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Inborn Errors of Metabolism
Published in Praveen S. Goday, Cassandra L. S. Walia, Pediatric Nutrition for Dietitians, 2022
Surekha Pendyal, Areeg Hassan El-Gharbawy
Liver transplant is recommended in GSD I when adenomas are large and numerous and have a high risk of becoming cancerous. Transplantation is curative for the hypoglycemia and most other complications of GSD I except for neutropenia in GSD Ib. Renal dialysis and transplant is required in some patients for advanced kidney failure.
Immuno-Pathologic Basis of COVID-19 and the Management of Mild and Moderate Cases
Published in Srijan Goswami, Chiranjeeb Dey, COVID-19 and SARS-CoV-2, 2022
Debdeep Dasgupta, Srijan Goswami, Chiranjeeb Dey
SARS-CoV-2 is an enveloped coronavirus having positive-sense single-stranded RNA (+ssRNA) as the genetic material. It has four membrane proteins out of which the spike (S) protein is of major concern from a pathophysiological perspective. The spike protein helps SARS-CoV-2 attach to the membrane of the host cell by a specialized receptor angiotensin converting enzyme-2 (ACE-2) receptor. The virus colonizes the nasopharynx from which it spreads by respiratory droplets and aerosols. The virus that contains droplets is produced by speaking, coughing, or sneezing. After exposure, the mean incubation period is about five days, which can be extended up to 14 days (Kumar and Al Khodor, 2020). The shedding of viral particles begins ~two to three days before the onset of symptoms until five days after the appearance of symptoms. The infected individual may be completely symptomatic or may show serious clinical symptoms. Patients have fever, cough, myalgia, headache, anosmia, dysgeusia, and diarrhea. Severe patients may present with COVID pneumonia with hypoxia and thrombotic episodes. Extrapulmonary manifestations affect nearly every organ, for example, cardiac complications include thrombosis, myocardial infarction, myocarditis, and arrhythmias. Neurologic complications can include myoclonus, seizures, delirium, and encephalitis. Renal damage is common, with about one-third of patients who require mechanical ventilation also requiring renal dialysis. Finally, death can occur due to ARDS and multiorgan failure (WHO, 2014, 2020a, c, d).
Respiratory, endocrine, cardiac, and renal topics
Published in Evelyne Jacqz-Aigrain, Imti Choonara, Paediatric Clinical Pharmacology, 2021
Evelyne Jacqz-Aigrain, Imti Choonara
Treatment must be based on the cause if known or suspected. In renal disease intravascular fluid (“saline”) overload is often under-estimated, and tissue oedema is not well correlated with intravascular volume. Thus the drug of choice may be a diuretic in the infant or child whose main problem is systemic fluid overload, with hypertension as part of the clinical picture. Renal function will profoundly influence management, particularly use of drugs. Urine output should be monitored and, if serum creatinine is outside the normal range, caution given in the use of drugs excreted though the renal route. Renal dialysis or filtration may be required. Renin-dependent causes will influence the use of ACE-inhibitors, particularly if renovascular. Catecholamine-driven hypertension needs specialist advice regarding both oc-adrenergic and β-blockade, with particular attention to problems around surgery for phaeochromocytoma.
Efficacy and safety of expanded hemodialysis in hemodialysis patients: a meta-analysis and systematic review
Published in Renal Failure, 2022
Yuchao Zhao, Liangying Gan, Qingyu Niu, Mengfan Ni, Li Zuo
A systematic review was performed by searching PubMed, the Cochrane Library, and Embase databases for relevant studies published from inception to 19 May 2021. The search language was limited to English. The following search terms were used in PubMed and were changed depending on the rules of each database: (HDx) OR (Expanded HD) OR (HDx) OR (Medium cut off) OR (Mid cutoff) OR (Medium-cut off) OR (Mid-cut off) OR (MCO) OR (Mid cutoff) OR (MCO-HD) OR (MCO HD) OR (Theranova).(HD OR Renal Dialysis [MeSH Terms]) OR (HD) OR (Dialyzer) OR (Dialyzer) OR (Membrane).1 AND 2.
Liposome supported peritoneal dialysis in rat amitriptyline exposure with and without intravenous lipid emulsion
Published in Journal of Liposome Research, 2019
Robin Chapman, Martyn Harvey, Paul Davies, Zimei Wu, Grant Cave
Peritoneal dialysis is the renal replacement dialytic technique used in 11% of renal dialysis patients worldwide (Fresenius Medical 2015). The peritoneum is the membrane lining the abdominal cavity and receives 1.5% of cardiac output with capillaries separated from the abdominal cavity by a thin layer of mesothelium. Dialysis is effected through introduction of fluid to dwell in the abdominal cavity, equilibration of dialysate with peritoneal capillary blood contents, and subsequent fluid drainage. This dialytic technique is presently regarded as having no role in the clinical management of intoxication (Yates et al.2014). In LSPD the affinity of dialysate for intoxicant is increased by the addition of liposomes with an internal pH gradient held in suspension. This technique has demonstrated markedly augmented removal of verapamil, haloperidol, and amitriptyline in animal models. Additionally LSPD has been shown to reduce the duration of hypotension when used in a rat model of oral verapamil intoxication (Forster et al.2014). The technique is also experimentally effective at increasing ammonia clearance (Agostoni et al.2016). Ammonia is implicated in the cerebral edema seen with hepatic failure and LSPD has orphan drug development status in Europe in the treatment of acute and chronic hepatic failure.
Validity, reliability and minimal detectable change of the balance evaluation systems test (BESTest), mini-BESTest and brief-BESTest in patients with end-stage renal disease
Published in Disability and Rehabilitation, 2018
Cristina Jácome, Inês Flores, Filipa Martins, Conceição Castro, Charlotte C. McPhee, Ellen Shepherd, Sara Demain, Daniela Figueiredo, Alda Marques
Three renal dialysis centers participated and clinicians in each center identified eligible patients according to the following criteria: (i) had ESRD; (ii) were receiving adequate hemodialysis or peritoneal dialysis for at least three months (Kt/V >1.2) and (iii) were aged 18 years or older. Patients were excluded if they (i) had an hospitalization within the past three months due to worsening of their health status; (ii) had comorbidities that interfered with independent ambulation (e.g., hip fracture, lower limb amputation); (iii) were taking medication that could cause dizziness or affect their balance (e.g., psychotropic medications); (iv) had severe respiratory (e.g., chronic obstructive pulmonary disease), neurological (e.g., Parkinson’s disease, multiple sclerosis), musculoskeletal (e.g., severe osteoarthritis) or psychiatric (e.g., psychosis, schizophrenia) disorders, that could interfere with the measurements. After screening, patients were contacted by the clinician, who explained the purpose of the study and asked about their willingness to participate. When patients agreed to participate, an appointment with the researchers was scheduled at their dialysis center. Written informed consent was obtained prior to data collection.