Clinical Course in a Patient With Neutrophil-Specific Granule Deficiency and Rapid Detection of Neutrophil Granules as a Screening Test

Shigemura, Tomonari; Yamazaki, Takashi; Shiohara, Masaaki; Kobayashi, N.; Naganuma, K; Koike, K; Agematsu, Kazunaga

doi:10.1007/s10875-014-0082-8

Cited by 10 publications

(4 citation statements)

References 14 publications

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“…Additionally, reductions in active CDP levels cause its repression of C/EBP-genes, such as gp91 phox , to be relieved, thereby allowing the C/EBP--induced transcription of both C/EBP-␦ and specific granule proteins (93,166). Congenital absence of specific granules is a rare disorder that is characterized by atypical neutrophil structure and function as well as frequent and severe bacterial infections (167).…”

Section: Granulopoiesismentioning

confidence: 99%

The Ontogeny of a Neutrophil: Mechanisms of Granulopoiesis and Homeostasis

Lawrence

Corriden

Nizet

2018

Microbiol Mol Biol Rev

180

173

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Comprising the majority of leukocytes in humans, neutrophils are the first immune cells to respond to inflammatory or infectious etiologies and are crucial participants in the proper functioning of both innate and adaptive immune responses. From their initial appearance in the liver, thymus, and spleen at around the eighth week of human gestation to their generation in large numbers in the bone marrow at the end of term gestation, the differentiation of the pluripotent hematopoietic stem cell into a mature, segmented neutrophil is a highly controlled process where the transcriptional regulators C/EBP-α and C/EBP-ε play a vital role. Recent advances in neutrophil biology have clarified the life cycle of these cells and revealed striking differences between neonatal and adult neutrophils based on fetal maturation and environmental factors. Here we detail neutrophil ontogeny, granulopoiesis, and neutrophil homeostasis and highlight important differences between neonatal and adult neutrophil populations.

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Section: Granulopoiesismentioning

confidence: 99%

The Ontogeny of a Neutrophil: Mechanisms of Granulopoiesis and Homeostasis

Lawrence

Corriden

Nizet

2018

Microbiol Mol Biol Rev

180

173

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“…Flow cytometric analysis of neutrophil granule proteins may aid in rapidly confirming the diagnosis. Severe deficiencies in defensins, lactoferrin, BPI, human cathelicidin antimicrobial peptide 18 (hCAP18), and neutrophil gelatinase-associated lipocalin (NGAL) confirm SGD (18). Finally, molecular analysis of C/EBPε definitively confirms the diagnosis.…”

Section: Discussionmentioning

confidence: 97%

Brain Abscess as Severe Presentation of Specific Granule Deficiency

et al. 2020

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“…15 Shigemura et al reported the case of a 32-year-old Japanese woman with neutrophil-specific granule deficiency who had a small impetigo lesion on her face and experienced the rapid spread of a facial abscess to a pulmonary abscess via the blood stream. 16 It was also reported that molluscum contagiosum cases were more likely to have a prior or cooccurring diseases, including impetigo, compared to controls. 17…”

Section: Impetigomentioning

confidence: 98%

Bacterial Skin Infections: Epidemiology and Latest Research

Mistik¹,

Uludağ²,

Kartal³

et al. 2015

TJFMPC

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Skin infections are very common throughout the world. The prevalence of skin infections has been reported as follows: pyoderma (prevalence range 0.2-35%, 6.9-35% in Sub-Saharan Africa), tinea capitis (1-19.7%), scabies (0.2-24%, 1.3-17% in Sub-Saharan Africa), viral skin disorders (0.4-9%, mainly molluscum contagiosum), pediculosis capitis (0-57%). Bacterial skin infections are the most common type of skin infections. The most common bacterial skin infections are reported as impetigo, folliculitis, furunculosis and abscesses, cellulitis, scarlet fever, erysipelas, erythrasma, necrotizing fasciitis and some others. In addition to these, ecthyma, carbunculosis, and mycobacterium skin infections are discussed in this review. The latest research gives us the information on the recent change in the bacterial etiology and treatment of bacterial skin infections. The increase in methicillin-resistant S. aureus (MRSA) in impetigo has recently shown us the necessity of being more careful in regard to its treatment. There are also new measures to prevent skin infections, such as the use of maternal zinc supplementation.

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Clinical Course in a Patient With Neutrophil-Specific Granule Deficiency and Rapid Detection of Neutrophil Granules as a Screening Test

Abstract: Severe bacterial infections sometimes occur and spread rapidly in SGD. Detection of neutrophil granules by flow cytometry is useful for a rapid diagnosis and a screening of SGD.

Cited by 10 publications

References 14 publications

The Ontogeny of a Neutrophil: Mechanisms of Granulopoiesis and Homeostasis

The Ontogeny of a Neutrophil: Mechanisms of Granulopoiesis and Homeostasis

Brain Abscess as Severe Presentation of Specific Granule Deficiency

Bacterial Skin Infections: Epidemiology and Latest Research

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