• Clinical and Experimental Pediatrics
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• v.50 no.2
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• pp.178-181
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• 2007

Purpose : The renal manifestations of tuberous sclerosis complex (TSC) are remarkably diverse, including polycystic kidney disease, simple renal cysts, renal cell carcinomas, and angiomyolipomas. All of these occur in children as well as adults in TSC. Angiomyolipomas, which can cause spontaneous life-threatening hemorrhages, are by far the most prevalent and the greatest source of morbidity. Here, we will address our experience, adding to the literature on pediatric patients with TSC requiring evaluation and treatment for renal manifestations. Methods : A retrospective analysis was made on 19 patients in whom TSC was diagnosed between May 2001 and Oct. 2005 at Severance Hospital. All patients had clinical diagnoses of TSC as defined by the 1998 tuberous sclerosis complex consensus conference. Results : The patients consisted of 13 boys and 6 girls with a mean age of 7.3 years (range 1 to 22). The renal disease associated with TSC included angiomyolipoma in nine patients (47.4 percent), renal simple cyst in one (5.3 percent), hydronephrosis in one (5.3 percent) patient. Eight patients (42.1 percent) presented with normal kidney contours at abdominal ultrasonography. One patient underwent renal replacement therapy due to chronic renal insufficiency after nephrectomy. Hemorrhage from angiomyolipoma was not detected. Conclusion : In our review of 19 cases of TSC, renal manifestations are reported in 57.9 percent of patients. Asymptomatic angiomyolipoma associated with TSC grow gradually, although severe hemorrhages are rare. So patients with TSC should be followed up with serial computerized tomography or abdominal ultrasonography. And also, renal function should be monitored conservatively.

• The Journal of the Korean life insurance medical association
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• v.2 no.1
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• pp.218-232
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• 1985

Congenital hereditary disease is in devided into Infantile type and Adult type, Adult type is hidden for many years and keeps normal renal function till middle age. Cyst is stimultaneously made in both sides and becomes lowered in renal function in 30's to 40's. Infantile type is generally born with the big kidneys, renal failure, undergrowth of intrahepatic bile duct. Both infantile and childhood type have ureteral dilatation and portal hypertension In infantile type, it is mostly developed into renal failure, but generally faces death as a result of hepatic disease. The reason of death is that an abnormal condition of recessive autosome affects the liver and kidneys. While the incidence of infantile type is rare as $0.017{\sim}0.07%$ and it is autosomal recessive heredity, Adult type can rarely exist in infantile period. Though it exists in middle period, 50% of patients can live for 2-4 years after the first symptom incidence and 25% can less than 2 years. It is hard to cure completely in medicine and surgery. Three difficulties in familial incidence are comparative decrease of the donor who have no affection on renal transplantation. For another consideration it is to show the family history for several generations. We, the Med. Dept. of Dae Han Kyouk Life Insurance Co. Ltd., used the ultrasonic apparatus in diagnosing the one case of adult type bilateral polycystic kidney and then doubted the family history. As a result of inspecting the family we experienced bilateral polycystic kidney from 3 persons out of 4 who can be inspected. The results are as follows: 1) We could confirm the polycystic kidney from 3 persons out of 4(75%). 2) Then when they came for check up, chief complaint was the pain in all 3 cases(100%). 3) Accompanying disease was hypertension in 2 cases(67%). 4) In early disease incidence, we couldn't observe the specific change in pathological opinion. 5) All 3 cases are not accompanied with cystic lesion in liver, spleen, pancreas.

• Korean Journal of Veterinary Research
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• v.61 no.2
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• pp.17.1-17.4
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• 2021

A seven-year-old, castrated male, Korean domestic shorthair cat was referred because of a kidney abnormality. Radiography revealed left renal agenesis and right kidney enlargement. Ultrasonography and computed tomography (CT) showed amorphous calcified materials in a cyst-like lesion of the right kidney. In the excretory phase of the CT images, the lesion was opacified with contrast materials, indicating communication with the collecting system. Based on these findings, the cat was diagnosed with a calyceal diverticulum. A calyceal diverticulum may cause various clinical symptoms related to the urinary system. The excretory phase of CT is useful for diagnosing a calyceal diverticulum.

• Childhood Kidney Diseases
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• v.1 no.2
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• pp.144-150
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• 1997

Purpose : The kidney is one of the most common sites of cyst formation. Cystic diseases of the kidney are a diverse group of clinicopathologic entities and variable prognosis. They span a wide range of both age of presentation and severity of the renal disease. And many of them are systemic disorders, sharing similar process of cyst formation in other organs. Recently, development of imaging studies has been contributing widely to the diagnosis of the diseases. Treatment, however, is not established satisfactorily. We performed this study to evaluate the occurrence and treatment of cystic diseases of the kidney. Methods : We reviewed retrospectively the medical records of 44 patients with cystic diseases of the kidney in the Department of Pediatrics, during last 11 years. Results : In the 44 patients with cystic diseases of the kidney, 31 patients(71%) had multicystic dysplastic kidney and 11(35%) of them received nephrectomy due to differentiation from neoplasms or severe abdominal distension. Seven patients(16%) had polycystic kidney disease, and all of them were infantile type. Five patients(11%) were diagnosed as having a simple renal cysts. Progression to renal failure was noted in none of the cases. In 14(32%) out of total 44 patients, the diagnosis was made in neonatal or infantile pelted. Conclusion : The incidence of cystic diseases of the kidney appeared very low, but further investigation on their pathogenesis, classification, and indication of treatment is needed.

• Clinical and Experimental Reproductive Medicine
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• v.48 no.1
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• pp.85-90
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• 2021

We present data from three Caucasian men with Zinner syndrome who attended our center for the treatment of primary couple's infertility. Each patient was scheduled for conventional testicular sperm extraction (cTESE) and cryopreservation. Sperm analysis confirmed absolute azoospermia. Patient 1 had right and left testis volumes of 24 mL and 23 mL, respectively; left seminal vesicle (SV) agenesis, severe right SV hypotrophy with right renal agenesis. Follicle-stimulating hormone (FSH) was 3.2 IU/L. Patient 2 exhibited right and left testis volumes of 18 mL and 16 mL, respectively; a left SV cyst of 32 × 28 mm, ipsilateral kidney absence, and right SV agenesis. FSH was 2.8 IU/L. Patient 3 showed a testicular volume of 10 mL bilaterally, a 65 × 46 mm left SV cyst, right SV enlargement, and left kidney agenesis. FSH was 32.0 IU/L. Sperm retrieval was successful in all patients. Nevertheless, cTESE should be performed on the day of oocyte retrieval.

• Journal of Veterinary Clinics
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• v.29 no.2
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• pp.130-133
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• 2012

The purpose of this study is to assess the relationship between glomerular filtration rate (GFR) and age by using dynamic computed tomography (CT) and Patlak plot analysis in dogs. Fifteen dogs were used in this study. CT-GFR study was performed under general anesthesia using propofol and isoflurane. 1 ml/kg dosage of 300 mgI/ml iohexol was administered at a rate of 3 ml/s during GFR measurement. CT-GFR was determined with a single-slice dynamic acquisition and Patlak plot analysis. The individual and global GFR values were calculated to plasma clearance per body weight (ml/min/kg). Bodyweight ($mean{\pm}SD$) ranged from 2.0 to 5.7 kg ($3.31{\pm}1.13$ kg). Age ranged from 3 years to 13 years old ($7.14{\pm}3.30$). $Mean{\pm}SD$ creatinine ($0.53{\pm}0.34 $mg/dl), phosphorus ($4.1{\pm}1.2$ mg/dL), and albumin ($3.3{\pm}0.3$ mg/dL) concentrations and urine protein-to-creatinine ratios (all ratios were < 0.5) were within reference ranges. Abdominal ultrasonography revealed small-sized renal calculi, mineralization, or renal cyst at eight dogs. The global CT-GFR ranges shown in this study was 2.57 to 6.60 ml/min/kg. In this study, there was no trend toward weight-adjusted CT-GFR with increasing age. We found no relationships between age-related kidney dysfunction in fifteen dogs. Small-sized renal calculi or cysts did not affect renal function in this study. However, it is thought that a large sample size may have been required to document an age effect.

• Journal of the Korean Society of Radiology
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• v.83 no.5
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• pp.1109-1115
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• 2022

Renal angiomyolipomas (AMLs) are typically solid tumors, but there have been few reports of a rare cystic variant of AML. AML with epithelial cysts, where the epithelial cyst has a cuboidal epithelial lining, account for the majority of them. Next, epithelioid AML (EAML) with cystic changes due to hemorrhage and necrosis, which is composed of epithelioid cells with abundant eosinophilic cytoplasm, have also been reported. These rare cystic types of AML can be mistaken for other cystic tumors, such as cystic renal cell carcinoma, in preoperative imaging. We report the imaging findings of a rare case of EAML with epithelial cysts.

• Journal of Veterinary Clinics
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• v.31 no.5
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• pp.466-468
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• 2014

Polycystic kidney disease (PKD) is characterized by multiple cysts within the renal parenchyma and is a common heritable disease in humans, dogs, and cats. However, a few cases of PKD have been described in captive pygmy hippopotamuses. Bilateral PKD was observed in a 33-year-old, 198-kg female pygmy hippopotamus during its necropsy in Seoul Zoo on 15 January 2013. The diagnosis of PKD was confirmed by gross findings and histopathological examination. One kidney was slightly enlarged, and the lower portion of other kidney contained a large cyst filled with light yellow, watery fluid. Both kidneys had numerous, variably sized fluid-filled cysts of 2 to 20 mm in diameter. Considerable portions of the renal cortex and medulla were replaced by cysts. Microscopic inspection showed that the cysts were lined with low cuboidal to flat epithelial cells. The present case report of PKD in a pygmy hippopotamus is the first in Korea.

• Journal of Veterinary Clinics
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• v.18 no.1
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• pp.70-73
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• 2001

Five-month-old a female mongrel puppy weighing 3.5 kg showed no systemic disorder and particular discomfort except abdominal distension at the first visit. On physical examination an irregular abdominal mass was palpated. One month later she was clumsy and uncoordinated. In addition, lethargy and anorexia were appeared. Then she became comatose and died in spite of initial therapy. In radiographic examination enlargement of both sides of kidney was observed. The hematological examination the dog had WBC of 16,250/$\mu$l, RBC of $7.2{\times}10^6$ $\mu$l, PCV of 32%, total protein of 8.0 g/dl, and fibrinogen of 900 mg/dl. In serum chemistry BUN was 87.4 mg/dl and creatinine was 5.1 mg/dl. Urinalysis revealed pH of 5.6, SG of 1.009 and protein of 500 mg/dl. In urine sediment test many RBCs, leukocytes, inflammatory cells and a few epithelial cells were observed. On histopathologic examination the size of right and left kidney were 15 cm, 16 cm in length, 6 cm, 6 cm in widths, respectively. Both sides of kidney were filled with brown-orange fluid and had irregular capsular surface. The cysts of various sizes were located throughout the cortex and medulla. No abnormality was found in any other organs. Histologically, cyst was lined by cuboidal to slightly flattened tubular epithelium and surrounded by mature fibrous connective tissue. Glomeruli, tubule and renal pelvis remained normal between cysts and exfoliated epithelial cells.

• Clinical and Experimental Pediatrics
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• v.46 no.9
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• pp.913-917
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• 2003

Cardiac rhabdomyomas are relatively uncommon and associated with tuberous sclerosis in 40-50% cases. We report a 10-month-old infant with tuberous sclerosis who presented with ventricular arrythmias and status epilepticus. There were hypopigmented macules on the body, periventricular calcifications, renal cyst and cardiac rabdomyomas just below the aortic valve. The patient required resection of left ventricular subaortic masses due to sustained arrythmia in spite of intravenous amiodarone therapy. The pathologic examination confirmed the diagnosis of rhabdomyoma. The patient had no more arrythmia during the 14 month follow up period. Although cardiac rhabdomyomas may spontaneously regress, surgery is often necessary and frequently resolves the underlying arrythmia.