per 100 ml. and
platelet-count usually around 70,000 per c.mm. We suggest that there is either a genetically determined " hypersensitivity " of the bone-marrow to the infectioushepatitis virus or that in certain rare circumstances the virus can directly damage the erythropoietic cells. The coincidence of infectious hepatitis and aplastic anaemia in two siblings supports the theory that some individuals a
mav
have
an
inherited susceotibilitv.
Fourth Medical Department of the Postgraduate Medical School,
Budapest, Hungary.
MARIANNA BOGA PAUL A. SZEMERE.
Testis, uterus, and fallopian tube. A large nodule is
seen at
the upper pole of the testis.
pelvis. The testis measured 5 x 4 x3 cm. On section, a brownish-grey nodule was seen incorporated into the testis itself, and histological examination revealed that the testis consisted of broad seminiferous tubules with rather abundant Leydig cells in between. The sharply demarcated nodule was composed of Leydig cells. Ultrastructurally these cells looked like normal adult Leydig cells, though no Reinke crystalloids were seen. It is arguable whether the nodule in the testis should be called adenoma or focal Leydig-cell hyperplasia. The testis was of average size, so the total amount of Leydig cells was evidently increased, though no exact volumetric measurements were done. What is more important, from our point of view, is the fact that the Leydig cells seemed to be mature and active, as judged by their ultrastructure, unlike another case of ours, in which the testis was small and the Leydig cells immature. Our intention is not to invalidate the measurements of Faulds and Lennox, especially since this case seems to be atypical. We are not aware of any other case of testicular feminisation with nodules of Leydig cells in the testis; nodules, if present, are usually composed of Sertoli cells.’ We feel that electron microscopic examination gives a more direct insight into the functional state of Leydig cells than estimation of their volume, although the two methods might well be complementary. Department of Pathology and Department of Obstetrics and Gynæcology, Medical Faculty, University of Zagreb, Yugoslavia.
I. DAMJANOV A. BUNAREVIĆ P. DROBNJAK V. GRIZELJ.
1. 2.
Faulds, J. S., Lennox, B. Lancet, 1971, i, 345. Damjanov, I., Drobnjak, P., Grizelj, V. Am. J. Obstet. Gynec. 1971,
3.
110, 594. Teter, J., Boczkowski, K. ibid. 1966, 94, 813.
the results of surgery in these
cases
with concomitant
ulcers, but the evidence suggests that vagotomy and drainage by pyloroplasty is usually successful. Obviously pyloroplasty will reverse the effects of pyloric stenosis, but it will also permit greater reflux of duodenal contents into the stomach and might be expected to prevent healing of the gastric ulcer. I suggest, though, that as a result of a wide pylorus, stomach emptying is rapid and refluxed duodenal contents do not stay in the stomach long enough to maintain the abnormal physiological state necessary for the persistence of the gastric ulcer. Perth Radiological Clinic, 8 King’s Park Road, West Perth, Western Australia 6005.
ERIC M. BATESON.
TRAINING IN CLINICAL PHARMACOLOGY
LEYDIG-CELL HYPERPLASIA IN TESTICULAR-FEMINISATION SYNDROME SIR,-In a letter early this year, Dr. Faulds and Professor Lennoxreported that the number and volume of Leydig cells in the testis of a patient with testicular feminisation was not increased. This is not true of all cases, as the following case-report shows. The patient was 20 years of age and presented with the chief complaint of amenorrhcea. On examination she had a moderate amount of pubic and axillary hair. The clitoris was somewhat enlarged, the vagina short and narrow, the uterus small, and in the inguinal canals on both sides a retractile testis was palpable. The breasts were moderately well developed, and she had the psychosomatic habitus of a woman. Chromosomal analysis revealed a 46,XY pattern. One testis was removed together with the uterus (see figure) and the other was fixed in the
SiR,ŅIs it too much to hope that the rigorous expertise of the clinical pharmacologist outlined by Dr. Prichard and Dr. Turner (Sept. 18, p. 653) can be extended to include some knowledge of human psychology and behaviour ? Enormous quantities of drugs are, we all know, in daily use in attempts to relieve all manner of psychological disturbance and bodily symptoms of psychological origin (quite apart from the specialised use of psychoactive drugs in overt psychiatric illness). Few can doubt that personality variables and social and situational factors can profoundly influence treatment responses, and that our interest in these areas needs to go further than double-blind-trial design, important though this may be. Perhaps the psychologist and the sociologist merit a place in the team, along with the biochemists, biometricians, pharmacists, electronic engineers, and the like ? University College London, C. J. LUCAS. WC1E 6BT. INFECTIOUS HEPATITIS AND APLASTIC ANÆMLA—IN TWO SISTERS SiR,ŅTo the series of cases of infectious hepatitis and aplastic anaemia reported during the past few months, I should like to add an account of two sisters with the diseases. In 1958, two sisters aged 19 and 17, acquired infectious hepatitis and were treated in the county hospital of Ozd (under the care of Dr. Kirchknopf). In 1963, the older sister showed all the signs of aplastic anaemia, including a hypoplastic bone-marrow, and despite therapeutic efforts she died a few months later. The first signs of aplastic anaemia in the other sister appeared in 1966. The bone-marrow was repeatedly found to be hypoplastic, and she was treated with high doses of anabolic steroids and prednisolone. During the first year she needed bloodtransfusions every fourth or fifth week. In the second and third years, however, she improved strikingly, and no transfusion was necessary for 14 months. In April, 1970, she relapsed after a " common cold ". She was transfused, and her condition improved again. At present she is fairly well, with a haemoglobin of 8-9 g. per 100 ml. and
platelet-count usually around 70,000 per c.mm. We suggest that there is either a genetically determined " hypersensitivity " of the bone-marrow to the infectioushepatitis virus or that in certain rare circumstances the virus can directly damage the erythropoietic cells. The coincidence of infectious hepatitis and aplastic anaemia in two siblings supports the theory that some individuals a
mav
have
an
inherited susceotibilitv.
Fourth Medical Department of the Postgraduate Medical School,
Budapest, Hungary.
MARIANNA BOGA PAUL A. SZEMERE.
Testis, uterus, and fallopian tube. A large nodule is
seen at
the upper pole of the testis.
pelvis. The testis measured 5 x 4 x3 cm. On section, a brownish-grey nodule was seen incorporated into the testis itself, and histological examination revealed that the testis consisted of broad seminiferous tubules with rather abundant Leydig cells in between. The sharply demarcated nodule was composed of Leydig cells. Ultrastructurally these cells looked like normal adult Leydig cells, though no Reinke crystalloids were seen. It is arguable whether the nodule in the testis should be called adenoma or focal Leydig-cell hyperplasia. The testis was of average size, so the total amount of Leydig cells was evidently increased, though no exact volumetric measurements were done. What is more important, from our point of view, is the fact that the Leydig cells seemed to be mature and active, as judged by their ultrastructure, unlike another case of ours, in which the testis was small and the Leydig cells immature. Our intention is not to invalidate the measurements of Faulds and Lennox, especially since this case seems to be atypical. We are not aware of any other case of testicular feminisation with nodules of Leydig cells in the testis; nodules, if present, are usually composed of Sertoli cells.’ We feel that electron microscopic examination gives a more direct insight into the functional state of Leydig cells than estimation of their volume, although the two methods might well be complementary. Department of Pathology and Department of Obstetrics and Gynæcology, Medical Faculty, University of Zagreb, Yugoslavia.
I. DAMJANOV A. BUNAREVIĆ P. DROBNJAK V. GRIZELJ.
1. 2.
Faulds, J. S., Lennox, B. Lancet, 1971, i, 345. Damjanov, I., Drobnjak, P., Grizelj, V. Am. J. Obstet. Gynec. 1971,
3.
110, 594. Teter, J., Boczkowski, K. ibid. 1966, 94, 813.