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PubMed1970

Intraglomerular fibrin, platelet aggregation, and subendothelial deposits in lipoid nephrosis.

We have investigated the formation of fibrin, platelet aggregates, and subendothelial deposits in lipoid nephrosis. Fibrin formation was found in 10 cases of active lipoid nephrosis. Platelet aggregates were found in eight cases and subendothelial deposits in nine. Fibrin and platelets were also found in cases of nephrotic syndrome due to other causes, and in glomerulonephritis. Fibrin was generally absent in lipoid nephrosis in remission and in benign recurrent hematuria. It is suggested that what seems to be a lower incidence in females is more apparent than real and that fibrin or related material may be present in a less easily identifiable form. Steroid therapy apparently had no effect on the presence or absence of fibrin. Most instances were associated with elevated serum cholesterol and alpha(2)-globulin. It is suggested that elevated serum lipids as well as the disease process in the kidney play a role in this phenomenon. It is further suggested that intraglomerular fibrin formation could lead to irreversible renal damage in lipoid nephrosis.

Adolescent↗
PubMedDate not supplied

Clinical and molecular abnormalities in lipoid proteinosis.

Lipoid proteinosis (hyalinosis cutis et mucosae) is a rare, autosomal recessive disease. The main clinicopathological features comprise skin and mucous membrane infiltration and scarring with deposition of hyaline material. In this report, we describe a 6-year-old boy in whom a diagnosis of lipoid proteinosis was first suspected when he presented with blisters and erosions at 4 years, a history of life-long dysphonia and a previous epileptic convulsion. The diagnosis was confirmed by histology and identification of a homozygous frameshift mutation, 501insC, in exon 6 of the gene encoding extracellular matrix protein 1, ECM1. Lipoid proteinosis may show protean clinical features and be difficult to diagnose on clinical grounds alone. This case report illustrates that lipoid proteinosis may show protean clinical features and yet remain undiagnosed for many years. Although the gold standard for definite diagnosis remains histology, molecular characterisation of the gene mutation will add to our understanding of genotype-phenotype correlation and perhaps to the development of a rationale for future therapeutics.

Child↗
PubMed2006

Lipoid proteinosis: case report and review of the literature.

Lipoid proteinosis is a rare autosomal recessive disorder, characterized histologically by infiltration of periodic acid Schiff-positive hyaline material into the skin, upper aerodigestive tract, and internal organs. Classical clinical features include skin scarring, beaded eyelid papules, and laryngeal infiltration leading to hoarseness. Moreover, the infiltrates in the tongue and its frenulum limit lingual movements and cause speech difficulties. Usually, the hoarse voice is present at birth or in early infancy, as the first manifestation. In more severe cases, diffuse infiltration of the pharynx and larynx might cause respiratory distress, at times requiring tracheostomy. The disorder has recently been shown to result from loss-of-function mutations in the extracellular matrix protein 1 gene on chromosome 1q21. The function of the protein extracellular matrix protein 1 gene is still unclear, although an important role in skin physiology and homeostasis has been hypothesized. In this report, the case is described of a 6-year-old girl with lipoid proteinosis. Histopathological examination of a laryngeal biopsy specimen showed massive deposits of eosinophilic, periodic acid Schiff-positive, and diastase resistant material in the lamina propria corroborating the clinical diagnosis of lipoid proteinosis. Molecular analyses in this patient also confirmed the clinical diagnosis. The proposita was a compound heterozygote for a new small rearrangement (543de1TG/ins15) in exon 6, and a nonsense mutation (Arg243Stop) in exon 7. Together with previously documented mutations in the extracellular matrix protein 1 gene, this study supports the hypothesis that exons 6 and 7 are the most common sites for extracellular matrix protein 1 gene mutations in lipoid proteinosis.

Child↗
PubMed2005

Lipoid pneumonia due to Mexican folk remedies: cultural barriers to diagnosis.

OBJECTIVE: To describe 2 cases of lipoid pneumonia in Mexican American infants after administration of vegetable- or animal-derived oils and the cultural barriers to diagnosis. Various folk remedies have been documented in the international medical literature that involve the oral or nasal administration of vegetable- or animal-derived oils to children for the treatment of common ailments, including nasal stuffiness, constipation, and colic. Lipoid pneumonia is a known complication of such practices in Mexico, India, Saudi Arabia, and other countries. METHODS: Case reports of 2 Mexican American infants with respiratory distress and interviews with 30 immigrant families of Mexican origin. RESULTS: In both cases, language and cultural barriers resulted in a delayed diagnosis of lipoid pneumonia. Interviews with immigrant families confirmed that oil administration to children is a common traditional therapy in Mexican cultures. CONCLUSIONS: These findings underscore the need for primary care providers to be aware of the traditional practice of oil administration to infants in many cultures, its pathophysiological consequences, the potential cultural barriers to timely diagnosis, and the opportunity to prevent cases of lipoid pneumonia through anticipatory guidance.

Bronchoscopy↗
PubMed1999

Exogenous lipoid pneumonia: high-resolution CT findings.

The aim of this study was to assess high-resolution computed tomography (HRCT) findings of exogenous lipoid pneumonia. High-resolution computed tomography was obtained in 25 patients with proven exogenous lipoid pneumonia resulting from aspiration of squalene (derived from shark liver oil). Diagnosis was based on biopsy (n = 9), bronchoalveolar lavage (n = 8), or sputum cytology and clinical findings (n = 8). The clinical history of taking squalene was confirmed in all patients. The CT findings were classified into three patterns: diffuse ground-glass opacity, consolidation, and interstitial abnormalities. Distribution of the abnormalities, duration of taking squalene, predisposing factors for aspiration, and route of administration were analyzed. Ten patients showed diffuse ground-glass opacity pattern. Seven of 10 patients had predisposing conditions such as unconsciousness, pharyngeal dysmotility, or motor disturbances, and 6 patients had a recent history of taking large amount of squalene through nasal route. Seven patients who had consolidation pattern had a history of taking squalene for several months and did not have any predisposing factor. All of the 5 patients who had a pattern of interstitial abnormalities had a history of taking squalene longer than 1 year and showed segmental distribution of interstitial thickening with interposing ground-glass opacities. Three patients simultaneously had two different patterns at different lobes of the lung. The HRCT findings of lipoid pneumonia are ground-glass opacities, consolidation, and interstitial abnormalities. These HRCT findings with appropriate inquiries could be useful for diagnosis of exogenous lipoid pneumonia.

Adult↗
PubMed1999

Exogenous lipoid pneumonia: HRCT, MR, and pathologic findings.

The objective of this study was to describe high-resolution CT (HRCT) and MR findings of exogenous lipoid pneumonia and to correlate them with pathologic findings. A retrospective review of the medical records of our institution revealed seven patients with a diagnosis of lipoid pneumonia based on clinical data, chest films, bronchoalveolar lavage, and follow-up. Both HRCT and MR imaging were reviewed by two readers. Pathologic examination of the resected specimen or surgical biopsies were also reviewed in the four available cases. The HRCT findings were pulmonary consolidations (n = 6) with fatty (n = 3) or unspecific but low attenuation values (n = 3), areas of ground-glass opacities (n = 5), septal lines, and centrilobular interstitial thickening (n = 5). In five of the seven cases, a crazy-paving pattern of various spread was also present, either isolated (n = 1) or surrounding a pulmonary consolidation. In two cases traction bronchiectasis and cystic changes consistent with fibrosis were seen. At MR imaging (n = 2) a pulmonary consolidation of high signal intensity on T1-weighted image consistent with lipid content was present in one case. Pathologic examination (n = 4) showed the coexistence of lobules with lesions of various ages, sometimes in contiguous lobules, within the same patient. Recent lesions were those with alveolar fill-in by spumous macrophages and almost normal alveolar walls and septae. In more advanced lesions, lobules were filled in with larger vacuoles often surrounded by inflammatory infiltrates of alveolar walls, bronchiolar walls, and septa. The oldest lesions were characterized by fibrosis and parenchymal distortion around large lipid-containing vacuoles. The HRCT findings reflect pathologic findings in exogenous lipoid pneumonia. Although non-specific, consolidation areas of low attenuation values and crazy-paving pattern are frequently associated in exogenous lipoid pneumonia and are indicative of the diagnosis.

Adult↗
PubMed1998

A patient with congenital lipoid adrenal hyperplasia evaluated by serial abdominal ultrasonography.

UNLABELLED: Adrenal enlargement was followed by serial ultrasonography in an infant with congenital lipoid adrenal hyperplasia (lipoid CAH) from day 12 until 2 years and 4 months of age, when they were no longer detectable. Contrary to other types of CAH in which the configuration changes soon after replacement therapy, this infant with lipoid CAH showed persistent adrenal cortex enlargement due to massive accumulation of lipids and cholesterol resulting in a damaged glandular cyto-architecture. CONCLUSION: ultrasonographically persistent enlargement of the adrenals after replacement therapy is suggestive of the lipoid form of CAH.

Adrenal Cortex↗
PubMed1983

Physical separation of the aqueous phase and lipoidal lamellae from multilamellar liposomes: an analytical and preparative procedure.

An efficient and rapid method to separate the aqueous phase from lipoidal lamellae after mechanical disruption of multilamellar liposomes with preservation of the phospholipid bilayer organization has been designed. Liposome suspensions were subjected to a few short bursts of sonication at a temperature below the transition temperature (Tc) of the lowest melting phospholipid component. This was followed by ultracentrifugation. The aqueous supernatant contained more than 90% of the encapsulated aqueous elements (6-carboxyfluorescein, bovine serum albumin) and less than 1% of the lipids. The washed pellet consisted of lipoidal lamellae devoid of any encapsulated aqueous phase. The lipoidal lamellae contained more than 99% phospholipids and were contaminated with less than 0.1% aqueous components. Such a method should allow the localization of any component within the aqueous phase or lipoidal lamellae of the liposomes.

Chemical Phenomena↗
PubMed1990

Wide spectrum of steroids serving as substrates for the formation of lipoidal derivatives in ZR-75-1 human breast cancer cells.

Recently, several natural steroids have been found to be esterified to long-chain fatty acids (FAE) in various mammalian tissues. The purpose of the present study was to determine the ability of a series of 3H-labeled steroids to serve as substrates for the formation and accumulation of such non-polar derivatives in intact cells, using the hormone-responsive ZR-75-1 human breast cancer cell line as model. All 14 steroids tested were found to be converted, directly or following further metabolism, to lipoidal ester derivatives. The percentage of intracellular steroids recovered as FAE derivatives was usually substantial (14-90%), especially in the case of C-19 steroids (75-90%). The composition of the lipoidal steroid fractions recovered from the labeled cell extracts was characterized by chromatographic comparison with synthetic steroid FAEs and by saponification of the steroid FAEs and identification of the released steroidal moieties. Following metabolism, most steroid substrates were converted into multiple lipoidal esters. Furthermore, 5 alpha-androstane-3 alpha, 17 beta-diol, 5 alpha-androstane-3 beta, 17 beta-diol, as well as androst-5-ene-3 beta, 17 beta-diol formed lipoidal diesters in addition to the monoester form. The high level of intracellular steroid FAE accumulation reported in this study suggests that these yet poorly known steroid derivatives may play important functions in the regulation of steroid hormone metabolism and action.

Breast Neoplasms↗
PubMed2000

[Acute lipoid pneumonia caused by accidental aspiration of vaseline used in nasogastric intubation].

Exogenous lipoid pneumonia is a rare disease which is often underdiagnosed. Exogenous lipoid pneumonia has traditionally been considered a chronic process secondary to continued aspiration or inhalation of fatty substances. In its acute form exogenous lipoid pneumonia sometimes coincides with accidental massive aspiration of lipidic material, as has been traditionally described for fire eaters. We review the literature and report a case of acute lipoid pneumonia secondary to accidental aspiration of vaseline used to place a nasogastric tube, which had been inserted for gastric lavage after a suicide attempt by flurazepam ingestion.

Acute Disease↗
PubMed1998

Incorrect folding of steroidogenic acute regulatory protein (StAR) in congenital lipoid adrenal hyperplasia.

Steroidogenic acute regulatory protein (StAR) rapidly stimulates the movement of cholesterol into adrenal and gonadal mitochondria to mediate the acute steroidogenic response; StAR mutations cause potentially lethal congenital lipoid adrenal hyperplasia (lipoid CAH). Bacterially expressed wild-type StAR and four amino acid replacement/deletion mutants that cause lipoid CAH were purified to apparent homogeneity. Sedimentation equilibrium ultracentrifugation showed that all five proteins were monomeric and fit a globular protein model of the correct molecular mass. Circular dichroism (CD) spectra of both the wild-type and mutants showed minima near 208 and 222 nm, confirming the presence of substantial alpha-helical structure. However, subtle differences in the CD signals of the wild-type and mutants in the far-UV and stronger differences in near-UV indicated differences in protein folding. The amide I and II bands in the 1400-1700 cm-1 region of Fourier transform infrared spectra showed that the proteins fell into two groups. The wild-type and a partially active conservative mutant were predominantly alpha-helical with some intramolecular beta-sheet. By contrast, three mutants that lost charged residues retained much of their alpha-helical structure, but also tended to form intermolecular beta-sheets. Urea at 2.0 or 4.0 M had less effect on the CD spectrum of the wild-type than of the mutants, particularly those having lost a charged residue; 50 mM guanidinium hydrochloride did not alter the CD spectrum of the wild-type, but elicited dramatic changes to the secondary structure in all four mutants. Despite this, thermal melting curves of the mutant proteins in 50 mM guanidinium hydrochloride showed surprising stability, even exceeding that of the wild-type protein. These data suggest that the StAR amino acid replacement mutants that cause lipoid CAH are inactive because of fairly gross errors in protein folding, probably due to the loss of salt bridges that stabilize the tertiary structure.

Adrenal Hyperplasia, Congenital↗
PubMed1997

Analysis of the steroidogenic acute regulatory protein (StAR) gene in Japanese patients with congenital lipoid adrenal hyperplasia.

Genomic DNA from 19 Japanese patients with congenital lipoid adrenal hyperplasia (lipoid CAH) representing 16 different families was examined to identify the genetic alterations of steroidogenic acute regulatory protein (StAR). Ten of 19 patients had a 46,XX karyotype and nine had a 46,XY karyotype. Six of the 46,XX patients have experienced spontaneous pubertal changes including breast development and irregular menstruation whereas none of the 46,XY subjects displayed pubertal changes. Eight different mutations were identified. Sixteen patients were either homozygotes or compound heterozygotes for the Q258X mutation. The seven other mutations identified were 189delG, 246insG, 564del13bp, 838delA, Q212X, A218V and M225T. The 189delG, 246insG, 546del13bp and Q212X mutants encode truncated proteins. COS-1 cells transfected with expression vectors encoding cDNAs for the mutant StAR proteins which affect the C-terminus, 838delA, A218V and Q258X, exhibited no steroidogenesis enhancing activity. However, the M225T mutant retained some steroidogenic activity. The patient with the M225T mutation had late onset of this disorder and some capacity to secrete testosterone in response to hCG. These findings suggest: (i) that the Q258X mutation can be used as a genetic marker for the screening of Japanese for lipoid CAH, (ii) that the C-terminus of StAR plays an important role in the protein's activity and (iii) that there are differences in the extent of functional impairment of the testis and ovaries in lipoid CAH.

Adrenal Hyperplasia, Congenital↗
PubMed1986

Immunogenic activity of gonococcal protein I in mice with three different lipoidal adjuvants delivered in liposomes and in complexes.

For several reasons the major outer membrane protein from Neisseria gonorrhoeae (gonococcal protein [PI]) is an attractive component for a gonococcal vaccine. This paper describes the influence of two different physical forms of PI on its immunogenic activity. To this end PI was delivered in liposomes and in protein-detergent complexes. In both forms PI was present in a multimeric form. The liposomes were composed of phosphatidylcholine and cholesterol. The effect of dicetylphosphate as a negatively charged amphiphile and three lipoidal adjuvants was investigated. Two lipoidal adjuvants (Avridine and dimethyldioctadecylammoniumbromide) were positively charged amphiphiles, whereas the third one (tridecyl N-acetylmuramyl-L-alanyl-D-isoglutaminate) was neutral. The protein-detergent complexes were also tested in the presence of the lipoidal adjuvants and in an AlPO4-adsorbed form. The liposome preparations were characterized for their size, charge, and residual amount of detergent. The immunogenic activity of PI in all forms was tested in mice. The results of the antibody assays showed that PI in the liposomes was more immunogenic than PI in the complexes. A second dose with liposomes induced only a small booster effect, whereas such a dose with the complexes produced pronounced booster effects. The incorporation of the positively charged lipoidal adjuvants in the liposomes resulted in enhanced booster effects. The highest immunogenic activity of PI after two injections, however, was observed in the complexed form adsorbed to AlPO4.

Animals↗
PubMed1997

Spontaneous puberty in 46,XX subjects with congenital lipoid adrenal hyperplasia. Ovarian steroidogenesis is spared to some extent despite inactivating mutations in the steroidogenic acute regulatory protein (StAR) gene.

Congenital lipoid adrenal hyperplasia (lipoid CAH) is the most severe form of CAH in which the synthesis of all gonadal and adrenal cortical steroids is markedly impaired. We report here the clinical, endocrinological, and molecular analyses of two unrelated Japanese kindreds of 46,XX subjects affected with lipoid CAH who manifested spontaneous puberty. Phenotypic female infants with 46,XX karyotypes were diagnosed with lipoid CAH as newborns based on a clinical history of failure to thrive, hyperpigmentation, hyponatremia, hyperkalemia, and low basal values of serum cortisol and urinary 17-hydroxycorticosteroid and 17-ketosteroid. These patients responded to treatment with glucocorticoid and 9alpha-fludrocortisone. Spontaneous thelarche occurred in association with increased serum estradiol levels at the age of 10 and 11 yr, respectively. Pubic hair developed at the age of 12 yr 11 mo in one subject and menarche was at the age of 12 yr in both cases. Both subjects reported periodic menstrual bleeding and subsequently developed polycystic ovaries. To investigate the molecular basis of the steroidogenic lesion in these patients, the StAR gene was characterized by PCR and direct DNA sequence analyses. DNA sequence analysis revealed that one patient is homozygous for the Gln 258 Stop mutation in exon 7 and that the other patient is a compound heterozygote with the Gln 258 Stop mutation and a single A deletion at codon 238 in the other allele causing a frame-shift, which renders the StAR protein nonfunctional. These findings demonstrate that ovarian steroidogenesis can be spared to some extent through puberty when the StAR gene product is inactive. This is in marked contrast to the early onset of severe defects in testicular and adrenocortical steroidogenesis which are characteristics of this disease.

Adolescent↗
PubMedDate not supplied

Endogenous lipoid pneumonia associated with undifferentiated connective tissue disease (UCTD).

BACKGROUND: Lipoid pneumonia is a rare pulmonary disease, a form of pneumonia that has no classical radiological appearance, thus it can imitate other lung diseases. Lipoid pneumonia is usually classified into two major groups, depending on whether the source of oil/fat in the respiratory tract is from an exogenous or endogenous source. Undifferentiated connective tissue disease is a term used by rheumatologists to define a group of diffuse connective tissue disorders that lack definitive characteristics of any particular well-defined disorder. MATERIAL AND METHODS: A case study is reported of concomitant undifferentiated connective tissue disease and endogenous lipoid pneumonia. RESULTS: Histologically the macrophages appeared filled with lipid and were similar to atherosclerotic foam cell macrophages. Antibiotic and antimycotic treatments were ineffective. However, with concomitant steroid treatment, the patient exhibited absence of lung infiltration as well as other symptoms and was discharged. Therefore it is concluded that the lipoid pneumonia was steroid dependent. CONCLUSION: Since the patient's condition responded to steroid treatment, and it is clear that steroids inhibit phospholipase activity, the authors speculate that the subsequent decreased endoperoxide production may diminish lipid uptake by macrophages via decreasing modification of LDL or other lipid sources.

Adult↗
PubMed1982

Ovarian lipoid cell tumor. Steroid hormones and ultrastructure.

A virilizing, ovarian lipoid cell tumor was removed from a 19-year-old woman with amenorrhea. Light- and electron-microscopic studies of tumor morphology were combined with biochemical measurements of testosterone, dihydrotestosterone, estrone, estradiol, and progesterone. Electron microscopy demonstrated cells with the typical ultrastructural features of steroidogenesis. Analysis of extracts from tumor tissue showed high concentrations of progesterone, testosterone, dihydrotestosterone, estrone, and estradiol. These extracts from the ovarian lipoid cell tumor contained the following quantities of steroids: testosterone 450 ng/g tissue; dihydrotestosterone 56 ng/g tissue; estrone 233 pg/g tissue; estradiol 10896 pg/g tissue; and progesterone 512 ng/g tissue. Ovarian vein blood of the ovary with the lipoid cell tumor showed these results: testosterone 3276 ng/dl; dihydrotestosterone 311 ng/dl; estrone 69 pg/ml; estradiol 258 pg/ml; and progesterone 8.1 ng/ml. Because of the ultrastructural and biochemical similarities of this tumor to granulosa lutein cells it is concluded that this ovarian lipoid cell tumor probably originated from ovarian stromal cells.

Adrenal Rest Tumor↗
PubMed1993

[The solubility of drugs in lipoid vehicles].

A method for determination of drug substances solubility in lipophilic solvents is presented. The solubility was determined in lipophilic suppository bases meltings, in pharmaceutical lipoids as Oleum Ricini, Oleum Arachidis, Cera perliquida, Paraffinum perliquidum and in chemically defined lipoids as n-hexadecane, 1-hexadecane, cetylic alcohol, palmitic acid, cetylpalmitate. Consequences from chemical constitution of substances for solubility are discussed, also consequences from chemical constitution and dielectric constants of lipoidic solvents for their solution behavior. For the substances investigated, the apparent partition coefficients in the two-phase systems lipoid/phosphate buffer pH 7,4 and n-octanol/phosphate buffer pH 7,4 were determined, also the solubility in phosphate buffer. The results show, that connections between partition coefficients and solubility in lipophilic or aqueous phases do not exist. On the other hand, an indirect proportionality between water solubility and lipoid solubility also does not exist. In consequence, interpretations of drug release from lipophilic systems have to be proceeded from exact knowledge of partition behavior and solubilities in both the lipophilic and aqueous phase.

Chemistry, Pharmaceutical↗
PubMedDate not supplied

[Lipoid nephrosis in a child with advanced Hodgkin's lymphoma].

This is a case of an eight year old male, with Hodgkin's disease grade IV-B, with initial clinical presentation of nephrotic syndrome. The renal biopsy showed minimal change lesions. He had a complete remission of the nephrotic syndrome and Hodgkin's disease after been given prednisone and chemotherapy respectively. The remission of the nephrotic persisted 22 months and the Hodgkin's disease, 23 months. It has been established that cellular immunity is altered in Hodgkin's disease. The etiopathogenesis of lipoid nephrosis is unknown. There are clinical and experimental evidences showing cellular immunity in patients with lipoid nephrosis, suggesting a possible involvement of this mechanism in the etiopathogenesis of lipoid nephrosis. This could explain a relationship between the nephrotic syndrome (lipoid) and Hodgkin's disease. Our patient, besides Hodgkin's disease, showed clinical, biochemical and histologic changes, as well as clinical course compatible with nephrotic syndrome with minimal changes lesions.

Adolescent↗