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bearing sperm 3 and YY bivalents in meiosis 4 have beenobserved in XYY males, only very few cases of XYY infather and son and no XXY offspring of XYY males areknown. Triple-X females are usually fertile-four XXYsons and one XXX/XX daughter,5 as well as a child withtrisomy 21,6 have been reported. Thus, the excess ofchromosomally normal children born to either XYY orXXX parents is overwhelming. It is unclear at what

stage of gametogenesis or fertilisation selection takes place.The absence of increased incidence of spontaneous abortionand the apparent viability of XXX, XXY, and XYY em-bryos resulting from meiotic non-disjunction in chromo-somally normal parents make selection during early embryo-genesis unlikely.

Genetic counselling for this couple has been generallyreassuring. The clinical findings in the propositus are notdiagnostic for a sex-chromosomal, autosomal, or geneticsyndrome. They are likely to be unrelated to the parents’chromosome abnormalities. In case of future pregnancies,fetal chromosome monitoring has been recommended,with the possibility in mind that a genetic predispositionto non-disjunction could result in other than sex chromo-somal trisomies as well.

Division of Medical Genetics,Departments of Pediatrics and

Medicine,University of California,

San Diego School of Medicine,La Jolla, California 92037,

U.S.A.

Department of Pediatrics,Naval Regional Medical Center,

San Diego,California 92134, U.S.A.

UTA FRANCKEOLIVER W. JONES.

MICHAEL J. MORAN.

POSSIBLE MOSAIC XXX-XXY MARRIAGEWITH ABNORMAL OFFSPRING

SIR,-Dr Howard’s report on an XXX-XXY marriage(Dec. 21, p. 1526), discovered through routine chromosomeanalysis, prompts us to report a marriage between a possibleXX/XXX woman and an XY/XXY man, whose abnormalchromosome complements were discovered because ofabnormal offspring.The first child of the couple was born in 1961, at which time

no chromosomal investigation was done. She had clinical Downsyndrome with severe congenital heart-disease and died at theage of one week.The second child, born in 1963, had few clinical signs of

Down syndrome but had congenital heart-disease. Chromosomestudies of 20 metaphases in blood-lymphocytes showed 17 witha normal male karyotype and 3 with a 47,XY,G+ chromosomecomplement.The third child was born in 1972. She has developed normally

so far, and there is no evidence of clinical Down syndrome.No chromosome studies have been done.The fourth pregnancy was monitored by examination of

cultured amniotic-fluid cells. In 150 metaphases examined afemale karyotype was found, without evidence of trisomy ofany chromosome. The woman is in her seventh month of

pregnancy at the time of this report.

The XX/XXX mosaic constitution of the mother’slymphocytes must be regarded as tentative only, since in 55metaphases examined only 1 was found with a 47,XXXkaryotype, the identification of the extra C being made byfluorescence. In 36 metaphases from the husband, 2 werefound with a 47,XXY complement, proven by fluorescencebanding. Clinically, the parents were normal except that

3. Diasio, R. B., Glass, R. H. Lancet, 1970, ii, 1318.4. Hultén, M., Pearson, P. L. Ann. hum. Genet. 1971, 34, 273.5. Barr, M. L., Sergovich, F. R., Carr, D. H., Shaver, E. L. J. Can.

med. Ass. 1969, 5, 247.6. Singer, J., Sachdeva, S., Smith, G. F., Hsia, D. Y. Y. J. med. Genet.

1972, 9, 238.

both had a slightly elevated palmar axial triradius in bothhands.The probability of a mosaic XXX-XXY marriage must

be even lower than the probability of a non-mosaic XXX-XXY marriage, although the frequency of mosaics withoutclinical symptoms might be the same in the marriage-agepopulation as in the newborn population. On the otherhand, the frequency of mosaics is not known at all at

the present time.We have also observed another family with 2 mosaic

mongoloid children. The first baby, a boy, had 25%G-trisomic cells in cultures from peripheral blood but only1-5% in fibroblast cultures. The second child, another boywith typical clinical Down syndrome, had only 1-5%trisomic cells in 340 metaphases analysed from blood-lymphocyte cultures. Both children died from severe

congenital heart-disease. The third child was a normal boy,clinically and chromosomally. The mother elected to haveher fourth pregnancy interrupted in view of the uncertaintyin detecting a weak mosaic in amniotic-fluid-cell cultures.In 50 metaphases examined from the aborted female fetus1 was found to be G-trisomic and 1 G-tetrasomic.The parents’ karyotypes from peripheral-blood lympho-

cytes were normal. Two questions arise from the observa-tion of these two families:

(1.) Is it fortuitous that all 4 mongoloid children from 2 mosaicfamilies had severe congenital heart-disease ?

(2.) Is prenatal diagnosis justified in families with provenfamilial chromosomal mosaicism ?

We should be interested to know how other geneticists,pxdiatricians, and gynaecologists feel about these two issues.

Institute of Genetics,Basle University Children’s Hospital,CH-4000 Basle, Switzerland.

E. M. BÜHLERG. KOSZTOLÁNYIG. R. STALDER.

PRACTOLOL AND SCLEROSING PERITONITIS

Sir should like to comment on the interesting paperby Dr Brown and his colleagues (Dec. 21, p. 1477), describ-ing sclerosing peritonitis in association with practolol.I agree that a causal relationship must seriously be con-sidered.From their discussion of differential diagnosis, one

might, however, conclude that non-specific fibrosing periton-itis is a newly recognised entity. This, I would suggest, isnot the case. As early as in 1942, Hartmann, a Germaninvestigator, described four patients with " fibroplasticperitonitis," a disorder which seems to be closely similarto the condition now observed. He also reviewed muchearlier literature on the same condition. During the

following years, several articles on this apparently very raretype of peritonitis were published in German literature,reviewed by Geisthövel and Kalfhaus in 1965.2

Fibroplastic peritonitis is defined as a chronic non-specificabdominal inflammation of unknown origin which causesthe development of a greyish-white pseudomembranousdeposit on the visceral peritoneum, predominantly of thesmall intestine and the mesentery, and on the parietalperitoneum. The small intestine is invested in a fibrous

capsule and may resemble a large ovarian cyst or, charac-teristically, may have the appearance of an accordion.As a result of encapsulation of the small intestine by thesclerous membrane, it becomes rigid, and ultimately a

mechanical ileus may develop.The Netherlands Centre for Monitoring of Adverse

Drug Reactions has now received reports on 5 patients whodeveloped sclerosing peritonitis, and in accordance withthe publication by Brown et al. it was concluded that

1. Hartmann, W. Dt. Z. Chir. 1942, 255, 173.2. Geisthövel, W., Kalfhaus, G. Mūnch. med. Wschr. 1965, 107, 781.