Literature Search
Six sets of search strategies were developed and executed in Ovid MEDLINE between May and June 2019 to identify studies that addressed the key questions for each of the topic groups. Search strategies combined both topic-specific controlled subject headings (MeSH terms) and relevant keywords in order to capture the broad topics of interest for each topic. Searches for topics addressed in the previous version of the guideline were limited to the date the last literature search was conducted in 2006, and topics which were not addressed in the previous guideline were expanded to include publications since 1990.
Anemia / Iron Deficiency and Management of Anticoagulation
| 1 | telangiectasia, hereditary hemorrhagic/ | 3148 |
| 2 | hereditary h$emorrhagic telangiectasia$.mp. [mp=title, abstract, original title, name of substance word, subject heading word, floating sub-heading word, keyword heading word, organism supplementary concept word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier, synonyms] | 1847 |
| 3 | (Osler adj2 Weber adj2 Rendu).mp. [mp=title, abstract, original title, name of substance word, subject heading word, floating sub-heading word, keyword heading word, organism supplementary concept word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier, synonyms] | 938 |
| 4 | or/1-3 | 3761 |
| 5 | limit 4 to (english language and humans and yr="1990- 2019") | 1866 |
| 6 | exp Anemia/ | 156539 |
| 7 | exp Vitamin B 12 Deficiency/ | 11021 |
| 8 | exp Hemolysis/ | 28388 |
| 9 | exp Folic Acid Deficiency/ | 4823 |
| 10 | (anem$ or iron deficie$).mp. [mp=title, abstract, original title, name of substance word, subject heading word, floating sub-heading word, keyword heading word, organism supplementary concept word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier, synonyms] | 186564 |
| 11 | 5 and (6 or 7 or 8 or 9 or 10) | 106 |
| 12 | exp Hematologic Agents/ | 584824 |
| 13 | (anticoagula$ or antithromb$).mp. [mp=title, abstract, original title, name of substance word, subject heading word, floating sub-heading word, keyword heading word, organism supplementary concept word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier, synonyms] | 146153 |
| 14 | exp Atrial Appendage/ | 2899 |
| 15 | 5 and (12 or 13 or 14) | 83 |
Liver Vascular Malformations
| 1 | telangiectasia, hereditary hemorrhagic/ | 3148 |
| 2 | hereditary h$emorrhagic telangiectasia$.mp. [mp=title, abstract, original title, name of substance word, subject heading word, floating sub-heading word, keyword heading word, organism supplementary concept word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier, synonyms] | 1847 |
| 3 | (Osler adj2 Weber adj2 Rendu).mp. [mp=title, abstract, original title, name of substance word, subject heading word, floating sub-heading word, keyword heading word, organism supplementary concept word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier, synonyms] | 938 |
| 4 | or/1-3 | 3761 |
| 5 | limit 4 to (english language and humans and yr="2006- 2019") | 1212 |
| 6 | (liver or biliary or hepatic or portal).mp. [mp=title, abstract, original title, name of substance word, subject heading word, floating sub-heading word, keyword heading word, organism supplementary concept word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier, synonyms] | 1205347 |
| 7 | 5 and 6 | 240 |
GI Bleed
| 1 | telangiectasia, hereditary hemorrhagic/ | 3148 |
| 2 | hereditary h$emorrhagic telangiectasia$.mp. [mp=title, abstract, original title, name of substance word, subject heading word, floating sub-heading word, keyword heading word, organism supplementary concept word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier, synonyms] | 1847 |
| 3 | (Osler adj2 Weber adj2 Rendu).mp. [mp=title, abstract, original title, name of substance word, subject heading word, floating sub-heading word, keyword heading word, organism supplementary concept word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier, synonyms] | 938 |
| 4 | or/1-3 | 3761 |
| 5 | limit 4 to (english language and humans and yr="2006- 2019") | 1212 |
| 6 | (gastr$ or GI).mp. [mp=title, abstract, original title, name of substance word, subject heading word, floating sub-heading word, keyword heading word, organism supplementary concept word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier, synonyms] | 756254 |
| 7 | 5 and 6 | 164 |
| 8 | exp colonoscopy/ or exp endoscopy, gastrointestinal/ | 85291 |
| 9 | fecal immunochemical test$.mp. | 636 |
| 10 | (colon cancer screen$ or screen$ for colon cancer or colorectal cancer screen$ or screen$ for colorectal cancer or colonoscopic screen$).mp. [mp=title, abstract, original title, name of substance word, subject heading word, floating sub-heading word, keyword heading word, organism supplementary concept word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier, synonyms] | 7430 |
| 11 | 5 and (8 or 9 or 10) | 31 |
| 12 | 7 or 11 | 168 |
Epistaxis
| 1 | telangiectasia, hereditary hemorrhagic/ | 3148 |
| 2 | hereditary h$emorrhagic telangiectasia$.mp. [mp=title, abstract, original title, name of substance word, subject heading word, floating sub-heading word, keyword heading word, organism supplementary concept word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier, synonyms] | 1847 |
| 3 | (Osler adj2 Weber adj2 Rendu).mp. [mp=title, abstract, original title, name of substance word, subject heading word, floating sub-heading word, keyword heading word, organism supplementary concept word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier, synonyms] | 938 |
| 4 | or/1-3 | 3761 |
| 5 | limit 4 to (english language and humans and yr="2006- 2019") | 1212 |
| 6 | Epistaxis/ | 4646 |
| 7 | (nose$ or nasal).mp. [mp=title, abstract, original title, name of substance word, subject heading word, floating sub-heading word, keyword heading word, organism supplementary concept word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier, synonyms] | 162481 |
| 8 | 5 and (6 or 7) | 293 |
Pregnancy
| 1 | telangiectasia, hereditary hemorrhagic/ | 3134 |
| 2 | hereditary h$emorrhagic telangiectasia$.mp. [mp=title, abstract, original title, name of substance word, subject heading word, floating sub-heading word, keyword heading word, organism supplementary concept word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier, synonyms] | 1831 |
| 3 | (Osler adj2 Weber adj2 Rendu).mp. [mp=title, abstract, original title, name of substance word, subject heading word, floating sub-heading word, keyword heading word, organism supplementary concept word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier, synonyms] | 929 |
| 4 | or/1-3 | 3735 |
| 5 | limit 4 to (english language and humans and yr="1990- 2019") | 1854 |
| 6 | exp Pregnancy/ | 861509 |
| 7 | pregnan$.mp. [mp=title, abstract, original title, name of substance word, subject heading word, floating sub-heading word, keyword heading word, organism supplementary concept word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier, synonyms] | 962143 |
| 8 | 5 and (6 or 7) | 61 |
Pediatrics
| 1 | telangiectasia, hereditary hemorrhagic/ | 3134 |
| 2 | hereditary h$emorrhagic telangiectasia$.mp. | 1831 |
| 3 | (Osler adj2 Weber adj2 Rendu).mp. | 929 |
| 4 | or/1-3 | 3735 |
| 5 | limit 4 to (english language and humans and yr="1990- 2019") | 1854 |
| 6 | exp Pediatrics/ | 55263 |
| 7 | (pediatr$ or paediatr$ or adolescen$ or children).mp. | 2768438 |
| 8 | (6 or 7) and 5 | 298 |
| 9 | limit 5 to "all child (0 to 18 years)" | 381 |
| 10 | 8 or 9 | 399 |
Genetic Testing
Note: A supplemental search was conducted to address key questions regarding genetic testing for both the pediatrics and pregnancy topic groups, in order to capture relevant studies that didn’t specifically include pregnancy or pediatric terms. The search strategy was as follows:
| 1 | telangiectasia, hereditary hemorrhagic/ | 3134 |
| 2 | hereditary h$emorrhagic telangiectasia$.mp. [mp=title, abstract, original title, name of substance word, subject heading word, floating sub-heading word, keyword heading word, organism supplementary concept word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier, synonyms] | 1831 |
| 3 | (Osler adj2 Weber adj2 Rendu).mp. [mp=title, abstract, original title, name of substance word, subject heading word, floating sub-heading word, keyword heading word, organism supplementary concept word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier, synonyms] | 929 |
| 4 | or/1-3 | 3735 |
| 5 | limit 4 to (english language and humans and yr="1990- 2019") | 1854 |
| 6 | exp Genetic Testing/ | 42765 |
| 7 | 5 and 6 | 55 |
| 8 | 7 not (results from full pediatric or pregnancy searches as executed separately) | 31 |
Inclusion Criteria
- Patients diagnosed with or suspected of having hereditary hemorrhagic telangiectasia
- Intervention: As specified for each panel topic, based upon key questions
- Comparison: Any, including placebo, regular treatment, or no intervention
- English language publications
- Human subjects
- ≥ 5 subjects
- Original results published in an indexed journal (i.e. no data from abstracts/posters included)