Prenatal diagnosis of short-rib polydactyly syndrome type 3 (Verma-Naumoff type) by three-dimensional helical computed tomography

We present a case of short-rib polydactyly syndrome (SRPs) type 3 in which accurate prenatal diagnosis was feasible using both ultrasonography and 3D-CT. SRP encompass a heterogeneous group of lethal skeletal dysplasias. However, the phenotypes overlap with those of nonlethal skeletal dysplasias (i.e. Ellis-van Creveld syndrome and Jeune syndrome). As accurate prenatal diagnosis of SRP is helpful for parents, we used 3D-CT in the early third trimester to examine a fetus suggested to have phenotypes of 'short-rib dysplasia group' on ultrasonography. 3D-CT showed mild modification of the vertebral bodies, small ilia with horizontal acetabula and triangular partial ossification defects, and subtle metaphyseal irregularities of the femora. These CT findings and an extensive literature search regarding the phenotypes of various diseases categorized as short-rib dysplasia group led to a correct prenatal diagnosis of SRP type 3. This case exemplified the usefulness of 3D-CT for the precise prenatal diagnosis of skeletal dysplasias.     

Bone marrow stromal cells protect and repair damaged neurons through multiple mechanisms

A surprising shortage of information surrounds the mechanism by which bone marrow stromal cells (BMSC) restore lost neurologic functions when transplanted into the damaged central nervous system. To clarify the issue, the BMSC were cocultured with the neurons using two paradigms: the cell-mixing coculture technique and three-dimensional coculture technique. The green fluorescent protein (GFP)-expressing BMSC were cocultured with the PKH-26-labelled neurons, using cell mixing coculture technique. GFP-positive, PKH-26-negative cells morphologically simulated the neurons and significantly increased the expression of MAP-2, Tuj-1, nestin, and GFAP. GFP/nestin-positive, PKH-26-negative cells increased from 13.6% +/- 6.7% to 32.1% +/- 15.5% over 7 days of coculture. They further enhanced Tuj-1 expression when cocultured with neurons exposed to 100 microM of glutamate for 10 min. About 20-30% of GFP-positive cells became positive for PKH-26 through coculture with the neurons, but the doubly positive cells did not increase when cocultured with glutamate-exposed neurons. Alternatively, the BMSC significantly ameliorated glutamate-induced neuronal damage when cocultured with the three-dimensional coculture technique. The protective effect was more prominent when coculture was started prior to glutamate exposure than when coculture was started just after glutamate exposure. ELISA analysis revealed that the BMSC physiologically produce NGF, BDNF, SDF-1alpha, HGF, TGFbeta-1, and IGF-1 and significantly enhanced the production of NGF and BDNF when cocultured with glutamate-exposed neurons. These findings strongly suggest that the BMSC may protect and repair the damaged neurons through multiple mechanisms, including transdifferentiation, cell fusion, and production of growth factors.     

Surgical treatment of cervical spondylosis in the elderly: surgical outcomes, risk factors, and complications

The incidence of cervical spondylosis in the elderly is increasing with the higher proportion of elderly individuals among the Japanese population. The present study retrospectively analyzed the clinical and radiological features of this clinical entity in 107 patients with cervical spondylosis aged 70 years or older surgically treated between 1995 and 2005. The patients were divided into Group 1 (n = 60) aged between 70 and 74 years, and Group 2 (n = 47) 75 years or older. Patients with localized compression within 2 levels responsible for the neurological symptoms underwent anterior fusion, and patients with a narrow spinal canal were treated by laminoplasty. The pre- and postoperative neurological status (Neurosurgical Cervical Spine Scale) and postoperative complications were compared. More patients in Group 2 required laminoplasty but most patients underwent single-level anterior fusion. There was no statistically significant difference in the surgical results. Group 2 had a much higher ratio of anterior fusion at the C3-4 level. Many of the aged patients had multiple risk factors. Surgical decompression for cervical spondylosis is beneficial even in elderly patients. Single-level anterior fusion to treat the lesion most responsible for the symptoms is the least invasive choice. Elderly patients tend to present with multiple risk factors so require careful perioperative management.     

Trierixin, a novel Inhibitor of ER stress-induced XBP1 activation from Streptomyces sp. II. structure elucidation

Trierixin, a new member of the triene-ansamycin group, has been isolated from the fermentation broth of Streptomyces sp. AC654 as an inhibitor of ER stress-induced XBP1 activation. The structure of trierixin was determined on the basis of its spectroscopical and chemical properties. Trierixin possessed a 21-membered macrocyclic lactam, which contains a methylthio-benzenediol structure, and a cyclohexanecarbonylalanine moiety. Trierixin is thus elucidated as 21-thiomethylmycotrienin II.     

Changes in nocturnal pineal melatonin synthesis during the perimenopausal period: Relation to estrogen levels in female rats

To evaluate changes in melatonin synthesis during the perimenopausal period in the female rat and to determine the effects of estrogen on melatonin synthesis, pineal levels of tryptophan, melatonin and norepinephrine and activities of N-acetyltransferase (NAT) and hydroxyindole-O-methyltransferase (HIOMT) were determined. Homogenates for assay were prepared from the pineal glands of female virgin Sprague-Dawley rats between 4 and 24 months of age in the middle of the dark period of a daily light/dark cycle. Serum 17 beta-estradiol (E2) concentrations were also determined. Pineal melatonin levels significantly decreased from month 4 12 and significantly increased from month 12 16, decreasing thereafter. Serum E2 concentrations significantly decreased from month 12-16, and remained low thereafter. No significant changes in tryptophan or norepinephrine were seen. NAT activities paralleled the time course of changes in melatonin. HIOMT activities decreased gradually from month 4 24. Subcutaneous implantation of an E2 capsule between months 12 and 16 resulted in significant decreases in levels of melatonin and NAT activity at month 16. Ovariectomy at month 4 or 12 led to significant increases in the levels of melatonin and NAT activity. These findings represent a temporal increase in pineal melatonin synthesis during the perimenopausal period, and suggest that the increase in melatonin synthesis activity at that time might result from decreasing levels of endogenous estrogen. The effect of estrogen on melatonin synthesis appeared to involve modulation of NAT activity.     

Characteristics of Ceftibuten Uptake into Caco-2 Cells

The characteristics of ceftibuten uptake into Caco-2 cells grown in a collagen-coated dish were examined. Ceftibuten showed stereoselective and pH-dependent uptake. The pH-dependency of ceftibuten was more marked than that of cefaclor or cephalexin, but all three antibiotics showed maximal uptake at pH 5.5. Ceftibuten uptake was linear for the initial 1 hr and then reached a plateau. The initial uptake (15 min) was markedly reduced by the addition of 2,4-dinitrophenol or FCCP (a protonophore), or by lowering the incubation temperature. The uptake of ceftibuten into the brush-border membrane vesicles prepared from cultured Caco-2 cells showed an overshoot in the presence of an H⁺-gradient. These findings indicated that the uptake of ceftibuten was energy-dependent, especially H⁺-gradient-dependent. Uptake inhibition by various compounds was compared using Caco-2 cells. Amino acids and a tetrapeptide did not inhibit uptake, whereas di- or tri-peptides were effective inhibitors. These observations suggest that ceftibuten is taken up by a carrier-mediated transport system(s) for dipeptides. Various antibiotics differed in their ability to inhibit uptake, with cyclacillin showing maximum inhibition. Differences in the inhibitory effect may be accounted for by the heterogeneity (multiplicity) of the transport systems.     

Allergic fungal sinusitis caused by Bipolaris spicifera and Schizophyllum commune.

There have been very few reports in Japan of patients with allergic fungal sinusitis (AFS). We describe two cases caused by Bipolaris spicifera and Schizophyllum commune. The patients were a 70-year-old male (Case 1) and a 55-year-old female (Case 2). Both presented with nasal obstruction and purulent nasal discharge. CT scans revealed each to have a soft tissue mass extending from the ethmoid sinus to the sphenoid sinus. In addition, pathological studies on the contents of the paranasal sinuses of both patients revealed the presence of fungal elements in the allergic mucin. Microbiological studies resulted in the recovery of Bipolaris spicifera from Case 1 and Schizophyllum commune from Case 2. To date there have been no reports of AFS due to these two fungi in Japan. It is very important in the diagnosis of AFS to demonstrate the presence of fungal elements in the allergic mucin. Squash cytology of the paranasal sinus contents was especially useful for proving the presence of fungi.     

Mediastinal parathyroid cyst with tracheal constriction.

A 63-year-old man visiting a physician for slight dyspnea, attributed to a lump on his neck, was found in ultrasonography and computed tomography to have a cyst extending from the left lobe of the thyroid gland to the superior mediastinum. Radiography showed right deviation of the trachea. The cyst disappeared after fine-needle aspiration, but cyst fluid subsequently reaccumulated and he was admitted to our hospital. No abnormalities were detected in tests of thyroid and parathyroid function or blood chemical analysis. The cyst was surgically removed and diagnosed as a nonfunctioning parathyroid cyst, based on the high-intact parathyroid hormone in cyst fluid. The patient recovered fully and has shown no recurrence in the 11 months to data since surgery.