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Diagnosis: Oligomaganephronia

Oligomeganephronia is a type of renal hypoplasia that results from a quantitative defect of the renal parenchyma with a reduced number of nephrons. This condition differs histopathologically from simple hypoplasia, in which the renal mass is reduced but the number of nephrons is normal. Oligomeganephronia may occur as a sporadic defect or in association with numerous syndromes. Oligomeganephronia results from arrested development of the metanephric blastema at 14-20 weeks' gestation, with subsequent hypertrophy of glomeruli and tubules in the kidney. This hypertrophy and hyperfiltration results in further nephron injury and sclerosis. Eventually, this progressive loss of nephrons leads to end-stage renal disease (ESRD). As with all such disorders, complications such as growth failure, metabolic bone disease, anemia, metabolic acidosis, and other disorders of fluid and electrolyte metabolism may occur. (Lane PH. Oligomeganephronia. In: eMedicine, consulted on July 5th 2017 [Link]). The incidence of the disease is uncertain because it is difficult to diagnose. There are no any special clinical manifestations in patients with OMN. Renal pathology is the only way to diagnose OMN, so missed diagnosis always happens without renal pathology.

In neonates, kidney disease is often suspected with spontaneous pneumothorax, feeding problems, or laboratory finding abnormalities. Oligomeganephronia is usually found in infants in their first year of life and presents with anorexia, vomiting, and failure to thrive. After the first year of life, individuals with oligomeganephronia most often present with short stature, polyuria and polydipsia, or proteinuria. This condition may be incidentally diagnosed when renal abnormalities are discovered during the course of another illness. Small kidney size depicted on ultrasonogram usually establishes diagnosis of hypoplasia. Exhaustive histologic examination of the kidney is the only way to establish an absolute diagnosis of oligomeganephronia. The number of glomeruli is reduced, and the number of glomerular generations varies from 2-6 (normally ≥ 10). Existing glomeruli and tubules are enlarged, and glomerular diameters of 300-400 microns are common. As the disease progresses, segmental sclerosis and hyalinosis of glomeruli are present. Tubular atrophy with interstitial fibrosis occurs (Lane PH. Oligomeganephronia. In: eMedicine, consulted on July 5th 2017 [Link]).

See the chapter: Cystic renal diseases and developmental defects of our Tutorial (only Spanish version).

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References

• Lane PH. Oligomeganephronia. In: eMedicine, consulted on July 5th 2017 [Link]
• Yang XD, Shi W, Li D, Peng T. Oligomeganephronia: case report and literature review. Srp Arh Celok Lek. 2014;142(11-12):732-5 [PubMed link]
• Hopkins K, Mowry J, Houghton D. Congenital oligomeganephronia: computed tomography appearance. Clin Pract. 2013 Nov 8;3(2):e31 [PubMed link]

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