Acute Rhabdomyolysis : Importance of MRI and Bone Scintigraphy

Rhabdomyolysis is a potentially life-threatening disease which may result from a variety of causes. We describe the features of magnetic resonance imaging(MRI) and bone scintigraphy, and their importance for diagnosis and treatment of a patient with rhabdomyolysis.

False Positive Immunoglobulin M Antibody to Cytomegalovirus in Child with Infectious Mononucleosis Caused by Epstein-Barr Virus Infection

A 16-month-old boy was admitted because of cough that had lasted for 10 days. The patient showed severe hepatomegaly incidentally, and dual positivity of Immunoglobulin (Ig) M to Epstein-Barr virus (EBV) viral capsid antigen (VCA) and cytomegalovirus (CMV). On the basis of seroconversion to Epstein-Barr nuclear antigen (EBNA) Ig G positivity and reduced CMV Ig M titer with persistently negative CMV Ig G, a definite diagnosis of EBV-induced infectious mononucleosis was established 1 year 2 month later.

Bilateral Nephromegaly as a Presenting Symptom of Acute Lymphoblastic Leukemia

Bilateral renal enlargement is a very rare manifestation as the primary presenting feature of acute lymphoblastic leukemia. We are reporting an unusual clinical picture of a 9-month-old male patient diagnosed as precusor B-cell lymphoblastic leukemia, who showed bilateral nephromegaly without any hepatosplenomegaly at the time of initial presentation.

Continuous Renal Replacement Therapy in Children

Continuous renal replacement therapy (CRRT) has been used in critically ill children for more than 20 years in the world, but in Korea, although it is becoming the preferred method of acute therapy in pediatric intensive care units, there has been a few experiences and no consensus of CRRT up to now. This review describes the basic technique of CRRT, highlights the between the adult and pediatric prescription, and elaborates on the main controversies in the application of CRRT in children.

Vesicoureteral Reflux and Urinary Tract Infection in Children with Congenital Hydronephrosis

PURPOSE: We checked voiding cystourethrography (VCUG) in patients with congenital hydronephrosis (CHN) and followed up the occurrence of urinary tract infection(UTI) in these patients. And we tried to figure out the relationship between CHN and VUR and UTI. METHODS: We performed a retrospective study in 122 CHN patients who received VCUG from 2002 to 2008 at Ajou University Hospital. We executed logistic regression, chi-square test and T-test using version 16.0 SPSS package for the statistical analysis. P<0.05 was regarded as significant. RESULTS: Most patients with CHN were male (73%) and there was a tendency of preferences for unilateral (77%) and left (84%) occurrence of hydronephrosis. The incidence of VUR was 18.9 % among 122 CHN patients and no laterality difference was found to the severity of VUR or hydronephrosis. The incidences of VUR and UTI was higher in group of higher grade (III-IV) CHN patients, when compared to the lower group, respectively. When VUR was present in CHN patients, the incidence of UTI during infancy was also higher. CONCLUSION: The incidence of VUR was higher in patients with higher grades of CHN. The incidence of UTI during infancy was higher when VUR was present in the CHN patients.

Successful Treatment of Hypercalcemia During Continuous Renal Replacement Therapy in Patient with Rhabdomyolysis Following Cardioversion and Cardiopulmonary Resuscitation / 대한신장학회지

Rhabdomyolysis is a common clinical and laboratory syndrome resulting from reversible skeletal muscle injury, with release of muscle cell contents into the plasma. Cardioversion, and cardiopulmonary resuscitation may produce rhabdomyolysis and myoglobinuria. We report a 5-year-old boy surviving after cardiopulmonary resuscitation and repeated 5 times of cardioversion. He showed elevated serum BUN and creatinine levels, requiring hemodialysis treatment. We had tried 5 times of intermittent hemodialysis, but oliguria was continued and there was no change of serum BUN and creatinine. His urine output was less than 100 cc per day and he showed severe edema and weight gain of 7 kg, and so we started the continuous renal replacement therapy (Prismaflex(R), gambro). After 12 days of continuous venovenous hemodiafiltration (CVVHDF), his urine output recovered and his BUN, creatinine, liver enzyme, creatine kinase, and lactate dehydrogenase levels returned to normal. During the treatment of CVVHDF, he had shown persistent hypercalcemia, and so we changed dialysate and replacement solution from hemosol B0 to calcium free solution. The hypercalcemia was controlled successfully using this calcium free pharmacy-made bicarbonate solution.

A case of newly developed pulmonary lesion during the antitubercular agents in tuberculous pleurisy : A paradoxical response / 소아과

Paradoxical response refers to the enlargement of old lesions or unexpected appearance of new lesions after initial improvement following treatment with antitubercular agents. Various types of paradoxical responses have been reported in the world, but they are rarely reported in Korean children. We report the case of a 17-year-old boy who was diagnosed with tuberculous pleurisy and was treated appropriately. Although the tuberculous pleurisy initially responded to medication with resolution of the pleural fluid, a new pulmonary lesion subsequently developed 3 weeks after the initiation of treatment that eventually cleared with continuation of the original drug regimen.

Caroli's Syndrome with Autosomal Recessive Polycystic Kidney Disease in a Two Month Old Infant

Caroli's syndrome is a rare congenital disorder that involves intrahepatic bile duct ectasia and congenital hepatic fibrosis, frequently seen with concomitant autosomal recessive polycystic kidney disease (ARPKD). Literature on infants with ARPKD is rare. Here, we present a case of a two month old boy who was diagnosed with Caroli's syndrome and ARPKD.

Distal Renal Tubular Acidosis Complicated with Periodic Hypokalemic Paralysis

A 5-year-old girl was admitted because of an acute onset of weakness in her extremities. She had experienced a similar episode before but had recovered spontaneously. She had previously been diagnosed with distal renal tubular acidosis(RTA) at the age of 2 months. During the period of acute paralysis, her serum potassium level was 1.8 mmol/L and the muscle enzymes were markedly raised suggesting massive rhabdomyolysis. Although hypokalemia is common in renal tubular acidosis, acute paralytic presentation is uncommon and is rarely described in children. We report a case of distal RTA complicated with hypokalemic paralysis with a brief review of related literatures.

A Case of Gitelman Syndrome Presented with Epileptic Seizure

Both Gitelman syndrome and Bartter syndrome are autosomal recessively inherited renal tubular disorders characterized by hypokalemic metabolic alkalosis, salt wasting and normal to low blood pressure. Gitelman syndrome is caused by mutations in the thiazide-sensitive Na- Cl cotransporter (NCCT) and distinguished from Bartter syndrome, which is associated with mutations of several genes, by the presence of hypomagnesemia and hypocalciuria. In most of the patients with Gitelman syndrome, the disease manifests with transient episodes of muscular weakness and tetany in the adult period, but, often, is asymptomatic. We report here an 11 years-old female with Gitelman syndrome who presented with aggravation of epileptic seizure. The diagnostic work-up showed typical clinical features of metabolic alkalosis, hypokalemia, hypomagnesemia and hypocalciuria. We also identified a heterozygote mutation(642CGC(Arg)>TGC(Cys)) and an abnormal splicing in the SLC12A3 gene encoding NCCT.

A Systemic Lupus Erythematosus Patient with Protein Losing Enteropathy

Protein-losing enteropathy (PLE) is a rare manifestation of systemic lupus erythematosus (SLE), and it is an uncommon cause of hypoalbuminemia without proteinuria. We describe a case of an 11 year-old girl who had diarrhea and periorbital edema as the initial symptoms of SLE. PLE was diagnosed by 24-hour stool alpha-1 antitrypsin clearance and 99mTc-human serum albumin scintigraphy.

A Case of Lesch-Nyhan Syndrome

Lesch-Nyhan syndrome is an X-linked recessive disorder characterized by hyperuricemia, choreoathetosis, spasticity, mental retardation, and compulsive, self-injurious behavior. This disorder results from a complete deficiency of the purine salvage enzyme, hypoxanthine-guanine phosphoribosyl transferase(HPRT). We report here on a case of Lesch-Nyhan syndrome in a 1-year, 7-month-old male who presented with frequent vomiting, failure to thrive, and developmental delay. The diagnostic work-up revealed hyperuricemia, hyperuricosuria, and medullary nephrolithiasis. The HPRT activity in the erythrocytes was undetectable with a biochemical assay. We also identified de novo mutation which was a deletion of the 649th base, adenosine, in HPRT gene(649delA) by analysis of cDNA using RT-PCR technique coupled with direct sequencing.

A Clinicopathological Study on the Prognosis of IgA Nephropathy in Children

PURPOSE: This study was performed to determine the natural history of histologically confirmed IgA nephropathy in pediatric patients who presented with hematuria and proteinuria. PATIENTS AND METHODS: We reviewed the clinical course of 57 patients diagnosed with IgA nephropathy at the age of 15 years or younger from 1981 to 2000. All patients presented with hematuria or minimal proteinuria( or =40 mg/m2/day), hypertension, and chronic renal failure. RESULTS: The mean age at presentation was 9.5+/-2.8 years(4 to 15 years) and 42(74%) were male. Isolated gross hematuria was observed in 20 patients(35%), microscopic hematuria in 3(5%), minimal proteinuria in 4(7%), both gross hematuria and minimal proteinuria in 15(26 %), and both microscopic hematuria and minimal proteinuria in 15(26%). During a median follow-up of 7.0+/-3.5 years, 38(67%) had complete resolution of hematuria and proteinuria, 12(21%) had persistently abnormal urinalysis without development of adverse events. Only 7(12%) developed adverse events:4(7%) developed severe proteinuria, 1(2%) became hypertensive, and 2(3%) developed impaired renal function. By univariate analysis using the chi-square test, the age at presentation(>10 years)(P<0.01) and poor histological classes of the Lee or Haas classification at onset(P<0.05) were significantly correlated with adverse events, whereas sex and clinical signs at onset were less concordant. CONCLUSION: We can conclude that the prognosis of IgA nephropathy diagnosed in early childhood is better and a good correlation exists between the clinical manifestations of this disease and the histological classes.

Correlation between Clinicomorphologic Findings and Clinical Outcome in Childhood Henoch-Schonlein Purpura Nephritis

PURPOSE: Henoch-Schonlein purpura(HSP) is usually a self-limited disease with a good eventual outcome. The prognosis of HSP is mainly determined by the renal involvement. In this study, We evaluated children with biopsy-proven Henoch-Schonlein purpura nephritis about the clinical outcome correlated with renal manifestation and morphologic findings. METHODS: The clinical features, initial laboratory and pathologic findings, and clinical outcome were evaluated in 60 children with biopsy-proven Henoch-Schonlein purpura nephritis at Yonsei University Severance Hospital during the period from Jan. 1990 to Dec. 2002. RESULTS: The ratio of male to female patients was 1.2:1. The interval between the onset of Henoch-Schonlein purpura and renal manifestation was less than 3 months in 81% of the patients. Initial renal manifestation was microscopic hematuria in 100% of patients, isolated hematuria in 15%, acute nephritic syndrome in 7%, nephrotic syndrome in 22% of patients. Renal manifestation correlated with clinical outcome. Grade II and III were the most common in histologic grades of ISKDC. Renal pathologic finding correlated with clinical outcome. CONCLUSION: Renal manifestation and pathologic findings correlated with the clinical outcome. It is necessary to evaluate the correlation between pathologic findings and treatment.

A Case of Acute Acalculous Cholecystitis Superimposed on the Nephrotic Syndrome

The nephrotic syndrome is characterized by generalized edema, hypoproteinemia(40 mg/m2/hr), and hypercholesterolemia(>200 mg/dL). It is reported that hypoalbuminemia, which is one of the four diagnostic criteria of the nephrotic syndrome, is associated with gallbladder wall thickening. An explanation for the thickened wall in hypoalbuminemic states is the accumulation of fluid in the subserosal layer of the gallbladder wall which contains the most areolar tissue. This report describes a patient who was initially diagnosed with the nephrotic syndrome at the age of 4 and subsequently developed acute acalculous cholecystitis at the age of 5.8 with an albumin level of 1.3 g/dL. The patient responded to fluid therapy, nasogastric suction, and broad spectrum antibiotics.

Clinical Comparison of Henoch-Schonlein Purpura Nephritis in Children and Adults

PURPOSE: Henoch-Shonlein purpura(HSP) is a systemic vasculitis that involves multiple organs, especially the kidney, which is the most important organ in determining the prognosis of the disease. The morbidity of HSP nephritis in adults is low and there have been little research done on its clinical course so far. Therefore, we have compared the clinical course of HSP nephritis in children and adults in Korea. METHODS: We retrospectively analyzed 81 cases of HSP nephritis in children younger than 15 years of age, and 25 cases of adults older than 15 years of age who were admitted to Yonsei University Medical College Severance Hospital from Jan. 1986 to May 2003. RESULTS: The male to female ratio was 1.5:1 in children and 1.3:1 in adults. The incidence of HSP nephritis for both age groups was found to be increased during the autumn and winter. Infection was the predisposing factor in 39 cases(48.1%) of children, 16 cases(64.0%) of adults, and drugs were the predisposing factor in 8 cases(9.9%) of children and 4 cases (16.0%) of adults. All patients initially presented with microscopic hematuria. Thirteen cases (16.0%) of children and 7 cases(28.0%) of adults initially showed proteinuria of nephrotic range. Thirty four cases(42.0%) of children and 4 cases(16.0%) of adults showed normal urinalysis after treatment. Asymptomatic urinary abnormalities were found in 41 cases(50.6%) of children and 18 cases(72.0%) of adults. Complications such as nephrotic syndrome and hypertension were found in 3 cases(3.7%) of children and 2 cases(8.0%) of adults. Three children(3.7%) and 1(4.0%) adult required dialysis or renal transplantation. Follow-up renal biopsies were performed on 21 children, of whom 10 cases(47.6%) did not show any histologic change, 9 cases(42.9%) showed low grade changes, and 2 cases(9.5%) showed high grade changes. Prognosis was gloomy when proteinuria of nephrotic range and high grade of abnormal histology were present at diagnosis, and there was no significant difference between the two groups(P<0.05) CONCLUSION: This study showed that there was no difference in terms of the clinical features and courses between the children and adults with HSP nephritis. Proteinuria of nephrotic range and the severity of abnormal histologic changes at diagnosis were found to be associated with a bad prognosis, therefore we recommend that patients with these features require long term follow-up and management.

Clinical Comparison of Henoch-Schonlein Purpura Nephritis in Children and Adults

PURPOSE: Henoch-Shonlein purpura(HSP) is a systemic vasculitis that involves multiple organs, especially the kidney, which is the most important organ in determining the prognosis of the disease. The morbidity of HSP nephritis in adults is low and there have been little research done on its clinical course so far. Therefore, we have compared the clinical course of HSP nephritis in children and adults in Korea. METHODS: We retrospectively analyzed 81 cases of HSP nephritis in children younger than 15 years of age, and 25 cases of adults older than 15 years of age who were admitted to Yonsei University Medical College Severance Hospital from Jan. 1986 to May 2003. RESULTS: The male to female ratio was 1.5:1 in children and 1.3:1 in adults. The incidence of HSP nephritis for both age groups was found to be increased during the autumn and winter. Infection was the predisposing factor in 39 cases(48.1%) of children, 16 cases(64.0%) of adults, and drugs were the predisposing factor in 8 cases(9.9%) of children and 4 cases (16.0%) of adults. All patients initially presented with microscopic hematuria. Thirteen cases (16.0%) of children and 7 cases(28.0%) of adults initially showed proteinuria of nephrotic range. Thirty four cases(42.0%) of children and 4 cases(16.0%) of adults showed normal urinalysis after treatment. Asymptomatic urinary abnormalities were found in 41 cases(50.6%) of children and 18 cases(72.0%) of adults. Complications such as nephrotic syndrome and hypertension were found in 3 cases(3.7%) of children and 2 cases(8.0%) of adults. Three children(3.7%) and 1(4.0%) adult required dialysis or renal transplantation. Follow-up renal biopsies were performed on 21 children, of whom 10 cases(47.6%) did not show any histologic change, 9 cases(42.9%) showed low grade changes, and 2 cases(9.5%) showed high grade changes. Prognosis was gloomy when proteinuria of nephrotic range and high grade of abnormal histology were present at diagnosis, and there was no significant difference between the two groups(P<0.05) CONCLUSION: This study showed that there was no difference in terms of the clinical features and courses between the children and adults with HSP nephritis. Proteinuria of nephrotic range and the severity of abnormal histologic changes at diagnosis were found to be associated with a bad prognosis, therefore we recommend that patients with these features require long term follow-up and management.

Clinical Manifestations of Ureteral Duplication in Children

PURPOSE: Ureteral duplication is a common urologic anomaly and early diagnosis may prevent irreversible renal damage. We thus aimed to evaluate the benefits of prenatal sonography in early detection. METHODS: We retrospectively studied 55 children with duplicated systems who were admitted to Severance Hospital, Yonsei University, Seoul, Korea from January 1998 to July 2003. RESULTS: The mean age at diagnosis was 2.3 years old. 89% of the children had complete duplicated ureter. 47% of the children visited the hospital with an initial complaint of abnormal prenatal sonographic findings. Among these patients, 70.8% had DMSA defects. The mean differential renal function(DRF) in the DMSA scan was 48.2% in the group diagnosed prenatally. In the postnatally-diagnosed group, DMSA defects were found in 67.7% patients and the mean DRF was 45.5%. CONCLUSION: In comparison with the past studies, the mean age at diagnosis is becoming younger, and the proportion of abnormal prenatal sonography as an initial complaint larger. Other clinical manifestations were similar. The difference of the renal damage between the prenatally diagnosed group and the postnatally diagnosed group was not statistically significant. A multi-center study may help to prove the importance of prenatal sonography in early diagnosis and treatment of ureteral duplication.

Clinical Manifestations of Ureteral Duplication in Children

PURPOSE: Ureteral duplication is a common urologic anomaly and early diagnosis may prevent irreversible renal damage. We thus aimed to evaluate the benefits of prenatal sonography in early detection. METHODS: We retrospectively studied 55 children with duplicated systems who were admitted to Severance Hospital, Yonsei University, Seoul, Korea from January 1998 to July 2003. RESULTS: The mean age at diagnosis was 2.3 years old. 89% of the children had complete duplicated ureter. 47% of the children visited the hospital with an initial complaint of abnormal prenatal sonographic findings. Among these patients, 70.8% had DMSA defects. The mean differential renal function(DRF) in the DMSA scan was 48.2% in the group diagnosed prenatally. In the postnatally-diagnosed group, DMSA defects were found in 67.7% patients and the mean DRF was 45.5%. CONCLUSION: In comparison with the past studies, the mean age at diagnosis is becoming younger, and the proportion of abnormal prenatal sonography as an initial complaint larger. Other clinical manifestations were similar. The difference of the renal damage between the prenatally diagnosed group and the postnatally diagnosed group was not statistically significant. A multi-center study may help to prove the importance of prenatal sonography in early diagnosis and treatment of ureteral duplication.

Two Cases of Pancreatitis in Children with Hemolytic Uremic Syndrome

An epidemic outbreak of food poisoning due to enterohemorrhagic Escherichia coli(EHEC) occurred throughout Korea from May to September, 2003. Patients infected via contaminated school lunch foods were found mainly in Seoul and Gyeonggi province. As a result of this food poisoning, 36 patients were diagnosed as hemolytic uremic syndrome(HUS) up to mid- September, 2003 and five of them visited our hospital. Before the outbreak this year, we had experienced only 23 cases of HUS, including diarrhea associated (D+) and atypical (D-) forms, for the last 14 years, and there had been no case with pancreatic enzyme elevation. Unlike the past HUS cases, those of this year presented with severe abdominal pain and spiking elevation of pancreatic enzyme levels in 2 cases. We report 2 cases of transient pancreatitis combined with HUS in children during the epidemic outbreak in 2003 with a brief review of related literatures.