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B Gruber

Publications and source records attributed to B Gruber.

At least 37 records · Page 2Linked to original sources
PubMed1994

Oromandibular-limb hypogenesis syndrome following chorionic villus sampling.

A female infant with hypoglossia-hypodactyly syndrome is presented. Her mother underwent chorionic villus sampling (CVS) at 9 weeks gestation. This is the seventh reported case of oromandibular-limb hypogenesis syndrome associated with maternal CVS, the first from the United States. These seven CVS-related cases represent a significant increase over this syndrome's 'natural' incidence of 1 per 175,000 live births. An excess of limb reduction deformities has also been seen following CVS. These seven cases are reviewed, and the possible etiology of this rare condition is discussed.

Chorionic Villi Sampling↗
PubMed1994

Correlation between senescence and DNA repair in cells from young and old individuals and in premature aging syndromes.

Cellular aging appears to be related to and perhaps caused by diminished DNA repair. To elucidate direct correlations between DNA repair capacity and senescence various parameters of cellular aging and DNA repair were studied simultaneously. Of special interest are features of DNA repair and senescence in cultured diploid fibroblasts derived either from healthy young or elderly probands as well as from patients suffering from premature senescence syndromes (Werner syndrome, Cockayne syndrome, ataxia telangiectasia and Down syndrome). Here we demonstrate the striking parallelism between reduced maximal lifespan, elevated levels of spontaneous chromosomal breaks, higher incidence of formation of micronuclei, a significant prolongation of cell cycle duration and a diminished reactivation of in vitro injured plasmid after transfection in cells from old individuals and from patients with premature senescence syndromes, suggesting a causal relationship between senescence and DNA damage.

Adolescent↗
PubMed1994

Malignant vestibular schwannoma.

A 61-year-old woman underwent a translabyrinthine resection of a right intracanulicular acoustic neuroma, which had been detected in the work-up of sudden hearing loss. At the time of surgery, the tumor was roughly twice as large as indicated by the magnetic resonance scan taken only 2 months previously. The tumor eroded the vertical and transverse crests and extended well into the cerebellopontine angle. It was impossible to distinguish the facial nerve proximal to the geniculate ganglion. All visible tumor was resected, along with the facial nerve. Histological evaluation showed a highly cellular tumor, with many mitoses and areas of necrosis, meeting the criteria for malignant schwannoma. The patient has no stigmata of neurofibromatosis, and has no known relatives with that condition. This case is only the fourth reported of a malignant vestibular schwannoma. The relationships between vestibular schwannoma, neurofibromatosis, and malignancy are discussed.

Case Reports↗
PubMed1994

Elevated plasma stromelysin levels in arthritis.

OBJECTIVE: To determine whether plasma concentrations of stromelysin-1 and gelatinase A are increased in patients with various forms of arthritis. METHODS: A sensitive and specific sandwich enzyme linked immunosorbent assay (ELISA), which employs a murine monoclonal antibody and a rabbit polyclonal antibody to human stromelysin-1, was used to measure plasma stromelysin-1 in 53 healthy subjects, 113 patients with various forms of arthritis and connective tissue diseases, and 65 patients with cancer. Gelatinase A was also measured in these patients using specific polyclonal and monoclonal antibodies to gelatinase A in an ELISA: RESULTS: The plasma concentration of stromelysin-1 (X +/- SEM) was significantly increased (p < 0.001) in patients with rheumatoid arthritis (RA) (187 +/- 14 ng/ml) and systemic lupus erythematosus (SLE) (258 +/- 35 ng/ml) as compared to both healthy control subjects (50 +/- 4 ng/ml) or patients with cancer (61 +/- 20 ng/ml). Plasma stromelysin-1 was also significantly increased in smaller groups of men with osteoarthritis (OA) and gout. In contrast, plasma concentrations of gelatinase A were not significantly increased in patients with RA, OA or gout. In healthy subjects, the concentration of stromelysin-1 was significantly higher in men than women. No correlation was noted between plasma stromelysin-1 levels and age. CONCLUSION: The detection of elevated plasma levels of stromelysin-1 in patients with RA is consistent with increased stromelysin-1 concentrations in inflamed synovial tissues in this disease. The origin of increased plasma stromelysin-1 in SLE is speculative. Measurement of plasma stromelysin-1 may be useful in the diagnosis and management of patients with various forms of arthritis.

Adult↗
PubMed1994

DNA damage and repair in normal and neoplastic cells treated with adriamycin.

Adriamycin (ADR), a common antineoplastic drug, was used to study DNA repair synthesis, cell cytotoxicity and DNA single strand breaks in normal human fibroblasts--CLV98 and human melanoma cells--ME18. No repair synthesis was observed in ME18 and CLV98 cells exposed to adriamycin in concentrations up to 10(-5) M. ME18 cells were less sensitive to ADR treatment than CLV98 cells. Adriamycin-induced DNA single strand breaks (at ADR concentration: 1 microgram/ml) were incompletely repaired in ME18 cells and unrepaired in CLV98 cells within 24 h after drug removal. Within 48 h strand breaks were completely repaired in both kinds of cells. No repair of single strand breaks was observed in ME18 and CLV98 cells after drug treatment in the concentration of 5 micrograms/ml.

Cell Line↗
PubMed1993

Mycosis fungoides involving the cervical esophagus.

Review of the otolaryngologic literature reveals no case reports of mycosis fungoides involving the esophagus. Post-mortem studies report 12 cases of esophageal involvement in 131 autopsies of patients with mycosis fungoides. We describe a 54-year-old man with mycosis fungoides involving the larynx, hypopharynx, and esophagus. Treatment consisted of radiation to this area, with resolution of the patient's hoarseness and dysphagia. The charts of 96 patients with mycosis fungoides treated at our institution were retrospectively reviewed. Three additional cases involving the aerodigestive tract, but not the esophagus, were found. Esophageal mycosis fungoides was an incidental finding in two of seven autopsies at our institution.

Esophageal Neoplasms↗
PubMed1993

Kainic acid seizures cause enhanced expression of cholecystokinin-octapeptide in the cortex and hippocampus of the rat.

Immunocytochemistry and in situ hybridization techniques were used for investigating changes in cholecystokinin immunoreactivity and mRNA in the cerebral cortex and hippocampus after kainic acid-induced limbic seizures in the rat. Marked increases in cholecystokinin mRNA concentrations were observed in layers II/III and V/VI of the cerebral cortex, in CA1 pyramidal neurons of the hippocampus, and in presumptive basket cells of the dentate gyrus 1 and 2 days after the acute seizures. Whereas cholecystokinin mRNA contents returned to normal in the cerebral cortex and the CA1 sector at later intervals, high concentrations were observed in basket cells even 2 months after the initial seizures. Accordingly, cholecystokinin-like immunoreactivity was intensified in the cerebral cortex, CA1 sector and in presumed basket cells of the hippocampus 30 days after kainic acid. Besides its high content in basket cells, cholecystokinin-like immunoreactivity was primarily present in neuronal fibers or diffusely distributed in the respective brain area. In the hippocampus, strongly enhanced staining for cholecystokinin was also observed in the alveus, the stratum lacunosum moleculare, and in the inner molecular layer, suggesting increased concentrations of the peptide in afferent and efferent fibers of the hippocampus. The present experiments suggest a strong activation of cholecystokinin systems in the brain after kainic acid-induced limbic seizures in the rat. This is indicated by pronounced increases in cholecystokinin mRNA in the cortex and individual cell types of the hippocampus (basket cells, granule cells, and CA1 pyramidal neurons). The subsequent increases in cholecystokin immunoreactivity even surpass those in mRNA. The observed changes may be part of the self-defense mechanisms that protect the animals during subsequent epileptic episodes.

Amygdala↗
PubMed1993

Kainic acid seizures in the rat: differential expression of chromogranin A, carboxypeptidase H and peptidylglycine alpha-amidating monooxigenase in subfields of the hippocampal formation.

Using in situ hybridization histochemistry concentrations of mRNAs encoding chromogranin A (ChA), carboxypeptidase H (CPH) and peptidylglycine alpha-amidating monooxigenase (PAM) have been investigated in the hippocampus after kainic acid (KA)-induced limbic seizures in the rat. Increased concentrations (by 150%) of ChA and CPH mRNAs were found in the granule cell layer 24 h after KA injection. At the same time PAM mRNA levels were only slightly elevated (by 50%). Whereas the increases in CPH and PAM transcripts were only transient, ChA mRNA concentrations in the granule cell layer were elevated up to 2 months after the initial seizures. In contrast, in the pyramidal cell layers of all hippocampal subfields (CA1 to CA3) ChA mRNA concentrations were significantly reduced (by 40-70%) 1-60 days after KA. PAM and CPH messages were slightly reduced in the pyramidal cell layer of CA1 but not in CA2 and CA3. The experiments demonstrate that KA-induced limbic seizures cause sustained changes in the expression of ChA mRNA. At the same time the expression of two enzymes involved in post-translational processing of neuropeptides, PAM and CPH, becomes only transiently altered. Synthesis of ChA may be regulated differently in the strata granulosum and pyramidale during epileptic seizures.

Animals↗
PubMed1993

Experience with one-stage laryngotracheal reconstruction.

Eight patients (ages 10 months to 5 years) underwent laryngotracheal reconstruction (LTR) without intraluminal stenting or tracheostomy. All patients had moderate to severe acquired or congenital subglottic stenosis. Six patients were tracheostomy-dependent prior to reconstruction. The other two children were intubated and would have required tracheostomy for airway control. LTR and decannulation/extubation were accomplished as a one-stage procedure. Autologous rib cartilage was used (anterior graft only--6 patients, anterior and posterior graft--3 patients) and patients were intubated for 7-10 days. Pulmonary complications from the week-long intubation and intensive care unit stay were common, but easily managed. Seven of eight patients were successfully decannulated/extubated.

Cartilage↗
PubMed1993

Blastomycosis of the skull base.

Blastomycosis is usually considered a pulmonary or cutaneous disease. A patient with blastomycosis of the infratemporal fossa temporal bone and sinuses without pulmonary disease is presented. Extensive skull base involvement required surgical debridement for relief of pain, and long-term systemic antifungal therapy. The literature is reviewed and discussed.

Journal Article↗
PubMed1993

Ingestion of caustic cosmetic products.

Twelve children over a 6-year period (1985 to 1991) underwent aerodigestive tract endoscopy after ingestion of lye-containing cosmetic products. All children were less than 2 years of age and had facial and/or oral cavity burns. Eleven of these patients ingested alkaline hair products such as "activators" or "relaxers." Endoscopy revealed pharyngeal burns in five patients but no laryngeal or esophageal burns in any patient. Erythema of the esophageal mucosa was seen in one patient. One patient ingested a solution used in fingernail decorating. This patient sustained extensive superficial burns of the pharynx, larynx, and esophagus and required intubation and admission to the intensive care unit. No long-term sequelae developed. Cosmetic products containing caustic chemicals represent a significant health risk to small children, especially because child-proof packaging is not standard for these products.

Burns, Chemical↗
PubMed1993

Temporal meningiomas presenting as chronic otitis media.

Two cases of temporal bone meningiomas presenting as chronic drainage ears are presented. One case was indicated by computer tomographic scan at the time of initial surgery to be limited to the middle ear, but recurred 9 months later with extensive cerebellopontine angle extension and cranial nerve palsies. The tumor in the second case extended from the middle ear to the cavernous sinus, with extensive intradural involvement. Meningiomas involving the middle ear must be considered to have intracranial extension, and magnetic resonance is the imaging study of choice.

Diagnosis, Differential↗
PubMed1992

Temporal lobe epilepsy of the rat: differential expression of mRNAs of chromogranin B, secretogranin II, synaptin/synaptophysin and p65 in subfield of the hippocampus.

We have investigated by in situ hybridization changes in the content of mRNAs encoding for chromogranin B, secretogranin II, synaptin/synaptophysin and p65 after kainic acid-induced seizures and pentylenetetrazol kindling. Kainic acid seizures resulted in marked but transient increases in secretogranin II mRNA concentrations in the granule cell layer and throughout the pyramidal cell layers of the hippocampus (by 100-500%) as well as in various areas of the cerebral cortex (by up to 900%) and the thalamus (up to 300%) 12 h after injection of the toxin. Chromogranin B mRNA concentrations were persistently increased in granule cells (but not in pyramidal cells) of the hippocampus (suprapyramidal blade, 450%) and in cortical areas (250%) at all time intervals after kainic acid injection (12 h to 60 days). Accordingly chromogranin B immunoreactivity was enhanced in the terminal field of mossy fibers and in the inner part of the molecular layer 30 days after kainic acid. Secretogranin II immunoreactivity was also markedly increased in CA1, the paraventricular thalamic nucleus and in the central amygdala. In rats kindled with pentylenetetrazol only chromogranin B (by 200%) but not secretogranin II mRNA was increased in dentate granule cells. In contrast to the mRNAs of these secretory proteins concentrations of mRNAs encoding synaptin/synaptophysin and p65, two membrane proteins of synaptic vesicles, were not altered in any of these brain structures. These data demonstrate that in brain the biosynthesis of chromogranin B and secretogranin II is regulated like that of neuropeptides which is consistent with a role of these secretory polypeptides as precursors of functional peptides. Activation of neurons induces an increased synthesis of neuropeptides but not a concomitant synthesis of membrane proteins of synaptic vesicle. This might lead to an increased quantal content available for transmission.

Animals↗
PubMed1992

Functional changes in neuropeptide Y- and somatostatin-containing neurons induced by limbic seizures in the rat.

The influence of sustained epileptic seizures evoked by intraperitoneal injection of kainic acid on the gene expression of the neuropeptides somatostatin and neuropeptide Y and on the damage of neurons containing these peptides was studied in the rat brain. Injection of kainic acid induced an extensive loss of somatostatin and, though less pronounced, of neuropeptide Y neurons in the inner part of the hilus of the dentate gyrus. Neuropeptide Y-immunoreactive neurons located in the subgranular layer of the hilus, presumably pyramidal-shaped basket cells, were spared by the treatment. Although neuropeptide Y messenger RNA was not detected in granule cells of control rats, it was found there after kainic acid seizures at all time intervals investigated (12 h to 90 days after injection of kainic acid). High concentrations of neuropeptide Y messenger RNA were especially observed 24 h after injection of kainic acid. At this time neuropeptide Y messenger RNA was also transiently observed in CA1 pyramidal cells. Neuropeptide Y synthesis in granule cells in turn gave rise to an intense immunoreactivity of the peptide in the terminal field of mossy fibers which persisted for the entire time period (90 days) investigated. In addition, neuropeptide Y messenger RNA concentrations were also drastically elevated in presumptive basket cells located at the inner surface of the granule cell layer, especially at the "late" time intervals investigated (30-90 days after kainic acid). These data support the concept that extensive activation of granule cells by limbic seizures contributes to the observed neuronal cell death in CA3 pyramidal neurons and interneurons of the hilus. Consecutively, basket cells containing neuropeptide Y and presumably GABA might be activated and participate in recurrent inhibition of granule cells. Neuropeptide Y-immunoreactive fibers observed in the inner molecular layer at "late" time intervals after kainic acid may result either from collateral sprouting of mossy fibers or from basket cells extensively expressing the peptide. It is speculated that neuropeptide Y synthesized and released at a high rate from granule cells and basket cells may exert a protective action against seizures.

Animals↗
PubMed1991

Visual-field asymmetry in face recognition as a function of face discriminability and interstimulus interval.

Reaction times were measured for recognizing laterally presented faces as a function of two levels of face discriminability and three interstimulus-interval lengths between pairs of successively presented faces. Contrary to many face-recognition studies, no left visual-field/right-hemisphere advantage was observed for the recognition task. Instead, a right visual-field/left-hemisphere advantage occurred at the longest interstimulus interval (500 msec.) on trials on which faces were different and at the shortest interstimulus interval (5 msec.) on trials on which the faces were identical. It appears that visual-field asymmetries in face recognition may vary due to the characteristics of the stimuli and procedures used in face-recognition studies.

Brain↗