Objective: To estimate the prevalence of female sexual dysfunction (FSD) in diabetic and non diabetic Jordanian women. Research design and methods: Data were collected from 1137 married females using the Arabic translation of the Female Sexual Function Index ( FSFI ) Questionnaire . Results: Prevalence of sexual dysfunction in diabetic females 50 years or older was 59.6% versus 45.6% in non diabetics ( P=0.003). Diabetic women had more dysfunction of desire, arousal, lubrication and orgasm than non diabetics. Glycemic control, smoking, dyslipidemia, hypertension, autonomic neuropathy and peripheral neuropathy did not have significant effect on FSD .Age, BMI, duration of diabetes, the presence of CAD, nephropathy and retinopathy had a negative effect on FSD . Conclusion: Prevalence of FSD among Jordanian females was found to be significantly higher in diabetic women compared to non diabetic women.

We report on two brothers, born to double first cousin Jordanian Arab parents, with a syndrome comprising severe hypertelorism with upslanted palpebral fissures, brachycephaly, abnormal ears, sloping shoulders, enamel hypoplasia, and osteopenia with repeated fractures. Both have severe myopia, mild to moderate sensori-neural hearing loss and borderline intelligence. Results of chromosome analysis were normal as was a FISH assay for subtelomeric rearrangements. The father has mild hypertelorism but the family history is otherwise unremarkable. We think that this represents a previously unrecognized autosomal or X-linked recessive syndrome.(c) 2007 Wiley-Liss, Inc.

OBJECTIVE: To estimate the prevalence and severity of erectile dysfunction (ED) and its correlations among Jordanian men with diabetes. METHODS: We conducted this study at the National Center for Diabetes, Endocrinology and Genetics, Amman, Jordan, between January and August 2004. The study included 988 married diabetic men. Patients were interviewed by one of our medical staff based on a health care questionnaire and an Arabic translation of the 15-item International Index of Erectile Function. Scores of the questions in each of the 5 sexual function domains were summed up. Dysfunction was categorized as absent, mild, moderate or severe. RESULTS: The overall prevalence of ED was 62%; and we found that 30.3% had severe ED. The prevalence increased with age from 26.5%(13 out of 49) of patients <40 years of age to 91%(87 out of 96) in the age group >/= 70 years. Severity of ED increased with age as well. Multivariate logistic regression analysis identified age, glycemic control, hypertension, coronary artery disease, retinopathy and neuropathy as independent risk factors of ED. Among patients with ED, 7% reported having treatment for ED. CONCLUSION: Prevalence of ED among Jordanian diabetic patients is high. It increases with age and poor glycemic control. Other independent risk factors include: hypertension, coronary artery disease, retinopathy and neuropathy.

Cockayne syndrome is a rare autosomal recessive condition characterized by growth failure and multisystem progressive degeneration. We report and describe this syndrome in a Jordanian brother and sister with Cockayne syndrome with first cousin parents. Clinical features included short stature, cachectic senile look, neurological deterioration, photosensitivity, mental retardation, hearing impairment and carious teeth. The phenotype is compatible with a mild variant of type I Cockayne syndrome. They showed an exaggerated response to growth hormone provocation test, with slightly elevated basal insulin-like growth factor 1 levels. The radiological findings of thinning of ribs and slender femora with narrow medullary canals have not previously been reported in this syndrome. We discuss the implications of these findings.

BACKGROUND: Proper management of patients with Kallmann syndrome (KS) allows them to attain a normal reproductive health. The purpose of this study is to demonstrate the presentation modalities, phenotypes and the modes of inheritance among 32 patients with Kallmann syndrome in Jordan. Recognition of the syndrome allows for prompt proper management and provision of genetic counselling. SUBJECTS: Over a period of five years (1999-2004), the clinical and inheritance profiles of 26 male and 6 female patients with Kallmann syndrome from 12 families were evaluated at the National Center for Diabetes, Endocrinology and Genetics in Jordan. RESULTS: The patients belonged to twelve Jordanian and Palestinian families and their age at presentation ranged from 4 - 46 years. Nine boys aged 4-14 years presented with cryptorchidism and microphallus, all other males presented with delayed puberty, hypogonadism and/or infertility. The main presentation among six female patients was primary amenorrhea. Intrafamilial variability in clinical phenotype was specifically evident for renal abnormalities and sensorineural hearing impairment. Familial KS was diagnosed in 27 patients belonging to five families with the X-linked mode of inheritance and two families with the autosomal recessive mode of inheritance. CONCLUSIONS:(1) the majority of cases in this study represented the X-linked form of KS, which might point to a high prevalence of Kal 1 gene in the population.(2) Genetic counselling helps these families to reach a diagnosis at an early age and to decide about their reproductive options.(3) Children presenting with cryptorchidism and microphallus in our population should be investigated for KS.

BACKGROUND: Data from developed countries about precocious puberty are abundant; data from developing countries are limited. Causes are different, and diagnostic and treatment possibilities are very expensive. The present study aimed to display the spectrum of initial presentation and aetiology among children with precocious puberty and to assess any association between th e clinical features and the underlying cause of the condition. PATIENTS AND METHOD: Forty-three girls and seven boys with precocious puberty were diagnosed at the Endocrine Clinic of Jordan University Hospital and at The National Center for Diabetes, Endocrinology and Genetics, Amman, Jordan, between the 1984 and 2003. RESULTS: Mean age for the girls with precocious puberty was 4.1 years +/- 2.5 SD and for the boys was 2.4 years +/- 1.9 SD. Among the girls, 21% presented with breast development only, 9% with pubic hair appearance only and 70% with multiple signs. All the boys presented with pubic hair appearance and enlarged genitalia. Organic causes for precocious puberty were detected in 42% of the girls and in all the boys. Idiopathic precocious puberty was more common among the girls presenting with breast development only (89%) compared with those presenting with multiple presenting signs (50%), and also was more common among girls presenting between 6 and 8 years (82%) than among those presenting < 6 years of age (42%). Congenital adrenal hyperplasia was diagnosed in four boys and four girls, and hypothyroidism in three girls. CONCLUSION: Precocious puberty in the girls was usually of idiopathic origin when it presented with breast development only and at age older than 6 years. Congenital adrenal hyperplasia and hypothyroidism could represent important causes for precocious puberty in our community.

OBJECTIVE: Metformin, an oral hypoglycemic agent, has several other metabolic and hormonal effects. This study aims at identifying the metabolic effect of metformin on androgens in diabetic men. METHODS: The study was conducted at The National Center for Diabetes Endocrinology and Genetics, Jordan University Hospital, Amman, Jordan from April 2001 to September 2001. We studied 15 men with type 2 diabetes mellitus by measuring fasting serum glucose, insulin, glycosylated hemoglobin, total and free testosterone, sex hormone binding globulin, dehydroepiandrosterone sulphate, 17-OH progesterone, luteinizing hormone, and follicle stimulating hormone before and after a short course of metformin. RESULTS: There was a significant decrease in fasting serum glucose and glycosylated hemoglobin and increase in the level of 17-OH progesterone. The remainder of the measured parameters did not show any significant change. Although serum glucose and glycosylated hemoglobin decreased insulin levels were not changed. CONCLUSION: In contrast to normal men there was no change in androgen levels in diabetics but the 17-OH progesterone was elevated.

OBJECTIVE: To detect feet changes and to identify risk factors leading to amputation among type 2 diabetics. METHODS: A total of 1142 patients with type 2 diabetes mellitus; 595 males (52%), and 547 females (48%) were seen between January and December 2001 at the National Center for Diabetes, Endocrinology, and Genetics (NCDG) Amman, Jordan. The mean age was 56.1 years (SD=10.2) and the mean duration of diabetes was 9 years (SD=7.1). All patients had a complete medical assessment including history, physical examination, glycosylated hemoglobin (HbA1c)(the mean of the last 4 readings) and microalbuminuria. Statistical analysis were performed to identify significant risk factors leading to amputation using Epi info, version 6 software. RESULTS: Mean HbA1c was 7.4%(SD=1.4). The prevalence of hypertension was 52%, retinopathy 45% and microalbuminuria 33%. Impaired vibration, position and protective sense were found in 19%, 13%, and 18%. The prevalence of all amputations was 5%. The following were strong predictors of amputation; duration of diabetes (P= 0.04), smoking (P=0.01), microalbuminuria (P=0.02), retinopathy (P=0.008), legs hair loss (P=0.003), neurological deficit (P=0.0001), ulceration (P=0.00001) absent dorsalis pedis (P=0.0006) and insulin therapy (P=0.0001). The rate of amputation was directly proportional to high HbA1c >= 8%(P=0.01). Age and gender were not found to have an impact on prevalence of amputation. CONCLUSION: Prevalence of amputation correlates with duration of diabetes, poor glycemic control, smoking, neurological impairment, peripheral vascular disease and microalbuminuria.

OBJECTIVE: To study the effect of metformin on androgens in normal men. METHODS: A total of 12 healthy males volunteered to participate in the study. A blood sample was obtained from each of them and analyzed for the following: Testosterone (total and free), sex hormone binding globulin dehydroepiandrosterone sulphate, 17-hydroxyprogesterone, luteinizing hormone, and follicle stimulating hormone. In addition, each participant was subjected to a glucose tolerance test and his insulin level was measured. Metformin 850 mg twice daily for 2-weeks was given to each subject after which the above tests were repeated. A paired t-test was used to assess the statistical significance of any observed differences before and after metformin. RESULTS: After metformin administration, there was a significant reduction in serum level of total testosterone (p=0.0001), free testosterone (P=0.002), and 17 hydroxyprogesterone (p=0.0001). There was also a significant increase in serum level of sex hormone binding globulin (p=0.009) and dehydroepiandrosterone sulphate (P=0.0008). Serum levels of luteinizing hormone and follicle stimulating hormone showed no significant changes. Similarly, there were no changes in fasting plasma glucose, fasting serum insulin, weight, or blood pressure. CONCLUSION: Metformin administration was associated with a reduction in total testosterone, free testosterone, and 17-hydroxyprogesterone and an increase in sex hormone binding globulin and dehydroepiandrosterone sulphate in normal males. The clinical significance of these findings needs further investigation.