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Background: Acute pancreatitis following cardiac surgery is a rare complication. Early recognition of risk factors and vigilance toward initial symptoms can prevent significant morbidity and mortality. Bowel ischemia and the release of inflammatory mediators during cardiopulmonary bypass play key roles in the development of acute postoperative pancreatitis. Management is generally conservative, following the modified Atlanta 2012 criteria. This paper reviews the literature and emphasizes the importance of early investigation of abdominal pain after cardiac surgery. Case presentation: We report a case of severe acute pancreatitis that developed after double valvular replacement, performed during a 3-h cardiopulmonary bypass in a patient who initially had endocarditis and underwent dialysis 3 times. Considering the risk factors, the patient was diagnosed early and managed conservatively, leading to a positive outcome. Conclusions: Anesthesiologists must not disregard abdominal pain after cardiac surgery in the context of risk factors, as it may indicate gastrointestinal complications, including pancreatitis, which can be among the most dangerous complications if not treated promptly. [ABSTRACT FROM AUTHOR]

Background: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease caused by immune hyperactivation. The overall survival (OS) of adults with secondary HLH remains suboptimal and new treatment strategies are needed. Objectives. This study aimed to compare the efficacy of different regimens in the treatment of secondary HLH in adults and analyze the prognostic factors affecting patient survival. Materials and methods: The clinical data of 245 adults with secondary HLH admitted to our hospital from January 2016 to October 2021 were analyzed retrospectively. The patients were divided into 3 groups according to different treatment regimens: corticosteroids therapy + chemotherapy + supportive treatment group (JHZ group), chemotherapy + supportive treatment group (HZ group) and corticosteroids therapy + supportive treatment group (JZ group). The clinical efficacy was compared among the 3 groups after treatment, and progression-free survival (PFS) and overall survival (OS) were calculated. Additionally, risk factors associated with prognosis were also analyzed with Cox regression analysis. Results: The objective response rate (ORR) in the JHZ group was higher than that in the HZ group and JZ group, but there was no significant difference between the 3 groups. Also, the patients in the JHZ group had the longest OS and median PFS. Further Cox regression analysis suggested that hyperbilirubinemia was an independent risk factor for OS in secondary HLH patients. Conclusions: A combination of corticosteroids therapy, chemotherapy and supportive therapy is superior to the other 2 regimens in the clinical benefit in the treatment of secondary HLH in adults, and thus may be a preferred and feasible treatment regimen. Moreover, hyperbilirubinemia was a risk factor for prognosis that has crucial guiding significance for clinical treatment of patients with secondary HLH. [ABSTRACT FROM AUTHOR]

Crigler–Najjar Syndrome (CNS) is a rare genetic disorder caused by mutations in the UGT1A1 gene, leading to impaired bilirubin conjugation and severe unconjugated hyperbilirubinemia. CNS presents in the following forms: CNS type 1 (CNS1), the more severe form with the complete absence of UGT1A1 activity, and CNS type 2 (CNS2), with partial enzyme activity. This narrative review aims to provide a detailed overview of CNS, highlighting its clinical significance and the need for new, more effective treatments. By summarizing current knowledge and discussing future treatments, this article seeks to encourage further research and advancements that can improve outcomes for CNS patients. The literature analysis showed that CNS1 requires aggressive management, including phototherapy and plasmapheresis, but liver transplantation (LT) remains the only definitive cure. The timing of LT is critical, as it must be performed before the onset of irreversible brain damage (kernicterus), making early intervention essential. However, LT poses risks such as graft rejection and lifelong immunosuppression. CNS2 is milder, with patients responding well to phenobarbital and having a lower risk of kernicterus. Recent advancements in gene therapy and autologous hepatocyte transplantation offer promising alternatives to LT. Gene therapy using adeno-associated virus (AAV) vectors has shown potential in preclinical studies, though challenges remain in pediatric applications due to liver growth and pre-existing immunity. Autologous hepatocyte transplantation avoids the risk of rejection but requires further research. These emerging therapies provide hope for more effective and less invasive treatment options, aiming to improve the quality of life for CNS patients and reduce reliance on lifelong interventions. [ABSTRACT FROM AUTHOR]

Background: Infantile jaundice affects up to 84% of term infants and is a primary cause of re-hospitalization. Phototherapy, the primary therapeutic intervention, may be associated with several complications. Therefore, employing additional compensatory therapies could reduce the duration of phototherapy and its adverse effects. Methods: A single-blind randomized clinical trial was conducted on newborns with indirect hyperbilirubinemia (IHB) undergoing phototherapy. Patients were randomly assigned to two groups: the intervention group received phototherapy and 10mg/kg of Ursodeoxycholic acid, taken twice a day with water as the solvent, while the control group received phototherapy and a placebo (water). Total bilirubin levels were measured every 12 hours, and the groups were compared regarding the reduction duration of bilirubin <10 mg/dl and the duration of phototherapy. Data analysis used SPSS-V22 software, with statistical tests done at a significance level of <0.05. Results: In total, 128 neonates, comprising 56 (43.8%) males and 72 (56.2%) females, with a mean gestational age of 39.02±0.86 weeks and a mean age of 2.77±1.45 days, were subjected to analysis. After 96 hours, the mean bilirubin level was 9.23±2.81 mg/dl overall, 7.27±2.12 mg/dl in the case group, and 10.91±2.17 mg/dl in the control group (p<0.001). The mean time to reach a bilirubin level below 10 mg/dl was 51.66±17.27 hours overall, 58.38±10.05 hours for the case group, and 62.61±14.39 hours for the control group (p<0.001). Conclusion: Ursodeoxycholic acid (UDCA) effectively reduces elevated bilirubin levels and the duration of phototherapy when employed as an adjunct treatment to phototherapy in cases of infantile indirect hyperbilirubinemia. [ABSTRACT FROM AUTHOR]

Background: Neonatal jaundice is a commonly seen clinical condition globally, including in India seen in nearly 60% of subjects born at term and 80% of neonates born pre-term in their first week of life and is the most common cause of readmission in neonates. Aim: The present study aimed to assess the predictive value of umbilical cord blood albumin level for developing neonatal jaundice in healthy neonates born at term and the usefulness of cord blood albumin for predicting subsequent significant neonatal jaundice development. Materials and Methods: The present study assessed 200 healthy term neonates where cord blood albumin level was estimated at birth. Estimation of total serum bilirubin was done for all the neonates at birth. Estimation of total serum bilirubin was done in all the neonates with clinically suspected jaundice at the age of 72-96 hours. The neonates were divided into three groups I, II, and III depending on cord albumin levels of <2.8g/dL, 2.8-3.3 g/dL, and >3.3 g/dL respectively. The main outcome assessed was concerning serum bilirubin =17 mg/dL, exchange transfusion, and the need for phototherapy. Results: Groups I, II, and III had 42, 70, and 88 newborns respectively. In Group I, 85.7% (n=36) neonates had total bilirubin of >17 mg/dL, among which 76.19% (n=32) needed phototherapy, and 9.52% (n=4) subjects required exchange transfusion. In Group II. 65.7% (n=46) neonates developed jaundice whereas phototherapy was needed in 34.2% (n=24) neonates and no subjects needed exchange transfusion. In Group III, 34.09% (n=30) neonates developed jaundice here 2.2% (n=2) subjects needed phototherapy, and no subjects needed exchange transfusion which depicted a statistically significant difference with p<0.001. Conclusion: The present study concludes that cord blood albumin level of =2.8g/dL is a significant risk factor for the development of neonatal hyperbilirubinemia that needs early intervention, whereas, cord blood albumin >3.3 g/dL is usually the safe criteria for early discharge. [ABSTRACT FROM AUTHOR]

Hemolytic disease of the fetus and newborn (HDFN) results from the destruction of the newborn's red blood cells or the fetus by the mother's immune globulin G antibodies. Although HDFN is often caused by RhD and ABO incompatibility, it can also be seen due to minor blood groups such as Kell, Kidd, Duffy, P, MNS, or Rh subgroup (C, c, E, e) alloantibodies. Our study aims to share our experiences regarding a rare cause of HDFN. The files of patients who were followed up with the diagnosis of jaundice in a third-level Neonatology Unit of a university hospital between January 2014 and September 2023 were retrospectively examined. Eleven patients with Kell incompatibility were included in the study. There was no ABO/RhD incompatibility in any case. RhD subgroup incompatibility and Kell incompatibility were present in four cases. Phototherapy was applied to all patients. The patient, whose total bilirubin level was high despite phototherapy, was treated with an exchange transfusion. No complications were observed due to treatment or high total bilirubin. No significant difference was observed between the parameters evaluated according to the gender variable. It should be kept in mind that severe hemolysis and related deaths may occur due to Kell incompatibility. In order to reduce and prevent severe hemolysis due to Kell and other minor blood groups in newborns, transfusing blood products suitable for minor blood groups to women of childbearing age (especially pregnant women) may be a correct approach as a country policy. [ABSTRACT FROM AUTHOR]

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Introduction: Gestational Diabetes Mellitus (GDM), a common medical complication of pregnancy, is an independent risk factor for obstetric complications and adverse perinatal outcomes. Insulin has been the treatment modality for many years. Recent studies indicate that metformin, an oral hypoglycaemic agent, may be a safe and effective alternative. Aim: To evaluate and compare the effectiveness of metformin and insulin in managing glycaemic control, and assessing maternal and newborn outcomes among women diagnosed with GDM. Materials and Methods: The present prospective cohort study was conducted in the Department of Obstetrics, Fernandez Hospital, Hyderabad, Telangana, India, from December 2020 to November 2021. The study population included women attending antenatal check-ups after meeting the inclusion and exclusion criteria. Those who failed to respond to lifestyle modifications were started on either metformin or insulin as per the attending physician’s advice. A total of 369 pregnant women were included in the study. Parameters such as weight gain during pregnancy, need for additional pharmacologic treatment, maternal hypoglycaemia, onset of Hypertensive Disorders of Pregnancy (HDP), gestational age at delivery, mode of delivery and neonatal details such as birth weight, occurrence of Large for Gestational Age (LGA), Appearance, Pulse, Grimace, Activity and Respiration (APGAR) score, birth injuries, neonatal hypoglycaemia, hyperbilirubinemia, respiratory distress, and neonates requiring Neonatal Intensive Care Unit (NICU) admissions were recorded. Data were subjected to statistical analysis. Analysis was performed using Statistical Package for the Social Sciences (SPSS) software version 22.0. A p-value of

Background and Objectives: Double-volume exchange transfusion (DVET) is a life-saving procedure that replaces a neonate’s blood volume twice over with adult donor blood to treat neonatal hyperbilirubinemia and prevent kernicterus. This study assessed DVET’s effectiveness in reducing total serum bilirubin (TSB) concentration in neonates at risk of kernicterus and examined the disease conditions that necessitate DVET. Methods: This observational study assessed the effectiveness of DVET in 104 neonates at risk of kernicterus. Neonates meeting the American Academy of Pediatrics guidelines were selected. Blood samples were collected before and 24 h after DVET for TSB estimation and complete blood count analysis. Blood components were selected based on ABO, Rh, and other minor blood group incompatibility. DVET was performed using the push–pull method. Results: This study of DVET in 104 neonates with hyperbilirubinemia found that DVET is an effective treatment, reducing TSB levels by 61.72% on average. The most common cause of hyperbilirubinemia was idiopathic (25.6%), followed by ABO incompatibility (22.12%) and anti-D alloimmunization (17.31%). In hypothyroid, preterm, and sepsis cases, hemoglobin levels decreased by an average of 17.7%, 12.9%, and 13.9%, respectively, after DVET. However, in cases of minor alloimmunization and anti-D alloimmunization, hemoglobin levels increased by an average of 29% and 40.5%, respectively, after DVET. The maximum decrease in TSB level was observed in cases of ABO incompatibility with sepsis (82%), while the minimum decrease was seen in neonates undergoing DVET due to sepsis (50%). The percentage reduction of TSB level after DVET was similar for different etiologies, ranging from 50.2% to 65.4%. Conclusion: DVET is a rarely used procedure in the age of phototherapy and intravenous immunoglobulin for neonatal hyperbilirubinemia. However, it is the only effective treatment for very high bilirubin levels to prevent kernicterus or bilirubin encephalopathy.

Abstract Background Acute pancreatitis following cardiac surgery is a rare complication. Early recognition of risk factors and vigilance toward initial symptoms can prevent significant morbidity and mortality. Bowel ischemia and the release of inflammatory mediators during cardiopulmonary bypass play key roles in the development of acute postoperative pancreatitis. Management is generally conservative, following the modified Atlanta 2012 criteria. This paper reviews the literature and emphasizes the importance of early investigation of abdominal pain after cardiac surgery. Case presentation We report a case of severe acute pancreatitis that developed after double valvular replacement, performed during a 3-h cardiopulmonary bypass in a patient who initially had endocarditis and underwent dialysis 3 times. Considering the risk factors, the patient was diagnosed early and managed conservatively, leading to a positive outcome. Conclusions Anesthesiologists must not disregard abdominal pain after cardiac surgery in the context of risk factors, as it may indicate gastrointestinal complications, including pancreatitis, which can be among the most dangerous complications if not treated promptly.

Background/Aims: Bile duct invasion (BDI) is rarely observed in patients with advanced hepatocellular carcinoma (HCC), leading to hyperbilirubinemia. However, the efficacy of pretreatment biliary drainage for HCC patients with BDI and obstructive jaundice is currently unclear. Thus, the aim of this study was to assess the effect of biliary drainage on the prognosis of these patients. Methods: We retrospectively enrolled a total of 200 HCC patients with BDI from multicenter cohorts. Patients without obstructive jaundice (n=99) and those who did not undergo HCC treatment (n=37) were excluded from further analysis. Finally, 64 patients with obstructive jaundice (43 subjected to drainage and 21 not subjected to drainage) were included. Propensity score matching was then conducted. Results: The biliary drainage group showed longer overall survival (median 10.13 months vs 4.43 months, p=0.004) and progression-free survival durations (median 7.00 months vs 1.97 months, p

Abstract This study aimed to assess the magnitude of hematological toxicity and associated factors in newborns with hyperbilirubinemia. A cross-sectional study was conducted from April to December 2023. A total of 247 newborns were included. The data were collected using questionnaires and a data extraction sheet. Four 4 ml of blood was collected. A Sysmex KX-21 analyzer was used for blood analysis, and a Mindray BS-240 analyzer was used for bilirubin measurement. The data were entered into Epi-data and analyzed by SPSS. The logistic regression was used. The P value was set at 0.05. Before phototherapy, the hematological toxicities, such as anemia, leucopenia, and thrombocytopenia, were 45.7%, 22.2%, and 6.1%, respectively, whereas after phototherapy, anemia and thrombocytopenia, significantly increased, but the leucopenia, significantly decreased. The risk of developing anemia increased, 3.5, 2.7, and 2.1-fold among newborns with bilirubin > 18 mg/dl, with Rh blood group incompatibility, and treated with intensive phototherapy, respectively. Both low birth weight and intensive phototherapy increased the incidence of thrombocytopenia by 2 and 3.4-fold, respectively. Hematological toxicity was found to be a severe public health issue in newborns. Thus, strict follow-up and early detection of toxicity by considering aggravation factors are necessary.

Background: As hyperbilirubinemia is one of the key contributors to brain damage and is crucial to identify those instances that are at risk as it is more prevalent and severe among the preterms. Many studies are available in the literature for terms babies but there is paucity of data for pre terms. Aims and Objectives: The aim of this study was to assess serum bilirubin at 24 h of live birth as a indicator of significant hyperbilirubinemia in preterm neonates. Materials and Methods: It was a prospective observational study conducted in the department of pediatrics of tertiary care center, Jodhpur on 150 preterms born within 33–36 weeks 6 days after considering the exclusion criteria in the study. Area under the receiver operating characteristic (ROC) curve was used to identify significant hyperbilirubinemia within 1 week of life, along with it the cutoff value of total serum bilirubin (TSB) at 24 h of life was calculated. Results: A total of 150 enrolled preterm newborns were followed up. TSB at 24 h of life was proved to be significant using the ROC curve, where area under the curve is 0.96 which was statistically significant. Among 150 preterms, 70 newborns with TSB >4.2 mg/dL developed jaundice, and 65 babies (98.48%) required phototherapy. Hence, preterms with TSB >4.2 mg/dL have a significant risk of developing hyperbilirubinemia. (Sensitivity: 98.48%, specificity: 94.05%, negative predictive value: 98.75%, and positive predictive value: 92.86%). Conclusion: TSB for preterms at 24 h of life can support to predict the significant hyperbilirubinemia successfully. Preterms with cutoff value of serum bilirubin level >4.2 mg/dL are observed to be more prone to develop significant hyperbilirubinemia requiring appropriate intervention at the earliest.

Abstract Liver-related side effects are a known complication of treatment with elexacaftor/tezacaftor/ivacaftor (ETI) for cystic fibrosis (CF). Gilbert’s syndrome is caused by a genetic mutation that reduces activity of the enzyme UDP glucuronosyltransferase 1 polypeptide A1 (UGT1A1), causing elevated levels of unconjugated bilirubin in the blood and duodenal bile. The presence of Gilbert’s syndrome and CF might represent additive risk factors for liver-related adverse events during ETI treatment. This case series describes six people with CF (pwCF) in whom previously unknown Gilbert’s syndrome was unmasked after initiation of treatment with ETI. Although all patients had some level of hepatic dysfunction and/or elevated levels of bilirubin after initiation of ETI, the clinical course varied. Only one patient had to stop ETI therapy altogether, while the others were able to continue treatment (some at a reduced dosage and others at the full recommended daily dosage). All patients, even those using a lower dosage, experienced clinical benefit during ETI therapy. Gilbert’s syndrome is not a contraindication for ETI therapy but may be mistaken for a risk factor for liver-related adverse events in pwCF. This is something that physicians need to be aware of in pwCF who show liver adverse events during ETI therapy.

Samira Said Lançoni,1,2 Jocilene Pedroso Albuquerque,1,3 Adriane Muller Nakato,2 Gislayne Castro e Souza de Nieto,4 Cláudio Marcelo Tkac,5 Marcos Fabiano Sigwalt,1,3 Percy Nohama,2 Larissa Riskalla Talamini Souto,2 Paulo Afonso Nunes Nassif1,3 1Graduate Program in Principles of Surgery, Faculdade Evangélica Mackenzie do Paraná, Curitiba, Paraná, Brasil; 2Graduate Program on Health Technology, Pontifícia Universidade Católica do Paraná, Curitiba, Paraná, Brazil; 3Hospital Universitário Evangélico Mackenzie, Curitiba, Paraná, Brasil; 4Department of Pediatrics, Universidade Positivo, Curitiba, Paraná, Brazil; 5School of Medicine and Life Sciences, Pontifícia Universidade Católica do Paraná, Curitiba, Paraná, BrazilCorrespondence: Samira Said Lançoni, Graduate Program on Health Technology, Pontifícia Universidade Católica do Paraná Rua Imaculada Conceição 1155, Curitiba, Paraná, CEP 80215-030, Brazil, Tel +55 41 99977-8255, Email samiraslanconi@hotmail.comPurpose: To analyze leptin levels in placental tissue and premature infants undergoing phototherapy and to evaluate the potential for prescribing passive exercise after phototherapy in this population.Patients and Methods: This analytical, longitudinal, prospective cohort study included 108 parturients and their respective premature infants. Variables examined included weight, gestational age, body mass index, sex, serum leptin levels in placental tissue, serum bilirubin levels, and reticulocyte count.Results: When comparing each group to a leptin threshold, statistically significant differences were observed at all evaluated time points for placental leptin levels (p < 0.001). Additionally, reticulocyte count decreased in relation to rebound time (p < 0.004). No correlations were found between leptin/bilirubin levels, leptin/reticulocytes, onset of nutrition, and BMI/leptin levels.Conclusion: The findings regarding leptin levels suggest that prescribing passive exercises to premature infants undergoing phototherapy may be feasible because this intervention did not increase leptin levels.Keywords: neonatal intensive care unit, prematurity, leptin, hyperbilirubinemia, child obesity

Background/Objectives: Intrahepatic cholestasis of pregnancy (ICP) is characterised by unexplained intense pruritus during pregnancy. While serum bile acid (BA) is the standard diagnostic marker for ICP, we explored the potential of serum calprotectin as an alternative diagnostic marker for ICP. Methods: Leftover serum specimens with known serum BA levels, collected from non-pregnant women and pregnant women with an ICP, were used to measure serum calprotectin levels using the Human calprotectin L1/S100-A8/A9 ELISA kit. Results: Serum calprotectin levels were measured in 79 pregnant women with ICP (median [interquartile range] 28 year; serum BA 20 [13.7–35.7] μMol/L; calprotectin159 pg/mL [122.2–212.3]); 43 pregnant women without ICP (age 28 years; serum BA 3.6 [2.1–5.8] μMol/L; calprotectin 146.5 pg/mL [75.8–194.8]), and 59 non-pregnant women (age 28 years; serum BA 3.5 [1.6–5.1 μMol/L; calprotectin 82.4 pg/mL [48.8–137.2]). Compared to non-pregnant women, calprotectin levels were significantly elevated among pregnant women with (p < 0.001) or without ICP (p = 0.01). Calprotectin levels were comparable between pregnant women with and without ICP (p = 0.15). The areas under the ROC curve, to differentiate the presence and absence of ICP, were 0.940 (0.903–0.977; p < 0.001) and 0.681 (0.604–0.759; p < 0.001) for BA and calprotectin, respectively. Conclusions: Serum calprotectin is raised in pregnant women regardless of the presence or absence of ICP and had an inferior diagnostic performance for ICP compared to BA. This information is crucial for understanding the challenges in ICP diagnosis and the limitations of serum calprotectin as an alternative marker. [ABSTRACT FROM AUTHOR]

Background: Maintaining the dose-intensity of cancer treatment is an important prognostic factor of aggressive hematological malignancies. The objective of this study was to assess the long-term outcomes of intensive care unit (ICU) survivors with acute myeloid leukemia (AML) or aggressive B-cell non-Hodgkin lymphoma (B-NHL) with emphasis on the resumption of the intended optimal regimen of cancer treatment. Patients and methods: We conducted a retrospective (2013–2021) single-center observational study where we included patients with AML and B-NHL discharged alive from the ICU after an unplanned admission. The primary endpoint was the change in the intended optimal cancer treatment following ICU discharge. Secondary endpoints were 1-year progression-free survival and overall survival rates. Determinants associated with modifications in cancer treatment were assessed through multivariate logistic regression. Results: Over the study period, 366 patients with AML or B-NHL were admitted to the ICU, of whom 170 survivors with AML (n = 92) and B-NHL (n = 78) formed the cohort of interest. The hematological malignancy was recently diagnosed in 68% of patients. The admission Sequential Organ Failure Assessment (SOFA) score was 5 (interquartile range 4–8). During the ICU stay, 30 patients (17.6%) required invasive mechanical ventilation, 29 (17.0%) vasopressor support, and 16 (9.4%) renal replacement therapy. The one-year survival rate following ICU discharge was 59.5%. Further modifications in hematologic treatment regimens were required in 72 patients (42%). In multivariate analysis, age > 65 years (odds ratio (OR) 3.54 [95%-confidence interval 1.67–7.50], p < 0.001), ICU-discharge hyperbilirubinemia > 20 µmol/L (OR 3.01 [1.10–8.15], p = 0.031), and therapeutic limitations (OR 16.5 [1.83–149.7], p = 0.012) were independently associated with modifications in cancer treatment. Post-ICU modifications of cancer treatment had significant impact on in-hospital, 1-year overall survival and progression-free survival. Conclusion: The intended cancer treatment could be resumed in 58% of ICU survivors with aggressive hematological malignancies. At the time of ICU discharge, advanced age, persistent liver dysfunction and decisions to limit further life-support therapies were independent determinants of cancer treatment modifications. These modifications were associated with worsened one-year outcomes. [ABSTRACT FROM AUTHOR]

Introduction: Hepatic visceral crisis (VC), characterized by a rapid total bilirubin increase with disease progression, poses a life-threatening risk in advanced breast cancer (ABC). International consensus guidelines define VC and touch on impending VC (IVC). Limited data exist on systemic treatments for hepatic VC/IVC. This study explores the safety and efficacy of cisplatin monotherapy in patients with Human Epidermal Growth Factor Receptor 2- negative breast cancer (BC) and hepatic IVC/VC. Methods: In this retrospective single-center cohort study data of patients treated with cisplatin monotherapy (60–80 mg/m2, every 3–4 weeks) between 2016 and 2023 at a reference Cancer Centre in Southern Poland were analyzed. Results: 33 female patients (24/33 hormonal-positive) with the mean age 53.84 years were included. Participants progressed on median 2 prior palliative systemic treatment lines. In 10/23 patients hepatic VC and in 23/33 IVC (rapid, symptomatic liver progression; extensive liver involvement; alanine or aspartate aminotransferase > 2 × normal limit; significant increases in lactate dehydrogenase, alkaline phosphatase, or gamma-glutamyl transferase) were identified. Median progression-free survival was 1.87 months and median overall survival 2.67 months. 33% of the patients presented stable disease or partial response. Eight patients experienced adverse events grade ≥ 3: in five the dose of cisplatin was reduced; two stopped the treatment. Conclusion: Due to the hepatotoxicity of BC-active drugs, specific recommendations for systemic treatment are scarce. Our study explored cisplatin's potential use, finding it to be a viable option in patients with performance status 0 or 1 experiencing hepatic IVC/VC, irrespective of liver function parameters and other factors. [ABSTRACT FROM AUTHOR]

Background: Hemolytic disease of the fetus and newborn (HDFN) is caused by maternal antibodies attacking fetal blood cell antigens. Despite routine antenatal anti-D prophylaxis, intrauterine transfusions (IUTs) are still needed in some HDFN cases. Methods: We conducted a retrospective cohort study on newborns with HDFN born in the 1st Department of Obstetrics and Gynecology of the Medical University of Warsaw. We analyzed 274 neonates with HDFN, identifying 46 who required IUT due to fetal anemia and 228 who did not. The laboratory results, management, and outcomes were compared between these groups. Results: Comparative analysis showed that newborns treated with IUT were more likely to have significant anemia, hyperbilirubinemia, and iron overload, indicated by a high ferritin concentration. These neonates more often required top-up transfusions, phototherapy, intravenous immunoglobulin infusions, and exchange transfusions. The length of stay was longer for newborns who received IUT. Conclusions: HDFN requiring IUT is associated with a greater number of complications in the neonatal period and more often requires additional treatment compared to HDFN not requiring IUT. [ABSTRACT FROM AUTHOR]

Background: As hyperbilirubinemia is one of the key contributors to brain damage and is crucial to identify those instances that are at risk as it is more prevalent and severe among the preterms. Many studies are available in the literature for terms babies but there is paucity of data for pre terms. Aims and Objectives: The aim of this study was to assess serum bilirubin at 24 h of live birth as a indicator of significant hyperbilirubinemia in preterm neonates. Materials and Methods: It was a prospective observational study conducted in the department of pediatrics of tertiary care center, Jodhpur on 150 preterms born within 33-36 weeks 6 days after considering the exclusion criteria in the study. Area under the receiver operating characteristic (ROC) curve was used to identify significant hyperbilirubinemia within 1 week of life, along with it the cutoff value of total serum bilirubin (TSB) at 24 h of life was calculated. Results: A total of 150 enrolled preterm newborns were followed up. TSB at 24 h of life was proved to be significant using the ROC curve, where area under the curve is 0.96 which was statistically significant. Among 150 preterms, 70 newborns with TSB >4.2 mg/dL developed jaundice, and 65 babies (98.48%) required phototherapy. Hence, preterms with TSB >4.2 mg/dL have a significant risk of developing hyperbilirubinemia. (Sensitivity: 98.48%, specificity: 94.05%, negative predictive value: 98.75%, and positive predictive value: 92.86%). Conclusion: TSB for preterms at 24 h of life can support to predict the significant hyperbilirubinemia successfully. Preterms with cutoff value of serum bilirubin level >4.2 mg/dL are observed to be more prone to develop significant hyperbilirubinemia requiring appropriate intervention at the earliest. [ABSTRACT FROM AUTHOR]

Background: Data from birth registries can be studied to assess the prevalence of prenatal alcohol use and associated maternal and neonatal outcomes. Methods: Linked maternal and neonatal data (2015–2018) for alcohol-exposed pregnancies were obtained from the Better Outcomes Registry and Network (BORN) Ontario. Descriptive statistics were generated for maternal demographics, prenatal substance use, mental health/substance use history, and neonatal outcomes. Logistic regression models were performed to assess the odds of prenatal heavy (binge or weekly) alcohol and other substance use based on mental health/substance use history and other maternal demographics, and the impacts of heavy alcohol use and other prenatal substance exposures on neonatal outcomes. Results: A total of 10,172 (2.4%) women reported alcohol use during pregnancy. One-third had pre-existing or current mental health and/or substance use problems, which was associated with significantly higher odds of heavy alcohol use during pregnancy. Prenatal exposure to heavy alcohol use was associated with increased odds of neonatal abstinence syndrome (2.5 times); respiratory distress syndrome (2.3 times); neonatal intensive care unit (NICU) admission (58%); and hyperbilirubinemia (57%). Prenatal exposure to one or more substances in addition to alcohol was associated with significantly higher odds of fetal/maternal/placental pregnancy complications; preterm birth; NICU admission; low APGAR scores; one or more confirmed congenital anomalies at birth; respiratory distress syndrome; and intrauterine growth restriction. Conclusions: It is crucial to routinely screen childbearing-age and pregnant women for alcohol and other substance use as well as mental health problems in order to prevent adverse maternal and neonatal outcomes. [ABSTRACT FROM AUTHOR]

Background: Niemann-Pick disease type C poses a significant challenge within the landscape of rare genetic disorders, marked by its connection to variants in the NPC1 or NPC2 genes. This autosomal recessive lipid storage disorder unfolds with a relentless progression of neurological deterioration and a distinctive hallmark of hepatosplenomegaly. Case presentation: This case report delves into the intricate presentation of a 9-year-old Iraqi boy exhibiting heightened walking instability and speech slurring. His medical history unfolds a series of challenges, including neonatal hyperbilirubinemia, hepatosplenomegaly, and recurrent nasal bleeding. A comprehensive physical examination reveals motor and neurological abnormalities such as an inability to squat and rise, vertical gaze palsy, and dysdiadochokinesia. Further investigations, encompassing laboratory tests and imaging studies, coupled with the identification of foamy cells in bone marrow smears, raise significant concerns about Niemann-Pick disease type C. By utilizing whole exome sequencing, we pinpointed a previously unreported homozygous variant—c.2925_2928delCTGC; p.Cys976PhefsTer6—found within exon 20 (NM_000271.5) of the proband's NPC1 gene. Conclusions: This study significantly advances our understanding of the c.2925_2928del (C976Ffs*6) variant in the NPC1 gene, shedding light on the complexities of Niemann-Pick disease type C. Beyond its scientific significance, the findings provide crucial insights for familial genetic counseling and prenatal diagnoses. This research expands our knowledge of the variant's genetic landscape, making it a valuable resource in both academic and clinical settings, particularly for families dealing with Niemann-Pick disease type C. [ABSTRACT FROM AUTHOR]