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Mediterranean Journal of Hematology and... 2023In patients with SCD, chronic liver damage is a common manifestation. More than 50% of SCD patients have elevated liver enzymes. Common underlying aetiologies include... (Review)
Review
In patients with SCD, chronic liver damage is a common manifestation. More than 50% of SCD patients have elevated liver enzymes. Common underlying aetiologies include sickle cell hepatic crisis, viral hepatitis, sickle cell intrahepatic cholestasis and hepatic sequestration in the acute setting, and cholelithiasis and iron overload in the chronic setting. Autoimmune hepatitis (AIH) is a rare disease that appears to occur more commonly in the sickle cell disease (SCD) population than in the general population. There are many schools of thought as to why this is the case, including the phosphatidylserine hypothesis, the heme inflammatory hypothesis, the complement generation hypothesis, and the transfusion alloimmunization hypothesis. Due to the natural history of the two illnesses, SCD is almost always diagnosed first in cases of dual pathology. Symptoms such as jaundice, fatigue, and abdominal pain are common in SCD, as are abnormal liver function tests (LFTs). These abnormalities, attributed to the other more frequent liver involvements in SCD, can lead to delays in AIH diagnosis in this population. Corticosteroids, sometimes with other immunosuppressive agents, such as azathioprine, are the cornerstone of acute AIH treatment. However, corticosteroid use in the SCD population has been shown to carry an increased risk of vaso-occlusive crises, providing a treatment dilemma. The following is a review of AIH in the SCD population, where we explore the pathophysiology behind the association between the two disorders, discuss an approach to investigating abnormal LFTs in SCD, and examine treatment options in this population with co-existing diseases.
PubMed: 38028400
DOI: 10.4084/MJHID.2023.060 -
Canadian Journal of Physiology and... Apr 2024Phototherapy is the standard treatment for neonatal jaundice. We aimed to review the efficacy and safety of fenofibrate as an adjunct therapy. Twelve databases were... (Meta-Analysis)
Meta-Analysis
Phototherapy is the standard treatment for neonatal jaundice. We aimed to review the efficacy and safety of fenofibrate as an adjunct therapy. Twelve databases were searched and a systematic review and meta-analysis were conducted. Mean change (MC), mean difference (MD), and risk ratios (RR) with a 95% confidence interval (CI) were calculated using a random effects model. The GRADE approach was used to evaluate the evidence's certainty. Nine randomized trials were included. The MC of total serum bilirubin (mg/dL) was significant at 12, 24, 36, 48, and 72 h with respective MC (95% CI) values of -0.46 (-0.61, -0.310), -1.10 (-1.68, -0.52), -2.06 (-2.20, -1.91), -2.15 (-2.74, -1.56), and -1.13 (-1.71, -0.55). The FEN + PT group had a shorter duration of phototherapy (MD: -14.36 h; 95% CI: -23.67, -5.06) and a shorter hospital stay (MD: -1.40 days; 95% CI: -2.14, -0.66). There was no significant difference in the risk of complications (RR: 0.89; 95% CI: 0.54, 1.46) or the need for exchange transfusion (RR: 0.58; 95% CI: 0.12, 2.81). The certainty of the evidence was very low for all outcomes. In conclusion, fenofibrate might be a safe adjunct to neonatal phototherapy. Larger randomized controlled trials are needed for the confirmation of these results.
Topics: Infant, Newborn; Humans; Fenofibrate; Hyperbilirubinemia, Neonatal; Jaundice, Neonatal; Phototherapy; Time Factors
PubMed: 38011686
DOI: 10.1139/cjpp-2023-0213 -
Frontiers in Public Health 2023In 2021, India contributed for ~79% of malaria cases and ~ 83% of deaths in the South East Asia region. Here, we systematically and critically analyzed data... (Review)
Review
INTRODUCTION
In 2021, India contributed for ~79% of malaria cases and ~ 83% of deaths in the South East Asia region. Here, we systematically and critically analyzed data published on malaria in pregnancy (MiP) in India.
METHODS
Epidemiological, clinical, parasitological, preventive and therapeutic aspects of MiP and its consequences on both mother and child were reviewed and critically analyzed. Knowledge gaps and solution ways are also presented and discussed. Several electronic databases including Google scholar, Google, PubMed, Scopus, Wiley Online library, the Malaria in Pregnancy Consortium library, the World Malaria Report, The WHO regional websites, and ClinicalTrials.gov were used to identify articles dealing with MiP in India. The archives of local scientific associations/journals and website of national programs were also consulted.
RESULTS
Malaria in pregnancy is mainly due to () and (), and on rare occasions to spp. and too. The overall prevalence of MiP is ~0.1-57.7% for peripheral malaria and ~ 0-29.3% for placental malaria. Peripheral infection at antenatal care (ANC) visits decreased from ~13% in 1991 to ~7% in 1995-1996 in Madhya Pradesh, while placental infection at delivery unit slightly decreased from ~1.5% in 2006-2007 to ~1% in 2012-2015 in Jharkhand. In contrast, the prevalence of peripheral infection at ANC increased from ~1% in 2006-2007 to ~5% in 2015 in Jharkhand, and from ~0.5% in 1984-1985 to ~1.5% in 2007-2008 in Chhattisgarh. Clinical presentation of MiP is diverse ranging from asymptomatic carriage of parasites to severe malaria, and associated with comorbidities and concurrent infections such as malnutrition, COVID-19, dengue, and cardiovascular disorders. Severe anemia, cerebral malaria, severe thrombocytopenia, and hypoglycemia are commonly seen in severe MiP, and are strongly associated with tragic consequences such as abortion and stillbirth. Congenital malaria is seen at prevalence of ~0-12.9%. Infected babies are generally small-for-gestational age, premature with low birthweight, and suffer mainly from anemia, thrombocytopenia, leucopenia and clinical jaundice. Main challenges and knowledge gaps to MiP control included diagnosis, relapsing malaria, mixed infection treatment, self-medication, low density infections and utility of artemisinin-based combination therapies.
CONCLUSION
All taken together, the findings could be immensely helpful to control MiP in malaria endemic areas.
Topics: Female; Humans; Infant, Newborn; Pregnancy; Abortion, Spontaneous; Anemia; India; Malaria; Malaria, Vivax; Placenta; Thrombocytopenia
PubMed: 37927870
DOI: 10.3389/fpubh.2023.1150466 -
Child: Care, Health and Development Jan 2024Accumulation of bilirubin above normal levels is considered a neurological risk factor for both premature and full-term newborns. This systematic review aimed to... (Review)
Review
BACKGROUND
Accumulation of bilirubin above normal levels is considered a neurological risk factor for both premature and full-term newborns. This systematic review aimed to determine the effect of neonatal hyperbilirubinemia on neurodevelopment in preterm and full-term newborns.
METHODS
PubMed, EMBASE, Cochrane Library, CINAHL, PsycINFO, Scopus and Lilacs databases were searched for articles published until 1 June 2022. The quality of cohort and case-control studies was assessed with the Newcastle-Ottawa Scale, and the MINCir scale was used to evaluate the methodological quality of therapy studies or the therapeutic procedures. Premature neonates without neurological conditions and those born at term with hyperbilirubinemia as the sole risk factor were included. Studies reporting one or more neurodevelopmental outcomes were included with an inter-group comparison of a hyperbilirubinemia group versus a non-hyperbilirubinemia or non-pathological hyperbilirubinemia group. The main outcomes were auditory function, visual function, cognitive function, motor function, behavior, global development and neurological risk.
RESULTS
The search identified 951 studies, 19 of which (n = 2210 newborns) were finally included. Fifteen of the cohort and case-control studies presented low risk of bias, and six studies showed high methodological quality. Within the preterm population, hyperbilirubinemia as the sole risk factor was not shown to affect neurodevelopment. Auditory, neurological and motor development alterations were found in the population of full-term newborns with hyperbilirubinemia, which were more evident during the first year of life.
CONCLUSIONS
Elevated bilirubin levels may be a trigger for the onset of neurodevelopmental disorders in full-term infants during the first year of life. More studies are warranted in the preterm population with hyperbilirubinemia to draw conclusions about its impact on their neurodevelopment.
Topics: Infant; Infant, Newborn; Humans; Hyperbilirubinemia, Neonatal; Bilirubin; Risk Factors; Neurodevelopmental Disorders; Case-Control Studies
PubMed: 37842871
DOI: 10.1111/cch.13183 -
World Journal of Gastrointestinal... Aug 2023Choledochal cysts (CC) are cystic dilatations of the biliary tract, usually diagnosed during childhood, with an estimated incidence in the general population of...
BACKGROUND
Choledochal cysts (CC) are cystic dilatations of the biliary tract, usually diagnosed during childhood, with an estimated incidence in the general population of 1:100000. Complications related to CC include rupture, biliary obstruction, and cholangitis. Maternal CC in pregnancy are rarely reported, and there are no guidelines on optimal management.
AIM
To systematically review maternal CC diagnosed during pregnancy or postpartum with regard to the clinical presentation of CC, the mode of treatment and delivery, and maternal outcomes.
METHODS
A literature search of cases and case series of maternal CC in pregnancy and postpartum was conducted using MEDLINE/PubMed, Web of Science, Google Scholar, and Embase. There were no restrictions on language or publication year. Databases were lastly accessed on September 1, 2022.
RESULTS
Overall, 71 publications met the inclusion criteria, reporting 97 cases. Eighty-eight cases were diagnosed during pregnancy and nine in the puerperium. The most common symptoms were abdominal pain (81.2%) and jaundice (60.4%). Interventions for CC complications were required in 52.5% of the cases, and 34% of pregnancies were induced. Urgent cesarean section (CS) was done in 24.7%. The maternal mortality was 7.2%, while fetal mortality was inconsistently reported. Cholangitis, CC > 15 cm, and bilirubin levels > 80 mmol/L were associated with a higher likelihood of urgent CS and surgical intervention for CC. Bilirubin levels positively correlated with CC size. There was no correlation between age and cyst dimension, gestational age at cyst discovery, and CC size.
CONCLUSION
Although rare, maternal CC in pregnancy should be included in the evaluation of jaundice with upper abdominal pain. Symptomatology and clinical course are variable, and treatment may range from an expectative approach to emergent surgical CC treatment and urgent CS. While most cases were managed by conservative measures or drainage procedures, CC > 15 cm and progressive cholangitis carry the risk of CC rupture and septic complications, which may increase the rates of unfavorable maternal and fetal outcomes. Therefore, such cases require specific surgical and obstetric interventions.
PubMed: 37701693
DOI: 10.4240/wjgs.v15.i8.1784 -
Cureus Sep 2023This research presents a systematic review focusing on rituximab's therapeutic applications in immunoglobulin G4 (IgG4)-related disease (IgG4-RD), a rare condition... (Review)
Review
This research presents a systematic review focusing on rituximab's therapeutic applications in immunoglobulin G4 (IgG4)-related disease (IgG4-RD), a rare condition characterized by immune-mediated systemic inflammation and tissue fibrosis, as well as the clinical features of IgG4-RD. While the disease commonly affects organs such as the bile ducts, lymph nodes, retroperitoneum, pancreas, and salivary glands, it can potentially involve other organs. This intricacy often leads to diagnostic challenges due to clinical overlaps with cancer, infections, and other autoimmune disorders. The diagnosis of IgG4-RD necessitates a comprehensive approach involving laboratory tests, imaging studies, and clinical assessments. Symptoms can vary, ranging from lymphadenopathy to jaundice, affecting multiple organs. Although elevated blood IgG4 levels and findings of tissue involvement and fibrosis on imaging can be suggestive, they lack the specificity for a definitive diagnosis. Early diagnosis is crucial for initiating corticosteroids and immunosuppressive to prevent further damage from IgG4-RD. This study highlights the therapeutic role of rituximab in managing this condition. Adhering to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) criteria, the research identifies and evaluates relevant literature across various electronic databases, including PubMed, ScienceDirect, and Google Scholar. This review includes 14 selected publications, comprising three systematic reviews, three observational studies, four narrative reviews, and four case reports. The study design ensures a comprehensive evaluation of rituximab's potential efficacy in treating IgG4-RD and its associated clinical characteristics. Based on this study, it can be concluded that IgG4-RD can potentially be treated with rituximab, particularly in cases of relapse and maintaining remission.
PubMed: 37701160
DOI: 10.7759/cureus.45044 -
Frontiers in Oncology 2023Biliary tract cancer (BTC) is a malignancy associated with unfavorable outcomes. Advanced BTC patients have a propensity to experience compromised immune and nutritional... (Review)
Review
BACKGROUND
Biliary tract cancer (BTC) is a malignancy associated with unfavorable outcomes. Advanced BTC patients have a propensity to experience compromised immune and nutritional status as a result of obstructive jaundice and biliary inflammation. Currently, there is a lack of consensus on the impact of the Controlling Nutritional Status (CONUT) score in the context of BTC prognosis. The purpose of this study is to conduct a meta-analysis on the association between CONUT and the prognosis of patients suffering from BTC.
METHODS
A defined search strategy was implemented to search the PubMed, Embase, and Web of Science databases for eligible studies published until March 2023, with a focus on overall survival (OS), relapse-free survival/recurrence-free survival(RFS), and relevant clinical characteristics. The prognostic potential of the CONUT score was evaluated using hazard ratios (HRs) or odds ratios (ORs) with 95% confidence intervals (CIs).
RESULTS
In this meta-analysis, a total of 1409 patients from China and Japan were involved in 9 studies. The results indicated that the CONUT score was significantly correlated with worse OS (HR=2.13, 95% CI 1.61-2.82, <0.0001) and RFS (HR=1.83, 95% CI 1.44-2.31, <0.0001) in patients with BTC. And, the analysis showed that a high CONUT score was significantly associated with clinical characteristics such as jaundice (OR=1.60, 95% CI=1.14-2.25, =0.006), poorly differentiated tumor (OR=1.43, 95% CI=1.03-1.99, =0.03), pT3 and 4 stage of the tumor (OR=1.87, 95% CI=1.30-2.68, =0.0007), and complications of Clavien-Dindo classification grade IIIa or higher (OR=1.79, 95% CI=1.03-3.12, =0.04).
CONCLUSION
This meta-analysis indicates that a high CONUT score can serve as a significant prognostic indicator for survival outcomes among patients diagnosed with BTC.
PubMed: 37664041
DOI: 10.3389/fonc.2023.1240008 -
Heliyon Jul 2023Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a genetic disorder caused by a structural abnormality in the enzyme. G6PD deficiency is most prevalent among... (Review)
Review
BACKGROUND
Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a genetic disorder caused by a structural abnormality in the enzyme. G6PD deficiency is most prevalent among African, Asian, and Mediterranean people. This study aimed to investigate how prevalent G6PD deficiency is in African neonates with jaundice.
METHODS
The public sources, such as PubMed, Science Direct, Google Scholar, and Africa Journal Online were searched for articles that reported the prevalence of G6PD deficiency published before March 21st, 2022. The Joanna Briggs Institute's (JBI) critical assessment checklist was used to evaluate the quality of individual studies. STATA-17 was used to do the statistical analysis. The pooled prevalence of G6PD deficiency in neonates with jaundice in Africa was calculated using a forest plot and a random effects model. I statistics and Galbraith plots were used to assess heterogeneity. Publication bias was assessed using a funnel plot and Egger's statistical test.
RESULTS
Ten studies involving 1555 neonates with jaundice were involved in the study. G6PD deficiency was prevalent in 24.60% of African neonates with jaundice (95% CI:12.47-36.74) with considerable heterogeneity (I = 100%). Nigerian neonates with jaundice had the highest G6PD deficiency (49.67%), whereas South Africans had the lowest (3.14%).
CONCLUSION
G6PD deficiency has been implicated in a significant portion of African neonates with jaundice, notwithstanding the need for greater research on predisposing variables from other countries. Therefore, it should be thought of performing screening and diagnostic laboratory tests for G6PD deficiency.
PubMed: 37539282
DOI: 10.1016/j.heliyon.2023.e18437 -
AJOG Global Reports Aug 2023This study aimed to assess the prognostic accuracies of Doppler ultrasound measures in predicting adverse perinatal outcomes for pregnancies complicated with preexisting... (Review)
Review
OBJECTIVE
This study aimed to assess the prognostic accuracies of Doppler ultrasound measures in predicting adverse perinatal outcomes for pregnancies complicated with preexisting or gestational diabetes mellitus.
DATA SOURCES
An online database search of MEDLINE, Cochrane, Embase, CINAHL, Scopus, and Emcare from inception to April 2022 was conducted.
STUDY ELIGIBILITY CRITERIA
Studies reporting singleton, nonanomalous fetuses of women with either preexisting (type 1 or 2 diabetes mellitus) or gestational diabetes mellitus during pregnancy were included. In addition, the included studies assessed cerebroplacental ratio and middle cerebral artery and/or umbilical artery pulsatility index in the prediction of either: preterm birth, cesarean delivery for fetal distress, APGAR (Appearance, Pulse, Grimace, Activity, and Respiration) score <7 at 5 minutes, neonatal intensive care unit admission (>24 hours), acute respiratory distress syndrome, jaundice, hypoglycemia, hypocalcemia, or neonatal death.
METHODS
The PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines were followed and 610 articles were identified, of which 15 were included. Two authors independently extracted prognostic data from each article and assessed the study applicability and risk of bias using the QUADAS-2 (Quality Assessment of Diagnostic Accuracy Studies-2) scoring criteria.
RESULTS
A total of 15 studies were included in the review and comprised prospective (n=10; 66%) and retrospective (n=5; 33%) cohorts. Sensitivity and positive predictive values varied widely across each Doppler measurement. Umbilical artery sensitivities were higher than those of cerebroplacental ratio and middle cerebral artery for hypoglycemia, jaundice, neonatal intensive care unit admission, respiratory distress, and preterm birth. Cerebroplacental ratio was the most reported index test; however, prognostic accuracy was worse than that of umbilical artery and middle cerebral artery Doppler across all adverse perinatal outcomes. Significant risk of bias was present in 14 (94%) studies, with substantial heterogeneity observed across studies in terms of study design and outcomes assessed.
CONCLUSION
Abnormal umbilical artery pulsatility index may be of more clinical value in predicting adverse perinatal outcomes compared with cerebroplacental ratio and middle cerebral artery pulsatility index in diabetic pregnancies. Further evaluation of umbilical artery Doppler measurements in diabetic pregnancies using standardized variables across studies is required for broader clinical application. The significant association between abnormal Doppler measurement and hypoglycemia may warrant further investigation.
PubMed: 37396341
DOI: 10.1016/j.xagr.2023.100241 -
European Journal of Pediatrics Sep 2023Neonatal jaundice is a common clinical condition that can progress to severe hyperbilirubinemia if identification and intervention are delayed. In this study, we aimed...
Neonatal jaundice is a common clinical condition that can progress to severe hyperbilirubinemia if identification and intervention are delayed. In this study, we aimed to analyze the current evidence on the accurate performance of smartphone applications to quantify bilirubin levels. PubMed, Embase, Emcare, MEDLINE, the Cochrane Library, and Google Scholar were searched from inception until July 2022. Grey literature was searched on "OpenGrey" and "MedNar" databases. We included prospective and retrospective cohort studies that recruited infants with a gestation of ≥ 35 weeks and reported paired total serum bilirubin (TSB) and smartphone app-based bilirubin (ABB) levels. We conducted the review using the guidelines of the Cochrane Collaboration Diagnostic Test Accuracy Working Group and reported using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses-diagnostic test accuracy (PRISMA-DTA) statement. The data were pooled using the random effects model. The outcome of interest was agreement between ABB and TSB measurements, provided as correlation coefficient, mean difference, and standard deviation. Certainty of evidence (COE) was assessed based on GRADE guidelines. Fourteen studies were included in the meta-analysis. The number of infants in individual studies ranged between 35 and 530. The pooled correlation coefficient (r) between ABB and TSB was 0.77 (95% CI 0.69 to 0.83; p < 0.01). Reported sensitivities for predicting a TSB of 250 µmol/L in individual studies ranged between 75 and 100% and specificities ranged from 61 to 100%. Similarly, a sensitivity of 83 to 100% and a specificity of 19.5 to 76% were reported for predicting a TSB of 205 μmol/L. Overall COE was considered moderate. Conclusion: Smartphone app-based bilirubin estimation showed a reasonable correlation to TSB levels. Well-designed studies are required to determine its utility as a screening tool for various TSB cut-off levels. What is Known: • Neonatal jaundice is a common clinical condition. Timely screening and intervention are necessary to prevent neurological morbidities • Transcutaneous bilirubinometer is a widely used non-invasive screening device but is mostly available in hospital settings and has cost limitations. Researchers have recently explored the utility of smartphone applications to estimate bilirubin levels in neonates. What is New: • This is the first systematic review and meta-analysis conducted to assess the performance of smartphone applications to detect neonatal hyperbilirubinemia. • Bilirubin estimates of newborn infants obtained through smartphone applications had a reasonable correlation with serum bilirubin levels.
PubMed: 37368007
DOI: 10.1007/s00431-023-05073-2