Deficiency of adenosine deaminase 2 leading to recurrent Hodgkin lymphoma: A case report.

Deficiency of adenosine deaminase 2 is a rare monogenic multi-organ disease of children and less often adults resulting from mutations in the adenosine deaminase 2 gene. We present a case of a 35-year-old Palestinian male with adenosine deaminase 2 deficiency and maturity-onset diabetes of the young type 2. The patient initially presented with complaints of swelling in his neck and night sweats, leading to a diagnosis of Hodgkin lymphoma. Subsequent evaluation revealed a recurrence of Hodgkin lymphoma, along with symptoms of otitis media, upper respiratory tract infection, and a rash around the mouth. Genetic testing confirmed mutations in the adenosine deaminase 2 gene and glucokinase genes, confirming the diagnosis of deficiency of adenosine deaminase 2 and maturity-onset diabetes of the young type 2, respectively. The patient was treated with Intravenous immunoglobulin, antiviral drugs, and oral hypoglycemic drugs, showing improvement in symptoms and laboratory tests. This case highlights the importance of considering rare genetic disorders in patients with unusual or refractory clinical manifestations, and the need for a multidisciplinary approach in such cases.

The Effect of Mechanical Alteration on Repair Bond Strength of S-PRG-Filler-Based Resin Composite Materials.

This study investigates the impact of mechanical alteration on resin composite surfaces and its subsequent effect on repair bond strength. A total of 100 resin composite disks were prepared and were allocated for 24 h or 1 year of artificial aging. Specimens were embedded in epoxy resin, and the composite surfaces were mechanically altered using either diamond burs or air abrasion with aluminum oxide or glass beads. A universal bonding material was applied and a 2 mm circular and 3 mm high repair composite cylinder were prepared using a Teflon mold. Then, the specimens were tested for their shear bond strength, and the de-bonded specimens were observed under a scanning electron microscope to determine the failure pattern. SPSS 26.0 statistical software was used to analyze the data. Two-way ANOVA showed a statistically significant effect of mechanical alteration and aging on the shear bond strength of S-PRG-filler-based resin composite (p < 0.05). Surface modification with a fine diamond bur showed a significantly higher bond strength in both 24-h- and 1-year-aged specimens. Surface modification with alumina significantly increased the bond strength of 1-year-aged specimens; however, it was statistically insignificant for 24 h-aged specimens. Mechanical alteration with a fine diamond bur and 50-micron alumina can improve the repair bond strength of the composite.

Concurrent glossopharyngeal neuralgia and oromandibular dystonia resolved after microvascular decompression of the trigeminal and glossopharyngeal nerve: A rare presentation.

Background: This type of pain syndrome occurs suddenly and briefly, beginning unilaterally from one side of the face. Modestly stimulating speech can provoke it, affecting the ear, tongue, throat, and jaw angle. Interestingly, it is the sensory distribution of the auricular and the pharyngeal branches of the cranial nerves IX and X. People have not had a confirmed case of glossopharyngeal neuralgia (GPN), along with oromandibular dystonia (OMD). Nevertheless, usually in the medical literature, this case report supplies information about a patient who has concurrent GPN and OMD. Case Description: A 36-year-old male patient presented with a history of sudden onset of increasing electric pains, which were centered in the middle of the forehead to the depth of the throat and accompanied by uncontrolled movements, repetitive tongue protrusions, jaw movements, and recurrent pervasive gagging reflexes. Magnetic resonance imaging showed that a vascular loop of the superior cerebellar and anterior inferior cerebellar artery on the left side had crossed over and compressed those nerves. Decompression surgery in the left glossopharyngeal and trigeminal nerves cured all the symptoms. Conclusion: The simultaneous occurrence of GPN and OMD is rare, complex, and challenging from the clinician's viewpoint in the management of similar but different pathologies. A detailed history was taken, and a radiological investigation was called to devise a management plan in the context of understanding the pathology of both disorders.

Atypical Teratoid/Rhabdoid Tumor of the Sellar Region in an Adult Male: A Case Report.

Atypical teratoid/rhabdoid tumor (AT/RT) is a rare, fast-growing, aggressive tumor that is almost exclusively seen in the pediatric population; it has a poor prognosis despite aggressive treatment. Adult cases were thought to be exclusively of women, with a total of 23 cases reported worldwide. We herein report a case of a 35-year-old male who posed a unique clinical and diagnostic challenge. To the best of our knowledge, this is the third case of a male patient with sellar AT/RT in the world.

The effects of pH on U(VI)/Th(IV) and Ra(II)/Ba(II) adsorption by polystyrene-nano manganese dioxide composites: Fourier Transform Infra-Red spectroscopic analysis.

Fourier Transform Infra-Red (FTIR) spectroscopy provides structural information of prime importance to understand ions coordination to adsorbents. This consequently aids in the design of improved ion exchange materials and help in deriving the optimum adsorption conditions. In the present work, the adsorption mechanism of both U(VI)/Th(IV) and Ra(II)/Ba(II) radionuclides couples onto polystyrene-nano manganese dioxide (PS-NMO) composite is reported in relation to the effect of working solution pH. The separation of each radionuclide couple; i.e. U(VI)/Th(IV) and Ra(II)/Ba(II); could be effectively achieved at pH = 3 and pH = 1 respectively. The pH values not only determine the species of the respected elements that are mainly present in aqueous solution before applying the adsorbent, but it also alters the structure of the composite adsorbent. FTIR spectroscopy showed that Th(IV) formed inner sphere complexes and occupied the A site in the dioxide layer, while U(VI) formed outer sphere complexes on the surface of the composite. Spectra subtraction showed that some aromatic bands and vinyl C-H bands were split or shifted to lower wavenumbers with the loading of Ba(II). This was attributed to changes in the composite stereochemistry to accommodate Ba(II). The working solution pH could be the key in the separation process of both U(VI)/Th(IV) and Ra(II)/Ba(II) from their mixture, and FTIR spectroscopy stands as a useful technique to explain the difference between metal ions responses to adsorbants.

Postoperative cerebrospinal fluid infection by Ralstonia mannitolilytica: Two case reports and a literature review.

Background: Ralstonia species are Gram-negative bacilli that are commonly found in moist environments, such as water and soil. They are opportunistic human pathogens, particularly found among immunocompromised patients, and are an infrequent cause of infection. The difficulty in correctly identifying and differentiating between Ralstonia species members using routine biochemical methods as well as their resistance to many classes of antibiotics poses a specific diagnostic and therapeutic challenge. Case Description: We report two cases from our neurosurgical unit complicated by postoperative cerebrospinal fluid infection caused by Ralstonia Mannitolilytica that posed a therapeutic challenge. Conclusion: Our hypothesis is contaminated irrigation fluids might be a significant cause of post-operative meningitis and prolonged hospital stay.

Effect of cranioplasty timing on the functional neurological outcome and postoperative complications.

BACKGROUND: The optimal timing for performing cranioplasty and its effect on functional outcome remains debatable. Multiple confounding factors may come into role; including the material used, surgical technique, cognitive assessment tools, and the overall complications. The aim of this study is to assess the neurological outcome and postoperative complications in patients who underwent early versus late cranioplasty. METHODS: A retrospective cohort study was conducted to investigate the neurological outcome and postoperative complications in patients who underwent cranioplasty between 2005 and 2018 at a Level l trauma center. Early and late cranioplasties were defined as surgeries performed within and more than 90 days of decompressive craniectomy, respectively. The Glasgow Outcome Score (GOS) and modified Rankin scale (mRS), recorded within 1 week of cranioplasty, were used to assess the neurological outcome. RESULTS: A total of 101 cases of cranioplasty were included in the study. The mean age of the patients was 31.4 ± 13.9 years. Most patients (n = 86; 85.1%) were male. The mean GOS for all patients was 4.0 ± 1.0. The mean mRS was 2.2 ± 1.78. Hydrocephalus was noted in 18 patients (early, n = 6; late, n = 12; P = 0.48). Seizures developed in 28 patients (early, n = 12; late, n = 16; P = 0.77). CONCLUSION: The neurological outcome in patients who underwent early versus late cranioplasty is almost identical. The differences in the rates of overall postoperative complications between early versus late cranioplasty were statistically insignificant. The optimal timing for performing cranioplasty is mainly dependent on the resolution of cerebral swelling.

Impact of COVID-19 on Saudi Neurosurgery Residency: Trainers' and Trainees' Perspectives.

INTRODUCTION: After the official announcement of the coronavirus disease-19 pandemic on March 11, 2020, the disease impacted most aspects of health care delivery, especially postgraduate education and training. METHOD: A cross-sectional, online questionnaire-based assessment was performed. The study participants involved neurosurgery residents and program directors (PDs) across the country between May 16 and May 27, 2020. RESULTS: Approximately 74 of 95 (77.9%) of the residents experienced an impact on their training calendar. Before the pandemic, 51 residents (53.3%) were involved in 2-3 surgeries per week, but during the pandemic, 66 (69.5%) were attending 0-1 case per week. Fifty-three residents (55.8%) agreed that academic sessions were affected despite the helpful effort of online teaching sessions. Thirty-four (35.8%) residents graded their anxiety during coronavirus disease-19 times as high. Ten PDs (58.8%) confirmed spending 3-5 hours per week on educational activities normally, whereas during the pandemic, 15 PDs (88.2%) reduced their educational hours to 0-2 hours per week. CONCLUSION: Our study showed that educational activities significantly decreased and shifted toward virtual teaching methods. Operative volume showed a substantial reduction for both junior and senior residents. Academic and clinical teaching was the main concern for PDs, and they faced challenges interviewing newly matched residents.

Endoscopic Reconstruction of Skull Base Defects Using Tutoplast.

BACKGROUND: Various graft materials that are classified as autografts, xenografts, and allografts based on their origin have been used to endoscopically repair skull base defects. Tutoplast® (Tutogen Medical GmbH), an allogeneic natural collagen matrix, is processed through chemical sterilization that preserves tissue biocompatibility and structural integrity. OBJECTIVE: To study the safety and efficacy of Tutoplast Fascia Lata and Tutoplast Temporalis Fascia® as primary graft materials in the endoscopic reconstruction of skull base defects of different sizes and etiologies and to compare the outcomes with those of other traditional graft materials based on our experience. METHODS: This is a multi-center retrospective chart review of patients who underwent cerebrospinal fluid leak (CSF) endoscopic reconstruction with Tutoplast Fascia Lata or Tutoplast Temporalis Fascia as either a stand-alone reconstruction material or a part of a multilayer reconstruction depending on the defect at Prince Sultan Military Medical City and King Faisal Specialist Hospital and Research Center in Riyadh, Saudi Arabia, between 2017 and 2020. Our inclusion criteria were CSF leak repair with Tutoplast and a transnasal endoscopic approach. We reviewed demographic data, intraoperative and postoperative complications, repair materials, repair failure, defect size and location. RESULTS: Tutoplast® was used as the primary graft material in 33 cases. Our main outcome was repair success with lack of post operative CSF leak, observed in 30 cases (90.9%). There was no significant association between postoperative CSF leaks and factors including different defect sizes, defect sites, demographic data, hospitalization duration, or postoperative radiation in oncological cases. CONCLUSION: Tutoplast alone or in combination with other materials can be used safely and effectively for skull base defects repair.

Infantile atypical subependymal giant cell astrocytoma.

Subependymal giant cell astrocytoma is a benign WHO grade I intraventricular tumor arise in patients with tuberous sclerosis complex. Previous reported described histopathological predictors of more aggressive forms, terms atypical SEGA in infantile age group. Other reports showed possible transformation of SEGA into glioblastoma, or misdiagnosis as glioblastoma due to the presence of atypical histopathological features. Here, we report a case of an infant who presented with right frontal extraventricular SEGA and underwent craniotomy with complete resection. Eight months later, he presented with fast recurrence in same location with midline shift and subfalcine herniation. Histopathological description showed high grade features including Ki labeling index of 60%, atypical mitotic figures, cellular plemorphism and necrosis. We also discussed the possible presence of different entity (termed atypical SEGA) which may have more aggressive clinical course, with literature review of predictors of SEGA aggressiveness and possible transformation/misdiagnosis as glioblastoma.

Predictors of Surgical Site Infection in Autologous Cranioplasty: A Retrospective Analysis of Subcutaneously Preserved Bone Flaps in Abdominal Pockets.

BACKGROUND: Decompressive craniectomy (DC) is a surgical procedure performed to manage intracranial hypertension. Once performed, patients are obligated to undergo another surgical procedure known as cranioplasty to reconstruct the cranial defect. Cranioplasty still has one of the highest rates of infection. The factors contributing to the high rate of surgical site infection (SSI) after cranioplasty are not well established. This study aims to estimate the incidence of SSI and determine its possible risk factors for patients who underwent cranioplasty using bone flaps subcutaneously preserved in abdominal pockets. METHODS: A retrospective cohort study was conducted to investigate the predictors of infection among patients who underwent cranioplasty from subcutaneously preserved bone flaps in abdominal pockets between January 2005 and December 2018 at a level l trauma center. RESULTS: A total of 103 cases of cranioplasty from subcutaneously preserved bone flaps were included in the study. The mean age of the patients was 31.2 ± 14.8 years (range, 5-67 years). The median interval between DC and cranioplasty was 115 days. The most frequent indication for DC was traumatic brain injury (76.4%). The incidence of SSI was noted in 15.7% of patients. The most significant predictors of infection in patients requiring cranioplasty were blood glucose levels and skull defect size (P = 0.03 and P = 0.02, respectively). CONCLUSIONS: Blood glucose levels and skull defect size were the only identifiable risk factors associated with SSI. Storing bone flaps in subcutaneous abdominal pockets is cost-efficient but carries considerable risk of infection.

Parent's knowledge and practice in home management of fever in their children in Riyadh, Saudi Arabia.

BACKGROUND: Fever in children is a common presenting complaint during health visits. Parents frequently have concerns about fever and perceive it as a disease rather than a symptom of illness. Parent's practice of home managing of fever varies according to their background and experience. OBJECTIVE: To explore parents' perception and practice in home management of fever in their preschool children in Riyadh, Saudi Arabia (SA). MATERIALS AND METHODS: This is a cross-sectional study. Data were collected from 250 parents attending three main family medicine centers at King Abdul-Aziz Medical City, Riyadh, SA, using self-administered questionnaire. RESULTS: Most of the parents (64%) defined fever correctly and 56% identified high fever. Almost all the parents (95%) believed fever is harmful, and febrile convulsion was the most concerned complication of fever (74%), followed by loss of consciousness, dehydration, brain damage, and hearing loss. Most of the parents (82%) touch their children to confirm fever, 68% use oral thermometer, and 63% use axillary thermometer. Most parents (84%) applied cold compression, 75% gave their children nonprescribed fever medication, 61% gave their children plenty of fluids, and 64% took their children to the doctor right away. Almost one-third of participants reported having difficulty either in choosing fever medicine or giving the proper dose and frequency. No difference in knowledge or practice was found in relation to difference in demographic characteristics of participants. CONCLUSION: Results of this study indicate poor knowledge and practice in regard to parent's management of febrile children, overuse of nonprescribed fever medication, and possible waste of health resources.

An innovative procedure for NORM scales treatment and radionuclides separation.

The present study focuses on the development of a new chemical treatment method for naturally occurring radioactive materials (NORM) scale wastes from the oil industry. The method consists of three-stages, including the separation of oil using the Fenton oxidation process at room temperature, the complete dissolution of soluble and slightly soluble salts and separation of Ra and Pb isotopes. The proposed method can be considered as an innovative procedure for NORM scales treatment and radionuclides separation, which in turn support the radioactive waste management with economic benefits.

Chemical fractionation of radium-226 in NORM contaminated soil from oilfields.

Contamination of soil with 226Ra is a common problem in the oilfields, leading to costly remediation and disposal programmes. The present study focuses on the chemical fractionation and mobility of 226Ra in contaminated soils collected from an oilfield using a three-step sequential extraction procedure (BCR). The total activity concentrations of 226Ra in contaminated soils were measured and found to be in the range from 1030 ± 90 to 7780 ± 530 Bq kg-1, with a mean activity concentration of 2840 ± 1840 Bq kg-1. The correlation between the total concentration of 226Ra and soil properties, mainly pH, LOI, Corg, clay and Ca, was investigated using the principal component analysis method (PCA). The chemical fractionation of 226Ra was studied using the sequential extraction method (BCR). The highest fraction of 226Ra (27-65%) was found to be in the acid-reducible fraction, which suggests that 226Ra is mainly bound to FeMn oxides. The BCR method showed that high percentages of 226Ra were found to be in mobile soil phases (between 45 and 99%). Consequently, groundwater contamination could occur due to the remobilization of 226Ra from soils under normal environmental conditions. However, the obtained results could be useful to reduce the volume of NORM wastes generated from the oilfields and decision-making process for final treatment and disposal of NORM-contaminated soil.

Dissolution of [²²6Ra]BaSO4 and partial separation of ²²6Ra from radium/barium sulfate: A new treatment method for NORM waste from petroleum industry.

Complete dissolution of [(226)Ra]BaSO4 precipitate was successfully performed using NaNO2 as a reducing agent in acidic solution at room temperature. Results showed a significant effect of acid and NaNO2 concentrations and temperature on the dissolution efficiency. The method was successfully used for separation of radium from NORM scale samples from the petroleum industry; sufficient volume reduction of NORM waste was achieved. The obtained (226)Ra solution was purified using two separation methods. The dissolution method can be of great interest in the development of radiochemical analysis of radium isotopes.

Characterization of NORM solid waste produced from the petroleum industry.

The accumulation of scales in the production pipe lines is a common problem in the oil industry, reducing fluid flow and leading to costly remediation and disposal programmes. Thus, an accurate determination of the activity of the radionuclides in scale samples is essential for environmental protection. The present study focuses on the characterization of naturally occurring radioactive materials (NORM) in scales generated from the petroleum industry to develop a suitable NORM waste management plan. The activity concentrations of 226Ra, 228Ra and 210Pb in 32 representative samples, collected from a number of drums at the NORM Decontamination Facility storage, were determined using gamma spectrometry. It was found that the highest concentrations were 2922, 254 and 1794 Bq g(-1) for 226Ra, 228Ra and 210Pb, respectively. A comparison to the reported worldwide values was made. Statistical approaches, namely Box plot, ANOVA and principal components analysis were applied on the total results. Maximal correlation was demonstrated by 226Ra activity concentration and count per second (cps) to density ratio. To obtain an accurate characterization of the radionuclides studied in the scale samples, method validation of gamma measurement procedure was carried out, in which minimum detectable activity, repeatability, intermediate precision and assessment of uncertainty were the parameters investigated. The work is a forefront for the proper and safe disposal of such radioactive wastes.

Distribution and baseline values of trace elements in the sediment of Var River catchment, Southeast France.

This study reports on the determination of trace element (TE)-Li, As, Co, Cs, Cu, Pb, U, and Zn-and major element (ME)-Si, Al, Fe, Mg, Ca, Mn, Na, and K-concentrations in 18 riverbed sediments and a sediment core from the Var River catchment using inductively coupled plasma mass spectrometry (ICP-MS). The results were compared with those of a reference sediment core, and the contribution of clay and organic carbon contents in the distribution of TE and ME in the sediment samples was investigated. The mean concentrations of the ME were comparable in both core and riverbed samples and were within the natural averages. In the case of TE, the concentrations were lower in riverbed sediment samples than those found in the sediment core. High mean concentration of As was observed (7.6 µg g(-1)) in both core and riverbed sediments, relatively higher than the worldwide reported values. The obtained data indicated that the natural high level of arsenic might be originated from the parent rocks, especially metamorphic rocks surrounding granites and from Permian sediments. Statistical approach, viz., Pearson correlation matrix, was applied to better understand the correlation among TE in both riverbed and sediment core samples. No significant metallic contamination was detected in the low Var valley despite of the localization of several industrial facilities. Therefore, results confirm that the concentrations of the TE obtained in the riverbed sediments could be considered as a baseline guide for future pollution monitoring program.