Tatsuo Fukushima

Zinc deficiency anemia and effects of zinc therapy in maintenance hemodialysis patients.

Quantitative adjuvant zinc therapy using polaprezinc was performed to examine the correlation between zinc concentration and anemia in maintenance hemodialysis patients to propose appropriate treatment. Anemia and serum zinc concentration were measured in 117 patients with chronic renal failure receiving outpatient maintenance hemodialysis at Tsuyama Chuo Kinen Hospital. Two bags of polaprezinc (containing zinc 34 mg/day) were administered to 58 patients with lower than normal zinc levels (Zn < 80 mg/dl) as adjuvant zinc therapy to assess anemia improvement. Zinc concentration and all anemia parameters showed significant positive correlation, indicating that anemia improves in patients with high serum zinc levels. Regarding the effects of adjuvant zinc therapy for improving anemia, hemoglobin levels were found to increase significantly to the highest value at 3 weeks. During treatment, the dosage of erythropoietin was reduced significantly from baseline at all assessment points. No zinc poisoning from therapy was seen, but two patients had diarrhea (1.9%). Zinc‐treated patients required iron therapy due to the development of iron deficiency. Most maintenance hemodialysis patients suffer from zinc deficiency anemia, and zinc‐based polaprezinc has been confirmed to be an effective and safe adjuvant zinc treatment. Most patients diagnosed as refractory anemia with no response to erythropoietin also suffer from zinc deficiency anemia, many of whom are expected to benefit from zinc therapy to improve their anemia. Possible zinc deficiency anemia should be considered in the treatment of refractory anemia with no response to erythropoietin.

A nosocomial parvovirus B19 infection-induced transient aplastic crisis in a patient with chronic renal failure.

A male patient aged 67 years with chronic renal failure (CRF), who had undergone hemodialysis since June 3, complained of dyspnea while walking on June 23, 1998. Rapidly progressive anemia and severe reticulocytopenia were noted. Serological tests showed that parvovirus B19- (B19) specific IgM antibody, but not IgG antibody, was present in the patients serum. B19 DNA was detected in the patients serum by the polymerase chain reaction (PCR). Therefore, a definite diagnosis of transient aplastic crisis induced by B19 was made. On June 10, prior to the appearance of this case, a female nurse aged 27 years working in our hemodialysis center, complained of cough, fever and arthralgia. Another female nurse, aged 35 years, developed similar symptoms on July 3. Both nurses had a positive IgM titer against B19, but were negative for IgG, indicating an acute B19 infection. These findings led us to suspect that this series of B19 infection was spread by nosocomial transmission. Although some cases of B19 infection have been reported to occur in laboratory staffs, the B19 nosocomial infection has not been described in the literature. We also suggest that a transient aplastic crisis due to B19 infection could lead to severe anemia in cases of CRF whose erythropoiesis is maintained by a recombinant human erythropoietin.

A Single-Base Mutation in Exon 31 Converting Glycine 852 to Arginine in the Collagenous Domain in an Alport Syndrome Patient

In a family with Alport syndrome, molecular analysis of the COL4A5 gene, which encodes the alpha 5(IV) chain of glomerular basement membrane collagen, revealed a GGA-->AGA change in exon 31, resulting in substitution of an arginine for a glycine in position 852 of the polypeptide chain, between interruptions 16 and 17 of the triple-helical collagenous domain. The mutation causes the MaeI restriction sites, and could be easily diagnosed in the family members through restriction analysis. This one point mutation can be expected to interrupt type IV collagen molecules.

THE ATTITUDE OF JAPANESE PHYSICIANS REGARDING GENETIC SERVICE FOR AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE (ADPKD)

SummaryA questionnaire carried out among Japanese physicians revealed that a strong demand for genetic counseling among patients and families with autosomal dominant polycystic kidney disease (ADPKD). In contrast, the awareness of physicians regarding genetic counseling for the disease seemed to be low. For example, 66.0% of the respondents to the questionnaire revealed a negative attitude to providing genetic counseling for patients, and 30.7% of the respondents did not know that most types of polycystic kidney disease are inherited disorders. With the advance of scientific research, the demand for genetic counseling among patients is bound to increase. Therefore, the providers of genetic counseling including the physicians are now pressed to improve their services.

IgA Nephropathy, Consanguinity and Hypertension

Shinsuke Nomura, MD, Lecturer, Nephrology Division, Department of Medicine, Kawasaki Medical School, Kurashiki, Okayama, 701-01 (Japan) Dear Sir, The cause and pathogenesis of the most common glomerular disease, IgA nephropathy, are not clearly understood. It had been assumed that IgA nephropathy develops randomly in individuals. However, a possible genetic influence in its pathogenesis has been recently suggested because of some clinical aspects; e.g., the association of specific HLA antigen with the development of the disease, regional and racial differences in its prevalence, and clustering of the disease in some families [1]. Therefore, it seems likely that the possible genetic influence in its pathogenesis may be responsible for the development of the disease in susceptible individuals. If a gene or genes considered to be associated with genetic susceptibility to IgA nephropathy exist, the phenotype(s) derived from the gene(s) may possibly be in evidence in patients afflicted with IgA nephropathy who are products of consanguineous mat-ings. Therefore, we are interested in the characteristic signs and findings found in these patients in this investigation to attempt to explore the hypothesized gene(s). Of 162 patients with IgA nephropathy who were followed up at our outpatient clinic, we identified 6 patients (3.7%) who were products of consanguineous matings using a questionnaire. Interestingly, it was revealed that 4 of these 6 developed hypertension during the early course of the disease, by a retrospective review of patients’ records. Two of the 4 had malignant hypertension and had lost renal function at age 28 and 41, respectively. Neither proteinuria and/or hematuria of all 6 patients have been eliminated since the diagnosis of the disease. However, these patients could not be distinguished from the other 156 patients by histo-logical or laboratory examinations. The 6 inbred patients may offer some clues to investigate the hypothesized gene(s). First, these findings may indicate that the inbred IgA nephropathy patients are vulnerable to hypertension. It may be possible to speculate that our patients whose parents were consanguineous mates might have been homozygotes of a susceptible gene for hypertension, or that the hypothesized gene(s) associated with genetic susceptibility to IgA nephropathy might have also played a role in the development of hypertension.