982

minantly autosomes. These findings tering of chromosomal anomalies

confirm the seasonal clusand our hypothesis that environmental factors may affect non-disjunction in all the chromosomes in the karyotype to a similar degree. of Biophysics and Genetics and Eleanor Roosevelt Cancer Research Institute, University of Colorado Medical Center, Denver, Colorado, U.S.A. and National Jewish Hospital and Research Center, Denver

Department

ARTHUR ROBINSON THEODORE T. PUCK

LIGATING THE UMBILICAL CORD effective methods of ligating the umbilical cord is by the use of rubber bands. However, these bands are difficult to apply with artery forceps, the usual method. They can be easily put on with the help of an ’Elastrator’, a simple device used by shepherds for cas-

SIR,-One of the cheapest and

most

pelvis and an enlarged bladder. The treated with co-trimoxazole, and she was referred for further urological investigations where, under anesthesia, she was found to have normal vulva, clitoral enlargement, and a high, stenosed, vaginal opening. There was a urogenital sinus above the stenosis, and a pelvic mass, which proved to be a hydrocolpus, compressing the bladder neck. This was drained and, later, a vaginoplasty was done. Follow-up t.v.p. showed great improvement of the hydronephrosis. This case provides further evidence that synthetic progesterones are teratogenic and should not be used during pregnancy. We thank Mr I. Williams, Hospital for Sick Children, London, for

ment

of the

ureters

urinary infection

in the

was

undertaking this patient’s urological investigations and care. Department of Child Health, St George’s Hospital, London SW17

I. F.

ROBERTS

R. J. WEST

PRENATAL DETECTION OF INTESTINAL OBSTRUCTION BY DISACCHARIDASE ASSAY IN AMNIOTIC FLUID

SIR,-Fetal intestinal obstructions have been diagnosed primarily by amniofetography. This technique, which relies upon intra-amniotic injection of radio-opaque dye, has a greater risk than amniocentesis alone but has been extensively used for management of polyhydramnios and pregnancies in which the

The Elastrator

trating lambs. It costs about 5and has two handles which, when squeezed together cause four short stubs to move apart in a square formation. The elastic band is put over these stubs, the handles are squeezed, and the whole is slipped over the cord end. When the handles are released the rubber band can be easily rolled onto the cord. The tool is stoutly made of niated metal and it can be immersed in sterilising solutions. Cumberland Infirmary, E. M. CARR-SAUNDERS Carlisle CA2 7HY

TERATOGENESIS AND MATERNAL PROGESTERONE

. SIR,-Fetal virilisation can result from synthetic progestertherapy during pregnancy,’and these agents may also cause other congenital malformations,’ yet they are still prescribed for threatened and habitual abortion even though there

one

is no evidence for their efficacy.5 A girl aged 4 months was referred to this department with a short history of cough, vomiting, and loose stools. She was mildly dehydrated and had a greatly enlarged clitoris. Her mother had taken oral dydrogesterone 20 mg daily from the 8th to the 20th week of her pregnancy and then 10 mg daily to term, and intramuscular hydroxyprogesterone hexanoate 250 mg had been given weekly from the 8th to 20th week. The infant had a Klebsiella urinary-tract infection, confirmed by suprapubic aspiration of urine. Plasma urea and electrolytes, plasma-17-hydroxyprogesterone, and urinary 11-oxygenation index were normal. An intravenous pyelogram (i.v.p.) showed bilateral hydronephrosis and hvdroureter with lateral displace-

1. Wilkins, L., Jones, H. W., Holman, G. H., Stempfel, R. S. J. clin. 2.

Endocr. Metab. 1958, 18, 559. Voorhess, M. L. J. Pediat. 1967, 71, 128.

3. Heinemen, O. P., Slone, D., Monson, R. R., Hook, E. B.,

Engl. J. Med. 1977, 296, 67. 4. Harlaps, S., Prywes, R., Davies, A M. Lancet, 1975, i, 682. 5. Shearman, R. P., Garrett, W. J. Br med. J. 1963, i, 292.

Shapiro,

S. New

fetus is at risk for intestinal malformations. Our finding that fetal intestinal disaccharidases were measurable in amniotic fluid’ suggested that obstruction of the lower part of the fetal intestine might be detected by low or absent disaccharidase activity. This paper reports a deficiency of disaccharidase activity in the amniotic fluid of a fetus born with anal imperforation. A 24-year-old woman, whose previous child had meningomyelocele, had amniocentesis at 16 weeks of pregnancy. When karyotyped, the amniotic-fluid cells had a normal male chromosomal pattern. The amniotic-fluid x-fetoprotein concentration was normal. The mother delivered spontaneously after 31 weeks of gestation. The infant’s birth-weight was 1860 g and his cranium circumference was 30 cm. Soon after birth, X-ray confirmed an oesophageal atresia and an imperforate anus. Disaccharidase activity was measured in the patient’s amniotic fluid (which was kept frozen at -60°C) and in control amniotic fluid samples2 by the method of Dahlqvist,3 and protein was measured by the method of Bohlen et al.1 In replicate tests, disaccharidase activity in amniotic fluid from the fetus with an imperforate anus was absent or considerably lower than normal. Sucrase, trehalase and lactase activities were absent (the normal activities of these enzymes at 15-21 weeks gestation are 26-203, 0-07-1-52, and 0.09—0.75 µmol/min/g of protein, n=27). The maltase and palatinase activities were 0.08 and 0-06 µmol/min/g of protein (normal values are 5.1-33. 1, n=27, and 041-3 43, n=20). We previously found normal disaccharidase activity in the amniotic fluid of a 14-week-old fetus at risk from the autosomal recessive type of multiple intestinal atresia.’ A normal infant was subsequently delivered. The disaccharidase activity in amniotic fluid may thus be useful in the early detection of anal imperforation and possibly atresia of the lower part of the fetal intestine. It should be pointed out, however, that amniocentesis must be done before 18 weeks of gestation, since the

1. Potier, M., Melançon, S. B , Dallaire, L. Biomedicine, 1976, 25, 167. 2 Dallaire, L., Potier, M., Melançon, S. B., Patrick, J. J. Obstet. Gynœc Brt Commonw. 1974, 81, 761. 3. Dahlqvist, A. Anal. Biochem. 1964, 7, 18. 4. Böhlen, P., Stein, S., Dairman, W., Undenfriend, S. Arch. Biochem Bio5.

phys. 1973, 155, 213. Dallaire, L., Perreault, G. Birth Defects orig. Art. Ser 1974, 10, 259

Ligating the umbilical cord.

982 minantly autosomes. These findings tering of chromosomal anomalies confirm the seasonal clusand our hypothesis that environmental factors may af...
154KB Sizes 0 Downloads 0 Views