The enigma of sickle cell hepatopathy: Pathophysiology, clinical manifestations and therapy.

Sickle cell disease (SCD) is one of the most common genetic disorders in the world predominantly affecting economically disadvantaged populations. There is a notable discrepancy between the growing adult SCD population and available diagnostic and therapeutic interventions for SCD. Sickle cell hepatopathy (SCH) is an all-inclusive term to describe the acute and chronic liver manifestations of SCD. The pathophysiology of SCH follows no defined pattern or sequence that poses challenges to clinicians and researchers alike. Evidence is lacking for this underreported disease at various levels from diagnostic to therapeutic options. This paper reviews the basic pathophysiology, clinical features, biochemical and radiological findings of various SCH manifestations and outlines the management of each condition. Old and new therapy options in SCD including hydroxyurea, red blood cell exchange transfusion, ursodeoxycholic acid, voxelotor, l-glutamine and crizanlizumab have been reviewed to investigate the role of these options in treating SCH. The role of liver transplant, haematopoietic stem cell transplant and gene therapy in SCH patients have been reviewed.

An emerging method in evaluation of reduced fetal movements using cerebroplacental ratio: A prospective case-control study.

AIM: Nearly half of the patients with stillbirths experience reduced fetal movements (RFM) in the preceding week. The standardized evaluation will help reduce stillbirths. Placental dysfunction is the underlying pathophysiology for RFM and low cerebroplacental ratio (CPR). We attempted to determine the application of CPR in evaluation of RFM. METHODS: A prospective case-control study with 100 patients each of cases and controls were studied at a tertiary care hospital. Middle cerebral artery pulsatility index (MCA-PI), umbilical artery PI (UA-PI), and CPR were calculated as multiples of median (MoM) in patients presenting with RFM after 30 weeks and their matched controls and followed up. CPR values of various gestation ranges and single versus multiple episodes of RFM were analyzed. RESULTS: Compared to controls, women with RFM showed a significantly low MCA PI MoM (0.94 vs 1.4; p = 0.00008) and low CPR MoM (1.48 vs 1.6; p = 0.015). Women with multiple RFM episodes had lower CPR MoM (1.2 vs 1.5; p < 0.00001) compared to single episode RFM. Multiple RFM episode patients had low MCA PI MoM (1.3 vs 1.4; p = 0.0038) and low CPR MoM (1.2 vs 1.6; p < 0.00001) compared to controls. There was a significantly low CPR in 32-34 weeks (1.35 vs 1.81; p = 0.004) and 36-38 weeks subgroups (1.39 vs 1.58; p = 0.002). No significant difference in birthweight centiles or AGA versus SGA categories between cases and controls was noted questioning current guidelines where ultrasound evaluation is done for RFM patients with FGR. CONCLUSION: Incorporation of routine CPR measurements can standardize evaluation of RFM patients, especially those with AGA to pick up patients at risk of poor outcome and bring down stillbirth rates.

Impact of Hypoglycemia on Hospitalized Patients With Hepatocellular Carcinoma.

Aims Hepatocellular carcinoma (HCC) is one of the common liver malignancies that presents a challenge to global healthcare. The impact and outcomes of hypoglycemia in HCC have not been studied in detail before. This study aimed to investigate the outcomes and prognosis associated with hypoglycemia in patients diagnosed with HCC, utilizing a large-scale database approach. Methods Using the Nationwide Inpatient Sample (NIS) database from 2017 to 2020, we conducted a comprehensive retrospective analysis to examine the incidence, risk factors, and clinical implications of hypoglycemia on HCC patients. The patients were divided into two groups: those with hypoglycemia and those without hypoglycemia. Univariate and multivariate logistic regression were used to conduct the analysis. STATA® version 17.0 software (StataCorp LLC, College Station, TX) was used for this purpose. Results Out of a total of 343,895 patients with HCC, the prevalence of hypoglycemia was present in 1.5% of this patient population. We found that hypoglycemia was common in the male population (68%). Compared with patients without hypoglycemia, patients who had hypoglycemia with HCC had higher mortality (42%, p-value < 0.05) and higher risks of secondary outcomes such as hepatic failure, spontaneous bacterial peritonitis (SBP), ascites, and portal vein thrombosis compared to patients who did not have hypoglycemia. The multivariate-adjusted odds ratio for hepatic failure was 2.7 (2.3-3.1), for SBP was 2.9 (1.8-3.0), for ascites was 1.6 (1.4-1.9), and for portal vein thrombosis was 1.2 (0.9-1.4). Conclusion In conclusion, hypoglycemia in HCC is associated with increased mortality and worse outcomes.

An audit of hysterectomy in a teaching hospital in India: Story of a decade.

BACKGROUND: The appropriateness of hysterectomy has gained an interest in scrutiny and debate. Periodic audits of the prevailing clinical practices are imperative for insight, and to formulate recommendations and guidelines. We report the temporal trends of hysterectomies, over the last 10 years in a teaching hospital. METHODS: Present study involved all patients who underwent hysterectomy at a teaching hospital, from January 1, 2012 to December 31, 2021. Patients were identified by medical record tracking using International Classification of Diseases-9 codes. Case records were reviewed for demography, indication for surgery, approach, complications, hospital stay, and histopathological correlation. RESULTS: Over the years the absolute number of hysterectomies in our hospital has ranged from 414 to 597 (mean 476), barring the coronavirus 19 pandemic year. The proportion of hysterectomy among all gynaecological admissions has ranged from 6% to 9%, except in 2020 where this proportion dropped down to 4%. The indications, age distribution, surgical approach, and complications have remained almost same. CONCLUSION: We report a static trend in hysterectomy over the past 10 years. This audit provides an insight for the need of shifting the abdominal to vaginal route, in carefully chosen patients. This will be beneficial for the patients, and for the trainees, where they can learn under supervision. Availability and patient education about the nonsurgical management options for benign gynecological conditions, as well as awareness about sequelae of hysterectomy, will bring down the rate in countries such as India.