ISSUE INFORMATION

Open Access

Issue Information

  • Pages: i-iv
  • First Published: 24 December 2022

ORIGINAL ARTICLE

Open Access

Phenomapping approach to interpreting coronary dimensions in febrile children

  • Pages: 233-240
  • First Published: 15 December 2022
Phenomapping approach to interpreting coronary dimensions in febrile children

Unsupervised hierarchical clustering analysis of phenotypic data including age, hemoglobin, white cell count, platelet count, C-reactive protein, erythrocyte sedimentation rate, albumin, alanine aminotransferase, aspartate aminotransferase, and coronary artery z scores was performed in 239 febrile children with (n = 180) and with (n = 59) Kawasaki disease (KD). Three phenogroups that differed in a clinical, laboratory, and echocardiographic parameters were identified. The phenomapping approach identified useful laboratory parameters that aid the diagnosis of KD in febrile children with relatively normal-sized coronary arteries.

Open Access

Allogeneic hematopoietic stem cell transplantation with the modified myeloablative conditioning regimen for children with chronic active Epstein–Barr virus infection

  • Pages: 250-259
  • First Published: 15 November 2022
Allogeneic hematopoietic stem cell transplantation with the modified myeloablative conditioning regimen for children with chronic active Epstein–Barr virus infection

Allogeneic hematopoietic stem cell transplantation (allo-HSCT) with the modified myeloablative conditioning (MAC) regimen is safe and effective for pediatric chronic active Epstein–Barr virus. The 3-year overall survival (OS) and 3-year event-free survival (EFS) rates were 88.8% ± 5.4% and 85.0% ± 5.7%, respectively. The 3-year OS and EFS did not significantly differ between the hemophagocytic lymphohistiocytosis (HLH) and non-HLH groups, or among the complete remission, partial remission, and activity disease groups. Patients with concomitant HLH and patients with the active disease before allo-HSCT also benefit from modified MAC-HSCT. Patients with grade III–IV acute graft-versus-host diseases (aGVHD) had lower 3-year OS and EFS rates than those without aGVHD or with grade I–II aGVHD.

RESEARCH LETTER

Open Access

Outcomes of complete surgical repair versus palliative intervention in neonates with Tetralogy of Fallot

  • Pages: 260-263
  • First Published: 23 October 2022
Outcomes of complete surgical repair versus palliative intervention in neonates with Tetralogy of Fallot

Using the US National Inpatient Sample dataset (2010 to 2018), we compared outcomes of neonates with Tetralogy of Fallot who had early primary surgical repair (1726 neonate) and those who had staged palliative intervention with transcatheter (1702 neonate) or surgical palliative shunt (2661 neonate).

REVIEW

Open Access

Newborn screening for genetic disorders: Current status and prospects for the future

  • Pages: 291-298
  • First Published: 24 October 2022
Newborn screening for genetic disorders: Current status and prospects for the future

The history of NBS began with PKU screening in the 1960s, but it expanded slowly. A revolutionary turning point came in the 1990s with the introduction of MS/MS. Enzymatic assay provides a reliable screening method for some disorders. Recently, NGS has emerged as a robust tool, raising concerns regarding its application in population screening.

LETTER TO THE EDITOR