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At least 595 records · Page 33Linked to original sources
PubMed1995

Erythropoietic protoporphyria presenting in an adult.

Erythropoietic protoporphyria is an inherited disorder of porphyrin metabolism, in which reduced activity of the enzyme ferrochelatase leads to accumulation of protoporphyrins in erythrocytes. Protoporphyrins are photoactivated by ultra-violet light causing tissue damage by release of free oxygen radicals, which manifests as photosensitivity. The majority of cases of erythropoietic protoporphyria present in childhood although sometimes symptoms are delayed until the second decade. We report here a case presenting in adulthood and discuss the risk of liver disease in the condition.

Adult↗
PubMed1982

Polymorphous light eruption: a common reaction uncommonly recognized.

Polymorphous light eruption (PMLE) is usually considered to be an uncommon complaint, although the prevalence in the general population has not been studied. In a survey of 271 apparently healthy subjects, 10% gave a history consistent with a diagnosis of PMLE. The clinical characteristics in the survey cases of PMLE were similar in most respects to those of patients presenting to a clinic with this disorder. However, there was one notable exception, in that there was a striking difference between the clinic and survey cases in the amount of sunlight required to trigger the eruption. Clinic patients required a mean exposure of 30 min as compared with over 3 h in the survey cases. These findings suggest that PMLE is a common disorder but that many individuals have a high threshold of response to sunlight exposure.

Adolescent↗
PubMed1998

Amiodarone-associated granuloma in bone marrow.

BACKGROUND: Amiodarone hydrochloride is classified as a Vaughan Williams class III antiarrhythmic agent, although class I, II, and IV effects may contribute to its favorable antiarrhythmic profile. It is associated with a wide variety of adverse effects, such as hypothyroidism, hyperthyroidism, interstitial pulmonary disease, hepatitis, coagulation disorders, skin photosensitivity, corneal microdeposits, alopecia, peripheral neuropathy, and cardiovascular arrhythmias. SUBJECTS: Bone marrow aspirations and biopsies were performed on two patients treated with amiodarone, on the first during a follow-up for myelofibrosis and on the second for a suspected lymphoproliferative disorder. Several bone marrow granulomas were found in both patients. The bone marrow specimens for tuberculosis and fungal stains were negative. CONCLUSIONS: The temporal relationship between the amoidarone therapy and the development of two cases of asymptomatic bone marrow granuloma suggest the possibility that this antiarrhythmic agent is involved in the etiology of these granulomas.

Aged↗