- Email: [email protected]
CYSTIC FIBROSIS WITH AND WTTHOUT MECONIUM ILEUS
161
identify patients who justify full appraisal for surgical treatment and who ultimately come to operation. Selecting only patients with a clear history of early febrile convulsions or well-defined structural lesions and using unsophisticated neurophysiological techniques (latterly including foramen ovale recording) as well as standard neuropsychological and neuroradiological appraisal we have done 13 operations with excellent results. The demand for investigation is accelerating. We already have a four to six month waiting list for admission. Whilst our facilities are rudimentary and the follow-up times for outpatients short, we obtain excellent results with 80% of patients operated upon being seizure-free postoperatively. We do not, doubt, however, that we are turning away some patients who might benefit from surgery because our investigational facilities are inadequate. We concur with the notion of supraregional specialisation to achieve the experience in assessment and the expertise in the functional neurosurgery necessary
to
offer the best results. But the
suggestion that only two such centres might be necesary in the UK seriously underestimates the size of the problem. Department of Neurological Science, Walton Hospital, Liverpool L9 1AE
JOHN MILES DAVID CHADWICK
CYSTIC FIBROSIS WITH AND WTTHOUT MECONIUM ILEUS
SIR,-Mornet
Upper: pharyngeal and tracheal activity. Lower: slight pharyngeal but no tracheal activity.
Farnihes with
reflux should now be transmission.
thought of as the underlying mechanism of
The disadvantages of a reduced bactericidal activity and the selection of multiresistent gram-negative intragastric species by broad-spectrum antibiotics in an intensive care setting’ call into question the routine use of stress ulcer prophylaxis in patients in acute respiratory failure. The value of antacid therapy may not outweigh the morbidity and mortality from severe bronchopulmonary infections3 which, our preliminary results suggest, is promoted by this treatment. Further clinical trials are needed to define which patients will benefit from preventive antacid treatment or alternative approaches such as sterilising the stomach by non-absorbable antimicrobial agents.
J. NACHTKAMP Departments of Surgery, Nuclear Medicine, and Internal Medicine
III,
Klinikum der RWTH, 5100 Aachen, West Germany
et all have
R. BARES G. WINKELTAU U. KLINGE M. M. LERCH
1 Gianella RA, Broitman SA, Zamcheck N Gastric acid barrier to ingested microorganisms in man studies in vivo and in vitro Gut 1972, 13: 251-56. 2 Atherton ST, White DJ Stomach as source of bacteria colonising respiratory tract during artificial ventilation Lancet 1978, ii. 968-69 3. du Moulin GC, Paterson DG, Hedley-White J, Lisbon A Aspiration of gastric bactena in antacid-treated patients a frequent cause of postoperative colonisation of the airway Lancet 1982; i. 242-45.
with
at
niecoizitaii
least one child ileus*
CFhaplorypeD,E2t
on
linkage
between different
SiR,—The points made in your editorial (Nov 12, p 1115)
are
experience emphasises the need for greater awareness among UK neurologists of the possibilities for helping patients with intractable epilepsy by a surgical approach. One of us (D. W. C.) has an extensive practice in epilepsy in a region with a catchment population of about three million. About two and a half years ago we began to develop a surgical service for the treatment of patients with epilepsy because of the long waiting times imposed on patients with epilepsy by referral to one or other of the two centres in the UK with an interest in the surgical treatment of eilepsy. We have been surprised at the frequency with which we
Mornet
12,’97 112%)) 155194 t80%)l
17/41 (41 %)) 62/74184%)I
8 % 12 r50%) 65 138% )/
6 79% 20 62% 26 162%z 10 I 24 %z
et
al
pallemS4. with ileus, D,EF, with ileus, D,E2F, without ileus, D,E2F2 without ileus, D,EF,
70 47 %.
*In our study there was only I CF cluld with neonatal ileus per family. CF was confirmed by sweat test tProbes XV-2c, KM-19, and J3-11 used to identify alleles D:-D2, B,-, and
F,-F respectively. tprequencies have been calculated within each group with or without ileus
frequency of CF families with ileus acccrds with previous reports23but is much less than in Mornet’s series. Our results
Our
confirm the strong association between DIE2 and the CF mutation in both clinical forms, and the frequency of this haplotype (19%) in our controls resembled that in Mornet’s (16%). Study of the frequencies of D,E2F2 and D1E2F1 haplotypes suggests that CF patients with and without ileus are not significantly different, in contrast with Momet’s findings. Although the CF population with ileus in our series and in that of Mornet et al are not significantly different from the two CF populations without ileus they do differ significantly in respect of these haplotypes. We thank R. Williamson for the recombinant plasmids used
to
prepare
probes.
Hôpital Debrousse, 69322 Lyon, France
SURGERY FOR TEMPORAL LOBE EPILEPSY
Our senes ( 105 children)
,Most frequent CF haplotypes in
Hormone Biochemistry Laboratory, INSERM Unit 34,
valid. Our local
reported
clinical forms of cystic fibrosis (CF) and haplotypes identified by association of restriction fragment length polymorphisms (RFLP). We can confirm some of their findings but not all:
INSERM Unit 91,
M. AUVINET Y. MOREL V. CHAMBON J. ANDRE
Creteil
M. VIDAUD M. GOOSSENS
Children’s Medical Clinic, Centre Hospitalier Lyon Sud
G. BELLON R. GILLY
Hôpital Henri Mondor,
1. Mornet E, Serre JL, Farall M, et al. Genetic differences between cystic fibrosis with and without meconium ileus. Lancet 1988; ii: 376-78. 2. Allan JL, Robbie M, Pelan PD, Danks DM. Familial occurrence of meconium ileus. Eur J Pediatr 1981; 135: 291-92. 3. Gilly R, Bellon G Mucoviscidose (maladie fibro-kystique du pancréas). Encycl Med Chir (Paris) Pédiatr 1988; 406: 10-12.
identify patients who justify full appraisal for surgical treatment and who ultimately come to operation. Selecting only patients with a clear history of early febrile convulsions or well-defined structural lesions and using unsophisticated neurophysiological techniques (latterly including foramen ovale recording) as well as standard neuropsychological and neuroradiological appraisal we have done 13 operations with excellent results. The demand for investigation is accelerating. We already have a four to six month waiting list for admission. Whilst our facilities are rudimentary and the follow-up times for outpatients short, we obtain excellent results with 80% of patients operated upon being seizure-free postoperatively. We do not, doubt, however, that we are turning away some patients who might benefit from surgery because our investigational facilities are inadequate. We concur with the notion of supraregional specialisation to achieve the experience in assessment and the expertise in the functional neurosurgery necessary
to
offer the best results. But the
suggestion that only two such centres might be necesary in the UK seriously underestimates the size of the problem. Department of Neurological Science, Walton Hospital, Liverpool L9 1AE
JOHN MILES DAVID CHADWICK
CYSTIC FIBROSIS WITH AND WTTHOUT MECONIUM ILEUS
SIR,-Mornet
Upper: pharyngeal and tracheal activity. Lower: slight pharyngeal but no tracheal activity.
Farnihes with
reflux should now be transmission.
thought of as the underlying mechanism of
The disadvantages of a reduced bactericidal activity and the selection of multiresistent gram-negative intragastric species by broad-spectrum antibiotics in an intensive care setting’ call into question the routine use of stress ulcer prophylaxis in patients in acute respiratory failure. The value of antacid therapy may not outweigh the morbidity and mortality from severe bronchopulmonary infections3 which, our preliminary results suggest, is promoted by this treatment. Further clinical trials are needed to define which patients will benefit from preventive antacid treatment or alternative approaches such as sterilising the stomach by non-absorbable antimicrobial agents.
J. NACHTKAMP Departments of Surgery, Nuclear Medicine, and Internal Medicine
III,
Klinikum der RWTH, 5100 Aachen, West Germany
et all have
R. BARES G. WINKELTAU U. KLINGE M. M. LERCH
1 Gianella RA, Broitman SA, Zamcheck N Gastric acid barrier to ingested microorganisms in man studies in vivo and in vitro Gut 1972, 13: 251-56. 2 Atherton ST, White DJ Stomach as source of bacteria colonising respiratory tract during artificial ventilation Lancet 1978, ii. 968-69 3. du Moulin GC, Paterson DG, Hedley-White J, Lisbon A Aspiration of gastric bactena in antacid-treated patients a frequent cause of postoperative colonisation of the airway Lancet 1982; i. 242-45.
with
at
niecoizitaii
least one child ileus*
CFhaplorypeD,E2t
on
linkage
between different
SiR,—The points made in your editorial (Nov 12, p 1115)
are
experience emphasises the need for greater awareness among UK neurologists of the possibilities for helping patients with intractable epilepsy by a surgical approach. One of us (D. W. C.) has an extensive practice in epilepsy in a region with a catchment population of about three million. About two and a half years ago we began to develop a surgical service for the treatment of patients with epilepsy because of the long waiting times imposed on patients with epilepsy by referral to one or other of the two centres in the UK with an interest in the surgical treatment of eilepsy. We have been surprised at the frequency with which we
Mornet
12,’97 112%)) 155194 t80%)l
17/41 (41 %)) 62/74184%)I
8 % 12 r50%) 65 138% )/
6 79% 20 62% 26 162%z 10 I 24 %z
et
al
pallemS4. with ileus, D,EF, with ileus, D,E2F, without ileus, D,E2F2 without ileus, D,EF,
70 47 %.
*In our study there was only I CF cluld with neonatal ileus per family. CF was confirmed by sweat test tProbes XV-2c, KM-19, and J3-11 used to identify alleles D:-D2, B,-, and
F,-F respectively. tprequencies have been calculated within each group with or without ileus
frequency of CF families with ileus acccrds with previous reports23but is much less than in Mornet’s series. Our results
Our
confirm the strong association between DIE2 and the CF mutation in both clinical forms, and the frequency of this haplotype (19%) in our controls resembled that in Mornet’s (16%). Study of the frequencies of D,E2F2 and D1E2F1 haplotypes suggests that CF patients with and without ileus are not significantly different, in contrast with Momet’s findings. Although the CF population with ileus in our series and in that of Mornet et al are not significantly different from the two CF populations without ileus they do differ significantly in respect of these haplotypes. We thank R. Williamson for the recombinant plasmids used
to
prepare
probes.
Hôpital Debrousse, 69322 Lyon, France
SURGERY FOR TEMPORAL LOBE EPILEPSY
Our senes ( 105 children)
,Most frequent CF haplotypes in
Hormone Biochemistry Laboratory, INSERM Unit 34,
valid. Our local
reported
clinical forms of cystic fibrosis (CF) and haplotypes identified by association of restriction fragment length polymorphisms (RFLP). We can confirm some of their findings but not all:
INSERM Unit 91,
M. AUVINET Y. MOREL V. CHAMBON J. ANDRE
Creteil
M. VIDAUD M. GOOSSENS
Children’s Medical Clinic, Centre Hospitalier Lyon Sud
G. BELLON R. GILLY
Hôpital Henri Mondor,
1. Mornet E, Serre JL, Farall M, et al. Genetic differences between cystic fibrosis with and without meconium ileus. Lancet 1988; ii: 376-78. 2. Allan JL, Robbie M, Pelan PD, Danks DM. Familial occurrence of meconium ileus. Eur J Pediatr 1981; 135: 291-92. 3. Gilly R, Bellon G Mucoviscidose (maladie fibro-kystique du pancréas). Encycl Med Chir (Paris) Pédiatr 1988; 406: 10-12.