• Childhood Kidney Diseases
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• v.1 no.2
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• pp.155-160
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• 1997

Purpose : Tethered cord syndrome is characterized by progressive motor and sensory disturbances in lower extremities, foot deformities caused by a pathologic fixation of spinal cord resulting in excessive stretching of the spinal cord. It is also frequently associated with urological symptoms include urinary frequency, incontinence, enuresis, urgency and recurrent urinary tract infection. Because there is few report in the literature about urological manifestations of theterd cord syndrome, we conducted a retrospective study on the patients diagnosed as tethered cord syndrome to delineate the characteristics of urologic manifestations in tethered cord syndrome and to establish the policy to evaluate patients who is suspected of tethered cord syndrome. Method : A retrospective study was conducted by reviewing the medical records of nine patients who was diagnosed as tethered cord syndrome from November 1991 to July 1996 in Yeungnam University Hospital. Result : 1) The age distribution of nine patients was as follows; 5 patients were under 2 years, 1 case from 2 to 6 years and 3 cases from 6 to 10 years. 2) Of 9 patients 6 had voiding frequency, urinary incontinence, enuresis, urgency and loss of micturation sense. Radiologic urodynamic studies revealed neurogenic bladder in 5 patients, hydronephrosis and hydroureter in 3, vesicoureteral reflux in 3. 3) Of 6 patients with urological abnormal manifestations 4 underwent spinal cord surgery (detethering). In spite of surgical intervention, the urological manifestations improved in only one patient. Conclusion : Urological abnormalities were common in tethered cord syndrome. Abnormal laboratory findings including urodynamic study were found even before the onset of urological symptoms. We have to concern tethered cord syndrome as one of common causes of voiding frequency and enuresis and to evaluate urological abnormalities as thetered cord syndrome is diagnosed.

• Childhood Kidney Diseases
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• v.26 no.2
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• pp.80-85
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• 2022

Purpose: To determine the prevalence, clinical manifestations, and outcomes of renal involvements in pediatric Alagille syndrome (ALGS). Methods: A total of 21 patients diagnosed with ALGS at age under 18 years who visited Samsung Medical Center from March 1999 to March 2022 were enrolled. ALGS was diagnosed either by clinical manifestations, targeted JAG1 sequencing, and/or liver biopsy. Medical records including sex, age, renal manifestations, urinalysis, serum creatinine, JAG1 sequencing, and ultrasonography were retrospectively reviewed. Results: The male to female ratio was 9:12. The mean age of patients at confirmative diagnosis of ALGS was 18.4 months. Sanger sequencing was performed for 17 patients. Sixteen of 21 patients (76.1%) showed JAG1 mutations. Renal involvement was found in 10 patients (47.6%). The most common type of anomaly was renal dysplasia (40%). One patient having renal dysplasia was pathologically confirmed with glomerular lipid deposition. Two patients (20%) manifested nephrocalcinosis/nephrolithiasis. Among eight renal-involved patients who survived, four (50%) progressed to chronic kidney disease stage 3. Two of these chronic kidney disease patients were diagnosed with hepatorenal syndrome. The other four patients had renal functions preserved, including two without any interventions and two who underwent urological interventions. Conclusions: The current study revealed a high prevalence of renal involvement in Korean pediatric ALGS with diverse phenotypes.

• Journal of Yeungnam Medical Science
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• v.26 no.1
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• pp.56-62
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• 2009

Appendicitis is a common cause of acute abdomen in pediatrics. Periappendiceal abscesses are frequently found in the pediatric population. Acute appendicitis in children can, at times, be a difficult clinical diagnosis because of its highly variable history? and physical manifestations and its unpredictable course. Despite the uncertainty of the diagnosis, appendicitis demands prompt treatment because of the risk of perforation, which occurs in approximately one third of cases. Urological manifestations of appendicitis and appendiceal abscess can vary. Acute appendicitis presenting with ureteral stenosis and hydronephrosis is very rare. Here, we report a case of acute appendicitis with perforation and left hydronephrosis in a 3-year-old female. This case presents a 3-year-old girl with dysuria having hydronephrosis that originated from a perforated appendix.

• Pediatric Infection and Vaccine
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• v.24 no.1
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• pp.60-64
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• 2017

Kawasaki disease (KD) is a systemic vasculitis that occurs predominantly in infants and young children. The etiology of KD is unknown and coronary heart disease is a major complication of KD. Acute scrotum is a rare complication of acute KD, and not as well recognized as other manifestations of the disease. We report a 2-month-old boy with acute scrotum in the acute phase of KD. He was treated with intravenous immunoglobulin (total 2 g/kg) and aspirin (50 mg/kg/day). The treatment was effective in resolving his fever and other clinical symptoms, but 2 days after starting treatment he experienced scrotal swelling. Scrotal ultrasound and transillumination were used in the diagnosis of acute scrotum. After 2 months, a follow-up testicular ultrasound revealed a remission of the acute scrotum. Subsequently, he has been followed up for KD.