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Biomedical subjects

K R Romeril

Publications and source records attributed to K R Romeril.

18 recordsLinked to original sources

Investigation of subjects with abnormal iron studies: role of the hepatic iron index.

AIM: Genetic haemochromatosis is a common disorder resulting in increased iron deposition in the liver and other organs but can be difficult to diagnose. The aim of this study was to assess the diagnostic value of the conventional tests for iron overload (percentage saturation of transferrin, serum ferritin and grading of iron staining on liver biopsy) and compare these with the newer quantitative biochemical measurements of liver iron. METHOD: A retrospective analysis was made of 108 consecutive patients referred for quantitative liver iron measurements. Iron studies were obtained in 66 of the 108 subjects of whom 60 had abnormal screening tests defined as percent saturation of transferrin (> 60%) and/or ferritin > 350 micrograms/L for females and > 450 micrograms/L for males. Based on clinical features, biochemical data and treatment outcome these 60 subjects were classified as either genetic haemochromatosis, nongenetic haemochromatosis or indeterminate. One patient with treated genetic haemochromatosis was excluded from subsequent analysis. RESULTS: Although the serum ferritin (p < 0.002), percentage saturation of transferrin (p < 0.001), histological iron grade (p < 0.0001) were significantly higher in the genetic haemochromatosis than nongenetic haemochromatosis group there was considerable overlap. Similarly for the hepatic iron concentration (HIC) (p < 0.0001) overlap occurred. The hepatic iron index (HIC/age) gave the best separation with only three cases being misclassified. A correlation between the HII and histological iron index (visualised iron score corrected for age) in 15 subjects gave an r value of 0.72. CONCLUSION: Based on this study we feel that in addition to visual grading of iron in liver biopsies, the hepatic iron index is helpful in establishing a diagnosis of genetic haemochromatosis.

Adult↗

Treatment of hairy cell leukaemia with recombinant alpha interferon.

Eleven patients with progressive hairy cell leukaemia (three nonsplenectomised) were treated with recombinant alpha-2 interferon (Intron-A or Roferon-A) subcutaneously three times per week at a dosage of 3 x 10(6) units. Ten patients completed at least ten weeks of therapy and could be evaluated; one patient died of haemorrhage from severe thrombocytopenia after only three weeks treatment. Nine of the ten patients responded and all of these are regarded as good partial remissions (normalisation of all blood parameters but still discernible hairy cells in the marrow). Responding patients have all been followed for a median of two years and in one case 3 1/2 years since commencement of therapy. The patients are all transfusion independent and free of infection. We conclude that alpha-2 interferon therapy for progressive hairy cell leukaemia is effective therapy in both splenectomised and nonsplenectomised patients.

Adult↗

Transfusion related HIV infection: the Wellington experience.

A case of transfusion-related AIDS is described which is believed to be the first published case to occur in New Zealand in a nonhaemophiliac patient. The human immunodeficiency virus (HIV) positive donor was shown to be the source of infection in five further HIV positive recipients.

Acquired Immunodeficiency Syndrome↗

Rhinocerebral mucormycosis complicating acute lymphoblastic leukaemia treated successfully: case report.

We report a case of rhinocerebral mucormycosis in a patient with acute lymphoblastic leukaemia undergoing remission induction therapy. The diagnosis was suspected on the characteristic clinical features and confirmed by histology and culture of infected tissue obtained by biopsy. Treatment was successful because of early institution of antifungal therapy, repeated surgical debridement and established of remission in the underlying disease.

Adult↗

Heinz body haemolytic anaemia after sniffing volatile nitrites.

Two cases are reported of Heinz body haemolytic anaemia after the sniffing of amyl nitrite and butyl nitrite for protracted periods. Nitrites are powerful oxidizing agents which are recognized to cause haemoglobin to be oxidized to methaemoglobin. One patient developed a symptomatic anaemia and also splenomegaly. Other possible causes of Heinz body formation were ruled out by the appropriate tests.

Adult↗