Simian-virus-40 footprints in human lymphoproliferative disorders of HIV- and HIV+ patients

BACKGROUND: In patients with spina bifida, traditional bowel management programs such as suppositories, retrograde enemas, and manual disimpaction have been largely unsatisfactory. The Malone antegrade continence enema (ACE) procedure has largely changed our approach to bowel management in this patient group. STUDY DESIGN: Over a 3-year period between January 1994 and January 1997, 27 patients with spina bifida underwent the Malone ACE procedure at our institutions. At the time of their ACE procedure, four patients underwent simultaneous continent urinary diversion and three had simultaneous small-bowel bladder augmentation. All the patients were evaluated for 9 months or more after their procedure, and 10 of the patients have been followed for more than 2 years. RESULTS: Postoperatively, predictable bowel control and continence were achieved in 19 of the 27 patients, but 6 had some rectal soiling requiring a sanitary pad. All patients were out of diapers and none reported stomal leakage. Eighteen of the 27 patients were able to manage independently and 9 required assistance. Two patients had stopped using their ACE stoma despite good technical results. The appendix was used as a catheterizable stoma in 15 of the 27 patients. The appendix was not available in 12 patients, so a tubularized cecal flap was used in 9 and a small-bowel neoappendix was created in 3. Complications included stomal stenosis in 5 patients, cecal-flap necrosis in 1, and stomal granulations in 3. CONCLUSIONS: We believe that the ACE procedure provides reliable colonic emptying and avoids fecal soiling in the majority of individuals, and we find it widely and enthusiastically accepted by patients with spina bifida.     

National Transplantation Pregnancy Registry: analysis of pregnancy outcomes in female liver transplant recipients

Outcomes from 48 pregnancies in 34 female liver transplant recipients were analyzed. Data were collected via interviews, questionnaires, and hospital records. All recipients were treated with cyclosporine-based immunosuppression except 2 patients treated with FK506 and 2 treated with no immunosuppression. The age at conception was 26.1 +/- 5.9 years (mean +/- SD) with a transplant interval (time from transplantation to conception) of 2.9 +/- 2.5 years. There were 49 outcomes (1 set of twins): miscarriage 9 (18%), therapeutic abortion 4 (8%), and live birth 36 (74%). No stillbirths or ectopic pregnancies were reported. Of the 36 live births, the gestational age was 36.9 +/- 3.5 weeks, the birthweight was 2,604 +/- 698 grams, 39% were premature (< 37 weeks), and 31% had low birthweight (< 2,500 grams). No birth defects or neonatal deaths (< 28 days) were reported. The newborn complication rate was 17% (n = 6), 5% in premature infants. The incidence of drug-treated hypertension was 46%; pre-eclampsia 21%; infectious complications 26%; and Caesarean section 47%. Recipients with hypertension had a higher proportion of premature infants (71%) than normotensive patients (38%) (P = .04 by Fisher's exact test). Acute rejection was diagnosed in 6 pregnancies, 2 of which were ended by therapeutic abortion. Four recipients who continued their pregnancies were treated with increased immunosuppression for rejection, and all delivered livebirths. There were two grafts lost within 6 months of pregnancy. The only maternal death occurred in a patient who required retransplantation for recurrent C hepatitis 3 months afte therapeutic abortion and died 6 months later. The other recipient with graft loss was successfully retransplanted for chronic rejection 6 months after delivery. We draw the following conclusions: (1) female liver transplant recipients can safely undergo pregnancy, although there is a high rate of premature and low birthweight infants; (2) pregnancies in this population should be considered high-risk and require close monitoring of liver function; and (3) altered graft function during pregnancy should be thoroughly investigated.     

Myotonic dystrophy kinase modulates skeletal muscle but not cardiac voltage-gated sodium channels

PURPOSE: To evaluate the technique used for and long-term results of percutaneous cecostomy tube placement for the treatment of fecal incontinence in children. MATERIALS AND METHODS: After an initial pilot study in 15 patients, 42 additional patients with fecal incontinence aged 2-20 (mean, 11.5) years and weighing 9.9-109.0 (mean, 39.2) kg underwent percutaneous cecostomy tube placement. Twenty-nine patients had spina bifida, nine had imperforate anus, three had cloacal anomalies, and one had Hirschsprung disease. Mean follow-up was 265 days (range, 8-503 days). RESULTS: Tube placement was successful in all patients. One patient developed local inflammation after accidental early retention-suture removal, which was treated with suture replacement and intravenous antibiotics. Another developed postprocedural ileus, which resolved. Late complications included constipation in one patient (treated with diet alteration), granulation tissue in seven patients (treated with silver nitrate cautery), and accidentally dislodged tubes in three patients (two successfully replaced at home and one replaced at the radiology suite). Vomiting related to the phosphate enema occurred in two patients. Resolution of soiling was achieved in all patients. CONCLUSION: Percutaneous cecostomy and antegrade enemas are very successful in achieving fecal continence and patient independence and acceptability, with minimal early and late complications.     

Oocyte donation to women of advanced reproductive age: pregnancy results and obstetrical outcomes in patients 45 years and older

We analysed the results of oocyte donation to women of advanced reproductive age (> or = 45 years old) and followed their pregnancies through to delivery in order to assess obstetrical outcomes. Patients (n = 162) aged 45-59 years (mean +/- SD; 47.3 +/- 3.4 years) underwent 218 consecutive attempts to achieve pregnancy. Oocytes (16.2 +/- 7.2 per retrieval) were provided by donors < or = 35 years old. Cleaving embryos (8.2 +/- 4.8 zygotes/couple) were transferred transcervically (4.5 +/- 1.1 per embryo transfer) to recipients prescribed oral micronized oestradiol and intramuscular progesterone. Following oocyte aspiration there were six instances of non-fertilization (2.8%) and 212 embryo transfers. A total of 103 pregnancies was established for an overall pregnancy rate (PR) of 48.6%, which included 17 preclinical pregnancies, 12 spontaneous abortions, and 74 delivered pregnancies (clinical PR 40.6%; delivered PR 34.9%). Multiple gestations were frequent (n = 29; 39.2% of pregnancies) and included 20 twins, seven triplets, and two quadruplets. Two of the triplet and both of the quadruplet pregnancies underwent selective reduction to twins. Antenatal complications occurred in 28 women (37.8% of deliveries) and included preterm labour (n = 9), gestational hypertension (n = 8), gestational diabetes (n = 6), carpel tunnel syndrome (n = 2), pre-eclampsia (n = 2), HELLP syndrome (n = 2), and fetal growth retardation (n = 2). 48 (64.8%) deliveries were by Caesarean section. The gestational age at delivery for singletons was 38.3 +/- 1.3 weeks (range 35-41 weeks), with birth weight 3218 +/- 513 g (range 1870-4775 g); twins 35.9 +/- 2.0 weeks (range 32-39 weeks), birth weight 2558 +/- 497 g (range 1700-3450 g); and triplets 33.5 +/- 0.7 weeks (range 32-34 weeks), birth weight 1775 +/- 190 g (range 1550-2100 g). Neonatal complications (4.6% of babies born) included growth retardation (n = 2), trisomy 21 (n = 1), ventricular septal defect (n = 1), and small bowel obstruction (n = 1). There were no maternal or neonatal deaths. We conclude that oocyte donation to women of advanced reproductive age is highly successful in establishing pregnancy. However, despite careful antenatal screening, obstetrical complications are common, often secondary to multiple gestation.     

Pregnancy in patients with pulmonary autograft valve replacement

AIM: The purpose of this study is to determine the outcome and complications of pregnancy in women with pulmonary autograft valve replacement for aortic valve disease. METHODS AND RESULTS: The records of all women who had undergone pulmonary autograft valve replacement at the National Heart Hospital (now Royal Brompton Hospital) since 1968 were reviewed. From 1968 to 1993, 27 hospital survivors were female and among eight of them there were 14 pregnancies. All women were in Ability Index 1 at time of pregnancy with normal ventricular function, mild aortic regurgitation (six), mild pulmonary regurgitation (three) and mild pulmonary stenosis (two). None took anticoagulants. There was no maternal death, thromboembolic or haemorrhagic event or evidence of deterioration in valve function during pregnancy. Except for one woman (Ability Index 3) who developed dilated cardiomyopathy without aortic or pulmonary valve disease 6 months after delivery, the women remained in Ability Index 1 after pregnancy. There was no significant progression of aortic regurgitation (mild after seven pregnancies), pulmonary regurgitation (mild after six) or right-sided obstruction (mild after four). Reoperation for right-sided obstruction was carried out in two patients 4 and 7 years after a second pregnancy (9 and 15 years after the pulmonary autograft). CONCLUSION: No valve-related complications occurred during pregnancy and pregnancy appeared to have no effect on the function of the pulmonary valve autograft or the right-sided homograft. The pulmonary autograft is thus an ideal procedure for a young female needing aortic valve replacement.     

The Malone antegrade colonic enema enhances the quality of life in children undergoing urological incontinence procedures

PURPOSE: Functional alterations of the gastrointestinal and genitourinary tracts, and physical limitations in children with spina bifida, imperforate anus and spinal cord injury challenge the ability to have independent fecal and urinary continence. Urologists have successfully helped these patients achieve urinary continence. We report our experience with the antegrade colonic enema procedure, which allows select individuals to achieve continence of stool, enhancing quality of life. MATERIALS AND METHODS: Since December 1992, 18 antegrade colonic enema procedures were performed in 12 female and 6 male patients 5 to 31 years old of whom 14 had spina bifida, 2 had imperforate anus and 2 had spinal cord injury. Simultaneous urological continence procedures were performed in 8 patients, including appendicovesicostomy in 4, augmentation cystoplasty in 2 and augmentation cystoplasty plus an ileal Mitrofanoff procedure in 2. Four patients previously underwent urological reconstruction. RESULTS: In 24 months of followup (average 6.6) all patients with a functioning stoma remained continent of stool and 17 were continent of urine. Complications related to the antegrade colonic enema procedure occurred in 4 children (22%) of whom 3 required further surgery. Three patients (17%) had minor stomal stenosis. CONCLUSIONS: The antegrade colonic enema procedure is easily performed and it should be considered for any child with significant physical limitations and/or refractory fecal incontinence before urological continence promoting procedures are done.     

Individual, family, and peer factors associated with the quality of sibling relationships in families of youths with spina bifida.

Based on a social-ecological theoretical framework, this study sought to identify key individual (attitude toward spina bifida), family (satisfaction with family functioning), and peer (peer support) factors associated with the quality of sibling relationships in families of youths with spina bifida. A cross-sectional sample of 224 siblings of youths with spina bifida, ages 11-18 years, and parents participated. Siblings completed self-report measures of attitude toward spina bifida, satisfaction with family functioning, peer support, and warmth and conflict in their relationships with brothers and sisters with spina bifida. The parent provided information on spina bifida severity and family demographics. Hierarchical regression analyses supported the overall model in explaining variance in sibling-reported relational warmth (Adjusted R2 = .29) and conflict (Adjusted R2 = .17). Satisfaction with family functioning was the only ecological factor consistently associated with warmth and conflict in the sibling relationship. Findings have clinical implications for pathways to support positive sibling interactions in families of youths with spina bifida. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

The incidence of spina bifida occulta in idiopathic scoliosis

A review of the roentgenograms of the spine of 100 patients with idiopathic scoliosis and 371 parents and siblings used as controls, showed an incidence of spina bifida occulta of 34 per cent and 41 per cent in their siblings. There was no increase in the incidence of spina bifida occulta in the patients with idiopathic scoliosis as compared with the control group. A review of the parents of these patients showed an incidence of spina bifida occulta of the lumbosacral area of 9 per cent in the mothers, and 13 per cent in the fathers, thus showing a decrease in the incidence of spina bifida occulta in the adult as compared with the teenager. The studies in the adult confirm the finding in the teenagers. There is no increased incidence of spina bifida occulta in idiopathic scoliosis.     

Maternal serum screening for Down syndrome and neural tube defects

OBJECTIVE: Evaluation of maternal serum screening for Down's syndrome (DS) and neural tube defects (NTDs). DESIGN: Longitudinal study. SETTING: Department of Obstetrics and Gynaecology, University Hospital Utrecht, the Netherlands. METHOD: 6362 pregnant women underwent serum screening for DS and (or) NTD between the 15th and 21st weeks of pregnancy between March 1991 and March 1996. Screening was performed using alpha-foetoprotein, unconjugated oestriol, human chorionic gonadotrophin and maternal age. The result of each individual test was a calculated risk for delivering a child with DS and (or) NTD. RESULTS: Nine out of 12 singleton pregnancies of a foetus with DS were detected. To this purpose, 573 women who, according to the serum screening had an increased risk of a child with the abnormality, were offered amniocentesis, which was performed in 471 of them. Two twin pregnancies with a total of 3 DS affected foetuses were also detected; one twin pregnancy of a DS foetus was screen-negative. The one case of spina bifida was screen-positive. The proportion of women eligible for invasive prenatal diagnosis because of maternal age increased from 9% to 25% in the course of the study. Of 1118 women aged > or = 36 years 913 (82%) declined invasive investigation compared with 40% in the general population. CONCLUSION: The results of the maternal serum screening program in Utrecht were comparable with other studies. Maternal serum screening is accepted as an alternative by women above 36 years, and allows to decrease the need for amniocentesis without a significant loss in detection rate.     

Delivery of the overweight woman. Analysis of 115 patients

OBJECTIVE: Our purpose was to assess the effect of weight gain during pregnancy on labor and delivery. Abnormal pregnancies were excluded from the study to avoid interaction with the management of labor. POPULATION AND METHODS: A retrospective monocentric case-control study was carried out. We analysed 115 pregnancies delivered in our institution between June 1994 and November 1994. The course of labor was studied in 2 groups of patients: a group of overweight patients and a control group. RESULTS: In the overweight patients, the frequency of induction of labor (25%) and of obstetrical analgesia (82%) was significantly higher than in the control group (respectively 7% and 64%), as well as the average duration of labour and the average duration of the rupture of the membranes. The rate of cesarean section was higher in the overweight patient group (16.7%) than in the control group (3.7%). The difference was not significant because of the small number of patients. CONCLUSION: These results show a more frequent requirement of induction of labour, analgesia, and cesarean section in overweight patients. This led us to propose a multidisciplinary management of overweight patients to minimize these different obstetrical risks during labor.     

The impact of family, peer, and school contexts on depressive symptoms in adolescents with spina bifida.

Objective: Based on social ecological theory, this study examined the joint relations among adolescents' family, peer, and school contexts and depressive symptoms in youth with spina bifida using cumulative, protective, and specific effects models. Method: Sixty families of adolescents with spina bifida and 65 comparison families reported on adolescents' positive experiences within these contexts and on depressive symptoms when youth were 14–15 and 16–17 years old. Results: Adolescents with spina bifida had fewer total positive contexts and less positive experience within peer and school contexts, as compared to typically developing adolescents. Greater total number of positive contexts and higher levels of positive experiences within family and school contexts were associated with fewer depressive symptoms for both groups; peer positive experiences were related to lower depressive symptoms for typically developing adolescents only. Conclusion: Adolescents with spina bifida have fewer positive contexts, which may place them at risk for higher levels of depressive symptoms. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Dorsal hemivertebrae in children's lumbar spines

STUDY DESIGN: Only small numbers patients with dorsal hemivertebrae have been reported. The natural course of this disease remains unpredictable, especially regarding the development of neurologic impairment. Two children with congenital dorsal hemivertebra with progressive kyphosis of the lumbar spine are presented. Onset and method of treatment are discussed. OBJECTIVES: Two boys with kyphosis due to dorsal hemivertebrae of the lumbar spine were followed with clinical examination, anteroposterior roentgenograms, and magnetic resonance imaging. One patient was in follow-up for more than 4 years postoperatively. The other patient, who has not undergone surgery, has been in follow-up for more than 2 years, undergoing reassessment every 4 to 6 months. SUMMARY OF BACKGROUND DATA: Spastic paralysis developed in one patient with dorsal hemivertebra and spina bifida. The other patient with dorsal hemivertebra and sacral agenesis had no neurologic deficit. METHODS: A dorsal approach with resection of the dorsal hemivertebra and short dorsal fusion with internal fixation was done in one patient. The other patient underwent clinical and radiologic follow-up. Method and onset of surgical treatment were compared with other studies. RESULTS: Progressive spasticity was seen in our first patient. This could be reversed by resection of the hemivertebra and monosegmental fusion. Alignment of the spine and normalization of the width of spinal canal were achieved. The second patient has not had a neurologic problem. Surgical treatment will be performed when indicated. CONCLUSIONS: A posterior approach is recommended for a dorsal hemivertebra. Decompression of the spinal canal can be achieved by resection of the dorsal hemivertebra and short dorsal fusion with internal fixation. Surgery should be done early to avoid late neurologic impairment.     

Granulocytic sarcoma as isolated extramedullary relapse after donor lymphocyte infusion in a patient with CML who relapsed after allogeneic bone marrow transplantation: a case report

Nineteen patients with spina bifida and bilateral dislocation of the hips were studied with a minimal follow-up of 10 years. The average age at review was 21 years (range, 10-31). Ten patients had an upper neurologic level (thoracic to L3), and nine had a low lesion (L4 to sacrum). Three patients had no hip surgery. A closed or open reduction was performed in 12 hips, supplemented by one or more surgical procedures. Of these, 10 remained enlocated, and two had redislocated. In all other hips, several surgical procedures were performed, aimed at improvement of hip-flexion deformity or stability. At follow-up, one patient had occasional pain in one hip, which was dysplastic. Of the 10 patients with a high lesion, only two were walkers, but both had bilateral dislocation of the hips at follow-up. In contrast, all nine patients were walkers, but four of these had bilateral dislocation at follow-up. We found that a level pelvis and good range of motion of the hips are more important for ambulation than is reduction of bilateral hip dislocation.     

Two cases of pulmonary dirofilariasis in Nagasaki Prefecture

BUT: Assess risk factors and pathogenesis of myocardial infarction in young women and evaluate cardiologic and obstetrical management of myocardial infarction during pregnancy as well as assess the prognosis of pregnancy in patients with a prior myocardial infarction. METHODS: Fra a personal observation of pregnancy after myocardial infarction, we reviewed the literature on two distinct themes: myocardial infarction during pregnancy and pregnancy after myocardial infarction. RESULTS: Pregnancy was deliberately authorized in a 21-year-old who had myocardial infarction 3 years earlier. The pregnancy was uneventful and cesarean section with epirual analgesia at 37 weeks gestation delivered a healthy 2,350 g boy. Unfortunately the patient died at her home 18 month later. Acardiac cause was retained although death due to ventricular rhythm disorder or recurrent myocardial infarction could not be confirmed due to lack of an autopsy. The prognosis of pregnancies after myocardial infarction would not appear to be catastrophic although this case raises some doubts as to whether pregnancy should be authorized in these patients. The major difference in mortality according to whether the infarction occurs before or during pregnancy would suggest that risk might be reduced by a haling period after the infarction. CONCLUSION: It appears that pregnancy in women with a prior myocardial infarction would be possible if sufficient precautions are taken. All situations increasing cardiac work should be avoided. Stress and pain must be avoided during delivery and the post-partum period. Intensive cardiological and obstetrical supervision supervision are required for correct management of the pregnancy.     

Comparative clinical and biochemical analyses of two methods of pancreatic cannulation in pigs

Over the last 16 years the evolution of 24 pregnancies in 17 women with biopsy-proven glomerular disease was analyzed. The underlying renal histology was IgA nephropathy in 8 cases, lupus nephritis in 7, mesangiocapillary glomerulonephritis type I in 1, and focal segmental glomerulosclerosis in 1. All but 2 had normal renal function before conception and 3 were hypertensive. Fetal survival rate was 75%. There were 6 preterm deliveries (33.3%), 3 newborns small for gestational age (17%), 1 stillbirth, and 5 therapeutic abortions. The perinatal mortality was 5.5%. De novo hypertension occurred in 8 pregnancies (33.3%). In 11 pregnancies (46%) increased proteinuria was diagnosed and in 6 (25%) a decline in maternal renal function was recorded. Permanent impairment of renal function was seen in 2 women with renal insufficiency before conception. Maternal hypertension and renal function impairment were associated more frequently with obstetric complications. In conclusion, pregnancy is safe for normotensive mothers with glomerular diseases and normal renal function. Hypertension and impaired renal function at conception seem to carry increased risk for mothers and fetuses. Low-dose immunosuppressive treatment during pregnancy is not harmful for the fetus.     

Trajectories of autonomy development across the adolescent transition in children with spina bifida.

Objective: The current study investigated individual growth in autonomy development across the adolescent transition, comparing the trajectories of children with and without spina bifida. Method: Individual growth curve modeling procedures were utilized to describe the developmental course of autonomy across four waves of data collection, from ages 9 to 15, and to test whether illness status [spina bifida vs. matched comparison group (N = 68 for both groups at Time 1)] would significantly predict individual variability in autonomy development. Potential moderators [child gender, SES, and Peabody Picture Vocabulary Test (PPVT) score] of the association between illness status and autonomy development were also examined. Results: Children with spina bifida demonstrated distinct developmental trajectories, though the nature of the group differences varied by type of autonomy development (emotional vs. behavioral), context (i.e. school vs. family), and reporter. Significant interactions with PPVT score and child gender were found. Conclusion: Overall, children with spina bifida show considerable developmental resiliency, but may lag behind their peers in specific areas of autonomy. Boys with spina bifida, and children with spina bifida who have lower than average levels of verbal intelligence, appear to be at greater risk for exhibiting delays in autonomy development. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Thrombophilia, anticoagulant therapy and pregnancy

Thromboembolic complications during pregnancy are the most common causes of maternal death. Here we report on thromboembolic prophylaxis of 60 pregnancies of 32 pregnant women with familial thrombophilia. Long-term Fraxiparine (Sanofi-Chinoin) as thromboprophylaxis was applied in 26 cases throughout pregnancy. UFH (Heparin-Ca inj.) was used in 11 cases, and there were 23 pregnancies without thromboembolic prophylaxis in our patient's case histories. Artificial abortions were not included in this paper. The ratio of successful pregnancies were: with Fraxiparine: 24/26 (92.3%), with UFH (Heparin-Ca): 8/11 (72.7%), without prophylaxis: 4/23 (17.4%). In the patient group treated with Fraxiparine there were no foetopathy, thrombocytopenia or bleeding complication. LMWH is recommended for pregnant women with familial thrombophilia. According to literature data and our own experiences the doses of LMWH in patients with familial thrombophilia, and -antiphospholipid syndrome, and -artificial heart value are suggested.     

Trajectories of family processes across the adolescent transition in youth with spina bifida.

The current study investigated change in family processes, including conflict, cohesion, and stress, across the adolescent transition, comparing the developmental trajectories of youth with and without spina bifida. Individual growth curve modeling procedures were utilized to describe the developmental course of family processes across 4 waves of data collection, from ages 9 to 15 years, and to test whether illness status (spina bifida vs. matched comparison group [N = 68 for both groups at Time 1]) would significantly predict individual variability in family processes. Potential moderators (child gender, socioeconomic status [SES], and child verbal ability) of the association between illness status and family functioning were also examined. Differences were found between the trajectories of family processes for families of youth with and without spina bifida. For families of youth with spina bifida, changes in family conflict and cohesion may be less dramatic than or inconsistent with what is expected during typical adolescence. Families of youth with spina bifida from low SES homes appear to demonstrate resilience in terms of family stress. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Three-dimensional gait analysis in spina bifida

This study was designed to determine gait patterns in children with lumbar and sacral neurologic level spina bifida. We studied a group of 28 children: 10 had L4-level lesions and a mean age of 11 years; eight had L5-level lesions and a mean age of 8 years; and 10 had S1-level lesions with a mean age of 12 years. A group of 15 normal children, mean age 10 years, was used for comparison. Each child underwent three-dimensional gait analysis using the Vicon system. We found that there were recognisable gait patterns for each level of spina bifida and that the abnormalities accurately reflected the muscle deficiencies present. The gait patterns approximated more closely to those of the normal group as the neurological level descended. The most important findings were of increased pelvic obliquity and rotation with hip abduction in stance (reflecting the gross Trendelenburg-type gait seen in these children) and persistent knee flexion throughout stance as a result of the absence of the plantar flexion-knee extension couple. We found that gait was not improved by tendon transfers performed either at the hip (posterolateral psoas transfer) or at the ankle (tibialis anterior transfer).