Comparing Plasmapheresis plus IVIg with Plasmapheresis plus IVIg plus Rituximab on the Management of Suspicious Antibody-Mediated Acute Rejection in Kidney Transplant Recipients.

BACKGROUND: There is no treatment of choice for the management of acute antibody-mediated rejection (ABMR) in kidney transplant recipients. Plasmapheresis ± intravenous immunoglobulin (IVIg) ± rituximab has been used in different regimens with contradictory results. OBJECTIVE: To compare three regimens of acute ABMR management including plasmapheresis + IVIg ± rituximab in two different rituximab regimens. METHODS: In this prospective, observational study kidney transplant recipients with suspicious ABMR were categorized into three groups. Group 1 patients were treated with plasmapheresis + IVIg. Groups 2 and 3 received weekly rituximab at a dosage of 375 mg/m2 for either 4 doses (group 2 or high dose) or 2 doses (group 3 or low dose) in addition to plasmapheresis + IVIg. RESULTS: 8, 15, and 9 patients were categorized in groups 1, 2, and 3, respectively. There was no difference among the groups in terms of demographic and clinical characteristics of recipients and donors. Although, 1-year graft (37.5%, 60.0%, and 66.7% for groups 1, 2, and 3, respectively; p=0.308) and patients survival (75.0%, 86.7%, and 77.8% for groups 1, 2, and 3, respectively; p=0.730) were not significantly different among studied groups, graft survival was 22%-30% higher in rituximab-treated groups. Estimated glomerular filtration rate at 12th month of follow-up did not differ among groups (56.3±19.6, 57.3±20.6, 48.7±16.1 mL/min/1.73 m2 for groups 1, 2, and 3, respectively; p=0.683). However, kidney function steadily improved over time in rituximab-treated patients. CONCLUSION: Adding high or low doses of rituximab to plasmapheresis + IVIg comparably increased graft survival in suspicious acute ABMR kidney recipients and steadily improved kidney function among survived allografts over time.

Confirmation of genetic homogeneity of syndactyly type 1 in an Iranian family.

Syndactyly type 1 (SD1) is the most common type of syndactyly, inherited in an autosomal dominant fashion and characterized by complete or partial webbings between the third and fourth fingers and/or between the second and third toes. We recently encountered an Iranian family in which 33 members in six generations were affected with SD1. As a locus of SD1 in a German family has recently been assigned to chromosome 2q34-q36, we performed a linkage analysis of the Iranian SD1 in order to know whether the disorder is genetically homogeneous. With the analysis on 15 affected and 16 unaffected persons in the Iranian family, using dinucleotide repeat polymorphisms as markers, we mapped the SD1 locus to 2q34-q36 with a maximum LOD score of 6.92 at a recombination fraction straight theta = 0.00 (penetrance = 1.00) for the D2S2179 locus. The result not only confirmed the gene assignment, but also suggests genetic homogeneity of the disease.

Bardet-Biedl syndrome type 3 in an Iranian family: clinical study and confirmation of disease localization.

Bardet-Biedl syndrome (BBS) is a group of autosomal recessive MCA/MR syndromes characterized by pigmentary retinopathy, postaxial polydactyly, hypogenitalism, obesity, and mental retardation. Five BBS loci have been identified; among them, BBS type 1 (BBS1) and type 3 (BBS3) are most common and most rare, respectively. We encountered an Iranian family that had seven affected members. All patients had a history of mild to severe obesity, but it was reversible in some patients by caloric restriction and exercise. All patients had pigmentary retinopathy, beginning as night blindness in early childhood and progressing toward severe impairment of vision by the end of the second decade. Polydactyly varied in limb distribution, ranging from four-limb involvement to random involvement or even to nonaffectedness. Six of the seven patients were not mentally retarded. Although kidney anomaly or an adrenal mass was pres- ent in two patients, the fact that one patient had seven children rules out reproductive dysfunction. Linkage analysis with microsatellite markers showed that the disease in the family was assigned to a region around marker loci at 3p13-p12 (maximum LOD score = 4.15 and recombination fraction straight theta = 0, at D3S1603 microsatellite marker), to which the BBS3 locus has been mapped. Haplotype analysis did not reduce the extent of the previously reported critical region of BBS3. A comparison of clinical manifestations of our patients with those of previously reported BBS3 patients did not support any type-specific phenotypes, though manifestations in our patients are similar to those in BBS3 patients of a family in Newfoundland.