Iron overload manifesting as apparent exacerbation of hepatic graft-versus-host disease after allogeneic hematopoietic stem cell transplantation.

Iron overload presenting as exacerbation of hepatic graft-versus-host disease (GVHD) after allogeneic hematopoietic stem cell transplantation has not been previously described. We report 6 patients with established hepatic GVHD in whom iron overload (median serum ferritin, 7231 mug/dL; median transferrin saturation, 77%) resulting from a lifetime median of 20 units of packed red blood cell transfusions was manifested by worsening of liver function. Liver biopsies performed in 4 patients confirmed severe iron overload and also hepatic GVHD. Analysis for the C282Y and H63D hemochromatosis gene mutation was negative for the homozygous state in all 6 patients. Erythropoietin-assisted phlebotomy resulted in normalization of liver function at a median of 7 months and of serum ferritin at a median of 11 months. Immunosuppressive therapy was successfully tapered in all 4 patients who completed the phlebotomy program, and this supported the impression that iron overload, rather than GVHD, was the principal cause of liver dysfunction. At a median follow-up of 50 months (range, 18-76 months) from the transplantation and 25 months (range, 5-36 months) from ferritin normalization, all 4 patients require maintenance phlebotomy. We conclude that iron overload can mimic GVHD exacerbation, thus resulting in unnecessary continuation or intensification of immunosuppressive therapy for GVHD, and that maintenance phlebotomy is necessary after successful iron-reduction therapy.     

Left pulmonary artery evaluation through transesophageal echocardiography.

INTRODUCTION: Transesophageal echocardiography (TEE) has become increasingly useful in the study of patients with suspected pulmonary thromboembolism. OBJECTIVE: The aim of this study was to prospectively evaluate the usefulness of TEE in the study of the distal part of the left pulmonary artery (LPA) as well as the influence of this procedure on total echocardiographic exam duration. METHODOLOGY: A prospective study in two groups of consecutive patients referred for TEE with a one- year interval between evaluation of Group A: 33 patients, 17 male, mean age 54 +/- 24 years, and Group B: 42 patients, 20 male, mean age 48 +/- 27 years (p = NS). The procedure was considered long when it took more than 3 min to evaluate the distal part of the LPA. RESULTS: In group A we were able to visualize the distal part of the LPA in 24 patients (73%) without significant prolongation of total exam duration in 16 patients (48% of group A). In one of the patients with suspected pulmonary thromboembolism thrombi were only observed in the distal part of the LPA. In group B we were able to visualize the distal part of the LPA in 36 patients (86%) without significant prolongation of total exam duration in 26 patients (61% of group B). CONCLUSIONS: 1. Visualization of the distal part of the LPA was possible in more patients, and with TEE time prolongation in less patients, in group B. These differences can be accounted for by the training of the operator in this technique. 2. The importance of visualization of this part of the LPA in guiding treatment in the subset of patients with pulmonary thromboembolism confirms the usefulness of this specific procedure.     

Human hepatocellular carcinoma tumor xenografts

Castrated or sham-operated male athymic mice were inoculated with cells from the human hepatocellular carcinoma cell line PLC/PRF/5. There were no significant differences between the two groups with respect to the number of animals developing tumors, the time to tumor development, or the subsequent rate of increase in either tumor base area or mouse serum alpha-fetoprotein concentration. Androgen receptors were assayed in nuclei obtained from three separate liver cancer cell lines and from normal adult human liver. Similar concentrations, ranging from 235 to 550 fmol/mg DNA, of nuclear androgen receptors were detected in all tissues. Low percentages of androgen receptors were retained on DNA-cellulose. Although the presence of receptors implies the potential for metabolic effects of androgens in normal and malignant liver, our in vivostudies suggest that castration does not alter significantly the growth of liver cancer xenografts in athymic mice.     

Improvement of oral colonic lavage with supplemental simethicone

We have noted that colons of patients prepared for colonoscopy with Golytely, a nonabsorbable electrolyte lavage solution, frequently contain foam which may obscure small mucosal lesions. Therefore, a randomized, blinded controlled trial was performed to determine the prevalence of Golytely-induced foam and the effect of supplemental simethicone in decreasing the prevalence of foam. Foam was present in 32% of colons prepared with Golytely alone but in none of the colons prepared with Golytely supplemented with simethicone. In addition, only 5% of colons prepared with supplemental simethicone had residual stool noted at the time of colonoscopy, a significant improvement over the 39% prevalence of residual stool in colons prepared with Golytely alone. Addition of simethicone to Golytely lavage decreases the prevalence of colonic foam and residual stool.     

Clinical relevance of the electrocardiogram in relatives of patients with hypertrophic cardiomyopathy

Eighty-nine first-degree relatives of 22 patients with an established diagnosis of hypertrophic cardiomyopathy underwent electrocardiographic and echocardiographic screening. Scalar electrocardiogram was abnormal in 30/89 (33.7%) relatives. Of these thirty, eleven had definite evidence of hypertrophic cardiomyopathy at echo; one had borderline hypertrophy and was considered neither affected nor unaffected; four had questionable signs of hypertrophy. The remaining 14 relatives had normal echo-cardiograms. Fifty-nine relatives (66.3%) had normal electrocardiograms; at echo 3 were considered to have borderline hypertrophy, 16 had questionable signs of hypertrophy and 40 were normal. In relatives of patients with hypertrophic cardiomyopathy an abnormal electrocardiogram may reflect different morphologic conditions: a real hypertrophic cardiomyopathy or a myocardial hypertrophy of uncertain significance. Furthermore, in these categories of subjects, an abnormal electrocardiogram with normal echo must be considered with caution.