BACKGROUND: Neutrophil gelatinase-associated lipocalin (NGAL), a bacteriostatic agent, is known to inhibit erythropoiesis leading to anemia. We aimed to investigate the associations of NGAL, anemia, and renal scarring in children with febrile urinary tract infections (UTIs).METHODS: We retrospectively reviewed the medical records of 261 children with febrile UTIs. The relationship between the presence of anemia and plasma NGAL levels was investigated. NGAL performance in comparison with serum C-reactive protein (CRP) at admission and after 72 hours of treatment was also evaluated for the prediction of renal scarring as well as acute pyelonephritis (APN) and vesicoureteral reflux (VUR).RESULTS: Plasma NGAL levels were elevated in patients with anemia compared with those without anemia. Multiple linear regression analysis showed an inverse relationship between NGAL levels and erythrocyte counts (standard β = −0.397, P < 0.001). Increased NGAL, but not CRP, was independently associated with the presence of anemia (odds ratio [OR], 2.37; 95% confidence interval [CI], 1.07–5.27; P < 0.05). Receiver operating curve analyses showed good diagnostic profiles of pre- and post-treatment NGAL for identifying APN, VUR, and renal scarring (all P < 0.05). For detecting renal scars, the area under the curve of post-treatment NGAL (0.730; 95% CI, 0.591–0.843) was higher than that of post-treatment CRP (0.520; 95% CI, 0.395–0.643; P < 0.05). The presence of anemia and elevated NGAL at admission (> 150 ng/mL) were independent risk factors for renal scarring in children with febrile UTIs. With anemia, NGAL levels increased consecutively in children with febrile UTI without renal involvement, with APN without scar, and with APN with renal scarring.CONCLUSION: Increased plasma NGAL levels may be associated with the presence of anemia and renal scarring in children with febrile UTIs.

BACKGROUND: Neutrophil gelatinase-associated lipocalin (NGAL), a bacteriostatic agent, is known to inhibit erythropoiesis leading to anemia. We aimed to investigate the associations of NGAL, anemia, and renal scarring in children with febrile urinary tract infections (UTIs). METHODS: We retrospectively reviewed the medical records of 261 children with febrile UTIs. The relationship between the presence of anemia and plasma NGAL levels was investigated. NGAL performance in comparison with serum C-reactive protein (CRP) at admission and after 72 hours of treatment was also evaluated for the prediction of renal scarring as well as acute pyelonephritis (APN) and vesicoureteral reflux (VUR). RESULTS: Plasma NGAL levels were elevated in patients with anemia compared with those without anemia. Multiple linear regression analysis showed an inverse relationship between NGAL levels and erythrocyte counts (standard β = −0.397, P < 0.001). Increased NGAL, but not CRP, was independently associated with the presence of anemia (odds ratio [OR], 2.37; 95% confidence interval [CI], 1.07–5.27; P < 0.05). Receiver operating curve analyses showed good diagnostic profiles of pre- and post-treatment NGAL for identifying APN, VUR, and renal scarring (all P < 0.05). For detecting renal scars, the area under the curve of post-treatment NGAL (0.730; 95% CI, 0.591–0.843) was higher than that of post-treatment CRP (0.520; 95% CI, 0.395–0.643; P < 0.05). The presence of anemia and elevated NGAL at admission (> 150 ng/mL) were independent risk factors for renal scarring in children with febrile UTIs. With anemia, NGAL levels increased consecutively in children with febrile UTI without renal involvement, with APN without scar, and with APN with renal scarring. CONCLUSION Increased plasma NGAL levels may be associated with the presence of anemia and renal scarring in children with febrile UTIs.

Renal cortical necrosis (RCN) is patchy or diffuse ischemic destruction of the renal cortex caused by significantly reduced renal arterial perfusion. It is a rare cause of acute kidney injury (AKI) and is associated with high mortality. Here, we review the case of RCN in a 15-year-old boy who developed AKI. A 15-year-old boy was referred to our hospital from a local hospital due to a sharp decrease in his renal function. He presented with acute flank pain, nausea with vomiting, and oliguria for the past two days. He had taken a single dose of antihistamine for nasal congestion. At our hospital, his peak blood pressure was 148/83 mmHg and he had a high body mass index of 32.9 kg/m². The laboratory data showed a blood urea nitrogen (BUN) of 28.4 mg/dL, a creatinine of 4.26 mg/dL, and a glomerular filtration rate estimated from the serum cystatin C of 20.2 mL/min/1.73m². Proteinuria (spot urine protein to creatinine ratio 1.66) with pyuria was observed. Kidney sonography showed parenchymal swelling and increased renal echogenicity. Due to rapidly progressing nephritis, steroid pulse therapy (750 mg/IV) was done on the second day of his admission and the patient showed complete recovery with normal renal function. However, the kidney biopsy findings revealed renal cortical hemorrhagic necrosis. Multifocal, relatively well-circumscribed, hemorrhagic necrotic areas (about 25%) were detected in the tubulointerstitium. Although RCN is an unusual cause of AKI, especially in children, pediatricians should consider the possibility of RCN when evaluating patients with rapidly decreasing renal function.
Acute Kidney Injury ; Adolescent ; Biopsy ; Blood Pressure ; Blood Urea Nitrogen ; Body Mass Index ; Child ; Creatinine ; Cystatin C ; Estrogens, Conjugated (USP) ; Flank Pain ; Glomerular Filtration Rate ; Humans ; Kidney ; Kidney Cortex Necrosis ; Male ; Mortality ; Nausea ; Necrosis ; Nephritis ; Obesity ; Oliguria ; Perfusion ; Proteinuria ; Pyuria ; Vomiting

PURPOSE: To evaluate the first results of surgical treatment using newly developed magnetically controlled growing rods (MCGR) for early onset scoliosis (EOS). MATERIALS AND METHODS: From January 2013 to January 2017, 5 patients, who underwent surgical treatment with MCGR for EOS and were followed for more than one year, were analyzed retrospectively. The demographic and radiology data, including age at surgery, diagnosis, number of lengthening, Cobb angle, T1–S1 length, T1–T12 length, and complications, were analyzed. RESULTS: The mean age of the patients was 6.0±2.7 years old. The subjects were 3 males and 2 females: 2 with neuromuscular scoliosis, 1 with syndromic scoliosis, 1 with idiopathic scoliosis, and 1 with congenital scoliosis. The mean number of lengthening was 9.8±2.9 times and the follow-up was 21.6±5.7 months. The Cobb angle improved from 82.0°±28.5° to 48.3°±28.8° at the last follow-up. The T1–S1 length increased from 283.1±72.7 mm to 342.6±86.3 mm at the last follow-up. The T1–T12 length increased from 163.1±50.5 mm to 202.3±65.5 mm at the last follow-up. One screw loosening complication was encountered and there were no neurological complications. CONCLUSION: The treatment using MCGR for EOS is effective and useful.
Diagnosis ; Female ; Follow-Up Studies ; Humans ; Male ; Retrospective Studies ; Scoliosis*

STUDY DESIGN: Retrospective study. OBJECTIVES: To evaluate the outcomes of dual growing rod treatment over a follow-up period of at least 2 years in patients with progressive pediatric spinal deformity. SUMMARY OF LITERATURE REVIEW: The dual growing rod treatment is safe and effective in curve correction and maintenance in patients with progressive pediatric spinal deformity. MATERIALS AND METHODS: Between 2009 to 2017, 14 patients who underwent dual growing rod treatment were followed up for more than 2 years. We analyzed their demographic and radiologic data, including age at surgery, sex, diagnosis, instrumented levels, number of total operations, number of lengthening procedures, interval of lengthening, Cobb angle, thoracic kyphosis angle, lumbar lordosis angle, T1-S1 length, and complications. RESULTS: The mean age of the patients was 11.0±2.9 years old. There were 10 male and 4 female patients, including 8 cases of neuromuscular scoliosis, 3 cases of idiopathic scoliosis, 2 cases of spondyloepiphyseal dysplasia, and 1 case of congenital scoliosis. The mean follow-up period was 42.4±14.0 months. The total number of operations was 6.6±2.6. The average number of lengthening procedures was 4.3±2.3 at an interval of 6.9±2.1 months. The Cobb angle improved from 60.4°±27.9° to 33.5°±19.7° after the initial treatment and 29.1°±16.4° after the last follow-up or final fusion. The T1-S1 length increased from 328.2±57.5 mm to 388.0±64.9 mm after the initial treatment and 424.9±64.4 mm after the last follow-up or final spinal fusion. The average growth rate was 11.5 mm/year. Six patients experienced 11 complications, of which 4 were Implant-related, and 7 were Infections. CONCLUSIONS: The dual growing rod technique is an effective and relatively safe treatment in patients with progressive pediatric spinal deformity.
Animals ; Congenital Abnormalities* ; Diagnosis ; Female ; Follow-Up Studies ; Humans ; Kyphosis ; Lordosis ; Male ; Osteochondrodysplasias ; Retrospective Studies ; Scoliosis ; Spinal Fusion

Eosinophilic gastroenteritis is a rare disease characterized by prominent eosinophilic tissue infiltration of the gastrointestinal tract. Here, we report a case of eosinophilic gastroenteritis in an 18-year-old patient with prolonged nephrotic syndrome who presented with abdominal pain and peripheral hypereosinophilia. During the previous 2 years, he had visited local Emergency Department several times because of epigastric pain and nausea. He had been treated with steroid-dependent nephrotic syndrome since 3 years of age. Tests ruled out allergic and parasitic disease etiologies. Gastroduodenoscopy with biopsy revealed marked eosinophilic infiltration in the duodenum. Renal biopsy findings indicated minimal change disease spectrum without eosinophilic infiltration. The oral deflazacort dosage was increased, and the patient was discharged after abdominal pain resolved. To our knowledge, this is the first report of eosinophilic gastroenteritis in a patient with minimal change disease.
Abdominal Pain ; Adolescent* ; Biopsy ; Duodenum ; Emergency Service, Hospital ; Eosinophilia ; Eosinophils* ; Gastroenteritis* ; Gastrointestinal Tract ; Humans ; Male* ; Nausea ; Nephrosis, Lipoid ; Nephrotic Syndrome* ; Parasitic Diseases ; Rare Diseases

Parkinson's disease (PD) and related Lewy body diseases are characterized by deposition of alpha-synuclein aggregates in both the central nervous system and peripheral nervous system. Synucleinopathy lesions spread to larger brain areas as the disease progresses, and prion-like cell-to-cell transmission of aggregated alpha-synuclein is thought to be the underlying mechanism for this pathological spreading. LRRK2 is another protein linked to the pathogenesis of PD, and its presence in Lewy bodies has attracted much attention as to whether LRRK2 and alpha-synuclein interplay during the pathogenesis of PD. However, the relationship between these two crucial proteins still remains unclear. In this review article, we will discuss the current state of knowledge in terms of how these proteins cause the disease and provide the hypothetical mechanisms by which LRRK2 might modify the generation and progression of synucleinopathy.
alpha-Synuclein ; Brain ; Central Nervous System ; Lewy Bodies ; Parkinson Disease* ; Peripheral Nervous System

Neurofibromatosis is a rare systemic disease, and genitourinary tract involvement is especially uncommon. Bladder is the most frequently involved organ in the genitourinary tract. Bladder neurofibromatosis may present as a diffuse infiltrative process or an isolated neurofibroma. The symptoms vary, ranging from urinary incontinence to retention. Treatment is usually conservative. The patient should be worked up to rule out other manifestation of tumor enlargement and followed to evaluate the development of new lesion. We report a case of the development of invasion of bladder in a patient with neurofibromatosis.
Humans ; Neurofibroma ; Neurofibromatoses ; Neurofibromatosis 1 ; Retention (Psychology) ; Urinary Bladder ; Urinary Incontinence ; Urinary Retention

Acute pyelonephritis (APN) is a relatively common bacterial infection in children. In previously healthy children, acute kidney injury (AKI) is very rare in the course of APN without urinary tract obstruction, renal hypoperfusion due to hypotension or exposure to nephrotoxic agents. We describe a case of AKI secondary to APN and renal abscess in a child with vesicoureteral reflux. With antibiotic treatment and adequate hydration, the patient was improved. APN should be included in the differential diagnosis of AKI and adequate treatment by proper antibiotics is crucial for full recovery of renal function.
Abscess ; Acute Kidney Injury ; Anti-Bacterial Agents ; Bacterial Infections ; Child ; Diagnosis, Differential ; Humans ; Hypotension ; Pyelonephritis ; Urinary Tract ; Vesico-Ureteral Reflux

PURPOSE: The incidence of community-acquired urinary tract infection (UTI) due to extended-spectrum beta-lactamase producing Escherichia coli (ESBL(+) E. coli) has increased worldwide. ESBL causes resistance to various types of the newer beta-lactam antibiotics, including the expanded spectrum cephalosporins and monobactams. We aimed to investigate the severity of UTI and associated genitourinary malformations in children with febrile UTI caused by ESBL(+) E. coli. METHODS: We retrospectively reviewed the medical records of 290 patients diagnosed as febrile UTI caused by E. coli between January 2008 and October 2010 at Korea University Medical center. We classified the patients into two groups with ESBL(+) and ESBL(-) E. coli group according to the sensitivity of urine culture. Fever duration, admission period, white blood cell (WBC) counts and C-reactive protein (CRP) in peripheral blood, the presence of hydronephrosis, cortical defects, vesicoureteral reflux (VUR) and renal scar were compared between the two groups. RESULTS: Patients with ESBL(+) E. coli were 32, and those with ESBL(-) E. coli were 258. If we excluded those tested with a sterile urine bag, patients with ESBL(+) E. coli were 22, and those with ESBL(-) E. coli were 212. Whether the results of sterile urine bag tests were included or not, there was no significant difference in all parameters between the two groups statistically. CONCLUSION: Our data shows that ESBL(+) E. coli may not be related to the severity of UTI and associated genitourinary malformations.
Academic Medical Centers ; Anti-Bacterial Agents ; beta-Lactamases ; C-Reactive Protein ; Cephalosporins ; Child ; Cicatrix ; Escherichia ; Escherichia coli ; Fever ; Humans ; Hydronephrosis ; Incidence ; Korea ; Leukocytes ; Medical Records ; Monobactams ; Retrospective Studies ; Urinary Tract ; Urinary Tract Infections ; Vesico-Ureteral Reflux