Hemolytic Jaundice Research Articles

Hemolytic Jaundice Due to G6PD Deficiency Causing Kernicterus in a Female Newborn

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an inherited deficiency of an enzyme necessary to protect the erythrocyte from oxidative stress and hemolysis. Without this enzyme, affected neonates are at risk for acute onset of hemolytic jaundice and severe sequelae, from hearing loss and mild retardation to kernicterus. In some populations, especially in blacks and those of Mediterranean ancestry, the incidence of G6PD deficiency has been reported to be as high as 10% to 14%. We describe a female newborn who had acute onset of hyperbilirubinemia leading to kernicterus in the first week of life. Investigation proved G6PD deficiency. This case suggests a need to screen for this disease or to follow serial bilirubin levels in populations at risk.

Carboxyhemoglobin Levels in Neonatal Immune Hemolytic Jaundice Treated with Intravenous Gammaglobulin

Carboxyhemoglobin Levels in Neonatal Immune Hemolytic Jaundice Treated with Intravenous Gammaglobulin

A congenital haemolytic anaemia and jaundice of Murray Grey calves.

A congenital haemolytic anaemia and jaundice of Murray Grey calves.

Preoperative transfusion in sickle cell anemia

American Journal of HematologyVolume 38, Issue 2 p. 156-157 Letter to the EditorFree Access Preoperative transfusion in sickle cell anemia Samuel Charache, Samuel Charache Professor of Medicine and Laboratory Medicine Johns Hopkins University School of Medicine Baltimore, MarylandSearch for more papers by this author Samuel Charache, Samuel Charache Professor of Medicine and Laboratory Medicine Johns Hopkins University School of Medicine Baltimore, MarylandSearch for more papers by this author First published: October 1991 https://doi.org/10.1002/ajh.2830380220Citations: 5AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL No abstract is available for this article. References 1 National Institutes of Health: Perioperative red cell transfusion. Cons Dev Conf Statement 7: 1– 6, 1988. 2 National SCA Preoperative Transfusion Study Group: Preoperative transfusion in sickle cell anemia (abstract). Blood 74: 184a, 1989. 3 Lane PK, Embury SH, Toy PTCY: Oxygen-induced marrow red cell hypoplasia leading to transfusion in sickle painful crisis. Am J Hematol 27: 67– 68, 1988. 4 Taylor C, Rogers G, Goodman C, Baynes RD, Bothwell TH, Bezwoda WR, Kramer F, Hattingh J: Hematologic, iron-related, and acute-phase protein responses to sustained strenuous exercise. J Appl Physiol 62: 464– 469, 1987. 5 Schooley JC, Kullgren B, Allison AC: Inhibition by interleukin-1 of the action of erythropoietin on erythroid precursors and its possible role in the pathogenesis of hypoplastic anaemias. Br J Haematol 67: 11– 7, 1987. 6 Maury CPJ, Lahdevirta J: Correlation of serum cytokine levels with haematological abnormalities in human immunodeficiency virus infection. J Int Med 227: 253– 257, 1990. 7 Owren PA: Congenital hemolytic jaundice. The pathogenesis of the “hemolytic crisis”. Blood, 3: 231– 248, 1948. 8 Pattison JR, Jones SE, Hodgson J: Parvovirus infections and hypoplastic crisis in sickle cell anaemia. Lancer 1: 664– 665, 1981. Citing Literature Volume38, Issue2October 1991Pages 156-157 ReferencesRelatedInformation

Etiopathogenesis of Craniosynostosis

Craniosynostosis is both etiologically and pathogenetically heterogeneous. Known causes include monogenic and chromosomal disorders, fetal head constraint, teratogens (aminopterin, diphenylhydantoin, retinoic acid, valproic acid), metabolic diseases (hyperthyroidism, rickets, mucopolysaccharidoses, mucolipidoses), hematologic disorders (thalassemias, sickle cell anemia, congenital hemolytic icterus, polycythemia vera), and malformations (microcephaly, encephalocele, shunted hydrocephaly, holoprosencephaly).

老齢Slc: Wistarラットにみられた自然発生白血病とラットへの可移植性

Spontaneous leukemia occurred in 36% of a group of 39 aged Slc: Wistar rats, which ranged in age at death from 99 to 123 (average age: 114) weeks old. The leukemic rats showed hemolytic anemia, jaundice and leukocytosis (21,000 to 360,000/mm3). Macroscopically, they were characterized by splenomegaly and hepatomegaly. The leukemia cells were rich in basophilic cytoplasm containing eosinophilic granules. Their nuclei were oval and occasionally revealed mitotic figures. These leukemia cells were consistently transplantable into both Slc: Wistar and F 344/NSlc rats. In the transplanted cases, the latency and survival periods were prolonged in proportion to decreases in the inoculated cell dose.

Green light phototherapy in newborninfants with ABO hemolytic disease

The efficacy of fluorescent green light phototherapy was compared with that of blue light phototherapy in the treatment of full-term infants with hemolytic disease and jaundice caused by ABO incompatibility. The efficacy of the treatment was expressed as actual (milligrams per hour) and quantum (milligrams per hour per square centimeter per megawatt) efficiency, taking into account the differential emission of energy from the green versus the blue fluorescent tubes. No statistically significant difference in the rate of serum bilirubin photodegradation was found between the two groups after treatment for 84.6 +/- 14.1 hours versus 81.5 +/- 14.2 hours with the green and the blue phototherapy, respectively. These results, coupled with the known effects of the blue light on the genetic apparatus of mammalian cells, support the application of the green light phototherapy for the treatment of neonatal hyperbilirubinemia caused by ABO incompatibility.

Esferocitosis hereditaria en el periodo de recién nacido

The clinical and hematological features of seven newborn infants with hereditary spherocytosis (HS) wereanalyzed, 5 of them had familial antecedents of the disease. All 7 patients with neonatal HS had early and intensejaundice. Phototherapy was employed in all of them, but 3 required exchange transfusions. The evolution of thesepatients showed the same patterns that those cases whose HS diagnosis was established at later ages. Another 29cases, recognized after the neonatal period, were also analized. In this later patients pathological neonatal jaundicewas noted in 44.8% of the cases. Probably HS with hemolytic jaundice in the newborns is undcrdiagnoscd andshould be investigated in every patient with neonatal jaundice of unknown origin: familial antecedents of US nuistbe inquired, specially if there is anemia, and complete hematological study (osmotic fragility test included)toghcther with a differential diagnosis with ABO incompatibility should also be done.(Key words: Hereditary spherocytosis, signs and symptoms, neonatal period, newborn infants, early manifestations).

抗Ｌｅ｀ｂ´抗体による溶血反応を呈した１症例

A case of hemolytic transfusion reaction due to Lewis b antibody was reported.The patient, 62-year-old farmer, was suffered from echinococcosis. He has no history to be transfused. His blood types were A, CCDee, Le (a-b-), MNSs, P2, kk, Fy (a+b-), Jk (a+b+), Xg (a+), Di (a-), and he had anti Lea & Leb antibodies.Right hepatic lobectomy was undergone. Total amount of bleeding in this operation was about 3, 000ml. And during the operation, 1, 600ml of Le (a-b-) blood and 800ml of Le(a-b+) blood was given.Two days after the operation, hemolytic jaundice and bleeding tendency from DIC appeared. Some part of the lost blood volume was replaced by incompatible Le (a-b+) blood, because Le (a-b-) blood was difficult to obtain due to its low frequency. Moreover few papers reported on hemolytic reaction by anti Leb antibody. Hemolytic reaction and DIC syndrome became even stronger as the days go by.After the transfusions of Le (a-b-) blood units alone, hemolytic anemia, jaundice and bleeding tendency were improved before long. Anti-Leb antibody titer which elevated initially decreased gradually after stoppage of using Le (b+) blood.According to these clinical features and laboratory data, we concluded that this hemolytic reaction was occured from Le (b+) incompatible blood transfusion.

Haemolytic Jaundice in a Neonate after Intra-amniotic Injection of Methylene Blue

A child developed haemolytic jaundice requiring exchange transfusion after intra-amniotic injection of methylene blue, a dye used for detecting premature rupture of membranes. Since no other cause of haemolysis could be found, methylene blue was strongly implicated as the causative agent and its use for this purpose should be avoided.

Hereditary Elliptocytosis with Hemolytic Jaundice following Infectious Mononucleosis

Hereditary Elliptocytosis with Hemolytic Jaundice following Infectious Mononucleosis

PREDICTION OF THE SEVERITY OF ABO HAEMOLYTIC DISEASE OF THE NEWBORN BY CORD BLOOD TESTS

Seventy-one ABO incompatible (heterospecific) infants and 71 controls, who were free from other potential causes of jaundice, were studied to ascertain which cord blood tests reliably predict the severity of ABO haemolytic disease of the newborn (ABO HDN). The modified Direct Antiglobulin Test (spin DAGT) was positive in all infants who required treatment for haemolytic jaundice and only DAGT positive children showed evidence of impending haemolytic anaemia or compensated haemolysis in cord or capillary blood. Cord serum bilirubin concentration had some predictive value, particularly when the level exceeded 85 mumol/l, but it was a less reliable indicator and had greater value if used in association with the DAGT. The elution test, which is frequently used as a diagnostic tool in ABO HDN, had no predictive value and we felt that its putative value is due to overdiagnosis of ABO HDN in jaundiced heterospecific infants. We conclude that the spin DAGT, despite the weakness of the reaction, reliably identifies infants at risk from severe ABO HDN and is sufficiently sensitive to be used as a single screening test for the early detection of the disorder.

Dynamics of protozoan population density, plasma glutamic oxalacetic transaminase and plasma bilirubin concentrations during histomoniasis in turkeys

Density of populations of Histomonas meleagridis in ceca of infected turkeys was recorded daily for the first 17 days postinoculation with 20,000 protozoa. The major features of population growth were a 5-day lag period followed by rapid growth and plateau on the order 10 7 protozoa/ml. Protozoan populations in hosts that failed to develop clinical histomoniasis remained close to the lag period density for the duration of the experiment. Plasma concentrations of glutamic oxalacetic transaminase (GOT) were found to be only weakly associated with the occurrence of clinical symptoms. Those results suggest that clinical symptoms are a cecal phenomenon, dependent on high population density of H. meleagridis. Evidence for elevation of both conjugated (direct) and protein-bound (indirect) bilirubin during histomoniasis suggests hemolytic jaundice, complicated with liver daage.

Hemolysis, Coagulation Defects, and Fulminant Hepatic Failure as a Presentation of Wilson's Disease

Fulminant hepatitis with acute hemolysis is a rare but well established presentation of Wilson's disease. 1.2 Several case reports 1.3 have suggested that coagulation disorders occur in conjunction with this clinical picture. We have recently cared for a patient with fulminant hepatic failure and acute hemolysis due to Wilson's disease in whom striking coagulation abnormalities were detected in the laboratory in the virtual absence of clinical hemorrhage. Report of a Case .—This 17-year-old girl was transferred to the Cleveland Clinic for evaluation of episodic hemolytic anemia. Five months before her admission, she had been evaluated elsewhere for hemolytic anemia and jaundice. Hemoglobin level during that admission was 5.1 g/dL; reticulocyte count, 33%; SGOT, 85 IU/L (normal, 8 to 33 IU/L); and lactic dehydrogenase, 490 units/dL (normal, 100 to 225 units/dL). This episode subsided spontaneously. She had no evidence of autoimmune hemolysis or systemic lupus erythematosus. For two weeks prior to

Acute renal failure following scorpion sting.

Fifteen cases of acute renal failure follwoing scorpion sting were studied. The onset of disease was characterized by the occurrence of hemoglobinuria within 24 h of the sting. Most of the patients developed oliguria, edema, hemolytic anemia, and hemolytic jaundice. Renal failure developed within a few days after the sting, and in five patients was severe enough to need dialysis. The onset of diuresis in oliguric patients occurred between 6 and 21 days following the sting. Renal biopsies were possible in four cases and showed mesangial proliferation, variable degrees of tubular changes, and mild interstitial infiltration. The pathogenesis of acute renal failure in these patients is discussed

The effects of intravenous injection of small amounts of copper sulphate in Nubian goats

Fifty mg. of copper sulphate was administered intravenously on each of three consecutive days to five Nubian goats. Three goats died within 3 to 4 days and the remaining two survived and were killed after 38 days. Anorexia, haemoglobinuria, jaundice, weakness and dyspnoea were the main clinical signs of acute copper toxicity. Haematological changes indicated the development of haemolytic jaundice and leukocytosis. An increase in the concentrations of bilirubin, ammonia, sodium and potassium and a decrease in total protein were detected in the serum. The outstanding pathological changes were necrosis and fatty change in the liver, dilatation and necrosis of the kidney tubules, petechial haemorrhages on the heart and splenic haemosiderosis.

A family with red cell pyrimidine 5'-nucleotidase deficiency

Congenital hemolytic anemia associated with pyrimidine 5′-nucleotidase deficiency is reported in two siblings. Both have had moderate chronic hemolytic anemia, splenomegaly, and jaundice since early infancy. The peripheral blood smear is characterized by striking red cell basophilic stippling. As this feature has been found in all previously reported cases, it should be the clue to the diagnosis.

A Family With Red Cell Pyrimidine 5'-Nucleotidase Deficiency

Congenital hemolytic anemia associated with pyrimidine 5'-nucleotidase deficiency is reported in two siblings. Both have had moderate chronic hemolytic anemia, splenomegaly, and jaundice since early infancy. The peripheral blood smear is characterized by striking red cell basophilic stippling. As this feature has been found in all previously reported cases, it should be the clue to the diagnosis.

Caloric Intake and Unconjugated Hyperbilirubinemia

Reduction in caloric intake was associated with a greater absolute rise in the serum bilirubin concentration in patients with Gilbert's syndrome and partial hepatic bilirubin uridine diphosphate glucuronyltransferase (UDPG-T) dysfunction compared to patients with hemolytic unconjugated hyperbilirubinemia and normal subjects. Two patients with overt hemolysis but an exaggerated response to caloric deprivation had reduced UDPG-T activities comparable to Gilbert's syndrome. The UDPG-T activities in the other patients with hemolytic jaundice were normal. The combination of fasting and novobiocin in 2 normal subjects produced a greater increase in bilirubin level than either fasting or novobiocin alone. These data suggest that theunderlying UDPG-T dysfunction, rather than the prefasting level of unconjugated hyperbilirubinemia, is responsible for the diet-induced hyperbilirubinemia in Gilbert's syndrome. The diet test appears to differentiate Gilbert's syndrome from hemolytic jaundice as well as from normal subjects, irrespective of the initial serum bilirubin concentration.

Hepatic neutral lipids, phospholipids and glycosphingolipids of a patient with histiocytosis X

Hepatic lipids of a patient with Histiocytosis X who had clinically hemolytic jaundice, intermittent fever and growth retardation and also showed histopathologically remarkable proliferation of enlarged reticular cells in liver, spleen, lymph node and bone marrow were isolated and analyzed. It was found that free fatty acids and lyso-glycerophospholipids markedly increased, whereas glycerophospholipids decreased. There was no increase in concentration of cholesterol and cholesterol esters. Relatively higher amounts of glucosyi ceramide, galactosyl ceramide, digalactosylglucosyl ceramide and hematoside seemed to be due to noticeable proliferation of enlarged reticular cells in liver. It was noted that neutral lipids and glycerophospholipids except free fatty acids gave lower percentages of unsaturated fatty acids. The fatty acid compositions of various sphingolipids contained more than 70% of longer chain fatty acids from C 20 to C 24.