Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0728731 (prematurity)
 7,134 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Glucose-6-phosphate dehydrogenase (G6PD) deficiency was detected in 16 (69.6%) of a group of 23 neonates who had unexplained moderate or severe jaundice. This proportion is significantly more than the 9.4% observed or the 22.2% expected in Jamaican neonates who are not moderately or severely jaundiced (P less than 0.003), and significantly more than the 12.6% observed or the 21.0% expected in older Jamaican children and adults (P less than 0.003). Phenobarbitone therapy and phototherapy reduced the need for exchange transfusion but this was necessary in eight patients. Two babies developed kernicterus and one died. On the other hand, only two of 21 neonates who were identified as G6PD deficient at birth subsequently became moderately or severely jaundiced, and this could be attributed to other causes in both cases. These findings indicate that apparently spontaneous neonatal jaundice is important in infants who have the G6PD A--enzyme. However, the jaundice is probably precipitated by unknown factors to which the G6PD deficient neonate is more susceptible than the infant who is not G6PD deficient. THere is also a slightly increased incidence of G6PD deficiency in neonates who develop jaundice because of ABO or Rh(D) iso-immune disease, infection or prematurity.
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PMID:Glucose-6-phosphate dehydrogenase deficiency and neonatal jaundice in Jamaica. 50 36

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

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

To quantitatively assess the impact of phototherapy (PT) in the prevention of kernicterus we calculated the rate of exchange transfusion (ET) in two large historical cohorts of Greek neonates (birthweight > or = 2.5 kg), one before (period I: 1957-61) and one after (period II: 1980-92) the introduction of PT. Overall, the introduction of PT was associated with a reduction of the rate of ET from 0.43% in period I to 0.05% in period II. The reduction was observed in all etiological categories but was more marked in the ABO-incompatible group. With an estimated rate of kernicterus without treatment of 0.085% (excluding rhesus hemolytic disease of the newborn and prematurity) we estimated that 4.2 and 0.36 infants were treated by ET for each spared kernicterus in periods I and II, respectively. Finally, in period II 185 infants were treated with PT or PT and ET for each spared kernicterus.
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PMID:The impact of phototherapy in the management of neonatal hyperbilirubinemia: comparison of historical cohorts. 869 78

This review has two objects: a brief recapitulation of the biological background of erythropoietin (EPO), and a review of its clinical utilization in hematology. EPO, both in its naturally occurring and recombinant form (rH-EPO), is a single chain glycoprotein with an approximate molecular weight of 30.000 to 34.000 kD. Its heavy glycosilation is essential for its activity in vivo, since asialoEPO is readily cleared by the heptic asialoglycoprotein receptor. This impedes the recombinant molecule's synthesis in biologic cultures other than mammalian cells (Chinese hamster's ovary cells), and inevitably increases costs. If in vitro glycosilation of E. coli-derived rH-EPO could be achieved, the clinical utilization of the product would be considerably enhanced, most especially when very high doses are necessary, as discussed later. There is no antigenic diversity between natural and recombinant EPO, so that out of the enormous clinical experience only one single case of immunization has been recorded. Almost paradoxically there are however three published cases of pure red cell aplasia (PRCA) caused by immunization against autologous EPO. It is now established that in adults EPO is synthetized in renal peritubular interstitial cells, although some residual activity remains in the liver. Hypoxia results in a rapid induction of EPO expression, although the role of the oxygen sensor system is still debated. Cellular targets are notoriously erythroid progenitors and precursors (BFU-E, CFU-E, early and intermediate erythroblasts). The global erythropoietic activity resulted in various effects (proliferation, differentiation, survival), but most probably each single effect is integrated with and complementary of the others. The utilization of rH-EPO in hematologic diseases came much later than its dramatic success in renal anemia. A variety of tools useful for assessing the possible beneficial effects of rH-EPO in clinical hematology has been proposed, among which a low level of endogenous EPO is a good predictor for therapeutic success. 'Hemopathic' anemia can be subdivided into three categories: patients with normal erythropoiesis due to inadequate EPO production (anemia of prematurity), patients with depressed but nonclonal erythropoiesis (chemotherapy, lymphoid malignancies such as multiple myeloma-MM and chronic lymphatic leukemia-CCL) and patients with at least partially clonal anemia, such as paroxysmal nocturnal hemoglobinuria (PNH), hemoglobinopaties, myelodysplastic syndromes (MDS) and others. Results in the first category of patients are, as expected, prompt and satisfactory with physiologic doses. Although therapeutic strategy for MM is moving fast to curative intents, the utilization of rH-EPO is indicated for the control of anemia in conservatively-treated patients. In the third category the most important and controversial area is MDS. Significant erythropoietic results are generally obtained in about 20% of patients; however, the association with G-CSF has considerably enhanced the response rate. In the field of bone marrow transplantation there is an inadequate production of endogenous EPO in the allogeneic setting, and randomized studies have shown the benefits of rH-EPO in this situation. However, the most important results have been obtained in post-major-ABO incompatible PRCA, when the removal of the recipient's isohemagglutinins does not resolve the anemia. High and very high doses of rH-EPO (even over 500 UI/kg/day for 2-4 weeks) may resolve this occasionally quite refractory condition. Although extremely expensive, this treatment may be life-saving when an otherwise successful allogeneic transplant is at the risk of failure because of this relatively uncommon but severe immunohematologic complication.
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PMID:[Erythropoietin: biochemical characteristics, biologic effects, indications and results of use in hematology]. 948 78

Neonatal disorders mean disturbance of normal state of body, organs and abnormal function of a newborn. Obstetricians play a major role to minimise the number of neonatal disorders. Prematurity, respiratory dysfunction, birth trauma, congenital malformations, neonatal infection and haemolytic disorders of the newborn are some examples of neonatal disorders commonly encountered. Preventive obstetrics is most important in reducing these disorders. Regular antenatal check-up, balanced diet, iron and folic acid tablet, avoiding repeated pregnancies are some measures which can prevent prematurity. Any factors which cause maternal hypoxia during pregnancy are responsible for foetal hypoxia. Proper antenatal care and avoidance of narcotic drugs in pregnancy are the pillars to combat respiratory dysfunction. Obstetricians play an important part to minimise birth trauma which is single handedly an important example of neonatal disorders. Proper antenatal care to detect any obstetrical anomaly reduces birth trauma to a large scale. In case of congenital anomalies, genetic counselling and early abortion in gross congenital anomaly are important aspects which can be looked after by the obstetricians. Neonatal infections can be minimised by the obstetricians themselves if they take care of any suspicious vaginal discharge in antenatal period. Dirty dressings are to be avoided in delivery time. Proper immunisation to the mother and also counselling of HIV transmission are also important. Haemolytic diseases of the newborn can be confronted by proper Rh and ABO blood groupings in antenatal period and proper intervention at the time of delivery.
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PMID:Neonatal disorders and obstetricians. 1167 12

Hyperbilirubinaemia is common in the newborn period, and while the vast majority of babies are unaffected, significant neurological impairment remains a risk associated with extremely high levels of bilirubin. There is concern internationally that the number of babies affected by severe neonatal hyperbilirubinaemia may be increasing. This review describes the most current published data pertaining to the incidence and causes of severe neonatal hyperbilirubinaemia in order to determine whether concern regarding the possible re-emergence of kernicterus in Australia is warranted. Seven incidence studies conducted internationally between 1988 and 2005 identify an estimated incidence of severe neonatal jaundice of between 7.1 and 45 per 100,000 births and of kernicterus at 0.4-2.7. Major pathophysiological causes or associations include ABO and other blood group incompatibility, glucose-6-phoshate-dehydrogenase deficiency, infection and haemolysis of other causes including spherocytosis. Other factors associated with poor outcomes include prematurity, male gender, ethnicity, breastfeeding and early hospital discharge. The management of severe neonatal jaundice requires multifaceted risk quantification in addition to the availability of adequate surveillance, particularly in the context of early hospital discharge. It is of concern that currently there is a paucity of incidence data in Australia relating to this potentially devastating yet generally preventable condition. Therefore, a surveillance study has been initiated through the Australian Paediatric Surveillance Unit. It is anticipated that these data will accurately define the incidence in Australia and hopefully guide strategies to prevent a condition that we may have prematurely considered to be of historical interest only.
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PMID:Severe neonatal jaundice: is it a rare event in Australia? 2207 56