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1.
Biochim Biophys Acta Mol Cell Res ; 1870(1): 119378, 2023 01.
Artigo em Inglês | MEDLINE | ID: mdl-36220452

RESUMO

Sulfhemoglobinemia is an incurable disease caused by an overdose of sulfur-containing drugs with oxidizing properties. Its diagnosis remains hindered due to the similarity of symptoms to other pathological state - methemoglobinemia, as well as contradictory information on the structure and characteristics of sulfhemoglobin. Herein, we present sulfhemoglobinemia model on living functional human erythrocytes, designed to recreate processes which could take place in a patient body in order to complement missing information and highlight distinctiveness of two hemoglobin (Hb) adducts formed after interaction with sulfur donors. Employed techniques, UV-Vis absorption, Raman, Fourier transformed infrared (FT-IR) and electronic circular dichroism (ECD) spectroscopies, allowed to distinguish and characterize Hb adduct with sulfur atom bounded directly to the iron ion (HbFeIII-SH), and irreversibly connected to the porphyrin ring (SHb - sulfhemoglobin). Presented herein results provided also new evidence on formation of both these hemoglobin adducts inside functional erythrocytes under oxidative conditions and during sulfur-containing drug presence, what can be further translated into future physiological studies. Moreover, we found that sulfur attachment to the porphyrin ring altered Hb structure and lead to changes in protein packing inside RBCs, eventually. Interestingly, measurement of blood drop smear by Raman spectroscopy occurred the most accurate method to differentiate HbFeIII-SH and SHb, indicating potential of this technique in sulfhemoglobinemia diagnosis.


Assuntos
Porfirinas , Sulfemoglobinemia , Humanos , Sulfa-Hemoglobina/análise , Sulfemoglobinemia/diagnóstico , Espectroscopia de Infravermelho com Transformada de Fourier , Hemoglobinas , Enxofre
2.
Rev Med Chil ; 150(10): 1401-1406, 2022 Oct.
Artigo em Espanhol | MEDLINE | ID: mdl-37358100

RESUMO

Sulfhemoglobin (SulfHb) is formed by hemoglobin (Hb) oxidation by sulfur compounds. Sulfhemoglobinemia is mainly associated with drugs or intestinal bacterial overgrowth. Patients present with central cyanosis, an abnormal pulse oximetry and normal arterial oxygen partial pressure. These features are shared with methemoglobinemia (MetHb) whose diagnosis requires an arterial co-oximetry. Depending on the device used, SulfHb may produce interference with this technique. We report two females aged 31 and 43 years, consulting at the emergency room with cyanosis. Both had a history of acute and chronic, high dose zopiclone ingestion. Pulse oximetry showed desaturation but with normal arterial oxygen partial pressure. Cardiac and pulmonary diseases were ruled out. Co-oximetry in two different analyzers showed interference or normal MetHb percentages. No other complications ensued, and cyanosis decreased over days. Since MetHb was discarded among other causes of cyanosis in a compatible clinical context, the diagnosis of sulfhemoglobinemia was made. The confirmatory method is not available in Chile. The presence of SulfHb is difficult to diagnose, confirmatory tests are not readily available, and it frequently interferes with arterial co-oximetry. This is attributed to a similar absorbance peak of both pigments in arterial blood. Venous co-oximetry can be useful in this context. SulfHb is a self-limited condition in most cases, however it must be differentiated from methemoglobinemia to avoid inappropriate treatments like methylene blue.


Assuntos
Metemoglobinemia , Sulfemoglobinemia , Feminino , Humanos , Metemoglobinemia/induzido quimicamente , Metemoglobinemia/diagnóstico , Sulfemoglobinemia/complicações , Oximetria/efeitos adversos , Cianose/complicações , Oxigênio
3.
Int J Lab Hematol ; 43(4): 837-844, 2021 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-34092029

RESUMO

INTRODUCTION: Methemoglobin (MetHb) and sulfhemoglobin (SHb) measurements are useful in the evaluation of cyanosis. When one or both values are elevated, additional analysis is important to establish the etiology of the disorder. Methemoglobinemia occurs from acquired or hereditary causes with diverse treatment considerations, while true sulfhemoglobinemia is only acquired and treatment is restricted to toxin removal. Some toxic exposures can result in a dual increase in MetHb and SHb. Hereditary conditions, such as M-Hemoglobin variants (M-Hbs), can result in increased MetHb and/or SHb values but are clinically compensated and do not require treatment if they are cyanotic but otherwise clinically well. METHODS: Herein, we report 53 hemoglobin variant cases that have associated MetHb and SHb levels measured by an adapted Evelyn-Malloy laboratory assay method. RESULTS: Our data indicate M-Hbs cause variable patterns of MetHb and SHb elevation in a fairly reproducible pattern for the particular variant. In particular, α globin chain M-Hbs can mimic acquired sulfhemoglobinemia due to an isolated increased SHb value. CONCLUSION: If the patient appears clinically well other than cyanosis, M-Hbs should be considered early in the evaluation process to differentiate from acquired conditions to avoid unnecessary testing and treatment regimens and prompt genetic counseling.


Assuntos
Cianose/sangue , Metemoglobina/análise , Sulfa-Hemoglobina/análise , Adolescente , Adulto , Criança , Pré-Escolar , Cianose/genética , Feminino , Variação Genética , Hemoglobina M/análise , Hemoglobina M/genética , Humanos , Lactente , Masculino , Metemoglobinemia/sangue , Metemoglobinemia/genética , Sulfemoglobinemia/sangue , Sulfemoglobinemia/genética , Adulto Jovem
4.
J Emerg Med ; 61(2): 147-150, 2021 08.
Artigo em Inglês | MEDLINE | ID: mdl-34034895

RESUMO

BACKGROUND: Sulfhemoglobinemia is a rare dyshemoglobinemia that presents similarly to methemoglobinemia. CASE REPORT: An 83-year-old woman with stage IV ovarian cancer presented to the Emergency Department after a near syncopal spell and was found to be cyanotic with a pulse oximetry reading of 71%. Pulse oximetry improved to only the mid-80s range with administration of high-flow oxygen. Her arterial blood gas on supplemental high-flow oxygen demonstrated a PaO2 of 413 mm Hg and methemoglobin of 1.2%, but also noted the interference of the co-oximetry with sulfhemoglobinemia. Further history revealed that the patient had recently been started on phenazopyridine. The phenazopyridine was stopped, an exchange transfusion was offered but declined, and the patient was discharged to home hospice. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: The diagnosis of sulfhemoglobinemia can be challenging given that routine co-oximetry does not identify it. The clue to the diagnosis is that the cyanotic-appearing patient has a normal or elevated PaO2 and seems to be less ill than expected, given the degree of cyanosis. Sulfhemoglobinemia does not reverse with the administration of methylene blue.


Assuntos
Metemoglobinemia , Sulfemoglobinemia , Idoso de 80 Anos ou mais , Cianose , Dispneia , Feminino , Humanos , Metemoglobinemia/diagnóstico , Azul de Metileno , Oximetria , Fenazopiridina
5.
J Pediatr Hematol Oncol ; 42(8): e765-e767, 2020 11.
Artigo em Inglês | MEDLINE | ID: mdl-31568176

RESUMO

Sulfhemoglobinemia (SulfHb) is a rare dyshemoglobinemia that can present with cyanosis in the absence of respiratory distress. It has been reported secondary to drug ingestion and chronic constipation. We present a case of SulfHb in an adolescent female with spina bifida and neurogenic bladder in the setting of an Escherichia coli urinary tract infection. An arterial blood gas differentiated a dyshemoglobinemia from other causes of hypoxemia. The resolution was achieved with antibiotics and red cell transfusion. Here we review the pathophysiology of SulfHb and contribute a unique case report to the limited body of literature on this topic.


Assuntos
Infecções por Escherichia coli/complicações , Escherichia coli/isolamento & purificação , Disrafismo Espinal/complicações , Sulfemoglobinemia/etiologia , Bexiga Urinaria Neurogênica/complicações , Infecções Urinárias/complicações , Antibacterianos/uso terapêutico , Criança , Infecções por Escherichia coli/microbiologia , Infecções por Escherichia coli/patologia , Feminino , Humanos , Prognóstico , Disrafismo Espinal/microbiologia , Disrafismo Espinal/patologia , Sulfemoglobinemia/tratamento farmacológico , Sulfemoglobinemia/patologia , Bexiga Urinaria Neurogênica/microbiologia , Bexiga Urinaria Neurogênica/patologia , Infecções Urinárias/microbiologia , Infecções Urinárias/patologia
6.
Forensic Sci Int ; 297: e1-e7, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30777401

RESUMO

The study reports the environmental, toxicological and histopathological forensic investigations applied on three victims of accidental death (father, mother and son), due to the fall in a volcanic pothole, during the touristic visit of the "Solfatara park", near Naples (Italy). At autopsy greenish skin discolouration was observed and all bodies showed the classical signs of asphyxial deaths, such as cyanosis and hemorrhagic pulmonary edema. Focal micro-hemorrhages were found in the brain at intracranial and subpial levels. The hemogasanalysis and spectrophotometric test on blood for Methemoglobin (MetHb), Carboxyhemoglobin (HbCO) and Sulfhemoglobin (SHb) showed pCO2, SHb and MetHb above the physiological levels. On biological specimens, toxicological analyses performed by GC/MS revealed high concentrations of hydrogen sulfide (H2S) and of thiosulfate (TS), its main metabolite. The monitoring of toxic gases on the death scene showed an unsafe environment, into the pothole, able to cause the sudden loss of consciousness of the victims with subsequent asphyxiation (knockdown effect). In particular, at the bottom of the hole, the maximum levels of H2S and carbon dioxide (CO2) were 2200 ppm and 98% respectively. For the family members, the cause of the death was assessed as acute poisoning by H2S and CO2. The fatalities, happened in quick succession as for a domino effect, were pretty similar to the asphyxial deaths by confined spaces, frequently observed in occupational setting. Fatalities secondary to accidental volcanic gases inhalation, such as H2S and CO2 in geothermal areas, have been already described but often without a forensic approach. To the best of our knowledge this is the first case that reports the accidental poisoning by volcanic gases involving three people, with different caracteristic of age and sex, allowing the correlation between toxicological and pathological results with the true levels of asphyxiating gas, measured on the death scene.


Assuntos
Acidentes por Quedas , Asfixia/etiologia , Dióxido de Carbono/envenenamento , Gases , Sulfeto de Hidrogênio/envenenamento , Erupções Vulcânicas , Adulto , Criança , Feminino , Humanos , Masculino , Metemoglobinemia/etiologia , Pessoa de Meia-Idade , Sulfemoglobinemia/etiologia , Tiossulfatos/sangue
7.
Annu Int Conf IEEE Eng Med Biol Soc ; 2017: 4570-4573, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-29060914

RESUMO

Methemoglobinemia and sulfhemoglobinemia are rare, but potentially life threatening, diseases that refer to an abnormal amount of methemoglobin or sulfhemoglobin in the blood, respectively. Unfortunately, blood samples containing abnormal quantities of methemoglobin or sulfhemoglobin have similar spectral characteristics. This makes it difficult to optically differentiate them and, hence, difficult to diagnose a patient with either disease. However, performing treatments for one of the diseases without a correct diagnosis can introduce increased risk to the patient. In this paper, we propose a method for differentiating the presence of methemoglobin and sulfhemoglobin in blood, under several conditions, using reflectance values measured at three wavelengths. In order to validate our method, we perform in silico experiments considering various levels of methemoglobin and sulfhemoglobin. These experiments employ a cell-based light interaction model, known as CLBlood, which accounts for the orientation and distribution of red blood cells. We then discuss the reflectance curves produced by the experiments and evaluate the efficacy of our method. In particular, we consider various experimental conditions by modifying the flow rate, hemolysis level and incident light direction.


Assuntos
Metemoglobina/análise , Sulfa-Hemoglobina/análise , Diferenciação Celular , Humanos , Metemoglobinemia , Sulfemoglobinemia
8.
Pediatrics ; 136(6): e1641-5, 2015 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-26553186

RESUMO

Sulfhemoglobinemia is a rare disorder characterized by the presence of sulfhemoglobin in the blood. It is typically drug-induced and may cause hypoxia, end-organ damage, and death through oxygen deprivation. We present here a case of non-drug-induced sulfhemoglobinemia in a 7-day-old preterm infant complicated by hemolytic anemia. Microbiota compositional analysis of fecal samples to investigate the origin of hydrogen sulphide revealed the presence of Morganella morganii at a relative abundance of 38% of the total fecal microbiota at the time of diagnosis. M morganii was not detected in the fecal samples of 40 age-matched control preterm infants. M morganii is an opportunistic pathogen that can cause serious infection, particularly in immunocompromised hosts such as neonates. Strains of M morganii are capable of producing hydrogen sulphide, and virulence factors include the production of a diffusible α-hemolysin. The infant in this case survived intact through empirical oral and intravenous antibiotic therapy, probiotic administration, and red blood cell transfusions. This coincided with a reduction in the relative abundance of M morganii to 3%. Neonatologists should have a high index of suspicion for intestinal pathogens in cases of non-drug-induced sulfhemoglobinemia and consider empirical treatment of the intestinal microbiota in this potentially lethal condition.


Assuntos
Anemia Hemolítica/complicações , Infecções por Enterobacteriaceae/complicações , Morganella morganii , Sulfemoglobinemia/complicações , Anemia Hemolítica/terapia , Infecções por Enterobacteriaceae/microbiologia , Infecções por Enterobacteriaceae/terapia , Fezes/microbiologia , Feminino , Microbioma Gastrointestinal , Humanos , Recém-Nascido , Recém-Nascido Prematuro , Sulfemoglobinemia/terapia
10.
J Biomed Opt ; 17(9): 97005, 2012 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-22975680

RESUMO

There are several pathologies whose study and diagnosis is impaired by a relatively small number of documented cases. A practical approach to overcome this obstacle and advance the research in this area consists in employing computer simulations to perform controlled in silico experiments. The results of these experiments, in turn, may be incorporated in the design of differential protocols for these pathologies. Accordingly, in this paper, we investigate the spectral responses of human skin affected by the presence of abnormal amounts of two dysfunctional hemoglobins, methemoglobin and sulfhemoglobin, which are associated with two life-threatening medical conditions, methemoglobinemia and sulfhemoglobinemia, respectively. We analyze the results of our in silico experiments and discuss their potential applications to the development of more effective noninvasive monitoring and differentiation procedures for these medical conditions.


Assuntos
Hemoglobinas/análise , Metemoglobinemia/diagnóstico , Metemoglobinemia/metabolismo , Pele/metabolismo , Análise Espectral/métodos , Sulfemoglobinemia/diagnóstico , Sulfemoglobinemia/metabolismo , Diagnóstico por Computador/métodos , Diagnóstico Diferencial , Reprodutibilidade dos Testes , Sensibilidade e Especificidade
11.
Rev Mal Respir ; 27(1): 80-3, 2010.
Artigo em Francês | MEDLINE | ID: mdl-20146957

RESUMO

INTRODUCTION: An acquired abnormality of haemoglobin is among the many causes of cyanosis, especially in patients with no identified cardiorespiratory cause. CASE REPORT: A 50-year-old woman, suffering from amyotrophic lateral sclerosis, was hospitalised for dyspnoea. Physical examination revealed cyanosis that persisted despite oxygen therapy. Discordance between the reduced arterial oxygen saturation and normal arterial oxygen tension led to a search for a dyshaemoglobinaemia as a possible cause. Use of co-oxymetry with spectrophotometry revealed sulphaemoglobinaemia. Sulphaemoglobinaemia is due to irreversible incorporation of a thiol radical into the porphyrin ring of a haem group. This decreases the affinity of haemoglobin for oxygen and thus reduces oxygen carrying capacity. A drug-induced cause is often identified. However, no previously described cause for sulphaemoglobinaemia was identified in our patient. The patient was currently being treated with thiocolchicoside (Miorel((R))). Thiocolchicoside was suspected as the cause because its chemical structure contains an easily hydrolysable thiol radical. Withdrawal of thiocolchicoside led to regression of the sulphaemoglobinaemia. CONCLUSIONS: This report underlines the importance of searching for an acquired abnormality of haemoglobin (methaemoglobinaemia or sulphaemoglobinaemia) in patients with cyanosis resistant to oxygen, in the absence of any cardiorespiratory abnormality. This case is the first to suspect thiocolchicoside as a possible cause of sulphaemoglobinaemia.


Assuntos
Esclerose Amiotrófica Lateral/tratamento farmacológico , Colchicina/análogos & derivados , Cianose/induzido quimicamente , Sulfemoglobinemia/induzido quimicamente , Compostos de Sulfidrila/sangue , Tranquilizantes/toxicidade , Esclerose Amiotrófica Lateral/sangue , Colchicina/farmacocinética , Colchicina/uso terapêutico , Colchicina/toxicidade , Cianose/sangue , Diagnóstico Diferencial , Quimioterapia Combinada , Feminino , Radicais Livres , Humanos , Pessoa de Meia-Idade , Sulfemoglobinemia/sangue , Tranquilizantes/farmacocinética , Tranquilizantes/uso terapêutico
12.
J Clin Rheumatol ; 15(3): 127-9, 2009 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-19300288

RESUMO

Rheumatologists are often asked to evaluate patients with Raynaud phenomenon. Occasionally, an alternate explanation is revealed such as acrocyanosis. Methemoglobinemia and sulfhemoglobinemia are rare causes of cyanosis that can be medication-induced. Both are known complications of therapy with phenazopyridine. We report an unusual case of a 45-year-old woman in whom sulfhemoglobinemia from chronic therapy with phenazopyridine was misdiagnosed as due to Raynaud phenomenon and limited scleroderma. This case illustrates the importance of taking into account medication-related adverse events when evaluating patients with Raynaud-like phenomenon.


Assuntos
Anestésicos Locais/efeitos adversos , Cianose/etiologia , Fenazopiridina/efeitos adversos , Doença de Raynaud/diagnóstico , Sulfemoglobinemia , Adulto , Diagnóstico Diferencial , Dispneia/etiologia , Feminino , Humanos , Polimedicação , Sulfemoglobinemia/induzido quimicamente , Sulfemoglobinemia/complicações , Sulfemoglobinemia/diagnóstico
15.
Clin Toxicol (Phila) ; 45(2): 189-92, 2007.
Artigo em Inglês | MEDLINE | ID: mdl-17364641

RESUMO

Sulfhemoglobinemia (SHb) is an uncommon cause of cyanosis that is predominantly drug-induced in adults. We report an unusual case of sodium sulfate-induced sulfhemoglobinemia in a 61-year-old woman after surgical polypectomy. Fractional hemoglobin derivates were assayed by spectrophotometry and high-performance liquid chromatography. The SHb ratio was 8.6% in the first sample and 3.77% a month later measured by spectrophotometry. In the blood hemolysate, a new peak was identified as SHb with high-performance liquid chromatography (HPLC). HPLC showed the presence of 9.37% SHb in the first sample and 4.88% a month later. After removing the suspected toxic agent the cyanosis decreased significantly. The findings underline the importance of routine SHb detection in cyanosis of unknown origin especially in emergency cases.


Assuntos
Cianose/diagnóstico , Pólipos Intestinais/cirurgia , Sulfatos/efeitos adversos , Sulfemoglobinemia/diagnóstico , Cromatografia Líquida de Alta Pressão , Constipação Intestinal/prevenção & controle , Cianose/sangue , Cianose/induzido quimicamente , Feminino , Humanos , Pessoa de Meia-Idade , Sulfatos/administração & dosagem , Sulfatos/uso terapêutico , Sulfa-Hemoglobina/análise , Sulfemoglobinemia/sangue , Sulfemoglobinemia/induzido quimicamente
17.
Ann Pharmacother ; 39(6): 1128-30, 2005 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-15886294

RESUMO

OBJECTIVE: To report a case of sulfhemoglobinemia in a patient receiving phenazopyridine for a urinary tract infection. CASE SUMMARY: A 63-year-old white woman presented to the emergency department with complaints of fatigue and bluish discoloration of her body that had gradually progressed over the previous 6-8 weeks. About 4 months prior to presenting to the emergency department, she had started taking phenazopyridine, an over-the-counter medication for symptoms of dysuria. Because the cyanosis did not improve after the patient received oxygen and methylene blue, sulfhemoglobinemia was suspected and confirmed by spectrophotometer analysis. DISCUSSION: Sulfhemoglobin is a green-pigmented molecule containing a sulfur atom in one or more of the porphyrin rings. It is a rare cause of cyanosis, which is usually drug induced. Sulfhemoglobinemia is suspected when a cyanotic patient has normal to near-normal oxygen tension, laboratory reports of elevated methemoglobin, and does not respond to methylene blue therapy. Sulfhemoglobinemia is relatively rare, despite the widespread use of drugs that have been reported to cause it. Predisposing factors, such as chronic constipation, present in our patient, have been suggested as a source of hydrogen sulfide. CONCLUSIONS: This case of sulfhemoglobinemia, which occurred after the patient took phenazopyridine, is considered a probable adverse event according to the Naranjo probability scale.


Assuntos
Fenazopiridina/efeitos adversos , Sulfemoglobinemia/induzido quimicamente , Administração Oral , Feminino , Humanos , Pessoa de Meia-Idade , Fenazopiridina/administração & dosagem , Sulfemoglobinemia/diagnóstico , Infecções Urinárias/tratamento farmacológico
18.
Ig Sanita Pubbl ; 60(4): 201-17, 2004.
Artigo em Italiano | MEDLINE | ID: mdl-15583709

RESUMO

Significant exposure to hydrogen sulfide may occur in workers at sulphureous thermal baths. Work-related exposure to hydrogen sulfide may be shown by measuring sulfhemoglobin (S-Hb) blood levels. In this study we measured S-Hb blood levels in two groups of workers at two different thermal baths and compared these with hydrogen sulfide concentrations in the air of the two work environments. Our results show that blood S-Hb levels can be considered a reliable measure of individual exposure to hydrogen sulfide.


Assuntos
Balneologia , Sulfeto de Hidrogênio , Exposição por Inalação , Sulfemoglobinemia/sangue , Feminino , Humanos , Itália , Masculino
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