scholarly journals Recurrent Focal Segmental Glomerulosclerosis in Renal Allograft Recipients: Role of Human Leukocyte Antigen Mismatching and Other Clinical Variables

2011 ◽  
Vol 2011 ◽  
pp. 1-9 ◽  
Author(s):  
Shimi Sharief ◽  
Shefali Mahesh ◽  
Marcela Del Rio ◽  
Vivian Telis ◽  
Robert P. Woroniecki
Keyword(s):  
Focal Segmental Glomerulosclerosis ◽  
Pediatric Population ◽  
Surrogate Marker ◽  
Human Leukocyte ◽  
Leukocyte Antigen ◽  
Clinical Variables ◽  
Segmental Glomerulosclerosis ◽  
Hla Compatibility

Recurrence of focal segmental glomerulosclerosis (FSGS) after renal transplantation impacts long-term graft survival and limits access to transplantation. We hypothesized that HLA donor/recipient matching could be used as a surrogate marker of recurrence. In a retrospective study of 42 pediatric and 77 adult subjects with primary FSGS, transplanted from 1990 to 2007 at a single center, we analyzed the degree of donor/recipient HLA compatibility and other clinical variables associated with FSGS recurrence. There were total of 131 allografts for primary FSGS (11 subjects were transplanted twice, and 1 had a third allograft) with 20 cases of FSGS recurrence (17 children) in the primary allograft, and two children who had FSGS recurrence in the second allograft. Fifty-two subjects (40%) were African American, and 66 (50%) Caucasians. Recurrent FSGS and controls were not different for age at transplant, gender, donor source, acute/chronic rejection episodes, and HLA matches. Recurrent FSGS was not associated with HLA mismatches; power equals 83%. Immunosuppressive regimen had no effect on recurrence of FSGS, . Recurrent FSGS is not associated with HLA mismatching, acute cellular or vascular rejection, and occurs primarily in the pediatric population.

scholarly journals Transplantation for bone marrow failure: current issues

Hematology ◽  
2016 ◽  
Vol 2016 (1) ◽  
pp. 90-98 ◽  
Author(s):  
Régis Peffault de Latour
Keyword(s):  
Bone Marrow Failure ◽  
Clonal Evolution ◽  
Human Leukocyte ◽  
Unrelated Donor ◽  
Hematopoietic Stem ◽  
Leukocyte Antigen ◽  
Sibling Donor ◽  
Alternative Donor

Abstract The preferred treatment of idiopathic aplastic anemia (AA) is allogeneic hematopoietic stem cell transplantation (HSCT) from a human leukocyte antigen (HLA)–identical sibling donor. Transplantation from a well-matched unrelated donor (MUD) may be considered for patients without a sibling donor after failure of immunosuppressive therapy, as may alternative transplantation (mismatched, cord blood or haplo-identical HSCT) for patients without a MUD. HSCT may also be contemplated for congenital disorders in cases of pancytopenia or severe isolated cytopenia. Currently, HSCT aims are not only to cure patients but also to avoid long-term complications, notably chronic graft-versus-host disease (GVHD), essential for a good quality of life long term. This paper summarizes recent advances in HSCT for idiopathic and inherited AA disorders. The effect of age on current transplantation outcomes, the role of transplantation in paroxysmal nocturnal hemoglobinuria, and the prevention of GVHD are also discussed. Emerging strategies regarding the role of up-front unrelated donor and alternative donor HSCT in idiopathic AA, along with advances in the treatment of clonal evolution in Fanconi anemia, are also examined.

scholarly journals Nonmyeloablative allografting for newly diagnosed multiple myeloma: the experience of the Gruppo Italiano Trapianti di Midollo

Blood ◽  
2009 ◽  
Vol 113 (14) ◽  
pp. 3375-3382 ◽  
Author(s):  
Benedetto Bruno ◽  
Marcello Rotta ◽  
Francesca Patriarca ◽  
Daniele Mattei ◽  
Bernardino Allione ◽  
...  
Keyword(s):  
Chronic Gvhd ◽  
Human Leukocyte ◽  
Response Duration ◽  
Tumor Burden ◽  
New Drugs ◽  
Newly Diagnosed ◽  
Leukocyte Antigen ◽  
Total Body

Abstract Despite recent advances, allografting remains the only potential cure for myeloma. From July 1999 to June 2005, 100 newly diagnosed patients younger than 65 years were enrolled in a prospective multicenter study. First-line treatment included vincristin, adriamycin, and dexamethasone (VAD)–based induction chemotherapy, a cytoreductive autograft (melphalan 200 mg/m2) followed by a single dose of nonmyeloablative total body irradiation and allografting from an human leukocyte antigen (HLA)–identical sibling. Primary end points were the overall survival (OS) and event-free survival (EFS) from diagnosis. After a median follow-up of 5 years, OS was not reached, and EFS was 37 months. Incidences of acute and chronic graft-versus-host disease (GVHD) were 38% and 50%, respectively. Complete remission (CR) was achieved in 53% of patients. Profound cytoreduction (CR or very good partial remission) before allografting was associated with achievement of posttransplantation CR (hazard ratio [HR] 2.20, P = .03) and longer EFS (HR 0.33, P < .01). Conversely, development of chronic GVHD was not correlated with CR or response duration. This tandem transplantation approach allows prolonged survival and long-term disease control in patients with reduced tumor burden at the time of allografting. We are currently investigating the role of “new drugs” in intensifying pretransplantation cytoreduction and posttransplantation graft-versus-myeloma effects to further improve clinical outcomes. (http://ClinicalTrials.gov; NCT-00702247.)

scholarly journals The Role of Molecular Typing for DQ2 and DQ8 Alleles Using Polymerase Chain Reaction Amplification in Children with Autoimmune Conditions

2020 ◽  
Vol 71 (6) ◽  
pp. 212-221
Author(s):  
Oana Belei ◽  
Andreea Dobrescu ◽  
Emil Radu Iacob ◽  
Daniela Iacob ◽  
Elena Amaricai ◽  
...  
Keyword(s):  
Polymerase Chain Reaction Amplification ◽  
Pediatric Population ◽  
Human Leukocyte ◽  
Intestinal Biopsy ◽  
Leukocyte Antigen ◽  
High Prevalence ◽  
Autoimmune Conditions ◽  
Reaction Amplification ◽  
Serologic Screening

The aim was to determine the prevalence of celiac disease (CD) in a pediatric population with juvenile idiopathic arthritis (JIA) and autoimmune hepatitis (AIH) compared to controls and to evaluate the clinical forms and human leukocyte antigen (HLA) alelles. Between September 2009-November 2019, 74 pediatric patients with JIA (1), 62 AIH (2) and 60 controls were assessed for CD. All children with one or more positive CD antibodies were submitted to gastrointestinal endoscopy with intestinal biopsy. All patients underwent HLA molecular assessment for DQ2/DQ8 alleles. Celiac prevalence after screening was 6.7% in the first group, 6.4% in the second group and 0% among controls. The results didn�t reveal significant differences regarding the CD prevalence among patients with JIA and AIH (p = 0.94). The majority of cases associated the silent form of disease (77.7). DQ2/DQ8 haplotypes were found in all CD cases. Of 69 children with JIA and no CD, three (4.3%) had DQ2 haplotype. Of 58 patients with AIH and no CD, 37 children (63.8%) presented DQ2/DQ8. According to the high prevalence obtained in this study, JIA and AIH are good parameters for stratification of asymptomatic cases in order to perform CD serologic screening. The absence of DQ2 or DQ8 haplotypes will make CD diagnosis unlikely and no further tests will be required.

scholarly journals Successful long-term management of recurrent focal segmental glomerulosclerosis after kidney transplantation with costimulation blockade

2020 ◽  
Author(s):  
Thomas Mühlbacher ◽  
Kerstin Amann ◽  
Moritz Mahling ◽  
Silvio Nadalin ◽  
Nils Heyne ◽  
...  
Keyword(s):  
Kidney Transplantation ◽  
Focal Segmental Glomerulosclerosis ◽  
Costimulation Blockade ◽  
Segmental Glomerulosclerosis ◽  
Remission Maintenance ◽  
Maintenance Immunosuppression ◽  
Therapeutic Concepts ◽  
Allograft Outcome

Abstract Recurrence of primary focal segmental glomerulosclerosis (FSGS) occurs in up to 50% of patients after kidney transplantation and is associated with poor allograft outcome. Novel therapeutic concepts directly target podocyte function via B7-1 with inconsistent response. We present the case of a 19 yr. old patient with recurrent primary FSGS early after living donor kidney transplantation. Plasmapheresis and rituximab did not induce remission. Repetitive abatacept administration was able to achieve partial remission. Maintenance immunosuppression was subsequently switched to a belatacept-based CNI-free immunosuppression, resulting in sustained complete remission with excellent allograft function throughout a follow-up of more than 56 months.

Narcolepsy: Differential Diagnosis or Etiology in Some Cases of Bipolar Disorder and Schizophrenia?

CNS Spectrums ◽  
2003 ◽  
Vol 8 (2) ◽  
pp. 120-126 ◽  
Author(s):  
Alan B. Douglass
Keyword(s):  
Bipolar Disorder ◽  
Differential Diagnosis ◽  
Psychiatric Patients ◽  
Human Leukocyte ◽  
Sleep Paralysis ◽  
Leukocyte Antigen ◽  
Psychotic Features ◽  
Hypnagogic Hallucinations ◽  
Rule Out

AbstractDoes narcolepsy, a neurological disease, need to be considered when diagnosing major mental illness? Clinicians have reported cases of narcolepsy with prominent hypnagogic hallucinations that were mistakenly diagnosed as schizophrenia. In some bipolar disorder patients with narcolepsy, the HH resulted in their receiving a more severe diagnosis (ie, bipolar disorder with psychotic features or schizoaffective disorder). The role of narcolepsy in psychiatric patients has remained obscure and problematic, and it may be more prevalent than commonly believed. Classical narcolepsy patients display the clinical “tetrad”—cataplexy, hypnagogic hallucinations, daytime sleep attacks, and sleep paralysis. Over 85% also display the human leukocyte antigen marker DQB10602 (subset of DQ6). Since 1998, discoveries in neuroanatomy and neurophysiology have greatly advanced the understanding of narcolepsy, which involves a nearly total loss of the recently discovered orexin/hypocretin (hypocretin) neurons of the hypothalamus, likely by an autoimmune mechanism. Hypocretin neurons normally supply excitatory signals to brainstem nuclei producing norepinephrine, serotonin, histamine, and dopamine, with resultant suppression of sleep. They also project to basal forebrain areas and cortex. A literature review regarding the differential diagnosis of narcolepsy, affective disorder, and schizophrenia is presented. Furthermore, it is now possible to rule out classical narcolepsy in difficult psychiatric cases. Surprisingly, psychotic patients with narcolepsy will likely require stimulants to fully recover. Many conventional antipsychotic drugs would worsen their symptoms and make them appear to become a “chronic psychotic,” while in fact they can now be properly diagnosed and treated.

scholarly journals PROTEINURIA IN FOCAL SEGMENTAL GLOMERULOSCLEROSIS: ROLE OF CIRCULATING FACTORS AND THERAPEUTIC APPROACH

Renal Failure ◽  
2001 ◽  
Vol 23 (3-4) ◽  
pp. 533-541 ◽  
Author(s):  
Luigi Moriconi ◽  
Ciro Lenti ◽  
Rodolfo Puccini ◽  
Antonio Pasquariello ◽  
Paolo Rindi ◽  
...  
Keyword(s):  
Focal Segmental Glomerulosclerosis ◽  
Therapeutic Approach ◽  
Segmental Glomerulosclerosis
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