The Significance of (99m)Technetium Dimercaptosuccinic Acid(DMSA) Scan as a Substitute for Voiding Cystourethrography(VCUG) in Evaluating Children with first Febrile Urinary Tract Infection

PURPOSE: We studied the value of clinical signs, laboratory findings and (99m)technetium dimercaptosuccinic acid(DMSA) scan in predicting the presence of vesicoureteral reflux(VUR) in children with first febrile urinary tract infection(UTI). METHODS: A retrospective analysis of 84 hospitalized children with first febrile UTI was performed. They underwent DMSA scan and voiding cystourethrography(VCUG) during the acute phase, and were divided into three groups according to the results of the VCUG: absence of VUR, mild(grade I-II) and severe VUR(grade III-V). We studied the relation of VUR to age, gender, fever duration, causative organism, white blood cell count, serum C-reactive protein and result of DMSA scan. RESULTS: Among 84 patients, 6 had mild and 17 had severe VUR. Thirty-eight had abnormal DMSA scan. results Patients with VUR were older than those without VUR(P<0.01). There was a lower probability of infection with Escherichia coli in patients with severe VUR than in those with mild and absent VUR(P<0.01). An abnormal DMSA scan correlated with the presence and severity of VUR(P<0.05). Severe VUR was present in 10.9% of patients with normal DMSA scan. The sensitivity, specificity, positive and negative predictive values of the DMSA scan in predicting the presence of VUR were 69.6%, 63.9%, 42.1%, and 84.8%, respectively. CONCLUSION: An abnormal DMSA scan correlated to the presence and severity of VUR, but the sensitivity, specificity and positive predictive value of the DMSA scan were low. Therefore, patient with an abnormal DMSA scan requires a VCUG. In order to prevent missing the 10.9% of patients with severe VUR but normal DMSA scans, a VCUG should be performed in patient with normal DMSA scan.

A Case of Pseudohypoaldosteronism Type l Diagnosed after Infancy / 대한소아내분비학회지

Pseudohypoaldosteronism (PHA) type l is a rare neonatal disease characterized by salt wasting, dehydration, hyperkalemia and metabolic acidosis. It is unresponsive to mineralocorticoid treatment with elevated aldosterone concentration. The three different modes of inheritance has been described. The autosomal dominant form has a mild clinical course and gradually improves with age. In this form, resistance to aldosterone seems to be restricted to the kidney. The autosomal recessive form displays generalized aldosterone resistance including kidney, colon, lung, sweat and salivary gland system. This form is more severe and requires life-long supplement with high-dose salt. The sporadic form is mild and resembles the autosomal dominant form. In this paper, we describe a male patient diagnosed as PHA type l at the age of 19 months. He presented with recurrent vomiting, diaphoresis accompanying hyponatremia, hyperkalemia, metabolic acidosis, elevated plasma renin activity and aldosterone level. Ultimately he has improved with treatment by oral sodium bicarbonate.

Tacrolimus versus Cyclosporine Immunosuppression in Pediatric Renal Transplantation: Pharmacokinetic Consideration / 소아과

Immunosuppressive therapy in pediatric renal transplant recipients is changing consequence of the increasing number of available immunosuppressive agents. The optimal use of immunosuppressive agents requires a thorough understanding of the pharmacokinetic characteristics, but the information on the pharmacokinetic characteristics of these drugs in pediatric transplant recipients is still limited. In general, patients younger than 5 years old show higher clearance rates, therefore the need for higher dosages in younger patients seems evident. By the therapeutic drug monitoring, trough(Cmin) and peak level(Cmax) are measured and the area under the blood concentration-time curve(AUC), which is taken as being representative of total systemic exposure can be calculated. Cyclosporine A (CSA) has poor bioavailability, which contributes to high inter- and intra-patient pharmacokinetic variability. CSA concentration measured 2 hours after administration(C2) has better correlation with the AUC than Cmin and is an alternative technique that predicts the AUC. Tacrolimus(Tac) has a great deal of inter-individual variability like CSA but intra-individual variability in systemic exposure is considered to be low. Both CSA and Tac are metabolized by a cytochrome P-450 enzyme isoform(CYP3A4). We should consider changing the dosages when CSA or Tac is used in combination with the medicines that inhibit or induce the CYP3A4. In case of steroid-free immunosuppressive therapy, the blood concentration of Tac should be frequently checked and dosage adjustment may be needed.

The Basic Data Analysis of Lupus Nephritis in Children

Granular cell tumor is mostly benign and thought to be of Schwann cell origin. The head and neck, particularly tongue, breast, and upper respiratory tract are frequently involved. Recently, we have experienced a case of granular cell tumor of the right thigh in a 30-year old male, diagnosed by fine needle aspiration cytology which revealed distinct cytologic features. The smear revealed cellular aspirates with clear background. The tumor cells showed uniform small nuclei and abundant eosinophilic, granular cytoplasm with hazy cell border. Mitoses were not found.

Nutcracker Syndrome Associated with Non-glomerular Hematuria in Childhood / 대한신장학회잡지

PURPOSE: This study was designed to aid the diagnosis and to predict the outcorne by understanding the clinical course of nutcracker syndrome in childhood. METHODS: The clinical, laboratory, radiological and cystoscopic data from the medical records of eleven children who were diagnosed as nutcracker syndrome by gross hematuria and pressure gradient criteria (>3mrnHg) were studied retrospectively and analyzed. RESULTS: Sex ratio of the cases was 7:4, and the median age of onset was 12.8 (3-14.3) years. Six cases showed persistent and 5 cases manifested interrnittent, exercise induced hematuria. Left flank pain (64%), abdominal pain (18%), left varicocele (9%) were associated in some of the children, but hematuria was the only symptom in 36Yo. Left renal vein entrapment was documented in 10 cases by ultrasonography. Out of the 5 cases studied by renal Doppler ultrasonography, 4 and 5 cases showed higher (>5) mean left renal vein diameter ratio (Distal/ Aortomesenteric portion) and mean peak velocity ratio respectively. Unilateral bleeding from left ureteral orifice was documented in 7 of the 9 cases at cystoscopy. The mean pressure gradient between proximal left renal vein and inferior vena cava was 4.4+/-1.6 (3-7) mmHg. Hematuria of 25% and 57% of the cases disappeared spontaneously in 3 and 5 years after onset respectively. Proteinuria disappear- ed in 3 of the 5 initial proteinuric cases. CONCLUSION: Nutcracker syndrome must be considered in the differential diagnosis of non-glomerular, especially gross hematuria in childhood, and Doppler ultrasonography can aid diagnosis non-invasively. The renal function remained stable, but 4396 of the cases continued to show hematuria still 5 years after onset.

Long-term Follow-up Study of Children with Minimal Change Nephrotic Syndrome (MCNS)

PURPOSE: Most of childhood MCNS has a long disease course with frequent relapses. This study was designed to analyze the long-term clinical course of childhood MCNS, focusing at relapsing pattern, treatment response and complications. Mothods : The medical records of 137 children with biopsy-proven MCNS observed during 1976 ti 1996 were analyzed retrospectively. They were classified as initial responders (111 patients, 81%) and nonresponders (26 patients, 19%) according to the response to initial oral prednisolone (60mg/m2/d) for 4 weeks. The detailed clinical courses were obtained in 126 patients. RESULTS: The incidences of hematuria, hypertension and azotemia were more frequent in initial responders than nonresponders. During follow-up, the proportion of patients with sustained remission increased gradually with decreasing rate of relapse. At the last follow-up, 77 patients (61%) revealed sustained remission, 36 (29%) repeated relapses, 9 (7%) persistent proteinuria, 3 (2%) renal failure, and 1 (1%) death. The responses to secondary drugs such as first and second course of cyclophosphamide, cyclosporin, levamisole and methylprednisolone pulse were 80%, 85.7%, 70%, 75%, and 40%, respectively. Major complications were infections including peritonitis (29 patients) and acute renal failure (10) patients.CONCLUSION: Long-term prognosis of childhood MCNS is determined by clinical courses rather than renal pathology. Although majority of childhood MCNS reveal good long-term prognosis, patients did well, few patients do not so. Early detection and more aggressive therapy of such patients are very helpful.

A Case of Successful Treatment of Pulmonary Alveolar Hemorrhage with Plasmapheresis in Child with Systemic Lupus Erythematosus

Pulmonary hemorrhage is a rare but possibly fatal complication of systemic lupus erythematosus (SLE). We report a case of massive pulmonary hemorrhage in a 14-year-old boy recently diagnosed as SLE. He developed massive pulmonary hemorrhage during the courses of i.v. methylprednisolone pulse therapy, and did not respond to i.v. cyclophosphamide. However, he rapidly improved through the use of plasmapheresis. Although various factors can precipitate pulmonary hemorrhage in SLE, our case was probably caused by an immune mediated mechanism since the hemorrhage responded promptly to plasmapheresis. This case illustrates the importance of plasmapheresis in the treatment of pulmonary hemorrhage which is not improved by methylprednisolone and cyclophosphamide. We report this case with a brief review of the related literatures.

A Case of Successful Treatment of Pulmonary Alveolar Hemorrhage with Plasmapheresis in Child with Systemic Lupus Erythematosus

Pulmonary hemorrhage is a rare but possibly fatal complication of systemic lupus erythematosus (SLE). We report a case of massive pulmonary hemorrhage in a 14-year-old boy recently diagnosed as SLE. He developed massive pulmonary hemorrhage during the courses of i.v. methylprednisolone pulse therapy, and did not respond to i.v. cyclophosphamide. However, he rapidly improved through the use of plasmapheresis. Although various factors can precipitate pulmonary hemorrhage in SLE, our case was probably caused by an immune mediated mechanism since the hemorrhage responded promptly to plasmapheresis. This case illustrates the importance of plasmapheresis in the treatment of pulmonary hemorrhage which is not improved by methylprednisolone and cyclophosphamide. We report this case with a brief review of the related literatures.

Lipoprotein and Lipid Abnormalities in Uremic Children with Maintenance Dialysis

Leiomyosarcoma of the soft tissue is a well-defined and characteristic entity histologically, but cytomorphological studes are lacking. A correlaive cytological study of 2 cases of leiomyosarcoma is presented. The smears from case 1 were rich in tumor cells and most cells were arranged in large sheets or clusters. The cells showed round to oval nuclei containing fine chromatin and small promiment nucleoli. The smears from case 2 were moderate in cellularity with loose clusters or isolated cells. The characteristic blunt-ended and cigar-shaped nuclei containing coarse chromatin and prominent nucleoli were identified in case 2. Nuclear atypia, prominent nucleoli and high cellularity permit diagnosis of malignancy, although the atypia is generally less pronounced than in the histology. The cytological diagnosis of leiomyosarcoma may be auxiliary in the diagnosis of recurrence or metastasis in the patients with alleged leiomyosarcoma.

Eosinophilic Peritonitis ( EP ) Complicated with Continuous Ambulatory Peritoneal Dialysis ( CAPD )

A 70-year-old female who was diagnosed as myxoid chondrosarcoma by fine needle aspiration of a pleural mass is described. She presented with left chest discomfort of 4 months' duration and aggravating dyspnea and chest pain for 2 months. Chest X-ray and CT scan revealed a large lobulated low density mass invading chest wall at the left pleural cavity and massive pleural fluid. Fine needle aspiration was done under the impression of mesothelioma or metastatic cancer. The aspirates from the mass were very cellular and composed of isolated or clustered forms of large plump cells. Abundant cytoplasm was bluish opaque and the margin was rounded in the isolated cells, whereas clustered cells show ill-defined cell borders and aggregating tendency. The nuclei were eccentric, round to ovoid, and had fine chromatin pattern and multiple small nucleoli. Cellular pleomorphism or mitotic figure was not definite. These findings were consistent with cytologic features of chondrosarcoma. Final diagnosis was confirmed as myxoid chondrosarcoma by mediastinoscopic biopsy and the tumor showed strong positivity for S-100 protein.

A case of Primary Sclerosing Cholangitis with Ulcerative Colitis

An association between primary sclerosing cholangitis and ulcerative colitis is well known. But, primary sclerosing cholangitis with ulerative colitis has been rarely reported in children. The prevalence of primary sclerosing cholangitis among ulcerative colitis patiens is 3% in children. Primary sclerosing cholangitis is characterised by inflammation and fibrosis of the intrahepatic and extrahepatic bile ducts. The diagnosis of primary sclerosing cholangitis based on biochemical, histologic and cholangiographic criteria. A twofold or greater elevation of serum alkaline phosphatase is required to suspect this diagnosis. The definitive diagnosis of primary sclerong cholangitis can usually made by cholangiography. The prognosis varies. No adequate treatment exists although a number of potential treatments have been evaluated. We experienced a case of primary sclerosing cholangitis with ulcerative colitis in a 14 year old girl. She was admitted with a history of intermittent bloody diarrhea and jaundice over a two year period. Hepatosplenomegaly and cholestasis had been noted. Abnormal liver function tests were noted. AST was 117U/l, ALT 179U/l, alkaline phosphatase 603U/l, gamma-GT 366U/l, total bilirubin 5.5mg/dl. An endoscopic retrograde cholecystopancreatography showed evidence of strictures, beading, and irregularities of intra and extrahepatic biliary system. Liver biopsy showed histologic findings compatible with a sclerosing cholangitis and evidence of periductular fibrosis. She sufferred from persistent cholestasis and sign of portal hypertension. She had developed recurrent episodes of variceal hemorrhages which had been successfully managed several times endoscopic variceal ligations.

Clinicopathological Study about Childhood Primary Nephrotic Syndrome Resistant to 4-week Daily Steroid Therapy

PURPOSE: Steroid-resistant nephrotic syndrome in children is difficult to manage and tends to progress to chronic renal failure. We studied clinicopathological correlations in primary nephrotic syndrome in children resistant to 4-week daily steroid therapy. METHODS: Among children who had been admitted to Seoul National University Children's Hospital during the period between Oct. 1985 and Jul. 1995 and diagnosed as primary nephrotic syndrome, 87 patients were selected for this study. They showed poor response to 4-week daily steroid therapy either initially (initial nonresponder) or subsequently in the disease course (subsequent nonresponder). The medical records including renal pathologic findings were analyzed retrospectively. RESULTS: The mean age at the onset of nephrotic syndrome was 7.3+/-4.1 years and male to female ratio was 62:25. Pathologically, 28 (32%) had minimal change lesion (MCL), 47 (54%) had focal segmental glomerulosclerosis (FSGS) and 12 (14%) had others. There were 15 (54%) initial nonresponders and 13 (46%) subsequent nonresponders in the MCL group, and there were 26 (55%) and 21 (45%), respectively, in the FSGS group. The incidence of hematuria was less frequent in the MCL group. The frequencies of hypertension and azotemia were not significantly different between in the MCL and the FSGS group. Among 10 patients with MCL in whom the steroid therapy were extended to 6 weeks, 3 patients responded subsequently. And 1 of 3 patients among the FSGS group responded to 8-week daily steroid therapy. The 2nd line drug therapy such as oral cyclophosphamide, intravenous pulsed methylprednisolone, enalapril, dipyridamole, etc. was tried in 26 patients with MCL and all 47 patients with FSGS. In the MCL group, 7 of 13 initial nonresponders and 9 of 13 subsequent nonresponders responded to these 2nd line drug therapies. In the FSGS group, 10 of 26 initial nonresponders and 11 of 21 subsequent nonresponders responded to these therapies. While only 1 subsequent nonresponder in the MCL group progressed to chronic renal failure, 9 initial and 4 subsequent nonresponders progressed in the FSGS group. CONCLUSIONS: The FSGS group formed about a half and the MCL group formed about a third of steroid-resistant nephrotic syndrome in children. Although the response to 2nd line drug therapies was not different between 2 groups, the incidence of progression to chronic renal failure was significantly higher in the FSGS group.