BACKGROUND: Hemophagocytic lymphohistiocytosis(HLH) is a rare and fatal disorder in children. Recently its clinical characteristics and etiology of uncontrolled activation of cellular immune system in genetically predirected patients have keen elucidated. The authors analyzed the clinical characteristics and response to immunosuppressive agents of HLH patients in single institute. METHODS: The authors retrospectively analyzed various clinical data including CSF and bone marrow examination at diagnosis and follow up period in the 6 patients who were diagnosed as HLH at Asan Medical Center during last 2 years. Antithymocyte globulin(ATG : 10 mg/kg/day) and methylprednisolone(methyl-PD: 5 mg/kg/day) for 5 consecutive days as induction treatment and cyclosporin A(CsA) as maintenance treatment after induction with weekly intrathecal methotrexate for 5-6 weeks were given to the recently diagnosed 3 patients. RESULTS: All the patients except one were infants. Persistent fever, hepatosplenomegaly and pancytopenia were observed in all the patients. Family history of suspicious HLH was observed in 4 patients(67%). The characteristic elevated serum triglyceride(TG) level and/or decreased fibrinogen level were noted in all. Mild to moderate CSF pleocytosls with increased lymphocytes and monocytes was also observed in all during the disease course. Variable degree of nonqr-Langerhans cell histiocytic infiltration and hemophagocytosis were observed in all the cases. Of the 3 patients treated with ATG and methyl-PD, one achieved complete remission and the others achieved partial remission within 2 weeks of treatment, but all expired after 5 months, remission with CsA. Regardless of treatment regimen, all the 6 patients expired due to CNS sequelae of HLH. CONCLUSIONS: HLH mainly developed in infants. Persistant fever, hepatosplenomegaly and pancytopenia were observed in most cases with the characteristic change of serum TG and/or fibrinogen level. HLH should be included in the differential diagnosis in patients with these features especially when the family history of suspicious HLH is present, and histologic comfirmation of HLH could be easily accomplished with bone marrow study in most cases. Remission induction of HLH could be achieved with immunosuppressive treatment but it was difficult to maintain long term remission.
Bone Marrow ; Bone Marrow Examination ; Child ; Chungcheongnam-do ; Cyclosporine ; Diagnosis ; Diagnosis, Differential ; Fever ; Fibrinogen ; Follow-Up Studies ; Humans ; Immune System ; Immunosuppressive Agents ; Infant ; Lymphocytes ; Lymphohistiocytosis, Hemophagocytic* ; Methotrexate ; Monocytes ; Pancytopenia ; Remission Induction ; Retrospective Studies

BACKGROUND: Despite the development of empiric antibiotics in treatment of neutropenic fever(NF) in pediatric cancer patients, bacterial infection is still the most important cause of death in these patients. In this study the authors analyzed clinical characteristics and tried to find out the possible risk factors of the pediatric cancer patients who succumbed to the documented bacterial infection after episodes of neutropenic fever. METHODS: 17 pediatric cancer patients expired after episodes of neutropenic fever(NF) in the pediatric department of Asan Medical Center from Mar. 1990 to Feb. 1996 were grouped by the results of bacterial culture. 7 cases (Group A) had documented bacterial culture results and 10 cases (Group B) had negative culture results. The clinical characteristics of these two groups were analyzed retrospectively. RESULTS: There were no differences in the types of cancer between the two groups. All the documented bacteria were gram negative organisms and all cultured from the blood. There were no differences between the two groups in the treatment duration, known risk factors of infection before and at the onset of fever, antibiotics administered, and interval from the onset of NF to the administration of antibiotics. The response rate of initial empiric antibiotics was lower in group A(14%) compared to Group B(40%), even though all the cultured organisms except 2 cases in Group A were sensitive to initial empirical antibiotics. Septic shock was the cause of death in 86% of Group A patients, but only in 10% of Group B patients. The other causes of death were progression of cancer, bleeding, hepatic failure, adult respiratory distress syndrome and multiple organ failure, which showed no difference between the 2 groups. The interval from the onset of fever to death was significantly shorter in Group A(6.2+/-2.3 days) compared to Group B(24.9+/-18.6 days). CONCLUSIONS: Bacterial infection is still the most frequent cause of death after NF. Most patients succumbed to the documented bacterial infection showed rapid progression of bacteremia to septic shock despite administration of sensitive antibiotics. The known risk factors of infection before the onset of NF and other treatment factors shows no differences between the two groups in this study. These results suggest that the bacteremia is obvious risk factor of poor outcome after NF episode, and antibiotics alone may be insufficient to prevent the rapid progression of septic shock in these patients.
Anti-Bacterial Agents ; Bacteremia ; Bacteria ; Bacterial Infections ; Cause of Death* ; Chungcheongnam-do ; Fever* ; Hemorrhage ; Humans ; Liver Failure ; Multiple Organ Failure ; Respiratory Distress Syndrome, Adult ; Retrospective Studies ; Risk Factors ; Shock, Septic

No abstract available.
Hyperlipidemias ; Skin Diseases

PURPOSE: To report a case of high-pressure paint gun injury to the eyeball and ocular adnexa. CASE SUMMARY: A 50-year-old woman was admitted after accidental high-pressure paint injection to her right eye while working. She complained of pain and severe swelling of the eyelids covered with paint. Slit lamp biomicroscopy showed multiple conjunctival lacerations, deposition of paint material on her conjunctiva, corneal edema and crystalline lens dislocation. Orbital computed tomography revealed infiltration of paint material into the retrobulbar space and rupture of medial rectus muscle. The paint was removed from the conjunctiva, Tenon's tissue, and medial orbit after the medial rectus muscle was disinserted. On the second postoperative day, the wound was irrigated and pus was drained from the conjunctiva wound. Two months after the operation, the patient underwent cataract removal due to high intraocular pressure associated with lens dislocation. Six months after the operation, eyeball movement was improved except medial gaze, and the cornea was stable with moderate corneal haziness. CONCLUSIONS: In high-pressure paint gun injury to the eye, detail evaluation, prompt removal of the foreign body and proper management of complications are necessary.
Cataract ; Conjunctiva ; Cornea ; Corneal Edema ; Dislocations ; Eye ; Eyelids ; Female ; Foreign Bodies ; Humans ; Intraocular Pressure ; Lacerations ; Lens Subluxation ; Lens, Crystalline ; Middle Aged ; Muscles ; Orbit ; Paint ; Rupture ; Suppuration

No abstract available.
Humans ; Lichens*

No abstract available.
Humans ; Lichens*

PURPOSE: To explore the expressions of P53 and phosphorylation-H2AX in varicocele-induced rat testes. MATERIALS AND METHODS: A total of 16 adult male Sprague-Dawley rats underwent an operation; 12 underwent an experimental varicocele and 4, as controls, were sham-operated. Groups of 4 varicocele-induced rats underwent a left orchiectomy after 2 or 3 weeks, or both orchiectomies after 4 weeks. The sham-operated rats underwent both orchiectomies after 4 weeks. Sections of both testes from each animal were studied. The changes in the expressions of P53 and phosphorylation of H2AX were determined using immunohistochemistry and western blot. RESULTS: Immunohistochemical staining of the left testes in the varicocele- induced rats showed that the expressions of P53 and phosphorylation of H2AX had not begun 2 weeks postoperatively, but remarkable results were observed after 3 and 4 weeks. Both testes of the varicocele-induced rats showed the expressions of P53 and phosphorylation of H2AX after 4 weeks, with the left testes being more distinctive in immunohistochemical staining compared to the right. Western blot of the left testes in the varicocele- induced rats also showed unclear expressions of P53 and gamma-H2AX after 2 weeks. Considerable distinction was seen after 3 and 4 weeks compared to the control group. CONCLUSIONS: Our results suggest that experimental varicocele is associated with increased sperm DNA damage. These changes may be related to abnormal spermatogenesis.
Adult ; Animals ; Blotting, Western ; DNA Damage ; Germ Cells* ; Humans ; Immunohistochemistry ; Male ; Orchiectomy ; Phosphorylation* ; Rats* ; Rats, Sprague-Dawley ; Spermatogenesis ; Spermatozoa ; Testis ; Varicocele*

PURPOSE: The aim of this study is to investigate the quality of basic life support (BLS) information for primary Korean-speaking individuals on the internet.METHODS: Using the Google © search engine, we searched for the terms ‘CPR’, ‘cardiopulmonary resuscitation (in Korean)’ and ‘cardiac arrest (in Korean)’. The accuracy, reliability and accessibility of web pages was evaluated based on the 2015 American heart association(AHA) guidelines for CPR & emergency cardiovascular care, the health on the net foundation code of conduct and Korean web content accessibility guidelines 2.1, respectively.RESULTS: Of the 178 web pages screened, 50 met criteria for inclusion. The overall quality of BLS information was not enough (median 5/7, IQR 4.75-6). 23(36%) pages were created in accordance with 2010 AHA guidelines. Only 24(48%) web pages educated on how to use the automated electrical defibrillator. The attribution and transparency of the reliability of pages was relatively low, 20(40%) and 16(32%). The web accessibility score was relatively high.CONCLUSION: A small of proportion of internet web pages searched by Google have high quality BLS information for a Korean-speaking population. Web pages based on past guideline were still being searched. The notation of the source of CPR information and the transparency of the author should be improved. The verification and evaluation of the quality of BLS information exposed to the Internet are continuously needed.
Cardiopulmonary Resuscitation ; Defibrillators ; Education ; Emergencies ; Heart ; Heart Arrest ; Internet ; Resuscitation ; Search Engine

PURPOSE: Sarcoidosis is a noncaseating granulomatous disorder that can affect any organ. In its early phase, sarcoidosis is clinically similar to tuberculosis. We report a case of sarcoidosis diagnosed through ocular and systemic evaluations in a patient who developed multiple conjunctival nodules during antituberculous treatment for nasal mucosal nodules. CASE SUMMARY: A 37-year-old woman who had been on antituberculosis medications for 9 months because of multiple nasal mucosal nodules was referred for conjunctival hyperemia and the multiple nodules. The ocular examination revealed multiple conjunctival nodules in the upper and lower fornices. Biopsy and systemic evaluations were performed. A conjunctival biopsy specimen showed noncaseating granulomatous inflammation compatible with sarcoidosis. Laboratory tests revealed hypercalciuria and increased levels of ACE. Chest computed tomography showed bilateral hilar and mediastinal lymphadenopathy. Subconjunctival steroid injection was done for the conjunctival nodules and systemic steroid treatment was started as well. Complete resolution of conjunctival lesions was obtained. CONCLUSIONS: In cases of chronic, multiple conjunctival and nasal mucosal nodules, sarcoidosis should be considered in the differential diagnosis.
Adult ; Biopsy ; Conjunctiva ; Diagnosis, Differential ; Female ; Humans ; Hypercalciuria ; Hyperemia ; Inflammation ; Lymphatic Diseases ; Nasal Mucosa ; Sarcoidosis ; Thorax ; Tuberculosis

PURPOSE: To compare the efficacy of time domain (TD) and spectral domain (SD) optical coherence tomography (OCT) in determining vitreomacular interface (VMI). METHODS: VMIs were evaluated with TD and SD OCT images crossing the fovea horizontally in 69 eyes (mean age 52.7 +/- 15.4 years) and were classified as follows: (1) no vitreomacular separation (VMS), (2) incomplete VMS, and (3) unknown. RESULTS: In TD OCT, no VMS was observed in 2 eyes (2.9%), incomplete VMS in 2 eyes (2.9%), and unknown in 65 eyes (94.2%). In SD OCT, no VMS was observed in 31 eyes (45.0%), incomplete VMS in 13 eyes (18.8%), and unknown in 25 eyes (36.2%). In 31 eyes with no VMS on SD OCT, 29 eyes (93.5%) presented unknown on TD OCT (p<0.0001). In 13 eyes with incomplete VMS on SD OCT, 2 eyes (15.4%) showed incomplete VMS and 11 eyes (84.6%) showed unknown on TD OCT (p<0.0001). TD OCT was also non-informative in all 25 eyes with unknown on SD OCT. CONCLUSIONS: SD OCT allows better visualization of VMI than TD OCT, especially in patients with no VMS.
Humans ; Tomography, Optical Coherence*