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Biomedical subjects

H Hamm

Publications and source records attributed to H Hamm.

At least 127 records · Page 7Linked to original sources
PubMed1991

[The "widow syndrome"].

The considerable increase in the number of elderly people in the general population in recent years has also resulted in an increase in the number of elderly patients, in particular in the office of the general practitioner. Among the over 65-year-olds, we now have about twice as many women as men, and widows outnumber widowers by about 6 to 1. As patients, widows often present with a typical general clinical picture that might be termed the "widow syndrome". This article describes this syndrome.

Aged↗
PubMed1991

Further delineation of the ichthyosis follicularis, atrichia, and photophobia syndrome.

We describe an 18-month-old male infant suffering from the ichthyosis follicularis, atrichia, and photophobia (IFAP) syndrome and further delineate the clinical phenotype. Severe retardation of growth and psychomotor development, chill-like seizures, bronchial asthma, urticaria, a proneness to skin infections and transient nail dystrophy observed in our patient are non-obligatory manifestations of this disorder. Histological examination of the atrichia revealed poorly developed, shortened hair follicles and a complete absence of sebaceous glands. The sex ratio of published cases suggests an X-linked recessive inheritance. The marked clinical variability of the IFAP syndrome might be the expression of a contiguous gene defect.

Alopecia↗
PubMed1991

Lipoprotein analysis in a chyliform pleural effusion: implications for pathogenesis and diagnosis.

A chyliform effusion is an uncommon high lipid pleural effusion that does not result from a leakage of the thoracic duct. Characteristically, it emerges from chronic pleurisy and contains high levels of cholesterol. The origin of this cholesterol is unknown, but it is often attributed to the degeneration of red and white blood cells. In this study we have carried out detailed lipoprotein analyses in a chyliform effusion, a chronic tuberculous effusion and three inflammatory effusions of recent onset, in an attempt to elucidate the process of cholesterol accumulation and possible lipoprotein alterations. Mean cholesterol was 92 mg/dl in the inflammatory exudates and 1,237 mg/dl in the chyliform effusion. In inflammatory effusions of recent onset most cholesterol was bound to low density lipoprotein (LDL) with corresponding apoprotein B levels. The chronic tuberculous exudate showed a shift of cholesterol binding towards high density lipoprotein (HDL). In the chyliform effusion most cholesterol was found in the HDL region. Our results suggest that in acute inflammation, the pleural barrier opens to plasma LDL. We hypothesize that in chronicity this cholesterol becomes trapped in the pleural space and undergoes a change in lipoprotein binding characteristics. In a chyliform effusion, cholesterol further accumulates and builds complexes containing triglycerides and proteins. In clinical routine, total cholesterol values above 200 mg/dl strongly suggest a chyliform effusion. Since triglyceride values may be as high as in chylous effusions (greater than 110 mg/dl), the diagnostic routine in all suspected high lipid effusions should involve cholesterol and triglyceride measurements.

Aged↗
PubMed1991

[Annular elastolytic giant cell granuloma--differential diagnosis of cutaneous granulomatosis].

Annular elastolytic giant cell granuloma (AEGCG) is a rare granulomatous skin disease characterized by phagocytosis of elastic fibres by multinucleated giant cells. A 46-year-old female patient developed multiple annular lesions with elevated borders and central atrophy on the limbs. Histological examination revealed the absence of elastic material in the granulomatous infiltrate and fragments of elastic fibrils in the cytoplasm of the multinucleated giant cells. Immunohistological investigation revealed that the ratio of CD4+ to CD8+ T-lymphocytes was 3:1. Our patient was treated with clofazimine orally, 200 mg daily for the first 10 days, then 100 mg daily. After 12 weeks a definite improvement was achieved. Our case demonstrates that AEGCG, which is mostly localized on head and neck, has to be taken into account in the differential diagnosis of generalized cutaneous granulomatous disease.

Biopsy↗
PubMed1991

[Squamous epithelial carcinoma at the site of lichen ruber hypertrophicus of the glans penis].

Reports of cancer development in lichen planus mostly concern the oral erosive form. The reported frequency of malignant transformation ranges between less than 1% and 10%. There have been a few isolated observations of malignant transformation of cutaneous lichen planus. In these cases, the hypertrophic type located on the lower limbs predominated. We report an unusual case of squamous cell carcinoma developing on the glans penis in the presence of hypertrophic lichen planus. Systemic treatment of the lichen planus with dapsone, retinoids, corticosteroids and topical cyclosporin A failed to prevent this development.

Carcinoma, Squamous Cell↗
PubMed1990

Regulation by light of cyclic nucleotide-dependent protein kinases and their substrates in frog rod outer segments.

Cyclic nucleotides (both cAMP and cGMP) stimulate the phosphorylation of several proteins of 65-70, 50-52, 21, 13, and 12 kD in rod outer segments (ROS) of the frog retina. Subcellular fractionation showed that phosphopeptides of 67, 21, 13, and 12 kD were soluble and phosphopeptides of 69, 67, 50-52, and 12 kD were membrane associated at physiological ionic strength. Components I and II, 13 and 12 kD, respectively, are the major cyclic nucleotide-dependent phosphoproteins of ROS and have been reported to be phosphorylated in the dark and dephosphorylated in the light. Under unstimulated conditions, phosphorylated Components I and II were found in the soluble fraction. Cyclic nucleotide stimulation of phosphorylation resulted in increased phospho-Components I and II in the soluble fraction, and phospho-Component II on the membrane. Light had no effect on the phosphorylation level of soluble Components I and II, but it caused a depletion within 1 s of the membrane-bound phospho-Component II. A half-maximal decrease in membrane-bound Component II was seen at 5 x 10(5) rhodopsins bleached per outer segment. The cyclic nucleotide-dependent protein kinase(s) were found primarily in the peripheral membrane fraction of ROS proteins. 8-bromo cyclic AMP was two orders of magnitude more effective than 8-bromo cyclic GMP at stimulating Component I and II phosphorylation. An active peptide of the Walsh inhibitor of cAMP-dependent protein kinase [PKI(5-22)amide] blocked the phosphorylation with an IC50 of 10 nM. Photoaffinity labeling studies with 8-N3-cAMP and 8-N3-cGMP revealed the presence of a 52-kD band specifically labeled with 8-N3-cAMP, but no specific 8-N3-cGMP labeling. These data suggest that cyclic nucleotide-dependent protein phosphorylation in ROS occurs via the activation of a cAMP-dependent protein kinase.

Animals↗
PubMed1990

[What is new in genetically-induced hair diseases?].

A profound knowledge of specific genetically determined anomalies of the hair may be of considerable value in the diagnosis of genetic syndromes. We give a review of a few recent developments in the field of genetic hair diseases. For example, the brittle hair due to sulphur deficiency (trichothiodystrophy) is nowadays regarded as genetically heterogeneous; three different syndromes can be distinguished: BIDS syndrome, Tay syndrome, and PIBIDS syndrome. Polarization microscopy revealed a striking resemblance of the hair anomalies found in trichothiodystrophy syndromes and those in acrodermatitis enteropathica. This surprising result indicates similar pathophysiological mechanisms. The Comèl-Netherton syndrome--long regarded as representing two different diseases--has recently been recognized as a clinically variable, but genetically homogeneous syndrome, which is most likely based on a single mutation ("lumping"). Minor's sweat test allows the recognition of women heterozygous for X-linked hypohidrotic ectodermal dysplasia and may help to appreciate seemingly non-specific hair findings, such as diffuse alopecia.

Ectodermal Dysplasia↗
PubMed1990

[Divided nevus--an embryological experiment of nature].

A "divided naevus" (or "kissing naevus") is defined as a congenital melanocytic naevus that occurs on adjacent parts of the upper and lower eyelid and may give the appearance of a single lesion when the eye is closed. We report on four cases of this rare naevus. Two of these were examined histologically and showed the features of compound naevi. The unique occurrence in nature of the divided naevus allows conclusions on the site and time of origin not only of the divided naevus, but possibly also of other congenital melanocytic naevi. The divided naevus must originate at the latest during the period of lid fusion, between the 9th and 20th week of gestation. The fact that the fusion involves only epithelial tissue suggests an epithelial origin of congenital melanocytic naevi, which may later develop secondarily into compound or intradermal naevi by way of "dropping-off".

Adolescent↗
PubMed1990

[Cholesterol in pleural effusions: a differential diagnostic aid].

In this prospective study, 70 patients with pleural effusions were investigated; the underlying disease was identified in 62 of these cases. Employing pre-defined criteria, 31 effusions were identified to be transudates, and 31 exsudates. The total protein, LDH and cholesterol were determined in order to establish their significance for the differentiation between transudates and exsudates. In 11-15% of the cases, the parameters protein and LDH led to a false classification of the effusions. The mean cholesterol concentration in malignant effusions was 94 mg/dl; in inflammatory effusions 76 mg/dl, and in transudates 30 mg/dl. By employing a threshold of 60 mg/dl to distinguish between transudates and exsudates, it proved possible to correctly classify 95% of the effusions. Lipoprotein studies using density gradient analysis revealed that the effusion cholesterol arises out of the serum, and is not a consequence of local cellular degeneration. Our results indicate that the determination of cholesterol levels is a simple and inexpensive method for distinguishing between transudate and exsudate.

Blood Proteins↗
PubMed1989

Mutation rate estimates are not compatible with autosomal dominant inheritance of the dysplastic nevus "syndrome".

Dysplastic nevi represent precursor lesions harboring an increased risk of evolving into melanoma. Their association with familial melanoma is usually considered a monogenic syndrome with autosomal dominant transmission. To test this concept we estimated the mutation rates. When derived directly from the sporadic occurrence of the trait, the mutation rate is exceedingly high (0.9%-2.5%), whereas, as estimated with the aid of Haldane's formula it would be 0.007% to 0.02%. Accordingly, newly arising mutation would outnumber eliminated mutations by 100:1. Even if only 80% of all old mutations are passed onto the next generation, this ratio of 100:1 would rapidly change. After only a few generations, 10% of the world population should be affected with the dysplastic nevus "syndrome". The apparent lack of a genetic equilibrium between newly arising and eliminated mutations is not compatible with autosomal dominant inheritance of the dysplastic nevus "syndrome."

Chromosome Aberrations↗
PubMed1989

Loose anagen hair of childhood: the phenomenon of easily pluckable hair.

We report two unrelated boys aged 4 and 9 years who had the recently delineated phenomenon of easily pluckable hair. Hair tufts could be pulled out without effort and without pain. Alopecia without any sign of scalp inflammation or scarring was noted. In the older child spontaneous remission occurred within 3 months, whereas the condition remained active in the younger boy for a follow-up period of 1 year. Trichograms from clinically involved and uninvolved areas disclosed a striking predominance of anagen hairs (98% to 100%) and complete absence of telogen hairs. A loss of the normal round or oval configuration of many hair shafts could be well visualized by the shrinking tube technique. Horizontal and transverse sections of scalp obtained for biopsy revealed marked cleft formations between hair shafts and regressively altered inner root sheaths. We suggest the name loose anagen hair of childhood for this newly described disease.

Alopecia↗
PubMed1989

Immunohistologic evidence for the malignant potential of congenital melanocytic nevi.

Monoclonal antibodies (MoAbs) against six well-defined progression-associated melanoma antigens (PAMAs) and a MoAb against the nuclear proliferation-associated antigen Ki67 were used for an immunoperoxidase study on 44 biopsies from 36 congenital melanocytic nevi (CMN). Twenty-nine common acquired nevi and 16 metastasizing primary melanomas were studied as controls. Two CMN of the series were giant CMN (greater than 20 cm), one of which later progressed to metastasizing melanoma. Of the remaining 34 CMN, four were histologically associated with a malignant melanoma. DNA flow cytometry was performed on adjacent cryostat sections of 37 biopsies from 28 CMN without melanoma. PAMAs were found expressed in CMN in the following frequencies: A-1-43: 36%; P358: 27%; PAL-M1: 20%; HLA-DR: 9%; Muc 18: 2%; A-10-33: 0%. No PAMAs were present in only 15 biopsies (34%). One single PAMA was found in 20 lesions (45%), and two or more PAMAs in nine lesions (21%). In all four CMN histologically associated with melanoma, and also in one biopsy from the giant CMN which later progressed to melanoma, at least two PAMAs were expressed. The latter biopsy was the only CMN in which the proliferation-associated antigen could be demonstrated. Only this biopsy and a single biopsy of the other giant CMN showed aneuploidy. We found expression of single PAMAs and co-expression of PAMAs in CMN in higher frequency than in common acquired melanocytic nevi. Co-expression of PAMAs was a feature of metastasizing primary melanomas. We conclude that co-expression of two or more PAMAs in a CMN might indicate its malignant potential, because this was found in all CMN proven to have progressed to malignant melanoma.

Antibodies, Monoclonal↗
PubMed1988

A monoclonal antibody to the alpha subunit of Gk blocks muscarinic activation of atrial K+ channels.

The activated heterotrimeric guanine nucleotide binding (G) protein Gk, at subpicomolar concentrations, mimics muscarinic stimulation of a specific atrial potassium current. Reconstitution studies have implicated the alpha and beta gamma subunits as mediators, but subunit coupling by the endogenous G protein has not been analyzed. To study this process, a monoclonal antibody (4A) that binds to alpha k but not to beta gamma was applied to the solution bathing an inside-out patch of atrial membrane; the antibody blocked carbachol-activated currents irreversibly. The state of the endogenous Gk determined its susceptibility to block by the antibody. When agonist was absent or when activation by muscarinic stimulation was interrupted by withdrawal of guanosine triphosphate (GTP) in the presence or absence of guanosine diphosphate (GDP), the effects of the antibody did not persist. Thus, monoclonal antibody 4A blocked muscarinic activation of potassium channels by binding to the activated G protein in its holomeric form or by binding to the dissociated alpha subunit.

Acetylcholine↗