Bone marrow transplantation has become an accepted treatment for malignancy(particulary leukemia and lymphoma), aplastic anemia, and certain inborn errors of metabolism. In addition to the problem of severe, prolonged myelosuppression, bone marrow transplantation is associated with several unusual complications. Among the complications such as GVHD, graft rejection, interstitial pneumonia and veno-occlusive disease, involvement of the gastrointestinal tract by GVHD is associated with high graft failure and mortality. Intestinal GVHD is usually manifest clinically as voluminous secretory diarrhea accompanied by abdominal cramping, ileus, nutritional depletion, and, at times, hemorrhage. We experienced a case of severe intestinal GVHD after allogeneic marrow transplantation for treatment of severe aplastic anemia. He received bone marrow from his elder sister, HLA-matched multiparous woman and suffered from large amount of watery diarrhea with skin rash 34 days after transplantation. 1n spite of prednisolone therapy the symptom was progressed. After sigmoidoscopic mucosal biopsy, intestinal GVHD was confirmed and we tried methylprednisolone pulse therapy. Skin lesion was improved but the amount of diarrhea was increased with intermittent abdominal cramping. We tried ALG(anti-lymphocyte globulin) and conservative management but the patient did not respond the therapy. He succumbed to pneumonia and acute respiratory insufficiency complicated with GVHD, 70days after transplantation.
Anemia, Aplastic* ; Biopsy ; Bone Marrow Transplantation* ; Bone Marrow* ; Colic ; Diarrhea ; Exanthema ; Female ; Gastrointestinal Tract ; Graft Rejection ; Hemorrhage ; Humans ; Ileus ; Leukemia ; Lung Diseases, Interstitial ; Metabolism, Inborn Errors ; Methylprednisolone ; Mortality ; Pneumonia ; Prednisolone ; Respiratory Insufficiency ; Siblings ; Skin ; Transplants

PURPOSE: Dendritic cells (DCs) are considered the most professional antigen-presenting cells (APCs) because of their unique role in initiating immunity against threatening antigens. Recently, hypertonicity has been suggested to be involved in the activation and development of immune cells such as T cells. And tonicity enhancer binding protein (TonEBP) has been thought to play a pivotal role in this process. Here, we studied the maturation status of DCs and expression of TonEBP in DCs exposed to hypertonic condition. METHODS: Murine bone marrow-derived DCs were generated in the presence of GM-CSF for 6 days, and then exposed to hypertonic media. We evaluated the functional capacities and maturation of DCs using flow cytometry, mixed lymphocyte reaction, and cytokine analysis. Also we investigated the expression of TonEBP in DCs cultured in variable hypertonic media. RESULTS: Mild hypertonicity made CD11c+ DCs to have up-regulation of CD40, 80, and 86. DCs exposed to 320 mOsm/kg media stimulated allogeneic T cell proliferation most effectively compared to DCs exposed to other tonic conditions. However, DCs exposed to 400 mOsm/kg media showed similar stimulatory capacities to isotonic control. Consistent with the phenotype changes, IL-1, 6, and TNF-alpha secretion increased in CD11c+ DCs exposed to mild hypertonic condition. Though we confirmed that TonEBP was expressed in CD11c+ DCs, the amount of upregulation was not dependent on the degree of hypertonicity. CONCLUSION: Our results suggest that hypertonicity enhances the maturation and activation of DCs.
Antigen-Presenting Cells ; Carrier Proteins ; Cell Proliferation ; Dendritic Cells ; Flow Cytometry ; Granulocyte-Macrophage Colony-Stimulating Factor ; Interleukin-1 ; Lymphocyte Culture Test, Mixed ; Osmolar Concentration ; Phenotype ; T-Lymphocytes ; Tumor Necrosis Factor-alpha ; Up-Regulation

BACKGROUND: Prediction of therapeutic response to radioactive iodine (RAI) in Graves disease is poorly understood. Although thyrotropin binding inhibitor immunoglobulin (TBII) level is a strong index for relapse after antithyroid drug treatment, conflicting results are described regarding its prognostic significance in Graves disease treated with RAI. This study is to evaluate possible prognostic factors including TBII wbich affect the outcome of RAI therapy in Graves disease. METHODS: Two hundred and one patients with Graves disease who were followed for over 12 months after RAI treatment were studied retrospectively. The subjects were divided into hypothyroid, euthyroid and hyperthyroid groups, based on the thyroid function evaluated at 12 months after RAI therapy. We evaluated the association of clinical parameters including patients age, goiter size, degree of hyperthyroidism and TBII index with outcome of RAI treatment. RESULTS: In Graves disease, response rate to RAI was 70.1% (hypothyroid 22.4% and euthyroid 47.7%) until 12th month. The mean age of hypothyroid group was 40+/-11 years, significantly older than that other groups (euthyroid: 33+/-12, hyperthyroid: 35+/-13, p<0.05). Initial level of thyroid function, duration of antithyroid drug treatment prior to RAI, goiter size and dosage of RAI were not significantly different between the groups. There were 61 patients who had both TBII tests before and after RAI. Twelve had negative TBII and 49 had positive TBII before RAI admini-stration. The rate of unremitted hyperthyroidism after RAI therapy was significantly lower in patients with negative TBII than in those with positive TBII prior to RAI treatment( 0% versus 46.9%, p<0.05). CONCLUSION: Graves patients with positive TBII prior to RAI therapy were associated with lower therapeutic response to RAI than those with negatve TBII. And old age was associated with the development of early hypothyroidism after RAI therapy. These results suggest these factors be also considered in the treatment of Graves disease with RAI.
Goiter ; Graves Disease* ; Humans ; Hyperthyroidism ; Hypothyroidism ; Immunoglobulins ; Iodine* ; Recurrence ; Retrospective Studies ; Thyroid Gland ; Thyrotropin

Autoimmune hepatitis (AIH) is a chronic, autoimmune disease of the liver that occurs when the body’s immune system attacks liver cells, causing the liver to be inflamed. AIH is one of the manifestations of a coronavirus disease 2019 (COVID-19), as well as an adverse event occurring after vaccination against severe acute respiratory syndrome coronavirus 2 (SARSCoV-2). Few cases of AIH have been described after vaccination with two messenger RNA (mRNA)-based vaccines—BTN162b2 (Pfizer-BioNTech) and mRNA-1273 (Moderna)—against SARS-CoV-2. Herein, we report a case of AIH occurring after Pfizer-BioNTech COVID-19 vaccine. A 27-year-old female presented with jaundice and hepatomegaly, appearing 14 days after receiving the second dose of Pfizer-BioNTech vaccine. Her laboratory results showed abnormal liver function with high total immunoglobulin G level. She was diagnosed with AIH with histologic finding and successfully treated with oral prednisolone. We report an AIH case after COVID-19 vaccination in Korea.

No abstract available.
Female ; Gonadotropin-Releasing Hormone* ; Pregnancy ; Pregnancy Outcome* ; Pregnancy*

Acute humoral rejection after renal transplantation is associated with a higher frequency of allograft dysfunction and graft loss. We report a case of acute humoral rejection which was treated successfully with plasmapheresis and intravenous immunoglobulin. A 31- year-old man developed azotemia after kidney transplantation. Kidney biopsy finding was compatible with antibody-mediated rejection, demonstrated by the infiltration of monocytes and neutrophils and the deposition of C4d on glomerulus and peritubular capillaries. We performed five plasmapheresis with concomitant treatment of intravenous immunoglobulin after each session. With aggressive treatment, there was improvement of oliguric acute renal failure, accompanied by decrease in the percentage of PRA and the titer of donor specific antibodies. Repeated kidney biopsy revealed persistent C4d staining on peritubular capillaries despite disappearance of donor specific antibodies. In conclusion, plasmapheresis and intravenous immunoglobulin are effective in treating acute humoral rejection.
Male ; Humans ; Biopsy

We report a case of microscopic polyangiitis, presenting with rapidly progressive glomerulonephritis, neuropsychiatric abnormalities, and urticarial vasculitis. A 65-year-old woman reported loss of appetite, significant weight loss, and a transient history of veering tendency. She was presented with a mild fever, cough, and sputum. Routine laboratory test revealed anemia, leukocytosis with a left shift, azotemia, and elevated highly sensitive C-reactive protein. The bilateral kidneys were observed to be enlarged (right kidney 16.3 cm, left kidney 18.2 cm) on an abdominal computed tomography. The perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) titer was >1:640 and MPO-ANCA was positive. Her chief complaints consisted of character change, visual illusion, and hearing loss. A skin rash with a bullous change resembling urticaria was further developed. Kidney biopsy demonstrated pauci-immune diffuse crescentic glomerulonephritis. Skin biopsy was compatible with urticarial vasculitis. Having received intravenous cyclophosphamide and oral prednisolone, she showed sustained improvement in renal function, as well as her neuropsychiatric symptoms and skin rash.
Aged ; Anemia ; Antibodies, Antineutrophil Cytoplasmic ; Appetite ; Azotemia ; Biopsy ; C-Reactive Protein ; Cough ; Cyclophosphamide ; Exanthema ; Female ; Fever ; Glomerulonephritis ; Hearing Loss ; Humans ; Illusions ; Kidney ; Leukocytosis ; Microscopic Polyangiitis ; Neuropsychology ; Prednisolone ; Skin ; Sputum ; Urticaria ; Vasculitis ; Weight Loss

No abstract available.
Craniocerebral Trauma* ; Head* ; Humans ; Tomography, Emission-Computed, Single-Photon*

A 68-year old man diagnosed with Middle East Respiratory Syndrome-Coronavirus (MERS-CoV) presented with multiple pneumonic infiltrations on his chest X-ray, and the patient was placed on a mechanical ventilator because of progressive respiratory failure. Urinary protein excretion steadily increased for a microalbumin to creatinine ratio of 538.4 mg/g Cr and a protein to creatinine ratio of 3,025.8 mg/g Cr. The isotope dilution mass spectrometry traceable serum creatinine level increased to 3.0 mg/dL. We performed a kidney biopsy 8 weeks after the onset of symptoms. Acute tubular necrosis was the main finding, and proteinaceous cast formation and acute tubulointerstitial nephritis were found. There were no electron dense deposits observed with electron microscopy. We could not verify the virus itself by in situ hybridization and confocal microscopy (MERS-CoV co-stained with dipeptidyl peptidase 4). The viremic status, urinary virus excretion, and timely kidney biopsy results should be investigated with thorough precautions to reveal the direct effects of MERS-CoV with respect to renal complications.
Aged ; Biopsy ; Coronavirus Infections/*diagnosis/virology ; Creatinine/blood/urine ; Dipeptidyl Peptidase 4/metabolism ; Humans ; In Situ Hybridization, Fluorescence ; Kidney/metabolism/*pathology ; Male ; Microscopy, Confocal ; Microscopy, Electron ; Middle East Respiratory Syndrome Coronavirus/*genetics/isolation & purification ; RNA, Viral/genetics/metabolism ; Reverse Transcriptase Polymerase Chain Reaction ; Serum Albumin/analysis

Seven hundred forty seven cases of gastrointestinal stromal tumors (GISTs) in Koreans who were diagnosed between 2001 and 2002 were analyzed to evaluate their occurrence and their clinical, pathologic and immunohistochemical findings. The most frequent location of tumor was in the stomach (63%), followed by the small intestine (30%), the colorectum (5%), and the esophagus (2%). c-kit expression was found in 93.6% of the cases, while CD34, SMA and S-100 protein was positive in 80.1%, 28.2%, and 20.2%, respectively. c-kit positivity was high in the stomach (94.2%) and small intestine (94.6%), while it was relatively low in the colorectum (85.0%), and esophagus (81.2%). The positivity for CD34 was correlated with the higher risk of GISTs (p=0.04). Follow up of the patients showed that 58 primary GISTs patients died and 20 of these patients were recurrent or metastatic at the time of diagnosis. The pathologic diagnosis to predict the risk of aggressive behavior of GISTs was correlated with the numbers of tumor, clinical stage, epithelioid histologic type, cellularity, cellular atypia, necrosis, and mucosal invasion (p= 0.00). GISTs with a poor prognosis were closely related to the clinical stage at presentation, the locations of the tumor, and the ages of the patients.
Actins/metabolism ; Adolescent ; Adult ; Aged ; Aged, 80 and over ; Antigens, CD34/metabolism ; Child ; Female ; Gastrointestinal Stromal Tumors/*epidemiology/metabolism/pathology ; Humans ; Immunohistochemistry ; Korea/epidemiology ; Male ; Middle Aged ; Prognosis ; Proto-Oncogene Proteins c-kit/metabolism ; Research Support, Non-U.S. Gov't ; S100 Proteins/metabolism