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At least 253 records · Page 14Linked to original sources
PubMed2001

Molecular and structural analysis of two novel StAR mutations in patients with lipoid congenital adrenal hyperplasia.

Mutations in the gene encoding steroidogenic acute regulatory protein (StAR) cause lipoid congenital adrenal hyperplasia. We report a novel homozygous splice site mutation (IVS1 + 2T --> G) in STAR in two sisters (46XY, 46XX) who presented with primary adrenal insufficiency at birth and a novel homozygous R182H missense mutation in the putative lipid transfer domain of StAR in a phenotypic female (46XY) with adrenal failure and a parotid tumor. These cases highlight the importance of StAR-dependent steroidogenesis during fetal development and early infancy and of the critical functional role of R182 in cholesterol transport.

Adrenal Hyperplasia, Congenital↗
PubMed1991

Conjunctival xerosis, arcus lipoides and Rieger's disease.

Three generations of a family with conjunctival xerosis, Rieger's anomaly (complete or incomplete) and arcus lipoides are described. The xerosis was found always to be accompanied by a Rieger anomaly, which had sometimes, but not always, been detected before. Independent heredity was not observed. In a number of cases the xerosis was bilateral and localised both temporally and nasally. The combination appears to be a stigma malformationis oculi. Correlation with a temporary vitamin A deficiency in early youth appears possible. At this age in such families caution is advisable in the administration of vitamin A inhibiting medicines such as steroids and neomycine.

Abnormalities, Multiple↗
PubMed1995

Hypertrophic osteoarthropathy caused by lipoid pneumonia.

A case of hypertrophic osteoarthropathy in a 5-year-old Saudi boy is presented. The child developed painful joints, was found to have clubbing of fingers and radiologically hypertrophic osteoarthropathy was demonstrated. The underlying cause of the condition was ingestion of animal fat in infancy, with a history of ghee oil aspiration which caused a chronic lung inflammation, lipoid pneumonia, proven by lung biopsy.

Child, Preschool↗
PubMed1988

Computed tomography in the early detection of congenital lipoid adrenal hyperplasia.

This paper describes the successful use of abdominal computed tomography (CT) scan in diagnosing congenital lipoid adrenal hyperplasia in a Japanese male with no virilization at 9 days of age. The CT unequivocally delineated massively enlarged adrenal glands of fat-tissue attenuation, enabling early replacement therapy.

Adrenal Hyperplasia, Congenital↗
PubMed1985

Steroid secretion by a lipoid cell tumor causing virilization and its diagnosis with computerized tomography.

Presented is the case of a virilized woman with a lipoid cell tumor of the ovary localized by computerized tomography. The major secretory products of the tumor were testosterone and estradiol; the production of androgens was responsible for the clinical features and hindered the effects of estrogens. Elevated levels of estradiol indicated important ovarian aromatase activity as reflected by large ovarian-peripheral venous gradients.

Adult↗
PubMed1981

Lipoidal degeneration of the retinal pigment epithelium.

Vacuolation of the retinal pigment epithelium in the foveomacular region of aging rhesus monkey eyes was re-examined with the eyes initially fixed in osmium tetroxide to retain the alcohol-soluble lipid. All vacuoles were found to be completely occupied by lipid material. Similar cells were positive to oil red O. The vacuolation of the pigment epithelial cell is considered to be a form of lipoidal degeneration.

Animals↗
PubMed1977

Cholesterol side-chain cleavage enzyme activity and cytochrome P-450 content in adrenal mitochondria of a patient with congenital lipoid adrenal hyperplasia (Prader disease).

An autopsied case with congenital lipoid adrenal hyperplasia (Prader disease) was presented. The cholesterol side-chain cleavage (SCC) enzyme activity in adrenal mitochondria of this case was assayed with [3H]cholesterol as substrate, combined with purified bovine adrenodoxin and adrenodoxin reductase, by measuring the amount of [3H]pregnenolone formed. The cytochrome P-450 content was also measured by recording the difference absorption spectra of carbon monoxide-complexed P-450. The cholesterol SCC enzyme activity in adrenal mitochondria of Prader disease was 0.81 nmol pregnenolone/nmol P-450 per min, which was approximately 10% of that in normal tissue. The content of cytochrome P-450 was 0.074 nmol/mg protein, which was about half of that in controls. These results indicate that there is a cholesterol SCC enzyme deficiency in adrenal mitochondria in this disease.

Adrenal Glands↗
PubMed1986

Changes in the metabolic pattern of estrogens as a function of age in cultured myometrial cells: synthesis of a lipoidal derivative of estradiol.

The effect of ageing on estradiol (E2) metabolism has been studied systematically in cultured ovine myometrial cells from the 2nd to the 25th subculture. Cell monolayers were incubated for various amounts of time with [3H]E2, and metabolites isolated from cells or medium by thin-layer chromatography (TLC). The main metabolites identified were estrone (E1), estriol (E3), 16-epi-E3 and a lipoidal derivative of E2 (LE2). The latter had an Rf of 0.90 and was recognized by its comigration with fatty acid on TLC and the release of E2 after alkaline hydrolysis. In contrast to the other metabolites, LE2 was found only in cells and was never secreted in the medium. In "young" cells (2nd subculture) the main metabolite was E1 which represented 16.3% of the total radioactivity after 2 h of incubation and 33% after 8 h both in cells and medium. LE2 appeared very slowly and represented only 13% after 8 h of incubation. In contrast in "old" cells (i.e. 10th subculture) LE2 had become the most abundant metabolite representing as much as 25% of the total cellular radioactivity. This change from one metabolic pattern to the other was progressive and associated with a decrease in 17 beta-hydroxysteroid dehydrogenase (SDH) activity. LE2 became prevalent relative to E1 around the 5th subculture. In conclusion, ageing in cultured myometrial cells is accompanied by a qualitative change in E2 metabolism, switching from E1 formation (an inactivation mechanism) to LE2 biosynthesis (a storage mechanism).

Animals↗
PubMed1989

Hans Horst Meyer and the lipoid theory of narcosis.

This year marks the 90th anniversary of the publication of Hans Horst Meyer's classic paper in which he proposed that the ability of a substance to produce narcosis or anesthesia is governed by its partition coefficient. In this article, Robert Lipnick describes the experiments carried out by Meyer and his colleagues which disproved the earlier theories that potency was determined by the presence of particular functional groups or their metabolites or by water or fat solubility, and which led to the formulation of the lipoid theory of narcosis.

Anesthesia↗
PubMed1987

Lack of therapeutic activity of the lipoidal amine CP-46,665 in rodent tumors and human non-seminomatous germ cell tumors growing in nude mice.

The alkyl-linked lipoidal amine 4-aminomethyl-1-[2,3-(di-n-decyloxy)-n-propyl]-4-phenylpiperidine (CP-46,665) was tested for therapeutic activity in 2 rodent tumor models and 2 human non-seminomatous germ cell tumors growing in nude mice. CP-46,665 failed to show therapeutic efficacy in 3-Lewis lung carcinoma (3-LL) growing in syngeneic C57Bl6-mice, in methylnitrosourea (MNU)-induced rat mammary carcinomas and in 2 human non-seminomatous germ cell tumor cell lines (H 12.1, H 12.7) growing in nu/nu NMRI-mice when given in a dose range including non-toxic doses and doses higher than the lethal dose for 10% of the treated animals (LD10).

Animals↗
PubMed1993

Chest radiographic findings in childhood lipoid pneumonia following aspiration of animal fat.

The pulmonary radiological manifestations of lipoid pneumonia following a cultural practice of forced feeding of rendered animal fat (ghee) in 24 Saudi children are described. The age range is 15 days to 11 years with 68% of cases being in infancy. The chest radiographic appearances encountered could be grouped into four broad patterns, viz, bilateral multilobar consolidations (BMLC) in 50% (12 cases), bilateral perihilar infiltrates (BPHI) with or without associated lobar consolidation in 21% (five cases), right perihilar infiltrates (RPHI) in 21% (five cases) and unilateral right multilobar consolidation (RMLC) 8% (two cases). The pneumoniae were non-resolving acute or chronic lung consolidations despite the usual antimicrobial chemotherapy. Associated nodular dense opacities (granulomas) were seen in the lower lobes of four of the 12 cases in the first group. Complications, seen also in this first group, included chronic collapse consolidation, fibrosis and death in three cases. In communities where the traditional practice of infant feeding with ghee exists, these pulmonary radiological patterns, although in some cases indistinguishable from those of bacterial pneumoniae, should raise the suspicion of ghee administration with consequent early diagnosis and energetic management to avert prolonged morbidity and potential death.

Child↗
PubMed2001

Studies on the effect of pH on the lipoidal route of penetration across stratum corneum.

The effect of pH on the excised skin permeability towards two model lipophilic compounds was studied in the range of pH 1.0-10.0 and 1.0-12.0, for hydrocortisone and testosterone, respectively. Suspensions of the model compounds in appropriate buffers (0.1-0.2 M) were applied to the intact skin, delipidized skin, skin pretreated with Azone or sodium lauryl sulfate solution and the percutaneous penetration was observed for 28 h. Within the studied pH range, up to pH 11.0 no change in the penetration through the skin and skin accumulation was observed for both compounds. Significant, 3-4-fold, increase of penetration of testosterone was noted only at pH 12.0, however the change was neither fast not progressing with time. None of the pretreatment procedure influenced the observed relationship between pH and skin permeability. It is concluded that the lipoidal route of penetration is not affected by solutions at pH within the range 1.0-11.0.

Anti-Inflammatory Agents↗
PubMed2003

[Lipoid pneumonia related to workplace exposure to paint].

A 49-year-old man with no known history of pulmonary disease was treated at our hospital after observation of an interstitial pattern on a chest film. The patient was a smoker and professional painter. Computed tomography of the chest showed a diffuse bilateral ground-glass pattern. The lung biopsy showed intra-alveolar lipid accumulation in the form of vacuoles of varying sizes surrounded by numerous focally multinucleated macrophages, establishing a definitive diagnosis of exogenous lipoid pneumonia. Given the patient's profession, he was recommended to avoid workplace exposure to paraffins and oily sprays. The clinical course was favorable after exposure was stopped, with improved lung function and symptoms.

Follow-Up Studies↗
PubMed1993

The human peripheral benzodiazepine receptor gene: cloning and characterization of alternative splicing in normal tissues and in a patient with congenital lipoid adrenal hyperplasia.

The mitochondrial benzodiazepine receptor (mBzR) appears to be a key factor in the flow of cholesterol into mitochondria to permit the initiation of steroid hormone synthesis. The mBzR consists of three components; the 18-kDa component on the outer mitochondrial membrane appears to contain the benzodiazepine binding site, and is hence often termed the peripheral benzodiazepine receptor (PBR). Using a cloned human PBR cDNA as probe, we have cloned the human PBR gene. The 13-kb gene is divided into four exons, with exon 1 encoding only a short 5' untranslated segment. The 5' flanking DNA lacks TATA and CAAT boxes but contains a cluster of SP-1 binding sites, typical of "house-keeping" genes. The encoded PBR mRNA is alternately spliced into two forms: "authentic" PBR mRNA retains all four exons, while a short form termed PBR-S lacks exon 2. While PBR-S contains a 102-codon open reading frame with a typical initiator sequence, the reading frame differs from that of PBR, so that the encoded protein is unrelated to PBR. RT-PCR and RNase protection experiments confirm that both PBR and PBR-S are expressed in all tissues examined and that expression PBR-S is about 10 times the level of PBR. Expression of PBR cDNA in pCMV5 vectors transfected into COS-1 cells resulted in increased binding of [3H]PK11195, but expression of PBR-S did not. It has been speculated that patients with congenital lipoid adrenal hyperplasia, who cannot make any steroids, might have a genetic lesion in mBzR. RT-PCR analysis of testicular RNA from such a patient, sequencing of the cDNA, and blotting analysis of genomic DNA all indicate that the gene and mRNA for the PBR component of mBzR are normal in this disease.

Adrenal Hyperplasia, Congenital↗
PubMed2003

Fatal lipoid pneumonia due to bronco-aspiration of isoparaffin after ingestion of an organophosphate insecticide.

A 66-year-old-male patient with a history of depression voluntarily ingested around 400 ml of an insecticide composed of 5% methylparathion, 75% isoparaffin, 8% etoxylated oleic acid, 4% 1,2,4-trimethylbenzene, 6% naphtha, 1% 1,3,5- trimethylbenzene, 0.4% propylbenzene and 0.3% xylene. The patient was conscious and alert at admission. Gastric lavage was performed and activated charcoal administered. There were no clinical symptoms of organophospate ingestion despite reduced concentrations of erythrocyte and plasma cholinesterase. Chest X-ray showed pulmonary infiltrate compatible with bronco-aspiration. The patient evolved to respiratory failure refractory to treatment and died from multiorganic failure 23 days after ingesting the insecticide. The pathological findings included a pulmonary fibrosis in the alveolar spaces which caused enlargement of the intra-alveolar septa. Abundant lipin-laden macrophages were observed within the alveolar spaces. We review the most relevant aspects of cases of fatal lipoid pneumonia and point out that on occasion severe or fatal intoxication is due to the substances accompanying the active ingredients.

Aged↗
PubMed1985

Adrenal insufficiency possibly due to lipoid adrenal hyperplasia: a case report.

An infant who presented with adrenal insufficiency possibly due to lipoid adrenal hyperplasia at the age of 66 days is described. This is the first time that this very rare disorder is reported from Saudi Arabia. Adrenocorticotrophic hormone (ACTH) stimulation test is useful in the ante-mortem diagnosis of this condition.

Adrenal Hyperplasia, Congenital↗