Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We reviewed jaundiced infants born between 1971 and 1989. Jaundice was diagnosed in infants whose serum bilirubin level was found to be 154 umol/l or greater. Of 88,137 livebirths, 10,944 (12.4%) were jaundiced. The most common aetiological factor was prematurity (20.3%), followed by ABO erythroblastosis (5.5%), sepsis (1.8%), Rh erythroblastosis (1.8%), bruising (1.3%), multifactorial (1.0%) and glucose-6-phosphate dehydrogenase deficiency (0.5%). In the remainder (67.8%) no cause was found or inadequate investigations were performed to determine a cause. During the period under review there was a significant increase (r = 0.91) in the proportion of newborn infants with jaundice of prematurity, in those not investigated (r = 0.92) and a decrease in the proportion with bruising (r = -0.90) as the cause. Phototherapy was used on 4,126 (37.7%) infants and exchange transfusion performed on 248 (2.3%). Causes of jaundice in infants requiring exchange transfusion were Rh erythroblastosis (108, 43.6%), ABO erythroblastosis (58, 23.4%), jaundice of prematurity (44, 17.7%) and a variety of causes in the remaining 38 (15.3%). Death occurred in 164 (1.5%) infants. In only 7 (4.3%), however, was the death possibly related to hyperbilirubinaemia or its treatment (Rh erythroblastosis (4), necrotizing enterocolitis following exchange transfusion (2) and pulmonary haemorrhage following exchange transfusion (1)). Phototherapy proved safe with no deaths attributable to its use.
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PMID:Jaundice: clinical practice in 88,000 liveborn infants. 144 22

Neonatal jaundice is a major clinical problem globally, especially in the Asian and south-east Asian regions. There is no universal definition of hyperbilirubinaemia, and comparisons of management and control of hyperbilirubinaemia in infants at different centres are difficult. G6PD deficiency, ABO incompatibility, low birth weight and sepsis are the common causes of neonatal jaundice, but there is a group of babies whose cause of neonatal jaundice has yet to be found. Genetic factors may be responsible for ethnic differences in the ability to eliminate bilirubin, while unidentified environmental factors may also play a role in the prevalence of neonatal jaundice. As a result of a surveillance programme for neonatal jaundice in Singapore, involving health education of doctors, nurses and the lay public, screening of the newborn and the early treatment of jaundice, we have not seen a single case of kernicterus in Singapore for more than 10 years.
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PMID:Neonatal jaundice in Asia. 159 89

To determine the susceptibility to sepsis in newborn infants deficient in glucose-6-phosphate dehydrogenase (G6PD), we screened 33,943 Saudi Arab infants. Deficiency of G6PD was found in 18%. Sepsis was determined by the presence of clinical signs of sepsis and confirmed by positive blood cultures. Sepsis was documented in 75 infants (2.2/1000). The incidence of sepsis was significantly higher in 6138 G6PD-deficient infants (3.4/1000) than in the 27,805 with normal G6PD activity (1.9/1000; p less than 0.02). The incidence of catalase-positive organism sepsis was higher in G6PD-deficient infants (2.9/1000) compared with those with normal G6PD activity (1/1000; p less than 0.0002), whereas the incidence of catalase-negative organism sepsis did not differ (p less than 0.2). Deficiency of G6PD was more common in infants with late sepsis (46%) than in those with early sepsis (21%) and in all infants screened (18%) (p less than 0.03 and p less than 0.001, respectively). We conclude that neonates with G6PD deficiency are more susceptible to late sepsis and to infection with catalase-positive organisms. The exact mechanism for the increased susceptibility is not clear, but a partial explanation could be lack of leukocyte bactericidal activity associated with G6PD deficiency, and an increased susceptibility to infection caused by hyperferremia resulting from lysis of G6PD-deficient erythrocytes.
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PMID:Incidence and causes of sepsis in glucose-6-phosphate dehydrogenase-deficient newborn infants. 271 88

We studied the interactions of the A- variety of glucose-6-phosphate dehydrogenase (G6PD) deficiency and sickle cell anemia (HbSS) to see if G6PD deficiency influenced laboratory and clinical features of HbSS. A total of 801 male patients over age 2 had G6PD electrophoresis on cellulose acetate membranes. Assays of both G6PD activity and hexokinase activity were then done on all samples that had an electrophoretic pattern other than the normal wild type (GdB). The collection of clinical data used a standardized protocol. Using cluster analyses we classified 10.4% males to be G6PD deficient, while 18.4% had the functionally normal GdA+ enzyme. The prevalence of G6PD deficiency did not change significantly when age was stratified by decade, suggesting little survival advantage or disadvantage of the combination of G6PD deficiency and HbSS. Compared to patients who were not G6PD deficient, there were no significant differences in the hemoglobin concentration, mean corpuscular volume, reticulocyte count, bilirubin, or SGOT level in patients with HbSS who had G6PD deficiency. The incidence of painful episodes, sepsis, or acute anemic episodes was similar in both groups. Our results are consistent with recent studies of smaller numbers of patients that have found little influence of G6PD deficiency upon HbSS. Specifically, we found no evidence that G6PD enhanced the severity of hemolysis or increased the incidence of acute anemic episodes or sepsis in HbSS.
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PMID:Effects of glucose-6-phosphate dehydrogenase deficiency upon sickle cell anemia. 334 44

A 52 yr old Caucasian female (F. E.) had hemolytic anemia, a leukemoid reaction, and fatal sepsis due to Escherichia coli. Her leukocytes ingested bacteria normally but did not kill catalase positive Staphylococcus aureus, Escherichia coli, and Serratia marcescens. An H(2)O(2)-producing bacterium, Streptococcus faecalis, was killed normally. Granule myeloperoxidase, acid and alkaline phosphatase, and beta glucuronidase activities were normal, and these enzymes shifted normally to the phagocyte vacuole (light and electron microscopy). Intravacuolar reduction of nitroblue tetrazolium did not occur. Moreover, only minimal quantities of H(2)O(2) were generated, and the hexose monophosphate shunt (HMPS) was not stimulated during phagocytosis. These observations suggested the diagnosis of chronic granulomatous disease. However, in contrast to control and chronic granulomatous disease leukocytes, glucose-6-phosphate dehydrogenase activity was completely absent in F. E. leukocytes whereas NADH oxidase and NADPH oxidase activities were both normal. Unlike chronic granulomatous disease, methylene blue did not stimulate the hexose monophosphate shunt in F. E. cells. Thus, F. E. and chronic granulomatous disease leukocytes appear to share certain metabolic and bactericidal defects, but the metabolic basis of the abnormality differs. Chronic granulomatous disease cells lack oxidase activity which produces H(2)O(2); F. E. cells had normal levels of oxidase activity but failed to produce NADPH due to complete glucose-6-phosphate dehydrogenase deficiency. These data indicate that a complete absence of leukocyte glucose-6-phosphate dehydrogenase with defective hexose monophosphate shunt activity is associated with low H(2)O(2) production and inadequate bactericidal activity, and further suggest an important role for NADPH in the production of H(2)O(2) in human granulocytes.
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PMID:Complete deficiency of leukocyte glucose-6-phosphate dehydrogenase with defective bactericidal activity. 440 Dec 71

This review has concentrated on clinical syndromes for which a congenital basis of polymorphonuclear neutrophil dysfunction has been identified. The first clinical syndrome found to be associated with dysfunctional polymorphs was chronic granulomatous disease of childhood. Identification of a cellular defect in oxidative metabolism and microbicidal activity of polymorphonuclear neutrophils from patients with CGD stimulated intense investigation of the function of phagocytes in several clinical entities characterized by increased susceptibility to infection. Other diseases with a probable congenital basis for polymorph dysfunction include Chediak-Higashi syndrome, myeloperoxidase deficiency, severe glucose-6-phosphate dehydrogenase deficiency, and Down's syndrome. Functional defects have also been identified in neutrophils with morphologic abnormalities, such as the Pelger-Huet anomaly and the May-Hegglin anomaly, and in neutrophils without alkaline phosphatase or with a disorder of the glutathione system. The evidence for a relation between these cellular disorders and susceptibility to infection is tentative. Patients with congenital disorders of polymorphonuclear neutrophil microbicidal function frequently suffer prolonged infections in spite of appropriate antimicrobial therapy, and severe lesions recur with discouraging frequency. These lesions are usually soft tissue or bone abscesses, and the etiologic agents are typically staphylococci, gram-negative enteric species, or fungi. The infectious disease problems of patients with phagocytic cell disorders are usually quite distinct from the problems of patients without immunoglobulins or with complement deficiency. Patients with agammaglobulinemia, for example, suffer recurrent septicemia or meningitis due to Streptococcus pneumonia or H. influenzae. Septicemia, especially with the pyogenic bacterial species, is unusual in patients with polymorphoinuclear dysfunction. A major contribution of the currently intense investigation of cells from patients with congenital disorders of phagocyte function has been the greatly increased understanding of the molecular events necessary for the normal function of these cells. The role of the oxidative metabolic burst during phagocytosis has been clearly identified as essential to the microbicidal function of polymorphs and monocytes, and the glutathione system has been identified as essential to the regulation of these oxidative reactions. It is anticipated that these studies may lead to practical methods for "stimulating the phagocytes" in patients with increased susceptibility to infection.
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PMID:Congenital disorders of the function of polymorphonuclear neutrophils. 625 30

A review is presented of jaundiced newborn infants during the 10-year period to 1980. Included are those whose serum bilirubin level was 154 mumol/l or more. Of 41,057 live births, 4,406 (10.7%) infants had hyperbilirubinaemia. The most common (19.9;%) aetiological factor was prematurity, followed by ABO erythroblastosis 7.1%; sepsis 3.4%; Rhesus erythroblastosis 2.7%; bruising 2.2%; multifactorial 1.0% and glucose-6-phosphate dehydrogenase deficiency 0.5%. Treatment was not undertaken in 2,855 (64.7%) infants, but 1,419 (32.2%) received phototherapy alone, 122 (2.7%) infants received both exchange transfusion and phototherapy and 10 (0.2%) infants received exchange transfusion alone. Of the infants requiring exchange transfusion 50.0% had Rhesus erythroblastosis, 28.0% ABO erythroblastosis, 10.6% jaundice of prematurity and the remainder were due to a variety of causes. Sixty-three (1.4%) infants died, with two deaths being related to the hyperbilirubinaemia, as their death was due to necrotizing enterocolitis following exchange transfusion. Phototherapy proved safe with no deaths directly attributable to its use.
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PMID:Jaundice: a 10 year review of 41,000 live born infants. 641 49

109 Nigerian neonates with serum bilirubin of 12 mg% and above, who presented at the Children's Emergency Room of the University College Hospital, Ibadan between November 1980 and February 1981 were investigated for bacterial sepsis and other causes of hyperbilirubinaemia. A detailed history of exposure to drugs likely to be icterogenic was also taken. Of the 109 infants, 67 (62%) were G6PD deficient and 41 (67%) of the latter had no obvious cause for the precipitation of jaundice. However 106 (97%) of the jaundiced infants had been exposed to agents capable of causing haemolysis in G6PD deficiency; 24 (22%) had bacteriologically proven septicaemia and in only five (4.6%) was sepsis the sole cause of hyperbilirubinaemia. There was no significant differences in the frequency of bacteriologically proven sepsis between the infants with normal or deficient G6PD status. Septicaemia however significantly increased the severity of jaundice among G6PD deficient infants. This study suggests that infection is common in severely jaundiced Nigerian infants and there is a need to reassess the role of exogenous agents in the pathogenesis of neonatal hyperbilirubinaemia in our community.
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PMID:A prospective study of the role of bacterial infection and G6PD deficiency in severe neonatal jaundice in Nigeria. 647 57

Thirty-five children with G6PD deficiency, who presented with acute intravascular haemolysis, were evaluated to define its aetiology, clinical features and ultimate outcome. All were boys with ages ranging from 6 months to 12 years. Pallor of abrupt onset and passage of cola-coloured urine were universal presenting symptoms. Incriminating factors responsible for haemolysis include hepatitis (7), malaria (4), bacterial sepsis (3) and drug intake (24), with more than one predisposing condition existing in some children. Marked elevations in serum bilirubin, coinciding with intravascular haemolysis, was a feature in all the seven children with hepatitis. Azotaemia was noted in 20 patients, of whom 14 did not have oliguria. All four children with malaria presented with protracted renal failure. Therapy focused on maintaining a high urine output in those without oliguria. A total of 15 peritoneal dialyses and five haemodialyses were required in six patients with acute renal failure, all of whom were oliguric. Supportive therapy consisted of blood transfusions and treatment of the predisposing diseases. Thirty-two children recovered completely while three died, the cause of death being severe anaemia and congestive cardiac failure, malaria with oliguric renal failure and hepatic encephalopathy, respectively.
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PMID:Acute intravascular haemolysis in glucose-6-phosphate dehydrogenase deficiency. 750 89

Of the 587 neonates born in ABUTH, Zaria, Nigeria and successfully followed up, 99 were clinically jaundiced (16.9%). Of these, only 38 (38%) had significant hyperbilirubinaemia (serum bilirubin above 170 umol/L). During the same period, 279 neonates were admitted through Emergency Paediatric Unit (EPU) of whom 70 (25%) were jaundiced and 64 (95%) of them had serum bilirubin above 170 umol/L. Jaundice was more severe and the incidence of kernicterus higher in babies born outside the hospital than in those born in hospital and periodically followed up. The incidence of kernicterus was 20.3% and 2.6% respectively. The pattern of aetiological factors was similar in the two groups of jaundiced neonates. Septicaemia (50%) and G6PD deficiency (40%) were the major aetiological factors. Exposure to traditional herbal medications, oxytocin induced/augmented labour, cephalhaematoma and tribal incidences did not play statistically significant roles. Jaundice due to Rh-incompatibility was not encountered. Results of this double prospective study were compared with the previous findings in this and other centres in Nigeria.
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PMID:Neonatal jaundice in Zaria, Nigeria--a second prospective study. 762 27


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