Tracheobronchial aspergillosis is an unusual form of invasive aspergillosis characterized by noninvasive or only superficially invasive tracheobronchitis with a propensity for dissemination. We report a two-year-old male who suddenly died of respiratory failure. Postmortem examination revealed a pseudomembrane covering the mucosa of larynx, trachea and bronchial tree of both lungs. This pseudomembrane was composed predominantly of Aspergillus hyphae. There was transmural necrotizing bronchitis with fungal invasion to the narrow zone of peribronchial tissue, and dissemination to the stomach and kidney. This form of pulmonary aspergillosis had not been reported in this country.
Aspergillosis* ; Aspergillus ; Autopsy* ; Bronchitis ; Humans ; Hyphae ; Kidney ; Larynx ; Lung ; Male ; Mucous Membrane ; Pulmonary Aspergillosis ; Respiratory Insufficiency ; Stomach ; Trachea

No abstract available.
Pneumonia*

Wilms' tumor is one of the most common primary malignant tumors of the kidney during infancy and childhood and is known to be originated from the primitive cells of metanephric blastema. It presents difficulties when encountered in deciding the presence of anaplasia or in differentiating it from other renal tumors of childhood with different biologic behavior because of its diverse histologic patterns and varying degrees of differentiation. Evaluation of clinical and histopathologic features in terms of prognostication was done of 32 cases of Wilms' tumor which were surgically resected and diagnosed in the period from January 1979 through June 1992. Immunohistochemical reaction for cytokeratin, vimentin, actin and desmin was also analysed on all cases of Wilms' tumor in conjunction with clear cell sarcoma of the kidney(CCSK), malignant rhabdoid tumor of the kidney(MRTK) and congenital mesoblastic nephroma(CMN) to assess the validity of immunohistochemistry in differentiating Wilms' tumor from these renal tumors. Twenty four(75%) cases were diagnosed before the age of 5 and 40.7% were under 2 years old. Mixed type was most common(62.5%), followed by epithelial, blastemal and stromal predominant type in descending order of frequency. Anaplasia was observed in 3 cases(9.4%), two of which were epithelial predominant type and one blastemal predominant type. Treatment modality and presence of anaplasia were significantly correlated with 5 year survival rate of patients. Immunohistochemical stain revealed that all epithelial component of Wilms' tumor were positive for cytokeratin and 56.3% of Wilms' tumor had blastemal component which were positive for both cytokeratin and vimentin. Twenty cases(62.5%) of Wilms' tumor had blastemal component which were positive for cytokeratin with a proportion of more than 5% of reactive cells. Stromal component of Wilms' tumor generally did not show differentiation into the specialized type of tissue and all revealed positive reactions for vimentin among which some revealed positive reactions for actin. Only 3 out of 6 cases with rhabdomyoblastic differentiation were positive for desmin. CCSK, MRTK and CMN which have different biologic behavior and treatment modality compared to Wilm's tumor showed positivity only for vimentin and/or actin. In summary, treatment modality and presence of anaplasia are significantly correlated with patients' survival and the immunohistochemical stain for cytokeratin is very helpful in confirming the presence of blastemal component and useful in the differential diagnosis of Wilms' tumor from other kinds of pediatric renal tumors.
Child ; Male ; Female ; Humans ; Diagnosis, Differential

BACKGROUND: 4-1BB mediated costimulatory signal is a recently identified immunotherapeutic strategy for treating autoimmune diseases without depressing the immune response. In this study, we investigated the expression of 4-1BB and 4-1BBL on the peripheral blood mononuclear cells (PBMC) and we assessed whether the serum levels of soluble (s) 4-1BB and s4-1BBL in the patients with Graves' disease (GD) and compared them with normal subjects. METHODS: Expression of 4-1BB and 4-1BBL on PBMC of GD patients was determined by flow cytometry. The concentrations of s4-1BB and s4-1BBL were assessed in the sera of GD patients by performing ELISA. RESULTS: 4-1BB was constitutively expressed on naive CD4+ and CD8+ T cells of the GD patients and this was increased by stimulation. 4-1BBL was also expressed on the antigen-presenting cells such as CD19+ B cells, monocytes and dendritic cells in GD patients. The serum levels of s4-1BB and s4-1BBL were significantly higher in GD patients than those in controls, and these levels were significantly correlated with the serum levels of thyroid-binding inhibitory immunoglobulin and free T4. CONCLUSION: These results indicate that effector T cells of GD patients can be activated through the 4-1BB-mediated costimulatory signal. Elevated s4-1BB and s4-1BBL levels in the sera of GD patients may affect modulation of the clinical course in GD patients.
Antigen-Presenting Cells ; Autoimmune Diseases ; B-Lymphocytes ; Dendritic Cells ; Enzyme-Linked Immunosorbent Assay ; Flow Cytometry ; Graves Disease ; Humans ; Immunoglobulins ; Monocytes ; T-Lymphocytes

PURPOSE: Narcolepsy is a specific neurologic syndrome consisting of excessive daytime drowsiness, cataplexy, and the additional phenomena of sleep paralysis and intense dreamlike hallucinations at sleep-wake transitions. We are reporting a patient with narcolepsy whose repeated self-injury caused traumatic eyeball perforation. METHODS: A 26-year-old male with narcolepsy injured himself, which resulted in eyeball perforation in his both eyes. We diagnosed him as traumatic corneal laceration, traumatic cataract, and vitreous opacity due to lens material in vitreous in both eyes. RESULTS: After improvement of narcoleptic symptom and healing of corneal perforation, we performed cataract extraction and pars plana vitrectomy under local anesthesia. During operation, we found preretinal gliosis at superior portion of optic disc and subretinal hemorrhage in the right eye. We observed successful improvement in visual acuity through intraocular lens implantation.
Adult ; Anesthesia, Local ; Cataplexy ; Cataract ; Cataract Extraction ; Corneal Perforation ; Gliosis ; Hallucinations ; Hemorrhage ; Humans ; Lacerations ; Lens Implantation, Intraocular ; Male ; Narcolepsy ; Sleep Paralysis ; Sleep Stages ; Visual Acuity ; Vitrectomy

Abrupt increase in the size of cervico-mediastinal tumor due to infection or spontaneous hemorrhage into cyst can induce severe tracheal compression and therefore sudden death. A 5 year old boy, who had a history of URI, had an enlarging cystic hygroma on the right side of the neck and anterior mediastinum. Under diagnosis of the cervico-mediastinal cystic hygroma, surgical removal was scheduled. After induction of anesthesia, intubation was done without any difficulty. A few minutes later, signs of partial airway obstruction were appeared. And within a very short period, total airway occlusion occurred. The tracheal tube was removed and manual ventilation was performed with positive airway pressure, but ineffective. We attempted to puncture cricothyroid membrane with 14 Gauge needle in order to ventilate manually. As soon as we puncture cricothyroid membrane, straw-colored fluid, not air, gushed out through a needle. After aspiration of about 200ml of cystic fluid, the obstructive signs disappeared and the patency of the airway was maintained. Intraoperatively, no more airway problems occured and vital signs were stable. And postoperatively, patient had no specific complications and discharged on the 7th day after operation.
Airway Obstruction* ; Anesthesia ; Child, Preschool ; Death, Sudden ; Diagnosis ; Hemorrhage ; Humans ; Intubation ; Intubation, Intratracheal* ; Lymphangioma, Cystic* ; Male ; Mediastinum ; Membranes ; Neck ; Needles ; Punctures ; Ventilation ; Vital Signs

Therapeutic irradiation can induce angiosarcoma. Radiation-induced angiosarcoma constitutes 20% of all angiosarcomas. Although its common site of origin is the skin and subcutaneous tissue, it rarely arises in small or large bowels with a presentation as multifocal abdominal angiosarcomatosis. We report a case of intra-abdominal angiosarcomatosis involving the jejunum, ileum, transverse colon, mesentery and right ovary in a 63-year-old female. It developed 10 years after therapeutic irradiation for squamous cell carcinoma of uterine cervix. She developed panperitonitis due to intestinal perforation. She died from sepsis 3 days after segmental resection of the small bowel and right oophorectomy. We reviewed the previously reported cases and describe the clinicopathologic features of this tumor.
Carcinoma, Squamous Cell ; Cervix Uteri ; Colon, Transverse ; Female ; Hemangiosarcoma ; Humans ; Ileum ; Intestinal Perforation ; Jejunum ; Mesentery ; Middle Aged ; Ovariectomy ; Ovary ; Sepsis ; Skin ; Subcutaneous Tissue

No abstract available.
Hypertension ; Leigh Disease*

Urinary tract anomaly is the most common anomaly in childhood. We reviewed medical records of 45 patients who were confirmed urinary tract anomalies on radiologic studies as Chuncheon Sacred Hospital, from Dec. 1984 to Sep. 1992. We analyzed incidence and clinical characteristics of urinary tract anomalies. The results obtained were as follows: 1) The age distribution were: 1)7 cases (37.8%) under 3 years, 6 cases (13.3%) from 4 to 6 years, 10 cases (22.2%) from 7 to 9 years, 9 cases (20.0%) from 10 to 12 years and 3 cases (6.7%) from 13 to 15 years. 2) Main symptoms were fever, dysuria and hematuria. 3) 26 cases (59.8%) were combined with urinary tract infection, and main causative organism were E. coli and Staphylococcus. 4) The most common anomalies were hydronephrosis, duplication of urinary tract and renal agenesis in order of sequence. 5) 34 cases (75.6%) were simple anomaly and 11 cases (24.4%) were complex anomalies. 6) Operative correction was needed on 20 cases.
Age Distribution ; Dysuria ; Fever ; Gangwon-do ; Hematuria ; Humans ; Hydronephrosis ; Incidence ; Medical Records ; Staphylococcus ; Urinary Tract Infections ; Urinary Tract*

No abstract available.
Carcinoma, Renal Cell* ; Genes, Tumor Suppressor*