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Biomedical subjects

M Lebwohl

Publications and source records attributed to M Lebwohl.

139 records · Page 8Linked to original sources
PubMed1987

Kaposi's sarcoma and acquired immunodeficiency syndrome. Postmortem findings in twenty-four cases.

Autopsy results on twenty-four patients with acquired immunodeficiency syndrome (AIDS) and Kaposi's sarcoma were reviewed. At postmortem, 29% of patients had evidence of visceral Kaposi's sarcoma without skin lesions. The most common sites for visceral involvement with Kaposi's sarcoma were as follows: lung (37%), gastrointestinal tract (50%), and lymph nodes (50%). At the time of death, only 25% of patients had evidence of cutaneous disease alone. The patients survived up to 36 months after the diagnosis of AIDS was made according to the specific diagnostic criteria established by the Centers for Disease Control. Many of the patients had severe, disseminated infections during the course of their AIDS illness. The most common infections diagnosed during the patients' clinical courses and/or at autopsy were cytomegalovirus (75%), candidiasis (50%), Mycobacterium avium intracellulare (50%), Pneumocystis carinii pneumonia (50%), bacterial pneumonia (33%), and herpes simplex virus (29%). Nearly 80% of the deaths were attributed to infection. In only one case was overwhelming Kaposi's sarcoma determined to be the cause of death.

Acquired Immunodeficiency Syndrome↗
PubMed1987

Subcutaneous fat necrosis of the newborn with hypercalcemia.

A newborn infant with subcutaneous fat necrosis and hypercalcemia is presented. Literature on the association between subcutaneous fat necrosis of the newborn and hypercalcemia is reviewed. Affected infants generally experience severe perinatal difficulties. Both vaginal deliveries and cesarean sections can be associated with subcutaneous fat necrosis.

Adipose Tissue↗
PubMed1987

Multiple hamartoma syndrome.

Multiple hamartoma syndrome, also known as Cowden's disease, is a rare genodermatosis with multiple organ system involvement affecting tissues derived from ectodermal, endodermal, and mesodermal tissue layers. We describe two previously unreported cases of multiple hamartoma syndrome in a father and daughter. Both show classic features of multiple hamartoma syndrome, as well as other mucocutaneous findings. The father has been shown to have substantial cutaneous deposits of amyloid in the absence of underlying plasma cell dyscrasia or malignancy. Both individuals have undergone excision of a unique fibroma that has features that have been reported only in multiple hamartoma syndrome and should be added to the criteria used to define the entity.

Adolescent↗
PubMed1986

Association between acrochordons and colonic polyps.

Fifty-four male and female patients with suspected colonic disease were examined for the presence of acrochordons prior to colonoscopy. Thirty-five of these patients (65%) had skin tags and twenty-six (48%) were found to have colonic polyps. In this study patients with acrochordons were significantly (p less than 0.005) more likely to have adenomatous polyps; thus, skin tags may be good markers for the presence of polyps in patients with suspected colonic disease. However, any association between colonic polyps and acrochordons in the general population requires further examination.

Adult↗
PubMed1986

Nerve growth factor receptors on dissociated neurofibroma Schwann-like cells.

Neurofibromatosis is a disorder which predominantly involves cellular elements of peripheral neural sheaths. Little is known about the regulation of differentiation and proliferation of cells comprising neurofibromas. Because nerve growth factor-like activity may be present in neurofibromas and the cells comprising neurofibromas are neural crest derivatives, we have investigated whether nerve growth factor (NGF) receptors are present on cells from dissociated dermal neurofibromas. Using 125I-NGF to measure binding to cultured cells in suspension and for autoradiography, we identified a population of cells having characteristics of Schwann cells which exhibited saturable 125I-NGF binding. This binding is characteristic of the "fast" (low affinity) NGF receptor, having a Kd of approximately 1 nM and a Bmax of at least 120 fmol/10(6) cells. Less than 20% of the bound 125I-NGF (5 ng/ml) is not displaced when transferred to 0 degrees C by an excess of unlabeled NGF (10 micrograms/ml) and is therefore bound to either "slow" (high affinity) sites or is rapidly internalized. NGF receptors with characteristics of fast sites have recently been reported on Schwann-like cells from chick dorsal root ganglia [Zimmerman, A., and Sutter, A. Beta nerve growth factor (beta NGF) receptors on glial cells. Cell-cell interaction between neurones and Schwann cells in culture of chick sensory ganglia. EMBO J., 2: 879-885, 1983]. The identification of NGF receptors on both fetal chick dorsal root ganglia and neurofibroma Schwann-like cells suggests that NGF may have a role in the regulation of Schwann cell function in both normal development and in neurofibromatosis.

Cells, Cultured↗
PubMed1985

Addition of short-contact anthralin therapy to an ultraviolet B phototherapy regimen: assessment of efficacy.

The purpose of this study was to determine whether the addition of short-contact anthralin therapy to an ultraviolet B phototherapy regimen would result in more rapid resolution of psoriatic plaques. A bilateral paired comparison between an ultraviolet B phototherapy regimen with and without short-contact anthralin was completed in eleven patients. Only two of the patients responded faster on the anthralin-treated side. Our results do not support the routine addition of short-contact anthralin therapy to ultraviolet B phototherapy regimens, but this combination may be more effective in a minority of patients.

Anthracenes↗
PubMed1985

Basement membrane proteins, interstitial collagens, and fibronectin in neurofibroma.

The distribution and nature of extracellular matrix proteins in neurofibroma tissue was studied by indirect immunofluorescence, immunoelectron microscopy, immunoblotting, and rotary shadowing. The most striking feature was an extensive network of basement membranes localized mainly around Schwann cells and small blood vessels. The major components, collagen IV, laminin, and nidogen, were mainly deposited in the lamina densa. Some laminin and nidogen could be extracted with 0.5 M NaCl and were shown by electrophoresis to have the characteristic chain and fragment patterns described previously for these proteins isolated from the mouse Engelbreth-Holm-Swarm (EHS) sarcoma. Fragments of collagen IV and collagen VI were solubilized by limited proteolytic digestion and identified after rotary shadowing. The more remote interstitial regions of the tumor contained cross-striated collagen fibrils which were composed of collagen III (diameter, 20-30 nm) or collagen I (diameter, 40-50 nm). Collagen fibrils thicker than 80 nm were not found. The interstitial regions also contained collagen VI as a fine filamentous network near cells and between collagen fibrils. Deposits of fibronectin were rather small and showed a scattered distribution. The data indicate that Schwann cells contribute considerably to matrix production in neurofibroma which may therefore be a suitable model for studying basement membranes of neuroectodermal origin.

Basement Membrane↗
PubMed1984

Metastatic Crohn's disease.

Cutaneous granuloma formation distant from the gastrointestinal tract in patients with Crohn's disease of the bowel has been called metastatic Crohn's disease. We report two patients with this entity, including the first to present with an erysipelas-like eruption of the face. A review of the world's literature reveals that all patients with metastatic Crohn's disease have had gastrointestinal disease involving the colon or rectum. Clinical features of the nine previously reported cases are reviewed.

Adult↗
PubMedDate not supplied

Elastin gene deletions in Williams syndrome patients result in altered deposition of elastic fibers in skin and a subclinical dermal phenotype.

Williams syndrome (WS) is a complex developmental disorder with multisystem involvement known to be the result of a microdeletion in the q11.23 region of chromosome 7. This deletion involves several genes, including the elastin gene. Although elastic fibers are important constituents of skin, little is known about the skin phenotype in WS patients. We have therefore studied the skin of four WS patients in which we've shown the deletion of one copy of the elastin gene. Physical examination and indirect immunofluorescent microscopy of elastin did not detect any major phenotypic or morphologic changes in the skin. We were able, however, to show subtle textural changes in skin and, by electron microscopy, that the amorphous component of elastic fibers in WS patients was consistently reduced when compared to normal controls. These findings indicate that deletion of one copy of the elastin gene results in reduced deposition of elastin in dermal elastic fibers, an altered elastic fiber ultrastructure, and a subclinical dermal phenotype in the children and young adult patients analyzed in this study.

Adult↗
PubMedDate not supplied

Calcification of elastic fibers in pseudoxanthoma elasticum.

Pseudoxanthoma elasticum is an inherited disorder of connective tissue that is characterized by calcification of elastic fibers with associated abnormalities of the skin, ocular, and cardiovascular systems. The genetic defect causing pseudoxanthoma elasticum has not been determined and the diagnosis relies on clinical recognition of skin lesions and histologic demonstration of calcification of elastic fibers that are clumped and fragmented in the dermis. The role of connective tissue components in the etiology of pseudoxanthoma elasticum is reviewed and includes changes in collagen, elastin, glycosaminoglycans, fibronectin, microfibrillar proteins, modifying enzymes of extracellular matrix proteins, fibroblasts, and fibrillins.

Elastin↗