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Biomedical subjects

H Wolff

Publications and source records attributed to H Wolff.

At least 73 records · Page 4Linked to original sources
PubMed1997

[Brazilian pemphigus foliaceus (fogo selvagem)].

Most of the clinical, histological and immunohistological features of fogo selvagem resemble those of idiopathic pemphigus foliaceus (PF). Both diseases are clinically characterized by small flaccid bullae evolving into to scaly and crusted lesions, sometimes with pustules, mainly in seborrheic areas of the skin. Mucosal surfaces are mostly spared. The main histologic feature of endemic pemphigus foliaceus is a subcorneal acantholytic blister. Standard immunofluorescence studies demonstrate intercellular IgG deposits throughout the entire epidermis. These IgG antibodies are mainly of the IgG4-subclass. Almost all patients have circulating IgG-autoantibodies in their serum directed against stratified epithelial desmosomes. The fogo selvagem autoantibodies and the PF antibodies are directed against the 160 kD desmosomal glycoprotein desmoglein 1 which together with plakoglobin (85 kD) forms a complex of adhesion proteins with desmosomes of stratified epithelia. Fogo selvagem occurs in endemic foci in some areas of Brazil and possibly in neighbouring South American countries, very often in children, adolescents and young adults. The etiology of fogo selvagem is still unknown. The frequent association with insect bites has lead to the concept of fogo selvagem being a transmissible disease with acquired immunity in adulthood. However, the infectious agent and possible vectors have not yet been identified.

Adolescent↗
PubMed1997

[Dorfman-Chanarin syndrome--a neutral lipid storage disease].

Dorfman-Chanarin syndrome is a rare, autosomal recessive inherited lipid storage disease with skin manifestations in form of congenital ichthyotic erythroderma. Demonstration of lipid vacuoles in neutrophils from peripherals blood smears in patients with ichthyotic erythroderma leads to the diagnosis. Other organ systems, such as CNS, liver, muscle, ears and eyes, are frequently involved. Since Dorfman-Chanarin syndrome may present with just skin findings, it should be included in the differential diagnosis of the congenital ichthyoses. Microscopic examination of peripheral blood smears is recommended in all patients with ichthyosis. We summarize the current data on the pathogenesis, diagnosis, differential diagnosis, and therapeutic options in Dorfman-Chanarin syndrome, give an overview of the clinical manifestations of the 23 patients affected with this rare disease.

Adolescent↗
PubMed1997

Development of T-tube tracts in piglets: effect of insertion method and material of T-tubes.

T-tube-related bile leakage is a considerable problem in liver transplantation but rather rare in surgery of biliary lithiasis. To investigate the effect of T-tube insertion method and material on the intraperitoneal T-tube tract, we performed a choledochotomy and insertion of T-tube (four of silicone, seven of latex, four of silicone with a latex sheath around the long arm) for 2 weeks on 15 piglets (choledochotomy group), and sutured a transected bile duct over a T-tube stent in nine piglets (five silicone, four latex), inserted similarly as in liver transplantations, for 6 weeks. Sixteen patients underwent cholectochotomy and T-tube drainage with a latex T-tube (n = 8) and latex-sheathed silicone T-tube (n = 8) for a median 9 (7-21) days. Histological examination of T-tube tracts in piglets was made, and complications after T-tube removal in the latex T-tube group were compared with those in the latex-sheathed silicone T-tube group. In piglets, latex T-tubes induced better tracts than silicone T-tubes (P < 0.05). Piglets in the choledochotomy group had tracts superior to those in the anastomotic stent group (P < 0.05). There was one bile leakage in the latex T-tube group, and none in the latex-sheathed silicone T-tube group. We conclude that T-tube tract development is affected by both the material and the insertion method of T-tubes. A silicone T-tube with a latex sheath around the long arm may also be a good choice for T-tube material in liver transplantation.

Adult↗
PubMed1997

Acne conglobata and Klinefelter's syndrome.

Acne conglobata has been reported to be more common and more severe in XYY chromosomal aberration than in normal subjects. Whereas only exceptional cases have been observed in the more common XXY Klinefelter karyotype. We report a 17-year-old male with both Klinefelter's syndrome and acne conglobata, spreading from face to upper arms, trunk, buttocks and upper legs. The serum level of testosterone was normal, whereas luteinizing hormone and follicle-stimulating hormone were elevated. Skin lesions responded well to a combined therapeutic regimen including oral isotretinoin and prednisolone. An overview of severe acne in relation to chromosomal abnormalities is given and diagnostic and therapeutic options discussed.

Acne Vulgaris↗
PubMed1996

Human papillomavirus infections in nonmelanoma skin cancers from renal transplant recipients and nonimmunosuppressed patients.

BACKGROUND: Nonmelanoma carcinomas of the skin represent the most frequent cancers among the Caucasian population worldwide. They occur with high frequency in renal allograft recipient patients after prolonged immunosuppression. PURPOSE: We analyzed tumors obtained from both immunosuppressed and nonimmunosuppressed patients for human papillomavirus (HPV) DNA. METHODS: Twenty-nine specimens of nonmelanoma carcinomas of the skin were obtained from 19 renal allograft recipient patients; these included 20 specimens of squamous cell carcinoma (SCC) from 11 patients, five specimens of basal cell carcinoma (BCC) from four patients, and four specimens of carcinoma in situ (CIS) from four patients. Forty-one specimens of nonmelanoma carcinomas of the skin were obtained from 32 nonimmunosuppressed patients; these included 26 SCC specimens from 19 patients, 11 BCC specimens from nine patients, and four keratoacanthoma (benign epithelial tumor) specimens from four patients. A polymerase chain reaction method involving use of degenerate oligonucleotide primers, in which the conserved region of the open reading frame of the HPV L1 (major capsid protein) gene is amplified, was used to amplify total cellular DNA purified from individual tumors. The DNA of each specimen was subjected to 16 different amplification reactions; different primer combinations were used in order to increase the sensitivity and specificity of HPV detection. Resulting products were probed with a radioactively labeled, degenerate oligonucleotide. HPV-specific DNA was either sequenced directly after elution from the gel or amplified with semi-nested, degenerate primers, after which the products were cloned and sequenced. Sequences were compared with all known papillomavirus sequences. RESULTS: Thirteen (65%) of the 20 SCC specimens and three of the five BCC specimens from immunosuppressed (renal allograft recipient) patients contained identifiable HPV-related sequences, among them 13 putative novel HPV genomes. In addition, all other malignant tumor specimens from this patient group revealed faint signals upon amplification and hybridization; the origin of these signals has not been identified in the present study. In nonimmunosuppressed patients, eight (31%) of 26 SCC specimens and four (36%) of 11 BCC specimens contained sequences of HPV types. Two putative novel HPV sequences could be identified in this group. Faint signals of yet undetermined origin were observed in eight of the SCC specimens and in two of the BCC specimens. Two of four keratoacanthoma specimens contained sequences of known HPV type. (Keratoacanthoma is a nonmalignant lesion for which the natural history has not been defined.) The spectrum of HPV types in both groups of patients differed substantially. CONCLUSIONS: These data point to the frequent presence of HPV sequences in SCCs and BCCs of the skin. The etiologic relationship of these infections to the respective malignant tumors remains to be evaluated. IMPLICATIONS: The presence of HPV DNA in a large percentage of specimens of nonmelanoma carcinomas of the skin from immunosuppressed patients, as well as from nonimmmunosuppressed patients, renders a papillomavirus infection as a possible factor in the etiology of this disease.

Amino Acid Sequence↗
PubMed1996

Characterization of H4: a mouse T lymphocyte activation molecule functionally associated with the CD3/T cell receptor.

The monoclonal antibody C398.4A was produced by immunizing Armenian hamsters with the mouse T cell clone D10.G4.1. It recognizes a molecule selectively expressed by activated mouse T cells and was named H4. H4 is expressed on the T cell surface about 24 h after activation and peaks at day 7. By contrast, it is not expressed by resting or activated B cells, macrophages, or fibroblasts. It is also expressed by CD4 or CD8 single-positive mature thymocytes. Immunoprecipitation showed that H4 is a disulfide-linked dimer, precipitating as a broad band at about 50-65 kDa under nonreducing conditions and at 25 and 29 kDa under reducing conditions. Deglycosylation of the reduced H4 by N-glycanase gave rise to a single band of about 21 kDa, suggesting that the two chains may be differentially glycosylated forms of the same protein. The H4 expression pattern and biochemical features, together with cross-blocking, co-capping, co-modulation, and immunoprecipitation preclearing experiments showed that H4 is different from other known co-stimulatory molecules such as CD69, CD2, Ly-6, CD25, OX-40, Mac-1 and LFA-1. By in vitro kinase assay, H4 was found to co-precipitate a tyrosine kinase activity that phosphorylated substrates of about 29 and 25 kDa. Co-modulation and co-capping experiments showed that H4 is physically associated with the CD3/T cell receptor. These data suggest that H4 may function as a T cell-specific co-stimulatory molecule and play a role in the T cell response when the activation stimulus is limited either because the antigen is only available in low concentration or has a low agonistic activity.

Animals↗
PubMed1996

Biocompatibility of latex and silicone T tubes in the porcine common bile duct: an experimental study.

Toxicity of materials used in indwelling drains or catheters has the potential to damage surrounding tissues. The biocompatibility of biliary T-tubes was investigated using in groups of piglets (total 30 animals). In group 1 (n=16) a choledochotomy was performed with insertion of a latex T-tube (n=6) or a silicone T-tube (n=8), or without a T-tube (n=2). In group 2 (n=14) the common bile duct (CBD) was 3/4-transected, and the lesion was sutured over a latex T-tube (n=4) or a silicone T-tube (n=5), or without a T-tube (n=5). The groups were reoperated upon after 2 and 6 weeks respectively, and the CBD was harvested for scanning electron microscopy and light microscopy. The T-tubes were examined for cell culture toxicity with a DNA synthesis inhibition test. According to the cell culture tests, latex T-tubes were toxic and silicone T-tubes nontoxic. T-tubes induced moderate to pronounced fibrosis and epithelial damage in the CBD wall, but did not induce excessive fibrosis or scarring at the site of CBD suture. In the 6-week study period, however, the grade of tissue reactions in the CBD did not correlate with the toxicity of the T-tube materials, but rather reflected a foreign body reaction and mechanical pressure. Although gross anatomical changes did not occur, neither material seemed to be completely harmless for porcine CBD wall.

Animals↗
PubMed1996

[Eruptive vellus hair cysts].

Eruptive vellus hair cysts are rare epidermal cysts with vellus hairs, which appear in children or young adults. A 21-year-old patient is reported who developed asymptomatic comedo-like papules on his face, neck and chest. Histopathologically, epidermoid cysts with a fine stratum granulosum, laminated keratinous material and numerous vellus hairs were found in the mid-dermis. Evaluation of immediate family members revealed that the patient's father was similarly affected. Eruptive vellus hair cysts and steatocystoma multiplex are clinically similar, but have distinctive histopathological features and should be regarded as distinct entities.

Adult↗
PubMed1996

[Electroencephalographic changes in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency].

Electroencephalographic changes have been detected in children with premature pubarche. These findings might be the result from past or current central nervous system dysfunction, or from increased sex steroids. In order to test the last assertion we have performed electroencephalographic studies in 10 patients (7F, 3M) with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency, of whom 4 classic and 6 nonclassic forms of the syndrome. Traditional neurologic examination was performed in 7 patients, the result being considered normal in everyone, while evolutional neurologic examination detected hyperactive disturbance and attention deficit in two patients. In another patient, the clinical findings were compatible with delay of neuropsychomotor development. Quantitative electroencephalography with brain mapping in patients over three years-old (n = 9), and conventional EEG in the remaining patient, were considered abnormal for age in 8 patients (80%), the record being characterized as slow in 7 patients. The high rate of electroencephalographic changes in this sample, as well as in premature pubarche, suggests that these findings might result from high inappropriate for age androgenic levels, and warns about the importance of neurologic examination and follow-up in children with CAH.

Adolescent↗
PubMed1996

Multiple keratoacanthomas, giant keratoacanthoma and keratoacanthoma centrifugum marginatum: development in a single patient and treatment with oral isotretinoin.

A 78-year-old man is described, who over 18 years developed three different types of keratoacanthoma: multiple keratoacanthomas, keratoacanthoma centrifugum marginatum and giant keratoacanthoma. Histological examination of the different neoplasms showed similar changes, all typical of a keratoacanthoma. In situ hybridisation revealed no human papilloma virus in the tumours. Complete examination showed no associated internal malignancy. After repeated surgical treatment oral isotretinoin treatment was administered (1 mg/kg per day). This treatment produced clearing of existing keratoacanthomas and, during a period of 2 months, further keratoacanthoma formation was completely suppressed. Treatment was stopped after 3 months by the patient because of side-effects. Numerous keratoacanthomas developed during the following 6 weeks.

Administration, Oral↗
PubMed1995

The biologic significance of white blood cells in semen.

OBJECTIVE: To analyze the data available on the biologic significance of white blood cells (WBC) in semen of infertility patients. DATA RESOURCES: The relevant literature was reviewed. RESULTS: It is not possible to identify reliably WBC by conventional sperm staining techniques. The peroxidase method is sufficient for quantification of granulocytes, but immunocytology is the gold standard for the detection of all WBC populations in semen. Granulocytes are the most prevalent WBC type in semen (50% to 60%), followed by macrophages (20% to 30%) and T-lymphocytes (2% to 5%). The prevalence of leukocytospermia (> 10(6) WBC/mL semen) among male infertility patients is approximately 10% to 20%. There is controversy on the significance of WBC in semen. Whereas some authors did not observe sperm damage in the presence of leukocytospermia, others have found evidence that WBC are significant cofactors of male infertility: [1] seminal WBC numbers were higher in infertility patients than among fertile men; [2] leukocytospermia was associated with decreased sperm numbers and impaired sperm motility; [3] WBC damaged sperm function and hamster ovum penetration in vitro and were important prognostic factors for IVF-ET failure. Because of absence of clinical symptoms, the origin of WBC is difficult to determine. Normally, most WBC appear to originate from the epididymis because vasectomized men show very few WBC in semen. On the other hand, leukocytospermic samples show low citric acid levels, pointing to asymptomatic prostatitis as a source of WBC in semen. Surprisingly, approximately 80% of leukocytospermic samples are microbiologically negative. In some cases Chlamydia trachomatis might have triggered a persistent inflammatory reaction leading to leukocytospermia. Sperm damage by WBC can be mediated by reactive oxygen species, proteases and cytokines. Furthermore, genital tract inflammation facilitates the formation of sperm antibodies. As seminal plasma has strong anti-inflammatory properties and because there is only short contact between sperm and WBC in prostatitis and seminal vesiculitis, inflammations of the epididymis and testis are likely to have the largest impact on sperm. CONCLUSIONS: There is ample evidence that WBC can affect sperm function. Further studies are needed to define cofactors that increase or decrease the risk of sperm damage by WBC.

Autoantibodies↗
PubMed1995

Biocompatibility of various indwelling double-J stents.

The biocompatibility of 8 double-J stent brands was evaluated both in vivo, by scanning electron microscopy and histological analysis of pig ureters intubated for 6 weeks, and in vitro, by cell culture methods. In vivo findings showed that superficial epithelial destruction was milder in the ureters intubated with hydrogel-coated stents than in those intubated with other stents. Inflammatory reactive changes were milder in the ureters intubated with silicone stents than in those intubated with other stents, while encrustation was more severe on silicone and Grey stents than the others. In vitro, the 3 stent brands made of "modified polyurethane" were found to be cytotoxic while the others were not. Overall, silicone and hydrogel-coated stents seemed to be more biocompatible than the others, and hydrogel-coated stents to be more suitable than silicone stents for long-term ureteral stenting, being less prone to encrustation. Although they are of an experimental nature, these findings may be of relevance to clinical practice.

Animals↗
PubMed1994

[Andrologic variables for in vitro fertilization].

The purpose of the study was to identify semen parameters relevant for in vitro fertilization (IVF). A total of 151 ejaculates from 130 patients were analyzed. There was a significant association between successful IVF, high sperm concentration and good sperm motility. Progressive sperm motility correlated best with successful fertilization of oocytes (r = +0.427) and was the only parameter significantly linked to pregnancy. Very low values were sufficient for fertilization in vitro: a sperm concentration of 4.5 x 10(6)/ml and a progressive sperm motility of 20%. The results of analysis of fructose, citrate and neutral alpha-glucosidase as markers of seminal vesicle, prostate and epididymal functions were not different in fertilizing and non-fertilizing semen samples. Moderate increases in granulocyte elastase levels, between 250 and 1000 ng/ml, did not influence IVF. However, two patients with genital tract inflammation (PMN-elastase > 1000 ng/ml) did not fertilize despite excellent semen parameters. These results stress the importance of andrological factors for in vitro fertilization outcome.

Adult↗