Deficiency of antidiuretic hormone: a rare cause of massive polyuria after kidney transplantation / 소아과

A 15-year-old boy, who was diagnosed with Alport syndrome and end-stage renal disease, received a renal transplant from a living-related donor. On postoperative day 1, his daily urine output was 10,000 mL despite normal graft function. His laboratory findings including urine, serum osmolality, and antidiuretic hormone levels showed signs similar to central diabetes insipidus, so he was administered desmopressin acetate nasal spray. After administering the desmopressin, urine specific gravity and osmolality increased abruptly, and daily urine output declined to the normal range. The desmopressin acetate was tapered gradually and discontinued 3 months later. Graft function was good, and urine output was maintained within the normal range without desmopressin 20 months after the transplantation. We present a case of a massive polyuria due to transient deficiency of antidiuretic hormone with the necessity of desmopressin therapy immediately after kidney transplantation in a pediatric patient.

Acute Kidney Injury after Using Contrast during Cardiac Catheterization in Children with Heart Disease

Acute kidney injury (AKI) is closely associated with the mortality of hospitalized patients and long-term development of chronic kidney disease, especially in children. The purpose of our study was to assess the evidence of contrast-induced AKI after cardiac catheterization in children with heart disease and evaluate the clinical usefulness of candidate biomarkers in AKI. A total of 26 children undergoing cardiac catheterization due to various heart diseases were selected and urine and blood samples were taken at 0 hr, 6 hr, 24 hr, and 48 hr after cardiac catheterization. Until 48 hr after cardiac catheterization, there was no significant increase in serum creatinine level in all patients. Unlike urine kidney injury molecule-1, IL-18 and neutrophil gelatinase-associated lipocalin, urine liver-type fatty acid-binding protein (L-FABP) level showed biphasic pattern and the significant difference in the levels of urine L-FABP between 24 and 48 hr. We suggest that urine L-FABP can be one of the useful biomarkers to detect subclinical AKI developed by the contrast before cardiac surgery.

Effects of Age and Sex on the Pharmacokinetics of Tacrolimus during Pediatric Kidney Transplantation: A Single Center Study

PURPOSE: The pharmacokinetics of tacrolimus, one of the most widely used immunosuppressive drugs, are known to vary by sex, age, and ethnicity during pediatric transplantation. This study assessed the pharmacokinetic characteristics and associated factors of tacrolimus in Korean children receiving a kidney transplant. METHODS: We retrospectively reviewed the pharmacokinetic data (therapeutic dose, trough level, clearance, and half-life) of 9 children who were given tacrolimus as one of their initial immunosuppressive drugs after kidney transplantation. In addition, we compared the findings to data from 10 adult kidney transplant recipients. RESULTS: The mean age of our pediatric patients was 13.9 years, and the male-to-female ratio was 4:5. The mean dose of tacrolimus was 0.19+/-0.14 mg/kg/day. The mean dose of tacrolimus for males was 0.23+/-0.12 mg/kg/day, which was significantly higher than the dose for females (0.16+/-0.14 mg/kg/day). The trough level was not significantly different between both groups. The clearance rate of tacrolimus for males was also significantly higher than females. Although the dosage of tacrolimus for patients over the age of 12 years was lower (0.18+/-0.13 vs. 0.21+/-0.16 mg/kg/day) and the trough level was higher (8.2+/-4.5 vs. 7.2+/-4.2 mg/mL) than that for patients under the age of 12 years, there was no significant difference between them. However, there were significant differences between children and adults in dose, clearance, and half-life of tacrolimus. CONCLUSION: Out study suggests that the pharmacokinetics of tacrolimus tends to vary with sex and age. Therefore, large-scale prospective studies are required to verify the proper therapeutic dosage of tacrolimus in Korean children.

Comparison of the Therapeutic Efficacy of Methylprednisolone Pulse Therapy and Oral Steroid Therapy in Children with IgA Nephropathy and HSP Nephritis Combined with Proteinuria

PURPOSE: The purpose of this study was to assess the therapeutic efficacy of methylprednisolone pulse therapy in children with IgA nephropathy and Henoch-Schonlein Purpura (HSP) nephritis combined with proteinuria. METHODS: We retrospectively reviewed the clinical records of 21 patients who were diagnosed with IgA nephropathy and HSP nephritis based on percutaneous renal biopsy. Of the 21 patients, 15 were diagnosed with IgA nephropathy and 6 were diagnosed with HSP nephritis. They had mild to severe proteinuria at the time of diagnosis or during follow-up. Group 1 (n=7) received methylprednisolone pulse therapy three times every couple of months, and Group 2 (n=14) received oral steroid therapy. The follow-up periods for Group 1 and 2 were 14.0 (9-54) months and 26.5 (14-34) months, respectively. There was no significant difference in the follow-up duration between the two groups. RESULTS: The average age at diagnosis and biopsy was lower in Group 1 compared to Group 2, but it was not significantly different. At admission, all patients in both groups had hematuria and 5 patients (71.4%) of Group 1 and 14 patients (100%) of Group 2 had proteinuria. Before treatment, there was no significant difference of spot urine protein/creatinine ratio between the two groups. During follow-up, 7 patients of Group 1 (100%) and 10 patients of Group 2 (71.4%) showed complete improvement of proteinuria and the spot urine protein/creatinine ratio in Group 1 was significantly lower than Group 2. CONCLUSION: In patients with IgA nephropathy and HSP nephritis with proteinuria, methylprednisolone pulse therapy was more effective than oral steroid therapy in the reduction of proteinuria. To investigate the effects on long-term prognosis, large-scale prospective studies are needed.

Clinical manifestations of IgA nephropathy combined with thin glomerular basement membrane nephropathy in children

BACKGROUND: Immunoglobulin A nephropathy (IgAN) and thin glomerular basement membrane nephropathy (TBMN) are the most common causes of persistent hematuria during childhood. The objective of this study is to determine the difference in clinicl features and laboratory findings between pediatric patients with IgA deposited TBMN and IgAN alone. METHODS: Between January 2000 and March 2009, 95 children were diagnosed with IgAN by renal biopsy. Clinical features and laboratory findings of patients with isolated IgAN and with IgAN plus TBMN were compared; the children diagnosed with IgAN were compared to 127 children who had been diagnosed with TBMN alone during the same period. RESULTS: There were 71 (74.7%) of a total 95 patients that were diagnosed with isolated IgAN (Group 1); in 24 (25.3%) of the 95 patients IgAN was combined with TBMN (Group 2). There was marked difference in the gender distribution between Group 2 and isolated TBMN patients. The degree of proteinuria and pathologic severity was higher in Group 1 compared with Group 2. Gross hematuria was present in both groups. There were no distinguishing features in the other laboratory parameters. CONCLUSION: Patients with both IgAN and TBMN seem to have similar clinical features to patients with isolated IgAN; however, the latter tend to have better pathologic and laboratory findings, compared to the patients with IgAN alone.

Polyclonal gammopathy related to renal bleeding in a peritoneal dialysis patient / 소아과

Polyclonal gammopathy represents the diffuse activation of B cells and is usually related to inflammation or immune-related diseases. However, the mechanisms leading to polyclonal gammopathy are essentially speculative. Generally, infectious, inflammatory, or various other reactive processes may be indicated by the presence of a broad-based peak or band in the gamma region on serum protein electrophoresis results. A 15-year-old girl, who had been receiving peritoneal dialysis, presented with polyclonal gammopathy and massive gross hematuria. Renal artery embolization was performed, after which the continuous bleeding subsided and albumin-globulin dissociation resolved. This is a rare case of polyclonal gammopathy related to renal bleeding.

A Case of Urachal Remnant Abscess with Gross Hematuria

Since urachal abnormalities are uncommon and have various clinical manifestations such as umbilical discharge, periumbilical pain, recurrent urinary tract infection and abdominal mass according to its structure, it is not easy to diagnose. We report our experience of a patient with urachal remnant abscess who presented with gross hematuria initially, and improved after the management with intravenous antibiotics and percutaneous drainage of abscess.

Clinical Usefulness of Ambulatory Blood Pressure Monitoring in Children and Adolescents

PURPOSE: With increasing prevalence of hypertension (HTN) in children and adolescent, pediatricians have become more interested in blood pressure (BP) measurements. The ambulatory blood pressure monitoring (ABPM) is known to be useful to differentiate true HTN and white coat HTN. The object of this study is to assess the clinical usefulness of ABPM in Korean children and adolescents. METHODS: A retrospective review of 51 patients in Kyungpook National University Hospital from January 2002 to February 2010 was done. All patients were 6-18 years old and underwent ABPM. We calculated the mean value of ABP, BP load, nocturnal dip and compared the results with the patients' diagnosis and characteristics. RESULTS: The mean age of the 51 patients was 17.8+/-1.8 years and 19 children were obese. 37 patients (72.5%) were truly hypertensive and 1 patient was diagnosed as masked HTN and 7 children (14%) as white coat HTN. The rest of the patients were normotensive. Among patients with white coat HTN, 5 were in a prehypertensive state. Mean systolic and diastolic BP load of patients with true HTN were significantly higher than non-hypertensive children (P<0.001). Although the nocturnal dip of all patients were below 10%, there was no statistical significance. The obese patients showed higher systolic and diastolic BP. Their systolic and diastolic BP load were significantly higher than non-obese patients (P<0.001). CONCLUSION: ABPM in children and adolescents seems to be a valuable tool in the assessment of white coat HTN and in the confirmation of true HTN. A considerable number of white coat HTN patients are revealed to be in a prehypertensive state and need close follow-up.

Correlation between Karyotype and Phenotype in Turner Syndrome

PURPOSE: In spite of the karyotype and phenotype diversity in Turner syndrome patients, there are few reports about such differences in Korea. We reviewed the data of chromosome abnormalities, clinical manifestations, and comorbidities of Turner syndrome patients in Kyungpook National University Hospital to compare them to the recent hypotheses about sex chromosome gene loci related to Turner symptoms. MATERIALS AND METHODS: We identified the cytologic findings of 92 patients with Turner syndrome and the clinical findings of 62 patients among them. RESULTS: 54.3 percent of patients had 45,X while 45.7 percent showed other karyotype combinations (45,X/46,XX, 45,X/46,XX/47,XXX, 46,X,del(Xp), 46,X,del(Xq), 45,X/46,X,del(Xq), 46,X,i(Xq), 45,X/46,X,i (Xq)). The Turner symptoms found included short neck, high arched palate, broad chest, Madelung deformity, short metacarpals, scoliosis, cubitus valgus, low hair line, webbed neck, edematous extremities, pigmented nevus, and sexual infantilism. The specific diseases associated Turner syndrome included renal abnormalities, congenital heart disease, hearing defects, diabetes mellitus, hyperlipidemia, and decreased bone density. The phenotype of the mosaicism group was milder than that of the monosomy group. In the case of 46,X,del(Xp) and 45,X/46,X,del(Xq) groups, all had skeletal abnormalities, but the 46,X,del(Xq) group had none. In the case of 46,X,del(Xp) group, all showed short statures and skeletal abnormalities, but no sexual infantilism was observed. In the case of 46,X,i(Xq) and 45,X/46,X,i(Xq) groups, they all showed delayed puberty and had primary amenorrhea. CONCLUSION: It is important to study karyotype-phenotype correlations in patients with Turner syndrome to obtain interesting information about the genotype-phenotype correlations related to the X chromosome.

The Rare Causes of Rhabdomyolysis; Parainfluenza Virus type I Infection and Hypernatremia

Rhabdomyolysis, the clinical syndrome caused by the injury to skeletal muscle resulting in the release of muscle cell contents into the systemic circulation, has been described in association with various factors. The causes include crush injury, skeletal muscle overuse, heat, drug, abuse of alcohols and metabolic disorders as well as several types of viral and bacterial infections. We report two cases of rhabdomyolysis, which were complicated by uncommon causes, parainfluenza virus type I infection and hypernatremia.

Effect of Inonotus Obliques Extracts on Proliferation and Caspase-3 Activity in Human Castro-Intestinal Cancer Cell Lines

We studied the effects of hot water extract of Inonotus obliquos mushroom on the proliferation and apoptosis of the human colon adenocarcinoma, HT-29 and the human stomach adenocarcinoma, SNU-484 cell. Cells were maintained with Dulbecco's modified Eagle medium/Ham's F-12 nutrient mixture supplemented with 10% fetal bovine serum at 37 degrees C in a humidified CO2. For the cell proliferation experiments, cells were seeded in 35 mm dishes, and were treated with the various concentrations of the extract for the different time course. Apoptosis was measured by caspase-3 activity. When we incubated HT-29 cells for 24, 48, 72, and 96 hours after treatments, the cell proliferation was more suppressed with more treatment time. In case of the human stomach cancer cell, SNU484, the extract significantly decreased the cell number. Thus, the treatment of 1.5 mg/ml extract decreased almost half of the cell number. Caspase-3 activity in HT-29 was increased by the treatment of mushroom extracts. In SNU484, caspase-3 activity tended to increase in proportion to the amounts of the extracts and the treatment of Inonotus obliquos affected the activity a lot. Therefore, Inonotus obliquos is suggested for the prevention of gastro-intestinal cancer and strongly recommended for the treatment of stomach cancer.