7-day weighed food diaries suggest patients with hereditary hemorrhagic telangiectasia may spontaneously modify their diet to avoid nosebleed precipitants.

Hereditary hemorrhagic telangiectasia (HHT) poses substantial burdens due to nosebleeds and iron deficiency resulting from recurrent hemorrhagic iron losses. Recent studies by our group found surprising links between HHT nosebleeds and certain food groups. In this letter, we report 7-day weighed food diary assessments of an unselected group of 25 UK patients with HHT whose nosebleeds ranged from mild to severe (median epistaxis severity score 4.66, range 0.89- 9.11). The diaries provide evidence that food items most commonly reported to provoke nosebleeds were ingested by fewer HHT patients, compared to food items less commonly reported to provoke nosebleeds (chi-squared p <0.001).

Dietary supplement use and nosebleeds in hereditary haemorrhagic telangiectasia - an observational study.

Understanding potential provocations of haemorrhage is important in a range of clinical settings, and particularly for people with abnormal vasculature. Patients with hereditary haemorrhagic telangiectasia (HHT) can report haemorrhage from nasal telangiectasia in real time, and suggested dietary factors may precipitate nosebleeds. To examine further, nosebleed severity, dietary supplement use, and blood indices were evaluated in an unselected group of 50 HHT patients recruited from a specialist UK service. Using the validated Epistaxis Severity Score, nosebleed severity ranged from 0 to 9.1 out of 10 (median 3.9). Using a Food Frequency Questionnaire, 24/50 (48%) participants reported use of dietary supplements in the previous year. A third (18/50; 36%) had used self prescribed, non-iron containing dietary supplements, ingesting between 1 and 3 different supplements each day. Eight (16%) used fish oils. Despite having more severe epistaxis (p = 0.012), the 12 iron supplement users had higher serum iron concentrations, and were able to maintain their red blood cell indices. In contrast, there was no evident benefit for the participants using non iron supplements. Furthermore, platelet counts and serum fibrinogen tended to be lower in fish oil/supplement users, and one fish oil user demonstrated reduced in vitro platelet aggregation. In conclusion, in this small study, a third of HHT patients used non-iron dietary supplements, and one in six ingested fish oils, unaware of their known anti-platelet activity. The scale of use, and potential of these "natural health supplements" to exacerbate nosebleeds has not been appreciated previously in HHT.

Clinical Experience of Use of High-dose Intravenous Methylprednisolone in Children With Acute Moderate to Severe Colitis.

OBJECTIVES: Treatment of acute severe colitis (ASC) has been associated with high morbidity and high colectomy rate in children. In the prebiologics era, our centre used short-term high-dose intravenous corticosteroids (IVCS) at 2 to 30 mg ·â€Škg ·â€Šday. We conducted a retrospective review to compare efficacy of different dosing regimes of IVCS. METHODS: Thirty-four children treated with IVCS for ASC were included over 8 years. Patients were studied as 2 groups with similar pretreatment patient characteristics. Group 1 (standard dose) received IVCS at 2 mg ·â€Škg ·â€Šday and group 2 (high dose) received IVCS at 10 to 30 mg ·â€Škg ·â€Šday. Safety, efficacy, and follow-up of the entire cohort for >1 year were studied. The median IVCS dose in the standard- and high-dose cohort was 1.5 mg ·â€Škg ·â€Šday (maximum 60 mg ·â€Škg ·â€Šday) and 24.8 mg ·â€Škg ·â€Šday (maximum 1000 mg ·â€Škg ·â€Šday), respectively. RESULTS: Pediatric Ulcerative Colitis Activity Index scores at day 5 were significantly lower in high-dose (15, interquartile range 8.5-20) than in standard-dose IVCS (30, interquartile range 20-30). IVCS side effects were minor and reversible. Overall, medical salvage therapy was required in 5.8% (2 children) before discharge, and in 17% (6 children) at follow-up after 1 year. The colectomy rate of the entire cohort was remarkably low with 0% during admission and 11% (4 children) after 1 year, with a trend of less colectomies in high-dose (4.8%-1 child) than in standard-dose (23%-3 children). CONCLUSIONS: Our data show that in paediatric ASC, the short-term use of high-dose IVCS is safe and effective. Prospective studies are needed to define the role of IVCS within salvage therapy protocols.

Corticosteroid Dosing in Pediatric Acute Severe Ulcerative Colitis: A Propensity Score Analysis.

OBJECTIVES: We aimed to explore the optimal dosing of intravenous-corticosteroids (IVCS) using a robust statistical method on the largest pediatric cohort of acute severe colitis to date. METHODS: Two hundred eighty-three children treated with IVCS for ulcerative colitis were included and studied for 1 year (46% boys, age 12.1 ±â€Š3.9 years, disease duration 2 (interquartile range [IQR] 0-14) months, baseline Pediatric Ulcerative Colitis Activity Index 69 ±â€Š13 points). Confounding by indication was addressed by matching high- and low-IVCS dose patients according to the propensity score method, using 3 cutoffs (1 mg ·â€Škg ·â€Šmethylprednisolone to 40 mg ·â€Šday, 1.25 mg ·â€Škg to 50 mg ·â€Šday and 2 mg ·â€Škg to 80 mg ·â€Šday). RESULTS: The median IVCS dose in the entire cohort was 1.0 mg ·â€Škg ·â€Šday (IQR 0.8-1.4) and 44 mg ·â€Škg ·â€Šday (32-60). Ninety-four of 283 children were matched in the low-dose cutoff (1 mg ·â€Škg ·â€Šday), 218 of 283 were matched in the middle cutoff (1.25 mg ·â€Škg ·â€Šday), and 86/283 in the high dose cutoff (2 mg ·â€Škg ·â€Šday). No differences were found in 25 pretreatment baseline variables in the three cutoffs, implying successful matching. There were no statistical differences in the outcomes of the two lower cutoffs (including need for salvage therapy during admission and by 1 years, admission duration, and day-5 Pediatric Ulcerative Colitis Activity Index<35 points; all P > 0.05). In the high cutoff, the higher doses were somewhat better but this benefit reversed in a sensitivity analysis excluding one center. High doses were not associated with better outcome also in a propensity score-weighted regression model on the entire cohort. CONCLUSIONS: Our data support present guidelines that doses of IVCS >1 to 1.5 mg ·â€Škg ·â€Šday (maximum 40-60 mg ·â€Škg ·â€Šday) are not justified in acute severe colitis.

Hemorrhage-adjusted iron requirements, hematinics and hepcidin define hereditary hemorrhagic telangiectasia as a model of hemorrhagic iron deficiency.

BACKGROUND: Iron deficiency anemia remains a major global health problem. Higher iron demands provide the potential for a targeted preventative approach before anemia develops. The primary study objective was to develop and validate a metric that stratifies recommended dietary iron intake to compensate for patient-specific non-menstrual hemorrhagic losses. The secondary objective was to examine whether iron deficiency can be attributed to under-replacement of epistaxis (nosebleed) hemorrhagic iron losses in hereditary hemorrhagic telangiectasia (HHT). METHODOLOGY/PRINCIPAL FINDINGS: The hemorrhage adjusted iron requirement (HAIR) sums the recommended dietary allowance, and iron required to replace additional quantified hemorrhagic losses, based on the pre-menopausal increment to compensate for menstrual losses (formula provided). In a study population of 50 HHT patients completing concurrent dietary and nosebleed questionnaires, 43/50 (86%) met their recommended dietary allowance, but only 10/50 (20%) met their HAIR. Higher HAIR was a powerful predictor of lower hemoglobin (p = 0.009), lower mean corpuscular hemoglobin content (p<0.001), lower log-transformed serum iron (p = 0.009), and higher log-transformed red cell distribution width (p<0.001). There was no evidence of generalised abnormalities in iron handling Ferritin and ferritin(2) explained 60% of the hepcidin variance (p<0.001), and the mean hepcidinferritin ratio was similar to reported controls. Iron supplement use increased the proportion of individuals meeting their HAIR, and blunted associations between HAIR and hematinic indices. Once adjusted for supplement use however, reciprocal relationships between HAIR and hemoglobin/serum iron persisted. Of 568 individuals using iron tablets, most reported problems completing the course. For patients with hereditary hemorrhagic telangiectasia, persistent anemia was reported three-times more frequently if iron tablets caused diarrhea or needed to be stopped. CONCLUSIONS/SIGNIFICANCE: HAIR values, providing an indication of individuals' iron requirements, may be a useful tool in prevention, assessment and management of iron deficiency. Iron deficiency in HHT can be explained by under-replacement of nosebleed hemorrhagic iron losses.