An uncommon cleft subtype of unilateral cleft lip and palate.

The finding that the vomer plays a crucial role in maxillary growth suggests that the bilateral cleft configuration of unilateral cleft lip and palate (UCLP), in which the vomer is detached from the non-cleft-side secondary hard palate, negatively influences palatal development, and this hypothesis was tested. Sixty persons with complete UCLP, including those with the vomer detached from (n = 30, b-UCLP) and attached to (n = 30, u-UCLP) the secondary hard palate, were analyzed morphologically, with the use of cast models taken at 10 days, 3 mos, and 12 mos of age. The anterio-posterior palatal length at 12 mos of age in those with b-UCLP was significantly shorter than that in those with u-UCLP, by 8.7% (p < 0.05). In addition, palatal width development in the first year in those with b-UCLP was also significantly retarded. These results suggest that the uncommon bilateral cleft subtype in UCLP should be included in the cleft classification.

[Sugar beet photosynthesis under conditions of increasing water deficiency in soil and protective effects of a low-molecular-weight alcohol]. / Fotosintez sakharnoi svekly pri narastaiushchem defitsite vody v pochve i protektornoe vliianie nizkomolekuliarnogo spirta.

The rate of CO2 gas exchange, transpiration, stomatal conductivity, and efficiency of the use of water were assessed in leaves of the sugar beet (Beta vulgaris var. Saccharifera (Alef) Krass.) sprayed with 40% methanol, against the background of increasing water deficiency in soil. A decrease in the negative impact of water deficiency in experimental plants was shown to be due to the larger stress-resistance of the photosynthetic apparatus, higher rate of photosynthesis, and more effective use of water.

Two cases of acute respiratory distress syndrome resulting from adult-onset Still's disease.

Abstract Adult-onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown origin. Acute respiratory distress syndrome (ARDS) is a rare complication of AOSD, with only nine cases having been reported in the literature. Here, we describe two cases of AOSD complicated with ARDS that were successfully treated with immunosuppressive therapy, including corticosteroids. Although ARDS is a life-threatening complication in AOSD, early commencement of high-dose corticosteroids and mechanical ventilation improve the prognosis.

Clonal biases of peripheral CD8 T cell repertoire directly reflect local inflammation in polymyositis.

Polymyositis (PM) involves destruction of striated muscles by autoaggressive CD8 T cells, which accumulate and secrete cytotoxic effector molecules in the affected muscles. Previous studies of peripheral T cell repertoires from normal individuals and patients with viral infections have shown that primed CD8 T cells, unlike CD4 T cells, are prone to expand clonally and persist as large populations in the peripheral blood. These facts made us assume that autoaggressive myocytotoxic CD8 T cells would expand clonally in the peripheral blood from patients with PM. By clonal analyses of peripheral T cells from patients and age-matched controls, we show here that clonal expansion of CD8 T cells was more frequent in patients. This was not significant in CD4 T cells. In analogy to virus-specific T cells, the expanded T cells persisted as large populations over time. Analysis of the muscle biopsy specimens revealed that some of the expanded clones were infiltrating in the affected muscles from the same patients. These results provide the first evidence that local autoimmune reaction directly elicits significant biases in peripheral T cell repertoire. The expanded cells, which should be candidate autoaggressive T cells, were readily isolated from the peripheral blood for analysis of expressed genes including perforin. Thus, our findings should give us an immediate clue to analysis of the pathogenic T cells in PM.

Supernumerary ring chromosomes in dermatofibrosarcoma protuberans may contain sequences from 8q11.2-qter and 17q21-qter: a combined cytogenetic and comparative genomic hybridization study.

Dermatofibrosarcoma protuberans (DFSP) presents with characteristic cytogenetic features such as reciprocal t(17;22)(q22;q13) or, more commonly, supernumerary ring chromosomes containing sequences from chromosomes 17 and 22. Here, we report the identification of a novel abnormality in a 43-year-old woman with DFSP. Cytogenetic analysis of tumor cells showed the presence of a supernumerary ring chromosome as the sole anomaly. Amplification of 8q11.2 approximately qter and 17q21 approximately qter sequences was confirmed by comparative genomic hybridization (CGH); the present case apparently lacked amplification of chromosome 22. To our knowledge, this is the first case indicating that the ring chromosome in DFSP is possibly associated with amplified material from chromosomes 8 and 17.

Identification of syt-ssx fusion transcripts in both epithelial and spindle cell components of biphasic synovial sarcoma in small tissue samples isolated by membrane-based laser microdissection.

In order to confirm the presence of SYT-SSX fusion gene in epithelial and spindle cell components of synovial sarcoma, we performed a nested reverse transcriptase-polymerase chain reaction (RT-PCR) using microbeam microdissection of membrane-mounted native tissue (MOMeNT) technique applied on formalin-fixed, paraffin-embedded tumor specimens from two biphasic synovial sarcomas and a control tissue of adamantinoma. Small targeted portions of either an epithelial or spindle cell component of the tumor tissue were microdissected together with the supporter membrane, by using an ultraviolet (337-nm) pulsed laser microbeam coupled into a robot-stage microscope with infinity optics. The SYT-SSX fusion transcript was detected in epithelial and spindle cell components of both biphasic synovial sarcomas, but not in the control tissue. Southern blot analysis also confirmed that the detected messages were derived from the SYT-SSX fusion gene. In conclusion, the microbeam MOMeNT is a useful method for isolating selected small portions from tissue sections. The SYT-SSX fusion gene is present in both cellular components of biphasic synovial sarcoma and is involved in oncogenesis of the synovial sarcoma rather than in morphologic epithelial differentiation. Therefore, in spite of the variable proportions of each component, our results confirm that the synovial sarcoma is of monoclonal origin.

Conservative management of placental polyp with oral administration of methotrexate.

Surgical treatment is usually selected for placental polyp accompanied by massive bleeding but patients wishing to conserve their fecundity require conservative management. A 35-year old nullipara was diagnosed as having placental polyp on the basis of typical episodes, and detection of placental polypeptide hormones and blood flow by Doppler ultrasonography and dynamic magnetic resonance imaging. Oral administration of methotrexate (2.5 mg, three times a day for 5 days) was repeated for three cycles because surgical treatment was rejected. Serum human placental lactogen, blood flow and the polyp disappeared sequentially following chemotherapy. This report thus advocates considering conservative management for placental polyp.

[A case of dermatomyositis complicated with pneumomediastinum that was successfully treated with cyclosporin A].

We reported the case of a 39-year-old man with dermatomyositis (DM) complicated with subcutaneous emphysema and pneumomediastinum during steroid therapy. The patient had complained of muscle weakness, dyspnea and skin eruption on his anterior chest wall 6 months prior to admission. He was diagnosed as having DM on the basis of an elevation in myogenic enzymes, myogenic changes in electromyography, a skin biopsy and a muscle biopsy. Chest roentgenogram revealed interstitial pneumonia (IP) in the lower lobes of the lungs. The administration of prednisolone (60 mg/day) was initiated, which resulted in improvement of DM. Fifteen days after the initiation of the steroid therapy, the patient developed subcutaneous emphysema and pneumomediastinum. Additional administration of cyclosporin A (CsA) enabled us to rapidly taper the dose of prednisolone without aggravating the diseases. Several reports have shown that vasculitis might be involved in the pathogenesis of pneumomediastinum in DM patients. Infection and tissue fragility due to steroid therapy worsen the outcome of those patients. CsA therapy may improve the outcome through the anti-vasculitic- and steroid sparing-effects.

[A case of sarcoidosis characterized by a nasopharyngeal tumor and neurological lesions].

A 20-year-old man was admitted to a hospital complaining a slight fever lasting for 3 months associated with a dull headache and weight loss. A tumor was found in the nasopharynx of which biopsy specimen revealed granulomas with Langhans' giant cells. He was given antituberculous agents without symptomatic improvement, and transferred to our hospital. Serum levels of soluble IL-2 receptor and lysozyme were increased, and a significant uptake was observed by Ga scintigraphy at the nasopharynx and bilateral hilar lymphnodes. Furthermore, spinal fluid contained increased number of mononuclear cells, and T2-weighted MRI scans showed an enhanced lesion at the pituitary stalk. The specimen of both TBLB and repeated biopsy of the nasopharyngeal tumor showed granulomas without caseous necrosis. Taken together with these findings, a diagnosis of sarcoidosis with CNS involvement was finally made, and he made a favorable progress by treatment with prednisolone. This is an unique case which emphasizes importance of differential diagnosis of nasopharyngeal tumors with neurological manifestations in the clinicalsetting of rheumatology.

Effects of umbilical arterial resistance on its arterial blood flow velocity waveforms.

BACKGROUND: We propose to clarify the effect of umbilical arterial resistance on umbilical arterial blood flow patterns in normal pregnant women. SUBJECTS: Pulsed Doppler examination was administered to 145 pregnant women of between 28 and 31 weeks of normal gestation without any prenatal complications. METHODS: The arterial flow velocity waveforms from two different arteries, one in the intra-abdominal portion and the other at the placental insertion, were sampled with the color flow mapping method, and the peak systolic velocity and the resistance index were calculated. RESULTS: The peak systolic velocity and resistance index on the placental side were significantly lower than those of the fetal side. CONCLUSION: A major difference exists between the umbilical arterial flow velocity waveforms on the fetal and placental sides, which might be caused by difference in the umbilical arterial resistance.

Abundant expression of common cytokine receptor gamma chain (CD132) in rheumatoid joints.

OBJECTIVE: Activated macrophages upregulate surface expression of common cytokine receptor gamma chains (gammac, CD132), triggering of which induces secretion of proinflammatory cytokines. Rheumatoid synovial tissues contain a number of macrophages that play a pathogenic role by secreting proinflammatory cytokines. We studied the expression of gammac in the rheumatoid synovial tissues. METHODS: Cryosections of synovial tissues from patients with active rheumatoid arthritis (RA) or with osteoarthritis (OA) were stained with an anti-gammac Mab. Single-cell suspensions from the rheumatoid synovial tissues were stained with the same antibody and an anti-CD14 monoclonal antibody (Mab) for 2-color flow cytometric analysis. A soluble form of gammac in synovial fluids collected from rheumatoid or OA joints was quantitated by ELISA. RESULTS: Rheumatoid synovial tissues, but not the OA tissues, expressed gammac at a high level. Flow cytometric analysis showed that gammac were expressed by virtually all CD 14 positive synovial cells in RA. Synovial fluid derived from the rheumatoid joints contained a high concentration of soluble gammac. CONCLUSION: Membrane bound and soluble forms of gammac are abundant in rheumatoid joints. They might play a complex role in the pathology of RA.

Why abaxial illumination limits photosynthetic carbon fixation in spinach leaves.

Limitations of carbon fixation within spinach leaves due to light and CO2 were investigated. Under equivalent photon fluxes, carbon fixation was higher when leaves were irradiated adaxially compared to abaxially. Maximal carbon fixation occurred in the middle of the palisade mesophyll under adaxial illumination, whereas, maximal carbon fixation occurred in the spongy mesophyll under abaxial illumination. Total carbon fixation and the pattern of carbon fixation across leaves were similar, when leaves were irradiated with 800 micromol quanta m(-2) s(-1) either adaxially alone or adaxially plus abaxially (1,600 micromol quanta m(-2) s(-1)). In contrast, when both leaf surfaces were irradiated simultaneously with 200 micromol quanta m(-2) s(-1), total carbon fixation increased and carbon fixation in the middle of the leaf was higher compared to leaves irradiated unilaterally with the low light. Feeding 14CO2 through either the adaxial or abaxial leaf surface did not change the pattern of carbon fixation across the leaf. Increasing 14CO2 pulse-feeding times from 5 s to 60 s allowed more 14CO2 to be fixed but did not change the pattern of 14CO2 fixation across the leaf. We concluded that in spinach, the distribution of both light and Rubisco activity within leaves has significant effects on the patterns of carbon fixation across leaves; whereas CO2 diffusion does not appear to affect the carbon fixation pattern within spinach leaves.

Supernumerary ring chromosome in a Bednar tumor (pigmented dermatofibrosarcoma protuberans) is composed of interspersed sequences from chromosomes 17 and 22: a fluorescence in situ hybridization and comparative genomic hybridization analysis.

Cytogenetic analysis of Bednar tumor (pigmented dermatofibrosarcoma protuberans) has not been reported previously. Here, we report the identification of a supernumerary ring chromosome in a Bednar tumor by chromosome painting with fluorescence in situ hybridization (FISH) and comparative genomic hybridization (CGH). Chromosome painting with FISH demonstrated that the supernumerary ring chromosome was composed of discontinuous, interwoven sequences from chromosomes 17 and 22. Amplification of chromosomes 17 and 22 sequences was confirmed by CGH. These results indicate that Bednar tumor and dermatofibrosarcoma protuberans are characterized by the same chromosomal features. To our knowledge, this is the first report that the ring chromosome in Bednar tumor is composed of amplified material from chromosomes 17 and 22.

Neonatal thrombocytopenia induced by maternal anti-HLA antibodies: a potential side effect of allogenic leukocyte immunization for unexplained recurrent aborters.

Allogenic leukocyte immunization is one of several treatments tried for unexplained recurrent aborters, and is reported to have few maternal and neonatal side effects after the immunotherapy having been reported to date. In the present study, we report a rare case of neonatal thrombocytopenia (41000 cells/microl) observed in a female infant delivered by an unexplained habitual aborter. The mother was immunized with her husband's leukocytes once before pregnancy and twice at the 5th and 6th week of her successful pregnancy. Serological studies using mixed passive hemagglutination assays (MPHA) showed that maternal serum did not contain any antibodies which were reactive to 11 platelet-specific antigens, or to granulocyte antigens extracted from 9 persons. Lymphocyte cytotoxicity tests, however, showed that maternal serum but not infant serum had anti-HLA antibodies against both paternal and infant lymphocytes. Moreover, the maternal serum was found to have anti-HLA IgGs against platelet antigens extracted from the father and the infant. It is highly likely that this case of neonatal thrombocytopenia was caused by transplacental perfusion of maternal anti-HLA antibodies whose production was induced or enhanced by the allogenic leukocytes immunizations.

Fetal peripheral bronchial fluid flow during breathing movement in normal pregnancies: a preliminary study.

OBJECTIVE: To determine the fluid flow velocity waveforms in the fetal peripheral bronchus during fetal breathing movement by means of pulsed Doppler ultrasonography. DESIGN: A preliminary cross-sectional study. SUBJECTS: Twenty-eight normal pregnant women between 32 and 38 weeks of gestation. METHODS: Velocity waveforms from fetal peripheral respiratory fluid flow were acquired from the segmental bronchus (B6 or B8) which runs along the segmental artery (A6 or A8). The maximum velocity of the intrabronchial fluid flow and duration of inspiratory and expiratory phases were quantified. RESULTS: The flow velocity signal from the fluid in the fetal segmental bronchus of 17 of the 28 fetuses was detected. We observed two types of fetal breathing movement. CONCLUSION: During fetal breathing movement, the fluid in the fetal respiratory tract moves in the fetal segmental bronchus. This movement can be detected by pulsed Doppler velocimetry aided by power Doppler ultrasonography.

Species-independent inhibition of abnormal prion protein (PrP) formation by a peptide containing a conserved PrP sequence.

Conversion of the normal protease-sensitive prion protein (PrP) to its abnormal protease-resistant isoform (PrP-res) is a major feature of the pathogenesis associated with transmissible spongiform encephalopathy (TSE) diseases. In previous experiments, PrP conversion was inhibited by a peptide composed of hamster PrP residues 109 to 141, suggesting that this region of the PrP molecule plays a crucial role in the conversion process. In this study, we used PrP-res derived from animals infected with two different mouse scrapie strains and one hamster scrapie strain to investigate the species specificity of these conversion reactions. Conversion of PrP was found to be completely species specific; however, despite having three amino acid differences, peptides corresponding to the hamster and mouse PrP sequences from residues 109 to 141 inhibited both the mouse and hamster PrP conversion systems equally. Furthermore, a peptide corresponding to hamster PrP residues 119 to 136, which was identical in both mouse and hamster PrP, was able to inhibit PrP-res formation in both the mouse and hamster cell-free systems as well as in scrapie-infected mouse neuroblastoma cell cultures. Because the PrP region from 119 to 136 is very conserved in most species, this peptide may have inhibitory effects on PrP conversion in a wide variety of TSE diseases.

Characteristics of blood flow in intrauterine growth-restricted fetuses with hypercoiled cord.

OBJECTIVE: To clarify the characteristics of fetoplacental blood flow of growth-restricted fetuses with hypercoiled umbilical cord. SUBJECTS: Eight growth-restricted fetuses with hypercoiled cord. METHODS: Flow velocity waveforms of the umbilical cord artery and vein, fetal abdominal aorta and fetal inferior vena cava were analyzed. RESULTS: The resistance index in the umbilical artery in the hypercoiled cases was lower than that in normal fetuses. Early-diastolic reversed flow was observed in the abdominal aorta in some cases. In all cases, umbilical venous pulsation was observed in the entire cord until delivery. In one case, fetal heart failure occurred, resulting in pre-mature delivery. An atrophic type of single umbilical artery was observed in four cases. CONCLUSION: Fetal blood flow disturbance caused by a hypercoiled umbilical cord may be a cause of growth restriction.