Skip to main content
PMC home page
Archives of Disease in Childhood logoLink to Archives of Disease in Childhood
. 1979 Jan;54(1):32–38. doi: 10.1136/adc.54.1.32

The lungs in congenital bilateral renal agenesis and dysplasia.

A Hislop, E Hey, L Reid
PMCID: PMC1545185  PMID: 570375

Abstract

A detailed quantitative analysis was made of the lungs from 8 infants dying with bilateral renal agenesis or dysplasia. Total lung volume was reduced in all cases, particularly in those with renal agenesis. In both groups there was a reduction in number of airway generations, indicating interference with development at between 12 and 16 weeks' gestation. The alveoli in each acinus were reduced in size and, in some cases, number--although their stage of differentiation was normal for age--pointing to a disturbance of growth during later fetal life also. As liquor is largely non-renal in origin at least up to 16 weeks' gestation, it seems that there are factors other than the oligohydramnios interfering in early lung growth in these cases, such as reduced proline production by the kidney.

Full text

PDF
32

Images in this article

35Figure
on p.35

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. ASHLEY D. J., MOSTOFI F. K. Renal agenesis and dysgenesis. J Urol. 1960 Mar;83:211–230. doi: 10.1016/S0022-5347(17)65695-7. [DOI] [PubMed] [Google Scholar]
  2. Alcorn D., Adamson T. M., Lambert T. F., Maloney J. E., Ritchie B. C., Robinson P. M. Morphological effects of chronic tracheal ligation and drainage in the fetal lamb lung. J Anat. 1977 Jul;123(Pt 3):649–660. [PMC free article] [PubMed] [Google Scholar]
  3. BAIN A. D., SCOTT J. S. Renal agenesis and severe urinary tract dysplasia: a review of 50 cases, with particular reference to the associated anomalies. Br Med J. 1960 Mar 19;1(5176):841–846. doi: 10.1136/bmj.1.5176.841. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. BUCHER U., REID L. Development of the intrasegmental bronchial tree: the pattern of branching and development of cartilage at various stages of intra-uterine life. Thorax. 1961 Sep;16:207–218. doi: 10.1136/thx.16.3.207. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Bashour B. N., Balfe J. W. Urinary tract anomalies in neonates with spontaneous pneumothorax and/or pneumonediastrnum. Pediatrics. 1977 Jun;59 (Suppl)(6 Pt 2):1048–1049. [PubMed] [Google Scholar]
  6. Buchta R. M., Viseskul C., Gilbert E. F., Sarto G. E., Opitz J. M. Familial bilateral renal agenesis and hereditary renal adysplasia. Z Kinderheilkd. 1973 Aug 31;115(2):111–129. doi: 10.1007/BF00440537. [DOI] [PubMed] [Google Scholar]
  7. CARPENTIER P. J., POTTER E. L. Nuclear sex and genital malformation in 48 cases of renal agenesis, with especial reference to nonspecific female pseudoheramphroditism. Am J Obstet Gynecol. 1959 Aug;78(2):235–258. doi: 10.1016/0002-9378(59)90168-1. [DOI] [PubMed] [Google Scholar]
  8. Cain D. R., Griggs D., Lackey D. A., Kagan B. M. Familial renal agenesis and total dysplasia. Am J Dis Child. 1974 Sep;128(3):377–380. doi: 10.1001/archpedi.1974.02110280107016. [DOI] [PubMed] [Google Scholar]
  9. Chamberlain D., Hislop A., Hey E., Reid L. Pulmonary hypoplasia in babies with severe rhesus isoimmunisation: a quantitative study. J Pathol. 1977 May;122(1):43–52. doi: 10.1002/path.1711220108. [DOI] [PubMed] [Google Scholar]
  10. D'AGOSTINO A. N., KERNOHAN J. W., BROWN J. R. THE DANDY-WALKER SYNDROME. J Neuropathol Exp Neurol. 1963 Jul;22:450–470. doi: 10.1097/00005072-196307000-00007. [DOI] [PubMed] [Google Scholar]
  11. DAVIDSON W. M., ROSS G. I. Bilateral absence of the kidneys and related congenital anomalies. J Pathol Bacteriol. 1954 Oct;68(2):459–474. doi: 10.1002/path.1700680220. [DOI] [PubMed] [Google Scholar]
  12. Davies G., Reid L. Growth of the alveoli and pulmonary arteries in childhood. Thorax. 1970 Nov;25(6):669–681. doi: 10.1136/thx.25.6.669. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. EMERY J. L., MITHAL A. The number of alveoli in the terminal respiratory unit of man during late intrauterine life and childhood. Arch Dis Child. 1960 Dec;35:544–547. doi: 10.1136/adc.35.184.544. [DOI] [PMC free article] [PubMed] [Google Scholar]
  14. FULTON R. M., HUTCHINSON E. C., JONES A. M. Ventricular weight in cardiac hypertrophy. Br Heart J. 1952 Jul;14(3):413–420. doi: 10.1136/hrt.14.3.413. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Finegold M. J., Katzew H., Genieser N. B., Becker M. H. Lung structure in thoracic dystrophy. Am J Dis Child. 1971 Aug;122(2):153–159. doi: 10.1001/archpedi.1971.02110020087013. [DOI] [PubMed] [Google Scholar]
  16. GOLDSTON A. S., BURKE E. C., D AGOSTINO A., MCCAUGHEY W. T., MACCAUGHEY W. T. NEONATAL POLYCYSTIC KIDNEY WITH BRAIN DEFECT. Am J Dis Child. 1963 Nov;106:484–488. doi: 10.1001/archpedi.1963.02080050486016. [DOI] [PubMed] [Google Scholar]
  17. HAYWARD J., REID L. M. Observations on the anatomy of the intrasegmental bronchial tree. Thorax. 1952 Mar;7(1):89–97. doi: 10.1136/thx.7.1.89. [DOI] [PMC free article] [PubMed] [Google Scholar]
  18. Hack M., Jaffe J., Blankstein J., Goodman R. M., Brish M. Familial aggregation in bilateral renal agenesis. Clin Genet. 1974;5(2):173–177. doi: 10.1111/j.1399-0004.1974.tb01679.x. [DOI] [PubMed] [Google Scholar]
  19. Hislop A., Reid L. Intra-pulmonary arterial development during fetal life-branching pattern and structure. J Anat. 1972 Oct;113(Pt 1):35–48. [PMC free article] [PubMed] [Google Scholar]
  20. Hislop A., Reid L. New pathological findings in emphysema of childhood. 1. Polyalveolar lobe with emphysema. Thorax. 1970 Nov;25(6):682–690. doi: 10.1136/thx.25.6.682. [DOI] [PMC free article] [PubMed] [Google Scholar]
  21. Hislop A., Reid L. Weight of the left and right ventricle of the heart during fetal life. J Clin Pathol. 1972 Jun;25(6):534–536. doi: 10.1136/jcp.25.6.534. [DOI] [PMC free article] [PubMed] [Google Scholar]
  22. Kitagawa M., Hislop A., Boyden E. A., Reid L. Lung hypoplasia in congenital diaphragmatic hernia. A quantitative study of airway, artery, and alveolar development. Br J Surg. 1971 May;58(5):342–346. doi: 10.1002/bjs.1800580507. [DOI] [PubMed] [Google Scholar]
  23. LANDING B. H. Amnion nodosum: a lesion of the placenta apparently associated with deficient secretion of fetal urine. Am J Obstet Gynecol. 1950 Dec;60(6):1339–1342. doi: 10.1016/0002-9378(50)90016-0. [DOI] [PubMed] [Google Scholar]
  24. Leonidas J. C., Fellows R. A., Hall R. T., Rhodes P. G., Beatty E. C. Value of chest radiography in the diagnosis of Potter's syndrome at birth. Am J Roentgenol Radium Ther Nucl Med. 1975 Apr;123(4):716–723. doi: 10.2214/ajr.123.4.716. [DOI] [PubMed] [Google Scholar]
  25. Liberman M. M., Abraham J. M., France N. E. Association between pneumomediastinum and renal anomalies. Arch Dis Child. 1969 Aug;44(236):471–475. doi: 10.1136/adc.44.236.471. [DOI] [PMC free article] [PubMed] [Google Scholar]
  26. Mauer S. M., Dobrin R. S., Vernier R. L. Unilateral and bilateral renal agenesis in monoamniotic twins. J Pediatr. 1974 Feb;84(2):236–238. doi: 10.1016/s0022-3476(74)80608-6. [DOI] [PubMed] [Google Scholar]
  27. PATHAK I. G., WILLIAMS D. I. MULTICYSTIC AND CYSTIC DYSPLASTIC KIDNEYS. Br J Urol. 1964 Sep;36:318–331. doi: 10.1111/j.1464-410x.1964.tb09516.x. [DOI] [PubMed] [Google Scholar]
  28. POTTER E. L. BILATERAL ABSENCE OF URETERS AND KIDNEYS: A REPORT OF 50 CASES. Obstet Gynecol. 1965 Jan;25:3–12. [PubMed] [Google Scholar]
  29. Perlman M., Levin M. Fetal pulmonary hypoplasia, anuria, and oligohydramnios: clinicopathologic observations and review of the literature. Am J Obstet Gynecol. 1974 Apr 15;118(8):1119–1123. doi: 10.1016/0002-9378(74)90692-9. [DOI] [PubMed] [Google Scholar]
  30. Perlman M., Williams J., Hirsch M. Neonatal pulmonary hypoplasia after prolonged leakage of amniotic fluid. Arch Dis Child. 1976 May;51(5):349–353. doi: 10.1136/adc.51.5.349. [DOI] [PMC free article] [PubMed] [Google Scholar]
  31. Ratten G. J., Beischer N. A., Fortune D. W. Obstetric complications when the fetus has Potter's syndrome. I. Clinical considerations. Am J Obstet Gynecol. 1973 Apr 1;115(7):890–896. doi: 10.1016/0002-9378(73)90663-7. [DOI] [PubMed] [Google Scholar]
  32. Reale F. R., Esterly J. R. Pulmonary hypoplasia: a morphometric study of the lungs of infants with diaphragmatic hernia, anencephaly, and renal malformations. Pediatrics. 1973 Jan;51(1):91–96. [PubMed] [Google Scholar]
  33. Renert W. A., Berdon W. E., Baker D. H., Rose J. S. Obstructive urologic malformations of the fetus and infant--relation to neonatal pneumomediastinum and pneumothorax (air-block). Radiology. 1972 Oct;105(1):97–105. doi: 10.1148/105.1.97. [DOI] [PubMed] [Google Scholar]
  34. Reynolds E. O., Roberton N. R., Wigglesworth J. S. Hyaline membrane disease, respiratory distress, and surfactant deficiency. Pediatrics. 1968 Nov;42(5):758–768. [PubMed] [Google Scholar]
  35. SYLVESTER P. E., HUGHES D. R. Congenital absence of both kidneys; a report of four cases. Br Med J. 1954 Jan 9;1(4853):77–79. doi: 10.1136/bmj.1.4853.77. [DOI] [PMC free article] [PubMed] [Google Scholar]
  36. Stern L., Fletcher B. D., Dunbar J. S., Levant M. N., Fawcett J. S. Pneumothorax and pneumomediastinum associated with renal malformations in newborn infants. Am J Roentgenol Radium Ther Nucl Med. 1972 Dec;116(4):785–791. doi: 10.2214/ajr.116.4.785. [DOI] [PubMed] [Google Scholar]
  37. Strang L. B. Growth and development of the lung: fetal and postnatal. Annu Rev Physiol. 1977;39:253–276. doi: 10.1146/annurev.ph.39.030177.001345. [DOI] [PubMed] [Google Scholar]
  38. Whitehouse W., Mountrose U. Renal agenesis in nontwin siblings. Am J Obstet Gynecol. 1973 Jul 15;116(6):880–882. doi: 10.1016/0002-9378(73)91027-2. [DOI] [PubMed] [Google Scholar]

Articles from Archives of Disease in Childhood are provided here courtesy of BMJ Publishing Group

RESOURCES