Comparison of acute non-haemolytic transfusion reactions in female and male patients receiving female or male blood components.

To study the relationship between antibodies detected in patients' and/or donors' sera and the clinical features of acute non-haemolytic transfusion reactions (ANHTRs), and to determine any gender-related difference. ANHTRs range from urticaria to transfusion-related acute lung injury (TRALI). Antibodies to human leukocyte antigen (HLA), granulocytes, platelets, and/or plasma proteins are implicated in some of the ANHTRs. A higher antibody positivity is expected for females than for males. A comparative study of ANHTRs for antibody positivity and their clinical features between females and males for both patients and donors is helpful for characterizing ANHTRs including TRALI more clearly, but such studies are few and outdated. Two hundred and twenty-three ANHTR cases reported by 45 hospitals between October 2000 and July 2005 were analysed. The patients and 196 donors of suspect blood products were screened for antibodies to HLA Class I, HLA Class II, granulocytes, and platelets. The patients were also screened for anti-plasma protein antibodies. The types and severity of ANHTR did not differ significantly between female and male patients. The frequency of the anti-HLA antibodies, but not that of the non-HLA antibodies, was significantly higher in females. Non-HLA antibodies were significantly associated with severe reactions in females. All the TRALI cases had predisposing risk factors for acute lung injury, and 60% of the cases showed anti-leucocyte antibodies. Although the anti-HLA antibodies were detected more frequently in females than males, no significant association of ANHTRs including TRALI with gender, not only for patients, but also for donors, could be shown in this study.

Inflammatory vitiligo in Vogt-Koyanagi-Harada disease.

Vogt-Koyanagi-Harada disease is a rare disease characterized by uveitis, meningitis, dysacusis, alopecia, poliosis, and vitiligo. We describe a 48-year-old patient with Vogt-Koyanagi-Harada disease associated with thin inflammatory raised erythema and plaque-type inflammatory erythema superimposed on vitiligo. Interestingly, inflammatory raised erythema was separated from the perfect vitiligo, and the incomplete vitiligo lay between them initially. Thereafter, incomplete vitiligo became completely depigmented with diminution of inflammatory raised erythema. This is the second case of vitiligo with inflammatory raised borders associated with Vogt-Koyanagi-Harada disease. Our results of immunohistochemical and electron microscopic studies suggested the involvement of T-cell-mediated cytotoxicity and apoptosis in the development of skin lesions.

[Mental stress of visiting-nursing staff in cases of severely handicapped children with respirators].

We evaluated the actual condition of work and mental stress on visiting nursing staff, for the purposes of establishing a suitable visiting-nursing system for severely handicapped children. The subjects were 13 visiting nurses involved in the care of two children with Werdnig-Hoffman disease. The visiting-nursing staff was examined by questionnaire. Of these staff, 77% felt a burden from their work. They felt a burden due to the special needs of pediatric patients and the relationship between the staff and parents. The relationship between visiting-nursing staff and other medical staff, including doctors, was another main factor in this burden. The younger nurses felt this burden more strongly.

Anti-granulocyte antibody screening with extracted granulocyte antigens by a micro-mixed passive hemagglutination method.

BACKGROUND AND OBJECTIVES: Serologic tests for granulocyte antibodies, i.e., the granulocyte agglutination test and the granulocyte immunofluorescence test, require panels of typed granulocytes that cannot be preserved for more than a few hours. We have developed a new method in which granulocyte antigens, extracted into saline containing 3% sucrose, are coated onto U-type Terasaki plates. With this new method, we evaluated the micro-mixed passive hemagglutination test (EG-MPHA) for screening for granulocyte antibodies. MATERIALS AND METHODS: We tested the ability of the EG-MPHA to detect granulocyte antigens using 5 human antibodies specific for NA1, NA2, NB1, 5b, and Sar(a), and 8 different monoclonal antibodies for NA1, CD11a, CD11b, CD13, CD16, CD18 and HLA class I. Sera from 94 alloimmunized patients were screened by the chloroquine-treated EG-MPHA method. RESULTS: NA1, NA2, NB1, 5b, Sar(a), CD11a, CD11b, CD13, CD16, CD18 and HLA class I antigens were present in the extracted granulocyte antigen preparation. CD11b and HLA class I antigens were removed when the extracted granulocyte antigens were treated with chloroquine. Granulocyte antibody screening of sera from alloimmunized patients showed that approximately 30% of the anti-HLA-positive and 10% of the anti-HLA-negative sera were positive for granulocyte antibody by the chloroquine-treated EG-MPHA. The extracted granulocyte antigen panels could be stored frozen for at least 1 year at -80 degrees C. CONCLUSION: This new method is preferable for screening for granulocyte antibodies. In addition, it has the advantage of requiring only 5 microl of serum for each test.

Clinical features of isolated noncompaction of the ventricular myocardium: long-term clinical course, hemodynamic properties, and genetic background.

OBJECTIVES: A nationwide survey was conducted to clarify the clinical features of isolated noncompaction of the ventricular myocardium (INVM) in Japanese children in comparison with features previously described in patients with INVM. BACKGROUND: Isolated noncompaction of the ventricular myocardium is a rare disorder characterized by an excessively prominent trabecular meshwork. It is accompanied by depressed ventricular function, systemic embolism and ventricular arrhythmia. METHODS: A questionnaire specifically designed for this study was sent to 150 hospitals in Japan where a pediatric cardiology division exists. RESULTS: Twenty-seven patients were diagnosed by two-dimensional echocardiography, their ages ranging from one week to 15 years at presentation, with follow-up lasting as long as 17 years. The gross anatomical appearance and the extension of noncompacted myocardium predominantly at the apex observed on two-dimensional echocardiograms were similar to observations reported previously. Dissimilarities included a greater number of asymptomatic patients at initial presentation, a longer clinical course with gradually depressed left ventricular function, no systemic embolism, and rare ventricular tachycardia in the Japanese children. Cardiac catheterization disclosed normal left ventricular end-diastolic volume and increased left ventricular end-diastolic pressure in most cases, consistent with restrictive hemodynamics. A higher incidence of Wolff-Parkinson-White syndrome was found in the children, whereas left bundle branch block was rarer than reported in adults. Familial recurrence was high (44%) and included many women. CONCLUSIONS: In Japanese children, INVM can be found by screening examinations at asymptomatic stage, and it might have a longer dinical course with gradually depressed left ventricular function and restrictive hemodynamics. The pattern of familial recurrence we observed implies that INVM is a distinctive clinical entity with a heterogeneous genetic background.

Familial isolated noncompaction of ventricular myocardium.

We report a family in which two male sibs were affected with isolated noncompaction of ventricular myocardium (INVM). The familial occurrence of INVM suggests a genetic basis. We review the literature of familial and nonfamilial cases and discuss the inheritance pattern of INVM.

[Workers' stress after Hanshin-Awaji earthquake in 1995--symptoms related to stress after 18 months].

Stress-related symptoms were investigated in workers living in the area hit by the Hanshin-Awaji earthquake. Questionnaires were distributed among taxi and truck drivers, longshoremen, workers in factories, hospitals and local government offices 18 months after the event. Nearly 40% of them, 3,015 answered. The multiple regressive analysis showed that the degree of housing damage was the factor most relevant to the symptoms. Although the symptoms decreased as time passed, the group with seriously damaged housing still complained of severe stress-related symptoms even 18 months after the event. Symptoms related to post traumatic stress disorder (PTSD) were observed in 48.0% in the male group with seriously damaged housing immediately after the quake. The figure was 34.2% of after 3 months and 12.6% after 18 months. 21.8%, 12.9% and 3.7%, respectively, were the figures in the male group with nil or slightly damaged housing. The number of workers who complained of the recurrence and worsening of chronic diseases peaked 3 months after the event and had not changed much even after 18 months. Workers also complained of anxiety over the loss of work, job pressure, overwork, difficulty of commuting to and from the area, danger at work, decline in income and changes of job location.

[Mitral valve replacement and closure of ventricular septal defect in the first two months of life].

A 2-month-old female infant weighing 4.2 kg was admitted with severe congestive heart failure and respiratory distress. The patient was operated upon under a diagnosis of severe congenital mitral regurgitation and ventricular septal defect. There was thickening of the anterior leaflet and short thickened chordae. The valve was judged not amenable to repair and it was replaced with a 17 mm St. Jude Medical valve. She was extubated on the 5th postoperative day. Post operative anti-coagulant therapy was initiated with warfarin potassium, dipyridamole and ticlopidine hydrochloride. The postoperative course was uneventful and the patient is doing well 9 months after the operation.

Endothelin-1 production in coronary circulation in a new canine model of coronary thrombosis.

OBJECTIVE: The purpose of this study was to investigate whether endogenous endothelin-1 (ET-1) production in coronary circulation is associated with acute coronary thrombotic events in vivo. To achieve this goal, we have designed a new experimental canine model of coronary thrombosis. METHODS: In vivo occlusive thrombus was induced by the intracoronary application of radiofrequency energy (660 kHz, 50 W) in closed-chest dogs. Pathological and immunohistochemical examinations of thrombosed coronary artery were performed. In 12 dogs, plasminogen activator was administered intravenously and serial measurements of ET-1, thromboxane B2 (TXB2) and thrombin-antithrombin III complex (TAT) levels in plasma from the coronary sinus, aortic root and inferior vena cava were examined. RESULTS: Occlusive platelet-rich thrombi were attached to the deeply injured intimal surface. TAT and TXB2 increased rapidly soon after the intimal injury and declined after successful thrombolysis. In contrast, ET-1 in the coronary sinus was elevated after reperfusion and was significantly higher than in the aorta. Net ET-1 production in the coronary circulation showed a significant positive correlation with the peak TAT levels (r = 0.69, P < 0.05), but not with TXB2 or total occlusion time as an index of ischemic severity. CONCLUSIONS: Deep intimal injury leads to occlusive coronary thrombus. Thrombus formation and its subsequent lysis is associated with the activation and deactivation, respectively, of the coagulation cascade and platelets. Thrombin generation may stimulate ET-1 production in the coronary endothelium in acute coronary thrombotic events.

A case of large neonatal arteriovenous malformation with heart failure. Color Doppler sonography, MRI and MR angiography as early non-invasive diagnostic procedures.

We report a case of a large arteriovenous malformation (AVM) of neonatal onset with heart failure. Transfontanel color Doppler sonography revealed abnormal vessels in the early stage of the investigation. Magnetic resonance imaging (MRI) revealed numerous flow voids suggesting abnormal vessels, and magnetic resonance angiography (MRA) disclosed numerous bizarre abnormal vessels. Color Doppler sonography is a convenient and appropriate procedure for the early bedside diagnosis of neonatal AVMs. MRI and MRA can replace cerebral angiography for the diagnosis of neonatal AVMs.

Coagulation activity is increased in the left atrium of patients with mitral stenosis.

OBJECTIVES: This study investigated the hemostatic status of the right and left atria in patients with mitral stenosis. BACKGROUND: Systemic thromboembolism is a serious major complication in patients with mitral stenosis. However, the pathogenesis of thromboembolism in mitral stenosis is not fully understood. METHODS: We determined the plasma levels of biochemical markers for platelet activity (platelet factor 4 and beta-thromboglobulin) and status of thrombin generation (fibrinopeptide A and thrombin-antithrombin III complex) and fibrinolysis (D-dimer and plasmin-alpha 2-plasmin inhibitor complex) in specimens of blood obtained from the peripheral vein and right and left atria of 12 consecutive patients with mitral stenosis who were undergoing percutaneous mitral valvuloplasty. RESULTS: Plasma levels of platelet factor 4, beta-thromboglobulin, D-dimer and plasmin-alpha 2-plasmin inhibitor complex in the patients did not differ significantly between the right and left atria, whereas levels of fibrinopeptide A and thrombin-antithrombin III complex in the left atrium were significantly higher than those in the right atrium (fibrinopeptide A in the left and right atria 19.35 +/- 4.64 and 6.31 +/- 0.75 ng/ml [mean +/- SE], respectively, p < 0.02; thrombin-antithrombin III complex in the left and right atria 11.45 +/- 2.29 and 3.98 +/- 0.60 ng/ml, respectively, p < 0.01). Levels of fibrinopeptide A and thrombin-antithrombin III complex in the left atrium did not correlate with mean transmitral gradient, dimension of the left atrium or reciprocal of the mitral valve area. Peripheral blood plasma levels of von Willebrand factor antigen were significantly higher in the patients than in an age-matched control group of normal subjects (168 +/- 25% and 99 +/- 7%, respectively, p < 0.05) but showed no difference in the peripheral blood and right and left atria of the patients. CONCLUSIONS: The coagulation system is activated in the left atrium of patients with mitral stenosis even during anticoagulation.

[Surgical treatment of supravalvular aortic stenosis].

In the last 14 years, 7 consecutive patients with supravalvular aortic stenosis (SVAS) underwent surgical treatment for SVAS and/or for associated lesions. There were 5 male and 2 female patients ranging in age from 3 months to 12 years. Six of them had associated other cardiac anomalies; two had severe multiple peripheral pulmonary stenoses (PPS) and one each had ventricular septal defect (VSD), valvular pulmonary stenosis, coarctation of aorta with patent ductus arteriosus (PDA), total anomalous pulmonary venous return (TAPVR) with pulmonary branch stenosis, PPS and left lower pulmonary venous obstruction. The type of SVAS were localized in all and 5 of them underwent successful surgical repair of SVAS (3 with extended aortoplasty and 2 with patch aortoplasty). In 2 patients with VSD or valvular pulmonary stenosis, associated cardiac anomalies and SVAS were repaired simultaneously. Four patients had undergone previous operations, which included repair of severe multiple PPS by extended peripheral pulmonary arterioplasty (case 4, 6), repair of coarctation of aorta and division of PDA (case 5), repair of TAPVR (Ia + IIa) and pulmonary branch stenosis (case 7). There was no operative death and one patient died late postoperatively (case 7) due to right heart failure in a follow up period of 3 to 14 years. In conclusion, it is important to select the appropriate surgical treatment according to the location and the severity of associated other cardiac anomalies as well as the severity of SVAS, and extended peripheral pulmonary arterioplasty is considered to be a recommended method for the relief of severe multiple PPS associated with SVAS.

Congenital heart defects in Aarskog syndrome.

We report on 10 Japanese individuals from 3 families affected with Aarskog syndrome. Pulmonary stenosis and ventricular septal defect with spontaneous closure were detected respectively, in 2 of them as an uncommon finding. A review documented 169 non-Japanese cases (2 with congenital heart defects), while of 30 Japanese individuals reported till now, 4 (including ours) had cardiac anomalies. We propose that this combination is not coincidental and that in all cases of Aarskog syndrome a cardiac evaluation is indicated.

Endothelin production in pulmonary circulation of patients with mitral stenosis.

BACKGROUND: Although plasma endothelin concentrations are elevated in patients with pulmonary hypertension, the precise sites of endothelin production have not been defined. We investigated the endothelin production in the pulmonary circulation of patients with mitral stenosis and its effects on pulmonary vascular tone. METHODS AND RESULTS: We measured plasma concentrations of endothelin-1, angiotensin II, and thrombomodulin in blood samples obtained from the right and left atria of 10 consecutive patients with rheumatic mitral stenosis (mean age, 55 years; range, 39 to 68) who were undergoing percutaneous mitral valvuloplasty. Plasma levels of endothelin-1 were significantly higher in the left atrium than in the right atrium (3.25 +/- 0.45 versus 2.53 +/- 0.36 pg/mL, mean +/- SE, P < .001). The increased plasma endothelin-1 level in the left atrium, which reflected endothelin-1 production in the pulmonary circulation, was correlated with mean pulmonary artery pressure (r = .65, P = .04), mean pulmonary arterial wedge pressure (r = .67, P = .03), total pulmonary resistance (r = .68, P = .03), and 1/mitral valve area (r = .85, P = .002) but not with pulmonary vascular resistance (r = .04, P = .91). There were no significant differences in plasma levels of angiotensin II and thrombomodulin between the right and left atria (angiotensin II, 16.40 +/- 3.08 versus 15.50 +/- 4.85 pg/mL; thrombomodulin, 2.96 +/- 0.34 versus 2.85 +/- 0.37 ng/mL). CONCLUSIONS: Endothelin-1 production is increased in the pulmonary circulation of patients with mitral stenosis in response to increased pulmonary artery pressure but is not directly related to increased pulmonary vascular tone in this disorder.

Multicystic aneurysmal dilatation of bilateral coronary artery fistula.

Bilateral coronary artery fistula constitutes an uncommon subgroup of coronary artery fistulas that may have a distinct embryologic origin. Coronary artery fistulas usually show a tortuous arrangement upon coronary angiography, but aneurysmal dilatation is rare. We report here an extremely rare case of coronary artery fistula originating from both coronary arteries, which showed multicystic aneurysmal dilatation.

[Blalock-Taussig shunts in infants less than 6 months of age with special reference to the long-term administration of prostaglandin E1].

Classical Blalock-Taussig (B-T) shunt operation was performed in 14 neonates (group N) and 22 infants (group P). A mean age of group N patients was thirteen days and that of group P patients was two months. Preoperatively prostaglandin E1 (PGE1) was administered in all patients and was continued for a mean of 7 days (range 2 hours to 21 days) in group N, and a mean of 78 days (range 42 to 158 days) in group P. Body weight (4359 +/- 339 g), size of subclavian artery (3.7 +/- 0.5 mm in diameter) and size of pulmonary artery (6.4 +/- 1.2 mm in diameter) at operation of group P patients were larger than those (3212 +/- 490 g, 3.4 +/- 0.4 mm, 5.1 +/- 1.3 mm) of group N. But there were no differences in operation time (165 +/- 22 minutes in group N vs 174 +/- 25 minutes in group P) and clamp time of pulmonary artery (31 +/- 10 minutes in group N vs 30 +/- 5 minutes in group P) between 2 groups. There was no operative death in both groups. There were one late death in group N and three late death in group P. Five patients in group N and nine patients in group P required additional B-T shunt at the mean follow up interval of two years.(ABSTRACT TRUNCATED AT 250 WORDS)

[Long-term results of the Blalock-Taussig shunts].

One-hundred and thirty-six patients received the classical Blalock-Taussig shunts between 1980 and 1990. Their age range at operation was 4 days to 12.8 years and their median age was 13 months. The operative mortality rate was 0.7% (1/136). The survivors were followed up from 1 month to 11.5 years, 5.4 years in average. Twenty-five patients required another shunt and the mean interval to that procedure was 2.4 years (modified Blalock-Taussig 23, classical Blalock-Taussig 2, Glenn 1, internal mammary artery-pulmonary artery shunt 1). Forty-five patients received corrective operations, there were four operative deaths (Fontan 2, Rastelli 2). There were 15 late deaths of which three deaths were not cardiogenic. One year after operation, 91.0% of patients remained in well-palliated condition. At three years after operation, 76.4% of patients continued to be in well-palliated condition. There were twelve neonates in this series. Their age range was 4 to 26 days and their median age was 13 days. There was no operative death. Five patients required second shunt. There were two late deaths. At present, six patients continue to be in well-palliated condition 8 months to 9 years after first operation. Angiographic findings showed the stenotic change of the vascular anastomoses in 49.3% (35/71) of patients. This study suggests that polydioxanone suture (PDS) will be useful for the growth of the anastomoses in Blalock-Taussig operation.