• Childhood Kidney Diseases
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• v.1 no.2
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• pp.151-154
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• 1997

Purpose : The prevalence of renal malformation in Turner syndrome has been quoted as being greater than 50% in older series. Recently in 1988, Lippe's review gave a prevalence of 33%, significantly lower than all previous reports. In 1996, Flynn reported that renal malformation occurs in approximately 24% of all girls with Turner syndrome, and that it is seldom seen in girls with mosaic karyotype who form the predominant subgroup. The aim of this study was to evaluate prevalence of renal malformation by karyotype in Turner syndrome in Korea. Method : We evaluated 81 patients with Turner sundrome diagnosed in Yonsei University from Jan. 1987 to Dec. 1996. The patient entered in this study were those for whom both karyotype and ultrasound examination of the kidney were available. Result : 1) The karyotype showed: 45,X ; 29 cases (38%), mosaicism : 32 cases (40%), structural aberration ; 17 cases (22%). 2) Of the 29 cases of pure 45,X karyotype, 5(17%) had abnormal renal findings, while these were found in only 1 of the 30 mosaic cases(3.3%), and in 1 of the 17 structural aberration cases(6%). The malformation included 3 cases of horseshoe kidney, 2 cases of axial malrotation, hypoplastic kidney and simple cyst each one. There was no statistical significance between 3 groups (p=0.09). Conclusion : We conclude that renal malformation occurs in 9.2% in this study, therefore Korean girls with Turner syndrome have lower rates of renal malformation.

• Journal of Dental Anesthesia and Pain Medicine
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• v.17 no.3
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• pp.215-217
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• 2017

Branchio-oto-renal syndrome (BOR) is a rare autosomal dominant disorder. The features include branchial cysts, hearing loss, ear malformation, preauricular pits, retrognathia, congenital heart disease, and renal abnormalities. However, anesthetic management of these patients has seldom been reported. We report a case in which general anesthesia was performed for dental treatment in a patient with BOR. Airway management, renal function, and hemodynamic changes can be of critical concern during anesthetic management. A 13-year-old girl diagnosed with BOR had severe right hearing loss, right external ear malformation, renal abnormalities, and postoperative patent ductus arteriosus (PDA). Dental extraction under general anesthesia was scheduled for a supernumerary tooth. The procedure was completed with sufficient urine volume, adequate airway management, and stable hemodynamics.

• Childhood Kidney Diseases
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• v.19 no.2
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• pp.154-158
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• 2015

Purpose: This study aimed to evaluate the status of renal function and the presence of urinary abnormalities in early adult patients with Turner syndrome (TS). Methods: Sixty-three girls with TS, who are attending pediatric endocrine clinics in Busan Paik Hosp., were studied. Urine and blood chemistry tests were performed in every visiting times. Renal ultrasonography was performed in all patients at the initial diagnosis, and intravenous pyelography, DMSA renal scan and renal CT were also performed, if necessary. Results: Of the 63 patients, the karyotype showed 45,X in 32 (50.8%), mosaicism in 22 (34.9%) and structural aberration in 9 (14.3%). The renal function at the latest visit was shown as normal in all patients. Nephrotic syndrome had developed in one patient. Hematuria was observed in seven patients. Renal anomalies were observed in 20 of the 63 TS (31.7%). Of the 32 TS patients with 45,X karyotype, 13 (40.6%) had renal anomalies, while these were found in 7 (22.6%) of 31 TS patients with mosaicism/structural aberration. But there was no significant statistical difference between two karyotype groups. Conclusion: Based on this study, most of the patients with TS do not have any significant problems related to renal function until early adulthood, regardless of renal malformation or hematuria.

• Journal of Animal Reproduction and Biotechnology
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• v.34 no.3
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• pp.253-258
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• 2019

A female English bulldog was gave birth two neonates by cesarean section on the sixty one days after mating, but both neonates were died soon after birth. The bodies of neonates were diagnosed using radiography, ultrasonography, computed tomography and necropsy immediately after death. Both neonates had caudal regression syndrome, butterfly vertebra, hydrocephalus, umbilical hernia, cleft palate and bow-legged hind-limb. At necropsy, neonates had mild fetal anasarca, cleft lip and the skull was remained non-union. At thoracic cavity, only three ribs and thoracic spines were existed and patent ductus arteriosus was found. At abnormal cavity, the renal ectopia was found with abnormal morphology. In the present case, those English bulldog neonates with multiple congenital malformation syndromes seriously suffered vertebral column anomalies and that may induced by neural tube defects in during embryonic period. To prevent congenital malformation occurring in English bulldog, further in depth studies are needed for the breed specific genetic diversity and for the reason of behind genetic abnormality in these breed.

• Childhood Kidney Diseases
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• v.22 no.1
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• pp.12-16
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• 2018

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital malformation syndrome that is characterized by a triad of uterine didelphys, blind hemivagina, and ipsilateral renal agenesis. There is a wide variety of phenotypic presentation which is recognized as a spectrum of disease rather than a separate entity. The exact incidence and pathogenesis of HWW syndrome are yet to be investigated. While this disease typically involves adolescent girls who present with abdominal pain or a pelvic mass that is secondary to hematocolpos, nowadays, a majority of potential patients with HWW are being prenatally screened for renal anomalies. Therefore, it is recommended to search for uterovaginal anomalies whenever a multicystic dysplastic kidney or the absence of a kidney is noted in a newborn female, and the role of pediatric nephrologists has become ever more important for early recognition of the disease.

• Journal of Yeungnam Medical Science
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• v.12 no.1
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• pp.96-104
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• 1995

The transarterial embolization has been widely used to control bleeding. It has a variety of clinical utility; to reduce bleeding on the surgical field, to reduce the size of malignant tumor as a preopearative treatment, to treat arteriovenous malformation or arterial aneurysm as a curative method and to promote life quality of patient with diffuse or multiple hepatocellular carcinoma as a palliative treatment, etc. With the advance of modem technology, various embolic materials have been also developed. However, it has not been fully investigated of histopathologic changes of the embolized organs according to the embolic materials used. This study was undertaken to investigate the histopathologic changes of embolized renal artery in rabbit by various embolic materials, according to each embolic material and to time passed by after embolization. Of the 5 arteries embolized by ethylene vinyl alcohol copolymer(EVAL), one showed abscess formation in embolized kidney. The other 4 allowed to perform further pathologic study: within a week after embolization there was no any specific change in vessels, however, minimal endothelial hypertrophy was observed following 2 weeks of embolization. Of the 8 renal arteries embolized by N-buthyl-2-cyanoacrylate(Histoacryl), 4 showed total occlusion of the main renal arteries as well as renal infarction, which reflects the strong adhesiveness of Histoacryl to vascular wall. The other 4 showed fibrinoid degeneration in vascular wall within a week. However, further change was not observed thereafter. In all the 5 renal arteries embolized by polyvinyl alcohol(Ivalon), there were infiltration of inflammatory cells along the vessel walls, within one week, which represents vasculitis. They showed some fibrosis with appearance of giant cells in the vessel wall two weeks after embolization and also showed marked fibrosis of connective tissues surrounding vessels two months after embolization, respectively. The results suggest that EVAL is useful for the embolization of hypervascular lesion with limited arteriovenous fistula, Histoacryl for the curative treatment of the lesion with high blood flow or severe arteriovenous fistula, and Ivalan for palliative treatment of malignant tumor or arteriovenous malformation, respectively.

• Clinical and Experimental Pediatrics
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• v.49 no.7
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• pp.796-799
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• 2006

Multicystic dysplastic kidney and congenital cystic adenomatoid malformation of the lung are independent disorders, but both result from abnormal morphogenesis during embryogenesis. Congenital cystic adenomatoid malformation of the lung is associated with renal anomalies as well as other extrapulmonary anomalies and almost all cases with these anomalies are stillborn. We report a case of a 21-month-old male who was admitted with the impression of acute infectious gastroenteritis; multicystic dysplastic kidney with congenital cystic adenomatoid malformation of the lung was detected incidentally during evaluation.

• Korean Journal of Biological Psychiatry
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• v.5 no.2
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• pp.283-287
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• 1998

The mother was 24 years old, primipara, and had been taking carbamazepine 400mg(serum concentration $5.0-8.5{\mu}g/ml$) during pregnancy without any clinical seizures. A male baby with physical malformation was delivered on week 39. The malformation is extradigit(polydactily) on X-ray of right foot and left mild hydronephrosis on ultrasonography and renal scan with radioactive material. We reported this rare case and reviewed related articles about teratogenic effect of carbamazepine, mechanism of action and prevention of teratogenesis.

• Childhood Kidney Diseases
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• v.23 no.2
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• pp.116-120
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• 2019

Bilateral renal obstruction is a rare critical condition, requiring a prompt diagnostic approach and treatment to restore the renal function. The most commonly observed obstructive uropathy in children is congenital malformation, such as posterior urethral valves and bilateral ureteropelvic junction obstruction. Malignant pelvic masses obstructing the ureter are widely reported in adults but are rarely observed in children. The treatment of ureteral obstruction related to pelvic malignancy is a therapeutic challenge with a median survival duration of 3-7 months in adults; however, pediatric patients with pelvic malignancy leading to ureteral obstruction had better outcomes, with a reported 5-year mortality rate of 20%, than the adult patients. Here, we report a rare case of bilateral ureteral obstruction associated with pelvic rhabdomyosarcoma presenting with acute kidney injury treated by ureteral diversion with double J stent, and concommittent emergency hemodialysis, leading to restoration of good renal function. We suggest that bilateral ureteral obstruction should be released as soon as possible using surgical or interventional approach to minimize the obstruction period, and subsequential chemotherapy may contribute to improvement of survival and recovery of renal function.

• Journal of Veterinary Clinics
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• v.29 no.4
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• pp.352-355
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• 2012

Hypospadias is a rare congenital malformation of the urethra reported in cattle. The urethral lumen of male indigenous Korean calf is open along the ventral aspect of the penis in the perineal region. Renal abscess and renal stone formation causing urinary tact infection has not been reported in hypospadia calves. The objective of this study was investigation for renal abscess and renal stone formation through autopsy. Histopathological examination and laboratory tests were performed. At autopsy, the pustules were formed on the right renal cortex, and the renal medulla abscess were formed on right and left part of the renal pelvis. Histopathological finding, this case was diagnosed as severe acute suppurative and necrotizing pyelonephritis, and severe chronic interstitial nephritis with fibrosis and moderate multifocal acute cystitis with edema. Milky exudate of the kidney has been identified as Actinomyces meyeri using the VITEK-2 system for identification of bacteria, and the stone has been identified as carbonate apatite using FT-IR system for quantification analysis. This case report describe the hypospadias complicated with urinary tract infection due to carbonate apatite stones and Actinomyces meyeri.