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Biomedical subjects

H C Kim

Publications and source records attributed to H C Kim.

At least 19 recordsLinked to original sources
PubMed1992

A modified transfusion program for prevention of stroke in sickle cell disease.

Regular red blood cell transfusions reduce the rate of recurrent cerebral infarction in sickle cell disease but lead to accumulation of excessive iron. We studied the effect on the prevention of recurrent stroke and the volume of blood transfused of a modified transfusion program in which the pretransfusion percentage of hemoglobin S (HbS) was maintained at 50%, rather than the conventional 30%. Fifteen patients with sickle cell disease and cerebral infarction who had been free of recurrent stroke for at least 4 years during which the pretransfusion HbS was maintained below 30% were assigned to a transfusion program in which the HbS was allowed to increase to 50%. Transfusion regimens included simple transfusion and manual and automated partial exchange transfusion. The duration of follow-up was 14 to 130 months with a median duration of 84 months. None of the 15 patients had a recurrent cerebral infarction during 1,023 patient-months in which the target pretransfusion HbS was 50%. Analysis of this finding, using a binomial distribution, indicates that there is less than a 5% chance that the risk per patient of recurrent stroke in the first year of the modified transfusion program is greater than 18%. One 23-year-old patient had a fatal intraventricular hemorrhage when the HbS was 30% and a 21-year-old patient had a fatal subarachnoid hemorrhage in the 40th week of pregnancy when the HbS was 29%. Blood requirements with simple transfusions decreased by 17% to 48% (mean 31%) when the target pretransfusion HbS level was increased from 30% to 50% (P less than .001). Manual or automated partial exchange transfusions and a target HbS level of 50% in eight patients reduced blood requirements by 33% to 99% (mean 67%) in comparison with simple transfusion and a target HbS level of 30% (P less than .001). This study offers evidence that a target pretransfusion HbS level of 50% affords a continuing high rate of protection against recurrent cerebral infarction in sickle cell disease after 4 years of a conventional transfusion program. Increasing the target HbS level from 30% to 50% provides a major reduction in blood requirements and lowers the rate of iron accumulation.

Adolescent↗
PubMed1992

Purified factor IX using monoclonal immunoaffinity technique: clinical trials in hemophilia B and comparison to prothrombin complex concentrates.

Replacement therapy for hemophilia B (factor IX deficiency) using prothrombin complex concentrate (PCC) has been associated with serious complications of thromboembolic events and transmission of viral infections. Monoclonal antibody-purified factor IX (Mononine) provides a highly purified factor IX concentrate, while eliminating other vitamin K-dependent factors (II, VII, and X). Mononine was evaluated for in vivo recovery, half-life, and for its safety and efficacy in 10 patients with hemophilia B. The in vivo recovery of factor IX with Mononine was a 0.67 +/- 0.14 U/dL (mean +/- SD) increase per 1U/kg of infused factor IX, and the biologic half-life (t1/2), determined using the terminal phase of elimination, was 22.6 +/- 8.1 hours. Comparison of in vivo recovery of other vitamin K-dependent factors following a single infusion of either Mononine or PCC showed that, whereas Mononine infusion caused no changes in other vitamin K-dependent factors or in prothrombin activation fragment (F1+2), PCC infusion was associated with significant increases of factors II (2.7 U/dL per 1 U/dL of IX increase) and X (2.2 U/dL for 1 U/dL for 1 U/dL of IX). Patients who used Mononine as their sole therapeutic material during the 12-month period showed an excellent response in hemostasis for their bleeding episodes. Their experience with long-term use of Mononine was at least equivalent to their previous experience with PCC in the frequency and amount of factor usage. No patients developed antibody against mouse IgG or an increase in IX inhibitor during the 12-month period. These results indicate that monoclonal antibody-purified factor IX concentrate provides hemostatically effective factor IX replacement while avoiding extraneous thrombogenic substances.

Antibodies, Monoclonal↗
PubMed1992

Childhood-onset familial porphyria cutanea tarda: effects of therapeutic phlebotomy.

Cutaneous fragility at age 2 years with blistering, scarring, milia, and hypertrichosis at age 4 years were noted in an otherwise healthy girl who had no family history of porphyria. Results of porphyrin analyses of urine, serum, and red blood cells revealed a pattern consistent with porphyria cutanea tarda. Red blood cell uroporphyrinogen decarboxylase activity was diminished to approximately 50% of normal in the child and in her mother and maternal grandmother, who were without symptoms; activity was normal in her sister, father, and maternal grandfather. Therapeutic phlebotomies were followed by a biochemical and clinical remission.

Bloodletting↗
PubMed1992

Therapeutic effect of transcatheter oily chemoembolization therapy for encapsulated nodular hepatocellular carcinoma: CT and pathologic findings.

An emulsion of iodized oil and doxorubicin hydrochloride was intraarterially injected in 27 patients with encapsulated single nodular hepatocellular carcinoma (HCC). Computed tomography (CT) was performed 1-3 weeks after injection, and the lesions were resected 1-4 weeks thereafter. The percentages of tumor necrosis were evaluated in cut surfaces of resected specimens and were compared with the findings at iodized-oil CT. Six tumors with complete intratumor retention of iodized oil had 98% necrosis, and 21 tumors with incomplete retention had 64% necrosis. Two tumors with complete retention of iodized oil in the surrounding liver had 100% necrosis, while 16 tumors with partial retention and nine tumors without retention of iodized oil in surrounding liver had 74% and 62% necrosis, respectively. Complete retention of iodized oil in the tumor and surrounding liver demonstrated the best therapeutic effects. Evaluation of the pattern and distribution of iodized oil in the tumor and surrounding liver with iodized-oil CT is useful in the assessment of the therapeutic effects of transcatheter arterial embolization in encapsulated nodular HCCs.

Carcinoma, Hepatocellular↗
PubMed1992

A photodocumented study on lens thickness and densitometric value according to the type of cataract.

Photodocumented measurements of the lens thickness using an EAS-1000 Scheimpflug camera were performed in 261 cataract eyes (162 pure type) compared with 52 control eyes. The lens thickness in normal eyes usually increased with age by 0.015 mm annually. The cataractous lens, especially in the anterior subcapsular type combined with the superficial cortical and posterior subcapsular types of opacity, showed statistically significant lens thinning. The photodocumented values of lens thickness were higher in 7.9%, and those of the anterior chamber depth were lower in 5% compared with A scan ultrasonographic measurements.

Adult↗
PubMed1992

Mandibular angloplasty.

Bulging of the mandibular angle is considered to be unattractive in the Orient since it gives the face a quadrangular and muscular appearance. In spite of its muscular origin, surgery should be aimed toward bony reduction or osteoplasty and supplemental myotomy. An intraoral approach was utilized with an oscillating saw to resect a predetermined segment of bone (mandibular angloplasty). In 2 patients with a prominent bilateral mandibular angle, only the bony angle was resected. The improvement of facial esthetics was subtle, and the acceptance and satisfaction of the patients were very good. A review of the literature is included with comments on the diagnosis and treatment of benign masseteric hypertrophy.

Adult↗
PubMed1992

The experimental Seoul-type keratoprosthesis.

The most important problem in the transplantation of artificial cornea is the extrusion of keratoprosthesis. So we performed this study to know the conditions to keep the keratoprosthesis as long as possible after animal experiment with experimental keratoprosthesis of various material and structure. We concluded from the results that silicone barrier could not prevent the retroprosthetic membrane, and fluorosilicone was not adequate material as an optic. And the following conditions are required to maintain keratoprosthesis as long as possible; optic made of PMMA, retention plate made of PTFE with 0.6 mm thick, anterior direction of optic, large optic, intralamellar suture technique. Furthermore we postulated that enzymatic theory is more appropriate than aseptic necrosis theory about the extrusion of keratoprosthesis.

Animals↗
PubMed1992

Internal capsular lesion associated with dizziness.

It has been known that the vestibular system is concerned with feelings of dizziness or vertigo. The vestibulo-thalamic pathway has also been described previously. However, there has been no confirmative report so far regarding the pathway through the internal capsule to the cortex. We have experienced 13 patients with symptoms of dizziness and/or vertigo whose lesions are located only around the internal capsule, mainly at the posterior limb and/or the genu. It is suggestive that fibers with dizziness may pass through a part of the internal capsule, probably through some part of the posterior limb and/or the genu.

Adult↗
PubMed1991

The complete amino acid sequence of the human transglutaminase K enzyme deduced from the nucleic acid sequences of cDNA clones.

In order to study the expression and role of transglutaminases in the formation of the cross-linked cell envelope of human epidermis, we have used a synthetic oligonucleotide encoding the consensual active site sequence of known transglutaminase sequences. By Northern blot analysis, newborn foreskin epidermis expresses three different mRNA species of about 3.7, 3.3, and 2.9 kilobases while normal cultured epidermal keratinocytes express only the 3.7- and 2.9-kilobase species. The largest species corresponds to a known ubiquitous tissue type II or transglutaminase C activity, the smallest corresponds to a known type I or transglutaminase K activity, and the mid-sized component apparently encodes a transglutaminase E activity that has recently been shown to be expressed in terminally differentiating epidermis (Kim, H. C., Lewis, M. S., Gorman, J. L., Park, S. C., Girard, J. E., Folk, J. E. & Chung, S. I. (1990) J. Biol. Chem., in press). Using the active site oligonucleotide as a probe, we have isolated and sequenced cDNA clones encoding the transglutaminase K enzyme. The deduced complete protein sequence has 813-amino acid residues of 89.3 kDa, has a pl of 5.7, and is likely to be an essentially globular protein, which are properties expected from the partially purified enzyme. It shares 49-53% sequence homology with the other transglutaminases of known sequence, especially in regions carboxyl-terminal to the active site, and possesses sequences likely to confer its Ca2+ dependence. Interestingly, its larger size is due to extended sequences on its amino and carboxyl termini, absent on the other transglutaminases, that may define its unique properties.

Amino Acid Sequence↗
PubMed1978

Variations in globin synthesis in delta-beta-thalassaemia.

Peripheral blood globin synthesis studies were done in 11 patients with delta beta-thalassaemia trait, Hb S-delta beta-thalassaemia or delta beta/betao-thalassaemia from two black and two Caucasian families. All patients had elevated Hb F and normal or decreased Hb A2 levels and 10 had family studies confirming the diagnosis. In addition, four unrelated non-thalassaemic patients with elevated Hb F levels also had peripheral blood globin synthesis studies. The beta/alpha specific activity globin synthesis ratios in the three blacks with delta beta-thalassaemia trait were 0.60--1.04. In the four Caucasians with delta beta-thalassaemia traint, the beta/alpha ratios were 0.58--1.02. These results demonstrate a wide range of ratios overlapping those of normal controls (0.99 +/- 0.06). The betas/alpha ratios in three blacks with Hb S-delta beta-thalassaemia ranged from 0.66 to 1.00, similar to those of patients with delta beta-thalassaemia trait. In the black patient with delta beta/betao-thalassaemia, the gamma/alpha ratio was 0.67. The beta/alpha peripheral blood ratios in the four non-thalassaemia patients with elevated Hb F ranged from 1.00 to 1.11, similar to those of normal controls. These studies indicate that a decreased beta/alpha ratio is not an invariable finding in delta beta-thalassaemia in blacks or Caucasian patients and that globin synthesis data alone is insufficient to diagnose definitively heterozygotes for delta beta-thalassaemia or to distinguish this trait from non-thalassaemic haematological disorders associated with a normal percentage of Hb A2 and an elevated level of Hb F.

Adolescent↗
PubMed1977

Globin biosynthesis in sickle cell, Hb SC, and Hb C diseases.

The wide range of globin synthesis ratios reported in patients with sickle cell disease casts doubt on whether the presence of genes for alpha- or beta-thalassemia in combination with Hb S can be detected by globin synthesis studies. We have studied globin synthesis in 20 patients with Hb SS who had a mean betaA/alpha ratio of 1.05+/-0.04, similar to that of 28 control children. In nine of these patients the percentage of newly synthesized radioactive alpha-chains in dimer or monomer forms was 16.3%+/-1.3, also similar to the control subjects. The remainder of alpha-chain was in hemoglobin tetramer. In nine patients with Hb SC, the (non-alpha)/alpha ratio was 0.97+/-0.04, and the free alpha-chain pool radioactivity in four patients was 14.1%+/-4.2. In three patients with Hb CC, betac/alpha ratios were 0.99, 1.07, and 1.10. These results indicate that globin synthesis ratios and alpha-chain radioactivity in the free alpha-chain pool of peripheral blood of patients with Hb SS, Hb SC, and Hb CC have narrow ranges, close to those of nonthalassemic controls. The data provide a basis for detecting syndromes with Hb S or Hb C associated with alpha- or beta-thalassemia. This precise differentiation is important for clinical studies of severity in sickle cell disease and for genetic counseling.

Adolescent↗
PubMed1977

Detection of sickle alpha- or beta0-thalassemia by studies of globin biosynthesis.

Globin synthesis studies are useful in the analysis of thalassemia syndromes. We have applied globin synthesis and free alpha-chain pool studies of peripheral blood to characterize hematologic disorders where alpha- or beta-thalassemia was present in combination with HbS or HbC. In 60 non-thalassemic controls, the beta/alpha specific activity ratio was 1.01 +/- 0.06 (SD). In three patients with HbS-beta0-thalassemia, the (betas + gamma)/alpha ratios were 0.48-.067. In four patients with HbSS-alpha-thalassemia, the (BETAS/ALPHA RATIO was 1.26 +/- 0.18 (1.13-1.53). The radioactive free alpha-chain pool in three patients with HbS-beta0-thalassemia was elevated (35.1%-53.0%), while three patients with HbSS-alpha-thalassemia had decreased free radioactive alpha-chain pools (3.2%-6.4%); both were significantly different from the mean (15.1% +/- 2.6%) of the 17 iron-sufficient controls. Simultaneous studies of the fraction of newly synthesized alpha chain contained in the free alpha-chain pool in peripheral blood and bone marrow demonstrated that this fraction was larger in peripheral blood than in marrow, and that the differences between thalassemia patients and controls previously found in bone marrow using these methods were also present in peripheral blood. The results indicate that even when family studies are not possible, patients with HbS in combination with alpha- or beta0-thalassemia can be differentiated from those with homozygous sickle cell disease by globin synthesis and free alpha-chain pool studies using peripheral blood.

Child↗
PubMed1976

Erythroleukemia-like syndrome due to busulfan toxicity in polycythemia vera.

Over a 19-year period, a patient with polycythemia vera who had undergone a splenectomy received six courses of busulfan for recurrent thrombocytosis. The total dose of busulfan given for the sixth course was greater than that used for the previous ones. Severe pancytopenia followed, which persisted for 4 months. During this period there was marked erythroid hyperplasia in the bone marrow with striking dyserythropoiesis; PAS-positive red cell precursors, as well as moderate numbers of circulating normoblasts and evidence of chronic and acute hemolysis, were present. All of these findings reverted to normal without therapy, and the polycythemic state eventually recurred. These events are interpreted as an unusual marrow reaction following busulfan overdosage rather than a transient erythroleukemia.

Adult↗
PubMed1976

Isolation and in vitro differentiation of human erythroid precursor cells.

There is decreased beta-globin production in beta-thalassemic reticulocytes and nucleated erythroid cells. In this study, we have examined whether unbalanced globin synthesis is expressed at all stages of human erythroid cell maturation. In order to determine the pattern of globin synthesis in early erythroid cells during erythroid cell maturation, an in vitro culture system using human bone marrow erythroid precursor cells has been developed. Early erythroid precursor cells (proerythroblasts and basophilic erythroblasts) have been isolated from nonthalassemic and thalassemic human bone marrows by lysing more mature erythroid cells, using complement and a rabbit antiserum prepared against normal human red cells. In the presence of erythropoietin, differentiation and proliferation of erythroid cells in demonstrable in liquid suspension culture for 24-48 hr, as determined by morphological criteria and by an increase in globin synthesis. The ratio of alpha- to beta-globin chain synthesis in nonthalassemic cells in approximately 1 at all stages of erythroid cell differentiation during culture. In cells from four patients with homozygous beta- thalassemia there is decreased beta-globin synthesis compared to alpha-globin synthesis, both in early erythroid precursor cells and during their maturation in culture. These findings indicate that unbalanced globin chain synthesis is expressed at all stages of red cell maturation in homozygous beta-thalassemia.

Anemia, Sickle Cell↗
PubMedDate not supplied

Neonatal thrombosis with anticardiolipin antibody in baby and mother.

The anticardiolipin antibody is associated with increased risk of thrombosis, which is manifested with various clinical presentations, including vascular thrombosis, recurrent fetal wastage, and neurologic defects. We report a case of neonatal thrombosis occurring in the renal vein and inferior vena cava associated with moderate positive anticardiolipin antibody titer in the baby while the maternal serum showed even stronger anticardiolipin antibody of immunoglobulin G class. Subsequent follow-up over the 4 months postpartum period showed a disappearance of the antibody in the baby, whereas the mother's antibody persisted. This case illustrates the fact that the maternal anticardiolipin antibody can be transferred to the fetus and may be a risk factor for thrombosis in the neonates. Anticardiolipin antibody syndrome should be considered in the differential diagnosis of neonatal thrombosis.

Adult↗
PubMedDate not supplied

Hypointense boundary layer between slow flow and mural thrombus on spin-echo MR.

To evaluate the ability of spin-echo MRI to differentiate between slow flow and mural thrombus in aortic diseases, we reviewed the spin-echo MRI of 10 patients with intraaortic thrombus that had been documented by CT in 8 patients and aortography in 2 patients. Six patients had aortic aneurysms with mural thrombi, and four had aortic dissections with adherent mural thrombi within the false lumen. Five of seven gated oblique sagittal or coronal T1-weighted studies demonstrated hyperintense slow flow signal within the residual lumen. This hyperintense slow flow signal was accompanied by a parallel hypointense rim due to a boundary layer dephasing phenomenon. Eight axial T2-weighted MR studies demonstrated a hypointense zone due to fresh clot at the edge of a mural thrombus on even-echo images consistently. The hypointense boundary layer between slow flow and mural thrombus on either gated T1-weighted MRI or second-echo T2-weighted MRI not only predicted the presence of flow within the residual lumen but also clearly separated the area of slow flow from that of mural thrombus in 9 of 10 patients. Proper interpretation of spin-echo images may obviate the need for phase display imaging or gradient-echo imaging in differentiating between slow flow and mural thrombus.

Adult↗