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)

Misalignment of pulmonary veins with alveolar capillary dysplasia (MPV) has been reported to be a rare cause of persistent pulmonary hypertension of the newborn (PPHN) and to be fatal despite extracorporeal membrane oxygenation (ECMO). A full-term female neonate with PPHN was brought to the hospital for ECMO therapy at 2 days of age. On the 14th day of life, she was extubated early after the second run of ECMO, and underwent nitric oxide (NO) inhalation therapy in the incubator. She died of catheter-related sepsis on the 61st day of life. After autopsy findings revealed MPV, the longest survival with this disease was documented. NO inhalation therapy in the incubator may provide time for lung transplantation.
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PMID:Nitric oxide inhalation therapy for an infant with persistent pulmonary hypertension caused by misalignment of pulmonary veins with alveolar capillary dysplasia. 902 81

Almost all respiratory diseases except benign lung tumors and lung dysplasia entail acute lung injury. The many clinical conditions associated with acute lung injury include aspiration pneumonia, bacterial pneumonia, and sepsis. The fundamental cause of acute lung injury is pulmonary vascular hyperpermeability. Pulmonary vascular hyperpermeability can be attenuated by nitric oxide and cyclic GMP, and potentiated by oxygen radicals and elastase released from neutrophils. Adhesion molecule inhibition could become an effective therapy against acute lung injury, because the adhesion molecules are very important in the pathogenesis of this condition. Adhesion molecules could also be useful markers of disease activity in various lung diseases. Neutrophil elastase inhibitors may become important as therapeutic agents against acute exacerbations of idiopathic interstitial pneumonia, because this pathological condition is a type of acute lung injury. Similarly, N-acetyl cysteine could also become a useful therapeutic agent against idiopathic interstitial pneumonia, because it is a precursor of glutathione, which is the major antioxidant in the fluid lining of the bronchial epithelium.
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PMID:[Pathophysiology of acute lung injury]. 921 75

In Japan, chronic lung disease (CLD) is defined as an oxygen requirement greater than that obtainable in room air at 28 days of age, with symptoms of persistent respirator distress and a hazy or emphysematous and fibrous appearance upon chest x-ray. A total of 4964 infants weighing less than 1500 g at birth and born in 1990 were admitted to and cared for at level II and III neonatal care centers in Japan. A total of 4293 infants (86.3%) survived at 28 days after birth. Analyses of infants who developed CLD through their preceding illnesses and chest x-ray findings resulted in the classification of CLD into six types. Types I and II are defined as CLD following the acute stage respiratory distress syndrome (RDS). Type I is the typical case of bronchopulmonary dysplasia (BPD) as described previously, whereas Type II shows atypical radiological findings, namely only diffuse haziness without typical emphysema and fibrosis. Type III has a history of intrauterine inflammation. Chest x-ray shows the typical bubbling and cystic appearance described in the original report of Wilson-Mikity syndrome or neonatal pulmonary emphysema in the very low birth weight infant. Type III also has atypical radiological findings in cases with intrauterine infection. Type IV does not have a history of either intrauterine inflammation or RDS but shows typical emphysematous and fibrous appearance upon chest x-ray. Type V includes those with atypical chest x-ray appearance similar to Type II but without history of RDS and intrauterine inflammation. CLD is a heterogeneous condition which shows different spectra. However, the cardinal event is common to all types--the excessive inflammatory response caused by various insults to the immature airways and alveoli, such as oxygen, barotrauma, infection and so on. The excessive inflammatory response leads to lung tissue damage and the abnormal healing process due to immaturity, (such as vitamin A deficiency and insufficient oxygen radical scavenging system) and results in dysplasia and metaplasia of the respiratory system. The treatment of respiratory distress due to CLD also acts as an insult to the lungs and thus forms a vicious cycle. The different spectra of the disease are most possibly attributed to the difference in the timing and the kind of insults to the lungs. In Type I and II CLD, the insults are given in the first hours of life when the infants with surfactant deficiency receive high concentrations of oxygen for stabilization before the surfactant replacement. In Type III and III' CLD the insults are most likely given before birth. Excessive and sustained inflammatory response in the lungs with different onset times may result in the development of Type IV and V CLD. The strategy for the prevention of CLD should be different according to the origins and causes. The prevention of Type I and II CLD, or CLD following RDS, should be accomplished by successful prophylactic surfactant replacement therapy. Another procedure may be the application of high frequency oscillatory ventilation (HFOV) in the acute stage of RDS or at the time of stabilization right after birth. Types III and III' CLD present the most difficult challenge for prevention strategy because the disease process already started before birth. At the moment there are no effective measures for prevention. The strategy for the prevention of Type IV and V CLD may reside in the early detection and treatment of patent ductus arteriosus, sepsis and airway infection including pneumonia.
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PMID:Chronic lung disease of the very low birth weight infant--is it preventable? 967 27

A persistent Gartner's duct cyst associated with ipsilateral renal agenesis or dysplasia is rare. A vaginal cyst at the introitus as the presenting complaint is very rare, and has not been previously described in a neonate. Sepsis despite the presence of renal agenesis, or non- or poorly functioning renal tissue, is an indication for ureterectomy or nephroureterectomy on the affected side.
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PMID:Gartner's duct cyst with unilateral renal dysplasia presenting as an introital mass in a new born. 1037 45

Almost all of respiratory diseases except benign lung tumors and lung dysplasia entail acute lung injury (ALI). The many clinical conditions associated with acute lung injury include aspiration pneumonia, bacterial pneumonia and sepsis. Acute lung injury is the end results of common pathways initiated by a variety of local or systemic insults leading to diffuse damage to the pulmonary parenchyma. Despite the accumulation of abundant information regarding the physiological and cellular basis of lung injury and increasing sophisticated intensive care, an improvement in prognosis has lagged behind. It has become clear that there is not one mediator responsible for ALI, but rather a complex interplay exists between diverse proinflammatory (e.g., lipopolysaccharide, complement products, cytocains, chemocains, reactive oxygen species and arachidonic acid products) and anti-inflammatory (IL-10, IL-1-RA, PGI2) mediators. Early in the course of ALI, large numbers of neutrophils are sequestered in and emigrate from the pulmonary capillaries. The fundamental cause of ALI is pulmonary vascular hyperpermeability caused by the activated neutrophils which release oxygen radicals and proteases. In these processes several adhesion molecules play very important roles. Neutrophil elastase inhibitors become very useful therapeutic agents against acute exacerbation of idiopathic interstitial pneumonia (IIP), because this pathological conditions is a type of ALI. Similarly, N-acetyl cystein could also become a useful therapeutic agent against IIP, because it is a precursor of glutathione, which is the major antioxidant in the fluid lining of the bronchial epithelium.
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PMID:[The 74th Annual Meeting President Lecture. Pathogenesis and therapy of acute lung injury]. 1053 83

Use of intraperitoneal insulin in diabetic end-stage renal disease (ESRD) patients receiving continuous ambulatory peritoneal dialysis (CAPD) is known to result in improved glycemic control. This route of insulin administration, although standard in adult diabetic CAPD patients, has not previously been reported in children. A 12-year old boy with ESRD from renal dysplasia who also had insulin-dependent diabetes mellitus (IDDM) was treated with CAPD and intraperitoneal insulin prior to renal transplantation. Diabetes and renal dysplasia were both diagnosed at 11 weeks of age. When he reached end-stage he was initially started on hemodialysis via a central line but was switched to CAPD because of recurrent line sepsis. His IDDM had been poorly controlled up to that time. CAPD was performed using 4 exchanges per day of 1.5% dialysate with a fixed dose of insulin added to each bag and with adjustments made based on blood glucose. His glycemic control markedly improved, with a fall in his glycosylated hemoglobin from 13.6% to 6%. CAPD was continued for 7 months until a living-related renal transplant was performed. Two episodes of peritonitis occurred while the patient received CAPD (1 episode/3.5 patient-months). We conclude that the use of intraperitoneal insulin in children with IDDM and ESRD leads to improved glycemic control. The rate of peritonitis, however, may be increased in these children.
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PMID:Improvement of glycemic control by CAPD with intraperitoneal insulin in a child with IDDM and ESRD. 1064 38

In 151 (17.5%) of 861 patients with liver cirrhosis regularly screened by sonography and determination of alpha-fetoprotein a hepatocellular carcinoma (HCC) was detected. Diagnosis was verified by sonographically guided fine needle puncture and exceptionally by laparoscopy and direct puncture. In 34 patients (22.5%) selection criteria for operation were a tumour diameter under 5 cm, no central localisation in the liver and at least 5 mm distant from the main structures; furthermore multilocular HCC and intra- and extrahepatic metastases were contraindications. Additionally Child-Pugh-classification should be A + B and urea synthesis rate 6 g per day. 27 patients (80%) had esophagogastric varices seen at endoscopy and 20 (59%) had bleeding episodes from varices managed endoscopically or surgically. Types of surgical resections were segmentectomy [17], bisegmentectomy [10] and oncologically defined wedge resections [7] using controlled hypotension and interrupted occlusion of the hepatoduodenal ligament. 4 patients (11.8%) died within 30 days of liver failure [3] and sepsis [1]. All patients could be followed up for eleven years: 18 patients died after 1.5-10 years of liver failure, tumour recurrence or second tumour and a cause not associated with HCC, 12 patients are living. Kaplan-Meier survival curves show that survival at 5 years is 50% and at 10 years 34%. The main indicators for a good prognosis were clinically the HBsAG-activity, the Child-Pugh-classification and the application of autologous blood, pathologic-anatomically the classification and grading and histologically the absence of vascular invasion, absence of satellites and a number of mitoses under 7 in the visual field. For tumour recurrence dysplasia is of positive influence.--Liver resection remains the most widely used therapeutic option for treatment of HCC in cirrhosis. The early and long-term results can be improved by early diagnosis, strict selection of patients for operation and the use of well defined clinical, pathological and histological criteria.
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PMID:[Small unilocular hepatocellular carcinoma (0 < 5 cm) in patients with liver cirrhosis. Early diagnosis, surgical indications, resection and prognosis]. 1096 Sep 74

Single-system ureteral ectopia (UE) encompasses a spectrum of malformations involving the bladder trigone, ureter, and kidney. The clinical presentation is variable, and both diagnostic and therapeutic problems are common. Reduced renal function in these patients may result from primary dysplasia, obstruction, vesicoureteral reflux, or recurrent infection. Based on our experience of seven patients, suggestions for diagnostic procedures and criteria for renal saving versus nephrectomy are offered. The relationship between ostium localization, renal function, and long-term results was investigated. From 1972 to 1990, five female and two male patients were studied. During the same period, 31 patients with UE and duplex kidneys were seen. Ages ranged from 1 day to 7 years. A ureteric opening into the bladder neck was associated with dilatation of the ureter and renal pelvis. Two patients had vaginal ectopia and severe renal dysplasia. In one, a cyst of the vaginal wall (Gardner's cyst) was detected at birth. A male newborn had multicystic renal dysplasia on the left and ureteric ectopia to the ductus deferens on the right side. To our knowledge, he is the first patient reported with renal function totally dependent on a kidney with severe UE. Follow-up ranged from 4 to 9 years. One patient died in the postoperative period because of renal failure and sepsis. All the others are well and have normal creatinine values. Improvement of renal function was noted after ureteral reimplantation (URI) in patients with bladder-neck ectopia. The numbers of infections were also drastically reduced. Our observations suggest that the combination of ultrasound, cyst urethrography, and cystoscopy will be diagnostic in most patients. A suspicion of UE should be raised in symptomatic patients with apparently solitary kidneys, enuresis ureterica, or atypical obstructive uropathy. Reduced renal function in some patients with ectopia to the bladder neck will improve after URI. This may be of importance in patients with bilateral anomalies and marginal renal function.
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PMID:Long-term results of treatment of single-system ectopic ureters. 1105 50

We report a patient with longstanding Crohn's disease (CD) developing recurrent sepsis and impaired neutrophil function tests. His inflammatory bowel disease was controlled with local steroids and sulfasalazine with only short exposure to azathioprine. His blood counts remained within normal range, but the marrow showed mild dysplasia. Repeated cytogenetic examinations revealed trisomies 8 and 9, which are typical for therapy related myelodysplasia. Fluorescent in situ hybridization (FISH) study showed stable persistent trisomies, confined to the myeloid lineage, one year after discontinuation of sulfasalazine. The long-term use of immunodulating agents in patients with CD is not without risks, and early therapy related myelodysplasia might not be easily detected by blood count and morphology assessment alone.
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PMID:Clonal marrow abnormalities after azathioprine and sulfasalazine exposure in Crohn's disease: a cautionary tale. 1240 Jun 12

The characteristics of severe neutropenia with a delayed onset following administration of rituximab have been evaluated in 53 consecutively treated patients. All but one patient received rituximab for the treatment of non-Hodgkin's lymphoma. Eight episodes of grade 4 neutropenia were detected between 1 and 5 months after rituximab, when administered alone on five occasions, and on three occasions in combination with chemotherapy, where neutrophil counts had recovered prior to the development of neutropenia. In three episodes, the patients presented with sepsis. Development of neutropenia did not correlate with either the presence of detectable disease or the administration of further treatment. Neutropenia was associated with selective depletion of neutrophil precursors in all but one episode, where it was associated with generalized bone marrow hypoplasia. All episodes developed after a period of either normal or mildly depressed neutrophil counts following treatment with rituximab, and persisted for between several days and several months, before undergoing spontaneous recovery in four instances, and after administration of filgrastim in the remainder. Episodes of neutropenia were associated with disordered immune status manifested by lymphopenia and hypogammaglobulinaemia, raising the possibility that either disturbance of the balance of lymphocyte subsets or an immune dyscrasia induced by rituximab resulted in the development of this type of neutropenia.
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PMID:Delayed-onset neutropenia associated with rituximab therapy. 1278 3


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