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Congenital H-ficolin deficiency in premature infants with severe necrotising enterocolitis
  1. Luregn J Schlapbach1,
  2. Steffen Thiel2,
  3. Ulf Kessler3,
  4. Roland A Ammann1,
  5. Christoph Aebi1,4,
  6. Jens C Jensenius2
  1. 1Pediatric Intensive Care Unit, Department of Pediatrics, Inselspital, University of Bern, Bern, Switzerland
  2. 2Department of Medical Microbiology and Immunology, Bartholin Building, University of Aarhus, Aarhus, Denmark
  3. 3Department of Pediatric Surgery, Inselspital, University of Bern, Bern, Switzerland
  4. 4Institute for Infectious Diseases, University of Bern, Bern, Switzerland
  1. Correspondence to Dr Luregn J Schlapbach, Pediatric Intensive Care Unit, Department of Pediatrics, University of Bern, Inselspital, CH-3010 Bern, Switzerland; luregn.schlapbach{at}

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In a recent issue of Gut, Müller et al reported on excessive colitis in a murine model of mannan-binding lectin (MBL) deficiency.1 They showed that absence of MBL can lead to uncontrolled intestinal inflammation detrimental to the host. MBL is a pattern-recognition molecule activating the complement system by the lectin pathway. H-ficolin is structurally closely related to MBL and can activate the lectin pathway of complement independently of MBL. While low levels of MBL occur in 10% of Caucasians, H-ficolin deficiency is extremely rare: studies involving over 100 000 adults found no case of H-ficolin deficiency, suggesting it exerts crucial functions for the human immune system.2 The first report on the so far only patient diagnosed with H-ficolin deficiency was published only recently,3 describing a patient with repeated infections who presented …

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  • Funding Rossi Research Fund, Department of Pediatrics, University of Bern, Switzerland. Danish Medical Research Council.

  • Competing interests None.

  • Provenance and peer review Not commissioned; externally peer reviewed.