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61.
Retrospective evaluation of abdominal ultrasound examinations were made in 36 patients who came to autopsy within 1 month after the ultrasound study. Without knowledge of clinical or autopsy data, two observers made independent determinations of the midhepatic line measurement of the liver on the ultrasound study using supine and left lateral decubitus longitudinal scans. Autopsy determination of hepatomegaly was made using hepatic weight, patient's total body weight, and patient age correlated with pertinent clinical history. Results of the autopsy/ultrasound correlation demonstrated that those livers measuring 13.0 cm or less in the midhepatic line (both supine and left lateral decubitus positions) were normal in 93% of the cases. Similarly, it was demonstrated that when the liver measured 15.5 cm or greater, it was enlarged in 75% of the cases. Used together, these two criteria result in an 87% accuracy rate in determining the presence or absence of hepatomegaly. Approximately 25% of the cases in our study fell into the borderline category of 13.0–15.5 cm. 相似文献
62.
Apolline Imbard Nuria Garcia Segarra Marine Tardieu Pierre Broué Juliette Bouchereau Samia Pichard Hélène Ogier de Baulny Abdelhamid Slama Charlotte Mussini Guy Touati Marie Danjoux Pauline Gaignard Hannes Vogel François Labarthe Manuel Schiff Jean-François Benoist 《Molecular genetics and metabolism》2018,123(4):433-440
Background and objectives
Patients affected with methylmalonic acidemia (MMA) and propionic acidemia (PA) exhibit diverse long-term complications and poor outcome. Liver disease is not a reported complication. The aim of this study was to characterize and extensively evaluate long-term liver involvement in MMA and PA patients.Patients and methods
We first describe four patients who had severe liver involvement during the course of their disease. Histology showed fibrosis and/or cirrhosis in 3 patients. Such liver involvement led us to retrospectively collect liver (clinical, laboratory and ultrasound) data of MMA (N?=?12) or PA patients (N?=?16) from 2003 to 2016.Results
Alpha-fetoprotein (αFP) levels were increased in 8/16 and 3/12 PA and MMA patients, respectively, and tended to increase with age. Moderate and recurrent increase of GGT was observed in 4/16 PA patients and 4/12 MMA patients. Abnormal liver ultrasound with either hepatomegaly and/or hyperechoic liver was observed in 7/9 PA patients and 3/9 MMA patients.Conclusions
These data demonstrate that approximately half of the patients affected by MMA or PA had signs of liver abnormalities. The increase of αFP with age suggests progressive toxicity, which might be due to the metabolites accumulated in PA and MMA. These metabolites (e.g., methylmalonic acid and propionic acid derivatives) have previously been reported to have mitochondrial toxicity; this toxicity is confirmed by the results of histological and biochemical mitochondrial analyses of the liver in two of our MMA patients. In contrast to the moderate clinical, laboratory or ultrasound expression, severe pathological expression was found for three of the 4 patients who underwent liver biopsy, ranging from fibrosis to cirrhosis. These results emphasize the need for detailed liver function evaluation in organic aciduria patients, including liver biopsy when liver disease is suspected.Take home message
MMA and PA patients exhibit long-term liver abnormalities. 相似文献63.
Miyawaki I Matsumoto I Horie H Inada H Kimura J Funabashi H Seki T 《Regulatory toxicology and pharmacology : RTP》2011,60(3):323-331
Antiepileptic agents are known to cause adverse effects in human liver, including steatosis. Clobazam (CLB), a 1,5-benzodiazepine, is clinically used as an antiepileptic agent. In the previous study, 4-week treatment with CLB induced hepatomegaly in male rats. In the present study, the human risk of hepatomegaly was assessed and the causative mechanism in terms of cell proliferation and apoptosis, oxidative stress, and drug-metabolizing enzyme induction was elucidated by toxicological approach. Male SD rats were treated orally with 400 mg/kg CLB for 1, 3, 7, 14, or 28 days. The 28-day treatment was followed by 7 or 14 days of withdrawal. At the end of each treatment, the liver and plasma of each rat were examined. Liver weight increased from Day 3 of CLB treatment. This increase was mostly accompanied by hepatic centrilobular hypertrophy and proliferation of smooth endoplasmic reticulum (SER), and by an increase in microsomal proteins. Cyp2b1, Cyp3a1, Cyp3a2, and Ugt2b2 mRNA levels in the liver were upregulated as compared to the control group throughout the dosing period. On the other hand, the thiobarbituric acid reactive substance (TBARS) formulation, hepatocyte proliferation, and apoptosis, assumed to play roles in laying groundwork for effective induction of metabolizing enzymes, were increased only at the acute phase of treatment. These results suggested that CLB-induced hepatomegaly in male rats is mainly attributable to microsomal enzyme induction associated with Cyp2b1, Cyp3a1, Cyp3a2, and Ugt2b2 gene upregulation, but does not cause any toxicological concerns. 相似文献
64.
Kalenahalli Jagadishkumar Puja Jain Vaddambal G. Manjunath Lingappa Umesh 《Iranian journal of pediatrics.》2012,22(2):231-236
Objective
Hepatic dysfunction is common in dengue infection and the degree of liver dysfunction in children varies from mild injury with elevation of transaminases to severe injury with jaundice. This study was undertaken to assess the spectrum of hepatic involvement in dengue infection.Methods
110 children with serologically positive dengue fever aged between 2 months - 14 years were studied for their hepatic functions both clinically and biochemically after excluding malaria, enteric fever, Hepatitis A and Hepatitis B with relevant investigations.Findings
All cases were grouped into DF (Dengue fever), DHF (Dengue hemorrhagic fever) and DSS (Dengue shock syndrome) according to WHO criteria. The spectrum of hepatic manifestations included hepatomegaly (79%), hepatic tenderness (56%), jaundice (4.5%), raised levels of aspartate transaminase (AST)(93%), alanine transaminase (ALT)(78%), alkaline phosphatase (AP) (57%), prolonged prothrombin time (PT) (20%), reduced levels of serum albumin (66%) and abnormal abdomen ultrasound (65%).Conclusion
Hepatic dysfunction was observed more in DHF and DSS group compared to DF group. About 17.27% of children had >10 fold increase in the liver enzymes. There was no correlation between the degree of hepatic enlargement or hepatic tenderness with the abnormalities of liver functions. Any child with fever, jaundice and tender hepatomegaly in geographical areas where dengue is endemic, the diagnosis of dengue infection should be strongly considered. 相似文献65.
Vennervald BJ Booth M Butterworth AE Kariuki HC Kadzo H Ireri E Amaganga C Kimani G Kenty L Mwatha J Ouma JH Dunne DW 《Transactions of the Royal Society of Tropical Medicine and Hygiene》2005,99(2):150-160
Evaluating regression of morbidity associated with parasitic infections is an important component of community-based control programmes. We performed an intervention against Schistosoma mansoni infection, focusing on hepatosplenomegaly in the absence of periportal fibrosis, in a cohort of 67 Kenyan children aged 7-18 years from Makueni District, selected on the basis of hepatosplenomegaly detected by ultrasonography. Clinical and ultrasound examinations were conducted annually for three years after treatment, and the source of infection (a river) was regularly treated with molluscicide, thereby severely reducing exposure to schistosomiasis. Malaria transmission was uninterrupted. The prevalence of hard spleens, and the magnitude of clinically assessed splenomegaly along the mid-axillary and mid-clavicular lines decreased monotonically over time, independently of age, whereas clinically measured hepatomegaly along the mid-sternal line and the prevalence of firm livers decreased in an age-specific manner, being more pronounced amongst children aged 14 years or older at enrolment. Ultrasound data were less informative, and did not concur with clinical observations. These results demonstrate that praziquantel treatment reduces hepatosplenomegaly in the absence of exposure to S. mansoni, even with continuing exposure to malaria. The lack of complete resolution of hepatosplenomegaly in most children suggests, among other things, a residual organomegaly attributable to malaria. 相似文献
66.
Mabrut JY Dubois R Pelizzo G Floret D Frappaz D Chappuis JP 《Pediatric surgery international》2000,16(3):219-221
The authors report three cases of stage IV-S neuroblastoma in infants aged 4, 6, and 8 weeks, who despite chemo- and radiotherapy
required surgical intervention to urgently relieve major thoracoabdominal compression secondary to massive hepatomegaly. The
results were successful, with abdominal expansion being achieved by the introduction of a polytetrafluoroethylene prosthesis,
which was removed during the 2nd, 3rd and 7th postoperative month, respectively, after tumor regression. Two children were
in complete remission 32 and 38 months later, the 3rd died after 16 months of tumor progression.
Accepted: 23 February 1999 相似文献
67.
对50例长期饮酒后所致精神障碍患者的B超监测,了解其肝脏超声检查、诊断情况及经住院戒断治疗后的变化。结果,长期饮用高度白酒后形成慢性酒精中毒的患者,肝脏增大者占54%,而且其肝实质有不同程度的脂肪性变声象图。结论,病人经过住院戒酒治疗后,原异常超声声象图有改善,肿大的肝脏可逐渐恢复正常。 相似文献