1359

relapse during the gluten challenge was followed by remission after the second withdrawal of gluten from the diet. Department of Medicine, Hope Hospital, Salford

A. ELLIS

Department of Medicine, Nuffield Unit of Medical Genetics, University of Liverpool

B. D. LINAKER

growth of symptom-free persons with pseudohypoaldosteronism has not been described. As this syndrome is probably more common than previously thought, we suggest that plasma aldosterone and renin be measured in the evaluation of short stature, especially if there is a suggestive family history. Early salt treatment in proven symptom-free patients seems logical.

on

the linear

Pædiatric Department, Zahalon Government Hospital, Jaffa, Israel and Tel-Aviv University Medical School

A. HANUKOGLU D. FRIED A. GOTLIEB

INHERITANCE OF PSEUDOHYPOALDOSTERONISM

SIR,-The exact mode of inheritance in pseudohypoaldosteronism needs clarifying since few pedigrees have been reported. Dr Limal and colleagues (Jan. 7, p. 51) proposed autosomal dominance with variable expression. We have seen a female infant with pseudohypoaldosteronism family studies, confirm Limal’s suggestion. The girl was born after an uncomplicated full-term pregnancy, birth-weight 2600 g. She was admitted at 3 months of age because of failure to thrive and severe dehydration. Electrolyte determinations revealed persistent hyperkalsemia. The plasma-aldosterone at 4.5 months was 600 ng/dl. The infant was discharged at this time weighing 4120 g. The results of plasma aldosterone, renin, and urine aldosterone at the age of 6 months are shown in the table. Her mother had been in hospital several times during infancy because of vomiting and failure to thrive and had received parenteral fluid. Since the age of 1 -years she had gradually recovered, without any specific treatment. We examined five more members of the family who

symptom-free. The propositus and her mother, maternal grandmother, and two brothers had high aldosterone and renin values. Five siblings of the mother who are also clinically normal were not available for investigation. were

drawn in the morning after the patients had got up. Aldosterone was isolated from the methylene chloride extract of plasma and urine by immunological purification before radioimmunoassay.2 The normal ranges for plasma and urine aldosterone are 2-14 ng/dl and 2-14 ;j.g/24 h, respectively. The plasma-renin-activity (P.R.A.) was determined by angiotensin I radioimmunoassay kit (New England Nuclear). The normal base values range from 0.6 to 3-0

Blood-samples

were

ng/ml/h.

The affected family members, except for one brother, have short stature. Moreover, the propositus was just below the 3rd percentile at the age of 6 months and remained so until we began a high-salt diet at the age of 8.5months. The treatment resulted in a catch-up growth concomitant with the expected lowering of aldosterone and renin values. Catch-up growth in salt-losing syndromes after high-salt diet is well known.2-4 However, the influence of this treatment

1. Gomez-Sanchez, C., Kem, D. C., Kaplan, N. M. J. clin. Endocr. Metab.

PREVENTION OF CONGENITAL ADRENAL HYPERPLASIA to Dupont et al.’ and preliminary expericlinic, the 21-hydroxylase locus, defective in con-

SIR,-According ence

in

our

genital adrenal hyperplasia (C.A.H.) is closely linked to the HLA B locus. This means that it might well be possible to use specific antibodies to eliminate the 50% of the paternal sperms2,3 which carry the marker HLA B antigen and then carry out artificial insemination with the remaining spermatozoa. Using this method the chances for the offspring of heterozygote (for C.A.H. and father for HLA B) parents, should be improved to almost 50% carrier-free and 50% heterozygotes. The same could be applied to HLA B27 fathers with ankylosing spondylitis. Universitätskinderklinik München, 8 München 2, West Germany

JUAN N. WALTERSPIEL

FINE-NEEDLE PERCUTANEOUS TRANSHEPATIC CHOLANGIOGRAPHY

SiR,—Our experience confirms

your support for fine-needle percutaneous transhepatic cholangiography in the investigation of jaundice (May 27, p. 1139). In ninety-six consecutive cholangiograms done in this way there has been only one failure to outline the ducts, and there have been no serious com-

The diagnostic accuracy is excellent, as judged by definition of the level and cause of obstruction. Failure to enter the bileducts does not completely exclude obstruction as the intrahepatic ducts may be of normal fine calibre in some cases of gallstone obstruction. This diagnostic technique is safe and sure, largely due to the use of the lateral approach, slim needle, and prophylactic antibiotics. Drainage of the ducts is a therapeutic measure which requires the insertion of a much larger calibre flexible catheter. It is attended by greater risks such as bile leakage, of a degree that caused the older methods of transhepatic cholangiography

plications.

1975, 36, 795. 2. 3. 4.

Donnel, G. N., Litman, N., Roldau, M. Am. J. Dis. Child. 1959, 97, 813. Proesmans, W., Geussens, H., Corbeel, L., Eeckels, R. ibid. 1973, 126, 510. Rosler, A., Rabinowitz, D., Theodor, R., Ramirez, L. C., Ulick, S. J. clin Endocr. Metab. 1977, 44, 279.

1.

Dupont, B., Oberfield, S. E., Smithwick, E. M., Lee, T. D., Levine, Lancet, 1977, ii, 1309. 2. Fellous, M., Dausset, J. J. Nature, 1970, 225, 191. 3. Halim, A., Abbasi, K., Festenstein, H. Tissue Antigens, 1974, 4, 1.

RESULTS OF FAMILY STUDY

L. S.

1360 fall into disrepute. The decision to drain should not be taken lightly, and should be carefully weighed against the alternative of early surgery. We have not had to resort to drainage in any of our cases to date but have continued gentamicin cover up to the time of surgery, which should be undertaken within 48 h, in all cases of gallstone obstruction where the risk of to

ascending cholangitis is highest. Llandough Hospital, Penarth, Glamorgan CF6

1XX

G. DE B. HINDE P. M. SMITH

SEPTIC SHOCK

SIR,-The extensive experience of Dr Ledingham and Mr McArdle (June 3, p. 1194) is of great interest. However, they cannot be said to be "treating" septic shock: their skill lies in supporting the lungs and circulation in patients with sepsis. Indeed, as their mortality figures show, the patients still die of multiorgan damage, and this is known to be induced by endotoxin. The mortality of septic shock remains near 50% because most people do not have protective anti-lipid-A antibodies in any appreciable titre. Until immunisation or immediate serum therapy become the accepted mode of raising antibody titres, there will be no real progress. In Britain there are about 2000 deaths a year because of this.

cal

improvement, and the steroid dose was subsequently tapered without adverse effects. Perhaps methylprednisone and/or its hemisuccinate is preferable in patients with brain tumours who are also on anticonvulsants. One fact we must not forget is that the treatment of brain tumours with large doses of corticosteroids may prolong the survival of such patients in a functional and productive fashion, Munson Medical Center, Traverse City, Michigan 49684, U.S.A.

FREDERICK M. VINCENT

D.A.T. (DAUNORUBICIN, CYTARABINE, 6-THIOGUANINE) IN ACUTE MYELOID LEUKÆMIA SIR,-In 1977 we reported’ a remission-rate of 85% in twenty consecutive patients with acute myeloid leukaemia treated with the D.A.T. regimen-a combination of daunorubicin 50 mg/m2 on day 1, cytarabine 100 mg/m2 12-hourly by intravenous push and 6-thioguanine 100 mg/m2 12-hourly on days 1-5. Many inquirers have asked whether this remissionrate is being maintained. We have now treated forty consecutive patients with the D.A.T. regimen, of whom thirty-four (85%) entered complete remission. Figs 1 and 2 show that the median duration of survival is 104 weeks and the median duration of remission is 61 weeks. A second remission following a relapse was obtained in 45%.

Department of Therapeutics, Sheffield Royal Sheffield

Infirmary,

E. N. WARDLE

1.

Rees, J.

K.

H., Sandler,

R.

M., Challener, J. Hayhoe, F. G. J. Br. J. Cancer,

1977, 36, 770.

PHENYTOIN/DEXAMETHASONE INTERACTION SiR,-The interaction noted by Dr McLelland and Dr Jack (May 20, p. 1096) has serious implications for the "successful" treatment of patients with brain tumours. There is a dose dependency of dexamethasone and other steroids in patients with brain tumours,’-3 and if patients deteriorate on conven16 mg dexamethasone daily), the sitube considered hopeless. Thus, some patients may require as much as 96 mg dexamethasone a day to control their symptoms; the same is true for equivalent doses of methylprednisolone. Several reportsl-3 have cited patients who had progressively deteriorated on conventional doses of dexamethasone but who became symptom-free when dosages were increased. Although steroid side-effects were not uncommon, the quality of life for these patients was quite good and survival was prolonged. In part this dose dependency may depend on the concomitant administration of drugs (usually anticonvulsants) which accelerate the metabolism of the steroid preparation. Although dexamethasone is the most common steroid used in patients with brain tumours, there is evidence which suggests that methylprednisolone and its hemisuccinate salt are as effective and that they do produce significant clinical improvement and reduce both periventricular elastance and intra!cranial pressure.3 They also undergo less metabolic clearance than dexamethasone in patients who are concurrently receiving phenobarbitone or phentoin, a fact which is both experimentally and clinically proven. 3,4 I have treated two patients, both with glioblastoma multiforme of the parietal lobes, who were receiving dexamethasone and, phenytoin. Symptoms improved on this regimen, but returned in 6-8 weeks. The switch from dexamethasone to equivalent doses of methylprednisone resulted in prompt clini-

tional doses ation should

(usually not

Renaudm, J., Fewer, D., Wilson, C. B., Boldrey, E. B., Calogero, J., Enot, K. J. J. Neurosurg 1973, 39, 302. 2. Lieberman, A., LeBrun, Y., Glass, P., Goodgold, A., Lux, W., Wise, A., Ransohoff, J. J. Neurol. Neurosurg. Psychiat. 1977, 40, 678. 3. Miller, J. D., Sakalas, R., Ward, J. D., Young, H. F., Adams, W E., Vries, J. K., Becker, D. P. Neurosurgery, 1977, 1, 114. 4. Stjernholm, M. R., Katz, F. H. J. clin. Endocr. Metab. 1975, 41, 887. 1

Fig. 2-Actuarial curve: remission (34 patients).

Fine-needle percutaneous transhepatic cholangiography.

1359 relapse during the gluten challenge was followed by remission after the second withdrawal of gluten from the diet. Department of Medicine, Hope...
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