scholarly journals Late Onset Combined Immunodeficiency Presenting with RecurrentPneumocystis jiroveciPneumonia

2014 ◽  
Vol 2014 ◽  
pp. 1-7 ◽  
Author(s):  
Ilias Papakonstantinou ◽  
Ioannis G. Baraboutis ◽  
Lazaros Karnesis

Late onset combined immunodeficiency (LOCID) is a recently described variant of common variable immunodeficiency (CVID), involving adult patients presenting with opportunistic infections and/or low CD4+ lymphocyte counts. A 36-year-old male with unremarkable past medical history presented with fever, respiratory failure, and lymphocytopenia. He was found to havePneumocystis jirovecipneumonia (PJP), subsequently complicated by recurrent hospital-acquiredPseudomonas aeruginosapneumonia and immune reconstitution phenomena, attributed to restoration of immunoglobulin levels. Clinicians should be aware of LOCID, which could be confused with HIV infection/AIDS or idiopathic CD4 lymphocytopenia. In the English bibliography there is only one case report, where PJP was the initial presentation of CVID (that case would probably be classified as LOCID). Phenomena of immune reconstitution are described in various settings, including primary immunodeficiency, manifesting as temporary clinical and radiologic deterioration and leading to misperceptions of therapeutic failure and/or presence of alternative/additional diagnoses.

2019 ◽  
Author(s):  
Noureddine Sakhri ◽  
Fatima Zahra Meski ◽  
SOUMIA TRIKI

BACKGROUND Morbidity and mortality in HIV disease is due to immune-suppression leading to life-threatening opportunistic infections (OIs) during the natural course of the disease. In 2015, the HIV prevalence is low in general population and concentrated among key populations. OBJECTIVE This study aimed to assess the prevalence and CD4 correlates of OIs among adult HIV-infected patients attending antiretroviral health care in Morocco, during 2015. METHODS We conducted a cross-sectional survey among all adult PLHIV for admitted in the health care centers during 2015, who had acquired infection disease. Patients’ opportunistic infection status was determined through clinical diagnosis and laboratory investigations. CD4 count was determined using flow cytometry technique. The clinical stage of HIV was identified by the classification of Centers for Disease Control and Prevention (CDC). We collected Socio-demographic and clinical data from patients’ medical records. We performed statistical analysis by using Epi-Info 7.2.0.1 software. The appropriate test was applied, bivariate analysis was made and the differences were significant when p<.05. RESULTS 299 HIV-infected cases were included; 53% were males. The most represented age group was 25-34 years (36.1%). The mean age of the cases was 38.7 ± 16.8. The prevalence of OIs was 47.8%. Tuberculosis (65/299, 21.7%), Pneumocystis jiroveci pneumonia (40/299, 13.4%) and Oral candidiasis (22/299, 7.4%) were the most frequently observed OIs. CONCLUSIONS Tuberculosis, pneumocystis and oral candidacies were the leading OIs, encountered by HIV-infected cases. Preventive measures and early diagnosis of HIV associated to OIs are crucial.


2020 ◽  
Vol 7 (Supplement_1) ◽  
pp. S585-S585
Author(s):  
Harry Cheung ◽  
Marwan M Azar ◽  
Geliang Gan ◽  
Yanhong Deng ◽  
Elizabeth A Cohen ◽  
...  

Abstract Background Kidney transplant recipients (KTR) are at increased risk for infections immediately post-transplant due to intense immunosuppression. However, this risk decreases over time as immunosuppression is tapered. The incidence of infection in KTR many years after transplant is not well characterized. The aim of this study was to describe these “very-late onset infections” (VLIs) ≥ 10 years after KT. Methods We performed a retrospective chart review of patients age ≥ 18 years who underwent KT between 2003 and 2009 and who survived ≥ 10 years post-KT. VLIs included opportunistic infections (OIs) and non-OIs. Demographics, comorbidities, immunosuppression, and clinical data for VLIs ≥ 10 years from KT were collected. Simple logistic regression was performed to determine characteristics associated with risk for VLIs. Results Of 332 KTR that met the inclusion criteria, the majority were male (62.0%), white (59.6%), and the largest proportion was transplanted between the ages of 50-59 (28.3%); 220 (67.9%) were on mycophenolate-based regimens. The mean Charlson Comorbidity Index (CCI) was 4.7 (S.D. 2.0). Of 332, 103 (31.0%) KTR experienced ≥ 1 VLI amounting to 187 episodes. Compared to those without VLI, KTR with VLI were more likely to have diabetes (p=0.005), cardiovascular disease (p=0.004), low ALC (p &lt; 0.001) and require dialysis (p=0.002). Of 103 KTR with VLI, 16 (15.5%) had OIs, while 87 KTR (84.5%) had non-OIs, most commonly urinary tract infection (n=85, 45.5%), pneumonia (n=35, 18.7%) and gastrointestinal infection (n=18, 9.6%). The most commonly isolated pathogens were E. coli (n=30, 16%), K. pneumoniae (n=16, 8.6%), MSSA (n=7, 3.7%), and P. aeruginosa (n=7, 3.7%). Higher CCI, diabetes, dialysis, cerebrovascular, cardiovascular disease and lower ALC were associated with increased risk for VLI (p &lt; 0.05), while living donor KTR was protective (p=0.04). Additionally, every 1 year after transplant was associated with an increased risk of VLI (OR=1.31, p &lt; 0.001). Table 1: Demographics, comorbidities, immunosuppression, and clinical data for all patients Conclusion VLIs were common in long-term survivors of KT and included both conventional and opportunistic pathogens. Every additional year from transplant incurred additional risk for VLI, particularly for those with multiple co-morbidities and lower ALC. Disclosures All Authors: No reported disclosures


2021 ◽  
Vol 14 ◽  
pp. 175628642110355
Author(s):  
Maike F. Dohrn ◽  
Gisa Ellrichmann ◽  
Rastislav Pjontek ◽  
Carsten Lukas ◽  
Jens Panse ◽  
...  

Progressive multifocal leukoencephalopathy (PML) is a subacute brain infection by the opportunistic John Cunningham (JC) virus. Herein, we describe seven patients with PML, lymphopenia, and sarcoidosis, in three of whom PML was the first manifestation of sarcoidosis. At onset, the clinical picture comprised rapidly progressive spastic hemi- or limb pareses as well as disturbances of vision, speech, and orientation. Cerebral magnetic resonance imaging showed T2-hyperintense, confluent, mainly supratentorial lesions. Four patients developed punctate contrast enhancement as a radiological sign of an immune reconstitution inflammatory syndrome (IRIS), three of them having a fatal course. In the cerebrospinal fluid, the initial JC virus load (8–25,787 copies/ml) did not correlate with interindividual severity; however, virus load corresponded to clinical dynamics. Brain biopsies ( n = 2), performed 2 months after symptom onset, showed spotted demyelination and microglial activation. All patients had lymphopenia in the range of 270–1150/µl. To control JC virus, three patients received a combination of mirtazapine and mefloquine, another two patients additionally took cidofovir. One patient was treated with cidofovir only, and one patient had a combined regimen with mirtazapine, mefloquine, cidofovir, intravenous interleukin 2, and JC capsid vaccination. To treat sarcoidosis, the four previously untreated patients received prednisolone. Three patients had taken immunosuppressants prior to PML onset, which were subsequently stopped as a potential accelerator of opportunistic infections. After 6–54 months of follow up, three patients reached an incomplete recovery, one patient progressed, but survived so far, and two patients died. One further patient was additionally diagnosed with lung cancer, which he died from after 24 months. We conclude that the combination of PML and sarcoidosis is a diagnostic and therapeutic challenge. PML can occur as the first sign of sarcoidosis without preceding immunosuppressive treatment. The development of IRIS might be an indicator of poor outcome.


Author(s):  
Mio Sakai ◽  
Masahiro Higashi ◽  
Takuya Fujiwara ◽  
Tomoko Uehira ◽  
Takuma Shirasaka ◽  
...  

AbstractWith the advent of antiretroviral therapy (ART), the prognosis of people infected with human immunodeficiency virus (HIV) has improved, and the frequency of HIV-related central nervous system (CNS) diseases has decreased. Nevertheless, mortality from HIV-related CNS diseases, including those associated with ART (e.g., immune reconstitution inflammatory syndrome) remains significant. Magnetic resonance imaging (MRI) can improve the outlook for people with HIV through early diagnosis and prompt treatment. For example, HIV encephalopathy shows a diffuse bilateral pattern, whereas progressive multifocal leukoencephalopathy, HIV-related primary CNS lymphoma, and CNS toxoplasmosis show focal patterns on MRI. Among the other diseases caused by opportunistic infections, CNS cryptococcosis and CNS tuberculosis have extremely poor prognoses unless diagnosed early. Immune reconstitution inflammatory syndrome shows distinct MRI findings from the offending opportunistic infections. Although distinguishing between HIV-related CNS diseases based on imaging alone is difficult, in this review, we discuss how pattern recognition approaches can contribute to their early differentiation.


2018 ◽  
pp. bcr-2018-225234
Author(s):  
Jasmine Sethi ◽  
Raja Ramachandran ◽  
Harbir Singh Kohli ◽  
Krishan Lal Gupta

Idiopathic CD4 lymphocytopenia (ICL) is characterised by a low CD4 +lymphocyte count in the absence of HIV or other underlying aetiologies. We report a case of a 17-year-old girl with ICL with autoimmune hepatitis who developed isolated renal mucormycosis, which, to our knowledge, is the first reported case described in literature. Combination therapy with antifungals and surgical resection was done, and the patient improved. This case report illustrates the importance of timely multidisciplinary approach to recognise this highly fatal disease at an early stage.


2020 ◽  
Vol 7 (5) ◽  
pp. 777
Author(s):  
Narendra Singh ◽  
R. K. Varma ◽  
Richa Giri ◽  
Punit Varma ◽  
Seema Dwivedi ◽  
...  

Background: AIDS was the first recognized in U.S in summer of 1981 at centers for disease control and prevention reported the unexplained occurrence of pneumocystis jiroveci in previously healthy homosexual men in Los Angeles and Kaposi. First time in 1983 HIV syndrome was isolated from a patient of lymphadenopathy. Person with positive HIV serology who have ever had a CD4 lymphocyte count below 200celles/mcl and CD4 lymphocyte percentage below 14% are considered to have AIDS (CMDT 2017).Methods: This study was continuous longitudinal, prospective and retrospective, observational, at ART plus Centre, Kanpur K.P.S. institute of medicine (G.S.V.M. medical college) included the all patients on ART1 attending in Centre were screened for treatment failure based on clinical, immunological and virological criteria’s as decided by SACEP from 2016 to 2018.Results: Total numbers of patients are 118 among them 71 female and 47 males, age groups between 30-40 there are 54 patients. In study treatment with ART patients Hb levels more than 10%, Mean value before 10.85±1.31 and mean value after treatment was 10.5±1.31, TLC before 6970.94±6309.93 after treatment 6800.25±2522.99, Serum Bilirubin before and after treatment 0.69±0.49 and 0.95±1.13. Mean value of before and after treatment serum creatinin1.80±11.34 and 0.88±0.38.Conclusions: There is increased in serum creatinine and SGPT /SGOT and decrease in Hb levels in treatment of second line ART treatment so it should be monitored every monthly interval.


2017 ◽  
Vol 4 (4) ◽  
pp. 1485
Author(s):  
Vishal Manohar Jadhav ◽  
Yashwant Raghu Gabhale ◽  
Mamatha Murad Lala ◽  
Nikita Dilip Shah ◽  
Mamta Vijay Manglani

Background: To determine the clinical spectrum and prevalence of opportunistic infections (OIs) in HIV infected children and correlate the occurrence of opportunistic infections with their CD4 count and Anti-retroviral treatment (ART).Methods: A total of 100 HIV infected children diagnosed with opportunistic infections were included in the study. Demographic details, clinical examination and relevant investigations were done for all the children. Clinical spectrum of OIs and HIV staging was recorded. CD4 counts were done at baseline and were repeated at 6 monthly intervals.Results: Mean age of the patients was 7.08±3.48 years (ranging from 6 months to 15 years) at enrollment with male to female ratio of 1.2:1. Fever (91%) was a common presenting symptom followed by weight loss (74%), cough (37%), abdominal pain (29%) and breathlessness (16%). CD4 count was significantly associated with presence of opportunistic infection in the study group. Tuberculosis - pulmonary (32%) and extra-pulmonary (29%) was the most common oppurtunistic infections, followed by oral thrush (13%), Herpes zoster (10%), Molluscum Contagiosum (9%), Pneumocystis jiroveci pneumonia (3%), Parvovirus infection (3%) and Pruritic Papular Eruptions (2%). 70% children were on ART as per clinical and immunological staging of HIV.Conclusions: Low CD4 count is significantly associated with severe opportunistic infections, therefore drop in CD4 count should serve as an alarming signal for the treating physician. High index of suspicion is required to detect opportunistic infections and therefore CD4 counts should be done more frequently to predict occurrence of OIs. 


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