Background <p>Clinical practice guidelines do not recommend tonsillectomy combined with steroid pulse therapy (TSP) as the initial treatment of severe IgA nephropathy (IgAN) in children. Therefore, the long-term prognosis following TSP is unknown in IgAN children with heavy proteinuria.</p> Methods <p>This retrospective study aimed to determine the long-term outcome of children with IgAN and heavy proteinuria (urinary protein-to-creatinine ratio, ≥ 1.0&#xa0;g/g) following TSP as the initial treatment. The primary endpoint was the probability of achieving treatment-free clinical remission (CR: disappearance of both hematuria and proteinuria) without developing kidney complications at the last follow-up.</p> Results <p>After initiating TSP in 43 patients (median age, 10.4&#xa0;years), CR was achieved in 41 patients (95%) at a median of 9.6&#xa0;months. During the observation period (median, 7.5&#xa0;years), four patients experienced proteinuria recurrence that required additional therapy. At the last follow-up (median age, 18.2&#xa0;years), 37 patients (86%) achieved treatment-free CR without developing kidney complications (favorable group), whereas six patients received a renin–angiotensin system inhibitor for persistent proteinuria (unfavorable group). The age and proportion of glomeruli with crescents at IgAN diagnosis were significantly lower in the favorable group than in the unfavorable group. Multivariable analysis showed that a younger age at IgAN diagnosis (&lt; 12.2&#xa0;years) was an independent predictive factor for favorable outcomes. There were no serious adverse events and no significant negative changes in the anthropometric outcomes.</p> Conclusions <p>Initial treatment with TSP may result in treatment-free CR in children with IgAN and heavy proteinuria, particularly in patients diagnosed at a young age.</p>

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Long-term outcomes of initial tonsillectomy and steroid pulse therapy in children with IgA nephropathy and heavy proteinuria: a single-center retrospective study

  • Nozomi Nomura,
  • Shuichiro Fujinaga,
  • Hiroki Miyano,
  • Yoshiyuki Ohtomo,
  • Daishi Hirano,
  • Mayu Nakagawa,
  • Koji Sakuraya,
  • Amane Endo,
  • Hiromichi Shoji

摘要

Background

Clinical practice guidelines do not recommend tonsillectomy combined with steroid pulse therapy (TSP) as the initial treatment of severe IgA nephropathy (IgAN) in children. Therefore, the long-term prognosis following TSP is unknown in IgAN children with heavy proteinuria.

Methods

This retrospective study aimed to determine the long-term outcome of children with IgAN and heavy proteinuria (urinary protein-to-creatinine ratio, ≥ 1.0 g/g) following TSP as the initial treatment. The primary endpoint was the probability of achieving treatment-free clinical remission (CR: disappearance of both hematuria and proteinuria) without developing kidney complications at the last follow-up.

Results

After initiating TSP in 43 patients (median age, 10.4 years), CR was achieved in 41 patients (95%) at a median of 9.6 months. During the observation period (median, 7.5 years), four patients experienced proteinuria recurrence that required additional therapy. At the last follow-up (median age, 18.2 years), 37 patients (86%) achieved treatment-free CR without developing kidney complications (favorable group), whereas six patients received a renin–angiotensin system inhibitor for persistent proteinuria (unfavorable group). The age and proportion of glomeruli with crescents at IgAN diagnosis were significantly lower in the favorable group than in the unfavorable group. Multivariable analysis showed that a younger age at IgAN diagnosis (< 12.2 years) was an independent predictive factor for favorable outcomes. There were no serious adverse events and no significant negative changes in the anthropometric outcomes.

Conclusions

Initial treatment with TSP may result in treatment-free CR in children with IgAN and heavy proteinuria, particularly in patients diagnosed at a young age.