Comparison of neutrophil distribution patterns in Jordans' anomaly among major automated hematology analyzers

Inaba, Tohru; Ishizuka, K.; Suzuki, Akira; Yuasa, S.; Saito, Kensuke; Kodama, Masashi; Hongo, Fumiya; Fujita, Naohisa; Hirano, Ken‐ichi

doi:10.1111/ijlh.12842

Cited by 3 publications

(1 citation statement)

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“…We reported that myocardial scintigraphy with iodine-123-β-methyl iodophenyl-pentadecanoic acid (BMIPP) [31, 32], a radioactive analogue of LCFA, was useful in detecting abnormal LCFA metabolism in patients with TGCV [33, 34]. In addition, we reported the use of automated hematology analyzers to detect Jordans’ anomaly in patients with PNPLA2 mutation [35–37]. Recently, we developed CT-based TG imaging to detect myocardial and coronary TG deposition [34, 38] and selective immunoinactivation assay to measure functional ATGL activities using peripheral leukocytes [39].…”

Section: Development Of Diagnostic Methods For Tgcvmentioning

confidence: 99%

Triglyceride deposit cardiomyovasculopathy: a rare cardiovascular disorder

Hirano

Ikeda

et al. 2019

Orphanet J Rare Dis

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Triglyceride deposit cardiomyovasculopathy (TGCV) is a phenotype primarily reported in patients carrying genetic mutations in PNPLA2 encoding adipose triglyceride lipase (ATGL) which releases long chain fatty acid (LCFA) as a major energy source by the intracellular TG hydrolysis. These patients suffered from intractable heart failure requiring cardiac transplantation. Moreover, we identified TGCV patients without PNPLA2 mutations based on pathological and clinical studies . We provided the diagnostic criteria, in which TGCV with and without PNPLA2 mutations were designated as primary TGCV (P-TGCV) and idiopathic TGCV (I-TGCV), respectively. We hereby report clinical profiles of TGCV patients. Between 2014 and 2018, 7 P-TGCV and 18 I-TGCV Japanese patients have been registered in the International Registry. Patients with I-TGCV, of which etiologies and causes are not known yet, suffered from adult-onset severe heart disease, including heart failure and coronary artery disease, associated with a marked reduction in ATGL activity and myocardial washout rate of LCFA tracer, as similar to those with P-TGCV. The present first registry-based study showed that TGCV is an intractable, at least at the moment, and heterogeneous cardiovascular disorder.

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