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Biomedical subjects

I Shapiro

Publications and source records attributed to I Shapiro.

18 recordsLinked to original sources

Neu-Laxova syndrome: prenatal ultrasonographic diagnosis, clinical and pathological studies, and new manifestations.

A diagnosis of the Neu-Laxova syndrome (NLS) was made by ultrasonography at 32 wks of gestation. Ultrasonographic examination showed intrauterine growth retardation (IUGR), Dandy-Walker anomaly, choroid plexus cysts, receding forehead and microcephaly, bilateral cataract without prominent eyes, scalp edema with no generalized edema, retrognathia, curved penis, and flexion deformities of limbs. The findings in this case are consistent with NLS; however, they did not fit any of Curry's [1982] groups. Massive swelling of hands and feet were among the main manifestations in classic NLS cases. In the case presented herein, edema was noted only in the scalp. This might shed further light on the question of variability vs. heterogeneity in the NLS. This case shows the existing possibility of an early diagnosis of NLS and adds Dandy-Walker anomaly and choroid plexus cysts as new findings to this syndrome.

Abnormalities, Multiple

Doppler flow velocity waveforms in fetal surveillance of twins: a prospective longitudinal study.

In 37 consecutive twin pregnancies, monthly Doppler sonographic measurements were made of blood flow velocity waveforms of the internal carotid and umbilical artery of each fetus. A total of 197 Doppler studies were performed. Of the 74 infants, 23 were small for gestational age (SGA). Thirty-five of the SGA Doppler studies were abnormal, giving an overall sensitivity for predicting SGA fetus of 58% and a positive predictive value of 71%. These data are not as sensitive and specific as our earlier data; however, Doppler criteria preceded sonographic diagnosis of SGA by a mean interval of 3.7 weeks and demonstrated better sensitivity and specificity. A combination of these parameters improves sensitivity to 84%. We conclude that Doppler velocimetry complements real-time ultrasonography for the early diagnosis of abnormal growth in twin pregnancies.

Blood Flow Velocity

[Fetal echocardiography].

Fetal echocardiography is an ultrasonic technique for the diagnosis of cardiac defects and fetal arrhythmias. During 1981-1990 we examined 1800 women referred for elective examinations from all over the country. Most, at high risk for fetal cardiac anomalies, were examined in the 22nd week of gestation. Others were examined whenever a fetal arrhythmia or a cardiac defect was suspected. In 251 fetuses (13.9%) pathological cardiac conditions were found, and structural heart defects in 51 (2.8%). When a cardiac defect with poor prognosis was diagnosed during the 2nd trimester, termination of pregnancy was suggested. In 35% of these cases termination of pregnancy was accepted. In 12% of the women examined spontaneous intra-uterine fetal death had occurred, while in 52% live babies were born, but half died within 6 months. In 215 fetuses (11.9%) a cardiac arrhythmia was found, which in 73% consisted of supraventricular premature beats. In 15.8% the fetus was endangered by the arrhythmia, mainly supraventricular tachycardia, atrial fibrillation or atrial flutter. Treatment was by drugs administered to the mother. In 1 case of complete A-V block echocardiographic follow-up demonstrated rapid deterioration of heart function. Delivery was induced and a pacemaker was implanted in the newborn.

Arrhythmias, Cardiac

Delirium: phenomenologic and etiologic subtypes.

While all delirious patients have clouding of consciousness (alteration of attention) and cognitive dysfunction, the level of alertness of different patients may range from stuporous to hyperalert. We, therefore, developed an analog scale to rate the alertness of delirious patients, and a separate scale to rate the severity of their clouding of consciousness. Based on these scales, patients were categorized overall as relatively "activated" (relatively alert despite clouding of consciousness), or "somnolent" (relatively stuporous along with clouding of consciousness). Cognitive function was estimated using the Mini-Mental Status Exam. Separate ratings were made of hallucinations, delusions, illusions, and agitated behavior. Activated and somnolent patients had similar ages, overall severity of delirium, and Mini-Mental Status Exam scores. Activated patients, however, were more likely to have hallucinations, delusions, and illusions than somnolent patients, and were more likely to have agitated behavior. Patients with hepatic encephalopathy were more likely to have somnolent delirium, while patients with alcohol withdrawal appeared more likely to have activated delirium. These data indicate that phenomenologic subtypes of delirium can be defined on the basis of level of alertness. These subtypes are validated in part by their differing associations with symptoms unrelated to alertness. These subtypes may have different pathophysiology, and thus, potentially different treatments.

Aged

Fetal blood flow velocity waveforms in pregnancies complicated by intrauterine growth retardation.

Fifty-three pregnancies suspected for a small-for-gestational age fetus (SGA) by ultrasonographic weight estimation (below the 10th percentile of Brenner nomograms) were studied using pulsed Doppler (Duplex) for recording fetal blood flow velocity waveforms from the umbilical artery and fetal internal carotid artery. The pulsatility index (PI) for each artery was calculated as an index of vascular resistance. The gestational age ranged from 29 to 40 weeks. In 42 cases an SGA newborn was delivered (according to Brenner tables corrected for maternal parity and fetal sex), 34 newborns were SGA according to Usher and McLean (2 SD below the mean). Fetal structural and/or chromosomal defects were found in four cases, all of which showed symmetric intrauterine growth retardation and normal PI values in the fetal internal carotid artery. After excluding the infants with congenital abnormalities and using nomograms constructed for our own population, the ratio between the PI of the umbilical artery and the internal carotid artery was found to be the best predictor of SGA (sensitivity 84.2%, specificity 90.9%, positive predictive value 97.0%, negative predictive value 62.5%). Using the more demanding criteria for SGA according to Usher and McLean, sensitivity improved to 100%, specificity was 80.0%, positive predictive value 87.9%, and negative predictive value 100%.

Blood Flow Velocity

Evaluation of relationship between hearing threshold and loudness discomfort level in sensorineural hearing loss.

The relationship between hearing level and loudness discomfort level (LDL) for narrow-band noise was evaluated in two groups of patients with sensorineural hearing loss. Group I had thresholds ranging from 25-60 dB SPL and Group II's thresholds ranged from 65-100 dB SPL. LDLs were determined for narrow bands of noise centered at 500, 1000, 2000, and 4000 Hz. The LDLs for Group II were greater than those for Group I and the differences were statistically significant. It is speculated that one reason for others not finding differences as a function of hearing level may be the absence of severe to profound hearing loss in the test populations.

Adolescent

Oculomotor programming disturbances in the dementia syndrome.

Three types of oculomotor programming disturbances are described in patients with the dementia syndrome. This report describes two of these abnormalities, poorly regulated gaze patterns and a programming type of gaze perseveration. All patients showed poorly regulated gaze patterns in contrast to the normal controls, whereas on the whole only the moderately affected patients showed the programming type of gaze perseveration. The third type of oculomotor programming disturbance is efferent perseveration of gaze (a hypokinetic, "staring" type of gaze pattern) and can be seen with severe dementia. The possible significance of these findings is discussed.

Aged

Comparison of unaided MCLs and use-gain of hearing aids.

Unaided MCLs for narrow bands of noise centered at 1 000, 2 000, 3 000 and 4 000 Hz were compared with the use-gain of hearing aids worn by 10 subjects with sensorineural hearing loss. In addition, for eight subjects, MCLs measured at the time of the hearing aid evaluation were compared with MCLs observed at the time of hearing aid follow-up. Results of the study suggest that (1) hearing aid use-gain can be accurately predicted on the basis of the MCLs for the narrow bands of noise, and (2) the hearing-impaired subjects were highly consistent in their MCL judgments for narrow-band noise.

Consumer Behavior

Children's use of CROS hearing aids.

Ten children with unilateral hearing loss, aged 7 to 17 years, who were fit with contralateral routing of signal (CROS) hearing aids were followed up for three years to assess the suitability of this type of hearing aid fitting on children. The determination of the success of the fitting was based on follow-up visits, chart review, and response to a questionnaire sent to the parents. The fitting was considered to be successful if the child accepted the aid and if parents and teachers observed substantial improvement in academic performance and/or social behavior. Seven children were considered to be successful users of their CROS aids; one was unavailable for follow-up; one did better with a BICROS aid; and one did not do well with amplification. Our experience suggests that children with unilateral hearing loss can benefit from CROS amplification.

Adolescent

Hearing aid fitting by prescription.

A procedure for the specification of hearing aid response characteristics is described. Thresholds of discomfort and most comfortable levels for narrow bands of noise centered at 0.25, 0.5, 1, 2, 3 and 4 kHz are determined. The thresholds of discomfort relate to the maximum power output of the hearing aid and the most comfortable levels determine the gain and frequency response that will be required. The data derived from these measurements are given to the hearing aid dispenser who provides a hearing aid that matches the prescribed response characteristics. This technique requires less time than the conventional hearing aid evaluation procedure, and it provides the audiologist with measures of auditory function that can be directly related to hearing aid parameters.

Audiometry

Prediction of most comfortable loudness levels in hearing aid evaluation.

Most comfortable loudness levels (MCLs) are used to determine the gain required in hearing aids. The relationship between MCL and the midpoint between pure-tone threshold and threshold of discomfort was evaluated to see if the MCL could be predicted from the midpoint values. The range of differences between midpoint and MCL precluded making such predictions.

Adolescent

Congenital mitral stenosis.

This report presents a 15 year review of the surgical treatment of 9 patients with congenital mitral stenosis seen at the Columbus Children's Hospital. The over-all mortality rate was 45 per cent. Seven patients had associated lesions, mostly coarctation of the aorta and patent ductus arteriosus. In the planning of the operative procedure, distal obstructive lesions of the left heart should generally be relieved first. The mitral valve should be explored with the use of cardiopulmonary bypass and the anatomic type of the valve determined. Type I valves will often respond to open valvulotomy, whereas Type II and III valves must be replaced.

Adolescent