Clinical manifestations of cholesterol crystal embolism with subungual haemorrhages: a possible relationship?
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Embolism, Cholesterol↗
Biomedical subjects
M Song
Publications and source records attributed to M Song.
Netherton's syndrome: a severe neonatal disease. A case report.
A male infant was born with generalized erythroderma and scaling; the newborn demonstrated poor neonatal development and developed several complications such as hypernatremic dehydration, septicemia, gastroenteritis and seizures. In the neonatal period, the erythema faded, but exfoliation persisted. The parents are healthy but related. One older brother, who died at the age of 3 months, had shown the same clinical picture in the neonatal period and was diagnosed with congenital psoriasis. All clinical investigations, including serum immunoglobulins, complement levels and lymphocyte counts, were normal. Only raised total IgE and multiple positive specific IgE reactions were noted. Skin biopsy revealed an image of ichthyosis. Polarization microscopy of scalp hair showed trichorrhexis nodosa and discrete focal twisting of the hair shaft. This clinical picture and all histological findings are compatible with the indications of Netherton's syndrome. The purpose of this report is to call attention to this severe presentation of congenital ichthyosis in the neonatal period and to the difficulty of a correct diagnosis when confronted with congenital erythroderma.
Consanguinity↗
Pharmacokinetics and efficacy of pulse oral versus intravenous calcitriol in hemodialysis patients.
Because intravenous (iv) calcitriol has greater bioavailability than oral calcitriol, it may be more efficacious in suppressing parathyroid hormone (PTH) secretion. In this study, the pharmacokinetics and efficacy of pulse oral and i.v. calcitriol were compared. Patients were randomized to receive 2 micrograms of i.v. or oral calcitriol after each dialysis. Two pharmacokinetic studies (PK1, PK2) were performed 10 days apart, during which the patients received calcitriol after each dialysis. Calcitriol bioavailability was determined from the area under the curve (AUCtime interval (hours) in pg/mL per h). After the PK phase, PTH was lowered to < 200 pg/mL by titrating calcitriol to a maximum of 12 micrograms/wk over 4 wk. Calcitriol was then maintained for another 18 wk unless serum calcium exceeded 11.5 mg/dL or Ca x P product exceeded 70; when these limits were reached, calcitriol was held and then restarted at a lower dose. After i.v. administration, peak serum calcitriol exceeded that achieved orally but by 1 h, calcitriol levels were similar. The AUC0-0.5 (105 +/- 12, i.v.; 9 +/- 4, oral) and AUC0.5-1 (68 +/- 6, i.v.; 30 +/- 7, oral) were higher with i.v. (P < 0.05), but cumulative AUC0-48 did not differ. Individual t1/2 values ranged from 10 to 129 h for PK1 and from 10 to 50 h for PK2. The t1/2 for oral calcitriol was 38 +/- 14 h for PK1 and 30 +/- 4 h for PK2 (not significant (NS)). The t1/2 for i.v. calcitriol was 26 +/- 5 h for PK1 and 19 +/- 3 h for PK2 (NS, PK1 versus PK2 and oral versus i.v.). When the PK1 oral and i.v. data were combined, the mean t1/2 was 32 +/- 7 h whereas the t1/2 for PK2 (oral and i.v.) was 22 +/- 3 h (P < 0.05). Baseline PTH levels were 510 +/- 90 pg/mL and 499 +/- 79 pg/mL, oral and i.v., respectively. Serum PTH level at 22 wk was not different between oral and i.v. groups, 153 +/- 38 pg/mL and 214 +/- 124 pg/mL in i.v. (NS). The percentage of PTH suppression was 66 +/- 7.4% in the oral group and 69 +/- 12% in the i.v. group (NS). A major degree of serum iPTH suppression occurred during the initial 4 wk of treatment, concomitant with a rise in serum calcium levels. Adverse effects were similar between groups, as were the average dosages of calcitriol and phosphate binders. In conclusion, the efficacy of intravenous and pulse oral calcitriol were similar in hemodialysis patients with secondary hyperparathyroidism. The early rise in serum calcium levels observed with treatment may have contributed significantly to the suppression of serum iPTH levels. The difference in bio-availability between the different routes does not have a clinically apparent effect. The t1/2 varied widely among individuals, whereas exposure to calcitriol may decrease the t1/2.
Administration, Oral↗
[Role of intralesional administration of bleomycin in the treatment of warts].
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Antimetabolites, Antineoplastic↗
Purification and characterization of alpha 1-antitrypsin secreted by recombinant yeast Saccharomyces diastaticus.
The secreted human alpha 1-antitrypsin (alpha 1AT) produced by yeast was purified from the culture medium by ultrafiltration, ammonium sulfate fractionation (60-75% saturation), protamine sulfate treatment, and ion-exchange chromatography. Molecular mass of the purified alpha 1AT was 52 kDa, which is similar to that of human plasma alpha 1AT. Yeast-produced alpha 1AT was fully functional as an inhibitor compared with the plasma form. Unlike plasma alpha 1AT, however, treatment of the yeast-produced alpha 1AT with endoglycosidase H decreased the molecular mass to that of recombinant alpha 1AT produced in Escherichia coli, indicating the high-mannose type N-linked glycosylation of the secreted alpha 1AT. Glycosylation in yeast cells enhanced kinetic stability of alpha 1AT towards heat deactivation.
Amino Acid Sequence↗
Genes encoding human serum amyloid A proteins SAA1 and SAA2 are located 18 kb apart in opposite transcriptional orientations.
The three expressed genes of the human serum amyloid A gene family (SAA1, SAA2 and SAA4) have been isolated in four contiguous clones selected from a chromosome 11 cosmid library. Analysis of these clones revealed that SAA1 and SAA2 are located 18 kb apart in opposite transcriptional orientations, while SAA4 lies 11 kb downstream from SAA2 in the same orientation: 3'(SAA1)5'-18 kb-5'(SAA2)3'-11 kb-5'(SAA4)3'. A fifth SAA clone isolated from this library was noncontiguous with the other four and contained the SAA3 pseudogene.
Chromosomes, Human, Pair 11↗
[Systemic sarcoidosis with cutaneous lesions: literature review].
Sarcoidosis is a systemic disease of unknown etiology. The diagnosis is based on consistent clinical, biological and radiological findings supported by histologic evidence of noncaseating epithelioid granulomas. Skin lesions occur in about a quarter of patients with sarcoidosis. The purpose of this article is to analyse through the clinical presentation of dermatological lesions, a potential link with systemic disease.
Adolescent↗
A particular subtype of ichthyosis congenita type III. Clinical, light, and electron microscopic features.
We report the case of a second patient with the extraordinary ultrastructural findings of vacuolated structures intermingled with membranes in the perinuclear part of the upper epidermal cells. Clinical, light microscopic, and electron microscopic features of this particular presentation of ichthyosis congenita type III have already been presented by K. M. Niemi and L. Kanerva in 1989. Although our patient has more or less the same light and electron microscopic findings, the clinical picture is more severe. The patient was born as a collodion baby. Later, he showed signs of generalized severe involvement with large scales, erythrodermia, and itching. Successful therapy with retinoids resulted in complete removal of the hyperkeratosis but left the striking reticulate skin pattern. Noting the heterogeneous clinical presentation, the specific electron microscopic findings are diagnostic. No biochemical data on this disease are known.
Adult↗
Eosinophilic pustular folliculitis in three atopic children with hypersensitivity to Dermatophagoides pteronyssinus.
Three children will be described who present recurrent episodes of pruritic papulopustular follicular lesions on the face, the extremities and the trunk. The episodes lasted for 1-3 months with intermittent remission. Each flare was accompanied by hypereosinophilia and an increased total IgE titer. RAST and prick tests were positive for Dermatophagoides pteronyssinus (DPT). Laboratory tests disclosed no infectious or parasitic etiology. Histological examination showed eosinophilic pustular folliculitis (EPF) in each of the 3 cases. The lesions responded well to topical corticosteroids. The aim of this article is to underline the importance of hypersensitivity reactions (in these particular cases to DPT) in the pathogenesis of EPF.
Adolescent↗
Atomic force microscopy of the cornea and sclera.
We report the first investigation of the extracellular matrix of cornea and sclera using an atomic force microscope (AFM), and evaluate the potential of this new technique. We were able to obtain 2-3 nanometre resolution of both tissues in a condition close to their native state. The AFM was able to resolve surface features on the collagen fibrils, as well as providing unique images of crossbridge structures between collagen fibrils in both cornea and sclera.
Animals↗
[Detection of serum sialic acid and its clinical significance in patients with oral and maxillofacial neoplasms.].
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Journal Article↗
[An experimental study of middle ear implantable hearing device].
The partially middle ear implantable hearing device was designed and tested in 7 cats with conductive hearing loss and 5 cats with mixed hearing loss. It was composed of sound processor, connector and ossicular vibrator. The ossicular vibrator was made from piezoelectric ceramic bimorph which was constructed in the form of flat chip 6mm in length, 1mm in width and 0.5mm in thickness. The incus of cat was removed and the lip attachment of the vibrator was attached to the head of the stapes. The base of the vibrator was fixed to the bone wall of bulla with medical glue. The average values of the response threshold of ABR evoked by click via this vibrator got reduction of 37dB and 39dB respectively in conductive hearing loss group and mixed hearing loss group. The gain curve of hearing was flat in the range from 0.5 to 8kHz frequencies. The ABRs evoked by click via ossicular vibrator was similar to that recorded preoperatively in the wave shape. This result indicates that this device is a highly efficient sound conducting divice to inner ear.
Animals↗
Controlling cancerous pain with analgesic powder for cancers.
Analgesic powder for cancers, composed of more than 20 Chinese drugs, was applied externally to 91 patients with various kinds of cancers for management of cancerous pain. The results showed that it was remarkably effective in 42 cases, fairly effective in 22, effective in 22, and ineffective in 5, the total effective rate being 94.51%. Animal experiments indicated that the pain threshold was evidently higher in mice treated with this powder on the site of femoral artery of the hind limbs than that of the controls without application of this powder.
Administration, Cutaneous↗
Restoration of normal ultrastructure after expression of the alpha 1 subunit of the L-type Ca2+ channel in dysgenic myotubes.
Muscular dysgenesis (mdg) is a spontaneous mutation affecting the alpha 1 subunit of the skeletal L-type Ca2+ channel. mdg/mdg mice suffer from a skeletal muscle disease characterised by low levels of the slow Ca2+ current, lack of contractile activity, and immature organisation of skeletal muscle. Microinjections of a cDNA encoding alpha 1 into mutant myotubes restore excitation-contraction coupling. We checked here that dysgenic myotubes transfected with expression vectors, including a full-length alpha 1 cDNA, also recover normal ultrastructural features. Transfection of alpha 1 cDNA partially deleted on the 5' end leads to the recovery of a good structural organisation without any improvement in the mutant physiological phenotype. These results suggest that: (i) the proper expression of alpha 1 is required for the full muscle differentiation of muscular dysgenesis myotubes, and (ii) portions of the alpha 1 molecule may be involved in the structural organisation of a muscle fiber, independent of its known functional properties.
Animals↗
Effect of iron regulation on expression and hemin-binding function of outer-sheath proteins from Treponema denticola.
The effect of the iron-chelating compounds EDDA and BPD on polypeptide regulation in the putative oral pathogen Treponema denticola was studied. SDS-PAGE analysis of the T. denticola strains grown in the presence of EDDA or BPD, i.e. iron-limiting environmental conditions, revealed the expression of 44 and 43 kDa polypeptides in the outer sheath, a 73 kDa polypeptide in the cell membrane, and a 16 kDa polypeptide in the soluble cell fraction. The hemin-binding activity of purified outer sheaths from T. denticola TD-4 grown in the presence of 6.4 mM EDDA was significantly greater than that observed in control (absence of EDDA) outer sheaths. Both activities were inhibited by proteinase K. SDS-PAGE, LDS-PAGE and TMBZ staining revealed the 44 and 43 kDa outer-sheath polypeptides to be expressed by T. denticola strains GM-1. MS-25, ATCC 33520 and ATCC 33404 (TD-4), strains which possessed strong hemin-binding activity. The 44 kDa hemin-binding polypeptide was purified by 1% CHAPS solubilization, HPLC, and SDS-preparative electrophoresis. N'-terminal sequence analysis indicated the purified 44 kDa polypeptide to belong to a new, undescribed group of polypeptides possessing hemin-binding activity.
2,2'-Dipyridyl↗
Degradation of endogenous plasma membrane fibronectin concomitant with Treponema denticola 35405 adhesion to gingival fibroblasts.
Treponema denticola adhesion and degradation of fibronectin (Fn) on human gingival fibroblasts (HGF) were studied by immunofluorescence and enzyme-linked immunosorbent assays. The number of adherent bacteria increased and the amount of immunoreactive Fn decreased as a function of increasing T. denticola concentration. The distribution of cell-bound Fn was punctate in micrographs. Anti-human Fn impaired bacterial adhesion to HGF. Phenylmethylsulfonyl fluoride inhibited Fn degradation but not adhesion. Sonicated extracts and diluted spent growth medium degraded HGF Fn but, unlike intact T. denticola cells, they hardly stimulated F-actin rearrangements.
Bacterial Adhesion↗
Recombinant murine serum amyloid A from baculovirus-infected insect cells: purification and characterization.
Serum amyloid A (SAA) is an extremely sensitive acute-phase reactant and precursor to the subunit protein in reactive amyloid deposits. Although the mouse has long served as an informative experimental model, both the function of SAA and the pathogenic mechanism of amyloid formation remain unknown. The production of SAA by a heterologous system was pursued as means of generating readily-renewable amounts of SAA of defined sequence. Murine SAA2 has been expressed in and purified from baculovirus-infected insect cells. Using the transfer vector pBlueBac, SAA2 cDNA was cloned into baculovirus DNA such that expression was under the control of the polyhedrin promoter. Lysates prepared from infected cells contained three amyloid A-immunoreactive forms which accumulated intracellularly over a three day periods. The form having the lowest relative molecular mass, 12.5 kDa, co-migrated in SDS-polyacrylamide gels with the SAA2 present in murine acute-phase serum. Recombinant SAA2 was purified by Sepharose CL-6B chromatography followed by chromatofocusing between pH 8 and pH 5. Amino-terminal sequencing of the purified 12.5 kDa sample confirmed the first 20 residues of mature murine SAA2. After incubation with normal mouse serum, purified recombinant SAA2 fractionated exclusively with lipoprotein complexes, suggesting that it was bound to HDL. Based on this observation, we believe that recombinant SAA can serve as a suitable substitute for the native protein in physiologically relevant studies.
Amino Acid Sequence↗
Basal cell nevus syndrome and congenital hydrocephaly.
A case of basal cell nevus syndrome or Gorlin's syndrome is reported in a newborn. The skin condition is associated with congenital hydrocephaly and skeletal malformations. To our knowledge, this is the first case of basal cell nevus syndrome with skin tumors present at birth and localized on the fingers.
Basal Cell Nevus Syndrome↗