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K Ajlouni

Publications and source records attributed to K Ajlouni.

At least 19 recordsLinked to original sources

Consanguineous marriages in Jordan: why is the rate changing with time?

The objective of this study was to explore the secular trend in consanguinity in Jordan and the subtypes of consanguineous marriages that may be undergoing a change. A total of 1032 individuals attending a diabetic clinic in Amman were interviewed. The questionnaire provided information on consanguinity status and date of marriage among three generations: the persons interviewed, their parents, parents of their spouses and their offspring. Data on consanguinity status among 5401 marriages was obtained. Generations were named generation 1 for marriages contracted before 1950, generation 2 for marriages contracted between 1950 and 1979, and generation 3 for marriages contracted after 1980. For generations 1, 2, and 3, first-cousin marriages constituted 20.2, 28.5 and 19.5% of all marriages, respectively, while the subtype of paternal parallel first-cousin marriages constituted 75.6, 60.3 and 43.3% of all first-cousin marriages, respectively. The offspring of first-cousin parents were significantly more prone to marry their relatives than the offspring of non-consanguineous parents, with rates of first-cousin marriages among offspring of first-cousin parents and non-related parents constituting 25.3 and 17.1% of all marriages, respectively. For generations 1, 2 and 3, the average coefficient of inbreeding was 0.0135, 0.02 and 0.0142, respectively. In conclusion, first-cousin marriage rate among a representative population from Amman showed a significant decline among marriages contracted after 1980 compared to marriages contracted between 1950 and 1979, but not to marriages contracted before 1950. The proportion of paternal parallel first cousins among first-cousin marriages showed a steady decline from one generation to the next.

Consanguinity↗

Bleeding tendency in Wolfram syndrome: a newly identified feature with phenotype genotype correlation.

Wolfram syndrome (WS) is a recessively inherited disorder associated with recognised clinical features. Bleeding tendency was noticed in some of our patients, although this has not been reported before. We therefore studied this problem in all our WS patients and tried to postulate a possible pathogenesis. At the same time, a genetic linkage study provided evidence of locus heterogeneity of this syndrome and showed that the majority of our patients belong to the second WS locus identified in that study. Our study group consisted of 13 WS patients, belonging to WSF2 locus (group I). Controls consisted of 4 healthy siblings of WS patients (group II) and 7 diabetics who do not have WS (group III). Relevant clinical data were obtained, and a coagulation screen was carried out for all groups. All individuals in the three study groups have normal platelet count, thrombin time (TT), prothrombin time (PT), activated partial thromboplastin time (aPTT), clot retraction, Factor VIII activity (FVIIIc) and von Willebrand factor antigen (vWAg). Eleven of the WS patients have prolonged template bleeding time (BT) compared with both control groups. Patients with WS have a longer BT (mean 9.6 min, 95% CL 8.61-10.53 min) than the siblings group (mean 6.75 min, 95% CL 5.52-7.98 min) and the diabetic group (mean 5.49 min, 95% CL 4.56-6.42 min). The differences between the study group and controls are statistically significant, p = 0.02 and 0.0002, respectively. In the three groups, platelet aggregation studies were normal using adenosine diphosphate (ADP), ristocetin and epinephrine. Aggregation with collagen was either absent or impaired, with failure of secondary wave being noticed in 11 of the WS patients (85%) and normal in the control groups. The pathogenesis of this problem is not known, but could be due to an inhibitory effect of vWAgII, deficiency of thrombospondin or a defect in the platelet membrane GPIa/IIa. Bleeding diathesis is a new additional feature to the clinical spectrum of WS, which is probably a feature of the disorder WFS2 and not WFS1, as bleeding has never been reported in the latter. This provides further evidence for the phenotypic and genotypic heterogeneity of this complex disorder and may provide clues to the search for the second gene responsible for this phenotype.

Adolescent↗

Growth status of Jordanian schoolchildren in military-funded schools.

OBJECTIVE: To study the growth status of Jordanian boys and girls in comparison with the Centers for Disease Control (CDC) growth charts. DESIGN: Cross-sectional study. SUBJECTS AND DATA COLLECTION: A total of 5826 boys and 1414 girls, aged 6.5-17.5 y, were included in the study. Height and weight were measured. Body mass index (BMI) was calculated as weight (kg) divided by the square of the height (m). Socioeconomic data were collected using a structured questionnaire. RESULTS: The height-for-age values fluctuated between the 5th and 10th percentiles of the CDC for both sexes, and then after the age of 8.5 and 14.5 y for boys and girls, respectively, values were just above the 10th percentile. The body weight-for-age values were just above the 25th percentile for boys and fluctuated between the 25th and 50th percentiles for girls; then after the age of 14.5 and 12.5 y for boy and girls, respectively, values fluctuated between the 25th and 50th percentiles for boys and just above the 50th percentile for girls of the CDC values. BMI values for boys were just above the 50th percentile of the CDC and for girls values fluctuated between the 50th and 75th percentiles until the age of 13.5, then values matched the 75th percentile of the CDC. CONCLUSION: The height of Jordanian children ranges from the 5th to the 10th percentile of the CDC reference values during schools years. Girls have a tendency toward obesity after puberty.

Adolescent↗

Homozygosity mapping identifies an additional locus for Wolfram syndrome on chromosome 4q.

Wolfram syndrome, which is sometimes referred to as "DIDMOAD" (diabetes insipidus, diabetes mellitus, optic atrophy, and deafness), is an autosomal recessive neurodegenerative disorder for which only insulin-dependent diabetes mellitus and optic atrophy are necessary to make the diagnosis. Researchers have mapped Wolfram syndrome to chromosome 4p16.1, and, recently, a gene encoding a putative transmembrane protein has been cloned and mutations have been identified in patients. To pursue the possibility of locus heterogeneity, 16 patients from four different families were recruited. These patients, who have the Wolfram syndrome phenotype, also have additional features that have not previously been reported. There is an absence of diabetes insipidus in all affected family members. In addition, several patients have profound upper gastrointestinal ulceration and bleeding. With the use of three microsatellite markers (D4S432, D4S3023, and D4S2366) reported to be linked to the chromosome 4p16.1 locus, we significantly excluded linkage in three of the four families. The two affected individuals in one family showed homozygosity for all three markers from the region of linkage on chromosome 4p16.1. For the other three families, genetic heterogeneity for Wolfram syndrome was verified by demonstration of linkage to chromosome 4q22-24. In conclusion, we report the unique clinical findings and linkage-analysis results of 16 patients with Wolfram syndrome and provide further evidence for the genetic heterogeneity of this disorder. We also provide data on a new locus that plays a role in the etiology of insulin-dependent diabetes mellitus.

Adolescent↗

Familial disorder of sex determination in seven individuals from three related sibships.

In humans, the sex of an individual is determined by the Y-chromosome-related SRY gene, which causes the differentiation of the undifferentiated gonads into testicular tissue. True hermaphrodites without a Y chromosome and XX males represent a sex determination error in which testicular tissue develops despite the absence of the SRY gene. Familial forms of XX true hermaphrodites and XX males exist in the literature, which also contains the two forms co-existing in the same family. In this report, we present a large family with seven affected individuals with phenotypes ranging from XX male to XX true hermaphrodite with predominance of female characteristics. We suggest that XX maleness and XX true hermaphroditism represent a continuum of the same disorder. We speculate on the mode of inheritance of this disorder in this particular family.

Adolescent↗

Incidence of insulin-dependent diabetes mellitus in Jordanian children aged 0-14 y during 1992-1996.

An attempt was made by the Jordanian National Center for Diabetes, Endocrine and Genetic Diseases (NCDEGD) to identify all cases of type 1 diabetes among Jordanian children aged 0-14 y. Data were obtained retrospectively for the years 1992-1994 and prospectively for the years 1995 and 1996, including full name, national identifying number, date of birth, date of diagnosis and family history. The incidence was calculated as the number of cases per 100,000 population, according to the national census of 1994. The incidence rate for these years (1992 through 1996) was 2.8, 2.9, 3.2, 3.6 and 3.6 per 100,000 population, respectively. The male:female ratio was (1:1.03). Seasonal variation at clinical onset was noticed, with maximum incidence in the winter months and minimum incidence in the summer months. In conclusion, the incidence of type 1 diabetes mellitus in Jordanian children aged 0-14 y is among the lowest in the region, but is rising.

Adolescent↗

Obesity in Jordan.

OBJECTIVES: To study the prevalence of obesity among semi-urban communities in Jordan and its association with a number of factors. DESIGN: A sample of households was systematically selected from four Jordanian towns namely, Sarih, Sikhra, Southern Mazar and Subha-Subhieh. All subjects aged > or = 25 y within the selected households, were invited to participate in the study. A total of 2836 subjects were actually included in the study, with an overall response rate of 70.5%. Study procedures were carried out in the local health centres in each town over a one-month period. RESULTS: The overall prevalence of obesity (body mass index, BMI > or = 30 kg/m2) was 49.7%; 32.7% in males and 59.8% in females. Obesity was more prevalent in the older age groups, illiterate people, diabetic and hypertensive subjects, and those with hypercholesterolaemia (HC), hypertriglyceridaemia (HTG), high level of low density lipoprotein cholesterol (LDL), and low level of high density lipoprotein cholesterol (HDL). After adjusting for age and gender, obesity was associated with diabetes mellitus (DM) (odds ratio (OR) 1.4, 95% confidence limit (CL) 1.1, 1.8), hypertension (HT) (OR 2.2, 95% CL 1.7, 2.8), HC (OR 1.3, 95% CL 1.1, 1.5), HTG (OR 2.3, 95% CL 2.0, 2.8), elevated LDL (OR 1.5, 95% CL 1.2, 1.8) and low HDL (OR 2.3, 95% CL 1.8, 2.9). CONCLUSION: Obesity seems to be a common disorder among adult Jordanians. More attention should be paid to this problem at the national level.

Adult↗

Diabetes and impaired glucose tolerance in Jordan: prevalence and associated risk factors.

OBJECTIVES: To study the prevalence of diabetes mellitus (DM) and impaired glucose tolerance (IGT) and their risk factors in Jordan. DESIGN: A cross-sectional study in four Jordanian communities was conducted: Sarih in the north, Sikhra in the middle of the country, Mazar in the south and Subha-Subheih in the east. Within each community, a systematic sample of households was selected. All subjects > or =25 years of age within the selected households were invited to participate in the study. A total of 2836 subjects were actually included in the study with an overall response rate of 70.5%(45% in males and 86% in females). World Health Organization criteria were adopted for the diagnosis of DM (fasting plasma glucose > or =7.8 mmol L(-1) or plasma glucose of > or =11.1 mmol L(-1), 2 h after an oral anhydrous glucose load of 75 g) and IGT (fasting plasma glucose <7.8 mmol L(-1)and plasma glucose between 7.8 mmol L(-1) and 11.1 mmol L(-1), 2 h after an oral glucose load of 75 g). RESULTS: The overall prevalence of DM was 13.4%: 14.9% in males and 12.5% in females. IGT was found in 9.8% of the study population: 9.0% in males and 10.3%, in females. Factors independently related to DM using stepwise logistic regression analysis were sex, age, family history of DM, hypertension, hypercholesterolaemia (HC), and hypertriglyceridaemia (HTG). CONCLUSION: Diabetes mellitus and IGT are common among adult Jordanians. Considering the high prevalence of this sickness makes it imperative to formulate a national plan to face this disease and its complications.

Adult↗

Plasma vasopressin responses in postpartum hypopituitarism: impaired response to osmotic stimuli.

The neurohypophyseal function was assessed in a group of 15 patients with postpartum hypopituitarism by measuring plasma arginine-vasopressin concentrations during 5% hypertonic saline infusion. None of the patients had symptoms of diabetes insipidus and all patients were on adequate cortisone and thyroxine replacement therapy before testing. The mean basal plasma vasopressin value in the patients (0.6 +/- 0.1 pmol/l) was significantly lower than that in the normal subjects (2.9 +/- 0.3 pmol/l; p < 0.01), whereas the mean serum sodium, plasma osmolality, plasma renin activity and serum aldosterone values were similar in the two groups. During the osmolar load (5% hypertonic saline), the patients revealed varying degrees of arginine-vasopressin responses to the increase in plasma osmolality. Three patients showed normal arginine-vasopressin responses, 10 had subnormal responses, and 2 had no response. During the dehydration test, the patients revealed significantly lower maximum urine osmolalities (p < 0.0025) with significantly higher concurrent mean plasma osmolality (p < 0.0025) than the controls. None of the patients showed overt polyuria at the time of the study. The results indicate the impaired osmoregulation of arginine-vasopressin secretion to an osmolar stimuli in patients with postpartum hypopituitarism, suggesting neurohypophyseal damage. In patients with Sheehan's syndrome, partial diabetes insipidus seems to be much more frequent than previously believed.

Adult↗

Chronic propranolol administration impairs glucagon release during insulin-induced hypoglycemia in normal man.

Failure of a plasma glucagon rise in response to insulin-induced hypoglycemia was demonstrated in normal subjects taking therapeutic doses of the nonselective beta-adrenergic blocker, propranolol, for 7 days before testing. This is the first study to examine glucose homeostasis in normal subjects exposed to chronic propranolol therapy and helps to explain the development of spontaneous hypoglycemia in patients, either diabetic or nondiabetic, during beta-adrenergic receptor blockade. Previous studies of the acute parenteral effects of propranolol administration failed to show any significant effect on glucagon secretory dynamics in response to insulin-induced hypoglycemia. In the present study, however, chronic oral administration of propranolol resulted in severe impairment of the expected glucagon rise in response to hypoglycemia and was associated with severe hypoglycemia in one normal subject.

Adult↗

The effect of acute hypercalcemia on prolactin release in man.

The serum prolactin response to acute hypercalcemia during calcium infusion was studied in 7 normal subjects. Prolactin, and calcium levels were determined at 30 minute intervals during the 210 minutes of the infusion. The infusion performed consisted of either normal saline at 3 ml/min for 210 minutes, or 15 mg calcium/kg at 3 ml/min for 180 minutes followed by normal saline infusion for the final 30 minutes. Calcium concentration increased from a base line of 10.0 +/- 0.26 (mean +/- SEM) to a maximal level of 13.6 +/- 0.26 (mean +/- SEM) mg/100 ml, in response to calcium level in response to normal saline infusion. Prolactin level decreased significantly (P less than 0.05) from 9.5 +/- 1.45 (mean +/- SEM) to 3.7 +/- 0.2 (mean +/- SEM) ng/ml at 180 minutes.

Adolescent↗

Effect of glucose of growth hormone, prolactin and thyroid-stimulating hormone response to diazepam in normal subjects.

The effect of hyperglycemia on growth hormone, thyroid-stimulating hormone, and prolactin response to oral diazepam (10 mg) was assessed in 7 normal subjects. A peak growth hormone response of 13.5 +/- 0.3 ng/ml (mean +/- SEM) significantly above the base line (p less than 0.001) was achieved when diazepam was given alone. Hyperglycemia after glucose load abolished this response. There was no significant response of thyroid-stimulating hormone or prolactin after the administration of oral diazepam with and without glucose.

Adult↗

Acute myelofibrosis and malignant hypercalcemia.

A 33 year old man presented with symptoms of one week's duration; he had a serum calcium of 22.5 mg/dl and a markedly hypercellular bone marrow. Despite therapy with saline diuresis, furosemide mithramycin, total parathyroidectomy and corticosteroids, symptomatic hypercalcemia was poorly controlled. Inappropriate serum parathyroid hormone (PTH) levels were found before and after parathyroidectomy whereas assays of the peripheral blood for osteoclast-activating factor and prostaglandin E (PGE2) were negative. An elevated leukocyte alkaline phosphate level, the inability to aspirate marrow, the marked generalized hyperplasia of all hematopoietic marrow elements, the focal accumulations of blastic cells and increasing reticulin fiber formation led to the diagnosis of acute myelofibrosis. A single course of cytosine arabinoside and thioguanine therapy was followed by profound hyperphosphatemia, hypocalcemia and death. The rarity of hypercalcemia with myeloproliferative disorders is documented by a review of the world literature, and the possible mechanism for hypercalcemia in this patient is discussed.

Acute Disease↗