Dyakonov waves generation at uniaxial chiral-plasma interface.

Numerical analysis of Dyakonov waves generation has been carried out at uniaxial chiral-plasma planar interface. The extended electromagnetic wave theory is utilized, and an impedance boundary conditions approach is employed to obtain characteristics equation. Effective mode index and attenuation under the different values of collisional frequency, plasma frequency and chirality in the THz frequency range for three cases for uniaxial chiral media are discussed. These results can be used in the field of photonics and integrated optics to fabricate nanophotonic devices in the THz frequency range.

Postoperative outcomes of acute-on-chronic liver failure in infants and children with biliary atresia.

INTRODUCTION: Acute-on-chronic liver failure (ACLF) is associated with increased mortality and morbidity in patients with biliary atresia (BA). Data on impact of ACLF on postoperative outcomes, however, are sparse. METHOD: We performed a retrospective analysis of patients with BA aged <18 years who underwent LT between 2011 and 2021 at our institution. ACLF was defined using the pediatric ACLF criteria: ≥1 extra-hepatic organ failure in children with decompensated cirrhosis. RESULTS: Of 107 patients (65% female; median age 14 [9-31] months) who received a LT, 13 (12%) had ACLF during the index admission prior to LT. Two (15%) had Grade 1; 4 (30%) had Grade 2; and 7 (55%) had Grade ≥3 ACLF. ACLF cohort was younger at time of listing (5 [4-8] vs. 9 [6-24] months; p < .001) and at LT (8 [8-11] vs. 16 [10-40] months, p < .001) compared to no-ACLF group. Intraoperatively, ACLF patients had higher blood loss (40 [20-53] vs. 10 [6-19] mL/kg; p < .001) and blood transfusion requirements (33 [21-69] vs. 18 [7-25] mL/kg; p = .004). Postoperatively, they needed higher vasopressor support (31% vs. 10.6%; p = .04) and had higher total hospital length of stay (106 [45-151] vs. 13 [7-30] days; p = .023). Rate of return to the operating room, hospital readmission rates, and 1-year post-LT survival rates were comparable between the groups. CONCLUSION: Despite higher perioperative complications, survival outcomes for ACLF in BA after LT are favorable and comparable to those without ACLF. These encouraging data reiterate prioritization during organ allocation of these critically ill children for LT.

Estimating risk of prolonged mechanical ventilation after liver transplantation in children: PROVE-ALT score.

BACKGROUND: Children at high risk for prolonged mechanical ventilation (PMV) after liver transplantation (LT) need to be identified early to optimize pulmonary support, allocate resources, and improve surgical outcomes. We aimed to develop and validate a metric that can estimate risk for Prolonged Ventilation After LT (PROVE-ALT). METHODS: We identified preoperative risk factors for PMV by univariable analysis in a retrospective cohort of pediatric LT recipients between 2011 and 2017 (n = 205; derivation cohort). We created the PROVE-ALT score by mapping multivariable logistic regression coefficients as integers, with cutoff values using the Youden Index. We validated the score by C-statistic in a retrospectively collected separate cohort of pediatric LT recipients between 2018 and 2021 (n = 133, validation cohort). RESULTS: Among total 338 patients, 21% (n = 72) were infants; 49% (n = 167) had cirrhosis; 8% (n = 27) required continuous renal replacement therapy (CRRT); and 32% (n = 111) required management in hospital (MIH) before LT. Incidence of PMV post-LT was 20% (n = 69) and 3% (n = 12) required tracheostomy. Independent risk factors (OR [95% CI]) for PMV were cirrhosis (3.8 [1-14], p = .04); age <1-year (8.2 [2-30], p = .001); need for preoperative CRRT (6.3 [1.2-32], p = .02); and MIH before LT (12.4 [2.1-71], p = .004). PROVE-ALT score ≥8 [Range = 0-21] accurately predicted PMV in the validation cohort with 73% sensitivity and 80% specificity (AUC: 0.81; 95% CI: 0.71-0.91). CONCLUSION: PROVE-ALT can predict PMV after pediatric LT with a high degree of sensitivity and specificity. Once externally validated in other centers, PROVE-ALT will empower clinicians to plan patient-specific ventilation strategies, provide parental anticipatory guidance, and optimize hospital resources.

The use of tracheostomy to support critically ill children receiving orthotopic liver transplantation: a single-center experience.

BACKGROUND: Children with end-stage liver disease and multi-organ failure, previously considered as poor surgical candidates, can now benefit from liver transplantation (LT). They often need prolonged mechanical ventilation (MV) post-LT and may need tracheostomy to advance care. Data on tracheostomy after pediatric LT are lacking. METHOD: Retrospective chart review of children who required tracheostomy in the peri-LT period in a large, freestanding quaternary children's hospital from 2014 to 2019. RESULTS: Out of 205 total orthotopic LTs performed in 200 children, 18 (9%) required tracheostomy in the peri-transplant period: 4 (2%) pre-LT and 14 (7%) post-LT. Among those 14 needing tracheostomy post-LT, median age was 9 months [IQR = 7, 14] at LT and 10 months [9, 17] at tracheostomy. Nine (64%) were infants and 12 (85%) were cirrhotic at the time of LT. Seven (50%) were intubated before LT. Median MV days prior to LT was 23 [7, 36]. Eight (57%) patients received perioperative continuous renal replacement therapy (CRRT). The median MV days from LT to tracheostomy was 46 [33, 56]; total MV days from initial intubation to tracheostomy was 57 [37, 66]. Four (28%) children died, of which 3 (21%) died within 1 year of transplant. Total ICU and hospital length of stay were 92 days [I72, 126] and 177 days [115, 212] respectively. Among survivors, 3/10 (30%) required MV at home and 8/10 (80%) were successfully decannulated at 400 median days [283, 584]. CONCLUSION: Tracheostomy though rare after LT remains a feasible option to support and rehabilitate critically ill children who need prolonged MV in the peri-LT period.

First direct evidence of involvement of a homozygous loss-of-function variant in the EPS15L1 gene underlying split-hand/split-foot malformation.

Split-hand/split-foot malformation (SHFM) is a severe form of congenital limb deformity characterized by the absence of 1 or more digits and/or variable degree of median clefts of hands and feet. The present study describes an investigation of a consanguineous family of Pakistani origin segregating SHFM in an autosomal recessive manner. Human genome scan using SNP markers followed by whole exome sequencing revealed a frameshift deletion (c.409delA, p.Ser137Alafs*19) in the EPS15L1 gene located on chromosome 19p13.11. This is the first biallelic variant identified in the EPS15L1 gene underlying SHFM. Our findings report the first direct involvement of EPS15L1 gene in the development of human limbs.

Homozygous XYLT2 variants as a cause of spondyloocular syndrome.

Spondyloocular syndrome (SOS) is a rare autosomal recessive, skeletal disorder. Two recent studies have shown that it is the result of biallelic sequence variants in the XYLT2 gene with pleiotropic effects in multiple organs, including retina, heart muscle, inner ear, cartilage, and bone. The XYLT2 gene encodes xylosyltransferase 2, which catalyzes the transfer of xylose (monosaccharide) to the core protein of proteoglycans (PGs) leading to initiating the process of PG assembly. SOS was originally characterized in 2 families A and B of Iraqi and Turkish origin, respectively. Using DNA from affected members of the same 2 families, we performed whole exome sequencing, which revealed 2 novel homozygous missense variants (c.1159C > T, p.Arg387Trp) and (c.2548G > C, p.Asp850His). Our findings extend the body of evidence that SOS is caused by homozygous variants in the XYLT2 gene. In addition, this report has extended the phenotypic description of SOS by adding follow-up data from 5 affected individuals in one of the two families, presented here.

Exome sequencing revealed a novel splice site variant in the ALX1 gene underlying frontonasal dysplasia.

Frontonasal dysplasia (FND) is a heterogeneous group of disorders characterized by hypertelorism, telecanthus, broad nasal root, wide prominent nasal bridge, short and wide nasal ridge, broad columella and smooth philtrum. To date one X-linked and three autosomal recessive forms of FND have been reported in different ethnic groups. We sought to identify the gene responsible for FND in a consanguineous Pakistani family segregating the disorder in autosomal recessive pattern. Genome-wide homozygosity mapping using 250KNsp array revealed five homozygous regions in the selected affected individuals. Exome sequencing found a novel splice acceptor site variant (c.661-1G>C: NM_006982.2) in ALX1. Sanger sequencing confirmed the correct segregation of the pathogenic variant in the whole family. Our study concludes that the splice site variant identified in the ALX1 gene causes mild form of FND.

Treatment-Resistant Immunoproliferative Small Intestinal Disease (IPSID) Leading to Lymphoma.

Immunoproliferative small intestinal disease (IPSID) is a distinct variant of mucosa-associated lymphoid tissue (MALT) lymphoma, often linked to chronic Campylobacter jejuni infection. Characterized as an extra-nodal marginal zone B-cell lymphoma, IPSID predominantly affects the proximal small intestine. It features lymphoplasmacytic infiltration and deposition of monotypic α-heavy chains in the lamina propria, leading to blunted intestinal villi, malabsorption, and protein-losing enteropathy. IPSID's clinical spectrum ranges from lymphoid infiltration to malignant diffuse large B-cell lymphoma. Similar to MALT lymphoma, early-stage IPSID can be resolved with antibiotic therapy. This case study documents a 50-year-old Nigerian woman presenting with recurrent watery diarrhea, abdominal pain, and weight loss, unresponsive to antibiotics. A 50-year-old female immigrant from Nigeria presented with recurrent watery diarrhea, abdominal pain, and significant weight loss, all refractory to antibiotic treatment. Initial diagnostic investigations revealed a positive Campylobacter stool antigen, mesenteric lymphadenopathy on CT and gallium scans, and diffuse mucosal lymphoplasmacytic infiltration with villi flattening on small bowel biopsies. An octreotide scan identified a reactive mesenteric lymph node, confirmed by surgical biopsy as reactive lymphadenitis. The patient was diagnosed with IPSID and commenced antibiotic therapy, which initially resolved her symptoms. However, she experienced frequent recurrences requiring multiple hospitalizations and repeated courses of intravenous antibiotics. Eventually, the disease progressed to lymphoma, necessitating chemotherapy initiation. This case underscores the diagnostic complexities of IPSID, particularly in distinguishing it from other causes of mesenteric lymphadenopathy. It also highlights the challenges in preventing disease progression from a benign to a malignant state despite appropriate antibiotic treatment. Given IPSID's prevalence in endemic regions, it should be considered in differential diagnoses for similar presentations. Continuous monitoring is crucial to evaluate therapeutic response and mitigate the risk of progression to lymphoma. IPSID presents a significant diagnostic and therapeutic challenge. This case exemplifies the necessity for heightened clinical awareness, especially in patients from endemic regions, and the importance of rigorous monitoring to prevent malignant transformation. Further research is warranted to elucidate the mechanisms behind IPSID progression in certain patients despite repeated antibiotic interventions.

Vitrifying expanded equine embryos collapsed by blastocoel aspiration is less damaging than slow-freezing.

The cryotolerance of equine blastocysts larger than 300 µm can be improved by aspirating blastocoele fluid prior to vitrification; however, it is not known whether blastocoele aspiration also enables successful slow-freezing. The aim of this study was therefore to determine whether slow-freezing of expanded equine embryos following blastocoele collapse was more or less damaging than vitrification. Grade 1 blastocysts recovered on day 7 or 8 after ovulation were measured (>300-550 µm, n = 14 and > 550 µm, n = 19) and blastocoele fluid was aspirated prior to slow-freezing in 10% glycerol (n = 14), or vitrification (n = 13) in 16.5% ethylene glycol/16.5% DMSO/0.5 M sucrose. Immediately after thawing or warming, embryos were cultured for 24 h at 38 °C and then graded and measured to assess re-expansion. Control embryos (n = 6) were cultured for 24 h following aspiration of blastocoel fluid, without cryopreservation or exposure to cryoprotectants. Subsequently, embryos were stained to assess live/dead cell proportion (DAPI/TOPRO-3), cytoskeleton quality (Phalloidin) and capsule integrity (WGA). For 300-550 µm embryos, quality grade and re-expansion were impaired after slow-freezing but not affected by vitrification. Slow-freezing embryos >550 µm induced additional cell damage as indicated by a significant increase in dead cell proportion and disruption of the cytoskeleton; neither of these changes were observed in vitrified embryos. Capsule loss was not a significant consequence of either freezing method. In conclusion, slow-freezing of expanded equine blastocysts collapsed by blastocoel aspiration compromises post-thaw embryo quality more than vitrification.

Prevalence of Gram positive bacteria in the affected individuals of Otitis media with effusion from the indigenous population of Southern Punjab, Pakistan: first report.

Otitis media with effusion (OME) is a type of otitis media (OM) characterized by the presence of fluid behind intact tympanic membrane and is one of the most common diseases of early childhood. It is an infectious disease associated with the presence of many pathogenic bacteria in the middle ear of affected individuals. This study was aimed to determine the prevalence of Gram-positive bacteria from the middle ear of OME patients in the population of Southern Punjab, Pakistan. The incidence of OME under comprehensive healthcare setting was investigated in patients who consulted at the department of ear, throat and nose, Bahawal Victoria Hospital (BVH), Bahawalpur, from December, 2019 to May, 2021. Ear swabs were taken from affected and normal individuals. After culturing bacteria from the ear swabs, microscopic analysis and biochemical tests were performed to characterize the cultured Gram-positive bacteria. Out of 352 patients examined, 109 (30.9%) patients had OME. Age of the participants ranged from 14 to 50 years; individuals between the ages of 14 and 22 years had the highest infection rates, while individuals between 40 and 50 years had the lowest rate of infection. Tympanic membrane perforation, fever, cough, sore throat, ear pain and hearing problem showed association with symptoms of OME. Microscopic analysis and biochemical characterization showed the presence of streptococci and staphylococci in all the studied samples. The most frequently isolated bacteria were Streptococcus pneumoniae, Streptococcus pyogenes, and Staphylococcus aureus with percentage of 53.3%, 20% and 13.3% respectively. Enterococcus faecalis (6.6%) and Staphylococcus epidermidis (6.6%) were also identified in the studied samples. This study will help in the better medical administration of OME affected individuals.

Elevated bile acids are associated with left ventricular structural changes in biliary atresia.

BACKGROUND: In children with biliary atresia (BA), pathologic structural changes within the heart, which define cirrhotic cardiomyopathy, are associated with adverse perioperative outcomes. Despite their clinical relevance, little is known about the pathogenesis and triggers of pathologic remodeling. Bile acid excess causes cardiomyopathy in experimental cirrhosis, but its role in BA is poorly understood. METHODS: Echocardiographic parameters of left ventricular (LV) geometry [LV mass (LVM), LVM indexed to height, left atrial volume indexed to BSA (LAVI), and LV internal diameter (LVID)] were correlated with circulating serum bile acid concentrations in 40 children (52% female) with BA listed for transplantation. A receiver-operating characteristic curve was generated to determine optimal threshold values of bile acids to detect pathologic changes in LV geometry using Youden index. Paraffin-embedded human heart tissue was separately analyzed by immunohistochemistry for the presence of bile acid-sensing Takeda G-protein-coupled membrane receptor type 5. RESULTS: In the cohort, 52% (21/40) of children had abnormal LV geometry; the optimal bile acid concentration to detect this abnormality with 70% sensitivity and 64% specificity was 152 µmol/L (C-statistics=0.68). Children with bile acid concentrations >152 µmol/L had ∼8-fold increased odds of detecting abnormalities in LVM, LVM index, left atrial volume index, and LV internal diameter. Serum bile acids positively correlated with LVM, LVM index, and LV internal diameter. Separately, Takeda G-protein-coupled membrane receptor type 5 protein was detected in myocardial vasculature and cardiomyocytes on immunohistochemistry. CONCLUSION: This association highlights the unique role of bile acids as one of the targetable potential triggers for myocardial structural changes in BA.

A Novel Approach to Dilation of Complete Colorectal Anastomotic Stricture Using Transillumination.

Colorectal anastomotic strictures are a well-known complication of low anterior resection. Depending on their location, strictures are amendable to different forms of endoscopic intervention. However, all endoscopic interventions are contingent on passing a guidewire across the anastomotic stricture. We present the case of a patient with a complete anastomotic stricture that could not be traversed by conventional endoscopy and the unique method by which a lumen was created in the anastomotic strictures that allowed for a guidewire to be passed across.

Cannabis use history is associated with increased prevalence of ascites among patients with nonalcoholic fatty liver disease: A nationwide analysis.

BACKGROUND: Recent studies have revealed the endocannabinoid system as a potential therapeutic target in the management of nonalcoholic fatty liver disease (NAFLD). Cannabis use is associated with reduced risk for NAFLD, we hypothesized that cannabis use would be associated with less liver-related clinical complications in patients with NAFLD. AIM: To assess the effects of cannabis use on liver-related clinical outcomes in hospitalized patients with NAFLD. METHODS: We performed a retrospective matched cohort study based on querying the 2014 National Inpatient Sample (NIS) for hospitalizations of adults with a diagnosis of NAFLD. The NIS database is publicly available and the largest all-payer inpatient database in the United States. The patients with cannabis use were selected as cases and those without cannabis were selected as controls. Case-control matching at a ratio of one case to two controls was performed based on sex, age, race, and comorbidities. The liver-related outcomes such as portal hypertension, ascites, varices and variceal bleeding, and cirrhosis were compared between the groups. RESULTS: A total of 49911 weighed hospitalizations with a diagnosis of NAFLD were identified. Of these, 3820 cases were selected as the cannabis group, and 7625 non-cannabis cases were matched as controls. Patients with cannabis use had a higher prevalence of ascites (4.5% vs 3.6%), with and without cannabis use, P = 0.03. The prevalence of portal hypertension (2.1% vs 2.2%), varices and variceal bleeding (1.3% vs 1.7%), and cirrhosis (3.7% vs 3.6%) was not different between the groups, with and without cannabis use, all P > 0.05. Hyperlipidemia, race/ethnicity other than White, Black, Asian, Pacific Islander or Native American, and higher comorbidity score were independent risk factors for ascites in the cannabis group. Among non-cannabis users, obesity and hyperlipidemia were independent protective factors against ascites while older age, Native American and higher comorbidity index were independent risk factors for ascites. CONCLUSION: Cannabis was associated with higher rates of ascites, but there was no statistical difference in the prevalence of portal hypertension, varices and variceal bleeding, and cirrhosis.

Ferrous Sulfate-Induced Esophageal Injury Leading to Esophagitis Dissecans Superficialis.

Medication-induced esophagitis is a well-known but relatively rare clinical diagnosis, most common in patients with preexisting esophageal dysmotility, obstruction, or altered anatomy. Esophagitis dissecans superficialis (EDS) is a rare endoscopic finding characterized by sloughing of large fragments of the esophageal mucosal lining. The causes of EDS include prior trauma, heavy smoking history, ingestion of alcoholic and hot beverages, and immunosuppression. We present a unique case of EDS secondary to ferrous sulfate-induced pill esophagitis. The patient was a 94-year-old male who presented with dysphagia to solids, odynophagia, and weight loss. Esophagogastroduodenoscopy (EGD) revealed EDS. Biopsies demonstrated vacuolar degeneration at the midlevel of the epithelium with overlying hyperkeratosis and parakeratosis, with noted black/brown pigment present at the level of the split in the epithelium. The patient was started on a liquid diet with no oral administration of pills. EGD was repeated and showed a significant improvement in esophageal mucosa and resolution of strictures. Although medication-induced esophagitis is not classically associated with EDS, specific circumstances that are associated with pill esophagitis may lead to progression to EDS. In the case of our patient, prolonged contact of ferrous sulfate to the esophageal mucosa is thought be a result of an enlarged left atrium and pulmonary arteries secondary to longstanding coronary artery disease and an enlarged left bronchus secondary to chronic obstructive pulmonary disease and right pneumonectomy. These anatomical changes likely led to an extended duration of contact and are believed to have led to erosion of the superficial esophageal mucosa, eventually progressing to EDS.

A Rare Presentation of Poorly Differentiated Lung Carcinoma with Duodenal Metastasis and Literature Review.

Lung cancer is a common malignancy which is frequently found to metastasize to distant sites including bone, liver, and adrenal glands. There are rare reports of metastases to the gastrointestinal (GI) tract, with the duodenum being the most uncommon. We present a rare case of a poorly differentiated lung carcinoma metastasizing to the duodenum. This case enhances the medical literature as it provides additional distinct features to the clinical and histological presentation of metastatic lung carcinoma to the GI tract. A 61-year-old male with a history of poorly differentiated lung carcinoma presented with worsening dizziness, fatigue, and early satiety. He had extensive workup done in the past for hemoptysis including a computerized tomography scan of the chest which showed a new lobulated, apical lesion and hilar lymphadenopathy. He ultimately had a transthoracic fine-needle aspiration (FNA) of the mass and was later diagnosed with poorly differentiated lung carcinoma. On examination, the patient was noted to be pale, tachycardic, and hypotensive. The patient was noted to have an acute drop in his hemoglobin requiring fluid resuscitation, multiple blood transfusions, and evaluation with an esophagogastroduodenoscopy. He was found to have an oozing ulcer in the third portion of the duodenum whose biopsies showed poorly differentiated carcinoma with areas of neuroendocrine differentiation, similar to his lung biopsy results, which was consistent with metastatic lung carcinoma.

Idiopathic Terminal Ileal Ulceration Mimicking Crohn's in the Setting of Antiretroviral Therapy Initiation.

Antiretroviral therapy (ART) has precipitously decreased the morbidity associated with human immunodeficiency virus but can unmask and exacerbate opportunistic infections and autoimmune diseases. Various diseases have been reported in association with ART initiation, but there is scant literature describing inflammatory colitis in the setting of ART initiation. We present a 39-year-old man with chronic untreated human immunodeficiency virus and central nervous system toxoplasmosis who developed persistent diarrhea after initiation of ART. A comprehensive infectious workup was negative. Computed tomography demonstrated terminal ileum enteritis, which was confirmed by colonoscopy. Biopsy of the terminal ileum revealed fibrinous exudate and granulation tissue.

Endoscopic Retrograde Cholangiopancreatography Leading to Pharyngeal Perforation.

Endoscopic retrograde cholangiopancreatography (ERCP) had become the favored method to access the pancreaticobiliary system because it is a safer and less invasive method compared to surgery. However, as with any procedure, ERCP comes with its own risks and potential complications. We present a unique case of a patient who underwent ERCP and developed necrotizing infection of the neck and a submandibular abscess. The patient is a 66-year-old female who presented to an outside hospital with complaint of right upper quadrant abdominal pain, workup of which revealed choledocholithiasis. ERCP was attempted; however, cannulation was unsuccessful. The patient was discharged home after the procedure, but within 48 h she presented to our institution complaining of left-sided neck pain, dysphagia, and drooling. CT of the neck revealed extensive gas and fluid collections at the left submandibular space. The patient was taken to the operating room for drainage of the left neck abscess. Drainage and irrigation of the abscess yielded Streptococcus mitis and Hemophilus parainfluenza. The rest of patient's hospital course was uncomplicated, and she was discharged with appropriate follow-up. In the case of our patient, ERCP was complicated by a perforation of the hypopharynx. Pharyngeal perforation can be subclassified into supraglottic and infraglottic. The most frequent cause of perforations is due to increased pressure in an intrinsically weak anatomical region of the pharynx. Such perforations are commonly due to the advancement of the endotracheal tube or transthoracic echo probe, but can also be due to advancement of an endoscope.

Severe Gastrointestinal Mucositis Following Concurrent Palbociclib and Palliative Radiation Therapy.

BACKGROUND/AIM: Palbociclib is an FDA-approved cyclin-dependent kinase inhibitor for the treatment of advanced breast cancer. Limited information is available regarding the toxicity of palbociclib and concurrent radiation therapy. CASE REPORT: Herein, we report a case of esophageal toxicity in a patient treated with palbociclib and radiation therapy. A 63-year-old woman was treated with palbociclib followed by palliative radiation therapy. The patient presented three days after completing radiation therapy with severe odynophagia, and dysphagia and was found to have grade 2-3 esophageal ulcers. Palbociclib and radiation therapy was held on admission, and a resolution of her symptoms and improvement in her oral intake was noted at which time she was restarted on palbociclib with no further radiation treatment. CONCLUSION: Caution is advised when patients are undergoing concurrent palbociclib and even low-dose palliative radiation treatment. In these patients, providers should maintain a high index of suspicion for toxicities such as dermatitis or mucositis.