• Journal of Chest Surgery
• /
• v.17 no.1
• /
• pp.150-156
• /
• 1984

Paraesophageal hiatus hernia represents a rare hiatal hernia that are treated surgically. The completely asymptomatic paraesophageal hernia often does not reach the clinician or surgeon. But the presence of a symptomatic paraesophageal hernia is sufficient indication for surgery. The paraesophageal hernia may be approached either transthoracically or transabdominally. The general technique is essentially the same, whichever route is used. From either transthoracic or transabdominal approach, following reduction of the viscus and elimination of the sac, the diaphragmatic opening is then closed with interrupted heavy dacron or silk sutures in paraesophageal hiatus hernia. But if the phrenoesophageal membrane often is destroyed when the esophagogastric junction and the distal esophagus have been mobilized, it becomes important to fix the esophagogastric junction below the diaphragm, so that it does not slide through the hiatus and produce a sliding-type hiatus hernia in future. We have experienced one case of paraesophageal hiatus hernia which was accompanied with severe anemia in child. We preferred to approach through left thoracotomy incision and then pushed down the stomach into the abdominal cavity with complete excision of the hernial sac. We employed Belsey Mark V procedure using of Teflon felt pledgets with the mattress sutures against development of sliding-type hiatus hernia in postoperative period. postoperative course has been uneventful and good for about 3 months to this time.

• Biomedical Science Letters
• /
• v.15 no.2
• /
• pp.161-166
• /
• 2009

Human erythropoietin(EPO) is a glycoprotein that enhances red blood cell production by stimulating proliferation and differentiation of erythroid progenitor cells in the bone marrow. Patients with chronic kidney disease(CKD) suffer from anemia caused by reduced production of EPO in the kidney. Recombinant human EPO protein has been used successfully for the treatment of anemia associated with CKD. Recently, attention has been paid to the development of side effect of EPO, pure red cell aplasia(PRCA), in some patients with CKD. PRCA is a rare disorder of erythropoiesis that leads to a severe anemia due to an almost complete cessation of red blood cell production. EPO-related PRCA is caused by the production of EPO-neutralizing antibodies(Abs) that eliminate the biological activity of EPO as well as endogenous EPO in patients undergoing therapy. Since 1988, almost 200 cases worldwide have been reported with Ab-positive PRCA after receiving EPO therapeutics. The underlying mechanisms of the breaking of immune tolerance to self-EPO have been investigated. Modification of formulation, organic compounds of container closures, and route of administration has been suggested for the possible mechanism of increased immunogenicity of EPO. A number of assays have been used to detect Abs specific to EPO. These assays are generally grouped into two major categories: binding Ab assays and neutralizing Ab assays(bioassays). There are several types of binding Ab assays, including radioimmunoprecipitation assay, enzyme-linked immunosorbent assay, and the BIAcore biosensor assay. In vitro cell-based bioassays have been utilized for the detection of neutralizing Abs. Finally, the recent experience with anti-EPO Abs may have considerable implications for the future development and approval of EPO preparations. Also, considering that millions of patients are being treated with EPO, clinicians need to be aware of signs and consequences of this rare but severe clinical case.

• Radiation Oncology Journal
• /
• v.30 no.4
• /
• pp.165-172
• /
• 2012

Purpose: To retrospectively evaluate the outcome and toxicity of total lymphoid irradiation (TLI) based conditioning regimen for allogeneic hematopoietic stem cell transplantation (HSCT) in severe aplastic anemia (SAA) patients who experienced an engraftment failure from prior HSCT or were heavily transfused. Materials and Methods: Between 1995 and 2006, 20 SAA patients received TLI for conditioning of HSCT. All patients were multi-transfused or had long duration of disease. Fifteen (75%) patients had graft failure from prior HSCT. In 18 (90%) patients, the donors were human leukocyte antigen identical siblings. The stem cell source was the peripheral blood stem cell in 15 (75%) patients. The conditioning regimen was composed of antithymocyte globulin plus TLI with a median dose of 750 cGy in 1 fraction. The graft-versus-host disease (GVHD) prophylaxis used cyclosporine with methotrexate. Results: With a median follow-up of 10.8 years, graft failures developed in 6 patients. Among them, 3 patients received their third HSCT to be engrafted finally. The Kaplan-Meier overall survival rate was 85.0% and 83.1% at 5 and 10 years, respectively. The incidence of acute and chronic GVHD was 20% and 20%, respectively. None of the patients have developed a malignancy after HSCT. Conclusion: In our study, TLI based conditioning in allogeneic HSCT was feasible with acceptable rates of GVHD in SAA patients who experienced graft failure from prior HSCT or was at a high risk of graft rejection. We achieved relatively better results of engraftment and survival with a long term follow-up.

• Clinical and Experimental Pediatrics
• /
• v.58 no.6
• /
• pp.199-205
• /
• 2015

Severe aplastic anemia (SAA) is a life-threatening disorder for which allogeneic hematopoietic stem cell transplantation (HSCT) is the current available curative treatment. HSCT from matched sibling donors (MSDs) is the preferred therapy for children with acquired SAA. For patients who lack MSDs, immunosuppressive therapy (IST) is widely accepted as a first-line treatment before considering HCT from an unrelated donor (URD). Given the recent progress in HSCT using URDs for childhood SAA, well-matched URDs became a realistic alternative for pediatric patients who have no suitable related donors and who are refractory to IST. However, it is quite challenging to treat patients with refractory SAA who lack suitable related or URDs. Even though haploidentical HSCT from genetically mismatched family members seemed to be an attractive procedure with the amazing benefit of readily available donors for most patients, early attempts were disappointing because of refractory graft-versus-host disease (GVHD) and excessively high transplant-related mortality. Recent advances with effective ex vivo depletion of T cells or unmanipulated in vivo regulation of T cells, better supportive care, and optimal conditioning regimens have significantly improved the outcome of haploidentical transplant. Besides considerable progress in the treatment of malignant diseases, recent emerging evidences for haploidentical HSCT in SAA has provided additional therapeutic options for patients with refractory diseases. Further improvements to decrease the rates of graft failure, GVHD, and infectious complications will facilitate the emergence of haploidentical HSCT as a front-line therapy for treating acquired SAA in children and adolescents who have no suitably matched donors.

• Clinical and Experimental Pediatrics
• /
• v.58 no.7
• /
• pp.267-269
• /
• 2015

Antithymocyte globulin (ATG) is used as an immunosuppressive treatment (IST) to deplete clonal suppressor T cells in patients with severe aplastic anemia (SAA). The depletion of suppressor T cells by ATG may affect the activation of B cells, which results in an increased risk for autoimmune conditions. A 12-year-old boy was diagnosed with idiopathic SAA. As he did not have an human leukocyte antigen-matched sibling, he was treated with rabbit ATG (3.5 mg/kg/day for 5 days) and cyclosporine. Five months later, he became transfusion independent. However, 23 months after IST, he complained of mild hand tremors, sweating, weight loss, palpitations, and goiter. Results of thyroid function tests revealed hyperthyroidism (free thyroxine, 3.42 ng/dL; thyroid stimulating hormone [TSH], <0.01 nIU/mL; triiodothyronine, 3.99 ng/mL). Results of tests for autoantibodies were positive for the antimicrosome antibody and TSH-binding inhibitory immunoglobulin, but negative for the antithyroglobulin antibody and antinuclear antibody. He was treated with methimazole, and his symptoms improved. The patient has been disease free for 39 months after IST and 9 months after methimazole treatment. This case report suggests that although rare, rabbit ATG may have implications in the pathogenesis of autoimmune hyperthyroidism. Our findings suggest that thyroid function tests should be incorporated in the routine follow-up of SAA patients treated with ATG.

• Journal of The Korean Dental Society of Anesthesiology
• /
• v.13 no.4
• /
• pp.203-207
• /
• 2013

Aplastic anemia (AA) is a serious hematologic disease characterized by hypocellular bone marrow and deficient production of erythrocytes, granulocytes and platelets. Serious complications such as uncontrolled bleeding and bacteremia can occur. A case of severe AA are presented with dental considerations. A 4-year-old boy had been referred from Seoul National University Hospital for dental examination before the hematopoietic stem cell transplantation (HSCT). Treatments were planned under general anesthesia, due to the poor compliance. Following medical consult, dental treatments were performed after platelet transfusion and antibiotic prophylaxis. Postoperatively, neither significant bleeding nor complictation was observed. On the time of the treatment planning. the anesthesiologist and dentist should perform a complete hematological assessment. It is imperative not only platelet counts but also other leukocyte counts are under safe boundaries. It is mandatory to follow strict aseptic precautios for all anesthetic and surgical maneuvers. In severe thrombocytopenic patients, platelet transfusion should be considered. Also, it is recommended to establish a good oral hygiene.

• Biomedical Science Letters
• /
• v.1 no.1
• /
• pp.81-88
• /
• 1995

RDW and MCV are thought to be the highly sensitive blood cell parameters in the differentiation of iron deficiency anemias. Through the medical records of 227 anemic patients and the physical checking results of 143 healthy persons in a General Hospital during the recent five years, the authors evaluated various blood cell parameters including RDW and MCV. Iron deficiency anemia, aplastic anemia and other anemias associated with chronic disease were shown as the three major causes of anemias in Korea. In the patients of iron deficiency anemia MCV was very low(62.9$\pm$13.7fl), while RDW was very high(19.3$\pm$4.8) showing much lower MCV and much higher RDW in severe IDA compared with in mild IDA. To differentiate iron deficiency anemias form other anemias, a discriminant function was developed from some blood cell parameters like MCV, MCH, MCHC, RDW and platelets(D.F.=0.26-0.012MCV -0.130MCH +0.073MCHC +0.052RDW+0.003PLT).

• Journal of Periodontal and Implant Science
• /
• v.28 no.1
• /
• pp.187-191
• /
• 1998

Aplastic anemia is a disease characterized by general lack of bone marrow activity; It may affect not only the red blood cells but also the white blood cells and platelets, resulting in pancytopenia. Spontaneous gingival hemorrhage is present in some cases and it is related to the blood platelet deficiency. This case report presents the periodontal treatment of a patient with aplastic anemia. A 43-year-old female was referred for continuous gingival bleeding after periodontal treatment. Periodontal findings revealed generalized gingival imflammation, oozing of blood from gingival crevice, and it was diagnosed as adult periodontitis. Root planing and extraction of the upper left third molar with poor prognosis were put into operation after elevation of the platelet count with platelet transfusion. The extraction socket was sutured with 3-0 silk. Bleeding continued even after digital compression at the upper right second premolar, second molar, and left canine areas, which presented severe inflammation. Although platelets were transfused repeatedly, platelet count did not stay elevated since survival rate of the transfused platelets were low due to alloimmunization. Thrombin gauze packing was not effective. Bleeding ceased 3 days after treatment with transfusion of donor platelets. 20 days after the treatment, the gingiva was generally healthy except upper right second premolar and lateral incisor areas. The result of periodontal treatment was good, but bleeding control after treatment was troublesome. In the periodontal treatment of patient with aplastic anemia, elevation of the platelet count with platelet transfusion seems to be the best method for hemorrhage control.

• Journal of Chest Surgery
• /
• v.21 no.4
• /
• pp.706-710
• /
• 1988

Intravascular hemolysis occurs in the majority of patient with mechanical valve prosthesis. The primary cause is mechanical trauma to red cells from turbulent blood flow through the prosthesis. Degree of hemolysis is dependent upon the type, size and material of valve and aggravated by paravalvular leakage. Clinically important hemolytic anemia is required medical management or consideration of reoperation. In severe hemolysis, reoperation is recommended without delay when seems to be renal failure. In this case, postoperative severe mechanical hemolysis was developed immediately after aortic valve replacement with St. Jude medical valve in a 13 year-old male patient. Neither significant paravalvular leakage nor valvular dysfunction was found through redo, but the mechanical valve was strongly suspected the cause of severe hemolysis. The St. Jude Medical valve was changed with Ionescu-Shiley bioprosthesis and any significant clinical problems were not noted through the postoperative course.

• Journal of Chest Surgery
• /
• v.29 no.4
• /
• pp.444-448
• /
• 1996

Hemolytic anemia due to tiny prosthetic paravalvular leakage is one of a complication of prosthetic valve replacement. Mild Hemolysls usually occurs after aortic valve replacement with mechanical valve but rarely occurs in mitral valve position especially in case of tissue valve. Cardiac valves fabricated from biologic material are associated with a reduced incidence of hemolytic anemia. Hemolysis was reported in patients with an lonescu-Shiley bovine pericardial xenograft prosthesis in the aortic position but not in the mitral site. A 41-year-old female patient was admitted due to sudden development dark colored urine. About 10 years ago the patient was underwent MVR (Mitral Valve Re lacement) with fTmm lonescu-Shiley valve due to MR (Mitral regurgitation). Echocardiographic examination showed mild degree of mitral regurgitation with valvular thickening. However, there was no definitive evidence of paravalvular leakage. The peripheral blood smear showed nomochromic normocytic anemia, but the hematologic and urinary examination revealed severe hemolytic evidence. Mitral valve replacement with St. Jude Medical valve (27mm) was done and intraoperatively, a tiny paravalvular leakage was found which was regarded as the point of hemolysis. The hemolytic evidence completely disappeared. We are reporting a case of severe hemolytic anemia due to tiny prosthetic paravalvular leakage with a review of the literature.