No abstract available.
Diagnosis* ; Multiplex Polymerase Chain Reaction* ; Muscular Dystrophy, Duchenne* ; Polymerase Chain Reaction*

Coxiella burnetii(C. burneii)was first recognized as the agent of Q fever in 1937. Q fever is an acute self-limited febrile illness. However, it manifests with several clinical symptoms depending upon the organs that are involved. The association of C. burnetii with human neoplasia has been rarely reported. We prospectively studied the 55 patients with fever of unknown origin, pneumonia, hepatosplenomegaly, lymphadenopathy, leukemia, lymphoma, and immunodeficiency and 14 persons who contacted the Q fever patients. The patient's sera were tested for antibodies specific for C. burnetii, using indirct fluorescent antibody techniques (IFA). 1) We serologically confirmed 23 C. burnetii infection. The 23 children with Q fever ranged in age from 0 to 15 years, with mean age of 4 years 11 months. Seventeen were boys and 6 were girls. 2) Characteristic symptoms and signs were fever (9/12 cases), rash (8/14 cases), hepatosplenomegaly (8/8 cases)and lymphadenopathy (14/27 cases). Five cases among 14 asymptomatic cases who contacted Q fever patients showed positive IFA test. One suffered from irregular uterine contraction, 4 weeks after contact with a Q fever patient. 3) There were no history of exposure to domestic animal carriers or contaminated dust, or drinking raw milk except one family. Three attending doctors and her father infected by a patient with Q fever. These suggested the person to person transmission of Q fever in a family and house staffs infected by a patient of Q fever. 4) Q fever (9 cases), acute lymphoblastic leukemia (2 cases), acute myelomonocytic leukemia (1 case), hairy cell leukemia (1case), Kawasaki disease (4 cases) and congenital dyserythropoietic anemia (1 case) showed positive IFA test. 5) Of 9 cases who suffered from lnly Q fever, 7 cases were confirmed hairy cell formation in their peripheral blood. One case was diagnosed as hairy cell leukemia after bone marrow study. Of 7 cases who showed hairy cells, all had hepatomegaly, 6 cases had lymphedenopathy and 5 cases showed splenomegaly. All except 1 case who was not followed cured after treatment. 6) We treated Q fever patients with rifampin and/or ciprofloxacin, and/or tetracyclin (over 8 year-old of age)for 2-4 weeks. One 25 month-old patient with hairy cell leukemia was treated with rifampin, ciprofloxacin and tetracyclin for 4 weeks, and rifampin for 8 months. A pregnant patient was administered with rifampin, and treated with rifampin and ciprofloxacin after delivery. We gave rifampin in one nweborn baby. In conclusion, we suggest that Q fever should be considered in the differential diagnosis of patients with FUO, hepatosplenomegaly and/or immunodeficiency.
Anemia, Dyserythropoietic, Congenital ; Animals, Domestic ; Antibodies ; Bone Marrow ; Child ; Child, Preschool ; Ciprofloxacin ; Coxiella burnetii* ; Coxiella* ; Diagnosis, Differential ; Drinking ; Dust ; Exanthema ; Fathers ; Female ; Fever ; Fever of Unknown Origin ; Fluorescent Antibody Technique ; Hepatomegaly ; Humans ; Internship and Residency ; Leukemia ; Leukemia, Hairy Cell ; Leukemia, Myelomonocytic, Acute ; Lymphatic Diseases ; Lymphoma ; Milk ; Mucocutaneous Lymph Node Syndrome ; Pneumonia ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Prospective Studies ; Q Fever* ; Rifampin ; Splenomegaly ; Uterine Contraction

No abstract available.

No abstract available.
Facial Bones* ; Humans

No abstract available.
Frontal Sinus*

Immunosuppressive medications and regimens have evolved with significant advancements in the understanding of the immunologic process after solid organ transplantation. Medications can block the communication between antigen-presenting cells and T-cells, the activation and proliferation of T-cells, antibody production by plasma cells, and the activation of the complement system by antibodies. T-cell depleting antibodies and interleukin-2 receptor blockers are commonly used during induction therapy. Calcineurin inhibitors, antimetabolites, antiproliferative agents, and corticosteroids are commonly used in maintenance therapy regimens. These medications decrease the rates of rejection episodes and markedly increase the survival rates of short-term grafts. However, in terms of the survival rate of long-term grafts, there is still room for improvement. Opportunistic infections, development of cancer, metabolic diseases, and calcineurin inhibitor toxicity are hurdles in the improvement in survival rates of long-term grafts. Therefore, many efforts are being taken to overcome these hurdles, such as the development of new drugs, individualization of immunosuppression, and induction of immune tolerance.

Paneth cell-rich carcinoma is essentially an adenocarcinoma with a predominance of Paneth cells. A 60-year-old male patient was admitted with a history of abdominal distension for several months. Endoscopic examination revealed a large ulceroinfiltrative tumor involving most of the areas of the stomach. The biopsy of the lesion confirmed poorly differentiated adenocarcinoma and total gastrectomy was followed. The submitted total stomach contained a diffuse infiltrative Borrmann type IV mass with ulceration, 8.0 3.5 cm, at the body along the lesser curvature. Microscopically, it was composed of Paneth cell differentiated cancer cells and poorly differentiated tubular adenocarcinoma cells. The Paneth cell differentiation was characterized by cytoplasmic coarse eosinophilic granules, which were PAS-positive and positive reaction for lysozyme. Electron microscopic examination showed numerous, spherical, electron-dense, homogeneous granules corresponding to those in Paneth cells as well as mucin granules in the signet-ring cells, and various intermediate forms in some cancer cells, which might be immature in the Paneth cell lineage.
Adenocarcinoma ; Biopsy ; Cell Differentiation ; Cell Lineage ; Cytoplasm ; Eosinophils ; Gastrectomy ; Humans ; Male ; Middle Aged ; Mucins ; Muramidase ; Paneth Cells ; Stomach* ; Ulcer

No abstract available.
Nasal Cavity* ; Neurilemmoma*

The papillary eccrine adenoma is a rare benign sweat gland neoplasm charscterized by potentislly locally aggressive clinical course. We report herein a case of papillsry eccrine adenoma in a 28-year-old female who presented single intradermal tumor on the right postauricular ares. Histopathological findings showed dilated ducts containing eosinophilic or keratinous materials and intraluminal papillary rowths.
Adenoma* ; Adult ; Eosinophils ; Female ; Humans ; Sweat Gland Neoplasms

We experienced an autopsy case of esophageal atresia with tracheoesophageal fistula and other multiple congenital anomalies in a 4 days old male infant. Esophageal atresia with tracheoesophageal fistula was type C by Gross Classification. Combined anomalies were malrotation of left kidney, one left accessory renal artery form right renal artery and one left accessory renal vein from right renal vein, duodenal atresia and annular pancreas. Until now, the esophageal atresia with tracheoesophageal fistula and other multiple congenital anomalies (7 congenital anomalies) are not reported in the korean literature, except our case. Our autopsy case, it suggests that esophageal atresia with tracheoesophageal fistula has often close relation-ship with other multiple congenital anomalies.
Infant ; Male ; Female ; Humans