Development of continuous measurement system for hydrogen and impurity gases using detector tube.

A relatively accurate, inexpensive, simple, and continuous quantification system for hydrogen and impurity gas(es) using a detector tube was developed in this study. Additionally, different detector tubes can be applied to measure different types of gases in a wide range from ppm order to % level. We optimized this system and evaluated its accuracy as well as the behavior of released H2 and impurity (NH3) gases from a hydrolysis of ammonia borane using a Pt/Al2O3 catalyst. The accuracy of hydrogen quantitation achieved by this system was comparable to that of commercial mass flow meters, and the accuracy of ammonia quantitation was 10% or 5% relative standard deviation, which depends on the detector tube. The concentration of released NH3 was evaluated by image analysis with a time-lapse video of the detector tube and succeeded in analyzing from ppm to % order. The H2 and NH3 release behaviors agreed with pH, and the percentage of reaction was estimated by NMR measurement of the reacted solution. These results confirmed the accuracy of this system.

Effects of water turbulence on variations in cell ultrastructure and metabolism of amino acids in the submersed macrophyte, Elodea nuttallii (Planch.) H. St. John.

The interactions between macrophytes and water movement are not yet fully understood, and the causes responsible for the metabolic and ultrastructural variations in plant cells as a consequence of turbulence are largely unknown. In the present study, growth, metabolism and ultrastructural changes were evaluated in the aquatic macrophyte Elodea nuttallii, after exposure to turbulence for 30 days. The turbulence was generated with a vertically oscillating horizontal grid. The turbulence reduced plant growth, plasmolysed leaf cells and strengthened cell walls, and plants exposed to turbulence accumulated starch granules in stem chloroplasts. The size of the starch granules increased with the magnitude of the turbulence. Using capillary electrophoresis-mass spectrometry (CE-MS), analysis of the metabolome found metabolite accumulation in response to the turbulence. Asparagine was the dominant amino acid that was concentrated in stressed plants, and organic acids such as citrate, ascorbate, oxalate and γ-amino butyric acid (GABA) also accumulated in response to turbulence. These results indicate that turbulence caused severe stress that affected plant growth, cell ultrastructure and some metabolic functions of E. nuttallii. Our findings offer insights to explain the effects of water movement on the functions of aquatic plants.

Effects of NAD kinase 2 overexpression on primary metabolite profiles in rice leaves under elevated carbon dioxide.

The concentration of carbon dioxide (CO2) in the atmosphere is projected to double by the end of the 21st century. In C3 plants, elevated CO2 concentrations promote photosynthesis but inhibit the assimilation of nitrate into organic nitrogen compounds. Several steps of nitrate assimilation depend on the availability of ATP and sources of reducing power, such as nicotinamide adenine dinucleotide phosphate (NADPH). Plastid-localised NAD kinase 2 (NADK2) plays key roles in increasing the ATP/ADP and NADP(H)/NAD(H) ratios. Here we examined the effects of NADK2 overexpression on primary metabolism in rice (Oryza sativa) leaves in response to elevated CO2. By using capillary electrophoresis mass spectrometry, we showed that the primary metabolite profile of NADK2-overexpressing plants clearly differed from that of wild-type plants under ambient and elevated CO2. In NADK2-overexpressing leaves, expression of the genes encoding glutamine synthetase and glutamate synthase was up-regulated, and the levels of Asn, Gln, Arg, and Lys increased in response to elevated CO2. The present study suggests that overexpression of NADK2 promotes the biosynthesis of nitrogen-rich amino acids under elevated CO2.

Nucleotide sequencing and transcriptional analysis of two tandem genes encoding glucosyltransferase (water-soluble-glucan synthetase) in Streptococcus cricetus HS-6.

Two tandem genes encoding glucosyltransferase synthesizing water-soluble glucan (GTF-S) were cloned from the lambda gene library of Streptococcus cricetus HS-6 (serotype a) using anti-GTF-S antibody, and the nucleotide sequences were analyzed. The two genes (ORF1 and ORF2) were identified as streptococcal glucosyltransferases based on the following evidence: [1] the deduced amino acid sequences of their products have an active site for catalytic action and C-terminal repeated units for dextran binding, and [2] a homology search revealed that the ORF1 and ORF2 products are homologous to the GtfS protein (77.4%) of S. downei Mfe28 and GtfT protein (83.8%) of S. sobrinus OMZ176, respectively, which are both known to have GTF-S activity. Therefore, ORF1 and ORF2 might be designated gtfS and gtfT of S. cricetus, respectively. A Northern blotting and RNase protection assay suggested that the gtfS and gtfT of S. cricetus are transcribed as a single bicistronic mRNA as well as separate monocistronic mRNAs. Primer extension analysis indicated multiple transcriptional start points for each gene.

Structure and enzymatic properties of genetically truncated forms of the water-insoluble glucan-synthesizing glucosyltransferase from Streptococcus sobrinus.

Glucosyltransferase-I (GTF-I: 175 kDa) of a cariogenic bacterium, Streptococcus sobrinus 6715, mediates the conversion of water-soluble dextran (alpha-1,6-glucan) into a water-insoluble form by making numerous alpha-1,3-glucan branches along the dextran chains with sucrose as the glucosyl donor. The structures and catalytic properties were compared for two GTF-I fragments, GTF-I' (138 kDa) and GS (110 kDa). Both lack the N-terminal 84 residues of GTF-I. While GTF-I' still contains four of the six C-terminal repeats characteristic of streptococcal glucosyltransferases, GS lacks all of them. Electron microscopy of negatively stained samples indicated a double-domain structure for GTF-I', consisting of a spherical head with a smaller spherical tail, which was occasionally seen as a long extension. GS was seen just as the head portion of GTF-I'. In the absence of dextran, both fragments simply hydrolyzed sucrose with similar K(m) and k(cat) values at low concentrations (<5 mM). At higher sucrose concentrations (>10 mM), however, GTF-I' exhibited glucosyl transfer activity to form insoluble alpha-1, 3-glucans. So did GS, but less efficiently. Dextran increased the rate and efficiency of the glucosyl transfer by GTF-I'. On removal of the C-terminal repeats of GTF-I' by mild trypsin treatment, this dextran-stimulated transfer was completely lost and the dextran-independent transfer became less efficient. These results indicate that the N-terminal two-thirds of the GTF-I sequence are organized as a structurally and functionally independent domain to catalyze not only sucrose hydrolysis but also glucosyl transfer to form alpha-1,3-glucan chains, although not efficiently; the C-terminal repeat increases the efficiency of the intrinsic glucosyl transfer by the N-terminal domain as well as rendering the whole molecule primer-dependent for far more efficient insoluble glucan synthesis.

[Usefulness of neuroendoscopy and a neuronavigator for removal of clival chordoma].

We report a case of large clival chordoma. The patient was a 56-year-old male who was admitted to our hospital with left eye ptosis and diplopia of 2 months duration. On admission, neurological examinations revealed oculomotor nerve palsy of the left eye. Skull radiographs with polytomographs demonstrated marked destruction of the clivus. A plain computed tomography (CT) scan revealed a large iso-attenuated mass in the clivus, extending anteriorly into the sphenoidal sinus, superiorly into the suprasellar cistern, bilaterally into the petrous apex, posteriorly into the prepontine cistern and caudally into the foramen magnum. An enhanced CT scan demonstrated a slightly enhanced tumor. A high-resolution bone-window CT scan revealed marked destruction of the clivus and bilateral petrous apex. Magnetic resonance imaging (MRI) scans disclosed a large enhanced mass extending superiorly into the suprasellar cistern, bilaterally into the petrous apex and inferiorly into the foramen magnum. The tumor extended so widely that we decided on a one-stage operation via a transsphenoidal sublabial transseptal approach and transoral transpalatal approach. At surgery, we employed a neuronavigator and Codman 4-mm rigid neuroendoscope with 0 degree, 30 degrees and 70 degrees angled lenses. The tumor was very soft and suckable, and could be easily removed by applying CUSA, a pituitary curette and suction. The neuronavigator was particularly useful because the surgeon had a real-time two-dimensional representation of the position of the tip of this device in the corresponding imaging space intraoperatively. The neuroendoscope also proved useful, since remnant tumor tissues that could not be seen under an operating microscope were frequently recognized near or around the entrance of the tumor cavity, cavernous sinus region and petroclival junction area. The surgeon was able to remove these remnants safely by checking on the neuroendoscope monitor. The tumor was excised completely. The dead space of the tumor cavity was reconstructed using a free rectus abdominis muscle flap. Postoperatively, cerebrospinal fluid leakage and meningitis were recognized, but improved following spinal drainage for one week and intrathecal injection of antibiotic. The oculomotor nerve palsy of the left eye also showed good recovery at one month after the operation. Recently, skull base surgery has undergone considerable developments. Neuroendoscopes and neuronavigators are very helpful for the neurosurgeon in performing skull base tumor surgery safely and with precision, although further instrument modifications are needed.

Mucin-producing adenocarcinoma of the lung, with special reference to goblet cell type adenocarcinoma: immunohistochemical observation and Ki-ras gene mutation.

To clarify its biological nature, 10 samples of goblet cell-type adenocarcinoma of the lung were collected and compared with 10 other pulmonary mucin-producing adenocarcinomas with respect to immunohistochemical features and the presence of Ki-ras gene mutation in codons 12 and 13. Goblet cell-type adenocarcinomas lacked immunoreactivity for surfactant apoprotein and S-100 protein-positive Langerhans cells, which was in marked contrast to other mucin-producing adenocarcinomas. In addition, the mucin gene products, MUC-1 and MUC-2 glycoproteins were immunohistochemically stained. The results showed that MUC-1 glycoprotein is frequently expressed by mucin-producing adenocarcinomas except the goblet cell-type. Ki-ras gene mutation was detected in 12 of 20 (60%) mucin-producing adenocarcinomas. These mutations were exclusively found in codon 12 and G to A transitions were the most frequent type of alteration in the Ki-ras gene. In goblet cell-type adenocarcinomas, the frequency of Ki-ras gene mutation was 80% consisting of G to A transitions and G to T transversions in six and two tumors, respectively. Therefore, goblet cell-type adenocarcinomas differed from other mucin-producing adenocarcinomas in terms of immunohistochemical and molecular biological features, suggesting that goblet cell-type adenocarcinomas are distinctly different from other subtypes of adenocarcinomas.

[Large cystic neurinoma: a case report].

We report a case of large cystic acoustic neurinoma. A 52-year-old male was admitted to hospital with a history of progressive dysphagia, gait disturbance and diplopia for 2 months. On admission, neurological examinations revealed Bruns' type nystagmus to the left side, hypesthesia in the distribution of the second and third divisions of the left trigeminal nerve, and partial paresis of cranial nerves IX, X, and XII on the left side, and truncal ataxia. A pure-tone threshold audiogram indicated the presence of 32 dB hearing loss in the left ear. Speech discrimination was 80%. Caloric vestibular responses were absent on the left side. Skull radiographs with polytomographs of the internal auditory canal (IAC) were normal. Bony changes in the IAC were not found by high-resolution bone-window computed tomography (CT) scan. A plain CT scan revealed a large low-attenuated cystic mass in the left cerebellopontine angle (CPA), which was associated with displacement of the fourth ventricle. An enhanced CT scan demonstrated a thin rim-enhancement in the cyst wall. Magnetic resonance imaging (MRI) scans disclosed a large rim-enhanced cystic mass extending superiorly into the tentorial incisura and inferiorly into the foramen magnum. At surgery via a left suboccipital approach, a large cystic mass was found at the left CPA arising from the VIIIth nerve, and compressing the Vth, VIth, VIIth and lower cranial nerves. The cyst was filled with a xanthochromic fluid and was firmly attached to the internal auditory meatus (IAM). However no tumor extension into the IAM was confirmed. The tumor was excised completely. The postoperative course was uneventful, except for impairment of the VIIth and VIIIth nerves. At 6 months after the first operation, the facial nerve had improved up to grade III (Hause-Brackmann stage). Histological examinations revealed a typical benign acoustic neurinoma with predominant representation of Antoni B tissues. The cyst wall contained numerous abnormal sinusoid and telangiectasia-like vessels which showed occasional thromboses. The vessel walls displayed endothelial proliferations and were frequently hyalinized. Hemosiderin deposits and hemosiderin-containing phagocytes were also found near these vessels. Myxoid degeneration and necrosis were evident in vast areas. These degenerative changes appeared to be the principal causes of the large cystic formation. 16 cases including our case have been reported. The broad characteristics of the clinical symptoms and radiological findings of these tumors are discussed.

Oligodontia: a radiographic comparison of subjects with Down syndrome and normal subjects.

Panoramic radiographs and clinical records were used to investigate developmentally absent permanent teeth in 98 subjects with Down syndrome (trisomy-21). This retrospective study was based on the records and panoramic radiographs of subjects from approximately five years of age (the age at which mineralization of the permanent tooth germ could be identified) through to their most recent records. The time period covered by records ranged from 6 to 28 years. The majority of subjects with Down syndrome (63%) exhibited oligodontia, and many subjects were missing two or more teeth (53%). The most frequently absent teeth were the lower lateral incisors (23.3%), the upper second premolars (18.2%), the upper lateral incisors (16.5%), and the lower second premolars (15.3%). In general, the distribution of the developmentally absent teeth was similar for teeth in homologous positions (i.e., left and right canines, etc.) on either side of the midline or between the maxilla and the mandible. The only significant exceptions to this pattern were seen with the central incisors and the second molar. This study's findings suggest a high risk of oligodontia in subjects with Down syndrome.

[A ruptured aneurysm of the distal posterior inferior cerebellar artery associated with acute subdural hematoma of the posterior fossa: a case report].

We report a case of distal posterior inferior cerebellar artery (PICA) aneurysm associated with acute subdural hematoma (SDH). The patient was a 68-year-old female who was found unconscious at home and transferred to the emergency medical center in a state of deep coma. Her consciousness on admission to the center was 200P (Japan coma scale), E1V1M2 (Glasgow Coma Scale), and the Hunt & Kosnik grade was grade IV. She was in a state of decerebrate condition. Computed tomography (CT) scans revealed diffuse subarachnoid hemorrhage that was located mainly in the posterior fossa, as well as intraventricular hemorrhage in the third and fourth ventricles. It also disclosed an intracerebellar hematoma (ICH) of the vermis and an acute SDH of the left posterior fossa. The first cerebral angiographic examinations on admission demonstrated no aneurysm. However, emergency surgery was performed immediately in order to improve her poor condition. Ventricular drainage and removal of the acute SDH were carried out. Postoperatively, her consciousness improved gradually to 20P. Ventricular peritoneal shunt was performed three weeks later. Her consciousness improved up to 3P and she showed only slight truncal ataxia. She was admitted to our hospital for rehabilitation at two months after the first surgery. Repeated angiography was performed and demonstrated an aneurysm in the telovelotonsillar segment of the left PICA. The aneurysm was successfully clipped via a midline suboccipital approach. Her postoperative course was uneventful, and she continues to undergo rehabilitation. Aneurysms of the posterior fossa associated with acute SDH are extremely rare. Only two cases have been reported for distal PICA aneurysm cases. The CT scans in our patient revealed not only SAH but also SDH in the posterior fossa and ICH in the vermis. Over 100 cases of distal PICA aneurysms have been described in the literature. We analyzed the relationship between the portions with the ruptured aneurysms and CT findings. Aneurysms which were located at the proximal portion of the distal PICA mainly showed SAH and IVH. On the other hand, ICHs of the vermis and cerebellum were characteristic CT findings of ruptured aneurysms which were located more distal to the telovelotonsillar segment, and were evident in 14% of cases of such aneurysms. ICH and SDH were not found in aneurysms which were located in portions more proximal to the telovelotonsillar segment. These characteristic findings were related to the complex anatomical courses of the PICA. The distal portions of the PICA run between the vermis and cerebellar hemisphere, so that if an aneurysm ruptures at these portions, ICHs in the vermis and cerebellum tend to occur. In cases such as ours, because of the characteristic CT findings, effort to detect a distal PICA aneurysm should be made at first surgery, along with ventricular drainage and removal of the SDH. The surgical procedures and outcome of cases with distal PICA aneurysms are also discussed.

The regulatory effect of fermentable sugar levels on the production of leukotoxin by Actinobacillus actinomycetemcomitans.

The relationship between sugar availability and RTX (repeats in toxin) cytotoxin (leukotoxin) production in the periodontopathic bacterium, Actinobacillus actinomycetemcomitans, was investigated using a chemostat. A actinomycetemcomitans 301-b produced significant amounts of leukotoxin in anaerobic fructose-limited chemostat cultures at a dilution rate of 0.15 h-1 and at pH 7.0. When the growth limitation was relieved by pulsing the cultures with 50 or 150 mM fructose (final concentrations), leukotoxin production immediately stopped and the amount of cellular leukotoxin decreased until the culture was returned to fructose-limited conditions. Leukotoxin synthesis was also repressed in the chemostat cultures by pulsing with glucose but not with the non-fermentable sugar analog, alpha-methyl-D-glucoside. Leukotoxin production was also repressed by fructose in chemostat cultures of ATCC 33384, which is generally recognized as a non-leukotoxin-producing or minimally leukotoxic strain.

[Intraorbital conjunctival cyst after a penetrating orbital injury: a case report].

We report a case of intraorbital conjunctival cyst following a penetrating orbitocranial injury. The patient was a 28-year-old male who was hospitalized with exophthalmos, retrobulbar pain and upper gaze disturbance of his left eye. When he was 4 years old, a thin iron rod had penetrated intracranially through the inner angle of his left orbit. He was hospitalized and treated conservatively for about two weeks. The left eye ball was intact and visual acuity was normal, although bloody fluid had continuously flowed out from the left inner angle of the conjunctival wound for a few days. He had been febrile to 39 degrees C and complained of headache for one week. Subsequentry, the symptoms gradually improved through conservative therapy. When he was a junior high school student, he noticed exophthalmos of his left eye. However, he had never been examined closely, until he was 28 years old. We suspect that he had suffered from meningitis caused by the penetrating orbitocranial injury, and had fortunately improved under the conservative therapy. On admission to our hospital, a craniogram showed fracture of the left orbital roof, and coronal and three-dimensional computed tomography (CT) scans clearly demonstrated the orbital fracture. CT revealed a cystic mass in the retrobulbar space, and a porencephalic cyst in the medial basal frontal lobe. On magnetic resonance imaging (MRI) scans, both cysts were of low intensity on T1-weighted imaging, and of high intensity on T2-weighted images. Coronal and sagittal MRI scans showed that the two cysts were connected with each other through the fracture in the orbital roof. We diagnosed therefore that the orbital cyst was a herniated porencephalic cyst of the frontal lobe. Surgery was performed by a transcranial approach. The porencephalic cyst adhered to the fractured lesion of the frontal base but did not extend into the orbita. The intraorbital cyst was totally removed by opening the orbital roof including the fractured lesion. The cyst contained milky fluid. Postoperatively, the exophthalmos, retrobulbar pain and upper gaze disturbance showed gradual improvement. On histological examination, the cyst was found to be lined by non-keratinized stratified squamous epithelium and was diagnosed as a conjunctival cyst. This case was considered to be one of traumatic conjunctival cysts caused by a penetrating orbitocranial injury. Orbital conjunctival cysts have been reported to comprise about 10% of orbital epidermoid and dermoid cysts. Of these cysts, traumatic conjunctival cysts are rare, and only a few cases have been described. The etiology and therapy of orbital conjunctival cysts are discussed.

[Analysis of the proliferative potential of meningiomas with MIB-1 monoclonal antibodies].

Tumor recurrence was observed in 12 (11.3%) out of 106 cases of intracranial meningioma followed for more than 5 years. Proliferative potential was evaluated immunohistochemically with MIB-1 monoclonal antibodies in 37 cases of non-recurrent meningioma and 12 cases (21 samples) of recurrent meningioma. The proliferating cell index (PCI) was much higher in the non-recurrent meningiomas than the recurrent meningiomas (10.6 +/- 7.7 [mean +/- SD] versus 1.9 +/- 1.5). Most recurrent meningiomas had high PCI values, greater than 3%. High PCI values of more than 5% were found in 13 (62%) of the 21 samples of recurrent meningioma. However, only 4 of the 37 cases of nonrecurrent meningioma had high PCI values with MIB-1 of more than 3%. The 12 cases of recurrent meningioma were classified into 3 groups: 6 cases in which both the initial and recurrent meningiomas were benign (Group I), 5 cases in which the meningioma at the time of the initial operation was benign, but the recurrent meningioma was malignant (Group II), and one case in which malignant meningioma was diagnosed at the time of the initial operation (Group III). The PCI values with MIB-1 in most of the recurrent meningiomas were higher at the time of recurrence than at the time of the initial operation. Malignant meningiomas, such as anaplastic and atypical meningioma, and some meningotheliomatous meningiomas among the benign meningiomas recurred and had higher PCI with MIB-1 values than other meningiomas. It is concluded that PCI with MIB-1 is important as a predictive factor for the recurrence of meningiomas. Meningiomas having a PCI value with MIB-1 of more than 3% in particular should be followed carefully.

[Triple primary intracranial tumors of different cell types: a case report].

We report a case of triple intracranial tumors of different cell types without phacomatosis. The patient was a 77-year-old female who was hospitalized with left hemiparesis and vomiting. Computed tomography (CT) scans revealed a large tumor mass in the right frontal lobe and relatively small tumor masses in the medial right frontal and parietal lobes. Other tumors were also detected in the sella turcica, left sphenoidal wing, left anterior clinoidal process and left cerebellar convexity. This case was considered to be one of metastatic brain tumors, and surgery was performed for the right frontal tumor because of its mass effect. The tumor was so highly vascular that it could not be totally removed. Postoperatively, the mass effect showed a gradual increase on CT scans because of intratumoral hemorrhage and peritumoral edema. The patient's consciousness level gradually fell, and she died of pneumonia and cardiac insufficiency 1 month after the operation. The surgical specimen of the tumor was diagnosed as glioblastoma multiforme by histological examination. At autopsy, the small tumors in the medial frontal lobe and parietal lobe were found to be clearly separated from the large right frontal tumor and were diagnosed as multicentric glioblastoma multiforme. The sellar tumor revealed chromophobe pituitary adenoma and was diagnosed as a prolactinoma by immunohistochemical examination. The tumors in the left sphenoidal wing and left cerebellar convexity were diagnosed as transitional meningiomas. Multiple primary intracranial tumors of different cell types without phacomatosis are relatively rare, but almost 100 reported cases could be found in the literature.(ABSTRACT TRUNCATED AT 250 WORDS)

Structure determination of galacto-oligosaccharides by pyridylamination and NMR spectroscopy.

Galacto-oligosaccharides formed from lactose by the action of some beta-galactosidases were subjected to gel chromatography on Bio-Gel P-2, and the resulting oligosaccharide fractions were converted into pyridylamino (PA) derivatives. Each PA-oligosaccharide fraction, which consisted of several isomers in a given size-class, was then subjected to HPLC on an ODS column. Twenty-one individual galacto-oligosaccharide components were isolated in this way. The structures of most of these compounds, namely six disaccharides, five trisaccharides, two tetrasaccharides, and a pentasaccharide, were determined by 13C-NMR spectroscopy. The results obtained will be useful for the study of the activity of various galacto-oligosaccharides on the growth of Bifidobacterium species.

Metabolic and energetic aspects of the growth response of Streptococcus rattus to environmental acidification in anaerobic continuous culture.

Streptococcus rattus, a serotype b strain of mutans streptococci, was grown in an anaerobic glucose-limited chemostat. The molar growth yield of glucose [Yglucose, g dry wt (mol glucose)-1] together with the maximum growth yields (Ymax) and maintenance coefficients for glucose utilization and calculated ATP generation were estimated as a function of pH. When the pH was lowered from 7.0 to 5.0, Yglucose decreased, with a concomitant gradual change in the composition of the end product from a mixture of formate, acetate and ethanol to one mostly of lactate. Whereas the Ymax for glucose decreased without any change in the Ymax for ATP on acidification, both of the maintenance coefficients markedly increased. Kinetic and immunochemical examinations indicated the presence of an F1F0-type proton-translocating ATPase in the membrane fraction prepared from bacterial cells grown under acidic conditions; no detectable level of the enzyme was found in cells grown at neutral pH. However, when incubated with glucose under non-growing conditions, these acid-adapted and unadapted cells showed an insignificant difference in the ability to maintain the intracellular pH alkaline relative to the acidic environments. These results suggest that the organism responds and adapts to environmental acidification by sacrificing some energy cost in terms of both the efficiency of glucose utilization to generate ATP and the extra maintenance required to continue biomass production as efficiently as under neutral pH.

[The two familial occurrence of von Hippel-Lindau disease].

Five cases of von Hippel-Lindau disease in two families were reported. In one family there were 2 cases in a mother (55 years old) and her daughter (26 years old) which had multiple hemangioblastomas in the optic nerve, cerebellum and spinal cord in each case. Retinal angioma appeared in the case of daughter, but not in the mother. In the other family there were 3 cases in a father (28 years old), his son (11 years old) and daughter (14 years old). All of them had retinal angioma and cerebellar hemangioblastoma. Two cases out of 5 cases in the two families were associated with abdominal organ diseases which were renal cell carcinoma in a case and pancreas cyst in the other. The familial occurrence of von Hippel-Lindau disease in Japan was recognized in 21 families with 103 cases including our cases. On the study of reported cases hemangioblastoma frequently occurred in multiple region such as cerebellum, medulla oblongata and frequently presented with renal cell carcinoma, pheochromocytoma and cyst in pancreas, kidney and liver. The location of hemangioblastoma and combined disease was similar in cases in each family. The age of onset of von Hippel-Lindau disease in the second generation was younger than that of the first generation.

[Clinical study on the recurrence of meningiomas: correlating factors for the recurrence and therapeutic prognosis of meningiomas].

Authors have studied 128 cases of meningiomas which were operated on and examined histologically. 12 cases (11.3%) of 106 cases of the meningiomas followed up for more than 5 years after surgery had recurrence after the primary operation. The distribution of age and sex, the tumor location, the histological type, the extent of tumor evacuation and AgNORs counts and BrdU labeling index for the evaluation of tumor proliferation were studied comparatively in the two groups, the recurrent and nonrecurrent meningiomas. 8 cases (19.0%) of 42 cases of the meningiomas in the age-group lower than 50 years old were recurrent cases, whereas 4 cases (6.0%) of 67 cases in the age-group over 50 years old were non-recurrent. Concerning the sex distribution of the meningiomas in our cases recurrent rate was 20.5% among males and 6.0% among females. The tumor locations of the meningiomas did not correlate significantly with recurrence. The extent of tumor removal demonstrated Simpson grade I (47 cases), grade II (37 cases), grade III (17 cases), grade IV (5 cases) on primary operation. Recurrence was found in 11 cases (13.1%) of 84 cases operated on by total removal of Simpson grade I or grade II. 8 cases of 11 cases had recurrence within 5 years after the primary operation of Simpson grade I or II. Total removal of the tumor was important in order to bring about long survival after operation. But correlation between the extent of tumor removal and recurrence was not able to be recognized. Histologically, all of the malignant, atypical and papillary type of meningiomas treated in the primary operations had recurrence.(ABSTRACT TRUNCATED AT 250 WORDS)

[Turner's syndrome with pituitary hyperplasia: a case report].

Turner's syndrome, a sex-chromosome abnormality, is often accompanied by cardiovascular disorders, such as coarctation of the aorta. We encountered a case of Turner's syndrome with meningioma and pituitary hyperplasia which resulted in death from dissection of the aorta. The patient was 36-year-old female who was diagnosed as having Turner's syndrome of mosaic-type at the age of 15. She had accepted sex-hormone replacement with estrogen and progesterone over 20 years. She lost consciousness and was transferred to our institute on June 20th, 1992. She was in shock but her condition began to improve after intensive treatment. CT scan revealed a calcified mass lesion at the left frontal convexity and a markedly enhanced round mass lesion at the suprasellar region. Angiography showed tumor stain of the suprasellar region fed by the posterior ethmoidal artery. These tumors were diagnosed as multiple meningiomas. She developed dyspnea on June 24th and chest X-ray showed right pleural fluid collection and cardiomegaly. This condition was diagnosed as congestive heart failure. Her condition was getting worse and she suffered abrupt cardiac arrest on June 28th. Autopsy revealed dissection of the aorta as the cause of death. The tumor of the convexity was meningioma, and the suprasellar lesion was diagnosed as pituitary hyperplasia. It is well known that frequent excess dose injection of estrogen can induce pituitary adenoma or hyperplasia in rats. In this case, the presence of pituitary hyperplasia was thought to be the result of long-term injection of estrogen.(ABSTRACT TRUNCATED AT 250 WORDS)

Pituitary adenoma combined with Rathke's cleft cyst--case report.

A rare pituitary adenoma associated with Rathke's cleft cyst was discovered incidentally in a 44-year-old male admitted after head trauma. Neurological and physiological examination found no abnormalities, except for panhypopituitarism. Computed tomography and magnetic resonance imaging demonstrated a solid mass in the sellar cavity with suprasellar extension, associated with a cystic mass extending into the third ventricle. The tumor was removed subtotally by the transcranial approach. Light microscopy demonstrated that the cyst wall was composed of ciliated columnar cells, cuboidal cells, and goblet cells, and the solid part indicated chromophobe pituitary adenoma. Immunohistochemistry demonstrated that a few adenoma cells were positive for prolactin and the cyst wall cells were positive for cytokeratin and negative for S-100 protein.