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Long survival in hypoplastic left heart syndrome
53
(1-99 mmol/1 with normal serum proteins). A CT scan on day 5 demonstrated oedema extending backwards to the bursa omentalis, and a small necrotic zone in the pancreatic isthmus. On day 16, the posterior extension had vanished; the gland remained oedematous but there was no abdominal pain and the only abnormal test was a serum amylase of 300 IU/1. The cause of oedematous (and slightly necrotic) acute pancreatitis in our patient is unclear. There was no cholelithiasis, alcoholism, hyperlipidaemia, or pancreatoxic drug intake. An anatomical abnormality of the pancreas is a possibility; endoscopic retrograde pancreatography was ruled out because the procedure might have worsened the pancreatitis. Two other, possibly interrelated, mechanisms may be involved-namely, sensitivity to octreotide, a suspicion raised by the history of severe diarrhoea after only three injections; or a sharp reduction in exocrine pancreatic secretion, resulting in a more viscous mucosal flow, with mucus obstruction and subsequent pancreatitis. We suggest careful clinical and laboratory monitoring of pancreatic function, even during short-term administration of octreotide. Department of Internal Medicine, Diabetology, and Endocrinology, and Department of Surgery, Hôpital Pasteur, 06002 Nice, France
A. FREDENRICH C. SOSSET
J. L. BERNARD J. L. SADOUL P. FREYCHET
G, Beyer J, Krause U, Neufeld M, del Pozo E. Long-acting and selective suppression of growth hormone secretion by somatostatin analogue SMS 201-995 in acromegaly. Lancet 1984; ii: 782-84. 2. van Liessum PA, Hopman WP, Pieters GF, et al. Postprandial gallbladder motility during long-term treatment with the long-acting somatostatin analogue SMS 201-995 in acromegaly. J Clin Endocrinol Metab 1989; 69: 557-62. 3. Sadoul JL, Benchimol D, Thyss A, Freychet P. Acute pancreatitis following octreotide withdrawal. Am J Med (in press). 1. Plewe
Withdrawing antiepilepsy drugs SIR,-In treated epilepsy many patients attain a seizure-free period. Several studies have tackled subsequent questions-such as, how long to continue with antiepileptic drugs and how to discontinue medication. Nonetheless there are no generally accepted guidelines. Very few studies have compared the consequences of different policies on seizure-free interval, speed of
reduction, and withdrawal. Todt1 and Groh2 concluded that the risk clearly declined only after a remission of at least three years. Janz et all noted that a study on the discontinuation of antiepileptic drugs will always be a record of experience rather than of relapse
prospective trial. Methodological problems apart, prospective studies are restricted by ethical questions. For example, it has not been proved that abrupt withdrawal is worse than slow withdrawal-but who would dare to undertake a study of abrupt withdrawal? The UK Medical Research Council (May 18, p 1175) set up a prospective, pragmatic, multicentre randomised trial comparing slow withdrawal with continued therapy to study the relative risks of recurrence and to identify prognostically important risk factors. The study confirmed that epilepsy of long duration is an adverse factor, thus underlining the importance of the timely introduction of antiepileptic therapy. However, even a sample size of over 1000 patients could not give any firm, additional data for further a
strategies. In this randomised trial the actuarial relapse-rate at 2 years was 41% for the withdrawal group and 22% for those continuing therapy. In the Innsbruck seizure clinic’s experience of withdrawal attempts in 101 patients the relapse rate was 53-5%. 95% of those who relapsed achieved another remission, sometimes at doses smaller than those they had been on at the start of the withdrawal attempt. From our experience we would oppose the arguments of those physicians holding a conservative view on termination of therapy. Such continuation of the therapy offers no absolute safeguard while a withdrawal attempt offers a 50% chance of freedom from seizures without medication. After weighing up the consequences of a relapse (especially in respect of driving or
employment), clinicians should take into account a patient’s wishes antiepileptic prophylaxis. The prospective MRC study still provides safe guidelines.
to terminate
E. KRISTMANN
G. LUEF D. SOUCEK G. BAUER
Department of Neurology, University Hospital, 6020 Innsbruck, Austria
epilepsy in childhood: results of a prospective follow-up study. Epilepsia 1984; 25: 137-44. 2. Groh CH. Zur Frage der Heilbarkeit kindlicher Epilepsien. Klin Wochenschr 1975; 87: 1. Todt H. The late prognosis of
1-23. 3.
Janz D, Kern A. Mössinger H-J, Puhlmann H-U. Ruckfallprognose wahrend und nach Reduktion der Medikamente bie Epilepsiebehandlung. In: Remschmidt H, ed. Epilepsie 1981. Stuttgard: Thieme, 1983; 17-24.
Long survival in hypoplastic left syndrome
heart
SiR,—Dr Stuart and colleagues (April 20, p 957) state that no infant with hypoplastic left heart syndrome survives more than 4 months. We report an atypical case of prolonged survival. A newborn male baby had cyanosis and heart failure and was diagnosed elsewhere. He was sent home, and two months later he presented to our hospital, where the diagnosis was confirmed by echocardiography, chest radiography, and electrocardiography (ECG). His family was told of his short life expectancy and he was sent home. 10 months later, at age 12 months, he returned to our hospital with fever and respiratory insufficiency caused by pneumonia, which improved with oxygen and antibiotics. He weighed 83 kg and his height was 80 cm. Psychomotor development was normal. A systolic murmur, grade II/VI was detected in the 2nd left parasternal space with reinforced second sound; he had hepatomegaly of 4 cm. Pulse oximetry showed oxygen saturation of 70%. We noted cardiomegaly due to enlarged right atrium and ventricle, and left ventricle hypoplasia with a 4 mm diameter ascending aorta. Blood flow to the aorta was through the ductus arteriosus. Right ventricle and pulmonary trunk and veins were very dilated. He had an atrial septal defect and tricuspid insufficiency. Heart transplantation was proposed but in the interim he had pulmonary aspiration with secondary refractory cardiogenic shock and died. His very long survival and his acceptable physical and psychomotor development, despite not receiving cardiological treatment, emphasises the importance of adequate assessment and consideration of the possibility of heart transplantation in all children with this condition. M. J. RUIZ J. LÓPEZ-HERCE AMELIA TAMARIZ M. A. GARCÍA-TERESA
Pediatric Intensive Care Unit and Cardiology, Niño Jesús Hospital, Autonoma University of Madrid, 28009 Madrid, Spain
Epidermal growth factor in necrotising enteritis SIR,-Epidermal growth factor (EGF) stimulates epithelial cell proliferation in several tissues,’ including gastrointestinal mucosa.2 These properties have not found any notable application in intestinal disease.3 We report the use of intravenous EGF in an infant with necrotising enteritis. An 8-month-old girl was admitted with a 3-day history of anorexia, vomiting, and bloody diarrhoea. X-ray of the abdomen revealed fluid levels with dilated loops of bowel. Intussusception was not confirmed at laparotomy but the whole bowel was found to be dilated and dusky. She was critically ill postoperatively with disseminated intravascular coagulation and circulatory collapse, and she passed sloughed mucosa per rectum. A second laparotomy nine hours after the first revealed discoloration and dilatation of the entire small and large bowel with considerable free fluid in the peritoneum. Mesenteric blood vessels bled, albeit sluggishly. A jejunostomy was fashioned; mucosal sloughing was considerable. She required intensive care with ventilatory support and
(1-99 mmol/1 with normal serum proteins). A CT scan on day 5 demonstrated oedema extending backwards to the bursa omentalis, and a small necrotic zone in the pancreatic isthmus. On day 16, the posterior extension had vanished; the gland remained oedematous but there was no abdominal pain and the only abnormal test was a serum amylase of 300 IU/1. The cause of oedematous (and slightly necrotic) acute pancreatitis in our patient is unclear. There was no cholelithiasis, alcoholism, hyperlipidaemia, or pancreatoxic drug intake. An anatomical abnormality of the pancreas is a possibility; endoscopic retrograde pancreatography was ruled out because the procedure might have worsened the pancreatitis. Two other, possibly interrelated, mechanisms may be involved-namely, sensitivity to octreotide, a suspicion raised by the history of severe diarrhoea after only three injections; or a sharp reduction in exocrine pancreatic secretion, resulting in a more viscous mucosal flow, with mucus obstruction and subsequent pancreatitis. We suggest careful clinical and laboratory monitoring of pancreatic function, even during short-term administration of octreotide. Department of Internal Medicine, Diabetology, and Endocrinology, and Department of Surgery, Hôpital Pasteur, 06002 Nice, France
A. FREDENRICH C. SOSSET
J. L. BERNARD J. L. SADOUL P. FREYCHET
G, Beyer J, Krause U, Neufeld M, del Pozo E. Long-acting and selective suppression of growth hormone secretion by somatostatin analogue SMS 201-995 in acromegaly. Lancet 1984; ii: 782-84. 2. van Liessum PA, Hopman WP, Pieters GF, et al. Postprandial gallbladder motility during long-term treatment with the long-acting somatostatin analogue SMS 201-995 in acromegaly. J Clin Endocrinol Metab 1989; 69: 557-62. 3. Sadoul JL, Benchimol D, Thyss A, Freychet P. Acute pancreatitis following octreotide withdrawal. Am J Med (in press). 1. Plewe
Withdrawing antiepilepsy drugs SIR,-In treated epilepsy many patients attain a seizure-free period. Several studies have tackled subsequent questions-such as, how long to continue with antiepileptic drugs and how to discontinue medication. Nonetheless there are no generally accepted guidelines. Very few studies have compared the consequences of different policies on seizure-free interval, speed of
reduction, and withdrawal. Todt1 and Groh2 concluded that the risk clearly declined only after a remission of at least three years. Janz et all noted that a study on the discontinuation of antiepileptic drugs will always be a record of experience rather than of relapse
prospective trial. Methodological problems apart, prospective studies are restricted by ethical questions. For example, it has not been proved that abrupt withdrawal is worse than slow withdrawal-but who would dare to undertake a study of abrupt withdrawal? The UK Medical Research Council (May 18, p 1175) set up a prospective, pragmatic, multicentre randomised trial comparing slow withdrawal with continued therapy to study the relative risks of recurrence and to identify prognostically important risk factors. The study confirmed that epilepsy of long duration is an adverse factor, thus underlining the importance of the timely introduction of antiepileptic therapy. However, even a sample size of over 1000 patients could not give any firm, additional data for further a
strategies. In this randomised trial the actuarial relapse-rate at 2 years was 41% for the withdrawal group and 22% for those continuing therapy. In the Innsbruck seizure clinic’s experience of withdrawal attempts in 101 patients the relapse rate was 53-5%. 95% of those who relapsed achieved another remission, sometimes at doses smaller than those they had been on at the start of the withdrawal attempt. From our experience we would oppose the arguments of those physicians holding a conservative view on termination of therapy. Such continuation of the therapy offers no absolute safeguard while a withdrawal attempt offers a 50% chance of freedom from seizures without medication. After weighing up the consequences of a relapse (especially in respect of driving or
employment), clinicians should take into account a patient’s wishes antiepileptic prophylaxis. The prospective MRC study still provides safe guidelines.
to terminate
E. KRISTMANN
G. LUEF D. SOUCEK G. BAUER
Department of Neurology, University Hospital, 6020 Innsbruck, Austria
epilepsy in childhood: results of a prospective follow-up study. Epilepsia 1984; 25: 137-44. 2. Groh CH. Zur Frage der Heilbarkeit kindlicher Epilepsien. Klin Wochenschr 1975; 87: 1. Todt H. The late prognosis of
1-23. 3.
Janz D, Kern A. Mössinger H-J, Puhlmann H-U. Ruckfallprognose wahrend und nach Reduktion der Medikamente bie Epilepsiebehandlung. In: Remschmidt H, ed. Epilepsie 1981. Stuttgard: Thieme, 1983; 17-24.
Long survival in hypoplastic left syndrome
heart
SiR,—Dr Stuart and colleagues (April 20, p 957) state that no infant with hypoplastic left heart syndrome survives more than 4 months. We report an atypical case of prolonged survival. A newborn male baby had cyanosis and heart failure and was diagnosed elsewhere. He was sent home, and two months later he presented to our hospital, where the diagnosis was confirmed by echocardiography, chest radiography, and electrocardiography (ECG). His family was told of his short life expectancy and he was sent home. 10 months later, at age 12 months, he returned to our hospital with fever and respiratory insufficiency caused by pneumonia, which improved with oxygen and antibiotics. He weighed 83 kg and his height was 80 cm. Psychomotor development was normal. A systolic murmur, grade II/VI was detected in the 2nd left parasternal space with reinforced second sound; he had hepatomegaly of 4 cm. Pulse oximetry showed oxygen saturation of 70%. We noted cardiomegaly due to enlarged right atrium and ventricle, and left ventricle hypoplasia with a 4 mm diameter ascending aorta. Blood flow to the aorta was through the ductus arteriosus. Right ventricle and pulmonary trunk and veins were very dilated. He had an atrial septal defect and tricuspid insufficiency. Heart transplantation was proposed but in the interim he had pulmonary aspiration with secondary refractory cardiogenic shock and died. His very long survival and his acceptable physical and psychomotor development, despite not receiving cardiological treatment, emphasises the importance of adequate assessment and consideration of the possibility of heart transplantation in all children with this condition. M. J. RUIZ J. LÓPEZ-HERCE AMELIA TAMARIZ M. A. GARCÍA-TERESA
Pediatric Intensive Care Unit and Cardiology, Niño Jesús Hospital, Autonoma University of Madrid, 28009 Madrid, Spain
Epidermal growth factor in necrotising enteritis SIR,-Epidermal growth factor (EGF) stimulates epithelial cell proliferation in several tissues,’ including gastrointestinal mucosa.2 These properties have not found any notable application in intestinal disease.3 We report the use of intravenous EGF in an infant with necrotising enteritis. An 8-month-old girl was admitted with a 3-day history of anorexia, vomiting, and bloody diarrhoea. X-ray of the abdomen revealed fluid levels with dilated loops of bowel. Intussusception was not confirmed at laparotomy but the whole bowel was found to be dilated and dusky. She was critically ill postoperatively with disseminated intravascular coagulation and circulatory collapse, and she passed sloughed mucosa per rectum. A second laparotomy nine hours after the first revealed discoloration and dilatation of the entire small and large bowel with considerable free fluid in the peritoneum. Mesenteric blood vessels bled, albeit sluggishly. A jejunostomy was fashioned; mucosal sloughing was considerable. She required intensive care with ventilatory support and