Hipoplasia pulmonar en el adulto: embriología, presentación clínica y métodos diagnósticos. Experiencia propia y revisión de la literatura

Comet, R.; Marín, A.; Sans, J.; Domingo, C.

doi:10.1016/S0300-2896(15)30505-6

Archivos de Bronconeumología

ISSN: 0300-2896

Archivos de Bronconeumologia is an international journal that publishes original studies whose content is based upon results of research initiatives dealing with several aspects of respiratory medicine including epidemiology, respiratory physiology, pathophysiology of respiratory diseases, clinical management, thoracic surgery, pediatric lung diseases, respiratory critical care, respiratory allergy and translational research. Other types of articles such as editorials, reviews, and different types of letters are also published in the journal. Additionally, the journal expresses the voice of the following scientific societies: the Spanish Respiratory Society of Pneumology and Thoracic Surgery (SEPAR; https://www.separ.es/), the Latin American Thoracic Society (ALAT; https://alatorax.org/), and the Iberian American Association of Thoracic Surgery (AIACT; http://www.aiatorax.com/).

It is a monthly journal in which all manuscripts are sent to peer-review and handled by the editor or an associate editor from the team and the final decision is made on the basis of the comments from the expert reviewers and the editors. The journal is published solely in English. All the published data is composed of novel manuscripts not previously published in any other journal and not being in consideration for publication in any other journal..

The journal is indexed at Science Citation Index Expanded, Medline/Pubmed, Embase and SCOPUS. Access to any published article is possible through the journal's web page as well as from Pubmed, ScienceDirect, and other international databases. Furthermore, the journal is also present in X, Facebook and Linkedin. Manuscripts can be submitted electronically using the following web site: https://www.editorialmanager.com/ARBR/.

Indexed in:

Current Contents/Clinical Medicine, JCR SCI-Expanded, Index Medicus/Medline, Excerpta Medica/EMBASE, IBECS, IME, SCOPUS, IBECS

Subscribe:

Impact factor

The Impact Factor measures the average number of citations received in a particular year by papers published in the journal during the two preceding years.

© Clarivate Analytics, Journal Citation Reports 2022

Impact factor 2023

8.7

Citescore

CiteScore measures average citations received per document published.

Citescore 2023

3.5

SJR

SRJ is a prestige metric based on the idea that not all citations are the same. SJR uses a similar algorithm as the Google page rank; it provides a quantitative and qualitative measure of the journal's impact.

SJR 2023

0.464

SNIP

SNIP measures contextual citation impact by wighting citations based on the total number of citations in a subject field.

SNIP 2023

0.482

View more metrics

Open Access Option

Journal Information

Previous article | Next article

Vol. 34. Issue 1.

Pages 48-51 (January 1998)

More article options

Pages 48-51 (January 1998)

DOI: 10.1016/S0300-2896(15)30505-6

Full text access

Hipoplasia pulmonar en el adulto: embriología, presentación clínica y métodos diagnósticos. Experiencia propia y revisión de la literatura

Pulmonary hypoplasia in adults: embryology, clinical presentation and diagnostic methods. Our experience and review of the literature

Visits

5811

Download PDF

R. Comet, A. Marín, J. Sans, C. Domingo*

Servicio de Neumología. Consorci Hospitalari del Pare Taulí. Sabadell

R.M. Mirapeixa, E. Castañerb

a Departament de Ciéncies Morfológiques. UAB. Bellaterra. Barcelona

b Servicio de Radiología. Consorci Hospitalari del Pare Taulí. Sabadell

This item has received

5811 Visits

Article information

Abstract

Bibliography

Download PDF

Statistics

Presentamos 5 pacientes diagnosticados de hipoplasia pulmonar (HP) en la edad adulta (edad > 18 años); el rango de edad en el momento del diagnóstico fue de 45-67 años. La HP fue izquierda en 4 casos y las malformaciones asociadas fueron: 2 agenesias renales, 2 hernias diafragmáticas y una hernia hiatal. El funcionalismo respiratorio mostró: FEV1 0,88±0,32 1; FVC 1,30±0,52 1; FEV1/FVC 68,5±5%. La gasometría arterial (con Fi02 0,21) fue de: Pa02 63,6±24; PaC02 47,1±11,2mmHg. Tres casos fueron remitidos para control de su insuficiencia respiratoria (dos precisaron oxigenoterapia domiciliaria) y 2 casos para estudio de radiografía de tórax patológica. El diagnóstico se realizó mediante tomografía computarizada (TC) torácica. Las enfermedades congénitas debidas a la detención del desarrollo pulmonar en la fase seudoglandular son raramente diagnosticadas en el adulto (hemos recogido 48 casos -incluyendo los actualespublicados en la literatura), siendo la HP muy infrecuentemente diagnosticada (posiblemente por atribuirse los cambios observados en la radiografía a procesos infecciosos antiguos). La clínica es muy variable, y depende en gran medida del hábito tabáquico y de las infecciones respiratorias de repetición. La TC torácica es hoy día la técnica diagnóstica de elección.

Palabras clave:

Hipoplasia pulmonar en adultos

Malformaciones asociadas

Tomografía computarizada torácica

We report five patients diagnosed of pulmonary hypoplasia (PH) in adulthood (age > 18 years, range 45 to 67 years). PH was left-sided in four cases. The malformations found were 2 cases of renal agenesis, 2 diaphragmatic hernias and 1 hiatal hernia. Lung function data were FEV1 0.88±0.32 L; FVC 1.30±0.52 L; FEV1/FVC 68.5±5%. Arterial gases (with Fi02 0.21) were PaO2 63.6±24 and PaC02 47.1±11.2mmHg. Three cases were referred for monitoring of respiratory insufficiency (2 requiring home oxygen therapy) and two for radiographic study of chest disease. The diagnoses were made by computerized tomography (CT) of the chest. Congenital diseases due to underdeveloped lungs in the pseudoglandular phase are rarely diagnosed in adults (48 cases, including ours, have been found in the literature), and PH is rarely diagnosed, possibly because the anomalies observed are attributed to old infections. Clinical presentation is highly variable, depending in large measure on a history of smoking and repeated respiratory infections. Chest CT is at present the diagnostic tool of choice.

Key words:

Adult pulmonary hypoplasia

Associated malformations

Computed tomography, chest

Full text is only aviable in PDF

Bibliografía

[1.]

P. Schneider.

Die Morphologie der Missbildungen des menschen und der tierer.

G Fisher Jena, 3 (1912), pp. 812-822

[2.]

E. Boyden.

A developmental anomalies of lungs.

Am J Surg, 89 (1955), pp. 79-89

Medline

[3.]

D.M. Sherer, J.M. Davis, J.R. Woods.

Pulmonary hypoplasia: a review.

Obst Gynecol Survey, 45 (1990), pp. 792-803

[4.]

Y. Nakamura, K. Harada, I. Yamamoto, Y. Uemura, K. Okamoto, S. Fukuda, et al.

Human pulmonary hypoplasia. Statistical, morphological, morphometric and biochemical study.

Arch Pathol Lab Med, 116 (1992), pp. 635-642

Medline

[5.]

W.J. Larsen.

Human embryology.

Churchill Livingstone, (1993), pp. 111-125

[6.]

Y. Nakamura, I. Yamamoto, S. Fukuda, T. Hashimoto.

Pulmonary acinar development in diaphragmatic hernia.

Arch Pathol Lab Med, 115 (1991), pp. 372-376

Medline

[7.]

J. Osborne, J. Masel, J. McCredie.

A spectrum of skeletal anomalies associated with pulmonary agenesis: possible neural crest injuries.

Pediatr Radiol, 19 (1989), pp. 425-432

Medline

[8.]

A. Vélez, F. Omeñaga, A. Santana, A. Ruiz, C. Ramos, C. García-Vao, et al.

Síndrome de la tríada en el período neonatal. Estudio anatomoclínico de 11 casos.

Ann Esp Pediatr, 25 (1986), pp. 345-351

[9.]

C. Della Pona, G. Rocco, A. Rizzi, M. Robustellini, G. Rossi, B. Crasti.

Lobar hypoplasia.

Eur Respir J, 4 (1993), pp. 1.140-1.142

Medline

[10.]

R.M. Mirapeix, C.H. Domingo, J.R. Sañudo, J.M. Mata.

Unusual association of two unilateral anomalies present in adulthood: pulmonary hypoplasia and renal agenesis.

Surg Radiol Anat, 17 (1995), pp. 177-179

Medline

[11.]

A.N. Husain.

Neonatal pulmonary hypoplasia: an autopsy study of 25 cases.

Pediatr Pathol, 13 (1993), pp. 475-484

Medline

[12.]

Y. Hosoda, J.E. Rossman, P.L. Glick.

Pathophysiology of congenital diaphragmatic hernia IV: renal hyperplasia is associated with pulmonary hypoplasia.

J Pediatr Surg, 28 (1993), pp. 464-470

Medline

[13.]

Y. Nakamura.

Pulmonary disorders in infants.

Acta Pathol Japon, 43 (1993), pp. 425-432

[14.]

J. Zlotogora, M. Sagi, Y.O. Shabany, R.Y. Jarallah.

Syndrome of tetraamelia with pulmonary hypoplasia.

Am J Med Genet, 47 (1993), pp. 570-571

http://dx.doi.org/10.1002/ajmg.1320470427 | Medline

[15.]

A.R. Valle.

Agenesis of the lung.

Am J Surg, 89 (1955), pp. 90-100

Medline

[16.]

D.L. Maltz, A.S. Nadas.

Agenesis of the lung. Presentation of eight new cases and review of the literature.

Pediatrics, 42 (1968), pp. 175-188

Medline

[17.]

A. Mas, R.M. Mirapeix, C.H. Domingo, J.R. Sañudo, M.D. Torremorell, A. Marin.

Pulmonary hypoplasia presented in adulthood as a chronic respiratory failure: report of two cases.

Respiration, 64 (1997), pp. 240-243

Medline

[18.]

R.A. Smith, A.O. Bech.

Agenesis of lung.

Thorax, 13 (1958), pp. 28-33

Medline

[19.]

R.L. Soulen, R.V. Cohen.

Plain film recognition of pulmonary agenesis in the adult.

Chest, 60 (1971), pp. 185-187

Medline

[20.]

S.S. Shenoy, G.J. Culver, H.S. Pirson.

Agenesis of lung in an adult.

AJR, 133 (1979), pp. 755-757

http://dx.doi.org/10.2214/ajr.133.4.755 | Medline

[21.]

F. Bernal Estévez, J.L. Bravo Bravo, R. Rami Porta, M. Aroca González, A. Alix Trueba, F. Lahoz, et al.

Hipoplasia pulmonar. A propósito de un caso.

Rev Clin Esp, 173 (1984), pp. 57-60

Medline

[22.]

M.D. Arteche, L. Oleaga, A. Agote, A. Echeberria, G. Vega-Hazas.

Malformaciones congénitas bronquiales.

Radiología, 31 (1989), pp. 289-295

[23.]

J.M. Mata, J. Cáceres, J. Lucaya, J.A. García-Conesa.

CT of congenital malformations of the lungs.

Radiographics, 10 (1990), pp. 651-674

http://dx.doi.org/10.1148/radiographics.10.4.2377765 | Medline

Tools

Indexed in:

Follow us:

Subscribe:

Subscribe to our newsletter