PURPOSE: To analyze functional gains after TKA in patients with hemophilic arthropathy. MATERIALS AND METHODS: From January 1989 to December 1996, 16 total knee replacements were performed in 11 patients with hemophilia. The average age at operation was 30 years and nine months. The average follow-up period was 3.2 years (range, 1 to 7 years). There were 14 knees of hemophilia A and 2 knees of hemophilia B. Preoperatively, all patients complained of severe pain and had marked knee joint deformity of grade IV or V by Arnold and Hilgartner classification. The knee rating scale of Hospital for Special Surgery was used for clinical assessment. RESULTS: The average preoperative range of motion was 87.9 degrees (range, 25-125 degrees) and 94 degrees (range, 70-125 degrees) postoperatively. The average flexion contracture was 20.7 degrees (range, 0-40 degrees) preoperatively and 4.7 degrees (range, 0-15 degrees) postoperatively. The knee rating scale improved from 44.3 points (range, 40-76) to 88.8 points (range, 81-97). There was one case of superficial necrosis of skin. CONCLUSIONS: The range of motion was not improved significantly after total knee replacements in hemophilic patients. However, knee pain and bleeding episodes have decreased prominently.
Arthroplasty ; Arthroplasty, Replacement, Knee ; Classification ; Congenital Abnormalities ; Contracture ; Follow-Up Studies ; Hemophilia A ; Hemophilia B ; Hemorrhage ; Humans ; Knee Joint ; Knee* ; Necrosis ; Range of Motion, Articular ; Skin

BACKGROUND: Therapeutic plasma exchange is the therapeutic procedure to improve clinical course by removing various antibodies, immune complexes, toxic materials or by replacing insufficient plasma elements. But there are many debates about its effects although many immunologic diseases improve their clinical courses by plasma exange. The aim of this study is to examine clinical aspects of plasma exchange. METHODS: We investigated retrospectively 30 patients who had been done a total of 128 plasma exchange procedures during the last 5 years at Korea University Anam Hospital. We judged clinical improvement according to diseases, replacing solutions and examined the changes of blood chemistries, side effects etc. RESULTS: There were 14 males and 16 females. The patients' age ranged from 8 to 76 years old and mean age was 45 years old. Total 30 patients had been carried out a total of 128 plasma exchange procedures and the mean was 4.3 times. The frequency of plasma exchange was from once up to 12 times and five times is the most frequent number carried out (27%). The most frequent diseases were multiple myeloma and myasthenia gravis which were 17% respectively and other diseases in the decreasing order of frequency were hemolytic uremic syndrome (HUS), Guillian-Barre syndrome, fulminant hepatitis etc. Double lumen catheter into femoral vein(73%) was most frequently used for the vascular access. The clinical improvement was obtained in 50% of total cases and the clinical results in myasthenia gravis and Guillian-Barre syndrome were excellent. There were 8 patients(27%) who died during treatment period, but the cause of death was not related to plasma exchange procedure itself. As a replacement solution, 5% albumin alone(23%) was used most frequently and the others were fresh frozen plasma(FFP) alone(20%), FFP plus Hartman's soultion plus albumin(20%), FFP plus albumin(17%) etc. Serum total calcium concentration was significantly decreased from 8.5 to 7.9 mg/dL(p=0.017) and platelet was also decreased from 154,666/mm3 to 100,222/mm3(p=0.02) after plasma exchange but the other chemistries were not changed. The frequent side effects were nausea, vomiting, fever, chill, hypotension, itching sensation and muscle cramps. CONCLUSION: The plasma exchange is one of the useful therapeutic procedures in some immunologic diseases especially for the myasthenia gravis and Guillian-Barre syndrome. It is a relatively safe procedure, but gastrointestinal side effects such as nausea, vomiting in early period may be a problem. And the careful monitoring of serum calcium concentration, platelet count before and after plasma exchange is required.
Aged ; Antibodies ; Antigen-Antibody Complex ; Blood Platelets ; Calcium ; Catheters ; Cause of Death ; Female ; Fever ; Hemolytic-Uremic Syndrome ; Hepatitis ; Humans ; Hypotension ; Immune System Diseases ; Korea ; Male ; Middle Aged ; Multiple Myeloma ; Muscle Cramp ; Myasthenia Gravis ; Nausea ; Plasma Exchange* ; Plasma* ; Platelet Count ; Pruritus ; Retrospective Studies ; Sensation ; Vomiting

BACKGROUND: The angiotensin-converting enzyme (ACE) has a major role in the degradation of bradykinin, tachykinin, substance P which are associated with bronchial hyperresponsiveness and inflammation. The other role of ACE is the genesis of angiotensin II which causes bronchial smooth muscle contraction. The deletion polymorphism of ACE gene(DDtype) may be related to the high serum level of ACE. OBJECTIVE: We studied to evaluate an association between the insertion /deletion polymorphism of the ACE gene and asthma, and its severity. Materials and methods: Sixty asthmatic patients and 44 healthy controls were enrolled. Severity of asthma was classified by the guideline of NHLBI/WHO workshop. The ACE genotypes of all the subjects were determined by polymerase chain reaction. RESULTS: The distribution of ACE genotypes were not significantly different between healthy controls and asthma group (p)0.05). In asthmatic patients, the genetic polymorphism was similar between different severity groups (p) 0.05). Conchcsion: It is suggested that I/D polymorphism of the ACE gene may not be associated with development of asthma. The severity of asthma may not be influenced by I/D polymorphism of the ACE gene.
Angiotensin II ; Asthma* ; Bradykinin ; Education ; Genotype ; Humans ; Inflammation ; Muscle, Smooth ; Polymerase Chain Reaction ; Polymorphism, Genetic ; Substance P ; Tachykinins

OBJECTIVES: The respiratory compensation threshold(RCT) is thought to be one of parameters by some for the evaluation of physical performance despite its controversial status. The purpose of this investigation is to establish the reference values of the RCT in healthy Korean adults, and to examine the usefulness of this parameter in evaluating physical performance. METHODS: Symptom-limited maximal exercise test by Bruce protocol was carried out, and RCT and AT were determined by the aid of the computerized V-slope method, along with the VO2 max, in 441 healthy Korean adults consisting of 284 men and 157 women aged from 20`s to 60`s. In addition, correlation between RCT and AT was observed, and regression equations of the RCT were also derived. RESULTS: The RCT VO2 was tended to decrease with age in men, but not in women and the RCT VO2 of women was 26-30% less than that of men. The ratio of RCT VO2 to VO2 max (RCT VO2/VO2 max) was tended to increase with age and the RCT V was significantly correlated with AT VC4 and VO2 max in both sexes. CONCLUSION: Our study reveals that the RCT is another useful submaximal index along with the AT in evaluating physical performance. The regression equation of the RCT was RCT VO2(L/min)=1.4232-0.0102 A(age in year)+0.0204 W(weight in kg)-0.4889 S (sex, coded O for males and 1 for females).
Adult* ; Anaerobic Threshold ; Compensation and Redress* ; Exercise Test ; Female ; Humans ; Male ; Reference Values

McCune-Albright syndrome (MAS) is a sporadic disease that's characterized by polyostotic fibrous dysplasia, cafe-au-lait pigmentation of the skin, and multiple endocrinopathies, including sexual precocity, hyperthyroidism, acromegaly, and hypercortisolism. Recent evidence has shown that the clinical manifestations are caused by a postzygotic activating missense mutation in the gene coding for the alpha-subunit of Gs protein that stimulates c-AMP formation in the affected tissues. Substitution of the Arg(201) residue in Gsalpha with cysteine or histidine have been identified in many MAS patients and Arg(201) to Gly or Leu mutations have also been recently identified. We identified the Arg(201) to His mutation in the gene encoding Gsalpha in the thyroid tissue from a 36-year-old man who was suffering with polyostotic fibrous dysplasia and hyperthyroidism.
Acromegaly ; Adult ; Clinical Coding ; Cushing Syndrome ; Cysteine ; Fibrous Dysplasia, Polyostotic* ; Histidine ; Humans ; Hyperthyroidism* ; Mutation, Missense ; Pigmentation ; Skin ; Thyroid Gland

Diabetes mellitus is well known to be associated with various structural and functional liver abnormalities. If diabetic patients are accompanied by hepatomegaly or abnormal findings from a liver function test, the most common pathological findings are steatosis and glycogenosis. The steatosis is characterized by deposition of macrovesicular fat droplets in the hepatocytes, which is common in obese, type 2 diabetes mellitus. If macrovesicular steatosis is combined with mixed inflammatory infiltrate, without evidence of alcoholic hepatitis, the case could be diagnosed as nonalcoholic steatohepatitis (NASH). NASH has the possibility of progressing to cirrhosis. Secondary glycogenosis is common in uncontrolled type 1 diabetes mellitus, and is completely reversible. A 22-year-old male, with uncontrolled type 1 diabetes mellitus, was admitted with anorexia, nausea and right upper quadrant pain. Hepatomegaly and elevated aminotransferases were noted. He was diagnosed as diabetic glycogenosis using computed tomogram and liver biopsy. The hepato megaly and liver function test abnormalities were markedly improved with glycemic control
Anorexia ; Biopsy ; Diabetes Mellitus ; Diabetes Mellitus, Type 1 ; Diabetes Mellitus, Type 2 ; Fatty Liver ; Fibrosis ; Glycogen Storage Disease* ; Hepatitis, Alcoholic ; Hepatocytes ; Hepatomegaly* ; Humans ; Liver ; Liver Function Tests ; Male ; Nausea ; Transaminases ; Young Adult