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Reply to: Protein S deficiency after varicella
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Editorial correspondence
The Journal of Pediatrics August 1996
We agree with Levin et al. that protein S deficiency after varicella has a spectrum of clinical presentation, but if purpura fulminans is present the disease is clearly severe and medical attention is likely to be sought at an early stage. However, in the absence of purpura fulminans the diagnosis may be delayed and the presence of deep vein thrombosis, which is uncommon in childhood, may be overlooked. It is therefore important to be aware of this uncommon but potentially fatal complication in an illness that is widespread throughout the childhood population.
REFERENCE
D. Pashankar, MD, MRCP Anthony Robinson, MD, FRCP, DCh Department of Paediatrics Duchess of York Children's Hospital Manchester M20 2LR, United Kingdom
To the Editor:
R. C. Tait, MRCP, MRCPath Department of Haematology Thrombosis Reference Centre Withington Hospital Manchester, United Kingdom 9/35/73079 REFERENCES
1. Levin M, Eley BS, Louis J, Cohen H, Young L, Heyderman RS. Postinfectious purpura fulminans caused by an autoantibody directed against protein S. J Pediatr 1995;127:355-63. 2. Nguyen P, Reynaud J, Pouzol P, Munzer M, Richard O, Francois P. Varicella and thrombotic complications with transient protein C and protein S deficiencies in children. Eur J Pediatr 1994;153:646-9. 3. D'Angelo A, Della Valle P, Cfippa L, Pattarini E, Grimaldi LME, Vigano D' AS. Autoimmune protein S deficiency in a boy with severe thromboembolic disease. N Engl J Med 1993;328:1753-7.
Reply
1. D'Angelo SV, Valle PD, Crippa L, Pattarini E, Grimaldi LME. Autoimmune protein S deficiency in a boy with severe thromboembolic disease. N Engl J Med 1993;328:1753-7.
Familial fetal pleural effusions We read with interest the case report by Thibeanlt et al.1 of familial pulmonary lymphatic hypoplasia associated with fetal pleural effusions. Although this is the first reported case of pulmonary lymphatic hypoplasia associated with fetal pleural effusions, we would like to point out that three other cases of familial fetal pleural effusions have also previously been described. 24 In these cases pulmonary lymphatics were apparently normal at autopsy. Thibeanlt et al. 1 made reference to two reported familial occurrences of congenital pulmonary lymphangiectasia. 5, 6 Fetal pleural effusions occurred in one of these families. 6 Most cases of fetal pleural effusion appear to occur sporadically, but both X-linked recessive2, 3 and autosomal recessive4, 6 inheritance have been suggested in reports of familial cases. The outcome for these infants has been variable, with survival occurring in several cases. It is possible that survival may depend on the underlying pulmonary lymphatic abnormality, as well as the degree of pulmonary hypoplasia and gestation at the time of birth. From the information available, it seems likely that fetal pleural effusions may occur in the presence of apparently normal pulmonary lymphatics or as a result of a variety of underlying abnormalities of lymphatic vessels. Until now these have not been well documented in pathology texts. Alternatively, previously described difficulties with postmortem preparation of specimens may have led to underdiagnosis of these abnormalities.6 These points have important implications in the counseling of families with recurrent cases of fetal pleural effusion, as well as improving our knowledge of this poorly understood condition.
Grenville F. Fox, MBChB, MRCP Karel K. O'Brien, MBBCh BAO, FRCPC Department of Neonatology Mount Sinai Hospital Toronto, Ontario M5G 1X5 Canada
To the Editor: The case report by Dr. Pashankar and colleagues adds further information on the range of thromboembolic complications that may be associated with acquired protein S deficiency after varicella infection. At one end of the spectrum are patients who have had pulmonary emboli or thrombotic events affecting other internal organs but in whom no cutaneous manifestations are seen. At the other end of the specmam are cases such as those we reported with purpura fulminans coexisting with thrombosis of major vessels, and the development of thrombosis or emboli within other organs. Although Dr. Pashankar et al. have not presented evidence that their case was mediated by auto antibodies, the fact that our patients with purpum fulminans and the single case reported by D'Angelo et al. 1 were mediated by autoantibodies, suggests that autoantibody production is likely to be the underlying mechanism for the thrombotic disease.
Michael Levin, FRCP, PhD Paediatric Infectious Diseases Unit Department of Paediatries St. Mary's Hospital Medical School London W2 1NY, United Kingdom 9/35/73080
9/35/74005 REFERENCES
1. Thibeault DW, Zalles C, Wickstrom E. Familial pulmonary lymphatic hypoplasia associated with fetal pleural effusions. J Pediatr 1995;127:979-83. 2. Defoort P, Thiery M. Antenatal diagnosis of congenital chylothorax by gray scale sonography. J Clin Ultrasound 1978;6:47-8. 3. Reece EA, Lockwood CJ, Rizzo N, Pilu G, Bovicelli L, Hobbins JC. Intrinsic intrathoracic malformations of the fetus: sonographic detection and clinical presentation. Obstet Gynecol 1987;70:627-32. 4. King PA, Ghosh A, Tang MH, Lain SK. Recurrent congenital chylothorax. Prenat Diagn 1991;11:809-11.
Editorial correspondence
The Journal of Pediatrics August 1996
We agree with Levin et al. that protein S deficiency after varicella has a spectrum of clinical presentation, but if purpura fulminans is present the disease is clearly severe and medical attention is likely to be sought at an early stage. However, in the absence of purpura fulminans the diagnosis may be delayed and the presence of deep vein thrombosis, which is uncommon in childhood, may be overlooked. It is therefore important to be aware of this uncommon but potentially fatal complication in an illness that is widespread throughout the childhood population.
REFERENCE
D. Pashankar, MD, MRCP Anthony Robinson, MD, FRCP, DCh Department of Paediatrics Duchess of York Children's Hospital Manchester M20 2LR, United Kingdom
To the Editor:
R. C. Tait, MRCP, MRCPath Department of Haematology Thrombosis Reference Centre Withington Hospital Manchester, United Kingdom 9/35/73079 REFERENCES
1. Levin M, Eley BS, Louis J, Cohen H, Young L, Heyderman RS. Postinfectious purpura fulminans caused by an autoantibody directed against protein S. J Pediatr 1995;127:355-63. 2. Nguyen P, Reynaud J, Pouzol P, Munzer M, Richard O, Francois P. Varicella and thrombotic complications with transient protein C and protein S deficiencies in children. Eur J Pediatr 1994;153:646-9. 3. D'Angelo A, Della Valle P, Cfippa L, Pattarini E, Grimaldi LME, Vigano D' AS. Autoimmune protein S deficiency in a boy with severe thromboembolic disease. N Engl J Med 1993;328:1753-7.
Reply
1. D'Angelo SV, Valle PD, Crippa L, Pattarini E, Grimaldi LME. Autoimmune protein S deficiency in a boy with severe thromboembolic disease. N Engl J Med 1993;328:1753-7.
Familial fetal pleural effusions We read with interest the case report by Thibeanlt et al.1 of familial pulmonary lymphatic hypoplasia associated with fetal pleural effusions. Although this is the first reported case of pulmonary lymphatic hypoplasia associated with fetal pleural effusions, we would like to point out that three other cases of familial fetal pleural effusions have also previously been described. 24 In these cases pulmonary lymphatics were apparently normal at autopsy. Thibeanlt et al. 1 made reference to two reported familial occurrences of congenital pulmonary lymphangiectasia. 5, 6 Fetal pleural effusions occurred in one of these families. 6 Most cases of fetal pleural effusion appear to occur sporadically, but both X-linked recessive2, 3 and autosomal recessive4, 6 inheritance have been suggested in reports of familial cases. The outcome for these infants has been variable, with survival occurring in several cases. It is possible that survival may depend on the underlying pulmonary lymphatic abnormality, as well as the degree of pulmonary hypoplasia and gestation at the time of birth. From the information available, it seems likely that fetal pleural effusions may occur in the presence of apparently normal pulmonary lymphatics or as a result of a variety of underlying abnormalities of lymphatic vessels. Until now these have not been well documented in pathology texts. Alternatively, previously described difficulties with postmortem preparation of specimens may have led to underdiagnosis of these abnormalities.6 These points have important implications in the counseling of families with recurrent cases of fetal pleural effusion, as well as improving our knowledge of this poorly understood condition.
Grenville F. Fox, MBChB, MRCP Karel K. O'Brien, MBBCh BAO, FRCPC Department of Neonatology Mount Sinai Hospital Toronto, Ontario M5G 1X5 Canada
To the Editor: The case report by Dr. Pashankar and colleagues adds further information on the range of thromboembolic complications that may be associated with acquired protein S deficiency after varicella infection. At one end of the spectrum are patients who have had pulmonary emboli or thrombotic events affecting other internal organs but in whom no cutaneous manifestations are seen. At the other end of the specmam are cases such as those we reported with purpura fulminans coexisting with thrombosis of major vessels, and the development of thrombosis or emboli within other organs. Although Dr. Pashankar et al. have not presented evidence that their case was mediated by auto antibodies, the fact that our patients with purpum fulminans and the single case reported by D'Angelo et al. 1 were mediated by autoantibodies, suggests that autoantibody production is likely to be the underlying mechanism for the thrombotic disease.
Michael Levin, FRCP, PhD Paediatric Infectious Diseases Unit Department of Paediatries St. Mary's Hospital Medical School London W2 1NY, United Kingdom 9/35/73080
9/35/74005 REFERENCES
1. Thibeault DW, Zalles C, Wickstrom E. Familial pulmonary lymphatic hypoplasia associated with fetal pleural effusions. J Pediatr 1995;127:979-83. 2. Defoort P, Thiery M. Antenatal diagnosis of congenital chylothorax by gray scale sonography. J Clin Ultrasound 1978;6:47-8. 3. Reece EA, Lockwood CJ, Rizzo N, Pilu G, Bovicelli L, Hobbins JC. Intrinsic intrathoracic malformations of the fetus: sonographic detection and clinical presentation. Obstet Gynecol 1987;70:627-32. 4. King PA, Ghosh A, Tang MH, Lain SK. Recurrent congenital chylothorax. Prenat Diagn 1991;11:809-11.