Skip to main content
SpringerLink
Log in

Sonographie extrapulmonaler Befunde bei Kindern und Jugendlichen mit zystischer Fibrose

Ultrasound in extrapulmonary findings in children and adolescents with cystic fibrosis

  • Originalien
  • Published:
Monatsschrift Kinderheilkunde Aims and scope Submit manuscript

Zusammenfassung

Hintergrund

Die zystische Fibrose ist die zweithäufigste autosomal-rezessive Erbkrankheit der kaukasischen Bevölkerung. Das Ausmaß pulmonaler Veränderungen und die kardiopulmonale Insuffizienz bestimmen den Verlauf der Erkrankung. In den meisten Fällen wird die Diagnose bereits im Neugeborenenalter aufgrund gastrointestinaler Symptome gestellt. Die abdominellen Veränderungen sind ebenso wie die pulmonale Pathologie unterschiedlich stark ausgeprägt. Ziel dieser Arbeit ist die Darstellung des sonomorphologischen Korrelats intraabdomineller Veränderungen bei Patienten mit zystischer Fibrose.

Patienten und Methode

72 Patienten mit zystischer Fibrose werden derzeit gemeinsam von den Kinderärzten und Kinderradiologen unserer Klinik betreut. Die sonographischen Befunde wurden im Rahmen regelmäßiger Verlaufsuntersuchungen am Ultraschallgerät Elegra (Siemens) sowie Sonos 4500 (Agilent Technologies) erhoben.

Ergebnisse

Die zystische Fibrose manifestiert sich neben der Lunge in individuell unterschiedlicher Ausprägung und Häufigkeit v. a. an Pankreas, Leber, Gallenblase und Gallenwegen sowie dem Magen-Darm-Trakt.

Schlussfolgerung

Die Sonographie erwies sich als geeignetes bildgebendes Verfahren zur Diagnostik und Verlaufsbeurteilung sowie Erkennung von Komplikationen intraabdomineller Veränderungen bei Patienten mit zystischer Fibrose.

Abstract

Background

Cystic fibrosis has the second highest incidence of an autosomal recessive hereditary disease in the Caucasian population. Pulmonary alteration in association with heart failure are limiting prognostic factors. Diagnose of the disease is mostly established in neonates due to intestinal symptoms. These symptoms, as well as pulmonary manifestations, exist with varying severity. The aim of this study is the presentation of intestinal findings during ultrasound of patients with cystic fibrosis.

Patients and Methods

Currently, the pediatricians and specialists in pediatric radiology in our clinic care for 72 patients with cystic fibrosis. The findings using ultrasound (Elegra, Siemens and Sonos 4500, Agilent Technologies) were regularly recorded during routine examinations.

Results

Cystic fibrosis causes alterations of varying severity and frequency, mainly affecting the pancreas, liver, bladder and intestinal tract.

Conclusion

Ultrasound is a suitable imaging method for diagnosis and the detection of complications due to intestinal alterations in patients with cystic fibrosis. It is also suitable for following the course of the disease.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Abb. 1
Abb. 2
Abb. 3
Abb. 4
Abb. 5
Abb. 6

Literatur

  1. Aitken ML (1995) Cystic fibrosis. Curr Opin Pulm Med 1: 425–434

    Article  PubMed  CAS  Google Scholar 

  2. Argons GA, Corse WR, Markowitz RI et al. (1996) Gastrointestinal manifestation of cystic fibrosis: radiologic-pathologic correlation. Radiographics 16: 871–893

    Google Scholar 

  3. Berrocal T, Pajares MP, Zubillaga AF (2005) Pancreatic cystosis in children and young adults with cystic fibrosis: sonographic, CT, and MRI findings. AJR Am J Roentgenol 184: 1305–1309

    PubMed  Google Scholar 

  4. Bronstein MN, Sokol RJ, Abman SH et al. (1992) Pancreatic insufficiency, growth, and nutrition in infants identified by newborn screening as having cystic fibrosis. J Pediatr 120: 533–540

    Article  PubMed  CAS  Google Scholar 

  5. Brown JA, Mason AC, Cooperberg PL (1999) Gastrointestinal manifestations of cystic fibosis in adults: pictorial essay. Can Assoc Radiol J 50: 165–169

    PubMed  CAS  Google Scholar 

  6. Chaudry G, Navarro OM, Levine DS (2006) Abdominal manifestations of cystic fibrosis in children. Pediatr Radiol 36: 233–240

    Article  PubMed  Google Scholar 

  7. Constantine S, Au VWK, Slavotinek JP (2004) Abdominal manifestations of cystic fibrosis in adults: a review. Austral Radiol 48: 450–458

    Article  CAS  Google Scholar 

  8. Doull IJ (2001) Recent advances in cystic fibrosis. Arch Dis Child 85: 62–66

    Article  PubMed  CAS  Google Scholar 

  9. Durie PR, Kent G, Phillips MJ et al. (2004) Characteristic multiorgan pathology of cystic fibrosis in a long-living cystic fibrosis transmembrane regulator knockout murine model. Am J Pathol 164: 1481–1493

    PubMed  Google Scholar 

  10. Efrati O, Barak A, Modan-Moses D et al. (2003) Liver cirrhosis and portal hypertension in cystic fibrosis. Eur J Gastroenterol Hepatol 15: 1073–1078

    Article  PubMed  Google Scholar 

  11. Feigelson J, Yvette P, Poquet M (2000) Imaging changes in the pancreas in cystic fibrosis: a retrospective evaluation of 55 cases seen over a period of 9 years. J Pediatr Gastroenterol Nutr 30: 145–151

    Article  PubMed  CAS  Google Scholar 

  12. Iannaccone G, Antonelle M (1980) Calcification of the pancreas in cystic fibrosis. Pediatr Radiol 9: 85–89

    Article  PubMed  CAS  Google Scholar 

  13. Jackson R, Pencharz PB (2003) Transition of care between paediatric and adult gastroenterology. Cystic fibrosis. Best Pract Res Clin Gastroenterol 17: 213–235

    Article  PubMed  Google Scholar 

  14. King LJ, Scurr ED, Murugan N et al. (2000) Hepatobiliary and pancreatic manifestations of cystic fibrosis: MR imaging appearances. Radiographics 20: 767–777

    PubMed  CAS  Google Scholar 

  15. Marcus-Soerkarman D, Offermans J, Van den Ouweland AM et al. (2005) Hyperechogenic fetal bowel: counseling difficulties. Eur J Med Genet 48: 421–425

    Article  Google Scholar 

  16. Moskowitz SM (2005) Cystic fibrosis lung disease: genetic influences, microbial interactions, and radiological assessment. Pediatr Radiol 35: 739–757

    Article  PubMed  Google Scholar 

  17. Oppenheimer EH, Esterley JR (1975) Hepatic changes in young infants with cystic fibrosis: possible relation to focal biliary cirrhosis. J Pediatr 86: 683–689

    Article  PubMed  CAS  Google Scholar 

  18. Pawel BR, de Chadarevian JP, Franco ME (1997) The pathology of fibrosing colonopathy of cystic fibrosis: a study of 12 cases and review of the literature. Hum Pathol 28: 395–399

    Article  PubMed  CAS  Google Scholar 

  19. Rathaus V, Werner M, Freud E et al. (2006) Sonographic findings of the genital tract in boys with cystic fibrosis. Pediatr Radiol 36: 162–166

    Article  PubMed  Google Scholar 

  20. Riordan JR, Rommens JM, Kerem B et al. (1989) Identification of cystic fibrosis gene: cloning and characterization of complementary DANN. Science 245: 1066–1073

    Article  PubMed  CAS  Google Scholar 

  21. Simon-Bouy, Muller F (2003) Hyperechogenic fetal bowel: collaborativ study of 682 cases. J Gynecol Obstet Biol Reprod 32: 459–465

    CAS  Google Scholar 

  22. Smymth RL, van Velzen D, Smyth AR (1995) Fibrosing colonopathy in cystic fibrosis: results of a case-control study. Lancet 346: 1247–1251

    Article  Google Scholar 

  23. Waters BL (1998) Cystic fibrosis with fibrosing colonopathy in the absence of pancreatic enzymes. Pediatr Dev Pathol 1: 74–78

    Article  PubMed  CAS  Google Scholar 

  24. Wilschanski M, Durie PR (1998) Pathology of pancreatic and intestinal disorders in cystic fibrosis. J R Soc Med 91: 40–49

    PubMed  Google Scholar 

  25. Yamamoto M, Molina-Gomes D, Girodon-Boulandet E et al. (2006) Mid-trimester hyperechogenic bowel in a fetus of Japanese origin carrying a new mutation of CFTR gene (L548Q). Prenat Diagn 26: 6–8

    Article  PubMed  Google Scholar 

  26. Ziegler MM (1994) Meconium ileus. Curr Probl Surg 31: 731–777

    Article  PubMed  CAS  Google Scholar 

Download references

Interessenkonflikt

Es besteht kein Interessenkonflikt. Der korrespondierende Autor versichert, dass keine Verbindungen mit einer Firma, deren Produkt in dem Artikel genannt ist, oder einer Firma, die ein Konkurrenzprodukt vertreibt, bestehen. Die Präsentation des Themas ist unabhängig und die Darstellung der Inhalte produktneutral.

Author information

Authors and Affiliations

  1. Kinderradiologie, Institut für Diagnostische und Interventionelle Radiologie, Friedrich-Schiller-Universität Jena, Kochstraße 2, 07745 , Jena, Deutschland

    U. Zimmermann, H.-J. Mentzel, S. Vogt & W.A. Kaiser

  2. Klinik für Kinder- und Jugendmedizin, Friedrich-Schiller-Universität Jena, Jena, Deutschland

    J. Mainz & F. Zintl

Authors
  1. U. Zimmermann
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. H.-J. Mentzel
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. S. Vogt
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. J. Mainz
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. F. Zintl
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. W.A. Kaiser
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to H.-J. Mentzel.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Zimmermann, U., Mentzel, HJ., Vogt, S. et al. Sonographie extrapulmonaler Befunde bei Kindern und Jugendlichen mit zystischer Fibrose. Monatsschr Kinderheilkd 155, 735–740 (2007). https://doi.org/10.1007/s00112-007-1504-y

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00112-007-1504-y

Schlüsselwörter

Keywords

Search

Navigation