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THEHE RADIOLOGIC demonstration of a single kidney or of bilateral small kidneys in a child presenting with signs and symptoms of renal insufficiency commonly results in the diagnosis of renal hypoplasia. However, small kidneys may be the result of many etiologic factors, including a failure of normal renal differentiation and development, disproportionate growth of the organs, and contraction secondary to scarring.It is difficult to evaluate and clearly identify which of these factors has operated to produce the small kidneys found in a given clinical problem. Furthermore, the terminology as currently applied to describe these conditions is unsatisfactory. A review of the existing literature, and the classifications and definitions which have been employed are needed.Bilateral dwarfed kidneys which on microscopic study were normal have not occurred in our experience. The small kidneys which we have studied have invariably shown histologic evidence of either dysplasia or chronic inflammatory disease or combinations of these alterations. Thus, the term renal hypoplasia, which implies only a failure of maturation, seems inap¬ propriate for these cases.Our experience includes 11 children whose renal disease was initially diagnosed as bilateral these 11 children suggests that the small kid¬ neys found were the result of the processes listed above rather than a failure of renal maturation alone.Six of the 11 cases had the clinical findings characteristic of a slowly progressive nonbacterial inflammatory disease of the renal parenchyma. The histological appearance of the kidneys of these patients demonstrated extensive glomerular fibrosis, interstitial fibrosis, and tubular atrophy. A pathologic diagnosis of chronic glomerulonephritis best characterized these changes. We believe that the kidneys in these six children were small because of this in¬ flammatory process and should be called atrophie (secondary atrophy).The five remaining cases had clinical pictures similar to those patients described above but at autopsy had gross malformations of the genitourinary tract compatible with an error in differentiation of the kidney. Microscopic ex¬ amination of the kidneys in these five children revealed pathologic changes of dysplasia. In ad¬ dition, histologie evidence of chronic pyelone¬ phritis was present in four patients while changes compatible with chronic glomerulone¬ phritis were present in one patient from this group. We believe that the small kidneys found in these cases were the result of an inflamma¬ tory process occurring in a dysplastic kidney.The purpose of this communication is to re¬ port our experience regarding these cases. It is hoped that through reviewing the pathology of these and other cases, an understanding of the etiologic factors producing dwarfed kidneys can be found. A classification for dwarfed kid¬ neys will be presented and hypotheses regarding their pathogenesis will be formulated.
THEHE RADIOLOGIC demonstration of a single kidney or of bilateral small kidneys in a child presenting with signs and symptoms of renal insufficiency commonly results in the diagnosis of renal hypoplasia. However, small kidneys may be the result of many etiologic factors, including a failure of normal renal differentiation and development, disproportionate growth of the organs, and contraction secondary to scarring.It is difficult to evaluate and clearly identify which of these factors has operated to produce the small kidneys found in a given clinical problem. Furthermore, the terminology as currently applied to describe these conditions is unsatisfactory. A review of the existing literature, and the classifications and definitions which have been employed are needed.Bilateral dwarfed kidneys which on microscopic study were normal have not occurred in our experience. The small kidneys which we have studied have invariably shown histologic evidence of either dysplasia or chronic inflammatory disease or combinations of these alterations. Thus, the term renal hypoplasia, which implies only a failure of maturation, seems inap¬ propriate for these cases.Our experience includes 11 children whose renal disease was initially diagnosed as bilateral these 11 children suggests that the small kid¬ neys found were the result of the processes listed above rather than a failure of renal maturation alone.Six of the 11 cases had the clinical findings characteristic of a slowly progressive nonbacterial inflammatory disease of the renal parenchyma. The histological appearance of the kidneys of these patients demonstrated extensive glomerular fibrosis, interstitial fibrosis, and tubular atrophy. A pathologic diagnosis of chronic glomerulonephritis best characterized these changes. We believe that the kidneys in these six children were small because of this in¬ flammatory process and should be called atrophie (secondary atrophy).The five remaining cases had clinical pictures similar to those patients described above but at autopsy had gross malformations of the genitourinary tract compatible with an error in differentiation of the kidney. Microscopic ex¬ amination of the kidneys in these five children revealed pathologic changes of dysplasia. In ad¬ dition, histologie evidence of chronic pyelone¬ phritis was present in four patients while changes compatible with chronic glomerulone¬ phritis were present in one patient from this group. We believe that the small kidneys found in these cases were the result of an inflamma¬ tory process occurring in a dysplastic kidney.The purpose of this communication is to re¬ port our experience regarding these cases. It is hoped that through reviewing the pathology of these and other cases, an understanding of the etiologic factors producing dwarfed kidneys can be found. A classification for dwarfed kid¬ neys will be presented and hypotheses regarding their pathogenesis will be formulated.
Twenty patients with hypoplastic kidney (12 men and 8 women, left kidney in 13 cases, right kidney in 7 cases) were observed between 1961 and 1971. The age of the patients ranged from 14 to 60 years. The anomaly predominated (65% of the patients) in the third and fourth decades of life; in this age the diagnosis of the true nature of the condition was often due to complications requiring examination. Carefully taken history and complete radiological survey are essential for the recognition. The value and characteristic features yielded by particular investigation are discussed. In 8 patients clincial diganosis was confirmed at operation. Most common complications of hypoplastic kidney included hypertension, lithiasis, hydronephrosis, pyelonephritis and periodic hematuria. In 9 pateints (4 women and 5 men) renal hypoplasia was associated with other anomalies of the genitourinary tract.
Hypoplasia is defined in the Merriman-Webster dictionary as “a condition of arrested development in which an organ, or part, remains below the normal size, or in an immature state.” The degree of reduced size is not definitional. Renal hypoplasia, however, has historically been defined as a more marked reduction in renal mass such that presentation in childhood is the norm. There are 3 commonly recognized types of renal hypoplasia, simple hypoplasia, oligomeganephronic hypoplasia (oligomeganephronia) and segmental hypoplasia (Ask-Upmark kidney). They have in common a reduction in the number of renal lobes. A fourth type, not widely recognized, is cortical hypoplasia where nephrogenesis is normal but there is a reduction in the number of nephron generations. Recently there has been great interest in milder degrees of reduced nephron mass, known as oligonephronia because of its association with risk of adult-onset hypertension and chronic kidney disease. Since the last pathology review of this topic was published by Jay Bernstein in 1968, an update of the renal pathology findings in renal hypoplasia is provided with a review of 18 new cases. The renal hypoplasias are then framed within the modern concept of oligonephronia, its diverse causes and prognostic implications.
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