Malakoplakia is a chronic inflammatory disorder that usually affects the urinary tract in immunocompromised patients and rarely extends to adjacent organs. Due to its mass-like presentation, malakoplakia is often clinically misdiagnosed as a neoplastic lesion. We describe the case of a 51-year-old female with renal malakoplakia and hepatic extension and large intraperitoneal abscesses that had been misdiagnosed as malignancy. She was diagnosed with myasthenia gravis 12 years prior and had been treated with oral corticosteroids and immunosuppressants. Radical nephrectomy concomitant with abscess drainage was performed. The final pathology was compatible with malakoplakia, and the patient was successfully treated with antibiotics. Although renal malakoplakia is a rare disease, it should be included in the differential diagnosis of patients with a renal mass who have a history of recurrent urinary tract infections or evidence of immunosuppression.
Abscess ; Adrenal Cortex Hormones ; Anti-Bacterial Agents ; Diagnosis, Differential ; Drainage ; Female ; Humans ; Immunocompromised Host ; Immunosuppression ; Immunosuppressive Agents ; Kidney ; Liver ; Malacoplakia ; Middle Aged ; Myasthenia Gravis ; Nephrectomy ; Rare Diseases ; Urinary Tract ; Urinary Tract Infections

Malakoplakia is a chronic inflammatory disorder that usually affects the urinary tract in immunocompromised patients and rarely extends to adjacent organs. Due to its mass-like presentation, malakoplakia is often clinically misdiagnosed as a neoplastic lesion. We describe the case of a 51-year-old female with renal malakoplakia and hepatic extension and large intraperitoneal abscesses that had been misdiagnosed as malignancy. She was diagnosed with myasthenia gravis 12 years prior and had been treated with oral corticosteroids and immunosuppressants. Radical nephrectomy concomitant with abscess drainage was performed. The final pathology was compatible with malakoplakia, and the patient was successfully treated with antibiotics. Although renal malakoplakia is a rare disease, it should be included in the differential diagnosis of patients with a renal mass who have a history of recurrent urinary tract infections or evidence of immunosuppression.
Abscess ; Adrenal Cortex Hormones ; Anti-Bacterial Agents ; Diagnosis, Differential ; Drainage ; Female ; Humans ; Immunocompromised Host ; Immunosuppression ; Immunosuppressive Agents ; Kidney ; Liver ; Malacoplakia ; Middle Aged ; Myasthenia Gravis ; Nephrectomy ; Rare Diseases ; Urinary Tract ; Urinary Tract Infections

PURPOSE: This study was carried out to investigate the awareness of the weaning of food using questionnaires, and the relationship with the weight gain in young infants. METHODS: From September 2005 to December 2005, we performed a survey on 141 guardians of young infants aged from 6 to 18 months, who visited the pediatric out-patient department at Dongguk University Medical Center. We calculated the total score for each responder from ten questions on the weaning of food and assessed the body weight percentile of each of the young infants. RESULTS: The most commonly reported information source for weaning food was 'the friends around' by 62 respondents (44.0%); 54 (38.3%) responded that the definition of weaning food was the preparatory step before starting a solid diet. Most used a spoon (90.8%) to feed when weaning food with a thin gruel of rice (78.7%). The time for weaning of food was before breast or infant formula feeding (55.3%). Addition of cow's milk was around 12 months (77.3%). The mean score was 6.21; however this did not show a statistically significant correlation with weight gain in young infants. CONCLUSION: The overall awareness of weaning of food has improved; however, information from doctors has decreased. Although the relationship between the awareness of weaning of food and the growth of young infants was not statistically significant, further studies on weaning of food, with larger and controlled sample sizes may provide important information.
Academic Medical Centers ; Body Weight ; Breast ; Surveys and Questionnaires ; Diet ; Friends ; Gyeongsangbuk-do* ; Humans ; Infant Formula ; Infant* ; Milk ; Outpatients ; Sample Size ; Weaning* ; Weight Gain

The complement system is composed of more than 25 different proteins and is usually divided into classical and alternative pathways. Complement component 7(C7) is one of the five terminal complement proteins that, upon activation of either the classical or the alternative pathway, interacts sequentially to form a large protein-protein complex, called membrane attack complex(MAC). Assembly of the MAC on target cells results in the formation of transmembrane pores that can lead to the killing of the cells. C7 deficiency is an autosomal recessive disorder that is mostly reported in Caucasians. The gene for C7 has been assigned to chromosome 5p13. To date, 15 different molecular defects leading to total or subtotal C7 deficient defects have been reported. C7 deficiency is associated frequently with recurrently bacterial infections, especially meningitis caused by Neisseria meningitidis. We report a case of a hereditary C7 deficiency associated with meningococcal meningitis.
Bacterial Infections ; Complement C7 ; Complement System Proteins ; Homicide ; Membranes ; Meningitis ; Meningitis, Meningococcal* ; Neisseria meningitidis

PURPOSE: The role of postoperative adjuvant chemo-radiotherapy in the treatment of patients with non-small cell lung cancer (NSCLC) remains unclear. This study was undertaken to evaluate the survival outcomes, relapse patterns, prognostic factors and complications of postoperative adjuvant MVP chemotherapy and radiotherapy. MATERIALS AND METHODS: The study involved some 96 patients who had undergone curative resection of stage II and III NSCLC between 1991 and 1996. Among these, 94 patients who completed their adjuvant treatment were analyzed. Surgery consisted of pneumonectomy (33%), single lobectomy (54%) or bilobectomy (13%). Within 4 weeks of curative resection, two cycles of MVP chemotherapy (mitomycin C 8 mg/m2, vinblastine 8 mg/m2, cisplatin 60 mg/m2) were started at 4 weeks intervals. Conventionally fractionated radiotherapy was given 3 weeks after chemotherapy to a total dose of 50 Gy in completely resected patients and 55~60 Gy in patients with positive resection margins. RESULTS: The TNM classification of the AJCC, as revised in 1997, was used for pathologic staging. The number of patients at AJCC stages IIa, IIb, IIIa, and IIIb were 4, 40, 45, and 5, respectively. A pathologically positive bronchial resection margin was found in nine patients. At the time of analysis, death was recorded in 29 patients (31%), though five had died without evidence of lung cancer. Overall 2-year, 3-year, and 5-year survival rates for all patients were 74.2%, 70.2%, and 65%, respectively, locoregional diseasefree survival (LRDFS) rates were 88.6%, 83.7%, 74.3%, at 2-years, 3-years, and 5-years, and distant metastasis disease-free survival (DMDFS) rates were 67.7%, 65.0%, and 63.6%, respectively. In the multivariate model, a primary tumor size of more than 5 cm and the level of pathologically positive nodes were found to be associated with poor overall survival, LRDFS and DMDFS. Although positive bronchial resection margin affected overall survival, LRDFS and DMDFS were unaffected. With respect to the first site of relapse, distant metastasis occurred more frequently (N=33, 35%) than locoregional recurrence (N=15, 16%). Grade 3 esophagitis in two patients and weight loss of more than 10% in five patients were observed during adjuvant treatment. Grade 4 pulmonary toxicity was observed in one patient after radiotherapy and this patient ultimately died 5 months after treatment. CONCLUSION: The postoperative adjuvant MVP chemotherapy and radiotherapy regimen showed relatively low locoregional recurrence and distant metastasis rates and good survival rate with acceptable toxicity. A prospective randomized trial, which compares this regimen to surgery alone or postoperative adjuvant radiotherapy is needed.
Carcinoma, Non-Small-Cell Lung* ; Chemotherapy, Adjuvant* ; Cisplatin ; Classification ; Disease-Free Survival ; Drug Therapy ; Esophagitis ; Humans ; Lung Neoplasms ; Neoplasm Metastasis ; Pneumonectomy ; Prospective Studies ; Radiotherapy* ; Radiotherapy, Adjuvant ; Recurrence ; Survival Rate ; Vinblastine ; Weight Loss

Treatment-related myelodysplastic syndrome (t-MDS) and acute myelogenous leukemia (t-AML) are now well established as complications of cytotoxic chemotherapy. We experienced a 28-yr-old female patient who developed t-MDS/t-AML with characteristic chromosomal abnormalities including 11q23 chromosomal rearrangement following high-dose chemotherapy with autologous stem cell transplantation (ASCT) for non-Hodgkin's lymphoma. The patient was admitted with bulky abdominal masses of B cell lineage non-Hodgkin's lymphoma. After 2 cycles of systemic chemotherapy of the Vanderbilt regimen, the patient underwent ASCT with high dose chemotherapy of the BEAC regimen. She also received radiation of 48 Gy for the residual periportal lymphadenopathy. The initial cytogenetic analysis of the infused mononuclear cells revealed a normal karyotype. Twenty two months after the ASCT, pancytopenia was noted and her bone marrow aspirate showed dysplastic hemopoiesis with myeloblasts up to 12% of nonerythroid nucleated cells. The patient was diagnosed as t-MDS (refractory anemia with an excess of blasts). Cytogenetic analysis showed complex chromosomal abnormalities including 11q23 rearrangement, which is frequently found in topoisomerase II inhibitor-related hematologic malignancies. Four months later, it was noted that the t-MDS had evolved into an overt t-AML. Cytogenetic analysis showed an evolving pattern with more complex abnormalities. The patient was treated with combination che-motherapy, but her leukemic cells were resistant to the therapy.
Adult ; Antineoplastic Agents, Phytogenic/adverse effects ; Antineoplastic Combined Chemotherapy Protocols/*adverse effects ; B-Lymphocytes/cytology ; Bone Marrow Cells/pathology ; Carmustine/*adverse effects ; Chromosome Aberrations ; Chromosomes, Human, Pair 11 ; Combined Modality Therapy/adverse effects ; Cyclophosphamide/*adverse effects ; Cytarabine/*adverse effects ; Etoposide/*adverse effects ; Female ; Gene Rearrangement ; Hematopoietic Stem Cell Transplantation/*adverse effects ; Humans ; Leukemia, Myeloid, Acute/*etiology/genetics ; Lymphoma, Non-Hodgkin/*therapy ; Myelodysplastic Syndromes/*etiology/genetics ; Neoplasms, Second Primary/*etiology ; Pelvis ; Pregnancy ; Pregnancy Complications, Neoplastic/*therapy ; Transplantation, Autologous

BACKGROUND: New therapeutic modalities such as high dose chemotherapy and stem cell support have been tried to prolong the survival period of the patients with multiple myeloma. However, little is known about the criteria for the application of those new therapies. There are only a few reports for the prognostic factors of multiple myeloma in Korea. The purpose of this study is to analyze the prognostic factors affecting chemotherapy response and survival in patients with multiple myeloma. METHODS: We retrospectively analysed the clinical records of 122 patients who were newly diagnosed as multiple myeloma by SWOG criteria, between November, 1989 and April, 1997 at Asan Medical Center. RESULTS: 1) The peak incidence was the 7th decade and male to female ratio was 1.3:1. The most common presenting symptom at first diagnosis was bone pain. 2) Initial clinical stage was as followed: stage I in 17.2% , stage II in 16.4% and 66.4% in III. The immunoglobulin classes were IgG in 51.6%, light chain only in 25.4%, IgA in 16.4%, IgD in 4.1%, and non-secretory type in 2.5%. Plasma cell types in bone marrow were classified as plasmablastic type in 45.9%, plasmacytic type in 54.1%. 3) Eighty two patients who recieved chemotherapy more than 3 cycles were evaluable for chemotherapy response. Overall response rate was 69.5%. Factors affecting response to chemotherapy were serum creatinine level, plasma cell type, total plasma cell percentage and plasmablast percentasge of total nucleated cells in bone marrow. 4) For total 122 patients, overall median survival period was 21 months, and estimated 5 year survival rate was 23.5%. Factors affecting survival were serum creatinine, corrected calcium, albumin, beta2-microglobulin level, response to chemotherapy, total plasma cell percentage and plasmablast percentage in bone marrow. CONCLUSION: Bone marrow findings at initial diagnosis are significantly associated with response to chemotherapy and survival duration.
Bone Marrow ; Calcium ; Chungcheongnam-do ; Creatinine ; Diagnosis ; Drug Therapy* ; Female ; Humans ; Immunoglobulin A ; Immunoglobulin D ; Immunoglobulin G ; Immunoglobulin Isotypes ; Incidence ; Korea ; Male ; Multiple Myeloma* ; Plasma Cells ; Retrospective Studies ; Stem Cells ; Survival Rate

Myelodysplastic syndrome is a closely related group of acquired bone marrow disorders characterized by ineffective and dysplastic hematopoiesis. These clonal disorders frequently progress to acute leukemia. Acute myelomonocytic leukemia with eosinophilia is characterized by an increase in abnormal eosinophils in the bone marrow, relatively good clinical course and inv (16) chromosomal abnormality. We experienced one case of refractory anemia with excess blasts which progressed to refractory anemia with excess blasts in transformation and finally to acute myelomonocytic leukemia with eosinophilia showing peculiar chromosomal abnormalities of der (1;7).
Adult ; Anemia/pathology ; Anemia/genetics ; Anemia/etiology ; Bone Marrow/pathology ; Case Report ; Chromosomes, Human, Pair 16* ; Disease Progression ; Eosinophilia/pathology ; Eosinophilia/genetics* ; Eosinophilia/etiology ; Human ; Inversion (Genetics)* ; Karyotyping ; Leukemia, Myelocytic, Acute/pathology ; Leukemia, Myelocytic, Acute/genetics* ; Leukemia, Myelocytic, Acute/etiology ; Male ; Myelodysplastic Syndromes/pathology ; Myelodysplastic Syndromes/genetics* ; Myelodysplastic Syndromes/complications

we studied the 12 patients, operated only sagittal split ramus osteotomy without genioplasty or maxillary osteotomy in department of oral and maxillofacial surgery, Hanyang university hospital from 1996.1. 1. to 1998. 7. 20. Preoperative and postoperative cephalometric view was measured to know the change of upper lip position and shape after mandibular setback. The result were obtained as follows. 1. The ratio of upper lip change amount to lower incisor horizontal movement was 15.1%. 2. The ratio of lower facial profile between Sn-Stm and Stm-Mes was changed from 1 : 2.352 to 1 : 2.069 after operation. 3. Post-operative upper lip was flattened 72.4% compared with pre-opreative one. 4. The vermilion zone of the upper lip increased 56% horizontally, 5.8% vertically after operation. 5. The vermilion zone ratio of the lower lip to the upper lip was change from 1 : 1.253 to 1 : 1.348. 6. The distance between esthetic line and Ls was changed from -3.958mm to -1.15mm.]]>
Genioplasty ; Humans ; Incisor ; Lip ; Maxillary Osteotomy ; Orthognathic Surgery ; Osteotomy ; Osteotomy, Sagittal Split Ramus ; Prognathism ; Surgery, Oral

BACKGROUND: Essential thrombocythemia (ET) is a rare chronic myeloproliferative disorder characterized by an extremely high platelet count in the circulating blood and abnormal proliferation of the megakaryocytes in bone marrow, resulting in splenomegaly, thromboembolic or hemorrhagic complications. We studied the presence of nuclear hyperploidy of the megakaryocytes in bone marrow, the presence of abnormal response to the individual reagent on platelet aggregation test, and its clinical implication. METHODS: We analyzed the 43 cases of ET at the Asan Medical Center between January, 1989 and March, 1999. The Polycythemia Vera Study Group criteria were used to diagnose ET. RESULTS: Nuclear hyperploidy was observed at 43 cases (100%). Platelet aggregation test was done at 32 (74.4%) cases, of which 27 (84.4%) cases showed abnormal response to more than one reagent, 16 (50%) cases to more than two reagents. Abnormal response to epinephrine and collagen was most common, but 5 cases showed normal response. By individual reagent, 1 (3%) cases to adenosine diphosphate, 1 (3%) case to ristocetin, 22 (69%) cases to epinephrine, 19 (59%) cases to collagen showed abnormal response. CONCLUSION: We observe that nuclear hyperploidy of the megakaryocyts and abnormal response on platelet aggregation test are frequent in ET in this study.
Adenosine Diphosphate ; Blood Platelets* ; Bone Marrow ; Chungcheongnam-do ; Collagen ; Epinephrine ; Indicators and Reagents ; Megakaryocytes* ; Myeloproliferative Disorders ; Platelet Aggregation* ; Platelet Count ; Polycythemia Vera ; Ristocetin ; Splenomegaly ; Thrombocythemia, Essential*