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Original Articles
- Beckwith-Wiedemann Syndrome with Unusual Sialoadenomegaly.
- Hye Seung Han, Seung Sook Lee, Suk Keun Lee, Je G Chi
- Korean J Pathol. 1996;30(10):939-942.
- 1,875 View
- 19 Download
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Abstract
PDF - Beckwith-Wiedemann syndrome is a rare clinical entity characterized by exomphalos, macroglossia, macrosomia, and renal hyperplasia/dysplasia. Although its entity is established, its etiology and obligatory features have not been settled. We report an autopsy case with the unusual involvement of the salivary gland. This infant was born to a 37-year-old mother as a normal full-term spontaneous delivery. At 11 days of age she developed with purulent eye discharge and weak sucking, and died suddenly. At autopsy the baby weighed 2,630 gm and the head circumference was 35 cm. She showed thick and prominent skin folds, bilateral aural fissures, macroglossia, hepatomegaly, cardiomegaly, dysmorphic kidneys, and nesidioblastosis. Both kidneys showed dysplastic tubules and hyperplastic cortical tissue enclosing the medulla. In this case there were characteristic findings in major and minor salivary glands with both acinar and ductal hyperplasia, and hypertrophy of mammary glands. Besides, she had generalized depletion of subcutaneous fat, immature buccal fat, patent ductus arteriosus, hyperlobation of the right lung, two accessory spleens, and hyperplasia of basophils and chromophobes in the pituitary gland. The lungs showed diffuse interstitial pneumonia and multiple fibrin thrombi. There were no adrenal cytomegaly, umbilical hernia and exophthalmos.
- Renal Dysplasia: A Clinicopathologic Review of Six Cases.
- Gil Hyun Kang, Jong Ok Kim, Bum Kyung Kim, Kwang Sun Suh
- Korean J Pathol. 1997;31(1):34-39.
- 1,717 View
- 16 Download
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Abstract
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- Renal dysplasia results from aberrant histogenesis in metanephric differentiation. It is characterized morphologically by abnormal organization and a persistence of primitive structures, such as cartilage, undifferentiated mesenchyme, and immature tubules. Six cases of renal dysplasia from five children and one adult are reviewed. Five patients were female and one patient was male. The chief complaint was urinary incontinence in four patients, dysuria in one patient, and the sixth patient suffered from vesicoureteral reflux. No evidence of family history of renal dysplasia in any patient was seen. According to Risdon's classification, three cases were hypoplastic dysplasia, one case was dysplasia in a duplex system, one case was dysplasia in a triplex system, and one case was dysplasia with vesicoureteral reflux. The ipsilateral ectopic ureteral orifice was identified in four patients, two of which drained into a Gartner's duct cyst, and the orifice was suggested in one patient. On histologic examination, all cases showed primitive ducts surrounded by concentrically arranged primitive mesenchyme. Nests of metaplastic cartilage were observed within the stroma in three of the six cases.
- Congenital Cystic Disease of the Kidney overview and a classification.
- Mee Joo, Yeon Mee Kim, Chong Jai Kim, Yeon Lim Suh, Jeong Wook Seo, Je Geun Chi
- Korean J Pathol. 1997;31(3):233-243.
- 1,704 View
- 22 Download
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Abstract
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- The congenital renal cystic disease encompasses a complex group of pathologic and clinical entities. We retrospectively reviewed 42 cases of congenital renal cystic lesions classified into four Potter types in a series of 2,063 consecutive autopsies from 1981 to 1996. According to our study based on morphologic, clinical, genetic features and associated anomalies, type I and III are relatively compatible with Potter's original definition. However, it was reasonable that type II and IV are classified to the same group because of: 1) very similar histologic findings representing dysplastic kidney, 2) many associated anomalies, 3) no evidence of inheritance, and 4) presence of a combined type. Syndrome associated cysts, such as Meckel-Gruber syndrome, were also separately classified. If the dysplastic evidence was insufficient for diagnosis to the dysplastic kidney in type II and IV, then these cases would be better classified into a cystic disease associated with congenital hydronephrosis. We propose a classification of the congenital cystic disease of the kidney to be: 1) dysplastic kidney, 2) cystic disease associated with congenital hydronephrosis, 3) polycystic kidney, and 4) syndromic cystic disease.
Case Report
- Multicystic Renal Dysplasia with Ipsilateral Ectopic Ureteral Orifice and Seminal Vesicle Cyst: A case report.
- Hyun Jin Son, Joo Heon Kim, Myoung Jae Kang
- Korean J Pathol. 2000;34(4):310-313.
- 1,991 View
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Abstract
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- Renal dysplasia results from aberrant metanephric histogenesis caused fundamentally by a defect in inducer tissue or responding tissue. Dysplastic kidneys vary tremendously in gross and microscopic appearance but are characterized by abnormal organization and a mixed population of primitive structures, such as fetal or immature cartilage, dysplastic ducts, immature tubules, and undifferentiated mesenchyme. We report a case of unilateral multicystic renal dysplasia associated with an ipsilateral ectopic ureteral orifice entering a seminal vesicle cyst in a 33-year-old man. He was admitted due to primary infertility which had developed three years ago. The his semen analysis revealed oligospermia. No evidence of a family history of renal dysplasia was reported. Microscopic examination showed that the entire kidney was composed of cysts lined by flattened cells, dysplastic ducts and immature tubules surrounded by collars of spindle cells, primitive mesenchyme, and a few aberrantly formed glomeruli.
Original Articles
- PAX2 Expression in Renal Dysplasia.
- Yoon Hee Lee, Woo Hee Jung, Soon Won Hong, Hyeon Joo Jeong
- Korean J Pathol. 2007;41(2):69-78.
- 1,987 View
- 23 Download
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Abstract
PDF
- BACKGROUND
Renal dysplasia is the abnormal development of the kidney. The condition is usually detected in childhood along with other urinary tract anomalies, but can remain unnoticed until adulthood. It was recently reported that a PAX2 gene mutation plays a major role in the development of renal dysplasia. The aim of this study was to examine the expression of PAX2 in dysplastic kidneys of children and adults.
METHODS
A total of 30 cases diagnosed with renal dysplasia after a nephrectomy were examined. PAX2 expression was evaluated using immunohistochemistry. Apoptosis was detected using an Apop Tag detection kit.
RESULTS
In the dysplastic kidneys, there was strong PAX2 expression in the epithelia of the primitive ducts in both children and adults, but the degree was significantly lower in adults (p=0.007). However, the mesenchyme surrounding the primitive ducts of children showed stronger staining for the smooth muscle actin antibody and trichrome than the adults. The apoptosis index was significantly higher in the primitive duct epithelia than in the surrounding normal collecting duct epithelia (p=0.000).
CONCLUSIONS
PAX2 is overexpressed in the primitive ducts of renal dysplasia, which is sustained until adulthood and is associated with increased apoptosis. However, a decrease in PAX2 expression in the dysplastic epithelia and mesenchymal cuff of adults suggest a gradual regression of the dysplastic elements with time.
- Multicystic Renal Dysplasia associated with Ectopic Ureteral Orifice.
- Shin Mong Kang, Yong Koo Park, Ju Hie Lee, Soo Eung Chai, Moon Ho Yang
- Korean J Pathol. 1988;22(3):331-335.
- 1,903 View
- 12 Download
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Abstract
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- A case of unilateral multicystic renal dysplasia was reported in view of rarity, and a review of literature 23-year-old female was admitted to Kyung Hee University Hospital with a complaint of urinary incontinence. An excretory urogram revealed non-visualization of the right kidney. The right ureter had opening at the 2 cm above the vaginal introitus. The resected right kidney revelaed multilobulated outer surface with multiloculated cystic struture on cut surface. The associated anomaly was uterine didelphy. The condition was treated by nephroureterectomy.
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