Acute interstitial pneumonia is a fulminant disease of unknown etiology that usually occurs in a previously healthy person and produces the histologic findings of the organizing phase of diffuse alveolar damage. We experienced an autopsy case of acute interstitial pneumonia of unknown etiology. The patient was a 48 year old man who had been healthy and had not been exposed to organic dusts or other toxic materials. The chief complaints represented were dyspnea and a dry cough for several weeks before hospitalization, and the chest radiographs showed bilateral interstitial infiltrates. Patchy consolidation of air space was also identified and ground-glass attenuation similar to those described in ARDS was detected on high-resolution computed tomography. Steroid pulse therapy, mechanical ventilation, and antibiotics for superimposed bacterial infection were performed, but the symptoms did not improve and the patient died of generalized respiratory insufficiency and severe hypoxemia 2 1/2 months after hospitalization. At autopsy the macroscopic and microscopic findings were confined mainly to the lungs. On the whole, both lungs were firm in consistency and the external surface showed a cobblestone appearance. The cut surface showed almost complete replacement of the normal lung parenchyma with gray to yellow fibrous tissue with a little residual functional area remaining. The pathology of both open lung biopsy and autopsy tissue showed marked hyperplasia of type II pneumocytes, hyaline membrane formation, thickening of the alveolar wall due to extensive fibroblast proliferation, and relatively abundant young collagen deposition in the interstitium. An immunohistochemical stain for cytokeratin revealed epithelial hyperplasia and showed that the alveolar spaces were markedly shrunken by fibrous tissue.
Anoxia ; Anti-Bacterial Agents ; Autopsy* ; Bacterial Infections ; Biopsy ; Collagen ; Cough ; Dust ; Dyspnea ; Fibroblasts ; Hospitalization ; Humans ; Hyalin ; Hyperplasia ; Keratins ; Lung ; Lung Diseases, Interstitial* ; Membranes ; Middle Aged ; Pathology ; Pneumocytes ; Pulmonary Fibrosis ; Radiography, Thoracic ; Respiration, Artificial ; Respiratory Insufficiency

Schinzel-Giedion syndrome is a rare, distinct dysmorphic syndrome characterized by congenital hydronephrosis, skeletal dysplasia, and severe developmental retardation, likely to be inherited as an autosomal recessive trait, but not yet confirmed. This syndrome is characterized by coarse facial features such as midfacial retraction, bulging forehead, short nose with anteverted nostrils, low-set malformed ears, protruding large tongue, and hypertelorism. Skeletal and limb defects, choanal stenosis, simian creases, hypospadias, microphallus, hypertrichosis, and intractable seizures are the frequently associated clinical findings. Urogenital involvement is a major component of the syndrome, and this problem sometimes is associated with nephrocalcinosis and urinary tract infection in the clinical course of the disease. We report a 22 month-old girl with Schinzel-Giedion syndrome complicated by medullary nephrocalcinosis and urinary tract infection due to Klebsiella pneumoniae. This patient had also been suffering from postnatal growth deficiency, intractable seizure, spastic tetraplegia, delayed development and severe mental retardation.
Constriction, Pathologic ; Ear ; Extremities ; Female ; Forehead ; Humans ; Hydronephrosis ; Hypertelorism ; Hypertrichosis ; Hypospadias ; Infant ; Intellectual Disability ; Klebsiella pneumoniae ; Male ; Nephrocalcinosis ; Nose ; Quadriplegia ; Seizures ; Tongue ; Urinary Tract Infections

PURPOSE: Long-term prognosis of Henoch-Schonlein purpura(HSP) is determined by the existence and severity of renal involvement. We evaluated the relationship between various clinical features of HSP and the development of renal involvement using univariate and multivariate analyses for early detection and proper management of HSP nephritis. METHODS: We performed a retrospective study of 200 children who were diagnosed as HSP and were initially free from renal involvement, from 1998-2003. Clinical features of each patient as risk factors of renal involvement were evaluated by univariate and multivariate analysis. Significant risk factors proven by univariate analysis were paired and re-analyzed to see if there are any interactions between them. RESULTS: A univariate analysis showed that sex, previous disease, severe abdominal symptoms, persistent purpura and steroid administration were associated with developing renal involvement. Among them, severe abdominal symptoms, persistent purpura, and male sex were statistically significant when they were re-analyzed after pairing, and they also increased the risk of renal involvement in a multivariate analysis. CONCLUSION: Severe abdominal symptoms, persistent purpura, and male sex are independent risk factors of renal involvement in HSP and it is presumed that interaction between them could be another factor of HSP nephritis.
Abdominal Pain ; Child ; Humans ; Male ; Multivariate Analysis* ; Nephritis ; Prognosis ; Purpura ; Purpura, Schoenlein-Henoch* ; Retrospective Studies ; Risk Factors*

PURPOSE: Obesity has become a pandemic and especially the prevalence of childhood obesity has increased remarkably over the past years. Childhood obesity is often accompanied by the expected morbidities such as hyperlipidemia, fatty liver and glucose intolerance. We evaluated the pattern of alteration in the body mass index (BMI), serum adiponectin concentration, insulin resistance (HOMA-IR), and complication rates after one year follow-up. METHODS: Forty two obese children (age: 8-15 years, 95th percentile of BMI for age and sex) were examined twice annually on June, 2004 and July, 2005. The body mass index (BMI) and sexual maturity rating (SMR) were determined annually by the same examiner and serum fasting glucose, liver enzyme, lipid profiles, adiponectin, insulin and HOMA-IR were measured and annual results were compared. Upper abdominal sonography was performed to detect fatty liver. RESULTS: BMI (25.3+/-2.9 kg/m2 vs. 26.0+/-2.9 kg/m2) and HOMA-IR (3.6+/-1.8 vs. 4.7+/-1.9) have increased significantly after one-year follow-up. The serum adiponectin concentration (17.2+/-5.2 g/mL vs. 12.6+/-5.2 g/mL) has decreased after one year. The prevalence of hyperlipidemia and fatty liver were not changed significantly but that of hyperinsulinemia increased after one-year. HOMA-IR showed a positive correlation with BMI and SMR, but was negatively correlated with serum adiponectin concentration. CONCLUSION: Obese children in our study showed significant increment of insulin resistance during one year. These results suggest that well-organized and continuous obesity control is required especially for obese adolescent to prevent complication of obesity.
Adiponectin* ; Adolescent* ; Body Mass Index ; Child* ; Fasting ; Fatty Liver ; Follow-Up Studies* ; Glucose ; Glucose Intolerance ; Humans ; Hyperinsulinism ; Hyperlipidemias ; Insulin Resistance* ; Insulin* ; Liver ; Obesity ; Pandemics ; Pediatric Obesity ; Prevalence

Eosinophilic gastroenteritis is an unusual disease characterized by eosinophilic infiltration of gastrointestinal tract, peripheral eosinophilia, and gastrointestinal symptoms. There are mucosal, muscular, and subserosal types, of which the subserosal type is the rarest. We experienced a 32-year old male patient with the subserosal type of eosinophilic gastroe-nteritis, who was presented with abdominal distension. The ascitic fluid revealed increased eosinophil count. With oral prednisolone, diarrhea and ascites rapidly disappeared and eosinophils in peripheral blood returned to normal. We report this case with a review of relevant literature.
Adult ; Ascites* ; Ascitic Fluid ; Diarrhea ; Eosinophilia ; Eosinophils* ; Gastroenteritis* ; Gastrointestinal Tract ; Humans ; Male ; Prednisolone

PURPOSE: Unilateral ureteral obstruction(UUO), a well established experimental model of renal injury, gives rise to tubulointerstitial fibrosis, tubular dilatation and cellular atrophy. Angiotensin(ANG) II may take the prime role in the regulation of this response. The objectives in the current investigation were to determine whether the renal response to UUO involves the dedifferentiation of tubular epithelial cells to mesenchymal cells(expressing vimentin, V) whether the response implicates the transformation of fibroblasts to myofibroblasts(expressing alpha-smooth muscle actin, SMA), and whether these responses depend on the action of ANG II or not. METHODS: Unilateral ureteral ligation and sham operations were performed in 12 adult male Sprague-Dawley rats. An additional 18 rats received exogenous ANG II at 50 ng/min or vehicle for one week using an osmotic minipump inserted into the interscapular area. Rats were sacrificed on postoperative day seven or day 14. To know the expression of vimentin(V) and alpha-smooth muscle actin (SMA) proteins, immunohistochemical staining and Western blot assay were done. RESULTS: In immunohistochemical staining, following UUO, V-positive cells appeared markedly in the interstitium and tubular cells within dilated tubules. UUO also markedly increased alpha-SMA expression in the interstitium surrounding dilated tubules. In Western blotting, UUO increased V(five times of Sham) and alpha-SMA(2.5 times of Sham) expression. ANG II infusion increased alpha-SMA significantly(two times of control), but not V expression in Western blotting. CONCLUSION: Phenotypic transformation of fibroblasts to myofibroblasts following UUO may depend on ANG II, but dedifferentiation of tubular epithelial cells may depend on other mechanisms rather than ANG II.
Actins ; Adult ; Angiotensin II* ; Angiotensins* ; Animals ; Atrophy ; Blotting, Western ; Dilatation ; Epithelial Cells* ; Fibroblasts* ; Fibrosis ; Humans ; Ligation ; Male ; Models, Theoretical ; Myofibroblasts ; Rats* ; Rats, Sprague-Dawley ; Ureter* ; Ureteral Obstruction* ; Vimentin

PURPOSE: In preterm infants with brain injury, periventricular echodensity is increased transiently or becomes cyst with neurologic sequelae. This study was done to identify the infants who need early rehabilitation treatment by evaluating the risk factors and neurologic outcome in cystic change group and transient echodensity group. METHODS: Thirty one preterm infants with periventricular echodensity on cranial ultrasonography were divided into cystic change group and transient echodensity group. The prenatal, natal, postnatal risk factors and neurologic outcome were evaluated in each group. RESULTS: The risk factors for periventricular cystic change included gestational age, ventilator use, oxygen administration, surfactant use and anemia. The risk factors for transient periventricular echodensity were the same as the cystic change group except gestational age. The development of cerebral palsy occurred in 3 cases out of 10 cases, 2 cases out of 21 cases in each group. CONCLUSION: The preterm infants with periventricular cysts or transient periventricular echodensity need to be observed closely for the development of adverse neurological outcomes especially of those with aforementioned risk factors.
Anemia ; Brain Injuries ; Cerebral Palsy ; Gestational Age ; Humans ; Infant ; Infant, Newborn ; Infant, Premature* ; Oxygen ; Rehabilitation ; Risk Factors* ; Ultrasonography ; Ventilators, Mechanical

PURPOSE: In preterm infants with brain injury, periventricular echodensity is increased transiently or becomes cyst with neurologic sequelae. This study was done to identify the infants who need early rehabilitation treatment by evaluating the risk factors and neurologic outcome in cystic change group and transient echodensity group. METHODS: Thirty one preterm infants with periventricular echodensity on cranial ultrasonography were divided into cystic change group and transient echodensity group. The prenatal, natal, postnatal risk factors and neurologic outcome were evaluated in each group. RESULTS: The risk factors for periventricular cystic change included gestational age, ventilator use, oxygen administration, surfactant use and anemia. The risk factors for transient periventricular echodensity were the same as the cystic change group except gestational age. The development of cerebral palsy occurred in 3 cases out of 10 cases, 2 cases out of 21 cases in each group. CONCLUSION: The preterm infants with periventricular cysts or transient periventricular echodensity need to be observed closely for the development of adverse neurological outcomes especially of those with aforementioned risk factors.
Anemia ; Brain Injuries ; Cerebral Palsy ; Gestational Age ; Humans ; Infant ; Infant, Newborn ; Infant, Premature* ; Oxygen ; Rehabilitation ; Risk Factors* ; Ultrasonography ; Ventilators, Mechanical

PURPOSE: Primary breast lymphoma is a very rare disease, accounting for 0.4-0.5% of all breast malignancies. Due to the rarity, there are only limited reports of this disease in Korean women. In this reason, we report the experience of a single institution in Korea with primary breast lymphoma (PBL). METHODS: We retrospectively reviewed the medical records of 9 patients with PBL and evaluated the clinicopathologic characteristics and treatment outcomes. RESULTS: All nine patients were female and had diffuse large B-cell lymphoma (DLBL). The median age at diagnosis was 47.9 years and the median tumor size was 3.8 cm in diameter. The most common symptom was a painless palpable mass. Five patients were classified as stage IEA and four patients were IIEA according to the Ann Arbor staging system. Four patients underwent excisional biopsy and one patient underwent a lumpectomy with sentinel lymph node biopsy due to uncertain histology of the preoperative core needle biopsy. Nine patients received anthracycline containing combined chemotherapy; among them, five patients were treated with a rituximab containing regimen. Four patients received radiotherapy combined with chemotherapy. A complete response was achieved in eight patients. During the 44 months of the median follow-up period, three cases of relapse occurred, and among them, two patients died due to disease progression. CONCLUSION: Most PBLs are B-cell origin, with DLBL being the most common histologic type. A combined treatment modality has been known to have positive effects on prognosis, and surgery should be limited to a diagnostic purpose.
Accounting ; Antibodies, Monoclonal, Murine-Derived ; B-Lymphocytes ; Biopsy ; Biopsy, Large-Core Needle ; Breast ; Combined Modality Therapy ; Female ; Follow-Up Studies ; Humans ; Korea ; Lymphoma ; Lymphoma, B-Cell ; Mastectomy, Segmental ; Medical Records ; Nitriles ; Prognosis ; Pyrethrins ; Rare Diseases ; Recurrence ; Retrospective Studies ; Rituximab ; Sentinel Lymph Node Biopsy ; Treatment Outcome

Sequential lymphoma is defined as two different types of lymphoma that occur in the same patient at different anatomic sites and times. In most cases, the two distinct histologies belong to the same lineage (B- or T-cell lymphoma), though cases with both have been observed. A few cases of Epstein-Barr virus (EBV)-positive diffuse large B-cell lymphomas arising in patients with angioimmunoblastic T cell lymphoma (AITL) have been reported. Immune deficits inherent in AITL, combined with the immunosuppressive effects of the therapy, may have allowed unchecked EBV-induced proliferation of latently or newly EBV-infected B cells with eventual clonal selection and progression to aggressive B-cell lymphoma. Here, we report a case of AITL in which EBV-positive diffuse large B-cell lymphoma (DLBCL) arose 9 months after the initial diagnosis of AITL.
B-Lymphocytes ; Herpesvirus 4, Human ; Humans ; Lymphoma ; Lymphoma, B-Cell ; Lymphoma, T-Cell ; T-Lymphocytes