PURPOSE: The present study investigates the long-term effects of intravenous immunoglobulin (IVIg) therapy for the treatment of moderate to severe childhood atopic dermatitis (AD). Previous research indicates that IVIg can treat severe AD; however, the effectiveness of IVIg has not been confirmed in prospective, blinded clinical trials. METHODS: Forty eligible children with moderate to severe AD, as defined by the criteria of Hanifin and Rajka, were enrolled in a randomized, placebo-controlled study. After the completion of an initial screening visit (V0), the patients were randomly allocated into therapy (n=30) and control (n=10) groups (V1). Thirty children were each treated with three injections of 2.0 g/kg IVIg at 1-month intervals over a 12-week period. Ten children were treated with placebo. Assessments were conducted after each injection (V2, V3, and V4) and at 3 (V5) and 6 months (V6) after completed treatment. RESULTS: The disease severity index was significantly decreased at V5 compared with the value at V1 (P<0.05). There were no significant changes in the total IgE level or total eosinophil count in peripheral blood at the last injection (V4) compared with the value at V1. The interleukin (IL)-5/interferon (IFN)-gamma ratio was assessed in T-helper 1 (Th1) and Th2 cells. The ratio significantly decreased between V1 and V5, after which it increased, such that the ratio at V6 was not significantly different from that at V1. Compared with the level at V1, the intercellular cell adhesion molecule-1 level at V4 did not differ significantly, but the level at V5 was lower. CONCLUSIONS: This study suggests that IVIg therapy may clinically improve AD in patients after 3 months of therapy, but the improvement may decline by 6 months after therapy.
Cell Adhesion ; Child ; Dermatitis, Atopic ; Eosinophils ; Humans ; Immunization, Passive ; Immunoglobulin E ; Immunoglobulins ; Immunoglobulins, Intravenous ; Interleukins ; Mass Screening ; Prospective Studies ; Th2 Cells

BACKGROUND/AIMS: Nonalcoholic steatohepatitis (NASH) is the progressive form of non-alcoholic fatty liver disease (NAFLD), and its prevalence is increasing worldwide. Liver biopsy remains the best way to diagnose NASH and establish the presence of fibrosis, but has not been performed easily in children because of its invasiveness. We analyzed the ultrasonographic and histopathologic findings of pediatric NAFLD patients, and studied to find their association with clinical characteristics and laboratory findings. METHODS: The study involved 18 obese children ranging from 7 to 15 years of age, who were diagnosed with NASH by liver biopsy. We performed the abdomen ultrasonography before the liver biopsy. We reviewed their pathology slides and classified them by NASH CRN (Clinical Research Network) scoring system. We also reviewed the abdomen ultrasonographic findings of the patients and classified them into grade of 1, 2, and 3. We reviewed the medical records of the patients and investigated their clinical characteristics and laboratory findings. RESULTS: The ultrasonographic grades had significant association with NAFLD activity score, grade of steatosis amount, ballooning change, and portal inflammation. Serum triglyceride level was significantly higher in patients who showed high grade steatosis and high NAFLD activity score. CONCLUSIONS: This study showed that serum triglycerides and ultrasonographic findings are highly correlated with pathologic findings in children with NAFLD.
Adolescent ; Biopsy, Fine-Needle ; Child ; Disease Progression ; Fatty Liver/diagnosis/pathology/ultrasonography ; Female ; Humans ; Male ; Obesity/complications ; Retrospective Studies ; *Severity of Illness Index ; Triglycerides/blood

We report a patient with whole neuroaxis dissemination of a sporadic supratentorial hemangioblastoma (HB) for more than 15 years. A 68-year-old female patient presented with severe radiating pain in the right leg. Gadolinium-enhanced lumbar spine MRI showed an intradural mass (2.5 cm in diameter) at the L4 level. The patient had been severely disabled for 22 years after a previous intraventricular brain tumor resection. At that time, the diagnosis was angioblastic meningioma, which was thought to be incorrect. At 14 years after the brain surgery, gamma knife radiosurgery was performed three times for newly developed or recurred supratentorial and infratentorial tumors in the cerebrospinal fluid pathway. The patient underwent lumbar spinal surgery, and a gross total removal of the mass was performed, which confirmed the histopathological diagnosis of HB. We reexamined the old histopathological specimen of the intraventricular tumor from 20 years ago and changed the diagnosis from angioblastic meningioma to supratentorial HB. Six months after spinal surgery, the patient underwent a second spinal surgery and brain surgery, and the histopathological diagnosis was HB following both surgeries, which was the same following the first spinal surgery. Here, we report a sporadic supratentorial HB patient who showed cranial and spinal disseminations for more than two decades along with a literature review

The paper provides a comprehensive overview of the growth and development of Hwasun Neurosurgery at Chonnam National University Hwasun Hospital over the past 18 years. As the first brain tumor center in Korea when it was established in April 2004, Hwasun Neurosurgery has since become one of the leading institutions in brain tumor education and research in the country. Its impressive clinical and basic research capabilities, dedication to professional education, and numerous academic achievements have all contributed to its reputation as a top-tier institution. We hope this will become a useful guide for other brain tumor centers or educational institutions by sharing the story of Hwasun Neurosurgery.

BACKGROUND: A successful cord blood (CB) bank requires long-term storage of a large number of CB units with good quality. To provide an optimal storage condition and to solve issues of space, many techniques have been developed to remove the plasma and RBC with maintenance of the quality and cell dose. We compared the results of the use of two different automated systems with the hydroxyethyl starch (HES) sedimentation method used in routine manual processing. METHODS: A total of 38 donated CB units with informed consent (18 June 2007~16 July 2007) were randomly selected and enrolled. We performed volume reduction of 20 units with the Sepax(R) S-100 (Sepax), 18 units with the AXP(TM) AutoXpress Platform (AXP), and compared the levels of nucleated cell recoveries, viability, and CD34+ cell/total nucleated cells (TNC) with the levels of 20 units processed using the HES method in the same period. The correlations of the initial TNC with cell recoveries were also evaluated. RESULTS: The mean values of the TNC recoveries, viabilities, and CD34+ cell/TNC among the three methods were similar (P=0.75; P=0.13; P=0.75). However, the MNC recovery was significantly higher with the use of the AXP system (89.2%, P<0.001) than the other methods. The Sepax system also showed a reduced SD for TNC recovery. Moreover, cell recovery rates were independent of the initial TNC counts. CONCLUSION: The Sepax and AXP automated cord blood process systems allow similar cell recoveries and viability as compared with the HES method. With the results of this study, a cord blood bank may be able to use an automated system considering the facility, personnel and the workload.
Fetal Blood* ; Informed Consent ; Plasma ; Starch

BACKGROUN: Despite improvements in immunosuppressive therapy for use in renal transplantation, acute graft rejection remains a risk factor of chronic rejection and a major cause of graft loss and patient death. Recently, daclizumab, an anti IL-2 receptor monoclonal antibody has been shown to reduce the incidence of acute rejection. METHODS: To investigate the immunosuppressive effect of daclizumab and the incidence of acute rejection, we administered daclizumab intravenously (1 mg/kg of body weight within 24 hours before transplantation and once every other week afterward, for a total of 5 doses) in combination with cyclosporine microemulsion (CsA), steroid and mycophenolate mofetil (MMF) to 68 transplant recipients RESULTS: Among them 62 were undergoing their first transplantation and 6 were undergoing their second transplantation. 32 patients received living-related transplants and 36 patients received living-unrelated transplants: their HLA match were as follows:1 case with 1 Ag match, 13 cases with 2 Ag matches, 18 cases with 3 Ag matches, 3 cases with 4 Ag matches, 1 case with 5 Ag matches. The clinical characteristics of patients treated with daclizumab were as follows: 42 were male, 26 were female; the mean age of recipients was 42.94 +/- 11.2 years and that of donor was 34.1 +/- 9.9 years. The underlying renal diseases were glomerulonephritis (n=47), reflux nephropathy (n=6), diabetic nephropathy (n=12), polycystic kidney disease (n=2) and acute renal failure (n=1). During the observed period (17.41 +/- 4.34 months; min. 6 months, max. 26 months), 2 cases had acute rejection in the third month after transplantation and 1 case in the 6th month after transplantation, 1 case in the 24th month after transplantation (4/68, 5.8%). In the historical control, 20.8% of acute rejection (10/48) were noted in CsA, MMF and steroid regimen group and 36% of acute rejection (22/60) in CsA, azathioprine and steroid group. Serum creatinine level was 1.21 +/- 0.23, 1.31 +/- 0.25, 1.35 +/- 0.28 and 1.34 +/- 0.31 (mg/dL) during the 1st, 3rd, 6th month and 1 year after transplantation respectively. 10 patients developed herpes-zoster infection and 6 patients had CMV infection. 1 patient expired due to CMV pneumonitis on the 3 months after transplantation. The 2-year graft survival rate was 98.5% with daclizumab and 45 months graft survival rates were 92.9% and 89.3% for MMF group and azathioprine group respectively. CONCLUSION: Daclizumab, used in combination with CsA, MMF and steroid, reduced acute rejection episodes without serious short term side effects. Further observation is needed to evaluate the graft survival rate and uncover any long-term side effects.
Acute Kidney Injury ; Azathioprine ; Body Weight ; Creatinine ; Cyclosporine ; Diabetic Nephropathies ; Female ; Glomerulonephritis ; Graft Rejection ; Graft Survival ; Humans ; Incidence ; Kidney Transplantation* ; Male ; Pneumonia ; Polycystic Kidney Diseases ; Receptors, Interleukin-2 ; Risk Factors ; Tissue Donors ; Transplantation ; Transplants

It has been well known that long-term immune suppression in renal transplant patients increases the possibility of complications. Infectious disease is one of the representative complications. We experienced a case of nocardiosis with cytomegalovirus infection after third renal transplantation in China. Nocardiosis is an important opportunistic infection in immunosuppressed patients, lymphoma, sarcoidosis, and organ transplant patients. CMV can cause severe hepatitis, pneumonitis, enteritis, endometritis, and encephalitis. It can depress bone marrow, and impair the immune system so as to increase other bacterial infection and trigger rejections. Third renal transplantation causes long-term immune suppression or over-immune suppression on transplant patients. Very few cases of third renal transplantation have been reported in Korea. We reduced the dose of immune- suppressants, and treated it successfully with ganciclovir and Trimethoprim/Sulfamethoxazole (Bactrim(R)).
Bacterial Infections ; Bone Marrow ; China* ; Communicable Diseases ; Cytomegalovirus ; Cytomegalovirus Infections ; Encephalitis ; Endometritis ; Enteritis ; Female ; Ganciclovir ; Hepatitis ; Humans ; Immune System ; Kidney Transplantation* ; Korea ; Lymphoma ; Nocardia Infections* ; Opportunistic Infections ; Pneumonia ; Sarcoidosis ; Transplants

Polyoma virus (PV) nephropathy is a known cause of graft loss after renal transplantation. In a renal transplant patient suspected of graft rejection, it is important to discriminate between PV induced interstitial nephritis and acute cellular rejection, because of similar pathologic findings. After the loss of the first allograft secondary to PV nephropathy, transplant graft nephroureterectomy before retransplantaton may have an influence in the recurrence of PV nephropathy. However, this question has not been completely resolved. Case: A 23-year-old male underwent first renal transplantation from his HLA haploidentical 25 year-old-sister. His renal function had been good with cyclosporine, steroid and azathioprine until 9 months after transplantation, when his serum creatinine level rose to 2.2 mg/dL. A renal biopsy revealed features of tubulitis and we confirmed PV nephropathy through a positive PV monoclonal antibody reaction to inclusion body. After gradual loss of graft function, he underwent hemodialysis. After 48 months of hemodialysis, the patient underwent cadaveric renal retransplantation without transplant graft nephroureterectomy. Thrombocytopenia and suspected delayed graft function occurred after 2 days of transplantation. A graft biopsy revealed thrombotic microangiopathy. Improved graft function was attained after a temporary stop of tacrolimus and ATGAM(R) bridging therapy. The patient is maintaining satisfactory graft function 33 months after retransplantation without clinical and serological evidence of recurrent PV infection.
Allografts ; Azathioprine ; Biopsy ; Cadaver ; Creatinine ; Cyclosporine ; Delayed Graft Function ; Graft Rejection ; Humans ; Inclusion Bodies ; Kidney Transplantation ; Male ; Nephritis, Interstitial ; Polyomavirus* ; Recurrence ; Renal Dialysis ; Tacrolimus ; Thrombocytopenia ; Thrombotic Microangiopathies ; Transplants* ; Young Adult

A 75-year-old man with chronic cholangitis and a common bile duct stone that was not previously identified was admitted for right upper quadrant pain. Acute cholecystitis with cholangitis was suspected on abdominal computed tomography (CT); therefore, endoscopic retrograde cholangiopancreatography with endonasal biliary drainage was performed. On admission day 5, hemobilia with rupture of two intrahepatic artery pseudoaneurysms was observed on follow-up abdominal CT. Coil embolization of the pseudoaneurysms was conducted using percutaneous transhepatic biliary drainage. After several days, intrahepatic artery pseudoaneurysm rupture recurred and coil embolization through a percutaneous transhepatic biliary drainage tract was conducted after failure of embolization via the hepatic artery due to previous coiling. After the second coil embolization, a common bile duct stone was removed, and the patient presented no complications during 4 months of follow-up. We report a case of intrahepatic artery pseudoaneurysm rupture without prior history of intervention involving the hepatobiliary system that was successfully managed using coil embolization through percutaneous transhepatic biliary drainage.
Aged ; Aneurysm, False ; Arteries ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangitis ; Cholecystitis, Acute ; Common Bile Duct ; Drainage ; Embolization, Therapeutic ; Follow-Up Studies ; Hemobilia ; Hepatic Artery ; Humans ; Rupture ; Tomography, X-Ray Computed

Although the prevalence of hereditary hemolytic anemia (HHA) is relatively low in Korea, it has been gradually increasing in recent decades due to increment in the proportions of hemoglobinopathies from immigrants of South East Asia, raising awareness of the disease among clinicians, and advances in diagnostic technology. As such, the red blood cell (RBC) Disorder Working Party (WP), previously called HHA WP, of the Korean Society of Hematology (KSH) developed the Korean Standard Operating Procedures (SOPs) for the diagnosis of HHA in 2007. These SOPs have been continuously revised and updated following advances in diagnostic technology [e.g., flow cytometric osmotic fragility test (FOFT) and eosin-5-maleimide (EMA) binding test], current methods for membrane protein or enzyme analysis [e.g., liquid chromatography-tandem mass spectrometry (LC-MS/MS), ultra-performance liquid chromatography-tandem mass spectrometry (UPLC-MS/MS), high-performance liquid chromatography (HPLC)], and molecular genetic tests using next-generation sequencing (NGS). However, the diagnosis and treatment of HHA remain challenging as they require considerable experience and understanding of the disease. Therefore, in this new Korean Clinical Practice Guidelines for the Diagnosis of HHA, on behalf of the RBC Disorder WP of KSH, updated guidelines to approach patients suspected of HHA are summarized. NGS is proposed to perform prior to membrane protein or enzyme analysis by LC-MS/MS, UPLC-MS/MS or HPLC techniques due to the availability of gene testing in more laboratories in Korea. We hope that this guideline will be helpful for clinicians in making diagnostic decisions for patients with HHA in Korea.