Measurement of the first ionization potential of lawrencium, element 103.

The chemical properties of an element are primarily governed by the configuration of electrons in the valence shell. Relativistic effects influence the electronic structure of heavy elements in the sixth row of the periodic table, and these effects increase dramatically in the seventh row--including the actinides--even affecting ground-state configurations. Atomic s and p1/2 orbitals are stabilized by relativistic effects, whereas p3/2, d and f orbitals are destabilized, so that ground-state configurations of heavy elements may differ from those of lighter elements in the same group. The first ionization potential (IP1) is a measure of the energy required to remove one valence electron from a neutral atom, and is an atomic property that reflects the outermost electronic configuration. Precise and accurate experimental determination of IP1 gives information on the binding energy of valence electrons, and also, therefore, on the degree of relativistic stabilization. However, such measurements are hampered by the difficulty in obtaining the heaviest elements on scales of more than one atom at a time. Here we report that the experimentally obtained IP1 of the heaviest actinide, lawrencium (Lr, atomic number 103), is 4.96(+0.08)(-0.07) electronvolts. The IP1 of Lr was measured with (256)Lr (half-life 27 seconds) using an efficient surface ion-source and a radioisotope detection system coupled to a mass separator. The measured IP1 is in excellent agreement with the value of 4.963(15) electronvolts predicted here by state-of-the-art relativistic calculations. The present work provides a reliable benchmark for theoretical calculations and also opens the way for IP1 measurements of superheavy elements (that is, transactinides) on an atom-at-a-time scale.

Involvement of Acetobacter orientalis in the production of lactobionic acid in Caucasian yogurt ("Caspian Sea yogurt") in Japan.

Lactobionic acid was first found in a Caucasian fermented milk product popularly known as "Caspian Sea yogurt" in Japan. The presence of lactobionic acid in the fermented milk was indicated by the results of both high-performance anion-exchange chromatographic analysis with pulsed amperometric detection and mass spectrometric analysis. Thereafter, the acid was purified from the yogurt and analyzed by nuclear magnetic resonance. A substantial amount of lactobionic acid was found to be accumulated in the upper layer of the yogurt, especially within 10 mm from the surface. A total of 45 mg of lactobionic acid per 100 g of the upper yogurt layer was collected after 4 d of fermentation. The annual intake of lactobionic acid in individuals consuming 100 g of the yogurt every day would be 0.5 to 1.0 g. A lactose-oxidizing bacterium was isolated from the fermented milk and was identified as Acetobacter orientalis. Washed A. orientalis cells oxidized monosaccharides such as d-glucose at considerable rates, although their activities for substrates such as lactose, maltose, and cellobiose were much lower. When A. orientalis cells were cultivated in cow's milk, they exhibited lactose-oxidizing activity, suggesting that this bacterium was the main organism involved in the production of lactobionic acid in the yogurt.

The use of magnetic resonance imaging in diagnosing infantile neuroaxonal dystrophy.

We used MRI to investigate the brains of four children ranging from 3 to 10 years of age with infantile neuroaxonal dystrophy. T2-weighted imaging revealed characteristic findings of marked cerebellar atrophy and diffuse hyperintensity of the cerebellar cortex. At autopsy, one child had extensive astrogliosis and neuronal loss with shrinkage of the cerebellar cortex, in addition to typical widespread changes of neuroaxonal dystrophy. The characteristic hyperintensity of the cerebellar cortex on T2-weighted imaging probably is secondary to the extensive gliosis and shrinkage of the cerebellar cortex. These cerebellar findings on MRI may permit early diagnosis of infantile neuroaxonal dystrophy.

A new type of fulminant group A streptococcal infection in obstetric patients: report of two cases.

A new type of fulminant group A streptococcal infection in obstetric patients is described. The illness occurs in late stage of pregnancy, and is preceded by an episode of upper respiratory tract infection. This is followed by sudden onset of septicemia, subsequent hematogenous infection of the myometrium by the bacteria, and development of acute purulent myometritis. Shock and multiorgan failure ensue rapidly. Prognosis of both the mother and fetus is very poor, as recognition of this serious condition is difficult until the late stage of the disease. This type of infection is entirely different from classical puerperal sepsis in that the illness starts before delivery, and that there was no evidence of ascending bacterial infections of the birth canal, such as acute endometritis or chorioamnionitis, in affected mothers. The underlying mechanism for this serious infection remains unknown.

Familial amyotrophic lateral sclerosis with posterior column degeneration and basophilic inclusion bodies: a clinical, genetic and pathological study.

We report an autopsy case of familial amyotrophic lateral sclerosis (FALS). The patient was a Japanese woman with hereditary burden. Family history revealed 12 patients with FALS over four generations. She developed muscle weakness of the proximal part of the upper extremities at age 42, followed by dysarthria, dysphagia, muscle weakness and atrophy in the lower extremities, spasticity, hyperreflexia and Babinski's sign. At age 44, she needed ventilatory support. At age 45, she died of bronchopneumonia. The total duration of the disease was three years and one month. Genetic study showed the absence of a mutation in the Cu/Zn superoxide dismutase-1 gene. Neuropathological examination revealed not only neuronal loss in the upper and lower motor neuron and Clarke's column, but also degeneration of the pyramidal tracts, middle root zone of the posterior column and posterior spinocerebellar tract. Bunina bodies and Lewy body-like inclusion bodies were absent. A few basophilic inclusion bodies were present in the neurons of the brain stem and anterior horn of the lumbar cord. Based on these clinical, genetic and pathological findings with a review of the literature, we concluded that our case was the first reported case of FALS with posterior column involvement and basophilic inclusion bodies.

[Purpura fulminans complicating pneumococcal sepsis: a case report].

An unusual case of a 67-year-old man is reported with fulminant pneumococcal sepsis. He had been healthy before, and the identified predisposing factors were only that he was a chronic alcohol drinker and was a HCV carrier. He presented signs of acute renal failure, liver dysfunction, adult respiratory distress syndrome and disseminated intravascular coagulation. Subsequently purpura fulminans (symmetrical peripheral gangrene) with major extremity involvement developed. He finally survived with amputation of both legs, right forearm and two fingers of left hand. Purpura fulminans is a rare catastrophic disease, with initial hemorrhagic skin lesions that progress to gangrene. It usually follows an infectious illness, and although it most commonly occurs in children, it can occur in adults with predisposing factors such as alcoholic, asplenia, AIDS and so on. In adults, pneumococcus and meningococcus are microorganisms that have been reported most frequently as caused agents in Europe and America. But in Japan the previously reported adult case was the only one complicating Xanthomonas maltophilia sepsis, and none accompanying pneumococcal sepsis. Congenital protein C deficiency is recognized to be able to cause purpura fulminans especially in patients with risk factors. In our case, protein C antigen was decreased in the acute stage but gradually increased later toward normal, so this decrease was thought to be concomitant with the initial disseminated intravascular coagulation rather than compatible with protein C deficiency.

[Case report of toxic shock-like syndrome due to group A streptococcal infection].

A case of streptococcal toxic shock like syndrome occurring in a 44-year-old previously healthy Japanese male is reported. He initially had a sore throat, low grade fever, diarrhea and mild pains in a lower extremities. Shortly thereafter, he rapidly developed a high fever, profound hypotension, multifocal epidermal necrosis, and sever purulent fasciitis and myositis in both lower extremities, which required above knee amputation of both legs. He later developed disseminated intravascular coagulopathy, adult respiratory distress syndrome, acute renal failure, coma and necrotizing inflammation of both arms and external genitalia despite treatment. He died on the 13th hospital day. Streptococcus pyogenes was isolated from the necrotic muscles and right knee joint. The organism was typed as M3, T3, was sensitive to penicillins, and was found to be producing streptococcal pyogenic exotoxin A in vitro. This is the first case report of streptococcal toxic shock like syndrome in Japan.

[Fulmonant group A streptococcal infection accompanied by massive pulmonary hemorrhage and subsequent asphyxia: a case report].

A case of fulminant group A streptococcal infection occurring in a 6-year-old Japanese child is reported. She was accompanied by massive pulmonary hemorrhage and subsequent asphyxia. She initially had pharyngalgia with fever. The cephalosporin antibiotic was given orally for 3 days. Three days after that recurrence of fever and pharyngalgia was noted. Twelve hours later tachypnea and a sudden onset of hemoptysis was noted. She manifested DOA (dead on arrival) and died in the emergency room. Autopsy revealed the presence of numerous cocci in the vessels and massive pulmonary hemorrhage. Streptococcus pyogenes was isolated from the blood. The serotype of this group A streptococcal organism was typed as M4, T4, which produces exotoxin type B and C, which was sensitive to the penicillins.

[A case report of toxic shock-like syndrome associated with prevalence of streptococcal pharingitis in the family].

A case of streptococcal toxic shock-like syndrome in a previously healthy, 57 year old Japanese female has been reported. Initially, she had a sore throat and low grade fever for 5 days. Because of sudden severe pain on the extremities and erythema on bilateral forearms, she was hospitalized. On admission, her conciousness was clear. Although profound hypotension, anuria and prolonged blood coagulation were observed. Antibiotics, fluid therapy and dopamine were given. Four hours after admission, she died in spite of resuscitation efforts, by sudden cardiac arrest. Streptococcus pyogenes was isolated in her blood. At the same time as when she died, three of the five people of the patient's family living with her, had pharingitis or pneumonia. From the pharynxs of the three people with pharingitis, Streptococcus pyogenes was also isolated. The serotype of all organisms was T11, and they produced exotoxintype B in vitro. This case suggests that infection of Streptococcus pyogenes is not essential for the development of toxic shock-like syndrome.

[Report of surveillance on streptococcal toxic shock syndrome in Japan and presentation of the criteria].

A survey was made on the situation of Group A Streptococcal toxic shock syndrome (STSS) based on questionnaires. The survey was divided into two parts. The first survey was done by sending out an outline of the STSS inquiring if any STSS cases were observed by mail to university hospitals, residence training hospital and other major hospitals totaling 2512 institutes. The second survey was subsequently done to the institutes that had STSS cases asking for the clinical course, data and sampling of the bacteria. The diagnosis of STSS was confirmed based on the diagnostic criteria induced by the working group of the United States. We have found 97 cases of STSS which 48.5% had fatal outcomes. There was no significant sex difference in the onset or the mortality rate. It occurred more in the older population, and occurred through out Japan but was not found to be epidemic. The first case was backed in 1978 and it began to increase since 1993, reaching its peak in 1994 and now decreasing in number. Most of the isolated Group A streptococcus were of type M1 and M3. We have modified the United States diagnostic criteria creating a new Japanese criteria, which includes the symptoms of the central nervous system in the term MOF. The aim for the Japanese criteria is to search for the etiology of the disease. The Japanese criteria requires that the disease progresses rapidly and that the patient be free from any conditions that might suppress the immunal system.

[Fulminant streptococcal infection and sudden death in a pregnant woman: a case report].

A case of fulminant streptococcal infection occurring in a 28-year-old pregnant female is reported. She initially developed pharyngitis and high fever during the 33rd week of gestation. This was treated with oral piperacillin for two days with temporary amelioration. Recurrence of high fever, however, was noted shortly thereafter, which was followed by stillbirth of twin babies, and subsequent development of refractory hypotension and demise of the mother. Autopsy revealed presence of numerous cocci and fibrin thrombi in systemic circulation, and this was thought to be the immediate cause of her death. The causative organism was identified as Streptococcus pyogenes, M3, T3, and was found to be producing streptococcal pyrogenic exotoxin A in vitro. The underlying mechanism for this serious infection remains unknown.

[Evaluation of cross circulation in conjoined twins].

This report describes the evaluation of cross circulation in conjoined twins. Female thoracoomphalopagus conjoined twins were delivered by cesarean section after 37 week's gestation. CT, MRI and echography were performed. A partial communication of pericardium and sternum, and a union of the liver were found. For the preoperative evaluation of cross circulation, a bolus of indigo carmine was injected, and the pigment appeared in the urine of the other twin. RI angiography showed that radionuclides in one twin were similar to those in the other after 5-10 minutes. During the operation, an injected bolus of SCC to one twin was not effective for the other twin. Inhaled isoflurane in one twin did not appear in the expired gas of the other twin. After the intentional hemorrhage for 3 minutes from one twin, the hemoglobin concentration of one of the twin was the same as that of the other. This demonstrates that acute hemorrhage from one twin seems to result in a significant and rapid decrease of hemoglobin in both babies equally. However, intravenous infusion of drugs to one of the twins requires a relatively long time to take effect in the other baby. We must be careful in anesthetic management of the surgical separation of conjoined twins.

Nuclear chemistry. Synthesis and detection of a seaborgium carbonyl complex.

Experimental investigations of transactinoide elements provide benchmark results for chemical theory and probe the predictive power of trends in the periodic table. So far, in gas-phase chemical reactions, simple inorganic compounds with the transactinoide in its highest oxidation state have been synthesized. Single-atom production rates, short half-lives, and harsh experimental conditions limited the number of experimentally accessible compounds. We applied a gas-phase carbonylation technique previously tested on short-lived molybdenum (Mo) and tungsten (W) isotopes to the preparation of a carbonyl complex of seaborgium, the 106th element. The volatile seaborgium complex showed the same volatility and reactivity with a silicon dioxide surface as those of the hexacarbonyl complexes of the lighter homologs Mo and W. Comparison of the product's adsorption enthalpy with theoretical predictions and data for the lighter congeners supported a Sg(CO)6 formulation.

In vitro measurement of tissue integrity during saccular aneurysm embolizations for simulator-based training.

In the domain of endovascular neurosurgery, the measurement of tissue integrity is needed for simulator-based training and for the development of new intravascular instruments and treatment techniques. In vitro evaluation of tissue manipulation can be achieved using photoelastic stress analysis and vasculature modeling with photoelastic materials. In this research we constructed two types of vasculature models of saccular aneurysms for differentiation of embolization techniques according to the respect for tissue integrity measurements based on the stress within the blood vessel model wall. In an aneurysm model with 5 mm dome diameter, embolization using MicroPlex 10 (Complex 1D, with 4 mm diameter loops), a maximum area of 3.97 mm² with stress above 1 kPa was measured. This area increased to 5.50 mm² when the dome was touched deliberately with the release mechanism of the coil, and to 4.87 mm² for an embolization using Micrusphere, (Spherical 18 Platinum Coil). In a similar way trans-cell stent-assisted coil embolization was also compared to human blood pressure simulation using a model of a wide-necked saccular aneurysm with 7 mm diameter. The area with stress above 1kPa was below 1 mm² for the pressure simulation and maximized at 3.79 mm² during the trans-cell insertion of the micro-catheter and at 8.92 mm² during the embolization. The presented results show that this measurement system is useful for identifying techniques compromising tissue integrity, comparing and studying coils and embolization techniques for a specific vasculature morphology and comparing their natural stress variations such as that produced by blood pressure.

Pathogenesis of hypofibrinogenemia in familial hemophagocytic lymphohistiocytosis.

In order to investigate the cause of hypofibrinogenemia in familial hemophagocytic lymphohistiocytosis (FHL), formalin-fixed and paraffin-embedded tissue sections of the spleen obtained at autopsy were examined by using immunohistochemical methods for the presence of fibrinogen antigens, and such antigens were detected in approximately 10% of the histiocytes in a diffuse staining pattern. This finding indicates uptake of fibrin and/or fibrinogen molecules by activated histiocytes and suggests that hypofibrinogenemia of FHL is caused by the direct action of activated histiocytes on factor X through Mac-1 receptors, subsequent activation of the common pathway of the coagulation protease cascade, and uptake of fibrin and/or fibrinogen molecules by such cells.

Severe pulmonary hemorrhage in patients with serious group A streptococcal infections: report of two cases.

Severe pulmonary hemorrhage was observed in two patients who died of serious group A streptococcal infections. These two patients initially presented with fever and sore throat. This was followed by sudden onset of septicemia caused by the bacteria and by the subsequent development of severe pulmonary hemorrhage. Hemoptysis, cyanosis, and dyspnea were observed prior to death in both cases. This pulmonary lesion resulted in asphyxia and sudden death in one patient. Pathological examinations of the lung revealed severe intraalveolar hemorrhage, with no evidence of inflammation or necrosis of the pulmonary tissue. There was no evidence of aspiration of blood due to hemorrhage in the upper respiratory or alimentary tract. This visceral lesion appears to be an hitherto undescribed, novel clinicopathologic feature of patients with serious group A streptococcal infections.

[Case of synovial sarcoma in an infant].

The case of a 4-month old boy with monophasic synovial sarcoma of the left thigh is reported. He died of pulmonary metastasis 11 months after surgical removal of the primary tumor. Histologically, the tumor was composed of densely packed, small spindle cells; there were occasional islands and clusters of polygonal cells. Ultrastructurally, the closely packed polygonal cells displayed occasional desmosomelike attachments between apposing cytoplasmic membranes of neighboring cells. Many small abortive duct-like intercellular spaces with elongated cytoplasmic filopodias were seen. These findings were suggestive of epithelial differentiation of some of the tumor cells. In general, synovial sarcoma is a disease of young adults and is rare in children.

Transmission of Streptococcus pyogenes causing toxic shock-like syndrome among family members and confirmation by DNA macrorestriction analysis.

Streptococcus pyogenes can cause severe infections, such as toxic shock-like syndrome (TSLS). The transmission and pathogenesis of TSLS are poorly understood, and information is needed to develop prevention strategies. Four cases were identified in which the organism was transmitted among patients with TSLS and their family members. DNA macrorestriction endonuclease analysis using pulsed-field gel electrophoresis demonstrated the spread of S. pyogenes clones that caused TSLS among the family members. Although 14 persons related to the case-patients experienced only colonization or self-limited disease, 3 developed invasive infections (pneumonia, severe pharyngitis requiring hospitalization, and puerperal sepsis). These findings indicate that antimicrobial prophylaxis for close contacts of patients with TSLS should be considered and reinforce the need for further studies on epidemic control of TSLS.

Constant involvement of the Betz cells and pyramidal tract in multiple system atrophy: a clinicopathological study of seven autopsy cases.

We investigated clinicopathologically the pyramidal signs, including spasticity, hyperreflexia, and Babinski's sign, and the involvement of the pyramidal tract and primary motor cortex, in seven Japanese autopsy cases of multiple system atrophy (MSA). Pyramidal signs were observed in six (86%) of the seven autopsy cases. Hyperreflexia and Babinski's sign were each evident in five patients, but spasticity was observed in only one patient. Loss of Betz cells and presence of glial cytoplasmic inclusions in the primary motor cortex were noticed in all seven cases. Astrocytosis in the fifth layer of the primary motor cortex was noticed in five cases, but its presence was not related to the duration of the disease. Involvement of the pyramidal tract in the spinal cord, particularly of the small myelinated fibers, was observed in all seven cases, but no involvement of the pyramidal tract in the midbrain was evident in any of the six cases in which this structure was examined. In MSA, pyramidal signs were shown to be present more frequently than believed before, and the clinicopathological correlation between pyramidal signs and involvement of the pyramidal tract was obvious. Constant involvement of Betz cells in MSA has not been reported. Our clinicopathological findings may also make a contribution to the understanding of the clinicopathological hallmarks of MSA.