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Constrictive pericarditis with dwarfism in two siblings (mulibrey nanism).

Two siblings with marked dwarfism, now 11 and 19 years of age, have been followed from infancy. The girl had frequent episodes of pneumonitis and presented at age 4 years with hepatic enlargement and ascites which proved to be due to constrictive pericarditis. The boy presented with growth failure and pseudohydrocephalus. He had fibrous dysplasia of the tibia and a pathologic fracture; acute hepatic congestion followed physical activity at age 13 years and led to the diagnosis of constrictive pericarditis. Muscle function was normal, there was no evidence for a primary liver disorder, and mental development was normal so that the coined word "mulibray" seemed inappropriate. Pericardiectomy produced only partial improvement; both patients have hepatic enlargement and continue to need diuretics. A third patient with dwarfism, frequent respiratory infections, and pericardial calcification has certain features of the syndrome.

Adolescent↗

Hypoxic airway constriction in infants of very low birth weight recovering from moderate to severe bronchopulmonary dysplasia.

We hypothesized that infants recovering from severe bronchopulmonary dysplasia have airway constriction that is, at least in part, related to borderline hypoxia. If this hypothesis were correct, pulmonary resistance should decrease with the administration of oxygen. To test this hypothesis, we studied 10 infants recovering from severe bronchopulmonary dysplasia (study weight 2490 +/- 275 gm; birth weight 1010 +/- 89 gm; postnatal age 73 +/- 7 days; postconceptional age 38.5 +/- 1.6 weeks) and 10 matched control infants (study weight 2430 +/- 179 gm; birth weight 2320 +/- 195 gm; postnatal age 25 +/- 4 days; postconceptional age 37.5 +/- 0.8 weeks). Resistance and compliance were measured by means of a mask with a flowmeter and an esophageal balloon (with the PEDS computer program). Measurements in both groups were made in quiet sleep, without sedation, during the inhalation of room air and during the fifth minute of oxygen inhalation. We found that (1) total pulmonary resistance, significantly higher in infants with bronchopulmonary dysplasia than in control infants, decreased from 206.1 +/- 47 cm H2O.L-1.sec-1 during inhalation of room air to 106.5 +/- 20.9 during inhalation of 100% oxygen (p less than 0.05) and (2) pulmonary dynamic compliance, lower in infants with bronchopulmonary dysplasia than in control infants, increased significantly with the administration of 100% oxygen. The results suggest that infants with bronchopulmonary dysplasia have airway constriction and that this is alleviated by inhalation of oxygen.

Airway Obstruction↗

Development of Peyronie's disease with the use of a vacuum constriction device.

Vacuum constriction devices are generally considered to be a safe, effective, noninvasive means of producing erections in impotent men. However, long-term study of the effect of subatmospheric pressure on penile structures and vasculature has not been performed. We report the development of Peyronie's disease in a patient after 4 years of complication-free vacuum constriction device use.

Constriction↗

Constrictive albuginitis: report of 3 cases.

Three sterile patients with constrictive albuginitis are described. The most common presenting symptom is a long duration of scrotal pain and the testicles are small and firm. Testicular biopsy is characterized by peritubular fibrosis. Spermatogenesis may be complete but the cells are degenerated. The condition is bilateral. At operation the tunica albuginea is thick, yellowish and rigid and, microscopically, it shows excessive fibrosis and hyalinosis. Sterility is related to the constrictive effect of the tunica albuginea. Treatment by decompression albugineotomy was successful in 2 patients. The benefits derived from the operation are discussed. A primary degenerative process of the tunica albuginea is suggested as causal.

Adult↗

Constrictive pericarditis associated with combined idiopathic retroperitoneal and mediastinal fibrosis.

Idiopathic retroperitoneal fibrosis and mediastinal fibrosis are localized expressions of a systemic sclerosing disease of unknown cause which, on rare occasions, may coexist in the same patient. Although pericardial involvement may occur, recurrent constrictive pericarditis that is unrelieved by pericardiectomy has not previously been reported in association with either idiopathic retroperitoneal or mediastinal fibrosis. Reported herein is a case of recurrent constrictive pericarditis that was unrelieved by two pericardiectomies , and autopsy revealed unsuspected combined idiopathic retroperitoneal and mediastinal fibrosis. The clinical, echocardiographic, and pathologic findings are described.

Echocardiography↗

Subacute constrictive pericarditis from Serratia marcescens bacteremia.

A case report of subacute constrictive pericarditis associated with disseminated Serratia marcescens infection and bacteremia in a patient with chronic tubulointerstitial nephritis and uremia is described. Although not substantiated by clinical history, the renal pathologic features were similar to those of ethylene glycol-induced tubulointerstitial nephritis. The patient did not have a history of heroin addiction. The importance of predisposing factors such as uremia, invasive vascular procedures, tracheal intubation, peritoneal dialysis, and pericardiocentesis in Serratia infection in susceptible persons is discussed, as are possible roles of uremia, pericardiocentesis, and pericardiotomy in the pathogenesis of constrictive pericarditis in the present case.

Adult↗

Treatment of penile strangulation caused by constricting devices.

Constricting devices placed on the penis present a challenge to urologists. Various nonmetallic and metallic objects are placed on the penis to increase sexual performance or because of autoerotic intentions. We describe five different cases of strangulating objects (wedding ring, metal plumbing cuff, bull ring, hammer- head, and plastic bottle neck) and demonstrate that each case needs individual handling in removing the object. The treatment of penile strangulation is decompression of the constricted penis to facilitate free blood flow and micturition. It requires no particular skill but does require resourcefulness to perform the removal simply and effectively, and with as little discomfort for the patient as possible.

Adult↗

Aorta collagen metabolism in spontaneously hypertensive and aortic-constricted rats: variations in enzyme activities concerned with disaccharide unit synthesis and degradation according to blood pressure and age.

Two enzyme activities concerned with collagen disaccharide unit metabolism (UDP-glucose: collagen glucosyltransferase and glucosyl-galactosyl-hydroxylysine glucohydrolase) have been studied in the thoracic aortic wall together with 4-prolyl hydroxylase activity and 4-hydroxyproline content in spontaneously hypertensive rats (SHR) at the prehypertensive, hypertensive and sustained hypertensive stages (respectively 32 days, 12 weeks and 19 weeks of age). They were compared with values observed in age-matched normotensive Wistar Kyoto rats (WKY). The same studies have been performed in parallel on aortic-constricted rats (ACR) 8 days after suprarenal constriction of the abdominal aorta. Negative regressions of all three specific activities as function of age were observed. The most striking difference observed between the SHR and the WKY was the increase of glucosyltransferase specific activity, already found at the prehypertensive stage and continuing thereafter; the glucohydrolase specific activity was increased only during the establishment of hypertension whereas no modification was found with prolyl hydroxylase at any stage. However, a diminution of hydroxyproline concentration was seen at all ages while total hydroxyproline mass remained unaffected. The alterations of the aortic collagen metabolism observed in the ACR recall those seen in the SHR at the prehypertensive stage: the only significant modification was that of glucosyltransferase activity. Correlation was found between glucosyltransferase activity and blood pressure level in the two animal models.

Age Factors↗

Role of input from saphenous afferents in altered spinal processing of noxious signal that follows sciatic nerve constriction in rats.

An investigation was made into a possible contribution of saphenous nerve to altered central processing of noxious information following sciatic nerve chronic constriction (CCI) in rats. Spinal sciatic neuron spontaneous and noxious evoked activities were recorded in CCI rats, CCI rats with saphenous nerve sectioned just before the sciatic nerve constriction and sham operated rats. The results show (1) high values of spontaneous and noxious evoked activities with prolonged afterdischarges in CCI rats with intact saphenous, (2) comparable high values of spontaneous activity, but significantly reduced noxious evoked activity and afterdischarges in CCI rats with sectioned saphenous, (3) values in the normal range in the sham rats. Potential mechanisms underlying these results are discussed.

Action Potentials↗

Increases in the phosphorylation of cyclic AMP response element binding protein (CREB) and decreases in the content of calcineurin accompany thermal hyperalgesia following chronic constriction injury in rats.

Plasticity in the spinal dorsal horn may underlie the development of chronic pain following peripheral nerve injury or inflammation. In this study, we examined whether chronic constriction injury of the sciatic nerve was associated with changes in the immunoreactive content of cyclic AMP response element binding protein (CREB), protein kinase A (PKA), and calcineurin Aalpha and Abeta in the spinal dorsal horn. In animals exhibiting thermal hyperalgesia as a behavioral sign of neuropathic pain 7 days after loose ligation of the sciatic nerve (chronic constriction injury), there was a significant increase in the content of phosphorylated (activated) CREB (pCREB). In contrast, following the typical disappearance of thermal hyperalgesia 28 days after loose ligation surgery, there were no differences in pCREB content between control and sciatic ligation animals. The increased CREB activation associated with thermal hyperalgesia was accompanied by significant decreases in the content of both calcineurin Aalpha and Abeta. In contrast, there were no differences in the content of non-phosphorylated CREB, and phosphorylated or non-phosphorylated PKA between control and sciatic ligation animals either 7 or 28 days after surgery. These data established a close association in the expression of thermal hyperalgesia with CREB activation and decreased calcineurin content in the spinal dorsal horn. The data revealed a significant but reversible shift in the manner in which spinal neurons processed sensory information following peripheral nerve injury, and lent further support to the notion that plasticity in the spinal dorsal horn may have contributed to the development of chronic pain.

Animals↗

The effect of thalidomide treatment on vascular pathology and hyperalgesia caused by chronic constriction injury of rat nerve.

Tumor necrosis factor alpha (TNF) may be involved in the pathogenic mechanisms of neuropathic pain by affecting endothelial cells and by upregulation of receptor sensitivity in afferent nerve fibers. To test the hypothesis that TNF plays a role in the vascular changes and the pain-related behavior in an experimental painful neuropathy in rats produced by tying loosely constrictive ligatures around one sciatic nerve, we investigated the effect of thalidomide, a selective blocker of TNF-production in activated macrophages. In rats in which treatment with thalidomide was started preoperatively, there was diminished mechanical allodynia and thermal hyperalgesia during the early stage of the disease. TNF immunohistochemistry revealed reduced endoneurial immunoreactivity on day 5 post surgery as compared to sham-treated animals. The pathologic vascular changes were also reduced in thalidomide-treated rats. Starting treatment with thalidomide at a time point when hyperalgesia was already present did not alter the course of the pain-related behavior. We conclude that preemptive treatment with a substance that blocks production of TNF reduces pain-related symptoms and pathologic vascular changes in the chronic constriction injury model of neuropathic pain.

Analysis of Variance↗

Posterior interosseous nerve paralysis with multiple constrictions.

We report four cases of posterior interosseous nerve paralysis with multiple constrictions. At surgery the constrictions were found between the arcade of Frohse and a point of bifurcation of the supinator motor branch. External neurolysis with epineurotomy using the microscope was performed in all cases, and full recovery was obtained.

Adult↗

Spontaneous anterior interosseous nerve palsy with hourglass-like fascicular constriction within the main trunk of the median nerve.

Interfascicular neurolysis was performed in nine patients with spontaneous anterior interosseous nerve palsy. In eight of these patients, an hourglass-like constriction in the fascicles forming the anterior interosseous nerve was found within the main trunk of the median nerve at 2-7.5 cm above the medial epicondyle. The clinical signs and symptoms of these eight patients were similar to those that have been described to isolated neuritis. While the etiology remains unknown, when spontaneous anterior interosseous nerve palsy is suspected to be caused by isolated neuritis, interfascicular neurolysis should be performed to confirm the lesion and to discover whether fascicular constriction is present.

Adult↗

Diastolic heart failure. Constrictive, restrictive, and pericardial.

Clinical heart failure with normal systolic function is suggestive of diastolic dysfunction. This can result from myocardial or pericardial disorders. Myocardial disorders are a broad range of pathologies leading to restrictive physiology. Amyloidosis is a prototype of restrictive cardiomyopathy leading to diastolic dysfunction. Pericardial disorders leading to diastolic heart failure are usually in the form of constrictive physiology. Differentiation between restrictive and constrictive pathologies is often difficult and require careful attention to hemodynamic and Doppler echocardiographic features.

Cardiomyopathy, Restrictive↗

Simulation of acute myopericarditis by constrictive pericardial disease with endomyocardial fibrosis due to methysergide therapy.

Methysergide ( Sansert ) has been associated with numerous fibrotic disorders. In particular, multiple cardiac lesions have been described in cases where methysergide was thought to have played a causative role. A patient is described who presented with cardiac findings suggestive of acute myopericarditis . An inflammatory myocarditis was subsequently excluded by endomyocardial biopsy. Hemodynamic findings suggested the presence of constrictive pericarditis or restrictive cardiomyopathy, or both. Radiographic evidence of constrictive pericarditis and biopsy evidence of endocardial fibrosis were documented in this patient with a long history of interrupted methysergide therapy.

Acute Disease↗

[Ascites due to constrictive pericarditis].

INTRODUCTION: We report a case in which ascites revealed chronic constrictive pericarditis (CCP) of tuberculous origin. OBSERVATION: A 65 year-old man had developed ascites the past over few weeks and oedema of the lower limbs. Treatment was surgical and consisted in pericardiectomy. DISCUSSION: Chronic constrictive pericarditis has become rare in industrialised countries with the reduction in the incidence of tuberculosis. Currently, its principle aetiologies identified are previous cardiac surgery and radiotherapy. Treatment is usually surgical and gives excellent results. This clinical case clearly illustrates the interest of systematic biological analysis of ascites, since it may reveal a curable disease.

Aged↗

Progressive constriction of the anterior capsular opening following intact capsulorhexis.

The continuous curvilinear capsulorhexis has rapidly increased in popularity as the procedure of choice when using phacoemulsification for cataract extraction. Only recently, however, have complications of this technique been reported. We review the complications of a continuous curvilinear capsulorhexis and present three cases involving progressive constriction of the postoperative anterior capsular opening. One patient had a history of myotonic muscular dystrophy, another had pars planitis, and the third had high myopia. A review of ocular findings in myotonic dystrophy and pars planitis is also presented, and the possible pathophysiology of this progressive constriction is explored.

Adult↗