Uncommon histopathological oral findings in an autopsied infant with cystic fibrosis.

Authors

  • Eduardo Rodrigues Fregnani Faculty of Odontology of Piracicaba-UNICAMP
  • Ana Lúcia Carrinho Ayrosa Rangel Department of Oral Pathology, Faculty of Odontology of Piracicaba-UNICAMP
  • Fábio Augusto Ito Department of Oral Pathology, Faculty of Odontology of Piracicaba-UNICAMP
  • Ilana Halpern Department of Pathology, Faculty of Medicine, São Paulo University (USP).
  • Paulo Hilário Nascimento Saldiva Department of Pathology, School of Medicine of São Paulo-USP
  • Pablo Agustin Vargas Department of Oral Pathology, Faculty of Odontology of Piracicaba-UNICAMP

DOI:

https://doi.org/10.20396/bjos.v2i7.8641718

Keywords:

Cystic fibrosis. Histopathology. Salivary glands. Tongue and autopsy

Abstract

Cystic Fibrosis or Mucoviscidosis is a hereditary genetic disease that attacks mainly children and young adults. It is characterized by a general dysfunction of the exocrine glands. We described uncommon histopathological findings in tongue and sublingual gland of a patient with Cystic Fibrosis. The 11-month-old female patient, had a history of repeated bronchopneumonia, chronic diarrhea and malnutrition during internment. On investigation, a high dose of chlorine was detected in the perspiration, and the diagnosis of Mucoviscidosis was made. The patient did not respond to treatment, and died in August 1997. During the autopsy, was noted lungs with bronchiectasis and bronchopneumonia with abscesses, stomach containing a large quantity of thick mucus and hepatomegaly. The organ fragments were fixed in formalin and the histological cuts were stained with H/E, Gomori-Grocott, ZiehlNeelsen and Brown-Hopps. Microscopically, the lung tissue showed chronic bronchitis and abscesses with hemorrhagic areas, and in the pancreas the ducts were dilated and filled with thick mucus, as well as evident cystic areas. On the tongue and sublingual gland we noted dilated ducts full of thick mucus and cystic areas. Furthermore, on the tongue areas with squamous ductal metaplasia and in the sublingual gland periductal fibrosis were found.

Downloads

Download data is not yet available.

Metrics

Metrics Loading ...

Author Biographies

Eduardo Rodrigues Fregnani, Faculty of Odontology of Piracicaba-UNICAMP

Realizou o curso de graduação em Odontologia pela Universidade Estadual de Campinas (2000), Mestrado em Estomatopatologia pela Universidade Estadual de Campinas (2003) e Doutorado em Oncologia pela Fundação Antônio Prudente (2008). É especialista nas áreas de Endodontia e Patologia Oral pelo Conselho Federal de Odontologia.

Ana Lúcia Carrinho Ayrosa Rangel, Department of Oral Pathology, Faculty of Odontology of Piracicaba-UNICAMP

Possui graduação em Odontologia pela Universidade de Taubaté (1998), Mestrado, (2001), Doutorado (2004) e Pós-doutorado (2007) em Estomatopatologia pela Universidade Estadual de Campinas (UNICAMP). 

Fábio Augusto Ito, Department of Oral Pathology, Faculty of Odontology of Piracicaba-UNICAMP

Possui graduação em Odontologia pela Universidade Norte do Parana (2000), mestrado em Estomatopatologia pela Universidade Estadual de Campinas (2003) e doutorado em Estomatopatologia pela Universidade Estadual de Campinas (2006).

Ilana Halpern, Department of Pathology, Faculty of Medicine, São Paulo University (USP).

Médica dermatopatologista e patologista geral. Graduada em Medicina pela Faculdade de Medicina da Universidade de São Paulo (FMUSP) , residência médica em Anatomia Patológica pelo HCFMUSP, Doutora em Patologia pela FMUSP, especialização em Dermatopatologia pelo HCFMUSP e especialista em Anatomia Patológica pela Sociedade Brasileira de Patologia/AMB. Diploma Internacional de Dermatopatologia (International Board Certification in Dermatopathology) do International Committee for Dermatopathology (ICDP).

Paulo Hilário Nascimento Saldiva, Department of Pathology, School of Medicine of São Paulo-USP

bachelor's at Medicina from Universidade de São Paulo (1977) and doctorate at Pathological Anatomy and Clinical Pathology from Universidade de São Paulo (1983). He is currently full professor at Universidade de São Paulo. Has experience in Medicine, focusing on Pathological Anatomy and Clinical Pathology, acting on the following subjects: air pollution, doencas respiratorias, fisiopatologia pulmonar, poluição atmosférica and poluição do ar.

Pablo Agustin Vargas, Department of Oral Pathology, Faculty of Odontology of Piracicaba-UNICAMP

Dr Vargas is a dental graduate of the UNESP-Araraquara-Brazil and obtained a Master of Science and PhD titles from the UNICAMP-Piracicaba-Brazil in Oral Pathology. He was chairman of the post-graduate course of Oral Pathology-UNICAMP for 4 years (2000-2004). He was also president of the II International Congress of Dentistry of the UNICAMP, which was held in Piracicaba, Brazil in 2004. He was Honorary Senior Lecturer of the Department of Oral Pathology of the University of Sheffield-UK (2005-2007). He has been teaching Oral Pathology in the School of Dentistry of Piracicaba-UNICAMP. He was vice-chair of the Department of Oral Diagnosis-UNICAMP and Latin America and Caribbean Councillor of the International Association of Oral Pathologists (IAOP) (2008-2012). He was local president of the XVI International Congress of Oral Pathology and Medicine (IAOP-SOBEP Joint Meeting), which was held in São Pedro, Brazil in 2012. He has authored or co-authored 1 chapter and 164 papers (134 indexed in PUBMED) on Oral Pathology and Medicine. He has been acting as reviewer of numerous international periodicals as Oral Oncology, Oral Diseases, Indian Journal of Cancer, Yonsei Medical Journal and Medicinal Oral Patologia Oral Cirurgia Oral. He belongs to the editorial board of 2 international journals: 1- Oral Oncology; 2- Oral Surg Oral Med Oral Path Oral Radiol. He was granted with FRCPath (The Royal College of Pathologists-UK) in January-2013 and Extraordinary Professor at University of Pretoria-South Africa. He is a diagnostic pathologist of oral lesions in Piracicaba and his main research fields are molecular pathology of salivary gland lesions, cysts and odontogenic tumours, and salivary gland involvement in AIDS patients

References

Kimura HM, Rozov T, Bardieri D, Nakaie CMA, Cardieri JMA. Fibrose cística: análise de 24 casos. Rev Paul Med 1982; 99: 13-7.

Ludwig A, de Souza GM, Léon CHB, Moritz MCR, Barbi N. Análise de casos de fibrose cística em uma família branca e em uma família negra. Arq Cat Med 1983; 12: 15-20.

Lunardi CA, Perez ABA, Mattar G. Fibrose cística em criança negra. Relato de caso. Ped Mod1987; 22: 328-30.

Wood BP. Cystic Fibrosis. Radiol 1997; 204: 1-10.

Farias L, Rosário Filho NA, Kovalhuk L, Miasaki N, Chaves SM, Recco RAC et al. Aspectos clínicos da Fibrose Cística. Experiência no Hospital de Clínicas da UFPR, 1980-1996. Pediatria 1997; 19: 241-8.

Morales MM, Capella MAM, Lopes AG. Structure and function of the cystic fibrosis transmembrane conductance regulator. Braz J Med Biol Res 1999; 32: 1021-8.

David TJ. Cystic fibrosis. Arch of Dis Child 1990; 65: 152-7.

Rosenstein JB, Langbaum TS, Winn K. Unexpected diagnosis of cystic fibrosis at autopsy. South Med J 1984; 77: 1383-5.

Eid SN, Shoemaker LR, Samiec TD. Vitamin A in Cystic Fibrosis: Case Report and Review of Literature. J Ped Gastroenterol Nutr 1990; 10: 265-9.

Duggan C, Clin AA, Agil A, Higgins L, Rifai N. Vitamin A status in acute exacerbations of cystic fibrosis. Am J Clin Nutr 1996; 64:635-9.

Fritz ME, Caplan DB, Leever D, Levitt J. Composition of parotid saliva on different days in patients with cystic fibrosis. Am J Dis Child 1972; 123: 116-7.

Mandel ID, Kutscher A, Denning CR, Thompson RH, Zegarelli EV. Salivary studies in cystic fibrosis. Am J Dis Child 1967; 113: 431-8.

Kolberg H, Danielson A, Glitterstam R, Henriksson R, Marklund S. Studies on the parotid saliva in cystic fibrosis. Acta Paed Scand 1982; 71: 321-2.

Wotman S, Mandel ID, Mercadante J, Denning CR. Parotid and submaxillary calcium in human cystic fibrosis. Arch Oral Biol 1971; 16: 663-5.

Jagels AE, Sweeney EA. Oral healthy of patients with cystic fibrosis and their siblings. J Dent Res 1976; 55: 991-6.

Primosch RE. Tetracycline discoloration, enamel defects, and dental caries in patients with cystic fibrosis. Oral Surg 1980; 50: 301-8.

Tynan JJ, Komiyama K. Cystic fibrosis and oral health. J Can Dent Assoc 1984; 50: 833-5.

Tunkel DE, Naclerio RM, Baroody FM, Rosenstein BJ. Bilateral maxillary sinus mucoceles in an infant with cystic fibrosis. Otolaryngol Head Neck Surg 1994; 111: 116-20.

Escobar Castro H, Llopis Carrasco MV, Alzira de Aguilar V, Camarero Salces C, Suarez Cortina L. Mucocele: una forma de presentación de la fibrosis quística en la enfacia. Revisión de la literatura. An Esp Pediatr 1992; 37: 327-30.

Rosenstein JB , Zeitlin PL. Cystic fibrosis. Lancet 1998; 351: 277-82.

Downloads

Published

2015-11-12

How to Cite

1.
Fregnani ER, Rangel ALCA, Ito FA, Halpern I, Saldiva PHN, Vargas PA. Uncommon histopathological oral findings in an autopsied infant with cystic fibrosis. Braz. J. Oral Sci. [Internet]. 2015 Nov. 12 [cited 2022 Nov. 26];2(7):353-6. Available from: https://periodicos.sbu.unicamp.br/ojs/index.php/bjos/article/view/8641718

Issue

Section

Article

Most read articles by the same author(s)