Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Kostmann syndrome or infantile genetic agranulocytosis, part one: Celebrating 50 years of clinical and basic research on severe congenital neutropenia

27 Nov 2006

Abstract excerpt

UNLABELLED: Congenital neutropenia in man was first reported 50 years ago by the Swedish paediatrician Rolf Kostmann. He coined the term "infantile genetic agranulocytosis" for this condition, which is now known as Kostmann syndrome. Recent studies have demonstrated a lack of antibacterial peptides and severe periodontitis in these patients despite recombinant growth factor treatment. Moreover, an increased...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Blood disorders and treatments
Share to Neonatal Health and Biochemistry

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Source

Identifiers

PMID: 17129957
DOI: 10.1080/08035250601087607

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Kostmann syndrome or infantile genetic agranulocytosis, part one: Celebrating 50 years of clinical and basic research on severe congenital neutropeniaPMID 17129957Read ↗

Kostmann disease and other forms of severe congenital neutropenia.Semantic proximity Read ↗

Kostmann syndrome or infantile genetic agranulocytosis, part two: Understanding the underlying genetic defects in severe congenital neutropenia.Semantic proximity Read ↗

Eponym. Kostmann disease.Semantic proximity Read ↗

Periodontal disease in patients from the original Kostmann family with severe congenital neutropenia.Semantic proximity Read ↗

Kostmann syndrome and severe congenital neutropeniaSemantic proximity Read ↗

Severe congenital neutropenia.Semantic proximity Read ↗

Kostmann's Disease and HCLS1-Associated Protein X-1 (HAX1).Semantic proximity Read ↗

Neurological findings and genetic alterations in patients with Kostmann syndrome and HAX1 mutations.Semantic proximity Read ↗

A pediatric case of congenital neutropenia with SRP54 gene mutation in which monocytosis and gingival swelling were useful in differentiating from autoimmune neutropenia.Semantic proximity Read ↗

Deficiency of antibacterial peptides in patients with morbus Kostmann: an observation study.Semantic proximity Read ↗

Compound heterozygous HAX1 mutations in a Swedish patient with severe congenital neutropenia and no neurodevelopmental abnormalities.Semantic proximity Read ↗

Assignment of the gene locus for severe congenital neutropenia to chromosome 1q22 in the original Kostmann family from Northern Sweden.Semantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.