No abstract available.
Kidney Diseases, Cystic*

Multilocular renal cyst is an uncommon entity of uncertain nature. By recent years only n few cases had reported in the literature. The difficulties encountered in distinguishing this entity from the Wilms' tumor indicate that this disease warrants great emphasis. We have experienced a case of unilateral multilocular cystic kidney treated with nephrectomy.
Kidney ; Kidney Diseases, Cystic* ; Nephrectomy ; Wilms Tumor

The cystic disease of the kidney include a heterogeneous group of developmental, hereditary, and acquired disorders. Based on extensive microdissection studies, Potter concluded all renal cystic diseases could be categorized into four types. We have experienced 5 cases of cystic kidney disease which were confirmed by aoutopsy and classified as Type I, Type II, Type III, Boderline between types II and III and Type IV according to Potter's classification. We report these cases with a review of literatures.
Classification* ; Kidney ; Kidney Diseases, Cystic* ; Microdissection

Laparoscopic management of renal cystic disease is a safe and effective method for treating those cysts that have indications for surgical intervention. Long term results of treatment of simple cysts, peripelvic cysts, indeterminate cysts and autosomal dominant polycystic kidney disease are so good. The approach - transperitoneal or retroperitoneal - depend on the disease, anatomic location and the preference and expertise of surgeons
Laparoscopy ; Kidney Diseases, Cystic ; Therapeutics ; surgery

PURPOSE: The location of renal cysts is related with the symptoms and signs. Parapelvic cysts are a rare form of simple renal cysts, and they are much more commonly associated with the symptoms of obstruction, pain, infection and stone formation. We evaluated the surgical outcomes of laparoscopic renal cyst marsupialization for treating the peripheral and parapelvic types of renal cysts. MATERIALS AND METHODS: Between November 1993 and April 2007, 87 patients(91 cases) who presented with symptomatic renal cysts underwent laparoscopic renal cyst marsupialization. There were 76 cases in the peripheral cyst group and 15 cases in the parapelvic cyst group. The mean age was 59.2 years(age range: 20-77) for the patients with peripheral renal cysts and 62.9 years(age range: 47-79) for the patients with parapelvic cysts. RESULTS: The mean operative time was 108 minutes(30-280) for the patients with peripheral renal cysts and 144 minutes(80-270) for the patients with parapelvic cysts, and there was a significant difference(p=0.031). However for the other factors, including the time for the first oral intake, the time to remove the drain and the length of the hospital stay, there were no significant differences(p=0.671, 0.088, 0.268, respectively). Complications, including bleeding and leakage, were detected in 11 patients(14.4%) of the peripheral cyst group and in 4 patients(26.6%) of the parapelvic cyst group; there was a statistical difference(p=0.035). Both groups had a high success rate, 98.7% in the peripheral cyst group and 100% in the parapelvic cyst group. CONCLUSIONS: We found that laparoscopic renal cyst marsupialization was a standard treatment with a high success rate and a fast recovery time. However, performing this for parapelvic cysts showed a longer operative time and a higher complication rate as compared with peripheral cysts.
Hemorrhage ; Humans ; Kidney Diseases, Cystic ; Laparoscopy ; Length of Stay ; Operative Time

Multicystic dysplastic kidney is the most frequent cause of abdominal mass in the neonate, but its presentation is variable depending on the size of cystic kidney, state of the opposite kidney and associated anomalies. multicystic dysplastic kidney also represents a spectrum of pathology from unilateral multicystic kidney through segmental and focal multicystic dysplasia to bilateral multicystic kidney. Herein we report 5 cases of MCK with different presentation and histology, a bilateral MCK associated with horseshoe kidney, a large MCK with uremia, a focal segmental MCK with contralateral UPJ obstruction, a small focal segmental MCK with contralateral megaureter and a small MCK detected by ultrasonogram for localization of impalpable testis.
Humans ; Infant, Newborn ; Kidney ; Kidney Diseases, Cystic ; Multicystic Dysplastic Kidney* ; Pathology ; Testis ; Ultrasonography ; Uremia

Autosomal recessive polycystic kidney disease (ARPKD) is a severe form of polycystic kidney disease that is characterized by enlarged kidneys and congenital hepatic fibrosis. The clinical spectrum of this condition shows wide variation. Approximately 30-50% of affected individuals die in the neonatal period, while others survive into adulthood. ARPKD is caused by mutations in the polycystic kidney and hepatic disease 1 (PKHD1) gene on chromosome 6p12, which consists of 86 exons variably assembled into many alternatively spliced transcripts. We report a case of a pathogenic PKHD1 frameshift mutation, c.889_931del43, which was identified using direct full sequencing, associated with enlarged cystic kidneys and dilatation of intrahepatic bile duct, as observed on imaging studies.
Bile Ducts, Intrahepatic ; Dilatation ; Exons ; Fibrosis ; Frameshift Mutation ; Kidney ; Kidney Diseases, Cystic ; Polycystic Kidney Diseases ; Polycystic Kidney, Autosomal Recessive*

Segmental cystic disease of the kidney is a rare form of cystic disease of the kidney that manifests as variable sized, numerous cysts that are localized in a segment of one kidney. Morphologically and pathologically, it is indistinguishable from autosomal dominant polycystic kidney disease except for its unilateral localization, the lack of an autosomal dominant genetic background and the progressive deterioration of the renal function. We experienced a case of surgically confirmed segmental cystic disease of the kidney in a 49-year-old patient and we report on its ultrasonographic and CT findings, along with a brief review of the relevant literature.
Humans ; Kidney ; Kidney Diseases, Cystic ; Middle Aged ; Polycystic Kidney Diseases ; Polycystic Kidney, Autosomal Dominant ; Tomography, X-Ray Computed

A multilocular cyst of kidney is a rare pathological entity, which has been reported in the literature under several names. These various names reflect the controversy surrounding their nature. This tumor is traditionally regarded as benign in nature and a nephrectomy has to be performed because of the difficulty in its accurate diagnosis. Malignant recurrence of a multilocular cyst of kidney has an even rarer incidence, with only a few cases having been reported. We report a case of 50-year-old male, with an incidentally detected right renal cystic mass. This mass was pathologically confirmed as a multilocular cyst of kidney after a radical nephrectomy, which locally recurred as a malignant mesoblastic nephroma.
Diagnosis ; Humans ; Incidence ; Kidney Diseases, Cystic ; Kidney* ; Male ; Middle Aged ; Nephrectomy ; Nephroma, Mesoblastic ; Recurrence*

The primary cilium of renal epithelia acts as a transducer of extracellular stimuli. Polycystin (PC)1 is the protein encoded by the PKD1 gene that is responsible for the most common and severe form of autosomal dominant polycystic kidney disease (ADPKD). PC1 forms a complex with PC2 via their respective carboxy-terminal tails. Both proteins are expressed in the primary cilia. Mutations in either gene affect the normal architecture of renal tubules, giving rise to ADPKD. PC1 has been proposed as a receptor that modulates calcium signals via the PC2 channel protein. The effect of PC1 dosage has been described as the rate-limiting modulator of cystic disease. Reduced levels of PC1 or disruption of the balance in PC1/PC2 level can lead to the clinical features of ADPKD, without complete inactivation. Recent data show that ADPKD resulting from inactivation of polycystins can be markedly slowed if structurally intact cilia are also disrupted at the same time. Despite the fact that no single model or mechanism from these has been able to describe exclusively the pathogenesis of cystic kidney disease, these findings suggest the existence of a novel cilia-dependent, cyst-promoting pathway that is normally repressed by polycystin function. The results enable us to rethink our current understanding of genetics and cilia signaling pathways of ADPKD.
Calcium ; Cilia* ; Genetics ; Kidney Diseases, Cystic ; Polycystic Kidney, Autosomal Dominant* ; Transducers ; TRPP Cation Channels*