Intraoperative Diagnosis and Surgical Management of Encapsulating Peritoneal Sclerosis: A Case Report.

Encapsulating peritoneal sclerosis (EPS) is a serious complication of chronic peritoneal dialysis (PD) that results in encapsulation of the bowel in a thick, fibrocollagenous membrane. Given its rare and complex nature, diagnosis of EPS often arises late in the disease process or intraoperatively. We report the case of an 86-year-old male with a history of renal failure managed with PD who presented with multiple hospital admissions for recurrent abdominal pain and symptoms of small bowel obstruction. Open laparotomy revealed encasement of the entire abdominal cavity in a cocoon-like membrane, consistent with EPS, which was successfully managed with extensive excision and adhesiolysis. This discussion, enriched by unique radiographic insights and delineation of a surgical strategy, seeks to enhance the understanding of this underreported disease characterized by a lack of definitive treatment and an enigmatic pathophysiology.

A Rare Case of Iron Overload in Hereditary Spherocytosis: A Case Report.

Hereditary spherocytosis (HS) is a hereditary hematologic disorder characterized by fragile spherical red blood cells that are susceptible to hemolysis. HS patients are often asymptomatic or present with anemia; however, serious complications of chronic hemolysis can include cholelithiasis and aplastic crisis. Splenectomy is considered the standard surgical treatment in moderate and severe forms of HS, with the main complication being a life-long risk of infection. Interestingly, our case suggests a possibility of secondary hemochromatosis as a complication of chronic hemolysis seen in HS. A vast majority of hemochromatosis patients possess a genetic predisposition, which increases their serum iron level and iron storage within the reticuloendothelial system. However, we present a case in which the genetic panel for common mutations associated with hemochromatosis resulted as negative. This case emphasizes the need for increased awareness regarding the potential development of idiopathic hemochromatosis in patients with long-standing HS, allowing for prompt intervention and preventing the associated complications.

Preparation and In-Vitro Characterization of Solid Lipid Nanoparticles Containing Artemisinin and Curcumin.

BACKGROUND: Malaria remains a formidable public health obstacle across Africa, Southeast Asia, and portions of South America, exacerbated by resistance to antimalarial medications, such as artemisinin-based combinations. The combination of curcumin and artemisinin shows promise due to its potential for dose reduction, reduced toxicity, synergistic effects, and suitability for drug delivery improvement. OBJECTIVE: This research aims to enhance the solubility and dissolution rates of curcumin and artemisinin by employing Solid Lipid Nanoparticles (SLNs). Oral delivery of both drugs faces challenges due to their poor water solubility, inefficient absorption, and rapid metabolism and elimination. METHOD: The study focuses on formulating and optimizing Solid Lipid Nanoparticles (SLNs) encapsulating artemisinin (ART) and curcumin (CUR). SLNs were developed using the hot homogenization method, incorporating ultrasonication. Drug-excipient compatibility was evaluated using Differential Scanning Calorimetry (DSC). Lipid and surfactant screening was performed to select suitable components. A 3² full factorial design was utilized to investigate the influence of lipid and surfactant concentrations on key parameters, such as entrapment efficiency (%EE) and cumulative drug release (%CDR). Additionally, evaluations of % entrapment efficiency, drug loading, particle size, zeta potential, and in-vitro drug release were conducted. RESULTS: Successful development of artemisinin and curcumin SLNs was achieved using a full factorial design, demonstrating controlled drug release and high entrapment efficiency. The optimized nanoparticles exhibited a size of 114.7nm, uniformity (PDI: 0.261), and a zeta potential of -9.24 mV. Artemisinin and curcumin showed %EE values of 79.1% and 74.5%, respectively, with cumulative drug release of 85.1% and 80.9%, respectively. The full factorial design indicated that increased lipid concentration improved %EE, while higher surfactant concentration enhanced drug release and %EE. Stability studies of the optimized batch revealed no alterations in physical or chemical characteristics. CONCLUSION: The study successfully developed Solid Lipid Nanoparticles (SLNs) for artemisinin and curcumin, achieving controlled drug release, high entrapment efficiency, and desired particle size and uniformity. This advancement holds promise for enhancing drug delivery of herbal formulations.

Current Practices in Pediatric Asthma Care.

This article is a comprehensive review of the latest knowledge and developments on pediatric asthma. It serves as a guide for general practitioners and subspecialists who treat asthma. The pathophysiology and critical features of asthma that should be addressed and the latest therapies available are discussed. The areas where further investigation is needed are also highlighted.

Dermoscopic Perspective of Pityriasis Versicolor in a Cross-Sectional Study.

Background: Pityriasis versicolor is a common superficial fungal infection which is usually easily diagnosed with Wood's lamp examination and 10% potassium hydroxide mount. However, these modalities have varying sensitivity and specificity. Aims and Objectives: This study aimed to ascertain the dermoscopic features of pityriasis versicolor lesionally as well as perilesionally using dermoscopy, a non-invasive diagnostic tool. Materials and Methods: In this cross-sectional study, consecutive patients with pityriasis versicolor underwent dermoscopic examination of lesions and 2 cm around lesions, noting lesional and perilesional features. Semi-objective grading of pigmentation, scaling, and vascularity was done. The association between parameters was determined using heat maps and violin plots with Kolmogorov-Smirnov test. Lesional analysis was performed since lesions at different sides showed disparate features. Results: A total of 353 lesions from 233 patients (males = 150/233; 64.38% and females = 84/234; 36.05%) were studied. On lesional dermoscopy, pigmentary and scaling abnormalities were universal. 258/353 (73.1%) of lesions showed vascular abnormalities. Perilesionally, scaling (223/353; 63.17%) followed by pigmentation (205/353; 58.07%) and vascular changes (111/353; 31.44%) constituted the most common dermoscopic abnormalities and were noted in 294/353 (83.29%) of lesions overall. Increased disease duration corresponded with increased intensity of perilesional pigmentation alterations, perifollicular (P = 0.04), and follicular scales (P = 0.02). Conclusion: Awareness of dermoscopic features could improve the diagnostic accuracy in doubtful cases of pityriasis versicolor. Vascular findings are common and may point to an underlying inflammatory pathogenesis. Perilesional findings constitute early dermoscopic features of pityriasis versicolor and hint at the need for treatment beyond the confines of lesions. Larger follow-up studies and research into immunopathogenesis may be of further benefit.

Digital Technology Characteristics and Literacy Among Families With Children With Asthma: Cross-Sectional Study.

Background: The use of digital technology in pediatric asthma management has emerged as a potential tool for improving asthma management. However, the use of digital tools has the potential to contribute to the inequitable delivery of asthma care because of existing social factors associated with asthma disparities. Our study focused on parents' chosen language and sociodemographic factors that might shape the use of digital technology in asthma self-management. Objective: This study aims to estimate and compare patient, family, and technology-related characteristics by parents' chosen language (English or Spanish) and compare a digital literacy measure by sociodemographic factors. Methods: Survey data were collected from July to December 2021 from parents of children with asthma who were seen by a Chicago pediatric health system pulmonary provider. Questions assessed patient and family characteristics, digital technology use, and digital literacy, measured using the validated eHealth Literacy Scale (eHEALS). Chi-square tests and multivariable logistic regression were used for comparisons, and Kruskal-Wallis tests were used for comparing median eHEALS scores by social characteristics. Results: Of the 197 parents surveyed, 24.4% (n=49) of parents identified as a race categorized as other, 37.1% (n=67) as White, and 38.6% (n=75) as Black; 47.2% (n=93) identified as Hispanic/Latino/Latina. Additionally, 79.7% (n=157) of parents preferred English, and 20.3% (n=40) preferred Spanish. English-speaking parents were more likely to report having a data plan for their smartphone (117/157, 74.5%) or high-speed internet (138/157, 87.9%) compared to Spanish-speaking parents (smartphone: 23/40, 58%; P=.03; internet: 27/40, 68%; P=.002). Compared with Spanish-speaking parents, English-speaking parents were less likely to report having a lot or some concern about paying for internet (28/40, 70% vs 83/157, 52.9%; P=.046) or about data privacy (35/40, 88% vs 105/157, 67.5%; P=.01). Digital literacy scores differed significantly by race, income, education level, and language. In a multivariable model, language was not a significant factor for having high-speed internet service (P=.12) or concern about paying for internet at home (P=.60), but it was a significant factor for concerns about data privacy (P=.04). Conclusions: The significant differences in technology-related characteristics suggest that digital connectivity, affordability, and data privacy may also be important factors in considering digital technology use in asthma care.

Codesign of remote data collection for chronic management of pediatric home mechanical ventilation.

INTRODUCTION: Outpatient monitoring of children using invasive home mechanical ventilation (IHMV) is recommended, but access to care can be difficult. This study tested if remote (home-based) data collection was feasible and acceptable in chronic IHMV management. METHODS: A codesign study was conducted with an IHMV program, home nurses, and English- and Spanish-speaking parent-guardians of children using IHMV (0-17 years; n = 19). After prototyping, parents used a remote patient monitoring (RPM) bundle to collect patient heart rate, respiratory rate (RR), oxygen saturation, end-tidal carbon dioxide (EtCO2 ), and ventilator pressure/volume over 8 weeks. User feedback was analyzed using qualitative methods and the System Usability Scale (SUS). Expected marginal mean differences within patient measures when awake, asleep, or after a break were calculated using mixed effects models. RESULTS: Patients were a median 2.9 years old and 11 (58%) took breaks off the ventilator. RPM data were entered on a mean of 83.7% (SD ± 29.1%) weeks. SUS scores were 84.8 (SD ± 10.5) for nurses and 91.8 (SD ± 10.1) for parents. Over 90% of parents agreed/strongly agreed that RPM data collection was feasible and relevant to their child's care. Within-patient comparisons revealed that EtCO2 (break-vs-asleep 2.55 mmHg, d = 0.79 [0.42-1.15], p < .001; awake-vs-break 1.48, d = -0.49 [0.13-0.84], p = .02) and RR (break-vs-asleep 16.14, d = 2.12 [1.71-2.53], p < .001; awake-vs-break 3.44, d = 0.45 [0.10-0.04], p = .03) were significantly higher during ventilator breaks. CONCLUSIONS: RPM data collection in children with IHMV was feasible, acceptable, and captured clinically meaningful vital sign changes during ventilator breaks, supporting the clinical utility of RPM in IHMV management.

Stevens-Johnson Syndrome in Adult Patient Secondary to COVID-19 Infection: Case Report.

COVID-19 is a global pandemic caused by a novel zoonotic RNA virus named SARS-CoV-2. Various cutaneous manifestations associated with COVID-19 have been described, including urticarial rash, confluent erythematous rash, papulovesicular exanthem, chilblain-like acral pattern, livedo reticularis, and purpuric vasculitis pattern. Here, we are presenting a case of a 45-year-old male with mucocutaneous features of Stevens-Johnson syndrome.

Prolonged Ocular Foreign Body Found on Repeat Visit to a Second Emergency Department.

We describe a case where the patient presented to the emergency department (ED) with ocular irritation in the right eye with concomitant blurry vision that had been persistent for a week. The cause of this patient's ocular irritation and worsening visual acuity was determined to be a retained foreign body of the limbus. The foreign body had been in the patient's eye for about four months before he began to experience these symptoms. The four-month duration was established based on initial symptoms and a prior ED visit with no noted eye injury or foreign body detection, as well as the degree of overlying epithelization. This case highlights the importance of obtaining a thorough history and physical examination while emphasizing the high index of suspicion needed for translucent foreign bodies. Here, an inert foreign body erupted four months after injury. Additionally, this case stresses the importance of transition of care for ophthalmologic conditions. Consideration of any social determinants of health that could prevent as an example.

Halting the Progression of Cicatricial Alopecia with Injectable Platelet-Rich Fibrin.

Discoid lupus erythematosus (DLE) is a chronic variant of cutaneous lupus erythematosus characterized by well-defined erythematous plaques with adherent scales and follicular plugging. The affected scalp shows erythema, edema, atrophy, alopecia, and telangiectasia. Trichoscopy of DLE shows branching capillaries, white patches, keratin plugs, reduced follicular ostia, and white dots and blue-gray dots arranged in speckles pattern. Prompt diagnosis and aggressive, early multimodal therapy helps in preventing disfiguring hair loss and psychosocial sequelae. Hereby, we present a case of reversal of hair loss in DLE with newer modalities of treatment such as injectable platelet-rich fibrin.

SPT6 functions in transcriptional pause/release via PAF1C recruitment.

It is unclear how various factors functioning in the transcriptional elongation by RNA polymerase II (RNA Pol II) cooperatively regulate pause/release and productive elongation in living cells. Using an acute protein-depletion approach, we report that SPT6 depletion results in the release of paused RNA Pol II into gene bodies through an impaired recruitment of PAF1C. Short genes demonstrate a release with increased mature transcripts, whereas long genes are released but fail to yield mature transcripts, due to a reduced processivity resulting from both SPT6 and PAF1C loss. Unexpectedly, SPT6 depletion causes an association of NELF with the elongating RNA Pol II on gene bodies, without any observed functional significance on transcriptional elongation pattern, arguing against a role for NELF in keeping RNA Pol II in the paused state. Furthermore, SPT6 depletion impairs heat-shock-induced pausing, pointing to a role for SPT6 in regulating RNA Pol II pause/release through PAF1C recruitment.

At-home end-tidal carbon dioxide measurement in children with invasive home mechanical ventilation.

BACKGROUND: Carbon dioxide concentration trending is used in chronic management of children with invasive home mechanical ventilation (HMV) in clinical settings, but options for end-tidal carbon dioxide (EtCO2 ) monitoring at home are limited. We hypothesized that a palm-sized, portable endotracheal capnograph (PEC) that measures EtCO2 could be adapted for in-home use in children with HMV. METHODS: We evaluated the internal consistency of the PEC by calculating an intraclass correlation coefficient of three back-to-back breaths by children (0-17 years) at baseline health in the clinic. Pearson's correlation was calculated for PEC EtCO2 values with concurrent mean values of in-clinic EtCO2 and transcutaneous CO2 (TCM) capnometers. The Bland-Altman test determined their level of agreement. Qualitative interviews and surveys assessed usability and acceptability by family-caregivers at home. RESULTS: CO2 values were collected in awake children in varied activity levels and positions (N = 30). The intraclass correlation coefficient for the PEC was 0.95 (p < 0.05). The correlation between the PEC and in-clinic EtCO2 device was 0.85 with a mean difference of -3.8 mmHg and precision of ±1.1 mmHg. The correlation between the PEC and the clinic TCM device was 0.92 with a mean difference of 0.2 mmHg and precision of ±1.0. Family-caregivers (N = 10) trialed the PEC at home; all were able to obtain measurements at home while children were awake and sometimes asleep. CONCLUSIONS: A portable, noninvasive device for measuring EtCO2 was feasible and acceptable, with values that trend similarly to currently in-practice, outpatient models. These devices may facilitate monitoring of EtCO2 at home in children with invasive HMV.

Chronic cough and weight loss in an adolescent marijuana smoker.

Marijuana is the most widely used illicit drug in the United States. As marijuana becomes legalized in more states and its use increases among adolescents, pediatricians must be aware of the impact of marijuana on pediatric health. Marijuana smoking as well as cigarette smoking has been associated with numerous lung diseases, including chronic bronchitis and bullous lung diseases. This case report postulates that regular marijuana smoking may be associated with pulmonary Langerhans cell histiocytosis, a severe lung disease that lacks definitive treatment and can cause respiratory failure. Given the potential risk of life-threatening lung diseases, pediatricians must screen adolescents with respiratory symptoms for marijuana use. In addition, this case underscores the need for further research and improved understanding of the relationship between marijuana smoking and lung disease.

Primary Ciliary Dyskinesia: A Rare and Often Underdiagnosed Disease.

Primary ciliary dyskinesia (PCD) is a rare autosomal recessive disease with clinical features of ear, sinus, and pulmonary infections that overlap with common respiratory illnesses of childhood. It is a progressive disorder that has significant influence on quality of life, lung function, and survival. Given the considerable overlap of symptoms between common illnesses and PCD, a high index of suspicion by primary care providers is needed to consider the diagnosis. There is not a single "gold standard" diagnostic test for PCD and multiple diagnostic methods coupled with specialized expertise is often needed to make the diagnosis. Patients with PCD also have comorbidities requiring a multidisciplinary approach for optimal clinical management. It is important for primary care physicians to recognize the PCD clinical phenotype and have a diagnostic framework for these patients. [Pediatr Ann. 2022;51(2):e82-e85.].

Molecular genotyping of Indian blood group antigens amongst regular voluntary blood donors of Surat city, Gujarat, India.

BACKGROUND: There is paucity of data related to the prevalence of the rare blood group antigens amongst South Gujarat blood donor population due to unavailability and high cost of antisera. Therefore it is difficult to screen donors for such rare antigens by gold standard haemagglutination assay. The single nucleotide polymorphism (SNPs) of Ina and Inb antigens is the base of the PCR based detection methods that help to detect these alleles in regular voluntary blood donors. MATERIALS & METHODS: Blood samples of 200 unrelated regular voluntary blood donors wee collected. DNA was extracted using phenol-chloroform method and genotyped for Indian (Ina/IN*01, Inb/IN*02) blood group alleles by Sequence Specific PCR. Ina antigen positivity was confirmed by serology test. RESULTS: Four donors were found heterozygous for Ina antigen i.e. In (a + b+) by SS-PCR and their Ina positivity were confirmed by in-house polyclonal Anti-Ina reagent. SS-PCR was standardized using known heterozygous sample of a blood donor. The frequency of Ina antigen (2.0 %) was higher than Caucasians, lower than Iranians and Arabs while comparable to those reported among Indians of Mumbai city. CONCLUSION: In absence or unavailability of antisera particularly for low frequency alleles like Ina, such PCR based method would be extremely helpful to prepare rare donor registry by screening blood donors' at large scale. Red cells of Ina positive donors can be used as in-house reagent red cells for screening and identification of corresponding antibody.

SPT5 stabilization of promoter-proximal RNA polymerase II.

Based on in vitro studies, it has been demonstrated that the DSIF complex, composed of SPT4 and SPT5, regulates the elongation stage of transcription catalyzed by RNA polymerase II (RNA Pol II). The precise cellular function of SPT5 is not clear, because conventional gene depletion strategies for SPT5 result in loss of cellular viability. Using an acute inducible protein depletion strategy to circumvent this issue, we report that SPT5 loss triggers the ubiquitination and proteasomal degradation of the core RNA Pol II subunit RPB1, a process that we show to be evolutionarily conserved from yeast to human cells. RPB1 degradation requires the E3 ligase Cullin 3, the unfoldase VCP/p97, and a novel form of CDK9 kinase complex. Our study demonstrates that SPT5 stabilizes RNA Pol II specifically at promoter-proximal regions, permitting RNA Pol II release from promoters into gene bodies and providing mechanistic insight into the cellular function of SPT5 in safeguarding accurate gene expression.

A ChIP-exo screen of 887 Protein Capture Reagents Program transcription factor antibodies in human cells.

Antibodies offer a powerful means to interrogate specific proteins in a complex milieu. However, antibody availability and reliability can be problematic, whereas epitope tagging can be impractical in many cases. To address these limitations, the Protein Capture Reagents Program (PCRP) generated over a thousand renewable monoclonal antibodies (mAbs) against human presumptive chromatin proteins. However, these reagents have not been widely field-tested. We therefore performed a screen to test their ability to enrich genomic regions via chromatin immunoprecipitation (ChIP) and a variety of orthogonal assays. Eight hundred eighty-seven unique antibodies against 681 unique human transcription factors (TFs) were assayed by ultra-high-resolution ChIP-exo/seq, generating approximately 1200 ChIP-exo data sets, primarily in a single pass in one cell type (K562). Subsets of PCRP mAbs were further tested in ChIP-seq, CUT&RUN, STORM super-resolution microscopy, immunoblots, and protein binding microarray (PBM) experiments. About 5% of the tested antibodies displayed high-confidence target (i.e., cognate antigen) enrichment across at least one assay and are strong candidates for additional validation. An additional 34% produced ChIP-exo data that were distinct from background and thus warrant further testing. The remaining 61% were not substantially different from background, and likely require consideration of a much broader survey of cell types and/or assay optimizations. We show and discuss the metrics and challenges to antibody validation in chromatin-based assays.

A case of disseminated subcutaneous phaeohyphomycosis caused by Exserohilum rostratum with CARD9 mutation.

Phaeohypomycosis is a rare cutaneous and subcutaneous fungal infection caused by dematiaceous fungi. They have a widespread global distribution occasionally affecting humans. A 26-year-old woman presented with multiple skin lesions over her face and extremities for last 7 years, unresponsive to systemic amphotericin B and itraconazole. Further investigations revealed CARD9 mutation and phaeohyphomycosis caused by the pigmented fungus Exserohilum rosatratum. Lesions subsequently improved with oral flucytosine and itraconazole.

Etiological prevalence and antifungal sensitivity patterns of dermatophytosis in India - A multicentric study.

BACKGROUND: The prevalence of dermatophytes varies with season, geographical area, socio-economic factors and effective management strategies. AIMS: The aim of the study was to assess the prevalence of pathogenic dermatophytes, clinical types of dermatophyte fungal infection, and in vitro antifungal drug susceptibility testing against dermatophytes. METHODS: Three hundred and ninety five patients with dermatophytosis were enrolled from five cities (Mumbai, Delhi, Lucknow, Kolkata and Hyderabad) across India. All patients were subjected to clinical examination and investigations, including potassium hydroxide microscopy, fungal culture and antifungal drug susceptibility testing. RESULTS: Trichophyton rubrum was the most common species identified (68.4%), followed by T. mentagrophytes (29.3%). Within species, T. mentagrophytes was prevalent in humid environmental conditions (Mumbai and Kolkata), whereas T. rubrum was prevalent in noncoastal areas (Delhi, Lucknow and Hyderabad). Tinea corporis (71.4%) and tinea cruris (62.0%) were the common clinical types observed. antifungal drug susceptibility testing data indicated that minimum inhibitory concentration required to inhibit the growth of 90% of organisms (MIC-90) was lowest for griseofulvin (0.25-3.0 µg/mL). Among oral antifungals, the mean MIC of itraconazole was within the range (0.84 [0.252] µg/ mL), whereas high mean MIC values were reported for terbinafine (0.05 [0.043] µg/mL). Among topical agents, lowest mean MIC values were reported for luliconazole (0.29 [0.286] µg/mL), eberconazole (0.32 [0.251]) µg/mL and amorolfine (0.60 [0.306]) µg/mL. LIMITATIONS: Lack of correlation between in vitro antifungal susceptibility and clinical outcome and absence of defined MIC breakpoints. CONCLUSION: T. rubrum was the most common, followed by T. mentagrophytes as an emerging/codominant fungal isolate in India. Tinea corporis was the most common clinical type of dermatophytosis. Mean MIC of terbinafine was above the reference range, while it was within the range for itraconazole; griseofulvin had the lowest mean MIC. Luliconazole presented the lowest mean MIC values across cities.