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1/25. Segmental multicystic dysplastic kidney in an adult woman.

    We report a case of unilateral segmental multicystic dysplastic kidney (SMCDK) in an adult woman. A 42-year-old woman presented with abdominal distension and gross hematuria. The preoperative diagnosis was cystic renal cell carcinoma, and a radical nephrectomy was performed. Histopathologically, the resected kidney was SMCDK with severe hydronephrosis.
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2/25. Joubert syndrome: an affected female with bilateral colobomata.

    Joubert syndrome is an autosomal recessive disease characterised by hypoplasia or agenesis of the cerebellar vermis, a syndrome of episodic apnoea-hyperpnoea, rhythmic protrusion of the tongue, abnormal eye movements, hypotonia, ataxia, and psychomotor retardation. Extracerebral malformations include multicystic kidney disease, congenital hepatic fibrosis, sacral dermoid cyst and polydactyly. We report the clinical and pathological findings of a 15-year-old girl with Joubert syndrome diagnosed at autopsy. This patient had bilateral colobomata, which has not been previously described in females with Joubert syndrome.
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keywords = kidney disease, kidney
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3/25. multicystic dysplastic kidney and Kallmann's syndrome: a new association?

    BACKGROUND: Kallmann's syndrome is characterized by anosmia and hypogonadotrophic hypogonadism. Radiographic studies of teenagers and older subjects with the X-linked form of the syndrome have shown that up to 40% have an absent kidney unilaterally. Although this has been attributed to renal "agenesis", a condition in which the kidney fails to form, little is known about the appearance of the developing urinary tract either pre- or post-natally in individuals with Kallmann's syndrome. methods: We describe two brothers who had features of Kallmann's syndrome, most probably of the X-linked variety, who both had a major urinary-tract malformation detected before birth. RESULTS: The brothers were found to have unilateral multicystic dysplastic kidneys on routine antenatal ultrasound scanning and both underwent surgical nephrectomy of these organs post-natally. Immunohistochemical studies on the younger sibling revealed hyperproliferative dysplastic kidney tubules which overexpressed PAX2, a potentially oncogenic transcription factor, and BCL2, a cell-survival factor, surrounded by metaplastic, alpha smooth-muscle actin-positive stroma: similar patterns have been observed in patients with non-syndromic multicystic dysplastic kidneys. CONCLUSIONS: Our results describe a new type of urinary-tract malformation associated with Kallmann's syndrome. However, since multicystic kidneys tend to involute, only when more Kallmann's syndrome patients are screened in utero or in early childhood using structural renal scans, will it be possible to establish whether multicystic kidney disease is a bona-fide part of the syndrome.
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ranking = 2.1180930398833
keywords = kidney disease, kidney
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4/25. Secondary endocardial fibroelastosis associated with Pompe disease and multicystic dysplastic kidney.

    An infant with secondary endocardial fibroelastosis (EFE) associated with glycogen storage disease II (Pompe disease) and multicystic dysplastic kidney (MCDK) is described. She had had refractory heart and renal failure from the early neonatal period. In spite of administration of cathecholamines and diuretics, ventilator support, and peritoneal dialysis, her heart failure due to reduction of left ventricular contractility progressively worsened. She died on the 40th day after admission. Histological examination of a left ventricular autopsy specimen showed prominent thickening of the endocardium due to fibroelastosis, and a lacework-like structure due to accumulation of glycogen in the cardiomyocytes. The EFE was derived from degeneration of the smooth muscle in the endocardium and cardiomyocytes due to glycogen storage. In addition, we supposed that the renal failure due to MCDK made the preload for the ventricles increase and accelerated her heart failure.
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5/25. Laparoscopic removal of multicystic dysplastic pelvic kidney.

    A patient presented to us with a symptomatic, polycystic, nonfunctioning, pelvic kidney, which was removed laparoscopically. Laparoscopic removal of multicystic pelvic kidney has not been reported in an adult. The technique we adopted is described, and the relevant literature is reviewed.
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6/25. A family study and the natural history of prenatally detected unilateral multicystic dysplastic kidney.

    PURPOSE: We document the inheritance pattern of multicystic dysplastic kidney in 3 affected families and screen first-degree relatives of a cohort of children with prenatally detected multicystic dysplastic kidney for renal anomalies. The study also afforded an opportunity to document the natural history of prenatally detected multicystic dysplastic kidney. MATERIALS AND methods: We identified 3 families during clinical treatment of children with prenatally detected multicystic dysplastic kidneys. Other members of these families were evaluated with renal ultrasonography. For the family screening study index cases were identified from a fetal uropathy database. A total of 94 first-degree relatives (52 parents, 35 full siblings and 7 half siblings) of 29 children with prenatally detected multicystic dysplastic kidneys were studied with urinary tract ultrasonography, blood pressure measurement, urinalysis and plasma biochemistry. RESULTS: Two families had affected sibling pairs, 1 of which also had a half sibling with vesicoureteral reflux. The third family included 3 individuals with multicystic dysplastic kidney and 1 with renal agenesis thought to have resulted from involution of multicystic dysplastic kidney. This family is consistent with autosomal dominant inheritance with variable expressivity and reduced penetrance. In the screening study ultrasonography did not demonstrate significant renal anomalies in any of the 94 first-degree relatives of the multicystic dysplastic kidney index cases. Followup assessment of prenatally detected multicystic dysplastic kidneys in index cases demonstrated total involution in 52% at a median age of 6.5 years with no multicystic dysplastic kidney related morbidity. CONCLUSIONS: multicystic dysplastic kidney can be familial but is most commonly a sporadic anomaly. Formal screening of relatives is not recommended. Followup data on a cohort of children with prenatally detected multicystic dysplastic kidney add further support to conservative management.
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keywords = kidney
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7/25. Multicystic congenital mesoblastic nephroma.

    This report describes an unusual example of congenital mesoblastic nephroma cellular variant that presented in a 1-week-old neonate as a multicystic tumor of the kidney. Extensive pseudocystic cavitation resulted from progressive accumulation of ground substance in a loosely myxoid tissue composed of stellate- and spindle-shaped cells that compressed and infiltrated renal tissue. The cells of the tumor were positive for vimentin and smooth muscle actin. The patient is alive and well 16 years after surgery. Differential diagnosis from segmental cystic dysplasia, cystic intralobar nephrogenic rest, cystic nephroma, cystic partially differentiated nephroblastoma, cystic nephroblastoma, and cystic clear cell sarcoma of the kidney, all of which may present at this age, is discussed.
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ranking = 0.33333333333333
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8/25. Normal nephrogenesis occurs in the early stage of bilateral multicystic dysplastic kidneys.

    We present a case report about bilateral multicystic dysplastic kidneys (MCDKs). pregnancy was terminated at 21 weeks' gestation and the fetus was examined by autopsy. The kidneys were replaced by many cysts and there was no parenchyma. However, on histological examination, there was normal nephrogenesis amid the cystic/dysplastic tissue. This suggests that normal nephrogenesis occurs before the secondary changes leading to MCDKs.
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9/25. Incidentally detected nephrogenic rests in the setting of congenital obstructive uropathy.

    PURPOSE: Nephrogenic rests (NR) are clusters of cells similar to renal blastema. NR are frequently seen in kidneys with Wilms' tumor (WT) and are seen with higher frequency in nephrectomy specimens from obstructed and/or multicystic dysplastic kidneys (MCDK) compared to autopsy series of normal kidneys. The significance of NR and their role in tumorigenesis is largely unknown. We report the findings of two cases with NR associated with ureteral ectopy/obstruction and review the relevant literature. MATERIALS AND methods: Two cases of upper pole heminephrectomy associated with ectopic upper pole ureter and resultant hydronephrosis were found to have nephrogenic rests present on pathologic examination. A literature search was done to review recent developments in the understanding of NR and their significance, primarily to guide patient recommendations regarding follow-up. RESULTS: Recent developments in the understanding of NR include the description of intralobar versus perilobar nephrogenic rests and prognostic considerations associated with each. However, the implications of finding nephrogenic rests in upper pole hemi-nephrectomy specimens associated with ureteral ectopy is not well delineated. CONCLUSIONS: The role of NR in tumorigenesis is still poorly understood. Because of the still undefined relationship with WT we recommend patients with incidentally detected NR be followed with serial abdominal ultrasounds for the first 5 years of life.
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keywords = kidney
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10/25. Spontaneous improvement of hypertension in multicystic dysplastic kidney: a case report.

    We report a case of transitory hypertension associated with unilateral multicystic dysplastic kidney (MCDK). A newborn girl with MCDK, detected by prenatal ultrasonography, was conservatively treated and has been followed for 18 months at the Pediatric Nephrourology Unit (HC-Belo Horizonte, brazil). Arterial hypertension was observed at about 4 months of age and was associated with high levels of plasma renin activity and circulating angiotensin, and also with changes in renal Doppler ultrasonography. For these reasons, a nephrectomy was initially proposed. However, a spontaneous improvement of blood pressure levels was noticed at about 10 months of age. Serial Doppler ultrasonography showed involution of the affected renal volume and decreased arterial flow. The anatomical improvement was observed simultaneously with normalization of the peripheral renin and angiotensin values. The literature is reviewed regarding the relationship between hypertension and MCDK.
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