Siamese twins in the United Arab Emirates

In the years 1985-1992, ten pairs of conjoined twins were born in the United Arab Emirates (UAE): one dicephalus, two teratopagi, and seven thoracoomphalopagi, one of which was still-born and three who were cared for in other hospitals. The first pair of thoraco-omphalopagus twins died of acute lymphoblastic leukaemia 6 months after successful separation. The management of the third set of twins gave rise to moral and ethical problems often encountered in such situations, while one of the teratopagi was a unique case of a parasite projecting from the mouth of the normal twin.     

Hirsutism in the United Arab Emirates: a hospital study

In a study of 102 women (Arabs and Asians) with hirsutism, polycystic ovary syndrome was diagnosed in 93 patients (91%) including 26 cases with hirsutism and regular menstrual periods. In more than half of the cases the diagnosis was made on the basis of transabdominal ultrasonography, which was the single test with the highest yield. Luteinising hormone hyperresponsiveness to gonadotropin-releasing hormone was positive in only seven of 50 patients tested. Late onset congenital adrenal hyperplasia due to 21-hydroxylase deficiency was identified in two cases, thus forming a prevalence of 8% in those who underwent adrenocorticotropin stimulation. In one case, polycystic ovary syndrome was an associated condition. In this study of a population of women with hirsutism and regular menses, the majority had polycystic ovaries. It is suggested that the term 'idiopathic or racial' hirsutism should not be applied unless a thorough evaluation has failed to reveal a diagnosable underlying disorder.     

Tracheomalacia with esophageal atresia and tracheoesophageal fistula in fetal rats

BACKGROUND: Many patients who have esophageal atresia and tracheoesophageal fistula (EA-TEF) have associated tracheomalacia, which is thought to be one of the reasons for respiratory complications after surgical correction of the abnormality. METHODS: In this study, tracheas from Adriamycin-induced EA-TEF fetal rats were examined histologically and relevant cross-sectional parameters of the tracheas were measured. RESULTS: The tracheal lumen in tracheomalacia was small and irregular, losing its normal "D" shape. In most rats, the cartilaginous ring was broken into two to four segments, making the trachea lose its rigid support. The submucosa was thickened with prominent bulging of its membranous part into the tracheal lumen. The ratio of the inner luminal cross-sectional area to the outer tracheal cross-sectional area in EA-TEF rats was 15.7%, compared with a control ratio of 47.2%. In EA-TEF rats, the length of the cartilaginous ring was significantly shortened (P < .001), but not the length of membranous trachea, thus resulting in a cartilaginous/membranous (C/M) ratio of 1.55:1, markedly lower than that of normal rats (4.34:1, P < .001). The reduction of anterior-posterior diameter of the tracheal lumen was more marked than that of the transverse diameter. CONCLUSIONS: These observations suggest that the trachea in EA-TEF rats has a smaller lumen and is more flaccid than normal, making it prone to airway obstruction. The fact that tracheomalacia developed only in fetuses who had EA-TEF indicates that the factors that result in EA-TEF also cause tracheomalacia.     

Adolescents' perceptions of family functioning in the United Arab Emirates

A sample of 710 subjects between 14 and 19 years of age were examined to assess psychological health of United Arab Emirates (UAE) families with adolescent members. The Family Functioning Questionnaire (FFQ) based upon the adolescent's perception of his or her family life and climate contains seven dimensions that constitute the family functioning process: family structure, emotional fulfillment, internal relations, behavioral control, value transmission, basic requirements, and external relations. Results indicate that the adolescents perceived their families to be operating more toward the functional than dysfunctional end of the scale. Males scored their families more functional in family structure and in external relations more significantly than did females. Females perceived their families as more functioning in internal relations, behavioral control, and basic requirements. However, a trend was found in favor of males on emotional fulfillment. Family functioning scores were higher in upper social economic status (SES) families than in other SES levels.     

Febrile episodes in children with cancer in the United Arab Emirates

We report the first patient with a partial trisomy and a partial monosomy of the long arm of chromosome 4: 46,XY, inv dup(4)(pter-->q32::q32-->q26), del(4)(q32-->qter). The boy died from a complex cardiac defect (monoventricle, monoatrium and truncus arteriosus) in combination with a diaphragmatic hernia. In addition he had preaxial polydactyly of the right hand. We compare the clinical features with data from the literature. The phenotype of the patient mainly resembles that in patients with a terminal deletion 4q32.     

Prognostic classification for esophageal atresia and tracheoesophageal fistula: Waterston versus Montreal

Since 1962, the Waterston classification has been used to stratify neonates who have esophageal atresia (EA) and/or tracheoesophageal fistula (TEF) into prognostic categories based on birth weight, the presence of pneumonia, and the identification of other congenital anomalies. In response to advances in neonatal care, the surgeons from the Montreal Children's Hospital proposed a new categorization system in 1993 in an attempt to define the current risk factors for patients who have EA/TEF. In the Montreal experience only two characteristics independently affected survival: preoperative ventilator dependence and associated major anomalies. The goal of this study was to determine which system had the greatest validity for the evaluation of prognosis in our patients with EA/TEF. The charts of 94 patients who had EA/TEF treated between 1972 and 1991 were reviewed. Patients were classified using both the Waterston and Montreal systems. Groups were compared with Fisher's Exact test using a 95% confidence level for statistical significance. Eleven infants were ventilator dependent preoperatively; 62 children had major associated anomalies, 8 of which were considered life threatening. Sixteen children died within 4 years, eight during their initial hospital stay. Five of the eight early postoperative deaths occurred in the highest-risk patients (Waterston C or Montreal II). Analysis was performed for multiple risk factors and mortality. As in the Montreal study, the presence of life-threatening and major congenital anomalies represented significant risk factors for death. Pulmonary disease as delineated by ventilator dependence appeared to be more accurate than pneumonia. This study confirms the accuracy of the Montreal classification in defining prognosis for EA/TEF. The Montreal system more accurately identifies children at highest risk than the Waterston classification.     

Clinical features of Crimean-Congo haemorrhagic fever in the United Arab Emirates

Crimean-Congo haemorrhagic fever (C-CHF) re-emerged recently in the United Arab Emirates. The clinical outcome of 11 cases of viral haemorrhagic fever patients admitted to hospital between June 1994 and January 1995 is described. Four cases were laboratory confirmed retrospectively as C-CHF, the other patients were diagnosed likely to have the same disease on epidemiological and clinical grounds. In 72.7% of the patients, infection was fatal. Symptoms started 3.5 days before hospitalization. On admission, 81.8% of patients had high fever, 45.5% were vomiting, 63.6% had diarrhoea, 45.5% had haemorrhagic signs, and 18.2% had throat pain. Fatalities occurred 6.8 days after admission. Survivors were hospitalized for 9.3 days. Nosocomial transmission was not observed.     

A new variant of esophageal atresia with tracheoesophageal fistula and duodenal atresia: a diagnostic dilemma

A new variant of esophageal atresia (EA) with tracheoesophageal fistula (TEF) associated with duodenal atresia is reported. The TEF was between the lower pouch and the trachea, with a cystic dilatation in the midportion. The tracheal end of the fistula was obstructed by a membranous septum at both ends of a cystic dilatation, leading to a diagnosis of pure EA (gasless abdomen). After the lower pouch was opened beyond the cystic dilatation, 100 ml nonbilious fluid was obtained. A laparotomy revealed a type III atresia of the first part of the duodenum.     

Lung abscess: an unusual presentation of congenital tracheoesophageal fistula without atresia

Tracheoesophageal fistula (TEF) without atresia is rare and usually presents with symptoms from birth. In this report, a 9-year-old boy presented with productive cough of 4 month's duration and was shown to have a right lung abscess seen on chest radiograph. His parents denied earlier respiratory symptoms or illnesses. Rigid bronchoscopy showed a fistulous opening of about 1 mm in diameter in the posterior wall of the trachea about 16 cm from the upper incisor teeth. Cannulation with a ureteral catheter demonstrated that the fistulous opening communicated with the esophageal lumen. The tracheoesophageal fistula was 1 cm long and was divided through a right supraclavicular incision. The postoperative period was uneventful, and the patient was discharged on the third postoperative day. This case demonstrated that TEF should be considered in any patient presenting with chronic respiratory problems even after a prolonged symptom-free period.     

Histopathologic study of esophageal atresia and tracheoesophageal fistula in an animal model

Authors propose one study of retinal cells population in different stages of ontogenesis and one study of the pigmentogenesis process at the level of uveal tract and external retinal stratum. The study was achieved with embryonic and fetal technique of paraffin inclusion. Concomitantly with loading with pigment of the external retinal stratum and so pigmentary uveal tract is present. Dynamics of the retinol cells population varies with stages and chronological age, the number of pigmented uveal cells increasing proportionally but at different parameters with the pigmented retinal stratum one simultaneous with age under influence specially of humoral factors.     

An unusual case of esophageal atresia and double distal tracheoesophageal fistula

This report describes a neonate with a very rare and an unusual variety of esophageal atresia and tracheoesophageal fistula. The anomaly consisted of esophageal atresia and double distal tracheoesophageal fistula. The two fistulae as well as part of the distal esophagus were made up of tracheobronchial tissues. The embryology of the anomaly is also discussed.     

Duodenal volvulus in free-living green turtles from coastal United Arab Emirates

Post-mortem examinations performed during May and August of 1997 on three free-living green turtles (Chelonia mydas) in the United Arab Emirates revealed that all had stomachs full of fresh seagrass (approximately 99% of the total ingesta) and presented with a duodenal volvulus involving a length of approximately 100 cm. Duodeni appeared empty and necrotic with diffuse purple-black mucosa. No apparent signs of obstruction by foreign objects, acute endoparasitism, or other disorders were observed. In all cases, duodenal volvulus was diagnosed, the cause of which may have been dietary in origin. The rise in water temperature, with an associated rise in the temperature of seagrass, thus enhancing the over-fermentation of ingesta with the subsequent liberation of excessive amounts of gas was the probable cause of volvulus formation. Ingestion of foreign bodies and physical movement also are discussed as causes of digestive disorders. It is recommended that handlers should avoid 360 degrees rotation when overturning turtles onto their carapaces.     

Surveying the counselling needs of families with disablement in the United Arab Emirates

We have previously described spontaneous but reversible hair loss that clinically and histologically resembles human alopecia areata in a colony of C3H/HeJ mice. Alopecia areata in humans is associated with antibodies to hair follicles. This study was conducted to determine whether C3H/HeJ mice with hair loss have a similar abnormal antibody response to hair follicles. Eighteen C3H/HeJ mice with alopecia, 12 unaffected littermates, and 15 control mice were examined for circulating antibodies to C3H/HeJ anagen hair follicles by indirect immunofluorescence and against extracts of isolated C3H/HeJ and human anagen hair follicles by immunoblotting. Using both procedures, antibodies to anagen hair follicles were present in all C3H/HeJ mice with alopecia but in none of the control mice. The antibodies were also present in some unaffected C3H/HeJ littermates but were absent in mice of an unrelated strain with inflammatory skin disease and alopecia, indicating that their appearance did not result from the hair loss. These antibodies reacted to hair follicle-specific antigens of 40-60 kDa present in murine and human anagen hair follicles. These antigens were also reactive with human alopecia areata antibodies. Some of the antibodies in both C3H/HeJ mice and humans with alopecia areata reacted to antigens of 44 and 46 kDa, which were identified as hair follicle-specific keratins. This study indicates that C3H/HeJ mice with hair loss have circulating antibodies to hair follicles similar to those present in humans with alopecia areata. These findings confirm that these mice are an appropriate model for human alopecia areata and support the hypothesis that alopecia areata results from an abnormal autoimmune response to hair follicles.     

Mal de Meleda: a report of four cases from the United Arab Emirates

Scanning electron microscopy was used to study the effect of cyclophosphamide (Cy) on molar development in 18 Sprague-Dawley rats from 15 to 48 days of age after birth. Doses of 30 mg/kg body weight of Cy dissolved in 1 ml 0.9% NaCl were given to the rats at 10 and 13 days of age. Eighteen control rats had injections of 1 ml 0.9% NaCl at the same ages. The most obvious changes in the experimental teeth were found in the developing roots of the first and second molars and in both the crown and roots of the third molar. The roots of the first and second molars were short and showed apical closure in the experimental rats. In addition to the disturbances in crown and root formation, the third molars were also significantly reduced in total size as compared with the third molars in the control rats.     

Relationship between esophageal atresia with tracheoesophageal fistula and vertebral anomalies in mammalian embryos

During the last years it became obvious that a lot of families of long-range repetitive DNA elements are located within the genomes of mammals. The principles underlying the evolution of such families, therefore, may have a greater impact than anticipated on the evolution of the mammalian genome as a whole. One of these families, called chAB4, is represented with about 50 copies within the human and the chimpanzee genomes and with only a few copies in the genomes of gorilla, orang-utan, and gibbon. Members of chAB4 are located on 10 different human chromosomes. FISH of chAB4-specific probes to chromosome preparations of the great apes showed that chAB4 is located, with only one exception, at orthologous places in the human and the chimpanzee genome. About half the copies in the human genome belong to two species-specific subfamilies that evolved after the divergence of the human and the chimpanzee lineages. The analysis of chAB4-specific PCR-products derived from DNA of rodent/human cell hybrids showed that members of the two human-specific subfamilies can be found on 9 of the 10 chAB4-carrying chromosomes. Taken together, these results demonstrate that the members of DNA sequence families can evolve as a unit despite their location at multiple sites on different chromosomes. The concerted evolution of the family members is a result of frequent exchanges of DNA sequences between copies located on different chromosomes. Interchromosomal exchanges apparently take place without greater alterations in chromosome structure.     

13 pairs of ribs--a predictor of long gap atresia in tracheoesophageal fistula

BACKGROUND/PURPOSE: Results of treatment of tracheoesophageal fistula (TEF) have improved over the years. However, long gap atresias continue to be a problem and require modification in the conventional operation. Preoperative diagnosis of a long gap atresia in a case of TEF is difficult, and often the defect is noted only at thoracotomy, thus necessitating multiple intraoperative changes in the position of the neonate. In the past 5 years the authors have treated 61 cases of TEF. Of these, 12 had a long gap atresia. Nine of these 12 patients had 13 pairs of ribs. None of the patients with a short gap atresia had 13 pairs of ribs. Hence, the presence of 13 pairs of ribs is a good indicator of long gap atresia. In a child who has TEF with 13 pairs of ribs, suitable modifications in operative procedure can be planned. The report also discusses the possible embryological basis of this association.