123rc/medical_text huggingface dataset:
Prevalence of inflammatory bowel disease in family members of Jewish Crohn's disease patients in Israel. A familial study of 189 Jewish Crohn's disease patients was conducted in order to evaluate the risk for inflammatory bowel disease (IBD) in relatives of the patients and to try to understand better the genetic component in the etiology of the disease among Jews. One hundred fifty-seven patients filled out questionnaires that were verified by personal interviews. In 10 families (6.6%), a first-degree relative of the propositus was found to have IBD, seven Crohn's disease and three ulcerative colitis. Among first-degree relatives, siblings were more frequently affected: of 400, five had Crohn's disease and one ulcerative colitis. Among 304 parents, two had Crohn's disease and two ulcerative colitis, while none of the propositi's children had IBD. The prevalence of first-degree relatives with Crohn's disease was similar in the 98 and 45 families of Ashkenazi and non-Ashkenazi origin: 5.1% and 4.4%, respectively. The risk for siblings of the probands to be affected were also similar in the two groups: 1.5% and 1.8%; while parents of the probands were affected only in the group of Ashkenazi Jews.
Congenital dilatation of the bile duct in 100 instances and its relationship with anomalous junction [published erratum appears in Surg Gynecol Obstet 1991 Mar;172(3):246] Congenital dilatation of the bile duct (CDBD) or choledochal cyst has been demonstrated to be associated with an anomalous junction of the pancreaticobiliary ductal system. Multifarious clinical signs and symptoms of CDBD have been shown to be closely related with the presence of this anomalous junction. In the present study, 100 instances of CDBD treated surgically at our institutions during a 30 year period were classified into two types according to the morphologic features of dilatation of the bile duct; there were 77 instances of the cystic type and 23 of the cylindric type. Morphologic features of the lesion, clinical signs and symptoms and laboratory findings in these 100 instances were clinically analyzed. In almost all of the patients who were less than one year of age, the disease was of the cystic type and patients presented with either a palpable mass or jaundice as the main symptom. In patients more than one year of age, the disease was of either the cystic or cylindric type. A history of episodes of characteristic abdominal pain accompanied by elevated levels of serum amylase was present in 70 of the patients with the cystic type of disease and in all of the patients with the cylindric type. Histologic sections from the patients showed glandular formation with chronic inflammation, possibly a result of refluxed activated pancreatic juice; in contrast, histologic sections from the remaining patients of all ages showed only thickening of the fibrous layer. Thus, such variable morphologic features and clinical signs and symptoms in CDBD are highly dependent on two factors--the age at onset and the reflux of pancreatic juice into the bile duct through the common channel.
Growth hormone for short stature not due to classic growth hormone deficiency. The advent of recombinant DNA technology has resulted in potentially unlimited supplies of growth hormone. Sufficient quantities are now available not only for the long-term, uninterrupted treatment of GH-deficient children but potentially for the treatment of non-GH-deficient patients with other short stature or growth attenuating disorders. Short-term studies have demonstrated an improvement in the growth rates of subjects with isolated short stature, Turner syndrome, and chronic renal failure; and additional studies are under way to assess the efficacy of GH therapy of other short stature syndromes. However, the long-term efficacy and possible adverse effects of GH treatment in these situations is not known. Until there has been more experience, GH deficiency should remain the primary indication for GH treatment. Growth hormone should not be considered routine therapy for other conditions associated with or resulting in short stature. However, research should continue in these areas to define which children may benefit from GH treatment.
The relapse of hairy cell leukemia after a 32-year remission. The optimum therapy for hairy cell leukemia (HCL) is controversial. Dramatic responses to interferon-alpha and 2'-deoxycoformycin overshadow the established role of splenectomy in HCL, and patients currently can avoid surgery altogether. A case is presented of clinical remission lasting 32 years after splenectomy--a prolonged normal life, without medication or medical intervention. When relapse occurred, the patient's response to interferon-alpha was prompt and predictable. Prognostic variables are an important consideration at the time of diagnosis in predicting the response to splenectomy.
Monoclonal antibody-purged autologous bone marrow transplantation therapy for multiple myeloma. Eleven patients with plasma cell dyscrasias underwent high-dose chemoradiotherapy and anti-B-cell monoclonal antibody (MoAb)-treated autologous bone marrow transplantation (ABMT). The majority of patients had advanced Durie-Salmon stage myeloma at diagnosis, all were pretreated with chemotherapy, and six had received prior radiotherapy. At the time of ABMT, all patients demonstrated good performance status with Karnofsky score of 80% or greater and had less than 10% marrow tumor cells. Eight patients had residual monoclonal marrow plasma cells and 10 patients had paraprotein. Following high-dose melphalan and total body irradiation (TBI) there were seven complete responses, three partial responses, and one toxic death. Granulocytes greater than 500/mm3 were noted at a median of 21 (range 12 to 46) days posttransplant (PT) and untransfused platelets greater than 20,000/mm3 were noted at a median of 23 (12 to 53) days PT in 10 of the 11 patients. Natural killer cells and cytotoxic/suppressor T cells predominated early PT, with return of B cells at 3 months PT and normalization of T4:T8 ratio at 1 year PT. Less than 5% polyclonal marrow plasma cells were noted in all patients after transplant. Three of the seven complete responders have had return of paraprotein, two with myeloma, and have subsequently responded to alpha 2 interferon therapy. Eight patients are alive at 18.9 (8.9 to 43.1) months PT and four remain disease-free at 12.3, 17.5, 18.9, and 29 months PT. This preliminary study confirms that high-dose melphalan and TBI can achieve high response rates without unexpected toxicity in patients who have sensitive disease, and that MoAb-based purging techniques do not inhibit engraftment. Although the follow-up is short- and long-term outcome to be determined, relapses post-ABMT in these heavily pretreated patients suggest that ABMT or alternative treatment strategies should be evaluated earlier in the disease course.
Choledochotomy for calculous disease in the elderly. To determine the risk of operations on the biliary tract in the elderly, we retrospectively examined 56 patients 80 years of age or older undergoing choledochotomy for calculous disease and compared their outcomes with those of a contemporary group of 257 patients undergoing similar surgery. Thirty of 56 elderly patients (54%) required urgent surgery for acute cholangitis, acute cholecystitis, or obstructive jaundice (serum bilirubin greater than 5 mg/dL), compared with 97 of 257 younger patients (38%) (p = 0.029). Mortality in elderly patients was significantly higher (5 of 56) than in younger (6 of 257) patients (p = 0.015). In three of the five patients who died, urgent surgery was performed for obstructive jaundice, reflecting the higher mortality experienced by jaundiced patients in the entire series (9.4%). Biliary cultures grew organisms in 25 of 27 elderly patients (93%). This is in contrast to findings of bactibilia in 62 of 118 patients (53%) under the age of 80 (p = 0.001). Similarly, preoperative blood cultures were positive more often in elderly (63%) than in younger patients (26%) (p = 0.007). Mean postoperative hospital recovery was longer in the elderly group (16.2 +/- 8.5 days) than in the younger group (12.2 +/- 9.3 days) (p = 0.002). In conclusion, elderly patients, particularly those with obstructive jaundice, are at higher risk for complicated biliary tract disease necessitating choledochotomy. They appear to be more susceptible to bactibilia and bacteremia, and require longer hospital recovery than younger patients.
A 5.3-kb deletion including exon XIII of the protein S alpha gene occurs in two protein S-deficient families. Genomic DNA samples from 12 protein S-deficient families with hereditary thrombophilia were analyzed by Southern hybridization using protein S cDNA probes. Protein S-deficient members of families A and B possessed identical restriction fragment length polymorphisms, which suggest the absence of 5.3 kb from one of their protein S alpha alleles. The abnormal alleles from individuals A7 and B1 were amplified by the polymerase chain reaction using a forward primer in intron K and a reverse primer in exon XIV. The amplified DNA was cloned and sequenced. Sequence comparison with the normal protein S alpha gene showed that most of intron L (roughly 4.7 kb), the entire exon XIII (151 bp), and about a quarter of intron M (407 bp) were missing from both the A7 and B1 clones. Exon XIII contains all three potential N-glycosylation sites in human protein S. This deletion may result in RNA transcripts in which exon XII is spliced to exon XIV. Such an arrangement would generate a stop codon at position 463 and consequently produce a nonglycosylated protein S molecule truncated by 173 amino acids.
Flosequinan: a vasodilator with positive inotropic activity. Flosequinan is an oral arterial and venous vasodilator that is currently under investigation for the treatment of congestive heart failure. The effects of flosequinan on ventricular performance and myocardial contractility were studied in 10 patients with severe congestive heart failure during right and left cardiac catheterization. Sixty minutes after a 100 mg oral dose of flosequinan, the peak rate of rise in left ventricular pressure (dP/dt) increased from 940 +/- 180 to 1050 +/- 240 mm Hg/sec (p less than 0.05), while left ventricular end-diastolic pressure decreased from 32 +/- 5 to 26 +/- 8 mm Hg (p less than 0.05), and cardiac index increased (2.1 +/- 0.4 to 2.3 +/- 0.5 L/min/m2, (p less than 0.05). The mean pulmonary artery pressure and vascular resistance decreased from 40 +/- 8 to 33 +/- 12 mm Hg (p less than 0.05) and from 330 +/- 240 to 290 +/- 170 dyne-sec/cm5 (p less than 0.05), respectively. Heart rate, mean aortic pressure, right atrial pressure, systemic vascular resistance, and serum norepinephrine levels did not change significantly. The increase in left ventricular peak dP/dt that was concomitant with a decrease in left ventricular end-diastolic pressure, and no change in systemic arterial pressure or sympathetic tone, argue for a direct positive inotropic effect of flosequinan.
Oral contraceptive therapy and the surgical management of ENT patients: a review of current clinical practice. The current policy of British Otolaryngologists with regard to the preoperative cessation of the oral contraceptive pill is reported. This is based on a confidential questionnaire sent to all members of the British Association of Otolaryngologists. The overall response rate was 66%, 91% of which were from practising otolaryngologists and forms the basis of this report, the remaining 9% being from respondents not engaged in active surgery. Although there is evidence to show an increased risk of developing thromboembolic complications after major abdominal, gynaecological and hip surgery in those patients taking the oestrogen-containing contraceptive pill, the risk following minor and intermediate surgery (which forms the bulk of the otolaryngologist's workload) is not known. Not surprisingly therefore the results of the survey show a varied policy across the country with 36.5% of respondents choosing to continue the pill and 25% always stopping the pill preoperatively. The remainder elect to stop the pill only in certain circumstances.
Helicobacter pylori infection: a simplified diagnostic approach. We evaluated the diagnostic accuracy of endoscopic finding of nodular antritis and rapid urease test (RUT) in order to simplify the approach to the diagnosis of Helicobacter pylori (H. pylori) infection. Forty-four consecutive patients (mean age 7.9 yr, range 6-13 yr) referred because of recurrent abdominal pain as the main symptom, were prospectively investigated for the presence of H. pylori. H. pylori positivity or negativity was defined as the concordance of two of the following tests: RUT, microbiologic culture, and histologic examination on bioptic samples. RUT sensitivity was 100%, whereas specificity was 87.5%. The presence of nodular antritis had a sensitivity of 96.4% and specificity of 87.5% in H. pylori infection diagnosis. The predictivity value of combined RUT and nodular antritis, whether positive or negative, was 100%. Only in case of discordance do we suggest the utilization of other expensive tools for diagnosis of H. pylori infection.
Human papillomaviruses: pediatric perspectives on a family of multifaceted tumorigenic pathogens As summarized here human papillomaviruses are associated with a wide spectrum of epithelial lesions, ranging from benign warts to invasive carcinomas. They have been difficult to study in part because they have not yet been propagated in tissue culture. Fortunately advances in molecular biology have allowed characterization of HPV genomes and identification of some HPV gene functions. In addition to their clinical importance HPVs represent an important tool for exploring virus-cell interactions, gene expression, cellular differentiation and cancer. HPV infections are not only common but also difficult to treat and prevent. Depending on the HPV type and location, the modes of HPV transmission may involve casual physical contact, sexual contact and perinatal vertical transmission. HPV DNA genomes replicate at a low copy number in basal cells and, as most clinicians know, are difficult to eradicate. There is often a long latent period and subclinical infections, and HPV DNA can be found in normal tissue adjacent to lesions. HPVs can cause widely disseminated lesions, especially in the immunocompromised host and in epidermodysplasia verruciformis. Aside from the rare carcinomas, the most serious life-threatening HPV-induced illness in children is recurrent respiratory papillomatosis. Somewhat surprisingly in malignant lesions HPV DNA is also found as fragments incorporated into the cellular genome. Unlike retroviruses such as human immunodeficiency virus which integrate into the cellular genome as part of their life cycle, HPV integration is a terminal event for viral replication. Such integration may be critical, however, for viral-induced abnormal cell growth. Perhaps the most important implication of the finding that some anogenital cancers are in part sexually transmitted infectious diseases is that they may be preventable. The data overwhelmingly suggest that avoidance of exposure to HPV via abstinence or monogamy in both partners markedly reduces the risk of cervical cancer. A more realistic goal, however is prevention of HPV transmission by the use of barrier method contraceptives, which may be protective against development of cervical carcinoma. The America Association of Pediatrics Committee on Adolescents has outlined the obligation of pediatricians to be actively involved in adolescent education on sexually transmitted diseases. Certainly a fundamental knowledge of HPV epidemiology, the risks of HPV-related sequelae and prevention of HPV infection are important considerations for adolescent sexuality. Although helpful, such awareness alone falls far short of making an impact on sexual behaviors. A significant reduction in HPV infection rates could be achieved only by inundating adolescents at an early age with a highly visible society-wide campaign directed at these issues.
Dietary preference for sweet foods in patients with dementia Using a telephone survey, patients with probable Alzheimer's disease (n = 31) and vascular dementia (n = 14) were compared with elderly normal controls (n = 43) in preferences for different foods. Patients with Alzheimer's disease had a greater preference than normal controls for relatively high-fat, sweet foods and for high-sugar, low-fat foods, but did not significantly differ in preference for other foods, including those high in complex carbohydrates and protein. Vascular dementia patients showed a similar pattern, not significantly different from that for Alzheimer's patients. Results did not consistently support a hypothesis that increased sweet preference is a nonspecific form of disinhibited behavior related to declining mental status, nor was a hypothesis relating sweet preference to serotonin activity within the brain consistently supported. Results provide preliminary evidence that craving for sweet food may be a significant part of the clinical syndrome of dementia, but further research is needed to delineate the psychological and biological mechanisms accounting for it.
Coronary stent implantation in acute vessel closure 48 hours after an unsatisfactory coronary angioplasty. We report the implantation of a balloon-expandable stent in a patient with acute vessel closure in the state of evolving myocardial infarction following 48 hr after unsatisfactory coronary angioplasty. The stent was implanted after successful recanalization of an occluded left anterior descending artery, with repeated unsatisfactory results of balloon angioplasty. Adjunct thrombolytic therapy was contraindicated. No residual stenosis was documented in immediate control angiograms, or after 24 hr, 3 weeks, and 4 months.
Preoperative measurement of pulmonary vascular resistance in complete transposition of the great arteries. Transposition of the great arteries is frequently complicated by the early onset of pulmonary vascular disease. It is difficult to measure pulmonary blood flow by the Fick principle because the pulmonary arteriovenous oxygen content difference is small and bronchial blood flow is increased in this condition. In eight patients (mean age 7.7 years, range 3 months to 29 years) with transposition of the great arteries mass spectrometry was used to measure oxygen uptake and predict pulmonary end capillary blood oxygen content. The effects of the bronchial circulation were studied by computer modelling. There was close agreement between pulmonary end capillary and pulmonary vein blood oxygen contents but the resultant percentage difference in arteriovenous content difference was significant (mean (SE of difference)) (14.5(3.8)%). The effect of the bronchial circulation was to give spuriously high estimates of pulmonary blood flow. The error was greatest when oxygen consumption was low and aortic blood was very desaturated.
Regional and systemic distribution of anti-tumor x anti-CD3 heteroaggregate antibodies and cultured human peripheral blood lymphocytes in a human colon cancer xenograft. Anti-tumor antibody (317G5) covalently coupled to an anti-CD3 antibody (OKT3) produces a heteroaggregate (HA) antibody that can target PBL to lyse tumor cells expressing the appropriate tumor Ag. The i.v. and i.p. distribution of radiolabeled HA antibody 317G5 x OKT3 and of radiolabeled cultured human PBL were studied in athymic nude mice bearing solid intraperitoneal tumor established from the human colon tumor line, LS174T. Mice were injected with 125I-labeled HA antibody, 125I-labeled anti-tumor mAb, or 111In-labeled PBL, and at designated timepoints tissues were harvested and measured for radioactivity. 125I-317G5 x OKT3 localized specifically to tumor sites. Tumor radioactivity levels (percent injected dose/gram) were lower with 125I-317G5 x OKT3 HA antibody than with 125I-317G5 anti-tumor mAb, but were similar to levels reported for other anti-tumor mAb. The major difference in radioactivity levels observed between i.v. and i.p. administration of 125I-317G5 x OKT3 was an increase in hepatic radioactivity after i.v. HA antibody administration. HA antibodies produced from F(ab')2 fragments, which exhibit decreased m. w. and decreased Fc receptor-mediated binding, demonstrated improved tumor:tissue ratios as compared to intact antibody HA. 125I-317G5 F(ab')2 x OKT3 F(ab')2 antibody levels were equivalent to intact HA antibody levels in tumor, but were lower than intact HA antibody levels in the blood, bowel, and liver. Tumor:bowel ratios (20:1 at 48 h) were highest when 317G5 F(ab')2 x OKT3 F(ab')2 was injected i.p. Autoradiography confirmed that anti-tumor x anti-CD3 HA antibodies localized specifically to intraperitoneal tumor; that i.p. administered HA antibodies penetrated tumor directly; and that i.v. administered HA antibodies distributed along tumor vasculature. Cultured human PBL distributed in moderate concentrations to intraperitoneal tumor when administered i.p., but not when administered i.v. The poor localization of i.v. injected PBL to tumor may reflect species disparity in homing receptors and/or endothelial ligands, a problem which may be overcome with a syngeneic model. These results suggest that regional therapy with HA antibodies and PBL may offer advantages over systemic therapy for initial clinical trials.
Acute osteomyelitis in children. Reassessment of etiologic agents and their clinical characteristics. One hundred thirty-five children with acute osteomyelitis were identified by chart review during a 7-year period, January 1, 1980, through December 31, 1986. Bacteriologic causes were detected in 75 (55%) of the patients. Staphylococcus aureus, Haemophilus influenzae type b, and Pseudomonas aeruginosa were identified in 34 (25%), 16 (12%), and eight (6%) children, respectively. Staphylococcus aureus occurred in all age groups, H influenzae type b occurred only in children younger than 3 years and was the number one cause of disease in this group. Pseudomonas aeruginosa occurred exclusively in children older than 9 years. Children with H influenzae type b had clinical and laboratory findings that were almost indistinguishable from a matched group of children with osteomyelitis due to other known bacteria, although children with H influenzae type b tended to have more joint effusions (63% vs 27%), less lower extremity disease (22% vs 70%), and fewer positive cultures from bone or joint aspirates (41% vs 89%). Unlike most pediatric cases of osteomyelitis, the ones due to P aeruginosa did not represent the hematogenous route of infection; penetrating injury to the foot was present in every case. Children with P aeruginosa infections were older than 9 years (100%), predominantly male (88%), often afebrile (83%), and never bacteremic. These data provide guidelines for the initial work-up and management of osteomyelitis in children.
Isotonic high-sodium oral rehydration solution for increasing sodium absorption in patients with short-bowel syndrome. We compared the effect of a standard oral rehydration solution and a high-sodium polymeric-glucose solution on sodium absorption in short-bowel syndrome. Six patients with high jejunostomy were tested in a random order with the standard solution or a solution containing maltodextrins (18 g Glucidex 12/L) enriched with 2.5 g NaCl/L. Solutions were administered via a nasogastric tube at a rate of 2 mL/min. Jejunal effluent was collected during an 8-h period. The net 8-h fluid absorption was not significantly different in the two periods. Glucose absorption was greater than 90% of the administered amount for both solutions. Net sodium absorption was greater for the maltodextrin solution than for the standard solution (56 +/- 12 vs 24 +/- 20 mmol, P less than 0.05). We conclude that replacement of glucose with maltodextrins and addition of sodium in the standard oral rehydration solution results in improved sodium absorption in short-bowel syndrome.