Multiple endocrine neoplasia type 1 (MEN 1) is an autosomal dominant disorder characterized by two or more tumors of the parathyroid gland, duodenum-pancreas, and anterior pituitary. Membranous nephropathy is the most common manifestation of paraneoplastic glomerulopathy. However, minimal change disease in patients with MEN 1 has yet to be reported. Here, we report a case of minimal change disease in a 59-year-old man with MEN 1, along with a review of the relevant literature.

Branchio-oto-renal (BOR) syndrome is a rare autosomal dominant disorder that is characterized by preauricular pits, branchial fistula, branchial cyst, hearing impairment, and kidney anomalies. Hearing impairment is the single most common feature of BOR syndrome, affecting 89% of patients. Preauricular pits (77%), kidney anomalies (66%), branchial fistula (63%), external auditory canal anomalies (41%) are also common. For most patients, BOR syndrome does not affect life expectancy. The major life-threatening feature of this condition is kidney dysfunction, which occurs with about 6% of kidney anomalies. Therefore, once BOR syndrome is recognized in a patient, careful evaluation to detect renal anomalies and treatment of any kidney involvement are necessary. No case reports of BOR syndrome involving adult-onset end-stage kidney disease have been published in the Korean medical literature. We report a case of end-stage kidney disease in a 19-year-old male patient with BOR syndrome, together with a review of the pertinent literature.
Branchio-Oto-Renal Syndrome* ; Branchioma ; Ear Canal ; Fistula ; Hearing Loss ; Humans ; Kidney ; Kidney Failure, Chronic ; Life Expectancy ; Male ; Renal Insufficiency* ; Young Adult

Henoch-Schönlein purpura (HSP) is the most common vasculitis in children, mainly affecting the small vessels of the skin, joints, gastrointestinal tract, and kidneys. Although most cases of HSP resolve spontaneously without sequelae, serious nephrological and intestinal problems may occur in some cases. We experienced a case of HSP complicated by simultaneous intussusception and nephritis in a 14-year-old boy who developed a sudden abdominal pain and gross hematuria on the 11th day after onset of the disease. Imaging studies revealed intussusception that required emergency laparotomy. Despite treatment with steroid and angiotensin-converting enzyme inhibitors, nephritis and nephrosis progressed for 4 weeks, and renal biopsy was performed to confirm the diagnosis. Cyclosporin A therapy was started, and remission of proteinuria was achieved after 5 months. However, the nephritis recurred and worsened to end-stage renal failure during 15 years of follow-up.
Abdominal Pain ; Adolescent ; Angiotensin-Converting Enzyme Inhibitors ; Biopsy ; Child ; Cyclosporine ; Diagnosis ; Emergencies ; Follow-Up Studies ; Gastrointestinal Tract ; Hematuria ; Humans ; Intussusception* ; Joints ; Kidney ; Kidney Failure, Chronic ; Laparotomy ; Male ; Nephritis* ; Nephrosis ; Proteinuria ; Purpura* ; Renal Insufficiency ; Skin ; Vasculitis

Adrenocortical carcinoma (ACC) is a rare disease with poor prognosis. We experienced a case of recurrent ACC with stomach metastasis which had been completely cured a long while ago. A 52-year-old man who presented with right thigh pain was hospitalized. We found a lumbar spine mass on magnetic resonance imaging scan. And this lesion was identified as metastatic adrenocortical carcinoma. The patient had been treated as stage II non-functioning ACC 19 years ago. At that time radical resection and adjuvant chemotherapy were successfully done. And the follow-up evaluation was discontinued, since it had been checked as no evidence of disease (NED) state for 5 years. But this time, there were multiple metastatic sites revealed in positron emission tomography-computed tomography scan including stomach. Therefore, we report a case of ACC herewith that it could be recurred even though long-term NED state was passed after treatment and stomach could be a metastatic site of ACC.
Adrenocortical Carcinoma* ; Chemotherapy, Adjuvant ; Electrons ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Neoplasm Metastasis* ; Prognosis ; Rare Diseases ; Recurrence ; Spine ; Stomach* ; Thigh

It is known that rheumatoid arthritis (RA) patients show increased incidence of multiple myeloma (MM), despite its rarity. Only one case of MM with seronegative RA was reported in Korea, thus far. We report a case of MM with seropositive RA. The patient was a 66 year old female who had been diagnosed with seropositive RA 4 years ago. Over the last 1 month, the patient experienced general weakness and weight loss of 10 kg. It was found that her serum creatinine had increased and her urine analysis showed proteinuria. To evaluate renal failure and proteinuria, renal biopsy, bone marrow biopsy and electrophoresis were carried out. A diagnosis of myeloma cast nephropathy was made. We report this rare case of MM represented as acute renal failure during the treatment for RA, and include a review of the literature.
Acute Kidney Injury* ; Arthritis, Rheumatoid* ; Biopsy ; Bone Marrow ; Creatinine ; Diagnosis ; Electrophoresis ; Female ; Humans ; Incidence ; Korea ; Multiple Myeloma* ; Proteinuria ; Renal Insufficiency ; Weight Loss

Sarcoidosis, systemic inflammatory disease characterized by non-caseating granulomas, is rarely associated with renal failure in a kidney transplant. We report a 51-year-old woman with a kidney transplant who was diagnosed to have renal sarcoidosis. After 7 years of renal transplantation, the patient presented with relatively rapid deterioration of renal function and, subsequently, she underwent kidney transplant biopsy. Renal biopsy revealed interstitial nephritis with non-caseating granulomas compatible with granulomatous interstitial nephritis (GIN). She was also found to have granulomatous lymphadenitis and skin lesions. Diagnosis of sarcoidosis was made based on histopathologic findings, the high serum angiotensin converting enzyme level and exclusions of other causes of GIN including tuberculosis, ANCA associated glomerulonephritis and tubulointerstitial nephritis and uveitis syndrome. The patient was started on oral prednisolone, and subsequently her renal function improved.
Antibodies, Antineutrophil Cytoplasmic ; Biopsy ; Female ; Glomerulonephritis ; Granuloma ; Humans ; Kidney ; Kidney Transplantation ; Lymphadenitis ; Nephritis, Interstitial ; Peptidyl-Dipeptidase A ; Prednisolone ; Renal Insufficiency ; Sarcoidosis ; Skin ; Transplants ; Tuberculosis ; Uveitis

PURPOSE: Neoadjuvant chemotherapy is the standard treatment for patients with locally advanced breast cancer and is increasingly considered for patients with operable disease. Recently, as many clinical trials have demonstrated favorable outcomes of anthracycline-taxane based regimen, this approach has been widely used in the neoadjuvant setting. METHODS: We compared women who received adriamycine and docetaxel (AD) with adriamycin, cyclophosphamide followed by paclitaxel (AC-T) as neoadjuvant chemotherapy. The AD group was scheduled for six cycles of AD (50 mg/m2 and 75 mg/m2, respectively) at a 3-week interval. The AC-T group was scheduled for four cycles of adriamycin and cyclophosphamide (50 mg/m2 and 500 mg/m2, respectively) followed by four cycles of paclitaxel (175 mg/m2) at a 3-week interval. RESULTS: The responses of chemotherapy were equivalent (overall response rate [AD, 75.7% vs. AC-T, 80.9%; P = 0.566], pathologic complete response [pCR] rate [breast and axilla: AD, 10.8% vs. AC-T, 12.8%; P = 1.000; breast only: AD, 18.9% vs. AC-T, 14.9%, P = 0.623], breast conserving surgery rate [P = 0.487], and breast conserving surgery conversion rate [P = 0.562]). The pCR rate in the breast was higher in the human epidermal growth factor receptor 2 (HER2) positive cases (HER2 positive 33.3% vs. negative 10%, P = 0.002). Although nonhematologic toxicities were comparable, hematologic toxicities were more severe in the AD group. Most women in the AD group suffered from grade 3/4 neutropenia (P < 0.001) and neutropenic fever (P < 0.001). CONCLUSION: Tumor responses were not different in various variables between the two groups. However, AC-T was a more tolerable regimen than AD in patients with breast cancer receiving neoadjuvant chemotherapy.
Breast ; Breast Neoplasms ; Cyclophosphamide ; Doxorubicin ; Female ; Fever ; Humans ; Mastectomy, Segmental ; Neoadjuvant Therapy ; Neutropenia ; Paclitaxel ; Polymerase Chain Reaction ; Receptor, Epidermal Growth Factor ; Receptor, erbB-2 ; Taxoids

Granulocytic sarcoma is a localized extramedullary solid tumor composed of immature myeloid cell and is usually associated with acute myeloid leukemia or myelodysplastic syndrome. Although it can involve any site, commonly in lymph nodes, skin, bone and soft tissue, the involvement of breast is unusual. Especially, the involvement of the breast as a pattern of relapse after bone marrow transplantation is extremely rare. We have experienced 2 cases of granulocytic sarcoma after bone marrow transplantation. One case was a 39-year-old woman with right breast mass diagnosed with granulocytic sarcoma. She had received an unrelated bone marrow transplantation due to biphenotype acute leukemia 3 years before our presentation. Another case was a 48-year-old woman with acute myeloid leukemia, who was diagnosed with granulocytic sarcoma on both breasts 8 months after allogenic bone marrow transplantation. We also discuss the clinicopathologic features of granulocytic sarcoma in breast after bone marrow transplantation.
Bone Marrow ; Bone Marrow Transplantation ; Breast ; Female ; Humans ; Leukemia ; Leukemia, Myeloid, Acute ; Lymph Nodes ; Myelodysplastic Syndromes ; Myeloid Cells ; Recurrence ; Sarcoma, Myeloid ; Skin

BACKGROUND: Underweight refers to the weight range in which health risk can increase, since the weight is lower than a healthy weight. Negative attitudes towards obesity and socio-cultural preference for thinness could induce even underweight persons to attempt weight control. This study was conducted to investigate factors related to weight control attempts in underweight Korean adults. METHODS: This was a cross-sectional study on 690 underweight adults aged 25 to 69 years using data from the Korea National Health and Nutrition Examination Survey, 2007-2010. Body image perception, weight control attempts during the past one year, various health behaviors, history of chronic diseases, and socioeconomic status were surveyed. RESULTS: Underweight women had a higher rate of weight control attempts than underweight men (25.4% vs. 8.1%, P < 0.001). Among underweight men, subjects with the highest physical activity level (odds ratio [OR], 7.75), subjects with physician-diagnosed history of chronic diseases (OR, 7.70), and subjects with non-manual jobs or other jobs (OR, 6.22; 12.39 with reference to manual workers) had a higher likelihood of weight control attempts. Among underweight women, subjects who did not perceive themselves as thin (OR, 4.71), subjects with the highest household income level (OR, 2.61), and unmarried subjects (OR, 2.08) had a higher likelihood of weight control attempts. CONCLUSION: This study shows that numbers of underweight Korean adults have tried to control weight, especially women. Seeing that there are gender differences in factors related to weight control attempts in underweight adults, gender should be considered in helping underweight adults to maintain a healthy weight.
Adult* ; Body Image ; Body Weight ; Chronic Disease ; Cross-Sectional Studies ; Family Characteristics ; Female ; Health Behavior ; Humans ; Korea* ; Male ; Motor Activity ; Nutrition Surveys* ; Obesity ; Single Person ; Social Class ; Thinness* ; Weight Loss ; Weight Perception

PURPOSE: Idiopathic granulomatous mastitis (IGM) is a rare chronic inflammatory disease of unknown etiology. The diagnosis of IGM requires that other granulomatous lesions in the breast be excluded. Tuberculous mastitis (TM) is also an uncommon disease that is often difficult to differentiate from IGM. The purpose of this study is to develop a new algorithm for the differential diagnosis and treatment of IGM and TM. METHODS: Medical records of 68 patients (58 with IGM and 10 with TM) between July 1999 and February 2009 were retrospectively reviewed. RESULTS: The mean age of the patients was 33.5 (IGM) and 40 (TM) years (p=0.018). The median follow-up was 84 months. Of the total 10 patients with TM, 5 patients had a history of pulmonary tuberculosis. The most common symptoms of the diseases were breast lump and pain. However, axillary lymphadenopathy was more seen in TM (50%) compared to IGM (20.6%) (p=0.048). TM showed more cancer-mimicking findings on radiologic study (p=0.028). In IGM, 48 patients (82.7%) underwent surgical wide excision and 21 patients (36.2%) were managed with corticosteroid therapy and antibiotics. All of the TM patients received anti-tuberculosis medications and 9 patients (90%) underwent wide excision. The mean treatment duration was 2.8 months in IGM and 8.4 months in TM. Recurrence developed in 5 patients (8.6%) in IGM and 1 patient (10%) in TM. CONCLUSION: This study shows different characteristics between IGM and TM. The IGM patients were younger and had more mastalgia symptoms than the TM patients. Axillary lymphadenopathy was seen more often in TM patients. Half of the TM patients had pulmonary tuberculosis or tuberculosis lymphadenitis. Surgical wide excision might be both therapeutic and useful for providing an exact diagnosis.
Anti-Bacterial Agents ; Breast ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Granulomatous Mastitis ; Humans ; Immunoglobulin M ; Lymphadenitis ; Lymphatic Diseases ; Mastitis ; Mastodynia ; Medical Records ; Recurrence ; Retrospective Studies ; Tuberculosis ; Tuberculosis, Pulmonary