Oral health-related quality of life in Fanconi anemia: a cross-sectional study.

PURPOSE: To evaluate the oral health-related quality of life (OHRQoL) of individuals diagnosed with Fanconi anemia (FA). METHODS: A cross-sectional study was conducted with FA patients from two Brazilian referral centers. Participants underwent a complete dental, periodontal, and oral mucosa examination, as well as assessment of resting salivary flow. The short version of the Oral Health Impact Profile (OHIP-14) questionnaire was administered. Descriptive and bivariate analyses were performed, followed by multivariate analysis to examine the impact of independent variables on OHRQoL. RESULTS: The study included 20 (57.1%) males and 15 (42.9%) females, with a mean age of 18.9 years. Oral leukoplakia (OL) was found in 18 individuals. The overall OHIP-14 score was 9.9 ± 10.5. Individuals aged ≥ 16 years had higher OHIP-14 scores, indicating worse OHRQoL for physical pain (p = 0.007), psychological discomfort (p = 0.001), physical disability (p = 0.03), psychological disability (p = 0.001), handicap (p = 0.004), and overall score (p = 0.007). Females reported more negative OHRQoL than males for physical pain (p = 0.02), psychological discomfort (p = 0.03), psychological disability (p = 0.009), and overall score (p = 0.02). Individuals with OL had an overall OHIP-14 score 1.83 times higher than those without OL (95% CI: 1.02-3.28; p = 0.04). Lower salivary flow correlated with higher overall OHIP-14 scores (95% CI: 0.14-0.84; p = 0.01). CONCLUSION: This study represents the first attempt to evaluate OHRQoL in individuals with FA. The presence of OL and reduced salivary flow were identified as predictors of a negative impact on OHRQoL. It is imperative to integrate patients' quality of life in the clinical treatment protocols for the FA population.

The Bloody Crossroads: Interactions between Periodontitis and Hematologic Diseases.

Periodontitis is a common oral condition that can have a significant impact on the overall health of the body. In recent years, attention has been paid to potential relationships between periodontitis and various hematological disorders. This publication aims to present information available in the literature on this relationship, focusing on examples of red blood cell disorders (such as aplastic anemia and sickle cell anemia) and white blood cell disorders (such as cyclic neutropenia, maladaptive trained immunity, clonal hematopoiesis, leukemia, and multiple myeloma). Understanding these associations can help physicians and dentists better diagnose, monitor, and treat patients associated with both groups of conditions, highlighting the need for interdisciplinary care for patients with oral disorders and hematologic diseases.

[Changes of parameters associated with anemia of inflammation in patients with stage â ¢ periodontitis before and after periodontal initial therapy].

OBJECTIVE: To investigate the differences and similarities of parameters associated with anemia of inflammation between patients with stage Ⅲ periodontitis and periodontally healthy volunteers, and to explore the influence of periodontal initial therapy on those indicators. METHODS: Patients with stage Ⅲ periodontitis and periodontally healthy volunteers seeking periodontal treatment or prophylaxis at Department of Periodontology, Peking University School and Hospital of Stomatology from February 2020 to February 2023 were enrolled. Their demographic characteristics, periodontal parameters (including probing depth, clinical attachment loss, bleeding index), and fasting blood were gathered before periodontal initial therapy. Three months after periodontal initial therapy, the periodontal parameters of the patients with stage Ⅲ periodontitis were re-evaluated and their fasting blood was collected again. Blood routine examinations (including white blood cells, red blood cells, hemoglobin, packed cell volume, mean corpuscular volume of erythrocytes, and mean corpuscular hemoglobin concentration) were performed. And ferritin, hepcidin, erythropoietin (EPO) were detected with enzyme-linked immunosorbent assay (ELISA). All data analysis was done with SPSS 21.0, independent sample t test, paired t test, and analysis of covariance were used for comparison between the groups. RESULTS: A total of 25 patients with stage Ⅲ periodontitis and 25 periodontally healthy volunteers were included in this study. The patients with stage Ⅲ periodontitis were significantly older than those in periodontally healthy status [(36.72±7.64) years vs. (31.44±7.52) years, P=0.017]. The patients with stage Ⅲ periodontitis showed lower serum hemoglobin [(134.92±12.71) g/L vs. (146.52±12.51) g/L, P=0.002] and higher serum ferritin [(225.08±103.36) µg/L vs. (155.19±115.38) µg/L, P=0.029], EPO [(41.28±12.58) IU/L vs. (28.38±10.52) IU/L, P < 0.001], and hepcidin [(48.03±34.44) µg/L vs. (27.42±15.00) µg/L, P=0.009] compared with periodontally healthy volunteers. After adjusting the age with the covariance analysis, these parameters (hemoglobin, ferritin, EPO, and hepcidin) showed the same trends as independent-sample t test with statistical significance. Three months after periodontal initial therapy, all the periodontal parameters showed statistically significant improvement. The serum hemoglobin raised [(146.05±15.48) g/L vs. (133.77± 13.15) g/L, P < 0.001], while the serum ferritin [(128.52±90.95) µg/L vs. (221.22±102.15) µg/L, P < 0.001], EPO [(27.66±19.67) IU/L vs. (39.63± 12.48) IU/L, P=0.004], and hepcidin [(32.54±18.67) µg/L vs. (48.18±36.74) µg/L, P=0.033] decreased compared with baseline. CONCLUSION: Tendency of iron metabolism disorder and anemia of inflammation was observed in patients with stage Ⅲ periodontitis, which can be attenuated by periodontal initial therapy.

Fanconi anemia-isogenic head and neck cancer cell line pairs: A basic and translational science resource.

Fanconi anemia (FA) is a heritable malformation, bone marrow failure and cancer predisposition syndrome that confers an exceptionally high risk of squamous carcinomas. These carcinomas originate in epithelia lining the mouth, proximal esophagus, vulva and anus: their origins are not understood, and no effective ways have been identified to prevent or delay their appearance. Many FA-associated carcinomas are also therapeutically challenging: they may be multi-focal and stage-advanced at diagnosis, and most individuals with FA cannot tolerate standard-of-care systemic therapies such as DNA cross-linking drugs or ionizing radiation due to constitutional DNA damage hypersensitivity. We developed the Fanconi Anemia Cancer Cell Line Resource (FA-CCLR) to foster new work on the origins, treatment and prevention of FA-associated carcinomas. The FA-CCLR consists of Fanconi-isogenic head and neck squamous cell carcinoma (HNSCC) cell line pairs generated from five individuals with FA-associated HNSCC, and five individuals with sporadic HNSCC. Sporadic, isogenic HNSCC cell line pairs were generated in parallel with FA patient-derived isogenic cell line pairs to provide comparable experimental material to use to identify cell and molecular phenotypes driven by germline or somatic loss of Fanconi pathway function, and the subset of these FA-dependent phenotypes that can be modified, complemented or suppressed. All 10 FANC-isogenic cell line pairs are available to academic, non-profit and industry investigators via the "Fanconi Anemia Research Materials" Resource and Repository at Oregon Health & Sciences University, Portland OR.

The tendency of anemia of inflammation in periodontal diseases.

Anemia of inflammation (AI) is associated with inflammatory diseases, and inflammation-induced iron metabolism disorder is the major pathogenic factor. Earlier studies have reported a tendency of AI in periodontitis patients, but the explicit relationship and possible pathological mechanisms remain unclear. Here, the analyses of both periodontitis patients and a mouse model of ligature-induced experimental periodontitis showed that periodontitis was associated with lower levels of hemoglobin and hematocrit with evidence of systemic inflammation (increased white blood cell levels) and evidence of iron restriction (low serum iron along with a high serum hepcidin and ferritin levels), in accordance with the current diagnosis criteria for AI. Moreover, periodontal therapy improved the anemia status and iron metabolism disorders. Furthermore, the increased level of hepcidin and significant correlation between hepcidin and key indicators of iron metabolism emphasized the pivotal role of hepcidin in the pathogenesis of periodontitis-related AI. Administration of the signal transducer and activator of transcription 3 (STAT3) inhibitors Stattic suggested that the IL-6-STAT3-hepcidin signaling pathway participated in this regulatory process. Together, these findings demonstrated that periodontitis should be considered an inflammatory disease that contributes to the development of AI; furthermore, IL-6-STAT3-hepcidin signaling pathway plays a key regulatory role in the pathogenesis of periodontitis-related AI. Our study will provide new insights into the systemic effects of periodontitis, while meaningfully expanding the spectrum of inflammatory diseases that contribute to AI.

Association between anemia and depression: results from NHANES 2005-2018 and mendelian randomization analyses.

BACKGROUND: The relationship between anemia and depression remains controversial. OBJECTIVE: To explore the association between anemia/hemoglobin and depression. METHODS: The data for our cross-sectional study were obtained from the National Health and Nutrition Examination Survey (NHANES) 2005-2018. Weighted multivariate logistic regression was performed to examine the association between anemia/hemoglobin and depression. Inverse variance weighted (IVW), weighted-median, and MR-Egger were used in MR analyses to assess the causal relationship between anemia/hemoglobin and depression. Heterogeneity and directional pleiotropy were assessed using the Cochrane Q test and Egger-intercept test, respectively. Sensitivity analysis was conducted by the leave-one-out approach. All analyses were carried out using IBM SPSS 24.0 and R version 4.2.2. RESULTS: A total of 29,391 NHANES participants were included in this study. After adjusting for all covariates, the association between anemia/hemoglobin and depression was not significant (P < 0.05). IVW estimates revealed that broad anemia had no significant effect on the risk of depression (OR = 1.00, 95% CI = 0.99-1.01, P = 0.432). Findings of weighted median and MR-Egger were consistent with those from IVW (weighted median: OR = 1.00, 95% CI = 0.99-1.02; P = 0.547; MR-Egger: OR = 1.01, 95% CI = 0.98-1.03, P = 0.605). The results of three MR Analyses methods also showed no causal association between hemoglobin and depression. CONCLUSIONS: Our findings do not support a causal association between anemia and depression. The association between hemoglobin concentration and depression was not statistically significant either.

Immunohematological evaluation of red cell alloimmunization and statistical assessment of various adsorption techniques in warm autoimmune hemolytic anemia.

Adsorption techniques are widely applied to detect underlying masked alloantibodies in warm autoimmune hemolytic anemia (WAIHA). We established various adsorption techniques with an aim to detect alloimmunization in WAIHA This study conducted over a period of nine years included 298 patients of WAIHA. Complete immunohematological evaluation was performed on these 298 samples following departmental protocols. Clinical and laboratory details of patients were obtained from patient files. Various adsorption methods were performed and statistically evaluated in the study. Out of 479 cases of autoimmune hemolytic anemia, WAIHA comprised of 62.2 % (N = 298). A total of 139 (46.6 %) serum samples revealed autoantibodies. Adsorption study was performed in 101 (72.7 %) indicated samples and 24 (23.8 %) of these showed 26 alloantibodies. Among the patients subjected to adsorption study hemolytic marker were significantly deranged in the alloimmunization group (p < 0.01). Polyethylene glycol (PEG) adsorption was the quickest (52.2-54.6 min) of all adsorption techniques with minimum (1.3-1.5) numbers of adsorptions needing for complete removal of serum antibodies. The LISS-papain (LP) technique was found to be more sensitive and specific compared to the other two techniques. The agreement between PEG adsorption and LP adsorption was found to be 'perfect' (96.4 %) with a Cohen's kappa (k) value of 0.9. We conclude that identification of alloantibody specificities underlying a warm autoantibody is critical for a safe and effective transfusion. All WAIHA patients with history of blood transfusion, pregnancy or both should be subjected to adsorption study. Selection of a suitable adsorption technique depends on multiple important factors.

Low hemoglobin levels predict increased radiation-induced trismus rates in nasopharyngeal cancer.

PURPOSE: To investigate the predictive significance of hemoglobin (Hb) values in the incidence of radiation-induced trismus (RIT) in locally advanced nasopharyngeal carcinoma (LA-NPC) patients who received concurrent chemoradiotherapy (C-CRT). METHODS: Data of LA-NPC patients were examined before and after C-CRT and to confirm the presence of RIT, maximum mouth openings (MMO) were measured; RIT is defined as an MMO of ≤35 mm. All Hb values were derived from complete blood count tests obtained on the first day of C-CRT. The receiver operating characteristic (ROC) curve analysis was used to scrutinize a possible connection between pre-treatment Hb values and RIT status. RESULTS: Two hundred and twenty three patients were included in the study and RIT was diagnosed in 46 (20.6%) patients. The Hb cutoff in ROC curve analysis that separated the patients into two groups was 12.05 g/dL [Area under the curve (AUC): 82.7%; sensitivity: 72.9%; and specificity: 71.3%]. RIT was significantly more prevalent in the Hb ≤ 12 g/dL group than in its counterpart (41.9% vs. 7.3%; p < 0.001). In multivariate analysis, Hb ≤ 12, anemia, pre-C-CRT MMO < 41.4 mm, and masticatory apparatus doseV58 Gy < 32% groups were found to be independently associated with significantly increased rates of RIT. CONCLUSION: Low pre-C-CRT Hb and anemia status are novel biological markers that independently predict higher RIT rates in LA-NPC undergoing C-CRT.

Mechanisms underlying the adaptive pulp and jaw bone trabecular changes in sickle cell anemia.

OBJECTIVE: Mechanisms underlying the oral outcomes in sickle cell anemia (HbSS) have been less explored. This study aimed to investigate the association of morbimortality indicators and hydroxyurea use with adaptive pulp and jaw bone trabecular changes in HbSS. METHODS: This cross-sectional study included 123 individuals with HbSS. The exposures were the morbimortality indicators of HbSS (number of vaso-occlusive crises, organ damage, hemoglobin level, and leukocyte count) and the use of hydroxyurea for HbSS treatment. The outcomes were adaptive pulp and jaw bone trabecular changes confirmed by radiographic examination. Associations were estimated by Poisson regression in crude and adjusted analyses for sex, skin color, socioeconomic class, and age. RESULTS: The vaso-occlusive crises (mean ratio (MR) = 3.5, p = 0.045), lower hemoglobin (MR = 2.4, p = 0.037), and higher leukocyte count (MR = 2.17, p = 0.036) were risk factors, while the use of hydroxyurea was inversely associated with adaptive pulp changes (MR = 0.23, p = 0.024). The vaso-occlusive crises were associated with jaw bone trabecular changes (MR = 1.33, p = 0.02). CONCLUSION: Adaptive pulp changes may be a potential clinical marker of chronic vasculopathy in HbSS. The use of hydroxyurea may reduce the frequency of adaptive pulp changes.

Anemia as a risk factor for restenosis in femoropopliteal arterial disease after drug-coated balloon angioplasty.

OBJECTIVE: This study aimed to investigate the relationship between anemia and restenosis in patients with femoropopliteal arterial disease following drug-coated balloon (DCB) angioplasty. METHODS: 194 patients treated with DCB for femoropopliteal lesions were retrospectively analyzed for up to 12 months of follow-up between January 2017 and September 2020. Baseline clinical and procedural characteristics were compared between the anemia and non-anemia patients, and predictors of restenosis were identified using logistic regression. RESULTS: 32.5% of the patients undergoing DCB angioplasty had anemia. Patients with anemia were significantly older, with higher rates of hypertension, coronary artery disease, chronic renal insufficiency, and diabetes, and with lower rates of smoking and male gender. In the multivariate analysis, anemia was independently and significantly associated with a higher risk of restenosis (OR, 3.872; 95% CI, 1.556-9.638; P = 0.004). CONCLUSION: Anemia is independently associated with restenosis in patients treated with DCB for femoropopliteal arterial disease. Patients with lower baseline hemoglobin might have more chances to develop restenosis at follow-up.

Craniofacial Characteristics and Orthodontic Treatment of Diamond Blackfan Syndrome: Two Case Reports.

Diamond Blackfan anemia (DBA) is a chronic congenital form of erythrocytic hypoplasia in which erythroid precursor cell levels are low. DBA reflects ribosomal dysfunction and is accompanied by hematopoietic cell apoptosis, anemia, and various somatic symptoms. We report the characteristic symptoms of the craniofacial region and the orthodontic treatments of two DBA cases. Case 1 was a 12-year-old female. The typical physical and facial characteristics of DBA were lacking. On initial examination, she exhibited a skeletal Class II jaw and end to end molar relationships and a large overjet. An edgewise appliance was placed after extraction of the first maxillary premolars. After 3 years and 11 months, an appropriate overjet and overbite, rigid intercuspation, and an acceptable profile were evident without any clinical adverse effects. Case 2 was a 13-year-old female. She exhibited a skeletal Class I jaw relationship, a spaced dental arch, the maxillofacial dysplasia characteristic of Binder syndrome, hypoplasia of the right mandibular condyle, and labial protrusions of the maxillary and mandibular incisors. We placed an edgewise appliance and after 1 year and 7 months, the occlusion was optimal in the absence of any adverse effects. Our two DBA cases exhibited a broad spectrum of physical and dentofacial symptoms. Patients with DBA are often prescribed combined steroid/bisphosphonate therapies. Both agents are likely to affect alveolar bone remodeling after tooth extraction and orthodontic tooth movement. Careful consideration of medication with reference to various dentofacial characteristics is necessary.

The Impact of Anemia-Related Early Childhood Caries on Parents' and Children's Quality of Life.

Background and Objectives: Today, oral diseases are well-known for their effects, not only on daily life but also on quality of life (QoL). Dental caries, especially early childhood caries (ECC), are considered a public health concern as regards their impact on the life quality of children and parents from multiple aspects. The present research was conducted to assess the effect of anemia on oral-health-related quality of life (OHRQoL) in terms of children and parents. Materials and Methods: The current study was performed in two independent stages. In the first stage, the Turkish version of the Early Childhood Oral Health Impact Scale (ECOHIS), and in the second stage, the Turkish version of the Parental-Caregivers Perceptions Questionnaire (P-CPQ) were used to measure the effect of anemia-related dental caries among children and parents. SPSS and Jamovi software were used for all calculations, graphs and comparisons. Results: A total of 204 participants (child-parent pairs) were incorporated in the present study. A considerable number of children (81.5%) reported occasional or more frequent oral/dental pain. Secondly, the subscale scores were determined for child symptoms (2.25 ± 0.067), child function (6.8 ± 0.22), child psychology (3.87 ± 0.128), self-image and social interaction (1.74 ± 0.063), parental distress (3.82 ± 0.143), and family function (3.5 ± 0.121). Additionally, more than half of the parents (56.3%) responded "fair" for the health of their children's teeth, lips, jaws and mouth. Similarly, the child's overall well-being was stated as being affected "a lot" by the condition of their child's teeth, lips, jaws or mouth by half of the parents (49.5%). Conclusions: Anemia-related dental caries has a highly negative impact on the quality of life of children and parents according to both of the questionnaires. Therefore, children with high scores should be prioritized for preventive procedures and timely dental treatments.

Iron deficiency anemia associated factors and early childhood caries in Qingdao.

BACKGROUND: Iron deficiency anemia (IDA) has been shown to be related to early childhood caries (ECC). However, data on the relationship, if any, between IDA-associated factors and ECC remain scant. This study aimed to explore the interplay between IDA-associated factors and ECC. METHODS: This study randomly sampled a total of 1598 children in Qingdao city, and analyzed the severity of ECC using decayed-missing-filled teeth index, while the rate of caries was analyzed following the WHO recommendations. The correlation between IDA and ECC was analyzed by both the chi-square test and Mann-Whitney U test. In addition, we designed an electronic questionnaire and employed the disordered multi-classification logistic regression to interrogate the relationship between the IDA-associated factors and ECC. RESULTS: Children with IDA had higher rates and severe ECC than those without IDA (p < 0.001). Children who were breastfed until 2 years old had a higher risk of IDA and ECC, compared to those who were not {OR 3.453 (1.681-7.094)}. Compared with children who had no history of IDA at the age of 2 years or below, those with IDA history had a higher risk of IDA and ECC {OR 8.762 (3.648-21.041)}. In addition, children who had a maternal history of IDA at pregnancy were at a higher risk of IDA and ECC compared to those who had no IDA history at pregnancy {OR 4.913 (2.934-8.226)}. Our data showed that children from a family with an annual income lower than 50,000 Renminbi (RMB) had a higher risk of IDA and ECC compared to those with an annual family income higher than 200,000 RMB {OR 3.421 (1.505-7.775)}. On the other hand, compared with children taking iron supplements, children who did not were at a higher risk of ECC and IDA {OR 5.602 (1.858-16.896)}. CONCLUSION: Factors such as low family income, history of IDA in children aged 2 years or younger, IDA history during pregnancy, children breastfed until 2 years old, and those not taking iron supplements were significantly associated with the occurrence of ECC and IDA.

Daprodustat Compared with Epoetin Beta Pegol for Anemia in Japanese Patients Not on Dialysis: A 52-Week Randomized Open-Label Phase 3 Trial.

BACKGROUND: Daprodustat is an oral agent that stimulates erythropoiesis by inhibiting the prolyl hydroxylases which mark hypoxia-inducible factor for degradation through hydroxylation. Its safety and efficacy (noninferiority) were assessed in this 52-week, open-label study. METHODS: Japanese patients not on dialysis (ND) (N = 299) with anemia of CKD (stages G3, G4, and G5) with iron parameters of ferritin >100 ng/mL or transferrin saturation >20% at screening were randomized to daprodustat or epoetin beta pegol (continuous erythropoietin receptor activator [CERA], also known as methoxy polyethylene glycol-epoetin beta). After initiation of the study, the daprodustat starting dose for erythropoiesis-stimulating agent (ESA)-naïve participants was revised, and daprodustat was started at 2 or 4 mg once daily depending on baseline hemoglobin. ESA users switched to daprodustat 4 mg once daily. CERA was started at 25 µg every 2 weeks for ESA-naïve patients and 25-250 µg every 4 weeks for ESA users based on previous ESA dose. In both treatment groups, dose was adjusted every 4 weeks based on hemoglobin level and changed according to a prespecified algorithm. The primary endpoint was mean hemoglobin level during weeks 40-52 in the intention-to-treat (ITT) population. ESA-naïve patients who entered before the protocol amendment revising the daprodustat starting dose were excluded from the ITT population. RESULTS: Mean hemoglobin levels during weeks 40-52 were 12.0 g/dL in the daprodustat group (n = 108; 95% confidence interval [CI], 11.8-12.1) and 11.9 g/dL for CERA (n = 109; 95% CI 11.7-12.0); the difference between the groups was 0.1 g/dL (95% CI -0.1 to 0.3 g/dL). The lower limit of the 95% CI of the difference was greater than the prespecified margin of -1.0 g/dL. The mean hemoglobin level was within the target range (11.0-13.0 g/dL) during weeks 40-52 for 92% of participants in both groups. There was no meaningful difference in the frequencies of adverse events. CONCLUSIONS: Oral daprodustat was noninferior to CERA in achieving and maintaining target hemoglobin levels in Japanese ND patients. Daprodustat was well tolerated, with no new safety concerns identified.

Effectiveness and safety of tacrolimus with or without eltrombopag, as a part of immunosuppressive treatment of aplastic anemia in adults: a retrospective case series.

First-line treatment of aplastic anemia(AA) and for AA patients ineligible for hematopoietic stem cell transplantation (HSCT) has consisted of antithymocyte globulin (ATG), the calcineurin inhibitor cyclosporine A (CsA), and more recently eltrombopag. However, at our institution, we have successfully substituted another calcineurin inhibitor, tacrolimus, as a part of immunosuppressive threatment (IST) for AA due to more favorable toxicity profile. Since there is limited data on the use of tacrolimus in aplastic anemia, we conducted a retrospective review of twenty patients treated with tacrolimus-based immunosuppressive therapy (IST) as a first- or second-line treatment. The overall response rate was comparable to that of patients treated with CsA (18 patients). However, there were no cutaneous side effects observed in patients receiving tacrolimus, a relatively common finding with CsA use. Our data suggest that tacrolimus-based IST is a potential option in AA and might have a more favorable toxicity profile compared to CsA.

Potential effective treatment of shortening continuous erythropoietin receptor activator treatment interval combined with iron supplementation in hemodialysis patients.

Our previous randomized controlled trial comparing the total dose of weekly versus biweekly continuous erythropoietin receptor activator (CERA) therapy to maintain optimal hemoglobin (Hb) levels showed no significant differences between the two therapies. This post-hoc analysis assessed whether the total dose of weekly versus biweekly CERA therapy to maintain Hb levels among HD patients differed among groups with or without iron supplementation. Of 107 patients, 40 received intravenous iron supplementation due to iron deficiency (iron group) and 67 did not (non-iron group). In the iron group, the weekly therapy tended to require a lower total CERA dose compared with the biweekly therapy (274 ± 274 vs 381 ± 223 µg/12 weeks, P = 0.051). Changes in circulating hepcidin levels, a negative regulator of intestinal iron uptake, after 2 weeks of CERA treatment were significantly lower in the weekly therapy compared with the biweekly therapy (-4.2 ± 6.3 vs 11.1 ± 7.3 ng/mL, P = 0.015). In the non-iron group, there were no significant differences in total CERA dose or changes in hepcidin levels between the two therapies. Shortening the CERA treatment interval combined with iron supplementation may lead to the more efficient treatment of HD patients with iron deficiency.

A case series of medication-related fibrovascular hyperplasia following hematopoietic stem cell transplantation for Fanconi anemia.

Systemic medications categorized as diphenylhydantoin, calcineurin inhibitor and calcium channel blocker may have effects on the oral cavity by modifying the inflammatory and immune response and causing undesired tissue proliferative reactions. Calcineurin inhibitors are medications commonly used for long periods in patients undergoing allogeneic hematopoietic stem cell transplant (HSCT) and solid organ transplantation. Medication-related fibrovascular hyperplasia (MRFH) is an extra gingival hyperplastic nodular growth associated with medications use. This study reports five cases of pediatric patients (6 to 12-years-old) diagnosed with Fanconi anemia (FA) after HSCT who presented similar oral mucosal lesions associated with the use of cyclosporine, phenobarbital and amlodipine. After excision of the lesions, histopathological analysis described them as pyogenic granuloma (PG). As the aetiology of the lesions manifested by the patients was associated with the use of medications, the final diagnosis was MRFH. Despite the clinical and histopathological similarity between PG and MRFH, it is fundamental to know the aetiological agent for achieving definitive diagnosis and correct management. Considering the etiologic agent (medication) and histopathological findings, it is suggested that the most appropriate term for this manifestation should be "medication-related fibrovascular hyperplasia". The correct nomenclature related to extra gingival hyperplastic lesions identified in patients on medications with potential to induce hyperplastic reactions should be adopted to facilitate scientific communication and improve the treatment.

Efficacy of continuous erythropoietin receptor activator for end-stage renal disease patients with renal anemia before and after peritoneal dialysis initiation.

BACKGROUND: Serial management of renal anemia using continuous erythropoietin receptor activator (CERA) throughout the peritoneal dialysis initiation period has rarely been reported. We investigated the efficacy and dosage of CERA treatment from pre- to post-peritoneal dialysis initiation for anemia management in patients with end-stage renal disease. METHODS: Twenty-six patients (13 men; mean age 60.9 years) who started peritoneal dialysis between April 2012 and April 2018 were investigated. Serial changes in hemoglobin levels, transferrin saturation and ferritin levels, CERA dosage, and the erythropoietin resistance index (ERI) over a 48 week period were retrospectively examined. RESULTS: Mean hemoglobin levels increased significantly from 10.5 g/dL at 24 weeks prior to the peritoneal dialysis initiation to 11.5 g/dL at 4 weeks post-initiation. The proportion of patients with hemoglobin levels ≥ 11 g/dL increased significantly after peritoneal dialysis initiation. The mean CERA dosage was 57.0 µg/month at 24 weeks prior to dialysis initiation, 86.5 µg/month at initiation, and 72.0 µg/month at 4 weeks post-initiation. Thus, the dosage tended to increase immediately before peritoneal dialysis initiation and then decreased thereafter. Hemoglobin levels were significantly lower, while the CERA dosage for maintaining hemoglobin levels and ERI tended to be higher at dialysis initiation in patients with diabetes than in those without diabetes. CONCLUSION: Treatment with CERA prior to and during the peritoneal dialysis initiation achieved fairly good anemia management in patients with and without diabetes. The CERA dosage could be reduced in patients without diabetes after dialysis initiation.

Toxic stress, health and nutrition among Brazilian children in shelters.

BACKGROUND: Living in a shelter is an adverse experience that generates toxic stress. This situation can cause the dysregulation of the hypothalamic-pituitary-adrenal axis and exert a negative impact on health.The aim of the present study was to determine the association between toxic stress and social, clinical and nutritional characteristics in children at welfare institutions in a city of northeastern of Brazil. METHODS: An analytical, cross-sectional study was conducted with male and female children up to 60 months of age who live in shelters. Hair cortisol was used for the assessment of stress (immunoassay). The anthropometric data collected were height for age, body mass index for age, arm circumference for age, and head circumference for age (expressed in z-scores). We also evaluated food intake using markers proposed by the Brazilian Dietary and Nutritional Vigilance Surveillance System as well as the occurrence of dental caries and anemia. RESULTS: Sixty-three children one to 60 months of age participated in the present study. Asthma was the most frequent disease (11.1%). The prevalence of short stature, anemia and dental caries in the sample was 22.2, 22.2 and 9.4%, respectively. Cortisol levels ranged from 0.93 pg/mg to 391.29 pg/mg (median: 6.17 pg/mg). Higher cortisol levels were found in children with illnesses (p = 0.012) and those who had been hospitalized after being admitted to the institutions (p = 0.001). CONCLUSIONS: The majority of children had unhealthy eating behavior. The cortisol concentrations found in the present study were suggestive of dysregulation of the hypothalamic-pituitary-adrenal axis. Hypercortisolism was associated with illness and hospitalization.

Associations of early childhood caries with salivary beta defensin-3 and childhood anemia: a case-control study.

BACKGROUND: Human beta defensin-3 (HßD-3) is an antimicrobial peptide present in saliva that protects tooth surfaces from microbial attack. These peptides are part of innate immunity so levels may be affected by different systemic diseases like anemia. Therefore, anemia may predispose an affected child to an increased risk of dental caries. The objectives of this study were to determine the association of early childhood caries (ECC) with HßD-3 levels and observe the association of HßD-3 levels with childhood anemia. METHODS: A total of 80 children admitted in a pediatric medical ward, age 48-71 months, of either sex were included in the study. The included children were categorized as cases (children with ECC n = 40) and controls (children without ECC n = 40). Children were further segregated into the anemic and non-anemic sub-groups based on the hospital record of hemoglobin level. The salivary concentration of HßD-3 was measured by Enzyme-Linked Immuno-sorbent assay (ELISA). IBM SPSS version 20 software was used for statistical analysis. Two sample t-test and one-way ANOVA were used to compare mean values while spearman was used for correlations at p < 0.05. RESULTS: The mean Salivary HßD-3 level in cases (8.87 ± 4.30) was significantly higher (p = 0.042) as compared to controls (7.23 ± 2.57). Salivary HßD-3 level in patients with caries and without anemia was highest (10.80 ± 4.50) whereas salivary HßD-3 level in the presence of caries and anemia was lowest (6.94 ± 3.13) amongst all groups. This difference was statistically significant (p = 0.001). Salivary HßD-3 level was found to be moderately correlated with cases (p = 0.002). An inverse correlation was found between salivary HßD-3 level and anemia (r = -0.479, p = 0.002). CONCLUSION: Anemia may affect the innate immunity of children, and may result in a decreased level of salivary HßD3, thus increasing vulnerability to decay.