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M Zerah

Publications and source records attributed to M Zerah.

66 records · Page 4Linked to original sources
PubMed1990

Prevalence of nonclassical steroid 21-hydroxylase deficiency based on a morning salivary 17-hydroxyprogesterone screening test: a small sample study.

Early morning salivary 17 alpha-hydroxyprogesterone (17-OHP) determination differentiates patients with non-classical 21-hydroxylase deficiency (NC21OHD) from those who are not affected. Using this test, we have conducted a trial screening study for NC21OHD and have compared the study results with previously reported figures for the frequency of this disorder. Testing was performed on 258 subjects recruited from among the medical students and employees of the New York Hospital-Cornell Medical Center. In 2 of the 249 admissible subjects, the 0700-0900 h salivary 17-OHP level was within the range for NC21OHD patients (0.72-6.7 nmol/L; n = 8). These 2 individuals were subsequently confirmed to be affected by ACTH testing. Of the subjects with morning salivary 17-OHP levels below the cut-off point of 0.72 nmol/L, 29 were recalled for ACTH testing and were confirmed to be unaffected. Prevalence of NC21OHD in the test population was determined according to ethnic group. Our study gives a prevalence by screening of 1.14% among caucasians, which agrees with values of 0.81% and 1.06% obtained by different analytical methods. Further, both affected subjects were Ashkenazi Jews, and the prevalence of 3.23% among study members from this group concurs with increased rates of 3.64% and 4.97% already reported. On the basis of a small population sample, screening so far confirms the claim that NC21OHD is the most common autosomal recessive human disorder. Using values from ACTH-proven unaffected subjects (n = 47) and NC21OHD patients (n = 10), we establish preliminary normative data for morning salivary 17-OHP levels of 0.172 nmol/L for unaffected subjects (95% confidence interval, 0.05-0.54 nmol/L) and 1.76 nmol/L for NC21OHD-affected subjects (95% confidence interval, 0.42-7.32 nmol/L).

17-alpha-Hydroxyprogesterone↗
PubMed1989

Ruptured intracranial aneurysms. The influence of sex and fibromuscular dysplasia upon prognosis.

From a statistical analysis of a series of 214 cases of ruptured intracranial aneurysms, it was demonstrated that only four parameters among those collected on the admission day, are predictive of the final functional result: sex (p less than 0.001); age (p less than 0.02); clinical grade (p less than 0.001) and angiographic spasm (p less than 0.01). The surprisingly poor prognosis in women was explained by the higher frequency of spasm (p less than 0.005). Aneurysms in women predominated on the intracranial carotid artery (38%) and were frequently multiple (12%). A further angiographic study on 87 cases was then carried out including a systematic investigation of the cervical part of the vessels; it permitted one to identify angiographic features of fibromuscular dysplasia (FMD) on the cervical vessels in 20 cases. In those cases it was also observed that there was a marked female predominance (F/M = 5.6), a frequent localization on the internal carotid artery (50%), a high rate of multiple aneurysms (60%) and a poor prognosis related to spasm (50%). Therefore, intracranial aneurysms appear far more frequently than usually reported, to be related to FMD. The worse prognosis of ruptured intracranial aneurysms in females can be, at least partly explained by the association with FMD and the frequent occurrence of spasm. A careful investigation of patients exhibiting features of FMD in order to detect intracranial aneurysms before rupture, is suggested.

Arterial Occlusive Diseases↗
PubMed1987

Morning salivary 17-hydroxyprogesterone is a useful screening test for nonclassical 21-hydroxylase deficiency.

A screening test for nonclassical 21-hydroxylase deficiency (NC 21-OHD) has been established based on early morning salivary 17-hydroxyprogesterone (17-OHP) measurements. Saliva and serum samples were collected simultaneously between 0700 and 1100 h from 57 normal subjects (37 women and 20 men) and from 15 untreated patients (6 women and 9 men) with NC 21-OHD. The salivary (mean +/- SD, 524 +/- 508 pg/mL) and serum (10,548 +/- 5,998 pg/mL) 17-OHP concentrations in all 15 patients were unequivocally higher than the levels in normal subjects (saliva, 51 +/- 24 pg/mL; serum, 1,564 +/- 787 pg/mL). Salivary 17-OHP levels in patients with NC 21-OHD were significantly higher at 0700-0900 h (828 +/- 653 pg/mL) than at 0900-1100 h (314 +/- 227 pg/mL), while no such change was found in normal subjects. The salivary 17-OHP concentration was 1.3-6.9% of its serum concentration, and there was an excellent correlation (r = 0.93) between salivary and serum concentration in both normal subjects and patients with NC 21-OHD. In conclusion, early morning salivary 17-OHP measurement is an excellent screening test for the diagnosis of NC 21-OHD, since it accurately reflects serum 17-OHP levels, and sample collection is easy and noninvasive. We propose that salivary 17-OHP determination be used in population screening programs for NC 21-OHD to establish the true frequency of this disease.

17-alpha-Hydroxyprogesterone↗
PubMed1987

[Intracranial arterial aneurysm. Differences in origin and prognosis in men and women].

A statistical model was defined from a series of 144 patients referred for a subarachnoid hemorrhage due to intracranial aneurysm rupture, occurred less than 3 days before admission. This model was then prospectively applied on a second series of 70 patients and allowed to find out four datas predicting the final functional result: age (p = 0.02), clinical status (p = 0.001), angiographic evidence of spasm (p = 0.01) and sex (p = 0.001). Concerning sex, functional result appears worse in women than in man. This more severe prognosis of aneurysmal rupture in woman is explained by the higher frequency of spasm (p less than 0.0005). This fact and the predominance of multiple aneurysms and localizations on the supraclinoid internal carotid artery (38.3% in females and 17.7% in males) suggest a different origin of intracranial aneurysms according to sex. Fibromuscular dysplasia might correspond to the datas here observed, and is currently explored.

Adolescent↗
PubMed1986

[Medical treatment of staphylococcal epiduritis].

The authors report a case of extensive spinal epidural staphylococcal abscess responsible for spinal compression. All symptoms regressed after conservative management consisting of parenteral antibiotic therapy with Gentamycin and Vancomycin.

Abscess↗
PubMed1985

A retromedullary arteriovenous fistula associated with the Klippel-Trenaunay-Weber syndrome. A clinicopathologic study.

An intraspinal vascular malformation associated with the Klippel-Trenaunay-Weber (KTW) syndrome initially was thought to be intramedullary on angiographic findings. Postmortem examination revealed an entirely posterior extramedullary arteriovenous fistula (AVF) fed by the anterior spinal artery. The association of the KTW syndrome with a so-called intramedullary AVF has been described in the literature without any pathologic confirmation (11 cases). Our case emphasizes the difficulty of determining the exact morphology and location of spinal AVF on arteriography. The association of the KTW syndrome with a retromedullary AVF can be explained on a developmental basis.

Angiomatosis↗
PubMed1981

[Effect of availability of computer tomography on activity in an emergency neuroradiological department (author's transl)].

Overall activity in an emergency neuroradiological department was altered when computed tomography facilities became available, as shown by comparative studies conducted during one year without and one year with the use of this investigational method. More examinations were conducted during the year the apparatus was available, and the diagnostic approach to patients with head or brain injuries was modified, computed tomography replacing the arteriography used as an initial examination during the first year. These findings can be related to the rapidity, safety, and diagnostic precision of computed tomography examinations.

Brain↗
PubMedDate not supplied

Congenital intraspinal lipomas: histological analysis of 234 cases and review of the literature.

The clinical, radiologic, and pathologic data from a series of 234 patients hospitalized in the Pediatric Neurosurgical Department of the Necker-Enfants Malades Hospital, Paris, for congenital intraspinal lipomas and operated on from 1976 to 1995 were examined. Histological studies showed that these lesions may be simple lipomas, similar to those developing elsewhere in the body, or they may be more complex forms including in addition to the lipomatous component a variety of unusual ectopic tissues of ectodermal, mesodermal, and/or endodermal origin. These complex forms indica te the malformative nature of these tumors. When they contain elements that are truly foreign to the region, the possibility of teratoma with a tumoral potential should be considered. Data found in the literature and from Necker-Enfants Malades Hospital are discussed.

Child↗