Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report here the cases of 15 children in whom nephrotic syndrome developed, from among 164 children (55% male, 90% black) followed in our acquired immunodeficiency syndrome clinic from 1984 through 1990. Mean age at onset of nephrotic syndrome was 4.9 +/- 2.6 years. Fourteen patients were black and one was Hispanic. Seventy-three percent of our patients with nephrotic syndrome were girls. The mean duration of clinical acquired immunodeficiency syndrome before development of nephrotic syndrome was 1.7 +/- 1.1 years. In eight patients, nephrotic syndrome appeared between 3 and 11 months after intravenous infusions of immune globulin or albumin were administered as part of a research protocol; this incidence (8/47) was higher than the incidence of nephrotic syndrome among those who did not receive intravenous infusions (7/117, p less than 0.05). Tissue for histologic examination was available for 80% of the patients, and histologic examination demonstrated mesangial hypercellularity (5 patients), focal segmental glomerulosclerosis (4 patients), minimal change disease (2 patients), and IgM nephropathy (1 patient). Deposition of one or more immunoglobulins was noted in all but one patient studied with immunofluorescence. Corresponding electron-dense deposits were seen by electron microscopy in 78% of specimens. Prednisone did not induce a remission of nephrotic syndrome in the 13 patients treated, whereas cyclosporine did so in the 3 patients to whom it was administered. Five patients were in the end stage of renal disease within 8 months. Successful maintenance peritoneal dialysis was performed in three patients, but 80% of patients have died of human immunodeficiency virus-related complications; one patient was lost to follow-up. We conclude that immune-complex deposition is consistently seen in children with human immunodeficiency virus-associated nephrotic syndrome. This nephrotic syndrome is resistant to steroid therapy, but we observed a remission of the proteinuria with cyclosporine therapy in three patients. For patients with end-stage renal disease, maintenance peritoneal dialysis may improve the quality of life.
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PMID:Nephrotic syndrome associated with acquired immunodeficiency syndrome in children. 194 75

Prednisone is used to treat a number of complications of the human immunodeficiency virus (HIV) infection, but there is little information about the immunologic consequences of such therapy. We gave prednisone to six boys with hemophilia who were infected with HIV in order to study its effects on their clinical status, serum immunoglobulins, lymphocyte populations, and serum HIV antigen concentrations. Most patients had clinical improvement while taking prednisone; platelet counts increased in five, weight increased in four, and adenopathy abated in two patients during the study. Serum IgG concentrations declined in all patients, but there were no significant changes in serum IgA or IgM concentrations. There was no consistent or significant change in the number of percentage of CD3, CD4, CD8, or CD19 lymphocytes. HIV antigen was present in the serum of one patient, and its concentration did not change during the study. None of the patients became HIV antigen seropositive during the study. These results indicate that prednisone administration was associated with clinical improvement without deleterious immunologic effects when given to children with HIV infection.
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PMID:Effects of prednisone on human immunodeficiency virus infection. 201 24

Five children with positive serology for human immunodeficiency virus (HIV) infection by enzyme-linked immunosorbent assay and Western blot were followed for chronic pulmonary disease. Lung biopsies were performed in all patients, and confirmed the diagnosis of pulmonary lymphoid hyperplasia. All children demonstrated progressive hypoxia and increasing alveolar capillary oxygen gradients over at least 1 year of follow-up. All children were on periodic intravenous gamma globulin treatment for a B-cell defect prior to the initiation of corticosteroid therapy. Prednisone was initially given at a dose of 2 mg/kg daily and was subsequently tapered to an alternate day regimen. All children showed improvement in oxygenation. No deterioration in immune function was noted, and there was no increase in bacterial infection. This study indicates that corticosteroids can successfully reverse the severe hypoxia that may result from pulmonary lymphoid hyperplasia in pediatric AIDS patients.
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PMID:Corticosteroid treatment for pulmonary lymphoid hyperplasia in children with the acquired immune deficiency syndrome. 244 50

Thrombocytopenia is a relatively frequent hematological complication of HIV (human immunodeficiency virus) infection. The incidence of thrombocytopenia in a cohort of 359 homo- or bisexual men with HIV infection was 3%, while it was 9% in a cohort of 321 HIV positive persons with a history of intravenous drug abuse. We followed 42 thrombocytopenic patients prospectively to study the clinical significance of thrombocytopenia in these patients. Thrombocytopenia was significantly more severe in intravenous drug abusers than in homo- or bisexual men: 52% of the drug abusers had thrombocyte counts below 10,000/mm3, compared with only 9% of the homo- or bisexual men. Symptoms of bleeding, almost always harmless skin or mucosal bleeding, were found in 45% of patients with a history of intravenous drug abuse and in 18% of the homo- or bisexual men. Life-threatening bleeding episodes did not occur during a median observation period of approximately one year. Prednisone was the most commonly used drug in symptomatic thrombocytopenia and had demonstrable effect only while being administered. After medication was stopped the thrombocyte counts usually fell to pretreatment values. Our findings suggest that therapy of HIV-associated thrombocytopenia should be reserved for severely symptomatic patients, particularly since this symptom of HIV infection rarely causes serious complications and we do not know the influence of drugs such as corticosteroids on the progression rate of HIV-infection.
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PMID:[HIV-associated thrombocytopenia]. 336 12

A 33 year-old male homosexual infected with human immunodeficiency virus type I developed an asymmetrical and painful neuropathy in the lower limbs. Neuro-muscular biopsy showed a necrotizing vasculitis. There were no clinical features indicative of systemic vasculitis. Prednisone therapy dramatically improved the neuropathy, without adverse effects. Although rare, necrotizing arteritis must be considered in HIV-1 patients with neuropathy, especially in case of mononeuropathy multiplex and when immunodepression is mild or absent, since a successful corticosteroid therapy can be prescribed.
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PMID:[Neuropathy caused by necrotizing vasculitis in HIV-1 infection]. 827 36

Renal transplantation continues to be the goal of therapy for children with end-stage renal disease. Patient age, primary renal disease, psychosocial status, living versus cadaver donor allograft, optimal immunosuppressive therapy, urologic status, and maximization of growth and development must be considered in determining the optimal time for transplantation. Immunizations should be up to date, and the immune status of both the donor and the recipient with regard to Epstein-Barr virus, cytomegalovirus, human immunodeficiency virus, and hepatitis A, B, and C must be known. Prednisone, imuran, cyclosporine, and T cell antibodies remain the mainstay of immunosuppression. However, new therapies, such as FK-506, rapamycin, mofetil, brequinar, leflunomide, and human leukocyte antigen-derived peptides, are under investigation for use in transplantation. Complications, including infection, rejection, and malignancy, continue to be problematic in pediatric renal transplantation. Although patient and graft survival has improved over time, outcomes in pediatric renal transplantation continue to lag behind those in young adults.
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PMID:Progress in renal transplantation for children. 862 Mar 69

Renal transplantation continues to be the goal of therapy for children with end-stage renal disease. Patient age, primary renal disease, psychosocial status, living versus cadaver donor allograft, immunosuppressive therapy, urologic status, and maximization of growth and development must be considered in determining the optimal time for transplantation. Immunizations should be up to date, and the immune status of both the donor and the recipient with regard to Epstein Barr virus (EBV), cytomegalovirus (CMV), varicella, human immunodeficiency virus (HIV) and Hepatitis A, B, and C must be known. Prednisone; cyclosporine or tacrolimus; and mycophenolate mofetil or azathioprine remain the mainstays of immunosuppression. However, new therapies such as sirolimus are under investigation for use in pediatric renal transplantation. Induction therapies include T-cell antibodies as well as the more recent addition of interleukin-2 receptor blockers. Complications including infection, rejection, and malignancy continue to be problematic in pediatric renal transplantation. There continues to be a strong focus on optimizing growth and development after transplant. Although patient and graft survival have improved over time, outcomes in pediatric renal transplantation continue to lag behind those in adults.
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PMID:Progress in renal transplantation for children. 1078 34

Pityriasis lichenoides (PL) is a cutaneous disease of unknown origin. In our 8-year-old female patient with the granulomatous form of common variable immunodeficiency (CVID), PL occurred together with massive splenomegaly and intra-abdominal lymphadenopathy. Prednisone was efficient for treatment of her splenomegaly and autoimmune cytopenias. However, PL was resistant to both topical and systemic steroid treatment. Healing of PL was achieved with the use of a super-potent topical steroid, clobetasol propionate. A defect of T-cell function in CVID may contribute to development of PL. In the granulomatous form of CVID, sarcoid-like granulomas are the most commonly reported cutaneous lesions. PL has not been previously reported.
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PMID:Pityriasis lichenoides in a girl with the granulomatous form of common variable immunodeficiency. 1186 May 73

Epstein-Barr virus (EBV)-associated lymphoproliferative disease occurs almost exclusively in immunocompromised states. This type of malignancy nevertheless developed in a woman without overt immunocompromise. She presented with rapidly progressive lower extremity weakness that was consistent with a chronic inflammatory demyelinating polyneuropathy (CIDP). CIDP has been documented in Hodgkin's disease but rarely in non-Hodgkin's disease. Diagnosis was confirmed by lymph node and sural nerve biopsies and by nerve conduction studies. Prednisone, rituximab, cyclophosphamide, doxorubicin, and etoposide were administered with regression of lymphadenopathy and improvement in neurologic symptoms. EBV-associated lymphoproliferative disease can thus develop in the absence of overt immunodeficiency and may trigger a demyelinating polyneuropathy.
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PMID:EBV-associated lymphoma and chronic inflammatory demyelinating polyneuropathy in an adult without overt immunodeficiency. 1192 Oct 25

A 48-year-old man with a history of sarcoidosis was transferred to the Mayo Clinic for evaluation and management of progressive neurologic decline. Two years before admission, he was admitted to a local hospital with mental status changes accompanied by ataxia and severe headache. A diagnosis of pulmonary and central nervous system sarcoidosis was made based on computed tomography of the head, lumbar puncture, and chest radiography. A mediastinoscopy with lymph node biopsy exhibited noncaseating granulomas and negative stains for microorganisms. Prednisone therapy was initiated at 80 mg/day. Clinical improvement was apparent for 13 months during steroid therapy until the slow taper reached a dosage of 20 mg/day. At that time, the patient was readmitted to the local hospital with severe confusion and skin lesions. When intravenous methylprednisolone therapy for presumed central nervous system sarcoidosis did not improve the patient's mental status, he was transferred to the Mayo Clinic. Physical examination of the thighs revealed large, well-marginated, indurated, irregularly bordered, violaceous plaques and rare, umbilicated, satellite papules with central hemorrhagic crusts (Fig. 1A). Superficially ulcerated plaques with a similar appearance to the thigh lesions were coalescing around the lower legs (Fig. 1B). A skin biopsy specimen of the thigh demonstrated abundant numbers of encapsulated organisms and minimal inflammatory response (Fig. 2). Skin, blood, and cerebrospinal fluid cultures confirmed the presence of Cryptococcus neoformans. Amphotericin and flucytosine combination therapy was initiated, and steroid dosages were gradually tapered. A test for human immunodeficiency virus was negative. The patient was dismissed from hospital after a complicated 2-month course resulting in improved mental status but progression of the lower extremity ulcerations as a result of polymicrobial infection.
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PMID:Cryptococcal infection in sarcoidosis. 1245 1


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