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Respiratory, endocrine, cardiac, and renal topics
Published in Evelyne Jacqz-Aigrain, Imti Choonara, Paediatric Clinical Pharmacology, 2021
Evelyne Jacqz-Aigrain, Imti Choonara
CF is inherited in an autosomal recessive fashion. The carrier incidence is around 1/25 with 1/2500 live births affected in Western Europe. The cystic fibrosis gene is located on chromosome 7. The commonest gene mutation in CF patients in the UK is AF508, with 52% of CF patients homozygous for this mutation. A defective form of the gene product, the cystic fibrosis transmembrane conductance regulator (CFTR), leads to abnormal transport of chloride ions at mucosal surfaces. This leads to the production of thick, sticky secretions in the lung, gastrointestinal tract, hepatobiliary system, pancreas and reproductive system. This presents some particular problems for drug delivery. Drugs (including bronchodilators, inhaled steroids, antibiotics and mucolytics) are frequently given by the inhaled route. Drug delivery to the lung may be inefficient in the normal airway, but in patients with CF, there are added obstacles of mucus plugging and ventilation-perfusion mismatch. There may be problems with the absorption of oral medication, such as fat soluble vitamins, due to fat malabsorption, liver disease and interruption of enterohepatic circulation by antibiotics. Frequent intravenous antibiotic treatment may be necessary and this may require the placement of an indwelling intravenous access device (e.g., Portacath®).
HPB Surgery
Published in Tjun Tang, Elizabeth O'Riordan, Stewart Walsh, Cracking the Intercollegiate General Surgery FRCS Viva, 2020
London Lucien Ooi Peng Jin, Teo Jin Yao
A 56-year-old woman presented to the emergency department with a 3-day history of fever and chills associated with malaise and right hypochondrial pain especially on deep inspiration. Examination showed a flushed facies, temperature of 38.5°C, and she was mildly tender in the right hypochondrium on deep palpation although Murphy's sign was negative. What are the possible differentials?In a woman who is obviously septic and the localisation pointing to the right hypochondrium would suggest some form of hepatobiliary sepsis.The most common condition in this setting would be acute cholecystitis although in this case the Murphy's sign is negative. Acute hepatitis is a possibility although one would expect a prodrome several weeks prior rather than such a short and acute history. Cholangitis is excluded as a differential as there is no jaundice. The presence of chills and rigours would suggest a focal collection of pus and this can be in the form of a gallbladder empyema or a liver abscess.Apart from doing the blood investigations to confirm an infective state and also for culture and sensitivity, there is a need for an urgent imaging to confirm the diagnosis and this can be quickly done with a bedside US of the hepatobiliary system.
Entamoeba histolytica
Published in Peter D. Walzer, Robert M. Genta, Parasitic Infections in the Compromised Host, 2020
William A. Petri, Jonathan I. Ravdin
The diagnosis of extraintestinal amebiasis is a challenge for physicians in developed countires who rarely encounter the disease (Table 9). Only a minority of patients will have concurrent diarrhea or E. histolytica isolated from their stool. When encountering a febrile patient with right upper quadrant abdominal pain, one should suspect an amebic liver abscess when the patient is from a group at high risk for amebic infection (such as an immigrant or a traveler from an endemic area) and can give a history of prior dysentery. Amebic serology is the key for confirming the diagnosis of extraintestinal amebiasis but is not always immediately available. Noninvasive imaging techniques of the hepatobiliary system should be used the first day of hospitalization to distinguish liver abscess from acute cholecystitis. A series of 75 patients admitted with fever, right upper quadrant pain, nausea, and vomiting from an area in Texas endemic for amebiasis were evaluated within 24 hr by hepatobiliary radionuclide and ultrasound scans (243). These scans identified nine patients with liver abscess subsequently proven to be amebic by serology and clinical response to therapy with metronidazole. Clinically these patients were indistinguishable from the patients with cholecystitis except for being in a younger age group. Patients with cholecystitis may present in a more acute manner, be more likely to give a history of prior abdominal complaints, and not have hepatomegaly on physical examination as often seen in amebic abscess.
The relationship between bile acids levels and the prognosis of patients with diabetes on maintenance hemodialysis: a retrospective study
Published in Renal Failure, 2023
Bin Li, Cong Peng, Yili Wang, Rong Ma, Ya Feng
The all-cause risk of patients with DM on MHD decreases with increased BAs levels. We obtained the cutoff value of death by fitting BAs and death risk with a restricted cubic spline. If the BAs level of a patient with DKD is less than 3.5 μmol/L, we needed to be alert to the increased risk of all-cause death. The cutoff value serves as a reference in clinical practice for identifying patients with an increased risk of end-stage renal disease. However, we must rule out a pathological increase in BAs caused by hepatobiliary system diseases. Our findings showed that, in addition to the conventional death risk factors for patients on MHD, such as age, albumin, and HbA1c, the all-cause death of patients in the higher BAs group was 0.55 times that of patients in the lower BAs group, indicating that BAs levels are play an important role in the prognosis of patients with DM on MHD. In addition, in the analysis of secondary endpoint events, we discovered that patients with higher BAs levels had a higher risk of cardiovascular death.
The level of serum total bile acid is related to atherosclerotic lesions, prognosis and gut Lactobacillus in acute coronary syndrome patients
Published in Annals of Medicine, 2023
Ting-Ting Liu, Jie Wang, Yan Liang, Xiao-Yuan Wu, Wen-Qing Li, Yu-Hang Wang, An-Ran Jing, Miao-Miao Liang, Li Sun, Jing Dou, Jing-Yu Liu, Yin Liu, Zhuang Cui, Jing Gao
The size of the circulating bile acid pool in ACS patients has not received enough attention. There were many reasons for this phenomenon. One reason was that bile acid levels were related to liver metabolic function, and some ACS patients had an acute liver injury, which may lead to abnormal bile acid metabolism. Another reason was that serum bile acid levels in most ACS patients were in the normal range, leading to a lack of awareness of it. In addition, the composition of bile acids was complex, and some studies focused on the relationship between a single component of bile acids and atherosclerosis. It was noteworthy these studies mostly used additional supplementation with a specific bile acid component, which was inconsistent with the absence of hyperbileacidemia in ACS. In our study, patients with combined hepatobiliary system disease were excluded. There was no difference in liver function among patients with different severity of coronary lesions. Our results suggested that lower levels of serum bile acid may indicate patients with more severe coronary lesions and a worse prognosis.
Factors associated with acute pancreatitis in patients with impacted duodenal papillary stones: a retrospective cohort study
Published in Scandinavian Journal of Gastroenterology, 2022
Ming Li, Ao Wang, Shaohua Ren, Zhenyu Wang, Qing Wang, Chengyue Gou, Weichuan Zhao, Li Zhang, Ning Li
This retrospective analysis included patients with IPS who underwent ERCP at the Endoscopy Center, Tianjin Nankai Hospital of Tianjin Medical University, Tianjin, China, between January 2009 and December 2018. The inclusion criteria were as follows: (1) the stone was impacted in the duodenal papilla; (2) ERCP and endoscopic sphincterotomy were performed successfully; (3) ERCP showed that the duodenal papilla had not been visibly expanded or damaged by the impacted stone; and (4) the bile duct was well-filled with contrast medium, allowing clear visualization of the hepatobiliary system. Patients were excluded from the analysis if any of the following criteria were met: (1) data required for the analysis were missing; (2) the diagnosis on ERCP was ambiguous; (3) the patient had serious comorbid conditions that might influence the accuracy of the analysis including chronic pancreatitis (which can alter the shape and function of the duodenal papilla) and diseases that affect liver function such as cirrhosis and hemolytic jaundice; and (4) the patient was taking oral lipid-regulating drugs (which can influence the assessment of blood lipids and affect liver function). The Ethics Committee of Tianjin Nankai Hospital of Tianjin Medical University approved the study (No. NKYY_YXKT_IRB_2021_019_01) and waived the requirement for consent due to the retrospective study design. However, all patients provided informed written consent before ERCP.