scholarly journals Hematin administration to an adult with lead intoxication

Blood ◽  
1979 ◽  
Vol 53 (5) ◽  
pp. 1007-1011
Author(s):  
JM Lamon ◽  
BC Frykholm ◽  
DP Tschudy
Keyword(s):  
Lead Poisoning ◽  
Aminolevulinic Acid ◽  
Acute Intermittent Porphyria ◽  
Lead Intoxication ◽  
Neurologic Manifestations ◽  
Heme Metabolism ◽  
Urinary Ala

Lead poisoning and acute intermittent porphyria (AIP) may exhibit similar neurologic manifestations, and they have in common elevated excretion of urinary aminolevulinic acid (ALA). Despite their similarities, the possible pathophysiologic connection between AIP and lead poisoning in not known. Because intravenous hematin administration has produced biochemical improvement in AIP, a hematin trial in lead intoxication was of interest with respect to some of the heme metabolism abnormalities observed in the condition. Significant diminution of urinary ALA and coproporphyrin excretion occurred in association with intravenous hematin administration.

scholarly journals Hematin administration to an adult with lead intoxication

Blood ◽  
1979 ◽  
Vol 53 (5) ◽  
pp. 1007-1011 ◽  
Author(s):  
JM Lamon ◽  
BC Frykholm ◽  
DP Tschudy
Keyword(s):  
Lead Poisoning ◽  
Aminolevulinic Acid ◽  
Acute Intermittent Porphyria ◽  
Lead Intoxication ◽  
Neurologic Manifestations ◽  
Heme Metabolism ◽  
Urinary Ala

Abstract Lead poisoning and acute intermittent porphyria (AIP) may exhibit similar neurologic manifestations, and they have in common elevated excretion of urinary aminolevulinic acid (ALA). Despite their similarities, the possible pathophysiologic connection between AIP and lead poisoning in not known. Because intravenous hematin administration has produced biochemical improvement in AIP, a hematin trial in lead intoxication was of interest with respect to some of the heme metabolism abnormalities observed in the condition. Significant diminution of urinary ALA and coproporphyrin excretion occurred in association with intravenous hematin administration.

scholarly journals Correlation between plasma 5-aminolevulinic acid concentrations and indicators of oxidative stress in lead-exposed workers

1997 ◽  
Vol 43 (7) ◽  
pp. 1196-1202 ◽  
Author(s):  
Cristine A Costa ◽  
Gilmar C Trivelato ◽  
Adriana M P Pinto ◽  
Etelvino J H Bechara
Keyword(s):  
Oxidative Stress ◽  
Free Radical ◽  
Lead Poisoning ◽  
Stress Responses ◽  
Aminolevulinic Acid ◽  
Aerobic Oxidation ◽  
Protoporphyrin Ix ◽  
Acute Intermittent Porphyria ◽  
Blood Concentrations ◽  
Exposed Workers

Abstract 5-Aminolevulinic acid (ALA), a heme precursor accumulated in acute intermittent porphyria and lead poisoning, undergoes metal-catalyzed aerobic oxidation at physiological pH to yield reactive free radical species (O2−·>, HO·, and ALA·). We analyzed the relationships between plasma ALA concentrations, blood concentrations of lead, protoporphyrin IX (PP-IX), superoxide dismutase (SOD), and methemoglobin (metHb), and urine chemiluminescence (CL) in samples collected from lead-exposed workers. All variables measured were substantially (P <0.01) higher (2–8-fold) in the lead-exposed workers (n = 60). Plasma ALA concentrations were, on average, 6-fold higher in lead-exposed workers. We observed positive linear relationships between ALA and lead (r = 0.992), ALA and PP-IX (r = 0.891), ALA and metHb (r = 0.984), lead and SOD (r = 0.948), ALA and urine CL (r = 0.987), and lead and PP-IX (r = 0.993). These data are consistent with our free radical hypothesis for lead poisoning, where ALA distribution to and accumulation in several organs may trigger oxidative stress responses.

scholarly journals Renal Mechanism for Excretion of Porphyrin Precursors in Patients with Acute Intermittent Porphyria and Chronic Lead Poisoning

Blood ◽  
1965 ◽  
Vol 26 (2) ◽  
pp. 181-189 ◽  
Author(s):  
ROBERT DRUYAN ◽  
BIRGITTA HAEGER-ARONSEN ◽  
WILFRIED VON STUDNITZ ◽  
JAN WALDENSTRÖM
Keyword(s):  
Lead Poisoning ◽  
Acute Intermittent Porphyria ◽  
Normal Subjects ◽  
Tubular Reabsorption ◽  
The Other ◽  
Lead Intoxication ◽  
Serum Concentrations ◽  
Lead Workers ◽  
Chronic Lead Intoxication

Abstract Serum concentrations of ALA and PBG have been measured in normal subjects, patients with AIP, and lead workers. Both porphyrin precursors are significantly increased in serum from porphyric patients, and serum ALA is elevated in lead workers. Endogenous clearance measurements, when compared with creatinine clearances, are consistent with significant tubular reabsorption of ALA and PBG under physiologic circumstances, and with an "overflow" mechanism for increased excretion in AIP or lead intoxication. Three of 8 porphyric patients showed an aminoaciduria; tyrosine was involved in 2, tryptophan in the other 3. Evidence to suggest a specific renal defect contributing to increased ALA excretion in chronic lead intoxication was not obtained.

Estimation of urinary delta aminolevulinic acid levels in gasoline and petrol pump workers as an index of lead exposure

2022 ◽  
Vol 8 (4) ◽  
pp. 274-277
Author(s):  
Sachin Patharkar ◽  
Neelam Patil ◽  
Siddhesh Thorat ◽  
Alka Nerurkar ◽  
Umesh Shinde ◽  
...  
Keyword(s):  
Lead Poisoning ◽  
Lead Exposure ◽  
Levulinic Acid ◽  
Aminolevulinic Acid ◽  
Petroleum Products ◽  
Safety Measures ◽  
Amino Levulinic Acid ◽  
Main Components ◽  
Urinary Ala ◽  
Petrol Pump

Lead poisoning is a phenomenon which with growing globalization is being a subject of worry.ALA i.e Amino levulinic acid is a precursor of hemoglobin, which is synthesized in mitochondria by two main components succinyl Co-A and glycine in presence of ALA-S i.e. amino levulinic acid-synthase. Urinary ALA (ALA-U) has been a recommended biomarker for lead exposure. Inhibition of Amino levulinic acid-dehydratase (ALA-D) results into activation of ALA-S which further synthesizes ALA, excess of ALA is accumulated in the blood, plasma, urine. Present manuscript is focused on the estimation of levels of ALA in the urine of gasoline and pertol pump workers, by acidifying the urine to extract out ALA and reading it colorimetrically as they are exposed to fumes released by gasoline, petrol, and petroleum products which contains lead. Awareness and safety measures such as protective masks and gears should be provided by the respective organisations to the workers.

scholarly journals Biochemical and hematological analysis in acute intermittent porphyria (AIP): a case report

2013 ◽  
Vol 85 (3) ◽  
pp. 1207-1214 ◽  
Author(s):  
ANNA R.R. DOS SANTOS ◽  
RAFAELA R. DE ALBUQUERQUE ◽  
MARIA J.R. DORIQUI ◽  
GRACIOMAR C. COSTA ◽  
ANA PAULA S.A. DOS SANTOS
Keyword(s):  
Biochemical Parameters ◽  
Contraceptive Use ◽  
Aminolevulinic Acid ◽  
Neuropsychiatric Symptoms ◽  
Acute Intermittent Porphyria ◽  
Signs And Symptoms ◽  
Acute Porphyria ◽  
Reactive Protein ◽  
Hematological Analysis ◽  
Hematological And Biochemical Parameters

Acute intermittent porphyria is the most common acute porphyria caused by a decrease in hepatic porphobilinogen deaminase activity, resulting in an accumulation of delta-aminolevulinic acid and porphobilinogen. This disease shows nonspecific signs and symptoms that can be confused with other diseases, thereby making the diagnosis difficult. We report a case of acute intermittent porphyria, reviewing clinical and laboratory aspects, highlighting the hematological and biochemical parameters during and after the crisis. A female patient, aged 28 years, suffered two crises, both presenting gastrointestinal disorders. The second presented neuropsychiatric symptoms. The analysis of hematological and biochemical parameters during the second crisis showed anemia, leukocytosis, hyponatremia, mild hypokalemia, uremia and elevated C-reactive protein. The initial treatment included glucose infusion, a diet rich in carbohydrates and interruption of porphyrinogenic drugs. Subsequently, treatment was maintained with oral contraceptive use. According to the observed data, signs and symptoms of gastrointestinal, neurological and psychiatric disorders, associated with laboratory results presented in this paper can be applied to screen acute porphyria, contributing to early diagnosis.

Deleading Dilemma: Pitfall in the Management of Childhood Lead Poisoning

PEDIATRICS ◽  
1987 ◽  
Vol 79 (2) ◽  
pp. 214-217
Author(s):  
Susana Rey-Alvarez ◽  
Theresa Menke-Hargrave
Keyword(s):  
Case Report ◽  
Lead Poisoning ◽  
Lead Level ◽  
Prevention Programs ◽  
Lead Intoxication ◽  
Supportive Evidence ◽  
Pediatric Practice ◽  
Childhood Lead Poisoning ◽  
Lead Poisoning Prevention ◽  
Poisoning Prevention

Deleading is a dangerous process which, if improperly done, can result in acute lead intoxication. The following case report illustrates what happened to an already-lead-poisoned child's lead level when he was not excluded from his apartment during deleading. Supportive evidence is provided from 12 additional cases recently seen by the same pediatric practice. The issues behind the failure of these families to vacate their apartments during deleading are discussed, and the need for lead-poisoning prevention programs to address these issues, particularly that of providing alternative shelter during deleading, is stressed. Society should adequately fund such programs so that they become a reality.

Urinary Delta-Aminolevulinic Acid (ALA) Levels in Lead Poisoning

1968 ◽  
Vol 17 (2) ◽  
pp. 164-171 ◽  
Author(s):  
Joseph R. Davis ◽  
Ronald H. Abrahams ◽  
William I. Fishbein ◽  
Enrique A. Fabrega
Keyword(s):  
Lead Poisoning ◽  
Aminolevulinic Acid

scholarly journals Molecular and Biochemical Studies of Acute Intermittent Porphyria in 196 Patients and Their Families

2002 ◽  
Vol 48 (11) ◽  
pp. 1891-1900 ◽  
Author(s):  
Raili Kauppinen ◽  
Mikael von und zu Fraunberg
Keyword(s):  
Diagnostic Accuracy ◽  
Aminolevulinic Acid ◽  
Clinical Manifestations ◽  
Acute Intermittent Porphyria ◽  
Reference Interval ◽  
Acute Attack ◽  
Fecal Excretion ◽  
Variant Form ◽  
Roc Curve Analysis ◽  
Biochemical Tests

Abstract Background: Acute intermittent porphyria (AIP) is a metabolic disease with clinical manifestations that mimic other abdominal, neurologic, or mental crises. We studied the diagnostic accuracy of current laboratory tests during an acute attack and in remission. Methods: Since 1966, we have studied all known Finnish AIP patients (n = 196) and their families (n = 45) and identified the porphobilinogen deaminase (PBGD) mutation in each family. Diagnoses or exclusions of AIP were based on clinical data (including family history), biochemical tests, and in 239 cases, mutation testing. We retrospectively evaluated the diagnostic accuracy of erythrocyte PBGD activity, urinary excretion of porphobilinogen (PBG) and δ-aminolevulinic acid, and urinary and fecal excretion of porphyrins in these patients. Results: Measurement of urinary PBG identified all 35 AIP patients studied during an acute attack. The mean excretion of PBG was 50-fold above the reference interval, although the intraindividual increases were modest (1.6- to 4.0-fold). In the mutation-screened population, urinary PBG analysis identified only 85% of 81 AIP patients studied during remission, but by ROC curve analysis it was nonetheless the best of the biochemical tests. It was increased ≤2-fold in 29% of healthy relatives. Erythrocyte PBGD activity was decreased in only 84% of AIP patients, with results within the reference interval mainly in the variant form of AIP; it was decreased in 23% of healthy relatives. Conclusions: Measurement of urinary PBG is the best biochemical test for AIP, although it is unspecific and does not distinguish AIP from other acute porphyrias. Because the acute increase in PBG is often modest, the medical history, signs, and symptoms must be evaluated carefully during an acute attack. In addition, because biochemical analyses often remain indeterminate in remission, mutation analysis is needed to exclude or confirm the diagnosis of AIP.

scholarly journals A Red Cell Enzyme Method for the Diagnosis of Acute Intermittent Porphyria

Blood ◽  
1974 ◽  
Vol 44 (6) ◽  
pp. 857-868 ◽  
Author(s):  
C. Richard Magnussen ◽  
Joel B. Levine ◽  
Joyce M. Doherty ◽  
Judy O. Cheesman ◽  
Donald P. Tschudy
Keyword(s):  
Aminolevulinic Acid ◽  
Acute Intermittent Porphyria ◽  
Mean Value ◽  
Red Cells ◽  
Red Cell ◽  
Enzyme Preparations ◽  
Cell Enzyme ◽  
Enzyme Method ◽  
Latent Disease ◽  
The Mean

Abstract A method has been devised for the measurement of uroporphyrinogen I synthetase ih red cells. By using trichloroacetic acid as a protein precipitant, heme is removed from the final solution, allowing accurate measurement of porphyrins. The method is highly reproducible and adaptable to varying incubation volumes and enzyme preparations. It is of great value as an enzyme diagnostic method for acute intermittent porphyria and appears capable of detecting patients with the latent disease who have normal urinary δ-aminolevulinic acid and porphobilinogen excretion. It also appears to distinguish other types of porphyria from acute intermittent porphyria. The mean value of the enzyme in red cells of patients with acute intermittent porphyria was approximately 50% that of normals, indicating that the mutation causes complete lack of catalytic activity in the mutant enzyme.

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