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PubMedDate not supplied

An experience with free scapular flap for reconstruction of lower extremity defects at Aga Khan University Hospital Karachi.

BACKGROUND: Reconstruction of large composite tissue defects with exposed vital structures and weight bearing surfaces are extremely difficult for reconstructive surgeons. Coverage of such difficult defects can be achieved with free tissue transfer provided microsurgical expertise is available. This study was carried out to determine the outcome of free scapular flaps performed for lower extremity defects. METHODS: Clinical records of the patients were reviewed and important variables included demographic data, etiology of soft tissue defects, site and size of defect, complications and percentage of wound coverage by flap. Patient satisfaction and objective assessment of flap coverage and motion of involved joints was made. RESULTS: There were 13 male patients with mean age of 30.46 years. The causes of tissue defects were road traffic accident in 8 cases, industrial accidents in 2 and 3 cases had bomb blast, gunshot and gas gangrene one in each case respectively. Nine scapular flaps were performed to reconstruct the defects around the foot, ankle and lower leg; two flaps for amputation stumps and two flaps for defects around the knee and popliteal fossa. Post flap surgery, 3 cases had vascular compromise which required urgent exploration of anastomosis. Two cases were revived and one flap did not survive. With respect to cosmetic appearance, coverage of required defect, performance of activities of daily living and functional range of motion of involved joints, 6 cases were graded excellent and 7 cases were good. CONCLUSION: In our cases of lower extremities defects, scapular flap helped to salvage the limb. In conclusion scapular flap is a versatile flap which can be used for lower extremity defects.

Accidents, Occupational↗
PubMed2005

[The effect of fibula flap graft on the restoration of the extremities with traumatic compound tissue defects].

OBJECTIVE: To evaluate the clinical effects of fibula flap grafts on the repair of the extremities with traumatic compound tissue defects. METHODS: In 12 cases, the fibula flap grafts were employed to restore the extremities with traumatic compound tissue defects. Of the 12 patients, 9 were males, 3 were females; their ages ranged from 12 to 45. There were 2 cases of tibia defect combined with fibula fracture, 2 cases of tibia defect, 2 cases of radius defect, 3 cases of ulna defect, 1 case of calcaneus defect, and 2 cases of first-metatarsus defect. The bone defect length ranged from 4.2 to 10.6 cm, 7.8 cm in average. The skin defect area ranged from 10.0 cm x 4.5 cm to 27.0 cm x 15.0 cm. The free transplantation of fibular flaps were used in 9 cases, the lapse operation were used in 2 cases, retrograde shift were used in 1 case. RESULTS: Post-operational vein crisis and common peroneal nerve traction injury were observed in category mentioned above respectively. All the 12 fibula flaps survived after proper treatments such as removal of great saphenous vein. Follow-ups were done for 6 to 24 months. Both the transferred fibula and the recipient broken end reflected bones were healed. Four patients underwent the second-phase reconstruction operation of tendon moving power. One wrist and 1 ankle underwent arthrodesis in 3 to 6 months. All the effects were satisfactory. CONCLUSION: The fibula flap grafts provide a relatively better alternative to repair the extremities with long bone compound tissue defects. In addition, the sensory function reconstruction of fibula flaps should be given full attention.

Adolescent↗
PubMed1992

Morphologic and biochemical study of sternal cartilage autografts for resurfacing induced osteochondral defects in horses.

Using biodegradable pins, sternal cartilage autografts were fixed into osteochondral defects of the distal radial carpal bone in ten 2 to 3-year-old horses. The defects measured 1 cm2 at the surface and were 4 mm deep. Control osteochondral defects of contralateral carpi were not grafted. After confinement for 7 weeks, horses were walked 1 hour daily on a walker for an additional 9 weeks. Horses were euthanatized at 16 weeks. Half of the repair tissue was processed for histologic and histochemical (H&E and safranin-O fast green) examinations. The other half was used for the following biochemical analyses: type-I and type-II collagen contents, total glycosaminoglycan content, and galactosamine-to-glucosamine ratio. On histologic examination, the repair tissue in the grafted defects consisted of hyaline-like cartilage. Repair tissue in the nongrafted defects consisted of fibrocartilaginous tissue, with fibrous tissue in surface layers. On biochemical analysis, repair tissue of grafted defects was composed predominantly of type-II collagen; repair tissue of non-grafted defects was composed of type-I collagen. Total glycosaminoglycan content of repair tissue of grafted defects was similar to that of normal articular cartilage. Total glycosaminoglycan content of nongrafted defects was 62% of that of normal articular cartilage (P less than 0.05). Repair tissue of all defects was characterized by galactosamine-to-glucosamine ratio significantly (P less than 0.05) higher than that of normal articular cartilage. These results at 16 weeks after grafting indicate that sternal cartilage may potentially constitute a suitable substitute for articular cartilage in large osteochondral defects of horses.

Animals↗
PubMed1991

Defective neutrophil function in the autoimmune mouse strain MRL/lpr. Potential role of transforming growth factor-beta.

Patients with systemic autoimmune diseases such as SLE and rheumatoid arthritis have increased rates of morbidity and mortality caused by infection. Although this increased risk of infection has been primarily attributed to therapeutic immuno-suppression, some reports exist of defective polymorphonuclear leukocytes (PMN) function in these patients. The purpose of the present work is to investigate the recruitment of PMN phagocytic function in a murine model of autoimmunity, the MRL/lpr mouse. PMN from MRL/lpr, but not from congenic MRL/n mice, exhibit a marked defect in the amplification of FcR-mediated phagocytosis stimulated by various inflammatory mediators. This defect is acquired and correlates with the onset of the autoimmune disease observed in this strain. In addition, MRL/lpr but not MRL/n PMN exhibit a defect in extravasation into the thioglycollate-inflamed peritoneum. Incubation of MRL/n PMN in MRL/lpr serum induces a defect in the amplification of PMN phagocytic function identical to that observed with MRL/lpr PMN. The activity in the serum that induces this defect is neutralized by an antibody to TGF-beta but not by control antibodies. Incubation of murine and human PMN with purified TGF-beta induces an identical defect in stimulated FcR-mediated ingestion. In addition, TGF-beta-treated MRL/n PMN fail to extravasate into the thioglycollate-inflamed peritoneum after injection into normal MRL/n recipient mice. In addition, direct injection of TGF-beta into MRL/n mice also reduces the percentage and number of PMN in the thioglycollate-stimulated peritoneal exudates of these mice. The defect in PMN extravasation and phagocytic function was not caused by failure of the defective PMN to modulate the expression of the adhesion molecules, Mac-1 and Mel-14. These data indicate that defects in PMN function can be observed in a murine model of autoimmunity and that spontaneous production of TGF-beta possibly may play a crucial role in the pathogenesis of the defective PMN function in this animal model.

Age Factors↗
PubMed2002

[Congenital heart defects in Iceland 1990-1999.].

OBJECTIVE: About 1% of live born children have congenital heart defects (CHD). Knowledge of the true incidence of CHD is important because of the risk of bacterial endocarditis in patients with heart defects. This knowledge could also serve as a basis for research on the etiology of CHD. The aim of our study was to investigate the incidence of CHD in children born in Iceland during a ten year period, from 1990 to 1999. A similar study on CHD was carried out in Iceland for children born 1985-1989. The incidence of CHD in the present study was compared to the previous and to similar studies from other countries. MATERIAL AND METHODS: Information about the patients were obtained from medical records from two hospitals covering the whole country, a private clinic of pediatric cardiologists, echocardiography database, autopsy reports and death certificates. Distribution of specific heart defects, age at diagnosis, symptoms leading to the diagnosis, referral pattern, treatment and quality of life was investigated. Other congenital defects, chromosomal defects and syndromes in the patients were studied. Premature children with patent ductus arteriosus (PDA) and children with patent foramen ovale (PFO) or atrial septal defect (ASD) smaller than four millimeters were excluded. We also studied the incidence of bicuspid aortic valve (BAV). All diagnoses were confirmed with echocardiography and in some cases cardiac catheterization. RESULTS: Between 1990 and 1999 there were 44,013 live births in Iceland, 740 children were diagnosed with a CHD or 1.7% of live born children. Yearly incidence varied from 1.04% of live births in 1991 to 2.34% in 1997. Male/female ratio was 1/1. The distribution of the defects was following: ventricular septal defect (VSD) 338 (45.7%), ASD 90 (12.2%), PDA 85 (11.5%), valvar pulmonal stenosis 48 (6.5%), BAV 38 (5.1%), coarctation of the aorta 28 (3.8%), tetrology of Fallot 22 (3.0%), transposition of the great arteries 14 (1.9%), aortic stenosis 11 (1.5%), common atrioventricular septal defect 10 (1.4%), mitral valve regurgitation 9 (1.2%), sub-aortic stenosis 7 (0.9%) and hypoplastic left heart syndrome 5 (0.7%). Other defects were less frequent. About 47% of children with CHD were diagnosed either before birth or before discharge from the delivery institution. A cardiac murmur on examination was the most common symptom leading to the diagnosis of CHD, 631 patients (85.3%). Extracardiac anomalies were seen in 89 patients (12.0%). Chromosomal abnormalities were seen in 36 patients of whom 28 had Down's syndrome. The majority or 499 patients have no symptoms but are still in follow-up, 20 patients have daily symptoms and/or receive medical treatment and 27 patients have died. Conslusions: Annual incidence of patients diagnosed with a CHD has increased during the study period. This is observed in minor CHD but the incidence of major defects does not alter. This yearly incidence (1.7%) is higher than in the previous study, where it was 1.1%. The difference can partly be explained by the BAVs, which were excluded in the 1985-1989 study. But the number of CHD diagnosed each year has increased, this being most pronounced in the last three years. The annual incidence in our study is also higher than in other population studies. The most likely explanation for the higher incidence in our study is the fact that access to pediatric cardiologists is very good in Iceland. Diagnosis, registration and follow-up is conducted by only a few cardiologists for the whole nation and takes place at a single pediatric cardiology center. Of 740 patients diagnosed with CHD in the study period 713 are alive. The outcome of the therapy is good and the majority of the patients has no symptoms.

English Abstract↗
PubMed2006

[Repair of abdominal wall defect after resection of abdominal wall endometriosis].

OBJECTIVE: To study the techniques to repair the fascia layer of abdominal wall after the resection of abdominal wall endometriosis (AWE). METHODS: Fifty-five AWE patients aged 28 approximately 38 underwent resection of the lesion. After the resection a defect fascia in abdominal wall larger than 2 cm(2) was seen in 29 patients (large fascia defect group), and in the other 26 patients the fascia defect was less than 2 cm(2) (small fascia defect group). In the large fascia defect group, 11 cases underwent routine closure of the abdominal wall, 2 underwent abdominal wall reconstruction by applying tension suture, 1 case underwent fascia layer/skin tension-relieving suture, 4 cases abdominal wall reconstruction by PDS-II suture, 4 cases underwent fascia patch grafting, and 7 cases underwent abdominal wall plastic repair plus fascia patch grafting, the different techniques being selected according to the size of the defect. Routine abdominal wall closure was performed on all the 26 patients in the small fascia detect group. The features of the lesion and operation, and the outcomes were compared. RESULTS: Primary healing was achieved in all the patients. In comparison with the small fascia defect group, the mean size of the masses measured by pre-operational ultrasonography of the large fascia defect group was significantly bigger [(3.8 +/- 1.4) cm vs. (2.5 +/- 1.1 cm)], the mean size of the masses resected in operation was significantly larger [(5, 5 +/- 2.0) cm vs. (3.7 +/- 1.9) cm, P = 0.004], the operation time was significantly longer [(66 +/- 42) min vs. (35 +/- 24) min, P = 0.002], and the intra-operational blood loss was significantly more [(52 +/- 50) ml vs. (23 +/- 19) ml, P = 0.006]. Relapse occurred in 1 case in the large fascia defect group. CONCLUSION: Ultrasonography helps estimate the extension of AWE before operation. Fascia layer/skin tension-relieving suture can be used in the fascia defect of abdominal wall larger than 2 cm(2). Abdominal wall plastic repair plus fascia patch grafting is capable of repairing larger fascia layer and skin defects of abdominal wall.

Abdominal Wall↗
PubMedDate not supplied

Evaluation of the hospital discharge diagnoses index and the birth certificate as sources of information on birth defects.

The hospital discharge diagnoses index (DI) for newborns and the birth certificate were evaluated as sources of information about birth defects by comparing them with the same births in the case registry of the California Birth Defects Monitoring Program (CBDMP). The CBDMP is an active surveillance system; the staff visit hospitals to identify children with birth defects diagnosed in the first year of life. The study population comprised 66,481 live births to residents of five counties in the San Francisco Bay area in 1983. Of these infants, 2,543 had at least one birth defect noted on the DI, and 1,623 were in the CBDMP registry; 1,020 with defects noted on the DI were also in the CBDMP registry. For this same population, 399 infants had one or more defects noted on the birth certificate; 304 of these were also in the CBDMP registry. Reporting of birth defects on the birth certificate was poor for every condition. Reporting on the DI was most reliable for oral clefts and chromosomal defects; for these defects, the DI omitted one-third of the cases but had identified only about 10 percent false-positive (that is, unverified) cases. Major central nervous system malformations were less well reported, with about one-third of them false-positive. For all other birth defects, the DI either omitted more than half of the cases, or more than half of the cases reported were false-positive cases. These findings raise questions about the validity of analytic studies of birth defects if the data are obtained only from the DI or the birth certificate.

Birth Certificates↗
PubMed1990

Evaluation of birth defect histories obtained through maternal interviews.

Etiologic studies of birth defects often use family history information provided by parents of patients. The validity of this information has not been adequately assessed. Using data from the Atlanta Birth Defects Case-Control study, we evaluated sensitivity, specificity, and positive predictive value of mothers' responses regarding the presence of birth defects in their offspring. A total of 4929 mothers of infants with major structural defects ascertained by the Metropolitan Atlanta Congenital Defects Program and a total of 3,029 mothers of normal infants were asked whether their babies had had a birth defect or a health problem diagnosed during the first year of life. Interviewers and coders of maternal responses were blinded to the case-control status of infants. Sensitivity (the proportion of case mothers who gave responses that could be coded as denoting a major birth defect) was 61%. Specificity (the proportion of control mothers who gave responses that could not be coded as denoting a major birth defect) was 98%. The positive predictive value (the proportion of mothers who gave a major-birth-defect response who in fact had babies with major birth defects) was estimated as 47%. Sensitivity, specificity, and positive predictive value varied by maternal sociodemographic factors such as race and education, as well as by type of defect. These results suggest that family history data obtained through maternal interviews should be cautiously interpreted and, if not properly validated, may alter estimates of recurrence risks.

Case-Control Studies↗
PubMed1983

Methods of repair of noncircumferential bile duct defects.

Although noncircumferential bile duct defects are uncommon, they are important because they require careful repair to avoid subsequent biliary stricture. I have encountered three of these defects in more than 1000 biliary operations. The method of repair chosen for a particular case depends on the pathologic defect, the potency of the ampulla, and the tissues available for use. If the ampulla must be bypassed, a Roux-en-Y jejunal reconstruction is applicable for most biliary defects. An anastomosis between the bile duct and duodenum may be suitable to repair low biliary defects. Occasionally, the gallbladder can be used as a conduit between the bile duct defect and the duodenum or jejunum. If the ampulla need not be bypassed, a Heineke-Mikulicz repair is suitable only for very short defects. A patch technique is a better choice for larger defects. Patches can be made of autogenous vein, gallbladder, knitted Teflon, or a serosal onlay patch of duodenum or jejunum. A vein patch is especially appealing because it will easily cover any extrahepatic defect. A different method was chosen in each of three cases. Side-to-side Roux-en-Y hepaticojejunostomy was used to repair a large cholecystocholedochal fistula with associated pancreatitis. Heineke-Mikulicz repair was employed for a short hepatic duct stricture. Saphenous vein patch was used to repair a long bile duct defect during a left hepatectomy for hepatocellular carcinoma. This patient presumably represents the second successful reported vein patch repair and the only one with subsequent studies showing the fate of the vein patch. The vein patch apparently acts as a temporary scaffold allowing the outgrowth of biliary epithelium from the remaining bile duct wall. Careful initial repair of these noncircumferential bile duct defects is essential to avoid a subsequent biliary stricture with its disastrous consequences.

Bile Ducts↗
PubMedDate not supplied

[The classification of defects in the dental arches of children and orthodontic treatment methods].

Dentition defects occurring in children are classified into 6 classes with subclasses in some of them. Class I includes dentition with defects in the frontal part, class II includes dentitions with distal defects. Both classes include special subclasses for dentition with defects of deciduous teeth, of permanent teeth with underdeveloped roots, and of permanent teeth with mature roots. Non-distal dentition defects (class III) are divided into two subclasses: unilateral and bilateral. Class IV includes dentition with multiple defects in various age groups. Dentition defects combined with maxillofacial abnormalities, deformations, and defects are classified as class V. Primary hypo- and anodontia are referred to class VI. Indications to fitting with removable plate prostheses, permanent bridge prostheses, and prosthetic devices of various design to repair the defects of various localization, extent, and the degree of development of the maxillofacial area are defined. Special attention is paid to fixation of prostheses, the most intricate problem in prostheses of dentition defects in children. The suggested classification is practically important for the diagnosis and planning of orthodontic treatment of children with dentition defects.

Activator Appliances↗
PubMed1995

Blood protein defects associated with thrombosis. Laboratory assessment.

This review has stressed the common hereditary and acquired blood protein defects associated with thrombosis. The most common of the hereditary defects appear to be antithrombin, protein C, and protein S deficiency, and the most common acquired defects are anticardiolipin antibodies and the lupus anticoagulant. Therefore, these are the defects which should first be searched for in an individual with unexplained thrombosis. If these more common defects are not found, the rarer defects, including HC-II, plasminogen, or TPA deficiency, dysfibrinogenemia, elevated PAI-1, or heterozygous homocystinemia should be looked for. The incidence of activated protein C co-factor deficiency (APC resistance) is not yet clear but may also represent a common defect. PAI-1 defects may, with time, be shown to be common. Finding these defects has important implications for therapy for the individual patient and for the institution of family studies to identify, inform, and possibly treat others at risk. It is expected that as knowledge of hemostasis expands, more hereditary and acquired defects, such as elevated lipoprotein(a) or defects of extrinsic (tissue factor) pathway inhibitor (EPI, TFPI), may be associated with enhanced risks for thrombosis.

Blood Protein Disorders↗
PubMedDate not supplied

Bone formation and reosseointegration in peri-implantitis defects following surgical implantation of rhBMP-2.

This study was designed to evaluate bone formation and reosseointegration following surgical implantation of recombinant human bone morphogenetic protein-2 (rhBMP-2) in peri-implantitis defects. Hydroxyapatite-coated dental implants were placed bilaterally in the mandibular and maxillary premolar area in four rhesus monkeys and were allowed to osseointegrate for 1 year. Cotton ligatures were then placed around the healing abutments, and plaque was allowed to accumulate for 11 months. Resulting circumferential peri-implantitis defects exhibited a large intrabony and horizontal component. At reconstructive surgery, peri-implantitis defects in contralateral jaw quadrants were randomly assigned to receive rhBMP-2 (0.43 mg/mL implant volume) in an absorbable collagen sponge carrier or a carrier control. The animals were sacrificed 4 months postsurgery, and block sections were prepared for histometric analysis. Summary statistics included means calculated per animal. Paired t tests were used to evaluate differences between experimental conditions (n = 4). Defect depth amounted to 3.4 +/- 0.9 mm and 3.2 +/- 0.9 mm for rhBMP-2 and control defects, respectively. Vertical bone gain in rhBMP-2 defects (2.6 +/- 1.2 mm) was significantly greater than in controls (0.8 +/- 0.8 mm; P < .01). Reosseointegration within the confines of the defect for rhBMP-2 defects (29.0 +/- 10.5%) differed significantly from that in the control (3.5 +/- 2.5%; P < .01). Reosseointegration within the extent of newly formed bone averaged 40.0 +/- 11.0% in rhBMP-2 defects as compared to 8.9 +/- 7.8% in the control (P < .01). Osseointegration in resident bone amounted to 69.5 +/- 6.9% and 72.6 +/- 8.0% for rhBMP-2 and control defects, respectively. There is significant evidence that rhBMP-2 has potential to promote bone formation and reosseointegration in advanced peri-implantitis defects in a demanding nonhuman primate model.

Alveolar Bone Loss↗
PubMed1999

Multiple ventricular septal defects: how and when should they be repaired?

BACKGROUND: Congenital heart lesions with multiple ventricular septal defects remain a surgical challenge. Traditional approaches often rely on either ventriculotomy for exposure or palliation with pulmonary artery banding. However, indications for repair versus palliation and for various approaches to surgical exposure are not clearly defined. METHODS: From July 1992 to January 1998, 45 patients with multiple (>/=2) ventricular septal defects (37 with associated lesions) underwent surgery. Median age was 86 days; all but 4 patients were infants. The mean number of defects was 3.7, and almost half of the patients had more than 3 defects. Apical muscular defects were present in 62% of patients. Thirty-one patients underwent primary complete repair through a right atriotomy or trans-semilunar valve approach (group 1), 8 had palliation (group 2), and 6 underwent complete repair after prior palliation elsewhere (group 3). No patient had a ventriculotomy. RESULTS: One early death occurred in a group 1 patient. Four patients who had had palliation (50%) underwent early reoperation for pulmonary artery band revision because of failure to thrive or band removal after spontaneous closure of the defects. At follow-up (median 22 months), there was 1 death in a group 2 patient (palliation) and 1 other group 2 patient required cardiac transplantation. The only late reoperation was for removal of the pulmonary artery band and closure of multiple apical defects in a group 2 (palliation) patient. No patients who underwent repair have hemodynamically significant residual defects. CONCLUSIONS: In our experience, palliation of multiple ventricular septal defects is associated with greater morbidity than primary repair. Multiple defects can almost always be repaired adequately in early infancy without ventriculotomy, although "Swiss-cheese" septum may be an indication for palliation.

Child, Preschool↗
PubMed2006

Maternal occupational exposure and risk of specific birth defects.

BACKGROUND: Gregg identified the teratogenic effect of maternal rubella infection in 1941 and since then there has been a focus on risk factors for birth defects. In nearly 70% of all birth defects, there is still no known risk factor and close to 30% of all pregnancies end in a foetal loss or spontaneous abortion, often because of a defect in the foetus. A large percentage of the workforce consists of women of reproductive age. METHODS: A search in MEDLINE for original literature and examination of the association between exposure during pregnancy and the risk of birth defects. RESULTS: Five specific birth defects were identified: neural tube defects, cleft lip and cleft palate, congenital heart defects, urinary tract defects and limb defects. The next step was to include studies with information on occupational exposure during pregnancy. CONCLUSION: There seems to be growing concern as to whether organic solvents, including glycol ethers, pesticides and heavy metals may play a teratogenic role. There is no convincing evidence linking occupational exposure during pregnancy and birth defects.

Cleft Lip↗
PubMed1989

Increased prevalence of ventricular septal defect: epidemic or improved diagnosis.

The Baltimore-Washington Infant Study is an ongoing case-control study of congenital cardiovascular malformations in infants in whom the clinical diagnoses have been confirmed by echocardiography, catheterization, surgery, or autopsy. An increase in the prevalence of ventricular septal defects was detected in 1,494 infants with congenital cardiovascular malformations between 1981 and 1984. The prevalence of congenital cardiovascular malformations increased from 3.6 to 4.5 per 1,000 live births (P less than .025) and the prevalence of ventricular septal defect increased from 1.0 to 1.6 per 1,000 live births (P less than .001). The increase in ventricular septal defects accounted for the total increase in congenital cardiovascular malformations. The prevalence of isolated ventricular septal defect increased from 0.67 to 1.17 per 1,000 live births (P less than .001). The prevalence of ventricular septal defect with associated coarctation of the aorta, patent ductus arteriosus, atrial septal defect, and pulmonic stenosis did not change. The prevalence of ventricular septal defect diagnosed by catheterization, surgery, and autopsy did not change; however, defects diagnosed by echocardiography increased from 0.30 to 0.70 per 1,000 live births (P less than .001). It is concluded that the reported increase in prevalence of ventricular septal defect is due to improved detection of small, isolated ventricular septal defects and that there is no evidence of an "epidemic."

Cardiac Catheterization↗
PubMed1998

Thirty-year incidence of infective endocarditis after surgery for congenital heart defect.

CONTEXT: The incidence of infective endocarditis after surgical repair of congenital heart defects is unknown. OBJECTIVE: To determine the long-term incidence of endocarditis after repair of any of 12 congenital heart defects in childhood. DESIGN: Population-based registry started in 1982. SETTING: State of Oregon. PARTICIPANTS: All Oregon residents who underwent surgical repair for 1 of 12 major congenital defects at the age of 18 years or younger from 1958 to the present. MAIN OUTCOME MEASURE: Diagnosis of infective endocarditis confirmed by hospital or autopsy records. RESULTS: Follow-up data were obtained from 88% of this cohort of 3860 individuals through 1993. At 25 years after surgery, the cumulative incidence of infective endocarditis was 1.3% for tetralogy of Fallot, 2.7% for isolated ventricular septal defect, 3.5% for coarctation of the aorta, 13.3% for valvular aortic stenosis, and 2.8% for primum atrial septal defect. In the cohorts with shorter follow-up, at 20 years after surgery the cumulative incidence was 4.0% for dextrotransposition of the great arteries; at 10 years, the cumulative incidence was 1.1% for complete atrioventricular septal defect, 5.3% for pulmonary atresia with an intact ventricular septum, and 6.4% for pulmonary atresia with ventricular septal defect. No children with secundum atrial septal defect, patent ductus arteriosus, or pulmonic stenosis have had infective endocarditis after surgery. CONCLUSION: The continuing incidence of endocarditis after surgery for congenital heart defect, particularly valvular aortic stenosis, merits education about endocarditis prophylaxis for children and adults with repaired congenital heart defects.

Adolescent↗
PubMed1992

Can maternal risk factors influence the presence of major birth defects in infants with Down syndrome?

Although the manifestations of Down syndrome (DS) are well known, certain major birth defects such as duodenal atresia and endocardial cushion defects are present in some infants but not others, suggesting the possible role of other genetic or environmental factors interacting with the trisomy genotype. To explore the possible role of maternal factors in the presence of major defects among DS infants, we examined data from an epidemiologic study of DS conducted in metropolitan Atlanta. Of 219 DS infants born between 1968 and 1980, 50 had recorded cardiac defects, 9 had selected gastrointestinal atresias and 4 had oral clefts. We evaluated the association of these defects with several maternal factors including age, race, first trimester cigarette smoking, alcohol use, and fever. We found that different maternal factors were associated with several defects: (1) mother's race with cardiac defects (40% in blacks vs. 17% in whites, P less than 0.01), (2) mother's age with oral clefts (6% for less than 25 years, 1% for 25-34, and 0% for greater than 34, P less than 0.05), and (3) maternal first trimester fever with gastrointestinal defects (15% in infants with history of fever and 3% in infants without a history of fever, P less than 0.01). We also observed an inverse relationship between maternal alcohol use and the presence of ventricular septal defect. These findings suggest that maternal risk factors may influence the clinical manifestations of DS. In addition to searching for a genetic basis for the DS phenotype, we suggest that the role of environmental factors and maternal exposures be specifically explored in clarifying the genesis of various birth defects in Down syndrome.

Alcohol Drinking↗
PubMed1992

Dietary folate and nonneural midline birth defects: no evidence of an association from a case-control study in Western Australia.

In a population-based case-control study of dietary folate and neural tube defects, information was collected by interview and self-administered questionnaires from the mothers of cases with only neural tube defects, from the mothers of matched control infants with defects other than neural tube defects, and from the mothers of matched live-born infants with no birth defects. The association of midline birth defects (excluding neural tube defects) with dietary folate intake in the first 6 weeks of pregnancy was assessed by restricting the analysis to the 59 mothers of infants with midline defects in the first control group and comparing them with their matched control infants in the second control group. The crude and adjusted odds ratios for dietary folate and for folic acid supplementation were close to one, and all confidence intervals embraced unity. These data do not provide evidence of an association between midline birth defects (excluding neural tube defects) and either dietary folate or folic acid supplementation.

Australia↗