Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0243026 (sepsis)
 52,417 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A twenty one years old man with obstructive hypertrophic cardiomyopathy with resting gradient and which develops subacute infectious endocarditis and acute mitral regurgitation by valvular apparatus destruction. During the course occurs refractory heart failure and sepsis. The association between these diseases and difficulties in management are analyzed, and literature is reviewed. It is emphasized the high mortality of this condition and indication for surgical referral, as well the necessity for infectious endocarditis prophylaxis in patients with obstruction at rest.
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PMID:[Refractory heart failure and sepsis in a patient with hypertrophic cardiomyopathy]. 828 67

Left ventricular (LV) noncompaction is a rare abnormality characterized by more than three trabeculations protruding from the LV wall, distal to the papillary muscles and visible in one echocardiographic image plane. The intertrabecular spaces are perfused from the LV cavity, as visualized on color Doppler imaging. Differential diagnoses of LV noncompaction are intraventricular thrombi, false tendons, aberrant bands, intramyocardial hematoma, cardiac metastases and the apical type of hypertrophic cardiomyopathy. Intramyocardial abscesses have not been reported as a differential diagnosis of LV noncompaction. In the patient presented, cardiac microabscesses due to candida sepsis mimicked LV noncompaction and should be considered in the differential diagnosis of LV noncompaction.
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PMID:Candida sepsis with intramyocardial abscesses mimicking left ventricular noncompaction. 1511 16

Hypertrophic obstructive cardiomyopathy with significant hypertrophy of the basal septum is the most frequently reported cause of left ventricular outflow tract (LVOT) obstruction. Additionally, other conditions such as dehydration, sepsis, vasodilatation, or mitral valve repair have been associated with LVOT obstruction. In this report, we present a case of a patient without hypertrophy who developed severe dynamic left ventricular outflow tract obstruction during catecholamine stimulation for shock that complicated severe pancreatitis. The present case serves as a reminder that hypovolemia together with a hyperdynamic state resulting from catecholamine administration may result in the development of dynamic LVOT obstruction even if baseline cardiac evaluation is unremarkable. Early detection and intensive efforts to reverse the underlying conditions, including cessation of catecholamine therapy and correction of hypovolemia are essential.
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PMID:Catecholamine therapy inducing dynamic left ventricular outflow tract obstruction. 1588 88

Hypertrophic cardiomyopathy (HCM) is the most common cause of left ventricular outflow tract (LVOT) obstruction. The LVOT obstruction is a consequence of the asymmetric septal hypertrophy and the mitral systolic anterior motion (SAM), causing both of them a dynamic gradient in LVOT. LVOT obstruction has been observed also in other conditions like hypertensive hypertrophy, dehydration, sepsis, vasodilatation, excessive sympathetic stimulation, pericardial tamponade, and after mitral valve repair and aortic valve replacement for aortic stenosis. We report in this document the case of two patients who developed a significant gradient at LVOT in the context of amine treatment during their admission into the intensive unit care. In both, cases there were no gradient, significant hypertrophy or SAM at baseline cardiac evaluation. We have met only one case reported in the literature matching those conditions. In order to treat this type of patients properly, it is essential to take in consideration this pathology.
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PMID:Dynamic left ventricular outflow tract obstruction secondary to catecholamine excess in a normal ventricle. 1629 Feb 26

In this report, we describe a brother and sister who presented at birth with short-limb skeletal dysplasia, polyhydramnios, prematurity, and generalized edema. Dysmorphic features included broad nose, thick ears, thin lips, micrognathia, inverted nipples, ulnar deviation at the wrists, spatulate fingers, fifth finger camptodactyly, nail hypoplasia, and talipes equinovarus. Other features included short stature, microcephaly, psychomotor retardation, B-cell lymphopenic hypogammaglobulinemia, sensorineural deafness, retinal detachment and blindness, intestinal malrotation with poor gastrointestinal motility, persistent hyponatremia, intermittent hypoglycemia, and thrombocytopenia. Cardiac anomalies included PDA, VSD, hypertrophic cardiomyopathy, and arrhythmias. The brother had a small penis with hypospadias, hypoplastic scrotum, and non-palpable testes. Skeletal findings included absent ossification of cervical vertebral bodies, pubic bones, knee epiphyses, and tali. Both sibs died before age 2 years, one of overwhelming sepsis and the other of cardiorespiratory failure associated with her cardiomyopathy. Metabolic studies showed a type 1 pattern of abnormal serum transferrin glycosylation. Fibroblasts synthesized truncated LLOs, primarily Man(7)GlcNAc(2), suggestive of CDG-Ig. Both sibs were compound heterozygotes for a novel 301 G > A (G101R) mutation and a previously described 437 G > A (R146Q) mutation in ALG12. Congenital disorders of glycosylation should be considered for children with undiagnosed multi-system disease including neurodevelopmental delay, skeletal dysplasia, immune deficiency, male genital hypoplasia, and cardiomyopathy.
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PMID:Expanding spectrum of congenital disorder of glycosylation Ig (CDG-Ig): sibs with a unique skeletal dysplasia, hypogammaglobulinemia, cardiomyopathy, genital malformations, and early lethality. 1750 7

Cardiac transplantation is currently the only established surgical approach to the treatment of refractory heart failure. Heart transplantation because of amyloid cardiomyopathy continues to generate controversy because of donor shortage and concerns about disease recurrence in the allograft. We reviewed the medical records for all patients who underwent heart transplantation at our institution from 1987 to 2007, and found that 4 patients were diagnosed as having amyloid cardiomyopathy after pathologic examination of the excised hearts. No operative mortality was noted; however, all of the patients died of sepsis after transplantation. Because of the poor results, we do not recommended performing transplantation in patients with amyloidosis. Preoperative surveys and evaluation for amyloidosis must be emphasized in patients with hypertrophic cardiomyopathy.
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PMID:Heart transplantation in patients with amyloidosis. 2043 Feb 6

A 55 year old male patient with a diagnosis of hypertrophic cardiomyopathy was admitted with features of sepsis related to cholangitis. Initial management with intravenous (i.v.) fluids and antibiotics did not cause any change in his general condition mandating an emergency endoscopic retrograde cholangio-pancreatography (ERCP). After successful retrieval of CBD stone on ERCP, patient had massive upper gastrointestinal bleed leading to hypotension and shock. Addition of inotropes had led to further deterioration in his clinical status with a mean arterial BP falling to 44 mm of Hg. His echocardiography showed a resting left ventricular outflow tract (LVOT) gradient of 90 mm of Hg and thus was taken up for emergency alcohol septal ablation (ASA). Immediately after ASA, patient had significant decrease in LVOT obstruction and rise of systemic arterial pressures. After 10 days of antibiotic therapy patient was discharged with a residual LVOT obstruction of 28 mm of Hg.
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PMID:Rescue alcohol septal ablation in sepsis with multiorgan failure. 2325 12

Heart transplantation started in Japan in 1999. Since then, 50 transplants have been performed at our center. We performed histopathological analyses of the 50 explanted hearts and the post-transplant biopsy specimens. The median age of recipients was 39 years. The primary diseases before transplant were idiopathic dilated cardiomyopathy in 33 patients (66%), hypertrophic cardiomyopathy in seven (14%), restrictive cardiomyopathy in one, arrhythmogenic right ventricular cardiomyopathy in one, and secondary cardiomyopathy in eight (16%). Before transplantation, 47 patients (94%) had left ventricular assist devices. No severe cardiovascular failure due to allograft rejection occurred. The post-transplant survival rate was 97.6% at 1 year and 93.1% at 10 years. One recipient was lost to sepsis from myelodysplastic syndrome in the fourth year, one died of multiple organ failure and peritonitis 8 months after transplant. Another patient died of recurrent post-transplant lymphoproliferative disorders (PTLD). Mild cardiac dysfunction occurred in seven recipients in the early postoperative period. Moderate acute cellular rejection occurred in six patients (12%), and antibody-mediated rejection occurred in three (6%). The number of heart transplants performed in Japan is very small. However, the outstanding 10-year survival rate is due to donor evaluation and post-transplant care resulting in low grade rejection. Pathological evaluation has also greatly contributed to the results.
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PMID:The pathological implications of heart transplantation: experience with 50 cases in a single center. 2514 28

Antenatal steroid treatment to enhance fetal lung maturity and surfactant treatment to prevent or treat respiratory distress syndrome have been major advances in perinatal medicine in the past 40 years contributing to improved outcomes for preterm infants. Use of postnatal steroids to prevent or treat chronic lung disease in preterm infants has been less successful and associated with adverse neurodevelopmental outcomes. Although early (in the first week of life) postnatal steroid treatment facilitates earlier extubation and reduces the risk of chronic lung disease, it is associated with adverse effects, such as hyperglycemia, hypertension, gastrointestinal bleeding and perforation, hypertrophic cardiomyopathy, growth failure, and cerebral palsy, and cannot be recommended. Early treatment with hydrocortisone may also improve survival without chronic lung disease, but concerns remain about possible adverse effects such as gastrointestinal perforation and sepsis, particularly in very preterm infants. Early inhaled budesonide also reduces the incidence of chronic lung disease but there are concerns that this may occur at the expense of increased risk of death. More studies of early low-dose steroids with adequate long-term follow-up are needed before they can be recommended for the prevention of chronic lung disease. Late (after the first week of life) postnatal steroids may have a better benefit-to-harm ratio than early steroids. A Cochrane Review shows that late steroid treatment reduces chronic lung disease, the combination of death and chronic lung disease at both 28 days and 36 weeks' corrected age, and the need for later rescue dexamethasone. Adverse effects include hyperglycemia, hypertension, hypertrophic cardiomyopathy, and severe retinopathy of prematurity but without an increase in blindness. Long-term neurodevelopmental effects are not significantly increased by late postnatal steroid treatment. Current recommendations are that postnatal steroid treatment should be reserved for preterm infants who are ventilator-dependent after the first 7-14 days of life and any course should be low dose and of short duration to facilitate endotracheal extubation. Budesonide/surfactant mixtures show some promise as a means of reducing chronic lung disease in preterm infants with severe respiratory distress syndrome, but further larger studies with long-term follow-up are needed before this treatment can be recommended as a routine intervention.
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PMID:Update on Postnatal Steroids. 2853 37