Neurofibromatosis type 1 (NF-1) is an autosomal dominant neurocutaneous disorder, which can affect different organs or systems of the body, including the cardiovascular system. One of the more serious aspects of the disease relates to arterial involvement. In particular, renal artery stenosis is one of the most common vascular abnormalities in patients with NF-1, and the manifestations vary, ranging from no symptoms to end-stage renal failure. Treatment usually consists of antihypertensive drugs, percutaneous transluminal angioplasty, or surgery. Other causes of hypertension should be ruled out and the patient followed up for close monitoring and proper management. We report a case of bilateral renal artery stenosis and hypertension in a patient with moyamoya disease associated with neurofibromatosis type 1. This report discusses the literature available on the current subject, its clinical features, diagnosis, and treatment.
Angioplasty ; Antihypertensive Agents ; Cardiovascular System ; Diagnosis ; Humans ; Hypertension* ; Kidney Failure, Chronic ; Moyamoya Disease* ; Neurocutaneous Syndromes ; Neurofibromatoses* ; Neurofibromatosis 1* ; Renal Artery Obstruction* ; Renal Artery*

Neurofibromatosis type 1 (NF-1) is an autosomal dominant neurocutaneous disorder, which can affect different organs or systems of the body, including the cardiovascular system. One of the more serious aspects of the disease relates to arterial involvement. In particular, renal artery stenosis is one of the most common vascular abnormalities in patients with NF-1, and the manifestations vary, ranging from no symptoms to end-stage renal failure. Treatment usually consists of antihypertensive drugs, percutaneous transluminal angioplasty, or surgery. Other causes of hypertension should be ruled out and the patient followed up for close monitoring and proper management. We report a case of bilateral renal artery stenosis and hypertension in a patient with moyamoya disease associated with neurofibromatosis type 1. This report discusses the literature available on the current subject, its clinical features, diagnosis, and treatment.
Angioplasty ; Antihypertensive Agents ; Cardiovascular System ; Diagnosis ; Humans ; Hypertension* ; Kidney Failure, Chronic ; Moyamoya Disease* ; Neurocutaneous Syndromes ; Neurofibromatoses* ; Neurofibromatosis 1* ; Renal Artery Obstruction* ; Renal Artery*

Background/Aims: Sarcopenia negatively affects the prognosis of cirrhotic patients, but clinical implications of changes in muscle mass remain unclear. We aimed to elucidate its role in the prognosis of outpatients with cirrhosis. Methods: Patients with cirrhosis who underwent annual abdominal computed tomography (CT) for hepatocellular carcinoma surveillance were included in the prospective cohort. The L3 skeletal muscle index (SMI) was adopted as a proxy for the amount of skeletal muscle, and the rate of SMI change between inclusion and after 1 year (ΔSMI/yr%) was calculated. Results: In total, 595 patients underwent a second CT after 1 year. Among them, 109 and 64 patients had sarcopenia and Child-Pugh class B/C decompensation at inclusion, which changed to 103 and 45 at the 1-year follow-up, respectively. During a median follow-up of 30.1 months after 1 year, 86 patients had at least one cirrhosis complication, and 18 died or received liver transplantation. In the development of cirrhosis complications, ΔSMI/yr% was independently associated, even after adjusting for the Child-Pugh and model for end stage liver disease (MELD)-Na scores. In addition, ΔSMI/yr% showed a good predictive performance for the development of cirrhosis complications within 6 months after 1-year follow-up in all subgroups, with a cut-off of -2.62 (sensitivity, 83.9%; specificity, 74.5%) in the overall population. SMI at 1-year and Child-Pugh score were independent factors associated with survival. In addition, changes in sarcopenia status significantly stratified survival. Conclusion ΔSMI/yr% was a good predictor of the development of cirrhosis complications in outpatients with cirrhosis, independent of Child-Pugh and MELD scores.

Background/Aims: Besifovir dipivoxil maleate (BSV) and tenofovir alafenamide fumarate (TAF) have been recently approved in Korea as the initial antiviral agents for chronic hepatitis B (CHB).However, the real-world outcome data for these drugs remain limited. Therefore, we conducted a noninferiority analysis using real-world data to compare the clinical outcomes of the two nucleotide analogs in treatment-naïve patients with CHB. Methods: We retrospectively investigated a cohort of patients with CHB who received BSV or TAF as first-line antiviral agents. The endpoints were virological response (VR) and liver-related clinical outcomes. Results: A total of 537 patients, consisting of 202 and 335 patients administered BSV and TAF, respectively, were followed up for 42 months. No significant difference was observed between the VRs of the patients from the two groups. The rates of biochemical response, virologic breakthrough, and incidence rates of hepatocellular carcinoma did not differ between the groups. However, the hepatitis B e antigen seroclearance rate was higher and the renal function declined less in the BSV group. Multivariable analysis indicated older age, alcohol abuse, cirrhosis and ascites, and lower serum HBV DNA level to be independently associated with increased hepatocellular carcinoma risk. The 1:1 propensity score-matched analysis with 400 patients showed VR rates of 85.0% and 88.7% in the BSV and TAF group patients, respectively, at 2 years. The absolute value of the 95% confidence interval for the difference (–0.04 to 0.12) satisfied the a priori limit of a noninferiority of 0.15. Conclusions BSV is noninferior to TAF in terms of VR, and their clinical outcomes are comparable to CHB.

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.
Academic Medical Centers ; Bacteriuria ; Biomarkers ; C-Reactive Protein ; Cicatrix ; Coinfection ; Fever ; Humans ; Hydronephrosis ; Korea ; Leukocytes ; Pyelonephritis ; Retrospective Studies ; Urinary Bladder ; Urinary Catheterization ; Urinary Catheters ; Vesico-Ureteral Reflux

BACKGROUND: Although there have been many studies on psoriasis, only a few studies have been conducted on pilosebaceous changes and even those results are controversial. While a few studies have been conducted on the morphological changes of the pilosebaceous unit in scalp psoriasis, comparative studies on the morphometric changes of pilosebaceous structures with a normal control group have not been conducted. OBJECTIVE: The purpose of this study is to elucidate the morphological change of the pilosebaecous unit in scalp psoriasis. METHODS: We conducted a comparative study an the morphometric changes of the pilosebaceous structure in 10 cases of scalp psoriasis with a normal control group. Results : The results of this study are summarized as follows : 1. Total hair counts, telogen hair counts, telogen hair counts, and the sizes of hairs and follicles in the lesional scalp showed no statistically significant increase than in the normal control. 2. The intrafollicular hyperkeratosis in infundibulum and sebaceous atrophy were more proininet in the lesional area than in the normal control area. These findings were also significantly prominent in the uninvolved normal-appearing area than in the normal control area. 3. Perifollicular inflammation, squamous metaplasia of the sebaceous gland and telangiectasia in the lesional and uninvolved areas were prominent, and these findings were not found in the normal control areas. Conclusion : The sebaceous atrophy and squamous metaplasia are very important findings of morphologic changes in psoriasis, and this paradoxical change may be an important clue to clarify the pathogenesis of psoriasis.
Atrophy ; Hair ; Inflammation ; Metaplasia ; Psoriasis ; Scalp* ; Sebaceous Glands ; Telangiectasis

Background: Several studies have recently suggested that liver disease and cirrhosis were risk factors for poor outcomes in patients with coronavirus disease 2019 (COVID-19) infections.However, no large data study has reported the clinical course of COVID-19 patients with chronic hepatitis B virus (HBV) infections. This study investigated whether HBV infection had negative impacts on the clinical outcomes of COVID-19 patients. Methods: We performed a nationwide population-based cohort study with 19,160 COVID-19-infected patients in 2020 from the Korean Health Insurance Review and Assessment database. The clinical outcomes of COVID-19 patients with chronic HBV infections were assessed and compared to those of non-HBV-infected patients. Results: Of the 19,160 patients diagnosed with COVID-19, 675 (3.5%) patients had chronic HBV infections. The HBV-infected patients were older and had more commodities than the non-HBV infected COVID-19 patients. During the observation period, COVID-19-related mortality was seen in 1,524 (8.2%) of the non-HBV-infected 18,485 patients, whereas 91 (13.5%) in HBV-infected 675 patients died of COVID-19 infection. Compared to patients without HBV infections, a higher proportion of patients with chronic HBV infections required intensive care unit (ICU) admission and had organ failures. However, odds ratios for mortality, ICU admission, and organ failure were comparable between the two groups after adjusting for age, sex, and comorbid diseases including liver cirrhosis and hepatocellular carcinoma. Conclusion COVID-19-infected patients with HBV infections showed worse clinical courses than non-HBV-infected COVID-19 patients. However, after adjustment, chronic HBV infection itself does not seem to affect the clinical outcomes in COVID-19 patients.

BACKGROUND/AIMS: The nonspecific clinical presentation of acute hepatitis A (AHA) mandates the detection of anti-hepatitis A virus IgM antibodies (IgM anti-HAV) in the serum for obtaining a definitive diagnosis. However, IgM anti-HAV might not be present during the early phase of the disease. The aim of this study was to determine the optimal time for repeating the IgM anti-HAV test (HAV test) in AHA patients with a negative initial test. METHODS: In total, 261 patients hospitalized with AHA were enrolled for this retrospective study. AHA was diagnosed when the test for IgM anti-HAV was positive and the serum alanine aminotransferase (ALT) level was > or =400 IU/L. Repeat HAV test was conducted after 1-2 weeks if the initial HAV test was negative but AHA was still clinically suspected. RESULTS: The results of the initial HAV test were negative in 28 (10.7%) patients. The intervals from symptom onset to the initial-HAV-test day and from the peak-ALT day to the initial-HAV-test day were significantly shorter in the negative-initial-HAV-test group, but on multivariate analysis only the latter was significantly associated with negative results for the initial HAV test (beta=-0.978; odds ratio [95% confidence interval]=0.376 [0.189-0.747]; P=0.005). The HAV test was positive in all patients when it was performed at least 2 days after the peak-ALT day. CONCLUSIONS: The results of HAV tests were significantly associated with the interval from the peak-ALT day to the HAV-test day. The optimal time for repeating the HAV test in clinically suspicious AHA patients with a negative initial HAV test appears to be at least 2 days after the peak-ALT day.
Acute Disease ; Adult ; Alanine Transaminase/blood ; Female ; Hepatitis A/*diagnosis ; Hepatitis A Antibodies/*blood ; Hepatitis A virus/*immunology ; Humans ; Immunoglobulin M/*blood ; Male ; Odds Ratio ; Retrospective Studies ; Time Factors

BACKGROUND/AIMS: Peginterferon plus ribavirin remains a standard therapy for patients with chronic hepatitis C (CHC) in Korea. We investigated the efficacy and long-term outcome of peginterferon and ribavirin therapy in Korean patients with CHC, particularly in relation to the stage of liver fibrosis. METHODS: The incidence of sustained virological response (SVR), hepatic decompensation, hepatocellular carcinoma, and liver-related death was analyzed in 304 patients with CHC; the patients were followed up for a median of 54 months. RESULTS: Among patients with HCV genotype 1, the SVR rate was 36.7% (18/49) and 67% (69/103) for patients with and without cirrhosis, respectively (p<0.001). For patients with non-1 HCV genotypes, the SVR rates were 86.0% (37/43) in cirrhotic patients and 86.2% (94/109) in noncirrhotic patients. SVR significantly reduced the risk of liver-related death, hepatic decompensation, and hepatocellular carcinoma, which had hazard ratios of 0.27, 0.16, and 0.22, respectively (all p<0.05). However, despite the SVR rate, patients with advanced fibrosis were still at risk of developing liver-related complications. CONCLUSIONS: A relatively high SVR rate was achieved by peginterferon plus ribavirin therapy in Korean patients with CHC, which improved their long-term outcomes. However, all CHC patients with advanced hepatic fibrosis should receive close follow-up observations, even after successful antiviral treatment.
Carcinoma, Hepatocellular ; Fibrosis ; Follow-Up Studies ; Genotype ; Hepatitis C ; Hepatitis C, Chronic* ; Hepatitis, Chronic* ; Humans ; Incidence ; Korea ; Liver Cirrhosis ; Ribavirin*

BACKGROUND/AIMS: We aimed to clarify the association of hepatitis B surface antigen (HBsAg)/hepatitis B core antigen (HBcAg) with the disease status and treatment response in patients with chronic hepatitis B (CHB). METHODS: We investigated 171 biopsy-proven entecavir-treated CHB patients (109 hepatitis B e antigen [HBeAg]-positive, 62 HBeAg-negative). HBcAg expression was positive when ≥10% of hepatocytes stained, and classified into nuclear, mixed, and cytoplasmic patterns. HBsAg expressions were intracytoplasmic (diffuse, globular, and submembranous) and membranous. The histologic activity index (HAI) and fibrosis stage followed Ishak system. RESULTS: In HBeAg-positive patients, older age, increased HAI score, advanced fibrosis, and reduced viral load were observed when HBcAg expression shifted from nucleus to cytoplasm in HBcAg-positive patients, and HBsAg expression from non-submembranous to submembranous in HBcAg-negative patients (all, p<0.05). In HBeAg-negative patients, only intracytoplasmic HBsAg expression patterns had clinical relevance with decreased ALT levels and viremia. In HBeAg-positive patients without favorable predictors of virologic response, negative HBcAg and membranous HBsAg expression predicted greater virologic response (both, p<0.05). The probability of HBeAg seroclearance was higher in patients with increased HAI or lacking HBcAg expression (both, p<0.05). Higher serum HBsAg levels and hepatocyte HBcAg positivity were associated with reduced serum HBsAg during first and post-first year treatment, respectively (both, p<0.05). CONCLUSIONS: Hepatocyte HBcAg/HBsAg expression is a good marker for disease status and predicting treatment response.
Cytoplasm ; Fibrosis ; Hepatitis B Core Antigens* ; Hepatitis B e Antigens ; Hepatitis B Surface Antigens* ; Hepatitis B* ; Hepatitis B, Chronic* ; Hepatitis* ; Hepatitis, Chronic* ; Hepatocytes* ; Humans ; Viral Load ; Viremia