Melricflash/CW_MedAbstractsAlt huggingface dataset:
Epidermal nevus syndrome: a neurologic variant with hemimegalencephaly, gyral malformation, mental retardation, seizures, and facial hemihypertrophy. The epidermal nevus syndrome (ENS) is a sporadic neurocutaneous disorder that consists of epidermal nevi and congenital anomalies involving the brain and other systems. From among over 60 patients with ENS presenting with neurologic manifestations, we identified 17 who had hemimegalencephaly based on pathologic or radiologic studies. Associated brain and neurologic abnormalities included gyral malformations in 12 of 12, mental retardation in 13 of 14, seizures in 16 of 17 (including 9 with infantile spasms), and contralateral hemiparesis in 7 of 12. All had ipsilateral epidermal nevi of the head, and several had ipsilateral facial hemihypertrophy. We concluded that these abnormalities comprise a recognizable neurologic variant of ENS that we believe represents the full expression of primary brain involvement. Several patients also had evidence of acquired brain lesions such as infarcts, atrophy, porencephaly, and calcifications, which are best explained by prior ischemia or hemorrhage. Given repeated observations of blood vessel anomalies in ENS patients, we hypothesize that underlying vascular dysplasia predisposes to these acquired lesions. The same cause may be invoked to explain the wide variety of neurologic symptoms reported in ENS patients without hemimegalencephaly. While the cause of ENS remains unknown, several observations suggest a somatic mutation. 

Category: nervous system diseases
Dural spinal cord arteriovenous malformation. After multiple hospital admissions and an inpatient rehabilitation stay, a 68-year-old woman was transferred to our rehabilitation facility with a paraparesis of unknown etiology. Previous studies included four CT scans and three MRIs which did not demonstrate the lesion. A myelogram was noncharacteristic. The correct diagnosis, confirmed by selective angiography, was ultimately contingent upon recognition of the clinical features and natural history of dural spinal cord arteriovenous malformations (SCAVM). The unusual combination of this multitude of nondiagnostic imaging studies in the uncommon dural SCAVM served to delay diagnosis and treatment. Such delay may have great functional consequences. This report illustrates the importance of suspecting SCAVM and recognizing its features. Emphasis is placed on the physiatrist's role in assuring proper diagnosis to expedite a timely treatment and to obtain the best functional outcome. A brief review of the classification, clinical features, pathophysiology, diagnosis, and prognosis of SCAVM is presented. 

Category: cardiovascular diseases
Proximal posterior cerebral artery occlusion simulating middle cerebral artery occlusion. We describe 12 cases of acute stroke in which clinical features of proximal posterior cerebral artery occlusion simulated the clinical syndrome of middle cerebral artery occlusion. The majority of patients developed contralateral hemiparesis, homonymous hemianopia, hemispatial neglect, and sensory loss or sensory inattention. All 8 patients with dominant hemisphere lesions were aphasic. Accurate diagnosis in each case was achieved only after a head CT, showing occipital lobe, thalamic, and inferomesial temporal lobe infarction. "Cortical" signs are probably explained by thalamic involvement. Recognition of this syndrome has implications for management and prognosis. 

Category: cardiovascular diseases
The effect of race on the relationship between fetal death and altered fetal growth. This population study examines racial differences in the relationship between birth weight and fetal death. An earlier report showed that a birth weight that results in a fourfold increased risk of stillbirth is not a constant birth weight percentile (2nd percentile at 25 weeks, 17th percentile at 42 weeks). This analysis was applied to 782,430 white and black births in Illinois from 1980 to 1984. Mean and 10th percentile growth for white and black infants is identical before 34 weeks' gestation and growth diverges by 250 gm at term, with white infants being larger. Race-specific birth weights resulting in quadrupling of the stillbirth rate were determined with an exponential regression analysis of the relationship between birth weight and fetal death rate for each gestational age. The data indicate (1) that the birth weights resulting in quadrupling the stillbirth rate are substantially above the Denver 10th percentile and the previously unpublished race-specific Illinois 10th percentiles and (2) that at term white infants demonstrate this constant risk at the 12th percentile, whereas black infants exhibit the risk at the 18th percentile. From these data we conclude that black fetuses are more sensitive than white fetuses to factors that adversely affect growth and that continued use of "race-neutral" data for clinical management in racially heterogenous populations will not accurately predict the risk of stillbirth. 

Category: general pathological conditions
Hypertension and diabetes in blacks. This article summarizes the current state of knowledge on the interrelationship between non-insulin-dependent diabetes mellitus, obesity, insulin resistance, and hypertension in our attempt to explore the pathophysiology of the high incidence of diabetes and hypertension in the Black population in the United States. A central role for hyperinsulinemia is proposed, and questions are raised that are suitable for investigative clinical trials. 

Category: cardiovascular diseases
Decision criteria for pure-tone detection used by two age groups of normal-hearing and hearing-impaired listeners. Response criteria from a yes-no task and detection thresholds from two test procedures were measured for four groups of adults: younger normal-hearing, older normal-hearing, younger hearing-impaired, and older hearing-impaired. The two test procedures were an audiological procedure (which does not control for response bias) and a 21FC adaptive procedure (which does control for bias). The signal was a 500 or 4000 Hz tone presented in quiet. All four groups showed an equally conservative response bias in the yes-no task. In addition, neither age nor hearing loss affected the difference (6.5 dB) between the two threshold measures. 

Category: general pathological conditions
Hepatitis B vaccination and interleukin 2 receptor expression in chronic renal failure. Only 50 to 60% of dialysis patients develop anti-HBs antibodies following hepatitis B vaccination. The nonresponder state correlates with impaired monocyte function, decreased interleukin-2 (IL-2) production of T cells, and an upregulation of the IL-2 receptor system. In the present study we examined anti-HBs production after hepatitis B vaccination and the in vitro expression of IL-2 receptors in nondialyzed patients with various degrees of chronic renal failure. Forty-four patients with impaired renal function were immunized with 2 micrograms recombinant hepatitis B vaccine and boostered after one and six months. Prior to the first injection IL-2 receptor expression of activated T cells was studied by an in vitro proliferation assay. Sixty-four healthy subjects served as controls. After completion of the third vaccination 55.0% of the patients acquired antibody titers greater than 10 U/liter. The seroconversion rate did not differ between patients with lower (less than 3.5 mg/dl) and higher (greater than 3.5 mg/dl) creatinine levels. In nonresponders IL-2 receptor expression (stimulation index, SI = 10.09 +/- 1.80) was elevated compared to healthy controls (SI = 4.62 +/- 0.35, P less than 0.002) or patients who responded with a high antibody titer (greater than 50 U/liter, SI = 3.12 +/- 0.43, P less than 0.001). Patients who produced low antibody titers (less than 50 U/liter) also presented with enhanced IL-2 receptor expression. These data show that an impaired antibody production following hepatitis B vaccination and an enhanced IL-2 receptor expression of T cells may already be present in early stages of chronic renal failure. 

Category: digestive system diseases
Adenovirus 12S E1A gene represses differentiation of F9 teratocarcinoma cells. The F9 teratocarcinoma cell line differentiates in vitro after treatment with retinoic acid and cAMP and has been a widely used model system for the study of the molecular events that are responsible for cellular commitment and differentiation during early development. Previous experiments have suggested intriguing parallels between the control of gene expression during F9 cell differentiation and the regulation of gene expression by adenovirus E1A. Transfection of a 12S E1A-expressing plasmid into terminally differentiated, nonproliferating F9 cells generates, at high frequency, colonies of dividing cells, each of which expresses E1A. Cell lines established from these colonies proliferate in the presence of retinoic acid and have lost the fully differentiated phenotype as characterized by the absence of expression of a series of differentiation-specific genes. We conclude that expression of the viral 12S E1A gene product interferes with retinoic acid-induced F9 cell differentiation. Moreover, the results suggest that the differentiation process, as defined by markers of terminal differentiation, may not be a permanent event but can be reversed by E1A expression. 

Category: neoplasms
Lymphoma, motor neuron diseases, and amyotrophic lateral sclerosis. We studied 9 patients with motor neuron disease and lymphoma. The following several observations have not been recognized in the past: (1) Motor neuron syndromes are associated with either Hodgkin's disease or non-Hodgkin's lymphoma. (2) The syndromes are not restricted to lower motor neuron disorders; 8 of 9 patients had definite or probable upper motor neuron signs as well, qualifying for the diagnosis of amyotrophic lateral sclerosis. Corticospinal tracts were affected in both postmortem examinations. (3) The combination of motor neuron disease and lymphoma is often accompanied by paraproteinemia (3 of 7 patients studied), increased cerebrospinal fluid protein content (6 of 9 patients), and cerebrospinal fluid oligoclonal bands (3 of 9 patients). (4) In 2 patients, asymptomatic non-Hodgkin's lymphoma was found only because the discovery of paraproteinemia gave impetus to examine the bone marrow. (5) Patients with both upper and lower motor neuron signs (amyotrophic lateral sclerosis) may show physiological evidence of conduction block in peripheral nerves or autopsy abnormalities in peripheral nerves. The cause of this syndrome is not known. Both lymphoma and motor neuron disease could have a common cause, possibly a retroviral infection. The frequency of paraproteinemia suggests that an immunological disorder may play a role in the pathogenesis of the neurological disorder. 

Category: nervous system diseases
Computed tomography-guided fine-needle aspiration of retroperitoneal lymph nodes in gynecologic oncology. Computed tomography-guided fine-needle aspiration was performed on 30 retroperitoneal lymph nodes in 29 patients with gynecologic cancer. There were no serious complications. Review of the cytologic material demonstrated malignant cells in 83% of the aspirates. Because the predictive value of a positive aspirate approaches 100%, therapy for metastatic disease can be initiated in these patients with the need for an open biopsy. Among five aspirates in which malignant cells were not seen, the cellularity of the specimen appeared to be the critical factor determining the predictive value of the aspirate. Whereas neither of two patients with negative aspirates of adequate cellularity has developed recurrent disease, two of three patients with hypocellular negative aspirates have. Because a hypocellular negative aspirate from a retroperitoneal lymph node may not be a true reflection of disease status, either repeat aspiration or open biopsy is advisable. 

Category: neoplasms
Intracranial pressure in African children with cerebral malaria Opening lumbar cerebrospinal fluid (CSF) pressure was measured with a paediatric spinal fluid manometer in 26 of 61 Kenyan children (mean age 39 months) with cerebral malaria. In all cases pressure was above normal (mean [SD]22.6 [7.4] cm CSF, range 10.5-36). Clinical features of our patients suggest that intracranial hypertension is important in the pathogenesis of cerebral malaria in children, especially as a cause of death. We suggest that raised intracranial pressure is secondary to increased cerebral blood volume. Lowering intracranial pressure may significantly reduce the mortality and morbidity of cerebral malaria. The potential risks and benefits of lumbar puncture should be considered carefully in patients with suspected cerebral malaria. 

Category: nervous system diseases
Experimental models of temporal lobe epilepsy: new insights from the study of kindling and synaptic reorganization. Temporal lobe epilepsy is a common localization-related epileptic syndrome characterized by complex partial seizures, ictal and interictal epileptic discharges arising from limbic structures of the temporal lobe, and association with hippocampal sclerosis. Temporal lobe epilepsy may follow perinatal injury and febrile convulsions, may be progressive, and frequently becomes refractory to standard antiepileptic therapy. The neurobiology that underlies these features of temporal lobe epilepsy is not known. Recent studies in experimental models have provided new insights that may help clarify the relationship of seizures, hippocampal sclerosis, and temporal lobe epilepsy. Observations from the study of the hippocampus with kainic acid-induced lesions, the kindling model, and other experimental models of epilepsy have demonstrated that seizures induce structural and electrophysiologic alterations in hippocampal pathways that may lead to increased excitability and could play a role in the development and progression of temporal lobe epilepsy. These alterations include mossy fiber synaptic reorganization, induction of NMDA-mediated synaptic transmission, and progressive hippocampal neuronal loss induced by brief kindled seizures. Some of the structural alterations induced by kindling have also been observed in the human epileptic temporal lobe, raising the possibility that mechanisms operative in kindling may play a role in the pathogenesis of hippocampal sclerosis and in the syndrome of human temporal lobe epilepsy. 

Category: general pathological conditions
Fludarabine: a new agent with marked cytoreductive activity in untreated chronic lymphocytic leukemia. Thirty-three patients with chronic lymphocytic leukemia (CLL) with advanced Rai stage (III-IV) or progressive Rai stage (0-II) disease were treated with fludarabine as a single agent. Eleven patients (33%) obtained a complete remission (CR), 13 (39%) a clinical CR with residual nodules as the only evidence of disease (nodular partial remission [PR]), and two patients (6%) achieved a PR for a total response rate of 79%. Response was rapid, usually occurring after three to six courses of treatment. The major morbidity was infection. Febrile episodes occurred in 13% of the courses (pneumonia 6%, minor infection 4%, and transient fever of undocumented cause 3%). Fludarabine appears to be the most cytoreductive single agent so far studied in CLL. 

Category: general pathological conditions
Induction of mixed erythroid-megakaryocyte colonies and bipotential blast cell colonies by recombinant human erythropoietin in serum-free culture. The effects of recombinant human erythropoietin (rEp) on murine hematopoietic progenitors were studied using a serum-free culture. A high concentration of rEp stimulated the formation of mixed erythroid-megakaryocyte colonies (EM colonies) and blast cell colonies, as well as erythroid colonies, erythroid bursts, and megakaryocyte colonies from normal mouse bone marrow cells. Direct effects of rEp on EM colony, megakaryocyte colony, and erythroid burst formation were confirmed by depletion of accessory cells such as T cells, B cells, and macrophages from crude bone marrow cells, and inhibition of the colonies by the addition of rabbit anti-rEp antibody to the culture in a dose-dependent fashion. Replating experiments were performed to confirm the differentiating ability of blast cell colonies grown in the presence of rEp. Most of the blast cell colonies yielded not only secondary erythroid colonies but also megakaryocyte colonies in the presence of 2 IU/mL rEp. Some of the blast cell colonies produced secondary EM colonies in the presence of 16 IU/ml rEp of 2 IU/mL rEp plus interleukin-3, although no granulocyte-macrophage colonies were found in the secondary culture. These results suggest that Ep acts not only as a late-acting factor that is specific for erythroid progenitors, but also as a bipotential EM-stimulating factor for murine hematopoietic cells. 

Category: general pathological conditions
Intracranial pressure and cerebrospinal fluid outflow conductance in healthy subjects. Conductance of cerebrospinal fluid (CSF) outflow (Cout) is an important parameter to be considered in patients with CSF circulation abnormalities. In patients with normal-pressure hydrocephalus it is the single most important parameter in determining if the patient needs CSF shunting. The lower normal limit for Cout has been estimated from the effect of shunting in patients with normal-pressure hydrocephalus, from patients retrospectively reevaluated after recovering from illness, and from patients with known abnormalities in the brain or the CSF system. The true value of Cout in normal individuals, however, has hitherto not been reported. In the present study, Cout has been measured by a lumbar infusion test in eight young volunteers with no suspicion of disease. The mean intracranial pressure (ICP) was 11 mm Hg and a linear relationship was found between CSF absorption and ICP. The mean Cout was 0.11 ml/min/mm Hg and the lower 95% confidence level was 0.10 ml/min/mm Hg. These values are in accordance with those obtained from previous studies. 

Category: nervous system diseases
Levels of serum granulocyte colony-stimulating factor in patients with infections. To clarify the physiologic roles of granulocyte colony-stimulating factor (G-CSF) in infectious states in vivo, we examined the serum levels of G-CSF in patients with infection. Serum samples from 24 patients in the acute stage of infection (14 men and 10 women, age 65 to 101, without hematologic disorders), as well as samples from 32 age-matched normal elderly volunteers were investigated. Sixteen of the initial 24 patients were reexamined after the recovery phase. G-CSF levels were examined by quantitative enzyme immunoassay. The G-CSF level in normal elderly controls, 25.3 +/- 19.7 pg/mL, was not different from that reported in other findings. There was no statistically significant relationship between their G-CSF level and peripheral white blood cell count or neutrophilic granulocyte count. The G-CSF level in the acute stage of infection was 731.8 +/- 895.0 pg/mL, with a range of 30 to 3,199 pg/mL. There was no significant difference in G-CSF levels between patients with respiratory tract infection and those with urinary tract infection. In all 16 cases examined, the serum G-CSF level in the acute stage of infection was significantly higher than that after recovery phase, the latter being the same as the level in normal elderly controls. G-CSF must therefore play a significant role in human infectious states in vivo. 

Category: general pathological conditions
Nosocomial infections due to Xanthomonas maltophilia (Pseudomonas maltophilia) in patients with cancer. From December 1985 to May 1986, Xanthomonas maltophilia (formerly known as Pseudomonas maltophilia) was isolated at an increased rate at our institution; 52 isolates of X. maltophilia were obtained in cultures of clinical specimens from 38 patients during that time. The records of 35 of these patients form the basis of this study. Twelve (71%) of the 17 infected and nine (50%) of the 18 colonized patients had received or were receiving antimicrobial therapy. Eleven of 17 patients (three of seven with septicemia, three of five with pneumonia, three with urinary tract infection, and two with wound infection) responded to antimicrobial therapy. Microbiologic studies of the potential environmental sources revealed growth of X. maltophilia in two water faucets and in one water sample from the medical intensive care unit. X. maltophilia is emerging as an important nosocomial pathogen in immunocompromised patients, especially those receiving broad-spectrum antimicrobial therapy. 

Category: general pathological conditions
Leukoaraiosis, intracerebral hemorrhage, and arterial hypertension. To investigate whether the observed association of leukoaraiosis with intracerebral hemorrhage is direct or mediated by risk factors, we compared 116 patients with intracerebral hemorrhage confirmed by computed tomography and 155 controls without intracerebral hemorrhage, evaluating the prevalence of leukoaraiosis and vascular risk factors. Leukoaraiosis was observed in 21 (18%) of the 116 patients and in 12 (8%) of the 155 controls (p less than 0.01). Only two (6%) of the 31 patients with lobar hemorrhage had leukoaraiosis on computed tomograms, compared with 17 (24%) of the 71 patients with basal ganglionic hemorrhage (p less than 0.05). Leukoaraiosis was significantly correlated with intracerebral hemorrhage after controlling for age and sex by using multiple logistic regression analysis, while the correlation disappeared after controlling for hypertension. Our results indicate that leukoaraiosis is not an independent risk factor for intracerebral hemorrhage. 

Category: general pathological conditions
Congenital craniofacial anomalies of ophthalmic importance. Congenital craniofacial abnormalities frequently require ophthalmic evaluation and surgical management. Called upon to perform as part of the craniofacial team managing the often severely deformed craniofacial patient, the ophthalmologist must bring a basic knowledge of craniofacial syndromes and developmental anatomy, as well as clinical acumen to help preserve or improve ocular and adnexal function. As an introduction to this area of ophthalmology, the clinical features, classification, appropriate facial embryology, assessment and surgical considerations of the various congenital craniofacial abnormalities are reviewed. The expanding availability of craniofacial surgeons and surgical teams along with improved surgical results will ultimately require an increasing involvement by many more ophthalmologists in the evaluation and management of these congenital abnormalities. 

Category: general pathological conditions
Management decisions in laryngeal carcinoma in situ. Twenty-one patients with laryngeal carcinoma in situ (n = 12) or carcinoma in situ with microinvasion (n = 9) were treated with laser mucosal dissection of the vocal cords and/or superficial laser cordectomy. The normal architecture of the vocal cords was preserved because the depth of vaporization was usually superficial in these early cases. Five patients had recurrence, new tumor, or persistence of abnormal tissue that required additional laser surgical treatments. With a follow-up range of 6 months to 4 years, all patients currently are free of laryngeal abnormality, and no patient has needed open laryngeal surgery or radiation therapy. Transoral endoscopic laser resection of laryngeal carcinoma in situ with or without microinvasion should be the treatment of choice for these early lesions. 

Category: neoplasms