History of abuse and organic difficulties in a convenience sample of 46 ultra-orthodox males with pedophilia.

BACKGROUND: Evidence has started to accumulate that relates pedophilia to a history of being a victim of sexual abuse as well as to comorbidity with organic vulnerabilities. During a naturalistic study regarding treatment of pedophilia, the authors had access to clinical and psychodiagnostic evaluations of Israeli Jewish ultraorthodox male pedophiles outside the forensic system. Using psychiatric examination as well as a battery of psychological tests, presence of history of trauma as well as comorbidity with organic vulnerabilities among this unique sub-group was examined. METHOD: This survey was part of a larger scale research on the effectiveness of Decapeptyl injections as treatment for pedophilia. All participants in the original research underwent comprehensive psychological assessment including an extensive clinical interview as well as psychological tests (Bender, Rorschach and TAT). Of the patients participating in the research, this survey focused on the group of 46 ultra-orthodox male pedophiles. Cross-tabs analyses were conducted in order to examine prevalence of history of trauma and organic vulnerabilities in this specific group. RESULTS: Based on self reports combined with corroborating reports (obtained from parents, educators and medical staff), together with indications in psychological tests, we found that 82.6% of participants were victims of sexual trauma as children and 87% suffer from some kind of organic vulnerability (learning disabilities, disinhibitions, etc.). LIMITATIONS: LIMITATIONS of this small convenience sample that influence ability to generalize are discussed. CONCLUSIONS: The current survey indicates that in this sample, the ultra-orthodox male pedophile was frequently a victim of childhood sexual trauma, and exhibited indications of organic vulnerabilities. This is more pronounced than findings in previous studies, and calls for further research in order to understand the underlying causes.

Prolonged remission of severe Cushing syndrome without adrenalectomy in an infant with McCune-Albright syndrome.

A 4 month-old girl presented with severe Cushing syndrome caused by McCune-Albright syndrome. After undergoing 19 months of pharmacologic suppression of cortisol production, she has been in clinical remission for more than 6 years. Adrenalectomy may be avoidable even in severe cases of Cushing syndrome associated with McCune-Albright syndrome.

Subclinical hypothyroidism in infertile women: the importance of continuous monitoring and the role of the thyrotropin-releasing hormone stimulation test.

The aim of our study was to assess the prevalence of subclinical hypothyroidism (SH) after administering a thyrotropin-releasing hormone (TRH) stimulation test among women with normal serum thyroid-stimulating hormone (TSH) levels and various causes of infertility. Eighty-seven infertile women (39 with ovulation disorders and 48 with other causes of infertility) had a TRH stimulation test on day 3 - 7 of their cycle. Exaggerated TSH response (>30 mIU/l at 20, 40 or 60 min) following intravenous injection of 400 microg TRH was defined as SH. The TRH test was performed 2 - 4 months after the first visit to the clinic. We found that the prevalence of SH was significantly higher among women with ovulation disorders (20.5%) than among women with normal ovulation (8.3%). In addition, we found that although basal TSH levels were normal at recruitment, 2 - 4 months later these levels were abnormally high in 8% of the women. All these women had an abnormal TRH test. We recommend performing TRH stimulation testing in women suffering from ovulation disorders who have normal basal TSH levels, followed by repeat assessments of thyroid function to enable treatment with thyroxine in cases with abnormal results.

17 beta-hydroxysteroid dehydrogenase 3 deficiency in the Mediterranean population.

Eighty-five males with 17 beta-HSD3 were identified among a highly inbred Arab population in Israel and 57 studied over a period of 25 years. The founders of this defect originated in the mountainous regions of present Lebanon and Syria, but most of the families now live in Jerusalem, Hebron, the Tel-Aviv area and, in particular, in Gaza, where the frequency of affected males is estimated at 1 in 100 to 150. Affected individuals are born with ambiguity of the external genitalia and reared as females until puberty. Thereafter marked virilization occurs, leading in many cases to the spontaneous adoption of a male gender identity and role. Adults develop a male habitus with abundant body hair and beard and the phallus and testes enlarge to adult proportions. Gender reassignment in infancy was only possible when enough erectile tissue was present at birth and developed into a normal size penis with testosterone. 17 beta-HSD3 deficiency can be reliably diagnosed by endocrine evaluation and mutation analysis. In adults the defect is characterized by markedly increased concentrations of androstenedione (A) with borderline low to normal testosterone (T) levels and a high A/T ratio. 5a-dihydrotestosterone (DHT) concentrations are moderately decreased, normal or high and dehydroepiandrosterone (DHEA) levels are high. The estrogen pathway is also impaired, even though both estrone (E-1) and estradiol-17 beta (E-2) levels are high. Children have low basal levels of all androgens, but the defect may be demonstrated after prolonged stimulation with human chorionic gonadotropin (HCG). LH and FSH levels are very high after puberty and normal in childhood. 17 beta-HSD3 isozyme is encoded by the chromosome 9q22 17 beta-HSD3 gene and expressed exclusively in testes. A point mutation in exon 3, codon 80 of the 17 beta-HSD3 gene, R80Q, caused by a single base substitution from CGG ( arginine) to CAG ( glutamine) was identified in both alleles of 24 individuals from 9 extended Arab families from Gaza, Jerusalem and Lod-Ramle. Twenty-one homozygote males (46,XY) were MPH with testicular 17 beta-HSD3 deficiency whereas the three homozygote females (46,XX) were asymptomatic, had normal internal and external genitalia, normal sexual development and revealed no biochemical evidence of 17 beta-HSD3 deficiency. The molecular pattern is compatible with an autosomal recessive mode of inheritance, sex dependent.