Torque Onset Angle of the Knee Extensor as a Predictor of Walking Related Balance in Stroke Patients

Objective@#To investigate the relationship between the torque onset angle (TOA) of the isokinetic test for knee extensors in the paretic side and walking related balance in subacute stroke patients. @*Methods@#We retrospectively reviewed patients with first-ever strokes who have had at least two isokinetic tests within 6 months of onset. 102 patients satisfied the inclusion criteria. The characteristics of walking related balance were measured with the Berg Balance Scale sub-score (sBBS), Timed Up and Go test (TUG), 10-m Walk Test (10MWT) and Functional Independence Measure sub-score (sFIM). The second isokinetic test values of the knee extensor such as peak torque, peak torque to weight ratio, hamstring/quadriceps ratio, TOA, torque stop angle, torque at 30 degrees, and peak torque asymmetry ratio between paretic and non-paretic limb were also taken into account. Pearson’s correlation, simple regression and multiple regression analysis were used to analyze the correlation between TOA and walking related balance. @*Results@#TOA of the knee extensor of the paretic limb showed significant correlations with BBS, sBBS, TUG, 10MWT, and sFIM according to Pearson’s correlation analysis. TOA also had moderate to good correlations with walking related balance parameters in partial correlation analysis. In multiple regression analysis, TOA of the paretic knee extensor was significantly associated with walking related balance parameters. @*Conclusion@#This study demonstrated that TOA of the paretic knee extensor is a predictable parameter of walking related balance. Moreover, we suggest that the ability to recruit muscle quickly is important in walking related balance.

The Effect of Theophylline on Improvement of Renal Function in Asphyxiated Neonates: A Systematic Review and Meta-analysis

The purpose of this systematic review and meta-analysis was to assess the preventive effect of theophylline on acute kidney injury and the ameliorative effect of theophylline on renal function in asphyxiated neonates. A literature search of the PubMed/Medline, Embase, and Cochrane Library databases for information published up to February 2019 was conducted. All studies that reported the incidence rate of acute kidney injury, serum creatinine level, and glomerular filtration rate after the randomized administration of theophylline or placebo were included. In total, eight studies involving 498 neonates were eligible. The incidence rate of acute kidney injury was significantly lower in the theophylline group than in the placebo group (risk ratio [RR]: 0.42, 95% confidence interval [CI]: 0.32–0.55, p < 0.001). The changes in serum creatinine level in the theophylline group were significantly higher than those in the placebo group from the first day of life to 3 and 5 days of age (weighted mean difference [WMD]: −0.51, 95% CI: −0.62 to −0.40, p < 0.001, and WMD: −0.26, 95% CI: −0.34 to −0.18, p < 0.001, respectively). The changes in glomerular filtration rate in the theophylline group were significantly higher than those in the placebo group from the first day of life to 3 days of age and the last day of follow-up (WMD: 12.30, 95% CI: 9.39–15.21, p < 0.001, and WMD: 9.35, 95% CI: 6.43–12.27, p < 0.001, respectively). These results suggested that theophylline has a beneficial effect on the prevention of acute kidney injury in neonates with perinatal asphyxia.

Predictive performance of target controlled infusion of propofol-MCT/LCT using the modified Marsh and Schnider models: a simulation study

BACKGROUND: Only two pharmacokinetic models of propofol are commercially available in the category of target controlled infusion (TCI) pumps: the modified Marsh and Schnider models. Both models were developed using propofol-LCT (long chain triglyceride). Depending on the excipient, the pharmacokinetic properties of fast-acting drugs, such as propofol, vary. Hence, it is necessary to evaluate the predictive performances of both models using propofol-MCT (medium chain triglyceride)/LCT, which is frequently used in clinical practice. METHODS: This was a computer simulation study, using data collected in the previous clinical analysis used to evaluate the predictive performance of a pharmacokinetic model of propofol-MCT/LCT. The infusion profiles for each patient were applied as inputs to both models. Simulations were performed using TCI software, and the simulated plasma concentrations of both models were calculated. RESULTS: In total, 217 plasma samples, obtained from 35 patients, were used to determine the predictive performance. The pooled median (95% CI) biases and inaccuracies were 9.6 (−1.7 to 15.4) and 32.1 (22.6–38.2) respectively, for the modified Marsh model, and −5.9 (−8.9 to −0.7) and 26.3 (21.7–27.8) respectively, for the Schnider model. CONCLUSIONS: Although the pooled bias and inaccuracy of the Schnider models were clinically acceptable (< 10–20% and approximately 20–30%, respectively), the Schnider model consistently produced negatively biased predictions. Conversely, even though the pooled inaccuracy of the modified Marsh model failed to meet this criterion, the value did not deviate significantly from the standard. Therefore, it is reasonable to conclude that both TCI models can be used for propofol-MCT/LCT.

Impaired angiogenesis in the enalapril-treated neonatal rat kidney / 소아과

PURPOSE: Nephrogenesis is normally accompanied by a tightly regulated and efficient vascularization. We investigated the effect of angiotensin II inhibition on angiogenesis in the developing rat kidney. METHODS: Newborn rat pups were treated with enalapril (30 mg/kg/day) or vehicle (control) for 7 days after birth. Renal histological changes were checked using Hematoxylin & Eosin staining. We also investigated the intrarenal expression of vascular endothelial growth factor (VEGF)-A, VEGF receptor 1 (VEGFR1), VEGFR2, platelet-derived growth factor (PDGF)-B, and PDGF receptor-beta with Western blotting and immunohistochemical staining at postnatal day 8. Expression of the endothelial cell marker CD31 was examined to determine glomerular and peritubular capillary density. RESULTS: Enalapril-treated rat kidneys showed disrupted tubules and vessels when compared with the control rat kidneys. In the enalapril-treated group, intrarenal VEGF-A protein expression was significantly higher, whereas VEGFR1 protein expression was lower than that in the control group (P<0.05). The expression of VEGFR2, PDGF-B, and PDGF receptor-beta was not different between the 2 groups. The increased capillary CD31 expression on the western blots of enalapril-treated rat kidneys indicated that the total endothelial cell protein level was increased, while the cortical capillary density, assessed using CD31 immunohistochemical staining, was decreased. CONCLUSION: Impaired VEGF-VEGFR signaling and altered capillary repair may play a role in the deterioration of the kidney vasculature after blocking of angiotensin II during renal development.

Correlation of B-type natriuretic peptide levels and echocardiographic parameters in preterm infants with patent ductus arteriosus / 소아과

PURPOSE: This study aimed to evaluate the correlation, according to postnatal age, between plasma B-type natriuretic peptide (BNP) levels and echocardiographic parameters for the assessment of patent ductus arteriosus (PDA) in preterm infants with respiratory distress. METHODS: We enrolled 42 preterm infants with respiratory distress who underwent serial echocardiographic evaluation with simultaneous plasma BNP measurements until ductal closure. The correlations between BNP levels and the following 4 representative echocardiographic parameters were studied: diameter of the ductus arteriosus (DA), ratio of the left atrial diameter to the aortic diameter (LA/Ao), ratio of the PDA diameter to the infant's left pulmonary artery diameter (PDA/LPA), and the antegrade diastolic flow of LPA (DFLPA). RESULTS: BNP levels were significantly correlated to the magnitude of the ductal shunt, comprising the DA diameter, PDA/LPA ratio, LA/Ao ratio, and antegrade DFLPA for the overall study period. The earliest significant correlation, starting from postnatal day 2, was observed between the LA/Ao ratio and BNP levels. The PDA/LPA ratio and the antegrade DFLPA showed significant correlations with BNP levels postnatal day 3 onward, and with the DA diameter, postnatal day 5 onward. CONCLUSION: BNP levels and echocardiographic parameters showed a positive correlation, but the significance of the correlations differed according to the postnatal age, especially during the first few days of life.

Familial Mediterranean fever presenting as fever of unknown origin in Korea / 소아과

Familial Mediterranean fever (FMF) is the most common Mendelian autoinflammatory disease, characterized by uncontrolled activation of the innate immune system that manifests as recurrent brief fever and polyserositis (e.g., peritonitis, pleuritic, and arthritis). FMF is caused by autosomal recessive mutations of the Mediterranean fever gene, MEFV which encodes the pyrin protein. Although FMF predominantly affects people from Mediterranean and Middle Eastern ethnic origins, 3 cases of FMF have been reported in Korea since 2012. We report another case of FMF in Korea in which the patient presented with a month-long fever without serositis. After treatment with colchicine was initiated, the patient’s symptoms quickly subsided. The response to colchicine was helpful for diagnosis. We compare the FMF genotypes in Korea with in other countries. Studying FMF cases in Korea will help establish the best MEFV exons to use for screening and diagnosis of Korean FMF.

A case of CHARGE syndrome featuring immunodeficiency and hypocalcemia

CHARGE syndrome (coloboma, heart defects, atresia choanae, retarded growth and development, genital hypoplasia, and ear abnormalities) is characterized by multiple malformations and is diagnosed using distinct consensus criteria. Mutations in the gene encoding chromodomain helicase DNA-binding protein 7 (CHD7) are the major cause of CHARGE syndrome. Clinical features of CHARGE syndrome considerably overlap those of 22q11.2 deletion syndrome. Of these features, immunodeficiency and hypocalcemia are frequently reported in patients with 22q11.2 deletion syndrome but are rarely reported in patients with CHARGE syndrome. In this report, we have described the case of a patient with typical phenotypes of 22q11.2 deletion syndrome but without the proven chromosome microdeletion. Mutation analysis of CHD7 identified a pathogenic mutation (c.2238+1G>A) in this patient. To our knowledge, this is the first case of CHARGE syndrome with immunodeficiency and hypocalcemia in Korea. Our observations suggest that mutation analysis of CHD7 should be performed for patients showing the typical phenotypes of 22q11.2 deletion syndrome but lacking the proven chromosome microdeletion.

Application of Array-Based Comparative Genomic Hybridization to Pediatric Neurologic Diseases

PURPOSE: Array comparative genomic hybridization (array-CGH) is a technique used to analyze quantitative increase or decrease of chromosomes by competitive DNA hybridization of patients and controls. This study aimed to evaluate the benefits and yield of array-CGH in comparison with conventional karyotyping in pediatric neurology patients. MATERIALS AND METHODS: We included 87 patients from the pediatric neurology clinic with at least one of the following features: developmental delay, mental retardation, dysmorphic face, or epilepsy. DNA extracted from patients and controls was hybridized on the Roche NimbleGen 135K oligonucleotide array and compared with G-band karyotyping. The results were analyzed with findings reported in recent publications and internet databases. RESULTS: Chromosome imbalances, including 9 cases detected also by G-band karyotyping, were found in 28 patients (32.2%), and at least 19 of them seemed to be causally related to the abnormal phenotypes. Regarding each clinical symptom, 26.2% of 42 developmental delay patients, 44.4% of 18 mental retardation patients, 42.9% of 28 dysmorphic face patients, and 34.6% of 26 epilepsy patients showed abnormal array results. CONCLUSION: Although there were relatively small number of tests in patients with pediatric neurologic disease, this study demonstrated that array-CGH is a very useful tool for clinical diagnosis of unknown genome abnormalities performed in pediatric neurology clinics.

Partial Retraction: Difference of Anxiety of Parents: before & after the VCUG

No abstract available.

A Case of Congenital Hypertrophic Cardiomyopathy

Congenital hypertrophic cardiomyopathy (HCMP) is a very rare congenital heart disease. Here, we report a case of neonatal HCMP, which was confirmed by two-dimensional echocardiography and autopsy. The HCMP rapidly progressed and the patient's condition deteriorated, despite the treatment for congestive heart failure.

Persistent Complete Atrioventricular Block after Recovery from Acute Fulminant Myocarditis / 대한내과학회지

Acute myocarditis can be caused by viral, bacterial, or protozoal infection, or drug toxicity. Fulminant myocarditis progresses rapidly and frequently leads to cardiogenic shock, so patients should be supported by extracorporeal membrane oxygenation (ECMO), an intra-aortic balloon pump (IABP), mechanical ventilation, or a temporary pacemaker to maintain hemodynamic status. Most patients recover with supportive therapy. However, a few patients have persistent atrioventricular (AV) block. We report the case of a 34-year-old male with persistent complete atrioventricular block after the regression of acute myocarditis. Ultimately, a permanent pacemaker was implanted.

The Clinical Comparison between Monomicrobial and Polymicrobial Urinary Infection in Febrile Pediatric Acute Pyelonephritis

PURPOSE: We investigated the clinical presentation of febrile pediatric patients with acute pyelonephritis (APN) with a mixed urine culture from an aseptic urine sample, and compared with that of those with a single culture. METHODS: We retrospectively reviewed the medical charts of 95 patients diagnosed as APN with fever between January 2008 and October 2010 at Korea University Medical Center. We classified the patients with APN into two groups with a positive single culture (S group) and a positive mixed culture (M group) from an aseptic urine sample of suprapubic bladder aspiration or urethral catheterization and compared the fever duration, laboratory markers such as serum white blood cell (WBC) counts and C-reactive protein (CRP) values in peripheral blood, and the presence of hydronephrosis, renal scar and vesicoureteral reflux (VUR) between the two groups (If presence of hydronephrosis, scar and VUR=1 and no=0). RESULTS: Total pediatric patients with febrile APN were 95 patients, a positive S group was 89 patients and a positive M group was 6 patients. Fever duration (S vs. M, 4.7+/-3.1 vs. 6+/-5.7 days), serum WBC (S vs. M, 18,630+/-6,483 vs. 20,153+/-7,660/microL) and CRP (S vs. M, 100.6+/-2.46 vs. 81.1+/-0.09 mg/L) values, and the presence of hydronephrosis, renal scar and VUR were not different between the two groups. CONCLUSION: Our data shows that there were no specific differences of clinical manifestation between a positive single urine culture and a positive mixed urine culture in pediatric APN. A mixed urine culture from an aseptic urine sample should be interpreted cautiously.

Clinical Significance of Extended-Spectrum beta-Lactamase Producing Escherichia coli in Pediatric Patients with Febrile Urinary Tract Infection

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.

Acute Kidney Injury Accompanied by Acute Pyelonephritis and Renal Abscess in a Child with Vesicoureteral Reflux

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.

A Case of Neurofibromatosis with Invasion of Bladder

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.

An Unusual Case of Left Ventricular Free Wall Rupture Caused by a Silent Myocardial Infarction

Left ventricular free wall rupture (LVFWR) is a serious complication of myocardial infarction. It presents with a very high mortality rate and can be rescued by accurate diagnosis and emergency surgery. LVFWR can occur with sudden overt clinical symptoms or present insidiously. This report highlights the case of a man with no prior history of coronary artery disease, who presented with LVFWR and pericardial effusion that evolved to severe bacterial pericarditis.

Analysis of Interictal Epileptiform Discharges in the Benign Childhood Epilepsy with Centrotemporal Spikes: Prediction of Seizure Outcome / 대한소아신경학회지

PURPOSE: The benign childhood epilepsy with centrotemporal spikes (BECTS), commonly affecting children between 3 and 13 years of age, is focal epilepsy with the presence of centrotemporal spikes on electroencephalography (EEG). The aim of our study was to assess the relationship between EEG findings and clinical prognosis in patients with BECTS. METHODS: From 2005 to 2010, patients with a diagnosis of BECTS with a follow-up period of over one year after the diagnosis were included in our study. We analyzed their first interictal EEG: frequency of interictal discharges and average voltage in each waking and sleep phase. In addition, multiple factors were reviewed from the medical records: the age at the time of seizure onset, frequency of seizures, use of antiepileptic drugs (AEDs), duration of AED therapy, age at the time of EEG normalization and age at the time of last seizure. RESULTS: Seventy-four patients were included in our study. Frequent interictal epileptiform discharges during sleep correlated with younger age of seizure onset (P=0.040), earlier onset of AED therapy (P=0.022), younger age at the time of seizure cessation (P=0.027), greater frequency of interictal epileptiform discharges during wakefulness (P=0.006), higher average voltage (P=0.001) and higher frequency of seizures after AED initiation (P=0.043). CONCLUSION: In BECTS patients, frequent interictal epileptiform discharges during sleep could be correlated with higher seizure frequency, even after cessation of AED. These findings would be helpful in predicting the prognosis of children with BECTS.

A Case of Acrodermatitis Enteropathica with Normal Serum Zinc Level in a Breastfed Preterm Infant

Acrodermatitis enteropathica due to zinc deficiency is characterized by periorificial dermatitis, alopecia, and intractable diarrhea. Here we report a case of transient acrodermatitis enteropathica in a 2-month-old preterm infant, whose skin is erythematous, scaly, eczematous, with an eruption starting from the periorificial area. While her serum zinc level was normal at 118.2 ug/dL, the serum alkaline phosphatase level was low at 48 IU/L and the serum alkaline phosphatase level get increased concordant with clinical improvement after zinc supplementation. Hence, this case shows that the diagnosis of zinc deficiency can be aided with a low level of serum alkaline phoaphatase although serum zinc level is normal.

Erratum: A Case of Imperforate Hymen with Acute Urinary Retention

No abstract available.

Clinical Significance of Electrolyte Imbalance in Pediatric Urinary Tract Infection

PURPOSE: Some hormonal and electrolyte abnormalities have been reported in pediatric patients with urinary tract infection (UTI). This study aimed to investigate the relationships between the imbalance of electrolytes and the severity of infection and associated urologic anomalies in children with febrile UTI. METHODS: We retrospectively reviewed 267 patients with febrile UTI who were admitted to Korea University Guro Hospital during the period from January, 2007 until February, 2010. According to the presence of hyponatremia or hyperkalemia, clinical parameters and associated renal anomalies, such as hydronephrosis, cortical defects and vesicoureteral reflux, were compared. RESULTS: 42.7% of all patients had decreased concentration of serum sodium. In patients with decreased concentration of serum sodium, cortical defects were significantly increased compared to normal patients (40.4% vs. 14.4%, P<0.05). White blood cell (WBC) counts (15,721+/-6,553/uL vs. 12,885+/-5,367/uL, P<0.05), C-reactive protein (CRP) (61.8+/-56.1 mg/L, vs. 29.9+/-39.8 mg/L, P<0.05), and erythrocyte sedimentation rate (ESR) (43.9+/-34.3 mm/hr vs. 27.4+/-26.8 mm/hr, P<0.05) in peripheral blood showed significant increases in the group with decreased concentration of serum sodium. Duration of fever, presence of gastrointestinal symptom, the incidence of hydronephrosis and vesicoureteral reflux did not differ between the two groups. None of the patients had significant hyperkalemia. CONCLUSION: We suggest that decreased concentration of serum sodium in febrile UTI might be a helpful marker for leukocytosis and increased CRP and ESR in peripheral blood, and acute pyelonephritis.