Two cases of atretic cephalocele, and histological evaluation of skin appendages in the surrounding skin.

Atretic cephalocele is a small skin-covered lesion, usually located at or near the mid-line of the scalp. Histologically, it is composed of syncytial cells expressing neurone-specific enolase and epithelial membrane antigen. The syncytial cells form capillary-like structures *(pseudovascular areas) and collagenic fibrosis with densely packed collagen bundles (fibrous areas). Such findings suggest that the atretic cephalocele is a mild form of cephalocele, with its pathogenesis lying in the spectrum of neural tube closure abnormalities. However, few descriptions of abnormalities of the skin overlying and surrounding atretic cephalocele are available. We report two cases of atretic cephalocele that showed hamartomatous change in the surrounding cutaneous appendages. These findings suggest that atretic cephalocele is associated with abnormalities not only of the neural tube, but also of the surrounding skin.

Skin lesions in a patient with IgG4-related disease.

IgG4-related disease (IgG4-RD) is a newly recognized condition that is characterized by raised levels of serum IgG4, tissue infiltration of IgG4-positive plasma cells and presence of fibrosis. It affects multiple organs, including the pancreas, bile duct, and lacrimal and salivary glands. Skin lesions have rarely been reported, and those that have were described as distributed mainly in the head and neck region. We report a case of IgG4-RD with autoimmune pancreatitis and skin lesions on the trunk and limbs. The skin lesions responded well to oral prednisolone (PSL); however, tapering of PSL to 5 mg/day resulted in recurrence. At present, the skin disease is well controlled at a dose of 7 mg/day. Interestingly, IgG4 levels fluctuated with changes in the PSL dose but did not coincide with the severity of the skin disease, implying that the raised levels of IgG4 did not directly influence the skin symptoms.

Primary leiomyosarcoma of the pulmonary artery confirmed by catheter suction biopsy.

A patient with clinical features consistent with pulmonary embolism showed no improvement despite therapy with tissue-plasminogen activator and full-dose heparin. Transvenous catheter suction biopsy was successful in establishing an antemortem histologic diagnosis of primary pulmonary artery leiomyosarcoma. Urgent surgical intervention was performed.

Molecular cloning and functional characterization of rat plasma protein S.

Plasma protein S is a cofactor of activated protein C (APC) in the regulation of the blood coagulation system. Rat protein S homogeneously purified from plasma showed cofactor activity for rat APC, but not for human APC when the APC cofactor activity was assayed using protein S- and C4b-binding protein (C4BP)-depleted human plasma. Rat plasma protein S was separated by gel chromatography into two forms, a free form and a form complexed with C4BP. Rat protein S forms complexes with rat and human C4BP in a solid-phase model with apparent dissociation constants (Kds) of 6.7 x 10(-8) and 1.2 x 10(-8) M, respectively, in the presence of 5 mM Ca2+. Human protein S also forms a complex with solid-phase human and rat C4BP with Kds of 6.3 x 10(-9) and 2.7 x 10(-8) M, respectively. Human C4BP strongly inhibited the APC cofactor activity of both human and rat protein S, whereas rat C4BP was only weakly inhibitory. The degree of the inhibitory activity of C4BP appears to depend on the affinity between protein S and C4BP. In order to evaluate the structure-function relationship of the rat protein S, the complete cDNA sequence of rat protein S was determined. This cDNA of 3,315 bp was composed of a 103-bp 5'-noncoding region, a 2,028-bp coding region that encodes a preprosequence of 41 amino acids, a mature protein S of 634 amino acids and a stop codon, and a 1,184-bp 3'-noncoding region. The rat mature protein S consisted of domains with distinct functions similar to those of human protein S, and with two potential Asn-linked glycosylation sites. The amino acid sequence of the mature form of rat protein S showed 80.4, 78.7, and 79.7% identity with those of human, bovine, and rabbit mature protein S, respectively. These findings suggest that despite the species-specificity of the APC cofactor activity of rat protein S, it is structurally very similar to human protein S. Expression of rat protein S mRNA (approximately 3.5 kb) was demonstrated by RNA blot analysis not only in the liver, but also in the lung, spleen, testis, and uterus of rats.

Surgical repair of extracardiac unruptured acquired Valsalva aneurysms.

Two cases of extracardiac unruptured Valsalva aneurysms due to rare causes are reported. One patient had been suffering from hyper eosinophilic syndrome. Operative corrections consisted of total replacement of the aortic root. The other patient had an aneurysm of just noncoronary sinus of Valsalva and a dilated ascending aorta due to cystic mucoid degeneration. Replacement of the ascending aorta with patch closure for the aneurysm was successfully performed.

Long-term follow-up after Carpentier-Edwards ring annuloplasty for tricuspid regurgitation.

BACKGROUND: Use of flexible rings for tricuspid ring annuloplasty is becoming popular. This study was undertaken to evaluate Carpentier-Edwards (C-E) rigid ring annuloplasty for tricuspid regurgitation (TR), secondary to mitral valve disease and clinical outcome on a long-term basis. METHODS: From December 1985 to March 1996, 45 patients with secondary TR underwent C-E ring annuloplasty. Thirty-nine patients (95.1%) were in New York Heart Association (NYHA) functional class III or IV. The mean follow-up was 96.7+/-48.5 months or 362.6 patient-years. RESULTS: There were three in-hospital and nine late deaths that were not related to tricuspid annuloplasty. Actuarial survival at 10 years was 68.3%. Echocardiographic studies showed that TR was well controlled within grade 2+ in all survivors. Residual pulmonary hypertension (PH) was recognized in 9 of 21 patients (42.9%) with preoperative PH, however, no TR was seen in 6 patients. A TR grade of 2+ was observed in 3 patients. Thirty of the total survivors (96.8%) were in NYHA class I and II, but 1 patient was in NYHA class III. The actuarial rate of freedom from tricuspid valve reoperation after 10 years was 97.5%. CONCLUSIONS: C-E ring annuloplasty is acceptable for repair of secondary TR and improvement in clinical status on a long-term basis.

Accomplishment of time-interval activation of esterase A4 by simple removal of pin fraction.

In the seasonal cycle of the silkworm Bombyx mori, an ATPase called esterase A4 (EA4) is thought to measure time interval as a diapause-duration timer. To address the mechanism by which EA4 measures the time, we present a simple EA4 screening method. By the method, EA4 activity can be assessed with a short incubation at 25 degrees C without the need to purify the enzyme further. The method largely overcomes methodological problems that remain unanswered. Besides, the results obtained by the method establish that the time measurement is based on the moment when the time-holding factor(s) PIN (peptidyl-inhibitory needle) is removed. EA4 may originally be a complex with PIN, and external time cues such as winter cold may induce the dissociation of the complex, which in turn results in the timer activation of EA4.

Aortoesophageal fistula-relief of massive hematemesis with an endovascular stent-graft.

A 59-year-old man with an esophageal carcinoma developed massive hematemesis due to aortoesophageal fistula after irradiation therapy reached 58 Gy. Emergent treatment with an endovascular stent-graft was successfully performed and the patient followed an uneventful course until he died of pneumonia 4.5 months later, which was caused by a tracheoesophageal fistula. Stent-graft repair is a safe and effective method to treat aortoesophageal fistula and may be an alternative to surgical resection.

[The effect and role of the intraaortic balloon pumping in off-pump coronary artery bypass].

Eighty consecutive patients who underwent off-pump coronary artery bypass (OPCAB) were studied. They were divided into group I (n = 10) which received preoperative intraaortic balloon pumping (IABP), and group II (n = 70) which did not receive IABP. The indications for preoperative IABP were severe left main coronary artery disease in 7 patients, severe 3 vessel disease in 3 patients, unstable angina in 5 patients, acute myocardial infarction in 3 patients. There was no operative mortality in both groups. The average number of distal anastomosis 2.7/patients in group I and 3.3/patients in group II. There was no differences in ventilator support time, length of stay in the intensive care unit and morbidity between 2 groups. The average postoperative IABP support time was 5.4 hours. There was no IABP-related complication in group I. IABP was very effective to perform OPCAB surgery safety. Preoperative IABP may be effective modality to support OPCAB surgery not only in emergent case but also in elective case.

[The effects of intravenous milrinone for the patient undergoing CABG].

The hemodynamic effects of intravenous infusion of milrinone were evaluated in 25 patients undergoing CABG using an internal mammary artery graft. Milrinone was administered to 9 patients at the time of weaning from cardiopulmonary bypass, at a dosage of 3 to 5 micrograms/kg/min. Postoperative cardiac function was compared in this group versus the other 17 patients who were treated without milrinone. We determined such parameters as cardiac index, wedge pressure and mean pulmonary pressure. Our findings did not show any significant difference between the 2 groups. We also studied a subject of low-output patients (EF < 0.5). In the patients with low-cardiac output, the use of milrinone in addition to standard postoperative administration of low-dose dopamine reduced mean pulmonary pressure and wedge pressure. Thus, milrinone not only improved the left ventricular function, but also expanded the pulmonary vascular bed.

[Results of surgical treatment for pT3 primary lung cancer].

We reviewed pT3 lung cancer for 86 cases (13.1%) out of 659 cases treated surgically for primary lung cancer between 1985 and march 1998. Five-year and ten-year survival rates for all pT3 cases were 48% and 40% respectively and those for pT3N0M0 cases were 67.2%. The operative mortality between 1990 and 1998 (2.4%) was better than that between 1985 and 1989 (6.7%). The extensive resection for pT3 lung cancer was evaluated to be appropriate. However, the prognosis of the patients who underwent combined resection of mediastinal pleura, pericardium or diaphragm was very poor. Five-year survival rate was significantly worse in patients with N2 disease (17.3%) than in patients with N0 disease (65.8%) (p < 0.05). Although the surgical indication for the patients with mediastinal pleura, pericardium or diaphragm disease and N2 disease is still controversial, there is not the extensive surgical indication.

Preoperative disinfection of the conjunctival sac in cataract surgery.

Conjunctival cultures were obtained from 488 patients undergoing cataract extraction. Bacterial isolation rates from the lids and conjunctiva were 84.6 and 36.7, respectively. Propionibacterium acnes was the only anaerobic organism identified. A significantly lower incidence of P. acnes was observed in the eyes after preoperative conjunctival irrigation using povidone-iodine solution (9%) compared with those using benzetonium chloride solution (30%). Topical use of ofloxacin and cefmenoxime for 1 or 2 days preoperatively, and irrigation of the conjunctival sac with a 16-fold-diluted povidone-iodine solution for at least 30 s immediately prior to cataract surgery are recommended.

Purification and properties of methyl formate synthase, a mitochondrial alcohol dehydrogenase, participating in formaldehyde oxidation in methylotrophic yeasts.

Methyl formate synthase, which catalyzes methyl formate formation during the growth of methylotrophic yeasts, was purified to homogeneity from methanol-grown Candida boidinii and Pichia methanolica cells. Both purified enzymes were tetrameric, with identical subunits with molecular masses of 42 to 45 kDa, containing two atoms of zinc per subunit. The enzymes catalyze NAD(+)-linked dehydrogenation of the hydroxyl group of the hemiacetal adduct [CH2(OH)OCH3] of methanol and formaldehyde, leading to the formation of a stoichiometric amount of methyl formate. Although neither methanol nor formaldehyde alone acted as a substrate for the enzymes, they showed simple NAD(+)-linked alcohol dehydrogenase activity toward aliphatic long-chain alcohols such as octanol, showing that they belong to the class III alcohol dehydrogenase family. The methyl formate synthase activity of C. boidinii was found in the mitochondrial fraction in subcellular fractionation experiments, suggesting that methyl formate synthase is a homolog of Saccharomyces cerevisiae Adh3p. These results indicate that formaldehyde could be oxidized in a glutathione-independent manner by methyl formate synthase in methylotrophic yeasts. The significance of methyl formate synthase in both formaldehyde resistance and energy metabolism is also discussed.

Five-year follow-up of valve replacement with the Jyros bileaflet mechanical valve.

Jyros bileaflet rotating valves were implanted as a clinical trial conducted in Japan, and the 5-year results were assessed. Nineteen patients underwent implantation of the valves: 14 in the mitral and 5 in the aortic position. The mean follow-up period was 65.4+/-15.7 months. There was 1 case of late death due to fatal arrhythmia and another case of cerebral thromboembolism (1.0% per patient year). All survivors were in New York Heart Association class I. On the early postoperative cinefluorography, 8 valves (42.1%) showed rotation of the leaflets. However, in the latest assessment 6 valves (33.3%) showed rotation, some valves had stopped rotation, and others had started to rotate during the follow-up period. The Jyros valve functions effectively, similar to other bileaflet valves. However, because the correlation between thromboembolism and the rotation mechanism is not clear, further follow-up of our patients and more implant studies are necessary to elucidate this issue.

Endothelin receptor antagonist triterpenoid, myriceric acid A, isolated from Myrica cerifera, and structure activity relationships of its derivatives.

As the first non-peptide endothelin receptor antagonist from a higher plant, a new triterpenoid, myriceric acid A (50-235) (1) was isolated from the bayberry, Myrica cerifera. Myriceric acid A (1) inhibited not only an endothelin-1-induced increase in cytosolic free Ca2+ concentration (IC50 = 11 +/- 2 nM) but [125I]endothelin-1 binding in rat aortic smooth muscle cells (Ki = 66 +/- 15 nM). Two new related triterpenoids, myriceric acid C (6), and myriceric acid D (8), were also isolated. Furthermore, the chemical modification of these natural products led to the synthesis of sulfated derivatives (13, 14, 15) which showed 1.5 to 20 times higher affinity for endothelin receptors. The structure activity relationships of myriceric acids and their derivatives are discussed.