Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0026827 (hypotonia)
 5,860 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The following report of our experience using isobaric Bupivacaine 0.5% in 6,228 operations (intrathecal technique) will consider the intra operative period. In 55.8% of cases the lower extremities were operated upon and in 44.2% the lower abdomen. In 1,487 cases (23.9%) side effects/complications were evident and a classification of such is as follows: anaesthetic technique 5.29%, cardiorespiratory 15.69%, operator/operative technique 6.13%. The percentage remained relatively constant irrespective of age but a higher pre op risk classification (A.S.A. System) resulted in a marked increase in the above figures. The principal side effects encountered were as follows: Bradycardia 7.5%, hypotonia 6.3%, insufficient analgesia 3.9%, extrasystoles 2.9%. Grave complications occured in 0.15% and within this group 4 patients died intraoperatively; a relationship with the mode of anaesthesia has not been proven.
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PMID:[Spinal anaesthesia using bupivacain--clinical experience of more than 6000 cases (author's transl)]. 49 24

Globoid cell leukodystrophy (GLD; Krabbe disease), is a rare heritable metabolic disorder in humans, dogs, mutant twitcher mice, and rhesus monkeys that is caused by a deficiency in the lysosomal enzyme galactocerebrosidase (GALC). GALC deficiency results in the accumulation of psychosine, which is toxic to oligodendrocytes and Schwann cells of the central and peripheral nervous systems. Clinical signs include hypotonia, mental regression, and death by 2 years of age in most human patients. Here we describe a domestic longhaired kitten with rapidly progressive neurologic disease and brain and spinal cord lesions characteristic of GLD. Pathologic hallmarks of the disease reflect the loss of oligodendrocytes and include myelin loss, gliosis, and the perivascular accumulation of large mononuclear cells with fine cytoplasmic vacuoles (globoid cells) in the peripheral and central nervous systems. Globoid cells were CD68 and ferritin positive, confirming their monocytic origin, and cytoplasmic contents were nonmetachromatic and periodic acid-Schiff positive.
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PMID:Globoid cell-like leukodystrophy in a domestic longhaired cat. 1212 53

Use of propofol in surgery on critical patients is limited because of the possibility of poorly corrected hypotonia. The effects of total anesthesia with propofol + fentanyl + nitric oxide under conditions of artificial ventilation of the lungs (tracrium) were evaluated during 63 operations on 42 patients with severe burns (ASA III-IV), divided into 2 groups depending on the protocol of anesthesia. The difference in the protocols consisted in the method of choice of anesthetic doses: in group A we proceeded from EEG data (bispectral index--BIS) and in group B relied only on general clinical and hemodynamic signs with a retrospective analysis of BIS. Prevention of hemodynamic disorders in both groups included infusion loading (7-8 ml/kg), dopamine (5-7 micrograms/kg/min) and decrease of the velocity of propofol infusion to 15 mg/kg/h during induction anesthesia. This method leveled the hypodynamic effects of propofol. Simultaneous monitoring of BIS showed that the propofol dose needed for adequate induction narcosis with subsequent intubation should be higher than the dose usually recommended for patients with ASA class III-IV. Use of BIS monitoring during the operation resulted in a decrease of the propofol and fentanyl doses. The authors do not recommend decreasing the velocity of propofol infusion below 3 mg/kg/h (at FiN2) = 0.6) because of the risk of awakening during narcosis.
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PMID:[Propofol-based protocol of general anesthesia for operations in patients with severe burns with ASA class III-IV]. 1222 78

ProSeal(TM) Laryngeal Mask Airway (PLMA) for children had been introduced in 2004, by Dr. Archie Brain. It has, in addition to Classic Laryngeal Mask Airway (CLMA), a drainage tube for providing a bypass channel for gastric contents to prevent regurgitation and pulmonary aspiration. A randomized prospective study was performed comprising of 60 ASA - I/II patients, between the age groups of 3 and 10 years, of either sex. All the patients were premedicated with oral Midazolam and Glycopyrollate. General anaesthesia with caudal epidural analgesia was given in all the cases. Inhalation with 8% Sevoflurane was used as a sole induction agent in all the patients. They were randomly divided into two groups. PLMA was inserted in patients of Group P and Endotracheal Tube (ETT) in patients of Group I. In all cases, after PLMA / ETT insertion; caudal epidural analgesia was given and general anaesthesia (GA) using Sevoflurane was provided for maintenance of anaesthesia. Muscle relaxant was not used in our study. We studied parameters such as number of attempts, ease of insertion and conditions during insertion, haemodynamic parameters, changes in SpO(2), EtCO(2), gastric insufflation, regurgitation, pulmonary aspiration, postoperative airway complications and so on. We found that insertion of PLMA as well as ETT was performed in the first attempt in all the patients. Ease of insertion and conditions during insertion were comparable in both the groups. Changes in SpO(2) and EtCO(2) were comparable. However, highly significant changes in haemodynamic parameters were observed in the ETT group. Complications such as sore throat (13.33% cases), coughing (40% cases), vomiting (3.33% cases) and hypoxia (3.33% cases) were observed in the ETT group. No gastric insufflation or regurgitation was noted in our study. Thus, we concluded that PLMA could be used as an effective and safe airway device in children compared to ETT undergoing general anaesthesia.
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PMID:Prospective randomized comparative study of use of PLMA and ET tube for airway management in children under general anaesthesia. 2066 47

Stiff Person Syndrome (SPS), typified by rigidity in muscles of the torso and extremities and painful episodic spasms, is a rare autoimmune-based neurological disease. Here we present the successful endotracheal intubation and application of TIVA without muscle relaxants on an SPS patient. A 46 years old male patient was operated with ASA-II physical status because of lumber vertebral compression fracture. After induction of anesthesia using lidocaine, propofol and remifentanil tracheal intubation was completed easily without neuromuscular blockage. Anesthesia was maintained with propofol, remifentanil and O2/air mixture. After a problem-free intraoperative period the patient was extubated and seven days later was discharged walking with aid. Though the mechanism is not clear neuromuscular blockers and volatile anesthetics may cause prolonged hypotonia in patients with SPS. We think the TIVA technique, a general anesthetic practice which does not require neuromuscular blockage, is suitable for these patients.
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PMID:Anesthesia in a patient with Stiff Person Syndrome. 2759 71

Down syndrome (DS) is a congenital disorder caused by trisomy 21 (T21). It is associated with cognitive impairment, muscle hypotonia, heart defects, and other clinical anomalies. At the same time, individuals with Down syndrome have lower prevalence of solid tumor formation. To gain new insights into aberrant DS development during early stages of mesoderm formation and its possible connection to lower solid tumor prevalence, we developed the first model of two types of DS iPSC-derived stromal cells. Utilizing bioinformatic and functional analyses, we identified over 100 genes with coordinated expression among mesodermal and endothelial cell types. The most significantly down-regulated processes in DS mesodermal progenitors were associated with decreased stromal progenitor performance related to connective tissue organization as well as muscle development and functionality. The differentially expressed genes included cytoskeleton-related genes (actin and myosin), ECM genes (Collagens, Galectin-1, Fibronectin, Heparan Sulfate, LOX, FAK1), cell cycle genes (USP16, S1P complexes), and DNA damage repair genes. For DS endothelial cells, our analysis revealed most down-regulated genes associated with cellular response to external stimuli, cell migration, and immune response (inflammation-based). Together with functional assays, these results suggest an impairment in mesodermal development capacity during early stages, which likely translates into connective tissue impairment in DS patients. We further determined that, despite differences in functional processes and characteristics, a significant number of differentially regulated genes involved in tumorigenesis were expressed in a highly coordinated manner across endothelial and mesodermal cells. These findings strongly suggest that microRNAs (miR-24-4, miR-21), cytoskeleton remodeling, response to stimuli, and inflammation can impact resistance to tumorigenesis in DS patients. Furthermore, we also show that endothelial cell functionality is impaired, and when combined with angiogenic inhibition, it can provide another mechanism for decreased solid tumor development. We propose that the same processes, which specify the basis of connective tissue impairment observed in DS patients, potentially impart a resistance to cancer by hindering tumor progression and metastasis. We further establish that cancer-related genes on Chromosome 21 are up-regulated, while genome-wide cancer-related genes are down-regulated. These results suggest that trisomy 21 induces a modified regulation and compensation of many biochemical pathways across the genome. Such downstream interactions may contribute toward promoting tumor resistant mechanisms.
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PMID:iPSC-derived progenitor stromal cells provide new insights into aberrant musculoskeletal development and resistance to cancer in down syndrome. 3276 7