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Results for “Hyperbilirubinemia”

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At least 19 recordsLinked to original sources

PubMed1977

Aminopyrine metabolism in the presence of hyperbilirubinemia due to cholestasis or hepatocellular disease. Combined use of laboratory tests to study disease-induced alterations in drug disposition.

Hepatocellular diseases, such as hepatitis, cirrhosis, or hepatic neoplasm, are associated with impaired metabolism of certain drugs, including aminopyrine, whereas cholestasis produced variable effects on aminopyrine metabolism. Reasons for the variable effects of cholestasis on hepatic aminopyrine metabolism were sought by performing in patients with hyperbilirubinemia the aminopyrine breath test (ABT), which consists of measurements of 14CO2 in breath 2 hr after oral administration of [14C]aminopyrine. Hyperbilirubinemia (total serum bilirubin less than 1.2 mg/100 ml) in these patients was due to hepatocellular disease or cholestasis. The ABT, defined as the percentage of the administered dose of 14C excreted in breath for 2 hr after [14C]aminopyrine administration, was 6.2 +/- 0.8% (mean +/- SD) in 107 control patients with normal total serum bilirubin. The ABT was severely abnormal (less than 3.1%) in 54 of 77 hyperbilirubinemic patients (70%) with hepatocellular disease and normal (greater than 4.5%) in only 5 of these patients (6%). In contrast, the ABT was severely abnormal in only 1 of 40 cases of cholestasis with hyperbilirubinemia and normal in 70% of these patients. Thus, aminopyrine metabolism is normal in most cases of hyperbilirubinemia due to cholestasis and is only rarely severely abnormal in these patients. On the other hand, severe abnormality in aminopyrine metabolism occurs in the majority of patients with hyperbilirubinemia due to hepatocellular disease. It therefore appears that the ABT may be useful in hyperbilirubinemia to distinguish patients with hyperbilirubinemia due to cholestasis form most patients with hyperbilirubinemia due to hepatocellular disease.

Aminopyrine↗

PubMed1977

Benign course of extreme hyperbilirubinemia in sickle cell anemia: analysis of six cases.

Since the approach to the management and outcome of extreme hyperbilirubinemia in patients with sickle cell anemia is not clearly defined, we reviewed our experience with marked hyperbilirubinemia in six children with sickle cell disease. Intrahepatic sickling (sickle hepatopathy) rather than hepatitis or biliary stones appeared primarily responsible for the extreme jaundice in at least four children and possibly in all six. Signs and symptoms were few, and laboratory abnormalities were not striking other than marked hyperbilirubinemia (total serum bilirubin concentrations ranging from 20.4 to 57.6 mg/dl with approximately one half conjugated). All of the children improved within days to weeks and currently are well, without recurrence of hyperbilirubinemia or evidence of chronic liver disease. The patients described here suggest that sickling within the liver, previously reported to be a serious and even fatal syndrome, usually is a benign and self-limited process.

Anemia, Sickle Cell↗

PubMed1978

Hyperbilirubinemia in inflammatory pancreatic disease: natural history and management.

Of 868 patients admitted with pancreatitis between 1971 and 1976, coexisting hyperbilirubinemia was noted in 125 (14%). The patient population was primarily composed of alcoholics (84%) with chronic pancreatic disease (75% Marsielles Class H or higher) which was of moderate severity (77% fewer than three prognostic signs). The hyperbilirubinemia in these 125 patients was due to extrahepatic obstruction in 22%, hepatocelluar disease in 31%, and was idiopathic in 47%. Transient hyperbilirubinemia (< 10 days duration) occurred most commonly in the idiopathic group. Transitory periductular pancreatic edema may account for the elevated bilirubin in some of these cases. Liver biopsy should be done whenever hyperbilirubinemia persists longer than ten days in patients with pancreatitis. If hepatocellular disease is not found, transhepatic or endoscopic retrograde cholangiography are indicated. If common bile duct obstruction is demonstrated, a brief trial of medical therapy is in order. Persistent conservative treatment, however, exposes the patient to the risk of cholangitis and biliary cirrhosis. In 13 of the 125 cases (10%), persistent extrahepatic obstruction proved to be due to compression of the common bile duct by inflammatory pancreatic tissue. In these circumstances, choledochoduodenostomy is recommended as the procedure of choice. In patients requiring biliary decompression, concommitant procedures upon the pancreas are occasionally indicated.

Acute Disease↗

PubMedDate not supplied

Cochlear and brain stem responses in hearing loss following neonatal hyperbilirubinemia.

The site of lesion in hearing loss following neonatal hyperbilirubinemia is unclear. Histopathological studies have implicated the brain stem auditory nuclei while other investigations have hinted at a lesion in the cochlea. In order to clarify this issue, attempts were made to record responses from the auditory pathway in 13 patients with hearing loss following neonatal hyperbilirubinemia. The neural response from the auditory nerve was absent in 11 of the 13 patients and present only in response to high intensity stimuli in 2 patients. However, the response of the cochlear hair cells (cochlear microphonic potential) was present in 9 of the 13 patients. In most other cases of sensorineural hearing loss, with no history of hyperbilirubinemia, the hair cell response was absent. This is functional evidence for auditory nerve damage in cases of hearing loss following neonatal hyperbilirubinemia while the hair cells are spared.

Adult↗

PubMedDate not supplied

Extreme hyperbilirubinemia in a patient with hereditary spherocytosis, Gilbert's syndrome, and obstructive jaundice.

Hyperbilirubinemia may be of several etiologies in the individual patient. An 18-year-old man presented with extreme hyperbilirubinemia (direct bilirubin 23.0 mg/dl, total bilirubin 60.0 mg/dl), hepatosplenomegaly, and anemia. Hematologic studies prelaparotomy documented the presence of hereditary spherocytosis. Intraoperative liver biopsy revealed moderately reduced levels of glucuronyl transferase activity (Gilbert's syndrome). Common bile duct obstruction secondary to choledocholithiasis was found, and a cholecystectomy and splenectomy were performed. This case stresses the potential confusion among several diseases which may present with hyperbilirubinemia.

Adolescent↗

PubMed1975

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.

Anemia, Hemolytic↗

PubMed1975

Cyclic premenstrual unconjugated hyperbilirubinemia. Report of two cases.

Two young women developed unconjugated hyperbilirubinemia that fluctuated with the menstrual cycle. They were otherwise healthy, and there was no evidence of hematologic or chronic liver disease. Serum unconjugated bilirubin rose concomitant with the rise of basal temperature during the premenstrual period, and it declined immediately after the end of menses. Administration of progesterone during the postmenstrual period elevated unconjugated bilirubin. A close relation of hyperbilirubinemia to female hormones was suggested. The condition may be called "constitutional unconjugated hyperbilirubinemia with a menstrual cycle.

Adult↗

PubMed1979

Chronic persistent hepatitis and unconjugated hyperbilirubinemia.

The authors studied 12 patients with chronic persistent hepatitis and persistent or intermittent mild unconjugated hyperbilirubinemia. Maximum serum total bilirubin concentration ranged from 2.1 to 3.6 mg/dl. Hemolysis was not evident. Hepatic bilirubin UDP-glucuronyltransferase activity assayed in each patient ranged from 0.16 to 0.39 U (mean +/- SEM = 0.27 +/- 0.02) compared to 0.68-1.99 (1.35 +/- 0.08) in 23 normals, 0.78-2.28 (1.41 +/- 0.05) in 53 patients with acute hepatitis, 0.34-1.74 (0.81 +/- 0.09) in 16 patients with anicteric chronic persistent hepatitis, and 0-0.62 (0.24 +/- 0.03) in 33 patients with Gilbert's syndrome. The mean UDP-glucuronyltransferase activity was significantly lower in anicteric chronic persistent hepatitis compared to normals, but higher than in Gilbert's syndrome. The incidence of unconjugated hyperbilirubinemia among first degree relatives was 0:32 in icteric chronic persistent hepatitis compared to 24:85 (28%) in Gilbert's syndrome. These results show that the likely cause for the unconjugated hyperbilirubinemia associated with chronic persistent hepatitis is an acquired depression of hepatic bilirubin UDP-glucuronyltransferase activity. The data suggest that the enzyme defect is related to chronic persistent hepatitis.

Adult↗

PubMed1975

Comparison of the effectiveness of phototherapy and exchange transfusion in the management of nonhemolytic neonatal hyperbilirubinemia.

The relative effectiveness of phototherapy and exchange transfusion for nonhemolytic neonatal hyperbilirubinemia was compared in two closely matched groups of infants. Although the exchange transfusion achieved an immediate reduction of bilirubin level, the "rebound" was rapid and tended to offset this reduction. The more gradual and steady effect of phototherapy resulted in a significantly lower serum bilirubin level at 1, 2, and 3 days after commencement of therapy; the rebound after phototherapy was small. Phototherapy was demonstrated to be more effective than exchange transfusion in achieving prolonged reduction of bilirubin levels for nonhemolytic hyperbilirubinemia. With more efficient lamps delivering more energy in the desired spectrum, it would seem feasible to treat hyperbilirubinemia of whatever etiology with this safer and more convenient form of therapy, though sometimes only as in adjunct therapy.

Bilirubin↗

PubMed1977

Unconjugated hyperbilirubinemia in very low birth weight infants.

In very low birth weight infants, the occurrence of bilirubin-related brain damage has been repeatedly observed at low serum bilirubin concentrations in close association with altered pathophysiologic status (hypoxia, acidosis, hypothermia, and so on). This increased susceptibility is accompanied by increased severity and duration of unconjugated hyperbilirubinemia as compared with more mature infants. Clinical manifestations of kernicterus in very low birth weight infants are almost always nonspecific. No single biochemical or physiologic measurement is sufficient to predict the risk for development of the bilirubin-related brain damage in this group. Prevention of bilirubin-related brain damage in very low birth weight infants requires not only the maintenance of physiologic and biochemical milieu within normal limits, but also specific therapy to alleviate unconjugated hyperbilirubinemia. Although exchange transfusion has been the mainstay of therapy for unconjugated hyperbilirubinemia, the increased morbidity and mortality associated with exchange transfusion in these immature infants and the need to maintain very low serum bilirubin concentrations suggest that prophylactic phototherapy may be more beneficial for this group.

Bilirubin↗

PubMed1979

Conjugated hyperbilirubinemia in infants with erythroblastosis fetalis.

In an attempt to identify the incidence of conjugated hyperbilirubinemia in infants with erythroblastosis fetalis the records of 67 infants were reviewed. Twenty-two infants were found to have direct bilirubin concentrations greater than 1.0 mg./dl. Among 11 infants who underwent intrauterine exchange transfusion, nine (82%) had conjugated hyperbilirubinemia. Of the remaining 56 infants without the procedure, only 13 (23%) showed evidence of this complication. To predict infants at risk of developing conjugated hyperbilirubinemia, the ratio of hematocrit and total bilirubin concentration in cord blood (H/B ratio) was examined.

Bilirubin↗

PubMed1977

Phenobarbital prophylaxis of neonatal hyperbilirubinemia. Evaluation of a nightly dose.

In an attempt to improve the success of a previous study of the prevention of neonatal hyperbilirubinemia employing phenobarbital in a single 30-mg nightly dose from 32 weeks' gestation until delivery, 246 patients were tested against 270 control subjects. A 39% reduction in significant hyperbilirubinemia was realized but this fell far short of previous findings with a larger dose. Premature infants received no apparent protection against jaundice. Mature infants had no severe jaundice (more than 16 mg/100 ml) and the resolution of hyperbilirubinemia was more rapid. No apparent difference in drowsiness or daytime sedation from previous experience was found. Female newborns responded much better (3:1) to prophylaxis. These disappointing results favor a return to the 1-g daily divided dose.

Birth Weight↗

PubMed1976

ABO hemolytic disease of the newborn, without hyperbilirubinemia.

ABO hemolytic disease of the newborn without hyperbilirubinemia is described in 17 full-term infants. The erythrocyte characteristics, such as reticulocytosis, microspherocytosis, and positive indirect antiglobulin (Coomb's) test, resembled those in ABO disease with hyperbilirubinemia. Erythrocyte acetylcholinesterase activity was reduced in this group to the same degree as in the more severely affected infants. Negro infants predominated over white, in a 2.5:1 ratio in this mild ABO group.

ABO Blood-Group System↗

PubMed1978

Hyperbilirubinemia and cholestasis.

Although the morphologist continues to describe cholestasis on the basis of precipitated bile seen on light microscopic sections of the liver or dilated canaliculi with loss of microvilli seen by electron microscopy, the physiologist can distinguish clearly between hyperbilirubinemia and cholestasis. Both bilirubin and bile acids are specifically removed from sinusoidal plasma by the normal hepatocyte and appear in bile in high concentration. Bilirubin conjugation and excretion appear to be governed by hepatocellular mechanisms that are, for the most part, separate from the conjugation and excretion of bile acids. Disturbances in bilirubin transport are recognized by hyperbilirubinemia which represents a number of clinical syndromes that can be classified by the nature of the block in the transport system. Serum bile acids appear to remain normal in hyperbilirubinemic syndromes. By contrast, cholestatic syndromes are characterized by marked bile acidemia with normal to slightly elevated bilirubin levels. Severe cholestasis, because of the marked reduction in bile flow, can however, engender jaundice. Further exploration of these excretory pathways will provide interesting new insights on the numerous cholestatic and hyperbilirubinemic syndromes that occur in nature.

Animals↗

PubMed1975

Effect of hyperbilirubinemia on the inner ear in Gunn rats.

The effect of hyperbilirubinemia on the inner ear was studied in fifteen jaundiced homozygous Gunn rats. Seven non-jaundiced heterozygous littermates served as controls. Histopathological examination of the temporal bone in these animals showed normal sensory and neuronal structures of the inner ear. This cannot be taken as conclusive evidence that a central, rather than a peripheral, lesion is the cause of hearing loss in hyperbilirubinemia.

Animals↗

PubMed1978

False-positive rubella hemagglutination-inhibition (HAI) titers in neonates and children with conjugated hyperbilirubinemia.

Two neonates, one with extrahepatic biliary atresia and one with cystic fibrosis, and a 9-year-old child with atresia of the common bile duct had conjugated hyperbilirubinemia and elevated rubella HAI titers when kaolin pretreatment of serum was used. A beta-lipoprotein fraction of the serum that is frequently found in association with biliary obstruction was shown to be the probable source of the rubella HAI inhibitor. This beta-lipoprotein was not removed by standard kaolin treatment of serum, but was removed almost completely by dextran sulfate--calcium chloride treatment. In the presence of conjugated hyperbilirubinemia, routine kaolin pretreatment of serum is an inadequate measure for the removal of interfering substances, as false-positive rubella HAI results are obtained consistently.

Antibodies, Viral↗

PubMed1978

Familial lecithin: cholesterol acyltransferase deficiency complicated with unconjugated hyperbilirubinemia and peripheral neuropathy. The first reported cases in the Far East.

Three Japanese patients with lecithin: cholesterol acyltransferase (LCAT) deficiency, the offspring of a consanguineous marriage, are described. In addition to the characteristic clinical and laboratory findings of the disease, our patients had hitherto unreported manifestations, namely unconjugated hyperbilirubinemia, peripheral neuropathy and marked hypocholesterolemia. Although the mechanism of the unconjugated hyperbilirubinemia is not clear, the role of impaired hepatic bilirubin uridine-diphosphate-glucuronyl transferase activity combined with another unknown factor(s) was postulated. Non-random assortment was observed between LCAT deficiency and haptoglobin types, as previously reported. The discovery of Japanese patients with LCAT deficiency indicates that the distribution of this hereditary metabolic disorder is not confined to the Western hemisphere.

Adult↗

PubMed1975

Chronic hyperbilirubinemia associated with hepatic uptake and storage impairment. A new syndrome resembling that of the mutant Southdown sheep.

A 19-year-old female complained of moderate jaundice since early childhood, without any other clinical abnormality. Apart from the hyperbilirubinemia (which comprised around 50% of conjugated bilirubin), usual hepatic function tests were normal. Oral cholecystogram showed a delayed opacification of a normal gallbladder. Liver histology and hepatic bilirubin UDP-glucuronyltransferase activity were normal. The plasma disappearance rates of bromosulfophthalein, dibromosulfopthalein, rose bengal, and indocyanine green were dramatically reduced. There was a marked impairment of the relative storage capacities of bromosulfophthalein and dibromosulfophthalein; this reduction was associated with, and thought to be the cause of, some decrease of the biliary transport maximum. This new form of chronic benign hyperbilirubinemia seems therefore primarily due to a defect of hepatic uptake and storage, and closely resembles the disease observed in the mutant Southdown sheep. Since it is not yet possible to quantify the Y- and Z-proteins from a needle biopsy of human liver, their role in the pathogenesis of the present disease remains speculative.

Adult↗