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World Journal of Clinical Pediatrics Jun 2024Acute fulminant liver failure rarely occurs in the neonatal period. The etiologies include viral infection (15%), metabolic/genetic disease (10%), hematologic disorders...
BACKGROUND
Acute fulminant liver failure rarely occurs in the neonatal period. The etiologies include viral infection (15%), metabolic/genetic disease (10%), hematologic disorders (15%), and ischemic injury (5%). Gestational alloimmune liver disease usually manifests as severe neonatal liver failure, with extensive hepatic and extrahepatic iron overload, sparing the reticuloendothelial system. Empty liver failure is a rare cause of liver failure where a patient presents with liver failure in the neonatal period with no hepatocytes in liver biopsy.
CASE SUMMARY
A 5-week-old male presented with jaundice. Physical examination revealed an alert but deeply icteric infant. Laboratory data demonstrated direct hyperbilirubinemia, a severely deranged coagulation profile, normal transaminase, and normal ammonia. Magnetic resonance imaging of the abdomen was suggestive of perinatal hemochromatosis. Liver biopsy showed histiocytic infiltration with an absence of hepatocytes. No hemosiderin deposition was identified in a buccal mucosa biopsy.
CONCLUSION
Neonatal liver failure in the absence of hepatocellular regeneration potentially reflects an acquired or inborn defect in the regulation of hepatic regeneration.
PubMed: 38947999
DOI: 10.5409/wjcp.v13.i2.92263 -
Endoscopic Ultrasound 2024Endoscopic treatment of obstructive jaundice and pancreatitis due to hepaticojejunostomy (H-J), pancreatojejunostomy (P-J) strictures, and tumor recurrence after...
BACKGROUND AND OBJECTIVES
Endoscopic treatment of obstructive jaundice and pancreatitis due to hepaticojejunostomy (H-J), pancreatojejunostomy (P-J) strictures, and tumor recurrence after pancreatoduodenectomy (PD) is technically challenging. Treatment of P-J strictures results in poor outcomes. Although conventional EUS that has an oblique view is not suitable for such patients, forward-viewing EUS (FV-EUS) may become a useful option. This study aimed to evaluate the feasibility and efficacy of FV-EUS in patients who have undergone PD.
METHODS
Patients with PD who were scheduled to undergo diagnosis and treatment using FV-EUS for H-J or P-J lesions were enrolled in this single-center prospective study. After observation of the P-J and H-J using FV-EUS according to a predetermined protocol, treatment using FV-EUS was performed as needed.
RESULTS
A total of 30 patients were enrolled, and FV-EUS was used to observe P-J and H-J in 24 and 28 patients, respectively. The detection rates of P-J and H-J by endoscopy were 50% (12/24) and 96.4% (27/28), respectively, and by EUS were 70.8% (17/24) and 100% (28/28), respectively. Of these, P-J and H-J were found by endoscopy only after EUS observation in 3 and 1 patient, respectively. The success rates of endoscopic treatment using FV-EUS were 66.7% (2/3), 95.2% (20/21), and 25% (1/4) for benign P-J strictures, benign H-J strictures, and tumor recurrence, respectively.
CONCLUSIONS
Endoscopic treatment using FV-EUS is feasible and effective for patients after PD. Moreover, FV-EUS increases the P-J lesion detection rate by adding EUS observation.
PubMed: 38947114
DOI: 10.1097/eus.0000000000000027 -
Research Square Jun 2024Little research has examined early life risk for symptoms of cognitive disengagement syndrome (CDS) despite a well-established literature regarding co-occurring outcomes...
BACKGROUND
Little research has examined early life risk for symptoms of cognitive disengagement syndrome (CDS) despite a well-established literature regarding co-occurring outcomes (e.g., attention-deficit/hyperactivity disorder). The current study estimated bivariate associations between early life risk factors and CDS in a large and representative sample of U.S. children.
METHODS
We conducted secondary analyses of baseline data from the Adolescent Brain Cognitive Development (ABCD) study (N = 8,096 children, 9-10 years old). Birthing parents reported early life risk factors on a developmental history questionnaire, including parental, prenatal, delivery and birth, and developmental milestone information. They also completed the Child Behavior Checklist, which includes a CDS subscale that was dichotomized to estimate the odds of elevated CDS symptoms (i.e., score > 70) in children related to risk indices.
RESULTS
We observed significantly elevated odds of CDS related to parental risk factors (i.e., unplanned pregnancy, pregnancy awareness after 6 weeks, teenage parenthood), birthing parent illnesses in pregnancy (i.e., severe nausea, proteinuria, pre-eclampsia/toxemia, severe anemia, urinary tract infection), pregnancy complications (i.e., bleeding), prenatal substance exposures (i.e., prescription medication, tobacco, illicit drugs), delivery and birth risk factors (i.e., child blue at delivery, child not breathing, jaundice, incubation after delivery), and late motor and speech milestones in children.
CONCLUSIONS
Several early-life risk factors were associated with elevated odds of CDS at ages 9-10 years; study design prevents the determination of causality. Further investigation is warranted regarding early life origins of CDS with priority given to risk indices that have upstream commonalities (i.e., that restrict fetal growth, nutrients, and oxygen).
PubMed: 38947040
DOI: 10.21203/rs.3.rs-4468007/v1 -
Cureus May 2024Acute lymphoblastic leukemia (ALL) is the most prevalent pediatric malignancy, accounting for approximately 25% of childhood cancers. Despite significant advancements in...
Acute lymphoblastic leukemia (ALL) is the most prevalent pediatric malignancy, accounting for approximately 25% of childhood cancers. Despite significant advancements in treatment protocols, ALL remains a complex disease, often presenting with various complications, including the rare metabolic disturbance of type B lactic acidosis. This case report details the clinical journey of a 14-year-old female with ALL who developed type B lactic acidosis during treatment. The patient presented with intermittent fever, abdominal pain, jaundice, and hepatosplenomegaly, accompanied by severe anemia and thrombocytopenia. Initial management included supportive care and chemotherapy initiation. Despite aggressive interventions, the patient's condition deteriorated, with escalating lactic acidosis and respiratory distress, leading to a critical need for tailored management strategies. This report underscores the importance of early recognition and comprehensive management of type B lactic acidosis in pediatric ALL, highlighting its multifactorial etiology and potentially life-threatening consequences. Enhanced clinical awareness and a multidisciplinary approach are crucial for improving outcomes in such complex cases.
PubMed: 38939285
DOI: 10.7759/cureus.61201 -
DEN Open Apr 2025A 79-year-old Japanese woman, who had undergone pancreaticoduodenectomy 6 months prior to presentation owing to pancreatic cancer, complained of jaundice with high...
A 79-year-old Japanese woman, who had undergone pancreaticoduodenectomy 6 months prior to presentation owing to pancreatic cancer, complained of jaundice with high fever. Computed tomography revealed proximal bile duct dilatation with complete hepaticojejunostomy anastomotic stricture (HJAS). We performed a single-balloon endoscopy for biliary drainage. The presence of a scar-like feature surrounding the anastomosis was identified as the HJAS. White-light imaging during single-balloon endoscopy revealed that the HJAS contained a milky whitish area (MWA), suggesting that a membranous and fibrosis layer affected continuous inflammation around the center of the anastomosis (within a scar-like feature). Endoscopic dilatation was performed using an endoscopic injection needle, with the MWA used as an indicator. A 23-gauge endoscopic injection needle was used to penetrate the center of the blind lumen within the MWA, and a pinhole was created in the stricture. After confirming the position of the proximal bile duct using a contrast medium with the needle, an endoscopic guidewire with a cannula was inserted into the pinhole. A through-the-scope sequential balloon dilator was used to dilate the stricture, and a plastic stent was inserted into the proximal bile duct. This endoscopic intervention led to positive outcomes. In cases of complete HJAS occlusion, an endoscopic approach to the bile duct is difficult because the anastomotic opening of the HJAS is not visible. Thus, puncturing within the MWA, which can be used as a scar-like landmark within a complete membranous HJAS, is considered a useful endoscopic strategy.
PubMed: 38939119
DOI: 10.1002/deo2.396 -
Medicina (Kaunas, Lithuania) May 2024Hepatobiliary ascariasis is caused by the entry of the nematode from the duodenum into the biliary duct. We report a case of an -induced extrahepatic biliary tract... (Review)
Review
Hepatobiliary ascariasis is caused by the entry of the nematode from the duodenum into the biliary duct. We report a case of an -induced extrahepatic biliary tract obstruction in a pediatric patient admitted to the hospital due to a wide spectrum of symptoms of biliary disease, which included abdominal pain in the upper abdominal quadrants, vomiting, and jaundice. Imaging tests-including ultrasound, magnetic resonance cholangiopancreatography (MRCP), and endoscopic retrograde cholangiopancreatography (ERCP)-were performed to confirm the diagnosis of biliary ascariasis. The tests did, in fact, demonstrate signs of this disease. Nevertheless, during the ERCP, only the remains of parasites in the form of tissue fragments were extracted. We also aim to discuss the prevalence of ascariasis in children, the causes of migration of parasites into the bile ducts, together with its clinical manifestations, as well as the diagnostic and treatment methods of this disease.
Topics: Humans; Ascariasis; Lithuania; Cholangiopancreatography, Endoscopic Retrograde; Child; Animals; Male; Ascaris lumbricoides; Cholangiopancreatography, Magnetic Resonance; Female
PubMed: 38929533
DOI: 10.3390/medicina60060916 -
Diagnostics (Basel, Switzerland) Jun 2024The liver, given its role as the central metabolic organ, is involved in many inherited metabolic disorders, including lysosomal storage diseases (LSDs). The aim of this... (Review)
Review
The liver, given its role as the central metabolic organ, is involved in many inherited metabolic disorders, including lysosomal storage diseases (LSDs). The aim of this manuscript was to provide a comprehensive overview on liver involvement in LSDs, focusing on clinical manifestation and its pathomechanisms. Gaucher disease, acid sphingomyelinase deficiency, and lysosomal acid lipase deficiency were thoroughly reviewed, with hepatic manifestation being a dominant clinical phenotype. The natural history of liver disease in the above-mentioned lysosomal disorders was delineated. The importance of Niemann-Pick type C disease as a cause of cholestatic jaundice, preceding neurological manifestation, was also highlighted. Diagnostic methods and current therapeutic management of LSDs were also discussed in the context of liver involvement.
PubMed: 38928715
DOI: 10.3390/diagnostics14121299 -
Diagnostics (Basel, Switzerland) Jun 2024This paper introduces a novel one-dimensional convolutional neural network that utilizes clinical data to accurately detect choledocholithiasis, where gallstones...
This paper introduces a novel one-dimensional convolutional neural network that utilizes clinical data to accurately detect choledocholithiasis, where gallstones obstruct the common bile duct. Swift and precise detection of this condition is critical to preventing severe complications, such as biliary colic, jaundice, and pancreatitis. This cutting-edge model was rigorously compared with other machine learning methods commonly used in similar problems, such as logistic regression, linear discriminant analysis, and a state-of-the-art random forest, using a dataset derived from endoscopic retrograde cholangiopancreatography scans performed at Olive View-University of California, Los Angeles Medical Center. The one-dimensional convolutional neural network model demonstrated exceptional performance, achieving 90.77% accuracy and 92.86% specificity, with an area under the curve of 0.9270. While the paper acknowledges potential areas for improvement, it emphasizes the effectiveness of the one-dimensional convolutional neural network architecture. The results suggest that this one-dimensional convolutional neural network approach could serve as a plausible alternative to endoscopic retrograde cholangiopancreatography, considering its disadvantages, such as the need for specialized equipment and skilled personnel and the risk of postoperative complications. The potential of the one-dimensional convolutional neural network model to significantly advance the clinical diagnosis of this gallstone-related condition is notable, offering a less invasive, potentially safer, and more accessible alternative.
PubMed: 38928692
DOI: 10.3390/diagnostics14121278 -
Journal of Medical Case Reports Jun 2024Acute hepatitis A infection is common among children in developing nations. The clinical presentation in children is usually asymptomatic and anicteric, and it is a...
BACKGROUND
Acute hepatitis A infection is common among children in developing nations. The clinical presentation in children is usually asymptomatic and anicteric, and it is a self-limiting infection. Rarely, it can be associated with extrahepatic complications such as pleural effusion, acalculous cholecystitis, and ascites.
CASE PRESENTATION
An 8-year-old middle eastern child presented with abdominal pain, jaundice in the sclera, yellowish color of urine, and poor appetite. In the last two days, abdominal distension developed. After conducting diagnostic investigations, the child was diagnosed with HAV hepatitis associated with bilateral pleural effusion, acalculous cholecystitis, and ascites. He was managed conservatively with vitamin K supplementation and supportive parenteral fluids. After 4 days, clinical improvement was observed.
CONCLUSION
Hepatitis A infections presented with extrahepatic manifestations like pleural effusion, acalculous cholecystitis, and ascites are very rare, especially in children. There have been some reports of these manifestations occurring in isolation, but for them to co-exist to our knowledge, this has only been reported in two cases in the literature, and this is the third case with all these three rare complications being presented simultaneously in a single child. Although HAV infection is an asymptomatic and self-limiting viral disease in childhood, it can manifest with rare extrahepatic complications, so pediatricians should be aware of this rare association to avoid unnecessary investigations.
Topics: Humans; Acalculous Cholecystitis; Hepatitis A; Ascites; Child; Pleural Effusion; Male; Vitamin K; Abdominal Pain
PubMed: 38918800
DOI: 10.1186/s13256-024-04627-8 -
Cureus May 2024Periampullary cancers, which include pancreatic adenocarcinoma, ampullary cancer, distal cholangiocarcinoma, and duodenal cancer, present diagnostic and management...
Periampullary cancers, which include pancreatic adenocarcinoma, ampullary cancer, distal cholangiocarcinoma, and duodenal cancer, present diagnostic and management challenges due to their aggressive nature and nonspecific symptoms. We describe a case of a female patient, age 20, who had obstructive jaundice brought on by a periampullary tumor. Despite difficulties in diagnosis and treatment, including failed endoscopic retrograde cholangiopancreatography (ERCP), the patient underwent a successful pancreaticoduodenectomy (Whipple's resection), and subsequent immunohistochemistry revealed adenocarcinoma with a mixed immunophenotype expressing duodenal and pancreatic markers. This example emphasizes the significance of taking young patients' periampullary tumors into account, the difficulties in diagnosing them, and the possibility of effective surgical surgery throughout this age range.
PubMed: 38915985
DOI: 10.7759/cureus.61013