Membranous Nephropathy-Like Apolipoprotein E Deposition Disease with Apolipoprotein E Toyonaka and Homozygous Apolipoprotein E2/2 without Dyslipidemia, with Characteristic Electron-Dense Deposits.

Recently, several cases of novel apolipoprotein E (apoE)-related glomerular disease known as membranous nephropathy (MN)-like apoE deposition disease with apoE Toyonaka (Ser197Cys) and homozygous apoE2/2 have been reported. However, the clinical and pathological characteristics are uncertain due to the small number of reports. Here, we report an additional case with various clinical and pathological characteristics. A 28-year-old Japanese man with mild proteinuria and hematuria underwent a kidney biopsy. Examination under a light microscope revealed mesangial proliferation, mesangial matrix expansion, and segmental spike lesion. An immunofluorescence study showed no immunoglobulin or complement depositions. In the electron microscopic (EM) examination, massive deposits with various electron densities in the subepithelial, subendothelial, and paramesangial areas were more prominent than those reported in previous cases, which resembled microbubbles or microcysts on higher magnification. The glomerular basement membrane (GBM) structure was partly degenerated by these deposits. Serum triglyceride and cholesterol levels were within the normal range. However, the serum apoE concentration was significantly high, and glomerular apoE accumulation was detected in immunohistochemistry. The DNA sequence revealed apoE Toyonaka and homozygous apoE2/2 similar to that of the previous cases with MN-like apoE deposition disease. MN-like apoE deposition disease can manifest as only mild hematuria and proteinuria without dyslipidemia. Various characteristic deposits associated with GBM degeneration can be observed in the EM study.

Anti-Fya-mediated delayed hemolytic transfusion reaction following emergency-release red blood cell transfusion: possible involvement of HLA-DRB1*04:03 in the Japanese population.

A 43-year-old Japanese male, who had undergone open liver surgery for tumor resection, presented with decreased hemoglobin levels on Day 13 post-emergency-release transfusion of 16 units of Fy(a +) red blood cells. As the anemia was accompanied by increased lactate dehydrogenase, indirect bilirubin, and reticulocytes, as well as decreased haptoglobin, it was attributed to hemolysis. In the diagnostic workup for hemolytic reaction, the direct antiglobulin test result for IgG was positive and the antibody dissociated from the patient's peripheral red blood cells was identified as anti-Fya (titer, 4). The hemolytic reaction was transient (approximately 10 days), of moderate severity, and did not result in any obvious organ damage. However, a single compatible red blood cell transfusion of 2 units was required on Day 17 after the causative transfusion. Notably, HLA typing revealed that the patient carried the HLA-DRB1*04:03 allele, which has been implicated in immunogenicity and induction of anti-Fya response in Caucasian populations. In summary, this is the first documented case of definitive anti-Fya-mediated delayed hemolytic transfusion reaction associated with HLA-DRB1*04:03 in the Japanese population.

Prolonged incubation period of hepatitis B in a recipient of a nucleic acid amplification test-negative hepatitis B virus window donation.

BACKGROUND: The occurrence of transfusion-transmitted hepatitis B virus (HBV) infection has fallen dramatically due to continuous improvements in pre-transfusion laboratory testing. However, the characteristics of transfusion-transmitted HBV infection caused by individual donor nucleic acid amplification test (ID-NAT)-negative blood products are unclear. CASE PRESENTATION: A 76-year-old woman with acute myeloid leukemia was diagnosed with transfusion-transmitted HBV infection after receiving apheresis platelets derived from an ID-NAT-negative blood donation. This case was diagnosed definitively as transfusion-mediated because complete nucleotide homology of a 1556 bp region of the HBV Pol/preS1-preS2-S genes and a 23 bp region of the HBV core promoter/precore between the donor and recipient strains was confirmed by PCR-directed sequencing. The case is uncommon with respect to the unexpectedly prolonged HBV-DNA incubation period of nearly 5 months after transfusion (previously, the longest period observed since the recent implementation of ID-NAT pre-transfusion laboratory testing in Japan was 84 days). Slow-replicating HBV genotype A2 may contribute to the prolonged incubation period; also, the quantity of apheresis platelets delivered in a large volume of plasma, and/or the immune response of the recipient suffering from a hematological neoplasm, may have contributed to establishment of HBV infection in the recipient. This was supported by analysis of three previously documented cases of transfusion-transmitted HBV infection by blood products derived from ID-NAT-negative donations in Japan. CONCLUSION: Continuous monitoring of HBV infection for longer periods (>3 months) may be required after transfusion of blood components from an ID-NAT-negative HBV window donation.

Low-grade myofibroblastic sarcoma of the palm.

We report here a case of an unusual spindle cell tumour of the palm with myofibroblastic differentiation, which was surgically excised. Histologically and immunohistochemically, it was a low-grade myofibroblastic sarcoma. After 25 months follow-up the patient is well and free of recurrence.

Radius lengthening for the treatment of Bayne and Klug type II and type III radial longitudinal deficiency.

PURPOSE: We performed radius lengthening to treat radial deviation of the wrist in patients with Bayne and Klug type II and type III radial longitudinal deficiencies. The purpose of this investigation was to review our results of radius lengthening for radial longitudinal deficiency. METHODS: Beginning in 1991 radius lengthening was performed to treat 4 patients with radial longitudinal deficiency whose mean age at the initial lengthening was 16 months. The corrections for radial deviation of the wrists were performed simultaneously by soft-tissue distraction of the wrist. According to Bayne and Klug's classification 2 patients had type II and 2 patients had type III deficiencies. All but 1 patient had lengthening several times to correct the recurring discrepancy between the radius and the ulna. RESULTS: The corrections were achieved just after the lengthening but deformities recurred because of growth discrepancies between the radius and the ulna as the children grew. The radius was lengthened by a mean of 28 mm, with a mean length gain of 79%. The mean period in the fixator was 136 days. Two patients had both functionally and cosmetically acceptable correction after several lengthening procedures. In the other patient we abandoned this treatment method because of severe bone absorption at the distal end of the lengthened radius. One patient died of cardiac disease after one lengthening. CONCLUSIONS: Radius lengthening for Bayne and Klug type II and type III deficiencies may be accomplished successfully with the primary benefit of maintaining wrist and forearm motion. It is likely that at least 3 lengthenings may be required and this may need to be accompanied by a soft-tissue distraction at the ulnar carpal joint as well. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic, Level IV.

Bone lengthening for congenital differences of the hands and digits in children.

PURPOSE: We investigated the growth of bones after lengthening to evaluate the effectiveness and usefulness of bone lengthening in congenital hand deficiencies. METHODS: Bone lengthening was performed to treat congenital hand deficiencies including hypoplastic thumb, hypoplasia of the small finger, cleft hand, metacarpal synostosis, brachymetacarpia, symbrachydactyly, and amniotic band syndrome. A total of 15 patients (1-13 years old) underwent lengthening of 23 bones. The average of distraction was 10 mm. The mean follow-up period was 59 months. The length of the bones at follow-up evaluation was measured and then compared with the length of the bones immediately after lengthening. The bone growth after lengthening, the percentage of growth after lengthening, and the growth rate were compared among diagnostic groups. RESULTS: In the cases involving older patients the bone length changed little and the growth plates of these patients closed naturally soon after lengthening. In contrast, in the cases in which the patients were younger than 7 years old the lengthened bones grew continuously. The amount of bone growth was different for each disease in the younger patients. In patients with amniotic band syndrome the lengthened bones grew at the same rate as the bones in the hand. Other diagnoses such as symbrachydactyly had a different growth pattern in the hand. CONCLUSIONS: We conclude that bone lengthening is a reliable method for the correction of congenital differences of the hand. The indications for this procedure must be determined while considering the relationship between the specific disease and subsequent bone growth.

Anterior interosseous nerve palsy after reduction and percutaneous pinning of open fractures of the radius and ulna.

We report a case of an anterior interosseous nerve palsy after closed reduction and percutaneous pinning of open fractures of the radius and ulna in an adult. Operative findings showed that the anterior interosseous nerve was trapped between the distal and proximal part of the fractured radius. Treatment by neurorrhaphy gave a satisfactory result.