BACKGROUND Autoimmune autonomic ganglionopathy(AAG),formerly known as acute pandysautonomia,is a rare,acquired,antibody-mediated,potentially curable autonomic disorder that presents with diffuse autonomic failure.High...BACKGROUND Autoimmune autonomic ganglionopathy(AAG),formerly known as acute pandysautonomia,is a rare,acquired,antibody-mediated,potentially curable autonomic disorder that presents with diffuse autonomic failure.High levels of anti-ganglionic nicotinic acetylcholine receptor(gAChR)serum antibodies are detected in approximately 50%of AAG cases,confirming the diagnosis.CASE SUMMARY We present the case of a 68-year-old man who developed autonomic failure gradually over a 2-year period.Recently,the patient was unable to stand upright for more than a few seconds before fainting.Additionally,he presented with decreased sweating,dry mouth,urinary retention,early satiety,weight loss,bloating,constipation,and erectile dysfunction.Neurological examination revealed dilated pupils that were unresponsive to light.Deep tendon reflexes were absent or diminished.Serologic evaluation revealed the presence of gAChR autoantibodies.An orthostatic hypotension test yielded a positive result.The patient did not respond to symptomatic therapy,including midodrine,fludrocortisone and atomoxetine.Second-line therapy with immunoadsorption produced a noticeable clinical improvement;however,orthostatic hypotension persisted.Sequential rituximab infusion therapy successfully led to a significant improvement in symptoms.CONCLUSION Our case report supports the benefit of combined immunomodulatory therapy for refractory AAG cases that are unresponsive to single-agent treatment.展开更多
Background: Human immunodeficiency virus (HIV) type 2 infection is predominantly found in West African nations, and approximately 1 - 2 million people are thought to be infected. HIV-associated nephropathy (HIVAN) occ...Background: Human immunodeficiency virus (HIV) type 2 infection is predominantly found in West African nations, and approximately 1 - 2 million people are thought to be infected. HIV-associated nephropathy (HIVAN) occurs in about 7% of patients with HIV-1 infection and is one of the most important causes of end-stage renal disease in this population. The only reported case of HIVAN related to HIV-2 infection was described by Izzedine et al. (2006). Aim: The aim of this paper is to report a case of HIVAN in an HIV-2-infected patient, a rarely described condition. Case presentation: We describe a case of a 40-year-old HIV-2-infected female from Angola hospitalized following a six-month history of fever, fatigue, anorexia and weight loss. Laboratory data revealed anaemia, leukopenia and renal dysfunction with nephrotic range proteinuria. Renal biopsy was performed, revealing findings consistent with HIVAN. Also, a presumed diagnosis of ganglionic tuberculosis was established. Conclusion: The slow progression of HIV-2 disease could explain the low frequency of this condition, however, more studies should be carried out for a better understanding of HIV-2 pathophysiology and its associated complications.展开更多
文摘BACKGROUND Autoimmune autonomic ganglionopathy(AAG),formerly known as acute pandysautonomia,is a rare,acquired,antibody-mediated,potentially curable autonomic disorder that presents with diffuse autonomic failure.High levels of anti-ganglionic nicotinic acetylcholine receptor(gAChR)serum antibodies are detected in approximately 50%of AAG cases,confirming the diagnosis.CASE SUMMARY We present the case of a 68-year-old man who developed autonomic failure gradually over a 2-year period.Recently,the patient was unable to stand upright for more than a few seconds before fainting.Additionally,he presented with decreased sweating,dry mouth,urinary retention,early satiety,weight loss,bloating,constipation,and erectile dysfunction.Neurological examination revealed dilated pupils that were unresponsive to light.Deep tendon reflexes were absent or diminished.Serologic evaluation revealed the presence of gAChR autoantibodies.An orthostatic hypotension test yielded a positive result.The patient did not respond to symptomatic therapy,including midodrine,fludrocortisone and atomoxetine.Second-line therapy with immunoadsorption produced a noticeable clinical improvement;however,orthostatic hypotension persisted.Sequential rituximab infusion therapy successfully led to a significant improvement in symptoms.CONCLUSION Our case report supports the benefit of combined immunomodulatory therapy for refractory AAG cases that are unresponsive to single-agent treatment.
文摘Background: Human immunodeficiency virus (HIV) type 2 infection is predominantly found in West African nations, and approximately 1 - 2 million people are thought to be infected. HIV-associated nephropathy (HIVAN) occurs in about 7% of patients with HIV-1 infection and is one of the most important causes of end-stage renal disease in this population. The only reported case of HIVAN related to HIV-2 infection was described by Izzedine et al. (2006). Aim: The aim of this paper is to report a case of HIVAN in an HIV-2-infected patient, a rarely described condition. Case presentation: We describe a case of a 40-year-old HIV-2-infected female from Angola hospitalized following a six-month history of fever, fatigue, anorexia and weight loss. Laboratory data revealed anaemia, leukopenia and renal dysfunction with nephrotic range proteinuria. Renal biopsy was performed, revealing findings consistent with HIVAN. Also, a presumed diagnosis of ganglionic tuberculosis was established. Conclusion: The slow progression of HIV-2 disease could explain the low frequency of this condition, however, more studies should be carried out for a better understanding of HIV-2 pathophysiology and its associated complications.
