Abstract

Proceedings of the 23rd Paediatric Rheumatology European Society Congress: part three

Highlights

  • Transient periosteal hyperostosis with dysproteinemia, named Goldbloom syndrome (GS), is a rare pediatric disease characterized by recurrent crisis of bone pain, fever, increased inflammatory markers and dysproteinemia

  • Both the empirical and the literature reveals that the meeting with the children who are afraid of needle stick can be challenging and difficult to manage

  • When meeting with children who are afraid of needle stick and meeting their families, it is important that the nurse takes into account several factors: the child’s age, developmental stage, previous experience and the situation the child is in

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Summary

Introduction

Transient periosteal hyperostosis with dysproteinemia, named Goldbloom syndrome (GS), is a rare pediatric disease characterized by recurrent crisis of bone pain, fever, increased inflammatory markers and dysproteinemia. Case I: A 12 year-old girl was admitted with malaise, rash on the legs and swelling of left ankle which started one week prior to her presentation On physical examination, she had hepatosplenomegaly, active arthritis of left ankle and nonspecific maculopapular rash on lower extremities. She had hepatosplenomegaly, active arthritis of left ankle and nonspecific maculopapular rash on lower extremities Her laboratory examination revealed elevated liver enzymes (ALT: 737 U/L, AST: 1018 U/L), increased total and direct bilirubin levels (3.34 mg/dl and 3.1 mg/dl, respectively), anemia, leukocytosis and thrombocytosis (hemoglobin 9.3 g/dl, WBC: 14.870/mm, platelet count 495.000/mm). Systemic lupus erythematosus (SLE) is rare disease in children, especially at an early age in boys Severe manifestations, such as macrophage activation syndrom (MAS) and Ro-associated vasculitis make the diagnostics and management of SLE difficult and challenging.

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