Search PubMed⌕ Search

Biomedical subjects

L Burd

Publications and source records attributed to L Burd.

At least 55 records · Page 3Linked to original sources

Management of pregnancy in sickle cell syndromes.

We have seen a marked decrease in maternal and perinatal morbidity and mortality among pregnant patients with sickle cell disease. This has been the result of coordinated efforts with the obstetric and hematologic teams. Patients are counseled prior to pregnancy regarding the risks and are given the opportunity to modify their life style to prepare for the additional metabolic burden of gestation. Once pregnant, they are instructed in the techniques to recognize and avoid complications. They are observed frequently for the appearance of pain crisis and other medical and obstetric complications. If complications are identified, they should be treated aggressively. Transfusion therapy is important in the management of patients; however, prophylactic transfusion does not change outcome. Although significant laboratory techniques aid in fetal and maternal supervision, the universal fundamentals of good clinical perinatal care provided through the combined efforts of the obstetrician and hematologist contribute to the framework for the modern management and successful outcome of patients with sickle cell disease during pregnancy.

Anemia, Sickle Cell↗

Prevalence of psychoactive drug use among North Dakota group home residents.

To determine the prevalence of psychoactive medication usage among persons with mental retardation residing in community settings in the state of North Dakota, we sent a questionnaire to all group homes serving persons with developmental disabilities. The return rate was 97%. Psychoactive medications were used by 37% of residents represented. Results were discussed in relation to previous studies that have primarily involved institutionalized populations.

Adolescent↗

Rett syndrome symptomatology of institutionalized adults with mental retardation: comparison of males and females.

Institutionalized adults with mental retardation (N = 297) were surveyed to determine the prevalence of symptoms of Rett syndrome. No symptom of the syndrome occurred more frequently in males than females. When symptoms were analyzed in clusters (e.g., severe mental retardation, no prenatal complications, walked before 15 months, and wide-based gait), no single cluster of symptoms appeared to differentiate males from females. However, as individuals, only females were found to meet the necessary criteria for a diagnosis of Rett syndrome. Symptoms were seen with equal frequency in this population and no one or two symptoms differentiated patients with and without Rett syndrome.

Adolescent↗

A prevalence study of Rett syndrome in an institutionalized population.

An institution for the mentally retarded was surveyed to determine the prevalence rate of Rett syndrome (RS). Four patients with definite RS and one with probable RS were identified in a population of 350, yielding a prevalence rate of 1 in 87, about 1% of institutionalized male and female patients with mental retardation. In this population of patients with severe and profound mental retardation (N = 297), 138 females were surveyed, suggesting a prevalence rate among females of 1 in 34 in an institutional population of persons with mental retardation. Surveys of institutions for persons with mental retardation may be an effective method to identify adults with RS.

Adolescent↗

Prevalence study of Prader-Willi syndrome in North Dakota.

A prevalence study of Prader-Willi syndrome (PWS) has been conducted in North Dakota. All pediatricians, neurologists, child psychiatrists, psychologists, and clinical geneticists were surveyed. The state's comprehensive evaluation center, the state hospital, the state institution for the mentally retarded, and group homes for the developmentally disabled, including one for persons with PWS, were also contacted. Seventeen patients were identified, 8 males, 8 females, and one patient whose sex was not specified. This suggests a prevalence rate of 1 per 16,062 in North Dakota.

Adolescent↗

Autism, profound mental retardation and atypical bipolar disorder in a 33-year-old female with a deletion of 15q12.

A case report of a 33-year-old woman with atypical bipolar disorder, autistic disorder, profound mental retardation and a chromosomal anomaly is presented. This patient is behaviourally similar to a patient reported by Akuffo et al. (1986), who apparently did not have a chromosomal investigation. The chromosomal anomaly and similarities between the two patients are discussed.

Adult↗

Crossed aphasia in early childhood.

A four-year-old child was admitted to hospital with an infarct of the right middle cerebral artery involving the frontoparietal area. His symptoms included left hemiplegia and aphasia. After two weeks, he had hemiparesis, word-finding and naming problems and enuresis. A year later he demonstrated elective mutism at school, had attention and short-term memory impairments, occasional enuresis and an average IQ. He was shy and withdrawn; this is interesting, since depression is usually associated with left-hemispheric lesions. It is suggested that an early period of mutism should be included among the criteria for the study of crossed aphasia in children, as this is a common occurrence in such cases. Even after recovery of speech, impairments in attention and academic skills may persist.

Aphasia↗

Asperger's syndrome: to be or not to be?

It has been questioned whether Asperger's syndrome (AS) is in fact a specific (high functioning) subgroup of autism, rather than a distinct entity. Thirteen AS patients were compared with 13 autistic patients and 13 developmentally disordered controls. While there was symptom overlap between AS and autism, patients could be separated into one or other group. However, current criteria are based on symptoms, and it is argued that studies of genetics and treatment response are needed to elucidate the relationship between these developmental disorders.

Adolescent↗

The hand apraxia scale.

A scale for the assessment of hand apraxia was developed on a population of 239 institutionalized mentally retarded adults. Guttman scaling indicates adequate reliability and validity.

Activities of Daily Living↗

Preliminary report on construction and validation of a pediatric sleep disturbance questionnaire.

A scale to assess sleep disturbance of children with developmental disabilities is reported. The scale has been used with a small sample of 14 children diagnosed as having Tourette Disorder, 36 physically handicapped children (cerebral palsy). Also, a group of 115 nonhandicapped Native American children were surveyed to assess whether the items might be appropriate for use with such children. The scale has 6 distinct factors and appears to have adequate reliability and validity.

Adolescent↗

A possible post-streptococcal movement disorder with chorea and tics.

A 14-year-old girl developed a movement disorder after a streptococcal infection. In the acute phase of the illness she exhibited simple and complex motor tics and chorea, but all abnormal movements ceased over the following eight months, without recurrence. This case raises questions about the relationship between tics, chorea and auto-immune reactivity.

Adolescent↗

Fetal alcohol syndrome: diagnosis and syndromal variability.

The diagnostic criteria for Fetal Alcohol Syndrome (FAS) are reviewed and the authors suggest a new diagnostic schema to allow for a more adequate description of the range of FAS. FAS is also reviewed by topic area. Associated problems believed to be caused by maternal alcohol ingestion are discussed.

Brain↗

Pervasive disintegrative disorder: are Rett syndrome and Heller dementia infantilis subtypes?

Children with developmental regression and emerging symptoms of autism have been given a variety of classifications. The authors compare two boys with Heller dementia with six girls with Rett syndrome. They all differed from children with classic autism in that they had normal prenatal and perinatal periods, followed by marked developmental regression, after which they acquired few or no skills. The boys differed from the girls in terms of estimated prevalence, age at onset, stereotypic breathing patterns, midline hand stereotypies, hand and gait apraxia and speech development. It is suggested that these children should be distinguished from those with classic autism, and should be classified as 'pervasive disintegrative disorder, Heller type' and 'pervasive disintegrative disorder, Rett type'.

Adolescent↗

Tourette disorder and bipolar symptomatology in childhood and adolescence.

Three boys with an early history of attention deficit disorder with hyperactivity developed Tourette disorder. At 13, 12 and eight years of age, respectively, each met DSM-III criteria for a manic episode or bipolar disorder. Each of the boys had a family history of affective or affective spectrum disorder. Lithium carbonate in a range of 0.8 to 1.2 meq/L markedly improved their bipolar symptomatology with Tourette symptoms improving in two patients. Further study is suggested to determine the significance of these findings.

Adolescent↗

Prophylactic red-cell transfusions in pregnant patients with sickle cell disease. A randomized cooperative study.

Prophylactic blood transfusion has come to be regarded as necessary in the treatment of patients with sickle cell disease during pregnancy. Because of the risks associated with blood products and reports of successful outcomes without the use of blood transfusion, we conducted a prospective randomized controlled study of this issue. Seventy-two pregnant patients with sickle cell anemia were randomly assigned to one of two treatment groups: 36 received prophylactic transfusions of frozen red cells, and 36 received red-cell transfusions only for medical or obstetric emergencies. Twenty-eight patients with sickle cell anemia who did not qualify for randomization (mainly because they had other medical disorders), 66 with sickle cell-hemoglobin C disease, and 23 with sickle cell-beta-thalassemia were also followed and received transfusions only for emergencies. There was no significant difference in perinatal outcome between the offspring of mothers with sickle cell disease who were assigned to treatment with prophylactic transfusions and those who were not (15 vs. 5 percent). The occurrence of a perinatal death in a previous pregnancy and the presence of twins in the present pregnancy were two major risk factors for an unfavorable outcome; when they were present, perinatal mortality was 50 percent. Perinatal mortality was somewhat higher in the two groups that were randomized than in the three groups that were not. Prophylactic transfusion significantly reduced the incidence of painful crises of sickle cell disease (P less than 0.01) and substantially reduced the cumulative incidence of other complications of this disorder (P = 0.07). Other medical and obstetric complications occurred with nearly equal frequency in the two randomized groups. Increases in costs, the number of hospitalizations, and the risk of alloimmunization were disadvantages of prophylactic transfusion. We conclude that the omission of prophylactic red-cell transfusion will not harm pregnant patients with sickle cell disease or their offspring.

Adult↗