Acute dural venous sinus thrombosis in a child with idiopathic steroid-dependent nephrotic syndrome: a case report

Nephrotic syndrome (NS) is a hypercoagulable state in which children are at risk of venous thromboembolism. A higher risk has been reported in children with steroid-resistant NS than in those with steroid-sensitive NS. The mortality rate of cerebral venous sinus thrombosis (CVST) is approximately 10% and generally results from cerebral herniation in the acute phase and an underlying disorder in the chronic phase. Our patient initially manifested as a child with massive proteinuria and generalized edema. He was treated with albumin replacement and diuretics, angiotensin-converting enzyme inhibitor, and deflazacort. Non-contrast computed tomography showed areas of hyperattenuation in the superior sagittal sinus when he complained of severe headache and vomiting. Subsequent magnetic resonance imaging revealed empty delta signs in the superior sagittal, lateral transverse, and sigmoid sinuses, suggesting acute CVST. Immediate anticoagulation therapy was started with unfractionated heparin, antithrombin III replacement, and continuous antiproteinuric treatment. The current report describes a life-threatening CVST in a child with steroid-dependent NS, initially diagnosed by contrast non-enhanced computed tomography and subsequently confirmed by contrast-enhanced magnetic resonance imaging, followed by magnetic resonance venography for recanalization, addressing successful treatment.

A pediatric case of idiopathic Harlequin syndrome / 소아과

Harlequin syndrome, which is a rare disorder caused by dysfunction of the autonomic system, manifests as asymmetric facial flushing and sweating in response to heat, exercise, or emotional factors. The syndrome may be primary (idiopathic) with a benign course, or can occur secondary to structural abnormalities or iatrogenic factors. The precise mechanism underlying idiopathic harlequin syndrome remains unclear. Here, we describe a case of a 6-year-old boy who reported left hemifacial flushing and sweating after exercise. He had an unremarkable birth history and no significant medical history. Complete ophthalmological and neurological examinations were performed, and no other abnormalities were identified. Magnetic resonance imaging was performed to exclude lesions of the cerebrum and cervicothoracic spinal cord, and no abnormalities were noted. His final diagnosis was classic idiopathic harlequin syndrome. Herein, we report the first pediatric case of idiopathic harlequin syndrome in Korea.

Antiallergic Effects of Trichostatin A in a Murine Model of Allergic Rhinitis

OBJECTIVES: Trichostatin A (TSA), an inhibitor of histone deacetylase, has been shown to play an important role in attenuating asthmatic inflammation. However, the effect of TSA in allergic rhinitis is not known. The aims of this study were to investigate the effect of TSA on allergic nasal inflammation and on the induction of regulatory T cells in a murine model of allergic rhinitis. METHODS: BALB/c mice were sensitized intraperitoneally with ovalbumin (OVA) and then challenged intranasally with OVA. TSA (1 mg/kg) was given to the treatment group, and multiple parameters of allergic responses were evaluated to determine the effects of TSA on allergic rhinitis. Allergic nasal symptom scores, including frequency of rubbing and sneezing, were checked. Eosinophil infiltrations were stained with Chromotrope 2R, and the expression levels of OVA-specific IgE, T-helper 1 (Th1) cytokine (interferon-gamma [IFN-gamma]), Th2 cytokines (interleukin [IL] 4 and IL-5) and Treg (Foxp3, IL-10, and transforming growth factor-beta [TGF-beta]) were measured by quantitative reverse transcription-polymerase chain reaction or enzyme-linked immunosorbent assay. RESULTS: TSA reduced the scores of allergic nasal symptoms and the amount of eosinophil infiltration into the nasal mucosa. TSA suppressed OVA-specific IgE levels and reduced expression of the IL-4 and IL-5. However, the expression of IFN-gamma was unchanged in the treatment group. The levels of Foxp3, IL-10, and TGF-beta were increased in pretreatment with TSA as compared to control group. CONCLUSION: This study shows that TSA induced antiallergic effects by decreasing eosinophilic infiltration and Th2 cytokines in a murine model of allergic rhinitis via regulation of Tregs. Thus, TSA may be considered a potentially therapeutic agent in treating allergic rhinitis.

Inhibitory Effect of Delphinidin on Extracellular Matrix Production via the MAPK/NF-kappaB Pathway in Nasal Polyp-Derived Fibroblasts

PURPOSE: Nasal polyps are associated with chronic inflammation of the mucous membranes in the nose and paranasal sinuses and involved in extracellular matrix (ECM) accumulation. Delphinidin promotes ECM degradation in hepatitis and cardiac fibrosis. The aims of this study were to examine the inhibitory effect of delphinidin on TGF-beta1-induced myofibroblast differentiation and ECM accumulation, and to determine the underlying mechanisms in nasal polyp-derived fibroblasts (NPDFs). METHODS: NPDFs were stimulated with TGF-beta1, with or without delphinidin, and the expression levels of alpha-SMA, fibronectin, and collagen type I were determined by RT-PCR, Western blot analysis, and collagen assay. The expression of alpha-SMA protein was measured by immunocytochemical staining. Mitogen-activated protein kinase and NF-kappaB activation induced by TGF-beta1 were determined by Western blot analysis. The transcriptional activity of NF-kappaB was measured by luciferase assay. RESULTS: The expression levels of alpha-SMA, fibronectin, and collagen type I increased in TGF-beta1-stimulated NPDFs. In TGF-beta1-induced NPDFs, delphinidin inhibited the expression of alpha-SMA, fibronectin, and collagen. Inhibitors of MAPK and NF-kappaB blocked the expression of alpha-SMA, fibronectin, and collagen type I. Delphinidin suppressed the activation of MAPK and NF-kappaB induced by TGF-beta1 stimulation. CONCLUSIONS: These results suggest that delphinidin may inhibit TGF-beta1-induced myofibroblast differentiation and ECM production through the MAPK/NF-kappaB signaling pathway in NPDFs.

A Nationwide Epidemiological Study of Nocturnal Enuresis in Korean Adolescents and Adults: Population Based Cross Sectional Study

We performed a nationwide epidemiological study to evaluate the prevalence and characteristics of nocturnal enuresis (NE) in Korean adolescents and adults. A questionnaire was sent via e-mail to 51,073 people aged 16-40 yr by stratified sampling according to age, sex, and region among a 200,000 internet survey panel pool. The questionnaire included following information; presence or absence of NE, frequency of NE, possible risk factors for NE, self-esteem scale score and depression score results, and measures for the treatment of NE. Among the 2,117 responders, 54 (2.6%) had NE (> or =1 enuretic episode within 6 months). Of 54 bedwetters, 9.3% wet > or =1 night per week and 20.5% wet > or =1 per month. The prevalence rates remained relatively stable with no apparent trend of reduction with age. The presence of sleep disturbance, family history, urgency, or urge incontinence increased the probability of NE episode significantly. The self-esteem score was lower (P=0.053) and the depression scale score was higher (P=0.003) in bedwetters compared with non-bedwetters. Overall 2.6% of Korean aged 16-40 yr have NE. The higher rate of urgency and urge incontinence in adolescent and adult enuretics suggests that bladder function has an important role in adolescent and adult NE.

Parent and Physician Perspectives on the Treatment of Primary Nocturnal Enuresis in Korea

PURPOSE: Parental decisions about the treatment of nocturnal enuresis (NE) are generally based on silent agreement with a physician's recommendation. However, physicians may have an insufficient understanding about parents' concerns and expectations regarding treatment. The aim of this study was to clarify the discrepancies between the perceptions of parents and physicians and to better understand the attitudes related to NE treatment. MATERIALS AND METHODS: A survey was conducted in six centers and included 105 parents and 102 physicians. Two questionnaires, one for parents and another for physicians, were prepared. Each contained items on demographic characteristics, concerns, and NE treatment preferences. The parents completed the questionnaire during their child's first clinical visit, and the physicians completed the questionnaire via e-mail or individual interviews. RESULTS: Low self-esteem was the most common concern among both parents and physicians. Parents showed a more serious concern regarding disease progression and sequelae than did physicians. In the parent group, parents of younger children were mainly concerned about growth, whereas parents of children with daytime symptoms were mainly concerned about disease progression and relationships. Treatment outcome preferences differed significantly between the two groups. Physicians preferred treatments yielding immediate results, whereas parents preferred long-term treatments that would result in low recurrence. CONCLUSIONS: This survey presents the differences between parents and physicians regarding NE concerns and therapeutic preferences. Our study may provide valuable insight for physicians regarding parental attitudes toward NE treatment.

Effect of renin inhibition on an experimental glomerulonephritis - a preliminary report / 소아과

PURPOSE: We performed this study in order to investigate the effect of direct renin inhibition on an experimental animal model with nephrotoxic serum nephritis and tried to give useful information for clinical research and renin inhibitor treatment. METHODS: Thirty BALB/c 6-week-old male mice were divided into 4 groups: control group (CO, n=5), control-treatment group with aliskiren (CT, n=5), disease group (DO, n=10), and disease treatment group with aliskiren (DT, n=10). Nephritis was induced by an intravenous injection of 0.25 mg/g weight of rabbit anti-GBM immunoglobulin G. Model 2002 Alzet mini-osmotic pumps (Durect Corp.) for aliskiren infusion were implanted into CT and DT. Each group strain was sacrificed serially one at a time on day 14. We estimated the protein-creatinine ratio in 12-hour-collected urine (UP/Cr) and measured the mesangial matrix score in the PAS-stained kidney of each strain. RESULTS: One strain at CT and DT died on day 6 and 7, respectively. Each group strain was sacrificed serially at a time on day 10 because DO were seriously ill. The UP/Cr of each group is as follows: CO, 31.24+/-6.54 mg/mg, CT, 23.38+/-13.60 mg/mg, DO, 112.72+/-10.97 mg/mg, DT 114.07+/-32.30 mg/mg. There was no significant difference between DO and DT. The mesangial matrix score of each group was CO, 0.23+/-0.10; CT, 0.13+/-0.03; DO, 1.90+/-0.48; and DT, 1.28+/-0.41, respectively, and there was a significant difference between DO and DT in the extent of mesangial matrix expansion (P=0.008). CONCLUSION: We found that renin inhibition was able to suppress the mesangial matrix expansion in experimental mice with acute nephritis, although there were no significant differences in UP/Cr.

Diagnosis and therapy for functional urinary incontinence in childhood / 소아과

Functional urinary incontinence, the absence of any neurologic or structural abnormality as a cause of urinary incontinence in children, is one of the most common clinical problems encountered in pediatric and urologic departments, and it can be socially and emotionally distressing for the affected children. The prevalence rates of functional urinary incontinence in school-aged children are not very high and differ between boys and girls. The underlying mechanisms of functional urinary incontinence are heterogenous and can be associated with the following dysfunctions of both the storage and voiding patterns of the bladder: overactive bladder, dysfunctional voiding, lazy bladder syndrome, HinmanAllen syndrome, giggle incontinence, and vaginal voiding. Treatment methods for urinary incontinence in children should be chosen according to these clinical conditions. Treatment modalities generally consist of the treatment of comorbid conditions such as urinary infection and constipation, behavior therapy to modify learned voiding patterns, and pharmacotherapy primarily with anticholinergics and alpha -adrenergic blockers. This review discusses the optimal treatment modalities, including treatment of the underlying voiding disorders, and diagnostic approaches related to functional urinary incontinence in children.

A Case of Hypophosphatemic Rickets associated with Epidermal Nevus Syndrome

Epidermal nevus syndrome is a rare disease consisting of epidermal nevus and multisystem pathologic conditions associated with anomalies in the central nervous system, bone, eye, heart, vasculature and genito-urinary system. Hypophosphatemic rickets has been rarely observed in association with this syndrome. We report a case of hypophosphatemic rickets associated with epidermal nevus syndrome with review of the literature.

Assesment of Bone Strength Using a New Quantitative Ultrasound Device in Chidren with Renal Diseases

PURPOSE: Metabolic bone diseases have been major problems in children with renal diseases and steroid treatment is the main precipitating factor reducing bone mineral density(BMD). This study was performed to assess the prevalence of osteoporosis and to evaluate the clinical factors associated with decreased BMD in children with renal diseases. METHODS: Forty-four children with renal diseases who were diagnosed at the pediatric nephrology division of Ajou University hospital since Oct. 1994 were included. Using a new quantitative ultrasound device, BMD and the prevalence of osteoporosis were evaluated. The clinical and serological data were analyzed in association with decreased BMD. RESULTS: A total of 44 patients were evaluated. The age at initial diagnosis was 6.7+/-4.2 years. At the time of evaluation, the chronological and bone age was 9.3+/-4.2 years and 8.2+/-4.6 years, respectively. The renal diseases included nephrotic syndrome 24(54.5%), Henoch Schonlein purpura nephritis 7(15.9%), IgA nephropathy 6(13.9%), reflux nephropathy(RN) 2 (4.5%), and other renal disease 5(%). The prevalence of osteoporosis was 11%. There was no difference in the clinical factors between the long-term and the short-term treated steroid groups. CONCLUSION: The prevalence of osteoporosis was 12% in 44 children with renal diseases. No significant factor was found in association with decreased BMD and there was no relationship between osteoporosis and steroid usage duration or cumulative dose. A new quantitative ultrasound, which is relatively easy to perform, especially in children, is expected to be in common use and will enable clinicians to evaluate metabolic bone disorders with ease.

Second Trial of Cyclosporin A-Induced Remission in Other Immunosuppressant Therapy-Resistant FSGS Patient

Focal segmental glomerulosclerosis(FSGS) has been detected in approximately 10% of cases of idiopathic nephrotic syndrome in children, and exhibits a poor response to initial steroid therapy, as well as a higher rate of progression to chronic renal failure and relapse after kidney transplantation. We describe a case of an eleven year-old boy with steroid-resistant FSGS who exhibited a response to a second trial of cyclosporin A(CsA) therapy. At the age of 26 months, this patient was diagnosed with steroid-resistant FSGS. For 9 years, he had undergone a gauntlet of therapies to induce remission; oral steroids, cyclophosphamide, methylprednisolone(mehylPd) pulse therapy, CsA, and ibuprofen therapy. Although these therapies failed to induce remission, the patient's renal function remained in the normal range during the nine years of treatment. At the age of ten years, the patient's proteinuria decreased, and complete remission was attained with a second administration of CsA, coupled with a low dose of oral steroids. This patient continues to receive CsA without relapse. Therefore, our major concern involves the possibility of relapse after the discontinuation of CsA therapy. Our findings in this case suggest that, in cases of refractory FSGS, if renal insufficiency does not emerge, aggressive therapy for the amelioration of proteinuria should be continuously pursued.

Acute Epididymo-orchitis in Newborn Infant

Acute scrotum in newborn infants is unusual and it is even more unusual with infectious origin. The causes of acute scrotum in newborn infants include hydrocele, testicular torsion, torsion of an appendix testis, epididymo-orchitis, meconium peritonitis, testicular tumor, scrotal hematoma and reducible or incarcerated inguinal hernia. Because of lack of typical symptoms and signs, the correct diagnosis of surgical emergencies such as testicular torsion and incarcerated inguinal hernia are often delayed. Recently, color Doppler ultrasonography and testicular scans are being used for differential diagnosis of acute scrotum, although the latter is often technically unsatisfactory due to small size of the testicles in newborn infants. We report a seven day-old male infant with acute scrotum who was diagnosed as acute epididymo-orchitis by color Doppler ultrasonography, and saved from unnecessary surgical interventions. Although color Doppler ultrasonography can not give diagnostic clue for acute scrotum, it can reduce the need for explorative surgery.

Fcalpha Receptor Gene Polymorphism and mRNA Expression in Children with IgA Nephropathy or Henoch-Scholein Nephritis / 대한신장학회잡지

BACKGROUND: The correlations among polymorphisms of the Fc alpha receptor gene (Fc alpha R), its mRNA expressions, and clinical features of IgA nephropathy (IgAN) and Henoch-Scholein nephritis (HSN) in children were analyzed. METHODS: In children with IgAN (n=26) and HSN (n=30), genotypes for -114 T/C and +6 T/C polymorphisms in the promoter region of Fc alpha R gene were determined by PCR-RFLP. And Fc alpha R mRNA expressions from the peripheral mononuclear cells (n=41) were semi-quantitatively measured by competitive RT-PCR. The clinico-pathological findings were reviewed retrospectively. RESULTS: The distributions of -114 T/C and +56 T/C genotypes were not different between total patients and controls. However, the frequency of -114 CC genotype was significantly higher in the HSN patients than in control (13.3% vs 2.0%, OR=7.54, p<0.05). The genotypes for the two polymorphisms did not correlate with either clinical presentations or disease courses. The Fc alpha R mRNA expression showed no difference between patients and controls, and did not correlate with serum IgA level, either. CONCLUSION: The -114 T/C polymorphism of the Fc alpha R gene contributes as a risk factor for the development of HSN in children. However, the genotypic differences do not affect Fc alpha R mRNA transcription and not correlate with clinical features and disease courses.

A Clinicopathological Study of Rapidly Progressive Glomerulonephritis in Children

PURPOSE: Rapidly progressive glomerulonephritis (RPGN) is a clinicopathologic entity characterized by extensive crescent formation and rapid deterioration of renal function within few months. For better understanding of its clinical course and designing better treatment strategies, a clinicopathological study of childhood RPGN was performed. METHODS: The clinical manifestations and pathological findings were reviewed retro spectively in 12 children who were diagnosed as having RPGN by clinical manifestations and renal biopsy during a period from 1991 to 2003. Several clinicopathological parameters were analyzed as prognostic factors. RESULTS: Among a total of 12 patients, 4 were male and 8 were female. The median onset age was 11.5 years(range 5.5-14.6 years), and the median period of follow-up was 25 months(range 7 months-6.6 years). According to the pathological classification, 10 patients (83%) were type II RPGN(immune-complex mediated glomerulonephritis), 2 patients were type III RPGN(pauci-immune glomerulonephritis), and none was type I RPGN(anti-glome rular basement membrane nephritis). All patients were treated with oral steroid in various combinations with methylprednisolone pulse therapy(10 patients, 83%), cyclophosphamide(8 patients, 67%), or plasmapheresis(4 patients, 33%). Clinical outcomes of 12 patients were complete remission in 1(8%), end-stage renal disease in 2(17%), chronic renal insufficiency with persistent proteinuria in 2(17%), and normal renal function with persistent proteinuria in 7(58%) at the last follow-up. Poor prognosis is associated with increased serum creatinine level, severe anemia and younger age at the time of diagnosis. CONCLUSION: Immune-complex mediated glomerulonephritis is the major cause RPGN in children and most cases showed improvement of renal function with aggressive management. For better understanding of this rare disease, a prospective multicenter study should be done.

Clinical Study of Hypophosphatemic Rickets

PURPOSE: Hypophosphatemic rickets is a hereditary disease, characterized by hypophosphatemia due to renal phosphate wasting, impaired renal production of 1,25-dihydroxyvitamin D3, rachitic bone deformities and impaired growth. The purpose of this study is to provide clinical profiles of patients with hypophosphatemic rickets in our hospital. METHODS: Between July 1983 and February 2004, 56 patients were diagnosed as having hypophosphatemic rickets. The medical records of these patients were reviewed retrospectively. Clinical manifestations, family histories, laboratory data, treatment outcomes were described. RESULTS: Fifty six patients were enrolled in this study. The average age at symptom onset and diagnosis were 20 months and 5 years respectively. Fourteen patients had family histories. The main clinical manifestations were bow legs and short stature. There was a significant negative correlation between the ages and the height z-scores at the time of diagnosis(r=-0.47, P=0.005). Initial laboratory data showed normocalcemia, hypophosphatemia, elevated serum alkaline phosphatase, decreased tubular reabsorption of phosphate and a normal range of 1,25-dihydroxyvitamin D3. Radiographic examinations of bone revealed fraying, widening and cupping of the metaphyseal ends. Treatment consisted of Joulie solution and vitamin D metabolites, and resulted in improved biochemical and radiographic findings. However, height z-scores remained essentially unchanged(P=0.224). Complications of treatment were frequently observed, including hyperparathyroidism, nephrocalcinosis, and hypercalciuria. Sixteen patients had corrective osteotomy and 4 of them underwent leg lengthening together. CONCLUSION: There was a gap of several years between the onset of symptoms and the diagnosis. Early treatment seems to be essential to growth. For the earlier treatment, the offsprings of affected parents should be followed up closely.

Characteristics and Recurrence Risk Factors of Urinary Tract Infection in Early Infancy

PURPOSE AND METHODS: Urinary tract infection(UTI) is one of the most important diseases of childhood, especially for young infants. To characterize the patients diagnosed with febrile UTI in their first 6 months of life and to explore the risk factors of recurrent UTI, a retrospective study was performed. RESULTS: Among the 90 patients studied, 77 were boys(86%). First episodes of UTI were diagnosed at the age of 2.5+/-1.4 months. These patients underwent ultrasonographic evaluation of urinary tract(n=90) and voiding cystourethrography(n=81) where 53 and 35 studies showed abnormal findings respectively, and a total of 45 cases of urinary tract anomaly including vesicoureteral reflux(VUR, n=35) were diagnosed. Normal findings on ultrasonography indicated decreased risk of VUR in boys of 1-3 months of age(n=30). 53 patients were followed up more than 6 months and 45 episodes of subsequent UTI developed in 29 patients during the first 6-month period. Patients with relapse were older than patients without relapse at the diagnosis of first UTI, but other clinical parameters including abnormal findings on the imaging studies and prophylactic antibiotics prescription were not different between the two groups. CONCLUSION: UTI in early infancy occurred mainly in male infants and half of the patients had structural anomalies. USG was of clinical value in detecting anomalies requiring surgical intervention, and to rule out high grade VUR in 1-3 months old boys. Results of the imaging study or prophylactic antibiotics could not modify the risk of recurrent UTI.

Focal Segmental Glomerulosclerosis in a Child with Prader-Willi Syndrome: A Case of Obesity-associated Focal Segmental Glomerulosclerosis

Obesity-associated focal segmental glomerulosclerosis(OB-FSGS) has been known to progress into advanced renal insufficiency, and its clinicopathological features include obesity, FSGS lesions with glomerulomegaly and, nephrotic-range proteinuria without edema. A 14- year old girl with Prader-Willi syndrome showed nephrotic-range proteinuria without hypoalbuminemia or edema. The renal biopsy revealed focal segmental glomerulosclerosis together with glomerular hypertrophy and an increased mesangial matrix. We report here a case of OB-FSGS as one of the renal problems of Prader-Willi syndrome, and we came to the conclusion that Prader-Willi syndrome is one of the possible disease entities that can lead to renal insufficiency through obesity.

A Clinicopathological Study of Idiopathic Membranous Nephropathy in Children

PURPOSE: Idiopathic Membranous Nephropathy(IMN) is a rare renal disease in children. To help better understanding of its clinical course and treatment strategies, we reviewed the clinical manifestations and pathological findings of children with IMN. METHODS: Among 58 cases with MN, from 1977 to 2003, 42(72.4%) were hepatitis B virus (HBV) associated and 16(27.6%), 6 males and 10 females, were idiopathic. All cases diagnosed after 2000 were IMN. Several clinicopathological findings(sex, onset age, proteinuria, serum albumin, cholesterol, creatinine clearance, tubulointerstitial changes, glomerular sclerosis, hypertension, renal vein thrombosis, the use of ACE inhibitor, and immunosuppressive therapy) were compared between the remission and the non-remission group of the patients with IMN. RESULTS: The median onset age was 13.4 years. Clinical manifestations were nephrotic syndrome(7 cases, 43.8%), gross hematuria(5 cases, 31.3%) and microscopic hematuria with proteinuria(3 cases, 18.8%). Hypertension, hypocalcemic tetany and renal vein thrombosis were accompanied in 2, 1 and 2 cases, respectively. In addition to the typical findings of MN, the kidney biopsies showed segmental sclerosis(5 cases, 31.3%) or global sclerosis(6 cases, 37.5 %), diffuse crescents(1 case), and mild(11 cases, 68.7%) or moderate tubulointerstitial changes(3 cases, 18.8%). Thirteen cases(86.7%) received oral steroid. Among them 2 cases received cyclophophamide and 1 received cyclosporin as well. Ten cases(62.5%) received ACE inhibitors. In the patients followed up, 7 cases(46.7%) became free from proteinuria (remission group) while 8(53.3%) presented continous proteinuria (non-remission group), two (13.3%) of which progressed to renal failure. Clinicopathological findings showed no significant differences between the two groups. CONCLUSION: With HBV vaccination, HBV associated MN decreased markedly and IMN has taken up most of MN in children. For better understanding of this rare disease, a prospective multicenter study of the clinical course and treatment strategies should be done

A Clinicopathological Study of Idiopathic Membranous Nephropathy in Children

PURPOSE: Idiopathic Membranous Nephropathy(IMN) is a rare renal disease in children. To help better understanding of its clinical course and treatment strategies, we reviewed the clinical manifestations and pathological findings of children with IMN. METHODS: Among 58 cases with MN, from 1977 to 2003, 42(72.4%) were hepatitis B virus (HBV) associated and 16(27.6%), 6 males and 10 females, were idiopathic. All cases diagnosed after 2000 were IMN. Several clinicopathological findings(sex, onset age, proteinuria, serum albumin, cholesterol, creatinine clearance, tubulointerstitial changes, glomerular sclerosis, hypertension, renal vein thrombosis, the use of ACE inhibitor, and immunosuppressive therapy) were compared between the remission and the non-remission group of the patients with IMN. RESULTS: The median onset age was 13.4 years. Clinical manifestations were nephrotic syndrome(7 cases, 43.8%), gross hematuria(5 cases, 31.3%) and microscopic hematuria with proteinuria(3 cases, 18.8%). Hypertension, hypocalcemic tetany and renal vein thrombosis were accompanied in 2, 1 and 2 cases, respectively. In addition to the typical findings of MN, the kidney biopsies showed segmental sclerosis(5 cases, 31.3%) or global sclerosis(6 cases, 37.5 %), diffuse crescents(1 case), and mild(11 cases, 68.7%) or moderate tubulointerstitial changes(3 cases, 18.8%). Thirteen cases(86.7%) received oral steroid. Among them 2 cases received cyclophophamide and 1 received cyclosporin as well. Ten cases(62.5%) received ACE inhibitors. In the patients followed up, 7 cases(46.7%) became free from proteinuria (remission group) while 8(53.3%) presented continous proteinuria (non-remission group), two (13.3%) of which progressed to renal failure. Clinicopathological findings showed no significant differences between the two groups. CONCLUSION: With HBV vaccination, HBV associated MN decreased markedly and IMN has taken up most of MN in children. For better understanding of this rare disease, a prospective multicenter study of the clinical course and treatment strategies should be done

A Case of Childhood Malignant Hyperthermia Complicated by Rhabdomyolysis

Mortality and morbidity of malignant hyperthermia has decreased markedly by the avoidance of succinylcholine, and the earlier detection and introduction of dantrolene. We report a fourteen-year-old boy who developed malignant hyperthermia during general anesthesia. He showed the earlier clinical signs, such as elevation of end-tidal CO2, tachycardia, and hypertension. After prompt administration of dantrolene, operation was continued with profopol and midazolam. Rhabdomyolysis and myoglobinuria followed, and were managed by hydration and alkalinization of urine. Azotemia did not occur, and he was discharged without any sequelae on the 10th postoperative day.