ORPHA:3161
Congenital pulmonary sequestration
Also known as: Congenital bronchopulmonary sequestration
Clinical definition (Orphanet)
pulmonary sequestration is a rare respiratory characterized by a cystic or solid mass of nonfunctioning primitive segmental lung tissue that does not communicate with the tracheobronchial tree and has anomalous systemic blood supply. Intralobar pulmonary sequestration may be asymptomatic or may present with recurrent pulmonary infections, hemoptysis, chest pain, cough and is usually diagnosed in older children and adults. Extralobar pulmonary sequestration present with respiratory distress, cyanosis, difficulty feeding or infection, may be associated with other anomalies and is mostly diagnosed in neonates or infants.
Is anyone studying this?
4,492
4,492 papers have been published on this condition. That is a real research literature — still far smaller than common diseases (breast cancer: over 700,000 papers). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=183) is 38.
4,492 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 38 (publications denominator n=183).
1,657 in the last 10 years · low confidence
Is a treatment being tested?
2
trials for this specific condition
2 interventional trials matched this specific condition name; 1 currently recruiting in our sample.
Data as of 26 July 2026
2 interventional trials — more than 59.2% of diseases in the trials denominator have none at all (151 of 255; this disease is at the 74.7th percentile).
low confidence · 74.7th percentile (trials denominator)
Do we know what causes it?
Not yet — the cause hasn't been pinned down in GenCC.
No strong gene–disease assertion joined for this Orphanet entity.
Who's working on it?
1,061
Distinct author names in 200 sampled papers — named people below.
Who's working on it?
People publishing on this condition (sampled Europe PMC records). Affiliation is the most recent found in that sample.
- 01Li J6 papers · 2026
Department of Thoracic Surgery, The Second Xiangya Hospital, Central South University, Changsha, Hunan, China.
Papers in Europe PMC - 02Liu Y5 papers · 2026
College of Basic Medicine, Army Medical University (Third Military Medical University), Chongqing, China.
Papers in Europe PMC - 03Zhao H5 papers · 2025
Department of Thoracic and Tumor Surgery, Children's Hospital Affiliated to Shandong University, Jinan, Shandong, 250022, China.
Papers in Europe PMC - 04Guo R4 papers · 2025
Department of Thoracic and Tumor Surgery, Children's Hospital Affiliated to Shandong University, Jinan, Shandong, 250022, China.
Papers in Europe PMC - 05He T4 papers · 2026
Department of Pediatric Surgery, West China Hospital of Sichuan University, NO. 37 GUOXUE Lane, Chengdu, 610041, Sichuan Province, China.
Papers in Europe PMC - 06Li H4 papers · 2025
Department of Pulmonary and Critical Care Medicine, Zhangzhou Affiliated Hospital of Fujian Medical University, Zhangzhou 363000, China.
Papers in Europe PMC - 07Luo D4 papers · 2026
Department of Pediatric Surgery, West China Hospital of Sichuan University, NO. 37 GUOXUE Lane, Chengdu, 610041, Sichuan Province, China.
Papers in Europe PMC - 08Xu C4 papers · 2026
Department of Pediatric Surgery, West China Hospital of Sichuan University, NO. 37 GUOXUE Lane, Chengdu, 610041, Sichuan Province, China. huaxixuchang@163.com.
Papers in Europe PMC - 09Yuan M4 papers · 2026
Department of Pediatric Surgery, West China Hospital of Sichuan University, NO. 37 GUOXUE Lane, Chengdu, 610041, Sichuan Province, China.
Papers in Europe PMC - 10Zhai Y4 papers · 2025
Department of Thoracic and Tumor Surgery, Children's Hospital Affiliated to Shandong University, Jinan, Shandong, 250022, China.
Papers in Europe PMC
Recruiting interventional trials
Trials testing a treatment from the matched ClinicalTrials.gov set
2 interventional trials matched after quoted-phrase search and title/condition post-filter.
Where to find support
We do not yet link condition-specific patient organisations. These umbrella groups support undiagnosed and ultra-rare families:
India — NPRD
Last verified 2026-07-26This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.
Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.
How we counted this
Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored below but are not added to the query string.
"Congenital pulmonary sequestration" OR "Congenital bronchopulmonary sequestration" OR "bronchopulmonary sequestration" OR "congenital sequestration of lung" OR "pulmonary sequestration" OR "sequestered lobe (pulmonary sequestration)"
MeSH descriptor terms unioned into the query: Bronchopulmonary Sequestration
ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):
"Congenital pulmonary sequestration" OR "Congenital bronchopulmonary sequestration" OR "bronchopulmonary sequestration" OR "congenital sequestration of lung" OR "congenital sequestration of the lung" OR "pulmonary sequestration" OR "sequestered lobe (pulmonary sequestration)"
Interventional trials matched via: both, phrase (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).
Study-type breakdown: 2 interventional · 0 observational · 0 expanded access. Only interventional studies enter the trial headline.
Cross-references (from Mondo): MESH:D001998 UMLS:C4020703 NCIT:C97124
Query health: ok — strategies attempted: phrase, mesh; with hits: phrase, mesh
Run this search on ClinicalTrials.gov
Confidence reasoning
- Preferred label is multi-word and distinctive
- No synonyms dropped by stoplist
- No label/synonym collisions with other diseases in this corpus
- Publication count (4492) is extremely high with unknown/missing prevalence — treat as possible over-matching, not proven research intensity