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Niemann-Pick disease
Published in William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop, Atlas of Inherited Metabolic Diseases, 2020
We have seen Niemann-Pick patients with hepatosplenomegaly whose history was that the spleen was not palpable early. Transaminases aspartate transaminase (AST) and alanine transaminase (ALT) are elevated, at least at times [26]. The alkaline phosphatase is also usually elevated. The cholesterol may be elevated in addition. There may be prolonged neonatal jaundice, and episodes of unexplained jaundice later. We have seen patients who presented in early infancy with acute jaundice, abnormal liver function tests, and hepatomegaly, suggesting a diagnosis of acute hepatocellular disease rather than a lipid storage disease. We have also seen a patient in whom two liver biopsies had been done in another institution and interpreted as fatty metamorphosis. At least one patient with Niemann-Pick disease was thought, on biopsy, to have glycogenosis [27]. Jaundice is a common terminal finding and some patients have developed disseminated intravascular coagulopathy. There may be lymphadenopathy.
Cyanides: Toxicology, Clinical Presentation, and Medical Management
Published in Brian J. Lukey, James A. Romano, Salem Harry, Chemical Warfare Agents, 2019
Gary A. Rockwood, Gennady E. Platoff Jr., Harry Salem
Histopathologically, there were foci of liver, renal tubular, and glomerular necrosis. It was also demonstrated (Okolie and Osagie, 2000) that aspartate transaminase activity in heart and serum was unaffected, but cardiac and pulmonary alkaline phosphatase was decreased. Histology was normal in pancreas and myocardium, but lungs had foci of edema and necrosis. These findings indicate that long-term p.o. dosing with CN causes hepatorenal and systemic pulmonary injury but not cardiac or pancreatic toxicity. In a multispecies (rat, pig, and goat) subchronic p.o. study with KCN, Soto-Blanco et al. (2001) did not find any biochemical or histological evidence for pancreatic exocrine or endocrine toxicity. In a 13 week study, rats and mice received up to 300 ppm in drinking water (Hébert, 1993), which resulted in a slight reduction in cauda epididymal weights (rats and mice) and reduced numbers of spermatid heads per testis (rats). Rats dosed with up to 500 ppm KCN in drinking water had dose-related decreases in hepatic and cardiac respiration and in cardiac, hepatic, and cerebral ATP concentration (Rickwood et al., 1987). These findings accord with CN producing mitochondrial dysfunction.
The liver
Published in Professor Sir Norman Williams, Professor P. Ronan O’Connell, Professor Andrew W. McCaskie, Bailey & Love's Short Practice of Surgery, 2018
Professor Sir Norman Williams, Professor P. Ronan O’Connell, Professor Andrew W. McCaskie
Bilirubin is synthesised in the liver and excreted in bile. Increased levels may be associated with increased haemoglobin breakdown, hepatocellular dysfunction resulting in impaired bilirubin transport and excretion or biliary obstruction. In patients with known parenchymal liver disease, progressive elevation of bilirubin in the absence of a secondary complication suggests deterioration in liver function. The serum alkaline phosphatase (ALP) is particularly elevated with cholestatic liver disease or biliary obstruction. It is important to note that routine laboratory analysis of ALP is not isoform-specific and so alkaline phosphatase from a skeletal source may also lead to elevation. The transaminase levels (aspartate transaminase (AST) and alanine transaminase (ALT)) reflect acute hepatocellular damage, as does the gamma-glutamyl transpeptidase (GGT) level, which may be used to detect the liver injury associated with acute alcohol ingestion. However, marked liver injury can occur in the presence of normal liver function tests. The synthetic functions of the liver are reflected in the ability to synthesise proteins (albumin level) and clotting factors (prothrombin time). The standard method of monitoring liver function in patients with chronic liver disease is therefore serial measurement of bilirubin, albumin and prothrombin time.
Bilateral Multiple Retinal Detachments Associated with Diffuse Large B-Cell Lymphoma: Masquerading as Vogt-Koyanagi-Harada Disease
Published in Ocular Immunology and Inflammation, 2023
The initial clinical impression was incomplete VKH disease3; however, considering the patient’s high fever and generalized symptoms, we performed additional systemic evaluation before administering systemic steroid pulse therapy. Lab test results revealed elevated levels of aspartate transaminase (81 IU/L) and alanine transaminase (31 IU/L). Moreover, lactic acid dehydrogenase (LDH) levels were remarkably high (2522 IU/L). Given that LDH levels are elevated in cases of myopathy and various malignancies,4 we performed abdominopelvic CT; it revealed a hepatic mass with multiple lymphadenopathy. Liver biopsy confirmed the diagnosis of DLBCL. On positron emission tomography-CT, a hypermetabolic nodular thickening was observed in the posterior poles of both eyes; furthermore, other lesions were observed along with the hypermetabolic lesions invading multiple organs such as the lungs, liver, spleen, bone marrow, bone, meninges, left maxillary sinus, and peritoneum. Accordingly, chemotherapy was initiated for DLBCL, which invades multiple organs with B symptoms. Post-chemotherapy, the patient’s visual symptoms rapidly improved. The BCVA recovered to 20/25 in both eyes and remained stable until the 3-year follow-up; furthermore, the subretinal fluid was completely resolved (Figure 2a,b).
Ingestion of Sudan IV-adulterated palm oil impairs hepato-renal functions and induces the overexpression of pro-inflammatory cytokines: A sub-acute murine model
Published in Egyptian Journal of Basic and Applied Sciences, 2022
Ofem E. Eteng, Ceaser A. Moses, Emmanuel I. Ugwor, Joe E. Enobong, Adio J. Akamo, Yewande Adebekun, Arikpo Iwara, Eyong Ubana
The activities of alanine transaminase (ALT), aspartate transaminase (AST), alkaline phosphatase (ALP), and lactate dehydrogenase (LDH) were determined in the serum, as well as in the liver and kidney homogenates, using commercially available kits and following the manufacturers’ instructions. The kits for ALT, AST, and ALP were products of Randox Laboratories Limited (Crumlin, United Kingdom), while the LDH kit was a product of Biosystems Diagnostics [Costa Brava, Barcelona, Spain). The total protein concentration was determined using the Coomassie blue method of [18] with serum albumin as standard. Serum levels of uric acid, blood urea nitrogen (BUN], and creatinine were also determined using commercially available kits produced by Randox Laboratories Limited, as per the manufacturer’s instructions.
Micafungin injection for the treatment of invasive candidiasis in pediatric patients under 4 months of age
Published in Expert Review of Anti-infective Therapy, 2022
Nahed Abdel-Haq, Stephanie M. Smith, Basim I. Asmar
In a recent phase 2 study by Auriti et al., micafungin was given at a dose of 8 mg/kg daily to 35 infants with proven or suspected invasive candidiasis including 28 who were ≤4 months of age [77]. Of those, 21 (60%) had proven invasive candidiasis and 20 (57.1%) completed the study treatment (≥14 days). Mean micafungin plasma level was highest at 1 hour post dose and decreased over 8 hours. Micafungin levels obtained via venous samples were slightly higher than the heal stick capillary samples. The resolution of the infection was achieved in 86.7% of patients who completed a minimum of 14 days of micafungin therapy. Among those, mycological eradication was accomplished in 61.9% at the end of treatment and 90.5% at the end of the study. Treatment-emergent AEs (TEAEs) were reported in 88.6% but none led to micafungin discontinuation or dose reduction. Five study patients died but no death was related to micafungin therapy. Micafungin at a dose of 8 mg/kg daily was effective and well tolerated in the study patients [77]. Transient elevations of aspartate transaminase and alanine transaminase were observed in 20.0% of patients [77].