bluuebunny/crossref_metadata_2025_split huggingface dataset:
Comparison of Optic Canal Diameter in Children With Malignant Infantile Osteopetrosis and Normal Children and the Effects of Hematopoietic Stem Cell Transplantation on the Optic Canal Diameter
Authors: Cao Wenhong , Wei Wenbin , Yu Gang , Wu Qian , Qin Maoquan 
Published: October 2018

Purpose:
To investigate the difference in the optic canal diameter between children with autosomal recessive malignant infantile osteopetrosis and normal children, and to assess the influence of hematopoietic stem cell transplantation on the optic canal diameter.

Methods:
Twenty pediatric patients with malignant infantile osteopetrosis and 22 normal control children were included in this study. Eleven patients with malignant infantile osteopetrosis underwent hematopoietic stem cell transplantation. The measurements included optical canal diameter and flash visual evoked potential. Comparisons of these measurements between patients with malignant infantile osteopetrosis and normal controls as well as before and after hematopoietic stem cell transplantation were performed. The correlation between age and optic canal diameter was analyzed using Pearson correlation analysis.

Results:

The mean optic canal diameter before hematopoietic stem cell transplantation was 1.65 ± 0.54 mm in patients with malignant infantile osteopetrosis and 3.38 ± 0.60 mm in the control group (
P
< .001). The mean optic canal diameter after hematopoietic stem cell transplantation was 2.72 ± 0.66 mm, which was significantly different from the pre-transplantation measurement (
P
< .001). The P2 latency for the flash visual evoked potential after hematopoietic stem cell transplantation (152.3 ± 36.4 ms) was significantly less than that before transplantation (165.5 ± 27.7 ms;
P
= .051). Pearson correlation analysis revealed a significant correlation between age and optic canal diameter (
r
= 0.722,
P
< .001).

Conclusions:
The optic canal was narrower in pediatric patients with malignant infantile osteopetrosis than in age-matched normal controls. This condition can be relieved through hematopoietic stem cell transplantation and the impaired conductibility of the optic nerve can be improved in some cases.

[
J Pediatr Ophthalmol Strabismus.
2019;56(1):35–42.]
Efficacy of Office-Based Nasolacrimal Duct Probing
Authors: Bach Austin , Vanner Elizabeth Ann , Warman Roberto 
Published: October 2018

Purpose:
To analyze the efficacy of nasolacrimal duct probing conducted in the office for nasolacrimal duct obstruction.

Methods:
A retrospective chart review was conducted of 1,294 patients. Of those, 1,227 patients who underwent office-based nasolacrimal probings of the nasolacrimal duct at a single tertiary care center were included.

Results:
A total of 82 (6.7%) patients needed reprobing. Of the 82 patients who underwent a second procedure, 35 (43%) underwent a second in-office probing with a success rate of 77%. The 8 (22%) patients who failed the second in-office probing underwent probing and Crawford stent placement in the operating room and their symptoms resolved. For the 47 (57%) patients who failed the primary in-office probing and underwent operating room probing and stent placement, only 1 (2%) needed a second operating room probing and stent placement. Logistic regression analyses indicated an increased likelihood of needing a secondary procedure with increased age at the time of the first probing.

Conclusions:
This large, retrospective analysis of office-based probings demonstrated a success rate of 93.3% with increased likelihood of not needing a second procedure with probing at a younger age. This study demonstrates an excellent success rate for in-office probings for patients both younger and older than 12 months. This not only shows a high rate of efficacy, but is also highly cost efficient when compared to primary probing in the operating room.

[
J Pediatr Ophthalmol Strabismus
. 2019;56(1):50–54.]
Surgical Outcome of Single Inferior Oblique Myectomy in Small and Large Hypertropia of Unilateral Superior Oblique Palsy
Authors: Akbari Mohammad Reza , Sadrkhanlou Samira , Mirmohammadsadeghi Arash 
Published: October 2018

Purpose:
To determine the efficacy of isolated inferior oblique myectomy on hypertropia in primary position, side gazes, and tilts, and its effect on comitancy and abnormal head posture in unilateral superior oblique palsy.

Methods:
Thirty-nine patients with unilateral superior oblique palsy who had inferior oblique overaction underwent inferior oblique myectomy. The hypertropia was measured in primary position, side gazes, and tilts preoperatively and postoperatively. Abnormal head posture was also assessed. Success was defined as primary position hypertropia of 5 prism diopters (PD) or less.

Results:

The mean distance hypertropia was 15.7 ± 7.7 PD (range: 3 to 30 PD) preoperatively and 1.5 ± 3.3 PD (range: 0 to 16 PD) postoperatively (
P
< .001). The mean reduction of distance hypertropia postoperatively was 14.2 ± 7.8 PD (range: 3 to 30 PD). The contralateral gaze hypertropia decreased from 21.7 ± 9.0 PD (range: 5 to 45 PD) preoperatively to 3.6 ± 5.1 PD postoperatively (range: 0 to 20 PD) and ipsilateral head tilt hypertropia decreased from 21.9 ± 8.4 PD (range: 8 to 40 PD) preoperatively to 5.0 ± 5.9 PD (range: 0 to 24 PD) postoperatively (
P
< .000 for both). Incomitance (contralateralipsilateral gaze hypertropia) decreased from 15.0 ± 7.4 PD (range: 3 to 35 PD) preoperatively to 2.8 ± 4.1 PD (range: 0 to 16 PD) postoperatively (
P
< .001). The success rate between the two groups of patients who had hypertropia of 15 PD or less and greater than 15 PD in primary position was not statistically different (94.7% vs 85%). Two patients underwent a second operation for residual hypertropia. There was no overcorrection. Thirty-two patients had abnormal head posture, which resolved postoperatively in 29 cases.

Conclusions:
Isolated inferior oblique myectomy is an effective procedure in resolving hypertropia up to 30 PD due to unilateral superior oblique palsy, with a minimal risk of overcorrection and low risk of undercorrection. It decreases incomitancy, is a self-titrating procedure, and resolves abnormal head posture efficiently.

[
J Pediatr Ophthalmol Strabismus
. 2019;56(1):23–27.]
Analysis of Macular Vessel Density and Foveal Avascular Zone Using Spectral-Domain Optical Coherence Tomography Angiography in Children With Amblyopia
Authors: Demirayak Bengi , Vural Asli , Onur Ismail Umut , Kaya Fatma Selin , Yigit Fadime Ulviye 
Published: October 2018

Purpose:
To quantify the foveal avascular zone and the whole, parafoveal, and foveal vessel density of superficial and deep capillary plexus in amblyopic eyes and age-matched controls and to compare the measurements.

Methods:

This cross-sectional study involved 49 eyes from 17 patients with amblyopia and 21 healthy children (aged 6 to 16 years). Optical coherence tomography angiography was performed for all participants and superficial capillary plexus, deep capillary plexus, and foveal avascular zone were evaluated. Data from amblyopic eyes, fellow eyes with unilateral amblyopia, and control eyes were compared using the Mann–Whitney
U
test.

Results:

The mean patient age was 8.6 ± 2.5 years in the amblyopia group and 9.6 ± 2.9 years in the control group. The mean foveal avascular zone measurements were 0.251 ± 0.1 mm
2
in the amblyopia group and 0.291 ± 0.1 mm
2
in the control group. The whole, foveal, and parafoveal vessel densities of superficial capillary plexus were 48.8% ± 3.7%, 23.8% ± 8.8%, and 50.9% ± 4.6% in the amblyopia group and 48.4% ± 2.5%, 19.3% ± 5.4%, and 51.3% ± 2.7% in the control group. The whole, foveal, and parafoveal vessel densities of deep capillary plexus were 51.8% ± 4.3%, 37.6% ± 5.8%, and 54.8% ± 4.2% in the amblyopia group and 54.4% ± 3.2%, 34.9% ± 7.4%, and 56.8% ± 3.2% in the control group. No statistically significant difference was detected in all measurements.

Conclusions:
Assessment of the foveal avascular zone and vessel density of superficial capillary plexus and deep capillary plexus with optical coherence tomography angiography revealed no difference between amblyopic eyes, controls, and fellow eyes of patients with unilateral amblyopia.

[
J Pediatr Ophthalmol Strabismus
. 2019;56(1):55–59.]
Descemet's Stripping With Endothelial Keratoplasty in 50 Eyes: A Refractive Neutral Corneal Transplant
Authors: Price Francis W , Price Marianne O 
Published: October 2018

ABSTRACT
PURPOSE: To evaluate early visual and refractive outcomes following treatment of corneal endothelial dysfunction with a corneal transplantation technique, Descemet's stripping with endothelial keratoplasty (DSEK).
METHODS: Visual and refractive outcomes of the first 50 consecutive cases of DSEK performed by a single surgeon between December 2003 and July 2004 were analyzed retrospectively. The DSEK technique consisted of stripping Descemet's membrane and endothelium from a recipient cornea and transplanting the posterior stroma and endothelium of a donor cornea through a 5-mm incision.
RESULTS: Results are reported for 50 eyes in 47 patients (30 women and 17 men). Mean patient age at surgery was 70±12 years (range: 34 to 89 years). Five eyes were treated for corneal edema or bullous keratopathy and 45 for Fuchs' endothelial dystrophy. Seven eyes were phakic and 43 were Pseudophakic. Six months after surgery, mean manifest cylinder was 1.5±0.94 diopters (D), unchanged from preoperative cylinder of 1.5±1.0 D. Mean manifest spherical equivalent refraction was 0.15 ±1.5 D at 6-month follow-up compared with -0.36±1.4 D preoperatively (P=.10) At 3- and 6-month follow-up, significant improvement was noted in mean best spectacle-corrected visual acuity compared with the preoperative mean of 20/100 (P=.007). At 6-month follow-up, 31 (62%) eyes refracted to ≥20/40 and 38 (76%) eyes saw ≥20/50.
CONCLUSIONS: Compared to standard penetrating keratoplasty, DSEK causes minimal refractive change and provides rapid visual recovery for patients with endothelial dysfunction. This technique maintains the structural integrity of the cornea by preserving the recipient's epithelium, Bowman's layer, and entire stromal thickness. [J Refract Surg. 2005;21:339-345.]
FcγRIIb on B Cells and Myeloid Cells Modulates B Cell Activation and Autoantibody Responses via Different but Synergistic Pathways in Lupus-Prone Yaa Mice
Authors: Lin Qingshun , Ohtsuji Mareki , Amano Hirofumi , Tsurui Hiromichi , Tada Norihiro , Sato Ryota , Fukuyama Hidehiro , Nishimura Hiroyuki , Verbeek J. Sjef , Hirose Sachiko 
Published: October 2018

Abstract
C57BL/6 (B6).FcγRIIb−/−.Yaa mice spontaneously develop lethal lupus nephritis. To define the cell type–specific role of FcγRIIb in Yaa-associated lupus, we established B cell– (CD19Cre.Yaa), myeloid cell– (C/EBPαCre.Yaa), and dendritic cell– (DC) (CD11cCre.Yaa) specific FcγRIIb-deficient B6.Yaa mouse strains. CD19Cre.Yaa mice developed milder lupus than B6.FcγRIIb−/−.Yaa mice, indicating that FcγRIIb deficiency on B cells is not sufficient for the development of severe disease. Surprisingly, C/EBPαCre.Yaa mice also showed autoantibody production and mild lupus similar to that in CD19Cre.Yaa mice, whereas CD11cCre.Yaa mice stayed disease free. These observations indicate that FcγRIIb deficiency in B cells and myeloid cells, but not DCs, contributes to the severe disease in B6.FcγRIIb−/−.Yaa mice. Flow cytometric analysis showed that the frequency of peripheral Gr-1− but not Gr-1+ monocyte was increased in B6.FcγRIIb−/−.Yaa and C/EBPαCre.Yaa but not CD19Cre.Yaa mice, suggesting a link between FcγRIIb deficiency on myeloid cells and the high frequency of Gr-1− monocytes. RNA sequencing revealed that compared with Gr-1+ monocytes, Gr-1− monocytes expressed higher levels of the B cell–stimulating cytokines BSF-3, IL-10, and IL-1β, the DC markers CD11c, CD83, and Adamdec1, and the antiapoptotic factors Bcl2 and Bcl6. In conclusion, in Yaa-associated lupus nephritis, FcγRIIb on B cells and myeloid cells modulates B cell activation via different but synergistic pathways. Gr-1− monocytes are the most likely candidate myeloid cells involved.
Seropositivity and Antibody Profiling of Patients Are Dramatically Impacted by the Features of Peptides Used as Immunosorbents: A Lesson from Anti–Citrullinated Protein/Peptide Antibody
Authors: Cornillet Martin , Babos Fruzsina , Magyar Anna , Sebbag Mireille , Verrouil Evelyne , Hudecz Ferenc , Serre Guy , Nogueira Leonor 
Published: October 2018

Abstract
Quantification of Abs toward a single epitope is critical to understanding immunobiological processes. In autoimmunity, the prognostic value of the serological profiles of patients draws much attention, but the detection of Abs toward a single epitope is not well controlled. Particularly, the rheumatoid arthritis (RA)–specific anti–citrullinated protein/peptide Abs (ACPA) are specific to a two-atom change on arginyl residues and are considered a heterogeneous family of Abs. As a model, we studied ACPA to decipher how peptide features used as immunosorbent impact Ab detection. We synthesized 30 peptides encompassing immunodominant epitopes of citrullinated fibrin differing by their length and biotin location and tested them using ELISA with 120 sera from RA and non-RA rheumatic disease controls, generating over 3000 experimental measurements. We showed that minor molecular changes in peptide chemical structure had dramatic consequences. Even when peptides exhibited the same epitope, measured Ab titers were extremely variable, and patients’ seropositivity was discordant in up to 50% of cases. The distance between epitope and biotin was the most critical parameter for efficient Ab detection irrespective of biotin position or peptide length. Finally, we identified a 15-mer peptide bearing a single citrullinated epitope detecting almost all ACPA-positive sera, thus revealing a high degree of homogeneity in RA autoimmune response. This integrative analysis deciphers the dramatic impact of the molecular design of peptide-based technologies for epitope-specific Ab quantification. It provides a model for assay development and highlights that the studies using such technologies can give a wrong perception of biological processes and therefore that medical use of data must be cautious.
Thymic Epithelial Cell Support of Thymopoiesis Does Not Require Klotho
Authors: Xing Yan , Smith Michelle J. , Goetz Christine A. , McElmurry Ron T. , Parker Sarah L. , Min Dullei , Hollander Georg A. , Weinberg Kenneth I. , Tolar Jakub , Stefanski Heather E. , Blazar Bruce R. 
Published: October 2018

Abstract
Age-related thymic involution is characterized by a decrease in thymic epithelial cell (TEC) number and function parallel to a disruption in their spatial organization, resulting in defective thymocyte development and proliferation as well as peripheral T cell dysfunction. Deficiency of Klotho, an antiaging gene and modifier of fibroblast growth factor signaling, causes premature aging. To investigate the role of Klotho in accelerated age-dependent thymic involution, we conducted a comprehensive analysis of thymopoiesis and peripheral T cell homeostasis using Klotho-deficient (Kl/Kl) mice. At 8 wk of age, Kl/Kl mice displayed a severe reduction in the number of thymocytes (10–100-fold reduction), especially CD4 and CD8 double-positive cells, and a reduction of both cortical and medullary TECs. To address a cell-autonomous role for Klotho in TEC biology, we implanted neonatal thymi from Klotho-deficient and -sufficient mice into athymic hosts. Kl/Kl thymus grafts supported thymopoiesis equivalently to Klotho-sufficient thymus transplants, indicating that Klotho is not intrinsically essential for TEC support of thymopoiesis. Moreover, lethally irradiated hosts given Kl/Kl or wild-type bone marrow had normal thymocyte development and comparably reconstituted T cells, indicating that Klotho is not inherently essential for peripheral T cell reconstitution. Because Kl/Kl mice have higher levels of serum phosphorus, calcium, and vitamin D, we evaluated thymus function in Kl/Kl mice fed with a vitamin D–deprived diet. We observed that a vitamin D–deprived diet abrogated thymic involution and T cell lymphopenia in 8-wk-old Kl/Kl mice. Taken together, our data suggest that Klotho deficiency causes thymic involution via systemic effects that include high active vitamin D levels.
Innate Lymphoid Cells Have Decreased HLA-DR Expression but Retain Their Responsiveness to TLR Ligands during Sepsis
Authors: Cruz-Zárate David , Cabrera-Rivera Graciela Libier , Ruiz-Sánchez Bibiana Patricia , Serafín-López Jeanet , Chacón-Salinas Rommel , López-Macías Constantino , Isibasi Armando , Gallegos-Pérez Humberto , León-Gutiérrez Marco Antonio , Ferat-Osorio Eduardo , Arriaga-Pizano Lourdes , Estrada-García Iris , Wong-Baeza Isabel 
Published: October 2018

Abstract
Sepsis, one of the leading causes of death in intensive care units, is caused by a dysregulated host response to infection that leads to life-threatening organ dysfunction. The proinflammatory and anti-inflammatory responses activated by the infecting microorganism become systemic, and the sustained anti-inflammatory response induces a state of immunosuppression that is characterized by decreased expression of HLA-DR on monocytes, T cell apoptosis, and reduced production of TNF-α by monocytes and macrophages in response to TLR ligands. Innate lymphoid cells (ILCs) are lymphocytes that lack Ag-specific receptors and lineage-specific markers; they express HLA-DR and are activated by cytokines and by direct recognition of microbial molecules. In this study, we evaluated if ILCs are affected by the anti-inflammatory response during sepsis. We found that the number of peripheral blood ILCs was decreased in septic patients compared with healthy volunteers; this decrease was caused by a reduction in ILC1 and ILC3 and is associated with apoptosis, because ILCs from septic patients expressed active caspase 3. ILCs from septic patients had decreased HLA-DR expression but increased expression of the activating receptors NKp46 and NKp44; they also showed a sustained expression of CD127 (IL-7R α-chain) and retained their capacity to produce TNF-α in response to TLR ligands. These results indicate that during sepsis, ILCs have decreased HLA-DR expression and die via apoptosis, similar to monocytes and T cells, respectively. However, other effector functions of ILCs (activation through NKp46 and NKp44, TNF-α production) may remain unaffected by the immunosuppressive environment prevailing in septic patients.
Increased Mitochondrial Biogenesis and Reactive Oxygen Species Production Accompany Prolonged CD4+ T Cell Activation
Authors: Akkaya Billur , Roesler Alexander S. , Miozzo Pietro , Theall Brandon P. , Al Souz Jafar , Smelkinson Margery G. , Kabat Juraj , Traba Javier , Sack Michael N. , Brzostowski Joseph A. , Pena Mirna , Dorward David W. , Pierce Susan K. , Akkaya Munir 
Published: October 2018

Abstract
Activation of CD4+ T cells to proliferate drives cells toward aerobic glycolysis for energy production while using mitochondria primarily for macromolecular synthesis. In addition, the mitochondria of activated T cells increase production of reactive oxygen species, providing an important second messenger for intracellular signaling pathways. To better understand the critical changes in mitochondria that accompany prolonged T cell activation, we carried out an extensive analysis of mitochondrial remodeling using a combination of conventional strategies and a novel high-resolution imaging method. We show that for 4 d following activation, mouse CD4+ T cells sustained their commitment to glycolysis facilitated by increased glucose uptake through increased expression of GLUT transporters. Despite their limited contribution to energy production, mitochondria were active and showed increased reactive oxygen species production. Moreover, prolonged activation of CD4+ T cells led to increases in mitochondrial content and volume, in the number of mitochondria per cell and in mitochondrial biogenesis. Thus, during prolonged activation, CD4+ T cells continue to obtain energy predominantly from glycolysis but also undergo extensive mitochondrial remodeling, resulting in increased mitochondrial activity.
Evidence for Neuroinflammation after Myocardial Infarction in a Mouse Model
Authors: Gouweleeuw Leonie , Pol Christine , Simonides Warner S. , de Kleijn Dominique PV , Schoemaker Regien G. 
Published: October 2018

Background:
The cardiovascular system and central nervous system are known to influence each other. Accordingly, neurological changes may occur after myocardial infarction (MI), which may be mediated by neuroinflammation. We investigated tumor necrosis factor alpha (TNF-α) and microglia activation in post-MI neuroinflammation.

Materials and Methods:
MI or sham surgery was induced in 28 male mice. Two weeks later, we performed echocardiography and dissected the brains for the western blot on TNF-α and its receptors (n = 10) or immunohistochemical stainings for microglia, doublecortin X (DCX), and TNF-α (n = 18). Plasma was collected for the measurement of circulatory cytokines.

Results:
The MI mice had an average infarct size of 38% of the left ventricle, heart failure was confirmed by decreased fractional shortening and increased lung weight. Plasma cytokine levels were unaltered. In brains of MI mice, there was a higher expression of TNF-α precursor protein, with trends for higher TNF-R1 and lower TNF-R2 expression. Furthermore, MI mice had more activated microglia in the inner blade of the dentate gyrus of the hippocampus. The amount of neurogenesis measured by DCX staining was unaltered.

Conclusions:
Our mouse model of MI showed signs of persistent neuroinflammation as indicated by raised levels of TNF-α precursor protein and an increased number of activated microglia in the hippocampus. The extent to which these neuroinflammatory hallmarks influence central nervous system functioning remain to be determined.
Primary free fibula reconstruction in life-threatening haemorrhage from high flow arteriovenous malformation of mandible
Authors: Ravula Parvathi , Rangachari Srikanth , Susarla Rammurti , Sambari Laxman , Jammula Srinivas Saraswathi 
Published: October 2018

ABSTRACT
Background: High flow arteriovenous malformation (AVM) of the mandible is rare, but it can present as a life-threatening emergency with severe intraoral bleeding for the first time. The gold standard of treatment for an AVM of the mandible is selective embolisation combined with resection and subsequent reconstructions. With the advent of advanced multidisciplinary techniques aimed at definitive therapy, surgical resection and primary reconstruction can provide an ideal anatomical and functional cure. There are no previous reports on primary resection and reconstruction for life-threatening haemorrhage from high flow AVM of the mandible. Aim: We discuss our approach aimed at definitive therapy in life-threatening intraoral bleeding from large high flow AVM of the mandible. Subjects and Methods: Four patients were managed for life-threatening intraoral bleeding during 2015–2017. Compression was applied over the bleeding point before the airway could be secured by endotracheal tube. Under general anaesthesia, the external carotid artery (ECA) was temporarily occluded using an umbilical tape loop ligature to control the bleeding. Emergency selective embolisation was done, followed by curative resection and primary mandible reconstruction using free fibula flap. Outcome assessed. Results: Temporary occlusion of the ECA successfully controlled the bleeding immediately and facilitated selective embolisation and definitive therapy. All the four cases were successfully reconstructed with a good outcome. There was no recurrence during the follow-up period. Conclusion: In life-threatening intraoral bleeding from large high flow AVM of the mandible, emergency selective embolisation followed by curative resection and primary reconstruction is safe in achieving an ideal cure.