Pleuroperitoneal communication-associated pleuritis as an uncommon cause of fever of unknown origin in a child on peritoneal dialysis: a case report

Pleuroperitoneal communication (PPC) is a rare mechanical complication of peritoneal dialysis (PD), which causes dialysate to move from the peritoneal cavity to the pleural cavity, resulting in pleural effusion. Typically, PPC is discovered through pleural effusion in PD patients who are not in volume overload status. A unique characteristic of the pleural effusion caused by PPC is that it is not resolved by increasing ultrafiltration by dialysis. In this report, we present a 7-year-old girl with PD after birth with the history of various infectious PD-related complications, presenting with fever ongoing for 6 months. PPC-associated pleuritis was suspected as the cause of fever, which eventually developed after long-term PD and induced complicated pleural effusion, lung inflammation, and prolonged fever for 6 months.

Two pediatric cases with hematuria, normal renal function and positive antineutrophil cytoplasmic antibodies

Antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis requires prompt diagnosis and treatment, since renal function at the time of diagnosis is significantly associated with renal outcomes. Here, we report two pediatric patients with ANCA-positive glomerulonephritis initially presenting with hematuria, mild proteinuria, and normal renal function. The first patient with a high myeloperoxidase-ANCA titer (>134 IU/mL) was diagnosed with rapidly progressive glomerulonephritis based on renal biopsy and treated with immunosuppressive therapy after 10 months of follow-up. The second patient with a low myeloperoxidase-ANCA titer (11 IU/mL) maintained normal kidney function without medication. Two cases showed different clinical course according to ANCA titer.

Management strategies for congenital isolated hydronephrosis and the natural course of the disease

Congenital isolated hydronephrosis encompasses a spectrum of physiologic states that spontaneously resolve and pathologic obstruction that necessitates surgical intervention. Distinguishing patients whose condition will resolve, those who will require stringent follow-up, and those who will eventually need surgical intervention present a challenge to clinicians, particularly because no unified guidelines for assessment and follow-up have been established. The recognition of the natural course and prognosis of hydronephrosis and a comprehensive understanding of the currently proposed consensus guidelines may aid in multidisciplinary treatment and in providing proper counseling to caregivers. In this review, we aimed to summarize the literature on the grading systems and management strategies for congenital isolated hydronephrosis.

A Case of Severe Hyperammonemic Encephalopathy Caused by Urinary Tract Infection in Obstructive Uropathy

Hyperammonemia is mainly caused by diseases related to liver failure. However, there are also non-hepatic causes of hyperammonemia, such as urinary tract infection (UTI) due to urease-producing organisms. Urease production by these bacteria induces a hydrolysis of urinary urea into ammonia that can cross the urothelial cell membrane and diffuse into blood vessels, leading to hyperammonemia. Delayed diagnosis and treatment of hyperammonemia can lead to lethal encephalopathy that can cause brain damage and life-threatening conditions. In the presence of obstructive uropathy, UTI by urease-producing bacteria can lead to more severe hyperammonemia due to enhanced resorption of ammonia into the systemic circulation. In this report, we present a case of acute severe hyperammonemic encephalopathy leading to brain death due to accumulation of ammonia in blood caused by Morganella morganii UTI in a 10-year-old girl with cloacal anomaly, causing obstructive uropathy even after multiple corrections.

Intellectual Functioning of Pediatric Patients with Chronic Kidney Disease:Results from the KNOW-Ped CKD

Background@#Chronic kidney disease (CKD) has a negative impact on growth and development in children and is a risk factor for neurocognitive impairment; however, there is limited research on the cognitive function of children and adolescents with CKD. This study therefore aimed to investigate the mean intelligence and risk factors for low intelligence in children and adolescents with CKD. @*Methods@#Eighty-one patients with CKD under 18 years old were included in the KoreaN cohort study for Outcomes in patients With Pediatric Chronic Kidney Disease (KNOW-Ped CKD). Participants completed either the Wechsler Intelligence Scale for Children (6–16 years), or Wechsler Adult Intelligence Scale (> 16 years). @*Results@#The mean full-scale intelligence quotient (IQ) was 91 ± 19; 24.7% of participants scored a full-scale IQ below 80. Participants with a short stature (height Z scores < −1.88), failure to thrive (weight Z scores < −1.65), more severe CKD stage (≥ IIIb), longer duration of CKD (≥ 5 years), and those who were Medicare or Medicaid beneficiaries, had significantly lower mean full-scale IQs. @*Conclusion@#On linear regression analysis, the association between the full-scale IQ, and longer duration of CKD and growth failure, remained significant after controlling for demographic and clinical variables. It is therefore necessary to investigate cognitive impairment in pediatric patients with CKD who exhibit growth failure or for a longer postmorbid period. It is believed that early interventions, such as kidney transplantation, will have a positive effect on IQ in children with CKD, as the disease negatively affects IQ due to poor glomerular filtration rate over time.

Intellectual Functioning of Pediatric Patients with Chronic Kidney Disease:Results from the KNOW-Ped CKD

Background@#Chronic kidney disease (CKD) has a negative impact on growth and development in children and is a risk factor for neurocognitive impairment; however, there is limited research on the cognitive function of children and adolescents with CKD. This study therefore aimed to investigate the mean intelligence and risk factors for low intelligence in children and adolescents with CKD. @*Methods@#Eighty-one patients with CKD under 18 years old were included in the KoreaN cohort study for Outcomes in patients With Pediatric Chronic Kidney Disease (KNOW-Ped CKD). Participants completed either the Wechsler Intelligence Scale for Children (6–16 years), or Wechsler Adult Intelligence Scale (> 16 years). @*Results@#The mean full-scale intelligence quotient (IQ) was 91 ± 19; 24.7% of participants scored a full-scale IQ below 80. Participants with a short stature (height Z scores < −1.88), failure to thrive (weight Z scores < −1.65), more severe CKD stage (≥ IIIb), longer duration of CKD (≥ 5 years), and those who were Medicare or Medicaid beneficiaries, had significantly lower mean full-scale IQs. @*Conclusion@#On linear regression analysis, the association between the full-scale IQ, and longer duration of CKD and growth failure, remained significant after controlling for demographic and clinical variables. It is therefore necessary to investigate cognitive impairment in pediatric patients with CKD who exhibit growth failure or for a longer postmorbid period. It is believed that early interventions, such as kidney transplantation, will have a positive effect on IQ in children with CKD, as the disease negatively affects IQ due to poor glomerular filtration rate over time.

Characteristics of pediatric rhabdomyolysis and the associated risk factors for acute kidney injury: a retrospective multicenter study in Korea

Background@#The clinical features of pediatric rhabdomyolysis differ from those of the adults with rhabdomyolysis; however, multicenter studies are lacking. This study aimed to investigate the characteristics of pediatric rhabdomyolysis and reveal the risk factors for acute kidney injury (AKI) in such cases. @*Methods@#This retrospective study analyzed the medical records of children and adolescents diagnosed with rhabdomyolysis at 23 hospitals in South Korea between January 2007 and December 2016. @*Results@#Among 880 patients, those aged 3 to 5 years old composed the largest subgroup (19.4%), and all age subgroups were predominantly male. The incidence of AKI was 11.3%. Neurological disorders (53%) and infection (44%) were the most common underlying disorder and cause of rhabdomyolysis, respectively. The median age at diagnosis in the AKI subgroup was older than that in the non-AKI subgroup (12.2 years vs. 8.0 years). There were no significant differences in body mass index, myalgia, dark-colored urine, or the number of causal factors between the two AKI-status subgroups. The multivariate logistic regression model indicated that the following factors were independently associated with AKI: multiorgan failure, presence of an underlying disorder, strong positive urine occult blood, increased aspartate aminotransferase and uric acid levels, and reduced calcium levels. @*Conclusions@#Our study revealed characteristic clinical and laboratory features of rhabdomyolysis in a Korean pediatric population and highlighted the risk factors for AKI in these cases. Our findings will contribute to a greater understanding of pediatric rhabdomyolysis and may enable early intervention against rhabdomyolysis-induced AKI.

Pneumocystis Pneumonia after Kidney Transplantationin Children

Pneumocystis pneumonia (PCP) is a rare disease in healthy people but a potentiallyfatal opportunistic infection by Pneumocystis jirovecii in immunocompromisedpatients with organ transplantation. We present three cases of PCP after kidneytransplantation in pediatric patients. First case was a 4-year-old boy diagnosedwith Denys-Drash syndrome and received living-donor kidney transplantationfrom his mother at age of 1. Second case was a 19-year-old male, with polycystickidney disease, who received kidney transplantation from his mother at the age of18. Third case was a 19-year-old female with chronic kidney disease of unknownetiology, who received kidney transplantation from her father at age of 15. Thesethree patients who were on immunosuppressive therapy and completed ofroutine PCP prophylaxis for 6 months had presented with cough and dyspneamore than 1 year after transplantation. Chest x-ray all showed diffuse haziness ofboth lung fields, and bronchoalveolar lavage from bronchoscopy revealed Pneumocystisjirovecii infection. All patients showed clinical resolution with intravenoustrimethoprim-sulfamethoxazole (TMP-SMX) therapy for at least 3 weeks and hadcontinued secondary prophylaxis for another 6–12 months. This report suggeststhat clinicians should have suspicion for the possibilities of opportunistic infectionsuch as PCP after kidney transplantation in children.

Primary Hyperoxaluria in Korean Pediatric Patients

BACKGROUND: Primary hyperoxaluria (PH), a rare inborn error of glyoxylate meta bolism causing overproduction of oxalate, is classified into three genetic subgroups: type 1–3 (PH1–PH3) caused by AGXT, GRHPR , and HOGA1 gene mutations, respectively. We performed a retrospective case series study of Korean pediatric patients with PH.METHODS: In total, 11 unrelated pediatric patients were recruited and their phenotypes and genotypes were analyzed by a retrospective review of their medical records.RESULTS: Mutational analyses revealed biallelic AGXT mutations (PH1) in nine patients and a single heterozygous GRHPR and HOGA1 mutation in one patient each. The c.33dupC was the most common AGXT mutation with an allelic frequency of 44%. The median age of onset was 3 months (range, 2 months-3 years), and eight patients with PH1 presented with end stage renal disease (ESRD). Patients with two truncating mutations showed an earlier age of onset and more frequent retinal involvement than patients with one truncating mutation. Among eight PH1 patients presenting with ESRD, five patients were treated with intensive dialysis followed by liver transplantation (n=5) with/without subsequent kidney transplantation (n=3).CONCLUSION: Most patients presented with severe infantile forms of PH. Patients with two truncating mutations displayed more severe phenotypes than those of patients with one truncating mutation. Sequential liver and kidney transplantation was adopted for PH1 patients presenting with ESRD. A larger nation-wide multicenter study is needed to confirm the genotype-phenotype correlations and outcomes of organ transplantation.

A Case of Infantile Nephrotic Syndrome associated with Neuroblastoma

Nephrotic syndrome in the first year of life, characterized by renal dysfunction and proteinuria, is associated with a heterogeneous group of disorders. These disorders are often related to genetic mutations, but the syndrome can also be caused by a variety of other diseases. We report an infant with nephrotic syndrome associated with a neuroblastoma. A 6-month-old girl was admitted with a 10% weight loss over 10 days and nephrotic-range proteinuria. She was ill-looking, and her blood pressure was higher than normal for her age. Her cystatin-C glomerular filtration rate was decreased, and levels of plasma renin, aldosterone, and catecholamines were elevated. Renal ultrasonography and abdominal computed tomography showed a retroperitoneal prevertebral mass encasing both renal arteries and the left renal vein. The mass was partially resected laparoscopically, and the pathologic diagnosis was neuroblastoma. Findings on a simultaneous renal biopsy were unremarkable. The patient was treated with chemotherapy and several anti-hypertensive drugs, including an alpha blocker. Two months later, the mass had decreased in size and the proteinuria and hypertension were gradually improving. In an infant with abnormal renin-angiotensin system activation, severe hypertension, and nephrotic-range proteinuria, neuroblastoma can be considered in the differential diagnosis.

Cytomegalovirus Infection in Pediatric Renal Transplant Recipients: A Single Center Experience

PURPOSE: To investigate the frequency, presentation, management, and outcome of cytomegalovirus (CMV) infection in pediatric patients who underwent renal transplantation. METHODS: We performed a retrospective chart review of 70 patients under the age of 18, who underwent renal transplantation between January 1990 and November 2014. A diagnosis of CMV infection was based on serology, molecular assays, antigenemia assays, and culture. CMV infection was defined as detection of virus and CMV disease was diagnosed when clinical signs and symptoms were present. RESULTS: The number of patients with CMV infection was 18 (25.7% of renal transplant recipients). Twelve were male (66.7%), and the mean±standard deviation (SD) age at infection was 13.3±3.9 years. Median time of infection after renal transplantation was 4 months (range 1.0-31.0 months). Pretransplantation CMV status in the infected group was as follows: donor (D)+/recipient (R)+, 11 (61.1%); D+/R-, 7 (38.9%); D-/R+, 0; and D-/R- 0. Nine patients had CMV disease with fever, leukopenia, thrombocytopenia, or organ involvement such as enteritis, hepatitis, and pneumonitis. The age of disease occurrence was 13.1±3.9 years and the median time to disease onset after renal transplantation was 8 months (range 1.0-31.0). Immunosuppressive agents were reduced or discontinued in 14 patients (77.8%), antiviral agents were used in 11 patients (61.1%), and all patients with CMV infection were controlled. CONCLUSIONS: A quarter of the patients had CMV infection about 4 months after renal transplantation. CMV infection was successfully treated with reduction of immunosuppressants or with antiviral agents.

Cytomegalovirus Infection in Pediatric Renal Transplant Recipients: A Single Center Experience

PURPOSE: To investigate the frequency, presentation, management, and outcome of cytomegalovirus (CMV) infection in pediatric patients who underwent renal transplantation. METHODS: We performed a retrospective chart review of 70 patients under the age of 18, who underwent renal transplantation between January 1990 and November 2014. A diagnosis of CMV infection was based on serology, molecular assays, antigenemia assays, and culture. CMV infection was defined as detection of virus and CMV disease was diagnosed when clinical signs and symptoms were present. RESULTS: The number of patients with CMV infection was 18 (25.7% of renal transplant recipients). Twelve were male (66.7%), and the mean±standard deviation (SD) age at infection was 13.3±3.9 years. Median time of infection after renal transplantation was 4 months (range 1.0-31.0 months). Pretransplantation CMV status in the infected group was as follows: donor (D)+/recipient (R)+, 11 (61.1%); D+/R-, 7 (38.9%); D-/R+, 0; and D-/R- 0. Nine patients had CMV disease with fever, leukopenia, thrombocytopenia, or organ involvement such as enteritis, hepatitis, and pneumonitis. The age of disease occurrence was 13.1±3.9 years and the median time to disease onset after renal transplantation was 8 months (range 1.0-31.0). Immunosuppressive agents were reduced or discontinued in 14 patients (77.8%), antiviral agents were used in 11 patients (61.1%), and all patients with CMV infection were controlled. CONCLUSIONS: A quarter of the patients had CMV infection about 4 months after renal transplantation. CMV infection was successfully treated with reduction of immunosuppressants or with antiviral agents.

Virus-associated Rhabdomyolysis in Children

PURPOSE: Virus-associated rhabdomyolysis is very rare. We report 15 patients with rhabdomyolysis caused by various viruses. METHODS: Fifteen patients who were diagnosed with rhabdomyolysis and a viral infection were included in this study. Clinical, laboratory, and radiologic findings were evaluated through retrospective chart reviews. RESULTS: Chief complaints were severe bilateral lower leg pain and leg weakness. The median age was 5.7 years. The male:female ratio was 2:5. The viral infections were caused by influenza virus B, parainfluenza virus, and rhinovirus. One patient with influenza virus B had coinfection with coronavirus. Median initial laboratory values and ranges were as follows:serum creatinine, 0.4 (0.1-0.5) mg/dL; serum aspartate transaminase, 124 (48-1,098) IU/L; serum alanine transaminase, 30 (16-1,455) IU/L; serum creatine kinase, 2,965 (672-16,594) IU; serum lactate dehydrogenase, 400 (269-7,394) IU/L; serum myoglobin, 644 (314-3,867) ng/mL; urine myoglobin, 3 (3-10,431) ng/mL. All patients recovered without complications. CONCLUSION: This is the first report of the simultaneous occurrence of rhabdomyolysis caused by various viruses. This is also the first report of rhinovirus-associated rhabdomyolysis.

Virus-associated Rhabdomyolysis in Children

PURPOSE: Virus-associated rhabdomyolysis is very rare. We report 15 patients with rhabdomyolysis caused by various viruses. METHODS: Fifteen patients who were diagnosed with rhabdomyolysis and a viral infection were included in this study. Clinical, laboratory, and radiologic findings were evaluated through retrospective chart reviews. RESULTS: Chief complaints were severe bilateral lower leg pain and leg weakness. The median age was 5.7 years. The male:female ratio was 2:5. The viral infections were caused by influenza virus B, parainfluenza virus, and rhinovirus. One patient with influenza virus B had coinfection with coronavirus. Median initial laboratory values and ranges were as follows:serum creatinine, 0.4 (0.1-0.5) mg/dL; serum aspartate transaminase, 124 (48-1,098) IU/L; serum alanine transaminase, 30 (16-1,455) IU/L; serum creatine kinase, 2,965 (672-16,594) IU; serum lactate dehydrogenase, 400 (269-7,394) IU/L; serum myoglobin, 644 (314-3,867) ng/mL; urine myoglobin, 3 (3-10,431) ng/mL. All patients recovered without complications. CONCLUSION: This is the first report of the simultaneous occurrence of rhabdomyolysis caused by various viruses. This is also the first report of rhinovirus-associated rhabdomyolysis.

Genotype and Phenotype Analysis in Pediatric Patients with Cystinuria

Cystinuria is an inherited disorder characterized by defective renal reabsorption of cystine and dibasic amino acids leading to nephrolithiasis. This study was conducted to analyze the genotypes and phenotypes of pediatric patients with cystinuria. Eight children from Seoul National University Hospital and Asan Medical Center presenting with cystinuria from January 2003 to June 2016 were retrospectively analyzed. Mutational studies were performed by direct sequencing. Two of the 8 were male and 6 were female. The median ages at onset and diagnosis were 1.5 (range, 0.3–13.6) and 2.6 (range, 0.7–16.7) years, respectively. The median followed up was 7.7 (range, 3.4–14.0) years. Mutational analyses were performed in 7 patients and revealed biallelic SLC3A1 mutations (AA genotype) in 4 patients, a single heterozygous SLC3A1 mutation (A- genotype) in 1 patient, biallelic SLC7A9 mutations (BB genotype) in 1 patient, and a single heterozygous SLC7A9 mutation (B- genotype) in 1 patient. Two of the mutations were novel. No genotype-phenotype correlations were observed, except for earlier onset age in patients with non-AA genotypes than in patients with the AA genotype. All patients suffered from recurrent attacks of symptomatic nephrolithiasis, which lead to urologic interventions. At the last follow-up, 3 patients had a mild-to-moderate degree of renal dysfunction. This is the first study of genotypic and phenotypic analyses of patients with cystinuria in Korea.

Lupus Anticoagulant-hypoprothrombinemia Syndrome as an Initial Manifestation of Systemic Lupus Erythematosus: A Case Report / 임상소아혈액종양

The lupus anticoagulant-hypoprothrombinemia syndrome, characterized by presence of lupus anticoagulant with acquired factor II deficiency, is a rare disease entity often presented with acute bleeding episodes. A 15-year-old girl was hospitalized with 3 month history of menorrhagia and easy bruising. Prothrombin time (31.3 sec, normal value: 10-13 sec) and activated partial thromboplastin time (72.5 sec, normal value: 27.5-34.7 sec) were markedly prolonged and partially corrected after mixing with normal plasma. Decreased Factor II activity (4%, normal range: 79-131%) or prolonged dilute Russell's viper venom time (89.8 sec, normal value: 25.4-34.3 sec), was consistent with lupus anticoagulant-hypoprothrombinemia syndrome. Antinuclear antibody, anti-double strand-DNA antibodies and anticardiolipin antibodies were also positive. Bleeding diathesis tends to wax and wane while 5 years of treatment with steroid combined with immunosuppressants, however, there was no more active bleeding episodes. Several years after diagnosis, myocarditis, pericarditis, seizure was occurred, fulfilled the diagnostic criteria of systemic lupus erythematosus.

Continuous Renal Replacement Therapy in the Neonatal Intensive Care Unit: A Single-Center Study

PURPOSE: Continuous renal replacement therapy (CRRT) has become an essential modality for the care of critically ill pediatric patients who require renal support. However, experience with CRRT in the neonatal population is not common in Korea. In this study, we aimed to investigate the clinical features, outcomes, and complications of CRRT in neonates in a single neonatal intensive care unit (NICU). METHODS: We reviewed the medical records of 17 neonates who underwent CRRT at a NICU of a tertiary hospital. The data included demographic characteristics, diagnosis, complications, and laboratory and CRRT parameters. RESULTS: The median age at initiation of CRRT was 6 days after birth. All patients were treated with CRRT in continuous venovenous hemodiafiltration mode, with a median treatment duration of 57 hours. The main indication for CRRT was an inborn error of metabolism (IEM), followed by congenital renal disease and multiorgan failure. In patients with an IEM, the median plasma ammonia level at the CRRT initiation was 1,232 micromol/L, and the mean duration until the ammonia level decreased to half of the peak ammonia level was 7.3+/-2.5 hours. The overall hospital mortality rate was 41.2%. The outcomes of the 10 survivors after discharge included death (n=2), loss to follow-up (n=3), and survival with developmental delay (n=4). CONCLUSION: Although CRRT was effective in lowering the plasma ammonia level of neonates with IEM, the associated mortality and morbidity were high. Hence, further studies are needed to optimize the CRRT protocol and to establish an effective patient referral system in Korea.

Lupus Anticoagulant-hypoprothrombinemia Syndrome as an Initial Manifestation of Systemic Lupus Erythematosus: A Case Report / 임상소아혈액종양

The lupus anticoagulant-hypoprothrombinemia syndrome, characterized by presence of lupus anticoagulant with acquired factor II deficiency, is a rare disease entity often presented with acute bleeding episodes. A 15-year-old girl was hospitalized with 3 month history of menorrhagia and easy bruising. Prothrombin time (31.3 sec, normal value: 10-13 sec) and activated partial thromboplastin time (72.5 sec, normal value: 27.5-34.7 sec) were markedly prolonged and partially corrected after mixing with normal plasma. Decreased Factor II activity (4%, normal range: 79-131%) or prolonged dilute Russell's viper venom time (89.8 sec, normal value: 25.4-34.3 sec), was consistent with lupus anticoagulant-hypoprothrombinemia syndrome. Antinuclear antibody, anti-double strand-DNA antibodies and anticardiolipin antibodies were also positive. Bleeding diathesis tends to wax and wane while 5 years of treatment with steroid combined with immunosuppressants, however, there was no more active bleeding episodes. Several years after diagnosis, myocarditis, pericarditis, seizure was occurred, fulfilled the diagnostic criteria of systemic lupus erythematosus.

Chronic Kidney Disease-mineral Bone Disorder and Active Vitamin D Analogs for Treating Severe Hyperparathyroidism in Children Receiving Chronic Peritoneal Dialysis

PURPOSE: The aims of this study were to assess the clinical and laboratory profiles of chronic kidney disease-mineral bone disorder (CKD-MBD) and to assess the effects of treatment of active vitamin D analogs on severe hyperparathyroidism (SHPT) in pediatric patients on chronic peritoneal dialysis. METHODS: This is a retrospective study included 53 patients who had been undergoing dialysis for more than 1 year, between January 2003 and December 2012. RESULTS: Even after treatment with phosphate binders and active vitamin D analogs, the mean+/-standard deviation of the percentage of time during peritoneal dialysis that the patients' serum concentrations of phosphorus, corrected total calcium, and parathyroid hormone (PTH) fell within the Kidney Disease Outcomes Quality Initiative recommended ranges was 25.06+/-17.47%, 53.30+/-23.03%, and 11.52+/- 9.51%, respectively. Clinical symptoms or radiological signs of CKD-MBD were observed in 10 patients (18.9%). There were significant differences in percentage of time that the serum intact PTH concentration was outside of the recommended range between patients with and without symptoms or signs of CKD-MBD (below recommended range, 11.74+/-7.37% vs. 40.77+/-25.39%, P<0.001; above the recommended range, 63.79+/-27.86% vs. 37.09+/-27.76%, P=0.022). Of the 25 patients with SHPT, high-dose alfacalcidol treatment was required in 13 patients that controlled SHPT in 7 of these patients, without marked complications. CONCLUSION: Despite our efforts to manage CKD-MBD, patients' met the recommended ranges from relevant guidelines at a low frequency. The treatment of high-dose active vitamin D analogs was required in about half of the patients with SHPT and effective in about half of them.

Renal Artery Aneurysm in a 13-year-old Child

Primary renal artery aneurysm has been estimated to account for an incidence of 0.015-1% with associated morbidities including renovascular hypertension and rupture. Renovascular hypertension associated renal artery aneurysms in children is not a common disease. In patients with complicated renal vascular disease, renal autotransplantation has been used as an alternative to percutaneous transluminal angioplasty, which may be hazardous in these situations. We report a case of a renal artery aneurysm in a 13-year-old Korean child presenting hypertension detected during school health examination. Preoperative workup demonstrated a 2.8x2.1x1.9 cm saccular aneurysm in the right renal hilum that was not amendable to endovascular repair. A surgical strategy including extracorporeal renal artery reconstruction with autotransplantation was applied in order to restore renal artery anatomy and to treat renovascular hypertension. Immediately he complained of severe right flank pain and postoperative doppler sonography revealed lack of perfusion. On the 5th day after autotransplantation, the patient underwent a transplant nephrectomy. He was well postoperatively and was found to have a normal kidney function and stable blood pressure control without antihypertensive medication. This is the first pediatric case of renal artery aneurysm in Korea who underwent extracorporeal repair followed by autotransplantation failure. More pediatric cases with renal artery aneurysm should be reported to identify therapeutic outcome and long term prognosis.