FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W1964451868> ?p ?o ?g. }

• W1964451868 endingPage "918" @default.
• W1964451868 startingPage "893" @default.
• W1964451868 abstract "Abstract The data from fifty personally observed cases of an uncommon disease, multiple syndrome disorder here designated familial paroxysmal polyserositis (FPP), are presented. Three fatal cases in which autopsy or renal biopsy findings are described in detail, death occurring in renal or cardiac failure. Symptoms and signs of serosal inflammation are characteristic. Elevated temperatures, although often quite brief in duration, may reach 105 °F. Of four chief syndromes of FPP, paroxysmal peritonitis is most frequent; either alone or in combination with paroxysmal pleuritis, second most common syndrome. Intermittent arthralgias, monoarthritis and paroxysmal pyrexia are less frequent. Occasionally, cutaneous eruptions are observed. Thirty-six of these patients are male; fourteen, female. Onset of FPP is chiefly in childhood or in youth. The earliest age at onset was seven months, latest forty-three years. Attacks, usually of two to three days' duration, often recur every few weeks. Paroxysmal peritonitis closely simulates an acute surgical emergency. Direct and rebound tenderness, at times with involuntary abdominal wall spasm, fever, gastrointestinal upset, constipation and usually leukocytosis, 15,000 to 20,000 cells per cu. mm., complete a picture of the false acute abdomen. Paroxysms of pleuritis may recur independently but more often precede or follow peritonitis. A transient small pleural effusion may be observed on roentgenogram. Paroxysmal pyrexia was predominant in only one instance. The surgical pathology of paroxysmal peritonitis may be localized or diffuse, with congestion or edema, with or without small amounts of serous fluid, or strands and clumps of fibrin may be present. Microscopically, there is an acute polymorphonuclear exudate with congestion of peritoneum and omentum. Cultures are sterile. Occasionally during attacks, but invariably during an interval free of attack, laparotomy is unrevealing. Enlargement of spleen, moderate in degree, was observed in five cases. Accelerated erythrocyte sedimentation rate is frequent, especially during attacks. Moderate anemia, not less than 10 gm. per cent hemoglobin, is occasional. Hepatic disturbance with minor histologic and biochemical abnormalities may accompany paroxysms. Evanescent small bowel changes are frequent during attacks of peritonitis. Cerebral dysrhythmia was observed in electroencephalogram in two cases in childhood and reported in a single adult case. Progressive renal disease is considered most significant organ involvement in FPP, but in fifty cases forty-eight were free of nephropathy. Two patients died in uremia; in one chronic glomerulonephritis was found at autopsy, and in other renal amyloidosis was found on biopsy. In a third case, death was due to rheumatic mitral stenosis. FPP is familial and genetic. It affects especially, but not exclusively, patients of Mediterranean origin, most often Jews (both Ashkenazi and non-Ashkenazi), Armenians and Arabs and, less commonly, Italians, Maltese and Greeks. Of fifty cases, forty-two occurred in Jews; thirty-five are definitely Ashkenazi, three probably Ashkenazi and four Sephardic; seven in Armenians; one in a Maltese. Ten familial cases include two families, Ashkenazi Jewish and Armenian, with five members affected. The role of hypersensitivity in FPP is discussed, with special reference to rheumatic fever. The incidence of atopy is shown to be about twice that in control subjects. Food allergy appeared to be demonstrated in only two of twenty-two cases studied. Endocrine factors involving especially formation of abnormal pyrogenic steroid metabolites probably derived from adrenal or gonadal hormones may play a significant role in FPP. Pregnancy is often associated with complete inhibition of attacks, and paroxysms may be strikingly associated with menstrual periods. In some cases emotional factors appear to initiate episodes of FPP. Paroxysmal peritonitis presents most urgent diagnostic problem, to be differentiated from common acute abdominal infections, acute intermittent porphyria and relapsing pancreatitis. The diagnosis of FPP must be considered with caution in patients with attacks beginning after age forty. Prognosis as to life is generally favorable. Forty-seven of fifty patients are alive despite many years of illness. Five are over age sixty, one being seventy-seven and free of attacks for fourteen years. In two patients progressive nephropathy developed. Most patients remain remarkably well between acute episodes. Rarely, unpredictable remissions occur which may last for years. The prognosis for complete recovery is poor, since FPP usually pursues a lifetime course. Treatment of FPP is generally unsatisfactory. The administration of corticosteroids has been distinctly helpful in aborting attacks in three of fifteen cases. Rarely, exclusion of a specific food, particularly milk and milk products, may produce a prolonged remission. A diet restricted in fat (20 gm./day) is of occasional benefit." @default.
• W1964451868 created "2016-06-24" @default.
• W1964451868 creator A5021688172 @default.
• W1964451868 date "1964-06-01" @default.
• W1964451868 modified "2023-09-23" @default.
• W1964451868 title "FAMILIAL PAROXYSMAL POLYSEROSITIS. ANALYSIS OF FIFTY CASES." @default.
• W1964451868 cites W1510297128 @default.
• W1964451868 cites W1510479719 @default.
• W1964451868 cites W1514131885 @default.
• W1964451868 cites W1525937482 @default.
• W1964451868 cites W1525971732 @default.
• W1964451868 cites W1534042084 @default.
• W1964451868 cites W1759343023 @default.
• W1964451868 cites W1780799479 @default.
• W1964451868 cites W1870109103 @default.
• W1964451868 cites W1964439776 @default.
• W1964451868 cites W1971570754 @default.
• W1964451868 cites W1973852720 @default.
• W1964451868 cites W1974574192 @default.
• W1964451868 cites W1980708320 @default.
• W1964451868 cites W1986710341 @default.
• W1964451868 cites W1989289095 @default.
• W1964451868 cites W1994311830 @default.
• W1964451868 cites W1996406293 @default.
• W1964451868 cites W2001452951 @default.
• W1964451868 cites W2006668097 @default.
• W1964451868 cites W2006827667 @default.
• W1964451868 cites W2008867503 @default.
• W1964451868 cites W2010313570 @default.
• W1964451868 cites W2017845883 @default.
• W1964451868 cites W2022177560 @default.
• W1964451868 cites W2023023799 @default.
• W1964451868 cites W2024725594 @default.
• W1964451868 cites W2029753592 @default.
• W1964451868 cites W2030867963 @default.
• W1964451868 cites W2031936792 @default.
• W1964451868 cites W2037263078 @default.
• W1964451868 cites W2048147428 @default.
• W1964451868 cites W2050854035 @default.
• W1964451868 cites W2065685825 @default.
• W1964451868 cites W2068836068 @default.
• W1964451868 cites W2069646776 @default.
• W1964451868 cites W2071454747 @default.
• W1964451868 cites W2072267671 @default.
• W1964451868 cites W2077504407 @default.
• W1964451868 cites W2078263700 @default.
• W1964451868 cites W2078264642 @default.
• W1964451868 cites W2082028177 @default.
• W1964451868 cites W2082848494 @default.
• W1964451868 cites W2083097280 @default.
• W1964451868 cites W2091265743 @default.
• W1964451868 cites W2130580918 @default.
• W1964451868 cites W2135540663 @default.
• W1964451868 cites W2159142992 @default.
• W1964451868 cites W2164748979 @default.
• W1964451868 cites W2167068828 @default.
• W1964451868 cites W2226379161 @default.
• W1964451868 cites W2264542991 @default.
• W1964451868 cites W2281960477 @default.
• W1964451868 cites W2322162332 @default.
• W1964451868 cites W2327451485 @default.
• W1964451868 cites W2336209135 @default.
• W1964451868 cites W2403283220 @default.
• W1964451868 cites W2405754951 @default.
• W1964451868 cites W2406365302 @default.
• W1964451868 cites W2409112094 @default.
• W1964451868 cites W2412119522 @default.
• W1964451868 cites W2416042183 @default.
• W1964451868 cites W2416680234 @default.
• W1964451868 cites W2427464771 @default.
• W1964451868 cites W2442590981 @default.
• W1964451868 cites W2467864841 @default.
• W1964451868 cites W2468703893 @default.
• W1964451868 cites W2793454548 @default.
• W1964451868 cites W3135773419 @default.
• W1964451868 cites W3140206895 @default.
• W1964451868 cites W1548565154 @default.
• W1964451868 cites W2794487002 @default.
• W1964451868 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/14162896" @default.
• W1964451868 hasPublicationYear "1964" @default.
• W1964451868 type Work @default.
• W1964451868 sameAs 1964451868 @default.
• W1964451868 citedByCount "52" @default.
• W1964451868 countsByYear W19644518682013 @default.
• W1964451868 countsByYear W19644518682015 @default.
• W1964451868 countsByYear W19644518682016 @default.
• W1964451868 countsByYear W19644518682018 @default.
• W1964451868 countsByYear W19644518682020 @default.
• W1964451868 countsByYear W19644518682022 @default.
• W1964451868 crossrefType "journal-article" @default.
• W1964451868 hasAuthorship W1964451868A5021688172 @default.
• W1964451868 hasConcept C126322002 @default.
• W1964451868 hasConcept C141071460 @default.
• W1964451868 hasConcept C2778923028 @default.
• W1964451868 hasConcept C2779134260 @default.
• W1964451868 hasConcept C2779504383 @default.
• W1964451868 hasConcept C2779537928 @default.
• W1964451868 hasConcept C2779634585 @default.

• next