Hypertriglyceridemia induced acute pancreatitis is a rare cause of pancreatitis in children. Hepatic lipase deficiency is an extremely rare cause of hypertriglyceridemia, reported in only a few families to date. Hepatic lipase is the enzyme involved in the hydrolysis of triglycerides and phospholipids in remnants of triglyceride-rich lipoproteins that have a role in the conversion of very low density lipoprotein remnants to low density lipoproteins. Hepatic lipase deficiency is inherited in an autosomal recessive pattern. Detection of heterozygous carriers of hepatic lipase mutations remains accidental at the population level, as affected persons with a heterozygous state of hepatic lipase mutation do not display specific lipoprotein abnormalities and also patients with complete hepatic lipase deficiency have inconstant phenotype. The proximal promoter of the LIPC gene consists of four polymorphic sites in complete linkage disequilibrium. Five missense mutations in encoding exons have been described and proved to be responsible for hepatic lipase deficiency to date: S267F, T383M, L334F, A174T, and R186H, affecting the activity and secretion of hepatic lipase. We identified a primary disorder of the lipid metabolism as the cause of the acute episode of pancreatitis in a four years old patient, consisting of hepatic lipase deficiency caused by a novel genetic variant of the LIPC gene, a gross deletion of the genomic region encompassing exon 1. This variant was not previously described in the literature in persons with LIPC-related disorders and its significance is currently uncertain, but in the presented clinical and paraclinical context, it has the characteristics of a pathological variant inducing a hepatic lipase deficiency phenotype.
Abstract. In this paper, we propose an electrical design (implemented on a PCB board) and an accompanying software design for controlling the automatic gear change. The designs complement the mechanical solutions developed in Part 1. The paper also analyses the issues encountered during the intermediate steps of the development of the electronic module, which is expected to be small and adaptable enough to be installed on a motorcycle without changing its ergonomics. The control software runs on the Arduino Nano board and is built as a state machine with one idle state, five active states that cover different stages of the gear change and one error state for preventing malfunctions in case of an unexpected event. The sketch uses 5,760 bytes (or 18%) of program storage space and 706 bytes (or 34%) of the dynamic memory.
Romania's forest policy is based on a long tradition in establishing and implementing the principles of sustainable management of forestry resources, based on a sound scientific and technical foundation. This resulted in a rigorous forestry regime, designed to operate efficiently under the conditions of state ownership over all forests. Over the past 30 years, Romania has gone through a period of transition towards a market economy with major institutional changes that took place in the forest sector through the restitution of forests and the privatisation of the administration, logging and forest industry sector. In this context, the regulatory system did not have the expected efficiency, which is reflected in a clear social tension, involving all stakeholders interested in and affected by the management of forests: private owners, public and private forest administration, logging and wood processing companies, environmental protection organisations, civil society, etc. The paper presents the process of elaborating a vision for sustainable forest management that would underlie a new public forest policy, in accordance with the international strategic documents and with the current challenges faced by the Romanian forest sector. The dialogue platform used in the elaboration of the vision has been coordinated by a university, precisely to ensure a unitary methodological approach to involve relevant experts and stakeholders in the process. The development of the vision used a stepwise participatory process based on the activity of a core group of experts followed by an extension of the group of experts, a public consultation process and a public debate. The result of the process is materialised in a set of eight sustainable forest management principles and four good governance principles. The implementation of the principles requires the identification, through an efficient and constructive communication between all stakeholders, of specific directions of action that have to be integrated in a new regulatory system that needs to be clear, easily monitored and effective in terms of practical results.
Introduction: The incidence of cholelithiasis has recently increased in the pediatric population, although it remains a rare disease among children. The purpose of this study is to highlight the management and the appropriate time to perform surgery. Materials and Methods: We performed a retrospective study over 10 years (2010-2010) and we found 123 patients who presented with biliary lithiasis and who underwent surgery in our department. We divided the study population into two groups depending on the time of admission and we found that in the 2010-2015 period we had a number of 33 patients, while in the last 5 years we admitted 90 patients with this diagnosis. Patients with other conditions such as bile duct atresia, choledochal cyst, hepatoblastoma that required a cholecystectomy were excluded from our study. Results: After analyzing the two groups we concluded that the number of surgical procedures increased from 33 to 90, which represents a 2.6 times increase. There was no significant difference between the two groups regarding the average age on admission (12.6 +/- 3.37 in the first group vs. 13.91 +/-3.34 in the second group). Surgical management of lithiasis cholecystitis registered an important difference in the two groups. In the first group, 9 patients (27.3%) underwent classic cholecystectomy while 24 (72.7%) – patients benefited from the laparoscopic approach, while in the second group just one patient underwent open surgery with 89 (98.9%) patients undergoing laparoscopic surgery. The percentage of patients who benefited from surgery in the same hospitalization remained high in both groups studied with a statistically significant p-value (p = 0.0417). Conclusion: The number of cases with cholecystitis and lithiasis is increasing in the pediatric population. Surgical management for these patients has become similar to that of adult patients, with minimally invasive surgery becoming the gold standard approach.
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