Mogamulizumab-induced photosensitivity in patients with mycosis fungoides and other T-cell neoplasms.

BACKGROUND: Mogamulizumab (Mog) is a defucosylated, therapeutic monoclonal antibody, targeting CCR4 and was first approved in Japan for the treatment of adult T-cell leukaemia/lymphoma (ATLL), followed by cutaneous T-cell lymphoma and peripheral T-cell lymphoma. OBJECTIVE: To retrospectively investigate development of photosensitivity in patients with mycosis fungoides and other T-cell neoplasms after treatment with Mog. METHODS: We treated seven cutaneous lymphoma patients with Mog. Upon combination treatment with narrow-band UVB, we noticed that four patients developed photosensitivity dermatitis following Mog therapy, including two cases of mycosis fungoides, one case of adult T-cell leukaemia/lymphoma and one case of EB virus-associated T-cell lymphoproliferative disorder. Phototest was performed with UVA and UVB, and immunohistochemical staining for CD4, CD8 and Foxp3 was conducted in both photosensitivity and lymphoma lesions. RESULTS: Phototest revealed that the action spectrum of the photosensitivity was UVB in three cases and both UVB and UVA in one case. Histopathologically, the photosensitive lesions were characterized by a lichenoid tissue reaction with a CD8+ T cell-dominant infiltrate, sharing the feature with chronic actinic dermatitis, an autoreactive photodermatosis with a cytotoxic T-cell response. Foxp3+ regulatory T cells (Tregs) were decreased in the photosensitivity lesions compared with the lymphoma lesions. CONCLUSION: Increased incidence of photosensitivity reaction was observed during Mog treatment. Decreased number of Tregs in the lesional skin suggests that this reaction is possibly induced by autoreactive cytotoxic T cells.

Clinical features of Bacteroides bacteremia and their association with colorectal carcinoma.

PURPOSE: We investigated the clinical features of Bacteroides bacteremia for 5 years to determine the risk factors for mortality and to ascertain whether bacteremia due to Bacteroides spp. is associated with colorectal carcinoma. METHODS: This study comprised a review of all patients with Bacteroides bacteremia at a teaching hospital in Tokyo from April 2003 to March 2008. We also conducted a case-control study between Bacteroides bacteremia and bacteremia due to other pathogens. RESULTS: During the study period, 25 cases of bacteremia were due to Bacteroides spp. Bacteroides bacteremia was associated with a high mortality rate (24%). Malignancy (76%) was the major comorbidity, followed by a history of surgery (40%). Colorectal carcinoma was the most frequent (n = 8, 32%) of the comorbid malignancies and was recognized as the primary infection site in six cases. Prevalence of colorectal carcinoma as comorbidity was significantly higher in Bacteroides bacteremia than in other bacteremia. CONCLUSIONS: In the Bacteroides bacteremia cases of this study, colorectal carcinoma was the major comorbidity and primary infection site. Colorectal carcinoma screening in Bacteroides bacteremia patients is potentially an important diagnostic marker for the early detection of this infection in the future.

Propionimicrobium lymphophilum and Actinotignum schaalii bacteraemia: a case report.

Propionimicrobium lymphophilum is an anaerobic Gram-positive bacillus that exists in human skin and urinary tract. The pathogenicity is, however, not well known. Only two cases of urinary tract infection have been described recently. In the case presented here, the bacterium was isolated, concomitant with Actinotignum schaalii, from blood culture of a patient with fever and difficulty of urination. The bacteria were identified by matrix-assisted laser desorption/ionization time-of-flight mass spectrometry and 16S rRNA sequencing. The case was successfully treated with ampicillin/sulbactam.

Predisposing factors of valve regurgitation in complete atrioventricular septal defect.

OBJECTIVES: We sought to determine the intrinsic risk factors of valve regurgitation in complete atrioventricular septal defect. BACKGROUND: Progression of regurgitation varies in each case, although the structure of the common atrioventricular valve itself is a predisposing factor. METHODS: In 90 consecutive patients undergoing surgical repair, we evaluated the preoperative and postoperative regurgitation, valve morphology, age at surgery and associated anomalies. A regurgitation jet with a high velocity reaching the deep left atrial wall by echocardiography was estimated as marked regurgitation. RESULTS: None of the 40 patients with Rastelli type C and an undivided inferior bridging leaflet had preoperative regurgitation in the first year of life, and 12% of them (95% confidence intervals [CI]: 0% to 28%) showed regurgitation at the age of 2. Of the remaining 50 with Rastelli type A and/or a divided inferior leaflet, regurgitation was determined in 21% (95% CI: 6% to 35%) of those 1 year old and in 49% (95% CI: 29%7 to 69%) of those 2 years old (p < 0.01). All patients underwent corrective surgery using the double-patch technique, with the "cleft" being sutured adequately. Irrespective of the valve morphology, regurgitation remained in 52% (12 of 23) of those with preoperative regurgitation, whereas regurgitation developed postoperatively in 28% (16 of 58) of those without regurgitation (p < 0.001). CONCLUSIONS: Those with Rastelli type C and an undivided inferior leaflet had a lesser degree of progression of preoperative regurgitation. However, regurgitation was likely to exist even after adequate repair once regurgitation had already advanced. Therefore, early primary repair before progression of the regurgitation may be the key to maintaining better competence of the atrioventricular valve.

Morphometric analysis of atrioventricular septal defect with common valve orifice.

OBJECTIVES: We sought to analyze morphometric features of atrioventricular septal defect (AVSD) in autopsy specimens and to consider the developmental implications of obstruction in either ventricular outflow tract. BACKGROUND: Left ventricular outlet obstruction (LVO) is more prevalent in patients with Rastelli type A morphology. When tetralogy of Fallot (ToF) complicates this malformation, there is usually a free-floating superior bridging leaflet. The reasons for these associations are uncertain. METHODS: In 133 hearts with AVSD and common atrioventricular (AV) valve orifice, we measured the degrees of horizontal and anterior deviation of the great arteries from the AV valve, the diameters of the ventricular outlets and the great arteries and the degree of deficiency of the ventricular septum. RESULTS: In Rastelli type A morphology, the great arteries were deviated more leftward than in type C morphology (p < 0.01). Type A hearts also had a relatively small aorta, with a long and narrow subaortic tract. The presence of obstruction in either ventricular outlet was associated with a more oblique arrangement of the great arteries, with the pulmonary trunk being more leftward than in hearts without LVO (p < 0.01). In combination with ToF, the aorta was dextroposed and the pulmonary trunk was located more posteriorly (p < 0.01). No heart with type A morphology showed ToF (p < 0.01). CONCLUSIONS: The geometric arrangement of the great arteries correlated significantly with obstruction in either ventricular outflow tract and with the Rastelli subtypes. Malrotation of the developing outlet septum may be an embryologic factor producing obstruction, with horizontal deviation of the outlets also influencing the morphology of the superior bridging leaflet.

Therapeutic use of helium-oxygen mixture in continuous positive airway pressure for early weaning from mechanical ventilation after cardiovascular surgery in infants.

Therapeutic use of helium-oxygen mixture in continuous positive airway pressure (He-CPAP) was employed for early weaning from mechanical ventilation of 11 infants who underwent cardiac surgery from August, 1974, to April, 1975. With the use of He-CPAP, a 20 to 30 mm. Hg elevation of PaO2 was usually observed and respiratory distress was reduced, as compared to results obtained with nitrogen-oxygen CPAP. In all cases, He-CPAP resulted in the possibility of relatively early removal of the endotracheal tube. Therefore, He-CPAP is recommendable for intermediate respiratory support between mechanical ventilation and nitrogen-oxygen CPAP.

A new method for prosthetic valve replacement in congenital aortic stenosis associated with hypoplasia of the aortic valve ring.

The smallest commercially available prosthetic valve has an external diameter of 17 mm. Therefore, prosthetic valve replacement has been unfeasible in cases in which the diameter of the aortic valve ring is smaller than this size. A new operative method was used on 2 patients with congenital aortic valve lesions associated with hypoplasia of the valve ring. The procedure consists of a longitudinal incision in the aortic septum placed in the midportion of the two coronary ostia, a vertical incision in the outflow tract of the right ventricle to join the septal incision, prosthetic aortic valve replacement, and patch reconstruction of the outflow tracts of both ventricles by means of two layers of a fusiform Dacron patch. A 23-year-old woman with congenital aortic stenosis associated with hypoplattic aortic valve ring underwent prosthetic placement of the aortic valve and is well 1 year after the operation. Our other patient, a 3-month-old infant with congenital aortic regurgitation, died on the first postoperative day because of postperfusion lung. However, her hemodynamic status had been good in the immediate postoperative period.

Conduit repairs of transposition complexes. A report of 14 cases.

Of 14 patients with transposition complexes undergoing conduit repairs, 10 had complete transposition of the great arteries (TGA) and 4 double-outlet right ventricle. All 14 had ventricular septal defect (VSD). Ten had pulmonary stenosis, one had an atrial septal defect, and one had patent ductus arteriosus. The ages of the patients ranged from 13 months to 18 years. Six patients were treated with nine palliative operations: four systemic-pulmonary artery shunts, three atrioseptectomies, and two pulmonary banding procedures. Four patients died immediately after the operation. Among the 10 operative survivors, there were no late deaths during the follow-up periods from 5 months through 6 years. Review of the factors influencine the early operative results has stressed the importance of correct timing of operation with or without palliative treatment, increased surgical experience, and the critical evaluation of intracardiac anatomy.

Properties of yeast expressed Aspergillus nidulans chitin synthase B which is essential for hyphal growth.

A complementary DNA of the Aspergillus nidulans chsB gene encoding chitin synthase, an essential gene for hyphal growth, was obtained by RT-PCR and expressed in Saccharomyces cerevisiae by using the GAL1 promoter in a multicopy plasmid. The biochemical characteristics of chitin synthase B (ChsB) expressed in S. cerevisiae were examined. The chitin synthase B produced in galactose medium showed zymogenicity due to activation by trypsin treatment and required Mg2+ ion to exert maximal activity. It was competitively inhibited by polyoxin D. The Ki value of the inhibitor was 10 microM, and the K(m) for the substrate was 1.6 mM. The activity was enhanced by the addition of N-acetylglucosamine. The optimal pH is 7.5 when Mg2+ is used. These characteristics are the same as those of other chitin synthases.

Valve morphology in complete atrioventricular septal defect: variability relevant to operation.

The remodeling of the atrioventricular valves in patients with complete atrioventricular septal defects is the crucial part of surgical repair. Variability in valve morphology is an important factor. This study evaluates the variability in morphology of the anterior and posterior leaflets in 30 heart specimens. All hearts had an anterior bridging leaflet: Rastelli type A in 12, type B in 2, and type C in 16. The posterior leaflet revealed four morphologic patterns: a right- and left-sided posterior leaflet, both inserting directly onto the crest of the ventricular septum (5 hearts); a common posterior leaflet attached to the septal crest by a membrane (2 hearts); a common posterior leaflet attached to the septal crest by multiple chordae (13 hearts); and a virtually free-floating posterior leaflet (11 hearts). The categorization is surgically relevant in making a distinction between hearts with and without an interventricular communication underneath the posterior leaflet. Surgically relevant variations occurred also in arrangement and positioning of chordae originating from the right septal side. There was no relationship between the Rastelli classification of the anterior leaflet and that of the posterior leaflet. The variability in morphology of the posterior leaflet and its attachments to the ventricular septum appear equally crucial for successful repair as that of the anterior leaflet.

Valvuloplasty for common atrioventricular valve regurgitation in cyanotic heart diseases.

Valvuloplasty for common atrioventricular valvular regurgitation and bidirectional cavopulmonary shunt were successfully performed in 3 children with univentricular heart and double-outlet right ventricle. The free margins of opposite leaflets of the common atrioventricular valve were sutured together to restore coaptation of the leaflets, and Kay-Reed's or DeVega's annuloplasty was carried out. Postoperative examinations revealed significant reduction of atrioventricular valve regurgitation and improvement of general condition in all patients.

Use of bovine lung heparin to obviate anaphylactic shock caused by porcine gut heparin.

Intracardiac repair in a 7-year-old girl with tetralogy of Fallot was hampered because of an anaphylactic reaction with cardiopulmonary collapse caused by porcine gut heparin. This patient had an extremely rare case of hypersensitivity to porcine gut heparin but not to bovine lung heparin. Uneventful radical operation could be performed with the use of bovine lung heparin for systemic heparinization during cardiopulmonary bypass.

Interventricular communication in complete atrioventricular septal defect.

BACKGROUND: Little attention has been paid to whether the interventricular communication in complete atrioventricular septal defect is different beneath the superior and inferior bridging leaflets, a feature of obvious surgical significance. METHODS: We searched for a defect under the bridging leaflets and examined the valve morphologies in 98 autopsied and 86 surgical patients. Of the overall specimens, 27 were associated with Fallot's tetralogy, and a further 20 had subaortic stenosis, aortic coarctation, or both. In the autopsied specimens, we also measured the degree of deficiency of the ventricular septum. RESULTS: No communication was found under the inferior bridging leaflet in 30% (29 of 98) of the specimens. All 29 hearts except two without such communications showed an undivided inferior leaflet. In contrast, all patients undergoing operation except 1 had a communication beneath both bridging leaflets (p < 0.001). The absence of a communication beneath the inferior leaflet was observed more in hearts with Fallot's tetralogy (seven of 14) or those with subaortic stenosis, aortic coarctation, or both (eight of 18) than in those without associated anomalies (14 of 66; p < 0.01). Those with a communication under the inferior leaflet showed a greater deficiency of the inlet ventricular septum than did those without it (p < 0.001). CONCLUSIONS: In a certain percentage of patients with complete atrioventricular septal defect, there will be no communication under the inferior bridging leaflet. Surgeons should be aware of this possibility, particularly when confronted with a patient with obstruction in either ventricular outlet.

Post-transfusion graft-versus-host disease following open heart surgery. Report of six cases.

Six cases of post-operative erythroderma after open heart surgery are described. About 10 days after seemingly uneventful recovery, all patients developed fever, erythroderma, liver enzyme elevation, pancytopenia, and an aplastic bone marrow. Their condition rapidly deteriorated, and they died within 20 days of the onset of symptoms. Skin biopsy specimens from two patients showed mild leukocytic infiltration in the epidermal basal layer and upper dermis. Immunostaining by the ABC method showed that most of these infiltrating cells were suppressor/cytotoxic T cells. HLA study of peripheral lymphocytes from two patients and their families revealed that the patients' HLA phenotypes were incompatible from their children's HLA findings. Y chromatin was present in the lymphocytes of the skin biopsy specimen of a female patient. Based on the clinical picture, skin biopsy, HLA study, and Y chromatin study, the authors strongly suspect post-transfusion GVHD as the etiology of postoperative erythroderma, although these patients lacked any known immunodeficiency.

[Successful surgical correction for an incomplete endocardial cushion defect in an elderly patient].

A 72-year-old female who presented with symptoms of severe congestive heart failure, was subsequently diagnosed as having an incomplete endocardial cushion defect. A severe left-to-right shunt and mitral and tricuspid valve regurgitation were noted. Closure of the ostium primum defect, mitral valve replacement, and tricuspid valve annuloplasty were performed simultaneously. The postoperative course was uneventful. A review of literature revealed that this is the oldest such surgical patient described in Japan to date. We believe that surgical correction can be effective even in elderly patients.

[One-stage surgery for Stanford type-A aortic dissection, annulo-aortic ectasia, and chronic constrictive pericarditis--a case report].

A case is reported of a rare combination of chronic constrictive pericarditis and aortic dissection. A 23-year-old male was diagnosed with constrictive pericarditis and annulo-aortic ectasia concomitant with type-A dissection. A pericardiectomy was performed under cardiopulmonary bypass, and an aortic segment, from the root to the arch, was totally replaced by a composite graft using selective cerebral perfusion. Both procedures were performed in one stage. An aged clot was found in the pericardial cavity. Sustained rupture of the aortic dissection into the pericardial cavity is considered to be a possible explanation for the development of constrictive pericarditis. The postoperative course was uneventful and the patient was discharged 34 days after the operation.

[Valvuloannuloplasty for atrioventricular valvular regurgitation in complicated cyanotic cardiac anomalies].

Valvuloannuloplasty for atrioventricular (AV) valvular regurgitation was performed in 13 patients with univentricular AV connection and double outlet right ventricle. The suturing-up of free margins of the anterior and posterior common leaflets, which divides the common orifice into two parts, was performed in 4 children with quadricuspid or quintacuspid common AV valve and effectively reduced the regurgitation. The semicircular annuloplasty with a thin GORE-TEX graft was carried out in two patients having tricuspid AV valve, and a favourable result was obtained. In bicuspid AV valvular regurgitation, two patients received the suturing-up of free margins of prolapsed leaflets, and the results were satisfactory. From these results we consider that the semicircular annuloplasty for dilated tricuspid AV valve and the suturing-up of free margins of dilated or prolapsing leaflets in quadricuspid or quintacuspid and bicuspid AV valves are the recommendable procedures. If more than mild AV valvular regurgitation remained after these valvuloplasties, the Kay's and/or DeVega's annuloplasties should be added for eliminating the regurgitation.

[Exercise capacity in children late after aortic valve replacement using the Konno procedure].

Between 1980 and 1996, aortic valve replacements using the Konno procedure were performed in 10 patients. In order to assess the latent problems after this procedure, the exercise capacity was evaluated in 6 patients (3 males and 3 females). Exercise stress tests by a bicycle ergometer with ramp protocol were performed and gas exchange responses using the mixing chamber method were measured. The mean age at the time of operation was 6.6 years (range 3 to 11) and the mean age at the time of exercise test was 11.4 years (range 8 to 15). All patients were NYHA class I except 2 patients who had residual PH. Maximum oxygen consumptions were 37 to 39 ml/kg/min in males and 23 to 34 ml/kg/min in females. Maximum heart rates ranged from 166 to 184 bpm and O2 pulses ranged from 4.6 to 7.1 ml/beat/BSA. In the 2 patients who had residual pulmonary hypertension, HR bursting and O2 pulse plateau & decline occurred, which may indicate presence of decreased stroke volume. In conclusion, all patients late after the Konno procedure could tolerate exercise beyond respiratory compensation and there were no arrhythmia during exercise. HR was satisfactorily increased but maximum oxygen consumption was lowered at about 70% of the normal level.

[Occlusion of left coronary ostium involving aortic stenosis and insufficiency].

Aortic valve replacement combined with enlargement of the valvar ring by the Nicks method and coronary artery bypass grafting via the left internal thoracic artery were performed for an 11-year-old girl with occlusion of the left coronary ostium. The left coronary sinus was covered with membranous tissue, so the left coronary ostium was not found. Aortic valve replacement with 21 mm prosthetic valve was possible by enlarging the valvar ring. The internal thoracic artery which is expected to grow with development of the body was used as the graft.