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O'Donnell-Pappas Syndrome


Presentation

  • Treatment Treatment for mycobacterial-associated IRIS depends on the presentation and disease severity. Most patients present with non-life threatening presentations which respond to the institution of appropriate antituberculous therapy.[aidsrestherapy.biomedcentral.com]
  • Minor cardiac anomalies and transient bradycardia and apnea may be present. Affected individuals may experience recurrent infections, which also tend to improve with age.[ncbi.nlm.nih.gov]
Lymphadenopathy
Pleural Effusion
Respiratory Distress
  • However a range of life threatening presentations, such as acute renal failure [ 6 ] and acute respiratory distress syndrome (ARDS) [ 11 ], are described and have significant morbidity and mortality.[aidsrestherapy.biomedcentral.com]
Jaundice
  • Abdominal TB-IRIS can present with nonspecific abdominal pain and obstructive jaundice. In most studies, TB-IRIS occurs within two months of ART initiation [ 6, 7, 10, 11, 25, 35, 45, 48 ].[aidsrestherapy.biomedcentral.com]

Workup

Mycobacterium Avium Complex
  • Examples of IRIS in response to intact organisms include, but are not limited to, the unmasking of latent cryptococcal infection [ 22 ] and infection with Mycobacterium avium complex (MAC) [ 4, 5, 23, 24 ].[aidsrestherapy.biomedcentral.com]
Pleural Effusion

Treatment

  • Otherwise, treatment is similar to TB-IRIS (see Mycobacterium tuberculosis IRIS – Treatment).[aidsrestherapy.biomedcentral.com]
  • Treatment of manifestations. Cyclic vomiting may be managed by identification and avoidance of triggers, daily maintenance medication, and early abortive treatment; G-tubes or GJ-tubes may decrease aspiration and improve nutrition.[ncbi.nlm.nih.gov]

Prognosis

  • Somatic ASXL1 pathogenic variants are known to be associated with a worse prognosis in persons with myeloid leukemia [ Abdel-Wahab et al 2012 ].[ncbi.nlm.nih.gov]

Etiology

  • Other etiologies Other less common infectious etiologies, as well as noninfectious etiologies, are listed in Table 1. Because these other infectious and non-infectious etiologies are rare, no recommendations exist for their management.[aidsrestherapy.biomedcentral.com]
  • The mode of inheritance is also unclear since the molecular etiology remains elusive.[ncbi.nlm.nih.gov]

Epidemiology

  • Atypical mycobacterial IRIS Epidemiology In addition to TB, atypical mycobacteria are also frequently reported as causative pathogens in IRIS.[aidsrestherapy.biomedcentral.com]
  • Isolated case reports suggest that individuals with BOS are at greater risk for Wilms tumor than the general population, but large-scale epidemiologic studies have not been conducted. Management. Treatment of manifestations.[ncbi.nlm.nih.gov]
Sex distribution
Age distribution

Prevention

  • Prevention of Primary Manifestations Adequate treatment of severe emesis can decrease hospitalizations, infectious exposures, and aspiration.[ncbi.nlm.nih.gov]
  • Results of trials addressing the optimal timing and duration of treatment of opportunistic infections will assist in developing guidelines for the prevention and management of IRIS.[aidsrestherapy.biomedcentral.com]

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