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Biomedical subjects

R K Joshi

Publications and source records attributed to R K Joshi.

At least 19 recordsLinked to original sources

Dyskeratosis congenita in a female.

We report a 9-year-old Saudi girl with dyskeratosis congenita. In addition to the known manifestations of this disease, she also had the additional features of tufts of hairs on the limbs, and an early onset of keratinized basal cell papillomas on her trunk.

Child

Autosomal recessive epidermolysis bullosa simplex. A case report.

We report a male child with autosomal recessive epidermolysis bullosa simplex presenting at birth. The patient subsequently developed cutaneous atrophy, nail dystrophy, milia and alopecia. He had growth retardation and anaemia, but there were no other associated abnormalities. Electron microscopy showed epidermolytic cleavage. The family history indicated an autosomal recessive mode of inheritance.

Consanguinity

Fumaric acid esters (FAEs) suppress CD 15- and ODP 4-positive cells in psoriasis.

A histological-immunohistological study was conducted to investigate the effect of systemically administered fumaric acid esters (FAEs) on epidermal thickness and composition of the inflammatory infiltrate in psoriatic plaques. The very first effect of systemic therapy with FAEs is the disappearance of CD 15-positive cells in the beneath the epidermis, accompanied by a significant reduction in T-helper cells beneath the epidermis, pointing to an immunosuppressive effect. This is followed after some delay by a reduction in acanthosis and hyperkeratosis. The reduction in infiltrating T-lymphocytes corresponds to that seen after systemic or intralesional therapy with cyclosporin. However, the normalization of the psoriatic plaques takes longer under the influence of FAEs than under cyclosporin.

Adult

A new quantum chemical approach in QSAR-analysis. Parametrisation of conformational energies into molecular descriptors JMn (steric) and JSn (electronic).

Two new types of structure-related molecular descriptors, JMn and JSn, have been developed using conformational energies from quantum chemical calculations. For this purpose propipocaine (CAS 3670-68-6) was chosen as a model and 42 analogues were studied. The quantum chemical calculations were performed applying AM1 and PCILO approximation methods. Appropriate mathematical models were designed to calculate steric parameter log JM1 and electronic parameters JS1 to JS6. The values obtained for these parameters were used in multiple linear regression analysis for the evaluation of the structure-activity relationship. Furthermore, a comparison between electronic parameters JSn and sigma (Hammett) was made. The results show, that these parameters can be used successfully in predicting the biological activity of compounds in this model. Although, JS5 values are comparable to sigma-Hammett, the electronic parameter JS2 gives a better correlation in QSAR-analysis involving two parameters JS2 and log JM1.

Anesthetics, Local

Successful treatment of Sweet's syndrome with doxycycline.

Two patients with Sweet's syndrome are described, both of whom were treated with doxycycline 200 mg daily, and responded well to therapy. Doxycycline appears to be an effective alternative to corticosteroids for the treatment of Sweet's syndrome.

Adult

Cutaneous granuloma with ataxia telangiectasia--a case report and review of literature.

A patient is reported who had ataxia telangiectasia with multiple cutaneous lesions mainly on the limbs, which showed atrophy and scarring. Histopathology of these skin lesions showed tuberculoid granulomas without frank collagen necrobiosis in the dermis. Chromosomal analysis revealed a translocation between 7 and 14 as well as deletion of chromosomes 4 and 6.

Ataxia Telangiectasia

Etretinate in the treatment of ulcerative lichen planus.

Ulcerative lichen planus (ULP) is a rare variant of lichen planus. It is resistant to all therapeutic modalities, and most of the patients with this disorder described in the literature have been successfully treated only by surgical skin grafting. We report a case with ULP successfully treated with etretinate.

Etretinate

Subcorneal pustular dermatosis and IgA myeloma.

A case of subcorneal pustular dermatosis (SCPD), as a presenting feature in a patient with multiple myeloma, is described. This is the seventh case report of this association and the first from the Middle East. Five of these cases, including the present report, have been of the IgA paraprotein type, IgA intraepidermal deposits have been described in a few patients with SCPD associated with IgA paraproteinaemias but have not been detected in our patient.

Female

White fibrous papulosis of the neck.

We report two middle-aged women who presented with multiple, discrete, non-follicular, oval, pale, asymptomatic lesions on the neck. Clinical and histopathological features were compatible with the entity of white fibrous papulosis of the neck.

Age Factors

Mycetoma: comparison of MR imaging with CT.

Magnetic resonance (MR) images obtained in 18 patients with pathologically confirmed mycetoma in the body (n = 4) or lower extremity (n = 14) were retrospectively reviewed and compared with computed tomographic (CT) scans in 15 patients and surgical findings in 10. T1-weighted images showed an infiltrating mass (same signal intensity as muscle) involving skin, subcutaneous fat, muscles, tendons, and other tissues. On T2-weighted images, the mass and affected structures showed moderately increased signal intensity. Bone marrow involvement was detected in seven patients and was best visualized on T1-weighted images. CT showed moderate enhancement of the infiltrative process in all patients. Bone changes, seen in nine, included coarse trabeculation, periosteal reaction, endosteal proliferation, and patchy destruction. MR imaging and CT were comparable and correlated well with surgery in showing the extent of soft-tissue involvement. Early bone changes (important for therapy planning for pedal mycetoma) were seen only at CT. The study showed that MR imaging is sensitive for assessing the extent of mycetoma in the soft tissues. CT should be the method of choice for staging pedal lesions because it can be used to detect early bone involvement.

Adult

Coagglutination test: a simple and rapid diagnostic technique for goat pox.

The coagglutination test was standardised using Staphylococcus aureus strain Cowan I (containing Protein A) coated with anti-goat pox serum for the detection of goat pox antigen of infected goat skin or kid kidney cell culture antigen. Agglutination was observed within 10 seconds in virus dilutions up to 10(-6). As the test is easy to perform it can be used for rapid diagnosis of goat pox.

Agglutination Tests

Dermal leishmaniasis and rifampicin.

In an uncontrolled study, the effectiveness of rifampicin was assessed in cutaneous leishmaniasis. Twelve of 15 patients (80%) showed good response. The treatment ranged from 2 to 12 weeks. Tolerance of the drug was excellent and apart from red discolouration of the urine, no other side effects were encountered. Rifampicin appears to have a place in the management of cutaneous leishmaniasis.

Adolescent

Papillon-Lefevre syndrome.

Two cases of Papillon-Lefevre Syndrome (PLS) in a family are reported. Association of recurrent infections, arachnodactyly, intracranial calcification and phalangeal abnormality have been observed. Because there is no history of consanguinity, and autosomal recessive pattern of inheritance is suggested.

Child