Revisiting the benefits of isovolemic hemodilution red cell exchange for sickle cell disease.

BACKGROUND: Isovolemic hemodilution red cell exchange (IHD-RCE) is a modified form of the standard red cell exchange (STD-RCE), intended to reduce red cell requirements in patients with sickle cell disease (SCD). This retrospective crossover analysis of nine patients aims to add to the limited existing literature on IHD-RCE and address the equipoise regarding whether the benefits of (a) decreased RBC usage per exchange and (b) increased interprocedure interval (via lower fraction of cells remaining, FCR) can be observed at the same time, in the same patient. METHODS: At a single center, we identified 37 patients with SCD undergoing chronic RCE between 2014 and 2021. We excluded those patients who did not have at least six consecutive procedures of each type (STD- and IHD-RCE), arriving at nine patients for analysis. RESULTS: When using greater decreases in hematocrit than previously published, we did not find that IHD-RCE resulted in any clinically apparent adverse events. We did find greater decreases in diastolic blood pressure and increases in heart rate in some patients, as compared to STD-RCE. After correcting for total blood volume, seven of the nine patients had significantly reduced red cell requirements with each IHD-RCE. Because the pattern of achieving a lower FCR than programmed was seen to an equal extent with both IHD-RCE and STD-RCE, none of the nine patients showed any statistical difference in actual FCR between procedure types. DISCUSSION: Our data do not support the observation of both IHD-RCE benefits, decreased red cell usage per exchange and lower FCR/increased interprocedure interval, simultaneously.

Congenital Malignant Rhabdoid Tumor of Neck.

Malignant rhabdoid tumors (MRT) are uncommon, highly aggressive tumors arising usually from the central nervous system and kidneys. Nonrenal and noncentral nervous systems MRT are rare in neonates. To the best of our knowledge, only five cases of congenital MRT of neck have been described till date. We present a rare case of congenital MRT of the neck in a neonate along with review of literature.

Abdominal Wall Yolk Sac Tumor in a Child.

Midline vascular abdominal wall lesions are likely to be mistaken for vascular malformations in young children. We report a case of large yolk sac tumor located in the anterior abdominal wall just below xiphisternum in a 20-month-old girl diagnosed by raised serum alpha fetoprotein levels and fine-needle aspiration cytology. Preoperative chemotherapy helped in reducing its size allowing wide resection and primary wound closure. This case is reported for the unusual location and role of chemotherapy in management.

Retrospective analysis of bleeding events after central venous catheter placement in thrombotic thrombocytopenic purpura.

BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is a thrombotic disorder caused by severe deficiency of ADAMTS13. Platelets are transfused prophylactically in non-TTP patients for central venous catheter (CVC) with a count <20 × 109/L to prevent bleeding. However, transfusing platelets in TTP prior to CVC placement remains controversial due to concern for arterial thrombosis and mortality. At our center, platelet transfusion is contraindicated in TTP, therefore, we analyzed data for bleeding complications following CVC placement. STUDY DESIGN AND METHODS: 95 acute episodes of TTP were identified. Twenty-six episodes were excluded for insufficient documentation or no CVC placement. The charts of 69 remaining episodes were reviewed. RESULTS: Of 69 TTP episodes, nine (13 %) had bleeding after a CVC placement. Of these, seven bleeds were minor, and the two were major related to the technical issues during femoral venous access causing arterial bleeds. Median platelet count before the CVC placement among those experiencing bleeding complications was 12 × 109/L (range 3-44) as compared to median count of 15 × 109/L (range 4-257) in those who did not bleed (p = 0.258). Among 44 episodes with a platelet count <20 × 109/L, seven (16 %) had bleeds. CONCLUSION: Major bleeding complications following CVC placement in TTP is uncommon and most likely related to technical challenges. Median platelet count was similar in patients who bled versus those who did not, suggesting that platelet transfusion is unnecessary to correct platelet count prior to a CVC placement in TTP.

Extrapulmonary Pleuropulmonary Blastoma in a 3-Year-Old Child: A Case Report and Review of Literature.

Pleuropulmonary blastomas (PPBs) are very rare, highly aggressive, dysembryonic neoplasms of thoracopulmonary mesenchyme. These have been reported in the pediatric population and account for only 0.5%-1% of all primary malignant lung cancers. They normally arise from lung tissue, however rarely the parietal pleura may be the tissue of origin (extra pulmonary PPB) which are extremely rare. Common age of presentation is three to 4 years. The prognosis is poor with distant metastasis to central nervous system and bone with survival rate of approximately 42.9% at 5 years. They are managed by aggressive multimodal therapies including surgery and chemotherapy. We report a case of a 3-year-old male child with Type 2 PPB of the left hemithorax, managed by surgical excision of the mass and adjuvant chemotherapy.

Comparison of low fixed dose versus standard-dose rituximab to treat thrombotic thrombocytopenic purpura in the acute phase and preemptively during remission.

The standard dose of rituximab used in B-cell hematological malignancies, 375 mg/m2 weekly, may be excessive for autoimmune conditions. Successful use of a low, fixed dose of 100-200 mg of rituximab, weekly for 4 weeks, has been reported in the literature in the treatment of autoimmune thrombotic thrombocytopenic purpura (aTTP). We retrospectively analyzed our rituximab data in aTTP over a 13-year-period for 39 patients, with the aim of comparing response and outcomes with a standard lymphoma-dose course versus a low fixed 100 mg-dose course. Compared to the standard dose (17 patients, 17 courses of 4 infusions), our patients who received a low dose (8 patients, 9 courses of 4 infusions) had a possibly lower baseline risk but did achieve a similar time to remission and number of plasma exchange procedures to remission. Preemptive low-dose courses for ADAMTS13 activity <50 % during remission (6 patients, 10 courses of 4 infusions) achieved a median peak ADAMTS13 activity of 99 %, in a median of 1 month, with no clinical relapses. Our results provide additional evidence for the efficacy of low-dose rituximab, with the benefit of much lower cost, less infusion time, and theoretically lower risk of toxicity.

Methadone continuation versus forced withdrawal on incarceration in a combined US prison and jail: a randomised, open-label trial.

BACKGROUND: Methadone is an effective treatment for opioid dependence. When people who are receiving methadone maintenance treatment for opioid dependence are incarcerated in prison or jail, most US correctional facilities discontinue their methadone treatment, either gradually, or more often, abruptly. This discontinuation can cause uncomfortable symptoms of withdrawal and renders prisoners susceptible to relapse and overdose on release. We aimed to study the effect of forced withdrawal from methadone upon incarceration on individuals' risk behaviours and engagement with post-release treatment programmes. METHODS: In this randomised, open-label trial, we randomly assigned (1:1) inmates of the Rhode Island Department of Corrections (RI, USA) who were enrolled in a methadone maintenance-treatment programme in the community at the time of arrest and wanted to remain on methadone treatment during incarceration and on release, to either continuation of their methadone treatment or to usual care--forced tapered withdrawal from methadone. Participants could be included in the study only if their incarceration would be more than 1 week but less than 6 months. We did the random assignments with a computer-generated random permutation, and urn randomisation procedures to stratify participants by sex and race. Participants in the continued-methadone group were maintained on their methadone dose at the time of their incarceration (with dose adjustments as clinically indicated). Patients in the forced-withdrawal group followed the institution's standard withdrawal protocol of receiving methadone for 1 week at the dose at the time of their incarceration, then a tapered withdrawal regimen (for those on a starting dose >100 mg, the dose was reduced by 5 mg per day to 100 mg, then reduced by 3 mg per day to 0 mg; for those on a starting dose >100 mg, the dose was reduced by 3 mg per day to 0 mg). The main outcomes were engagement with a methadone maintenance-treatment clinic after release from incarceration and time to engagement with methadone maintenance treatment, by intention-to-treat and as-treated analyses, which we established in a follow-up interview with the participants at 1 month after their release from incarceration. Our study paid for 10 weeks of methadone treatment after release if participants needed financial help. This trial is registered with ClinicalTrials.gov, number NCT01874964. FINDINGS: Between June 14, 2011, and April 3, 2013, we randomly assigned 283 prisoners to our study, 142 to continued methadone treatment, and 141 to forced withdrawal from methadone. Of these, 60 were excluded because they did not fit the eligibility criteria, leaving 114 in the continued-methadone group and 109 in the forced-withdrawal group (usual care). Participants assigned to continued methadone were more than twice as likely than forced-withdrawal participants to return to a community methadone clinic within 1 month of release (106 [96%] of 110 in the continued-methadone group compared with 68 [78%] of 87 in the forced-withdrawal group; adjusted hazard ratio [HR] 2·04, 95% CI 1·48-2·80). We noted no differences in serious adverse events between groups. For the continued-methadone and forced-withdrawal groups, the number of deaths were one and zero, non-fatal overdoses were one and two, admissions to hospital were one and four; and emergency-room visits were 11 and 16, respectively. INTERPRETATION: Although our study had several limitations--eg, it only included participants incarcerated for fewer than 6 months, we showed that forced withdrawal from methadone on incarceration reduced the likelihood of prisoners re-engaging in methadone maintenance after their release. Continuation of methadone maintenance during incarceration could contribute to greater treatment engagement after release, which could in turn reduce the risk of death from overdose and risk behaviours. FUNDING: National Institute on Drug Abuse and the Lifespan/Tufts/Brown Center for AIDS Research from the National Institutes of Health.

Ischemic stroke in a patient with moderate to severe inherited factor VII deficiency.

Thrombosis is known to occur in patients with rare inherited bleeding disorders, usually in the presence of a thrombotic risk factor such as surgery and/or factor replacement therapy, but sometimes spontaneously. We present the case of a 72-year-old African American male diagnosed with congenital factor VII (FVII) deficiency after presenting with ischemic stroke, presumably embolic, in the setting of atherosclerotic carotid artery stenosis. The patient had an international normalized ratio (INR) of 2.0 at presentation, with FVII activity of 6% and normal Extem clotting time in rotational thromboelastometry. He was treated with aspirin (325 mg daily) and clopidogrel (75 mg daily) with no additional bleeding or thrombotic complications throughout his admission. This case provides further evidence that moderate to severe FVII deficiency does not protect against thrombosis.

Making the invisible, visible: a cross-sectional study of late presentation to HIV/AIDS services among men who have sex with men from a large urban center of Brazil.

BACKGROUND: Late presentation to testing, treatment and continued care has detrimental impacts on the health of HIV-positive individuals as well as their sexual partners' health. Men who have sex with men (MSM) experience disproportionately high rates of HIV both globally and in Brazil. However, the factors that inhibit linkage to care among MSM remain unclear. METHODS: We conducted a cross-sectional study of HIV-positive MSM (n = 740) enrolled in HIV/AIDS services in a large urban center of Brazil from August 2010 to June 2011. Descriptive, bivariate and multivariate statistics were conducted using STATA 12 to examine the relationship between a range of variables and late presentation, defined as having a first CD4 count <350 cells/mm3. RESULTS: Within the sample, the prevalence of LP was 63.1%. Men who self-identified as heterosexual (AOR 1.54 and 95% CI 1.08 - 2.20) compared to men who self-identified as homosexual and bisexual were at increased odds of late presentation. Additionally, men age 30 and older (AOR 1.56, 95% CI 1.01 - 2.43) compared to individuals age 18-29 experienced increased odds of late presentation among MSM. CONCLUSIONS: The prevalence of LP in this population was higher than noted in the global literature on LP among MSM. Heterosexual men and older age individuals experienced substantial barriers to HIV care. The stigma around same-sex behaviors and the current focus of HIV prevention and treatment campaigns on younger age individuals may limit patients' and providers' awareness of the risk for HIV and access to available services. In addition to addressing HIV-specific barriers to care, developing effective strategies to reduce late presentation in Brazil will require addressing social factors - such as stigma against diverse sexualities - to concretely identify and eliminate barriers to available services. Only in so doing can we make currently invisible people, visible.

Linking D-Dimer and haematological parameters among Indian COVID 19 patients.

The severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) outbreak continues to place a significant strain on healthcare systems, economies, and patient management. Therefore, it is of interest to evaluate the role of D-Dimer and haematological parameters to identify severity and outcome of COVID 19 patients. Total 100 cases diagnosed with COVID 19 were recruited in the study and followed up for 6 months. The subjects were grouped into 2, Group 1: Newly Diagnosed COVID 19 Patients and Group 2: After 6 months of follow up COVID 19 Patients. We analyzed Hb, RBCs, WBCs, PT, APTT and D-Dimer and also, we taken CT values of the study subjects. A statistical analysis was done by using SPSS version 20.0. The WBCs and haemoglobin mean values are shown significant values between the study subjects, respectively with p-values < 0.001**. The PT and APTT significantly increased in newly diagnosed COVID 19 patients when compared to after 6 months of follow up at p-value < 0.001**. There was a positive correlation of WBCs, PT, APTT (r= 0.458, 526, 509) with D-Dimer and negatively correlated RBCS, Hb, CT (-0.056, 321, 526, 353), respectively at p < 0.001**. Thus, low platelet, high d-dimer, and fibrinogen may serve as risk markers for the progression of COVID-19 severity. Hence, COVID-19 patients may experience anaemia-related consequences as hypoxia, coronary and pulmonary failure due to low Hb concentration. Further, patients with COVID-19 also experience bleeding issues due to thrombocytopenia.

Association between intraoperative anatomical variation and histopathological parameters in cases of ureteropelvic junction obstruction in children: A cross-sectional study.

Introduction: The intraoperative anatomical findings (IOAF) of all ureteropelvic junction obstruction (UPJO) cases are not identical. Moreover, there is also controversy in the literature regarding histopathological (HP) findings in cases of UPJO. In the present study, we evaluated different IOAF and assessed their association with specific HP parameters. Materials and Methods: This was a cross-sectional study set-up, which was carried out in a tertiary care centre. Children with UPJO who underwent surgery between 2017 and 2020 were enrolled. The following IOAF were noted: Type of pelvis (extrarenal or intrarenal), insertion of the ureter (high or normal), presence of lower pole crossing vessel (CV), negotiation of UPJ segment with double J stent (3 Fr) and length of internal narrowing (LIN) at UPJ. The resected segment of UPJ was assessed at three levels (pelvis, UPJ and ureter) for various HP parameters including fibrosis, oedema, inflammation and smooth muscle hypertrophy (SMH). Results: Thirty-nine children were included in the study with a mean age of 31 months. The summary statistics of IOAF were intrarenal pelvis in 5 cases, high insertion of the ureter (HIU) in 9, CV in 6, negotiable UPJ in 23, and 16 cases showed LIN >1 cm. All cases showed SMH at the pelvis region and SMH with fibrosis at the UPJ region. At the pelvis region, there was an association between (1) HIU with oedema and chronic inflammation (CIF), (2) CV with CIF and (3) LIN with CIF and SMH. At the UPJ region, there was an association between (1) CV and negotiable UPJ with less fibrosis and (2) LIN with SMH. At the ureteric end, CV showed an association with less fibrosis and more CIF. Conclusion: All UPJO cases have some common HP findings. Although, some particular IOAF, i.e., presence of CV, negotiable UPJ, HIU and LIN showed association with specific HP parameters.

Autonomic dysfunction in idiopathic hypersomnia: an overlooked association and potential management.

There is a small yet robust body of literature regarding autonomic dysfunction in idiopathic hypersomnia as well as sleep disturbances in postural orthostatic tachycardia syndrome. This review aims at summarizing the current literature and highlighting gaps in the current knowledge. This article additionally presents the personal experience of one of the authors at the sleep center. CITATION: Adra N, Reddy M, Attarian H, Sahni AS. Autonomic dysfunction in idiopathic hypersomnia: an overlooked association and potential management. J Clin Sleep Med. 2022;18(3):963-965.

Anorectal Malformations: The Earlier the Diagnosis, the Better the Outcome.

OBJECTIVE: To estimate the impact of delayed presentation of anorectal malformation (ARM) in neonates and to compare the presenting characteristics and outcomes of early versus delayed presentation. METHODS: This is a prospective observational study of all neonates (age < 28 d) with ARM over 2 y. Delayed presentation was defined as presentation beyond 48 h of birth. Various presenting features and their early postoperative outcomes were compared. RESULTS: Nearly half (26, 48%) of the 54 neonates with ARM had delayed presentation. Early and late presenters did not differ in terms of gender, gestational age, birth weight, place of delivery, and type of ARM (p > 0.05 for all). Delayed group had lower weight at presentation (p = 0.008), higher incidence of severe abdominal distension (p = 0.05), and sepsis (p = 0.171) and required longer time for resuscitation (p = 0.007) and more inotropes (p = 0.015), preoperatively. Early postoperative outcomes including time for stoma to function, initiate feeds and time to reach full feeds were significantly delayed in late presenters. They also had more wound infections, longer hospital stay and higher mortality. CONCLUSIONS: Delayed diagnosis of ARM is associated with significantly higher morbidity and mortality. Adequate awareness and training of health workers for early identification of ARM by careful perineal examination of all newborns at birth is the need of the hour.

Adequate indoor air quality in nursing homes: An unmet medical need.

A small but growing body of literature indicates that concentrations of indoor particulate and gaseous pollutants in long-term care facilities (i.e., skilled nursing facilities) for older adults, hereafter referred to nursing homes, often exceed those recorded in nearby, comparable outdoor environments. Unlike the outdoors, indoor air quality (IAQ) in nursing homes is not regulated by legislation and is seldom monitored. To that end, residents of nursing homes commonly spend the vast majority of their time indoors where they are exposed to indoor air pollutants for long periods of time. Given that many nursing home residents, especially those of advanced age, are more susceptible to the effects of air pollutants, even at low concentrations, this prolonged exposure may adversely affect their health, well-being, quality of life and increase medical expenditures due to frequent, unscheduled acute care visits and hospitalizations. We propose an action plan for assessing IAQ in nursing homes, understanding the impacts of IAQ on adverse health outcomes of nursing home residents, and addressing vulnerabilities in these facilities to safeguard health, well-being, and quality of life of nursing home residents and minimizing unscheduled acute care visits and hospitalizations. We propose that IAQ should be regularly monitored in nursing homes to proactively identify and address vulnerabilities in these facilities and that resources should be provided for remedial interventions to improve IAQ in nursing homes, including but not limited to source control, improving ventilation and filtration, and deploying air cleaners where appropriate. This proactive approach may pave the way for establishing enforceable standards for indoor air quality in nursing homes that will promote health, well-being, and quality of life of nursing home residents and reduce medical expenditures.

Cerebral Air Embolism as Possible Cause of Stroke During Therapeutic Endobronchial Application of Argon Plasma Coagulation.

A 68-year-old male was admitted for evaluation of an endobronchial mass obstructing the right middle lobe (RML) and right lower lobe (RLL) of the lung. Flexible bronchoscopy revealed a notable endobronchial lesion in the bronchus intermedius that completely obstructed the RML and the RLL. Argon plasma coagulation (APC) at 30 watts and gas flow at 0.8 liters/minute to 1 liter/minute were applied to the tumor. In the recovery room, the patient was discovered to have a left-sided facial droop and left-sided weakness. The initial computed tomography (CT) scan of the brain and an angiogram of the head and neck were normal, but a repeat CT scan of the head several hours later was remarkable for an area of hypoattenuation in the right frontoparietal lobe concerning for infarct. A magnetic resonance imaging (MRI) brain scan confirmed acute to sub-acute cortical infarcts. Given the direct temporal relation between the onset of neurologic symptoms and the usage of APC with bronchoscopy, a cerebral air embolism (CAE) was thought to be the cause of the patient's acute stroke.

Late presentation to HIV/AIDS care in Brazil among men who self-identify as heterosexual.

OBJECTIVE: To analyze the factors associated with late presentation to HIV/AIDS services among heterosexual men. METHODS: Men infected by HIV who self-identified as heterosexual (n = 543) were included in the study. Descriptive, biivariate and logistic regression analyses were performed to evaluate the factors associated with late presentation (defined as individuals whose first CD4 count was <350 cells/mm3) in the study population. RESULTS: The prevalence of late presentation was 69.8%. The multivariate logistic analysis showed testing initiated by the provider (ORadjusted 3.75; 95%CI 2.45-5.63) increased the odds of late presentation. History of drug use (ORadjusted 0.59; 95%CI 0.38-0.91), history of having sexually transmitted infections (ORadjusted 0.64; 95%CI 0.42-0.97), and having less education (ORadjusted 0.63; 95%CI 0.41-0.97) were associated with a decreased odds of LP. CONCLUSIONS: Provider initiated testing was the only variable to increase the odds of late presentation. Since the patients in this sample all self-identified as heterosexual, it appears that providers are not requesting they be tested for HIV until the patients are already presenting symptoms of AIDS. The high prevalence of late presentation provides additional evidence to shift towards routine testing and linkage to care, rather than risk-based strategies that may not effectively or efficiently engage individuals infected with HIV.

Revisiting the use of condoms in Brazil.

INTRODUCTION: It is known that a single prevention strategy is not enough to control multiple HIV epidemics around the world and in Brazil. However, it is not only necessary to recognize the importance of condoms as part of the policy of HIV/AIDS prevention but also discuss its limits. In this article, we aim to investigate the use of condoms in Brazil, draw critical reflections, and understand how they can once again be highlighted in Brazil's prevention strategy going forward. METHODS: A narrative review of literature was conducted using keywords in PubMed. Reports from national surveys that guide the epidemiological and behavioral surveillance of the Brazilian Ministry of Health were also included. RESULTS: A total of 40 articles and 3 reports were included in the review and 11 intervention studies to promote the condom use; the main findings were as follows: 1) Despite the increase in national studies on sexual behavior, little attention is given to the role of condom use; 2) There are few studies examining the factors associated with condom use among key populations such as men who have sex with men (MSM), female sex workers (FSW), drug users (DU), and transvestites and transexuals (TT), while substantial studies focus on adolescents and women; 3) Evidence suggests that a combination of interventions is more effective. DISCUSSION: new prevention technologies must not lose sight of the critical importance of condoms, and efforts to reintroduce them should focus on the role of pleasure in addition to their potential to minimize the risk of HIV.