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Biomedical subjects

I Korom

Publications and source records attributed to I Korom.

At least 19 recordsLinked to original sources

[Ocular manifestations in porphyria cutanea tarda].

Ninety two patients suffering from porphyria cutanea tarda were examined ophthalmologically in a paired case-control study. The incidence of pinguecula and that of pterygium were 8 times and 2 times higher, respectively, in PCT patients that in the control group. The photodamage of the conjunctiva is presumed to be a result of the photoactivity of uroporphyrin in the tissues.

Conjunctiva

Photodamage of the conjunctiva in patients with porphyria cutanea tarda.

Ninety two patients with porphyria cutanea tarda (PCT) were examined ophthalmically in a paired case control study. The incidence of pinguecula and of pterygium was 8 and 2 times higher respectively, in PCT patients than in the control group. The photodamage to the conjunctiva is considered to be a result of the photoactivity of uroporphyrin in the tissues.

Case-Control Studies

[Amidaron-induced dermatopathy resulting from unnecessary Cordarone therapy of ventricular parasystole].

The case history of a patient is reported who was treated with a variety of antiarrhythmics over a period of years because of refractory ventricular "bigeminy". As the arrhythmia did not respond to any kind of therapy, amiodarone treatment was started, which the patient received in a maintenance dose of 600-400 mg/day for 4 years. More recently, a bluish-grey hyperpigmentation of the face and other areas of the skin exposed to sunlight developed. A cutaneous biopsy of the hand revealed pigment deposits and lamellated lysosomal inclusions characteristic for amiodarone dermatopathy. The interactive, computer-assisted analysis of the ventricular ectopic activity has clearly demonstrated its innocent, parasystolic nature. The differentiation between ventricular extrasystolic and parasystolic activity is essential, because the latter arrhythmia does not require specific antiarrhythmic pharmacotherapy.

Amiodarone

[Family studies on the incidence of multiple pigmented naevi, familial skin melanoma and other malignant tumors].

102 family members of 14 patients with primary melanoma and multiple atypical pigmented naevi were investigated. Additionally to 14 patients with melanoma, 31 family members were found to have multiple atypical pigmented naevi. Most of them had light complexion and poor pigmentation capacity. A relative high frequency of malignant tumors other than melanoma were observed among blood relatives without multiple naevi. Members of melanoma-prone families need regular medical surveillance.

Adult

[Pheochromocytoma in Recklinghausen neurofibromatosis].

The authors describe the case of a 38-year-old hypertensive woman who suffered from neurofibromatosis, pheochromocytoma, scoliosis and diabetes mellitus. Because of the residual pheochromocytoma surgical intervention was repeated. According to the available literature this is the first case in Hungary where pheochromocytoma was associated with neurofibromatosis.

Adrenal Gland Neoplasms

[Hyperpigmentation of the face].

A 49-year-old male patient is presented, who developed hyperpigmented macules on the face. An exact classification of the disorder was not possible on the basis of anamnestic data, histology and electron microscopy. An attempt was made to differentiate it from other known dyschromias of the face.

Dermabrasion

[Cutaneous paraneoplastic syndromes of the skin].

With paraneoplastic skin diseases, we distinguish between obligatory and facultative symptoms. These may be caused by the tumor via allergic or immunologic mechanisms, but mostly the mechanism is unclear (e.g. coincidental). After removal of the tumor, the signs and symptoms should disappear. It is important to avoid strong immunosuppressive treatment, because it may produce dissemination of the tumor.

Acanthosis Nigricans

Membranous nephropathy accompanied by angiolymphoid hyperplasia of the skin.

A 24-year-old female developed a painless swelling adjacent to the left ear. This was shown to be eosinophilic angiolymphoid hyperplasia (ALH). Three months later she developed a nephrotic syndrome. Renal biopsy revealed membranous nephropathy. This is the first non-Japanese case of dermal eosinophilic ALH and nephrotic syndrome; steroid treatment followed by surgical removal of the tumour resulted in complete remission in the renal lesion.

Adult

[Sclerodermiform porphyria].

We give a retrospective survey on the clinical, histological, biochemical, and pathogenetical aspects of sclerodermiform changes rarely accompanying porphyria cutanea tarda (PCT). Sclerodermiform changes were seen in 12 patients (2% of all our PCT cases). In these patients, no correlation was found between the severity of the dermatological signs and symptoms and the degree of disturbance in the porphyrin metabolism. Biochemical remission was not accompanied by improvement of the sclerodermiform changes. The proportion of porphyrins with 4 or 5 COOH-groups was higher than that of PCT patients without sclerosis. The findings are consistent with the view that the development of sclerodermiform changes cannot be merely explained by phototoxic reactions, but the "dark-effect" of the porphyrins may also play an important role in the pathogenesis.

Actin Cytoskeleton