Anemia Requiring Blood Transfusion Research Articles

Response, complications and risk of leukemic transformation of phosphorus-32 p treatment in philadelphia-negative chronic myeloproliferative syndromes

ObjectiveDescribe our experience in treatment with Phosphorus-32 P for refractory Philadelphia negative chronic myeloproliferative syndromes or with side effects to the usual treatment, its complications and risk of leukemic transformation. Material and methodsRetrospective descriptive study including 17 patients with a diagnosis of Philadelphia-negative chronic myeloproliferative syndrome treated with Phosphorus-32 P in our hospital from January 1985 to March 2017. Indications, response to treatment, as well as early and late complications have been analyzed. ResultsOf the 17 patients treated with 32 P (11 men, 6 women; mean age 79,8 years), 6 patients had Polycythemia Vera and 11 Essential Thrombocytosis. A single dose was administered in 9 of the subjects, the rest required two or more doses due to inadequate hematological response and/or relapse. The total dose range of Phosphorus-32 P administered was 116-951 MBq (median: 236 MBq). In 14 patients treated with Phosphorus-32 P, complete or partial response was achieved in hematimetry. In 11 patients, the response was complete, established as a platelet count < 400.000/mm3 in those diagnosed with Essential Thrombocythemia and a hematocrit < 45% in cases of Polycythemia Vera. The median follow-up of patients from the date of the first treatment of Phosphorus-32 P until study completion or death was 37 months (range: 5 - 230 months). Regarding early complications, 2 cases of anemia requiring blood transfusion were observed, and 1 case of mild thrombocytopenia. No leukemic transformation was identified. ConclusionsIn our experience, treatment with Phosphorus-32 P has been a useful therapeutic option in Philadelphia-negative chronic myeloproliferative syndromes in elderly patients who showed poor tolerance and/or resistance to first-line treatment. No leukemic transformation was identified.

Correlation between Component Size and Functional Knee Score in Patients Undergoing Bilateral Simultaneous Total Knee Arthroplasty: A Cross-Sectional Study

Abstract Background: Simultaneous bilateral total knee arthroplasty (simBTKA) has been a favored surgical solution to reduce costs and patient suffering. We aimed to evaluate the rate of asymmetry of component size in patients undergoing simBTKA and its impact on knee function as we believe that implant asymmetry may affect the functional outcomes in those patients. Methods: A cross-sectional study design was done on on 60 patients (120 knees) with simBTKA using patient-specific templating (PST). Patients were included if they had Kellgren–Lawrence Grade III-IV osteoarthritis. Revision surgeries, staged BTKA, or patients with bone defects, valgus deformity, severe varus deformity (defined as above 20°), and extra-articular deformities were excluded from the study. The outcome measures were interlimb component asymmetry, Knee Society Score (KSS), and range of motion (ROM). The comparison between pre- and postoperative findings was done using a dependent t-test. Results: A total of 29 (48.34%) patients had symmetrical femoral and tibial components, whereas the rest had asymmetry, of them, 11.7% had both femoral and tibial component size asymmetry. There were no statistically significant differences between the changes in KSS and ROM in the smaller implant and larger implant groups (P = 0.5 and P = 0.4, respectively). The total number of complications was eight and as follows: superficial infection, aseptic loosening, rupture of the patellar tendon after a bathroom fall, anemia requiring blood transfusion, residual varus deformity, deep venous thrombosis, periprosthetic fracture, and malalignment. Conclusion: There is no correlation between the interlimb component asymmetry and the knee function. However, there was statistically significant improvement from preoperative to postoperative KSS and ROM in small and large implants.

A phase I study of highly potent oral ATR inhibitor (ATRi) tuvusertib plus oral PARP inhibitor (PARPi) niraparib in patients with solid tumors.

3018 Background: Ataxia telangiectasia and Rad3-related (ATR) protein kinase and poly-ADP ribose polymerases (PARPs) are crucial components in the DNA damage response (DDR). Combining tuvusertib and niraparib may synergistically enhance synthetic lethality and increase apoptosis. Part B1 of the DDRiver Solid Tumors 301 study (NCT04170153) assessed this combination. Methods: Part B1 of this open-label multicenter dose-escalation phase Ib trial enrolled unselected patients with metastatic or locally advanced unresectable solid tumors refractory to standard treatment. The primary objective was to determine safety (including maximum tolerable dose and recommended dose(s) for expansion [RDE]). Secondary and tertiary objectives included determination of the pharmacokinetics (PK), pharmacodynamics (PD), and preliminary efficacy of different dosing regimens of tuvusertib plus niraparib. Multiple continuous and intermittent schedules were explored with tuvusertib doses ranging from 90–180 mg once-daily (QD) and niraparib doses from 100–200 mg QD. A partial ordering continual reassessment method was used to identify the RDEs. Results: As of 02 January 2024, 43 patients had been enrolled (all with baseline body weight &lt;77kg or platelet count &lt;150,000/mm3); 13 patients remained on treatment. Ten (26.3%) patients had dose-limiting toxicities (DLTs). The most common DLT was grade 3 anemia requiring blood transfusion (n=4; 10.5%); the most frequent grade ≥3 treatment-emergent adverse events were anemia (n=18; 41.9%), platelet count decrease (n=6; 14.0%) and fatigue (n=4; 9.3%). The PK of tuvusertib when combined with niraparib was consistent with that of tuvusertib monotherapy, suggesting a lack of any clinically meaningful mutual drug–drug interactions. Dose-dependent ɣ-H2AX inhibition (proximal PD marker) was observed at tuvusertib doses ≥130 mg QD. Preliminary efficacy data show 5 (15.6%) responses (3 confirmed) by RECIST v1.1 in 32 evaluable patients: 2 in patients with epithelial ovarian cancer (EOC; 1 with BRCA1-mutant [ BRCA1m] PARPi-resistant EOC, 1 with BRCA-wild type homologous recombination deficiency-positive EOC) and 1 each in patients with non-small cell lung cancer, estrogen receptor-positive HER2-negative BRCA1m breast cancer, and BRCA1m PARPi-resistant pancreatic cancer. Tuvusertib 180 mg QD and niraparib 100 mg QD or tuvusertib 90 mg QD and niraparib 200 mg QD, both given in a 1 week on/1 week off schedule, were identified as RDEs. Conclusions: The combination of tuvusertib plus niraparib, each administered on a 1 week on/1 week off schedule, has a manageable safety profile and is suitable for further investigation. A combination study in patients with PARPi-resistant EOC is planned. Clinical trial information: NCT04170153 .

Necrotising enterocolitis in a middle-income country: Early onset and risk factors for mortality.

There is limited literature available about necrotising enterocolitis (NEC) in low- and middle-income countries. This study sought to determine the proportion, pattern and risk factors for mortality among very low birth weight (VLBW) neonates with NEC in a middle-income setting. A retrospective observational cohort study was conducted on all infants with birth weights less than 1501 g admitted from 2018 to 2020 at Groote Schuur Hospital, Cape Town, South Africa. Data were obtained from the Vermont Oxford Network and hospital folders. A total of 104/1582 (6.6%) neonates were diagnosed with NEC with a median onset of 8 days of life. The mortality rate was 39.0%, compared to the all-cause mortality rate of 18.7% for VLBW neonates. Thirty-two (32.0%) neonates with NEC were transferred for surgery of whom 10 (31.3%) died. Small for gestational age (p = 0.13), NEC stage 2B or above (p = 0,002), a positive blood culture (p = 0.018), a raised C-reactive protein (p = 0.013), hyponatraemia (p = 0.004), anaemia requiring blood transfusion (p = 0.003) and thrombocytopenia requiring platelet transfusion (p = 0.033) were associated with mortality. A positive blood culture was found in 37.0% with a predominance of Klebsiella pneumoniae isolates. NEC has an early onset and a high mortality rate in our setting.

Gastrointestinal Bleeding in COVID-19 Infected Patients, and Management Outcomes.

Gastrointestinal bleeding in COVID-19-infection poses unique challenges to patients owing to the high risk of concomitant respiratory failure. However, endoscopic care providers are prone to transmission. This study aimed to understand the risk and management outcomes of gastrointestinal bleeding in COVID-19-infected patients. Data were abstracted from electronic patient medical records, using ICD 10 codes, and demographic and clinical data were collected, for COVID-19-infected patients who developed gastrointestinal (GI) bleeding. Complications related to COVID-19 infection and management outcomes of GI bleeding were studied. Statistically, descriptive analysis was used because of the small sample size. Eighteen COVID-19-infected patients developed episodes of GI bleeding, yielding a prevalence of 0.45%. Their mean age was 74.8 years, 55.5% were female, and 66.6% of patients (n=12) had upper GI bleeding symptoms, predominantly melena (55.5%), followed by coffee ground nasogastric aspirates (n=2). Only two patients (11.11%) had episodes of lower GI bleeding, and the remaining four patients (22.2%) had recurrent acute anemia requiring blood transfusion. The Glasgow-Blatchford score (GBS) at presentation ranged between 6 to 16 (mean 8.8) and seven patients (38.8%) underwent endoscopic evaluation for GI bleeding. The predominant comorbid conditions included hypertension (22.2%), diabetes mellitus (27.7%), chronic kidney disease (50%), ischemic heart disease (33%), atrial fibrillation (11.1%), and peripheral vascular disease (11.1%). The median hospitalization was 24.6 days (range: 3-54 days). The 30-day mortality rate in our cohort was 22.2%, (4/18) mainly noted in older patients aged> 60 years with comorbid conditions and severe COVID-19 infection. The prevalence of GI bleeding observed in our cohort was approximately 0.45%, significantly lower than the global prevalence observed, majority (66%) had upper GI bleeding. The exact reasons for the observed low prevalence of GI bleeding cannot be explained and will be the subject of future research.

Colorectal cancer in inflammatory bowel diseases in St. Petersburg: results of an analytical study

Introduction. Ulcerative colitis (UC) and Crohn’s disease (CD) belong to the category of inflammatory bowel disease (IBD) and are one of the most pressing problems of gastroenterology worldwide, including due to the development of specific life-threatening complications. Chronic inflammation in the colon in IBD is the cause of the development of one of these complications - colorectal cancer, which requires disabling colectomies and is also one of the leading causes of death among IBD patients. In the Russian population of IBD patients the prevalence of dysplasia and cancer of the colon mucosa has not been previously studied in large epidemiological studies. The aim of the study was to assess the incidence of colorectal cancer in patients with IBD in St. Petersburg. Materials and methods. Within the framework of this publication, the results of three studies were compared: a single-center retrospective cohort study conducted on the basis of the city center of IBD in St. Petersburg (516 patients with IBD who admitted to this medical institution for the period 2020-2023); a multicenter epidemiological study aimed at assessing the clinical features of IBD among the population of St. Petersburg (1072 patients with IBD who admitted to 42 outpatient clinics and 6 city hospitals in 2018-2020); registry study based on the general statistical module of the regional IBD Register of St. Petersburg, operating on the basis of a regional fragment of the unified healthcare state information system (12,858 patients with IBD, data collection period: 2019-2022). Results. As a result of comparing data from three studies, the incidence of IBD-associated colorectal cancer in St. Petersburg was calculated, which was 15.7 cases per 10,000 patients with UC per year and 11.5 cases per 10,000 patients with CD per year. Compared to the population of St. Petersburg as a whole, the likelihood of developing colorectal cancer in patients with UC was 2.9 times higher, in patients with CD - 2.4 times higher. At the same time, in a single-center study, when assessing the prevalence of IBD-associated colorectal cancer, it was found that compared with other life-threatening complications of IBD, it was much less common: toxic dilatation of the intestine occurred 3 times more often, intestinal perforation occurred 20 times more often, decompensated bowel stricture occurred 21 times more often, and severe anemia requiring blood transfusion occurred 36 times more common. Also, as a result of a single-center study, it was found that for 1 case of IBD-associated dysplasia of the colon mucosa, there were 5 cases of sporadic dysplasia. Conclusion. The selection of the most effective therapy and systematic endoscopic examination of patients with IBD will significantly reduce the likelihood of developing colorectal cancer, and systematic observation of the patient with regular endoscopic monitoring of the colon with multifocal biopsy will allow timely detection of dysplasia of the colon mucosa in accordance with current clinical guidelines. It can be assumed that within the framework of cancer prevention measures in the first years of follow-up of a patient with IBD from the onset of the disease, it is advisable to give priority to the risk of developing not only IBD-associated dysplasia, but also sporadic dysplasia of the colon mucosa.

An Observational Study on Impact of COVID-19 in Pregnancy: Clinical Profiles and Foetomaternal Outcomes in Caesarean Section Cases at a Tertiary Care Centre, Gujarat, India

Introduction: The Coronavirus Disease 2019 (COVID-19) pandemic, caused by Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) infection, has resulted in a crippled healthcare system worldwide. In this unprecedented situation, it is important to analyse the impact on the vulnerable population of pregnant women, especially those with high-risk pregnancies and those undergoing a Caesarean section (CS). Aim: To analyse the clinical profile, foetomaternal outcomes, and co-morbidities in COVID-19-affected pregnancy cases undergoing a CS. Materials and Methods: This was a retrospective observational study conducted at the Department of Obstetrics and Gynaecology, Government Medical College, and New Civil Hospital in Surat, Gujarat, India during the first wave of the COVID-19 pandemic from April 2020 to December 2020. The study included a total of 65 cases of COVID-19-positive mothers undergoing a C-section. Demographic parameters such as age, obstetric history, details of the C-section (like gestational age at the time of the procedure, indication, and category of the C-section), associated co-morbidities, severity of COVID-19- related symptoms and treatment, neonatal parameters {such as birth weight, Appearance, Pulse, Grimace, Activity and Respiration (APGAR) score, and COVID-19 status of the baby at birth} were analysed. Additionally, a comparison of the C-section rate and complications such as the development of severe anaemia requiring blood transfusion, postoperative development of urinary tract infections, surgical site infections, and the association of hypertensive disorders of pregnancy was conducted between COVID-19-affected and unaffected pregnancies undergoing a C-section during the study period. Data on COVID-19 negative cases were obtained from the monthly labour room statistics submitted to the department. The Chi-square test and Fisher’sexact test were used to compare parameters of the COVID-19 positive and negative groups and a p-value of less than 0.05 was considered statistically significant. Results: Out of a total of 6246 deliveries conducted at the institute during the study period, the CS rate among affected and non affected women was 44.5% (65 out of 146) vs. 32.8% (2006 out of 6100) {p=0.003, Relative Risk (RR) 1.3, 95% Confidence Interval (CI) 1.12-1.62}. The mean age of women with COVID19 undergoing a CS was 25.3 years. The difference in the rate of blood transfusion requirement in COVID-19-affected cases was 9 out of 65 (13.8%) vs. 120 out of 2006 (6%) in COVID-19 negative pregnancies (RR 2.3, 95% CI: 1.2-4.3). The difference in the rate of urinary tract infections in the postoperative period was statistically significant in COVID-19-infected patients, 5 out of 65 (7.7%) vs. 58 out of 2006 (2.9%) in non infected patients (p=0.0451, RR 2.6, 95% CI 1.1-6.4). The prevalence of hypertensive disorders among COVID-19 positive and negative mothers undergoing CS was very high in the present study (16 out of 65, 24.6% vs. 160 out of 2006, 8.1%, RR 3.08, 95% CI 1.9-4.8). Neonatal parameters like low APGAR score at birth were seen in 5 cases (8%), low birth weight in 20 cases (30%), and 3 babies (4.5%) were COVID-19 positive at birth. Conclusion: Higher rates of caesarean sections, increased prevalence of hypertensive disorders of pregnancy, anaemia requiring blood transfusion, and postoperative development of urinary tract infections among COVID-19-affected mothers undergoing a caesarean section.

The Impact of Preoperative Co-morbidities on Blood Transfusion Requirements Following Reverse Total Shoulder Arthroplasty.

Reverse total shoulder arthroplasty (RTSA) continues to increase in popularity as a surgical operation in the United States. As indications for this procedure expand, more attention is needed to evaluate perioperative risk factors and patient characteristics. Postoperative anemia requiring blood transfusion (BT) is a well-documented risk factor for increased in-house mortality although little has been studied on the relationship between RTSA and postoperative BT. The purpose of this study was to identify comorbidities and patient characteristics as risk factors for BT in patient's undergoing RTSA. Using the Nationwide Inpatient Sample (NIS) database, 59,925 RTSA patients (2016-2019) were analyzed, with 1.96% requiring postoperative BT. Demographics, comorbidities, and preoperative factors were compared between BT and non-BT groups via univariate and multivariate analyses. Overall prevalence of blood transfusion in all patients was 1.96%. Male sex (OR 1.75, p < 0.001), Asian ethnicity (OR 1.96, p = 0.012), age >80 (OR 1.51, p < 0.001), age >90 (OR 2.26, p < 0.001), CKD (OR 1.94, p < 0.001), and Parkinson's disease (OR 2.08, p < 0.001) were associated with increased BT odds. Cirrhosis exhibited the highest impact (OR 5.7, p < 0.001). Conversely, Caucasian ethnicity (OR 0.76, p = 0.023), uncomplicated DM (OR 0.73, p = 0.002), tobacco-related disorders (OR 0.43, p < 0.001), BMI >30 (OR 0.8, p = 0.011), and elective procedures (OR 0.16, p < 0.001) decreased BT odds. These results were useful with identifying several risk factors that predispose to a higher risk of postoperative BT in patients undergoing RTSA including male sex, people of Asian descent, age > 80, CKD, Parkinson's disease, and cirrhosis. These findings provide clinicians with information that may be helpful with preoperative planning and perioperative management of complex patient populations.

High Response Rates Following Point-of-Care Anti-CD19 CAR T-Cell Therapy in Adults with Relapsed/Refractory Acute B Lymphoblastic Leukemia

Background Tisagenlecleucel and brexucabtagene autoleucel have demonstrated remarkable efficacy and safety results in adult patients with relapsed/ refractory acute B lymphoblastic leukemia (R/R B-ALL). Long term follow-up of the ZUMA-3 trial recently reported a median duration of response (DOR) of 14.6 months and an overall response rate of 71% (Hadjivassileva, EBMT 2023). Methods and patients We report results of a phase 1b/2 single center clinical trial (NCT02772198) involving point-of-care (POC) anti-CD19/CD28 costimulatory domain chimeric antigen receptor (CAR) T-cell therapy in adults with R/R B-ALL. Inclusion criteria were age ≥18 years, failure of at least 2 prior therapies, and a preserved organ function. Patients underwent a single leukapheresis procedure. Fresh CAR T products were delivered for immediate infusion. Lymphodepletion included fludarabine and cyclophosphamide. Cell dose was 1x10 6 CAR T cells/kg. Primary endpoints were 1-month disease response and safety. Secondary endpoints were the minimal residual disease (MRD) negativity rate, overall survival, progression-free survival (PFS), and production feasibility. Last follow-up was July 2023. Results Between 03/2017-05/2023, 28 patients enrolled. CAR T-cells were successfully produced in 27 patients (96%) who were included in the analysis. The median age was 33 years (range 19-77), and 7 (25%) patients had Karnofsky Performance Status &amp;lt;90%. Six patients (22%) had positive Philadelphia chromosome, and 14 patients (52%) had an extramedullary disease at leukapheresis. Nineteen patients (70%) had ≥3 prior lines of therapy, 18 patients (67%) underwent prior allogeneic hematopoietic stem-cell transplantation (allo-HCT), and 20 patients (74%) had prior exposure to inotuzumab-ozogamicin or blinatumomab. At leukapheresis, 16 patients (60%) had an active disease, while 11 patients (40%) were in complete morphological response (CR; MRD positive, n=7 [26%], MRD negative or not evaluated, n=4 [15%]). The median time between leukapheresis and cell infusion was 11 days (IQR 10-11). Only 3 patients (11%) received bridging chemotherapy. Grade 3-4 cytokine release syndrome and immune effector cell-associated neurotoxicity syndromes were observed in 30% of the patients, each. Six (22%) and 16 (59%) patients were treated with tocilizumab or corticosteroids, respectively. Severe neutropenia (&amp;lt;0.5k/μl) and thrombocytopenia (≤50 K/μl) occurred in 81% and 70% of the patients, respectively, and anemia requiring blood transfusion occurred in 71%. Three patients (11%) had grade 3 cardiovascular events, and 1 patient (4%) had grade 3 pleural effusion. Bloodstream bacterial infection occurred in 3 (11%) patients. Cellular therapy-related mortality was observed in 2 (7%) patients (both due to septic shock). The 1-month overall response rate was 88% (81% CR; MRD negative CR, 63%). MRD was evaluated by RT-PCR in most of the patients. The median follow-up was 19.7 months (IQR 4.7-43.2). Two-year overall survival and PFS were 62% (95% CI: 42-91), and 56% (95% CI: 38-84), respectively. The median DOR was not reached (95% CI: 6.1 months-not reached). PFS was not affected by prior exposure to inotuzumab-ozogamicin or blinatumomab (hazard ratio (HR) 0.7, 95% CI: 0.17-2.68, p=0.6). All eligible patients who achieved a response were offered consolidation with allo-HCT. Eight patients (30%) underwent allo-HCT (2 nd allo-HCT in 3 of them). The DOR was not affected by consolidation of CR with allo-HCT (HR 0.9, 95% CI: 0.2-4.1, p&amp;gt;0.9). Nevertheless, 2 patients (7%) died due to allo-HCT related complications while in CR. Conclusion Treatment with POC CAR T-cells, produced in an academic center, achieved high response and survival rates in adults with R/R B-ALL. These results are also comparable to the FDA approved CAR T-cells. The use of POC CAR T abrogates the need for cryopreservation and shipment of the cells, thus allowing a short vein-to-vein time without the need of bridging therapy in most patients.

Abstract 17878: Clinical Profile, Procedure-Related Complications and Long-Term Outcomes in Men and Women With Aortic Stenosis Undergoing Transcatheter Aortic Valve Implantation: Insights From TAVI-NOR Study

Introduction: The incidence of aortic stenosis (AS) is increasing. If left untreated, AS progresses to clinical heart failure, reduced quality of life and functional capacity, and ultimately death. Hypothesis: Gender difference in survival benefits of transcatheter aortic valve implantation (TAVI) and frequencies of procedural complications. Methods: We included 600 consecutive, less selected, patients with symptomatic AS who underwent TAVI at our hospital. Clinical and echocardiographic data was analysed in SPSS. Data distribution was checked for normality. The inter-group comparison by Student’s t-test or chi square as appropriate. The association between gender and all-cause mortality assessed by Kaplan-Meier survival analysis with Log-rank test. Results: Mean age was 80.8±6.5 years of the study population. Women (49.3%) were older (82.2 ±5.3 years vs 79.5±7.3 years, p&lt;0.001), had more severe AS and higher left ventricular ejection fraction and more often had basal septal hypertrophy. The prevalence of hypertension and use of antihypertensive medications were higher in women. The prevalence of diabetes mellitus, coronary artery disease, previous stroke, overall abnormal ECG, and atrial fibrillation was higher in men. The severity of symptoms, and prevalence of chronic kidney or lung diseases were comparable. The frequencies of procedure-related paravalvular leakage (PVL) ≥ grade II and endocarditis during follow-up were low, but significantly higher in men than women. No significant difference between genders in procedure-related access site dissection and pseudo-aneurysm, anaemia requiring blood transfusion, or new-onset renal failure. Need of permanent pacemaker implantation following TAVI was also comparable between genders. Mean event-free survival was 59±24 months for men and 66±25 months for women (p&lt;0.001). Most apparent benefits was seen at 3-year follow-up where survival estimate for men was 84% and for women 92%. Conclusions: Results from TAVI-NOR study demonstrates that women with AS were older and had a higher burden of hypertensive heart disease, and showed a significantly better outcome. Men more frequently developed a significant PVL or endocarditis during follow-up.

Pyeloplasty in Children with Ureteropelvic Junction Obstruction and Associated Kidney Anomalies: Can a Robotic Approach Make Surgery Easier?

Robot-assisted pyeloplasty is widely used in pediatric surgery because of its well-known advantages over open or laparoscopic surgery. The aim is to explore our experience and evaluate the achievements we have made. We evaluated patients undergoing robotic pyeloplasty from January 2016 to November 2021, including those who presented with a ureteropelvic junction obstruction associated with other anomalies of the kidney. The parameters examined were: age, weight, associated renal malformations, conversion rate, operative time, and intra- and postoperative complications. Of 39 patients, 7 (20%) were included, of whom 5 (71%) were male and 2 (29%) were female. The mean age at surgery was 84 months (range 36-180 months), and the mean weight at surgery was 24.4 kg (range 11-40 kg). In five (71%) patients the ureteropelvic junction obstruction (UPJO) was left-sided and in two (29%) it was right-sided. In four (57%) cases, UPJO was associated with a horseshoe kidney, right-sided in one (25%) patient, and left-sided in the other three (75%). A 180° rotation of the kidney was present in one (14%) patient. Nephrolithiasis was present in two (29%) patients. The mean operative time was 160 min (range 140-240 min). The average bladder catheter dwell time was 1 day (range 2-3 days), while the average abdominal drainage dwell time was 2 days (range 2-4 days). The mean hospitalization time was 4 days (range 3-9 days). On average, after 45 days (range 30-65) the JJ ureteral stent was removed cystoscopically. No intraoperative complications were reported, while one case of persistent macrohematuria with anemia requiring blood transfusion occurred postoperatively. Ureteropelvic junction obstruction might be associated with other congenital urinary tract anomalies such as a duplicated collecting system, horseshoe kidney, or pelvic kidney. These kinds of malformations can complicate surgery and require more attention and accuracy from the surgeon. Our experience shows that, with regards to the robotic learning curve required for pyeloplasty, the treatment of the ureteropelvic junction in these situations does not present insurmountable difficulties nor is burdened by complications. The application of robot-assisted surgery in pediatric urology makes difficult pyeloplasties easier.

FINCA syndrome

FINCA (fibrosis, neuro-degeneration, cerebral angiomatosis) syndrome is a rare autosomal recessive early onset fatal disorder characterised by progressive cerebropulmonary symptoms, malabsorption, progressive growth failure, recurrent infection, chronic haemolytic anaemia and transient liver dysfunction. A 5 year old female baby born as a preterm 33 weeks (2nd twin of dichorionic diamniotic twin) to nonconsanguineous parents via intra-cytoplasmic sperm injection and surrogacy, delivered by emergency caesarean section (indication- pre-eclampsia in mother) with a birth weight of 1.8 kg. She had a stormy neonatal period and had anaemia requiring blood transfusion. Developmental delay was noted from early infancy which was not responding to developmental therapy, along with recurrent episodes of aspiration pneumonia. Her twin on the other hand had normal mile stones without any remarkable medical history. Examination and evaluation revealed features of chronic hemolysis. In view of constellation of developmental delay, recurrent infection, chronic hemolytic anaemia and cerebral atrophy beyond documented perinatal issues and dysmorphism, clinical exome sequencing was done which revealed missense variation in exon of NHLRC2 gene on chromosome 10 suggestive of FINCA syndrome. This case emphasized the need of genetic testing to resolve uncertainty in etiology and accurate prognostication.

Sex-Based Outcomes in Patients Undergoing WATCHMAN Device Implantation

Sex-based disparities in outcomes are reported for various cardiovascular procedures. This study aimed to assess the difference in outcomes in patients who underwent WATCHMAN device implant based on sex. Patients who underwent WATCHMAN device placement, from 2016 to 2018, were identified from the National Inpatient Sample database. The primary outcome was inpatient mortality, and the secondary outcomes were the length of stay (LOS), hospitalization cost (HOC), and periprocedural complications. A logistic regression model was built to perform an adjusted analysis for the outcomes. A total of 12,327 patients underwent WATCHMAN device placement. Female patients were older and more likely to have hypertension (p <0.01) and less likely to have peripheral arterial disease (5.6 vs 7.2, p <0.01), chronic kidney disease (21% vs 26%, p <0.01), and diabetes (18% vs 20%, p=0.03) and were also at a higher risk for certain periprocedural complications, including pericardiocentesis and anemia requiring blood transfusion (p <0.01 for all). In the unadjusted analysis, the female sex was associated with longer LOS (1.5 vs 1.3days, p <0.01) and inpatient mortality (0.23 vs 0.10, p=0.05). The HOC was numerically higher in women but statistically nonsignificant ($120,791 vs $118,554, p=0.1). In the stepwise, backward, multivariate regression analysis, the female sex was an independent risk factor for higher LOS (1.5 vs 1.3days, p <0.01, 95% confidence interval 1.3 to 1.4) after adjusting for potential confounders. The inpatient mortality and HOC were similar between 2 groups after adjusting for potential cofounders in the multivariate regression analysis. Our study suggests that the female sex is an independent risk factor for longer LOS among patients hospitalized for WATCHMAN device placement.

Inherited CD59 deficiency, where neurology and genetics intertwine

To describe the clinical phenotype of eight children diagnosed with CD59 deficiency and their ultimate neurological outcome. The data of our cases were extensively reviewed both clinical and ancillary tests; investigations included: neuroimaging, neurophysiological studies, and laboratory tests. All patients presented during early infancy with Guillain-Barre syndrome later they suffered repeated relapses leading to the diagnosis of chronic axonal neuropathy. Recurrent stroke and acute necrotizing encephalopathy were described, 2 patients in each group. One girl developed acute disseminated encephalomyelitis while one boy developed acute transverse myelitis. Overt hemolytic anemia requiring blood transfusion reported in six patients. Inherited CD59 deficiency is an autosomal recessive disorder which can have devastating neurological consequences. First line immunotherapy including intravenous immunoglobin, corticosteroids, and plasma exchange may have transient beneficial effect. Reports of targeted therapy with eculizumab might be lifesaving. Genetic counseling is crucial.

Maternal and fetal outcomes in pregnant patients with non-cirrhotic portal hypertension: A systematic review and meta-analysis.

Concerned studies with respect to the outcome of pregnant patients with non-cirrhotic portal hypertension are limited. Thus, a systematic review and meta-analysis of the available literature was conducted. A literature search was conducted from 1999 to December 2021 for studies evaluating pregnancy outcomes in patients with non-cirrhotic portal hypertension. Twelve studies were included in the meta-analysis. The pooled rate of variceal bleeding, ascites and severe anemia requiring blood transfusion were 9.6%, 2.3%, and 14.9%, respectively. The pooled rate of spontaneous miscarriage, gestational hypertension, delivery by cesarean section, and postpartum hemorrhage were 11.9%, 4.5%, 36.7%, and 4.7%, respectively. The pooled stillbirth rate was 2.5% and among the live births, the pooled rates of preterm birth, low birth weight, intensive care unit admission, and neonatal mortality were 21.6%, 18.7%, 15.5%, and 1.8%, respectively. Pregnancy in patients with non-cirrhotic portal hypertension is associated with increased maternal & fetal morbidity but mortality remains low.

Piropoiquilocitose hereditária no período neonatal - relato de caso

The Hereditary Pyropoikilocytosis is a rare and severe form of Elliptocytosis, characterized by an alteration in the skeleton of the red blood cell membrane. It is an autosomal dominant condition with variable penetration in elliptocytosis, which may be in homozygosity or double heterozygosity in Pyropoikilocytosis, starting from the neonatal period, with clinical manifestations of persistent jaundice. We report here a case of a newborn with persistent jaundice, associated with anemia requiring blood transfusion. The clinical evolution and laboratory findings were conclusive for the diagnosis of Hereditary Pyropoikilocytosis.

Iliopsoas bleeds in patients with hemophilia

Background: Iliopsoas bleed is a serious complication in people with hemophilia (PWH), with significant morbidity. Studies examining the profile, incidence, and outcomes in psoas bleeds are scarce and will shed more light and increase awareness about its management. Material and Methods: Data of 453 PWH with confirmed iliopsoas bleed treated at Bangalore Medical College were retrospectively analyzed. Results: Eighty-five (18.8%) PWH presented with total of 154 psoas bleeds. Their mean age was 21.96 years. Common symptoms were thigh/hip/groin pain (100.0%), hip flexion spasm (41.2%), numbness/tingling in quadriceps muscle (5.9%), abdominal tenderness (3.5%), hematuria, and anemia requiring blood transfusion in 2.4% each. Long-term complications were quadriceps atrophy (9.4%), permanent abnormal posture (10.6%), transient paresthesia in the distribution of femoral nerve (3.5%), and pseudo tumor in pelvis (1.2%). The overall average duration of therapy with clotting factor concentrate was 1.7 days with a mean duration of therapy of 1.4 days in patients without inhibitors and 2.8 days in patients with inhibitors (p = 0.010). The overall mean duration of hospitalization was 7.2 days with 5.3 days in PWH without inhibitors and 8.3 days in PWH with inhibitors (p = 0.342). Conclusion: Pain in hip joint/groin/hip flexion spasm suggests the possibility of an iliopsoas hematoma and early factor replacement therapy should be started to prevent complications. Early treatment at first sign of discomfort reduces the duration of treatment, and prevents severe complication and invasive interventions. Patients with inhibitors were overrepresented in the cohort who needed longer duration of factor replacement therapy.

Piropoiquilocitose Hereditária no período Neonatal - Relato de caso

The Hereditary Pyropoikilocytosis is a rare and severe form of Elliptocytosis, characterized by an alteration in the skeleton of the red blood cell membrane. It is an autosomal dominant condition with variable penetration in elliptocytosis, which may be in homozygosity or double heterozygosity in Pyropoikilocytosis, starting from the neonatal period, with clinical manifestations of persistent jaundice. We report here a case of a newborn with persistent jaundice, associated with anemia requiring blood transfusion. The clinical evolution and laboratory findings were conclusive for the diagnosis of Hereditary Pyropoikilocytosis.

Contribution of Environment in Comorbidities in Children with Sickle Cell Disease: A Prospective Observational Study at a Tertiary Care Center in India

Key words -environmental determinants, morbidity, sickle cell disease, children Sickle cell disease (SCD) presents with highly variable clinical consequences, which are noted even among identical twins residing in different geographical areas, but remain unexplained even after extensive genetic studies. India has 2nd largest number of births with SCD, only second to Africa. Different regions in India also have variability in climate and environment which also varies from season to season. Therefore, we aimed to study the contribution of environmental determinants associated with morbidity in children with SCD. We conducted a prospective observational study in hospitalized children diagnosed with SCD in a tertiary care center in Government medical college, Nagpur, Maharashtra in India. Our study included 88 females (41.51%) and 124 males (58.49%) in the age group of 6 months -14 years, but majority were between 5-10 years of age (50.94%). Children living outside a radius of 10 km from the hospital where study is conducted and children with systemic disorders were excluded. Convenience sampling method was used to examine the effect of several environmental factors including temperature, rainfall, relative humidity, wind speed, atmospheric pollutants such as nitrogen dioxide (NO2), sulfur dioxide (SO2) and ozone from 1st January 2020 to 31st December 2021. We correlated the association of these environmental factors with the severity of morbidity related to the duration of hospital stay, infection, vasoocclusive crises (VOC), intensive care utilization (ICU) & number of blood transfusions. In our study population maximum duration of hospital stay was 44 days and minimum of 2 days. The cause of admissions included VOCs (54.25%), severe anemia requiring blood transfusion (41.98%), and infections (34.91%). During our 2 years study period, range of maximum and minimum temperature was 22-46.8 and 0 -30.8degree Celsius, relative humidity of 20-100%, wind speed of 0-28 knots, rainfall of 0-100.3 mm, NO2 levels of 0-95 ug/mm3, SO2 levels of 0-39ug/mm3 & 0zone of 0-148ug/mm3. Maximum number of admissions (62.73%) occurred in the months of June - November. High relative humidity (p value =0.0001) was the most important factor leading to an increase in hospital admissions, followed by rainfall (p value =0.0004). Among air pollutants, increase in NO2(p value =0.0002) and SO2(p value =0.0014) levels were associated with higher number of admissions, whereas ozone levels had no significant impact on number of admissions (p value =0.1499). (More statistical analysis will be provided at the time of presentation). Surprisingly COVID-19 pandemic substantially reduced the number of SCD patients seeking medical attention in our tertiary care center. It is also likely that our results may be influenced by a change in the environmental exposure due to the pandemic. Also, the current observations are from a single city. However, our data provide a 'proof of principle' that the environment contributes to the comorbidities of SCD and offer support to undertake larger studies in different geographic regions of tropical countries. We conclude that environmental factors are important determinants of acute complications in children with SCD. Identification of such environmental factors will help develop strategies to prevent/reduce the comorbidities of SCD and improving the quality of life for children and perhaps adults with SCD.

Study of polyps in female reproductive tract in a tertiary care centre

To study the clinical presentation, management of polyps of female reproductive tract. To know the correlation between ultrasonological finding and Histopathological findings. This was a retrospective hospital based observational study. It was conducted in Cheluvamba hospital, attached to Mysore Medical college and Research Institute, Mysore. Data was collected from hospital registers who underwent surgery for diagnosis of polyp from the year 2018 to 2021 were included in the study. There were 50 cases in the study. They were managed according to their symptoms, age, desire for fertility and type of polyp. Polyps are most common in the age group 31 to 40 years (42%) and in parous women. The most common presenting complaint was heavy menstrual bleeding (82%). Based on USG and HPE the most common type of polyp was leiomyomatous polyp. Heavy menstrual bleeding caused severe anaemia in 44% of women who required blood transfusion for correction anaemia. No cases of malignancy were diagnosed.Polyps are an important cause of AUB. The resulting heavy menstrual bleeding can cause severe anaemia requiring blood transfusion, leading to morbidity. Clinical examination and ultrasound diagnosis correlates with histopathological diagnosis. Using clinical examination and ultrasound one can identify the site and type of polyp and decide on further management.