Respiratory dysfunction in myotonic dystrophy type 1: A systematic review.

Myotonic dystrophy type 1 (DM1) is one of the most common muscular dystrophies in adults. This review summarises the current literature regarding the natural history of respiratory dysfunction in DM1, the role of central respiratory drive and peripheral respiratory muscle involvement and its significance in respiratory function, and investigates the relationship between genetics (CTG repeat length) and respiratory dysfunction. The review included all articles that reported spirometry on 10 or more myotonic dystrophy patients. The final review included 55 articles between 1964 and 2017. The major conclusions of this review were (1) confirmation of the current consensus that respiratory dysfunction, predominantly a restrictive ventilatory pattern, is common in myotonic dystrophy and is associated with alveolar hypoventilation, chronic hypercapnia, and sleep disturbance in the form of sleep apnoea and sleep related disordered breathing; (2) contrary to commonly held belief, there is no consensus in the literature regarding the relationship between CTG repeat length and severity of respiratory dysfunction and a relationship has not been established; (3) the natural history and time-course of respiratory functional decline is very poorly understood in the current literature; (4) there is a consensus that there is a significant involvement of central respiratory drive in this alveolar hypoventilation however the current literature does not identify the mechanism for this.

Biases in young children's communication about spatial relations: containment versus proximity.

Four experiments examined 3- and 4-year-olds' ability to communicate about containment and proximity relations. One hundred twenty-eight children either described where a miniature mouse was hiding in a dollhouse or they searched for the mouse after the experimenter described where it was hiding. The mouse was always hidden with a small landmark that was either in or next to a large landmark. When describing where the mouse was hiding, children were more likely to successfully disambiguate the small landmark when it was in the large landmark (e.g., under the plant in the dresser) than when it was next to the large landmark (e.g., under the plant next to the dresser). When searching for the mouse, 3-year-olds were faster to initiate their searches when the small landmark was in the large landmark than when it was next to the large landmark. Together, these results suggest that there are informational biases in young children's spatial communication.

Effects of auditory stimuli on intracranial pressure and cerebral perfusion pressure in traumatic brain injury.

The primary goal of medical and nursing management in the traumatic brain-injured patient is to decrease intracranial pressure (ICP) and maintain adequate cerebral perfusion pressure (CPP). Little is known about what effect, if any, auditory stimulation has on these parameters. Therefore, an experimental study was conducted to examine the effects of various auditory stimuli on intracranial pressure (ICP) and cerebral perfusion pressure (CPP) in traumatic brain-injured patients. A convenience sample of fifteen participants with admitting Glasgow Coma Scale scores between 3 and 8 was studied. Three types of auditory stimuli: earplugs, a music tape and a tape of ICU environmental noise were applied to patients in a computer-generated random order. The tapes delivered sound at 70 decibels. Each intervention lasted 15 minutes. ICP and CPP were recorded at a 30 second resolution time through a bedside computer. Data were analyzed using analysis of variance for a cross over design. Results showed no statistically significant change in ICP or CPP during the study period. Further studies are needed to examine the effects of auditory stimuli on ICP and CPP in traumatic brain-injured patients.

Penetrating head injury.

Penetrating head injuries are a significant public health problem in the United States, with an estimated 33,000 gun-related deaths and many more nonfatal shootings per year. Initial treatment for a penetrating head injury is similar to that of a closed head injury. That is, all efforts must be made to prevent any secondary insults, hypoxia, or ischemia. This translates into the standard methodology of care of the trauma patient. The basic neurologic examination consists of using the Glasgow Coma Score and estimation of pupillary function and some estimation of brain stem function and motor power. The radiologic test of choice for a penetrating head injury is the computed tomography scan. There are several indications for surgery: the patient's condition will be improved or significant neurologic sequelae will be averted; the patient is sufficiently stabilized from any other injuries such that he or she can tolerate surgery; the condition of the patient is not so poor that surgery will have no effect; and the area of penetration is reasonably accessible to surgical intervention. Three main goals of medical management of a penetrating head injury include (1) control of hypertension, (2) maintenance of adequate cerebral circulation oxygenation, and (3) prevention of secondary complications. Outcome after a penetrating head injury is related to the extent of brain tissue damage caused either directly or indirectly by the missile as well as any indirect insults. The most significant indicator, particularly in terms of survival versus death, has consistently been the patient's presenting neurologic status. Some investigators have recommended that a patient presenting with a Glasgow Coma Scale Score of > 5 should not be treated. Others have said that patients with a low coma score and transventricular gunshot wounds should not be treated because of the high mortality. If the patient survives a penetrating head injury, he or she generally goes on to experience a relatively good functional outcome. Only if all components of a good treatment regimen are in place will patients and their families obtain the best possible outcome.

Behavioral assessment of the effects of psychotropic medications on demented nursing home residents.

The effects of different psychotropic medications were examined for the control of behavior problems associated with dementia in three elderly nursing home residents. A reversal design was used in which the medications were introduced and withdrawn, and their effects were assessed on various resident behaviors using behavioral and motor performance assessments. All medications were effective in decreasing aberrant behaviors, but they also increased sedation in all three subjects. Performance on the motor assessments decreased for all subjects after the introduction of medication and improved after the medication was withdrawn. The results highlight the utility of behavioral assessment strategies for assessing the effects of psychotropic medication on elderly nursing home residents.

The community pharmacist as a resource for the family physician.

The family physician's relationship with the community pharmacist has tended to be biased. The physician sees the pharmacist simply as a dispenser of drugs. Physicians and pharmacists are usually physically separated, lessening their chances of a collaborative working relationship. Family physicians' traditional sources of drug information include journals, colleagues and drug company literature. However, when they have some form of regular interaction with a pharmacist, physicians tend to see the pharmacist as a main source of drug information. The proper use of medication involves three critical relationships: doctor/patient, doctor/pharmacist, and pharmacist/patient. The doctor/pharmacist relationship has several components: individual consultations, regular team meetings, and establishment of a limited formulary for physicians and residents. There is evidence that compliance is improved when the pharmacist is involved in patient education.

Adrenocortical function: corticosterone levels in female BALB/c and C3H mice under various conditions.

A diurnal rhythmicity in plasma corticosterone levels was demonstrated in female BALB/cCrgl and C3H/Crgl mice, with and without mammary tumor virus. Removal of the adrenals followed by metopirone treatment reduced circulating corticoid to non-detectable levels in C3H but not in BALB/c mice. Dexamethasone strains. Neonatal exposure to exogenous hormones failed to cause any obvious change in corticoid levels. Bilateral ovariectomy of these neonatally treated mice at 40 days of age resulted in a subsequent lowering of plasma corticoid levels when compared with intact animals.