Hemolytic uremic syndrome (HUS) is one of the most common causes of acute renal failure in childhood and is primarily diagnosed in up to 4.5% of children who undergo chronic renal replacement therapy. |
Recent studies have established the association between hypotonic fluids administration and hospital-acquired hyponatremia in children. The present paper investigated the pattern of current practice in intravenous fluid prescription among Korean pediatric residents, to underscore the need for updated education. A survey-based analysis was carried out. Pediatric residents at six university hospitals in Korea completed a survey consisting of four questions. Each... |
Pseudohypoaldosteronism type 1 (PHA1) is a rare form of mineralocorticoid resistance characterized in newborns by salt wasting with dehydration, hyperkalemia and failure to thrive. This disease is heterogeneous in etiology and includes autosomal dominant PHA1 owing to mutations of the |
Bartter syndrome (BS) is a clinically and genetically heterogeneous inherited renal tube disorder characterized by renal salt wasting, hypokalemic metabolic alkalosis and normotensive hyperreninemic hyperaldosteronism. There have been several case reports of BS complicated by focal segmental glomerulosclerosis (FSGS). Here, we have reported the case of a BS patient who developed FSGS and subsequent end-stage renal disease (ESRD) and provided... |
Bartter syndrome (BS) is an autosomal recessively inherited rare renal tubular disorder characterized by hypokalemic metabolic alkalosis and hyperreninemic hyperaldosteronism with normal to low blood pressure due to a renal loss of sodium. Genetically, BS is classified into 5 subtypes according to the underlying genetic defects, and BS is clinically categorized into antenatal BS and classical BS according to onset... |
Purpose : Idiopathic nephrotic syndrome (NS) can be clinically classified as steroid-sensitive and steroid-resistant. The detailed mechanism of glucocorticoid action in NS is currently unknown. Methods : In this study, we investigated 3 known single nucleotide polymorphisms (SNPs) (ER22/23EK, N363S, and BclI) of the glucocorticoid receptor gene (the NR3C1 gene) in 190 children with NS using polymerase chain reaction-restriction fragment... |
Purpose : This study was performed to identify risk factors for urinary tract infection(UTI) by extended-spectrum β-lactamase(ESBL)-producing Escherichia coli in children. Methods : We analyzed 125 episodes of UTI which were developed in 112 children at the Seoul National University Children's Hospital from July to December 2002. Medical records of 74 episodes of UTI caused by E. coli were reviewed in... |
Purpose : To characterize the infants under 3 months of age with urinary tract infections(UTIs), and especially patients with bacteremia or meningitis Methods : Hospital records of all the infants under 3 months of age discharged from our hospital for 69 consecutive months with the diagnosis of initial episode of UTI were reviewed. UTI was defined when patients had fever with... |
Systemic capillary leak syndrome(SCLS) is a rare disorder of unknown etiology, which is characterized by recurrent attacks of hypotension, hemoconcentration, and hypoalbuminemia. Urinary or enteric loss of protein is not demonstrated. It is often associated with monoclonal gammopathy, but does not manifest multiple myeloma. Since Clarkson et al. described the first case in a 34- year-old woman, about 50 cases have been reported in... |
Ketogenic diet is a high-fat, low carbohydrate, low protein diet used in treatment of pediatric epilepsy since the 1920s. Currently it is used primarily to treat refractory childhood epilepsy. Few serious complications caused by ketogenic diet have been reported. Short-term complications include dehydration, hypoglycemia, vomiting, diarrhea, and refusal to eat. Long-term complications include kidney stones, recurrent infections, metabolic derangement, hypercholesterolemia,... |
Purpose : The pathophysiologic mechanisms and risk factors of renal functional deterioration are still controversial. We analyzed the factors associated with renal failure in children with primary vesicoureteral reflux. Methods : The medical records of 166 children who were diagnosed with primary vesicoureteral reflux from Jan. 1985 to Dec. 1996 in the Department of Pediatrics, Seoul National University Children's Hospital... |
Purpose : Wilson disease is a hereditary metabolic disorder characterized by an accumulation of copper in tissues which sometimes results in renal problems. This study was performed to evaluate the prevalences of the renal problems in Wilson disease and related risk factors. Methods : In 31 patients with Wilson disease, we looked for renal problems by history taking, physical examination... |
Oculocerebrorenal syndrome of Lowe(OCRL) is a rare X-linked disorder characterized by congenital cataract(oculo-), hypotonia, developmental delay, cognitive impairment(cerebro-), renal tubular dysfunction(renal), and growth retardation. Recently, the defective gene, OCRL-1 gene encoding [PtdIns(4,5)P2] 5-phosphatase, was cloned with mutations identified in patients. Although there have been about 200 cases of OCRL reported in English literature, only three reports have been published in... |
Purpose : This study was aimed to assess the clinical manifestations and courses of autosomal recessive polycystic kidney disease(ARPKD). Methods : The medical records of 10 children diagnosed as infantile or juvenile ARPKD at Seoul National University Children's Hospital between January, 1984 and December, 1996, were reviewed, retrospectively. Results : The average age at diagnosis was 3 8/12 years(4months-7 3/12 years)... |
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,... |
Seoul National University College of Medicine, Seoul, Korea 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... |
Purpose : Vitamin D dependent rickets(VDDR) is a rare, autosomal recessively transmitted disorder characterized by hypocalcemia, hypophosphatemia, increased alkaline phosphatase, secondary hyperparathyroidism and many other clinical features. Type Ⅰ VDDR arises from primary deficiency in the renal 1α-hydroxylase that produces 1,25(OH)2D3. So patients with type I VDDR require life long administration of vitamin D. Methods : There had been 6 children(4... |
Diffuse mesangial sclerosis(DMS) is one of the underlying pathology of congenital and infantile nephrotic syndrome. Infants with DMS develop nephrotic syndrome before 2 years of age and progress to end stage renal disease within 3 years of age. The authors experienced a case of isolated DMS in a 4-month-old male infant who had nephrotic syndrome for 1 month. The diagnosis... |
Purpose : Most neonatal abdominal masses are benign in nature, but early detection and management is important to avoid the development of complications. So, we studied the causes of neonatal abdominal masses and the efficacy of ultrasonography for early detection and diagnosis. Methods : We reviewed 36 newborn infants with abdominal masses who had been admitted to the nursery and Neonatal... |
Purpose : Medium chain acyl-CoA dehydrogenase(MCAD) deficiency is an autosomal recessive disoder of β oxidation of fatty acids and characterized by episodic hypoglycemia, vomiting, convulsion, encephalopathy, apnea, and sudden death related to fasting or infection resembling Reye syndrome or sudden infant death syndrome. In acute stage, mortality rate is very high and survivors have significant risk of developmental disability and chronic somatic illness. However,... |
Methylprednisolone(MP) is administered by means of intravenous pulse therapy especially in the patients with focal segmental glomerulosclerosis(FSGS). There have been reports of its complications in a few increasing frequency. We experienced three cases of cardiaccomplications during intravenous pulsetherapy.Therewere onecaseof2¡ÆA-V block(Mobitztype I) and two cases of bradycardia and hypotension. In the former,2¡ÆA-V block in the first case deveoloped 45 hours after... |
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... |
Purpose : Although routine urinalysis can detect leukocyturia, difficulties remain in differentiating subpopulations of leukocytes and various epithelial cells in urinary sediments. This study was performed to appreciate the value of cytodiagnostic urinalysis in the discrimination of leukocyturias of bacterial urinary tract infection (UTI) from those without UTI. Methods : Urine from 12 children with UTI (UTI group) and from 11 children without UTI (non-UTI... |
Mauriac's syndrome was described in the 1920s, as a triad of poorly controlled insulin dependent diabetes mellitus, profound growth retardation, and hepatomgaly. Following the wide availability of insulin and intensification of diabetic control, this entity has become quite rare. A 9-year-old female child was transferred to pediatric OPD of SNUCH because of hyperglycemia, short stature, and visual disturbance. Five years prior to admission, she... |
Purpose : This study was designed to examine the clinical characteristics and the effect of treatment in children with primary distal renal tubular acidosis (dRTA). Methods : Medical records of 4 children diagnosed as dRTA at Seoul National University Children's Hospital were reviewed, and the clinical manifestations, laboratory findings, diagnostic criteria and effects of treatments were investigated. Results : All cases presented with growth retardation.... |
Purpose : With development of socioeconomic status, the prevelance of adolesecnt obesity, which is a risk factor for cardiovascular disease, is increasing. Skinfold and body mass index are indirectly used measurement of obesity in clinical and epidemiologic studies. Triceps skinfold, subscapular skinfold and body mass index were measured in adolescents aged 12 to 17. Percentile values by age and sex and... |
Antiphospholipid syndrome is a thrombotic disorder and the serologic marker of the syndrome is antiphospholipid antibody(lupus anticoagulant, anticardiolpin antibody, or both). In a 13-year-old girl who presented with dyspnea, pulmonary embolism and femoral vein thrombosis were demonstrated by lung scan and abdominal ultrasonography. She had 3 out of 11 criteria for the diagnosis of SLE, such as thrombocytopenia, positive antinuclear antibody, low C3... |
Splenic infarction is a rare disease and usually detected as a complication of SBE, atrial fibrillation, hemoglobinopathy, or myeloproliperative disorders. Although thrombotic event, such as deep vein, cerebral or renal thrombosis, is a frequent complication of SLE, there have been only a couple of cases documenting splenic infarction complicated in SLE. This is, to our knowledge, the first case report... |
We report three cases of cystinuria, presenting with urinary stones. A 2-year-old girl presented with urinary difficulty, hematuria, dysuria of sudden onset, and her 7-month-old younger brother also was presented with urinary difficulty, irritability on urination & stone passage. Other 6-month-old boy was admitted due to sudden onset anuria. They had radioopague renal & ureter stones and stone analysis revealed mixed... |