Background: The clinical features of pediatric rhabdomyolysis differ from those of the adults with rhabdomyolysis; however, multicenter studies are lacking. This study aimed to investigate the characteristics of pediatric rhabdomyolysis and reveal the risk factors for acute kidney injury (AKI) in such cases. Methods: This retrospective study analyzed the medical records of children and adolescents diagnosed with rhabdomyolysis at 23 hospitals in South Korea between January 2007 and December 2016. Results: Among 880 patients, those aged 3 to 5 years old composed the largest subgroup (19.4%), and all age subgroups were predominantly male. The incidence of AKI was 11.3%. Neurological disorders (53%) and infection (44%) were the most common underlying disorder and cause of rhabdomyolysis, respectively. The median age at diagnosis in the AKI subgroup was older than that in the non-AKI subgroup (12.2 years vs. 8.0 years). There were no significant differences in body mass index, myalgia, dark-colored urine, or the number of causal factors between the two AKI-status subgroups. The multivariate logistic regression model indicated that the following factors were independently associated with AKI: multiorgan failure, presence of an underlying disorder, strong positive urine occult blood, increased aspartate aminotransferase and uric acid levels, and reduced calcium levels. Conclusions Our study revealed characteristic clinical and laboratory features of rhabdomyolysis in a Korean pediatric population and highlighted the risk factors for AKI in these cases. Our findings will contribute to a greater understanding of pediatric rhabdomyolysis and may enable early intervention against rhabdomyolysis-induced AKI.

PURPOSE: To investigate differences in clinical features, blood/urinary findings, and prognosis in different age groups of patients with Henoch-Schönlein purpura (HSP). METHODS: A total of 469 patients with HSP were analyzed retrospectively from June 2003 to February 2016. We classified patients into child or adult groups based on their age. RESULTS: The adult group had more patients with anemia (child vs. adult; 7.5% vs. 16.4%), and higher immunoglobulin A (IgA) (30.0% vs. 50.0%) levels, C-reactive protein (34.2% vs. 54.0%) and uric acid (3.1% vs. 12.1%) levels than the child group. The child group was highly positive for Mycoplasma pneumoniae immunoglobulin M (IgM) (34.4%). More patients in the child group presented with high levels of antistreptolysin O (24.7% vs. 2.9%) and high C4 (11.5% vs. 4.2%). Low C3 (1.1% vs. 10.2%) levels, and renal involvement with gross hematuria (8.6% vs. 21.5%), nonnephrotic proteinuria (1.1% vs. 11.2%), and nephrotic syndrome (1.1% vs. 6.0%) were common in the adult group. Adults also had poorer renal outcomes [persistent hematuria/proteinuria (10.5% vs. 32.8%), and chronic kidney disease (0% vs. 11.2%)] than the child group. Risk factors for renal involvement such as older age and higher level of uric acid were only found in the child group. The risk factors for poor renal outcome were nephrotic syndrome in the child group and gross hematuria in the adult group. CONCLUSION: In this study, child and adult groups presented with different clinical manifestations of HSP. We found that risk factors for renal involvement included age and high uric acid level in the child group. Moreover, nephrotic syndrome in the child group and gross hematuria in the adult group increased the risk of poor renal outcome.
Adult* ; Anemia ; Antistreptolysin ; C-Reactive Protein ; Child* ; Hematuria ; Humans ; Immunoglobulin A ; Immunoglobulin M ; Mycoplasma pneumoniae ; Nephrotic Syndrome ; Pneumonia, Mycoplasma ; Prognosis* ; Proteinuria ; Purpura* ; Renal Insufficiency, Chronic ; Retrospective Studies* ; Risk Factors ; Uric Acid

PURPOSE: To investigate differences in clinical features, blood/urinary findings, and prognosis in different age groups of patients with Henoch-Schönlein purpura (HSP). METHODS: A total of 469 patients with HSP were analyzed retrospectively from June 2003 to February 2016. We classified patients into child or adult groups based on their age. RESULTS: The adult group had more patients with anemia (child vs. adult; 7.5% vs. 16.4%), and higher immunoglobulin A (IgA) (30.0% vs. 50.0%) levels, C-reactive protein (34.2% vs. 54.0%) and uric acid (3.1% vs. 12.1%) levels than the child group. The child group was highly positive for Mycoplasma pneumoniae immunoglobulin M (IgM) (34.4%). More patients in the child group presented with high levels of antistreptolysin O (24.7% vs. 2.9%) and high C4 (11.5% vs. 4.2%). Low C3 (1.1% vs. 10.2%) levels, and renal involvement with gross hematuria (8.6% vs. 21.5%), nonnephrotic proteinuria (1.1% vs. 11.2%), and nephrotic syndrome (1.1% vs. 6.0%) were common in the adult group. Adults also had poorer renal outcomes [persistent hematuria/proteinuria (10.5% vs. 32.8%), and chronic kidney disease (0% vs. 11.2%)] than the child group. Risk factors for renal involvement such as older age and higher level of uric acid were only found in the child group. The risk factors for poor renal outcome were nephrotic syndrome in the child group and gross hematuria in the adult group. CONCLUSION: In this study, child and adult groups presented with different clinical manifestations of HSP. We found that risk factors for renal involvement included age and high uric acid level in the child group. Moreover, nephrotic syndrome in the child group and gross hematuria in the adult group increased the risk of poor renal outcome.
Adult* ; Anemia ; Antistreptolysin ; C-Reactive Protein ; Child* ; Hematuria ; Humans ; Immunoglobulin A ; Immunoglobulin M ; Mycoplasma pneumoniae ; Nephrotic Syndrome ; Pneumonia, Mycoplasma ; Prognosis* ; Proteinuria ; Purpura* ; Renal Insufficiency, Chronic ; Retrospective Studies* ; Risk Factors ; Uric Acid

Internal hernia is an uncommon cause of intestinal obstruction, accounting for 1%. The most common cause of internal hernia is left paraduodenal hernia (PDH), which causes acute or recurrent abdominal pain. Due to its nonspecific symptoms, diagnosis of PDH by physical examination is difficult. PDH in children is rarely reported. A 3-year-old-child presented with acute abdominal pain associated with multiple episodes of vomiting. He came to the emergency room three times with abdominal pain. Left PDH was identified by computed tomography. He was taken to the operating room. However cardiac arrest occurred during anesthesia, and he expired. Due to the rarity of PDH in children, it is difficult to consider it as a cause of recurrent abdominal pain. Therefore, considering the possibility of PDH in children with acute or recurrent abdominal pain is recommended.
Abdomen, Acute ; Abdominal Pain ; Anesthesia ; Child ; Diagnosis ; Emergency Service, Hospital ; Heart Arrest ; Hernia* ; Hernia, Abdominal ; Humans ; Intestinal Obstruction ; Operating Rooms ; Physical Examination ; Vomiting

Nephrogenic diabetes insipidus (NDI) can cause nonobstructive hydronephrosis. Congenital NDI (CNDI) is caused by a genetic mutation. This case report presents a 12-year-old girl who was incidentally diagnosed with nonobstructive hydronephrosis due to NDI caused by AQP2 gene mutation after being evaluated for microscopic hematuria found on routine health examination at school. The patient's medical and family history was unremarkable, and she complained of nocturia only at the time of the clinic visit. Bilateral hydronephrosis on abdominal ultrasonography prompted a water deprivation test, leading to diagnosis of NDI. Genetic study confirmed p.Asn (AAC)123Ser (AGC) in exon 2 of the AQP2 gene. Polyuria and hydronephrosis improved following arginine-vasopressin therapy. CNDI responsive to treatment should be considered as a possible cause of nonobstructive hydroureter.
Ambulatory Care ; Child ; Diabetes Insipidus, Nephrogenic* ; Diagnosis ; Exons ; Female ; Hematuria ; Humans ; Hydronephrosis* ; Nocturia ; Polyuria ; Ultrasonography ; Water Deprivation

BACKGROUND AND OBJECTIVES: Interleukin-21 receptor (IL-21R) gene polymorphism is related with the development of systemic vasculitis. In this study, we investigated the polymorphisms of IL-21R gene in patients with Kawasaki disease (KD). SUBJECTS AND METHODS: We genotyped the promoter region of IL-21R gene (-2500 bp to +1 bp) in 100 patients with KD and 100 healthy controls. All study subjects were Korean. We designed five pairs of primers and performed polymerase chain reaction (PCR) and direct sequencing. We analyzed whole promoter sequences of 200 individuals with comparison to reference sequences of IL-21R gene (NG_012222.1/NC_000016.9). RESULTS: We found five single nucleotide polymorphisms (SNPs) of which minor allele frequency (MAF) >0.01 in the promoter region of IL-21R gene. Those are -1681 G>T (chromosome site 27411802), -379 G>A (27413104), -332 G>C (27413151, rs2214537), -237 A>T (27413246), and -53 G>A (27413430). There is no significant difference in MAF of each SNP between patients with KD and healthy controls except -237 A>T. Twenty five patients with KD had more than 1 SNP in contrast to only seven healthy controls had. The patients with KD have significantly more IL-21R gene polymorphisms than controls (odds ratio: 3.0, 95% confidence interval: 1.6-5.6, p=0.0005). There was no significant correlation between IL-21R gene polymorphisms and the serum level of IL-21. The serum level of total IgE was not significantly correlated with the presence of IL-21R gene polymorphisms. CONCLUSION: Our data suggest that the genetic susceptibility profile for KD may include IL-21R gene.
Gene Frequency ; Genetic Predisposition to Disease ; Humans ; Immunoglobulin E ; Interleukins ; Mucocutaneous Lymph Node Syndrome ; Polymerase Chain Reaction ; Polymorphism, Genetic ; Polymorphism, Single Nucleotide ; Promoter Regions, Genetic ; Receptors, Interleukin-21 ; Systemic Vasculitis

PURPOSE: The serum level of immunoglobulin (Ig)E has been reported to be elevated in patients with Kawasaki disease (KD). We investigated whether interleukin (IL)-21, rather than IL-4, could be related to elevated serum levels of IgE in KD. METHODS: Sera from 48 patients with KD and 12 controls with high fever were collected to determine the level of IgE using an immunoassay system and the levels of IL-4 and IL-21 were determined using enzyme-linked immunosorbent assay kits. RESULTS: The median IL-21 level of KD patients was significantly elevated, at 499.5 pg/mL (range: <62.5-1,544 pg/mL), whereas that of controls was <62.5 pg/mL (<62.5-825 pg/mL; P<0.001). The median IL-4 level of KD patients was not elevated (4.0 pg/mL; 2.1-7.6 pg/mL). The median level of total IgE in KD patients was 58.0 IU/mL (5-1,109 IU/mL). No statistically significant correlation was found between IL-21 and total IgE levels (Spearman's R=0.2; P=0.19). CONCLUSIONS: Patients with KD have elevated levels of IL-21 in the serum. IL-21 may play a role in the pathogenesis of KD.
Enzyme-Linked Immunosorbent Assay ; Fever ; Humans ; Immunoassay ; Immunoglobulin E ; Immunoglobulins ; Interleukin-4 ; Interleukins ; Mucocutaneous Lymph Node Syndrome

Hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber disease, is a rare autosomal dominant vascular disorder involving arteriovenous malformation (AVM) due to angiodysplasia. HHT is characterized by mucocutaneous hemorrhagic telangiectasia and AVM of various different organs. The mutated genes in HHT encode proteins that mediate signaling by the transforming growth factor-beta superfamily, leading to migration and proliferation of endothelial cells to finally result in AVMs of various organs. Amongst various affected organs, pulmonary AVM is the most common. This 11-year-old female patient with a past and familial history of frequent epistaxis visited the hospital for abdominal pain and vomiting which developed on the same day. Nodular opacity was found in chest X-ray. Subsequently, AVMs in the lungs and the spleen were recognized in chest computed tomography along with detection of ENG gene mutation. Here we report a case of hereditary hemorrhagic telangiectasia diagnosed by incidental chest X-ray findings followed by genetic and radiologic studies.
Abdominal Pain ; Angiodysplasia ; Arteriovenous Malformations ; Endothelial Cells ; Epistaxis ; Female ; Humans ; Lung ; Proteins ; Spleen ; Telangiectasia, Hereditary Hemorrhagic ; Telangiectasis ; Thorax ; Vomiting

A 7-year-old boy with pancytopenia, cervical lymphadenopathy, interstitial pneumonia, and hepatosplenomegaly was diagnosed with Epstein-Barr Virus (EBV)-associated hemophagocytic lymphohistiocytosis. His clinical course was characterized by hepatorenal syndrome and myocarditis. Based on his serological markers for EBV and an immunochromatography test for scrub typhus, this case was inferred as an EBV infection that was reactivated during tsutsugamushi infection. We treated this patient with the HLH-2004 protocol and administered clarithromycin. Normal ferritin level was achieved within 8 weeks after starting chemotherapy and antibiotics.
Anti-Bacterial Agents ; Child ; Clarithromycin ; Epstein-Barr Virus Infections ; Ferritins ; Hepatorenal Syndrome ; Herpesvirus 4, Human ; Humans ; Immunochromatography ; Lung Diseases, Interstitial ; Lymphatic Diseases ; Lymphohistiocytosis, Hemophagocytic ; Myocarditis ; Pancytopenia ; Scrub Typhus

Acute renal failure means that the word does not contain a mild kidney injury. In addition, the criteria for acute renal failure per researcher are different, and it is difficult in interpreting the results of research on acute renal failure. Therefore, rather than acute renal failure, a new term "acute kidney injury" meaning to include all the levels of injury is introduced. In 2002, to diagnose by means of serum creatinine, glomerular filtration rate and urine output, a detailed classification of acute kidney injury, the RIFLE criteria has been proposed. In 2007, the RIFLE criteria by transforming, AKIN criteria has been proposed. The pediatric RIFLE criteria for children has also been proposed. The author reviews here these criteria by comparing them.
Acute Kidney Injury ; Child ; Creatinine ; Glomerular Filtration Rate ; Humans ; Kidney