Background: Several cases of pediatric acute hepatitis of unknown etiology related to adenoviral infections have been reported in Europe since January 2022. The aim of this study was to compare the incidence, severity, possible etiology, and prognosis of the disease with those in the past in Korea. Methods: The surveillance group collected data between May and November 2022 using a surveillance system. Acute hepatitis of unknown etiology was defined in patients aged < 16 years with a serum transaminase level > 500 IU/L, not due to hepatitis A-E or other underlying causes. For comparison, data from 18 university hospitals were retrospectively collected as a control group between January 2021 and April 2022. Results: We enrolled 270 patients (mean age, 5 years). The most common symptom was fever. However, the incidence was similar between 2021 and 2022. Liver function test results, number of patients with acute liver failure (ALF), liver transplantation (LT), death, and adenovirus detection rates did not differ between the two groups. None of the adenoviruspositive patients in either group experienced ALF, LT, or death. In the surveillance group, adenovirus-associated virus-2 was detected in four patients, one of whom underwent LT. Patients with an unknown etiology showed significantly higher bilirubin levels, a lower platelet count, and a higher LT rate than patients with a possible etiology. Conclusion The incidence of pediatric acute hepatitis of unknown etiology and adenovirus detection rate have not increased in Korea.

Polymyositis (PM) is a chronic inflammatory disease that predominantly affects muscles. Systemic organ involvement, including the respiratory and gastrointestinal tracts, is frequently observed in PM, but renal involvement is rare. Herein, we report the case of a 56-year-old woman presenting with weight gain, edema, and generalized myalgia. Laboratory tests revealed elevated creatinine kinase level, hypoalbuminemia, and proteinuria. Histopathological examination of muscle biopsy revealed inflammatory myositis, and a renal biopsy confirmed immunoglobulin A (IgA) nephropathy. Based on the clinico-pathological results, the patient was diagnosed with PM with IgA nephropathy. This is a report of a rare occurrence of IgA nephropathy in a patient with PM presenting with chronic glomerulonephritis.
Biopsy ; Creatinine ; Edema ; Female ; Gastrointestinal Tract ; Glomerulonephritis ; Glomerulonephritis, IGA* ; Humans ; Hypoalbuminemia ; Immunoglobulin A* ; Immunoglobulins* ; Middle Aged ; Muscles ; Myalgia ; Myositis ; Phosphotransferases ; Polymyositis* ; Proteinuria ; Weight Gain

It is important to identify therapeutic compounds with no adverse effects for use in the chemotherapy of patients with bone-related diseases. The aim of this study was to identify a new compound that inhibits osteoclast differentiation and bone resorption. Herein, we examined the effects of 1',2'-dihydrorotenone on osteoclast differentiation and bone resorption in vitro and in vivo. 1',2'-dihydrorotenone inhibited receptor activator of NF-kappaB ligand (RANKL)-induced osteoclast differentiation of cultured bone marrow macrophages (BMMs) in a dose-dependent manner. However, 1',2'-dihydrorotenone did not exert cytotoxic effect on BMMs. 1',2'-dihydrorotenone suppressed the expression of c-fos and NFATc1 as well as osteoclast-specific genes in BMMs treated with RANKL. Treatment with RANKL inhibited the expression of inhibitors of differentiation/DNA binding (Id)1, 2, and 3; however, in the presence of 1',2'-dihydrorotenone, RANKL did not suppress the expression of Id1, 2, and 3. Furthermore, 1',2'-dihydrorotenone inhibited bone resorption and considerably attenuated the erosion of trabecular bone induced by lipopolysaccharide treatment. Taken together, these results suggest that 1',2'-dihydrorotenone has the potential to be applied in therapies for bone-related diseases.
Bone Marrow ; Bone Resorption ; Humans ; Macrophages ; Osteoclasts ; Receptor Activator of Nuclear Factor-kappa B ; Rotenone

Several autoimmune and chronic inflammatory conditions have been consistently linked with an increased risk of hematologic malignancies. Although ankylosing spondylitis (AS) is a chronic inflammatory disease, previous studies have demonstrated that it is not associated with an increase in risk of malignant lymphomas. Cases of AS accompanied by hematologic malignancies such as multiple myeloma, chronic myelogenous leukemia, and Hodgkin's disease have been reported. In Korea, AS with non-Hodgkin's lymphoma or follicular lymphoma has not been reported. We experienced a 38-year-old male who had been diagnosed with follicular lymphoma with bone metastasis, who achieved complete remission after having been treated with chemotherapy, developed new inflammatory back pain. An MRI of his hip showed an active inflammation of the left sacroiliac joint and a positive HLA-B27. The patient was diagnosed with AS and was treated with naproxen, which improved the pain in his back and buttock.
Adult ; Back Pain ; Buttocks ; Hematologic Neoplasms ; Hip ; HLA-B27 Antigen ; Hodgkin Disease ; Humans ; Inflammation ; Korea ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Lymphoma ; Lymphoma, Follicular ; Lymphoma, Non-Hodgkin ; Male ; Multiple Myeloma ; Naproxen ; Neoplasm Metastasis ; Sacroiliac Joint ; Spondylitis, Ankylosing

Patients with hemophilia generally have a reduced frequency of coronary artery disease compared to the general population. As advances in the management of hemophilia have increased their life expectancy, the prevalence of coronary artery disease also has increased. However, there are no standard treatment guidelines for coronary artery disease in patients with hemophilia, especially in the field of coronary intervention. We report the case of a patient with severe hemophilia A who presented with acute coronary syndrome and was successfully treated with percutaneous coronary intervention.
Acute Coronary Syndrome ; Angioplasty ; Coronary Artery Disease ; Hemophilia A ; Humans ; Life Expectancy ; Percutaneous Coronary Intervention ; Prevalence

A previous report by this laboratory demonstrated that bacterial iron chelator (siderophore) triggers inflammatory signals, including the production of CXC chemokine IL-8, in human intestinal epithelial cells (IECs). Microarray-based gene expression profiling revealed that iron chelator also induces macrophage inflammatory protein 3 alpha (MIP-3alpha)/ CC chemokine-ligand 20 (CCL20). As CCL20 is chemotactic for the cells involved in host adaptive immunity, this suggests that iron chelator may stimulate IECs to have the capacity to link mucosal innate and adaptive immunity. The basal medium from iron chelator deferoxamine (DFO)-treated HT-29 monolayers was as chemotactic as recombinant human CCL20 at equivalent concentrations to attract CCR6+ cells. The increase of CCL20 protein secretion appeared to correspond to that of CCL20 mRNA levels, as determined by real-time quantitative RT-PCR. The efficacy of DFO at inducing CCL20 mRNA was also observed in human PBMCs and in THP-1 cells, but not in human umbilical vein endothelial cells. Interestingly, unlike other proinflammatory cytokines, such as TNF-alpha and IL-1beta, a time-dependent experiment revealed that DFO slowly induces CCL20, suggesting a novel mechanism of action. A pharmacologic study also revealed that multiple signaling pathways are differentially involved in CCL20 production by DFO, while some of those pathways are not involved in TNF-alpha-induced CCL20 production. Collectively, these results demonstrate that, in addition to some bacterial products known to induce host adaptive immune responses, direct chelation of host iron by infected bacteria may also contribute to the initiation of host adaptive immunity in the intestinal mucosa.
Calcium/metabolism ; Cell Movement/drug effects ; Chemokines, CC/genetics/*metabolism ; Deferoxamine/*pharmacology ; Egtazic Acid/analogs & derivatives/pharmacology ; HT29 Cells ; Humans ; Immunity, Mucosal/*drug effects ; Intestinal Mucosa/*drug effects/immunology/metabolism ; Iron Chelating Agents/*pharmacology ; Macrophage Inflammatory Proteins/genetics/*metabolism ; NF-kappa B/metabolism ; Phosphoprotein Phosphatase/physiology ; Protein Transport/drug effects ; Protein-Serine-Threonine Kinases/physiology ; RNA, Messenger/genetics/metabolism ; Receptors, Chemokine/metabolism ; Research Support, Non-U.S. Gov't

Susceptibilities of 5 different mice strains, including C3H/HeN, BALB/c, C57BL6, FvB and ICR, to Echinostoma hortense infection, was evaluated. The worm expulsion rate, worm size and egg production were observed from 1 to 8 weeks after infection with 30 metacercariae. C3H/HeN and ICR mice showed the highest worm maturation rates. The worm recovery rate and the number of eggs per gram (EPG) of feces was also higher in C3H/HeN and ICR mice than in BALB/c, C57BL6, and FvB mice. It is suggested that E. hortense is highly infectious to ICR and C3H/HeN mice, but not to the other strains of mice. Based on the results obtained, we believe that the susceptibility of different mouse strains to E. hortense infection is dependent on the genetic and immunologic background of mice.
Animals ; Echinostoma/*growth & development ; Echinostomiasis/genetics/*parasitology ; Feces/parasitology ; Female ; Genetic Predisposition to Disease ; Intestines/parasitology ; Mice/*parasitology ; Mice, Inbred BALB C ; Mice, Inbred C3H ; Mice, Inbred C57BL ; Mice, Inbred ICR ; Parasite Egg Count

PURPOSE: We performed this study to assess the incidence of venous complications, including portal vein and hepatic vein stenosis, in both split cadaveric and living donor liver transplants and to assess the diagnostic and therapeutic modalities of these lesions. METHODS: Seventy-six liver transplantations were performed in 75 children with split (5) or living donor (71) graft between December 1994 and March 2002. Patients' data were analyzed retrospectively with special emphasis on venous complications. RESULTS: Venous complications occurred in 14 patients (18.6%) including hepatic vein stenosis in 8, portal vein stenosis in 4, portal vein thrombosis in 1, and combined portal vein thrombosis and hepatic artery stenosis in 1 patient. Venous complications were accompanied by abnormality of liver fuction, ascites, progressed splenomegaly, and gastrointestinal bleeding. To diagnose the venous complications, Doppler ultrasonography was performd at first, and those were confirmed by angiography or CT. Hepatic vein stenosis was managed by percutaneous transhepatic angioplasty (6), angioplasty followed by reposition of graft (1), and supportive care only 1 patient. Portal vein complications were managed by angioplasty (4), angioplasty followed by mesocaval shunt (1), and combined revascularization and angioplasty (1). The overall survival rate was 86% (12 of 14 patients). CONCLUSION: Close surveillance of the complication of vascular anastomoses and multidisciplinary approach to treat of venous complication after pediatric liver transplantation have made it possible to reduce the graft loss and mortality. Aggressive and successful radiologic intervention should be considered to eliminate the need for surgical revision, portacaval shunting or retransplantation.
Angiography ; Angioplasty ; Ascites ; Cadaver ; Child ; Constriction, Pathologic ; Hemorrhage ; Hepatic Artery ; Hepatic Veins ; Humans ; Incidence ; Liver Transplantation* ; Liver* ; Living Donors ; Mortality ; Portacaval Shunt, Surgical ; Portal Vein ; Reoperation ; Retrospective Studies ; Splenomegaly ; Survival Rate ; Transplants ; Ultrasonography, Doppler ; Venous Thrombosis

The uterus with rudimentary horn occurs as a result of a lack of development during fetal life of the middle and lower parts of one of the Miillerian ducts, in which there is a failure of fusion of the two ducts, The incidence of this uterine anomaly is rare. Many cases of rudimentary uterine horn are not discovered since they do not become involved in a pregnancy, and thus remain symptomless and uncomplicated, and the diagnosis usually being made only with pregnancy with rupture. In this presentation, we describe a case of diagnosis and management of unruptured non-communicating rudimentary uterine horn pregnancy at the third trimester. The patient diagnosed by ultrasonography and MRI, was hospitalized for further evaluation and delivery under the impression of unruptured non-communicating rudimentary horn pregnancy. A living male infant weighing 1,930 gm was delivered by cesarean section at 33+l weeks due to impending preterm labor. Some articles concerned this subject were reviewed briefly to discuss relevant method of diagnosis, treatment, and clinical characteristics.
Animals ; Cesarean Section ; Diagnosis ; Female ; Horns* ; Humans ; Incidence ; Infant ; Magnetic Resonance Imaging ; Male ; Obstetric Labor, Premature ; Pregnancy Trimester, Third* ; Pregnancy* ; Rupture ; Ultrasonography ; Uterus

PURPOSE: To evaluate the MR imaging findings of liposarcomas of different histologic subtypes. MATERIALS AND METHODS: We evaluated MR images of 21 patients (5 men and 16 women, mean age, 55 years) with liposarcoma andcorrelated the findings with the results of histopathology. In the study group seven liposarcomas werewell-differentiated, seven were myxoid, three were mixed, two were pleomorphic, and one was round cell. RESULTS: On T1 -and T2 - weighted images, six of seven well-differentiated liposarcomas showed signal intensity equal tothe fat and hypointense septa, while the other showed low signal intensity on a T1 -weighted image, heterogeneoushigh signal intensity on a T2- weighted image, heterogeneous enhancement after the administration of contrastmedia and was dedifferentiate. Nine masses in seven patients with myxoid liposarcoma showed low signal intensityon T1-weighted images, six of the nine showed lace-like foci of high signal intensity. On T2 -weighted images, allmasses showed homogeneous high signal intensity. After administration of contrast media, five of seven massesshowed heterogeneous enhancement. Two of three mixed form were well-differentiated and myxoid types, and twosubtypes were separable on MR. Pleomorphic, round cell, mixed type myxoid and pleomorphic and unclassified casesshowed low signal intensity on T1-weighted images, heterogeneous high signal intensity on T2-weighted andheterogeneous enhancement. CONCLUSION: Using MR imaging, well-differentiated and myxoid liposcarcomas may bedifferentiated from other types.
Contrast Media ; Female ; Humans ; Liposarcoma* ; Liposarcoma, Myxoid ; Magnetic Resonance Imaging ; Male