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Biomedical subjects

Ia S Pustovoĭt

Publications and source records attributed to Ia S Pustovoĭt.

6 recordsLinked to original sources
PubMed2003

[Clinical manifestations of porphyrin metabolism disorders].

AIM: To characterize patients with various nosological unities [symbol: see text] of porphyria in accordance with their age, clinical symptoms, provoking factors, therapy and outcome. MATERIAL AND METHODS: Patients with acute intermittent porphyria (43), hereditary coproporphyria (8), variegate porphyria (3), porphyria cutanea tarda (7), hepatoerythropoietic porphyria (1), and hereditary erythropoietic porphyria (2) were studied. One patient was suspected of porphyria caused by deficiency of delta-aminolevulenic acid dehydrogenase. RESULTS: The patients were from the CIS. The overwhelming majority of them were young and middle-aged subjects. Rapid development of the disease and severe neurological symptoms were predominantly observed in patients with acute forms of porphyria. CONCLUSION: Early diagnosis of porphyrin metabolism disorders makes it possible to decrease abruptly the number of cases leading to severe complications, disability, and fatal outcome. The use of inexpensive methods of screening of porphyrin metabolism disorders provides a promising approach to solving this problem. These methods should be used in municipal hospitals. In addition, asymptomatic carriers of defective gene should be revealed at the preclinical stage using various methods of molecular genetic assay.

Acute Disease↗
PubMed2000

[Atypical electrolytic disorders in patients with diseases of the blood system].

AIM: To describe and analyze rare iatrogenic disturbances of water-electrolyte and acid-base balances in systemic blood diseases. MATERIAL AND METHODS: Five cases of life-threatening electrolytic imbalance resultant from therapy in patients with hemoblastoses and a patient with acute intermittent porphyria (AIP). RESULTS: Hyperkalemia arose in 2 patients treated with cyclosporin A and trimethoprim-sulfamethoxasole. One patient developed severe arrhythmia because of hypomagnesemia following long-term therapy with aminoglycosides and amphotericin B. In one AIP patient hyponatremia ending in coma was attributed to the disease activity and infusion therapy. This was the cause of coma. In a patient with acute leukemia and pneumonia amphotericin B therapy entailed distal renal tubular acidosis and marked dyspnea which was primarily mistaken for acute respiratory failure. Pathogenetic, diagnostic and therapeutic aspects of the above water-electrolyte defects are provided. CONCLUSION: The appearance of water-electrolyte imbalance in hemoblastosis patients calls up a detailed analysis of the going-on therapy whether the imbalance is iatrogenic.

Acidosis, Renal Tubular↗