incontinentia pigmenti

Summary

Summary: A genodermatosis occurring mostly in females and characterized by skin changes in three phases - vesiculobullous, verrucous papillomatous, and macular melanodermic. Hyperpigmentation is bizarre and irregular. Sixty percent of patients have abnormalities of eyes, teeth, central nervous system, and skin appendages.

Top Publications

  1. Meuwissen M, Mancini G. Neurological findings in incontinentia pigmenti; a review. Eur J Med Genet. 2012;55:323-31 pubmed publisher
    b>Incontinentia Pigmenti is a rare X-linked multisystem disorder with well described and pathognomonic skin manifestations...
  2. Kim B, Shin H, Won C, Lee J, Kim K, Kim M, et al. Incontinentia pigmenti: clinical observation of 40 Korean cases. J Korean Med Sci. 2006;21:474-7 pubmed
    b>Incontinentia pigmenti (IP) is an uncommon genodermatosis that usually occurs in female infants. It is characterized by ectodermal, mesodermal, neurological, ocular, and dental manifestations...
  3. Smahi A, Courtois G, Vabres P, Yamaoka S, Heuertz S, Munnich A, et al. Genomic rearrangement in NEMO impairs NF-kappaB activation and is a cause of incontinentia pigmenti. The International Incontinentia Pigmenti (IP) Consortium. Nature. 2000;405:466-72 pubmed
    Familial incontinentia pigmenti (IP; MIM 308310) is a genodermatosis that segregates as an X-linked dominant disorder and is usually lethal prenatally in males...
  4. Makris C, Godfrey V, Krähn Senftleben G, Takahashi T, Roberts J, Schwarz T, et al. Female mice heterozygous for IKK gamma/NEMO deficiencies develop a dermatopathy similar to the human X-linked disorder incontinentia pigmenti. Mol Cell. 2000;5:969-79 pubmed
    ..These symptoms and inheritance pattern are very similar to those of incontinentia pigmenti (IP), a human genodermatosis, synthenic with the IKK gamma/NEMO locus...
  5. Shah S, Gibbs S, Upton C, Pickworth F, Garioch J. Incontinentia pigmenti associated with cerebral palsy and cerebral leukomalacia: a case report and literature review. Pediatr Dermatol. 2003;20:491-4 pubmed
    b>Incontinentia pigmenti (IP) is a multisystem disorder with characteristic cutaneous signs. After the skin, the central nervous system is the next most affected system...
  6. Hadj Rabia S, Rimella A, Smahi A, Fraitag S, Hamel Teillac D, Bonnefont J, et al. Clinical and histologic features of incontinentia pigmenti in adults with nuclear factor-?B essential modulator gene mutations. J Am Acad Dermatol. 2011;64:508-15 pubmed publisher
    b>Incontinentia pigmenti (IP) is a multisystem disorder, in which cutaneous symptoms can be accompanied by dental, ocular, and central nervous system defects...
  7. Mayer E, Shuttleworth G, Greenhalgh K, Sansom J, Grey R, Kenwrick S. Novel corneal features in two males with incontinentia pigmenti. Br J Ophthalmol. 2003;87:554-6 pubmed
    b>Incontinentia pigmenti (IP) is a rare X linked genetic disorder, which predominantly affects females. The mutations are usually lethal in males...
  8. Nenci A, Huth M, Funteh A, Schmidt Supprian M, Bloch W, Metzger D, et al. Skin lesion development in a mouse model of incontinentia pigmenti is triggered by NEMO deficiency in epidermal keratinocytes and requires TNF signaling. Hum Mol Genet. 2006;15:531-42 pubmed
    ..Mutations disrupting the X-linked NEMO gene cause incontinentia pigmenti (IP), a human genetic disease characterized by male embryonic lethality and by a complex pathology ..
  9. Fusco F, Fimiani G, Tadini G, Michele D, Ursini M. Clinical diagnosis of incontinentia pigmenti in a cohort of male patients. J Am Acad Dermatol. 2007;56:264-7 pubmed
    Eighteen male patients with incontinentia pigmenti (IP) showed the characteristic clinical features and, when examined, histologic skin defects observed in female patients with IP...

More Information

Publications62

  1. Maingay de Groof F, Lequin M, Roofthooft D, Oranje A, de Coo I, Bok L, et al. Extensive cerebral infarction in the newborn due to incontinentia pigmenti. Eur J Paediatr Neurol. 2008;12:284-9 pubmed
    b>Incontinentia pigmenti (IP) is a rare X-linked dominant neuroectodermal multisystem disorder characterized by skin lesions following Blaschko lines...
  2. Hsiao P, Lin S, Chiang S, Wu Y, Chen H, Lin Y. NEMO gene mutations in Chinese patients with incontinentia pigmenti. J Formos Med Assoc. 2010;109:192-200 pubmed publisher
    b>Incontinentia pigmenti is a rare, X-linked, dominant genodermatosis affecting skin, teeth, eyes, and central nervous system...
  3. Bardaro T, Falco G, Sparago A, Mercadante V, Gean Molins E, Tarantino E, et al. Two cases of misinterpretation of molecular results in incontinentia pigmenti, and a PCR-based method to discriminate NEMO/IKKgamma dene deletion. Hum Mutat. 2003;21:8-11 pubmed
    Familial incontinentia pigmenti (IP) is a rare X-linked dominant disorder that affects ectodermal tissues...
  4. Fusco F, Paciolla M, Napolitano F, Pescatore A, D Addario I, Bal E, et al. Genomic architecture at the Incontinentia Pigmenti locus favours de novo pathological alleles through different mechanisms. Hum Mol Genet. 2012;21:1260-71 pubmed publisher
    IKBKG/NEMO gene mutations cause an X-linked, dominant neuroectodermal disorder named Incontinentia Pigmenti (IP)...
  5. Aradhya S, Courtois G, Rajkovic A, Lewis R, Levy M, Israel A, et al. Atypical forms of incontinentia pigmenti in male individuals result from mutations of a cytosine tract in exon 10 of NEMO (IKK-gamma). Am J Hum Genet. 2001;68:765-71 pubmed
    Familial incontinentia pigmenti (IP [MIM 308310]), or Bloch-Sulzberger syndrome, is an X-linked dominant and male-lethal disorder...
  6. Gautheron J, Pescatore A, Fusco F, Esposito E, Yamaoka S, Agou F, et al. Identification of a new NEMO/TRAF6 interface affected in incontinentia pigmenti pathology. Hum Mol Genet. 2010;19:3138-49 pubmed publisher
    ..We also showed that E57K mutation of NEMO found in a mild form of the genetic disease incontinentia pigmenti, resulted in impaired TRAF6 binding and IL-1beta signaling...
  7. Sebban Benin H, Pescatore A, Fusco F, Pascuale V, Gautheron J, Yamaoka S, et al. Identification of TRAF6-dependent NEMO polyubiquitination sites through analysis of a new NEMO mutation causing incontinentia pigmenti. Hum Mol Genet. 2007;16:2805-15 pubmed
    ..the molecular characterization of a new missense mutation of NEMO (A323P) which causes a severe form of incontinentia pigmenti (OMIM#308300), an inherited disease characterized predominantly by skin inflammation...
  8. Zou C, Zhao Z. Clinical and molecular analysis of NF-kappaB essential modulator in Chinese incontinentia pigmenti patients. Int J Dermatol. 2007;46:1017-22 pubmed
    To analyze clinical manifestation and gene of NF-kappaB essential modulator (NEMO) in 12 pediatric incontinentia pigmenti (IP) patients. Twelve pediatric probands with three of their mothers were enrolled in this study...
  9. Gigarel N, Frydman N, Burlet P, Kerbrat V, Steffann J, Frydman R, et al. Single cell co-amplification of polymorphic markers for the indirect preimplantation genetic diagnosis of hemophilia A, X-linked adrenoleukodystrophy, X-linked hydrocephalus and incontinentia pigmenti loci on Xq28. Hum Genet. 2004;114:298-305 pubmed
    ..disease genes such as haemophilia A, X-linked adrenoleukodystrophy, X-linked hydrocephalus and incontinentia pigmenti. We developed five single-cell triplex amplification protocols with microsatellite markers DXS1073, ..
  10. Song M, Chae J, Park E, Ki C. The common NF-?B essential modulator (NEMO) gene rearrangement in Korean patients with incontinentia pigmenti. J Korean Med Sci. 2010;25:1513-7 pubmed publisher
    b>Incontinentia pigmenti (IP) is a rare X-linked dominant disorder characterized by highly variable abnormalities of the skin, eyes and central nervous system...
  11. Pacheco T, Levy M, Collyer J, de Parra N, Parra C, Garay M, et al. Incontinentia pigmenti in male patients. J Am Acad Dermatol. 2006;55:251-5 pubmed
    b>Incontinentia pigmenti (IP) is a rare X-linked dominant genodermatosis that is typified by distinctive cutaneous findings and often by abnormalities of teeth, hair, nails, eyes, musculoskeletal system, and central nervous system...
  12. Zonana J, Elder M, Schneider L, Orlow S, Moss C, Golabi M, et al. A novel X-linked disorder of immune deficiency and hypohidrotic ectodermal dysplasia is allelic to incontinentia pigmenti and due to mutations in IKK-gamma (NEMO). Am J Hum Genet. 2000;67:1555-62 pubmed
    ..Recently, mutations in IKK-gamma (NEMO) have been shown to cause familial incontinentia pigmenti (IP). Unlike HED-ID, IP affects females and, with few exceptions, causes male prenatal lethality...
  13. Fiorillo L, Sinclair D, O Byrne M, Krol A. Bilateral cerebrovascular accidents in incontinentia pigmenti. Pediatr Neurol. 2003;29:66-8 pubmed
    b>Incontinentia Pigmenti is an X-linked dominant neurocutaneous disorder with central nervous system manifestations in 30% of cases, including seizures and mental retardation...
  14. Lee Y, Kim S, Kim K, Chang M. Incontinentia pigmenti in a newborn with NEMO mutation. J Korean Med Sci. 2011;26:308-11 pubmed publisher
    b>Incontinentia pigmenti (IP) (OMIM #308300) is a rare X-linked dominant neuroectodermal multisystemic syndrome due to mutations in the gene for NF-?B essential modulator (NEMO)...
  15. Kenwrick S, Woffendin H, Jakins T, Shuttleworth S, Mayer E, Greenhalgh L, et al. Survival of male patients with incontinentia pigmenti carrying a lethal mutation can be explained by somatic mosaicism or Klinefelter syndrome. Am J Hum Genet. 2001;69:1210-7 pubmed
    b>Incontinentia pigmenti (IP), or "Bloch-Sulzberger syndrome," is an X-linked dominant disorder characterized by abnormalities of skin, teeth, hair, and eyes; skewed X-inactivation; and recurrent miscarriages of male fetuses...
  16. Matsumoto N, Takahashi S, Toriumi N, Sarashina T, Makita Y, Tachibana Y, et al. Acute disseminated encephalomyelitis in an infant with incontinentia pigmenti. Brain Dev. 2009;31:625-8 pubmed publisher
    We report the case of a female Japanese infant who was diagnosed with incontinentia pigmenti (IP) on the basis of the clinical and pathological findings of characteristic skin lesions and the detection of deletion in the nuclear factor-..
  17. Schmidt Supprian M, Bloch W, Courtois G, Addicks K, Israel A, Rajewsky K, et al. NEMO/IKK gamma-deficient mice model incontinentia pigmenti. Mol Cell. 2000;5:981-92 pubmed
    ..and strikingly similar skin lesions in heterozygous females are hallmarks of the human genetic disorder incontinentia pigmenti (IP)...
  18. Bachevalier F, Marchal C, Di Cesare M, Antunes A, Truchetet F. [Lethal neurological involvement during incontinentia pigmenti]. Ann Dermatol Venereol. 2003;130:1139-42 pubmed
    We present a case of incontinentia pigmenti associated with lethal neurological involvement...
  19. Yamashiro T, Nakagawa K, Takada K. Case report: orthodontic treatment of dental problems in incontinentia pigmenti. Angle Orthod. 1998;68:281-4 pubmed
    b>Incontinentia pigmenti is an uncommon genodermatosis that occurs in female infants. The characteristic dental defects are partial anodontia and the presence of some peg-shaped teeth...
  20. Abe S, Okumura A, Hamano S, Tanaka M, Shiihara T, Aizaki K, et al. Early infantile manifestations of incontinentia pigmenti mimicking acute encephalopathy. Brain Dev. 2011;33:28-34 pubmed publisher
    We retrospectively reviewed six patients with incontinentia pigmenti (IP) who had encephalopathic manifestations during early infancy...
  21. Nouri Merchaoui S, Mahdhaoui N, Methlouthi J, Zakhama R, Seboui H. [Neonatal seizures revealing incontinentia pigmenti]. Arch Pediatr. 2011;18:1095-9 pubmed publisher
    b>Incontinentia pigmenti (IP) is a rare affection inherited as X-linked dominant disease. It is usually lethal in male infants. IP can affect ectodermal tissues such as the skin, teeth, eyes, bones, and the central nervous system...
  22. Arenas Sordo M, Vallejo Vega B, Hernández Zamora E, Gálvez Rosas A, Montoya Pérez L. Incontinentia pigmenti (IP2): familiar case report with affected men. Literature review. Med Oral Patol Oral Cir Bucal. 2005;10 Suppl 2:E122-9 pubmed
    b>Incontinentia pigmenti is a genodermatosis described by Garrod and in 1920 by Bloch, Sulzberger, Siemens y Bardach. It is an ectodermic disorder that affects skin, teeth, eyes and may also have neurological problems...
  23. Lee A, Goldberg M, Gillard J, Barker P, Bryan R. Intracranial assessment of incontinentia pigmenti using magnetic resonance imaging, angiography, and spectroscopic imaging. Arch Pediatr Adolesc Med. 1995;149:573-80 pubmed
    To evaluate patients with incontinentia pigmenti for evidence of cerebrovascular disease using magnetic resonance imaging techniques...
  24. Tada H, Yoshida S, Yamaji Y, Fujisawa K, Ishibashi T. NEMO mutational analysis in a Japanese family with incontinentia pigmenti. Eye (Lond). 2007;21:888-90 pubmed
  25. Franco L, Goldstein J, Prose N, Selim M, Tirado C, Coale M, et al. Incontinentia pigmenti in a boy with XXY mosaicism detected by fluorescence in situ hybridization. J Am Acad Dermatol. 2006;55:136-8 pubmed
    We report the case of a male infant with incontinentia pigmenti (MIM 308310) and low-grade XXY mosaicism...
  26. Martínez Pomar N, Munoz Saá I, Heine Suñer D, Martin A, Smahi A, Matamoros N. A new mutation in exon 7 of NEMO gene: late skewed X-chromosome inactivation in an incontinentia pigmenti female patient with immunodeficiency. Hum Genet. 2005;118:458-65 pubmed
    b>Incontinentia pigmenti is an X-linked genodermatosis, lethal in males...
  27. Milam P, Griffin T, Shapiro R. A dentofacial deformity associated with incontinentia pigmenti: report of a case. Oral Surg Oral Med Oral Pathol. 1990;70:420-4 pubmed
    ..This patient manifested classic ectodermal and mesodermal anomalies. We present this case to illustrate a rare etiologic factor in the development of dentofacial deformities that can be treated in the conventional manner. ..
  28. Mansour S, Woffendin H, Mitton S, Jeffery I, Jakins T, Kenwrick S, et al. Incontinentia pigmenti in a surviving male is accompanied by hypohidrotic ectodermal dysplasia and recurrent infection. Am J Med Genet. 2001;99:172-7 pubmed
    Familial Incontinentia pigmenti (IP) is a rare X-linked dominant condition. The affected cases have characteristic skin lesions, hair, eye, teeth and nail abnormalities and may also have neurological problems...
  29. Doruk C, Bicakci A, Babacan H. Orthodontic and orthopedic treatment of a patient with incontinentia pigmenti. Angle Orthod. 2003;73:763-8 pubmed
    b>Incontinentia pigmenti is an uncommon, inherited disorder with predominantly ectodermal manifestations that is associated with skin (100%)), dental (90%), skeletal (40%), central nervous (40%), and ocular (35%) deformities...
  30. Pascual Castroviejo I, Pascual Pascual S, Velazquez Fragua R, Martinez V. [Incontinentia pigmenti: clinical and neuroimaging findings in a series of 12 patients]. Neurologia. 2006;21:239-48 pubmed
    ..clinical, neuroimaging 51 with magnetic resonance imaging (MRI) and evolutive findings in 12 patients with incontinentia pigmenti (IP)...
  31. Wiederholt T, Poblete Gutierrez P, Ott H, Lehmann S, Grussendorf Conen E, Beermann T, et al. [Incontinentia pigmenti in a five-week-old girl]. Hautarzt. 2004;55:999-1001 pubmed
    ..Histological examination confirmed the clinical presumptive diagnosis of incontinentia pigmenti. We discuss the dinical features, diagnosis, and the molecular genetic basis of this rare inherited skin ..
  32. Minić S, Obradović M, Kovacevic I, Trpinac D. Ocular anomalies in incontinentia pigmenti: literature review and meta-analysis. Srp Arh Celok Lek. 2010;138:408-13 pubmed
    b>Incontinentia pigmenti (IP) is an X-linked genodermatosis in which skin changes are combined with dental, eye and central nervous system anomalies...
  33. Okan F, Yapici Z, Bulbul A. Incontinentia pigmenti mimicking a herpes simplex virus infection in the newborn. Childs Nerv Syst. 2008;24:149-51 pubmed
    b>Incontinentia pigmenti is a rare, X-linked dominant multisystem genodermatosis that presents at or soon after birth with characteristic cutaneous signs...
  34. Fusco F, Pescatore A, Bal E, Ghoul A, Paciolla M, Lioi M, et al. Alterations of the IKBKG locus and diseases: an update and a report of 13 novel mutations. Hum Mutat. 2008;29:595-604 pubmed publisher
    ..called nuclear factor-kappaB (NF-kB) essential modulator (NEMO), gene are the most common single cause of incontinentia pigmenti (IP) in females and anhydrotic ectodermal dysplasia with immunodeficiency (EDA-ID) in males...
  35. Obermann M, Weber R. Concomitant diagnosis of sarcoidosis and incontinentia pigmenti in an epileptic patient. Eur J Neurol. 2008;15:e36-7 pubmed publisher
  36. Smahi A, Courtois G, Rabia S, Doffinger R, Bodemer C, Munnich A, et al. The NF-kappaB signalling pathway in human diseases: from incontinentia pigmenti to ectodermal dysplasias and immune-deficiency syndromes. Hum Mol Genet. 2002;11:2371-5 pubmed
    ..b>Incontinentia pigmenti (IP) is the first genetic disorder to be ascribed to NF-kappaB dysfunction...
  37. Hennel S, Ekert P, Volpe J, Inder T. Insights into the pathogenesis of cerebral lesions in incontinentia pigmenti. Pediatr Neurol. 2003;29:148-50 pubmed
    We report the case of a neonate with incontinentia pigmenti and seizures on day 4 of life who underwent magnetic resonance imaging and angiography scanning at 8, 13, and 21 days of age...
  38. Berlin A, Paller A, Chan L. Incontinentia pigmenti: a review and update on the molecular basis of pathophysiology. J Am Acad Dermatol. 2002;47:169-87; quiz 188-90 pubmed
    b>Incontinentia pigmenti is an uncommon X-linked dominant disorder, lethal in the majority of affected males in utero and variably expressed in females...
  39. Montes C, Maize J, Guerry Force M. Incontinentia pigmenti with painful subungual tumors: a two-generation study. J Am Acad Dermatol. 2004;50:S45-52 pubmed
    We report 2 cases of painful subungual dyskeratotic tumors occurring in a mother and daughter with incontinentia pigmenti (IP) as a late manifestation of the disease...
  40. Rola M, Martins T, Melo M, Gomes R, Roseira J, Souto A. [Incontinence of pigment]. An Pediatr (Barc). 2004;60:601-2 pubmed
  41. Fryssira H, Kakourou T, Valari M, Stefanaki K, Amenta S, Kanavakis E. Incontinentia pigmenti revisited. A novel nonsense mutation of the IKBKG gene. Acta Paediatr. 2011;100:128-33 pubmed publisher
    To describe and evaluate the clinical and molecular findings of patients with incontinentia pigmenti (IP) in Greece. We examined 12 female patients, initially aged 2 weeks to 7 months with clinical diagnosis of IP...
  42. Godambe S, McNamara P, Rajguru M, Hellmann J. Unusual neonatal presentation of incontinentia pigmenti with persistent pulmonary hypertension of the newborn: a case report. J Perinatol. 2005;25:289-92 pubmed
    b>Incontinentia pigmenti (Bloch-Sulzberger syndrome) is a multisystem disorder with classical changing skin lesions...
  43. Chang T, Behshad R, Brodell R, Gilliam A. A male infant with anhidrotic ectodermal dysplasia/immunodeficiency accompanied by incontinentia pigmenti and a mutation in the NEMO pathway. J Am Acad Dermatol. 2008;58:316-20 pubmed publisher
    ..The same gene is mutated in incontinentia pigmenti (IP), and mutations that do not completely abolish NF-kappaB activity allow survival of male fetuses...
  44. Demirel N, Aydin M, Zenciroglu A, Okumus N, Tekgunduz K, Ipek M, et al. Incontinentia pigmenti with encephalocele in a neonate: a rare association. J Child Neurol. 2009;24:495-9 pubmed publisher
    b>Incontinentia pigmenti is a rare, X-linked dominant multisystem genodermatosis affecting ectodermal and mesodermal tissues. After the skin, the central nervous system is the second-most affected system...
  45. Huang J, Kondo H, Uchio E. A case of incontinentia pigmenti in Japan and its genetic examination. Jpn J Ophthalmol. 2007;51:142-5 pubmed
    b>Incontinentia pigmenti (IP) is a rare X-linked dominant genodermatosis with approximately one-third of cases with associated ocular complications...
  46. Scheuerle A. Male cases of incontinentia pigmenti: case report and review. Am J Med Genet. 1998;77:201-18 pubmed
    Male patients with Bloch-Sulzberger incontinentia pigmenti (IP type II) are rare and more severely affected than their female counterparts, with a significant occurrence of sex chromosome aneuploidy...
  47. Fusco F, Bardaro T, Fimiani G, Mercadante V, Miano M, Falco G, et al. Molecular analysis of the genetic defect in a large cohort of IP patients and identification of novel NEMO mutations interfering with NF-kappaB activation. Hum Mol Genet. 2004;13:1763-73 pubmed
    b>Incontinentia Pigmenti (IP) is an X-linked genodermatosis that is lethal for males and present in females with abnormal skin pigmentation and high variable clinical signs, including retinal detachment, anodontia, alopecia, nail dystrophy ..
  48. Conte M, Pescatore A, Paciolla M, Esposito E, Miano M, Lioi M, et al. Insight into IKBKG/NEMO locus: report of new mutations and complex genomic rearrangements leading to incontinentia pigmenti disease. Hum Mutat. 2014;35:165-77 pubmed publisher
    b>Incontinentia pigmenti (IP) is an X-linked-dominant Mendelian disorder caused by mutation in the IKBKG/NEMO gene, encoding for NEMO/IKKgamma, a regulatory protein of nuclear factor kappaB (NF-kB) signaling...
  49. Hadj Rabia S, Froidevaux D, Bodak N, Hamel Teillac D, Smahi A, Touil Y, et al. Clinical study of 40 cases of incontinentia pigmenti. Arch Dermatol. 2003;139:1163-70 pubmed
    To analyze the distribution of manifestations in a pediatric cohort and define guidelines for follow-up of incontinentia pigmenti (IP)...
  50. Dupuis Girod S, Corradini N, Hadj Rabia S, Fournet J, Faivre L, Le Deist F, et al. Osteopetrosis, lymphedema, anhidrotic ectodermal dysplasia, and immunodeficiency in a boy and incontinentia pigmenti in his mother. Pediatrics. 2002;109:e97 pubmed
    ..the clinical features of a second boy with this novel syndrome and his mother, who presented with signs of incontinentia pigmenti (IP)...
  51. Aradhya S, Woffendin H, Jakins T, Bardaro T, Esposito T, Smahi A, et al. A recurrent deletion in the ubiquitously expressed NEMO (IKK-gamma) gene accounts for the vast majority of incontinentia pigmenti mutations. Hum Mol Genet. 2001;10:2171-9 pubmed
    b>Incontinentia pigmenti (IP) is an X-linked dominant disorder characterized by abnormal skin pigmentation, retinal detachment, anodontia, alopecia, nail dystrophy and central nervous system defects...
  52. Goldberg M. The skin is not the predominant problem in incontinentia pigmenti. Arch Dermatol. 2004;140:748-50 pubmed
  53. Patrizi A, Neri I, Guareschi E, Cocchi G. Bullous recurrent eruption of incontinentia pigmenti. Pediatr Dermatol. 2004;21:613-4 pubmed