Primary systemic amyloid with nail dystrophy.
We report a patient with the classical cutaneous findings of primary systemic amyloidosis, due to myeloma. He had developed a nail dystrophy, which is a recognized, but rare, feature in systemic amyloid.
Biomedical subjects
Publications and source records attributed to M L Price.
We report a patient with the classical cutaneous findings of primary systemic amyloidosis, due to myeloma. He had developed a nail dystrophy, which is a recognized, but rare, feature in systemic amyloid.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
A case of plane xanthomatosis in a 67-year-old woman, suffering from chronic lymphatic leukaemia, who developed asymptomatic yellow plaques on the neck, upper arms and chest is reported. Histology was suggestive of xanthelasma. Development of plane xanthomata has been reported in association with various haematological malignancies and with benign monoclonal gammopathies, but not previously with chronic lymphatic leukaemia.
Factor XIIIa has previously been used to identify a subgroup of dermal dendritic cells which co-express class II MHC antigens, intercellular adhesion molecule-1 (ICAM-1) and lymphocyte function associated antigen-1 (LFA-1), leading to the suggestion that they play a central role in skin immune responses. To examine whether these cells may also be important constituents of other organs we have performed an immunohistochemical survey of normal tissue to identify the distribution of this factor XIIIa positive subgroup of dendritic cells in humans. Routine tissue sections from a range of organs were assayed for factor XIIIa expression using a standard peroxidase-antiperoxidase (PAP) immunolabelling technique. Dendritic, factor XIIIa positive cells were observed in all tissues studied, but were most numerous in skin and mucosal tissues (gastrointestinal tract, bladder). They were also observed associated with epithelial structures in lung and kidney, but were only rarely observed in liver, thyroid, testis and spleen. The distinctive distribution of these cells is consistent with an important role for them in immune responses at those sites.
We evaluated regional cerebral blood flow with technetium 99mTc hexamethylpropyleneamineoxime single photon emission computed tomography (SPECT) in 20 children and adolescents with neurologic dysfunction of varied etiology and abnormal electroencephalograms (EEGs). All patients were also examined with computed tomography (CT) and magnetic resonance imaging (MRI). Abnormal perfusion was found in 17 (85%) of 20 SPECT scans. Abnormal CT or MRI scans were noted in nine (45%) and in 10 (50%) of 20 cases, respectively. In eight (73%) of 11 cases with normal CT scans and in seven (70%) of 10 with normal MRI scans, the SPECT scan was abnormal. Abnormal regional cerebral blood flow on SPECT scans correlated better with EEG abnormalities than with neurologic examination or CT or MRI scan findings. We conclude that in children and adolescents with a spectrum of neurologic diseases and abnormal EEGs, abnormalities of brain structure or function are more likely to be documented by SPECT than by CT or MRI scans. SPECT findings correlate well with the location and type of EEG abnormality.
A recombinant 60 kDa Brucella abortus protein expressed in Escherichia coli was recognized in immunoblots by sera from mice experimentally infected with B. abortus and a dog experimentally infected with B. canis. Sera from humans and dogs with naturally acquired brucellosis also recognized this protein, which was designated BA60K. The gene encoding BA60K was localized within an 18 kb B. abortus genomic fragment and its direction of transcription determined by subcloning and maxicell analysis of selected restriction fragments. The nucleotide sequence of 1800 bases encompassing the predicted gene location was determined, revealing an open reading frame encoding a protein of 546 amino acids (predicted relative molecular mass of 57515). Solid phase micro-sequencing of BA60K eluted from two-dimensional polyacrylamide gels confirmed the predicted amino acid sequence. Comparison of the predicted amino acid sequence of BA60K with a protein sequence database revealed that BA60K shares 67.9% identity with the GroEL protein of E. coli, a member of the Hsp60 family of chaperonins. The immunodominant Hsp60 homologs from Legionella pneumophila, Chlamydia trachomatis and Mycobacterium tuberculosis were also found to share greater than 59% amino acid sequence identity with the BA60K protein. The identification of BA60K as a member of the Hsp60 family of chaperonins supports its role in stimulating a prominent host immune response during the course of Brucella infections. It also indicates that BA60K is an important candidate for studies aimed at identifying the antigens responsible for eliciting the protective immune response to brucellosis.
Changes in cardiac index (CI) and estimated systemic vascular resistance (ESVR) were assessed non-invasively using pulsed Doppler ultrasound during induction of anaesthesia. Ninety-six ASA I patients were allocated randomly to one of four groups to receive alfentanil 8 micrograms kg-1 followed by a dose of thiopentone, methohexitone, propofol or etomidate sufficient to obtund the eyelash reflex. CI increased significantly by 8% 1 min after administration of both methohexitone (P < 0.05) and propofol (P < 0.05), returning to pre-induction values thereafter. CI increased after thiopentone but the increase was not statistically significant. There was a significant decrease in CI of 16% after induction with etomidate (P < 0.001). ESVR decreased significantly from pre-induction values by 18% after methohexitone (P < 0.001) and 23% after propofol (P < 0.001). ESVR in the thiopentone group decreased, but this was not statistically significant. ESVR increased significantly by 12% 1 min after induction of anaesthesia with etomidate (P < 0.05) and then decreased towards pre-induction values. The results suggest that the cardiostability of etomidate may not be as complete in all groups of patients as previous studies have suggested.
Explore the source record for details and available documents.
Pre-existing epidural analgesia was rapidly extended in 36 consecutive patients presenting for emergency caesarean section by using 20 ml of 2% lignocaine + 1/200,000 adrenaline freshly prepared given by slow bolus injection. Despite a wide range of initial analgesic sensory levels the technique produced blocks that were dense with adequate anaesthesia for surgery in all patients within 12.5 min. The plasma local anaesthetic concentrations were well below the toxic range. Neonatal condition was good.
Explore the source record for details and available documents.
Two groups of people with psoriasis attended a short series of meetings conducted by a clinical psychologist. During these sessions the patients were given the opportunity to discuss between themselves problems created by their skin complaint and they were taught specific relaxation techniques for use whenever they felt under stress. Psychological tests showed that the participants were a noticeably anxious group compared to the general population. However, their levels of anxiety were significantly reduced by the end of the study. There was also a modest trend towards physical improvement. No such changes were seen in a matched control group.
Breslow tumour thickness was measured in frozen and paraffin wax sections from 21 excision biopsies of cutaneous malignant melanomas by two observers. There was no consistent variation between frozen and paraffin wax sections, with recorded differences ranging from +0.3 mm to -0.2 mm. Interobserver differences ranged from +0.4 mm to -0.2 mm. The interobserver variations exceeded the intraobserver variations, but neither were significant. These findings show conclusively that, when using high quality frozen sections, there is no significant difference between Breslow thickness measured in frozen or paraffin wax sections and therefore that frozen sections can be used to microstage melanoma. Interobserver variations seem to be a more likely source of erroneous measurements of tumour thickness.
Explore the source record for details and available documents.
A monoclonal antibody (LH 7.2) to the carboxy terminal of type VII collagen has been used in an indirect immunoperoxidase method on frozen sections of biopsies of benign compound and intradermal cellular naevi, dysplastic naevi, and malignant melanomas, to label the epidermal basement membrane. Benign intradermal naevi had an intact membrane with the dermal naevus cell component lying beneath it. Compound naevi showed proliferating junctional nests of naevus cells pushing through the membrane towards the dermis beneath. In contrast, dysplastic naevi and melanomas showed a progressive displacement of the membrane downwards. Thin lesions were confined above the membrane. With increasing tumour thickness, discontinuities in the membrane were seen and foci of invasive growth through the line of the membrane were identified. These studies suggest that the good prognosis of thin melanomas is associated with confinement of the tumour within an expanded epidermis above the basement membrane.
We have compared four monoclonal antibodies which label basement membrane components using an indirect immunoperoxidase method in frozen sections of skin biopsies. The antibodies LH 7.2 and GB3 showed expression limited to epidermal and adnexal basement membrane. Antibodies to laminin and type IV collagen also decorated dermal blood vessels and stromal components. The antibodies LH 7.2 and GB3 are more suitable for labelling epidermal basement membrane in the study of cutaneous lesions.
Explore the source record for details and available documents.