Acute Cholecystitis in a Patient With Situs Inversus.

A rare disorder called situs inversus partialis (SIP) is characterized by the transposition of organs in the abdomen or thoracic cavity from one side of the body to the other (the mirror image of normal). Autosomal dominant, autosomal recessive, rare genetic mutations, and X-linked recessive inheritance patterns have been identified to be involved in this condition. Laparoscopic cholecystectomies have been successfully performed on patients with SIT. Due to challenges in spatial orientation and the identification of anatomical variations brought on by the abdominal organs' mirror image, surgery is more complicated and takes longer. We describe a 40-year-old female case who had acute cholecystitis. Laparoscopic cholecystectomy was used to treat this patient, a highly effective procedure for both the treatment and care of these patients. Post-surgical examination and follow-up revealed improvement in the patient's condition without subsequent complications.

Alemtuzumab, Dual Graft-versus-Host Disease Prophylaxis, and Lower CD3+ T Cell Doses Equalize Rates of Acute and Chronic Graft-versus-Host Disease in Pediatric Patients Receiving Allogeneic Hematopoietic Stem Cell Transplantation with Matched Unrelated Donor Peripheral Blood Stem Cells or Bone Marrow Grafts.

Data comparing hematopoietic stem cell transplantation (HSCT) using bone marrow (BM) or peripheral blood stem cell (PBSC) grafts in children after alemtuzumab-based conditioning are lacking. We investigated whether in vivo T cell depletion using alemtuzumab could reduce the risk of severe acute graft-versus-host disease (aGVHD) and chronic GVHD (cGVHD) after HSCT with matched unrelated donor (MUD) BM or PBSCs. This retrospective multicenter study included 397 children (BM group, n = 202; PBSC group, n = 195) who underwent first MUD HSCT at 9 pediatric centers in the United Kingdom between 2015 and 2019. The median age at transplantation was 7.0 years (range, .1 to 19.3 years), and the median duration of follow-up was 3.1 years (range, .3 to 7.5 years). The 3-year overall survival was 81% for the entire cohort (BM group, 80%; PBSC group, 81%). The incidence of grade II-IV aGVHD was significantly higher in the PBSC group (31%) compared to the BM group (31% versus 19%; P = .003), with no difference in the incidence of grade III-IV aGVHD (BM, 7%; PBSC, 12%; P = .17). CD3+ T cell dose >5 × 108/kg and the use of PBSCs were independent predictors of grade II-IV aGVHD. When considering CD3+ T cell dose and GVHD prophylaxis, PBSC transplantation with a calcineurin inhibitor (CNI) and mycophenolate mofetil (MMF) and a CD3+ T cell dose ≤5 × 108/kg had a comparable grade II-IV aGVHD to BM transplantation plus a CNI (20% versus 18%; P = .52). PBSC transplantation was associated with a lower incidence of cGVHD compared to BM transplantation (6% versus 11%; P = .03). Within the limits of this study, we identified a potential strategy to reduce the risk of severe GVHD in pediatric PBSC recipients that includes a combination of in vivo T cell depletion using alemtuzumab and dual GVHD prophylaxis (with a CNI and MMF) and limiting the CD3+ T cell dose to ≤5 × 108/kg.

Valorization of lignocellulosic wastes for sustainable xylanase production from locally isolated Bacillus subtilis exploited for xylooligosaccharides' production with potential antimicrobial activity.

The worldwide availability of lignocellulosic wastes represents a serious environmental challenge with potential opportunities. Xylanases are crucial in lignocellulosic bio-hydrolysis, but the low enzyme productivity and stability are still challenges. In the current study, Bacillus subtilis (coded ARSE2) revealed potent xylanase activity among other local isolates. The enzyme production optimization revealed that maximum enzyme production (490.58 U/mL) was achieved with 1% xylan, 1.4% peptone, and 5% NaCl at 30 °C and pH 9. Furthermore, several lignocellulosic wastes were exploited for sustainable xylanase production, where sugarcane bagasse (16%) under solid-state fermentation and woody sawdust (2%) under submerged fermentation supported the maximum enzyme titer of about 472.03 and 485.7 U/mL, respectively. The partially purified enzyme revealed two protein bands at 42 and 30 kDa. The partially purified enzyme revealed remarkable enzyme activity and stability at 50-60 °C and pH 8-9. The enzyme also revealed significant stability toward tween-80, urea, DTT, and EDTA with Vmax and Km values of 1481.5 U/mL and 0.187 mM, respectively. Additionally, the purified xylanase was applied for xylooligosaccharides production, which revealed significant antimicrobial activity toward Staphylococcus aureus with lower activity against Escherichia coli. Hence, the locally isolated Bacillus subtilis ARSE2 could fulfill the xylanase production requirements in terms of economic production at a high titer with promising enzyme characteristics. Additionally, the resultant xylooligosaccharides revealed a promising antimicrobial potential, which paves the way for other medical applications.

Myeloproliferative Neoplasm Driven by ETV6-ABL1 in an Adolescent with Recent History of Burkitt Leukemia.

ETV6-ABL1 gene fusion is a rare genetic rearrangement in a variety of malignancies, including myeloproliferative neoplasms (MPN), acute lymphoblastic leukemia (ALL), and acute myeloid leukemia (AML). Here, we report the case of a 16-year-old male diagnosed with a MPN, 7 months post-completion of treatment for Burkitt leukaemia. RNA sequencing analysis confirmed the presence of an ETV6-ABL1 fusion transcript, with an intact, in-frame ABL tyrosine-kinase domain. Of note, secondary ETV6-ABL1-rearranged neoplastic diseases have not been reported to date. The patient was started on a tyrosine kinase inhibitor (TKI; imatinib) and, subsequently, underwent a 10/10 matched unrelated haematopoietic stem cell transplant. He is disease-free five years post-transplant. Definitive evidence of the prognostic influence of the ETV6-ABL1 fusion in haematological neoplasms is lacking; however, overall data suggest that it is a poor prognostic factor, particularly in patients with ALL and AML. The presence of this ETV6-ABL1 fusion should be more routinely investigated, especially in patients with a CML-like picture. More routine use of whole-genome and RNA sequencing analyses in clinical diagnostic care, in conjunction with conventional cytogenetics, will facilitate these investigations.

T-cell replete cord transplants give superior outcomes in high-risk and relapsed/refractory pediatric myeloid malignancy.

Stem cell transplant (SCT) outcomes in high-risk and relapsed/refractory (R/R) pediatric acute myeloid leukemia (AML) and myelodysplastic syndrome (MDS) have been historically poor. Cord blood (CB) allows T-cell replete CB transplant (TRCB), enabling enhanced graft-versus-leukemia. We consecutively collected data from 367 patients undergoing TRCB (112 patients) or other cell source (255 patients) SCT for pediatric AML/MDS in the United Kingdom and Ireland between January 2014 and December 2021. Data were collected about the patient's demographics, disease, and its treatment; including previous transplant, measurable residual disease (MRD) status at transplant, human leukocyte antigen-match, relapse, death, graft versus host disease (GvHD), and transplant-related mortality (TRM). Univariable and multivariable analyses were undertaken. There was a higher incidence of poor prognosis features in the TRCB cohort: 51.4% patients were MRD positive at transplant, 46.4% had refractory disease, and 21.4% had relapsed after a previous SCT, compared with 26.1%, 8.6%, and 5.1%, respectively, in the comparator group. Event free survival was 64.1% within the TRCB cohort, 50% in MRD-positive patients, and 79% in MRD-negative patients. To allow for the imbalance in baseline characteristics, a multivariable analysis was performed where the TRCB cohort had significantly improved event free survival, time to relapse, and reduced chronic GvHD, with some evidence of improved overall survival. The effect appeared similar regardless of the MRD status. CB transplant without serotherapy may be the optimal transplant option for children with myeloid malignancy.

Wooden Polymer Composites of Poly(vinyl chloride), Olive Pits Flour, and Precipitated Bio-Calcium Carbonate.

As a filler to be inserted into poly(vinyl chloride) (PVC), low-cost olive pits flour (OPF) and precipitated bio-calcium carbonate (PBCC)-produced PVC/OPF/PBCC composites have been used with high stability and rigidity compared to PVC. Hydrogen bonding is generated between OH cellulose in OPF and H in PVC. Composite tensile modulus increased in PVC grid in the presence of PBCC and OPF, possibly because of a filler restriction effect on the polymer chains. The hardness also increased as both OPF and PBCC increased. The mechanical tendency of the PVC/OPF composite was improved by adding a low content of PBCC particles with the PVC network, resulting in a smart distribution in the range of 10% by weight, and it was reduced by adding more than that percentage. The successful distribution of PBCC in PVC/OPF composite strengthened the mechanical path. The morphology and possible interface adhesion of components in the composite were demonstrated by scanning electron microscopy (SEM). The PVC SEM images showed a homogeneous, smart, and consistent surface, while the PVC/60 wt % OPF SEM images showed a large number of voids that suggested weak PVC/OPF interactions. The SEM images showed outstanding PBCC distribution in the PVC/OPF matrix for the PVC/50 wt % OPF/10 wt % PBCC composite. Due to the accumulation of PBCC particles producing cavities, the distribution of particles became nonhomogeneous at percentages above 10 wt %. At a low filler material, better spread of PBCC particles in the PVC grid was achieved. Owing to the polarity of OPF, the H2O absorption and thickness swelling of PVC/OPF/PBCC composites showed higher amounts than PVC. PBCC improved the thermal stabilization and the neutralization of Cl- negative ions as an acid acceptor of secondary PVC stabilization.

Comparison of the clinical and radiological manifestations of male patients with COVID-19 from different ethnicities.

AIM: The coronavirus disease 2019 (COVID-19) outbreak began in Wuhan, China, and quickly escalated into a significant pandemic threat. COVID-19 is associated with variable morbidity and mortality rates, which differ greatly from one country to another. This study aimed to investigate the clinical findings of SARS-CoV-2 infection in different ethnic groups, as well as to identify the radiological manifestations and various biomarkers for the assessment of COVID-19 patients. MATERIALS AND METHODS: The clinical data of 210 COVID-19 patients with respiratory disorders, who attended the chest clinic at Mouwasat Hospital, Jubail, in the Eastern area of the Kingdom of Saudi Arabia from April to May 2020, were thoroughly reviewed. The patients were divided into seven groups based on their ethnicities (Saudi, Egyptian, Nepali, Filipino, Pakistani, Bangladeshi and Indian). The differences in the clinical findings, laboratory data and radiological manifestations between these groups were statistically analysed. RESULTS: The study included 210 COVID-19 patients from seven ethnic groups (Saudi, Egyptian, Nepali, Filipino, Pakistani, Bangladeshi and Indian). Comorbidities were reported among 60.9% of patients, which were significantly higher among Filipinos at 73.3%. Dyspnoea was prevalent in the Saudi and Pakistani groups, while hypoxaemia was prevalent in the Indian group (40%). In terms of laboratory assessment, Bangladesh patients had the highest median of serum ferritin and lactate dehydrogenase (LDH) levels with a significant P value (<.001), while Saudi patients had the highest median of C-reactive protein (CRP) levels with a significant P value (<.001). According to computed tomography (CT) findings, structural destruction was the most common finding in bilateral parenchymal affection among 88.6% of patients. Filipinos and Bangladeshis had the highest morbidity rates. CONCLUSION: There were great variations in clinical, radiological and even laboratory findings among different ethnic groups of COVID-19 patients.

Automatic segmentation for synchrotron-based imaging of porous bread dough using deep learning approach.

In recent years, major capability improvements at synchrotron beamlines have given researchers the ability to capture more complex structures at a higher resolution within a very short time. This opens up the possibility of studying dynamic processes and observing resulting structural changes over time. However, such studies can create a huge quantity of 3D image data, which presents a major challenge for segmentation and analysis. Here tomography experiments at the Australian synchrotron source are examined, which were used to study bread dough formulations during rising and baking, resulting in over 460 individual 3D datasets. The current pipeline for segmentation and analysis involves semi-automated methods using commercial software that require a large amount of user input. This paper focuses on exploring machine learning methods to automate this process. The main challenge to be faced is in generating adequate training datasets to train the machine learning model. Creating training data by manually segmenting real images is very labour-intensive, so instead methods of automatically creating synthetic training datasets which have the same attributes of the original images have been tested. The generated synthetic images are used to train a U-Net model, which is then used to segment the original bread dough images. The trained U-Net outperformed the previously used segmentation techniques while taking less manual effort. This automated model for data segmentation would alleviate the time-consuming aspects of experimental workflow and would open the door to perform 4D characterization experiments with smaller time steps.

Neuroinflammatory Disease as an Isolated Manifestation of Hemophagocytic Lymphohistiocytosis.

Isolated neuroinflammatory disease has been described in case reports of familial hemophagocytic lymphohistiocytosis (FHL), but the clinical spectrum of disease manifestations, response to therapy and prognosis remain poorly defined. We combined an international survey with a literature search to identify FHL patients with (i) initial presentation with isolated neurological symptoms; (ii) absence of cytopenia and splenomegaly at presentation; and (iii) systemic HLH features no earlier than 3 months after neurological presentation. Thirty-eight (20 unreported) patients were identified with initial diagnoses including acute demyelinating encephalopathy, leukoencephalopathy, CNS vasculitis, multiple sclerosis, and encephalitis. Median age at presentation was 6.5 years, most commonly with ataxia/gait disturbance (75%) and seizures (53%). Diffuse multifocal white matter changes (79%) and cerebellar involvement (61%) were common MRI findings. CSF cell count and protein were increased in 22/29 and 15/29 patients, respectively. Fourteen patients progressed to systemic inflammatory disease fulfilling HLH-2004 criteria at a mean of 36.9 months after initial neurological presentation. Mutations were detected in PRF1 in 23 patients (61%), RAB27A in 10 (26%), UNC13D in 3 (8%), LYST in 1 (3%), and STXBP2 in 1 (3%) with a mean interval to diagnosis of 28.3 months. Among 19 patients who underwent HSCT, 11 neurologically improved, 4 were stable, one relapsed, and 3 died. Among 14 non-transplanted patients, only 3 improved or had stable disease, one relapsed, and 10 died. Isolated CNS-HLH is a rare and often overlooked cause of inflammatory brain disease. HLH-directed therapy followed by HSCT seems to improve survival and outcome.

Androgen therapy in inherited bone marrow failure syndromes: analysis from the Canadian Inherited Marrow Failure Registry.

Progressive cytopenia is a serious complication among paediatric patients with inherited bone marrow failure syndromes (IBMFS). Androgens have been used to improve blood counts in different bone marrow failure conditions. Little is known about efficacy and toxicity with new androgens (i.e., danazol) in different types of IBMFS. We identified 29 patients from the Canadian Inherited Marrow Failure Registry, who received oxymetholone or danazol. Sixteen (55%) had haematological response including patients with unclassified IBMFS (45%). Danazol showed a better toxicity profile and similar efficacy compared to oxymetholone. Androgens are an effective and safe option to ameliorate bone marrow failure in IBMFS.

Primary immunodeficiencies and their associated risk of malignancies in children: an overview.

Primary immunodeficiency disorders represent a heterogeneous spectrum of diseases, predisposing to recurrent infections, allergy, and autoimmunity. While an association between primary immunodeficiency disorders and increased risk of cancer has been suggested since the 1970s, renewed attention has been given to this topic in the last decade, largely in light of the availability of large registries as well as advances in next generation sequencing. In this narrative review, we will give an insight of the primary immunodeficiencies that are commonly responsible for the greater number of cancers in the primary immunodeficiency disorders population. We will describe clinical presentations, underlying genetic lesions (if known), molecular mechanisms for carcinogenesis, as well as some management considerations. We will also comment on the future directions and challenges related to this topic.Conclusion: The awareness of the association between several primary immunodeficiencies and cancer is crucial to provide the best care for these patients.What is Known: • Patients with primary immunodeficiency have an increased risk of malignancy. The type of malignancy is highly dependent on the specific primary immunodeficiency disorder.What is New: • Survival in patients with primary immunodeficiency disorders has been improving, and conversely also their lifetime risk of malignancy. • International collaboration and multinational registries are needed to improve our knowledge and therapeutic strategies.

Effect of different conditioning regimens on survival and engraftment for children with hemophagocytic lymphohistiocytosis undergoing allogeneic hematopoeitic stem cell transplantation: A single institution experience.

BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH), a rare hyperinflammatory immuneregulatory disorder, is a challenge in hematopoietic stem cell transplantation (HSCT) because of the high rate of mixed chimerism, relapse, and graft failure (GF) unless intensive myeloablative regimens are used. However, historically conventional myeloablative regimens (conv MA) are associated with high toxicity and mortality. PROCEDURE: We retrospectively compared transplant outcomes between three preparative regimens of varying intensities: Conv MA (n = 15), reduced-intensity conditioning (RIC, n = 12), and a treosulfan-based reduced-toxicity conditioning (RTC, n = 9). RESULTS: Patients in the RIC cohort had a higher incidence of mixed donor chimerism and five patients (42%) developed secondary GF (P = .002) compared to the other two regimens. There was a higher incidence of veno-occlusive disease and intensive care unit (ICU) admissions in the Conv MA cohort. With the RTC regimen, there was a similar 2-year overall survival (89, 73, and 83%; P = .87), but improved compound EFS (lack of relapse, GF, second transplant or additional donor cell infusions, or death; 89, 73, and 42%, P = .041) in RTC, Conv MA, and RIC regimen, respectively. CONCLUSIONS: The intensity of the preparative regimen has a significant impact on outcome of HSCT for HLH. The newly described treosulfan-based RTC provides for a stable graft with a reasonable toxicity profile.

[A CHILD IS LOST: THE PHENOMENON OF REFUSING TO BE HOSPITALIZED IN FAMILIES OF CHILDREN REFERRED TO THE DEPARTMENT OF EMERGENCY MEDICINE].

BACKGROUND: Discharge against medical advice (DAMA) in pediatrics may endanger the child, increase the rate of complications, morbidity or mortality. Despite the prevalence of this phenomenon in the world, we found only one study that examined the DAMA phenomenon in Israel. The study examined the phenomenon in one ER in general and did not distinguish between adults and children. OBJECTIVES: To describe the characteristics of children and parents who left the ER against medical advice for hospitalization and to examine the reasons given by the parents, and factors associated with this phenomenon. METHODS: A prospective study involving parents who refused to hospitalize their children despite a medical recommendation. Data was collected from medical records and telephone interviews after discharge. RESULTS: During the study, there were 16,376 visits to the pediatric ER, 3288 recommendations for hospitalization (20.07%) and 200 DAMA (6%). Reasons for parents refusing hospitalization can be categorized according to: child's health reasons, parents personal reasons and reasons related to the health system`s function. A total of 22 of the children returned to the ER for the same complaint and 12 of them were hospitalized (7.9% of the children who completed their participation in the study). DISCUSSION: Refusal of medical care for children is a disturbing phenomenon due to the negative consequences that may result from this. In order to minimize the extent of DAMA and its damage, it is very important to recognize the extent and understand the factors associated with this phenomenon.

Lymphocyte subset at time of Epstein-Barr viremia post-allogeneic hematopoietic stem cell transplantation in children may predict development of post-transplant lymphoproliferative disease: CD8:CD20 ratio as a sensitive predictor.

EBV-associated PTLD following allogeneic HSCT is a serious complication associated with significant mortality. In this retrospective study, we evaluated whether lymphocyte subset numbers and CD8:CD20 ratio at time of EBV viremia in children undergoing allogeneic HSCT could predict development of PTLD. Absolute lymphocyte count, lymphocyte subsets, and CD8:CD20 ratio at the time of EBV viremia were analyzed. Patients who were treated preemptively with rituximab for high blood EBV viral load were excluded. Out of 266 patients transplanted during the study period, 26 patients were included in the analysis. Patients were divided into two cohorts; cohort 1 included patients with EBV-associated PTLD (n = 5; four with proven, one with probable PTLD). Cohort 2 included patients with EBV viremia without PTLD (n = 21). Lymphocyte recovery was slower in the PTLD group. CD8:CD20 ratio was significantly lower in the PTLD group (median 0.15) compared to the non-PTLD group (median 2.4, P = .012). Using the ROC curve and 1 as the cutoff value, CD8:CD20 ratios were analyzed. In the PTLD group, 4/5 patients (80%) had a ratio <1 whereas in the non-PTLD group, all 21 patients had a ratio >1. Sensitivity and specificity were 80% and 100%, respectively. Negative and PPVs were 95% and 100%, respectively. Profoundly low T-cell count and CD8:CD20 ratio may be used to predict development of PTLD in the context of EBV viremia in children post-allogeneic HSCT. Further studies are needed to validate this finding.

Comparison between intermittent and continuous leukapheresis protocols for autologous hematopoietic stem cell collections in children.

BACKGROUND: Peripheral hematopoietic stem cell (HSC) collections are needed for autologous hematopoietic stem cell transplantation (HSCT). Since 2015, our institution has utilized a secondary chamber mononuclear cell (MNC) protocol on the Spectra Optia apheresis system. Recently, a new continuous mononuclear collection protocol (CMNC) was developed for the same device. As there is limited data available regarding the use of the CMNC protocol in children, we compared collection efficiency (CE2), side effects, and clinical feasibility between the two protocols in patients <18 years old. STUDY DESIGN AND METHODS: We prospectively collected clinical, laboratory, and technical collection data from HSC collection procedures performed with the Spectra Optia apheresis system utilizing the CMNC protocol. Data were compared to retrospectively collected data utilizing the MNC protocol. Data collection included donor demographics, precollection peripheral CD34+ cell counts, total CD34+ cells collected, collection efficiency, side effects, and collection product characteristics. RESULTS: A total of 96 HSC collection procedures were performed on 79 pediatric patients utilizing either the MNC (61 patients) or CMNC (18 patients) protocol. The collection efficiencies were comparable between MNC and CMNC cohorts (52.9% vs 54.9%, P = 0.711). Platelet loss was significantly lower in the CMNC cohort (P = 0.002), especially in children weighing <15 kg. Product volumes were higher with CMNC. No significant collection-related side effects were noted with either protocol. CONCLUSIONS: MNC and CMNC protocols have comparable collection efficiencies and are both feasible and safe for the use in children. Centers may choose between the methods depending on clinical needs.

The yield of monitoring adenovirus in pediatric hematopoietic stem cell transplant patients.

Human adenovirus (HAdV) is recognized as a serious pathogen after allogeneic hematopoietic stem cell transplantation (HSCT), causing morbidity and mortality. Currently, there is no universal agreement regarding routine HAdV surveillance after HSCT. We assessed the impact of HAdV weekly monitoring by polymerase chain reaction (PCR) on HAdV viremia rates and the risk factors that influence survival. Three-hundred and fifty-six pediatric allogeneic HSCT were done between 2007 and 2015. Until July 2011, HAdV testing was performed based on clinical suspicion (cohort 1, n = 175) and from August 2011, weekly blood-HAdV monitoring was done (cohort 2, n = 181) until day +100. Twenty-three patients (4 [2.3%] from cohort 1 and 19 [10.5%] from cohort 2, p = .001) were found with HAdV viremia and seven of them died. Both cohorts had a similar incidence of HAdV-associated mortality (3/175; 1.7% in cohort 1 and 4/181; 2.2% in cohort 2). Respiratory failure was the cause of death in all patients. Clinical symptoms appeared prior to or within 5 days of HAdV detection in cohort 2. In summary, weekly monitoring was associated with higher detection of HAdV. The study could not assess survival benefit due to small numbers of HAdV-positive cases. In many instances, symptoms occurred with the development of positive HAdV blood PCR results and hence, symptomatology could have triggered the test. Future studies are needed to provide data that help establishing a uniform approach for regular monitoring of HAdV post-transplant.

Perianal Infections in Children With Acute Myeloid Leukemia: A Report From the Canadian Infection in Acute Myeloid Leukemia Research Group.

Among 235 children with acute myeloid leukemia, 17 experienced 19 perianal infections. Among 12 episodes with definite abscess, 75% were severely neutropenic. Sixteen diagnostic imaging evaluations were performed; diagnostic yield was similar between computerized tomography of pelvis (5 of 10) and ultrasound (3 of 5). Consistent management approaches to perianal infection should be developed.

Hemophagocytic lymphohistiocytosis is a sign of poor outcome in pediatric Epstein-Barr virus-associated post-transplant lymphoproliferative disease after allogeneic hematopoietic stem cell transplantation.

EBV-related PTLD developing after HSCT is a potentially life-threatening disease. HLH is uncommon after allogeneic HSCT. Data on outcome of patients with PTLD and concomitant HLH after allogeneic HSCT are limited. In this retrospective study, we collected demographic, clinical, laboratory, and outcome data for 408 patients who underwent allogeneic HSCT from 2006 to 2015. Graft source included CB (n = 135; 33.1%), PBSCs (n = 34; 8.3%), and BM (n = 239; 58.6%). Eight out of 408 patients (2%) developed EBV-PTLD with a median age at HSCT of 5.9 years (range: 2.3-17.3). All eight patients received ATG as part of the conditioning regimen. Graft source was PBSC in three patients (37.5%), BM in four patients (50%), and CB in one patient (12.5%). Donors were matched unrelated in five patients (62.5%) and matched sibling in three patients (37.5%). Seven out of eight patients developed EBV-PTLD within the first 100-day post-HSCT. Lymph node biopsy revealed early lesions in three patients, polymorphic in three patients, and monomorphic PTLD in two patients. Three patients (37.5%) died within 1 month of EBV-PTLD diagnosis. All deceased patients developed HLH manifestations with two of them meeting HLH diagnostic criteria and one having an incomplete workup. PTLD after allogeneic HSCT with manifestations of HLH is associated with high mortality. Early identification and treatment of EBV-PTLD seems imperative to control the disease, especially if signs of HLH are evolving.

Incidental extraspinal findings in the lumbar spine during magnetic resonance imaging of intervertebral discs.

PURPOSE: To calculate the frequencies of incidental extraspinal findings and incidentally detected congenital anomalies or anatomical differences in the lumbar spine on magnetic resonance imaging (MRI) scans of intervertebral discs. MATERIALS AND METHODS: A total of 379 lumbar spine MRI cases were prospectively investigated in the period spanning from August 2016 to January 2018. Both 1.5 and 0.35 Tesla MRI units (Toshiba and Siemens Medical Systems) were used to examine patients with clinically suspected intervertebral disc abnormalities at three MRI diagnostic centers in Khartoum State, Sudan. RESULTS: Of the 379(100%) patients, 90(23.7%) patients were presented with incidental findings. Among the incidental findings, 39(10.3%) were renal cysts, 10(2.6%) were retroverted uteri, 5(1.3%) were Nabothian cysts, 4(1.1%) were ovarian cysts, 10(2.6%) were uterine fibroids, 3(0.8%) were endometrial thickening, 11(2.9%) were indicative of hydronephrosis, 4(1.1%) were uncovered prostatic enlargement, 2(0.5%) were atrophic kidney, and 1(0.3%) each was of an ectopic kidney and bladder wall thickening, respectively. CONCLUSIONS: A high percentage of extraspinal pathological findings were detected during MRI lumbar spine scans of intervertebral discs. Thus, it is important to be aware of the high percentage of patients who undergo further evaluation given the presence of unexpected findings, but for whom clinical confirmation of these abnormalities is not obtained.

Adenovirus Infection in Children With Acute Myeloid Leukemia: A Report From the Canadian Infection in Acute Myeloid Leukemia Research Group.

BACKGROUND: Children with acute myeloid leukemia (AML) are at high risk of life-threatening bacterial and fungal infection. However, little is known about the prevalence or severity of adenovirus infection in this population. Objective was to describe the characteristics, treatments and outcomes of adenovirus infection in children with newly diagnosed AML. METHODS: We performed a retrospective chart review based upon 2 multicenter cohort studies that focused on identifying risk factors for infection in children with AML. Inclusion criteria were patients with de novo AML who were ≤18 years of age at diagnosis with a clinical specimen positive for adenovirus. RESULTS: Among the 235 patients with AML, 12 (5.1%) had positive adenovirus testing. The most common site of isolation was stool (n = 11, 91.6 %), and the most frequent symptom was diarrhea (n = 11, 91.6 %). Two patients received specific treatment for adenovirus, namely intravenous immunoglobulin only in 1 patient and both intravenous immunoglobulin and inhaled ribavirin in a second patient. In 11 patients, adenovirus resolved uneventfully without recurrence, including 10 that received no adenovirus-specific therapy. However, 1 patient developed sepsis syndrome in the setting of disseminated adenoviral infection and died from multiorgan failure. CONCLUSION: In children with AML, adenovirus infection was rare and typically not associated with severe disease, even without specific treatment. However, disseminated and fatal disease can occur in this population. Further investigations are needed to identify pediatric AML patients at particular risk for severe adenovirus infection and to determine optimal treatment approaches in these patients.