• Clinical and Experimental Pediatrics
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• v.48 no.6
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• pp.669-674
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• 2005

Nephrogenic diabetes insipidus (NDI) is a disorder in which the secretion of antidiuretic hormone is normal, but the response of the renal collecting tubules to vasopressin is impaired. Compared with acquired NDI (a-NDI), which is secondary to chronic bilateral incomplete urinary tract obstruction with hydronephrosis, congenital NDI (c-NDI) is a very rare heritable disorder that usually follows the X- linked recessive pattern. Clinical symptoms of c-NDI can be non specific, and often the disease ultimately results in failure to thrive, or mental retardation. Recently, the diagnosis can be confirmed by direct sequencing analysis of the peripheral blood specimens. The long-term results of treatment for c-NDI are not satisfactory. Reports on the follow up of c-NDI cases are rare and there is no report on the cases treated with combinations of three drugs. We report herein a case of severe c-NDI in an 8 year-old-boy with a severely dysconfigurated urinary tract system. The patient and his mother showed a frameshift mutation on the AVPR2 gene on chromosome Xq28:.847_851delTGCTG (p.C283fsX90). The patient showed normal growth and development by treatment with combinations of hydrochlorothiazide ($65mg/m^2$), amiloride (0.3 mg/kg/d) and indomethacin ($100mg/m^2$), yet after five years he needed adjuvant cystostomy to relieve him from the residual symptoms of urgency with polyuria.

• The Korean Journal of Nuclear Medicine
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• v.9 no.1
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• pp.51-58
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• 1975

It is a great value to find an early detection of involvement of ureteric obstruction in the carcinoma of cervix. Little or no knowledge of the condition of the kidneys or the lower urinary tract are able to elucidate by the biochemical studies such as blood nitrogen or urine creatinine in carcinoma of cervix. Findings of urography delineates the condition of urinary tract stasis in the renal pelvis and ureters, however, slight stasis maybe difficult to demonstrate. On the other hand isotope nephrography is accepted as a sensitive method to observe renal function especially in regarding to the excretory function of kidney. It was attempted to analysis the findings of urography conjunction with isotope nephrography in 50 cases of unselected patients with invasive carcinoma of cervix through pre and post irradiation follow up studies. Urography was done as a routine procedure and.analysed emphasising changes of collecting systems and ureter condition. Isotope nephrography was carried out by means of $15{\mu}ci\;I^{131}$-Hippuran injected intravenously and the curves were analysed as follows. Parameter were; time of maximum amplitude ($T_{max}$), half time of maximum amplitude ($T\frac{1}{2}$), Kac and Kex value calculated from these two parameters in Tobe's method. The excretion index by Aurell defines the ratio between the maximum activity and the activity measured on the slope of the third phase ten minites after it has reached its maximum. Results: 1. 28.8% had an abnormal IVP suggestive of ureteric involvement before irradiation therapy and the patient of stage III and IV were the great part. 2. 21.7% had abnormal findings of per-irradiation renogram whom showed normal IVP. The other group showed normal IVP which group also showed normal renogram prior irradiation. 3. The more severe the ureteric involvement, the change of excretion index was greater. 4. Even in stage I and II patient, abnormal renogram was revealed in 12 cases (39.4%) among 31 cases. 5. All cases of TAH showed abnormal findings of IVP and renogram. 6. No. definite change of renogram was obtained just after the irradiation therapy (point $A:8000{\sim}9000rads,\;B:5000{\sim}6000rads,\;Co:11000{\sim}13000rads$). Each 3 month follow up study was performed and comparing with preirradiation study which showed significant changes of excretion index of renogram were 42.8% in $6{\sim}9$ month follow-up and 75% in $9{\sim}12$ month, respectively. 7. It seems to be important to observe the parameter Kex and excretion index of renogram to determine early abnormality of kidney excretory function by means of post-irradiation follow up study.

• Childhood Kidney Diseases
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• v.14 no.2
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• pp.203-209
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• 2010

Purpose : It has been a common medical practice to use prophylactic antibiotics to prevent recurrent urinary tract infections (UTI) in high risk situations such as urinary tract obstruction, vesicoureteral reflux, neurogenic bladder, or urinary stones. But sometimes, we meet difficult situation of breakthrough infections (BI) which might cause new or progressive renal scarring. The clinical characteristics of children contracting breakthrough UTI experienced in a single center were studied. Methods : The study was done retrospectively through medical records of 150 pediatric patients who had been cared in pediatric and urologic clinics of Pusan National University Hospital from Jan. 2001 till June 2006 and had prophylactic antibiotics to prevent recurrent UTI. Results : The starting age of prophylactic antibiotics of 150 patient was 1-76 months, and median age was 5 months. The BI developed 61 times in 43 patients (28.7%), 1.5 times per 100 patient-months. The BI occurred more frequently in patients with higher grade of VUR, and in the cases with abnormal DMSA scan. Co-trimoxazole was more effective than 2nd and 3rd generation cephalosporins to prevent UTI. The distribution of causative organisms was more diverse than usual UTI. The causative organisms were sensitive to the antibiotics used for prophylaxis in 29.5%, and resistant in 59.1%. After experience of BI, 40 percents of patients went to the surgical treatment including endoscopic injection of Deflux, 35% to new antibiotics for prophylaxis, 26% remain on the same antibiotics as the previous one. Conclusion : Based on our study results, preexisting renal scar might be one of the factors which should be considered in favor of early surgical interventions of VUR. Poor compliance and wrong selection of antibiotics such as cephalosporins are important underlying causes of breakthrough UTIs.

• Childhood Kidney Diseases
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• v.2 no.2
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• pp.161-168
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• 1998

Purpose : Lots of congenital anomalies of urinary tract including hydronephrosis are detected in fetus and neworn by popular use of prenatal ultrasonography. But there are little data available in Korea about natural course of hydronephrosis diagnosed antenatally by ultrasonography. So we intended to help management of these patients by analizing the follow up data of the neonates with hydronephrosis diagnosed antenatally. Methods : We evaluated 22 patients with neonatal hydronephrosis(33 renal units) who were diagnosed prenatally and confirmed postnatally. Especially patients with suspected ureteropelvic junction obstruction were followed regulary with renal ultrasonography and diuretic renography for 8-24 months. Results : 1) The etiologies of neonatal hydronephrosis diagnosed prenatally were suspected ureteropelvic junction obstruction($69.9\%$), vesicoureteral reflux($15.1\%$), primary megaureter($3.0\%$), double ureter with ureterocele($3.0\%$), ureteral stricture($3.0\%$), multicystic dysplastic kidney(3.0$\%$), and ureterovesical junction obstruction(3.0$\%$). 2) The follow up results of 23 renal units of suspected ureteropelvic junction obsruction: Except 4 renal units with palpable abdominal mass, of the remained 19 renal units, 14 units($73.6\%$) were improved spontaneously, 3 units($15.7\%$) remained stable, only2 units($10.5\%$) were aggravated. Conclusion : We concluded that in most cases of hydronephrosis there is no need for immediate surgery, and that nonoperative approach, using serial ultrasonography and diuretic renogram, is safe management of neonatal hydronephrosis diagnosed anteratally.

• Childhood Kidney Diseases
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• v.9 no.1
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• pp.69-75
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• 2005

Purpose : The most important management of congenital hydronephrosis consists of the early diagnosis and evaluation of the pathologic abnormalities of congenital hydronephrosis. This study was conducted to investigate the different causes of hydronephrosis and its clinical outcome. Methods : 54 live neonates who were hospitalized and diagnosed with congenital hydronephrosis at Chungnam National University Hospital from Aug. 1998 to Aug. 2003 were retrospectively analyzed. Results : Hydronephrosis(renal pelvic AP diameter $gt;5 mm) was postnatally detected in 54 cases(2.1%) among 2,539 neonates who were hospitalized from Aug. 1998 to Aug. 2003. There were three times more males than females. Additional imaging studies revealed that ureteropelvic junction obstruction was the most common postnatal diagnosis(48.7%), followed by multicystic dysplastic kidney, vesicoureteral reflux and duplication kidney with ureterocele. Spontaneous regression of hydronephrosis was revealed in 25 renal units(75.8% ) of mild hydronephrosis, 14 renal units of moderate hydronephrosis and 1 renal unit of severe hydronephrosis. Operative correction were carried out in 14 renal units(70%) of severe hydronephrosls. Conclusion : The most common established cause of congenital hydronephrosis in this study was ureteropelvic junction obstruction. There are many cases of spontaneous regression in mild to moderate congenital hydronephrosis. Urinary tract infections occur in many neonates with hydronephrosis. Therefore, early detection and evaluation of congenital hydronephrosis and continuous follow-up at regular intervals are necessary for conservation of renal function. (J Korean Soc Pediatr Nephrol 2005;9:69-75)

• Tuberculosis and Respiratory Diseases
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• v.57 no.4
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• pp.377-380
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• 2004

We report a rare case of idiopathic chylothorax and chyluria. A 31 year-old woman was referred to our hospital with a right-sided pleural effusion. Cream-colored pleural fluid and urine were confirmed as chylothorax and chyluria, respectively, by a lipoprotein electrophoresis. Even though she had previously underwent surgery for pelvic fibrosarcoma and experienced its recurrence, there has been no change of mass size and no evidence of thoracic duct or urinary tract obstruction as of the moment. Hence, idiopathic chylothorax and chyluira was diagnosed. Because she responded poorly to conservative treatment, thoracic duct ligation and pleurodesis were performend ; wherease chyluria was resolved spontaneously.

• Childhood Kidney Diseases
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• v.16 no.1
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• pp.63-67
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• 2012

Acute pyelonephritis (APN) is a relatively common bacterial infection in children. In previously healthy children, acute kidney injury (AKI) is very rare in the course of APN without urinary tract obstruction, renal hypoperfusion due to hypotension or exposure to nephrotoxic agents. We describe a case of AKI secondary to APN and renal abscess in a child with vesicoureteral reflux. With antibiotic treatment and adequate hydration, the patient was improved. APN should be included in the differential diagnosis of AKI and adequate treatment by proper antibiotics is crucial for full recovery of renal function.

• Korean Journal of Veterinary Research
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• v.39 no.4
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• pp.832-837
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• 1999

lntrarenal resistive index (RI) was determined in normal, sedated and diuretic kidneys. The mean RI values of normal dogs were $0.64{\pm}0.03$ without statistically significant difference between left and right kidney. Interrenal RI difference (${\Delta}RI$) was $0.03{\pm}0.01$. No significant relationship was found between mean intrarenal RI versus body weight. The mean intrarenal RI values after administration of acepromazine, xylazine, thiopental, and ketamine were $0.66{\pm}0.03$, $0.64{\pm}0.03$, $0.56{\pm}0.05$, and $0.50{\pm}0.05$, respectively. After administration of acepromazine or xylazine, a significant change of the RI was not found. But, a significant decrease of the RI following thiopental or ketamine administration could be observed as compared with the normal RI. The mean intrarenal RI values were $0.63{\pm}0.06$ and $0.62{\pm}0.04$ at 10 minutes and 30 minutes after injection of furosemide, respectively. No significant change of intrarenal RI was found after administration of furosemide. But, mannitol significantly decreased mean intrarenal RI to $0.57{\pm}0.02$ and $0.58{\pm}0.03$ at 30 minutes and 60 minutes, respectively. Based on the obtained results, values of 0.72 and 0.05 may be proposed as the reasonable upper limits of RI and ${\Delta}RI$ of normal average. Acepromazine or xylazine are recommended as anesthetics for renal diseases because they have less effect on the RI. Further study using mannitol injection to increase ${\Delta}RI$ may be suggested in unilateral urinary tract obstruction since mannitol more effectively changed mean RI than furosemide.

• Childhood Kidney Diseases
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• v.2 no.1
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• pp.60-68
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• 1998

Purpose: To determine the various prenatal factors related to the prenatal hydronephrosis diagnosed on prenatal ultrasonography. We also attempted to correlate the prenatal and neonatal renal pelvic anteroposterior diameter with the outcome in infancy Methods: Between 1985 and 1997. We retrospectively reviewed 105 renal unit (75 patient) with fetal hydronphrosis persisting postnatally. Investigation consisted of renal ultrasonography, voiding cystourethrography, diurectic renogram, and DMSA scan. Results: The 75 patient assesed had the following underlying cause: UPJ(Ureteropelvic juction) obstruction(52%). multicystic dysplastic kidney(10%). UVJ (Ureterovesicai juction) obstruction (10%) and no underlying cause (25%). Of theses cases 36 cases (40 renal unit) underwent operation, while 28 cases (50 renal unit) resoled spontaneously. 12% of mild hydornephrosis deteriorated. whereas 50% of modrate hydrophrosis and 81% of severe hydronephrosis required surgical correction. Attempting to find the renal unit that were at risk for deterioration. our study showed that urinary tract infection group and calyceal blunting group had a predictive role. Conclusion: It is necessary to follow up after birth dilatation of caylx or urinary tract infection are present. Early operation is considered when prenatal pelvic AP diameter greater is than 22 mm and postnatal diameter greater than 17 mm. This may make it possible to prevent further progression of renal damage and prompt treatment of asymptomatic hydronephrosis before complications occur.

• Childhood Kidney Diseases
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• v.10 no.1
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• pp.33-39
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• 2006

Purpose : Neonatal hydronephrosis is diagnosed with an incidence of 4.5-7% of pregnancies. Recently, early detection of neonatal hydroneprosis with antenatal ultrasonography has be-come possible. But consensus about its management has not been reached, especially concerning surgical intervention. The purpose of this study is to analyze the natural course of neonatal hydronephrosis and to determine the guideline of surgical intervention and follow up study. Methods : Sixty nine hydronephrotic kidneys were confirmed from April 2001 to April 2005. All cases were rechecked by ultrasonography once at least and had a minimum follow-up of 6 months. We classified the patients into 4 groups according to the anterior posterior pelvic diameter(APPD) on perinatal ultrasonography. Ultrasonography to measure the APPD diameter and Society for Fetal Urology(SFU) grade, $^{99m}Tc-MAG3$ scan were done to a set protocol. Pyeloplasty was peformed according to the protocol. Results : Pyeloplasty was not needed in most cases where the APPD was below 10 mm and where the SFU grade were less than grade 3. Many cases with APPD 10 mm above or SFU grade III above had undergone pyeloplasty. We found a correlation between obstruction grade on MAG3 scan and whether surgery was performed or not. Conclusion : If APPD is above 10 mm, SFU grade is above grade 3 or urinary tract obstruction is suspected by MAG3 scan, pyeloplasty must be considered. In cases where APPD is below 10mm and SFU grade is less than grade 3, we can observe the natural course of neonatal hydronephrosis with consecutive follow-up. (J Korean Soc Pediatr Nephrol 2006;10:33-39)