A clinical case report of cystic fibrosis and liver cirrhosis in a child

Authors

  • V. A. Klymenko Kharkiv National Medical University, Ukraine,
  • O. V. Piontkovska Kharkiv Regional Clinical Children’s Hospital No. 1, Ukraine,
  • O. V. Pasichnyk Kharkiv Regional Clinical Children’s Hospital No. 1, Ukraine,
  • N. M. Drobova Kharkiv National Medical University, Ukraine,
  • K. O. Yanovska Kharkiv National Medical University, Ukraine,
  • S. I. Bevz Kharkiv Regional Clinical Children’s Hospital No. 1, Ukraine,
  • N. T. Sindieieva Kharkiv Regional Clinical Children’s Hospital No. 1, Ukraine,

DOI:

https://doi.org/10.14739/2310-1210.2018.5.141713

Keywords:

children, cystic fibrosis liver, cirrhosis

Abstract

Purpose. To analyze the history of a child with cystic fibrosis (CF) and liver cirrhosis.

Results of research. This clinical case report demonstrates the course of CF in a patient with a severe CFTR mutation delF508. The disease had severe course, mostly due to hepato-renal disorders.

From birth the child had a poor appetite, insufficient weight gain. A cough bothered the child from age of three months, and then pneumonia was diagnosed with a protracted course. CF was diagnosed by positive sweat chloride test (85/107 mmol/L) and clinical manifestations at the age of eight months.

CFTR mutation delF508 was found during genetic testing. The patient had chronic airways colonization with S. aureus and P. aeruginosa. At 1 year of age the child was infected by S. aureus, at 6 year of age – P. aeruginosa. The child received pathogenetic treatment including inhaled antibiotic therapy (tobramycin, colistin). The child constantly received the replacement enzyme therapy, hepatoprotective drugs and multivitamins. Antibiotic therapy was prescribed only for bronchopulmonary exacerbation.

At 8 years of age the biliary cirrhosis, portal hypertension and splenomegaly (+ 6.0 cm) were found. Type 1 diabetes mellitus was diagnosed at 9 years of age. The child's condition worsened progressively mostly due to hepato-renal disorders. The child needed permanent hospital care.

The last patient’s hospitalization lasted for 85 bed-days, including 52 days in the Department of Anesthesiology and Intensive Care because of the condition worsening due to multiple organ dysfunction syndrome that caused death at 17 years of age.

Conclusions. This clinical case report demonstrates the course and outcomes of cystic fibrosis with severe CFTR mutation delF508 and cirrhosis.

 

References

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How to Cite

1.
Klymenko VA, Piontkovska OV, Pasichnyk OV, Drobova NM, Yanovska KO, Bevz SI, Sindieieva NT. A clinical case report of cystic fibrosis and liver cirrhosis in a child. Zaporozhye Medical Journal [Internet]. 2018Sep.24 [cited 2024Nov.23];(5). Available from: http://zmj.zsmu.edu.ua/article/view/141713

Issue

No. 5 (2018)

Section

Case Reports

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