Search PubMed⌕ Search

Biomedical subjects

D J Cripps

Publications and source records attributed to D J Cripps.

At least 37 records · Page 2Linked to original sources
PubMed1982

Epidemiology of hexachlorobenzene-induced porphyria in Turkey: clinical and laboratory follow-up after 25 years.

Cutanea tarda and mixed porphyric symptoms resulted from accidental ingestion of hexachlorobenzene (HCB)-treated seed grain in Turkey 25 years ago and involved about 4,000 persons with a 10% mortality. Twenty-five years later, 161 patients, 63 women and 98 men, with histories of HCB exposure as children or young adults were studied. Many patients had persistent abnormal porphyrin metabolism and prominent neurological, dermatologic, and orthopedic symptoms and signs. Thyromegaly was seen in 60% of the women and 27% of the men. The children born to porphyric mothers 25 years ago all died of pembe yara secondary to maternal milk and transplacental transfer of HCB. Current lactation specimens of porphyric patients show high HCB levels, up to 3.12 ppm, but the infant offspring appear normal.

Adult↗
PubMed1982

Action spectra of topical psoralens: a re-evaluation.

The action spectra for producing minimal phototoxic erythema with topical 0.I% trimethyl psoralen (TMP) and 8-methoxypsoralen (8-MOP) were determined with a double monochromator in the range of 295-380 nm. both psoralens induced photosensitivity in the range of 313-365 nm; TMP was 54% more effective than 8-MOP. There was no difference in the dose needed to produce minimal UV erythema or phototoxic erythema with 8-MOP and TMP at 295 and 305 nm, but at 313 nm with 8-MOP, photosensitivity was enhanced 3.5 times, and with TMP, sensitivity was enhanced 5.5 times. The peak sensitivity with 8-MOP was at 330 nm and for TMP it was 335 nm. No photosensitivity occurred above 380 nm. Results suggest that TMP and 8-MOP are significantly photoreactive at 320-335 nm. Commonly used UV-A light sources show peak emission around 360 nm. If there is a relationship between development of erythema and therapeutic effectiveness than this raises the possibility of alternative UV light sources for phototherapy with psoralens.

Furocoumarins↗
PubMed1981

Instrumentation and action spectra in light-associated diseases.

Instrumentation for studying action spectra in controls and various light-associated diseases is described. This study summarizes tests performed with a prism grating monochromator during the last 10 yr. There were 68 photodermatoses studied: xeroderma pigmentosum (XP) (1), lupus erythematosus (LE) (12), polymorphous light eruption (PLE) (23), solar urticaria (4), actinic reticuloid (2), halogenated salicylanilide photosensitivity and persistent light reactors (11), psoralen photosensitivity (6), and porphyria (9). A normal minimal erythema dose in the UVB (below 320 nm) was generally observed in polymorphous light eruption and lupus erythematosus. The most exquisite photosensitivity for delayed erythema was observed in actinic reticuloid, which in one case was 25-35 times more sensitive in the UVB range which was also observed but to a lesser extent in XP and in persistent light reactors. Persistence of erythema and edema at test sites was observed in XP, PLE, LE, and actinic reticuloid. A delay in development of erythema reaching a maximum at 72 hr was observed in XP and psoralen phototoxicity. Maximum photosensitivity occurred in solar urticaria. Three patients had peak sensitivity in the range of 310-313 nm and the 4th at 460 nm. Photosensitivity in the visible range was detected in 2 patients with solar urticaria, one with actinic reticuloid, and confirmed in 9 patients with porphyria (405 nm). Photosensitivity in the UVA (above 320 nm) occurred to some degree in all groups.

Bithionol↗
PubMed1981

Natural and artificial photoprotection.

A solar simulator is described with collimated filtered radiation incorporating an optical integrator to more evenly diffuse the xenon 2.5 KW light source. Populations can be catogorized into skin types I-VI and the minimal erythema dose (MED) in each skin type measured in Joules/cm2 was compared to give a relative suntan photoprotection factor or SPF compared with type I (1.0) so that type II had an SPF of 1.67, type III SPF of 2.5, type IV V had an SPF of nearly 4, whereas the darker Negroid skin (type VI) had an SPF of 9.68, or nearly 10 times that of type I. In addition, various studies were performed to determine the natural protection factor after natural sunlight (3 1/2 mo of summer sun). In 21 subjects the mean SPF was 2.33 when compared to the MED on unexposed skin on the buttocks. Five subjects who had received a mean total dose of 3.49 J/cm2 of UVB within a 4-week period had a mean SPF of 8.01 and 5 subjects who had received a mean total dose of 20 J/cm2 of UVA with PUVA therapy within 2 weeks had a mean SPF of 2.7. These studies were compared with the photoprotection ability of topical chemical protective agents with a range of 4-15.

Adolescent↗
PubMed1980

Porphyria turcica. Twenty years after hexachlorobenzene intoxication.

Porphyria involving more than 4,000 persons occurred in Eastern Turkey from 1956 to 1961 due to ingestion of hexachlorobenzene (HCB), a fungicide added to wheat seedlings. During a site visit in 1977, 32 porphyric Turks (mean age, 29 years) were examined. Porphyrin concentrations were determined with samples of urine and stool from 29 porphyric and 10 control Turks and compared with results from 40 subjects in the United States. Distinctive clinical features were found in porphyric subjects, including hyperpigmentation, hirsutism, severe scarring, short stature, pinched facies, small hands, painless arthritis, weakness, and enlarged thyroids. Porphyrin levels were still substantially elevated in five subjects. Hexachlorobenzene, which is fat soluble, was detected in maternal milk of one porphyric patient and in one other patient's fat. The level of HCB was not considered sufficient to account for persistence of porphyria but may be a clue to the cause of pembe yara (pink sore), in which many children who were breast-fed by affected mothers died with associated weakness, convulsions, and annular erythema.

Adipose Tissue↗
PubMed1979

Actinic reticuloid. A clinical, pathologic, and action spectrum study.

A 54-year-old man suffered from a chronic dermatitis that was more severe on light-exposed areas. Skin biopsy specimens revealed histopathologic findings consistent with those seen in mycosis fungoides. Ultrastructural studies demonstrated the existence of lymphoid cells with hyperconvoluted nuclei, typical of the mycosis fungoides cell or Sézary cell, within the epidermis, dermis, and peripheral blood. Light testing with a monochromator showed abnormal photosensitivity to both short- and long-wave ultraviolet light as well as to the violet, blue, and green wavelengths of visible light. The features of this patient's disease indicate actinic reticuloid.

Biopsy↗
PubMed1979

Photochemotherapy for mycosis fungoides: a clinical and histological study.

Nine patients with stages 2 or 3 mycosis fungoides (MF) and one with Sézary's syndrome were treated with oral methoxsalen and long-wave ultraviolet light. Previous therapy had either failed or achieved only partial control. Satisfactory clinical control was achieved in all but one case. Histological improvement was seen in all cases, but complete clearance of lymphoid infiltrate was noted mainly in epidermal and papillary dermal infiltrates. Lower dermal infiltrate remained largely unchanged. The case of Sézary's syndrome was inadequately controlled with systemic prednisone and cyclophosphamide, but addition of photochemotherapy improved the erythema and pruritus. This shows that photochemotherapy may have a place in MF treatment both alone and perhaps in conjunction with other treatments. An early relapse occurred on stopping photochemotherapy in four cases, but one patient has had three months' full remission.

Adult↗
PubMed1978

Erythropoietic protoporphyria: hepatic cirrhosis.

Observations on liver pathology and function were made on 12 patients with erythropoietic protoporphyria (EPP). Needle liver biopsies in 3 of 11 patients showed mild portal or periportal fibrosis without evidence of abnormal liver function. One patient (Case 12), died from hepatic cirrhosis and failure at the age of 11 years, the youngest yet reported. He was not suspected of pending liver disease until 3 months before death. The quantity of protoporphyrin present in his liver was approximately 5.75% of total liver weight. Fatal liver disease in EPP has occurred in 13 patients, 7 male and 6 female, in which the mean age of death was 38 years. The pathology of the liver in EPP is characteristic and is described.

Adolescent↗
PubMed1977

Griseofulvin causing hyperhemopexinemia and hepatic proliferation in mice: an in vivo and in vitro study.

Concentrations of hemopexin, a porphyrin-binding serum protein synthesized exclusively in the liver, increased significantly and concomitantly with levels of erythrocyte and liver protoporphyrin and coproporphyrin in mice made porphyric with 1% griseofulvin in the feed. Liver weights of porphyric mice increased remarkably. In comparison with controls, the ratio of the weight of normal to porphyric livers was at 10 days 1:2.1, at 21 days 1:2.8, and at 46 days 1:3.8. This increase in liver size was accompanied by increased cell division. The hepatic hyperplastic tissue fragments survived in vitro for several weeks and could be subcultured. The cultured cells, like those of the original liver, showed intense protoporphyrin fluorescence in the cytoplasm.

Animals↗
PubMed1977

Multiple glomus tumors.

A man had widespread, slowly evolving vascular lesions since infancy suggestive of the Blue Rubber Bleb Nevus Syndrome. His son had two painless lesions typical of Multiple Glomus Tumors. Many of the man's nodular lesions were painful. Post-excision recurrences were noted. Histologic studies of asymptomatic tumors from both cases showed irregular, dilated, vascular channels surrounded by narrow mantles of glomus cells, whereas a painful tumor had large foci of glomus cells with wider mantles around the flattened channels. Electron microscopy showed the glomus cells to be modified smooth muscle cells. The anatomy and pathology of glomus tumors are reviewed. Differentiation from other syndromes of multiple hemangiomata, particularly the Blue Rubber Bleb Nevus Syndrome is stressed. It is suggested that Multiple Glomus Tumors may be derived from simple cutaneous vessels and not the Sucquet-Hoyer canal of the normal cutaneous glomus body described by Masson in 1924.

Adult↗