shukoor65/lab-rag-model huggingface dataset:
Responsiveness to phenobarbital in an adult with Crigler-Najjar disease associated with neurological involvement and skin hyperextensibility. We present the case of a 23-yr-old man who had had since birth marked and sustained unconjugated non-hemolytic hyperbilirubinemia and who had had several attacks of grand mal seizures. Analysis of serum bilirubin by diazoreactive methods showed serum levels of unconjugated bilirubin as high as 445 mumol/L that were not affected by phenobarbital administration. However, analysis of serum bile pigments by high-pressure liquid chromatography demonstrated marked decrease of unconjugated bilirubin after phenobarbital treatment (from 432.4 mumol/L to 291.0 mumol/L) associated with slight increase of bilirubin monoconjugates and disconjugates (from 0.25 mumol/L to 0.42 mumol/L). Furthermore, in the past few years the patient had exhibited striking skin hyperextensibility and diaphragm eventration. This case confirms that alkaline methanolysis-high-pressure liquid chromatography is the most reliable method for assessment of serum fraction bilirubin levels; that clinical parameters such as neurological signs do not unequivocally discriminate between type I and II Crigler-Najjar disease and that response to phenobarbital treatment remains the main diagnostic tool.
Myocardial infarction in childhood: clinical analysis of 17 cases and medium term follow up of survivors. Between 1979 and 1989 17 patients aged two months to 12 years with acute myocardial infarction of any cause (other than after cardiac surgery) were seen at a children's hospital. Eight died from three days to three years after diagnosis (overall mortality 47%). The nine survivors, now aged 2-17 years, have been followed for one to 10 years (mean follow up five years) after infarction. The commonest causes of myocardial infarction in this series were anomalous origin of left coronary artery from the pulmonary artery (six patients (35%] and Kawasaki disease (five patients (27%]. The main symptoms of acute myocardial infarction were dyspnoea, vomiting, and difficulty feeding. Diagnosis was made in all patients by electrocardiography and confirmed by echocardiography, cardiac catheterisation, or at operation. All survivors were symptom free with excellent exercise capacity. The left ventricular ejection fraction in survivors ranged from 21% to 66%, and only one child was on regular cardiac medications. There were no cases of late sudden death. Twenty four hour Holter monitoring performed on survivors was normal (seven) or showed minor abnormalities only (one), suggesting that serious arrhythmia is rare after paediatric myocardial infarction. Myocardial infarction in children had a high early mortality; however, the incidence of serious arrhythmia was low in the survivors, who had a good exercise tolerance even when the left ventricular ejection fraction was low.
Polyneuropathy induced by m-tolyl methyl carbamate intoxication. A 55-year-old woman who attempted suicide by ingesting 200 ml of m-tolyl methyl carbamate (MTMC) is reported. She was comatose for 3 days and, upon recovery, had notable paraesthesia in her lower limbs and difficulty in walking. Neurological examination revealed sensorimotor polyneuropathy. Sural nerve biopsy revealed marked axonal degeneration with a moderate decrease of myelinated fibres.
Pseudoaneurysm of the popliteal artery as a complication of an osteochondroma. A review of the literature and a case report. False aneurysm of the femoral artery was associated with a solitary osteochondroma of the femur in a 22-year-old man. An extensive review of the literature and the problems related to diagnosis revealed that the aneurysm was apparently unique. Contrary to previous reports, computed tomography and angiography did not establish the diagnosis. The physical findings were more informative.
Mammographic and CT findings after breast reconstruction with a rectus abdominis musculocutaneous flap. This essay illustrates the radiologic appearance of the reconstructed breast and the abdominal wall after breast reconstruction with a transverse rectus abdominis musculocutaneous (TRAM) flap. The findings are based on a retrospective study of 42 mammograms, 17 abdominal CT scans, and two CT scans each of the chest and pelvis of patients who underwent this procedure.
Surgical management of severe aortic outflow obstruction in lesions other than the hypoplastic left heart syndrome: use of a pulmonary artery to aorta anastomosis. Between December 1985 and April 1990, 50 infants with a variety of congenital cardiac lesions other than the hypoplastic left heart syndrome underwent surgical relief of aortic outflow obstruction by creation of a pulmonary artery to aorta anastomosis. The patients were grouped anatomically by ventriculoarterial alignment. Nineteen had normally aligned great arteries (group I); 25 had transposition of the great arteries, all with a univentricular heart of left ventricular morphology (group II); and 6 had a double-outlet right ventricle (group III). All patients had either aortic stenosis with atresia, subaortic stenosis or a restrictive ventricular septal defect. Sixteen had normal arch anatomy; 34 had arch anomalies consisting of arch hypoplasia (n = 17), coarctation (n = 11), interruption of the arch (n = 4) and complex arch anomalies (n = 2). Surgery was performed at a median age of 10 days (range 2 to 184). Of the 50 infants, 33 survived. No significant difference in early survival (30 days) was noted among the groups of varying ventriculoarterial alignment (68% group I, 72% group II, 83% group III) (p greater than 0.05). Overall actuarial survival was 63% at 18 months. Analysis of actuarial survival by arch anatomy, although not statistically significant, revealed a trend toward better survival at 18 months postoperatively in infants with normal arch anatomy (81%) than in infants with arch anomalies (54%). Of the 33 survivors, 26 have proceeded to the next surgical stage, including the Fontan procedure in 8, superior cavopulmonary anastomosis in 13 and biventricular repair in 5.
Sudden death in infants sleeping on polystyrene-filled cushions. BACKGROUND. Infants are at risk for both the sudden infant death syndrome (SIDS) and accidental suffocation. On postmortem examination, however, it is difficult to distinguish one from the other without information from the scene of death. Healthy infants are assumed to be able to turn their heads and, if not otherwise restrained, to obtain fresh air. We assessed this assumption in an investigation of infant deaths that occurred during sleep on cushions filled with polystyrene beads. METHODS. We obtained data on 25 deaths from the U.S. Consumer Product Safety Commission. We also used mechanical and animal models to study physiologic aspects of ventilation relevant to these results, by simulating the effects on an infant of breathing into a cushion. We measured the effects of softness, malleability (molding of the cushion about an infant's head), airflow resistance, and rebreathing of expired gases. RESULTS. All 25 study infants were prone when found dead, and at least 88 percent were face down with nose and mouth obstructed by the cushion. SIDS was the diagnosis in 19 of the 23 infants who underwent autopsy. Our findings show, however, that the cushion would have limited movement of the infant's head to obtain fresh air, and the amount of rebreathing we estimated to have occurred in the infants was lethal in a rabbit model. CONCLUSIONS. Accidental suffocation by rebreathing was the most likely cause of death in most of the 25 infants studied. Consequently, there is a need to reassess the cause of death in the 28 to 52 percent of the victims of SIDS who are found with their faces straight down. Safety regulations setting standards for softness, malleability, and the potential for rebreathing are needed for infant bedding.
Complications of tissue plasminogen activator therapy after vitrectomy for diabetes. Human recombinant tissue plasminogen activator (25 micrograms) was injected into seven eyes of six patients who had developed massive fibrin deposition after vitrectomy surgery for diabetes. Six eyes had developed pupillary membranes and recurrence of tractional retinal detachment from fibrin membranes, and one eye had developed only a pupillary membrane. All pupillary membranes resolved within one hour of administration of tissue plasminogen activator, and five tractional retinal detachments resolved within 24 hours. All eyes developed evidence of intraocular bleeding after tissue plasminogen activator injection. Subsequently, six of seven eyes developed recurrence of fibrin accumulation and tractional retinal detachment.
Diet and nutrition in ulcer disease. In this era of H2-inhibitors, the available evidence does not support the need to place peptic ulcer disease patients on restrictive diets. The major goal of diet is to avoid extreme elevations of gastric acid secretion and the direct irritation of gastric mucosa. In view of this, only slight modifications in the patient's usual diet are recommended. Table 1 depicts a sample menu for chronic peptic ulcer disease. Frequent milk ingestion as previously prescribed is not encouraged. This is owing to the transient buffering effect and significant gastric acid secretion effect of milk. The fat content of milk has no influence on these effects. Spices, in particular black pepper, red pepper, and chili powder, may produce dyspepsia. One study shows red chili powder to have no detrimental effect on duodenal ulcer healing. It has also been proposed that daily pepper ingestion may have a beneficial adaptive cytoprotective response. While still controversial and under evaluation, peptic ulcer patients should avoid any spice that causes discomfort, especially during exacerbation of peptic disease. Currently, studies indicate that it is prudent to avoid alcohol. This is especially true for the concentrated forms, such as 40% (80 proof) alcohol. Coffee should be avoided on the basis of its strong acid secretagogue property. Coffee can induce dyspepsia. Whether noncoffee caffeine-containing beverages (tea, soft drinks) induce peptic ulcer is unknown, but they are acid secretion stimulators. Decaffeinated coffee has an acid stimulating effect as well. It is reasonable to have peptic ulcer patients restrict decaffeinated coffee and all caffeine-containing beverages. There appears to be no evidence to restrict dietary fiber. Some fiber-containing foods may possess factors that are protective against ulcer disease. According to the Mayo Clinic Diet Manual, previously recommended small frequent feedings have not been shown to be more effective than three meals per day in the treatment of chronic peptic ulcer disease. This reference cites authorities advising against extra feedings because of increased acid secretion and unnecessary complication of eating patterns. However, some patients claim to be relieved of symptoms with more frequent feedings, especially during acute phases. Citric acid juices may induce reflux and cause discomfort in selective patients. Stomach distention with large quantities of food should be discouraged. Although there is now little role for dietary therapy, one should note that bland and ulcer diets probably are not detrimental to most persons if they are used for a short time and may have some psychological benefit.(ABSTRACT TRUNCATED AT 400 WORDS).
Risk factors for gastrointestinal ulcers caused by nonsteroidal anti-inflammatory drugs (NSAIDs) [published erratum appears in J Fam Pract 1991 Jul;33(1):16] Thirteen million individuals in the United States take nonsteroidal anti-inflammatory drugs (NSAIDs) chronically for the relief of pain and inflammation caused by arthritis and related conditions. NSAIDs are valuable and in many cases essential to the management of arthritis pain. Long-term use of NSAIDs, however, can cause gastrointestinal (GI) ulcers and potentially life-threatening ulcer complications. Factors such as older age (greater than or equal to 60 years), a history of peptic ulcer disease, concomitant corticosterid use, cigarette smoking, alcohol use, and high dose or multiple NSAID therapy can increase the risk of GI damage and complications in the chronic NSAID user. Physician awareness of these risk factors is crucial so that the benefits and hazards of NSAID therapy in high-risk patients can be properly weighed. When the decision is made to prescribe NSAIDs, despite the presence of risk factors for NSAID-induced ulcers, patients should be closely monitored, and, in some cases, effective prophylaxis should be initiated. This is especially important since NSAID-induced gastrointestinal ulcers can be asymptomatic, causing serious ulcer complications without warning.
Nesidioblastosis: evidence for autosomal recessive inheritance. The risk of recurrence of nesidioblastosis is unclear. Thirty patients from 28 families were studied; the marriages were consanguineous in five families. The ratio of subsequent affected to unaffected siblings was similar to that of an autosomal recessive condition and the sex ratio was close to 1.
Isolated serum-free perfused rat kidneys release immunoreactive erythropoietin in response to hypoxia. The renal glycoprotein hormone erythropoietin (Epo) interacts with erythrocytic progenitors to stimulate their proliferation and differentiation in the bone marrow. The renal O2-sensing mechanism in the control of the synthesis of Epo is still poorly understood. Therefore, the capacity of isolated rat kidneys to produce Epo during hypoxic and anemic perfusion was studied. The kidneys were perfused at a constant perfusion pressure of 100 mm Hg with a substrate-enriched Krebs-Henseleit solution containing 60 g/liter BSA and freshly drawn human erythrocytes. Epo was measured by RIA. When the kidneys were perfused at an arterial pO2 of 720 or 150 mm Hg (hematocrit, 5%), Epo production was very low (0.1-0.2 U/g kidney within 3 h of perfusion). When the arterial pO2 was lowered to 35 or 20 mm Hg, Epo production increased to 0.4 and 0.9 U/g kidney, respectively. The release of Epo during hypoxic perfusion (pO2 35 and 20 mm Hg) was little affected by changes in the hematocrit, i.e. the O2-carrying capacity of the perfusion medium over a wide range (0-40%). These results indicate that the production of Epo in the isolated perfused kidney depends on the availability of O2 and can be modulated by changes in the arterial pO2.
Flexible sigmoidoscopy may be ineffective for secondary prevention of colorectal cancer in asymptomatic, average-risk men. Asymptomatic men (N = 114) 50 years of age or older had screening for colorectal neoplasia with flexible sigmoidoscopy followed by colonoscopy regardless of the sigmoidoscopic result. Our study objective was to determine the prevalence of patients having isolated adenomatous polyps in a proximal colonic segment in the absence of a distal index neoplasm within reach of the sigmoidoscope. Through the combined use of sigmoidoscopy and colonoscopy, adenomatous polyps were detected in 47 of 114 individuals (41%). A total of 88 adenomas was found. Seventeen patients had isolated neoplasms in proximal colonic segments in the absence of distal adenomas. These patients represented 15% of screened subjects (17 of 114) and 20% of individuals who lacked adenomas on sigmoidoscopy (17 of 84). The majority of proximal neoplasms were small (less than 1.0 cm), tubular adenomas. Flexible sigmoidoscopy may be ineffective for screening asymptomatic men for neoplasia. However, it remains to be determined if a 20% miss rate (for those with a normal sigmoidoscopic examination) is significant and whether small proximal adenomas are worth finding.
Temporal thresholds for neocortical infarction in rats subjected to reversible focal cerebral ischemia. We investigated the temporal threshold for focal cerebral infarction in the spontaneously hypertensive rat. The right middle cerebral artery and common carotid artery were occluded for 0, 1, 2, 3, 4, or 24 hours, and all the animals were sacrificed 24 hours after the onset of ischemia. Cortical infarct volumes and edema volumes were quantified in serial frozen sections of hematoxylin and eosin-stained tissue using image analysis. Upon occlusion, blood flow in the core of the ischemic zone, measured with laser-Doppler flowmetry, fell to a mean +/- standard deviation of 21 +/- 7% of the preocclusion baseline value (n = 26). During the first hour of ischemia, blood flow in the densely ischemic zone rose to 27 +/- 8% of baseline (n = 25). Release of the middle cerebral artery and common carotid artery occlusions rapidly restored cortical blood flow to 213 +/- 83% of baseline (n = 21). Focal ischemia of 1 hour's duration caused little or no infarction, while ischemic intervals of 2 and 3 hours produced successively larger volumes of infarcted cortex. Ischemic intervals of 3-4 hours' duration followed by approximately 20 hours of recirculation yielded infarct volumes that were not significantly different from those after 24 hours of permanent focal ischemia. The results indicate that 3-4 hours of focal cerebral ischemia in this rat model is sufficient to attain maximal infarction and suggest that recirculation or pharmacological interventions after this time will provide little benefit.
Air esophagogram and intestinal pseudoocclusion in a patient with scleroderma. Air in the esophagus is unusual because it is collapsible. Its finding on a chest roentgenogram, particularly when not associated with a fluid level indicative of stricture, should strongly suggest systemic sclerosis (scleroderma). We describe a patient with scleroderma with intestinal pseudoocclusion and an air esophagogram. Study of chest roentgenograms of 83 patients with scleroderma, including those of 7 with pseudoocclusion, revealed no other instance of air esophagogram. This radiological sign, although rare, should suggest scleroderma and may be particularly useful in patients with "systemic sclerosis sine scleroderma.".
Local anesthesia blocks the antiemetic action of P6 acupuncture. The incidence of postoperative illness was monitored for 6 hours in 74 women premedicated with nalbuphine, 10 mg, and undergoing short gynecologic operations of similar duration under methohexitalnitrous oxide-oxygen anesthesia. Each patient received P6 acupuncture for 5 minutes at the time of administration of premedication. In random order the site of the acupuncture had been previously infiltrated with normal saline solution in half of the patients and 1% lidocaine in the remaining patients. Postoperative emetic sequelae occurred significantly more often in those who received lidocaine compared with the group that received saline solution. This demonstrates the ability of a local anesthetic administered at the point of stimulation to block the antiemetic action of P6 acupuncture in a manner similar to that shown by others for analgesia.
Immunolocalization of the Bcl-2 protein within hematopoietic neoplasms. The Bcl-2 proto-oncogene was discovered at the t(14;18) breakpoint found in most follicular B-cell lymphomas and some diffuse large-cell lymphomas. Bcl-2 is unique among proto-oncogenes, being localized to mitochondria and extending cell survival by blocking programmed cell death. We examined Bcl-2 protein expression in 82 hematologic malignancies and reactive lymphoid processes. All lymphomas with Bcl-2 rearrangement demonstrated high levels of Bcl-2 protein. However, most follicular and diffuse lymphomas without Bcl-2 rearrangement also displayed intense Bcl-2 staining. In these cases, mechanisms other than classic translocation may be deregulation Bcl-2. The pattern of Bcl-2 staining in follicular lymphoma is the inverse of the pattern in reactive hyperplasia, confirming a role for Bcl-2 immunolocalization in routine diagnosis. Small lymphocytic malignancies, including small lymphocytic lymphoma, mantle zone lymphoma, and chronic lymphocytic leukemia, expressed intermediate levels of Bcl-2. Bcl-2 protein varied in plasma cell dyscrasias. Bcl-2 protein levels in T-cell lymphomas reflected their corresponding stage of development. No substantial Bcl-2 was present in the Reed-Sternberg cells of nodular sclerosing Hodgkin's disease. Chronic myelogenous leukemia was strongly positive for Bcl-2, consistent with the presence of Bcl-2 in normal myeloid progenitors. Immunohistochemistry identified an expanded spectrum of hematopoietic neoplasms in which Bcl-2 may provide a cell survival advantage.
Intestinal lymphonodular hyperplasia of childhood: patterns of presentation. In this retrospective analysis we searched for a constellation of signs or symptoms attributable to childhood lymphonodular hyperplasia (LNH). Of 147 children with documented LNH reviewed, 43% had lesions in the small bowel, and 57% in the large bowel. Children in this study presented with complaints of abdominal pain (58%) and bright red blood per rectum (32%). Physical examination revealed little except right lower quadrant (RLQ) abdominal tenderness and "fullness" in 35%. The pain was periumbilical, dull-cramping, rarely acute, and nonradiating. The hematochezia was most commonly streaky red in mucoid strands adhering to the stools, with no associated tenesmus. Three clinical patterns emerged: (a) Under 1 year of age most patients were male, with painless bleeding and pancolonic LNH. (b) Between 2 and 6 years, although the LNH was predominantly colonic, pain and bleeding occurred equally. (c) From 7 years old on, the main symptom was abdominal pain, but LNH distribution was nearly equal between the small bowel and the colon. To date, our long-term follow-up of the children with isolated LNH has revealed no sequelae.
Gastric carcinoids and their precursor lesions. A histologic and immunohistochemical study of 23 cases. A histologic and immunohistochemical study was carried out in 23 unselected nonantral gastric carcinoids and their precursor lesions classified according to Solcia et al. None of the patients showed Zollinger-Ellison syndrome. Two variants of carcinoids showing distinctive pathologic and pathogenetic characteristics were identified on the basis of presence or absence of associated chronic atrophic gastritis type A (A-CAG). Chronic atrophic gastritis type A was found in 19 cases showing either single or multiple neoplasms, tumor extension limited to the mucosa or submucosa, consistent endocrine cell precursor changes in extratumoral mucosa, and consistent hypergastrinemia and/or G cell hyperplasia. Associated precursor lesions were only hyperplastic in all but two cases with single carcinoids whereas they were also dysplastic in all but one case with multiple carcinoids. The four tumors arising in nonatrophic mucosa were all single, more aggressive, and not associated with extratumoral endocrine cell proliferations or with signs of gastrin hypersecretion. Tumor cells were diffusely immunoreactive for chromogranin A and synaptophysin but usually negative for chromogranin B or HISL-19. Scattered serotonin cells were found in ten carcinoids. They were more frequent in infiltrating than in intramucosal tumors as were the less represented pancreatic polypeptide cells whereas the reverse was found for alpha-subunit-containing cells. These results are of relevance for tumor pathogenesis and may provide the rationale for a less aggressive therapeutic approach in the patients.