SNAP-II predicts mortality among infants with congenital diaphragmatic hernia.

OBJECTIVE: Outcomes analysis in congenital diaphragmatic hernia (CDH) requires a validated risk-adjustment tool. The purpose of this study was to use the Canadian Neonatal Network (CNN) database to validate the Score for Neonatal Acute Physiology, Version II (SNAP-II) for prediction of mortality among CDH infants admitted to a neonatal intensive care unit (NICU), and to compare this to the predictive equation recently developed by the Congenital Diaphragmatic Hernia Study Group (CDHSG). STUDY DESIGN: Infants with CDH in the CNN database were identified. Bivariate and multivariable logistic regression models were used to identify risk factors predictive of mortality. Model predictive performance and calibration were assessed using the area under the receiver operator characteristic curve and the technique of Hosmer-Lemeshow, respectively, and compared with the CDHSG predictive equation. RESULTS: There were 88 patients with CDH among 19,507 admissions to CNN hospitals. The mortality rate among CDH patients surviving to NICU admission was 17%, and 12.5% received extracorporeal membrane oxygenation therapy. Gestational age and admission SNAP-II score predicted mortality. Model predictive performance and calibration were optimized with these variables combined. The CDHSG equation was equally predictive of mortality, but was only marginally calibrated. CONCLUSIONS: SNAP-II is highly predictive of mortality among patients with CDH, and can be used to risk-adjust these patients.     

Multiple sclerosis with higher cerebral dysfunction: a case report

Higher cerebral dysfunctions such as aphasia, apraxia and agnosia have seldom been reported in multiple sclerosis (MS). 12 year-old right-handed boy felt unsteadiness of the body and headache for several days. Two months later, he had the same episode and complained of visual disturbance, and weakness and sensory disturbance on the face and the extremities. Additionally, he showed amnestic aphasia, acalculia, ideomotor apraxia, finger agnosia and right-left disorientation. Cerebrospinal fluid examinations revealed increases IgG, myelin basic protein and neuron specific enolase (11%, 25 ng/ml and 28.8 ng/ml, respectively). X-ray CT scan and MRI-CT examinations revealed sclerotic lesions on the left parietal white matter and the right mid-brain. The diagnosis was made as MS. He was treated with m-PSL (methyl-prednisolone) pulse therapy for three weeks and consecutively treated with PSL for four weeks. He recovered gradually, but visual disturbance and facial palsy remained. After seven months MRI-CT showed a high signal intensity on the left parietal white matter in spite of the disappearance of the lesion on X-ray CT scan. We suggest that these higher cerebral dysfunctions may result from the lesion of the left parietal white matter which produces a disconnection between each cortical area.     

X-ray CT of Duchenne muscular dystrophy skeletal muscles--chronological study for five years]

To clarify when and how rapidly individual muscles are damaged in the course of Duchenne muscular dystrophy (DMD), we followed X-ray CT of whole body skeletal muscles from 29 cases of DMD patients (age ranging 3 to 23 years) for a period of 2 to 6 years. Each patient had 2 to 5 scans with an average of 3.3. We evaluated chronological changes of 23 muscles from the entire body using the muscle damage stage defined as follows. The stages were classified into the following 5 stages: stage 0; normal, 1; area of fatty replacement less than 10% of whole muscle area, 2; area of fatty replacement between 10 to 50%, 3; area of fatty replacement 50 to 90%, 4; almost complete fatty replacement. Each muscle had its own period of rapid degeneration starting at 5 to 10 years of age and, continuing 5 to 10 years. In some muscles such as gluteus maximus or quadriceps femoris, fatty replacement started at 5 years or earlier and progressed for five years, while other muscles such as splenius capitis, damage started much later, e.g. around 10 years of age and the progression was much slower. There was a variation of at least 5 years among individual patients in any muscle damage stage, reflecting the variability of clinical severity in each patient. On the basis of the above results we defined the whole body muscle damage index as a summation of the muscle damage stages of the following five muscles: gluteus maximus, quadriceps femoris, gracilis, medial head of gastrocnemius and splenius capitis.(ABSTRACT TRUNCATED AT 250 WORDS)     

Foregoing prehospital care: should ambulance staff always resuscitate?

Approximately 400,000 people die outside US hospitals or chronic care facilities each year. While there has been some recent movement towards initiating procedures for prehospital Do Not Resuscitate (DNR) orders, the most common situation in the US is that emergency medical systems (EMS) personnel are not authorized to pronounce patients dead, but are required to attempt resuscitation with all of the modalities at their disposal in virtually all patients. It is unfair and probably unrealistic for EMS personnel to have to make a determination of the validity of a non-standard prehospital DNR order (for example, a living will or a durable power of attorney for health care). Existing prehospital DNR protocols range from being very restrictive in the scope of patients allowed to participate and in their implementation, to those that are more liberal. Potential benefits of prehospital DNR orders include freeing up vital personnel and material for use by those who would more fully benefit, and alleviating the enormous emotional strain on patients, families, EMS personnel, and hospital medical staffs involved in unwanted resuscitations that only prolong the dying process. Given this, prehospital DNR orders present several legal and moral problems. These include proper patient identification, the nature of the document itself, precautions incorporated into a DNR system to prevent misuse, potential liability for EMS and hospital personnel, and potential errors in implementation. Functioning prehospital DNR systems need to include: 1) specific legislation detailing the circumstances in which such a document could be used, the wording of such a document, and protection from liability for those implementing the document's directives; 2) having the currently valid document immediately available to the EMS personnel or base station doctors; and 3) acceptable means of identifying the patient. Relatively few US jurisdictions as yet have a prehospital DNR order system, although it is an idea whose time is overdue. Society's imperative to use available technology has pushed us into a situation where a technique to save those with a potential to continue a meaningful and wanted existence is being used indiscriminately to prolong the agony of death.     

Thyroid function in severely disabled children]

Thyroid function tests were studied in 105 severely disabled children. Fifty-four cases (53%) showed following abnormalities. Serum TSH concentration was increased in 2 cases. Both serum T4 and T3 levels were decreased in 18 cases (17%). Only the serum T4 level was decreased in 25 cases (24%). Only the serum T3 level was decreased in 10 cases (9.5%). Two patients showed primary hypothyroidism. Two patients (monozygotic twins) were suspected to have subclinical hypothalamic-pituitary hypothyroidism caused by septo-optic-dysplasia. Abnormal thyroid functions were caused by anticonvulsants in most patients. The serum T4 level was correlated with the number of anticonvulsants, but not correlated with motor performance (daily activity) or feeding function. Four patients who had low T4 level or low T4 and T3 levels were received L-thyroxine supplementation, but no clinical improvement was observed. The serum TSH concentration was decreased and the TRH test showed hyporeaction in all of these cases. The low T3 level and normal T4 level were not related to anticonvulsant administration. Two of these patients were in poor nutritional condition, resulting in so called low T3 syndrome.     

Changes in women's mental and physical health from pregnancy through six months postpartum

A longitudinal study was conducted to investigate changes in women's mental and physical health around the time of childbirth, and to determine whether health was related to length of maternity leave. Thirty-seven married, employed first-time mothers completed questionnaires during pregnancy, and again at 6 weeks, 3 months, and 6 months postpartum. Results showed that, from pregnancy to the 6th postpartum month, the number of days that mothers were ill because of infections steadily increased. In addition, depressive symptoms for new mothers rose from pregnancy to the 6th week postpartum, and declined thereafter. For women who did not return to work during the period of the study, a significant decline in depressive symptoms was observed from the prenatal period through the 6th postpartum month. These findings demonstrate significant changes in mental and physical health for this group of first-time mothers.     

Effect of selenium on the connective tissue metabolism in rats]

Rats were chronically exposed to poisonous sodium selenite in concentrations 0.5 mg/kg b. m. for 10 weeks. As a result, hydroxyproline contents in blood serum increased, as well as its excretion with urine. It was proved that the procedure enhanced urinary glycosaminoglycane excretion and increased the contents of the protein-related glycosaminoglycanes in the serum. This was accompanied by increased quantities of keratane sulphate and heparin sulphate. The results confirmed that selenium poisonings influenced connective tissues.     

HEAD CIRCUMFERENCE AND INTELLECTUAL PERFORMANCE OF PATIENTS WITH DUCHENNE MUSCULAR DYSTROPHY

The head circumferences of 64 patients with Duchenne muscular dystrophy were measured and found to be greater than those of a normal population. The patients had relative, and in 12 cases, absolute macrocephaly. 47 of the 64 patients underwent intelligence testing and were found to be significantly intellectually impaired, particularly in verbal and language skills. There was no correlation between head circumference or absolute macrocephaly and intellectual performance. The cause of macrocephaly in these patients is unknown and its relevance to the aetiology of intellectual impairment in Duchenne muscular dystrophy is not yet clear.     

Myelinated fiber regeneration after crush injury is retarded in sciatic nerves of aging mice

To compare nerve regeneration in young adult and aging mice, the right sciatic nerves of 6- and 24-month-old mice were crushed at the sciatic notch. Two weeks later, both groups of mice were perfused with an aldehyde solution, and, after additional fixation, the sciatic nerves were processed so that the transverse sections of each nerve subsequently studied by light and electron microscopy included the entire posterior tibial fascicle 5 mm distal to the crush site. The same level was sectioned in unoperated contralateral nerves; these nerves served as controls. Electron micrographs and the Bioquant Image Analysis System IV were used to measure areas of posterior tibial fascicles and count the number of myelinated axons, the number of unmyelinated axons, and their frequency in Schwann cell units. In aging mice, the total number of regenerating myelinated axons was significantly reduced, but totals of regenerating unmyelinated axons in aging and young adults did not differ significantly. In aging mice, the frequency of Schwann cells that contained a single unmyelinated axon was greater, suggesting that before myelination began, Schwann cell ensheathment of axons also was slowed. After axotomy by a crush injury, the area of the posterior tibial fascicle was less than that in young adults and the distal disintegration of myelin sheath remnants also appeared to be retarded. The results indicate that responses of neurons, axons, and Schwann cells could be important in slowing the regeneration of myelinated fibers found in sciatic nerves from aging mice.