[Preservation of aortic root with severe destruction in Stanford type A acute aortic dissection].

From November 1999 to December 2008, 197 patients with Stanford type A acute aortic dissection underwent the surgical treatment on an emergency basis. In 19 cases, we preserved the severely destroyed aortic root using gelatin-resorcin-formalin (GRF) glue avoiding aortic root replacement. We examined the indication and limitation of repair of the destroyed aortic root. The 19 patients were classified into 3 groups (A, B and C). Group A consisted of 7 patients who had no aortic regurgitation (AR). Group B consisted of 6 patients who had moderate to severe AR. Group C consisted of 6 patients who had coronary involvement. We preserved the broken aortic root in group A and group B. But it seemed to be rather difficult to repair the destroyed aortic root in some cases of group C.

[Surgical treatment for acute type A aortic dissection; for better postoperative quality of life].

From November 1999 to December in 2005, 114 patients with acute type A aortic dissection underwent surgical treatment on an emergency basis. The overall in-hospital mortality was 7.9% (9 patients). Four were rupture cases before cardiopulmonary bypass. De novo postoperative stroke rate was 3.5% (4 patients). But all of them were discharged on foot. There were 6 rupture cases before operation. Unfortunately only 2 patients survived. Preoperative stroke due to malperfusion occurred in 19 cases (16.7%). Among them, those with clear consciousness had tendency to better social rehabilitation than those with drowsiness. We had experienced 2 vegetable states in the group of drowsiness after the operations. For better outcome, we must avoid rupture before operation and reconsider the timing of operation in the case of brain ischemia.

Surgical repair of double-orifice of the mitral valve in cases with an atrioventricular canal defects.

OBJECTIVE: A Double-orifice in the mitral valve is an uncommon congenital cardiac lesion which occurs as an isolated anomaly or in association with other cardiac malformation. This report deals with our surgical experience of a double-orifice of the mitral valve in cases with an atrioventricular canal defect. PATIENTS AND METHODS: From 1991 through 1999, ten patients were diagnosed to have a double-orifice of the mitral valve at Shizuoka Children's Hospital. Each patient had associated major cardiac malformations, among which atrioventricular canal defect underwent surgical management, with five of these undergoing complete correction with or without previous pulmonary artery banding. Of these 10, the five cases were enrolled in this study. Two of these had a complete type, and the other three had a partial type. The cleft in the left-sided atrioventricular valve was closed partially in four and left untouched in one. Bridging tissue, when present, was left intact. There was no regurgitation from any accessory orifice and no repair for an accessory orifice was needed. RESULT: There was no late death and no replacement of the valve with prosthesis. During follow-up ranging from 1 to 4 years, none of the patients developed severe stenosis or progressive regurgitation in the left-sided atrioventricular valve. CONCLUSION: Meticulous surgical management of a double-orifice in the mitral valve in association with atrioventricular canal defect an achieve an acceptable midterm result without developing severe dysfunction in the left-sided atrioventricular valve.

Arginine kinase from Nautilus pompilius, a living fossil. Site-directed mutagenesis studies on the role of amino acid residues in the Guanidino specificity region.

Arginine kinases were isolated from the cephalopods Nautilus pompilius, Octopus vulgaris, and Sepioteuthis lessoniana, and the cDNA-derived amino acid sequences have been determined. Although the origin and evolution of cephalopods have long been obscure, this work provides the first molecular evidence for the phylogenetic position of Cephalopoda in molluscan evolution. A crystal structure for Limulus arginine kinase showed that four amino acid residues (Ser(63), Gly(64), Val(65), and Tyr(68)) are hydrogen-bonded with the substrate arginine. We introduced three independent mutations, Ser(63) --> Gly, Ser(63) --> Thr, and Tyr(68) --> Ser, in Nautilus arginine kinase. One of the mutants had a considerably reduced substrate affinity, accompanied by a decreased V(max). In other mutants, the activity was lost almost completely. It is known that substantial conformational changes take place upon substrate binding in arginine kinase. We hypothesize that the hydrogen bond between Asp(62) and Arg(193) stabilizes the closed, substrate-bound state. Site-directed mutagenesis studies strongly support this hypothesis. The mutant (Asp(62) --> Gly or Arg(193) --> Gly), which destabilizes the maintenance of the closed state and/or perhaps disrupts the unique topology of the catalytic pocket, showed only a very weak activity (0.6-1.5% to the wild-type).

Melanoma in situ of the penis.

Melanoma of the penis is rare and the prognosis is very poor. We report a case of melanoma in situ localized on the penile shaft. Melanoma in situ of the penis is extremely rare. We emphasize that early diagnosis of melanoma in situ will improve the prognosis of melanoma of the penis.

[The repair of congenital coronary artery fistula in the neonatal period].

We operated on 10 cases diagnosed as congenital coronary artery fistula, including 2 critical neonates. Such neonates needed special care for perfusion pressure drop at the beginning of cardiopulmonary bypass and imperfect delivery of cardioplegia. This phenomenon is likely to depend on the shunt size of a fistula, that is much larger in such neonates than in older patients who could be operated on electively. It was helpful in the situation to crossclamp the main PA, compress the fistula and crossclamp the aorta quickly for a standstill. We prefer the closure of a fistula in the recipient chamber especially when an important area is supplied distally to the origin of the fistula. However, if a ventricle is a recipient chamber, the closure through coronary incision should be done as the neonates in our series. There were two hospital deaths due to vascular leakage syndrome and hypoxia, respectively. Aortic regurgitation (AR) got worse in two after the operation. They were found to have more dilatation and distortion in the Valsalva's sinus which appeared to affect the aortic annulus to some extent. It is likely due to long-standing larger shunt. Contrary to them, the patient operated on during neonatal period was followed by no increase of AR, though she had the largest shunt of our series. We have an impression it could be prevented by earlier operation.

Isolation and characterization of a thermophilic Bacillus sp. JF8 capable of degrading polychlorinated biphenyls and naphthalene.

Bacillus sp. strain JF8, which was isolated from compost, utilizes naphthalene and biphenyl as carbon sources at 60 degrees C. Biphenyl grown cells of strain JF8 barely degraded naphthalene while naphthalene grown cells did not degrade p-chlorobiphenyl, suggesting the existince of two independent degradation pathways. Isolation of JF8N, a mutant strain which can not utilize biphenyl as a carbon source while retaining the ability to utilize naphthalene, supports this hypothesis. Biphenyl grown cells of strain JF8 can degrade several polychlorinated biphenyl congeners including tetra- and pentachlorobiphenyl. bph and nah probes from mesophilic organisms failed to hybridize to strain JF8 DNA.

[Reoperation after correction of total anomalous pulmonary venous connection: particularly for postoperative pulmonary vein stenosis].

Postoperative pulmonary vein stenosis is a major complication after the correction of the total anomalous pulmonary venous connection. Six patients developed this complication 2 to 3 months after surgery. Five underwent reoperation (3 had the third operations) with only one survivor. Risk factors for developing the stenosis were 1. small common pulmonary vein with small branches occurring in the patients with the ages at the operation below 2 days and accordingly with the infradiaphragmatic connection and 2. Y-shaped common pulmonary vein with the confluence situated far below the left atrium. Turbulence seemed the major cause of the intimal thickening occurring in the common pulmonary vein shortly distant from the orifices of the branches. Typically the anastomotic orifice was not narrowed. Relief of the stenosis was done by the excision of the hypertrophied intimal tissue frequently cutting into the branches. Severe stenosis recurred in 4 patients necessitating another operation in 3 patients which was fatal in two. Prevention is important. Creation of a large anastomosis with the incision not extending into the branches and the use of the absorbable suture materials were effective. In the infradiaphragmatic connection incorporation of the divided stump of the descending vein into the anastomosis and a T-shaped incision of the left atrium were also important.

[Extended aortic arch anastomosis for coarctation of the aorta and interruption of the aortic arch in early infancy].

We performed extended aortic arch anastomosis, which was so called EAA procedure, for Coarctation of the Aorta (CoA) with hypoplastic aortic arch (HAA) and interruption of the aortic arch (IAA) in 17 infants under three months of age. The proximal anastomosis site was extended into ascending aorta in order that we could make non-obstructive pathway of systemic flow. During anastomosis, we employed mild systemic hypothermia and topical cooling of head and lumber lesion. Satisfactory anastomoses were performed without any neurological and renal complications except one case. Postoperative Doppler echographic evaluation revealed that the mean peak flow velocities at anastomotic site were under 2.0 m/sec at 1 and 2 years after surgery. We concluded that EAA procedure was useful for CoA with HAA and IAA in early infancy.

[Relief of pulmonary venous obstruction for asplenia syndrome associated with total anomalous pulmonary venous connection in neonates and infants].

Five patients of asplenia syndrome with pulmonary venous obstruction underwent TAPVC repair in the period of neonates (4 patients) and infant (1 patient). They also underwent associated procedures to reconstruct or adjust pulmonary blood flow (systemic-pulmonary shunts in 2, bilateral PDA banding in 1, pulmonary artery banding in 1) in accordance with individual anatomy of the pulmonary arteries. In four of them, they required subsequent surgical procedures for reduction of pulmonary blood flow because of intractable heart failure due to increase in pulmonary blood flow mostly at the early postoperative period. The increasing pulmonary blood flow was successfully controlled by early reduction procedures (1 patient: extrathoracically adjustable PA banding, 1 patient: tightening of shunt graft) in two patients. However, one patient died in the early postoperative period because of moribund preoperative condition. The remaining one patient underwent PA banding three month after the first operation, but died in late period due to heart failure. These results suggest that the adjustment of pulmonary blood flow is critically important for management of the patients of asplenia syndrome with TAPVC after the operation, and early decision of PA band re-adjustment (or other procedures to decrease pulmonary blood flow) is mandatory to improve the results.

Immunogenetic analysis of silicosis in Japan.

We previously reported that a gene in linkage disequilibrium with HLA-Bw54, DR4, and DRw53 might control the susceptibility to silicosis (K. Honda et al. 1988. N. Engl. J. Med. 319:1610). To further define the HLA-linked gene and other genetic factors for predisposition of silicosis, we determined for HLA-DQ and DP alleles using the polymerase chain reaction and sequence-specific oligonucleotide probes and made a restriction fragment length polymorphism (RFLP) analysis of the fourth component of complement (C4) genes, immunoglobulin lambda variable chain (IGLV) gene, and T-cell receptor alpha and beta genes in 46 Japanese patients with silicosis. The frequency of DQB1*0401 (relative risk [RR] = 2.2, P < 0.02) was increased and that of DQB1*0601 (RR = 0.36, P < 0.01) was decreased in the patients. RFLP analysis of C4 and IGLV genes showed significant association between silicosis and a specific RFLP pattern of C4A3-C4B5 allotype (RR = 2.3, P < 0.05) and that of IGLV 5.3 kb (RR = 0.33, P < 0.003). No other genetic markers showed significant association. Statistical analyses of the associated genetic markers revealed that the HLA-Bw54 was the allele that showed primary association with silicosis and the frequencies of the C4 and HLA-DQ alleles were suggested to be increased due to their linkage disequilibrium with the HLA-Bw54. We conclude that the major gene for silicosis may be mapped near the HLA-B locus.

[Postoperative management in neonate after open cardiac surgery].

Recent advances in diagnostic method and preoperative care have allowed us to perform surgical repair in neonate. Important to the successful outcome of open cardiac surgery in neonate is cautious management in postoperative period. So we investigated the hemodynamics in the first 72 hours following open heart surgery for TAPVC, TGA and PAIVS. The hemodynamics in acute period are different according to patient age. Especially, early neonates under 14 days present low systemic blood pressure and high central venous pressure. So we must manage them according to their hemodynamic characteristics.

[Silicosis and lung cancer].

A clinicopathological analysis was carried out on 50 cases of lung cancer with silicosis diagnosed from April 1975 to March 1988. All patients were males and the age distribution ranged from 47 to 85 years with a mean of 63.5 at diagnosis. They had been exposed to silica in tunneling for 3 to 42 years, with an average of 15.1. Forty eight cases smoked. Histologically, squamous cell carcinoma was the most common with 29 cases, followed by 10 small cell carcinomas, 6 adenocarcinomas, 4 large cell carcinomas and one adenosquamous carcinoma. Thirty seven tumors were located in peripheral regions, mostly upper lobe or S6, while 13 tumors were in large bronchi. As the most common histological types of lung cancer with silicosis were squamous cell carcinoma and small cell carcinoma, some carcinogens might be involved in tumorigenesis. Silica alone is not considered to be a carcinogen, however, silica containing adsorbed polycyclic aromatic hydrocarbons from cigarette smoking or from industrial pyrolysis can act as a carcinogen or promoter.

[Neutrophil chemiluminescence in pneumoconiosis].

Neutrophilic phagocytic activity and serum opsonic activity were examined in pneumoconiosis patients. One hundred patients aged 43 to 81 were classified into four types (types 1 to 4) according to the degree of chest X-ray findings. A simultaneous multiple measurement system for luminol-dependent chemiluminescence was used. Neutrophilic phagocytic activity and serum opsonic activity were shown by the time length to reach peak chemiluminescence (PT) and the height of peak chemiluminescence (PH). There were no significant differences of neutrophilic phagocytic activities and serum opsonic activities between normal persons and type 1 cases of mild disease. However, these activities were elevated in cases with more severe disease, and the most severe type 4 type cases tended to show lower activities than type 2 and type 3 cases. PT of total chemiluminescence was influenced by both neutrophilic phagocytic activity and serum opsonic activity, but PH only by neutrophilic phagocytic activity. Statistically significant correlation was observed between serum opsonic activity and serum CH50 levels.

A patient with hereditary spherocytosis and silicosis who developed an IgA(lambda) monoclonal gammopathy.

A patient with hereditary spherocytosis and silicosis who developed an IgA(lambda) monoclonal gammopathy is reported. It was thought that a combination of hereditary factors, a chronic hemolytic stimulus to the reticuloendothelial system and an adjuvant effect provided by presence of diffuse silica might have contributed to the development of monoclonal gammopathy.