scholarly journals Hemolytic Crisis following Naphthalene Mothball Ingestion in a 21-Month-Old Patient with Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency

2019 ◽  
Vol 2019 ◽  
pp. 1-3
Author(s):  
Maricel Dela Cruz ◽  
Muhammad Masood Khalid ◽  
Ahmed Mostafa ◽  
Muhammed Ershad ◽  
David Vearrier ◽  
...  
Keyword(s):  
Blood Transfusion ◽  
G6pd Deficiency ◽  
Pediatric Intensive Care ◽  
Dermal Absorption ◽  
Vulnerable Children ◽  
Household Item ◽  
Hemolytic Crisis ◽  
History Of ◽  
Haptoglobin Level ◽  
Glucose 6 Phosphate Dehydrogenase

Introduction. Naphthalene is an aromatic hydrocarbon that may be found in mothballs and deodorizers. Exposure can occur by ingestion or dermal absorption. We present a case of acute hemolysis requiring blood transfusion in a 21-month-old male with a history of glucose-6-phosphate dehydrogenase (G6PD) deficiency after ingestion of a naphthalene-containing mothball. Case Presentation. A 21-month-old male with G6PD deficiency presented to the emergency department three hours following an exploratory ingestion of a naphthalene-containing mothball. On arrival, the patient was tachycardic with normal blood pressure, temperature, respiratory rate, and oxygen saturation. Initial laboratory studies showed significant anemia with elevated reticulocyte count, blood urea nitrogen, total bilirubin, and lactate dehydrogenase. Haptoglobin level was low, and the methemoglobin level was unremarkable. The patient was admitted to the pediatric intensive care unit and underwent blood transfusion. Discussion. This case serves as a reminder that mothballs, a ubiquitous household item, can be hazardous when accessible to vulnerable children. Care should be taken to secure these products and prevent ingestion.

scholarly journals Late-Life Presentation of Unsuspected G6PD Deficiency

2018 ◽  
Vol 2018 ◽  
pp. 1-3
Author(s):  
Marcos Benchimol ◽  
Laura Bernardo Madeira ◽  
Ricardo de Oliveira-Souza
Keyword(s):  
G6pd Deficiency ◽  
Clinical Manifestations ◽  
Diagnostic Challenge ◽  
Packed Red Blood Cells ◽  
Wide Range ◽  
Hemolytic Crisis ◽  
History Of ◽  
Triggering Conditions ◽  
Red Blood Cell Count ◽  
Glucose 6 Phosphate Dehydrogenase

Deficiency of glucose-6-phosphate dehydrogenase (G6PD) is the commonest enzyme deficiency in humans with a wide range of possible clinical manifestations depending on the specific genetic variant in each case. Here we present the case of an 86-year-old male of African descent who acutely developed symptoms of G6PD deficiency immediately after he received methylene blue for treating methemoglobinemia. The contrast between a low SO2 on pulse oximetry and a normal arterial gas sampling raised the possibility of methemoglobinemia. The patient was treated with packed red blood cells and folic acid, and a rapid clinical improvement followed by normalization of the red blood cell count ensued. In view of the patient’s advanced age, the lack of a history of similar episodes in the past, and the normal laboratory results during the hemolytic crisis, this case remained a diagnostic challenge for over three months, when a follow-up measure of G6DP activity eventually confirmed the diagnosis. A latent deficiency of G6PD may become clinically manifest under the appropriate triggering conditions even in elderly patients and in the absence of past or current clinical and laboratory evidence of G6PD deficiency.

scholarly journals From Prejudice to Evidence: The Case of Rhizoma Coptidis in Singapore

2014 ◽  
Vol 2014 ◽  
pp. 1-7 ◽  
Author(s):  
Chin Ee Ho ◽  
You Li Goh ◽  
Chang Zhang
Keyword(s):  
Chinese Medicine ◽  
Traditional Chinese Medicine ◽  
G6pd Deficiency ◽  
Neonatal Jaundice ◽  
Therapeutic Effects ◽  
Evidence Based ◽  
Rhizoma Coptidis ◽  
History Of ◽  
Hepatoprotective Effects ◽  
Glucose 6 Phosphate Dehydrogenase

Rhizoma Coptidis (RC), commonly known ashuanglian, is a herb frequently used in Traditional Chinese Medicine (TCM) prescriptions. Known to have “clearing damp-heat, quenching fire and counteracting poison” properties, it was widely used in the Chinese community in Singapore. Berberine, an alkaloid isolated from RC, is known to have a wide array of therapeutic effects including antimicrobial, antineoplastic, and hepatoprotective effects. In 1978, RC was implicated in causing neonatal jaundice (NNJ) and kernicterus in neonates suffering from glucose-6-phosphate dehydrogenase (G6PD) deficiency, leading to the banning of RC and berberine in Singapore. More than three decades later, accumulating evidence-based studies pointing to the safety of RC for general public and better understanding of G6PD deficiency, the Health Sciences Authority (HSA) in Singapore reviewed and lifted the prohibition on RC and berberine, turning a brand new chapter in the history of TCM in Singapore. This paper aims to review the safety of RC and berberine, using the prohibition of use and subsequent lifting of ban on RC and berberine in Singapore as an illustration to highlight the importance of evidence-based studies in Traditional Chinese Medicine (TCM).

scholarly journals Clinical spectrum and severity of hemolytic anemia in glucose 6-phosphate dehydrogenase–deficient children receiving dapsone

Blood ◽  
2012 ◽  
Vol 120 (20) ◽  
pp. 4123-4133 ◽  
Author(s):  
Allan Pamba ◽  
Naomi D. Richardson ◽  
Nick Carter ◽  
Stephan Duparc ◽  
Zul Premji ◽  
...  
Keyword(s):  
Blood Transfusion ◽  
Hemolytic Anemia ◽  
G6pd Deficiency ◽  
Case Reports ◽  
Drug Induced ◽  
Once Daily ◽  
Maximum Decrease ◽  
Life Threatening ◽  
The Mean ◽  
Glucose 6 Phosphate Dehydrogenase

AbstractDrug-induced acute hemolytic anemia led to the discovery of G6PD deficiency. However, most clinical data are from isolated case reports. In 2 clinical trials of antimalarial preparations containing dapsone (4,4′-diaminodiphenylsulfone; 2.5 mg/kg once daily for 3 days), 95 G6PD-deficient hemizygous boys, 24 G6PD-deficient homozygous girls, and 200 girls heterozygous for G6PD deficiency received this agent. In the first 2 groups, there was a maximum decrease in hemoglobin averaging −2.64 g/dL (range −6.70 to +0.30 g/dL), which was significantly greater than for the comparator group receiving artemether-lumefantrine (adjusted difference −1.46 g/dL; 95% confidence interval −1.76, −1.15). Hemoglobin concentrations were decreased by ≥ 40% versus pretreatment in 24/119 (20.2%) of the G6PD-deficient children; 13/119 (10.9%) required blood transfusion. In the heterozygous girls, the mean maximum decrease in hemoglobin was −1.83 g/dL (range +0.90 to −5.20 g/dL); 1 in 200 (0.5%) required blood transfusion. All children eventually recovered. All the G6PD-deficient children had the G6PD A− variant, ie, mutations V68M and N126D. Drug-induced acute hemolytic anemia in G6PD A− subjects can be life-threatening, depending on the nature and dosage of the drug trigger. Therefore, contrary to current perception, in clinical terms the A− type of G6PD deficiency cannot be regarded as mild. This study is registered at http://www.clinicaltrials.gov as NCT00344006 and NCT00371735.

scholarly journals Glucose-6-phosphate Dehydrogenase Deficiency Induced Haemolysis in Male With Newly Diagnosed Diabetes During Diabetic Ketoacidosis Treatment: A Case Report

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A383-A383
Author(s):  
Eman Ebrahim Albasri
Keyword(s):  
Glucose Level ◽  
Plasma Glucose ◽  
G6pd Deficiency ◽  
Bacterial Infections ◽  
Epigastric Pain ◽  
Vital Signs ◽  
Epidemiological Data ◽  
Insulin Injection ◽  
History Of ◽  
Glucose 6 Phosphate Dehydrogenase

Abstract Background: Glucose-6-phosphate dehydrogenase (G6PD) deficiency could be a risk factor for diabetes, as suggested by various epidemiological data, but still a matter of debate. The occurrence of haemolysis during diabetes crises was reported in patients with G6PD deficiency, furthermore the fall in glucose level during the treatment of hyperglycemia is suggested as a possible cause for hemolysis in G6PD deficiency. We reviewed the mechanisms that may link the two diseases. Clinical Case: A 19-year-old male, with G6PD deficiency, presented to the emergency department in our institution with history of generalised weakness, epigastric pain, nausea, and vomiting. He gave history of polyuria, polydepsia and weight loss over a period of two weeks. Diagnosis of DM was confirmed by laboratory tests that showed a mild DKA: arterial pH 7.28, HCO3 18 mmol/l, plasma glucose 21 mmol/l, urinary ketones ++, and hemoglobin 16.1 g/dl (12.0–15.0 g/dl). DKA was treated according to DKA guidelines. Ketoacidosis remission was achieved two days after rehydration and treatment with continuous intravenous insulin infusion (0.05–0.1 IU/kg). The patient then started on subcutaneous intensive insulin injection therapy, three times daily before meals and once before bedtime. Fasting and post-prandial blood glucose levels decreased to 7-8mmol/L, and 10 –13 mmol/l respectively. On day 4, the patient developed dizziness while he was taking a bath, On examination he was pale and had icterus, however, the vital signs and systemic examination were normal, blood tests revealed a hemolytic anemia as follows: (normal values): Hemoglobin 9 g/dl (12.0–15.0 g/dl), reticulocytes 8.5% (0.5–1.5%), total bilirubin 82 umol/l (0–20 umol/l), unconjugated bilirubin 58 umol/l (3–15 umol/l), By the 7th day, hemoglobin levels had fallen to the lowest value of 8.3, then gradually raised to 10.2 g/dl, 2 weeks later, hemoglobin electrophoresis was normal, Coombs test was negative. G6PD activity was below 300 U/L (reduced activit<600) with two separate measurements. The patient had no bacterial infections and had not ingested haemolytic drugs. Hemolysis ceased spontaneously and hemoglobin increased gradually. Conclusion: G6PD deficiency and diabetes mellitus could aggravate each other, and In patients at risk of G6PD deficiency screening of the enzyme activity should be considered following diabetes decompensation, and in case of G6PD deficiency, it is advisable to correct plasma glucose level gradually to avoid the rapid drop in glucose availability, which may cause hemolysis in these patients.

scholarly journals Fine mapping of Glucose 6 Phosphate Dehydrogenase (G6PD) deficiency in rural area of South West Odisha using the clinical, hematological and molecular approach

2020 ◽  
Vol 12 (1) ◽  
pp. e2020015
Author(s):  
Ravindra Kumar ◽  
MPSS Singh ◽  
Soumendu Mahapatra ◽  
Sonam Chourasia ◽  
Malay Kumar Tripathi ◽  
...  
Keyword(s):  
G6pd Deficiency ◽  
Clinical History ◽  
South West ◽  
Normal Individuals ◽  
Pcr Rflp ◽  
History Of ◽  
Tribal Populations ◽  
Glucose 6 Phosphate Dehydrogenase ◽  
National Prevalence

Introduction: The aim of the study was to enumerate the clinical, hematological and molecular spectrum of G6PD deficiency in malaria endemic regions of south west Odisha. Methods: Diagnosis of G6PD deficiency was made by using the Di-chloroindophenol Dye test in from two south west districts (Kalahandi and Rayagada) of Odisha State. Demographic and clinical history was taken from each individual using a pre-structured questionnaire. Molecular characterization of G6PD deficiency was done using PCR-RFLP and Sanger sequencing. Results:  A total of 1981 individuals were screened, out of which 59 (2.97%) individuals were found G6PD deficient. Analysis revealed that G6PD deficiency was more in males (4.0%) as compared to females (2.3%). G6PD deficiency was significantly higher in tribal population (4.8%) as compared to non-tribal populations (2.4%) (p=0.012, OR=2.014, 95%CI =1.206-3.365). Individuals with history of malaria and G6PD deficiency have high risk of need of blood transfusion than G6PD normal individuals (p=0.026, OR=3.816, 95%CI=1.079-13.496). Molecular analysis revealed G6PD Orissa as the most common (88%) mutation 88% in the studied cohort. G6PD Kaiping (n=3), G6PD Coimbra (n=2) and G6PD Union (n=1) were also identified in studied cohort.  Conclusion: The cumulative prevalence of G6PD deficiency the present is below the estimated national prevalence. G6PD deficiency was higher in tribes as compared to non-tribes. Rare G6PD Kaiping and G6PD Union variants have been identified.

Methemoglobinaemia in Diabetic Ketoacidosis Patient

2021 ◽  
Vol 03 ◽  
Author(s):  
Magdy Mohamed ◽  
Nadem Javed
Keyword(s):  
Ascorbic Acid ◽  
Family History ◽  
Diabetic Ketoacidosis ◽  
G6pd Deficiency ◽  
Genetic Disorder ◽  
Red Blood Cell Transfusion ◽  
Case Presentation ◽  
History Of ◽  
Insulin Dependent ◽  
Glucose 6 Phosphate Dehydrogenase

Background: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a common X-linked genetic disorder. Case Presentation: In this paper, we report a case of a 41-years-old male patient with non-insulin-dependent diabetes and a family history of G6PD deficiency never known to have any previous hemolytic episodes, presented as a case of diabetic ketoacidosis with features of hemolytic anemia due to G6PD deficiency manifesting as methemoglobinemia and anemia. Conclusion: Our patient successfully managed with ascorbic acid and red blood cell transfusion. Clinicians should, therefore, be aware of the possibility of this uncommon association between diabetic ketoacidosis, G6PD deficiency, and methemoglobinemia which may be present in patients with G6PD deficiency and severe hemolysis.

scholarly journals SARS-CoV-2 Trigger in Severe Insulin Resistance With Acute Haemolytic Crisis in Diabetes and Glucose-6-phosphate Dehydrogenase Deficiency

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A397-A398
Author(s):  
Hareesh Joshi ◽  
Kenneth Earle
Keyword(s):  
Diabetes Mellitus ◽  
Insulin Resistance ◽  
Type 2 Diabetes ◽  
G6pd Deficiency ◽  
Insulin Infusion ◽  
Once Daily ◽  
Acting Insulin ◽  
History Of ◽  
Glucose 6 Phosphate Dehydrogenase

Abstract Background: We report a case of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection as trigger for increased insulin resistance and severe haemolytic crisis in a male with type 2 diabetes mellitus and glucose-6-phosphate dehydrogenase (G6PD) deficiency. Clinical Case: A 64-year-old man (BMI 25kg/m2, weight 75kg) with past medical history of type 2 diabetes mellitus (on metformin and sitagliptin; glycated haemoglobin 51 mmol/mol, n<42mmol/mol), hypertension, G6PD deficiency and gout was admitted to hospital with COVID pneumonitis and type 1 respiratory failure giving 5 days’ history of cough, shortness of breath, fatigue and tiredness. As per hospital guidelines, the patient was treated with amoxicillin/clavulanate 625mg three times daily and doxycycline 100mg once daily. Hydroxycholoroquine was not given in view of G6PD deficiency. There was no evidence of diabetic ketoacidosis and a short-acting insulin sliding scale was initiated at 2U/hr. Continuous positive pressure ventilation was offered for next 72 hours, however the patient failed to improve and required transfer to intensive care unit for intubation and mechanical ventilation. Computer tomography scan of pulmonary artery excluded embolism. The patient was fed via nasogastric tube post intubation. On day 8, the patient experienced a sudden drop in haemoglobin levels from 132 g/dl on day 1 to 68 g/dl, requiring multiple blood transfusions. The blood results demonstrated evidence of haemolysis with a rise in total and direct bilirubin and lactate dehydrogenase levels. The peripheral blood smear showed numerous bite cells with polychromasia suggesting an acute haemolytic crisis in the context of G6PD deficiency. A medication review revealed no evidence of drug-induced haemolysis. Later the patient was started on dexamethasone 6mg once daily (day 11) and on remdesivir 100mg once daily (day 15). During this time, the patient’s insulin infusion requirements had progressively increased from 2U/hr to 8U/hr (equivalent to 192 units/24 hr). On day 22, the patient’s clinical condition deteriorated with septicaemia requiring extended course of antibiotics. At this time, continuous insulin infusion was stopped and intermediate acting insulin (insulatard 36U twice daily; later increased to 48U twice daily) was started. In the next 10 days, the patient made good clinical recovery from sepsis with stable haemoglobin and blood sugar levels. He was extubated and transferred for rehabilitation. The patient was successfully weaned off insulin in the community with optimal diabetes control. Conclusion: This is a case demonstrating the possible role of SARS-CoV-2 in increased insulin resistance and severe haemolytic crisis on background of diabetes and G6PD deficiency.

scholarly journals Glucose-6-phosphate Dehydrogenase Deficiency: A Case Report

2017 ◽  
Vol 12 (1) ◽  
pp. 47-49
Author(s):  
Md Kamrul Hassan ◽  
Aloke Kumar Saha ◽  
Lakshman Chandra Kundu ◽  
Poly Begum ◽  
Abu Yousuf
Keyword(s):  
G6pd Deficiency ◽  
Neonatal Jaundice ◽  
Dehydrogenase Deficiency ◽  
Cultural Factors ◽  
Enzyme Defect ◽  
Asian Populations ◽  
Hemolytic Crisis ◽  
Life Threatening ◽  
Fava Beans ◽  
Glucose 6 Phosphate Dehydrogenase

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common hereditary enzyme disorder and more than 200 million people have a deficiency in this enzyme. G6PD deficiency is an X-linked enzyme defect, and one of its main signs is the presence of hemolytic anemia. It is a worldwide important cause of neonatal jaundice and causes life threatening hemolytic crisis in childhood. At later ages, certain drugs such as anti-malarial drugs and fava beans cause hemolysis among G6PD deficiency patients. The frequency and severity is influenced by genetic and cultural factors. It is common in Mediterranean, African and some East Asian populations but rare in Bangladeshi peoples. Genetic counseling may be of benefit for patients and their families. Other treatment is symptomatic and supportive.Faridpur Med. Coll. J. Jan 2017;12(1): 47-49

Rasburicase-induced haemolysis and methemoglobinemia: an ongoing issue

2021 ◽  
Vol 14 (3) ◽  
pp. e240967 ◽  
Author(s):  
Luai Madanat ◽  
Daniel Schoenherr ◽  
Elizabeth Wey ◽  
Ruby Gupta
Keyword(s):  
G6pd Deficiency ◽  
Blood Film ◽  
Caucasian Woman ◽  
Presumptive Diagnosis ◽  
Successful Resolution ◽  
History Of ◽  
Hypoxic Respiratory Failure ◽  
Glucose 6 Phosphate Dehydrogenase ◽  
Acute Anaemia

We report a case of a 91-year-old Caucasian woman with a history of chronic lymphocytic leukaemia who developed acute hypoxic respiratory failure (AHRF) requiring intubation for less than 24 hours after receiving rasburicase. Laboratory workup was significant for methemoglobinemia and acute anaemia, and blood film demonstrated evidence of oxidative haemolysis with bite cells. The patient was given a presumptive diagnosis of glucose-6-phosphate dehydrogenase (G6PD) deficiency and was managed conservatively with successful resolution of AHRF and stabilisation of haemoglobin level. Seven days after admission, she passed away due to subsequent complications; hence, follow-up G6PD level could not be obtained. Haemolytic anaemia and methemoglobinemia in the setting of recent rasburicase administration should raise clinical suspicion for G6PD deficiency. In non-emergent cases, patients should be screened prior to receiving rasburicase regardless of risk factors. Because rasburicase is often needed emergently, patients at high risk of tumour lysis syndrome should be screened early for G6PD deficiency.

Seizure following acute hemolysis caused by Glucose-6-phosphate dehydrogenase Deficiency

2019 ◽  
Author(s):  
Bahram Darbandi ◽  
Simin Sajudi ◽  
Vahid Aminzadeh ◽  
Kioomars Golshekan ◽  
Afagh Hassanzadeh Rad ◽  
...  
Keyword(s):  
Drinking Water ◽  
G6pd Deficiency ◽  
Dehydrogenase Deficiency ◽  
Statistical Significance ◽  
Cross Sectional Study ◽  
P Value ◽  
Cross Sectional ◽  
Exact Test ◽  
History Of ◽  
Glucose 6 Phosphate Dehydrogenase

Background: Storage of platelet concentrates (PCs) at room temperature (20-24°C) limits its storage time to 5 Background: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common inherited enzyme deficiency of the human red blood cells . Most of G6PD deficient individuals are asymptomatic, but acute hemolytic anemia may be presented with nausea, vomiting, abdominal pain, headache, jaundice, pallor, discoloration of the urine, chills, and fever. Seizure is reported as a rare symptom, as well. The present study aimed to investigate seizure following acute hemolysis caused by Glucose-6-phosphate dehydrogenase deficiency. Material and Methods:  This analytic cross-sectional study was conducted on all consecutive patients aged 1-12 years with G6PD deficiency hospitalized for hemolysis in 17 Shahrivar children hospital, Rasht, Iran, in 2016. Demographic characteristics and other variables such as place of inhabitants, type of drinking water, history of seizure in the patients and family, cause of hemolysis, hemoglobin level and hemoglobinuria on admission, and infection history prior to hemolysis were recorded. Data were analyzed by Mann-Whitney U test and Fischer Exact Test.  P-value < 0.05 indicated statistical significance and data were assessed by SPSS (version 20). Results:  The youngest patient was one year old and the oldest was 11 years old. Most of them were males (68.9%). Out of 244 patients, 8 ones (3.3%) experienced seizure. There was a significant correlation between seizure occurrence and family history of seizure (p=0.03) as well as fava bean consumption (p=0.019) as the causes of hemolysis; but not with infection as the cause of hemolysis, hemoglobin or hemoglobinuria level on admission, types of drinking water, place of living, and gender. Methemoglobinemia was considered as the main cause of the seizure. Conclusion: Although the rate of seizure was not so high (3.3%), it seems that seizure can be a critical and potentially life-threatening complication in these patients. Environmental factors may also play a role in the pathogenesis of the seizure in these patients.

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