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Biomedical subjects

E Sohar

Publications and source records attributed to E Sohar.

At least 19 recordsLinked to original sources
PubMed1992

Twin studies in familial Mediterranean fever.

Familial Mediterranean fever (FMF) is a genetic disease characterized by recurrent short episodes of fever, accompanied by peritonitis, pleuritis, or arthritis. The disease is almost completely ethnically restricted to patients of Mediterranean descent--Sephardic Jews, Armenians, Anatolian Turks, and Arabs. Although many family studies have been performed, no twin study has been reported as yet. We studied 21 di- and monozygotic twin sets, identified among the 1,943 FMF patients in our registry. Full concordance was observed in all the 10 monozygotic twin sets. In the 11 dizygotic twins, concordance for FMF disease was found in only 3 pairs. Variability in the clinical manifestations and degree of severity have been noted within twins. These findings provide definitive evidence for the genetic cause of FMF. They also support the single gene autosomal recessive model, and provide support for the contention that the lower observed than expected incidence found in FMF is due to genetically affected but clinically undiagnosed patients.

Adolescent↗
PubMed1992

Colchicine prevents kidney transplant amyloidosis in familial Mediterranean fever.

Twenty-one familial Mediterranean fever (FMF) patients who received a kidney transplant for terminal renal failure due to amyloidosis were studied retrospectively to evaluate the prophylactic effect of colchicine on graft amyloidosis. Proteinuria, highly suggestive of kidney transplant amyloidosis, developed in 11 patients within a median of 3 years after transplantation (range 0.5-10 years). In 10 patients, repeated urinalyses for protein were negative during a median of 5 years after transplantation (range 1-13). Patients who developed proteinuria or transplant amyloidosis received smaller colchicine doses than patients without proteinuria--mean 0.69 (range 0-1) versus 1.53 (range 1-2) milligrams per day (p = 0.0002), suggesting that colchicine prevents or delays development of transplant amyloidosis. This prophylactic effect of colchicine was complete at a dose of 1.5 mg/day or more and absent at a daily dose of 0.5 mg or less. In patients who received 1 mg/day, individual variability in the response to colchicine was observed. We conclude that the development of amyloidosis of the kidney transplant in FMF is inevitable at a colchicine dose lower than 1 mg/day, unpredictable at 1 mg/day and usually preventable with 1.5 mg/day or more.

Adolescent↗
PubMed1991

Long-term colchicine treatment in children with familial Mediterranean fever.

Three hundred fifty children (younger than age 16) who had familial Mediterranean fever (FMF) were given continuous prophylactic treatment with colchicine (1-2 mg/day) for 6-13 years. Complete remission of febrile attacks was achieved in 64% of the patients, and partial remission in 31%. Protracted attacks of arthritis virtually disappeared. None of the children developed amyloidosis while on the colchicine regimen. Side effects of colchicine were insignificant, and did not prompt permanent discontinuation of treatment in any of the children. Their growth, development, and subsequent fertility were normal. The efficacy of long-term colchicine treatment of children with FMF makes early diagnosis life saving.

Adolescent↗
PubMed1978

Cold-induced profuse sweating on back and chest. A new genetic entity?

Two sisters whose parents shared a grandfather had cold-induced sweating. Since childhood they had sweated profusely from the back and chest when exposed to environmental temperatures of 18 degrees to 7 degrees C. They had additional abnormalities--e.g., high palate and inability fully to extend the elbows--which neither their parents nor their sibs shared. The cold-induced sweating, which could not be stopped by a beta-adrenergic blocking agent, was abolished by postganglionic blockade with atropine sulphate. This indicates the possibility of a peripheral mechanism.

Administration, Oral↗
PubMed1978

Age and sex difference in response to short exposure to extreme dry heat.

Sixty volunteers, 33 males and 27 females (18-63 yr), were divided according to age and sex. They were exposed for 10 min to extreme dry heat: 80-90 degrees C dry bulb temperature and 3-4% relative humidity. Their rectal temperature, skin temperature at eight different points, weight, and heart rate were recorded prior to and immediately following the exposure. A mean rise of only 0.5 degrees C in rectal temperature was recorded following exposure as compared to a mean rise of 5.2 degrees C in mean weighted skin temperature (MWST). Female subjects showed a significantly higher rise in MWST than the male subjects. Similarly, a significantly higher rise in MWST was observed in elderly male subjects as compared to the youngest male group (P less than 0.05). The differences in MWST possibly resulted from differences in mean skin blood flow causing differences in skin conductance. Large individual variation in heat response was recorded in rectal temperature, as well as in weighted skin temperatures. The increase in skin temperature during the first 10 min of exposure to extreme dry heat may serve as an indicator for heat tolerance time, and may help predicting heatstroke susceptible individuals.

Adaptation, Physiological↗
PubMed1978

Rehabilitation of patients with NCA (neurocirculatory asthenia) through a short term training program.

Twenty-two patients, 18 years old, with the predominant somatic type of NCA, and a control group of 22 healthy subjects participated in a short term training course lasting three weeks. Following the training the NCA subjects showed a significant change in the systolic and diastolic blood pressure after 5 minutes of recumbency as well as in the systolic blood pressure after 5 minutes of standing, diastolic pressures during 50 W load and submaximal effort (100-150 W) - all the values being significantly lower following the training, except the diastolic pressure during 50 W load which was higher following the course. The prominent training effect was on the heart rate during light work loads (50 W) in which a decrease from a mean of 151.2 +/- 13.9 to 118.3 +/- 16.3 beats/min was recorded. This decrease indicates the better adaptation of the NCA patients to physical effort. Similarly, a significant increase in the mean VO2max from 31.5 +/- 6.1 to 37.7 +/- 5.9 ml O2/kg B.W./min was observed. No changes were recorded in the ECG after the short training course. Following the training all the symptoms typical of the NCA syndrome vanished. A short physical training of 3 weeks can start the procedure of rehabilitation of a well selected group of NCA patients with predominant somatic complaints. The rehabilitation must be continued throughout life by continued physical training.

Adolescent↗
PubMed1978

Orthostatic hypotension in amputees and subjects with spinal cord injuries.

Orthostatic examinations were performed with a tilt table on 64 male volunteers, of whom 21 had sustained amputation of one or both lower limbs and 23 were paraplegic due to spinal cord injuries (SCI), with 20 healthy subjects as controls. Following tilting, signs and symptoms of fainting appeared in five of the SCI subjects, four of whom had spinal injuries above the level of D5. These phenomena appeared in only two of the controls and in none of the amputees. The mean systolic and diastolic blood pressures of the amputees at rest and standing were higher than those recorded in the other two groups. The mean pulse pressures were lower in the amputees than in the other two groups. The changes in the systolic, diastolic, and pulse pressures were more profound in those subjects with high spinal cord injuries than in subjects with lower cord injuries. ECG changes and fainting were more frequent among the SCI subjects than in the other two groups.

Adult↗
PubMed1978

Optimal back-pack load for short distance hiking.

Twenty young men marched 6 and 12 km with a well-fitted back-pack load of 30 or 35 kg. Each subject served as his own control. No significant increase in mean heart rate, rectal temperature, or decrease in mean VO2 max and serum levels of glucose and muscle enzymes were recorded in the groups marching 6 km with 30 and 35 kg. Significant differences in the increases in mean heart rate, the decreases in VO2 max and the changes in blood glucose were noted between the two groups carrying 30 and 35 kg for 12 km. These significant differences were also supported by the subjective feelings of the volunteers. The present study shows the optimal back-pack load for healthy young men, marching at 6 km/hr on a paved level road to be 30 kg for 12 km and 35 kg for 6 km without considering the task too difficult and with no significant decrease in VO2 max. The results are relevant to hiking, rescue assignments, and military missions.

Adolescent↗
PubMed1977

Effect of a single dose of dimethyl sulphoxide on renal amyloidosis.

An amyloid-like fibrillar substance was obtained under certain chemical conditions from the urine of 11 patients with amyloid nephropathy who had been given a test dose of dimethyl sulphoxide. This fibrillar material could not be produced in the urine from 9 patients with proteinuria due to other causes. This finding may be useful in the diagnosis of amyloid nephropathy and could lead to a new approach to thearpy.

Administration, Oral↗