15 may be necessary not only for the efficient absorption of vitamin E but also for other fatsoluble vitamins. In contrast to cystic fibrosis, fat malabsorption is well controlled by pancreatic extract therapy but the growth retardation, bone changes and himatological abnormalities persist. The pathogenesis of the condition is not understood. Embryological development of pancreatic acinar cells and myeloid tissue occurs at about the same time, and it has been suggested that intrauterine infection may play a role in pathogenesis (Hruban et al. 1963). This seems unlikely as the condition may occur in siblings of successive pregnancies, and in one family both the father and daughter had neutropenia (Burke et al. 1967). A familial incidence has been noted by most authors, and our case with an affected sibling supports an autosomal recessive mode of inheritance for the condition. Acknowledgments: Thanks are due to the Wellcome Trust and to Roche Products Ltd for financial support. REFERENCES Burke V, Colebatch J H, Anderson C M & Simons M J (1967) Archives of Diseases in Childhood 42, 147 Doe W F (1973) Proceedings of the Royal Society of Medicine 66, 1125 Hruban Z, Oda Y, Warner N E & Wright F H (1963) Archives ofPathology 76, 226 Hudson E & Aldor T (1970) Archives of Internal Medicine 125, 314 Nezelof C & Watchi M (1961) Archives FranCaises de Pddiatrie 18, 1135 Shwachman H, Diamond L K, Oski F A & Khaw K (1964) Journal ofPediatrics 65, 645
Section ofPediatrics
305
iA A
.. , EX
Fig 1 Paroxysmal ventricular tachycardia. ECG, September 1973: A, immediately after exercise. B, after I min rest, showing coupled beats
Nevertheless he was given phenytoin 50 mg twice daily, with initial improvement. September 1973: dizziness and fainting following exercise recurred. He was shown to have exercise-induced paroxysmal ventricular tachycardia (Fig IA, B), but with no evidence of congenital or rheumatic heart disease, myocarditis or cardiomyopathy. Propranolol 5 mg three times daily controlled the rhythm and no further fainting episodes or dizziness have occurred. Recently the dose has been increased because a repeat exercise ECG showed recurrence of tachycardia, without clinical symptoms.
Comment Paroxysmal ventricular tachycardia (PVT) is a rare condition in childhood (Videbaeck et al. 1973) and may be associated with underlying Paroxysmal Ventricular Tachycardia heart disease (Anthony et al. 1966, Armbrust & of Unknown Etiology Levine 1950). The prognosis is generally better in Caryl W Darby' MB MRCP the idiopathic group (Palaganas et al. 1965) (Queen Elizabeth Hospitalfor Children, although sudden death in syncopal attacks due to London E2) superimposed ventricular fibrillation has been reported (Cohen et al. 1967). Boy, aged 9 No ECG recordings have been made during this Presented in December 1971, following two child's episodes of unconsciousness. His attacks fainting episodes during swimming. In March. have always been induced by exercise. He has 1970 had fainted whilst playing and sustained a never experienced palpitations, angina, dyspnoea minor head injury. Initial examination normal. or abdominal pain and has never been in conECG: sinus bradycardia (rate 55/min); attacks gestive cardiac failure, presumably because his thought to be due to vagal overactivity. Episodes episodes have been short-lived and self-limiting. of unconsciousness after exercise, usually associ- The resting ECG shows no changes in P wave ated with urinary incontinence, continued. EEG direction or intensity suggestive of the sick sinus (August 1972): slow, disorganized background syndrome (Scott & Macartney 1974) but the rhythm not diagnostic of idiopathic epilepsy. exercise ECG fulfils most of the criteria for PVT (Robinson & Hermann 1921). This shows an 'Present address: Hospital for Sick Children, Great Ormond Street, London WCI irregular heart rhythm, with auricular complexes
306
Proc. roy. Soc. Med. Volume 68 May 1975
16
independent of and slower than the ventricular complexes, which are bizarre and broadened. Meeting 29 June 1974 Ventricular captures or fusion beats occur when at Queen Mary's Hospitalfor Children, the rhythms become superimposed. His improve- Carshalton, Surrey ment on phenytoin is explained by its quinidinelike action on myocardial conductivity (Leonard The following cases were shown: 1958, Conn 1965). The initial clinical improvement delayed the diagnosis of the true cause of Moebius Syndrome Dr J Hammond the attacks. Propranolol, having both a /3adrenergic blocking action and a quinidine-like Unusual Hypothyroidism muscle depressant action, is being given for long- Dr A T Piesowicz term prophylaxis of this child's tachycardia and he has been advised to avoid strenuous activity. Ectopic Duplex Ureter Mr H B Eckstein Conduction defects may be further localized by intracardiac electrography, most recently desc- Negram Intoxication Dr Y F Ransley ribed by Krikler (1974). Using these techniques, abnormal or retrograde conduction in the bundle Juvenile Batten's (Spielmeyer-Vogt) of His may be demonstrated and in an intractable Disease Dr E M Brett arrhythmia surgery may be contemplated. Two Unusual Diaphragmatic Hernias REFERENCES Anthony C L, Stuart L D, Plunket D C & Czarnecki S W (1966) American Journal of Diseases of Children 112, 469 Arnbrust C A & Levine S A (1950) Circulation 1, 28 Cohen L S, Buccino R S, Morrow A G & Braunwald E (1967) Annals ofInternal Medicine 66, 945 Conn R D (1965) New England Journal of Medicine 272, 277 Krikler D M (1974) Lancet i, 974 Leonard W A (1958) Archives of Internal Medicine 101, 714 Palaganas M C, Fay J E & Delahaye D J (1965) Journal ofPediatrics 67, 784 Robinson G C & Hermann G R (1921) Heart 8,59 Scott 0 & Macartney F J (1974) Archives of Disease in Childhood49, 819 Videbaek J, Andersen E D, Jacobsen J R, Sandoe E & Wennevold A (1973) Acta pwdiatrica Scandinavica 62, 349
Dr R Bentall
The following short papers were presented:
Hyperuricemic Encephalopathy Dr C W Kesson Non-ketotic Hyperglycinmemia Dr C J de Groot The Changing Pattern of Spina Bifida Dr D M Forrest
Artificial Bladder Stimulation Mr J Nicholas Neurophysiological Techniques in Paxdiatrics Dr R Harris
The following cases were also presented:
Neurofibromatosis in Childhood Menkes' Kinky Hair Syndrome Dr J R Norman (for Dr J W Scopes) (Health Centre, Henfield, Sussex)
Mr T L Bowen (On account of illness, this paper was not read.)
Neonatal Herpesvirus Encephalitis Dr R S Brown (for Dr Brian Bower) (Radeliffe Infirmary, Oxford, OX2 6HE)
The following demonstrations were given: The Diamond Riding Centre for the Handicapped, Carshalton Mr K Webb and Mr M Bridson
The following Guest Lecture was delivered: Neonatal Hypoglycaemia Dr H Loeb and Dr A Pardou (Hopital Universitaire St Pierre, Bruxelles, Belgium)
Regional Infant Screening Service Mrs Judith Porter, Mr D J T Starr and Dr J Stern Medical Engineering Research Unit Mr W T F Bond
Section ofPediatrics
305
iA A
.. , EX
Fig 1 Paroxysmal ventricular tachycardia. ECG, September 1973: A, immediately after exercise. B, after I min rest, showing coupled beats
Nevertheless he was given phenytoin 50 mg twice daily, with initial improvement. September 1973: dizziness and fainting following exercise recurred. He was shown to have exercise-induced paroxysmal ventricular tachycardia (Fig IA, B), but with no evidence of congenital or rheumatic heart disease, myocarditis or cardiomyopathy. Propranolol 5 mg three times daily controlled the rhythm and no further fainting episodes or dizziness have occurred. Recently the dose has been increased because a repeat exercise ECG showed recurrence of tachycardia, without clinical symptoms.
Comment Paroxysmal ventricular tachycardia (PVT) is a rare condition in childhood (Videbaeck et al. 1973) and may be associated with underlying Paroxysmal Ventricular Tachycardia heart disease (Anthony et al. 1966, Armbrust & of Unknown Etiology Levine 1950). The prognosis is generally better in Caryl W Darby' MB MRCP the idiopathic group (Palaganas et al. 1965) (Queen Elizabeth Hospitalfor Children, although sudden death in syncopal attacks due to London E2) superimposed ventricular fibrillation has been reported (Cohen et al. 1967). Boy, aged 9 No ECG recordings have been made during this Presented in December 1971, following two child's episodes of unconsciousness. His attacks fainting episodes during swimming. In March. have always been induced by exercise. He has 1970 had fainted whilst playing and sustained a never experienced palpitations, angina, dyspnoea minor head injury. Initial examination normal. or abdominal pain and has never been in conECG: sinus bradycardia (rate 55/min); attacks gestive cardiac failure, presumably because his thought to be due to vagal overactivity. Episodes episodes have been short-lived and self-limiting. of unconsciousness after exercise, usually associ- The resting ECG shows no changes in P wave ated with urinary incontinence, continued. EEG direction or intensity suggestive of the sick sinus (August 1972): slow, disorganized background syndrome (Scott & Macartney 1974) but the rhythm not diagnostic of idiopathic epilepsy. exercise ECG fulfils most of the criteria for PVT (Robinson & Hermann 1921). This shows an 'Present address: Hospital for Sick Children, Great Ormond Street, London WCI irregular heart rhythm, with auricular complexes
306
Proc. roy. Soc. Med. Volume 68 May 1975
16
independent of and slower than the ventricular complexes, which are bizarre and broadened. Meeting 29 June 1974 Ventricular captures or fusion beats occur when at Queen Mary's Hospitalfor Children, the rhythms become superimposed. His improve- Carshalton, Surrey ment on phenytoin is explained by its quinidinelike action on myocardial conductivity (Leonard The following cases were shown: 1958, Conn 1965). The initial clinical improvement delayed the diagnosis of the true cause of Moebius Syndrome Dr J Hammond the attacks. Propranolol, having both a /3adrenergic blocking action and a quinidine-like Unusual Hypothyroidism muscle depressant action, is being given for long- Dr A T Piesowicz term prophylaxis of this child's tachycardia and he has been advised to avoid strenuous activity. Ectopic Duplex Ureter Mr H B Eckstein Conduction defects may be further localized by intracardiac electrography, most recently desc- Negram Intoxication Dr Y F Ransley ribed by Krikler (1974). Using these techniques, abnormal or retrograde conduction in the bundle Juvenile Batten's (Spielmeyer-Vogt) of His may be demonstrated and in an intractable Disease Dr E M Brett arrhythmia surgery may be contemplated. Two Unusual Diaphragmatic Hernias REFERENCES Anthony C L, Stuart L D, Plunket D C & Czarnecki S W (1966) American Journal of Diseases of Children 112, 469 Arnbrust C A & Levine S A (1950) Circulation 1, 28 Cohen L S, Buccino R S, Morrow A G & Braunwald E (1967) Annals ofInternal Medicine 66, 945 Conn R D (1965) New England Journal of Medicine 272, 277 Krikler D M (1974) Lancet i, 974 Leonard W A (1958) Archives of Internal Medicine 101, 714 Palaganas M C, Fay J E & Delahaye D J (1965) Journal ofPediatrics 67, 784 Robinson G C & Hermann G R (1921) Heart 8,59 Scott 0 & Macartney F J (1974) Archives of Disease in Childhood49, 819 Videbaek J, Andersen E D, Jacobsen J R, Sandoe E & Wennevold A (1973) Acta pwdiatrica Scandinavica 62, 349
Dr R Bentall
The following short papers were presented:
Hyperuricemic Encephalopathy Dr C W Kesson Non-ketotic Hyperglycinmemia Dr C J de Groot The Changing Pattern of Spina Bifida Dr D M Forrest
Artificial Bladder Stimulation Mr J Nicholas Neurophysiological Techniques in Paxdiatrics Dr R Harris
The following cases were also presented:
Neurofibromatosis in Childhood Menkes' Kinky Hair Syndrome Dr J R Norman (for Dr J W Scopes) (Health Centre, Henfield, Sussex)
Mr T L Bowen (On account of illness, this paper was not read.)
Neonatal Herpesvirus Encephalitis Dr R S Brown (for Dr Brian Bower) (Radeliffe Infirmary, Oxford, OX2 6HE)
The following demonstrations were given: The Diamond Riding Centre for the Handicapped, Carshalton Mr K Webb and Mr M Bridson
The following Guest Lecture was delivered: Neonatal Hypoglycaemia Dr H Loeb and Dr A Pardou (Hopital Universitaire St Pierre, Bruxelles, Belgium)
Regional Infant Screening Service Mrs Judith Porter, Mr D J T Starr and Dr J Stern Medical Engineering Research Unit Mr W T F Bond
