High Incidence of Posttransplant Lymphoproliferative Disease in Pediatric Patients with Cystic Fibrosis

2000 ◽  
Vol 161 (4) ◽  
pp. 1252-1255 ◽  
Author(s):  
ALAN H. COHEN ◽  
STUART C. SWEET ◽  
ERIC MENDELOFF ◽  
GEORGE B. MALLORY ◽  
CHARLES B. HUDDLESTON ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pediatric Patients ◽  
Lymphoproliferative Disease ◽  
High Incidence ◽  
Posttransplant Lymphoproliferative Disease

Rhinosinusitis in the Pediatric Patient with Cystic Fibrosis

2014 ◽  
Vol 10 (3) ◽  
pp. 198-201 ◽  
Author(s):  
Christopher Fundakowski ◽  
Rosemary Ojo ◽  
Ramzi Younis
Keyword(s):  
Cystic Fibrosis ◽  
Autosomal Recessive ◽  
Pediatric Patients ◽  
Surgical Intervention ◽  
Chronic Sinusitis ◽  
Genetic Disorder ◽  
Symptomatic Relief ◽  
Recurrence Rates ◽  
Polyp Recurrence ◽  
Sinonasal Polyposis

Cystic fibrosis (CF) is a common autosomal recessive genetic disorder where a deletion mutation and subsequent downstream alteration in transmembrane regulator proteins results in increased mucus viscosity. CF manifests clinically with chronic multisystem inflammation and recurrent infections. Nearly all children with CF have chronic sinusitis, and a large majority will have concurrent sinonasal polyposis. Chronic sinusitis and sinonasal polyposis in pediatric patients with CF can be managed conservatively initially, though most will fail medical management and require surgical intervention. Unfortunately, symptom resolution is marginal and polyp recurrence rates are high. Currently, no cure exists for CF and the mainstay of treatment is to provide symptomatic relief, and minimize disease morbidity.

Population Pharmacokinetics of Tobramycin Inhalation Solution in Pediatric Patients With Cystic Fibrosis

2017 ◽  
Vol 106 (11) ◽  
pp. 3402-3409 ◽  
Author(s):  
Xinting Wang ◽  
Stephan Koehne-Voss ◽  
SivaNaga S. Anumolu ◽  
Jing Yu
Keyword(s):  
Cystic Fibrosis ◽  
Population Pharmacokinetics ◽  
Pediatric Patients

Nutrition Management of Pediatric Patients Who Have Cystic Fibrosis

2009 ◽  
Vol 56 (5) ◽  
pp. 1123-1141 ◽  
Author(s):  
Suzanne H. Michel ◽  
Asim Maqbool ◽  
Maria D. Hanna ◽  
Maria Mascarenhas
Keyword(s):  
Cystic Fibrosis ◽  
Pediatric Patients ◽  
Nutrition Management

Increased Plasma YKL-40 Level and Chitotriosidase Activity in Cystic Fibrosis Patients

2021 ◽  
Author(s):  
Dilara Bal Topcu ◽  
Gökcen Tugcu ◽  
Berrin Er ◽  
Sanem Eryilmaz Polat ◽  
Mina Hizal ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pediatric Patients ◽  
Adult Patients ◽  
Enzyme Linked Immunosorbent Assay ◽  
Healthy Children ◽  
Plasma Samples ◽  
Stable Period ◽  
Valuable Marker ◽  
Chitotriosidase Activity ◽  
Relevant Factors

Abstract Background We investigated plasma YKL-40 levels and chitotriosidase (CHIT1) activity in patients with cystic fibrosis (CF) lung disease and evaluated clinically relevant factors that may affect their levels. Methods Plasma samples were obtained from pediatric (n = 19) and adult patients (n = 15) during exacerbation, discharge and stable period of the disease. YKL-40 levels and chitotriosidase activity were measured by enzyme-linked immunosorbent assay and fluorometric assay, respectively. Data were compared with healthy children and adults of similar age. Results YKL-40 levels of pediatric and adult CF patients at all periods were significantly higher than controls (p < 0.001 and p < 0.05). CHIT1 activities of adult patients at all periods were significantly higher compared to controls (p < 0.05). On the other hand, CHIT1 activities of pediatric CF patients were similar with controls. YKL-40 levels of exacerbation period of adult CF patients were negatively correlated with % FVC (r= -0.800, p = 0.014) and % FEV1 (r= -0.735, p = 0.008). YKL-40 levels in the exacerbation period of pediatric CF patients were negatively correlated with % FVC (r= -0.697, p = 0.0082) and % FEV1 (r= -0.720, p = 0.006). Conclusions CHIT1 activity may be a valuable marker of chronic inflammation in adult CF patients who suffer from CF for a longer period of time compared to pediatric patients. Increased YKL-40 levels in both pediatric and adult patients compared to controls may point to a role in between CF pathology. Furthermore, as YKL-40 levels are correlated with FEV1 and FVC in patients, it may be useful for the monitoring of pulmonary function in CF patient.

Cognitive Deficits in Pediatric Craniopharyngioma: An Updated Review

2021 ◽  
Author(s):  
Abdulrahman Al-Mirza ◽  
Omar Al-Taei ◽  
Tariq Al-Saadi
Keyword(s):  
Systematic Review ◽  
Cognitive Impairment ◽  
Cognitive Deficits ◽  
Pediatric Patients ◽  
Memory Deficits ◽  
Neurological Deficits ◽  
Children And Adolescent ◽  
Visual Spatial ◽  
High Incidence ◽  
Standard Tests

AbstractCraniopharyngiomas (CP) are brain tumors that often occur in children and adolescent that results in many neurological and endocrinological disorders. The aim of this systematic review is to provide updated version of studies used to formalize standard tests used for cognitive impairment in pediatric patients with craniopharyngioma. A systematic review was conducted in PubMed, EBSCO, ProQuest, Science Direct, Wiley Online, and Springer to identify studies assessing cognitive impairment in pediatric patients with craniopharyngioma. Academic and learning dysfunctions were reported in seven studies among 41 of 178 patients (23%). Visual–spatial deficits were reported in six studies. Speech and verbal dysfunctions were reported in three studies. Memory deficits were reported in eight studies among 61 of 197 patients (31%). Motor dysfunctions were reported in five studies. Sleep related issues were reported in four studies among 33 of 70 patients (47.1%). Patients with treated pediatric CP demonstrate a high incidence of neurological deficits including cognitive dysfunctions. Academic and learning dysfunctions, visual–spatial deficits, speech and verbal dysfunctions, memory deficits, and sleep-related issues were the most commonly reported cognitive deficits in the present study.

scholarly journals Interaction betweenPseudomonas aeruginosaandAspergillus fumigatusin cystic fibrosis

PeerJ ◽  
2018 ◽  
Vol 6 ◽  
pp. e5931 ◽  
Author(s):  
Jingming Zhao ◽  
Wencheng Yu
Keyword(s):  
Cystic Fibrosis ◽  
Antibiotic Sensitivity ◽  
Pathogenic Microorganisms ◽  
Mutual Inhibition ◽  
Sensing System ◽  
High Incidence ◽  
Volatile Organic ◽  
Metabolic Transformation ◽  
Small Colony ◽  
Combined Infection

BackgroundCystic fibrosis (CF) is a disease characterized by chronic airway infection with a high incidence and poor prognosis.Pseudomonas aeruginosaandAspergillus fumigatusare pathogens commonly found in CF patients. Clinically, these two microorganisms often coexist in the airway of CF patients. Combined infection withP. aeruginosaandA. fumigatusresults in worsening lung function and clinical condition.MethodsIn this review, we focus on the mutual inhibition and promotion mechanisms ofP. aeruginosaandA. fumigatusin CF patients. We also summarized the mechanisms of the interaction between these pathogenic microorganisms.ResultsP. aeruginosainhibitsA. fumigatusgrowth through the effects of phenazines, the quorum sensing system, iron competition, bacteriophages, and small colony variants.P. aeruginosainducesA. fumigatusgrowth through volatile organic compounds and subbacteriostatic concentrations of phenazines.A. fumigatusinterferes withP. aeruginosa, affecting its metabolic growth via phenazine metabolic transformation, gliotoxin production, and reduced antibiotic sensitivity.DiscussionCoexistence ofP. aeruginosaandA. fumigatuscan lead to both mutual inhibition and promotion. In different stages of CF disease, the interaction between these two pathogenic microorganisms may shift between promotion and inhibition. A discussion of the mechanisms ofP. aeruginosaandA. fumigatusinteraction can be beneficial for further treatment of CF patients and for improving the prognosis of the disease.

scholarly journals Posttransplant Lymphoproliferative Disease Presenting as an Extracranial Mass

2017 ◽  
Vol 2017 ◽  
pp. 1-5
Author(s):  
Reuben J. Arasaratnam ◽  
Alejandro Restrepo
Keyword(s):  
Soft Tissue ◽  
Early Recognition ◽  
Solid Organ Transplantation ◽  
Organ Transplant ◽  
Antibody Therapy ◽  
B Cell Lymphoma ◽  
Lymphoproliferative Disease ◽  
Solid Organ ◽  
Tissue Mass ◽  
Posttransplant Lymphoproliferative Disease

Posttransplant lymphoproliferative disease is a serious complication following stem cell and solid organ transplantation. Early recognition of the disease is important in facilitating timely therapy and improving long-term outcomes. We report a renal transplant recipient presenting with an extracranial frontoparietal soft tissue mass that was subsequently diagnosed as a B-cell lymphoma. The patient was treated successfully with immunosuppression reduction, anti-CD20 monoclonal antibody therapy, and cytotoxic chemotherapy. Our case highlights the importance of recognizing soft tissue masses in the head and neck as a potential clinical manifestation of PTLD in solid organ transplant recipients.

Frequent monitoring of Epstein-Barr virus DNA load in unfractionated whole blood is essential for early detection of posttransplant lymphoproliferative disease in high-risk patients

Blood ◽  
2001 ◽  
Vol 97 (5) ◽  
pp. 1165-1171 ◽  
Author(s):  
Servi J. C. Stevens ◽  
Erik A. M. Verschuuren ◽  
Inge Pronk ◽  
Wim van der Bij ◽  
Martin C. Harmsen ◽  
...  
Keyword(s):  
Whole Blood ◽  
Epstein Barr Virus ◽  
Lymphoproliferative Disease ◽  
Serum Samples ◽  
Barr Virus ◽  
Load Monitoring ◽  
Risk Patients ◽  
Posttransplant Lymphoproliferative Disease ◽  
Ebv Dna ◽  
Epstein Barr

Posttransplant lymphoproliferative disease (PTLD) is a frequent and severe Epstein-Barr virus (EBV)–associated complication in transplantation recipients that is caused by iatrogenic suppression of T-cell function. The diagnostic value of weekly EBV DNA load monitoring was investigated in prospectively collected unfractionated whole blood and serum samples of lung transplantation (LTx) recipients with and without PTLD. In PTLD patients, 78% of tested whole blood samples were above the cut-off value of quantitative competitive polymerase chain reaction (Q-PCR) (greater than 2000 EBV DNA copies per mL blood), with the majority of patients having high viral loads before and at PTLD diagnosis. Especially in a primary EBV-infected patient and in patients with conversion of immunosuppressive treatment, rapid increases in peripheral blood EBV DNA load diagnosed and predicted PTLD. In non-PTLD transplantation recipients, only 3.4% of the whole blood samples was above the cut-off value (P &lt; .0001) despite heavy immune suppression and cytomegalovirus (CMV)-related disease. These findings illustrate the clinical importance of frequent EBV DNA load monitoring in LTx recipients. The increased EBV DNA loads in PTLD patients were restricted to the cellular blood compartment, as parallel serum samples were all below cut-off value, which indicates absence of lytic viral replication. EBV+ cells in PTLD patients have a very short doubling time, which can be as low as 56 hours, thereby creating the need for high screening frequency in high-risk patients. Furthermore, it is shown that EBV and CMV can reactivate independently in LTx recipients and that EBV DNA load monitoring may be useful in discriminating PTLD from rejection.

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