Elevated FVIII levels in hereditary hemorrhagic telangiectasia: Implications for clinical management.

Objectives: The objective of this study was twofold: to determine the prevalence of arterial and venous thromboembolic events in the Norwegian Hereditary Hemorrhagic Telangiectasia (HHT) population, and to explore potential factors linked to such events, with particular emphasis on FVIII. Methods: Patients with an HHT diagnosis attending the Otorhinolaryngology Department at Oslo University Hospital-Rikshospitalet were included consecutively between April 2021 and November 2022. We recorded the participants' medical history with an emphasis on thromboembolic events. Measurements of blood constituents, including FVIII, FIX, vWF, hemoglobin, iron, ferritin, and CRP were performed. Results: One hundred and thirty-four patients were included in the study. The total prevalence of thromboembolic events among the participants was 23.1%. FVIII levels were high (>150 IU/dL) in the majority of HHT patients (n = 84) (68.3%) and were significantly associated with thromboembolic events (p < .001), as was age. Of the patients with high FVIII levels, 28 (33%) had experienced a thromboembolic event. Furthermore, FVIII levels were measured consecutively in 51 patients and were found to fluctuate above or below 150 IU/dL in 25% of these cases. Conclusion: Thromboembolic events are highly prevalent in the Norwegian HHT population and are significantly associated with FVIII levels. FVIII levels can fluctuate, and measurements should be repeated in HHT patients to assess the risk of thromboembolic events. Level of Evidence: 4.

[A child with periorbital swelling]. / Et barn med hevelse rundt øyet.

BACKGROUND: A young boy presented with acute ethmoiditis and recurrent periorbital swelling. The periorbital swelling was confirmed to be caused by a rare condition. CASE PRESENTATION: Debut symptoms were acute rhinitis, unilateral periorbital swelling, fatigue, and swelling in the temple region, probably caused by an insect bite. Magnetic resonance imaging (MRI) of the sinuses showed bilateral ethmoiditis and unilateral periorbital cellulitis without subperiostal abscess formation, and antibiotics were prescribed. Because of recurrence of the periorbital swelling, an interdisciplinary team started an investigation for a differential diagnosis. The infestation of Hypoderma tarandi was confirmed by recent history of hiking in reindeer habitat, typical clinical presentation and detection of IgG hypodermin C antibodies. INTERPRETATION: Human myiasis by Hypoderma tarandi is a rare condition in Norway. Typical clinical signs are unaffected general condition, migration of swelling in the forehead, recurrent unilateral periorbital swelling and normal CRP levels. Early-stage diagnosis and treatment will contribute to rapid symptom relief and prevent rare eye and intracranial complications. This case illustrates the importance of investigation for other conditions when the disease course is unexpected. The authors emphasise that knowledge about this disease is important in a variety of medical specialties.