Melricflash/CW_MedAbstractsAlt huggingface dataset:
IgD immune complex vasculitis in a patient with hyperimmunoglobulinemia D and periodic fever. We describe a 27-year-old Dutch woman with the hyperimmunoglobulinemia D and periodic fever syndrome. During febrile attacks she occasionally presented with skin lesions on the distal parts of her upper and lower extremities, with the histologic picture of a leukocytoclastic vasculitis. Clear perivascular deposits of IgD and C3 were presented in early lesional skin on immunofluorescence investigation. Circulating IgD immune complexes were demonstrated on several occasions, both during and in between clinical attacks. These findings are consistent with an IgD immune complex-mediated pathogenesis for the skin lesions. In 10 patients with other forms of immune complex vasculitis of the skin, minimal perivascular deposits of IgD were found in four cases. In these cases, however, IgD was never found as the solitary immunoglobulin class. 

Category: cardiovascular diseases
The impact of right ventricular infarction on the prevalence of ventricular arrhythmias during acute inferior myocardial infarction. To determine the impact of RV infarction on the prevalence and complexity of ventricular arrhythmias during inferior AMI, 57 patients with no prior MI were studied by 24-hour Holter monitoring on the first and tenth days of AMI. Based on radionuclear studies, patients were allocated into two groups: (1) group A, 21 patients (37 percent) with normal RVEF (greater than or equal to 40 percent); and (2) group B, 36 patients (63 percent) with depressed RVEF (less than 40 percent). There were no significant differences between the groups regarding age and LVEF. Values of RVEF were 47 +/- 6 percent and 31 +/- 6 percent, respectively (p less than 0.05). The RVEF had no influence on the prevalence and complexity of early and late arrhythmias. Stratification of patients in group B into two subgroups based on the extent of RV dysfunction did not reveal any differences in the occurrence of all forms of ectopy (when both groups were matched to group A). Therefore, patients with inferior AMI, with or without RV infarction, have a similar prevalence of arrhythmias. Ventricular ectopic beats may be related to the severity and spread of LV involvement, rather than to RV dysfunction. 

Category: general pathological conditions
Primary adenocarcinoma of the temporal bone. A case with 40-year follow-up. Adenomatous tumors of the temporal bone are rare neoplasms. This article records the 40-year course of a patient with adenocarcinoma of the temporal bone and reviews the literature pertinent to the biologic behavior, histologic appearance, prognosis, and treatment of this group of tumors. 

Category: neoplasms
Pharmacokinetics of tiaprofenic acid after oral administration in fasting patients during and between migraine attacks. This study examined the pharmacokinetics of 300 mg of tiaprofenic acid, a NSAID belonging to the 2-arylpropionic class, as a single oral dose, in 10 migraine patients during and out of migraine attacks. Plasma concentration of tiaprofenic acid was determined by HPLC analysis. Drug absorption appeared to be the same during and out of migraine attacks (absorption half life: during attack, 0.249 +/- 0.122 hr; out of attack, 0.249 +/- 0.105 hr; maximum plasma concentration: during attack, 37.8 +/- 9.8 ug/ml; out of attack, 40.1 +/- 13.2 ug/ml). The other pharmacokinetic parameters evaluated were not affected by headache attacks as well. We conclude that tiaprofenic acid absorption and metabolism are not affected by migraine attacks. Also, our data suggest that tiaprofenic acid might be useful in the treatment of migraine. 

Category: nervous system diseases
How stressful is retirement? Findings from the Normative Aging Study. The stressfulness of retirement both as a transitional event experienced during the past year and as a life stage was investigated. Transitional stress was assessed using a life events approach, and stage stress using a "hassles" approach. Respondents were 1,516 male participants in the Normative Aging Study, 45% of whom were retired. Among those retiring in the past year, respondents' own and spouse's retirement were rated the least stressful from a list of 31 possible events. Only 30% found retirement stressful. Retirement hassles were also less frequently reported and were rated less stressful than the work hassles of men still in the labor force. The only consistent predictors of both transitional and stage retirement stress were poor health and family finances; personality did not predict retirement stress. 

Category: general pathological conditions
Levels of disability among U.S. adults with arthritis. This article studies the excess levels of disability experienced by persons with arthritis, compared to persons without the disease. The data set is the Supplement on Aging (1984 National Health Interview Survey); it has information for a national probability sample of community-dwelling persons ages 55 + (N = 16,148). (1) Arthritis people have more difficulty in physical functions, personal care, and household care than do nonarthritis persons. The excess disability is greatest for physical functions (walking, reaching, stooping, etc.). Disabled arthritis people have especially high degrees of difficulty in physical activities that require endurance and strength. (2) Various models are tested for walking, grasping, shopping, and light housework to show how comorbidity propels disability for arthritis people and to show arthritis' own contribution to disability in the presence of other chronic conditions. Difficulties escalate for arthritis people when they have other concurrent conditions. These models affirm that arthritis has a pronounced effect on physical dysfunctions, but these are not readily translated into personal and household care problems. Apparently, arthritis people often make successful accommodations so their roles and daily activities are not seriously affected by the disease. 

Category: general pathological conditions
LDH isoenzymes in cerebrospinal fluid in various brain tumours. This study examined the isoenzymatic pattern of LDH in the cerebrospinal fluid (CSF) as well as the ratio between the five fractions of LDH among patients with various brain tumours, carcinomatous meningitis and control groups. LDH 1/LDH 2 less than 1 was found significant for carcinomatous meningitis (p less than 0.001) and brain metastases (p less than 0.001). LDH 1/LDH 2 ratio was found to be significantly lower in carcinomatous meningitis than in brain metastases (p less than 0.05). No LDH 1/LDH 2 ratios smaller than 1 were found in the other groups. The LDH 1/LDH 2 ratio smaller than 1 was found in the early stage of carcinomatous meningitis without other evidences of the involvement of the leptomeninges. Examination of LDH 1/LDH 2 can be found as an adjunctive method to identify brain metastases and carcinomatous meningitis at the initial stage. 

Category: neoplasms
Orthostatic hypotension after spinal cord injury: treatment with fludrocortisone and ergotamine. We report a case of a 28-year-old woman with C5 quadriplegia, unresponsive to conservative treatment for orthostatic hypotension. Ergotamine, daily combined with fludrocortisone, successfully prevented symptomatic hypotension. In this report, neural, renal, and hormonal blood pressure regulatory mechanisms are described. Experiences obtained from this case and the existing literature suggest that (1) in persons with quadriplegia, plasma catecholamine levels show little increase with sitting, indicating an inability to activate baroreceptor and chemoreceptor reflexes, (2) elevated plasma aldosterone and renin levels indicate a renal compensatory response to decreased renal perfusion secondary to low plasma volume and/or chronic hypotension, and (3) the net effect of ergotamine and fludrocortisone is probably a combination of plasma volume expansion and direct peripheral vasoconstriction. 

Category: cardiovascular diseases
Thyroid cancer. There have been important recent advances in our understanding of the biologic nature of thyroid cancer and in the early diagnosis of the disease. Despite these advances, there is still considerable controversy over the management of thyroid cancer, including the extent of surgery, the indications for the use of iodine-131, the effectiveness of thyroid-stimulating hormone suppression, and the prediction of outcome. In this review, the current status of the diagnosis and management of the various types of thyroid cancer are carefully reviewed and extensively documented. 

Category: neoplasms
The nature of opioid responsiveness and its implications for neuropathic pain: new hypotheses derived from studies of opioid infusions. In recent years, the observation that the response of patients to opioid drugs may be influenced by properties inherent in the pain or pain syndrome, such as its pathophysiology, has evolved into the belief that certain types of pain, e.g., neuropathic pains, may be unresponsive to these drugs. This concept has important implications for both clinical practice and basic understanding of opioid mechanisms. We critically evaluate opioid responsiveness, particularly as it relates to neuropathic pain, and propose a clinically relevant definition and a paradigm for its investigation. The paradigm is illustrated by analgesic responses to opioid infusion in 28 patients with neuropathic pains and by a detailed presentation of the pharmacokinetic and pharmacodynamic relationships in one of these patients, whose central pain responded promptly to an infusion of hydromorphone. From this analysis, we hypothesize that (1) opioid responsiveness in man can be defined by the degree of analgesia achieved during dose escalation to either intolerable side effects or the occurrence of 'complete' or 'adequate' analgesia; (2) opioid responsiveness is a continuum, rather than a quantal phenomenon; (3) opioid responsiveness is determined by a diverse group of patient characteristics and pain-related factors, as well as drug-selective effects; and (4) a neuropathic mechanism may reduce opioid responsiveness, but does not result in an inherent resistance to these drugs. Given the complexity of factors contributing to opioid responsiveness and the observation that outcome cannot be reliably predicted, opioids should not be withheld on the assumption that pain mechanism, or any other factor, precludes a favorable response. Both the clinical use of opioids and paradigms to investigate opioid responsiveness should include dose escalation to maximally tolerated levels and repeated monitoring of analgesia and other effects. 

Category: nervous system diseases
The iris in Williams syndrome. Forty three children with Williams syndrome and 124 control subjects had their eyes photographed. The photographs were examined by three ophthalmologists and four geneticists of varying experience. A stellate pattern was noted more often in the irides of patients with Williams syndrome (51%) than in those of the control subjects (12%), and was more difficult to detect, or was absent, in heavily pigmented irides. We conclude that the stellate pattern is of diagnostic importance, particularly if the pattern is carefully defined and the clinician is experienced. 

Category: cardiovascular diseases
Haemangiosarcoma of the maxillary antrum. Angiosarcomas are extremely rare in the head and neck and the histological diagnosis is often difficult. We present a case of a haemangiosarcoma of the maxillary antrum in a 33 year old male. The histological diagnosis and subsequent management are discussed. 

Category: neoplasms
Morphometry of the subepithelial circulation in sheep airways. Effect of vascular congestion. In order to quantitate the subepithelial microvascular volume and its relation to the airway lumen, we conducted a morphometric analysis of the vascular compartment in the wall of the trachea (within a 55-microns depth from the epithelial basement membrane) and of 1.0 and 0.5-mm bronchioles of sheep. The lungs were fixed by bronchial and pulmonary artery perfusion with glutaraldehyde under three experimental conditions: (1) bronchial artery pressure, 100 mm Hg pulmonary artery pressure, 20 mm Hg (control); (2) bronchial artery pressure, 100 mm Hg, pulmonary artery pressure, 40 mm Hg (pulmonary hypertension, PH); (3) bronchial artery pressure, 100 mm Hg, pulmonary artery pressure, 40 mm Hg (pharmacologic vasodilation with sodium nitroprusside, PH + V). Venous pressures were atmospheric. Under control conditions, the microvascular volume fraction comprised 12, 16, and 15% of the subepithelial tissue in the trachea and 1-mm and 0.5-mm bronchioles, respectively. PH increased the microvascular volume fraction in the bronchioles (p less than 0.05), but it had no effect on the microvasculature in the trachea. PH + V approximately doubled the microvascular volume fraction in the trachea and the bronchioles. PH increased the mean wall thickness, and PH and PH + V decreased the airway cross-sectional area in the 1-mm bronchioles. These observations demonstrate that the microvasculature constitutes a considerable volume fraction of the subepithelial airway tissue and that vascular congestion can narrow the bronchiolar lumen. 

Category: cardiovascular diseases
Characterization of a large deletion associated with a polymorphic block of repeated dinucleotides in the type III procollagen gene (COL3A1) of a patient with Ehlers-Danlos syndrome type IV. Ehlers-Danlos syndrome type IV (EDS IV) is an autosomal dominant condition characterized by extreme fragility of skin, blood vessels, intestine, gravid uterus, and lungs. The phenotype is accounted for by mutations affecting the integrity and/or synthesis of the precursor procollagen molecules of type III collagen. In this article, we report the elucidation of the molecular defect in an EDS IV patient whose type III collagen was previously found to be structurally abnormal. We utilized PCR in a two-step process involving first the localization of the mutation in the mRNA and then the characterization of the defect in the gene. The results established the patient's heterozygosity for a genomic deletion of about 7.5 kb which eliminates 1,026 nucleotides of coding sequences in the message. The mutation arose as a result of an exon-to-intron recombination. The deleted segment extends from the 13th nucleotide of exon 9 to within a DNA sequence of intron 24, which is composed of a series of dinucleotide repeats. Using PCR, we tested the polymorphic nature of this DNA element on several unrelated individuals. Analysis of amplified genomic products of 45 chromosomes recognized at least four distinct allelic forms that display frequencies ranging from 5% to 61%. Mendelian segregation of three of the four alleles was established by the same method in a 3-generation family. 

Category: general pathological conditions
Magnetic resonance imaging in Mayer-Rokitansky-Kuster-Hauser syndrome. Six girls with suspected Rokitansky syndrome underwent magnetic resonance imaging (MRI) and then sonography and laparoscopy to confirm the clinical diagnosis and define the precise characteristics of the malformation. The uterus was missing in all; four had mullerian rudiments in place of the uterus, which were cavitary in two as shown by sonography and in one by MRI. Both ovaries were present in all cases, normally located in four and extrapelvic in two; they were correctly identified by MRI in all cases and by sonography in four. Partially canalized vaginal rudiments were recognized by MRI in two girls and by sonography in one. One patient had unilateral renal agenesis, demonstrated by both MRI and sonography. Magnetic resonance imaging was more precise than laparoscopy and sonography in defining the anatomical characteristics of Rokitansky syndrome, and is less invasive and expensive than laparoscopy. 

Category: general pathological conditions
Final height in boys with untreated constitutional delay in growth and puberty. To determine the natural history and psychological impact of the growth pattern in boys with constitutional delay in growth and puberty (CDGP), 43 boys presenting with short stature due to CDGP were followed up to final height. At presentation mean (SD) chronological age was 14.0 (1.9) years, bone age delay 2.7 (1.0) years, standing height standard deviation score (SDS) -3.4 (0.6), and predicted adult height SDS -1.3 (0.7). Final adult height SDS was -1.6 (0.9), measured at 21.2 (2.6) years. There was no significant difference between final height and predicted adult height, but there was a significant difference between final height and measured mid-parental height. Psychological questionnaires showed no significant difference in self esteem, marital, or employment state between the CDGP group and a control group. There was no correlation between self esteem and final height, but 25 felt their growth delay had affected their success either at school, work, or socially and 20 would rather have had treatment to advance their growth spurt. This study supports the more frequent use of active medical treatment to advance growth in boys with CDGP, and shows that although boys with CDGP reach their predicted heights, this is short for their families. 

Category: general pathological conditions
Defective jejunal brush border membrane sodium/proton exchange in association with lethal familial protracted diarrhoea. The spectrum of clinical disease associated with specific defects in jejunal brush border membrane sodium/proton exchange is poorly defined and only two patients have been described so far. Jejunal brush border membrane transport studies were performed in a boy who presented with lethal familial protracted diarrhoea in the first few days of life. Using jejunal brush border membrane vesicles prepared from conventional jejunal biopsy specimens, initial sodium uptake under H+ gradient conditions was found to be only 6% of the mean control value. In contrast, sodium stimulated glucose uptake was normal. Our data confirm the importance of a congenital defect in this exchanger as a cause of severe sodium-losing diarrhoea and extend the spectrum of disorders characterised by a specific defect in brush border membrane Na+/H+ exchange to include some forms of lethal familial protracted diarrhoea. 

Category: digestive system diseases
Frequency of low-grade residual coronary stenosis after thrombolysis during acute myocardial infarction. The clinical, angiographic and demographic characteristics of 42 patients with low-grade (less than 50%) residual stenosis at the infarct lesion after thrombolysis for acute myocardial infarction (MI) were assessed. The study group (group I) represented 21% of 198 consecutive patients receiving thrombolytic therapy over a 59-month period. Data on the 156 remaining patients were pooled for comparison (group II). Group I patients were predominantly men (86%) who were cigarette smokers (81%). Group II patients were predominantly men (75%, p greater than 0.10) but were significantly older (52 +/- 12 vs 56 +/- 10 years, p = 0.02). Prior acute MI or angina was unusual in group I. Sixty percent had no significant (greater than 50%) residual coronary artery disease while 25% had residual single artery disease. Average significant (greater than 50% diameter stenosis) residual vessel disease was 0.6 +/- 1.0 for group I and 1.9 +/- 0.9 for group II (p less than 0.001). In group I, average residual infarct lesion diameter stenosis was 36 +/- 7% in the right anterior oblique and 34 +/- 8% in the left anterior oblique views. Thirty-nine group I patients were discharged with medical therapy and 100% follow-up was obtained over a mean interval of 18 +/- 17 months. Fifteen patients experienced chest pain after acute MI accounting for 17 discrete events. Fifty-nine percent of group I had a benign course on follow-up. Eight events were classified as unstable angina, 4 as acute MI and 5 as atypical angina. Documented coronary vasospasm occurred in 3. 

Category: cardiovascular diseases
Pseudocyst of the auricle: successful treatment with intracartilaginous trichloroacetic acid and button bolsters. Pseudocyst of the auricle is an asymptomatic, noninflammatory cystic swelling that typically involves the anthelix of the ear and results from an accumulation of fluid within an unlined intracartilaginous cavity. We report a patient with a recurrent pseudocyst of the auricle and describe a new surgical technique for treating this condition by applying 50% trichloroacetic acid to the intracartilaginous cavity and utilizing external button bolsters for compressive therapy. This therapeutic approach is simple to perform in the office, results in permanent resolution of the pseudocyst, preserves the normal architecture of the external ear, and provides excellent postoperative and long-term cosmetic results. 

Category: general pathological conditions
Application of INSTAT hemostat in the control of gingival hemorrhage in the patient with thrombocytopenia. A case report. Gingival bleeding in the patient with thrombocytopenia can be a difficult management problem. Primary therapy with platelet transfusions may not be sufficient to control the hemorrhage and adjunctive local therapy may be required. Currently, few local management techniques can effectively control this problem. INSTAT collagen absorbable hemostat can be used as a local adjunct with platelet transfusions and has certain application advantages over topical thrombin and microfibrillar collagen. Two successful techniques of INSTAT application to control gingival hemorrhage in a patient with severe thrombocytopenia in leukemic relapse are described. 

Category: general pathological conditions
Activation of erythropoietin receptors by Friend viral gp55 and by erythropoietin and down-modulation by the murine Fv-2r resistance gene. The leukemogenic membrane glycoprotein (gp55) encoded by Friend spleen focus-forming virus appears to bind to erythropoietin receptors (EpoR) sto stimulate erythroblastosis [Li, J.-P., D'Andrea, A.D., Lodish, H.F. & Baltimore, D. (1990) Nature (London) 343, 762-764]. To directly compare the effects of gp55 with erythropoietin (Epo), we produced retrovirions that encode either gp55, Epo, or EpoR. After infection with EpoR virus, interleukin 3-dependent DA-3 cells bound 125I-labeled Epo and grew without interleukin 3 in the presence of Epo. These latter cells, but not parental DA-3 cells, became factor-independent after superinfection either with Epo virus or with Friend spleen focus-forming virus. In addition, Epo virus caused a disease in mice that mimicked Friend erythroleukemia. Although Fv-2r homozygotes are susceptible to all other retroviral diseases, they are resistant to both Epo viral and Friend viral erythroleukemias. These results indicate that both gp55 and Epo stimulate EpoR and that the Fv-2 gene encodes a protein that controls response to these ligands. However, the Fv-2 protein is not EpoR because the corresponding genes map to opposite ends of mouse chromosome 9. These results have important implications for understanding signal transduction by EpoR and the role of host genetic variation in controlling susceptibility to an oncogenic protein. 

Category: neoplasms