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

Benzodiazepines are known to cause muscle hypotonia, but their effects on respiratory muscle function, particularly on diaphragm, have not yet been studied. Our aim was to look for any effect of lorazepam on respiratory muscle function in patients with chronic obstructive pulmonary disease (COPD). Nine stable COPD patients (mean +/- SD forced expiratory volume in one second (FEV1) 0.91 +/- 0.31 l) were included in the study. The following measurements were performed before and 1 hour after lorazepam administration (doses: 1.5 to 2 mg) by sublingual route: forced vital capacity (FVC), FEV1, maximal voluntary ventilation (MVV), arterial oxygen tension (PaO2), arterial carbon dioxide tension (PaCO2), minute ventilation (Ve), tidal volume (Vt), respiratory rate (f), inspiratory time/inspiratory plus expiratory time (Ti/Ttot)-, mean inspiratory flow (Vi), maximal inspiratory (MIP) and expiratory (MEP) pressures, maximal pleural pressure (Pplmax), transdiaphragmatic pressures (Pdi) and skeletal muscle strength and endurance. As expected, no change was noted in FVC, FEV1, FEV1/FVC (Table-1). Besides stability of expiratory flows, this denotes no change in collaboration in spite of the sedative effects of lorazepam. There was a 20% decrease in Ve, due to a Vt reduction and a small increase in PaCO2. These could be explained by the central effects of benzodiazepines. Skeletal muscle strength and endurance decreased significantly (22 and 50% respectively-Table 2), in accordance with the previously reported muscular actions of this pharmacological group. Respiratory muscle function parameters, MIP, MEP, MVV and Ppl showed significant reductions (10 to 20 per cent), as was the case with diaphragmatic function measured by Pdi (Muller maneuver with abdominal protrussion and maximal open-glottis expulsive maneuver) (Table 3). This study demonstrates that a single lorazepam dose reduces strength and endurance of respiratory muscle in chronic stable COPD patients.
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PMID:[Acute effect of lorazepam on respiratory muscles in stable patients with chronic obstructive pulmonary disease]. 964 Jul 77

The respiratory dysfunction in Duchenne muscular dystrophy (DMD) patients increases with age. We have attempted various methods of artificial respiration for them. Recently, a non-invasive positive pressure ventilation (NIPPV) became the first choice of respiration, but the chest respirator (CR) was still one of the choices. In our hospital, DMD patients with tracheostomy wearing a CR were alive for longer period, despite of respiratory dysfunction and complications. We studied 6 DMD patients with CR to evaluate nocturnal hypoxia index (NHI) by examining nocturnal periodic hypoxia, and measured oxygen saturation after 20 mg of clomipramine hydrochloride administration before sleep. Three patients had periodic nocturnal hypoxia which was prevented by the clomipramine administration. Two patients with tracheostomy did not exhibit hypoxia. We speculated that pharyngeal hypotonia during REM sleep induces periodic nocturnal hypoxia, therefore the tracheostomy would prevent hypoxia. Next, we examined the pharyngeal MRI on one DMD patient at the same disease stage who also had night NIPPV. We found that his soft palate and tongue shifted downward, which narrowed his pharynx during REM sleep. Consequently we have concluded that noctunnal periodic hypoxia is mainly caured by obstructive sleep apnea. For DMD patients with CR, the tracheostomy may be effective to prevent hypoxia during sleep.
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PMID:[The usefulness of tracheostomy in Duchenne muscular dystrophy ventilated by a chest respirator]. 1050 82

Infants with high upper airway obstruction (UAO) are managed with a variety of techniques to relieve their UAO. Among these techniques, the least invasive and safest is the nasopharyngeal tube (NPT). However, the traditional NPT is not always satisfactory, and tracheostomies need to be done. We recently described a modified NPT technique that, in contrast to the traditional tube, does not add airway dead space and resistance, is easy to use, is well-tolerated, has proven highly successful, and allows the simultaneous use of oxygen nasal prongs. This modified NPT has many advantages over the traditional NPT as a temporary management of high UAO that resolves with growth of the infant. This report highlights the respiratory care of 10 infants with high UAO (Pierre Robin syndrome, Down syndrome, Goldenhar syndrome, isolated microngathia, and idiopathic hypotonia) who were managed with a modified NPT. The modified NPT described potentially reduces the need for surgical intervention to relieve high UAO in infants.
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PMID:A modified nasopharyngeal tube to relieve high upper airway obstruction. 1073 18

CASE. Timmy was born at 32 weeks of gestation after an uncomplicated pregnancy until there was a spontaneous rupture of the membranes and preterm labor associated with chorioamnionitis. A 2-month hospitalization in the neonatal intensive care unit (NICU) was associated with pneumonia, a Grade II intraventricular hemorrhage, chronic lung disease, and a slow weight gain in the nursery. He was discharged to home with plans for ongoing care by his pediatrician. The primary care pediatrician attended a multidisciplinary conference with the NICU staff and Timmy's parents. At the time of discharge from the nursery, at 38 weeks postconceptual age, Timmy still required oral diuretics and supplemental oxygen, as well as other medications such as iron. Timmy's respiratory rates were between 40 and 60 breaths per minute at rest, with mild intercostal retractions. He was discharged with a cardiorespiratory monitor. The discharge examination revealed mild to moderate symmetrical hypotonia with intact deep tendon reflexes, shoulder girdle weakness, and a mild head lag. Timmy would regard a human face and a bright object and would follow them briefly. He became active and would thrash his extremities with minimal tactile, bright light, or auditory stimulation. Typically, he settled slowly with swaddling and a pacifier. Nursing was slow to develop; he was currently receiving one half of his calories at the breast and the remainder of his calories from bottle-feeding of fortified expressed breast milk. As she prepared for the first office visit with Timmy and his parents, the pediatrician asked herself, "What can I do to enhance the developmental outcome for this child?"
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PMID:"What can I do to enhance the development of a premature infant with chronic lung disease?". 1079 83

The technique of controlled hypotonia was developed for adequate management of anesthesia in endonasal microsurgical interventions on the paranasal sinuses in patients with chronic rhinosinusitis. A total of 92 patients with chronic polypous rhinosinusitis aged 18 to 65 years were operated using the following variants of anesthesia: local, local in combination with neuroleptanalgesia and/or ataralgesia with controlled hypotonia, endotracheal oxide-fluoroxene and endotracheal oxide-oxygen anesthesias with neuroleptanalgesia and/or ataralgesia with controlled hypotonia. This was achieved with clofelin. Controlled hypotonia reduces blood loss in endonasal microsurgery on the paranasal sinuses in patients with chronic polypous rhinosinusitis compared to patients unexposed to controlled hypotonia.
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PMID:[The use of controlled hypotonia in endonasal microsurgical interventions on the paranasal sinuses in patients with chronic rhinosinusitis]. 1101 83

This review presents our current knowledge on the genetic and phenotypic aspects of mitochondrial complex II gene defects. The mutations of the complex II subunits cause two strikingly different group of disorders, revealing a phenotypic dichotomy. Genetic disorders of the mitochondrial respiratory chain are often characterized by hypotonia, growth retardation, cardiomyopathy, myopathy, neuropathy, organ failure, and metabolic derangement. These disorders are transmitted through maternal lineage if the defective gene is located in the mitochondrial genome or may follow a Mendelian pattern if it is in the nucleus. Mitochondrial complex II (succinate:ubiquinone oxidoreductase) is the smallest complex in the respiratory chain and is composed of four subunits encoded by nuclear genes SDHA, SDHB, SDHC, and SDHD. Complex II oxidizes succinate to fumarate in the Krebs cycle and is involved in the mitochondrial electron transport chain. SDHA and SDHB encode the flavoprotein and iron-sulfur proteins, respectively, and SDHC and SDHD encode the two hydrophobic membrane-spanning subunits. While mutations in SDHA display a phenotype resembling other mitochondrial and Krebs cycle gene defects, those in SDHB, SDHC and SDHD cause hereditary paraganglioma. Paraganglioma is characterized by slow-growing vascular tumors of the paraganglionic tissue (i.e., adrenal and extra-adrenal paragangliomas, including those in the head and neck, mediastinum, abdomen, and pheochromocytomas). Paraganglioma caused by SDHD mutations occurs exclusively after paternal transmission, suggesting that genomic imprinting influences gene expression. Association of a mitochondrial gene defect with tumorigenesis expands the phenotypic spectrum of mitochondrial diseases and adds genomic imprinting as a new transmission mode in mitochondrial genetics. The phenotypic features of complex II gene mutations suggest that whereas the catalytic subunit SDHA mutations may compromise the Krebs cycle, those in other structural subunits may affect oxygen sensing and signaling.
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PMID:Phenotypic dichotomy in mitochondrial complex II genetic disorders. 1169 62

The inherited neurometabolic disease d-2-hydroxyglutaric aciduria is complicated by progressive neurodegeneration of vulnerable brain regions during infancy and early childhood, frequently presenting with hypotonia, epilepsy and psychomotor retardation. Here, we report that the pathogenetic role of the endogenously accumulating metabolite d-2-hydroxyglutarate (D-2), which is structurally similar to the excitatory amino acid glutamate, is mediated by at least three mechanisms. (i) D-2-induced excitotoxic cell damage in primary neuronal cultures from chick and rat involved N-methyl-d-aspartate (NMDA) receptor activation. Indeed, D-2 activated recombinant NMDA receptors (NR1/NR2A, NR1/NR2B) but not recombinant alpha-amino-3-hydroxy-5-methyl-4-isoxazole (AMPA) receptors in HEK293 cells. (ii) Fluorescence microscopy using fura-2 as a calcium indicator and the oxidant-sensitive dye dihydrorhodamine-123 revealed that D-2 disturbed intracellular calcium homeostasis and elicited the generation of reactive oxygen species. (iii) D-2 reduced complex V (ATP synthase) activity of the mitochondrial respiratory chain, reflecting an impaired energy metabolism due to inhibition of ATP synthesis but without affecting the electron-transferring complexes I-IV. Thus, D-2 stimulates neurodegeneration by mechanisms well-known for glutamate, NMDA or mitochondrial toxins. In conclusion, excitotoxicity contributes to the neuropathology of d-2-hydroxyglutaric aciduria, highlighting new neuroprotective strategies.
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PMID:NMDA receptor activation and respiratory chain complex V inhibition contribute to neurodegeneration in d-2-hydroxyglutaric aciduria. 1215 28

Patients with morbid obesity undergoing gastric bypass surgery present many unique challenges to the healthcare team. Obstructive sleep apnea (OSA) is a common comorbidity in the postoperative gastric bypass patient. The complexity in the management of these patients during and after anesthesia is immense. Anesthetic agents and analgesics increase the severity of symptoms of OSA. Systemic opioid administration depresses the respiratory drive and decreases oxygen saturation. In addition, normal muscle hypotonia during rapid eye movement sleep increases the chance of respiratory complications during the initial postoperative period. Continuous positive airway pressure can effectively treat sleep apnea in the postoperative gastric bypass patient. There are concerns that pressurized air can inflate the stomach and intestines and result in the disruption of the anastomoses of the gastric bypass patient. If weight loss is sufficient, it can lead to an improvement or even cure of symptoms of OSA.
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PMID:Potential complications of obstructive sleep apnea in patients undergoing gastric bypass surgery. 1604 Dec 30

A 27-year-old male intravenous drug user presented to the Emergency Department of St James's Hospital with a 1-week history of progressive dysphasia, dysphagia and difficulty 'holding his head up' and 'keeping his eyes open'. He also complained of increasing weakness in his upper limbs, as a result of which he kept dropping things. He was on a methadone program but was using both intravenous heroin and cocaine at the time of presentation. Examination of his motor function revealed generalized hypotonia, hyporeflexia and reduced power in both upper limbs. No sensory loss was observed. Co-ordination was intact. The clinical picture of a proximal symmetrical descending weakness and an absence of sensory loss was suggestive of botulism. Clostridium botulinum is a spore-forming, obligate anaerobe. The three forms of human botulism are food-borne, wound and intestinal. A fourth man-made form is produced from aerosolized botulinum toxin and results in inhalational botulism. A little as 1 g of aerosolized botulinum toxin has the potential to kill 1.5 million people. Toxin is detected in serum or stool specimens in only approximately 46% of clinically diagnosed cases. Treatment involves supportive care and early passive immunization with equine antitoxin. Patients should be regularly assessed for loss of gag and cough reflex, control of oropharyngeal secretions, oxygen saturation, vital capacity and inspiratory force. When respiratory function begins to deteriorate, anticipatory intubation is indicated. Early symptom recognition and early treatment with antitoxin are essential in order to prevent mortality, and to prevent additional cases, it is important to ascertain the presence of similar symptoms in contacts of the patient and local public health officials must be notified as one case may herald an outbreak. Given the continued threat of bioterrorism, the Centre for Disease Control Surveillance System in the United States must also be notified of any cases of botulism.
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PMID:Descending polyneuropathy in an intravenous drug user. 1617 64

Asphyxia i a condition caused by lack of oxygen in tissues and organs. The basic pathogenic mechanisms of asphyxia are: 1)hypoxemia, 2) ischemia. The effects of perinatal asphyxia on the brain of a neonatal baby are critical in development of hypoxic-ischemic encephalopathy. The diagnosis of hypoxic-ischemic encephalopathy is based on clinical data including course of pregnancy and delivery (Apgar score) and especially on the neurological status of the newborn (consciousness, tonus, convulsions, reflexes, vegetative functions, etc.) and it can be confirmed by biochemical analysis and neurological examinations. The aim of this paper is to determine the importance of prenatal and perinatal risk factors for hypoxic-ischemic encephalopathy, as well as their effects on the development of neurological complications and further neurological problems. The research included 148 newborn infants born in the period from January 1, 1996 to January 1, 1999, with gestational age of 27 to 42 weeks, with hypoxic ischemic lesions of the central nervosus system. The control group included 58 children of the same age and the same gestation, with generalized hypotonia ("floppy infant") but without any signs of hypoxic ischemic lesions of the central nervous system. In the group of examined newborn infants with hypoxic ischemic lesions, from 149 children 1 (0.67%) died, 87 (53.89%) had normal findings, whereas the handicap was established in 61 (40.94%). Perinatal asphyxia affects the fetus and newborn infants not by individual factors, but with at least three or four associated factors. The disorders caused by asphyxia are in inverse proportion to the duration and intensity of hypoxic insults and the gestational age of the newborn.
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PMID:Monitoring of neurological parameters in newborns with hypoxic-ischemic encephalopathy. 1763 93


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