Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Fifty infants ranging in age from 13 days to 18 months (mean age 6 months) and weighing from 1.7 to 8.2 kg (mean weight 4.5 kg) underwent patch closure of a ventricular septal defect (VSD) with use of deep hypothermic circulatory arrest. Seventeen infants were under 3 months of age. The principal indication for operation was intractable chronic congestive heart failure; All infants were below the third percentile for weight preoperatively; Three patients (6%) died postoperatively within the second month of life. There was no late mortality. Seven infants (14%) had seizures; these were associated with a low output state in 2 infants, with hypoxic episodes in 4 infants, and occurred postoperatively in 1 infant. Postoperatively, 8 (17%) of the surviving infants developed right bundle-branch block and left anterior hemiblock, and 16 (32%) developed right bundle-branch block alone. One year postoperatively, catheterization studies in 24 children revealed normal pulmonary artery pressure and pulmonary vascular resistance in all; there were no significant residual ventricular septal defects. Because of these results we continue to be enthusiastic about primary closure of VSD irrespective of age or weightk0
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PMID:Early and late results of closure of ventricular septal defect in infancy. 87 75

The long-term prognosis of 185 children with epilepsy, who continued to attend the Clinic for Epileptic Children, the Department of Pediatrics, the University of Tokyo, beyond the age of 18 years, was reported. The length of follow-up varied from three to 20 years, but most of them were followed longer than 10 years. The presumed etiology in these children was divided into a cryptogenic group (124, 67.0%) and a symptomatic group (61). The types of seizures were classified into grand mal (86 cases), focal seizure (27), petit mal absence (4), psychomotro seizure (5), infantile spasms (7), and so on. It may be noted that the highest frequency of grand mal was demonstrated, while the incidences of infantile spasms, myoclonic seizure, and akinetic seizure were low in the series. Only 28 children (15.1%) had complications of physical and/or mental handicaps. The follow-up study revealed that 140 patients (75.7%) had been seizure-free in the last 12 months. One hundred and fifteen of them had no seizures for five years or longer. On the other hand, electroencephalographic abnormalities generally continued for a long time after disappearance of seizures. Eightly-one of well-controlled patients were gradually decreasing the doses of anticonvulsants. As for seizure types, it is noted that focal seizure, psychomotor seizure, and infantile spasms were relatively difficult to be controlled. Except for 27 patients, most of them attended normal schools, including junior colleges or universities, and engaged in various occupations. Fifteen female patients had already married, and out of 13 babies who were born from these patients, there were one with ventricular septal defect, one with mental deficiency, and one with anencephaly, while the rest were entirely normal. Additional problems on withdrawal of anticonvulsants after a long-term seizure-free period, and what a medical system should be for treatment of epilepsy in children up to their adulthood were discussed.
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PMID:Long-term prognosis of epilepsy in children--a follow-up report beyond 18 years of age. 99 13

In a prospective study, 30 pregnant epileptic patients were followed through their pregnancy to determine the effect of seizures on pregnancy and its outcome and the effect of pregnancy on seizures. An attempt was made to correlate the serum hormone and anticonvulsant drug levels with seizure frequency, complications of pregnancy, occurrence of status epilepticus and teratogenicity. In 14 patients seizure frequency increased, in 15 it remained unchanged and in 1 patient it decreased. There were 2 spontaneous abortions, 2 patients had status epilepticus and 1 offspring of a patient had a ventricular septal defect. This patient was receiving carbamazepine and diphenylhydantoin. Patients with increased seizures frequency had significantly higher oestrogen levels, lower level of progesterone and lower level of anticonvulsant drugs as compared with those with no change in seizures. Patients who had abortions and those who developed status epilepticus had high serum oestrogen levels.
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PMID:Pregnancy and epilepsy. 276 Jun 52

The case of a 5-month-old black female child with a linear sebaceous naevus syndrome and multiple congenital anomalies is presented. Ocular malformations consisted of colobomatous changes of the lid and retina, dermoid of the conjunctiva, chorioretinal changes, and peripapillary atrophy of the optic nerve. Systemic findings included midline cleft of the secondary palate with involvement by the naevus, bilateral hearing loss, asymmetrical skull bones, ventricular septal defect, epidermal inclusion cyst, and developmental delay without seizures.
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PMID:Ocular findings in linear sebaceous naevus syndrome. 358 Mar 38

This report summarizes the spectrum of clinical and immunologic findings gathered prospectively in 13 patients with the DiGeorge syndrome. Our patients demonstrated marked variability in both the clinical manifestations and the degree of immunodeficiency, confirming the findings of earlier individual case reports and retrospective autopsy reviews. Ages at the time of presentation ranged from one day to 4 months. Congenital heart defects including truncus arteriosus, ventricular septal defect, interrupted aortic arch, and tetralogy of Fallot commonly brought these infants to medical attention within the first two weeks of life. Abnormal calcium homeostasis was found in all patients. Those patients presenting after the first month of life often had hypocalcemic seizures as the initial clinical manifestation. Parathyroid hormone levels and the number and location of parathyroid glands varied considerably. Immunologic evaluation revealed that total lymphocyte counts, percent T-cells, total T-cells, and T-lymphocyte function ranged from normal to severely depressed. The most consistent immunologic abnormality, found in 11 of the 13 patients, was a decrease in total T-cells. Sequential studies in five patients demonstrate that spontaneous resolution of immunodeficiency may occur in some, yet progressive loss of immune function may be observed in others. Complete immunologic evaluation and careful followup is mandatory in infants with persistent hypocalcemia and congenital heart disease who are suspected to have DiGeorge syndrome.
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PMID:Clinical and immunologic spectrum of the DiGeorge syndrome. 697 33

The acronym DOOR was first used by Cantwell in 1975 to describe a syndrome comprising sensorineural deafness, osteodystrophy, onychodystrophy, and mental retardation. To date, 16 cases of the syndrome have been documented in the literature. We present two sisters who died in early infancy with the clinical features of DOOR syndrome, both of whom in addition had cardiac defects and urinary tract abnormalities. Both infants had the classical clinical features of sensorineural deafness, seizures, hypoplastic nails, finger-like thumbs, and the characteristic facies of the syndrome. Autopsy in each case revealed the additional findings of a membranous ventricular septal defect and a septum secundum atrial septal defect. The first child had left-sided hydronephrosis and hydroureter, and the second sibling had bilateral hydronephrosis, hydroureter, and dilatation of the bladder. Congenital heart disease and renal abnormalities have not to our knowledge been previously described in association with the DOOR syndrome.
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PMID:Congenital heart disease and urinary tract abnormalities in two siblings with DOOR syndrome. 780 78

Many infants with cardiac anomalies undergo repair early in life. Both commonly used support techniques, deep hypothermic circulatory arrest (DHCA) and low-flow cardiopulmonary bypass (LFB), may be associated with adverse neurological outcomes, including seizures. In a single center study, 171 infants undergoing correction for D-transposition of the great arteries were randomized to one of these support techniques. Incidence and onset times of EEG seizures during continuous EEG-video monitoring in the first 48 h postoperatively and clinical seizures in the first postoperative week were compared. EEG seizures were characterized by time, duration, and localization of onset. Incidence of EEG seizures (20%) was more than 3 times that of clinical seizures (6%). Most infants with EEG seizures had multiple seizures beginning between 13 and 36 h postoperatively. Durations ranged from 6 s to 980 min. Onset of EEG seizures occurred most commonly in frontal and central regions. Factors associated with EEG seizures included randomization to DHCA, longer duration of circulatory arrest, and diagnosis of VSD. In this study EEG seizures were common following this type of cardiac surgery, illustrating the importance of EEG monitoring in detecting seizures. This data adds insight into mechanisms of seizures in infants undergoing cardiac surgery.
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PMID:Perioperative electroencephalographic seizures in infants undergoing repair of complex congenital cardiac defects. 906 Aug 52

E-type prostaglandins (PGE1) can effectively maintain the patency of the ductus arteriosus in neonates. Its use, therefore can be life saving in infants born with ductus dependent congenital heart disease. Although PGE1 is available for over two decades in western world, it has been introduced in India only since April, 1995. Various cardiac defects where PGE1 is useful include (a) lesions with ductus dependent pulmonary blood flow e.g. pulmonary atresia with or without ventricular septal defect, critical valvular pulmonic stenosis etc, (b) lesions with ductus dependent systemic blood flow e.g. critical aortic stenosis, coarctation of aorta, interruption of aortic arch etc, and (c) admixture lesions like transposition of great arteries. The drug is given as a continuous intravenous infusion. The initial dose is 0.05 to 0.4 ug/kg/min, infusion rate must be decreased to 0.01 ug/kg/min as soon as the desired effect is achieved as incidence of side effects is more at higher doses. Serious side effects include apnoea, hypotension, hyperthermia, seizures etc. We have used this drug in 43 infants ranging in age from one to forty five days. Beneficial response was seen in 41 of 43 infants and the major side effect was apnoea (seen in 5 of 32 spontaneously breathing infants). Unfortunately the high cost of the drug prohibits its wide spread and long term use. PGE1 is a life saving drug for infants born with ductus dependent congenital cardiac malformations. It helps in stabilizing these patients prior to further surgical palliation or correction.
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PMID:Prostaglandin E1: first stage palliation in neonates with congenital cardiac defects. 1077 66

We performed molecular analysis of a germline interstitial deletion of chromosome 4 [del(4)(q21.22q23)], which had been observed in a male infant manifesting early-onset hepatoblastoma (HBL). The chromosomal anomaly in this child was associated with a unique congenital syndrome including HBL, atrial septal defect, ventricular septal defect, patent ductus arteriosus, mental retardation, and seizures. However, the patient did not exhibit a megalencephaly typical of 4q21-22 deletions. His HBL was associated with an increasing serum alpha-fetoprotein level and rapid growth. To define the chromosomal deletion at the molecular level in this child, we analyzed his lymphoblasts with fluorescence in situ hybridization, using as probes a panel of BAC/PAC genomic clones containing STS markers covering the 4q12-27 region. The analysis revealed that the affected chromosome had an 8-cM deletion within 4q21-q22, flanked by markers D4S2964 and D4S2966. This microdeletion overlaps with the commonly deleted region at 4q21-q22 that was recently defined in adult hepatocellular carcinomas.
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PMID:An 8-cM interstitial deletion on 4q21-q22 in DNA from an infant with hepatoblastoma overlaps with a commonly deleted region in adult liver cancers. 1156 28

During the period January 1994 to December 1999, a total of 395 patients--adults and children--underwent various types of open heart surgical (OHS) procedures. The age range of these patients was 10 days to 77 years. Most (43.5%) of these patients underwent valve replacement--first time and 'redo'--as a result of rheumatic heart disease of varying duration and severity. The other large group was the complex congenital heart abnormalities (16%), followed by the group that underwent repair of atrial septal defect (13%), coronary artery bypass graft (13%) and repair of ventricular septal defect (12%). Open valve repair (2%) and excision of atrial myxoma (0.5%) were uncommon OHS procedures. Nine patients (2.3%) developed severe neurological complications (NCs) post-OHS. These included: a short period (< 48 hours) of irritability and fluctuating consciousness level; seizure activity associated with hemisparesis and/or blindness; severe encephalopathy and vegetative state. Four patients showed complete recovery, but one died as a result of complications of the anti-seizure medication. Of the other five, four died as a result of the NCs, and one was discharged in a vegetative state. Such severe neurological complications after an otherwise successful cardiac surgery represent a devastating outcome for patients and their families, and the social and economic impacts are enormous. Several risk factors were identified. The main ones being mitral valve replacement (MVR), especially 'redo' surgery, female gender, age over 60 years, high New York Heart Association functional class and post-operative hypotension. However, the true incidence of NCs (gross and subtle) in the study group cannot be ascertained. A detailed, structured neurological and neuropsychiatric assessment, both pre- and post-operatively, is therefore needed to document the true incidence of this complication. There is also the need for public education, especially for those with valvular disease, in order to encourage changes in attitude and behaviour towards continued follow-up care and valve surgery, both first time and 'redo'.
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PMID:Severe neurological complications post-open heart surgery. The Jamaican experience. 1199 21


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