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Maple syrup urine disease (branched-chain oxoaciduria)
Published in William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop, Atlas of Inherited Metabolic Diseases, 2020
William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop
Emergency treatment of an infant in coma requires prompt reduction of levels of leucine and the other branched-chain amino acids. This has formerly been approached by exchange transfusions, peritoneal dialysis, or both; but direct measurements have indicated the removal of small quantities of amino acids in this way. Continuous arteriovenous or venovenous hemodialysis, hemofiltration and hemodiafiltration are doubtless very effective [85, 86], but it is formidable in a young infant, and the prospect of repeat dialysis with each respiratory infection in the early years of life is impossible to consider. Saudubray and colleagues [85, 86] have reported on continuous venovenous extracorporeal hemodiafiltration as a very effective method for the lowering of high levels of leucine. In six neonatal infants and six children with later episodes, this approach was begun after six hours of conservative management including enteral amino acid mixtures, and leucine concentration, was over 1700 μmol/L. In each, the decrease in leucine was logarithmic and usually reached <1000 μmol/L in 24 hours, while the rate of decrease with enteral therapy after cessation of the diafiltration was a slower linear fall. Follow-up developmental levels in this series were encouraging; some had intelligence quotients over 100 with follow up of as long as three to five years.
Diagnostic Approach to Acute Kidney Injury in the Critical Care Unit
Published in Cheston B. Cunha, Burke A. Cunha, Infectious Diseases and Antimicrobial Stewardship in Critical Care Medicine, 2020
Sonali Gupta, Divyansh Bajaj, Sana Idrees, Joseph Mattana
The gradual removal of solute, water, and electrolytes, offered by CRRT, may make it the preferred therapy in certain circumstances where the patient is more susceptible to volume and osmotic/metabolic fluctuations. It may offer better hemodynamic and volume homeostasis in hemodynamically unstable patients with considerable volume overload and is generally a preferable option in patients with acute brain or liver injury, where rapid shifts in blood osmolality may contribute to an iatrogenic increase in intracranial pressure. The CRRT is also recommended in situations where extracorporeal life support therapies are applied. It can be performed using diffusive clearance (continuous venovenous hemodialysis [CVVHD]), convective clearance (continuous venovenous hemofiltration [CVVH]), or a combination of both (continuous venovenous hemodiafiltration [CVVHDF]). Although convective modalities offer greater clearance of middle molecules compared with diffusive clearance, this has not been shown to affect clinical outcomes in the AKI setting, and the choice between modalities should be determined by local expertise. A comparison of RRT modalities is provided in Table 24.5.
Sedative/Hypnotics
Published in Frank A. Barile, Barile’s Clinical Toxicology, 2019
Frank A. Barile, Anirudh J. Chintalapati
Should renal or cardiac failure, electrolyte abnormalities, or acid–base disturbances occur, hemodialysis is recommended. Although most cases of phenobarbital overdose respond well to cardiopulmonary supportive care, severe cases will also require hemodialysis or charcoal hemoperfusion. Neither of these procedures will remove significant amounts of short- or intermediate-acting barbiturates. Through ion exchange, hemodialysis is more effective in removing long-acting barbiturates than short-acting compounds, because there is less protein and lipid binding of the former. Recently, continuous venovenous hemodiafiltration was used successfully in a case of severe phenobarbital poisoning. The protocol effectively uses continuous renal replacement therapy in phenobarbital clearance associated with severe coma and hypotension.
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 demographic and laboratory baseline indicators were obtained from the electronic medical record system for the patients starting hemodialysis and the frequency and duration of dialysis was determined as the weekly frequency and duration of dialysis after the first dialysis for three months. According to the patient’s condition, hemodialysis was performed 2–4 times weekly, hemodiafiltration was performed once every half month, and the laboratory tests and examinations related to renal complications were performed once every three months. Hypertension was defined as the diagnosis of hypertension, use of antihypertensive drugs, and an average systolic blood pressure (SBP) >140 mmHg or average diastolic blood pressure (DBP) >90 mmHg. The primary endpoint was defined as all-cause death. The secondary endpoint was defined as cardiovascular deaths. A cardiovascular death was defined as death caused by heart failure, myocardial infarction, arrhythmia, cerebral hemorrhage, infarction and other cardiovascular events. BAs levels were determined using the enzyme circulation method. The association between bile acids and all-cause mortality in all MHD patients was assessed, then the association between bile acids and all-cause mortality in MHD patients with and without DM was investigated separately. Ultimately, we focused our study on the relationship between BAs and all-cause mortality in patients on MHD with DM.
Acute toxic kidney injury
Published in Renal Failure, 2019
Nadezda Petejova, Arnost Martinek, Josef Zadrazil, Vladimir Teplan
General recommendation for prevention and treatment of critically ill patients with AKI are based on: (1) correction of hypovolemia using isotonic crystalloids with avoidance of hyperhydration, (2) regular monitoring of ions (chloride) and acid base-balance with adequate correction when chloride rich solutions are used, (3) use of vasopressor – norepinephrine to maintain mean arterial pressure 65–70 mmHg, (4) use of diuretic agent to avoid fluid overload and preserve diuresis if needed, (5) tight control of serum glucose, (6) therapeutic drug monitoring in initiation of therapy with highly nephrotoxic antimicrobials (e.g., vancomycin, gentamicin), (7) initiation of renal replacement therapy (RRT) according to the clinical picture or laboratory findings, usually in stage 2 or 3 according to KDIGO [1,137]. Renal replacement therapy can be used in two forms according to the duration and intensity needed: intermittent or continuous hemodialysis, hemofiltration or hemodiafiltration. Patient hemodynamic instability is usually a crucial factor for initiating continuous RRT.
Toward an individualized determination of dialysis adequacy: a narrative review with special emphasis on incremental hemodialysis
Published in Expert Review of Molecular Diagnostics, 2021
Massimo Torreggiani, Antioco Fois, Linda Njandjo, Elisa Longhitano, Antoine Chatrenet, Ciro Esposito, Hafedh Fessi, Giorgina Barbara Piccoli
Hemodiafiltration is not necessarily performed in the same way in different settings: for instance, in France, for reasons of efficiency, convective therapies are preferred in younger patients with a long life-expectancy or a transplant project, while in Italy, HDF is often chosen to improve cardiovascular stability in fragile subjects [163]. Finally, according to the Japanese experience, HDF should be offered to prevent long-term complications such as dialysis-related amyloidosis (Table 3) [164–166]. Elderly patients, who are often affected by high comorbidity, might be particularly vulnerable to protein losses and this should be considered together with favoring an incremental dialysis approach [145,167–169].