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Frontiers in Oncology 2024Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is frequently accompanied with type 1 autoimmune pancreatitis (AIP). Isolated IgG4-SC which is not accompanied...
BACKGROUND
Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is frequently accompanied with type 1 autoimmune pancreatitis (AIP). Isolated IgG4-SC which is not accompanied with AIP is uncommon in clinical practice, and its manifestations are similar to those of hilar cholangiocarcinoma.
CASE PRESENTATION
A 55-year-old male presented with persistent aggravation of icteric sclera and skin. He was initially diagnosed with hilar cholangiocarcinoma and underwent surgery. However, positive IgG4 plasma cells were found in the surgical specimens. Thus, a pathological diagnosis of IgG4-SC was established. After that, steroid therapy was given and initially effective. But he was steroid dependent, and then received rituximab therapy twice. Unfortunately, the response to rituximab therapy was poor.
CONCLUSION
It is crucial to differentiate isolated IgG4-SC from hilar cholangiocarcinoma to avoid unnecessary surgery. Future studies should further explore effective treatment strategy in patients who do not respond to steroids therapy. It is also required to develop novel and accurate diagnostic approaches to avoid unnecessary surgical procedures.
PubMed: 38846973
DOI: 10.3389/fonc.2024.1385214 -
Annals of Medicine and Surgery (2012) Jun 2024It is common for the liver to be supplied blood by a hepatic artery branching off the coeliac trunk. Occasionally, a replaced common hepatic artery (RCHA), emerges from...
INTRODUCTION
It is common for the liver to be supplied blood by a hepatic artery branching off the coeliac trunk. Occasionally, a replaced common hepatic artery (RCHA), emerges from the superior mesenteric artery (SMA), can supply the liver in 1.5-4.0% of cases. Computed tomography (CT) angiography is a highly accurate method for identifying arterial anomalies, which may remain undetected until the time of surgery, leading to unexpected complications.
CASE PRESENTATION
A 53-year-old male exhibiting symptoms of decreased appetite, weight loss, vomiting, and altered sclera, urine, and stool colour, underwent a contrast-enhanced CT scan revealing biliary tract dilatation and pancreatic abnormalities, leading to a pancreaticoduodenectomy. During the surgery, an uncommon arterial finding-CHA from SMA-was noted. Pancreatic cancer was confirmed. The patient was discharged a week post-surgery without issues, emphasizing perioperative care progress.
DISCUSSION
The authors' study focused on the detection conditions of the same hepatic artery anomaly in eight cases reported between 2017 and 2023. In two of them the anomaly was discovered in cadaver by routine autopsy. In three cases, this variation was identified before the surgery, but in three other cases it wasn't detected until the surgical procedure. In the authors' case, due to multiple reasons, the anomaly remained undetectable until the surgery.
CONCLUSION
This study underscores the importance of thorough preoperative evaluation to grasp vascular variations for better patient care. Also, a noteworthy observation in our case is that the surgeon identified an expanded hepatic vessel, prompting further investigation into this anomaly.
PubMed: 38846897
DOI: 10.1097/MS9.0000000000002102 -
BMC Ophthalmology Jun 2024To explore the application and long-term clinical effects of modified Yamane technique in intrascleral intraocular lens (IOL) fixation combined with or without iris...
AIMS
To explore the application and long-term clinical effects of modified Yamane technique in intrascleral intraocular lens (IOL) fixation combined with or without iris reconstruction.
SETTINGS AND DESIGN
The data of patients receiving IOL fixation with modified Yamane technique in an ophthalmology department between December 2021 and August 2023 were analyzed retrospectively. The longest follow-up duration was > 12 months.
METHODS AND MATERIAL
The trailing haptic was fixed with the needle before the leading haptic. The silicone haptic stoppers were used to stabilize the IOL when iris reconstruction was combined. Preoperative and postoperative best-corrected visual acuity (BCVA), corneal endothelial cells (CECs), postoperative intraocular pressure (IOP), surgical indications and methods, and postoperative complications were recorded. Anterior segment optical coherence tomography (OCT) was used to evaluate IOL decentration and tilt. The paired sample t-test or Wilcoxon rank sum test were used to compare the results of the same index before and after the operation.
RESULTS
Twelve patients (12 eyes) were included in this cohort. There were 1 case of IOL dislocation, eight cases of lens dislocation or subluxation, and three cases of aphakia. Traumatic lens dislocation was the main cause of aphakia. Primary lens extraction was performed in previous surgeries, and all three were combined with pars plana vitrectomy (PPV). Four of 12 patients underwent IOL fixation and iris reconstruction. The mean age of participants was 63 ± 10.61 years. The mean BCVA increased from 0.89 ± 0.72 logMAR to 0.39 ± 0.56 logMAR at the last visit (p < 0.05). The postoperative relative refractive error was - 0.13 ± 0.42 D (-0.60 D to + 0.57 D). The OCT showed that the IOLs were well centered, with a mean decentration of 0.20 ± 0.13 mm and a mean tilt of 2.31°±0.93°. Ten patients did not experience any complications.
CONCLUSIONS
The modified Yamane technique in IOL fixation surgery, especially combined with iris reconstruction, reduces operation difficulty, increases operational stability and safety, and improves postoperative visual acuity without serious intra- or postoperative complications. The long-term improvement effect was remarkable.
Topics: Humans; Female; Male; Middle Aged; Retrospective Studies; Lens Implantation, Intraocular; Iris; Aged; Visual Acuity; Lenses, Intraocular; Sclera; Plastic Surgery Procedures; Tomography, Optical Coherence; Follow-Up Studies
PubMed: 38840075
DOI: 10.1186/s12886-024-03493-8 -
Osteogenesis Imperfecta type 1: like mother, like daughter - Challenges in the perinatal management.BMJ Case Reports Jun 2024A third gravida with osteogenesis imperfecta (OI) type 1, in her 20s, was referred from the Medical Genetics department at 12+ weeks with a prenatal diagnosis of OI type...
A third gravida with osteogenesis imperfecta (OI) type 1, in her 20s, was referred from the Medical Genetics department at 12+ weeks with a prenatal diagnosis of OI type 1 in this fetus for further management. She was wheelchair-bound and keen to continue this pregnancy. She had medical termination in her two previous pregnancies for OI in the fetuses. Ultrasound at 12+ weeks revealed a short-bent femur with sparing of the long bones of the upper limb. Serial ultrasound revealed progressive affliction of the long bones with falling growth profile and polyhydramnios. She was delivered at 36 weeks by caesarean for breech in labour under regional anaesthesia.A multidisciplinary approach, patient determination, and good partner support helped in the successful management of this pregnancy.The neonate had blue sclera, dentigerous imperfecta, bowing of the femur and relatively spared upper limbs. Growth was on the third centile. The mother says she brings the girl for follow-up every 3-6 months to give injection zoledronate. The mother confirms her girl can stand with support, crawl, and speak two-syllable words. Her daughter had to undergo femur corrective osteotomy rush nailing and hip spice application for a closed fracture of the left femur.
Topics: Humans; Osteogenesis Imperfecta; Female; Pregnancy; Infant, Newborn; Cesarean Section; Ultrasonography, Prenatal; Perinatal Care; Adult; Femur; Femoral Fractures
PubMed: 38839405
DOI: 10.1136/bcr-2023-258705 -
Cureus Jun 2024Posterior scleritis is a rare inflammatory eye condition affecting the posterior segments of the sclera and is more prevalent in females. Its clinical presentation,...
Posterior scleritis is a rare inflammatory eye condition affecting the posterior segments of the sclera and is more prevalent in females. Its clinical presentation, often nonspecific, includes ocular pain, headache, and vision loss. Misdiagnosis is common due to a lack of specific symptoms posing a potential threat to vision. The etiology is often tied to rheumatic diseases, such as rheumatoid arthritis (RA), systemic erythematous lupus (SLE), and anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis. Posterior scleritis poses diagnostic challenges, mimicking many other ocular conditions, hence necessitating a thorough clinical eye exam. Laboratory studies, including inflammatory markers and markers of rheumatic diseases, may identify underlying systemic diseases. Imaging, including B-scan ultrasound and magnetic resonance imaging (MRI), aids in accurate diagnosis. Treatment involves non-steroidal anti-inflammatory drugs (NSAID), as well as topical corticosteroids for mild disease and systemic corticosteroids for severe disease. Biologic therapy has become increasingly significant for refractory cases. A multidisciplinary approach involving ophthalmology and rheumatology is crucial in the management of this potential sight-threatening disease. This case report highlights a 46-year-old woman with a history of RA-associated posterior scleritis.
PubMed: 38831917
DOI: 10.7759/cureus.61537 -
BMC Ophthalmology Jun 2024The long-term safety and efficacy of repeated applications of subliminal transscleral cyclophotocoagulation (SL-TSCPC) with a focus on cumulative energy was evaluated in...
BACKGROUND
The long-term safety and efficacy of repeated applications of subliminal transscleral cyclophotocoagulation (SL-TSCPC) with a focus on cumulative energy was evaluated in glaucoma patients.
METHODS
In this retrospective, multicentric study the data of a total of 82 eyes with various causes of glaucoma that were treated with a single or multiple applications of SL-TSCPC were collected. Treatments were performed under general or local anesthesia with an 810 nm diode laser. Power was 2000 mW; duty cycle, 31.3%; total treatment duration, 80-320 s; equaling a total energy of 50-200 J per treatment session. Fifty-five eyes (55 patients) presented for all follow-ups, and these eyes were selected for further statistical analysis. The mean age was 60.0 ± 17.1 years, and 22 (40%) of the patients were female. Intraocular pressure (IOP) and dependence on further glaucoma medication were evaluated at 12 months following the initial treatment.
RESULTS
Eyes underwent 1 or 2 consecutive SL-TSCPC treatments. Median (min-max) baseline IOP of 34 (13-69) decreased to 21.5 (7-61), 22 (8-68), 20 (9-68), and 19.5 (3-60) mmHg at the 1, 3, 6, and 12-month postoperative timepoints respectively. The mean (± SD) IOP decrease at 12 months was 26 ± 27%, 39 ± 32%, and 49 ± 33% in the low (below 120 J, n = 18), medium (120-200 J, n = 24), and high (above 200 J, n = 13) cumulative energy groups respectively. At the 12-month timepoint, oral carbonic anhydrase use was discontinued in ¾ of the cases.
CONCLUSIONS
It was found that the repeated application of SL-TSCPC safely and efficiently decreases IOP in a Caucasian population with heterogenous causes of glaucoma, eyes with silicone oil responded to a greater extent. Inclusion of cumulative energy scales may contribute to better addressing repeated procedures in a standardized fashion.
Topics: Humans; Retrospective Studies; Female; Male; Middle Aged; Intraocular Pressure; Laser Coagulation; Ciliary Body; Aged; Sclera; Glaucoma; Adult; Lasers, Semiconductor; Visual Acuity; Aged, 80 and over; Follow-Up Studies; Treatment Outcome
PubMed: 38831268
DOI: 10.1186/s12886-024-03505-7 -
Journal of Clinical Research in... Jun 2024Osteogenesis imperfecta (OI) is a group of phenotypically and genetically heterogeneous connective tissue disorders that share similar skeletal anomalies causing bone...
INTRODUCTION
Osteogenesis imperfecta (OI) is a group of phenotypically and genetically heterogeneous connective tissue disorders that share similar skeletal anomalies causing bone fragility and deformation. This study aimed to investigate the molecular genetic etiology and determine the relationship between genotype and phenotype in OI patients with targeted next-generation sequencing (NGS).
METHOD
In patients with OI, a targeted NGS analysis panel (Illumina TruSight One) containing genes involved in collagen/bone synthesis was performed on the Illumina Nextseq550 platform.
RESULTS
Fifty-six patients (female/male: 25/31) from 46 different families were enrolled in the study. Consanguinity between parents was noted in 15 (32.6%) families. Clinically according to Sillence classification; 18(33.1%) patients were considered to type I, 1(1.7%) type II, 26(46.4%) type III and 11(19.6%) type IV. Median body weight was -1.1 (-6.8, - 2.5) SDS, and height was -2.3 (-7.6, - 1.2) SDS. Bone deformity was detected in 30 (53.5%) of the patients, while 31 (55.4%) were evaluated as mobile. Thirty-six (60.7%) patients had blue sclera, 13 (23.2%) had scoliosis, 12 (21.4%) had dentinogenesis imperfecta (DI), and 2 (3.6%) had hearing loss. Disease-causing variants in COL1A1 and COL1A2 genes were found in 24 (52.1%) and 6 (13%) families, respectively. In 8 (17.3%) of the remaining 16 (34.7%) families, the NGS panel revealed disease-causing variants in three different genes (FKBP10, SERPINF1, and P3H1). Nine (23.6%) of the variants detected in all investigated genes were not previously reported in the literature and were classified to be pathogenic according to ACMG guidelines pathogenity scores. In ten (21.7%) families, a disease-related variant was not found in a total of 13 OI genes included in the panel.
CONCLUSION
Genetic etiology was found in 38 (82.6%) of 46 families by targeted NGS analysis. In addition, 9 new variants were assessed in known OI genes which is a significant contribution to the literature.
PubMed: 38828893
DOI: 10.4274/jcrpe.galenos.2024.2022-12-8 -
[Zhonghua Yan Ke Za Zhi] Chinese... Jun 2024With the increasing prevalence of myopia among adolescents, the pathogenesis of this condition has garnered significant attention. Studies have discovered the expression... (Review)
Review
With the increasing prevalence of myopia among adolescents, the pathogenesis of this condition has garnered significant attention. Studies have discovered the expression of various hormone receptors in ocular tissues of both animals and humans. Additionally, changes in hormone levels accompany the development of myopia, although the exact relationships remain inconclusive. This article reviews the potential influences and mechanisms of action of endogenous hormones such as melatonin, serotonin, insulin, glucagon, sex hormones, vitamin D, and prostaglandins in ocular tissues including the retina, choroid, and sclera. It elaborates on the relationship between fluctuations in these hormone levels and the progression of myopia, aiming to provide guidance for exploring targets for myopia prevention and control.
Topics: Humans; Myopia; Melatonin; Vitamin D; Serotonin; Insulin; Glucagon; Animals; Gonadal Steroid Hormones; Prostaglandins; Hormones; Retina
PubMed: 38825955
DOI: 10.3760/cma.j.cn112142-20231106-00213 -
[Zhonghua Yan Ke Za Zhi] Chinese... Jun 2024To evaluate the efficacy of sutureless intrascleral intraocular lens (IOL) fixation with the modified Yamane technique. It was a retrospective case series study....
To evaluate the efficacy of sutureless intrascleral intraocular lens (IOL) fixation with the modified Yamane technique. It was a retrospective case series study. Patients undergoing sutureless intrascleral IOL fixation with the modified Yamane technique were included at Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University from January 2022 to September 2023. Uncorrected visual acuity (UCVA) and best-corrected visual acuity (BCVA), recorded as the logarithm of the minimum angle of resolution (logMAR), were measured before surgery and at 1 day, 3 days, 1 week, 1 month, and 3 months postoperatively. Refractive error and intraocular pressure were also checked. Anterior segment examination with a slit lamp microscope, fundus examination, anterior segment and posterior segment optical coherence tomography were performed. Intraoperative and postoperative ocular complications were documented. A total of 53 patients (53 eyes) were included in this study, comprising 40 males and 13 females, with a median age of 60 (49, 68) years. Among them, the proportion of a history of trauma was 22.6% (12/53). There was 1 eye with intraoperative vitreous hemorrhage (1.9%). All eyes had no obvious hypotony, no obvious inflammation in the anterior chamber, and no pupillary abnormalities at 1 week after surgery. The mean follow-up time was (8.0±3.3) months (range, 3 to 16 months). There was no iris capture, re-dislocation, or haptic exposure of the IOL during the follow-up. The corneal endothelial cell density was (2 236±704) cells/mm preoperatively and (1 964±628) cells/mm at 1 month, with significant difference (<0.001). The UCVA (logMAR) was 1.53±0.75 preoperatively, 0.18±0.17 at 1 month, 0.15±0.14 at 3 months, and 0.14±0.13 at the final visit (<0.001). The UCVA (logMAR) at 1 month was significantly different from that at 3 months and the final visit (both <0.05). At 1 month, 50.9% (27/53) of the eyes had an UCVA (logMAR)≤0.1, and the rate was 56.6% (30/53) at 3 months. The BCVA (logMAR) was 0.25±0.21, 0.03±0.06, 0.02±0.06, and 0.02±0.06 before surgery, at postoperative1 month, 3 months, and the final visit, respectively (<0.001). The BCVA (logMAR) at 1 month was not significantly different from that at 3 months and the final visit (both >0.05). The rate of the eyes with a BCVA (logMAR)≤0 was 81.1% (43/53) at 1 month and 83.0% (44/53) at 3 months. The IOL tilt was (5.18±2.60)° at postoperative 1 month and (5.08±2.48)° at postoperative 3 months, without statistically significant difference (>0.05). The IOL decentration was (0.35±0.24) mm at postoperative 1 month and (0.32±0.24) mm at postoperative 3 months, without statistically significant difference (>0.05). Sutureless intrascleral IOL fixation with the modified Yamane technique is simpler and more minimally invasive to achieve a stable and centered IOL implantation with fewer complications and good visual prognosis.
Topics: Humans; Middle Aged; Male; Female; Retrospective Studies; Aged; Lens Implantation, Intraocular; Lenses, Intraocular; Sclera; Visual Acuity; Sutureless Surgical Procedures
PubMed: 38825949
DOI: 10.3760/cma.j.cn112142-20240103-00008 -
Microscopy Research and Technique Jun 2024The anatomical structures forming the eyeball differ among dog breeds, both morphologically and morphometrically. This study was aimed at determining the morphometric...
The anatomical structures forming the eyeball differ among dog breeds, both morphologically and morphometrically. This study was aimed at determining the morphometric values of the eyeball layers of different dog breeds and the morphological structures of these layers using scanning electron microscopy. Thirty-two eyeballs of 17 dogs belonging to 9 different breeds that died from traffic accidents, falling from a height, and naturally were used. These dog breeds were grouped according to their brachycephalic and mesocephalic skull structures, and morphometric measurements of the eyeballs of each group were obtained. Scanning electron microscopy was used to examine the morphological structure of the eyeball layers. The studied dogs' eyeballs comprised three layers: outer, middle, and inner. Thickness measurements obtained from three different regions of the eyeball indicated that the equatorial region was the thinnest among all dog breeds. Moreover, the cornea, which is covered by the sclera along its edges, was thicker at the corneal limbus than at the corneal vertex. A positive correlation was observed between lens thickness and the number of ciliary processes, which varied according to the dogs' head structures. Notably, depression was observed in the posterior surface of the lens in brachycephalic dogs. The morphometric values of the eyeball layers in the brachycephalic and mesocephalic dog breeds were also determined. These values will help researchers study this subject, and the determined morphometric and morphological values will contribute to the anatomy literature. RESEARCH HIGHLIGHTS: This comprehensive study investigates the morphometric and morphological variations in the eyeball layers of different dog breeds, utilizing scanning electron microscopy to analyze eyeballs. It reveals significant breed-specific differences, particularly between brachycephalic and mesocephalic dogs, regarding eyeball layer thickness, corneal structure, lens thickness, and the number of ciliary processes.
PubMed: 38822703
DOI: 10.1002/jemt.24624