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At least 307 records · Page 17Linked to original sources
PubMed1992

Chronic intracerebral infusions of vasopressin and vasopressin antagonist modulate behavioral effects of interleukin-1 in rat.

To assess the role of sex dependent brain vasopressinergic transmission in the modulation of the neural effects of interleukin-1, castrated male rats that are deficient in vasopressin were implanted intracerebroventricularly with an Accurel collodion mini device containing 10 micrograms AVP whereas intact male rats were implanted with a similar device containing 50 micrograms of dPTyr(Me)AVP, a specific antagonist of the vasopressor-like receptors of vasopressin. Control rats in each sex group were implanted with an Accurel device containing distilled water. Acute intracerebroventricular injection of 1.25-2.50 ng recombinant human interleukin-1 beta decreased in a dose and time-dependent manner social investigation of a juvenile conspecific. This effect was more intense in intact rats chronically infused with dPTyr(Me)AVP and less intense in castrates infused with AVP. These results confirm the modulatory role of sex-dependent vasopressinergic neurotransmission on the neural effects of interleukin-1.

Analysis of Variance↗
PubMedDate not supplied

Standardization of 125I and 238Pu.

A simple distance-variable detection table on which two detectors can be mounted and moved back and forth facing each other centred on a sample source has been constructed for photon-photon and alpha-photon coincidence counting for 125I and 238Pu, respectively. Two NaI(Tl) detectors were used for the standardization of 125I. A Si detector and an NaI(Tl) detector were employed for the standardization of 238Pu. The NaI(Tl) detectors have a very thin aluminium window for measuring low-energy photons from 125I and 238Pu while the Si-detector is a usable-in-air ion-implanted detector for alpha particles from 238Pu. A thin collodion film was used for 238Pu samples, whereas a thick polyester film was used for 125I samples to prevent 125I from leaking from the sample. The key comparison result of 125I showed good agreement, and the result of 238Pu was also compared with that of liquid scintillation counting.

Algorithms↗
PubMed2004

Primary intraocular lymphoma: clinical, cytologic, and flow cytometric analysis.

PURPOSE: To compare cytologic with flow cytometric results of vitreous biopsy specimens obtained to rule out primary intraocular lymphoma (PIOL). STUDY DESIGN: Prospective noncomparative case series. PARTICIPANTS: Patients suspected of having PIOL who underwent vitreous biopsy were evaluated. METHODS: Patients underwent a standard 3-port vitrectomy and vitreous biopsy to rule out PIOL. Each undiluted specimen was split, and half was prepared for cytologic evaluation with the collodion bag method; the other half was submitted for flow cytometric immunophenotyping (FCI). The diluted specimen was processed as a cell block for cytology. MAIN OUTCOME MEASURES: Final diagnosis based on cytology and FCI. RESULTS: Ten of 14 patients had sufficient specimens for both cytologic and FCI evaluation. Three patients had chronic inflammation confirmed by both methods. Six patients had large cell lymphoma identified by both cytology and FCI. Two of those 6 patients initially had insufficient specimen for FCI. One patient had large cell lymphoma diagnosed cytologically that was initially negative for a clonal population by FCI. All lymphomas were B-cell type. CONCLUSIONS: Cytologic evaluation is an accurate diagnostic technique to evaluate for PIOL. FCI is useful for immunophenotyping PIOL. Multiple biopsies may be required to achieve a diagnosis.

Adult↗
PubMedDate not supplied

Gaucher disease: complexity in a "simple" disorder.

Gaucher disease, the recessively inherited deficiency of the enzyme glucocerebrosidase and the most common sphingolipidosis, has both non-neurological and neuronopathic forms and a continuum of diverse clinical manifestations. Studies of genotype-phenotype correlations reveal significant genotypic heterogeneity among clinically similar patients, and vastly different phenotypes among patients with the same mutations. The region surrounding the glucocerebrosidase gene (GBA) on chromosome 1q is particularly gene-rich, with a highly homologous pseudogene sequence 16 kb downstream. Recombination events within the GBA locus contribute to the etiology of some mutations in Gaucher disease. Studies of patients with Gaucher disease and atypical manifestations, including parkinsonism, myoclonic epilepsy, cardiac involvement and collodion skin, seek to define other genetic or environmental factors contributing to the phenotypes. Recent reports demonstrating an association between Gaucher disease and parkinsonism provide an example of heterozygosity for a Mendelian disorder acting as a risk factor for a complex disease. There are rare patients with Gaucher disease and differing genotypes who develop early onset, treatment-refractory parkinsonism. Neuropathology in a group of these patients showed alpha-synuclein-reactive Lewy bodies in brain regions specifically associated with Gaucher disease. Family studies of these probands suggested that the incidence of parkinsonism might be more frequent in obligate heterozygotes. In a complementary finding, the examination of GBA in autopsy samples from individuals with sporadic Parkinson disease identified alterations in the GBA sequence in 14% of the cohort. These studies provide evidence that altered glucocerebrosidase may contribute to a vulnerability to parkinsonism. Moreover, this research demonstrates how insights from rare, single gene disorders like Gaucher disease can provide a window into the etiology of more common, multifactorial genetic diseases.

Animals↗
PubMed1979

Extracorporeal immunoadsorption: initial experience in human systemic lupus erythematosus.

Extracorporeal immunoadsorption and filtration was used in treatment of a 29-year-old woman with severe lupus nephritis. For the previous 35 days single-stranded DNA (ssDNA) antibody and immune-complex levels had been continuously raised with only partial improvement on prednisone therapy. Levels of immune complexes and ssDNA antibodies were substantially reduced by extracorporeal plasma filtration and adsorption of DNA collodion charcoal. There were no major clinical complications. After perfusion, the reduction of ssDNA binding and immune complexes in serum was sustained, serum C'3 became normal, and serum creatinine and proteinuria improved. 28 days after perfusion, subendothelial glomerular deposits were much reduced compared with those in a pre-perfusion biopsy specimen. Selective immunoadsorption is a promising new approach to persistent lupus nephritis refractory to drug therapy alone.

Adult↗
PubMedDate not supplied

The effect of developmental exposure to cadmium (Cd) on visual evoked potentials (VEPs) and lipid peroxidation.

Pregnant Swiss albino rats were divided into three groups: control (C), gestational exposure of Cd (G-Cd), and gestational/postnatal exposure of Cd (GP-Cd) groups. Control animals received tap water, and the rats of GP-Cd group received Cd as CdC12 in their drinking water during the experimental period. The G-Cd group was given Cd during pregnancy, but given tap water after birth. Twenty-two days after birth, 15 rats (for each group) were taken from their mothers and continued to be treated with Cd (GP-Cd group) or tap water (C and G-Cd groups) for an additional 38 days. On postnatal day (PND) 60, flash visual evoked potentials (FVEPs) were recorded with disc electrodes attached with collodion 0.5 cm in front of and behind bregma. The mean latencies on N1, P2, and P3 were prolonged in the GP-Cd group compared with controls. The mean latency of P3 was also significantly different between G-Cd and GP-Cd groups. P1-N1 and N1-P2 amplitudes of VEPs were significantly decreased in the GP-Cd group compared with control group. N1-P2 amplitude of the G-Cd group was significantly lower than that of the control group. Thiobarbituric acid reactive substances (TBARS) were determined as an indicator of lipid peroxidation. Our data showed that pre- and postnatal Cd treatment caused a significant increase of lipid peroxidation in the brain.

Analysis of Variance↗
PubMed2003

Loricrin keratoderma: a novel disease entity characterized by nuclear accumulation of mutant loricrin.

Loricrin is the major protein of the cornified cell envelope, a structure that replaces the plasma membrane during keratinocyte terminal differentiation. Recently, unique heterozygous, insertion mutations in the loricrin gene have been found to underlie certain congenital skin abnormalities, the phenotypes of which vary considerably. Clinically, these patients can be diagnosed as suffering from an ichthyotic variant of Vohwinkel's syndrome (VS), progressive symmetric erythrokeratoderma, or congenital ichthyosiform erythroderma born as a collodion baby. Common clinical features include hyperkeratosis of the palms and soles with digital constriction. Histologic characteristics include parakeratotic hyperkeratosis with hypergranulosis and nuclear accumulation of mutant loricrin. The unique mutations in the glycine-rich domain of the mutant loricrin form arginine-rich nuclear localization sequences (NLSs) that disrupt differentiation of keratinocytes. This group of unique genodermatoses caused by distinct loricrin mutations is collectively termed as loricrin keratoderma (LK).

Animals↗
PubMed1999

Penetration studies of clotrimazole from semisolid formulation using step-scan FT-IR photoacoustic spectroscopy.

PURPOSE: The aim of this study was to elucidate the potential use of the step-scan FT-IR photoacoustic spectroscopy (PAS) for the non-destructive determination of drug penetration into membranes. METHODS: The penetration of clotrimazole from a 10% (w/w) suspension in Vaseline into a dodecanol-collodion acceptor membrane was studied by three methods: the step-scan FT-IR PAS with a phase modulation, a multilayer membrane system, and a modified libration model. Based on Fick's second law, the diffusion coefficient of the drug in the membrane was derived by numerical fitting of the experimental data. RESULTS: The three methods applied provided almost the same diffusion coefficient D = 2.2 10(-9) cm2/s for clotrimazole in the membrane. Because of the non-destructive mode of operation, the accuracy of results obtained by FT-IR PAS is much better than that attainable by other two methods. CONCLUSIONS: Step-scan FT-IR photoacoustic spectroscopy in conjunction with a phase modulation is useful to determine the penetration of drug through membranes. The fact that samples can be investigated without elaborate preparation is an advantage of this spectroscopic technique.

Acoustics↗
PubMed2003

Gaucher's disease: identification of novel mutant alleles and genotype-phenotype relationships.

A sequencing protocol for the acid beta-glucosidase (GCase) gene (GBA) was developed using a long-range PCR template. This protocol has an advantage of greater DNA yields over similar strategies. Seven Gaucher's disease patients had four novel and five other rare alleles. A non-pseudogene in-frame deletion (g.2600-2602delTAC) and a new complex mutation (null allele) were identified in Gaucher's disease type 1, i.e. the g.2600-2602delTAC deletion is associated with the non-neuronopathic variant. An F251L allele was found in a baby with the collodion skin phenotype. Three mutant alleles were identified in a single primary family with type 3. The patients' father at 45 years is healthy and is heteroallelic for the G202R and E326K alleles. Family studies indicated that E326K is in trans to G202R and L444P, and that isolated E326K is non-pathogenic in this family. A rare mutation R257Q was identified in a type 2 patient, providing an association with neuronopathic disease. A genotype L444P/L444P was noted in a 22-year-old non-neuronopathic patient. Complete gene sequencing showed a new complex allele consisting of L444P and g.7741T > C in the 3' UTR. Three additional complex alleles also involved the 3' UTR. Complete gene characterization in Gaucher's disease should allow greater insights into the correlation of specific alleles with phenotype.

Adolescent↗
PubMedDate not supplied

Is the perinatal lethal form of Gaucher disease more common than classic type 2 Gaucher disease?

In recent years there has been increased recognition of a severe perinatal lethal form of Gaucher disease, the inherited deficiency of lysosomal glucocerebrosidase. We previously reported a case of severe type 2 Gaucher disease which was seen in a medical center in Rotterdam and now present three new cases from two other families seen at the same center. Mutational analyses of these cases revealed two novel mutations, H311R and V398F, located in exons 8 and 9, respectively. The identification of four cases of lethal type 2 Gaucher disease in a single center seems to be a function of increased awareness of this phenotype, rather than of geographic clustering. The actual incidence of lethal type 2 Gaucher disease may be underestimated, as many cases may have been misclassified as collodion babies or hydrops of unknown cause.

DNA Mutational Analysis↗
PubMed1998

Ichthyosiform erythroderma associated with generalized pustulosis.

Two unrelated boys are described who were born as collodion babies and subsequently developed non-bullous ichthyosiform erythroderma and flares of generalized sterile pustulosis, similar to generalized pustular psoriasis. Both patients had a good response to treatment with oral retinoids.

Acute Disease↗
PubMed2000

Non-bullous congenital ichthyosiform erythroderma, with ocular albinism and Noonan syndrome.

A 21-year-old woman presented with non-bullous congenital ichthyosiform erythroderma; she was born a collodion baby. Associated features were ocular albinism, anterior segment dysgenesis of both eyes and Noonan syndrome. X-linked ichthyosis (steroid sulphatase deficiency) and X-linked ocular albinism have been mapped to the Xp22.3 region and cases have been reported with both conditions due to a partial short-arm deletion of the X chromosome. The ichthyosis and ocular albinism in the present case, however, are likely to be of the autosomal recessive type - a very rare association - and the combination with Noonan syndrome has not been reported previously.

Adult↗
PubMed2001

Use of cyanoacrylate tissue adhesive under a diaper.

OBJECTIVE: To examine the utility of cyanoacrylate tissue adhesive (Dermabond, Ethicon, Somerville, NJ, USA; an effective means of epithelial skin closure) under a diaper (i.e. constantly exposed to bodily fluids) in 25 children (not toilet-trained) undergoing inguinal surgery. PATIENTS AND METHODS: A retrospective review of charts between July 1998 and July 2000 was used to identify children who were not toilet-trained and who had undergone inguinal surgery (e.g. orchidopexy, hernia and hydrocele repair). In addition to the type of surgery, the method of closure was also assessed; in all cases reported the subcutaneous tissues were approximated with 4/0 polyglactin suture. From July 1998 to October 1999 all epithelial skin was closed with 5/0 poliglecaprone, benzoin, sterile strips and collodion; from October 1999 to July 2000 all skin was closed with cyanoacrylate tissue adhesive. RESULTS: The chart review identified 45 boys (mean age 15.3 months, range 1-26) of whom 26 (57%) had undergone either inguinal hernia or hydrocele repair, and 19 (42%) who had undergone orchidopexy. These procedures resulted in a total of 52 incisions in the 45 children. Of the 52 incisions, 27 were closed with suture and 25 with cyanoacrylate adhesive. All patients were followed up at 2 weeks; there were no instances of infection or wound dehiscence in either group. The incisions were cosmetically identical at 3 months of follow-up. CONCLUSION: Cyanoacrylate adhesive is a safe and effective method of skin closure that can be used under a diaper.

Child↗
PubMed2001

Resolution of recurrent perianal condylomata acuminata by topical cidofovir in patients with HIV infection.

Anogenital condylomata acuminata are the most frequent clinical manifestation of genital human papillomavirus (HPV) infection. Association between human immunodeficiency virus (HIV) and HPV infections is frequent (range: 26-60% in males). Topical cidofovir (a nucleotide analogue antiviral drug active against a broad range of DNA viruses) is a potential treatment for anogenital warts in immunocompromised patients. We treated three HIV-infected patients with HPV perianal condylomas with topical 1% cidofovir in flexible collodion once a day for 2 weeks. The treatment resulted in complete clearance of the HPV lesions. The patients experienced mild transient erythema without any other side-effects. None of the patients relapsed during the 10-14-month follow-up period.

AIDS-Related Opportunistic Infections↗
PubMedDate not supplied

Hemodialyzer membranes and configurations: a historical perspective.

The principle of hemodialysis (HD) was first described over a century ago while the first human HD treatment was performed in 1923 with collodion tubes. Since that time, a variety of different hemodialyzer configurations and membranes have been used. The purpose of this article is to provide a historical review of these various configurations and membranes. The rotating drum, coil, parallel flow, and hollow fiber artificial kidneys are discussed. Emphasis is placed on the factors that have influenced the shaping of the contemporary HD market.

Equipment Design↗
PubMed2002

Variability of inguinal hernia surgical technique: A survey of North American pediatric surgeons.

BACKGROUND/PURPOSE: The tradition of learning from mentors is a unique aspect of surgical training. With this in mind, the authors sought to document our roots by analyzing the technical variability of how pediatric surgeons perform their most frequent operation, the inguinal hernia, and compare these data with the original description by Drs William Ladd and Robert Gross. METHODS: A survey compiling the operative steps of an inguinal hernia repair as well as several key clinical situations involving hernias was mailed to pediatric surgeons in North America. These results then were compared with the original inguinal hernia technique by Drs Ladd and Gross. Results are recorded as the percent who concurred with their original description. RESULTS: A total of 447 of 640 (70%) surveys were returned. Geneologic data show that 81% of surgeons' hernia lineage could be traced to Drs Ladd and Gross. When compared with all respondents, Drs Ladd and Gross' hernia repair steps included incising Scarpa's fascia (61%), defining the external ring by pushing down with retractors (34%), incising the external oblique with scissors (18%), identifying the ileoinguinal nerve (81%), cleaning one underside of the external oblique (22%), bluntly spreading the cremasteric fibers (90%), elevating the sac with sharp dissection of the vessels (53%), opening the sac and inserting the forefinger into it (0%), bluntly dissecting the sac with forefinger and gauze (0%), ligating the sac with single ligature (22%) without twisting it (34%), leaving the distal sac untouched other than to drain fluid (78%), not inspecting the testicle (79%), performing a formal floor repair bringing external and internal oblique down to Poupart's ligament (10%), tightening the internal ring in both boys and girls (19% and 41%), using no local anesthetic (14%), closing Scarpa's fascia (94%), closing the skin with interrupted subcuticular sutures (49%), covering the incision with Collodion (48%), using the Stiles' dressing (0%), and only exploring the contralateral side if a hernia is suggested by history or physical examination (87% for boys, 60% for girls). The various other options surgeons use for their technique and their management decisions also are described. CONCLUSIONS: There is significant variability in the way pediatric surgeons perform inguinal herniorraphy. The differences from Drs Ladd and Gross' original description likely result from evolving techniques, experiences, and analysis of outcomes.

Child, Preschool↗
PubMed1971

Quantitative two-dimensional ultrastructural distribution of Rh o (D) antigenic sites on human erythrocyte membranes.

A method is described for determining the two-dimensional distribution of specific antigens on cell surfaces, and is applied to the D antigen of the Rh antigenic system. Rh-positive human erythrocytes are allowed to react with purified (125)I-labeled human anti-Rh(o)(D) gamma-globulin antibodies, and the sensitized cells are then lysed at an air-water interface. The residual cell membranes are spread flat by surface forces, and are picked up on a carbon-strengthened collodion-coated electron microscope grid. The membranes are then stained with ferritin-conjugated goat antibodies directed against human gamma-globulins. Only Rh-positive cells sensitized with anti-Rh(o)(D) antibodies bind the ferritin-conjugated antihuman gamma-globulins. The ferritin particles are found in small clusters on the membrane surface, and the number of such clusters per unit area agrees with the number of (125)I-labeled anti-Rh(o)(D) antibodies bound per unit area. The Rh(o)(D) antigenic sites appear to be molecularly dispersed on the membrane surface, but in a random two dimensional array.

Adult↗
PubMed2003

Autosomal recessive congenital ichthyosis in Sweden and Estonia: clinical, genetic and ultrastructural findings in eighty-three patients.

Congenital (non-bullous) ichthyosis is a rare group of keratinizing disorders which can be tentatively subclassified based on clinical criteria, analysis of transglutaminase 1 gene mutations and electron microscopy of epidermis. We studied 83 patients who were all on topical therapy and in 16 cases also on oral retinoids. Three main groups of patients were distinguished: (A) those with transglutaminase 1 gene mutations (n=44), (B) those without transglutaminase 1 gene mutations showing a coarse, generalized scaling (n=19), and (C) those without transglutaminase 1 gene mutations showing only fine or focal scaling (n=20). On clinical scoring, patients in group A were more hyperkeratotic and less erythematous than those in group B (p < 0.05). Anhidrosis was recorded in nearly all patients (> or = 80%), but ectropion and a collodion phenotype at birth were more common in group A versus other groups. Ultrastructurally, a high frequency of type I (Anton-Lamprecht's classification) was found in all three groups (37-63%), 20 cases of type II in group A and a few cases of types III and IV in groups B and C, respectively. In conclusion, transglutaminase 1 gene mutation is a major cause of congenital ichthyosis in Sweden and Estonia, and is often associated with severe scaling and ultrastructural type II in corneocytes. The transglutaminase-unrelated cases are more heterogeneous, probably reflecting a more varied aetiology.

Adolescent↗