256
agents, took 50 mg for 14 days with no significant alteration in blood glucose control or precipitation of hypoglycaemia. We suggest that fluconazole is a suitable treatment for vulvovaginitis in diabetic women and that if the patient is taking an oral hypoglycaemic agent there is little risk of clinical hypoglycaemia. Department of Medicine, University of Birmingham, East Birmingham Hospital, Birmingham B9 5ST, UK
BETH R. ROWE JOHN THORPE ANTHONY BARNETT
JD, Wilner KD. Drug interactions with fluconazole. Rev Infect Dis 1991; 12 (suppl 3): 328-29. 2. Report of an international trial. A comparison of single-dose oral fluconazole with 3-day intravaginal clotrimazole in the treatment of vaginal candidiasis. Br J Obstet Gynaecol 1989; 96: 226-32. 3. Brammer KW. Clinical experience with fluconazole in hospital practice. In: Richardson RG, ed. Fluconazole and its role in vaginal candidiasis. Int Congr Symp Ser Roy Soc Med 1989; 160: 29-31. 1. Lazar
Postpartum idiopathic polymyositis SIR,-Several lines of evidence suggest that autoimmune mechanisms have a role in the pathogenesis of idiopathic polymyositis.l We have lately seen a woman in whom polymyositis developed after delivery. This prompted us to look for a possible link between delivery and polymyositis, since several other putative immune disorders such as myasthenia gravis.1 systemic lupus erythematosus,3 and multiple sclerosis4 have been reported to present or to relapse postpartum. We therefore reviewed the records of our polymyositis patients diagnosed during the past decade. 22 patients fulfilled at least three of the following diagnostic criteria: (1) suggestive clinical picture, consisting of progressive symmetrical limb weakness of subacute onset; (2) increased serum muscle enzymes; (3) electromyographic (EMG) evidence of active muscle disease; (4) skeletal muscle biopsy showing muscle fibre necrosis and regeneration with inflammation; and (5) beneficial response to immunomodulating therapy. In 8 (36%) of the 22 women the disorder developed during childbearing age (age range 20-41 years) and 3 of them (38%) presented with polymyositis within three months of delivery. Patient 1-Leg weakness started to develop in a 21-year-old woman a month after delivery of her first child. The weakness slowly progressed to affect all four limbs and neck flexors. She was admitted seven months later and polymyositis was diagnosed on the basis of raised serum creatine kinase (CK) (4976 IU), EMG pattern with fibrillations, and characteristic muscle biopsy findings. Her symptoms were resistant to immunotherapy but eventually responded to a combination of methylprednisolone and
cyclophosphamide. Patient 2-A 23-year-old woman noticed muscle ache and weakness in her hands and legs within two months of giving birth to her second child. Although serum CK concentrations were normal, EMG results were suggestive of active muscle disease, and a muscle biopsy demonstrated muscle-fibre necrosis and inflammatory reaction. Muscle weakness responded to prednisone. In the next seven years she had several relapses, which were controlled with steroids. During the same period she had two normal pregnancies and deliveries, one of which was associated with mild exacerbation of disease postpartum. Patient 3-This woman gave birth to her first child at the age of 24. Two and a half months later, she had rapidly progressive hand weakness associated with swelling in her forearms, hands, and fingers. On admission she had upper limb weakness (3/5 [MRC scale] distally, and 4/5 in the shoulder girdle and upper arm muscles) and slight neck flexor weakness. Her condition deteriorated rapidly and within four weeks of disease onset she had striking weakness in all limbs. Polymyositis was diagnosed on the basis of a serum CK concentration of 5000 IU, typical EMG findings, and muscle histology. She responded well to prednisone therapy but tapering off trials resulted in clinical and biochemical relapses. At present, a year and a half after diagnosis, she is maintained on a combination of low-dose steroids and methotrexate. These three women had polymyositis that first manifested within a few months of delivery. Although an increased frequency of other putative immune disorders has been reported during the
postpartum period,2-4 we are unaware of other reports suggesting such a pattern in polymyositis. We believe that the association between polymyositis and delivery is not coincidental, although the exact pathogenetic mechanisms causing such conditions after delivery are poorly understood. Hormonal changes associated with pregnancy and delivery, such as altered progesterone and corticosteroid concentrations, have been proposed.5 Synthesis of corticosteroids, which suppress polymyositis, is increased during pregnancy and returns to normal after delivery. Additionally, the resolution of the natural immunosuppressive state that exists during pregnancy because of the action of substances with
immunosuppressive properties-such as alpha-fetoprotein’*—may be contributory. Whatever the mechanism is, this report adds yet another condition to the list of immune disorders presenting or worsening post delivery. Departments of Neurology and Medicine (Mount Scopus), Hadassah University Hospital, Hebrew University-Hadassah Medical School, Jerusalem 91120, Israel
ISRAEL STEINER LEA AVERBUCH-HELLER ODED ABRAMSKY EYAL RAZ
1. Dalakas MC. Polymyositis, dermatomyositis and inclusion body myositis. N Engl J Med 1991; 325: 1487-98. 2. Fraser D, Turner JWA. Myasthenia gravis and pregnancy. Proc R Soc Med 1963; 56: 379-81. 3. Garsenstein M, Pollack V, Kark RM. Systemic lupus erythematosus and pregnancy N Engl J Med 1962; 267: 165-69. 4. Korn-Lubetzki I, Kahana E, Cooper G, Abramsky O. Activity of multiple sclerosis during pregnancy and puerperium. Ann Neurol 1984; 16: 229-31. 5. Arnason BG, Richman DP. Effect of oral contraceptives on experimental demyelinating disease. Arch Neurol 1969; 21: 103-08. 6. Abramsky O, Brenner T, Mizrachi R, Soffer D. Alpha fetoprotein suppresses experimental allergic encephalomyelitis.J Neuroimmunol 1982; 2: 1-7.
Malignant hyperthermia and Hirschsprung’s disease SIR,-Dr Elechi and Dr Dakaraju (Nov 9, p 1206) suggest a link between malignant hyperthermia (MH) and Hirschsprung’s disease (HD). Between 1986 and 1991, 17 cases of biopsy-proven HD were seen at this hospital (12 males, 5 females; aged 24 days to 9 years). These patients had general anaesthesia 38 times, for temporary colostomy, definitive correction, and so on. 11 definitive operations were done (7 Swenson’s procedures). In the hospital the most commonly used agents for general anaesthesia are
succinylcholine and halothane.
Yet MH has never been encountered here since 1983, either in patients with HD or anyone else. We have not found any record of MH in three large series totalling 3325 patients with HD.1-3 The experience of HD as a common cause of large bowel obstruction in children in the Port Harcourt region of Nigeria may be related to genetic factors. It has been reported that a family history is positive in 3-7% of children with HD, and the familial incidence rose to 21 % as the aganghonic segment extended to caecum.4 Another suggestion is that MH has an autosomal dominant mode of inheritance.5 The frequency and co-existence of diseases with familial inheritance can be expected to be high in regions with homogeneous populations.
Departments of Paediatrics, Surgery, and Paediatric Surgery, Selçuck University Medical Faculty, Konya, Turkey
HALÛK YAVUZ AHMET ÖZEL ADNAN KAYNAK ALÂADDIN DILSIZ ÌBRAHIM ERKUL
Swenson O, Sherman JO, Fisher JH. Diagnosis of congenital megacolon: an analysis of 501 patients. J Pediatr Surg 1973; 8: 587-94. 2. Kleinhaus S, Boley SJ, Sheran M, Sieber WK. Hirschsprung’s disease: a survey of the members of the surgical section of the American Academy of Pediatrics. J Pediatr Surg 1979; 14: 588-97. 3. Ikeda K, Goto S. Diagnosis and treatment of Hirschsprung’s disease in Japan. An analysis of 1628 patients. Ann Surg 1984; 199: 400-05. 4. Lipson AH, Harvey J. Three-generation transmission of Hirschsprung’s disease. Clin Genet 1987; 32: 175-78. 5. Sessler DI. Malignant hyperthermia. J Pediatr 1986; 109: 9-14. 1.
CORRECTION Trends in child growth from a single cross-sectionalsurvey.-In the table in Prof C. A. Monteiro and Dr A. M. Torres’ letter Jan 18, p 192) the 1989 height for 22-year-old males should read 169 Ocm.
agents, took 50 mg for 14 days with no significant alteration in blood glucose control or precipitation of hypoglycaemia. We suggest that fluconazole is a suitable treatment for vulvovaginitis in diabetic women and that if the patient is taking an oral hypoglycaemic agent there is little risk of clinical hypoglycaemia. Department of Medicine, University of Birmingham, East Birmingham Hospital, Birmingham B9 5ST, UK
BETH R. ROWE JOHN THORPE ANTHONY BARNETT
JD, Wilner KD. Drug interactions with fluconazole. Rev Infect Dis 1991; 12 (suppl 3): 328-29. 2. Report of an international trial. A comparison of single-dose oral fluconazole with 3-day intravaginal clotrimazole in the treatment of vaginal candidiasis. Br J Obstet Gynaecol 1989; 96: 226-32. 3. Brammer KW. Clinical experience with fluconazole in hospital practice. In: Richardson RG, ed. Fluconazole and its role in vaginal candidiasis. Int Congr Symp Ser Roy Soc Med 1989; 160: 29-31. 1. Lazar
Postpartum idiopathic polymyositis SIR,-Several lines of evidence suggest that autoimmune mechanisms have a role in the pathogenesis of idiopathic polymyositis.l We have lately seen a woman in whom polymyositis developed after delivery. This prompted us to look for a possible link between delivery and polymyositis, since several other putative immune disorders such as myasthenia gravis.1 systemic lupus erythematosus,3 and multiple sclerosis4 have been reported to present or to relapse postpartum. We therefore reviewed the records of our polymyositis patients diagnosed during the past decade. 22 patients fulfilled at least three of the following diagnostic criteria: (1) suggestive clinical picture, consisting of progressive symmetrical limb weakness of subacute onset; (2) increased serum muscle enzymes; (3) electromyographic (EMG) evidence of active muscle disease; (4) skeletal muscle biopsy showing muscle fibre necrosis and regeneration with inflammation; and (5) beneficial response to immunomodulating therapy. In 8 (36%) of the 22 women the disorder developed during childbearing age (age range 20-41 years) and 3 of them (38%) presented with polymyositis within three months of delivery. Patient 1-Leg weakness started to develop in a 21-year-old woman a month after delivery of her first child. The weakness slowly progressed to affect all four limbs and neck flexors. She was admitted seven months later and polymyositis was diagnosed on the basis of raised serum creatine kinase (CK) (4976 IU), EMG pattern with fibrillations, and characteristic muscle biopsy findings. Her symptoms were resistant to immunotherapy but eventually responded to a combination of methylprednisolone and
cyclophosphamide. Patient 2-A 23-year-old woman noticed muscle ache and weakness in her hands and legs within two months of giving birth to her second child. Although serum CK concentrations were normal, EMG results were suggestive of active muscle disease, and a muscle biopsy demonstrated muscle-fibre necrosis and inflammatory reaction. Muscle weakness responded to prednisone. In the next seven years she had several relapses, which were controlled with steroids. During the same period she had two normal pregnancies and deliveries, one of which was associated with mild exacerbation of disease postpartum. Patient 3-This woman gave birth to her first child at the age of 24. Two and a half months later, she had rapidly progressive hand weakness associated with swelling in her forearms, hands, and fingers. On admission she had upper limb weakness (3/5 [MRC scale] distally, and 4/5 in the shoulder girdle and upper arm muscles) and slight neck flexor weakness. Her condition deteriorated rapidly and within four weeks of disease onset she had striking weakness in all limbs. Polymyositis was diagnosed on the basis of a serum CK concentration of 5000 IU, typical EMG findings, and muscle histology. She responded well to prednisone therapy but tapering off trials resulted in clinical and biochemical relapses. At present, a year and a half after diagnosis, she is maintained on a combination of low-dose steroids and methotrexate. These three women had polymyositis that first manifested within a few months of delivery. Although an increased frequency of other putative immune disorders has been reported during the
postpartum period,2-4 we are unaware of other reports suggesting such a pattern in polymyositis. We believe that the association between polymyositis and delivery is not coincidental, although the exact pathogenetic mechanisms causing such conditions after delivery are poorly understood. Hormonal changes associated with pregnancy and delivery, such as altered progesterone and corticosteroid concentrations, have been proposed.5 Synthesis of corticosteroids, which suppress polymyositis, is increased during pregnancy and returns to normal after delivery. Additionally, the resolution of the natural immunosuppressive state that exists during pregnancy because of the action of substances with
immunosuppressive properties-such as alpha-fetoprotein’*—may be contributory. Whatever the mechanism is, this report adds yet another condition to the list of immune disorders presenting or worsening post delivery. Departments of Neurology and Medicine (Mount Scopus), Hadassah University Hospital, Hebrew University-Hadassah Medical School, Jerusalem 91120, Israel
ISRAEL STEINER LEA AVERBUCH-HELLER ODED ABRAMSKY EYAL RAZ
1. Dalakas MC. Polymyositis, dermatomyositis and inclusion body myositis. N Engl J Med 1991; 325: 1487-98. 2. Fraser D, Turner JWA. Myasthenia gravis and pregnancy. Proc R Soc Med 1963; 56: 379-81. 3. Garsenstein M, Pollack V, Kark RM. Systemic lupus erythematosus and pregnancy N Engl J Med 1962; 267: 165-69. 4. Korn-Lubetzki I, Kahana E, Cooper G, Abramsky O. Activity of multiple sclerosis during pregnancy and puerperium. Ann Neurol 1984; 16: 229-31. 5. Arnason BG, Richman DP. Effect of oral contraceptives on experimental demyelinating disease. Arch Neurol 1969; 21: 103-08. 6. Abramsky O, Brenner T, Mizrachi R, Soffer D. Alpha fetoprotein suppresses experimental allergic encephalomyelitis.J Neuroimmunol 1982; 2: 1-7.
Malignant hyperthermia and Hirschsprung’s disease SIR,-Dr Elechi and Dr Dakaraju (Nov 9, p 1206) suggest a link between malignant hyperthermia (MH) and Hirschsprung’s disease (HD). Between 1986 and 1991, 17 cases of biopsy-proven HD were seen at this hospital (12 males, 5 females; aged 24 days to 9 years). These patients had general anaesthesia 38 times, for temporary colostomy, definitive correction, and so on. 11 definitive operations were done (7 Swenson’s procedures). In the hospital the most commonly used agents for general anaesthesia are
succinylcholine and halothane.
Yet MH has never been encountered here since 1983, either in patients with HD or anyone else. We have not found any record of MH in three large series totalling 3325 patients with HD.1-3 The experience of HD as a common cause of large bowel obstruction in children in the Port Harcourt region of Nigeria may be related to genetic factors. It has been reported that a family history is positive in 3-7% of children with HD, and the familial incidence rose to 21 % as the aganghonic segment extended to caecum.4 Another suggestion is that MH has an autosomal dominant mode of inheritance.5 The frequency and co-existence of diseases with familial inheritance can be expected to be high in regions with homogeneous populations.
Departments of Paediatrics, Surgery, and Paediatric Surgery, Selçuck University Medical Faculty, Konya, Turkey
HALÛK YAVUZ AHMET ÖZEL ADNAN KAYNAK ALÂADDIN DILSIZ ÌBRAHIM ERKUL
Swenson O, Sherman JO, Fisher JH. Diagnosis of congenital megacolon: an analysis of 501 patients. J Pediatr Surg 1973; 8: 587-94. 2. Kleinhaus S, Boley SJ, Sheran M, Sieber WK. Hirschsprung’s disease: a survey of the members of the surgical section of the American Academy of Pediatrics. J Pediatr Surg 1979; 14: 588-97. 3. Ikeda K, Goto S. Diagnosis and treatment of Hirschsprung’s disease in Japan. An analysis of 1628 patients. Ann Surg 1984; 199: 400-05. 4. Lipson AH, Harvey J. Three-generation transmission of Hirschsprung’s disease. Clin Genet 1987; 32: 175-78. 5. Sessler DI. Malignant hyperthermia. J Pediatr 1986; 109: 9-14. 1.
CORRECTION Trends in child growth from a single cross-sectionalsurvey.-In the table in Prof C. A. Monteiro and Dr A. M. Torres’ letter Jan 18, p 192) the 1989 height for 22-year-old males should read 169 Ocm.
