A review of the renal system and diurnal variations of renal activity in livestock

Kidneys are the main organs regulating water-electrolyte homeostasis in the body. They are responsible for maintaining the total volume of water and its distribution in particular water spaces, for electrolyte composition of systemic fluids and also for maintaining acid-base balance. These functions are performed by the plasma filtration process in renal glomeruli and the processes of active absorption and secretion in renal tubules, all adjusted to an 'activity-rest' rhythm. These diurnal changes are influenced by a 24-hour cycle of activity of hormones engaged in the regulation of renal activity. Studies on spontaneous rhythms of renal activity have been carried out mainly on humans and laboratory animals, but few studies have been carried out on livestock animals. Moreover, those results cover only some aspects of renal physiology. This review gives an overview of current knowledge concerning renal function and diurnal variations of some renal activity parameters in livestock, providing greater understanding of general chronobiological processes in mammals. Detailed knowledge of these rhythms is useful for clinical, practical and pharmacological purposes, as well as studies on their physical performance

A newborn with severe liver failure, cardiomyopathy and transaldolase deficiency

This paper describes the second patient found to be affected with a deficiency of transaldolase (TALDO1; EC 2.2.1.2). Clinically, this patient presented in the neonatal period with several signs of severe liver failure: severe coagulopathy, low serum protein, elevated blood ammonia, and hypoglycaemia. She had generalized oedema, moderate muscular hypotonia, and dysmorphic signs. Liver size was decreased, and the spleen was moderately enlarged. There was severe cardiomegaly. The clinical course was characterized by intractable liver failure and progressive myocardial hypertrophy. The child died at the age of 18 days from respiratory failure. In urine, elevations of erythritol, arabitol and ribitol were found, suggesting a deficiency of transaldolase. Enzyme studies in cultured fibroblasts showed undetectable transaldolase activity. DNA sequence analysis of the TALDO1 gene showed a homozygous missense mutation (575G>A), resulting in an amino acid alteration at position 192 (arginine to histidine, R192H). This amino acid is part of the catalytic site of the transaldolase protein. Discovery of this second patient affected with transaldolase deficiency and liver failure suggests that this disorder has a heterogeneous clinical presentation with highly variable severit

Paediatric acute liver failure and transplantation: the University of Essen experience.

To report our experience with 17 children who underwent a liver transplantation (LT) for acute liver failure (ALF). All LT procedures (deceased and living donor) were offered. Since 2003 Molecular Adsorbents Recycling System (MARS) was proposed as bridging procedure. We monitored the perioperative course and the short- and long-term outcomes. All children developed pretransplant hepatic encephalopathy (mostly grades II and III); six needed ventilator support and three haemodialysis. Median PELD/MELD score was 30. MARS was used in five children with poor pretransplant prognostic factors: all five survived the LT without sequelae. We performed 13 deceased donor LT (seven whole, five split and onr reduced) and four left lateral LDLT. Postoperative complications were observed in 10 children, requiring re-operation in seven. Two children developed irreversible neurological disorders. After a median follow up of 45 months, 16 children are still alive. About 1- and 5-year cumulative patient survival rates are 94% with a corresponding graft survival of 88% and 81%, respectively. The combination of experienced paediatric ICU management, the application of new liver support devices, and the capacity to offer both living and deceased donor transplant alternatives in a timely fashion represent the best formula to achieve optimal results in children with ALF