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Biomedical subjects

A Feinstein

Publications and source records attributed to A Feinstein.

At least 37 records · Page 2Linked to original sources

Pachyonychia congenita.

Pachyonychia congenita is a rare hereditary disorder characterized mainly by nail hypertrophy and dyskeratoses of skin and mucous membranes. A thorough literature survey since its first description in 1904 up to 1985 revealed 168 cases of pachyonychia congenita. There were no indications of any sex or ethnic group predilection. Based on this survey the following classification is suggested: type I (56.2% of cases), hyperkeratosis of nails, palmoplantar keratosis, follicular keratosis, and oral leukokeratosis; type II (24.9% of cases), clinical findings of type I plus bullae of palms and soles, palmar and plantar hyperhidrosis, natal or neonatal teeth, and steatocystoma multiplex; type III (11.7% of cases), clinical findings of types I and II plus angular cheilosis, corneal dyskeratosis, and cataracts; and type IV (7.2% of cases), clinical findings of types I, II, and III plus laryngeal lesions, hoarseness, mental retardation, hair anomalies, and alopecia.

Humans

Ganser symptoms, dissociation, and dysprosody.

Approximate answers (vorbeireden) are considered pathognomonic of the Ganser syndrome. Controversy has always surrounded the nosological status of this syndrome. Its recent inclusion in the DSM-III-R as a dissociative disorder is supported by this case report. The association of approximate answers, psychogenic amnesia, and dysprosody is reported here for the first time.

Adult

Isolation and characterisation of goat C-reactive protein.

A pentraxin was isolated from acute phase goat serum by its calcium-dependent affinity for agarose, and although it did not bind to phosphorylcholine immobilised on Sepharose, its binding to agarose was reversed by exposure to fluid phase phosphorylcholine. It was identified as goat C-reactive protein on the basis of its immunochemical cross-reactivity with human and bovine C-reactive protein. The molecule was composed of five identical, glycosylated, non-covalently associated subunits, each of molecular weight approx. 24,000. Acute phase serum levels in a small number of samples were not significantly different from normal levels (means 72 and 55 micrograms/ml, respectively), suggesting that goat C-reactive protein is not a major acute phase reactant. No other pentraxin was detected in goat serum.

Animals

Crystallization and preliminary crystallographic data for an antiprogesterone monoclonal antibody Fab' and steroid-Fab' complexes.

Crystals of an Fab' from an antiprogesterone monoclonal antibody (IgG1) have been grown from 1.5 M-ammonium sulfate, pH 5.5 to 8.5. The single crystals are hexagonal rods, space group P6222 (or P6422), with cell dimensions a = b = 135.2 A, and c = 124.2 A. Cocrystals of the Fab' with progesterone, pregnanedione and other related steroids have been grown. The complex crystals have different morphology but are isomorphous with the native crystals. A hydroxy-progesterone derivative obtained by substituting an iodo-benzoyl group at the 11 alpha-hydroxyl position looks promising as a suitable heavy-atom candidate in addition to other potential conventional heavy-atom derivatives. All crystals diffract to at least 2.8 A resolution and are suitable for high-resolution X-ray diffraction studies.

Animals

Monoclonal BALB/c anti-progesterone antibodies use family IX variable region heavy chain genes.

Variable region nucleotide sequences and respective translated amino acid sequences for three heavy chains (DB3, 11/32 and 10/8) and two light chains (DB3 and 11/32) of monoclonal mouse IgG1 anti-progesterone antibodies have been determined by primer extension mRNA sequencing. The three VH regions exhibit the same rarely observed VH IX gene family and have greater than 88% homology between them. Two associated light chain sequences are 95% homologous and belong to the V kappa I group. The N-terminal twenty two amino acids of the kappa light chain of the third antibody 10/8 have been determined by automated protein sequencing and are identical to those of 11/32. Thus, these three monoclonal anti-progesterones derived from separate fusions all use VHIX-V kappa 1 gene combinations.

Amino Acid Sequence

Painful piezogenic pedal papules in patients with Ehlers-Danlos syndrome.

Painful piezogenic pedal papules were observed in 10 of 29 patients with Ehlers-Danlos syndrome. The diagnosis was made by examining each patient while he or she stood with full body weight on the heels and by observing the appearance of the painful papules on the medial, posterior, and lateral aspects of both heels. Biopsy specimens of papules demonstrated a thickened and dense dermis. The subcutaneous fibrous trabeculae were thin, with resultant poor compartmentalization of the fat. Painful piezogenic pedal papules are due to herniation of subcutaneous fat into the dermis, which is possibly due to structural defects of the connective tissue. The high prevalence of painful piezogenic pedal papules in our group of patients (34.5%) makes its direct association with the Ehlers-Danlos syndrome highly feasible, most probably because of the connective tissue defect that occurs in patients with Ehlers-Danlos syndrome.

Adolescent

Ichthyosis-cheek-eyebrow (ICE) syndrome: a new autosomal dominant disorder.

A family presenting with ichthyosis vulgaris, prominent full cheeks, sparse lateral eyebrows and other craniofacial and musculoskeletal defects is described in detail. This constellation of physical findings represents a new syndrome, transmitted in an autosomal dominant fashion. For reasons of simplicity it has been termed the I (ichthyosis), C (cheek), E (eyebrow) syndrome.

Abnormalities, Multiple