Immature ovarian teratoma in a 20-year-old woman: A case report.

Immature ovarian teratomas are a rare subtype of germ cell tumours characterized by the presence of embryonic elements, particularly primitive neuroepithelium, and they typically affect young women. We report the case of a 20-year-old woman who presented with a growing abdominal mass that turned out to be a grade II immature teratoma after adnexectomy. This article reviews the clinical presentation, imaging features, and some of the main problems that arise in the management of immature ovarian teratomas.

Rectal taurocholate increases L cell and insulin secretion, and decreases blood glucose and food intake in obese type 2 diabetic volunteers.

AIMS/HYPOTHESIS: Glucagon-like peptide-1 (GLP-1) and peptide YY (PYY) are secreted from enteroendocrine L cells in response to numerous stimuli, including bile salts. Both have multiple effects that are potentially useful in treating diabetes and obesity. L cell number and hormone content in the intestine are highest in the rectum in humans. We investigated the effects of intrarectal sodium taurocholate on plasma GLP-1, PYY, insulin and glucose concentrations, and on food intake of a subsequent meal. METHODS: Ten obese type 2 diabetic volunteers were each studied on five separate occasions after an overnight fast and oral administration of 100 mg sitagliptin 10 h before the study. They then received an intrarectal infusion of either one of four doses of taurocholate (0.66, 2, 6.66 or 20 mmol, each in 20 ml of vehicle) or vehicle alone (1% carboxymethyl cellulose) single-blind over 1 min. Hormone and glucose measurements were made prior to, and for 1 h following, the infusion. The consumption of a previously selected favourite meal eaten to satiety was measured 75 min after the infusion. RESULTS: Taurocholate dose-dependently increased GLP-1, PYY and insulin, with 20 mmol doses resulting in peak concentrations 7.2-, 4.2- and 2.6-fold higher, respectively, than those achieved with placebo (p < 0.0001 for each). Plasma glucose decreased by up to 3.8 mmol/l (p < 0.001). Energy intake was decreased dose-dependently by up to 47% (p < 0.0001). The ED(50) values for effects on integrated GLP-1, insulin, PYY, food intake and glucose-lowering responses were 8.1, 10.5, 18.5, 24.2 and 25.1 mmol, respectively. CONCLUSIONS/INTERPRETATION: Therapies that increase bile salts (or their mimics) in the distal bowel may be valuable in the treatment of type 2 diabetes and obesity.

[Recurrent acute fatty liver of pregnancy: About a case and a review of the literature]. / La stéatose hépatique aiguë gravidique récidivante : à propos d'un cas et une revue de la littérature.

Acute fatty liver of pregnancy (AFLP) is a specific but rare hepatopathy that can usually complicate the third trimester of pregnancy. It is potentially fatal for the mother and the fetus. To our knowledge, only eight cases of recurrence have been published, we report a new case. The first episode presented by our 23-year-old patient was suspected in front of a cutaneous-mucosal jaundice with vomiting occurring on pregnancy of 35weeks of gestation (WG). Hyperleucytosis, abnormalities of the hepatic balance, as well as a hypoglycemia were biological elements supporting the diagnostic beam. On the other hand, medical imaging could not bring a clear confirmation. The evolution was favorable after deferred delivery by caesarean section for pulmonary maturation. Three years later, she presented to the obstetrical emergency room at 36weeks and six days of gestation, with a clinical and biological picture almost similar to that of the first episode. A caesarean section was then indicated for suspicion of recurrence. The evolution is favorable for the mother and her children. The interest of the communication on the risk of recurrence, the clinical and biological monitoring in particular in the third trimester of the subsequent pregnancy are imperative, in order to improve the prognosis of this pathology.

Carbohydrate restriction favorably alters lipoprotein metabolism in Emirati subjects classified with the metabolic syndrome.

BACKGROUND AND AIMS: Carbohydrate restriction (CR) has been shown to improve dyslipidemias associated with metabolic syndrome (MetS). We evaluated the effects of CR on lipoprotein subfractions and apolipoproteins in Emirati adults classified with the MetS. METHODS AND RESULTS: 39 subjects (15 men/24 women) were randomly allocated to a CR diet [20-25% energy from carbohydrate (CHO)] for 12 wk (CRD group) or a combination treatment consisting of CRD for 6 wk followed by the American Heart Association diet (50-55% CHO, AHA group) for an additional 6 wk. All subjects reduced body weight, LDL cholesterol and triglycerides (P<0.01). At baseline all subjects had low concentrations of medium VLDL and total HDL particles associated with the very low plasma triglycerides and HDL cholesterol in this population. After 12 wk, the large VLDL subfraction was decreased over time for subjects in the CRD group (P<0.01) while these changes were not observed in those subjects who changed to the AHA diet. The number of medium and small LDL particles decreased for all subjects rendering a less atherogenic lipoprotein profile. In agreement with these results, a significant decrease in apolipoprotein (apo) B was observed (P<0.01). The medium HDL subfraction and apo A-II, which can be considered pro-atherogenic, were also decreased over time in the CRD group only. CONCLUSIONS: These results suggest that weight loss favorably affects lipoprotein metabolism and that the CRD had a better effect on atherogenic VLDL and HDL than the low fat diet recommended by AHA.

[Major alpha-thalassemia: antenatal diagnosis, case report and literature review]. / Alpha-thalassémie majeure: à propos d'un cas de dépistage anténatal et revue de la littérature.

Homozygous alpha-thalassaemia or Bart's hydrops fetalis is a genetic disease with autosomal recessive transmission. The condition is lethal for the fetus because of hypoxia and anemia. For the mother there is an increased risk of the severe forms of preeclampsia and its complications. The diagnosis can be suspected in presence of suggestive ultrasonographic anomalies, where both parents come from South-East Asia or China. Confirmation is based on the identification of the typical deletions or mutation of the alpha globin gene by molecular genetics. We report a rare clinical case of Bart's hydrops fetalis diagnosed because of fetal growth retardation, fetal cardiomegaly and increased size of placenta on the 26 weeks fetal echography. This case underscores the need to include the alpha thalassemias in medical and midwifery education in countries where they were almost inexistent a generation ago.

Audit of a diabetes clinic at Tawam hospital, United Arab Emirates, 2004-2005.

The aim of this audit was to determine the current management of patients with diabetes compared to international standards and to benchmark the results against current international standards. A retrospective audit of medical records of diabetic patients attending Tawam Hospital, a tertiary healthcare facility in the Al Ain region in the Emirate of Abu Dhabi, United Arab Emirates was performed. A random sample of 30 patients (5% of the target group) was selected from the total number of 600 patients who visited the diabetes clinics in August 2005. An audit form was developed based on the priority aims and measures contained in the Institute for Clinical Systems Improvement (ICSI) Guidelines for the management of type 2 diabetes mellitus. Data analysis was carried out based on measurement specifications in the ICSI Guidelines. All patients had their blood pressure checked at their most recent appointment. All except one patient had an HbA1c test and a lipid profile performed during the study period. Although 75% of patients were referred for a dilated eye examination, only 47% complied. Approximately two-thirds of all patients had evidence of self-monitoring of blood glucose. Less than half of the patients were referred to the nutritionist and only a relatively small proportion of patients had advice on diet and exercise documented in their medical record. There was also lack of documentation, particularly for smoking status, foot examination, and body mass index (BMI). All patients received aspirin (ASA) treatment. The majority had a systolic blood pressure of 130 mmHg or less; just under half of patients had HbA1c of less than 7%. In comparison to the Center for Diseases Control (CDC) targets, we exceeded targets in three areas in relation to HbA1c testing, ASA treatment, and self-report monitoring blood glucose. We did not meet the stated target of 75% for patients having a dilated eye exam due to lack of patient compliance (more than three-quarters of all patients were referred but did not attend for appointment). Documentation of a comprehensive foot examination was present for only one-quarter of all patients compared to the recommended target of three-quarters of patients. Documentation of advice on diet and exercise was present for 40%, which did not meet the CDC target of 60%. Overall, the audit highlighted that Tawam Hospital is providing a good level of care to diabetic patients and compares favorably with international targets; however, key recommended actions have been identified for implementation to improve patient care and maintain a continuous improvement process through effective monitoring with prioritization to those related to preventative care.

Feminizing adrenocortical tumor: steroid hormone response to ketoconazole.

A 58-yr-old man presented with gynecomastia and elevated serum estrogens. The diagnosis of an estrogen-secreting adrenal tumor was made based upon the finding of a 4-cm left adrenal mass, elevated levels of estradiol in peripheral and left adrenal venous blood, and increased urinary 17-ketosteroids. In addition to marked elevations in estradiol and 17-ketosteroids there was an increased baseline level of 11-deoxycorticosterone and a slightly decreased level of 18-hydroxycorticosterone, suggesting the possibility of impaired P450c11 activity. The effect of ketoconazole administration (600 mg/day) for 4 weeks was studied. Urinary free cortisol and 17-ketosteroid excretion and serum testosterone levels fell acutely (1 week). Serum estradiol levels decreased gradually over the 4-week course. Plasma aldosterone levels were essentially unaltered and 18-hydroxcorticosterone levels fell gradually, but there were marked increases in 11-deoxycorticosterone and corticosterone. Coincident with the increase in 11-deoxycorticosterone there was an increase in blood pressure and a transient fall in serum potassium. We conclude that ketoconazole administration may result in a hypermineralocorticoid state. Therefore, the usefulness of ketoconazole therapy for steroid hormone-producing neoplasms will depend upon the individual tumor's steroidogenic profile.

Secretion and binding of insulin-like growth factor-I by a human medullary-thyroid carcinoma cell-line.

The TT human medullary thyroid carcinoma cell line secretes a 7.5 kD species with insulin-like growth factor (IGF-I) immunoreactivity and HPLC mobility and expresses IGF-I mRNA. Conditioned medium also contained IGF-binding activity with a pattern of displacement of [I-125]-IGF-I characteristic of IGF binding proteins. Western ligand analysis and immunobloting with anti-IGFBP4 antiserum identified a 34 kD IGF-I binding species. Northern analysis identified a 2.1 kb IGFBP4 mRNA species. Cell surface binding of [I-125]-IGF-I identified Type I IGF receptors. TT cells constitute a useful model to study the IGF-I autocrine system in the transformed state.

Conservative management of patients with intrathyroidal well-differentiated follicular thyroid carcinoma.

BACKGROUND: Total or near-total thyroidectomy for the treatment of follicular thyroid carcinoma (FTC). The prognosis of patients with low-risk FTC, however, is excellent, and thus total thyroidectomy may not be justifiable in such patients. METHODS: A retrospective review identified 61 patients diagnosed with intrathyroidal well-differentiated FTC between 1958 and 1991. RESULTS: Median age at diagnosis was 42 years (range, 15-78 years). Most patients (90.2%) had a lobectomy or subtotal thyroidectomy. Median tumor size was 3.0 cm (range, 0.9-9.5 cm). Fifty-eight patients (95.1%) received thyroid hormone supplementation, and 5 (8.2%) received radioactive iodine ablation postoperatively. Median follow-up was 11 years (range, 3-35 years). Local recurrence, metastasis, or both developed in 3 patients (4.9%), and all subsequently died of thyroid cancer. The cumulative 10- and 15-year cancer-specific survival rate was 96.5%. Factors significantly related to worse survival were oxyphilic histology (log-rank, P =.00) and tumor size of more than 4 cm (P =.001). However, neither was found to be an independent predictor of outcome by Cox multivariate analyses (P =.7 and.9, respectively). The extent of initial operation (unilateral versus bilateral procedure) was not significantly related to survival (P =.52). CONCLUSION: Conservative management consisting mainly of lobectomy or subtotal thyroidectomy and thyroid hormone supplementation is associated with favorable outcome of patients with intrathyroidal well-differentiated FTC.

Quantitative ultrasound of the calcaneus in Arabian women: relation to anthropometric and lifestyle factors.

OBJECTIVES: To determine factors influencing quantitative ultrasound (QUS) parameters of the calcaneus in a population-based sample of United Arab Emirates (UAE) women, and to compare QUS parameters of the calcaneus for healthy young UAE women with the manufacturer's reference ranges for other populations. METHODS: All subjects completed a questionnaire on reproductive and life style factors. Height and weight were measured, and body composition was determined by bioelectric impedence. Estimated bone mineral density (BMD), Speed of sound (SOS), broadband ultrasound attenuation (BUA) and quantitative ultrasound index (QUI) of the right calcaneus were determined by Sahara ultrasound. RESULTS: In premenopausal women (n=330), age, weight, body mass index (BMI), lean weight, fat weight, education, age at menarche, and number of pregnancies, correlated significantly with QUS parameters. Multiple regression analysis showed that age at menarche, number of pregnancies, and BMI, were the best predictors of QUS parameters although these factors explained only small amounts of the variance (R(2)=0.05). In postmenopausal women (n=81), age, BMI and physical activity were the best predictors of BUA (R(2)=0.35), SOS (R(2)=0.39), and QUI (R(2)=0.43). Mean estimated BMD, QUI and SOS for healthy young UAE women were significantly lower than the manufacturer's reference ranges for U.S. Caucasian, European Caucasian, and Chinese Asian healthy young women of the same age range (P<0.001 for all comparisons). Mean BUA was not significantly different, however. CONCLUSIONS: Menopausal status, age, BMI and physical activity are strong predictors of QUS parameters of the calcaneus in Arabian women. Healthy young Arabian women have lower estimated calcaneal BMD compared with the manufacturer's reference ranges for other populations.

Severe hypertriglyceridemia and acute pancreatitis during pregnancy: treatment with gemfibrozil.

OBJECTIVE: To describe the effect of gemfibrozil therapy during pregnancy in a woman with severe hypertriglyceridemia. METHODS: We present a case report, with details of lipid levels throughout several attempted pregnancies, and discuss other similar published studies. RESULTS: In a 22-year-old woman, severe acute pancreatitis due to hypertriglyceridemia developed during her first pregnancy. After a prolonged hospital course, the outcome was fetal demise. The patient was subsequently treated with gemfibrozil, which controlled the hypertriglyceridemia. A second pregnancy ended with a therapeutic abortion at 1 month because of the possible risk of pancreatitis. Gemfibrozil therapy was instituted but subsequently discontinued when she was discovered to be pregnant again, at approximately 10 weeks of gestation; the potential risks involved with the use of this drug during pregnancy were unknown. Because of the patient's strong desire to maintain the pregnancy, gemfibrozil treatment was resumed 1 week later in order to help prevent recurrent pancreatitis. The patient ultimately delivered a fullterm healthy boy, and she had no recurrence of pancreatitis or other complications. CONCLUSION: In selected patients at high risk for pancreatitis, the potential risk of gemfibrozil use during pregnancy may be offset by its benefits in the management of severe hypertriglyceridemia.

High prevalence of hypothyroidism in patients with primary pulmonary hypertension.

PURPOSE: To characterize the prevalence of hypothyroidism in a population with primary pulmonary hypertension (PPH). METHODS: Retrospective record review of 41 patients with PPH seen between 1991 and 1997 at a tertiary care center. Data abstracted included: history of previous thyroid disease, intake of thyroid supplement, and thyroid function tests. Hypothyroidism was defined as a serum thyroid stimulating hormone (TSH) level higher than 5.5 U/L, intake of thyroid supplement, or low serum thyroxine level. RESULTS: Of the 40 patients with PPH included in the study (11 men and 29 women), ages ranged from 11 to 76 years (mean 43.5 years). The mean pulmonary artery pressure was 58.7 mm Hg. Thirty-three patients had normal serum TSH levels (3 of whom were on levothyroxine supplement); 1 had low TSH; 5 had high TSH (range, 6.8-9.9 U/L, mean 8.4 U/L), and 1 had low thyroxine (T4 < 1.0 microg/dL). Nine of 40 patients (22.5%) had evidence of hypothyroidism, which is much more than expected in the general population of similar age range (2.8% in men, 7.5% in women) (p = 0.002). CONCLUSIONS: The prevalence of hypothyroidism in patients with PPH is high (22.5%). Patients with PPH should be investigated for the possibility of coexisting hypothyroidism.

Correlation of quantitative ultrasound parameters of the calcaneus with bone density of the spine and hip in women with prevalent hypovitaminosis D.

Quantitative ultrasound (QUS) of the calcaneus correlates modestly with axial dual-energy X-ray absorptiometry (DXA). Because bone mineral density (BMD) might be influenced by vitamin D status, we assessed the correlation between both techniques in 56 Arabian women, a population with high prevalence of hypovitaminosis D. The speed of sound (SOS), broadband ultrasound attenuation (BUA), and estimated BMD of the right calcaneus were determined by QUS. Spine and right hip BMD were measured by DXA scan. The serum 25-hydroxyvitamin D (25OHD) level was measured by radioimmunoassay. The correlations of QUS parameters (estimated calcaneal BMD, BUA, and SOS) with spine and hip BMD were modest (r = 0.50, r = 0.53, r = 0.41 for the spine and r = 0.54, r = 0.56, and r = 0.46 for the hip, respectively; p < 0.01 for all comparisons), but stronger in postmenopausal women. All postmenopausal women with low estimated calcaneal BMD (T-score pound -1) had a T-score pound -1 by DXA of the spine or hip. Of the 21 premenopausal women with spine or hip T-score pound -1 who had 25OHD measurements, 20 (95.2%) had levels below 50 nmol/L and 12 (57%) had levels below 30 nmol/L. The subgroup with 25OHD < 30 nmol/L had significantly lower spine (p < 0.01) and hip BMD (p < 0.05) than the subgroup with 25OHD >/= 30 nmol/L. QUS parameters were not significantly different between the two subgroups. The QUS and DXA correlated modestly well in women with prevalent hypovitaminosis D. QUS could be used in postmenopausal women with hypovitaminosis D to identify those at risk for osteoporotic fracture.

Clinical implications of the interaction between hypothyroidism and the cardiovascular system.

Hypothyroidism frequently causes cardiovascular manifestations that can complicate treatment of both the hypothyroidism and of any underlying heart disease. This review discusses mechanisms, pathophysiology, and management.

New drugs for reducing cardiovascular risk in women.

Atherosclerosis is largely preventable in women. Clinicians need to appreciate the gender-associated risks of cardiovascular disease and emphasize to their women patients that life-style changes can reduce cardiovascular risk. However, newer oral agents for diabetes and the statins for hyperlipidemia are important pharmacological adjuncts.

A clinical trial of chronic care diabetic clinics in general practice in the United Arab Emirates: a preliminary analysis.

Control of diabetes mellitus is a high priority for primary health care systems. One innovative method of diabetes care delivery is the use of structured diabetes care in primary care. This includes the use of chronic care diabetes clinics or mini-clinics operated by general practitioners in primary care. There is limited experience with this model in non-Western settings. This study sought to evaluate a multi-component structured approach to diabetes care in primary care including chronic care diabetes clinics in a newly developed country in the Arabian Gulf. The study design used was a controlled before-after methodology. Three primary health centers were chosen for the intervention with six of the remaining clinics in a Health District being used as controls. A multifaceted intervention was initiated in the intervention clinics composed of chronic care diabetes clinics, a diabetic flow chart, and educational programs for clinic nurses and doctors and patients. The study intervention took place over a period of 18 months with three diabetic outcomes (fasting blood glucose, blood pressure and cholesterol) and adherence to seven diabetes guidelines being compared for the year prior to the intervention and during the last 12 months of the intervention period. Knowledge and satisfaction questionnaires were also administered to intervention and control subjects at the end of the study. In this study, 219 subjects were enrolled (130 males and 89 females). They had a mean age of 51.6 years and a mean of 3.1 years of formal education. Of these 109 were enrolled in one of three clinics that had a chronic care diabetes clinic and 110 were enrolled in one of the six control clinics. Subjects had diabetes for a mean of 7.8 +/- 4.8 years and the majority was treated with pharmacological therapy. Baseline characteristics in the intervention and the control clinics were similar with the exception of younger age (p = 0.01) and a trend for more males (p = 0.06) in the intervention clinics. There was a statistically insignificant change noted with the intervention in the three clinical outcomes studied (fasting blood glucose, blood pressure and cholesterol) both in comparison to the control group before and after and within the intervention group. However most changes noted were in the expected direction of improvement; six of the seven guidelines were statistically improved in the intervention group when compared with the control group. Within the intervention group, adherence with five of seven guidelines was also statistically significantly increased with the remaining guidelines showing a trend in favor of improvement (fasting blood glucose measurements (p = 0.07) and urine determinations for protein (p = 0.07)). Knowledge questionnaire scores were similar between the intervention and control groups on completion of the study but 2 of 4 items on a satisfaction scale were statistically significantly higher in the intervention group. The intervention described in this setting was successful in improving adherence to diabetes guidelines and increased some aspects of satisfaction with diabetes care. The intervention did not result in a statistically significant improvement in clinical outcomes but changes noted were in the expected direction of improvement. The significant improvement in adherence to diabetes guidelines suggests that this intervention is a promising model for diabetes care for newly developed countries.

Acanthosis nigricans, hyperinsulinaemia and risk factors for cardiovascular disease.

We examined the association between acanthosis nigricans, hyperinsulinaemia and other risk factors for cardiovascular disease in patients from the United Arab Emirates. Height, weight and sitting blood pressure were recorded in 122 patients with acanthosis nigricans, and blood samples were obtained for measuring fasting cholesterol, high-density lipoprotein cholesterol, triglyceride and uric acid levels. In addition, a glucose tolerance test was performed and blood was sampled for insulin and glucose. Our results indicate that the patients with acanthosis nigricans had a high prevalence of abnormal glucose tolerance and hyperinsulinaemia. In addition, euglycaemic patients with hyperinsulinaemia had a cluster of risk factors for cardiovascular disease.

Bone density estimates and risk factors for osteoporosis in young women.

The bone mineral density (BMD) of the calcaneus was estimated in 185 young women from the United Arab Emirates, using SAHARA ultrasound. All participants completed a questionnaire on factors potentially associated with osteoporosis. In all, 29 (15.7%) of the women were classified as having osteopenia and none as having osteoporosis. Participants with osteopenia were more likely to have had a later onset of menarche, irregular periods, lower body mass index, and a positive family history of osteoporosis. Only late menarche and low body mass index, however, were independent predictors of osteopenia.

[Rocuronium and sugammadex use for the management of neuromuscular blockade in urgent abdominal surgery in a patient with Landouzy-Dejerine myopathy]. / Gestion de la curarisation par rocuronium et sugammadex pour chirurgie abdominale en urgence chez une patiente porteuse d'une myopathie facio-scapulo-humérale.

In patients with neuromuscular diseases, the use of rocuronium in the general anesthesia rapid sequence induction provides safety intubation conditions, but induces a deep and prolonged neuromuscular blockade. We report dose reduction to 0.8mg/kg for a 47-year-old female with Landouzy-Dejerine myopathy. Therefore, less dose of sugammadex was given to reverse the neuromuscular block.

Post-menopausal endometrial tuberculosis mimicking carcinoma: An important differential diagnosis to consider.

We report the case of a menopausal 74-year-old patient who presents pyorrhoea for 6months. We suspect initially a carcinoma process, but the anatomopathological examination takings obtained by biopsy curettage of the endometrial under hysteroscopy is in favor of an inflammatory infiltrate with epithelioid and giant cells of type Langhans and type Muller without caseous necrosis. The bacteriological direct examination after coloring of Gram, Ziehl-Neelsen and Sabouraud was negative. The bacteriological culture in the Löewenstein and Coletsos environment identified Mycobacterium tuberculosis. The searches for another source of the infection was negative both at the pulmonary and urinary levels. An antituberculous quadritherapy allows the fast clinical improvement. The tuberculosis remains frequent but rarely genital. It is especially the case of young women, from non industrialized countries, consulting for infertility. It is necessary to know how to evoke it front in pelvic symptoms, whatever the age is and to realize easily mycobacteriological examinations.