shukoor65/lab-rag-model huggingface dataset:
Partial hypoparathyroidism. A variant of transient congenital hypoparathyroidism. We encountered three children who had neonatal hypocalcemia followed by a period of normocalcemia and recurrence of hypocalcemia later in childhood. They were full-term infants with normal birth weights who developed hypocalcemia within the first 48 hours after birth. The hypocalcemia resolved in 1 week, 3 months, and 14 months in the three patients. The recurrences of hypocalcemia occurred at 4, 7, and 12 years of age. Their plasma parathyroid hormone concentrations were consistently low but detectable. We suggest that partial hypoparathyroidism is the underlying abnormality in these patients and that neonatal hypocalcemia in otherwise healthy infants indicates the need for calcium measurements during childhood and adolescence.
Comparison of the aortic homograft and the pulmonary autograft for aortic valve or root replacement in children. To assess late results of aortic homograft and pulmonary autograft valves implanted into the left ventricular outflow tract of children, we reviewed the case histories of 146 patients 18 years of age or younger who underwent aortic valve or root replacement between November 1964 and April 1990. One hundred three patients (mean, 12 +/- 3.9 years) received an aortic homograft and 43 (mean, 14 +/- 4.1 years) had their own pulmonary valve transferred to the aortic position. There were 54 valve and 49 root replacements with homografts and 36 valve and seven root replacements with autografts. Hospital mortality rate was 15.5% (16 patients) in the homograft group and 11.6% (five patients) in the autograft group. Survivors were followed up for a total of 867 (homograft) and 297 (autograft) patient-years. The late mortality rate was 16.7% (1.9% per patient-year) for patients with homografts and 13.2% (4.4% per patient-year) for patients with autografts, whereas the incidence for reoperation per patient-year was 2.9% and 2.0%, respectively. At 15 years actuarial rates for homografts and autografts for freedom from reoperation were 54% +/- 8.1% and 68% +/- 11.1%; freedom from endocarditis, 97% +/- 2.4% and 75% +/- 10.2%; and freedom from any complication, 41% +/- 6.5% and 50% +/- 10.3%. Valve degeneration occurred in 19 homografts (2.2% per patient-year), whereas there was no definite instance of primary tissue failure among the pulmonary autografts. This experience would indicate that either the homograft or the autograft valve can be used with acceptable results in children. However, the pulmonary autograft gives better long-term performance and, if growth potential is realized, may be the ideal valve substitute in children.
The relationship of sodium balance and concomitant diuretic therapy to blood pressure response with calcium channel entry blockers. Marked heterogeneity exists in the response of blood pressure to specific antihypertensive agents. In general, a single therapeutic agent can be expected to provide an adequate blood pressure response in 40% to 60% of hypertensive patients. The addition of a second agent usually extends the response rate to over 85%. The addition of a diuretic to nondiuretic regimens often produces an additive blood pressure-lowering effect. However, calcium channel entry blockers (CCBs) may be an exception, as diuretics do not appear to improve these agents' efficacy. CCBs have a dual mechanism for blood pressure reduction. In addition to their vasodilatory actions, they also promote diuresis and natriuresis. These features account for the striking efficacy of these agents when used as monotherapy in hypertensive patients. Studies have shown that dietary sodium restriction does not appear to improve the efficacy of CCBs, nor is a high-salt diet predictive of a better therapeutic outcome. Recent studies also indicate that the addition of a diuretic to a CCB does not produce a significant or consistent additive antihypertensive effect. Because CCBs do not produce the typical responses of the renin-angiotensin-aldosterone (RAA) system to sodium/volume depletion, their diuretic and natriuretic effects do not result in the same adverse metabolic consequences as experienced with traditional diuretic therapy.
Fibrin fragment D-dimer and fibrinogen B beta peptides in plasma as markers of clot lysis during thrombolytic therapy in acute myocardial infarction. The validity of markers in plasma of in vitro thrombolysis was investigated in 12 patients with extensive fibrinogen breakdown (greater than 80%, group 1) and in 12 patients with minimal breakdown (less than 20%, group 2). The patients were treated with 100 mg of recombinant tissue-type plasminogen activator (rt-PA) in the "Thrombolysis in Myocardial Infarction II" (TIMI II) trial. Cross-linked fibrin degradation product levels were measured with two variant enzyme-linked immunosorbent assays (ELISAs), both using a fibrin fragment D-dimer specific capture antibody. In one instance, a tag antibody was used that cross-reacts with fibrinogen (pan-specific tag ELISA); in the other, the tag antibody was specific for fibrin fragment D (fibrin-specific tag ELISA). Apparent concentrations of cross-linked fibrin degradation products at baseline were within normal limits with both assays in most patients. At 8 hours after rt-PA infusion, the measured cross-linked fibrin degradation products were increased about twofold to fourfold in group 2 with both assays. However, in group 1, levels were significantly higher with the pan-specific tag ELISA (5.8 +/- 4.2 micrograms/mL) compared with the fibrin-specific tag ELISA (1.5 +/- 1.3 micrograms/mL). This observation was most likely a result of detection of fibrinogen degradation products in the pan-specific ELISA. Apparent levels of fibrinopeptide B beta 1-42, a marker of fragment X formation, increased during thrombolysis from 4.2 +/- 2.8 pmol/mL to 2,000 +/- 230 pmol/mL in group 1 and from 4.1 +/- 2.1 pmol/mL to 300 +/- 43 pmol/mL in group 2, and were correlated significantly with the extent of fibrinogen breakdown (r = -0.8). Fibrinopeptide beta 15-42 levels increased from 4.3 +/- 3 pmol/mL to 70 +/- 19 pmol/mL in group 1, but did not increase in group 2. The apparent increase in group 1 could be explained by cross-reactivity of fibrinopeptide B beta 1-42 in the fibrinopeptide beta 15-42 assay. We conclude that cross-linked fibrin degradation product levels as measured with a pan-specific tag ELISA and fibrinopeptide beta 15-42 levels as measured with certain monoclonal antibody-based ELISA are influenced by the extent of fibrinogen degradation. Fibrinopeptide B beta 1-42 is a marker specific for fibrinogen breakdown. Cross-linked fibrin degradation product levels, measured with a fibrin-specific tag ELISA, appear to be markers specific for thrombolysis. Consequently, assays similar to the fibrin-specific tag ELISA may provide more accurate information when correlated with clinical endpoints.
Management of labyrinthine fistulas caused by cholesteatoma. The surgical management of labyrinthine fistulas caused by cholesteatoma remains controversial. Forty cases (41 ears) of labyrinthine fistulas were reviewed. This represented 10% of our total series of cholesteatomas in adults and children (426 ears). Clinical presentation, extent of disease, results of fistula testing and audiometric studies, and radiographic findings were analyzed. A canal wall-down procedure was performed in all but one patient. Generally an attempt was made to completely remove the cholesteatoma, to graft the fistulous area, and to reconstruct the middle ear mechanism in one stage. The matrix was preserved in patients with large fistulas where the involved ear was the only hearing one, when the matrix was adherent to the underlying optic duct, and in selected elderly persons. Long-term followup did not reveal a significant difference in hearing, degree of vertigo, or incidence of recidivism when those patients in whom the matrix was removed were compared with those in whom the matrix was preserved. The importance of recognizing the presence of a labyrinthine fistula preoperatively is stressed, along with the need to be prepared for an unexpected fistula. Operative management is described.
Catecholaminergic neurons in the parabrachial nucleus of normal individuals and patients with idiopathic Parkinson's disease. The parabrachial nucleus is believed to play a role in autonomic regulation. We have used the Fontana-Masson ammoniacal silver nitrate method and a tyrosine hydroxylase-immunostaining technique to demonstrate the presence of neuromelanin-containing catecholaminergic neurons in the parabrachial nucleus of normal individuals. In addition, we also show that there is a significant reduction of these catecholaminergic neurons and presence of Lewy bodies in the parabrachial nucleus of patients with idiopathic Parkinson's disease. These findings may be related to the several autonomic disturbances that may occur in idiopathic Parkinson's disease.
A loading dose/continuous infusion schedule of fludarabine phosphate in chronic lymphocytic leukemia. Using a loading dose/continuous infusion schedule, fludarabine phosphate was administered to 51 patients with previously treated chronic lymphocytic leukemia (CLL). All patients had evidence of active disease, and the majority had advanced Rai stages. Of the 42 patients assessable for response, 22 (52%) achieved a partial response, five (12%) had stable disease, and 15 (36%) progressed. Thirteen of the 22 responders improved their Rai stages with fludarabine therapy, including six patients who achieved stage 0. Response rates for pretreatment stages III and IV were 60% and 53%, respectively. Patients with final Rai stages 0 to II had better survival than those with stages III and IV. Patients who had undergone splenectomy before starting therapy were more likely to respond. Myelosuppression was the primary toxicity and did not appear to be cumulative. Severe leukopenia and thrombocytopenia, although infrequent, were associated with several deaths in the early cycles of treatment. Nonhematologic toxicity was mild with no serious neurotoxicity noted. Infections were common with 22 minor, 18 major, and 10 fatal episodes. Fludarabine phosphate by this alternative dosing schedule is effective in refractory advanced CLL and is well tolerated by the majority of patients.
Response to tumor necrosis factor in two cases of psoriasis. A 70-year-old woman was treated for renal cell carcinoma with recombinant human tumor necrosis factor, 450,000 U/m2 of body surface area, daily for 5 days, once a month. She had had psoriasis for 25 years. After two courses of recombinant human tumor necrosis factor her psoriasis resolved. A 40-year-old man with severe psoriasis was subsequently treated with a total of seven complete and incomplete 5-day courses of recombinant human tumor necrosis factor at doses of 50,000 to 300,000 U/m2/day. Significant toxicity (fever, chills, hypertension, and hypotension) was encountered. Partial resolution of the lesions was seen. Tumor necrosis factor is an active agent in psoriasis.
The effects of adenotonsillectomy on growth in young children. A history of poor weight gain can often be elicited in young children with chronic upper airway obstruction resulting from adenotonsillar hypertrophy. A series of 41 consecutive children under 3 years of age, who underwent inpatient adenotonsillectomy, were reviewed for changes in weight and height. Thirty-seven patients had adequate long-term follow-up. Of these, many had dramatic improvements in growth after adenotonsillectomy. Indications for surgery in this group were recurrent infection in three patients (7%), unilateral tonsillar mass in one patient (3%), and upper airway obstruction in 37 patients (90%). A clear history of sleep apnea was elicited in 59%. At the time of surgery, 19 of 41 patients (46%) were of the fifth percentile or lower for age-corrected weight. The inpatient hospital stay averaged 3.2 days. The postoperative complication rate was 27%, with postoperative stridor as the most common complication. After surgery, 28 children (75%) showed a change to a higher percentile for weight. Twenty-four (65%) had percentile changes of 15% or more. This change is significant according to results of the Wilcoxon signed-rank test (p less than 0.001). We conclude that a relationship exists between improved growth rate and adenotonsillectomy in our study group. The rapid improvement in growth appears to be most obvious in children with upper airway obstruction resulting from adenotonsillar hypertrophy. Upper airway obstruction (including andenotonsillar hypertrophy) should be suspected as a possible cause in the workup of children with suboptimum growth.
Oral almitrine in treatment of acute respiratory failure and cor pulmonale in patients with an exacerbation of chronic obstructive airways disease. The effects of oral almitrine bismesylate, a respiratory stimulant that acts on peripheral arterial chemoreceptors, was studied in patients with chronic obstructive airways disease and hypoxaemic cor pulmonale. Twenty three patients admitted to hospital with an acute exacerbation of ventilatory failure were randomised to receive either almitrine 100 mg twice a day reducing to 50 mg twice a day over 48 hours or placebo in addition to conventional treatment. On admission the mean (SE) values for blood gas tensions were PaO2 4.8 (0.3) and PaCO2 7.7 (0.3) kPa in the 12 patients who received almitrine and PaO2 4.9 (0.1) and PaCO2 7.6 (0.3) kPa in the 11 who received placebo. After three hours of oxygen therapy at 1 1/min there was a similar rise in PaO2 in both groups, 6.4 (0.2) kPa in those receiving almitrine and 6.6 (0.4) kPa in those receiving placebo. After 24 hours of oxygen therapy values of PaO2 were again similar at 6.3 (0.8) kPa and 6.7 (2.2) kPa respectively. Arterial blood gas tensions improved during the study in those who survived but no significant differences were apparent between the two groups. There were six deaths, five in the almitrine group and one in the placebo group. There were no differences between the groups in respiratory rate, results of spirometry, oxygen requirement, or degree of dyspnoea (on visual analogue scale). The results did not show any benefit from oral almitrine in patients with acute respiratory failure secondary to chronic obstructive airways disease. Plasma almitrine concentrations, however, were often below the optimum therapeutic range, suggesting impaired drug absorption.
Medication-induced esophageal injury: report of 17 cases with endoscopic documentation. We report on medication-induced esophageal injury (MIEI) in 17 patients (six male, 11 female) seen from October 1986 to May 1990. The mean age of patients was 27.3 (SD = 5.7) yr; mean duration of drug ingestion prior to the occurrence of symptoms 10.2 (SD = 11.5) days, and mean duration of symptoms before seeking medical attention 4.6 (SD = 3.8) days. Symptoms subsided after treatment, with a mean of 6 (SD = 2.5) days. Symptoms included odynophagia (in 17), chest pain (six), epigastric pain (three), and retrosternal pain (one). Symptoms occurred after the drug was stopped in three. MIEI was caused by doxycycline (seven), minocycline (five), Pantozyme (one), cloxacillin (one), unknown (two), and dicloxacillin + Danzen (one). Reclining after drug ingestion was the predominant risk factor. Endoscopy showed most ulcers to be multiple and at midesophagus. Barium swallows done in two patients were negative. There is no previous report of Pantozyme (pancreatic enzyme), Danzen (serratio-peptidase), cloxacillin, and dicloxacillin causing MIEI.
Human interleukin-9: genomic sequence, chromosomal location, and sequences essential for its expression in human T-cell leukemia virus (HTLV)-I-transformed human T cells. We have isolated the genomic sequence of human interleukin-9 (IL-9) based on its sequence homology with a human IL-9 cDNA isolated from human T-cell leukemia virus (HTLV)-I-transformed T cells by expression cloning. The entire genomic sequence has been determined and the gene consists of five exons and four introns. The human IL-9 gene is mapped to the long arm of human chromosome 5 at band 5q31-32, a region found to be deleted in a number of patients with acquired 5q- abnormalities and hematologic disorders. Several blocks of transcriptional control sequences have been identified at the 5'-flanking region of the human IL-9 gene that may play an important role in the control of IL-9 gene expression. The 5'-regulatory region of the human IL-9 gene also contains sequences identified in the 5'-flanking regions of other cytokine genes mapped to the long arm of human chromosome 5, including IL-3, IL-4, IL-5, and granulocyte-macrophage colony-stimulating factor and other T-cell growth factor genes including IL-2 and IL-6. The IL-9 gene is constitutively expressed in the HTLV-I-transformed human T cells and the expression of IL-9 in these cells can be further induced by 12-O-tetradecanoyl phorbol 13-acetate. Transient transfection analysis using the plasmid containing the 5'-flanking region of IL-9 gene upstream from the firefly luciferase ciferase report gene indicated that the 0.9-kb Smal-Sacl fragment of the IL-9 gene contains sequences required for the constitutive and activated expression of IL-9 gene in HTLV-I-transformed cells. These results will now allow us to study the regulatory mechanism of IL-9 gene expression in normal and leukemic human T cells.
Randomized controlled trial of prophylactic rifampin for peritoneal dialysis-related infections. Staphylococcal infections are a major cause of catheter infections and peritonitis in peritoneal dialysis patients. Since catheter-related infections are associated with nasal carriage of Staphylococcus aureus in this population, we studied the effect of intermittent rifampin, an antibiotic known to decrease S aureus nasal carriage, on catheter-related infections and peritonitis. We randomly assigned 64 patients to receive either rifampin 300 mg twice daily for 5 days every 3 months or no treatment. The rifampin-treated patients had a significant delay in time to first catheter-related infection (P less than 0.015) and significantly fewer catheter-related infections overall (P less than 0.001). The catheter-related infection rate in rifampin-treated patients was .26 per patient-year versus .93 per patient-year in untreated patients. Multivariate analysis defined baseline colonization of nares or catheter exit-site and prior renal transplant as risk factors for catheter-related infections. There was no significant difference in peritonitis rates between groups, although the trend was for a delayed time to first episodes and fewer episodes in rifampin-treated patients. Adverse effects necessitated withdrawal of rifampin in four patients. We conclude that intermittent rifampin administration is effective in decreasing catheter-related infections in a peritoneal dialysis population.
Dissecting the primary causes of genetic hypertension in rats. Blood pressure has a genetic component influenced by several to many genetic loci in both humans and animals; that is, blood pressure is a polygenic trait. Ultimately, the primary causes of genetic hypertension can only be established with genetic techniques. The primary tools for this analysis in animals are inbred strains of rodents selectively bred for differences in blood pressure and the genetic analysis of these strains by cosegregation techniques. This analysis determines whether it is possible to separate specific alleles at a candidate genetic locus from a component of blood pressure in genetically segregating populations. If the candidate alleles cosegregate with a component of blood pressure, these alleles must be the cause of the blood pressure differences or be linked on the same chromosome to alleles at other loci that cause blood pressure differences. If, on the other hand, the candidate alleles do not cosegregate with blood pressure, they cannot be the cause of the blood pressure differences. This analysis is straightforward in the case of a single-locus Mendelian candidate trait but is less informative if a candidate trait is itself polygenic. In this case, genetic analysis yields either 1) results that are compatible with, but not definitive proof for, a genetic role of the trait in causing blood pressure differences or 2) results that eliminate the trait as a cause of genetic differences in blood pressure. A complete listing of all the traits that have undergone genetic cosegregation analysis in rodents is given.
The diagnosis of reflex sympathetic dystrophy using an algometer. Thirty-three patients with reflex sympathetic dystrophy were studied prospectively to ascertain the pressure-pain threshold of affected and unaffected limbs. The affected side had a lower threshold which was found to be statistically significant. In all 18 patients with upper limb involvement, the pain threshold was reduced on the affected side, but this applied to only 11 of the 15 with leg involvement. This difference may be because patients with lower limb symptoms had been referred later in the course of the syndrome. We showed by repeated tests that after an average of 49 days there was a slow return to normality. The estimation of pressure-pain thresholds may help in the earlier diagnosis of reflex sympathetic dystrophy.
Mesh-constricted varicose and dilated veins used as arterial bypass grafts. To avoid non-autogenous vascular prostheses in coronary and peripheral vascular procedures, otherwise unusable dilated, varicose or thin-walled veins can be implanted as naturally endothelialized grafts after being calibrated by insertion into mesh tubes. In six sheep the 14 mm diameter jugular vein was inserted into a 12 cm long, 6 mm diameter Dacron mesh tube and implanted as a femoropopliteal graft. On the contralateral side the femoral vein with a maximal diameter of 7 to 8 mm was implanted. At control angiography after two months all grafts were patent, none of the wrapped grafts showed wrinkles or signs of anastomotic hyperplasia. Segments of wrapped veins were casted in paraffin under physiological pressure and cut transversely. In all cases the vein wall was pressed against the mesh tubes without forming folds. Mesh-wrapped varicose veins were used for three femorocrural and seven femoropopliteal reconstructions. All grafts showed a smooth flow surface at control angiography. Indentations occurred only at sites where thick-walled side branches had been ligated rather than oversewn. One popliteo-pedal reconstruction occluded after two months and one patient with a crural reconstruction died two months after surgery with a patent graft. The remaining grafts were patent after 11 (2-32) months. Oversized veins can be considerably constricted by tubes without forming wrinkles. By this technique varicose veins can be used as bypass grafts and in general vein grafts can be matched in size to the recipient vessel.