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Biomedical subjects

M Bakir

Publications and source records attributed to M Bakir.

At least 37 records · Page 2Linked to original sources
PubMed1999

Pattern of central nervous system anomalies in a population with a high rate of consanguineous marriages.

Nine thousand six hundred and ten births were prospectively studied in the three major hospitals in Al-Ain, United Arab Emirates (UAE) between October 1995 and January 1997. Babies suspected of, or diagnosed, as having central nervous system (CNS) abnormalities were evaluated by a neonatologist, a clinical geneticist and a pediatric neurologist. Brain computerized tomography/magnetic resonance imaging (CT/MRI) was performed on all babies suspected of having CNS abnormalities. In addition, metabolic screening and chromosome analysis were also performed when indicated. Of the 225 babies with congenital anomalies identified, 31 had CNS abnormalities (3.2/1000). Syndromic abnormalities of the CNS were present in 13 cases (42%), chromosomal abnormalities in one case (3.2%) and the rest included: neural tube defect (NTD) in 11 cases (36%), holoprosencephaly in two cases (6.4%) and hydrocephalus in four cases (12.9%). Detailed analysis of the syndromic types revealed that out of the 13 cases, 12 were inherited as autosomal recessive (AR) and in one case the inheritance was undetermined. Consanguinity with high level of inbreeding was present in 12 cases and the majority of the syndromes identified were extremely rare. The study indicates that CNS anomalies are fairly common in the UAE, particularly, the recessive syndromic types. Careful and detailed analysis of such anomalies is required so that accurate genetic advice can be given.

Central Nervous System↗
PubMed1999

Anterior segment anomalies of the eye associated with multiple skeletal abnormalities and early lethality: confirmation of an autosomal recessive syndrome.

We report two sibs from a complexly consanguineous family with joint contractures, skeletal abnormalities, anterior segment anomalies of the eye and early lethality. The features of these sibs are similar to the features of the sibs reported by Al-Gazali et al. (Clin Dysmorphol 3: 238-244, 1994). This report confirms the identity of this new autosomal recessive syndrome.

Abnormalities, Multiple↗
PubMed1999

Recurrence of the severe form of microgastria-limb reduction defect in a consanguineous family.

This report concerns two sibs from a consanguineous Sudanese family with microgastria-limb reduction defect associated with hydrocephalus and agenesis of corpus callosum. We suggest that these cases together with other previously reported cases of central nervous system (CNS) anomalies associated with microgastria-limb reduction defect could represent an autosomal recessive syndrome differing from the classical microgastria-limb reduction defect by its severity, presence of CNS anomalies and its pattern of inheritance.

Abnormalities, Multiple↗
PubMed1999

Complex consanguinity associated with short rib-polydactyly syndrome III and congenital infection-like syndrome: a diagnostic problem in dysmorphic syndromes.

Short rib-polydactyly syndromes (SRPS) are a heterogeneous group of recessively inherited lethal skeletal dysplasias. Four types have been recognised. However, overlap in the clinical and radiological features of the four types has led to difficulties in distinguishing between them. The congenital infection-like syndrome is an autosomal recessive syndrome characterised by mental retardation, microcephaly, seizures, and intracranial calcifications. We report a complex consanguineous family of Baluchi origin in whom short rib-polydactyly type III and congenital infection-like syndrome are segregating. Four children inherited SRPS III, one inherited congenital infection-like syndrome, and one inherited both. Although the radiological features in all the children with SRPS in this report were typical of type III, there was overlap in the clinical features with the other types of SRP syndromes. Furthermore, the child who inherited both SRPS III and congenital infection-like syndrome had CNS malformations in addition to periventricular calcification. CNS malformations have been described in SRPS types II and IV but not type III. This report further highlights the overlap between the different types of SRP syndrome. Moreover, it draws attention to the importance of considering the possibility of two recessive syndromes in the same child in complex consanguineous families when features overlap two syndromes.

Abnormalities, Multiple↗
PubMed1998

XK aprosencephaly.

We report a baby with aprosencephaly, preaxial limb defect and ambiguous genitalia. This combination of abnormalities have been reported previously and constitute the XK aprosencephaly syndrome.

Abnormalities, Multiple↗
PubMed1998

Agyria-pachygyria and agenesis of the corpus callosum: autosomal recessive inheritance with neonatal death.

We report three neonates, one boy and two girls, born to an inbred Arab family who had cortical dysplasia, probably agyria-pachygyria, and agenesis of the corpus callosum. All had asphyxia, intractable seizures, and increased muscle tone at birth and died in the neonatal period. Congenital microcephaly or dysmorphic features were absent. Cytogenetic abnormality, metabolic disorder, and intrauterine infection were excluded. These cases suggest a new cerebral dysgenesis syndrome with autosomal recessive inheritance.

Agenesis of Corpus Callosum↗
PubMed1997

Intranasal budesonide spray as an adjunct to oral antibiotic therapy for acute sinusitis in children.

BACKGROUND: The role of topical corticosteroids in the treatment of acute sinusitis has not been established in children. OBJECTIVE: An attempt was made to determine the impact of topical corticosteroids as an adjunct to antibiotic treatment in the management of childhood sinusitis. METHODS: In a double-blind, placebo-controlled study, 151 children with sinusitis were recruited from a general pediatric outpatient clinic and 89 completed a 3-week trial. Treatment consisted of amoxicillin-clavulanate potassium, 40 mg/kg/d tid, combined with bid nasal spray of either budesonide, 50 micrograms, to each nostril (n = 43) or placebo )n = 46_ for 3 weeks. Patients maintained daily symptom cards throughout the study and were examined by the same physician each week. RESULTS: Clinical symptoms and signs decreased significantly in both treatment groups in comparison to baseline (P < .01). We detected a significant improvement in the scores of the cough and nasal discharge at the end of second week in the budesonide group when compared with placebo (P < .05). Friedman nonparametric repeated measures ANOVA test revealed a significant decrease in the total weekly scores of cough during the second week of budesonide treatment (P < .001) in contrast to continuous decline during the second and third weeks in the placebo group (P < .001 and P < .05, respectively). While the nasal discharge score decreased significantly during the second week in the budesonide group (P < .01), no significant effect on the nasal discharge score was observed in the placebo group. CONCLUSION: These data suggest that topical corticosteroids may be a useful ancillary treatment to antibiotics in childhood sinusitis and effective in reducing the cough and nasal discharge earlier in the course of acute sinusitis.

Acute Disease↗
PubMed1997

Fibrochondrogenesis: clinical and radiological features.

We report an Omani child with fibrochondrogenesis. This neonatally lethal bone dysplasia is characterized by a distinctive facial appearance and specific radiological changes. Only seven cases have been reported previously. The clinical and radiological features together with the differential diagnosis is discussed.

Abnormalities, Multiple↗
PubMed1996

Severe facial clefting, limbic dermoid, hypoplasia of the corpus callosum, and multiple skin appendages: severe frontofacionasal "dysplasia" or newly recognised syndrome?

We report on a child with severe midline facial cleft, bilateral cleft lip and palate, telecanthus, S-shaped palpebral fissures, limbic dermoid, midface hypoplasia, hypoplastic corpus callosum, and multiple skin appendages. This case may be an example of severe frontofacionasal "dysplasia" or a newly recognised syndrome.

Abnormalities, Multiple↗
PubMed1995

Postoperative wound infections.

A prospective study of postoperative wound infection was carried out over a two year period in Cumhuriyet University Medicine Faculty Hospital in Sivas, Turkey. Examination of wounds, with cultures of all suspicious wounds using standard bacteriological methods was performed. Of a total of 4146 surgical wounds, 188 (4.53%), became infected. High infection rates were noted after colon resection (32.1%), gastric and oesophageal operations (21.1%), cholesystectomy (17.2%), and splenectomy (10.2%). Low infection rates were noted after thyroidectomy, mastectomy, caesarean section and abdominal hysterectomy. The commonest causative organisms were coagulase-negative staphylococci 21.7%, Staphylococcus aureus 19.7%, Escherichia coli 19.7%, Enterobacter spp. 17.6%, and Pseudomonas spp. 10.7%.

Bacterial Infections↗
PubMed1995

The prevalence and correlates of anaemia among young children and women of childbearing age in Al Ain, United Arab Emirates.

During 1992 and 1993, 309 children aged 1-22 months (mo) and their mothers visiting an immunization facility in Al Ain city, Abu Dhabi Emirate, United Arab Emirates, were studied to (1) define blood haemoglobin (Hb) levels; (2) estimate the prevalence of anaemia; (3) examine the role of iron deficiency in causing anaemia; (4) identify the correlates of anaemia prevalence; and (5) assess the acceptability to parents of an anaemia screening test for their young children. The levels of Hb in all subjects and the levels of serum ferritin in anaemic (Hb < 11 g/dl) children aged > or = 6 mo and in non-pregnant women with Hb < 11 g/dl were determined by a HemoCue Photometer and an enzyme immunoassay, respectively. Each woman was interviewed to obtain pertinent data. In children, anaemia was detected in 3% of those aged 1-2 mo (Hb < 9 g/dl), in 8% of those aged 3-5 mo (Hb < 10 g/dl), and in 25-39% of those aged > or = 6 mo (Hb < 11 g/dl). Of 19 children tested, ten (53%) were iron-depleted (serum ferritin < 12 micrograms/l). After multivariate adjustment, the only significant positive correlate of anaemia in children was older age (1-5 mo vs 6-22 mo; odds ratio [OR]: 9.51; 95% confidence interval [CI]: 3.92-23.08). Anaemia was detected in 14% of pregnant women (Hb < 11 g/dl) and 16% of non-pregnant women (Hb < 12 g/dl).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1995

Treatment of tuberculous meningitis in Turkey.

A prospective study was performed on 72 cases of tuberculous meningitis studying various treatments. 37 patients were treated with a combination of isoniazid, rifampicin, pyrazinamide and streptomycin for 2 months, followed by a combination of isoniazid and rifampicin for 6 months. 35 patients were treated with various combinations of antituberculous drugs for 12-16 months. Disappearance of symptoms took (mean) 17 days. Mean duration of therapy for the hospitalized patients was 36 +/- 4 days. Seven (9.7%) patients died, 5 in the short-course therapy group and 2 in the long-course therapy group. Sequelae persisted in 18 (31%) cases, 8 of which cases were in the short-course therapy group and 10 in the long-course therapy group. No relapse was observed in either of the groups.

Adolescent↗
PubMed1994

The value of radionuclide splenic scanning in the evaluation of asplenia in patients with heterotaxy.

Splenic anomalies frequently accompany conotruncal and atrioventricular septal malformations. Asplenia is a major factor in the mortality of newborns with the heterotaxy syndrome, requiring an early and accurate diagnosis. We evaluated the splenic status of five consecutive patients with heterotaxy syndrome by radionuclide splenic scanning with 99mTc-labelled and denatured red blood cells (RBCs) and by real-time abdominal ultrasonography. Examination and comparison of the findings using these diagnostic methods suggest that the former has some diagnostic pitfalls which arise from the symmetrical location of the liver in the abdomen. This leads to difficulty in the interpretation of overlapping signals from the blood pool of the liver and from the spleen.

Abnormalities, Multiple↗
PubMed1994

Direct intraperitoneal insemination and controlled ovarian hyperstimulation in subfertile couples.

METHOD: We performed direct intraperitoneal insemination in combination with superovulation and washed husband's spermatozoa as the treatment of infertility caused by oligospermia, cervical subfertility, or ovulatory dysfunction or idiopathic infertility in 162 couples during 360 cycles. RESULTS: The pregnancy rate per couple was 21.9% in the cervical-factor group, 20.9% in the idiopathic group, 4.9% in the oligospermia group, and 15.5% in the ovulatory dysfunction group. CONCLUSION: The pregnancy rate was 33.3% for all couples and 15.0% for all cycles.

Adult↗