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

A 46-year-old male presented with skin lesion on the tip of penis, occurring a month ago. Erythematous macules and grouped vesicles on the glans penis and upper side of prepuce area were noticed. In addition, scales were noticed on the tip of glans penis. The lesion was first diagnosed as herpes genitalis based on the clinical manifestation, but had failed to improve with systemic and topical antiviral and topical antibiotics. KOH examinations were performed on his upper glans penis, and we detected pseudo hyphae and spores. Fungal culture and polymerase chain reaction (PCR) study were revealed as Candida albicans. He was successfully treated with topical isoconazole cream twice a day. Candida balanitis refers to candida infections of glans penis. Infectious balanitis presented various clinical presentations, it is not easy to diagnose with clinical presentation only. Therefore, it is essential to include other examination such as KOH examination, microorganism exam, and/or biopsy in the differential diagnosis when cutaneous lesions are noticed in patients. Herein, we present a candida balanitis with atypical features that clinically mimicked herpes genitalis.
Anti-Bacterial Agents ; Balanitis* ; Biopsy ; Candida albicans ; Candida* ; Diagnosis, Differential ; Herpes Genitalis* ; Humans ; Hyphae ; Male ; Middle Aged ; Penis ; Polymerase Chain Reaction ; Skin ; Spores ; Weights and Measures

Primary cutaneous anaplastic large cell lymphoma (PCALCL) is a rare primary cutaneous lymphoma that is predominantly composed of large lymphoid cells that express the CD30 antigen. The skin lesion of PCALCL is usually single, ulcerative, and located on the trunk or extremities and rarely the palm. A 25-year-old woman presented with a plaque on the left palm for 20 days. The plaque was walnut-sized and purple to gray colored with erosion in the center. Histopathologic examination showed infiltration of large atypical cells in the dermis. The large tumor cells showed positivity for CD3, CD4, and CD30 and negativity for CD8, CD20, epithelial membrane antigen, and anaplastic lymphoma kinase. PET-CT showed no other hypermetabolic lesion except that on the left palm, and we finally arrived at a diagnosis of PCALCL. The patient was treated with an intralesional injection of methotrexate (25 mg/mL, 0.45 cc). After 3 months of treatment, the walnut-sized plaque had disappeared and a peripheral hyperpigmented patch remained.
Adult ; Antigens, CD30 ; Dermis ; Diagnosis ; Extremities ; Female ; Humans ; Injections, Intralesional ; Lymphocytes ; Lymphoma ; Lymphoma, Large-Cell, Anaplastic ; Lymphoma, Primary Cutaneous Anaplastic Large Cell* ; Methotrexate ; Mucin-1 ; Phosphotransferases ; Skin ; Ulcer