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

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.

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

The main objectives of this study were to investigate the concept of family function from the perspective of the contemporary Korean family, and to construct model of change of family function whit chronic illness. The hybrid model approach was applied in which three phases(theoretical phase, empirical phase, and analytic phase) of concept development were explored for family functioning. The study was conducted from 1997 to 1998. In empirical phase, two groups of purposive samples were drawn : normal family group composed of six families without ill family member, and ill family group composed of seven families of which wives have rheumatoid arthritis. Only families with child(or children)in primary or secondary schools were included in the study. The results were as follows: In theoretical phase, six dimensions of family concept were emerged : affective, structural, control, cognitive, financial, and reproductive dimension. In order to analyze the Korean normal family function in middle class with middle-age women, financial and reproductive dimension were not included. In empirical phase, five dimensions(affective, structural, control, cognitive, and external relationship) were fond from the normal family data. External relationship dimension is very important factor as a resource of the support, especially when their parents or siblings had no help or support to them. In the affective dimension, Korean family emphasized harmony and balance rather than affective expression harmony and balance rather than affective expression between couples and between parents and children. They also showed common goals of the families to solve their problems to control the family members. The priority of the goals was getting into the higher education of their children or helping their unhealthy parents or family members. Six dimension(affective, structural, control, cognitive, external relationship, and financial) of family functions were emerged from the ill family data. From the analysis of ill family data, types of restructuring house chore after wives illness were developed : (a) negociated, (b) accomodated, and (c) isolated, enduring types. Although the dimensions of family functioning identified in this study are similar to the conceptualizations that exist in the western literature, there where distinct differences in the nature of major themes and subconcepts under these family function dimensions.
Arthritis, Rheumatoid ; Child ; Chronic Disease* ; Education ; Family Characteristics ; Female ; Humans ; Parents ; Siblings ; Spouses

Parkinson's disease (PD) is characterized by selective and progressive degeneration of dopamine (DA)-producing neurons in the substantia nigra pars compacta (SNpc) and by abnormal aggregation of alpha-synuclein. Previous studies have suggested that DA can interact with alpha-synuclein, thus modulating the aggregation process of this protein; this interaction may account for the selective vulnerability of DA neurons in patients with PD. However, the relationship between DA and alpha-synuclein, and the role in progressive degeneration of DA neurons remains elusive. We have shown that in the presence of DA, recombinant human alpha-synuclein produces non-fibrillar, SDS-resistant oligomers, while beta-sheet-rich fibril formation is inhibited. Pharmacologic elevation of the cytoplasmic DA level increased the formation of SDS-resistant oligomers in DA-producing neuronal cells. DA promoted alpha-synuclein oligomerization in intracellular vesicles, but not in the cytosol. Furthermore, elevation of DA levels increased secretion of alpha-synuclein oligomers to the extracellular space, but the secretion of monomers was not changed. DA-induced secretion of alpha-synuclein oligomers may contribute to the progressive loss of the dopaminergic neuronal population and the pronounced neuroinflammation observed in the SNpc in patients with PD.
Blotting, Western ; Cell Line, Tumor ; Dopamine/*metabolism ; Humans ; Levodopa/pharmacology ; Neurons/*metabolism/pathology/*secretion ; Parkinson Disease/metabolism/pathology ; Substantia Nigra/metabolism/pathology ; alpha-Synuclein/*biosynthesis/*secretion

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*

The insertion of nasogastric tubes in comatose, obtunded or anesthetized patients is often difficult, frustrating and time-consuming. A large variety of methods inserting nasogastric tubes in those uncooperative patients have been reported. As a new effective method, we used Savary-Gilliard Wire Guide(R), which is designed for introducing Savary-Gilliard Dilator(R) into a strictured esophagus, for inserting a nasogastric tube in a comatose patient who was intubated with a ballooned tracheostomy tube. The insertion was successful in the first attempt and no complication occurred.
Coma* ; Esophagus ; Humans ; Tracheostomy

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

OBJECTIVE: Umbilical cord blood is an effective alternative to bone marrow as a source of hematopoietic stem cells for transplantation. But the amount of collected umbilical cord blood and its contents are limited and obtaining an adequate volume of umbilical cord blood is essential for successful transplantation. The aim of this study was to identify factors that influence the volume of umbilical cord blood. METHODS: A retrospective analysis of the maternal, neonatal and placental factors that were obtained by medical record review was conducted. The variables that were evaluated for this study were mother's age, parity, gestational age, presence of maternal diabetes mellitus, route of delivery, multiple births, neonatal sex and birth weight, and placental weight. Total 484 deliveries were evaluated from March 2003 to April 2004. The statistical significance of observed differences was calculated using t-test and multiple regression analysis; p-value<0.05 was considered significant. RESULTS: Gestational age, neonatal birth weight, placental weight, parity, number of fetus and maternal diabetes mellitus were significantly associated with a greater volume of collected umbilical cord blood. Obstetric factors that influenced the total nucleated cell concentration were gestational age, neonatal birth weight, placental weight, number of fetus, and route of delivery. CONCLUSION: To prolong a gestational age as far as possible, at least beyond the 37 completed weeks of gestation, and modifying a method of vaginal delivery or cesarean section rather than conventional vaginal delivery method can increase significantly the volume of collected cord blood and the yield of the concentration of total nucleated cell.
Birth Weight ; Bone Marrow ; Cesarean Section ; Diabetes Mellitus ; Female ; Fetal Blood* ; Fetus ; Gestational Age ; Hematopoietic Stem Cell Transplantation* ; Hematopoietic Stem Cells* ; Humans ; Medical Records ; Multiple Birth Offspring ; Parity ; Postpartum Period* ; Pregnancy ; Retrospective Studies ; Umbilical Cord*