Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The purposes of this prospective repeated measures study were to: (a) describe the occurrence of withdrawal symptoms with the use of a standardised protocol to slowly taper opioids and benzodiazepines; and (b) to test the predictive validity of an opioid and benzodiazepine withdrawal assessment scoring tool in critically ill infants and young children after prolonged opioid and benzodiazepine therapy. Fifteen children (6 weeks-28 months of age) with complex congenital heart disease and/or respiratory failure who received opioids and benzodiazepines for 4 days or greater were evaluated for withdrawal symptoms using a standardized assessment tool. Thirteen children showed moderate to severe withdrawal symptoms a median 3 days after commencement of tapering. Symptom intensity was not related to prior opioid or benzodiazepine exposure, extracorporeal membrane oxygenation (ECMO) therapy or length of tapering. Children who received fentanyl in addition to morphine more often exhibited signs of withdrawal. This study demonstrated that significant withdrawal symptoms occur in critically ill children even with the use of a standardised assessment tool and tapering management protocol. The predictive validity and utility of the Opioid and Benzodiazepine Withdrawal Score (OBWS) was adequate for clinical use, but areas for further improvement of the tool were identified. Problems with the clinical withdrawal prevention and management guidelines were also identified. More research is needed to establish the optimal methods for prevention and management of iatrogenic opioid and benzodiazepine withdrawal in paediatric critical care.
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PMID:Opioid and benzodiazepine withdrawal symptoms in paediatric intensive care patients. 1556 75

Persistent pulmonary hypertension (PPHN) and subsequent hypoxic respiratory failure is seen in association with numerous diseases and conditions in the neonate. This includes infections such as group B streptococcus, meconium aspiration syndrome, perinatal asphyxia, congenital diaphragmatic hernia, congenital heart disease, and as an idiopathic phenomenon. Conventional therapy of persistent pulmonary hypertension is discussed, as well as integrated with current treatment modalities such as surfactant replacement therapy and high frequency ventilation. The molecular action of nitric oxide including its relationship to neonatal cardiopulmonary transition at birth and the human neonatal clinical experience with term infants from 1992 to the present is explored. Also, the current use of inhaled nitric oxide in preterm infants is reviewed. Additionally, the follow-up of infants treated with inhaled nitric oxide is summarized, and novel therapies including inhaled prostacyclin and other pulmonary vasodilators such as sildenafil are introduced.
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PMID:Inhaled nitric oxide in the treatment of persistent pulmonary hypertension/ hypoxic respiratory failure in neonates: an update. 1585 81

Although initially designed for respiratory failure, venoarterial extracorporeal membrane oxygenation (VA ECMO) has become a mainstay of therapy in the treatment of patients with congenital heart disease, providing preoperative and postoperative support for infants with temporary impairment of myocardial function. Postoperative support for pulmonary hypertension has allowed dramatic improvements in lesions such as total anomalous pulmonary venous connection. VA ECMO has also proven to be an acceptable bridge to cardiac transplantation in patients too small for currently available ventricular assist devices. Intraoperative elective use of ECMO has also provided a surgical environment with some specific advantages over conventional cardiopulmonary bypass for lesions such as long segment tracheal stenosis. The results of "rescue ECMO" or extracorporeal cardiopulmonary resuscitation (ECPR) has shown that many children with congenital heart disease are very salvageable despite sudden decompensation and arrest. These techniques of VA ECMO have evolved and improved over the last two decades, allowing expanded application of this life saving support.
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PMID:ECMO and congenital heart disease. 1592 Nov 50

A 74-year-old woman with chronic auricular fibrillation, arterial hypertension, hypercholesterolemia, ischemic cardiopathy, and peripheral arteriopathy presented with purpuric lesions on the lower limbs (Fig. 1) and, to a lesser extent, on the anterior area of the chest. The mucous membranes were not affected. In 1989, she was diagnosed with anemia that evolved until 1998, when a bone marrow biopsy revealed a myelodysplastic syndrome unclassified in French-American-British Group (FAB). The patient has required periodic transfusions since February 1999. A skin biopsy of the purpuric lesions revealed a leukocytoclastic vasculitis; the lesions cleared with topical corticosteroid treatment. In May 1999, the patient presented with inflammatory and painful lesions localized on the vulva (Fig. 2), which had evolved over several days, without fever. No lesions were observed in other locations. A cutaneous biopsy showed an intense dermal edema and a diffuse and polymorphous dermal infiltrate involving the follicular structures. Exocytosis, spongiosis, and mucin deposits, demonstrated by Alcian blue stain, were observed in the follicular epithelium. Mature neutrophils were predominant in the dermal infiltrate, but a small number of eosinophils and immature cells were also present (Fig. 3). The myelogenous origin of the immature lining cells was further confirmed by positive staining of intracytoplasmic granules with naphthol-ASD chloroacetate sterase (Leder's stain). Vasculitis was not observed. Routine laboratory tests revealed 3030 leukocytes/mm(3) (60% neutrophils), a hemoglobin level of 8.4 g/dL, and 92,000 platelets/mm(3). Treatment with 30 mg/day of prednisone was started, and the lesions cleared slowly within 4 weeks. A new bone marrow biopsy in September 1999 showed a similar appearance to that taken in 1998. The patient died in January 2000 as a result of pneumonia with cardiac and respiratory failure. A 66-year-old man presented with a febrile syndrome that had evolved over 5 days, and painful and pruritic cutaneous lesions on the face and posterior neck (Fig. 4). Three months before, the patient was diagnosed with chronic myelogenous leukemia in acceleration phase. Examination revealed an edematous and erythematous face with pustular lesions on the surface, also involving the neck and the upper part of the back. The histopathologic examination revealed an intense edema and abscesses in the dermis. The infiltrate of these lesions was composed of mature neutrophils with the presence of abundant immature cells with a myelogenous aspect (Fig. 5). Analytical studies revealed 26,130 leukocytes/mm(3) (42% blasts). No specific treatment for Sweet's syndrome was administered and the lesions showed an improvement within 5 days. Eight days after admission, the patient died as a result of acute hemorrhage, before treatment for leukemia was initiated.
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PMID:Concurrent Sweet's syndrome and leukemia cutis in patients with myeloid disorders. 1610 72

Due to the short supply of donor organs available, many patients decompensate or die while waiting for transplantation. Options for mechanical support for infants and pediatrics with congenital heart disease are limited because of the patient's size and device availability. Extracorporeal membrane oxygenation (ECMO) is the most common means of cardiac and respiratory support for these patients. One of the many indications for ECMO use in cardiac patients is as a bridge to transplantation, with patients being transported to the operating room (OR) on ECMO support. Converting the ECMO circuit to an open cardiopulmonary bypass system in the OR minimizes the patient's exposure to new circuitry, decreases further donor exposures and provides continuous support for patients in cardiac and/or respiratory failure. In addition, the ability to use modified ultrafiltration post-bypass aids in reducing extracellular fluid, increasing the hematocrit and improving hemodynamic stability following an extended duration of ECMO and bypass support. The integrity of the ECMO circuit is maintained and can be converted back to ECMO for support postoperatively if needed.
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PMID:Extracorporeal membrane oxygenation to cardiopulmonary bypass with a single circuit exposure. 1623 27

To evaluate whether the system-based strategy for management of meconium aspiration syndrome (MAS) could reduce the morbidity and mortality rate of MAS in our institute, a prospective consecutive clinical observation was conducted. System-based strategy including appropriately trained the relevant medical staff to familiar with neonatal resuscitation program, early surfactant replacement or lavage following with high-frequency ventilator (HFV) and/or inhaled nitric oxide (iNO). Outcome measurements were the morbidity and mortality rates of MAS. All infants of MAS in the study period were included except cases of congenital malformations or cyanotic congenital heart disease (CHD). Oxygen, nasal continuous positive airway pressure (CPAP), and intermittent mandatory ventilation (IMV) were applied as clinically indicated. Surfactant was used as replacement or lavage therapy for MAS infants whose oxygen index (OI) exceeded 20 or value for AaDO2 exceeded 400 within 6 hours of age. High-frequency oscillator ventilation (HFO) was applied for infants of MAS that demonstrated intractable respiratory failure with conventional mechanical ventilation and 100% oxygen. Inhaled nitric oxide (iNO) was used with IMV or HFO for infants of persistent pulmonary hypertension (PPHN) when it was unresponsive to conventional therapy. Dexamethasone was prescribed in infants of severe hypotension that did not respond to dopamine and epinephrine. A series of 198 consecutive infants of MAS born in this hospital during 9 years were analyzed. There was no mortality. Fourteen infants developed PPHN, 11 had pneumothorax, 1 had pulmonary hemorrhage, 2 had neurologic sequelae because of severe asphyxia, and 2 developed bronchopulmonary dysplasia. Our results indicated that appropriately trained relevant medical staff with neonatal resuscitation program to avoid complicated MAS and early surfactant replacement or lavage following with HFO and/or iNO could reduce the morbidity and mortality rate of MAS even without extracorporeal membrane oxygenation (ECMO).
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PMID:System-based strategy for the management of meconium aspiration syndrome: 198 consecutive cases observations. 1630 81

We retrospectively reviewed the files of 19 extracorporeal life support (ECLS) applications performed after cardiac surgery in 15 patients from January 2002 to December 2004. We placed 16 arteriovenous ECLS applications with oxygenator, 2 venovenous ECLS applications with oxygenator, and 1 biventricular ECLS application without oxygenator (graft dysfunction after heart transplant). Mean age was 4.9 +/- 7 years (median 5.9 months, range 11 days to 21 years). All patients underwent surgery for congenital heart disease, except for one patient who had a heart transplant. Indications were hemodynamic failure in 12 cases, respiratory failure in 5 cases, and mixed failure in 2 cases. Four patients were undergoing cardiopulmonary resuscitation during ECLS placement (no deaths). Mean delay between surgery and ECLS placement was 3.2 +/- 3.4 days (median 2 days). Mean ECLS duration was 3.4 +/- 5.8 days (mean 6 days, range 3-16 days). Three patients had further surgery for residual lesions. Thirteen patients (86.7%) survived to ECLS weaning; 12 patients survived to hospital discharge (80%). No survivor presented obvious neurologic damage. Specific morbidity included reentry for bleeding, multiple transfusions, and mediastinitis. These results support early placement of ECLS in children whenever a severe postoperative hemodynamic or respiratory failure, refractory to medical treatment, is present.
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PMID:Postoperative extracorporeal life support in pediatric cardiac surgery: recent results. 1632 8

Infective endocarditis, a serious microbial infection of the cardiac endothelial surface, may involve any heart valve. However, right-sided endocarditis is uncommon in non-intravenous drug abusers without underlying heart disease, and the contextual involvement of the left-sided valve is exceptional. A 63-year-old man with no evidence of intravenous drug abuse or heart disease, presented with persistent fever, worsening of breath, and aphasia. His medical history was notable for mild arterial hypertension and serious lymphangitis with cutaneous erosions on the legs. Transesophageal echocardiography was performed for suspicious endocarditis and showed a pedunculated and highly mobile vegetation adhered to the atrial portion of the posterior leaflet of the mitral valve, protruding into the left ventricle through the valvar orifice. Another large vegetation was seen at the tricuspid valve surface and protruded into the right ventricle during diastole. Cerebral and thoraco-abdominal computed tomography scan revealed multiple embolism to the left kidney, spleen, lungs and central nervous system. Blood cultures identified Staphylococcus aureus. The only risk factor was large skin sepsis. Despite successful antibiotic therapy, the patient died for development of renal and respiratory failure.
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PMID:[Widespread embolism in tricuspid and mitral endocarditis caused by chronic lymphangitis. Case report]. 1653 26

The diagnosis of total anomalous pulmonary venous connection (TAPVC) is made when all four pulmonary veins drain anomalously to the right atrium or to a tributary of the systemic veins. It constitutes between 1% and 1.5% of all children with congenital heart disease and can be categorized by the site of drainage into the systemic circulation (supracardiac, 45%; infracardiac, 25%; cardiac, 25%; mixed, 5%). The clinical presentation is different if the pulmonary venous drainage is unobstructed (heart failure, mild cyanosis) or obstructed (respiratory failure, severe heart failure). Surgical management depends on the anatomic type. Obstructed TAPVC requires urgent surgical intervention, whereas unobstructed TAPVC can be dealt with electively; although this is usually operated on once the diagnosis is made. Postoperative pulmonary artery hypertension can be problematic. Recent surgical results with isolated TAPVC have improved, with operative mortality consistently at less than 10%. A particularly challenging group of patients are those with single ventricle physiology and TAPVC with high operative mortality and poor long-term survival.
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PMID:Surgical repair of total anomalous pulmonary venous connection. 1663 46

Differential diagnosis of neonatal respiratory distress includes pulmonary and systemic disorders and anatomic problems compromising respiratory system. We report on a 2770-g female born to a 29-year-old gravida 3, para 2 woman after 34 weeks of gestation. Antenatal ultrasound performed in week 8 and 21 was normal. The infant was delivered by cesarean section after amniotic membranes had been ruptured for less than 12 hours due to signs of fetal distress. The Apgar score was 3 and 3 at 1 and 5 minutes, respectively. The infant was intubated and resuscitated, and transferred immediately to the neonatal intensive care unit. She had an extremely protuberant and cyanotic abdomen. Dilated cutaneous collateral vessels were apparent in the periumbilical region. Abdominal sonography showed cystic multiloculated tumorous mass filled with dense, flocculent content at the level of hepatic portal. The tumorous mass occupied the majority of the abdomen with caudal extension toward the pelvis and dorsally toward the spine. The liver was displaced high under the diaphragm with the left liver lobe in the left hemiabdomen. On x-ray the lung were collapsed due to a large abdominal mass in the right hemiabdomen that displaced the right diaphragm and intestines contralaterally. She soon developed bilateral pneumothoraces. Drainage and continuous suction were started. The infant failed to improve despite all attempts and died. On autopsy, an extremely large, mobile, multichambered, solitary cyst was found. It was attached to the mesenteric side of the ileum by its own thin peduncular stalk and had no communication with the remainder of the gut. It occupied the majority of the abdomen. Histologic section revealed a well-developed smooth muscle wall and inner mucosa of small bowel type. Respiratory distress is a common problem in premature infants. The majority of cases are due to pulmonary disorders (e. g., hyaline membrane disease, meconium aspiration syndrome, pneumonia), hypothermia, metabolic acidosis, anemia, and congenital heart disease. Anatomic problems including space occupying lesions are less common. Duplications of the alimentary tract in infants and children are rare congenital anomalies. Although symptoms can occur at any age, they usually present during the first year. In our patient, intraabdominal mass caused severe respiratory distress and respiratory failure in the first hours of postnatal life. This had been seen before only as a complication of intrathoracic lesions extending into the abdominal cavity. Pathology revealed spherical intestinal duplication that was completely separated from the alimentary tract. Embryologically, it was a localized duplication. Respiratory distress in our patient was refractory to all means of mechanical ventilation. Poor lung compliance was the consequence of prenatal lung hypoplasia and inadequate postnatal lung expansion due to the duplication cyst space occupying character and its compressive effect. Prenatal diagnosis was the child's only chance for survival but it was not made. Duplications of the alimentary tract can present a diagnostic challenge even in the first hours of life. They should be included in the differential diagnosis of severe respiratory distress, especially in premature infants in which timely prenatal diagnosis cannot be always made. We propose their inclusion among other space occupying lesions that might be the cause of severe respiratory distress even in the earliest neonatal period.
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PMID:[Severe respiratory distress due to ileal duplication cyst in the newborn]. 1680 74


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