123rc/medical_text huggingface dataset:
Recurrent meningitis in a patient with congenital deficiency of the C9 component of complement. First case of C9 deficiency in Europe. We describe the first cases, to our knowledge, of C9 deficiency in Europe that were detected in a Swiss family, of which two members--one with a complete deficiency and the other with approximately half-normal C9 levels--experienced bacterial meningitis. The index patient, a 56-year-old white man with a history of purulent meningitis at the age of 23 years, presented with an acute meningococcal meningitis. No impairment of cellular immunity or immunoglobulin deficiency could be found. Complement assays showed a complete deficiency of the C9 component, while the other individual component levels were normal and the hemolytic activity (measured using the CH50 assay) was only slightly reduced. A family study revealed complete C9 deficiency in the patient's healthy brother and half-normal C9 concentrations in his sister, his son (who also had experienced an episode of bacterial meningitis), and his niece, consistent with an inherited C9 deficiency. This first case of recurrent meningitis in a white patient with complete C9 deficiency suggests that this complement defect may also be a risk factor for bacterial, especially neisserial, infections.
Colonic motor activity in acute colitis in conscious dogs. The changes in motor activity of the colon during acute colitis were investigated in six conscious dogs. The motor activity was recorded with seven strain-gauge transducers. Colitis was induced in the entire colon by luminal perfusion of acetic acid. The dogs exhibited urgency and diarrhea with mucus and blood during colitis. The mucosa was diffusely erythematous and friable and there were scattered ulcerations over the mucosal surface. The motor activity of the colon changed in several ways during colitis: (a) the total duration per hour and the mean duration of contractile states decreased significantly; (b) the cycle length of colonic migrating motor complexes was significantly prolonged, and the nonmigrating motor complexes were almost completely absent; and (d) the incidence of giant migrating contractions increased significantly. About half of the giant migrating contractions were followed by defecation. The remaining expelled mucus or gas. Sometimes, a migrating motor complex in the colon was also followed by defecation; this was never observed in the normal state. The motor activity of the colon was still decreased and the cycle length prolonged 21 days after induction of colitis. However, the dogs were asymptomatic at this time and the mucosa looked normal at colonoscopy. The incidence of giant migrating contractions was also normal at this time. It was concluded that the dog is a good model for the study of colitis because of the similarity of symptoms with human ulcerative colitis. The phasic contractions of the colon decreases during colitis but the incidence of giant migrating contractions is increased. The diarrhea in colitis may primarily be due to the large number of giant migrating contractions in the middle and the distal colon.
Sun-induced freckles in children and young adults. A correlation of clinical and histopathologic features. Sun-induced freckles are a risk factor for epidermal and melanocytic neoplasia. Whereas sun-induced freckles in children and older adults may be clinically indistinguishable, and sun-induced freckles in older adults usually consist of increased numbers of intraepidermal melanocytes, the histology of sun-induced freckles in children remains unsettled. Using L-3,4 dihydroxyphenylalanine (DOPA)-paraffin sections, the authors examined six sun-induced freckles and adjacent nonpigmented skin (ANP) in as many white male subjects, ages 10 to 23 years. Melanocyte frequency was expressed as the ratio of DOPA-reactive melanocytes to total epidermal basal unit cells. For each case, melanocyte frequencies in freckles were significantly greater than in ANP. Cellular atypia of melanocytes was noticed in four of six freckles. Reactivity of melanocytes for HMB-45 was noticed in two freckles studied, compared with no reactivity in three specimens of ANP studied. The authors conclude the sun-induced freckles in the young may consist of a hyperplasia of melanocytes (i.e., similar to solar lentigines in the elderly), sometimes with cellular atypia, and that these findings may be relevant to melanocytic neoplasia.
Site of action of continuous extrapleural intercostal nerve block. Continuous extrapleural intercostal nerve block has been shown in a randomized, controlled study to be effective in reducing postoperative pain after thoracotomy and in restoring pulmonary mechanics. To assess the extent of spread of bupivacaine infused through an extrapleurally placed cannula inserted at thoracotomy, iohexol (Omnipaque) was infused at 5 days postoperatively in 5 patients and computed tomography performed. The contrast medium was confined to the paravertebral space covering on average six intercostal spaces. This study demonstrated that anatomically, the site of action of the bupivacaine infused through an extrapleural cannula was primarily in the paravertebral space.
Endoscopic findings in Yersinia enterocolitica enterocolitis. The endoscopic findings in the colon and terminal ileum in eight cases of Yersinia enterocolitica enterocolitis infection were studied. The diagnosis was based on the isolation of Y. enterocolitica in the feces and/or elevated serum antibody titers to the organism. Total colonoscopy was performed between 7 and 38 days (mean, 24 days) after the onset of symptoms. In all patients, the terminal ileum was affected, followed by frequent involvement of the ileocecal valve and the cecum, and less frequently, the ascending colon. In the terminal ileum, round or oval elevations with or without ulcers were detected. Small ulcers were detected on the ileocecal valve and in the cecum. These findings were observed even 4 to 5 weeks after the onset of symptoms, suggesting a relatively long course for this disease.
Rectal examination in patients with pain in the right lower quadrant of the abdomen OBJECTIVE--To determine whether rectal examination provides any diagnostic information in patients admitted to hospital with pain in the right lower quadrant of the abdomen. DESIGN--Casualty officer or surgical registrar recorded symptoms and signs on admission on detailed forms. Final diagnosis was noted on discharge from hospital. SETTING--District general hospital. PATIENTS--1204 Consecutive patients admitted to hospital with pain in the right lower quadrant of the abdomen as their major complaint; 1028 had a rectal examination on admission. MAIN OUTCOME MEASURES--Odds ratio for each symptom and sign related to final diagnosis. Results of multiple logistic regression analysis for acute appendicitis. RESULTS--Right sided rectal tenderness, present in 309 of those examined, was more common in patients with acute appendicitis (odds ratio 1.34, p less than 0.05). This odds ratio was considerably less than that for other clinical signs--namely, tenderness in the right lower quadrant (odds ratio 5.09), rebound tenderness (3.34), guarding (3.07), and muscular rigidity in the abdomen (5.03). In the logistic regression analysis of patients with acute appendicitis, when allowance was made for the presence or absence of rebound tenderness, rectal tenderness on the right lost its significance. Six patients had masses palpable rectally, of which three were palpable on abdominal examination; the other three patients had acute appendicitis. No other unexpected diagnoses were established, and no useful additional diagnostic information was obtained by routine rectal examination. CONCLUSION--If patients presenting with pain in the right lower quadrant of the abdomen are tested for rebound tenderness then rectal examination does not give any further diagnostic information.
Ileal pouch-anal anastomosis. Reoperation for pouch-related complications. The aim was to assess the value of reoperative surgery for pouch-related complications after ileal pouch-anal anastomosis (IPAA) for chronic ulcerative colitis and familial adenomatous polyposis. Between January 1981 and August 1989, 114 of 982 IPAA patients (12%) seen at the Mayo Clinic had complications directly related to IPAA that required reoperation. Among the 114 patients, the complications prevented initial ileostomy closure in 33 patients (25%), occurred after ileostomy closure in 68 patients (60%), and delayed ileostomy closure in the remaining patients. The salvage procedures performed included anal dilatation under anesthesia for anastomotic strictures, placement of setons and/or fistulotomy for perianal fistulae, unroofing of anastomotic sinuses, simple drainage and antibiotics for perianal abscesses, abdominal exploration with drainage of intra-abdominal abscesses with or without establishment of ileostomy, and complete or partial reconstruction of the reservoir for patients with inadequate emptying. None of the reoperated patients died. Reoperation led to restoration of pouch function in two thirds of patients and, of these, 70% had an excellent clinical outcome. However approximately 20% of the 114 pouches required excision. Excision was common, especially among patients who had pelvic sepsis. Salvage procedures for pouch-specific complications can be done safely and will restore pouch function in two thirds of patients. Complications after reoperation, however, may ultimately lead to loss of the reservoir in one in five patients.
Ocular complications of rheumatic diseases of childhood. The association between inflammatory ocular and articular disease is a strong one, although the pathogenesis of this relationship is obscure. For the physician or surgeon caring for children with chronic arthritis, awareness of the risk of chronic asymptomatic anterior uveitis is essential if early diagnosis, treatment, and prevention of visual impairment are to be accomplished. This is particularly true for children with pauciarticular-onset juvenile rheumatoid arthritis or psoriatic arthritis. Acute symptomatic anterior uveitis is characteristic of disorders such as ankylosing spondylitis. Whereas this disease is initially more unpleasant for the child, it has a good long-term prognosis.
Nuclear DNA profiles in primary melanomas and their metastases. DNA-aneuploid primary melanomas are said to show a greater propensity to metastasize than those without an aneuploid profile. A higher aneuploid rate has also been reported in metastatic melanomas than in primary lesions. The ploidy profile was determined of 26 primary melanomas and their first subsequent secondaries on Feulgen-stained sections from routinely processed material using a computerized video image-analysis system. In 18 of 26 cases, both primary and secondary tumor showed similar corresponding DNA patterns. These were aneuploid in 13 of 18 of the cases. A hyperdiploid pattern was present in one case in both the primary and its metastasis, and a diploid pattern was seen in four cases. There were only eight cases in which a disparity between the nuclear ploidy profile in the primary and secondary tumor was evident. Although the results suggest that ploidy studies are not useful for predicting the metastatic potential of a primary melanoma, the maintenance of similar DNA profiles in the metastases in most of these cases was interesting in regard to tumor biology.
Relation between lipids and atherosclerosis: epidemiologic evidence and clinical implications. Coronary artery disease (CAD) is the leading cause of death in most developed countries. Therefore, elucidation of risk factors and associated mechanisms for CAD has been a high priority. Data from the Framingham Heart Study and other large-scale epidemiologic studies have identified major risk factors associated with CAD, demonstrating the adverse effects of increased total and low-density lipoprotein cholesterol levels and the protective effect of high-density lipoprotein cholesterol. Other more recent investigations, including the Lipid Research Clinics Trial and the Helsinki Heart Study, have shown that lowering total cholesterol and raising high-density lipoprotein cholesterol significantly reduce the risk for CAD. Because hypertension is also a significant risk factor for CAD, the assumption has been that blood pressure reduction should offer significant benefits in terms of CAD risk. However, despite their antihypertensive efficacy, diuretics and beta blockers have failed to significantly reduce CAD morbidity or mortality. The adverse effects of these antihypertensive agents on lipid profiles, glucose metabolism and other metabolic parameters may account for their disappointing performance in reducing CAD morbidity and mortality. As a result, newer agents such as angiotensin-converting enzyme inhibitors and calcium antagonists that appear to be free of such adverse effects have garnered considerable attention for their potential to reduce CAD risk.
Physical fitness and cardiovascular regulation: mechanisms of orthostatic intolerance. We studied three groups of eight men each--high, mid, and low fit (peak O2 consumption 60.0 +/- 0.8, 48.9 +/- 1.0, and 35.7 +/- 0.9 ml.min-1.kg-1)--to determine the mechanism of orthostatic intolerance in endurance athletes. Tolerance was defined by progressive lower body negative pressure (LBNP) to presyncope. Maximal calf vascular conductance (Gmax) was measured. The carotid baroreflex was characterized using both stepwise R-wave-triggered and sustained (2 min) changes in neck chamber pressure. High-fit subjects tended to have lower LBNP tolerance than mid- and low-fit subjects but similar baroreflex responses. Subjects with poor LBNP tolerance had larger stroke volumes (SV) (120 +/- 6 vs. 103 +/- 3 ml) and greater decline in SV with LBNP to -40 mmHg (40 +/- 2 vs. 26 +/- 4%). Stepwise multiple linear regression analysis revealed that Gmax and steady-state gain of the carotid baroreflex contributed significantly toward explaining interindividual variations in LBNP tolerance. Thus endurance athletes may have decreased LBNP tolerance, but apparently not as a simple linear function of aerobic fitness. Orthostatic tolerance depends on complex interactions among functional characteristics that appear both related (Gmax and SV) and unrelated (baroreflex function) to fitness or exercise training.
Prognostic factors in yolk sac tumors of the ovary. A clinicopathologic analysis of 29 cases. Twenty-nine ovarian cancer patients with yolk sac tumors and germ cell tumors with yolk sac tissue as a component of their disease (16 endodermal sinus tumor, 11 mixed germ cell tumors, one embryonal carcinoma, and one polyembryoma) were treated with cytoreductive surgery and combination chemotherapy. Prognostic factors were investigated in this group. Patients with Stage I disease had a more favorable prognosis (P less than 0.003) than those with Stages II and IV disease. The difference in prognosis was significant in cases where residual tumor was absent (P less than 0.003) and in cases where ascites was either absent or less than 100 ml in volume (P less than 0.05). Endodermal sinus tumor with either an intestinal (P less than 0.05) or microcystic pattern (P less than 0.01) was more common in survivors than in those who died. The age, preoperative serum alpha-fetoprotein level, maximum tumor size, and tumor weight had no significant correlation with prognosis. In advanced cases, chemotherapy regimens including cisplatin gave better results than those containing vincristine, dactinomycin, and cyclophosphamide (P less than 0.05). The optimal treatment of yolk sac tumors or tumors with yolk sac tissue as a component of the ovary is discussed in light of these results.
Lack of gene deletion for complement C4A deficiency in Japanese patients with systemic lupus erythematosus. The frequency of C4A gene deletion was studied in Japanese patients with systemic lupus erythematosus (SLE) and was compared with healthy controls. DNA preparations were extracted from peripheral blood leukocytes from 59 patients with SLE and from 166 healthy persons, and digested by restriction enzymes. They were hybridized with C4 complementary DNA by the Southern blotting method and the deletion of C4A gene was judged from restriction fragment length polymorphism. At the same time phenotypic C4A deficiency (C4AQ0) was measured. Our results showed that the frequency of phenotypic C4A deficiency was 44.1% in Japanese patients with SLE and this value was comparable with that (43.2%) in Caucasian patients. On the other hand the deletion of C4A gene was not found in Japanese patients with SLE (0%), or in healthy controls (0.6%). Our results indicate that C4AQ0 may contribute to the pathogenesis of SLE beyond the ethnical differences but Japanese patients with SLE have a different genetic background from Caucasian patients with the C4A gene deleted.
Epidermoid cyst (monodermal teratoma) of the testis. We report 14 patients with epidermoid cyst of the testis (monodermal teratoma). In 7 patients (5 treated within the last 5 years) the mass was excised and adjacent testicular tissue was biopsied. Seven patients underwent radical inguinal orchiectomy. Carcinoma in situ was not detected in any testicular tissue examined. There was no evidence of tumour recurrence in any patient after a mean follow-up of 10 years. Ultrasonographic appearance was not specific for a diagnosis of epidermoid cyst and exploratory surgery was required in all cases. Excision of the tumour and biopsy of adjacent testicular tissue to determine the presence or absence of carcinoma in situ is adequate treatment for this rare testicular neoplasm.
Clinical interaction of salt and weight change on blood pressure level. Studies that examine the effect of altered body weight or dietary sodium on blood pressure level are reviewed. Emphasis is placed on studies that compare the effects of weight reduction or sodium restriction in separate comparison groups or analyze the independent effect of the two intervention modalities in multiple regression analysis. Additional analyses of the Hypertension Prevention Trial data are presented. Most studies, where comparison can be made, suggest a greater effect for weight reduction than for the achieved, moderate, or short-term reduction in sodium intake on subsequent lowering of blood pressure; the lowering effect on blood pressure of weight reduction is independent of the effect of dietary sodium restriction. However, long-term (over a year) substantial reductions (up to 70 meq/day) of dietary sodium have not been achieved in comparative trials, nor are the effects of combined weight and sodium reduction in the prevention and treatment of hypertension quantified. Future studies and potential problems are discussed.
Hepatic cyst associated with Peutz-Jeghers syndrome. A solitary hepatic cyst, associated with Peutz-Jeghers syndrome, is described. To our knowledge, this association has not been previously reported. A developmental anomaly or hamartomatous nature of this lesion is suggested. Knowledge of this association may be helpful in the clinical diagnosis of this benign entity.
Aberrant regeneration in a case of syringobulbia: selective co-activation of abducens and facial nerves during saccades. A patient suffering from syringobulbia and syringomyelia exhibited a phasic contraction of the ipsilateral facial muscles, mainly the levator labii, whenever he looked to the left or right. Facial muscle twitches occurred exclusively with saccades. The selective co-activation of abducens and facial nerves is interpreted as the result of bilateral misrouting of regenerating neurons from the parapontine reticular formation to the facial nerve in the tegmentum pontis.
Lactic acidosis associated with high-dose niacin therapy. We have reported a case of lactic acidosis induced by ingestion of sustained-release nicotinic acid. Prompt recovery followed treatment with glucose-containing fluids and discontinuation of nicotinic acid therapy.
Insulin resistance and compensatory hyperinsulinemia: role in hypertension, dyslipidemia, and coronary heart disease. Resistance to insulin-stimulated glucose uptake and hyperinsulinemia may play a central role in the cause and clinical course of patients with non-insulin-dependent diabetes mellitus, high blood pressure, abnormalities of lipoprotein metabolism, and coronary heart disease. This article summarizes the evidence in support of this general hypothesis.
Changes in the postenteropathic form of the hemolytic uremic syndrome in children. An analysis was made of clinical and laboratory findings in children with the diarrheal form of the hemolytic uremic syndrome (HUS) treated at The Children's Hospital, Birmingham between 1970 und 1987. From 1982 the rate of referral increased, the prodromal illness more often consisted of bloody diarrhea, and the mean age at presentation doubled from 2 to 4 years. For patients with a good outcome there was an excess of males in the period 1970-81, and females in the period 1982-87. Moreover, in the years 1982-87 the disorder was distinguished from that of the earlier time by a positive correlation between adverse outcome and both neutrophil leukocytosis and a higher hemoglobin concentration at presentation. Prognostic scores obtained by logistic regression analysis were specific for each period. From July 1983 stool samples were analyzed for verocytotoxin-producing Escherichia coli (VTEC) and neutralizable verotoxin. Positive results were obtained in 39% of cases. The nature of HUS has changed and the new form of the disorder is associated with VTEC infection.
Germ cell tumor of testis in a patient with von Hippel-Lindau disease. Germ cell testicular tumor is a previously undescribed entity in association with von Hippel-Lindau disease. This case exemplifies the variety of pathologic entities encountered in von Hippel-Lindau disease and stresses the importance of thorough evaluation of the patient, as well as careful follow-up, to ensure early detection of potentially malignant lesions.
Detection of Coxsackievirus B3 RNA in myocardial tissues by the polymerase chain reaction. Coxsackievirus B3 is a possible etiologic agent in some forms of myocarditis and idiopathic dilated cardiomyopathy. A method for the detection of coxsackievirus B3 RNA was developed using the polymerase chain reaction based on the amplification of a cDNA copy of the positive-strand viral RNA. The fidelity of the method was established in two murine models for coxsackie B3 myocarditis. All cardiac specimens with adequate RNA for study from coxsackie B3-infected mice contained detectable viral RNA, in contrast to none of control specimens from noninfected mice. The sensitivity of the technique was established at approximately 1 to 100 plaque-forming units of virus per gram of tissue, and the specificity was established as limited to the coxsackievirus B3 serotype among nine viruses tested. In patients with myocarditis, one of five specimens contained detectable viral RNA, whereas none of 11 specimens from patients with idiopathic dilated cardiomyopathy or 21 myocardial specimens from patients with a wide variety of other cardiac disorders contained detectable coxsackie B3 viral RNA. The results show that the polymerase chain reaction is a useful means for detecting coxsackie viral RNA and its application should help in the evaluation of hypotheses concerning the infectious etiology of human myocarditis and idiopathic dilated cardiomyopathy.
Vagal schwannoma. A patient with a benign encapsulated intrathoracic vagal schwannoma is presented and the literature is reviewed. The right paratracheal tumor was identified incidentally on a chest film and excised using a right thoracotomy. Although rare, vagal schwannomas should be considered for any mediastinal mass along the vagus nerves especially when the paratracheal tumor produces minimal or no respiratory symptoms suggesting a slow-growing, encapsulated process.