scholarly journals Respiratory outcomes at five-year follow-up in children with MBL deficiency: a cohort study

2021 ◽  
Author(s):  
Manisha Ramphul ◽  
Anna Poghosyan ◽  
Javairiya Afzaal ◽  
Elizabeth Mcdermott ◽  
Lucy Cliffe ◽  
...  
Keyword(s):  
Conclusive Evidence ◽  
Recurrent Respiratory Tract Infection ◽  
Vaccine Responses ◽  
Microbiological Characteristics ◽  
Large Teaching Hospital ◽  
Mannose Binding ◽  
Radiological Changes ◽  
Respiratory Outcomes ◽  
Mbl Deficiency

Introduction Mannose-binding lectin (MBL) serum protein, is an important molecule of the innate immune system that is involved in antimicrobial recognition and clearing responses. There is no conclusive evidence that MBL deficiency is associated with adverse respiratory consequences. Aim We explored whether there is a difference in clinical, radiological and microbiological characteristics in children with MBL deficiency presenting with troublesome respiratory symptoms (frequent, recurrent, persistent or very severe), as compared to those who are MBL-sufficient. Methods We performed a retrospective study looking at MBL measurements in children over a period of 10 years in a large teaching hospital, with a minimum follow-up period of 5 years from the time of the MBL measurement to the year 2019. Results 32% of children with MBL deficiency and 30% of those with MBL sufficiency had positive microbiology. 23% of children with MBL deficiency and 24% of those with MBL sufficiency had radiological changes on plain radiographs. 28% of children with MBL deficiency and 33% of those with MBL sufficiency had suboptimal vaccine responses to primary immunisations. 67% of the MBL-deficient children had suboptimal vaccine responses to booster immunisations, compared to 40% of the MBL-sufficient group. Conclusion We conclude that there is no difference at five year follow-up in clinical, radiological and microbiological characteristics between children who are MBL-deficient as compared to those who have sufficient levels. These results add to the existing body of literature that shows no statistically significant association between MBL deficiency and susceptibility to recurrent respiratory tract infection in children.

scholarly journals A Population-based Study of Morbidity and Mortality in Mannose-binding Lectin Deficiency

2004 ◽  
Vol 199 (10) ◽  
pp. 1391-1399 ◽  
Author(s):  
Morten Dahl ◽  
Anne Tybjærg-Hansen ◽  
Peter Schnohr ◽  
Børge G. Nordestgaard
Keyword(s):  
Population Based ◽  
Caucasian Population ◽  
Mannose Binding Lectin ◽  
Case Control Studies ◽  
Common Diseases ◽  
Mannose Binding ◽  
Incidence Of Hospitalization ◽  
Binding Lectin ◽  
Mbl Deficiency

Reduced levels of wild-type mannose-binding lectin (MBL) may increase susceptibility for infection, other common diseases, and death. We investigated associations between MBL deficiency and risk of infection, other common diseases, and death during 24, 24, and 8 yr of follow-up, respectively. We genotyped 9,245 individuals from the adult Danish population for three MBL deficiency alleles, B, C, and D, as opposed to the normal noncarrier A allele. Hospitalization incidence per 10,000 person · yr was 644 in noncarriers compared with 631 in heterozygotes (log-rank: P = 0.39) and 658 in deficiency homozygotes (P = 0.53). Death incidence per 10,000 person · yr was 235 in noncarriers compared with 244 in heterozygotes (P = 0.44) and 274 in deficiency homozygotes (P = 0.12). After stratification by specific cause of hospitalization or death, only hospitalization from cardiovascular disorders was increased in deficiency homozygotes versus noncarriers (P = 0.02). When retested in two case control studies, this association could not be confirmed. Incidence of hospitalization or death from infections or other serious common disorders did not differ between deficiency homozygotes and noncarriers. In conclusion, in this large study in an ethnically homogenous Caucasian population, there was no evidence for significant differences in infectious disease or mortality in MBL-deficient individuals versus controls. Our results suggest that MBL deficiency is not a major risk factor for morbidity or death in the adult Caucasian population.

scholarly journals Microsurgery for vestibular schwannoma after Gamma Knife surgery: challenges and treatment strategies

2014 ◽  
Vol 121 (Suppl_2) ◽  
pp. 150-159 ◽  
Author(s):  
Cheng-Chia Lee ◽  
Hsiu-Mei Wu ◽  
Wen-Yuh Chung ◽  
Ching-Jen Chen ◽  
David Hung-Chi Pan ◽  
...  
Keyword(s):  
Gamma Knife ◽  
Vestibular Schwannoma ◽  
Radiation Effects ◽  
Tumor Resection ◽  
Treatment Strategies ◽  
Gamma Knife Surgery ◽  
Neurological Outcomes ◽  
Radiological Changes ◽  
Cyst Development

ObjectResection of vestibular schwannoma (VS) after Gamma Knife surgery (GKS) is infrequently performed. The goals of this study were to analyze and discuss the neurological outcomes and technical challenges of VS resection and to explore strategies for treating tumors that progress after GKS.MethodsIn total, 708 patients with VS underwent GKS between 1993 and 2012 at Taipei Veterans General Hospital. The post-GKS clinical courses, neurological presentations, and radiological changes in these patients were analyzed. Six hundred patients with imaging follow-up of at least 1 year after GKS treatment were included in this study.ResultsThirteen patients (2.2%) underwent microsurgery on average 36.8 months (range 3–107 months) after GKS. The indications for the surgery included symptomatic adverse radiation effects (in 4 patients), tumor progression (in 6), and cyst development (in 3). No morbidity or death as a result of the surgery was observed. At the last follow-up evaluation, all patients, except 1 patient with a malignant tumor, had stable or near-normal facial function.ConclusionsFor the few VS cases that require resection after radiosurgery, maximal tumor resection can be achieved with modern skull-based techniques and refined neuromonitoring without affecting facial nerve function.

scholarly journals Long-term radiological changes and functional outcomes after proximal row carpectomy: Retrospective study with 3 years’ minimum follow-up

2020 ◽  
Vol 106 (8) ◽  
pp. 1589-1595
Author(s):  
Charles Bijon ◽  
Marc Saab ◽  
Thomas Amouyel ◽  
Nadine Sturbois-Nachef ◽  
Elvire Guerre ◽  
...  
Keyword(s):  
Retrospective Study ◽  
Functional Outcomes ◽  
Proximal Row Carpectomy ◽  
Radiological Changes

scholarly journals The effect of lumbosacral manipulation on growing pains

2015 ◽  
Vol 20 (1) ◽  
Author(s):  
Dawid De Beer ◽  
Charmaine M. Bester
Keyword(s):  
Tibialis Anterior Muscle ◽  
Specific Treatment ◽  
Conclusive Evidence ◽  
Self Report ◽  
Response To Treatment ◽  
Rank Test ◽  
Positive Effects ◽  
Pressure Algometer ◽  
Growing Pains

Background: Growing pains are a frequent clinical presentation that continues to puzzle practitioners, with very little conclusive evidence in any medical field, including chiropractic.Objective: The aim of this study was to determine whether lumbosacral manipulations have an effect on growing pain symptoms.Methods: Thirty participants with growing pains between the ages of 4 and 12 years were recruited. The participants were placed into two groups of 15 participants each. Group 1 received lumbosacral manipulations to restricted joints as determined by motion palpation, while Group 2 never received any professional intervention. Often parent(s)/guardian(s) of children who suffer from growing pains will rub the child's legs and offer verbal reassurance in an attempt to console their children. Parent(s)/guardian(s) of both groups were encouraged to continue to do this throughout the duration of the trial. Instructions were given to the parents so that the same rubbing technique and rubbing cream (aqueous cream) were used. Subjective changes were tracked using a pain diary that the parent(s)/guardian(s) were asked to complete, a six-week post-study follow-up question regarding children's growing pains and the Oucher self-report pain scale. Objective measures consisted of pressure algometer readings of the tibialis anterior muscle belly.Results: The statistical data was analysed using the Friedman test, Manne—Whitney test and the Wilcoxon Signed-Rank test. The results demonstrated that both groups responded favourably to their specific treatment over time. However, the group that received lumbosacral manipulations proved to show a quicker response to treatment; and the post study follow-up of this same group showed markedly more positive feedback than the group that did not receive the treatment. These results highlighted the positive effects of chiropractic manipulation on growing pain symptoms.Conclusion: The results from this study, specifically the feedback from parent(s)/guardians(s) and the pain diaries, indicated that spinal manipulation is beneficial in the treatment of growing pains. The results also showed that other methods of treating growing pains, such as simple leg rubs, may also bring relief.

Investigation of aStreptococcus viridansPseudobacteremia Epidemic at a University Teaching Hospital

1989 ◽  
Vol 10 (9) ◽  
pp. 416-421 ◽  
Author(s):  
Deirdre L. Church ◽  
Heather E. Bryant
Keyword(s):  
Blood Culture ◽  
Teaching Hospital ◽  
Index Finger ◽  
Blood Cultures ◽  
University Teaching ◽  
Large Teaching Hospital ◽  
Disproportionate Number ◽  
Good Concordance ◽  
Contaminated Blood

AbstractFor several months in 1986, an outbreak of Streptococcus viridanspseudobacteremia occurred at a large teaching hospital. All sources of laboratory blood culture contamination were excluded. A retrospective epidemiological study indicated that one phlebotomist, “P,” collected a disproportionate number of the positive blood cultures. Further comparison of the paired blood culture results from the three months when the incidence was highest revealed a good concordance of results among all other phlebotomists (Kappa = 0.5), while P's results concurred with others less frequently than would be expected even by chance (Kappa < 0.0). Clinical follow-up showed that P did not routinely wear gloves while drawing blood and had eczema of the hands. Skin scrapings from the hands, right index finger/fingernail grew predominantlyS viridansspecies that were compatible with those recovered from contaminated blood cultures. This epidemic demonstrated the need for early detection of this source as a cause of nosocomial pseudobacteremia.

scholarly journals Response to Zoledronic Acid Infusion in Children With Fibrous Dysplasia

2020 ◽  
Vol 8 ◽  
Author(s):  
Sujit Kumar Tripathy ◽  
Shakti Swaroop ◽  
Sandeep Velagada ◽  
Debashree Priyadarshini ◽  
Rashmi Ranjan Das ◽  
...  
Keyword(s):  
Zoledronic Acid ◽  
Fibrous Dysplasia ◽  
Term Treatment ◽  
Acid Infusion ◽  
Café Au Lait Spots ◽  
Long Term Treatment ◽  
Radiological Response ◽  
Radiological Changes

Objective: This retrospective study evaluated the outcome and safety of long-term treatment with zoledronic acid, in both polyostotic and mono-ostotic fibrous dysplasia (FD) of children.Methods: The case records of children and adolescents with symptomatic FD who received zoledronic acid (0.1 mg/kg IV infusion over 1 h) and have completed at least 2 years follow-up were analyzed. The relevant details were recorded in a predesigned chart. Clinical assessment [pain assessment by visual analog scale (VAS) and incidence of new fracture], radiological changes (cortical thickening, ossification, and decrease in the diameter of the osteolytic lesions) and biochemical parameters [alkaline phosphatase (ALP)] were used to evaluate the improvement.Results: The mean age of presentation was 9.1 years, with four males and six females. All patients had symptomatic FD in the lower limb with complaints of pain, tenderness, swelling, or deformity. Four children had associated pathological fracture. The radiological evaluation with bone scan revealed polyostotic involvement in eight patients and mono-ostotic involvement in two patients. Three patients had associated systemic features like café-au-lait spots or precocious puberty. The fracture united within 3 months and the radiological improvement was evident in the form of filling of the osteolytic defect. The pain score in six patients showed significant improvement (VAS &lt; 3). The ALP level decreased to 544.12 ± 47.35 IU/L from an initial value of 895.75 ± 79.64 IU/L (p = 0.04) at 12 months. One patient had symptomatic hypocalcaemia after zoledronic acid infusion.Conclusion: The clinical and radiological response of zoledronic acid treatment in FD of children is promising. Further randomized control trials with a larger sample size are required to establish this drug as a first-line medical treatment in FD.

scholarly journals Cluster investigation of mixed O76:H19 Shiga toxin-producingEscherichia coliand atypical enteropathogenicE. coliinfection in a Spanish household

2013 ◽  
Vol 142 (5) ◽  
pp. 1029-1033 ◽  
Author(s):  
S. SÁNCHEZ ◽  
M. GARCÍA CENOZ ◽  
C. MARTÍN ◽  
X. BERISTAIN ◽  
M. T. LLORENTE ◽  
...  
Keyword(s):  
Shiga Toxin ◽  
Family Members ◽  
Conclusive Evidence ◽  
Disease Clusters ◽  
E Coli ◽  
Source Of Infection ◽  
Asymptomatic Carriage ◽  
Sheep Manure ◽  
Household Members

SUMMARYA Spanish household was identified through a Public Health follow up on a Shiga toxin-producingEscherichia coli(STEC)-positive 14-month-old girl reporting bloody diarrhoea, with the four household members experiencing either symptomatic or asymptomatic STEC and/or atypical enteropathogenicE. coli(aEPEC) shedding. In total, two different O76:H19 STEC strains and six aEPEC strains belonging to multiple serotypes were isolated and characterized in the household during a 5-month period. Prolonged asymptomatic shedding of O76:H19 STEC and O51:H49 aEPEC was detected in two family members. Although there was no conclusive evidence, consumption of vegetables fertilized with sheep manure was the suspected source of infection. This study highlights the risk of cross-infections posed by prolonged asymptomatic carriage and close household contact between family members, and illustrates the importance of molecular epidemiology in understanding disease clusters.

A Novel and Pivotal Role of the Mannose-Binding Lectin (MBL) Pathway of Complement Cascade (ComC) Activation in Triggering Mobilization of Hematopoietic Stem/Progenitor Cells (HSPCs)

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 4301-4301 ◽  
Author(s):  
Mateusz Adamiak ◽  
Ahmed Abdel-Latif ◽  
Janina Ratajczak ◽  
Mariusz Z Ratajczak
Keyword(s):  
Pattern Recognition ◽  
Mannose Binding Lectin ◽  
Pivotal Role ◽  
Classical Pathway ◽  
Complement Cascade ◽  
Alternative Pathways ◽  
Mannose Binding ◽  
Binding Lectin ◽  
Mbl Deficiency

Abstract Background . The complement cascade (ComC) is part of the innate immunity system, which is not adaptable and does not change over the course of an individual's lifetime; however, it can be recruited and brought into action by the adaptive immune system. The ComC has several pleiotropic effects, and, as we have previously demonstrated, it is required for mobilization of HSPCs during infection or tissue/organ injuries and responding to pharmacological mobilizing agents (Blood 2004, 103, 2071-2078). The ComC is activated by three pathways: the classical, alternative, and mannose-binding lectin (MBL) pathways. While a requirement for ComC activation and, in particular, the pivotal role of the distal part of complement activation and generation of C5 cleavage fragments was previously demonstrated by us (Leukemia 2009, 23, 2052-2062), mice with mutations to components of the classical and alternative pathways in which the distal pathway of C5 activation remained intact did not show impairment of HSPC mobilization (Leukemia 2010, 24, 1667-1675). However, no studies so far have been performed to address the role of the MBL pathway of ComC activation in triggering the mobilization of HSPCs. The MBL pathway is homologous to the classical pathway, but contains opsonin, MBL, and ficolins instead of C1q. MBL functions by pattern recognition, as opsonin binds to mannose residues on the surface of pathogens and certain cells, and activates the MBL-associated serine proteases, MASP-1, and MASP-2, which can then split C4 (into C4a and C4b) and C2 (into C2a and C2b) to form the classical C3-convertase, as in the classical pathway. Interestingly, it is known that ~10% of the population has defective activation of the MBL pathway. Hypothesis. We hypothesized for first time that the MBL ComC-activation pathway is involved in triggering ComC-mediated mobilization of HSPCs and that MBL deficiency results in poor mobilization. Materials and Methods . In our experiments, 2-month-old, MBL-deficient mice (MBL-/-) and normal wild type (WT) littermates were mobilized for 6 days with G-CSF or AMD3100. Following mobilization, we measured in peripheral blood (PB) i) the total number of white blood cells (WBC), ii) the number of circulating clonogenic colony-forming unit granulocyte/macrophage (CFU-GM) progenitors, and iii) the number of Sca-1+ c-kit+ lineage- (SKL) cells. In parallel, we evaluated activation of the MBL pathway in WT animals after administration of G-CSF and AMD3100. Results . We found that pattern recognition by the MBL ComC activation pathway is involved in pharmacological G-CSF- and AMD3100-induced mobilization of HSPCs, and activation of the MBL pathway was confirmed by ELISA in WT animals. As predicted, MBL KO mice were found to be poor mobilizers. Conclusions . We identified a previously unrecognized role of the MBL pathway in triggering ComC activation in the process of HSPC mobilization. This finding explains the pivotal role of the MBL pathway in triggering activation of the proximal part of the ComC and explains why, even with a deficiency in activation of classical and alternative pathway components, mobilization of HSPCs proceeds normally as long as the MBL pathway is intact. On the other hand, if the MBL pathway of the ComC is defective, neither classical nor alternative pathways can trigger optimal mobilization of HSPCs. Taking into consideration that ~10% of normal people are poor activators of the MBL pathway, we are currently investigating whether MBL deficiency correlates with poor mobilization in these patients. Disclosures No relevant conflicts of interest to declare.

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