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PubMed1980

Biosynthesis of lipoidal derivatives of pregnenolone and dehydroisoandrosterone by the adrenal.

The incubation of pregnenolone or dehydroisoandrosterone with bovine or rat adrenal homogenates leads to the formation of nonpolar metabolites of these steroids. The enzymatically prepared compounds have properties that are similar to the endogenous lipoidal derivatives of pregnenolone found in bovine adrenals (Hochberg, R. B., Bandy, L., Ponticorvo, L., and Lieberman, S. (1977) Proc. Natl. Acad. Sci. U. S. A. 74, 941-945) in that they are much less polar than the parent steroid and yield the parent steroid as a product of treatment with alkali. The lipoidal derivatives of both dehydroisoandrosterone and pregnenolone proved to be chromatographically heterogeneous. 17 alpha-Hydroxypregnenolone, 17 alpha-hydroxyprogesterone, progesterone, and testosterone were not converted into lipoidal derivatives when incubated with the bovine adrenal homogenate.

Adrenal Glands↗

PubMed1980

Exogenous lipoid pneumonitis.

The clinical and radiologic diagnosis of exogenous lipoid pneumonitis often goes unrecognized. A specific history of lipoid aspiration is rarely obtained unless specifically sought. We analysed the clinical, radiologic and pathologic findings in 11 patients with pathologically-proven exogenous lipoid pneumonitis. Lipid aspiration results in a predictable pathologic and consequently radiologic pattern of disease, often simulating bronchogenic carcinoma. Transthoracic percutaneous fine-needle aspiration biopsy is useful as a complementary procedure to bronchoscopy in making the diagnosis.

Adult↗

PubMed1993

Prenatal diagnosis of congenital lipoid adrenal hyperplasia.

BACKGROUND: There are no published reports of prenatal diagnosis of congenital lipoid adrenal hyperplasia, which is the rarest form of congenital adrenal hyperplasia. CASE: Congenital lipoid adrenal hyperplasia was diagnosed prenatally based on the existence of one affected sibling in the family, the presence of an amniotic fluid cell karyotype of 46,XY, the appearance of normal female genitalia on ultrasonography, relatively low amniotic fluid concentration of 17 alpha-hydroxyprogesterone, low maternal plasma and urinary concentrations of estriol, and a positive response to the dehydroepiandrosterone sulfate loading test. CONCLUSION: Congenital lipoid adrenal hyperplasia can be diagnosed prenatally. Treatment in early infancy can lead to normal mental and physical development.

Adrenal Hyperplasia, Congenital↗

PubMed1996

[Acute lipoid pneumonia. Report of 2 cases].

Lipoid pneumonia is a chronic infiltrative pulmonary process secondary to continued aspiration of exogenous lipids (laxatives and nasal drops). On occasions, an acute form of lipoid pneumonia which may lead to respiratory failure may be observed coinciding with accidental massive aspirations of lipidic material. Two cases of acute lipoid pneumonia secondary to paraffin aspiration in << fire eaters >> are presented.

Accidents, Occupational↗

PubMed1993

Inner ear damage due to lipoid nephrosis.

Inner ear pathology was studied in adult rats with lipoid nephrosis induced by puromycin aminonucleoside. Although no abnormality was observed in auditory brain-stem responses, significant changes were noted in the stria vascularis. The most striking observation was that intermediate cells were markedly swelled, there-by pressing adjacent marginal cells. Severely affected marginal cells have vacuoles and increased lysosomes and protruded toward the endolymphatic space. The organ of Corti remained virtually intact. Although the vestibular maculae were relatively normal, type I hair cells in the semicircular canal underwent a conspicuous vaculolization. These findings support a postulate that the inner ear is liable to damage in lipoid nephrosis.

Animals↗

PubMed1992

Lipoid proteinosis.

Lipoid proteinosis (Urbach-Wiethe disease) is a rare, recessively inherited disorder that is characterized by the deposition of hyaline-like material in the skin, oral cavity, and other tissues. It usually appears in infancy with hoarseness. We report a case of lipoid proteinosis in a 10-year-old boy that demonstrates the characteristic clinical, histologic, and ultramicroscopic features of this disease.

Child↗

PubMedDate not supplied

Selected disorders of connective tissue: pseudoxanthoma elasticum, cutis laxa, and lipoid proteinosis.

There has been progress made in the understanding of 3 Mendelian disorders: pseudoxanthoma elasticum, cutis laxa, and lipoid proteinosis cutis and mucosae. While they are primary connective tissue diseases, their names imply a connection to the skin, and in fact, it is often the dermatologist who makes the diagnosis. It seems rational that defects in various extracellular matrix proteins cause lipoid proteinosis or subtypes of cutis laxa, yet the discovery of a liver- and kidney-based transmembrane transporter as the culprit of pseudoxanthoma elasticum was rather surprising and may shed new light on elastic tissue homeostasis.

Adolescent↗

PubMed2004

Lipoid proteinosis of larynx: review of four cases.

Lipoid proteinosis is a rare autosomal recessive disorder characterized by intercellular deposition of an amorphous hyaline material. It mainly involves skin and mucosal membranes of upper aerodigestive tract as well as central nervous system, lung, lymph nodes and striated muscles. Etiology and pathogenesis are unknown. Infantile hoarseness is a common presenting feature of the disease due to infiltration of larynx. In two-thirds of the cases, voice changes are present at birth or in early infancy as the first manifestation. We present four patients with lipoid proteinosis involving skin, oropharynx and larynx.

Adolescent↗

PubMed2005

[Lipoid proteinosis].

Lipoid proteinosis is an infrequent disease characterized by the deposition of a PAS-positive diastase-resistant hyaline material in the skin and respiratory tract, although it can also be deposited in internal organs, in a generally asymptomatic manner. The earliest clinical manifestation is hoarseness. Clinical cutaneous manifestations come later, in the form of hyperkeratotic lesions located on the trunk, elbows, axillae, groins, backs of hands, palms and soles. A lesion typical of the disease is moniliform blepharosis, which consists of beaded papules along the eyelid margins. Also characteristic is the presence of comma-shaped intracranial calcifications in the temporal lobes. The course of the disease is progressive, with a normal life expectancy. It affects men and women equally, with worldwide distribution. The diagnosis is based on the clinical symptoms and the histology. At this time, there is no effective treatment for the disease. We present a case of lipoid proteinosis in a 23-year-old woman, with typical clinical and histological characteristics.

Adult↗

PubMedDate not supplied

Lipoid proteinosis: report of four siblings and brief review of the literature.

Lipoid proteinosis (Urbach-Wiethe disease) is a rare autosomal recessive disorder associated with deposition of periodic acid-Schiff (PAS)-positive hyaline material in various tissues including skin, mucous membranes, and internal organs. A family is reported in which four siblings (two boys and two girls) born to nonconsanguineous parents had lipoid proteinosis. All had the characteristic hoarseness of voice and three had skin lesions. The diagnosis was confirmed by the presence of typical features on light and electron microscopy.

Child↗

PubMed1981

Lipoid proteinosis: in vivo and in vitro evidence for a lysosomal storage disease.

Tissue and cultured fibroblasts derived from one patient with the classical findings of lipoid proteinosis have been used to examine pathologic mechanisms in the disease. Ultrastructural examination of the skin revealed not only extracellular deposits of finely granular, moderately electron dense material, but in addition the dermal fibroblasts characteristically demonstrated marked cytoplasmic vacuolization. Phase contrast microscopy of the cultured skin fibroblasts also showed strikingly abnormal cells with many inclusions, which by electron microscopy were delimited by a single membrane. Membranous lamellar material was also increased in these cells. Biochemical analysis of the fibroblasts revealed a 3- to 4-fold elevation in intracellular hexuronic acid. These morphologic and biochemical findings suggest certain similarities with known storage diseases and support the postulate that lipoid proteinosis may represent a lysosomal storage disease.

Cells, Cultured↗

PubMed1985

Lipoid proteinosis: an inherited disorder of collagen metabolism?

The dermal collagen of a patient with lipoid proteinosis was investigated by immunohistochemistry and biochemical analysis. The affected skin was found to contain significantly less collagen per unit dry weight than normal dermis but showed elevated levels of type 3 collagen with respect to type I. Purification of collagen types from affected skin after pepsin digestion showed no novel forms, but a doubling in the yield of type 5 collagen. These results correlated well with those of immunohistochemistry which showed a patchy, diffuse, widely distributed type 3 collagen and an increase in types 4 and 5 collagens associated with 'onion skin' endothelial basement membrane thickening. Estimation of collagen cross-links showed an abnormal pattern with a preponderance of the keto-imine form not normally associated with skin. These results strongly suggest that lipoid proteinosis involves a primary perturbation of collagen metabolism.

Adolescent↗

PubMed1985

Lipoid proteinosis.

Lipoid proteinosis is a rare autosomal recessive disorder that can affect the majority of organ systems, but most frequently presents due to its characteristic skin and mucous membrane changes. This was illustrated in a 27-year-old patient. Because its manifestations are easily misinterpreted, one might reasonably assume that the incidence of lipoid proteinosis may be higher than previously reported.

Adult↗

PubMed1984

Is house dust allergen a possible causal factor for relapses in lipoid nephrosis?

In three cases of minimal change nephrotic syndrome (lipoid nephrosis), an allergen sensitivity to house dust, cat hair and grass pollen was demonstrated by clinical and biological data. An increase in proteinuria after specific allergen contact and a decrease after withdrawal of the allergens, suggested that the cause was allergenic. Such cases demonstrate the necessity of an etiological investigation of particularly the atopic features in lipoid nephrosis. Results of these investigations can lead to specific therapy in selected cases.

Adolescent↗

PubMed1989

Corectopia and lipoid proteinosis.

Lipoid proteinosis (Urbach-Wiethe disease) is a rare autosomal recessive disorder associated with deposition of hyalinised material in the skin, mucous membrane, and brain. Corectopia has not been described in this disorder. A case is presented of lipoid proteinosis with bilateral corectopia.

Adult↗

PubMed1994

Case report: computed tomography findings in lipoid proteinosis: report of two cases.

Lipoid proteinosis (Urbach-Wiethe disease (Urbach, E and Wiethe, C, Lipoidosis cutis mucosae, Virchows Arch. Patholog. Anat., 273, 285-319 (1929)) is a rare generalized disease with autosomal recessive inheritance. It most often involves the skin and mucosal membranes of the aerodigestive tract; but also involves the central nervous system, lung, lymph nodes and striated muscles. We present the computed tomography findings in the cranium and larynx of two siblings with lipoid proteinosis.

Adult↗

PubMed1992

Gingival lesions in lipoid proteinosis.

Lipoid proteinosis is a rare heritable disease of the skin and mucous membranes characterized by subepithelial deposits of hyaline material. The morbid factors include disfiguring papulo-nodular lesions particularly of exposed skin, hoarseness of voice due to vocal cord infiltration, nodular deformation of the eyelids, and board-like rigidity of the tongue. Infiltration of the gingiva is usually unreported, and when mentioned has been equivocally related to the overall disease process. This patient demonstrates striking ulceration and hypertrophy with histologic evidence of heavy deposition of amorphous material confirming a gingival component to the lipoid proteinosis entity.

Adolescent↗

PubMed1999

Oral lipoid proteinosis.

This literature review of oral lipoid proteinosis and a case report of an infant with this condition describe an uncommon condition inherited as an autosomal recessive trait, but one which is more likely to occur in this country than elsewhere. The article discusses other lesions which could be mistaken for oral lipoid proteinosis, and offers some pertinent aspects of its diagnosis and management.

Child, Preschool↗