scholarly journals A rare cause of cyanosis: Congenital methemoglobinemia

Author(s):  
RAHMA GUEDRI ◽  
Nada Missaoui ◽  
Leila ESSADDAM ◽  
SAAYDA Ben Becher

A 13-month-old infant, born from a consanguineous marriage. She presented isolated cyanosis. Physical examination was normal. Pulsed oxygen saturation was 94% in room air. Methemoglobin level was 39.4%. The diagnosis of congenital methemoglobinemia type I was retained. She was successfully treated with methylene blue infusions and ascobic acid.

Author(s):  
Ali H. Jawad ◽  
Ahmed Saud Abdulhameed ◽  
Noor Nazihah Bahrudin ◽  
Nurul Nadiah Mohd Firdaus Hum ◽  
S. N. Surip ◽  
...  

Abstract In this work, sugarcane bagasse waste (SBW) was used as a lignocellulosic precursor to develop a high surface area activated carbon (AC) by thermal treatment of the SBW impregnated with KOH. This sugarcane bagasse waste activated carbon (SBWAC) was characterized by means of crystallinity, porosity, surface morphology and functional groups availability. The SBWAC exhibited Type I isotherm which corresponds to microporosity with high specific surface area of 709.3 m2/g and 6.6 nm of mean pore diameter. Further application of SBWAC as an adsorbent for methylene blue (MB) dye removal demonstrated that the adsorption process closely followed the pseudo-second order kinetic and Freundlich isotherm models. On the other hand, thermodynamic study revealed the endothermic nature and spontaneity of MB dye adsorption on SBWAC with high acquired adsorption capacity (136.5 mg/g). The MB dye adsorption onto SBWAC possibly involved electrostatic interaction, H-bonding and π-π interaction. This work demonstrates SBW as a potential lignocellulosic precursor to produce high surface area AC that can potentially remove more cationic dyes from the aqueous environment.


2019 ◽  
Vol 3 (01) ◽  
pp. 20-23
Author(s):  
Tasneem Ara ◽  
Qazi Smita Haque ◽  
Salma Afrose

Congenital heart diseases are common cause of congenital cyanosis with polycythaemia. Congenital methemoglobinemia is a rare cause of lifelong cyanosis with polycythemia. Congenital methemoglobinemia is caused either by enzyme deficiency or by an abnormal Hb (Hb M). Asymptomatic despite presence of severe cyanosis indicates this rare disorder. We are reporting a rare case of polycythemia with cyanosis due to congenital methemoglobinemia. The patient was referred to our centre and attended Hematology OPD (out-patient department) when his routine CBC revealed erythrocytosis. At that time, we found him severely cyanosed especially apparent on lips, tongue, hands and feet. He was diagnosed as a case of congenital methemoglobinemia with 38% blood methemoglobin level (normal value-0.00-2.00%). On view of life long persistent cyanosis, without any cardiopulmonary and neurological abnormality, consanguinity of parent’s marriage, dark colored blood with high methemoglobin level, a final diagnosis of Type I enzyme deficiency congenital methemoglobinemia was made. He was treated with oral ascorbic acid 250 mg twice daily. At follow up after 6 months his skin color improved and RBC count returned to normal. We are reporting this case of congenital methemoglobinemia for the first time in Bangladesh to emphasize the importance of this rare entity in the differential diagnosis of asymptomatic cyanosis with polycythemia.


1996 ◽  
Vol 89 (3) ◽  
pp. 141-143 ◽  
Author(s):  
G Roeggla ◽  
M Roeggla ◽  
A Podolsky ◽  
A Wagner ◽  
A N Laggner

Reports of acute mountain sickness (AMS) at moderate altitude show a wide variability, possibly because of different investigation methods. The aim of our study was to investigate the impact of investigation methods on AMS incidence. Hackett's established AMS score (a structured interview and physical examination), the new Lake Louise AMS score (a self-reported questionnaire) and oxygen saturation were determined in 99 alpinists after ascent to 2.94 km altitude. AMS incidence was 8% in Hackett's AMS score and 25% in the Lake Louise AMS score. Oxygen saturation correlated inversely with Hackett's AMS score with no significant correlation with the Lake Louise AMS score. At moderate altitude, the new Lake Louise AMS score overestimates AMS incidence considerably. Hackett's AMS score remains the gold standard for evaluating AMS incidence.


2002 ◽  
Vol 15 (1) ◽  
pp. 75-78 ◽  
Author(s):  
Robert P. Granko

The author reports a case of topical benzocaine-induced methemoglobinemia. Benzocaine is a topical anesthetic commonly used during minor surgical and cannulation procedures. Although not widely reported, benzocaine has the potential to induce severe or life-threatening methemoglobinemia. Clinical practitioners should be cognizant of this complication associated with benzocaine use and its appropriate management. The following report describes a case of topical benzocaine-induced methemoglobinemia in a 74-year-old, Caucasian woman who had undergone transesophageal echocardiography and who received topical benzocaine for local anesthesia. Ten minutes after the administration of benzocaine, her oxygen saturation levels decreased to 71%, requiring immediate attention. Co-oximetry (multiple wavelength spectrophotometry) measured methemoglobin levels indicated the presence of methemoglobinemia. The patient was subsequently treated with 60 mg of intravenous methylene blue. Within 10 minutes, the patient’s condition had improved. The primary etiology of methemoglobinemia is from exposure to oxidizing agents. Benzocaine is an oxidizing agent commonly used as a topical anesthetic. Topically applied benzocaine, in the patient described here and in other reported cases, produced elevated methemoglobin levels requiring treatment with a reducing agent. Methylene blue, within a specified dosage range, can serve as a reducing agent to reverse the effects of topically applied benzocaine-induced methemoglobinemia.


2019 ◽  
Vol 2 (22.2) ◽  
pp. 155-164
Author(s):  
Liang Zhang

Background: There is an increasing local application of methylene blue (MB) in the treatment of discogenic low back pain (LBP) and percutaneous transforaminal endoscopic discectomy (PTED) procedures. MB could generate DNA damage and induce apoptosis in different cell types; however, the effects of MB on intervertebral disc (IVD) annulus fibrosus (AF) cells are not clearly understood. Objective: The objective of this study was to investigate the effects of different concentrations of MB on rat AF cells in vitro. Study Design: This study used an experimental design. Setting: This research was conducted at the Orthopaedic Institute of the Clinical Medical College of Yangzhou University. Methods: AF cells were isolated and cultured with different concentrations of MB (0, 2, 20, and 200 μg/mL) and assessed to determine the possible cytotoxic effects of MB. The cell proliferation was detected by Cell Counting Kit-8 (CCK-8) assay. The inverted phase-contrast microscopy was used to perform morphological observation of apoptotic cells, and flow cytometry was used to measure the incidence of cell apoptosis. The mRNA and protein expression levels of apoptosis-associated genes (caspase-3, Bcl-2, and Bax) and other related genes (collagen type I, transforming growth factor β1 [TGF-β1], fibroblast growth factor [bFGF], and tissue inhibitor of metalloproteinase-1 [TIMP-1]) were analyzed by quantitative real-time PCR (RT-PCR) and Western blotting. Results: Our results indicated that MB reduced cell viability in a concentration- and timedependent manner. MB also induced marked AF cell apoptosis in a concentration-dependent manner observed by inverted phase-contrast microscopy, flow cytometry, and indicated by the increased expression of caspase-3. Both RT-PCR and Western blotting revealed significant upregulation of Bax and caspase-3 expression levels accompanied by decreased expression of Bcl2 in a concentration-dependent manner. Moreover, collagen type I, TGF-β1, bFGF, and TIMP-1 mRNA and protein levels were also found to be decreased by MB in a concentration-dependent manner. Limitations: Limitations of this study were the in vitro study design and lack of in vivo validation of the observed effects of MB on human IVD cells. Conclusions: Our results indicate that a high concentration of MB can not only inhibit proliferation and paracrine function of AF cells, but can also induce cell apoptosis in a concentration-dependent manner, suggesting that it is necessary to choose low concentrations of MB in practical application and limit the use of MB in the treatment of discogenic LBP to research protocols. Key words: Methylene blue, annulus fibrosus cell, proliferation, apoptosis, paracrine


2021 ◽  
Vol 3 (1) ◽  
pp. 107-112
Author(s):  
mariyatul khiptiyah ◽  
◽  
Teguh Rahayu Sartono ◽  
Indrastuti Normahayu ◽  
Wiwi Jaya ◽  
...  

Introduction: High altitude pulmonary edema (HAPE) is one of the acute, severe, non- cardiogenic disease that could be life threatening, occurs upon either the first or subsequent exposure to high altitude. It is triggered by a shortage of oxygen after ascending high altitude. The most effective therapeutic approach for HAPE is to immediately descend from high altitude and to give oxygenation, maintaining arterial saturation over 90%, as well as letting the patient rest from strenuous physical activity. The use of portable hyperbaric chamber is also deemed effective in certain circumstance, and nifedipine can also be used to treat HAPE, even as additional treatment in condition that the patients had yet to descend and oxygenation is still not administrable. Case Report: We reported a case at Rs. Dr. Saiful Anwar, a 23-year-old male with High Altitude Pulmonary Edema (HAPE). Diagnosis established from anamnesis, physical examination, and laboratory tests. Patient complained shortness of breath when climbing Mt. Semeru, in which the patient reached an altitude of 2700 mdpl in 2 days. Physical examination showed oxygen saturation 46-49% with NRBM 10 lpm, and rhonchi breath sound in all lung areas. Laboratory examination showed leukocytosis, blood gas analysis showed hypocarbia, severe hipoxemia, metabolic acidosis, and type I respiratory failure. Ches XRay showed wide, irregular infiltrate in both lungs. Based on those, the patient was diagnosed with HAPE. In this case, the patient was given oxygenation. Conclusion: In this case, the patient was diagnosed with HAPE based on anamnesis, physical examination, and laboratory tests. Oxygenation given to the patient improved his condition.


2021 ◽  
Author(s):  
Silvia Mendez-Flores ◽  
Angel Alexis Priego-Ranero ◽  
Daniel Azamar-Llamas ◽  
Hector Olvera-Prado ◽  
Kenia Illian Rivas-Redondo ◽  
...  

BACKGROUND Currently, therapeutic options for ambulatory COVID-19 patients are limited. OBJECTIVE To evaluate the safety, efficacy and effect of the intramuscular administration of polymerized type I collagen (PTIC) on hyperinflammation, oxygen saturation and symptom improvement in adult outpatients with symptomatic COVID-19. DESIGN Double-blind, randomised, placebo-controlled clinical trial of PTIC vs placebo. SETTING Single Third-level hospital in Mexico City (Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran) PARTICIPANTS Eighty-nine adult participants with a confirmed COVID-19 diagnosis and symptom onset within the 7 days preceding recruitment were included from August 31, 2020 to November 7, 2020 and followed for 12 weeks. Final date of follow-up was February 4, 2021. INTERVENTIONS Patients were randomly assigned to receive either 1.5 ml of PTIC intramuscularly every 12 h for 3 days and then every 24 h for 4 days (n=45), or matching placebo (n=44). MAIN OUTCOMES AND MEASURES The primary outcome was a mean reduction of at least 50% in the level of IP-10 compared to baseline. The secondary outcomes were mean oxygen saturation >92% while breathing ambient air and duration of symptoms. RESULTS Of 89 patients who were randomised, 87 (97.8%) were included in an intention-to-treat analysis; 37 (41.6%) were male and mean age was 48.5+/-14.0 years. The IP-10 levels decreased 75% in the PTIC group and 40% in the placebo group vs baseline. The comparison between treatment vs placebo was also statistically significant (P=0.0047). The IL-8 (44%, P=0.045), M-CSF (25%, P=0.041) and IL-1Ra (36%, P=0.05) levels were also decreased in the PTIC group vs baseline. Mean oxygen saturation >92% was achieved by 40/44 (90%), 41/42 (98%) and 40/40 (100%) of participants that received PTIC at 8, 15 and 97 days of follow-up vs 29/43 (67%), 31/39 (80%) and 33/37 (89%) of patients treated with placebo (P=0.001). The unadjusted accelerated failure time model showed that patients treated with PTIC achieved the primary outcome 2.70-fold faster (P<0.0001) than placebo. In terms of risk, the group of patients treated with PTIC had a 63% lower risk of having a mean oxygen saturation <92% vs placebo (P<0.0001). Symptom duration in patients treated with PTIC was reduced by 6.1+/-3.2 days vs placebo. No differences in adverse effects were observed between the groups at 8, 15 and 97 days of follow-up. CONCLUSIONS In this study, treatment with PTIC down-regulated IP-10, IL-8, M-CSF and IL-Ra levels, which could explain the PTIC effect on the higher proportion of patients with mean oxygen saturation readings > 92% and a shorter duration of symptoms as compared to patients treated with placebo. Although results are encouraging, larger randomised trials are needed.


1997 ◽  
Vol 66 (2) ◽  
pp. 204-208 ◽  
Author(s):  
H. Abe ◽  
K. Ikebuchi ◽  
S. J. Wagner ◽  
M. Kuwabara ◽  
N. Kamo ◽  
...  

2018 ◽  
Vol 10 (1) ◽  
Author(s):  
Dina Sameh Soliman ◽  
Mohamed Yassin

Methemoglobinemia is a rare overlooked differential diagnosis in patients presented with cyanosis and dyspnea unrelated to cardiopulmonary causes. Our patient is 29 year old Indian non-smoker male, his story started 6 months prior to presentation to our center when he had generalized fatigue and discoloration of hands. He presented with persistent polycythemia with elevated hemoglobin level. The patient was misdiagnosed in another center as polycythemia and treated with Imatinib. The diagnosis of PV was revisited and ruled out in view of negative JAK2, normal erythropoietin level and absence of features of panmyelosis. Clinical cyanosis and lowoxygen saturation in the presence of normal arterial oxygen tension was highly suggestive of methemoglobinemia. Arterial blood gas revealed a methemoglobin level of 38% (normal: 0-1.5%). Cytochrome B5 reductase (Methemoglobin reductase B) was deficient at level of <2.6 U/g Hb) (normal: 6.6-13.3), consistent with methemoglobin reductase (cytochrome b5) deficiency and hence the diagnosis of congenital methemoglobinemia was established. The role of Imatinib in provoking methemoglobinemia is questionable and association between Imatinib and methemoglobinemia never described before. In our case, there were no other offending drugs in aggravating the patients’ symptoms and cyanosis. The patient started on Vitamin C 500 mg once daily for which he responded well with less cyanosis and significant reduction of methemoglobin level. Congenital methemoglobinemia is a rare underreported hemoglobin disease and often clinically missed. Upon extensive review of English literature for cases of congenital methemoglobinemia due to deficiency of cytochrome b5 reductase, we found 23 cases diagnosed as type I (including the case reported here). 17 cases (~74%) of type I and 6 cases (27%) of type II. There is male predominance 73% versus 26% in females. Almost half of reported cases 12 cases (52%) are Indian, 2 Japanese, 3 English, 2 Arabic, one case Spanish and one case Italian. For type I, the median calculated age is 31 years with cyanosis and shortness of breath being the most common sign and symptoms. For type II: Six cases were reported in English literature, all in pediatric age group with median calculated age at presentation is 6 years with neurologic manifestations and mental retardation are the most common type II associated symptoms. Due to lack of systematic epidemiological studies, congenital methemoglobinemia is under diagnosed as it is under investigated and usually overlooked especially when presenting in adulthood and in absence of obvious acquired agents.


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