scholarly journals Good’s syndrome, a rare form of acquired immunodeficiency associated with thymomas

2019 ◽  
Vol 9 (2) ◽  
Author(s):  
Antonio Tamburello ◽  
Laura Castelnovo ◽  
Paola Faggioli ◽  
Daniela Bompane ◽  
Bruno Brando ◽  
...  
Keyword(s):  
Bacterial Infections ◽  
B Lymphocyte ◽  
Fungal Infections ◽  
Early Recognition ◽  
Pneumocystis Jiroveci ◽  
Treatment Protocols ◽  
Gradual Improvement ◽  
Good’S Syndrome ◽  
Cold Sores ◽  
Tract Infections

Good’s syndrome (GS) or thymomaassociated immunodeficiency is a rare clinical entity that should be ruled out in patients with thymoma who develop severe, recurrent bacterial infections and opportunistic viral and fungal infections. There are no treatment protocols established, hence, early recognition is imperative to avoid complications. We report the case of a 42-year-old female, known for a previous thymectomy for giant thymoma who has suffered for a long time from recurrent pulmonary and urinary tract infections and cold sores. In March 2016 she referred to our unit complaining of fever, cough, chest pain, and cold sores due to Herpes simplex virus (HSV), confirmed serologically as HSV-1. Chest X-ray showed left pneumonia due to Streptococcus pneumoniae. She started antibiotics (amoxicillin/clavulanic acid associated with azithromycin) with gradual improvement. Given her history she was studied for an underlying immunodeficiency: IgG, IgA, and IgM were significantly low or absent, as well as all IgG subclasses; blood and bone marrow aspirate leucocyte immunophenotyping showed complete absence of B lymphocytes and reduced CD4+ T cells. In light of: i) thymoma; ii) B lymphocyte deficit; iii) hypogammaglobulinemia; iv) recurrent infections, GS was diagnosed and pre-emptive immunoglobulin treatment, associated with HSV and Pneumocystis jiroveci prophylaxis (Acyclovir for HSV and Sulfamethoxazole- Trimethoprim for P. jiroveci) were started. Since then the patient has no longer presented any infectious episodes.

scholarly journals Good’s syndrome – Nothing Good about it: A Case Report.

2021 ◽  
Vol 4 (1) ◽  
pp. 77-80
Author(s):  
Sunitha Ramachandra ◽  
Lakshmi Rao ◽  
Bola Rajendra Kamath ◽  
Masoud Al Kindi
Keyword(s):  
Bacterial Infections ◽  
Mediastinal Mass ◽  
Fungal Infections ◽  
Early Recognition ◽  
Anterior Mediastinal Mass ◽  
Chronic Diarrhoea ◽  
Hiv Status ◽  
Good’S Syndrome ◽  
Microbial Infections ◽  
Common Variable

We report a rare case of Good’s Syndrome (GS) or thymoma with immunodeficiency in a 48-year-old male patient. This condition presents in the fourth or fifth decade of life.  GS should be suspected in a person presenting with recurrent bacterial infections with encapsulated organisms and opportunistic viral and fungal infections in the setting of thymoma, hypogammaglobulinemia and reduced or absent B cells. Our patient presented with chronic diarrhoea for the past three years,  repeated esophageal candidiasis and a superior anterior mediastinal mass. Duodenal biopsy showed CMV duodenitis. Lab investigations revealed low IgM levels [hypogammaglobulinemia] and the biopsy from the mediastinal mass was consistent with thymoma. This case is being written to highlight that unexplained repeated opportunistic microbial infections should prompt the clinician to suspect immunodeficiency in the background of a negative HIV status and to emphasize that GS is one of the causes of adult-onset immunodeficiency where early recognition and treatment can improve and alter the course of the disease as GS carries a worse prognosis compared to XLA [X-linked agammaglobulinemia] and CVID [common variable immunodeficiency].

Patients with Chromosome 11q-abberations are Characterized by a Combined Primary Immunodeficiency Involving Both B- and T-lymphocytes

2021 ◽  
Author(s):  
Elisabeth Janneke Huisman ◽  
A. Rick Brooimans ◽  
Samone Mayer ◽  
Marieke Joosten ◽  
Louis de Bont ◽  
...  
Keyword(s):  
T Lymphocyte ◽  
B Lymphocyte ◽  
Fungal Infections ◽  
Prophylactic Antibiotic ◽  
Tube Placement ◽  
Primary Immune Deficiency ◽  
Lymphocyte Function ◽  
Chromosome 11 ◽  
Infectious Burden ◽  
Tract Infections

Abstract Disorders of the long arm of chromosome 11 (11q) are rare and involve various chromosomal regions. Patients with 11q-disorders, including Jacobsen syndrome, often present with a susceptibility for bacterial, prolonged viral and fungal infections partially explained by hypogammaglobulinemia. Additional T-lymphocyte or granular neutrophil dysfunction may also be present. In order to evaluate infectious burden and immunological function in patients with 11q-disorders, we prospectively studied a cohort of 14 patients with various 11q aberrations. Clinically, 12 patients exhibited prolonged and repetitive respiratory tract infections, frequently requiring (prophylactic) antibiotic treatment (n=7), ear-tube placement (n=9) or use of inhalers (n=5). Complicated varicella infections (n=5), chronic eczema (n=6), warts and chronic fungal infections (n=4) were reported. Six patients were on immunoglobulin replacement therapy. We observed a high prevalence of low B-lymphocyte counts (n=8), decreased T-lymphocyte counts (n=5) and abnormal T-lymphocyte function (n=12). Granulocyte function was abnormal in 29% without an aberrant clinical phenotype. Immunodeficiency was found in patients with terminal and interstitial 11q-deletions and in one patient with 11q trisomy. Genetically, FLI1 and ETS1 are seen as causative for the immunodeficiency, but these genes were deleted nor duplicated in 5 of our 14 patients. Alternative candidate genes on 11q such as ATM, CD3-cluster, CBL and THYN1 may have a role in immune dysregulation in our patients. In conclusion, we present evidence that a combined primary immune deficiency may be present in patients with 11q-disorders leading to clinically relevant infections. Therefore, broad immunological screening and necessary treatment is of importance in this patient group.

508 Fungal Infections in Burn Patients from 2014–2019: A 5-year Retrospective Review

2020 ◽  
Vol 41 (Supplement_1) ◽  
pp. S90-S91
Author(s):  
Kaitlin A Pruskowski ◽  
Julie A Rizzo ◽  
Leopoldo C Cancio
Keyword(s):  
Mortality Rate ◽  
High Mortality ◽  
Bacterial Infections ◽  
Burn Injury ◽  
Fungal Infections ◽  
Early Recognition ◽  
Hospital Length Of Stay ◽  
Burn Patients ◽  
Average Hospital ◽  
Time From Injury

Abstract Introduction Topical and systemic antibiotic agents used to treat burn wounds allow for fungal growth. Fungal infections often occur later in the hospital course and carry a higher mortality rate than bacterial infections in critically ill burn patients. The primary objective of this study was to describe the incidence and characteristics of fungal infections in our burn center. Methods This was a retrospective chart review. Subjects who were admitted between January 2014 and February 2019 after sustaining a burn injury and received a systemic antifungal agent were included. All cultures and histopathology results that were positive for fungus were recorded. Results Ninety one subjects were included in this analysis. The average age was 42.3±16.9 years and 74 (81.3%) subjects were male. Seventy four (81.3%) subjects were admitted after flame burn. The average burn size was 40.1±24.2% TBSA. The average hospital length of stay was 57.2±44.8 days. Thirty eight (41.7%) subjects died during their hospital stay. The 91 subjects had 220 cultures positive for fungi: 29 blood cultures, 19 BAL cultures, 23 sputum cultures, 118 tissue cultures, and 31 urine cultures. The most common fungal genera isolated were: Candida (n=146), Aspergillus (n=55), and Fusarium (n=44). Twenty six (28.6%) subjects had histopathology results that showed fungus in non-viable tissue, with an average time from injury to fungus of 20.4±11.8 days. Twenty six (28.6%) subjects had histopathology results that showed fungus in viable tissue, with an average time from injury to fungus of 22.7±16.7 days. Of note, these were not the group that had fungus in non-viable tissue. Twelve (13.2%) subjects had angioinvasion, with an average time to angioinvasion of 26.6±22 days. The 91 subjects received 133 courses of antifungals. The average course of antifungal therapy was 7.8±8.9 days. The most common antifungals used were: micafungin (n=54), fluconazole (n=53), and liposomal amphotericin B (n=44). Conclusions Patients who were started on a systemic antifungal had a high mortality rate. Common fungi isolated included Candida and Aspergillus, which is consistent with previous literature. Further research is needed on the early recognition, accurate diagnosis, and timely management of fungal infections in this patient population. Applicability of Research to Practice Fungal infections after burn injury carry a high mortality rate. Early recognition and timely management of these infections is paramount.

Fungal diseases of the gastrointestinal tract

2017 ◽  
Author(s):  
Silke Schelenz
Keyword(s):  
Surgical Intervention ◽  
Fungal Infections ◽  
Early Recognition ◽  
Fungal Diseases ◽  
Gi Tract ◽  
Mucosal Infection ◽  
Contaminated Food ◽  
Life Threatening ◽  
Tract Infections ◽  
Underlying Risk

Fungal diseases of the gastrointestinal (GI) tract can occur because of an overgrowth of yeast in the gut, exposure to contaminated food and water, or as part of disseminated invasive fungal infections from other sites. The extent of the disease depends on the underlying risk factors, such as diabetes or immunosuppression, and ranges from colonization, localized infection, or fungaemia, to aggressive life-threatening GI tract infections. Candida spp. are the commonest cause of mucosal infection, although mould infections are increasingly reported. Serious invasive mould infections are difficult to diagnose as symptoms are often non-specific. Early recognition, prompt antifungal treatment, and surgical intervention can be lifesaving.

scholarly journals Cytokine Production Assays Reveal Discriminatory Immune Defects in Adults with Recurrent Infections and Noninfectious Inflammation

2014 ◽  
Vol 21 (8) ◽  
pp. 1061-1069 ◽  
Author(s):  
Jaap ten Oever ◽  
Frank L. van de Veerdonk ◽  
Leo A. B. Joosten ◽  
Anna Simon ◽  
Reinout van Crevel ◽  
...  
Keyword(s):  
Bacterial Infections ◽  
Cytokine Production ◽  
Fungal Infections ◽  
Diagnostic Value ◽  
Recurrent Infections ◽  
Peripheral Blood Mononuclear ◽  
Necrosis Factor Alpha ◽  
Ifn Γ ◽  
Tract Infections ◽  
Immune Defects

ABSTRACTCytokine production assays have been primarily used in research settings studying novel immunodeficiencies. We sought to determine the diagnostic value of cytokine production assays in patients with recurrent and/or severe infectious diseases (IDs) without known immunodeficiencies and unclassified noninfectious inflammatory disorders (NIIDs). We retrospectively examined cytokine production in whole-blood and peripheral blood mononuclear cell samples from 157 adult patients. A cytokine production rate of <5% of that of healthy controls was considered defective. While monocyte-derived cytokine (tumor necrosis factor alpha [TNF-α], interleukin-1β [IL-1β], and IL-6) production was rarely affected, 30% of all included patients had deficient production of interferon gamma (IFN-γ), IL-17A, or IL-22. Twenty-five percent of the NIID patients displayed defective IFN-γ production, whereas IL-17A production was generally unaffected. In the group of ID patients, defective IFN-γ production was found in 19% and 14% of the patients with viral and bacterial infections, respectively, and in 38%, 24%, and 50% of patients with mycobacterial, mucocutaneous, and invasive fungal infections, respectively. Defective IL-17A and IL-22 production was mainly confined to ID patients with mucocutaneous fungal infections. In conclusion, cytokine production assays frequently detect defective Th1 responses in patients with mycobacterial or fungal infections, in contrast to patients with respiratory tract infections or isolated bacterial infections. Defective IL-17A and IL-22 production was primarily found in patients with fungal infections, while monocyte-derived cytokine production was unaffected. Thus, lymphocyte-derived cytokine production assays are helpful in the diagnostic workup of patients with recurrent infections and suspected immunodeficiencies and have the potential to reveal immune defects that might guide adjunctive immunomodulatory therapy.

Rhino-Orbital and Rhino-Orbito-Cerebral Mucormycosis

2002 ◽  
Vol 127 (1) ◽  
pp. 22-31 ◽  
Author(s):  
Yoav P. Talmi ◽  
Anna Goldschmied-Reouven ◽  
Mati Bakon ◽  
Iris Barshack ◽  
Michael Wolf ◽  
...  
Keyword(s):  
Amphotericin B ◽  
Fungal Infections ◽  
Early Recognition ◽  
Underlying Disease ◽  
Treatment Protocols ◽  
Seasonal Incidence ◽  
End Stage ◽  
Aggressive Debridement ◽  
Immunocompromised Hosts

BACKGROUND: Rhino-orbito-cerebral mucormycosis (ROCM) is a devastating infection of immunocompromised hosts. We present our experience with 19 ROCM cases and attempt to define preferred diagnostic and treatment protocols. METHODS: All had tissue biopsies obtained studied by direct smear, histologic studies, and cultures. Imaging was obtained in 14 cases. RESULTS: Sixteen patients presented between August and November. Six had mixed fungal infections. Seven patients had end-stage underlying disease or infection and did not undergo surgery and 4 had an indolent form of disease. Patients were treated by surgery and by amphotericin B. The overall survival was 47%. CONCLUSIONS: ROCM may have seasonal incidence peaking in the fall and early winter. The therapeutic approach should be unchanged in cases of mixed fungal infections. Amphotericin B with aggressive debridement remains the mainstay of treatment. Early recognition and treatment are essential. A presentation and survival-dependent classification of ROCM are offered.

scholarly journals Utilization of Teledentistry in Antimicrobial Prescribing and Diagnosis of Infectious Diseases during COVID-19 Lockdown

2020 ◽  
Vol 14 (S 01) ◽  
pp. S20-S26
Author(s):  
Najla Dar-Odeh ◽  
Hamzah Babkair ◽  
Ahmad Alnazzawi ◽  
Shaden Abu-Hammad ◽  
Abdalla Abu-Hammad ◽  
...  
Keyword(s):  
Bacterial Infections ◽  
Fungal Infections ◽  
Clinical Manifestations ◽  
Lymphoid Hyperplasia ◽  
Diagnostic Features ◽  
Treatment Protocols ◽  
Professional Advice ◽  
Oral Infections ◽  
Antimicrobial Prescribing ◽  
Dental Practitioners

Abstract Objectives This study aimed to analyze Jordanian dentists’ inquiries on oral infections and antimicrobial prescribing using dental professional WhatsApp groups during coronavirus disease lockdown period. Materials and Methods Three professional WhatsApp groups of Jordanian dentists were reviewed and analyzed for inquiries posted during the period from January to May 2020. Inquiries were sent from patients to their dentists who posted these inquiries to the professional WhatsApp dental groups for consultation and professional advice. All queries regarding oral infections and antimicrobial prescribing were extracted and analyzed. Results Three WhatsApp professional groups with a total numbers of members of 750 dentists who posted queries about their patients were included in this study. Dentist members posted queries about their patients to these professional WhatsApp groups. There was a total of 32 inquiries regarding oral lesions and 11 consultations regarding prescribing and dental management of medically compromised patients giving a total of 43 consultations. Among which there were 19 inquiries on oral infections and 9 inquiries on antimicrobial prescribing giving a total of 28 consultations. Most common inquiries were on bacterial infections (localized dentoalveolar abscess, pericoronitis, cellulitis, and lymphoid hyperplasia of the tongue), viral herpetic infections, and Candida infections (erythematous and pseudomembranous candidiasis). Conclusions Many dental practitioners cannot distinguish the correct diagnostic features of oral infections particularly viral and fungal infections. Continuing education should be considered to focus on clinical manifestations of various oral infections. Further, educational activities that focus on variations in treatment protocols for various infections should be introduced particularly those that concern indications for antimicrobial prescribing.

Application of Innovative Hydroxyapatite Materials in 3D Printing for Biocompatible Voice Implants

2018 ◽  
Vol 69 (4) ◽  
pp. 840-842
Author(s):  
Wojciech Musialik ◽  
Marcin Nabialek ◽  
Slawomir Letkiewicz ◽  
Andrei Victor Sandu ◽  
Katarzyna Bloch
Keyword(s):  
3D Printing ◽  
Surgical Procedures ◽  
Bacterial Infections ◽  
Fungal Infections ◽  
Colloidal Silver ◽  
Bone Implants ◽  
External Ring ◽  
Voice Prostheses ◽  
3D Printers ◽  
Silver Composite

The paper presents the possibility of using an innovative hydroxyapatite filament Ca10(PO4)6(OH)2 for printing in 3D printers of bone implants and the possibility of using it during implantation with voice prostheses. The introduction of an additional colloidal silver composite in voice implants will contribute to the reduction of bacterial infections, fungal infections and granulomatous hyperplasia. The creation of a stable external ring of the vocal fistula will remove complications associated with it with enlargement of the fistula and leakiness of voice implants. The ability to print with a hydroxyapatite filament will allow digital pre-surgery modeling of bone implants suited to the needs of surgical procedures.

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