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1.
Zhonghua Yi Xue Za Zhi ; 104(3): 218-221, 2024 Jan 16.
Artigo em Chinês | MEDLINE | ID: mdl-38220448

RESUMO

The clinical characteristics, auxiliary examinations, skin and neuropathological features of 7 patients who had reticular cyanosis with peripheral neuropathy from the Department of Neurology, Huashan Hospital, Fudan University from January 2019 to December 2022 were retrospectively analyzed. Among the 7 patients, 5 were female and 2 were male.The age of onset of peripheral neuropathy was (39.8±21.3) years and the disease duration of peripheral neuropathy was (2.7±2.3) years. Three patients had acute onset and 4 patients had chronic onset. All the patients had limb numbness, with limb weakness in 6 patients and pain in 5 cases. Neuroelectrophysiological examination revealed 1 case of mononeuropathy, 2 cases of polyneuropathy, 2 cases of peripheral neuropathy, and 2 cases of sensory neuron neuropathy. Skin biopsy was performed in 3 patients, which presented hyperplasia and expansion of blood vessels in the dermis with lymphocyte infiltration. Nerve biopsy was performed in 3 patients, indicating axonal damage. Reticular cyanosis with peripheral neuropathy characterizes with numbness and weakness of limbs, most of which were accompanied by pain. Electrophysiological changes are in various forms. The pathological changes are dominated by the damage of axonal.


Assuntos
Livedo Reticular , Doenças do Sistema Nervoso Periférico , Adolescente , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Adulto Jovem , Cianose/complicações , Hipestesia/complicações , Livedo Reticular/complicações , Dor , Estudos Retrospectivos
2.
Cardiol Young ; 34(1): 145-150, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37254574

RESUMO

OBJECTIVE: It is often assumed, that adult patients with CHD (ACHD) have impairments regarding their cognitive function (CF) and health-related quality of life. In particular, it seems reasonable to assume that cyanosis may have a potential impact on CF as well as surgical or drug treatment into adulthood. This study assesses neuromental health aspects such as CF and health-related quality of life in ACHD patients. METHODS: Seventy-eight ACHD patients (female n = 39 (50%); 34.1 ± 12.9 years; cyanotic CHD n = 49 (62.8%) with a cyanosis duration of 159.8 ± 196.2 month) who underwent open heart surgery as first intervention were asked to participate during routinely follow-up in 2018. Wechsler Intelligence Scale IV was used for CF and the Short Form 36 Health Survey to assess health-related quality of life. RESULTS: Intelligence quotient measures showed significant differences comparing never cyanotic and with a cyanotic phase in verbal comprehension (p = 0.013). There was no association of CF with cyanosis duration, number of surgery or catheter, CHD severity, and time of first surgery. The group of early surgery showed significantly better results in physical function (p = 0.040) of health-related quality of life, and in comparison with their assigned reference, both groups showed significantly reduced results in all domains except in bodily pain and mental health. Full-Scale intelligence quotient correlates with physical function of health-related quality of life. CONCLUSIONS: The results show normal CF in ACHD. Health-related quality of life was weak in comparison with the reference. There is a need to improve the well-being of our ACHD with structured programmes, including physical activity programmes. This growing ACHD population should be focused in order of their needs, medical ones on one hand and on the other hand psychosocial matters.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Cardiopatias Congênitas , Adulto , Humanos , Feminino , Masculino , Qualidade de Vida/psicologia , Ponte Cardiopulmonar , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/cirurgia , Cianose/complicações
3.
Trop Doct ; 54(1): 39-41, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37807706

RESUMO

Acute diarrhoeal illness remains a common medical problem in children with nearly 1.7 billion cases globally every year. We report five infants who, following severe diarrhoea, developed methaemoglobinemia. This is an altered state of haemoglobin presenting with cyanosis and can pose a diagnostic dilemma. It should be suspected in young infants without cyanotic heart disease presenting with severe diarrhoea, sepsis and cyanosis disproportionate to their clinical status. Its outcome depends on prompt treatment, the severity of underlying sepsis and co-morbidity.


Assuntos
Metemoglobinemia , Sepse , Lactente , Criança , Humanos , Metemoglobinemia/complicações , Metemoglobinemia/diagnóstico , Metemoglobinemia/terapia , Diarreia/diagnóstico , Diarreia/etiologia , Cianose/etiologia , Cianose/complicações , Sepse/complicações
4.
Zhonghua Er Ke Za Zhi ; 62(1): 71-75, 2024 Jan 02.
Artigo em Chinês | MEDLINE | ID: mdl-38154981

RESUMO

Objective: To summarize the clinical features and prognosis of Budd-Chiari syndrome with hepatopulmonary syndrome (HPS) in children. Methods: The clinical data of a child who had Budd-Chiari syndrome with HPS treated at the Department of Pediatrics of the First Affiliated Hospital of Zhengzhou University in December 2016 was analyzed retrospectively. Taking "Budd-Chiari syndrome" and "hepatopulmonary syndrome" in Chinese or English as the keywords, literature was searched at CNKI, Wanfang, China Biomedical Literature Database and PubMed up to July 2023. Combined with this case, the clinical characteristics, diagnosis, treatment and prognosis of Budd-Chiari syndrome with HPS in children under the age of 18 were summarized. Results: A 13-year-old boy, presented with cyanosis and chest tightness after activities for 6 months, and yellow staining of the skin for 1 week. Physical examination at admission not only found mild yellow staining of the skin and sclera, but also found cyanosis of the lips, periocular skin, and extremities. Laboratory examination showed abnormal liver function with total bilirubin 53 µmol/L, direct bilirubin 14 µmol/L, and indirect bilirubin 39 µmol/L, and abnormal blood gas analysis with the partial pressure of oxygen of 54 mmHg (1 mmHg=0.133 kPa), the partial pressure of carbon dioxide of 31 mmHg, and the alveolar-arterial oxygen gradient of 57 mmHg. Hepatic vein-type Budd-Chiari syndrome, cirrhosis, and portal hypertension were indicated by abdominal CT venography. Contrast-enhanced transthoracic echocardiography (CE-TTE) was positive. After symptomatic and supportive treatment, this patient was discharged and received oxygen therapy outside the hospital. At follow-up until March 2023, there was no significant improvement in hypoxemia, accompanied by limited daily activities. Based on the literature, there were 3 reports in English while none in Chinese, 3 cases were reported. Among a total of 4 children, the chief complaints were dyspnea, cyanosis, or hypoxemia in 3 cases, and unknown in 1 case. There were 2 cases diagnosed with Budd-Chiari syndrome with HPS at the same time due to respiratory symptoms, and 2 cases developed HPS 1.5 years and 8.0 years after the diagnosis of Budd-Chiari syndrome respectively. CE-TTE was positive in 2 cases and pulmonary perfusion imaging was positive in 2 cases. Liver transplantation was performed in 2 cases and their respiratory function recovered well; 1 case received oxygen therapy, with no improvement in hypoxemia; 1 case was waiting for liver transplantation. Conclusions: The onset of Budd-Chiari syndrome with HPS is insidious. The most common clinical manifestations are dyspnea and cyanosis. It can reduce misdiagnosis to confirm intrapulmonary vascular dilatations with CE-TTE at an early stage. Liver transplantation is helpful in improving the prognosis.


Assuntos
Síndrome de Budd-Chiari , Síndrome Hepatopulmonar , Masculino , Humanos , Criança , Adolescente , Síndrome de Budd-Chiari/complicações , Síndrome de Budd-Chiari/diagnóstico , Síndrome de Budd-Chiari/terapia , Síndrome Hepatopulmonar/complicações , Síndrome Hepatopulmonar/diagnóstico , Síndrome Hepatopulmonar/terapia , Estudos Retrospectivos , Hipóxia/complicações , Oxigênio , Dispneia/complicações , Cianose/complicações , Bilirrubina
5.
Rinsho Shinkeigaku ; 63(11): 743-747, 2023 Nov 23.
Artigo em Japonês | MEDLINE | ID: mdl-37880116

RESUMO

The patient is a 44-year-old man. His parents are consanguineous. He experienced muscle weakness in his toe and distal tingling sensation in his feet at 42 years of age, which gradually progressed. Additionally, a marked cyanotic discoloration of the feet appeared and worsened progressively. Neurological examination revealed loss of tendon reflexes and distal muscle weakness in the lower extremities. Findings from nerve conduction studies indicated axonal polyneuropathy. Upon detection of the MME gene mutation, the patient was diagnosed with autosomal-recessive Charcot-Marie-Tooth disease 2T (ARCMT2T). In this case, cyanosis of the lower extremities possibly was associated with ARCMT2T, and it was suggested to be due to neprilysin deletion linked with the MME mutation. This represents the first documented occurrence of cyanosis as a distinctive feature of CMT with MME mutation.


Assuntos
Doença de Charcot-Marie-Tooth , Masculino , Humanos , Adulto , Doença de Charcot-Marie-Tooth/diagnóstico , Doença de Charcot-Marie-Tooth/genética , Doença de Charcot-Marie-Tooth/complicações , Neprilisina/genética , Mutação , Debilidade Muscular/complicações , Linhagem , Fenótipo , Cianose/complicações
6.
Blood Purif ; 52(9-10): 835-843, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37640010

RESUMO

INTRODUCTION: Hemodialysis uses municipal water that must be strictly purified and sterilized to be used for that procedure. Large amounts of decontaminants are often used, such as chlorine, and if these compounds are not subsequently removed they can be transferred to the blood of patients causing complications including methemoglobinemia. METHODS: In this case series study, dialysis patients in one unit were evaluated. We reviewed clinical characteristics and laboratory findings obtained on the day when the water supply was disinfected with chlorine, with the aim to quantify methemoglobin concentrations. Our objective was to characterize the clinical presentation and management of patients who presented with methemoglobinemia on a specific index day. We also reviewed reported cases in the literature regarding this underreported complication. RESULTS: Eight patients who presented with chlorine intoxication were evaluated. The methemoglobin concentrations were between 1.3% and 7.9% (reference value 0-1%). We believe this to be caused by water containing 0.78 mg/L of total chlorine. Seven patients presented with cyanosis, 4 with dizziness, 6 with dark brown blood, 4 with dyspnea, and 4 with headache and hemolytic anemia. Subjects were treated with supplemental oxygen, methylene blue, intravenous vitamin C, blood transfusions, and increased doses of erythropoietin. No patient died, and all continued with their usual hemodialysis sessions. CONCLUSION: Acute chlorine intoxication transferred by the water used during hemodialysis sessions can present with methemoglobinemia accompanied by cyanosis, oxygen desaturation, and hemolytic anemia. Chlorine levels should be carefully monitored in the water used for hemodialysis treatment.


Assuntos
Anemia Hemolítica , Metemoglobinemia , Humanos , Metemoglobinemia/induzido quimicamente , Metemoglobinemia/terapia , Metemoglobina/uso terapêutico , Cloro/toxicidade , Diálise Renal/efeitos adversos , Cianose/complicações , Cloretos , Anemia Hemolítica/complicações , Oxigênio , Água
7.
Am Surg ; 89(9): 3959-3961, 2023 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-37303171

RESUMO

Acquired methemoglobinemia is a potentially lethal medical condition caused by exposure to oxidizing xenobiotics, including antibiotics such as dapsone and inhaled anesthetics such as benzocaine. In this case report, we describe two presentations of acquired methemoglobinemia which presented to our surgical intensive care unit within one month. This highlights the potential connection between an emergent surgery or procedure and the development of methemoglobinemia in an environment where it is presumed that this condition would be extremely rare. High clinical suspicion for methemoglobinemia is warranted if the patient develops cyanosis or a decreased oxygen saturation unresponsive to supplemental oxygen when another etiology is not identifiable. If methemoglobinemia is suspected, a direct measurement of blood methemoglobin levels can be obtained to confirm the diagnosis. Prompt treatment with intravenous methylene blue is highly effective.


Assuntos
Metemoglobinemia , Humanos , Metemoglobinemia/induzido quimicamente , Metemoglobinemia/diagnóstico , Azul de Metileno/uso terapêutico , Benzocaína/efeitos adversos , Cianose/complicações , Anestésicos Locais/efeitos adversos , Cuidados Críticos
8.
Ital J Dermatol Venerol ; 158(2): 117-123, 2023 04.
Artigo em Inglês | MEDLINE | ID: mdl-37153946

RESUMO

BACKGROUND: Since the COVID-19 pandemic started, great interest has been given to this disease, especially to its possible clinical presentations. Besides classical respiratory symptoms, dermatological manifestations occur quite often among infected and non-infected patients, particularly in children. A prominent IFN-I response, that is generally higher in children compared to adults, may not only cause chilblain lesions, but it could also prevent infection and viral replication, thus justifying the negative swab results, as well as the absence of relevant systemic symptoms in positive cases. Indeed, reports have emerged describing chilblain-like acral lesions in children and adolescents with either proven or suspected infection. METHODS: Patients aged from 1 to 18 years old were enrolled in this study from 23 Italian dermatological units and were observed for an overall period of 6 months. Clinical pictures were collected along with data on the location and duration of skin lesions, their association with concomitant local and systemic symptoms, presence of nail and/or mucosal involvement, as well as histological, laboratory and imaging findings. RESULTS: One hundred thirty-seven patients were included, of whom 56.9% were females. Mean age was 11.97±3.66 years. The most commonly affected sites were the feet (77 patients, 56.2%). Lesions (48.5%) featured cyanosis, chilblains, blisters, ecchymosis, bullae, erythema, edema, and papules. Concomitant skin manifestations included maculo-papular rashes (30%), unspecified rashes (25%), vesicular rashes (20%), erythema multiforme (10%), urticaria (10%) and erythema with desquamation (5%). Forty-one patients (29.9%) reported pruritus as the main symptom associated with chilblains, and 56 out of 137 patients also reported systemic symptoms such as respiratory symptoms (33.9%), fever (28%), intestinal (27%), headache (5.5%), asthenia (3.5%), and joint pain (2%). Associated comorbid conditions were observed in 9 patients presenting with skin lesions. Nasopharyngeal swabs turned out positive in 11 patients (8%), whereas the remainder were either negative (101, 73%) or unspecified (25, 18%). CONCLUSIONS: COVID-19 has been credited as the etiology of the recent increase in acro-ischemic lesions. The present study provides a description of pediatric cutaneous manifestations deemed to be potentially associated with COVID-19, revealing a possible association between acral cyanosis and nasopharyngeal swab positivity in children and teenagers. The identification and characterization of newly recognized patterns of skin involvement may aid physicians in diagnosing cases of asymptomatic or pauci-symptomatic COVID patients.


Assuntos
COVID-19 , Pérnio , Exantema , Adulto , Feminino , Humanos , Adolescente , Criança , Lactente , Pré-Escolar , Masculino , COVID-19/complicações , COVID-19/diagnóstico , COVID-19/epidemiologia , Pérnio/diagnóstico , Pérnio/etiologia , Pérnio/epidemiologia , Estudos Retrospectivos , Pandemias , SARS-CoV-2 , Eritema/complicações , Exantema/complicações , Itália/epidemiologia , Vesícula/complicações , Cianose/complicações
9.
Cardiol Young ; 33(9): 1713-1714, 2023 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-36896981

RESUMO

A significant contributing factor to the progression of late cyanosis in individuals undergoing Kawashima operation is pulmonary arteriovenous malformations. Following the Fontan procedure, arteriovenous malformations may regress. However, in cases with extensive malformations causing severe cyanosis, lobectomy can also be a possible treatment approach. Thereby, we present our two-step treatment strategy in a late Fontan completion complicated by arteriovenous malformations in a Kawashima patient.


Assuntos
Fístula Arteriovenosa , Malformações Arteriovenosas , Técnica de Fontan , Humanos , Criança , Malformações Arteriovenosas/diagnóstico , Malformações Arteriovenosas/cirurgia , Técnica de Fontan/efeitos adversos , Fístula Arteriovenosa/diagnóstico , Fístula Arteriovenosa/cirurgia , Fístula Arteriovenosa/complicações , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgia , Artéria Pulmonar/anormalidades , Cianose/complicações
10.
Cardiol Young ; 33(2): 306-308, 2023 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-36648193

RESUMO

Right-to-left shunt in atrial septal defect without pulmonary hypertension is a rare condition and can present with complications such as cyanosis. This is a rare case of cyanosis caused by right-to-left shunt atrial septal defect related to prominent crista terminalis.


Assuntos
Comunicação Interatrial , Hipertensão Pulmonar , Dispositivo para Oclusão Septal , Humanos , Dispositivo para Oclusão Septal/efeitos adversos , Comunicação Interatrial/complicações , Comunicação Interatrial/diagnóstico , Átrios do Coração , Hipertensão Pulmonar/complicações , Cianose/complicações , Cateterismo Cardíaco/efeitos adversos
11.
Am Heart J ; 259: 9-20, 2023 05.
Artigo em Inglês | MEDLINE | ID: mdl-36681172

RESUMO

BACKGROUND: Patients with congenital heart disease (CHD) are at lifelong high risk of infective endocarditis (IE). The risk of IE presumably differs among different CHD, but little knowledge exists on the area. METHODS: In this observational cohort study, all CHD-patients born in 1977 to 2018 were identified using Danish nationwide registries and followed from the date of birth until first-time IE, emigration, death, or end of study (December 31, 2018). The comparative risk of IE among CHD-patients vs age- and sex-matched controls from the background population was assessed. The risk of IE was stratified according to the type of CHD and factors associated with IE including sex and relevant time-varying coefficients (ie, cyanosis, cardiac prostheses, diabetes mellitus, chronic kidney disease, and cardiac implantable electronic devices) were examined using Cox-regression analysis. RESULTS: A total of 23,464 CHD-patients (50.0% men) were identified and matched with 93,856 controls. During a median follow-up of 17.7 years, 217(0.9%) CHD-patients and 4(0.0%) controls developed IE, corresponding to incidence rates of 5.2(95%CI 4.6-6.0) and 0.02(95%CI 0.01-0.1) per 10,000 person-years, respectively. The incidence of IE was greatest among patients with tetralogy of fallot, malformations of the heart chambers (including transposition of the great arteries, univentricular heart, and truncus arteriosus), atrioventricular septal defects, and heart valve defects. Factors associated with IE among CHD-patients included male sex, cyanosis, cardiac prostheses, chronic kidney disease, and cardiac implantable electronic devices. CONCLUSIONS: CHD-patients have a substantially higher associated incidence of IE than the background population. With the increasing longevity of these patients, relevant guidelines concerning preventive measures are important.


Assuntos
Endocardite Bacteriana , Endocardite , Cardiopatias Congênitas , Transposição dos Grandes Vasos , Humanos , Masculino , Pessoa de Meia-Idade , Feminino , Incidência , Transposição dos Grandes Vasos/complicações , Fatores de Risco , Endocardite/complicações , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/epidemiologia , Cianose/complicações
12.
J Anesth ; 37(1): 32-38, 2023 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-36309903

RESUMO

PURPOSE: Congenital heart disease (CHD) is divided into two groups according to cyanosis status. Cyanotic CHD has a low level of systemic oxygenation and is accompanied by increased erythropoiesis. We hypothesized that pediatric patients with CHD would exhibit different thromboelastographic profiles according to their cyanosis status. METHODS: The study recruited 70 pediatric patients younger than 12 months who were undergoing surgery for CHD. Patients were allocated to the acyanotic group or cyanotic group after preoperative evaluations of their diagnosis and peripheral oxygen saturation in the operating room on room air. After inducing anesthesia, blood samples were collected. Hematologic and thromboelastographic profiles were evaluated. RESULTS: Demographic data were similar between groups. The thromboelastographic profiles did not differ significantly between the groups. Hematologic profiles generally did not significantly differ between groups, except hematocrit (Hct) was higher in the cyanotic group (41.7 ± 6.8% vs. 35.3 ± 5.3%, p < 0.001). In patients under 3 months of age, prothrombin time (PT) (cyanotic group 15.4 ± 1.1 s vs. acyanotic group 14.2 ± 2.4 s, p = 0.02) and international normalized ratio (INR) (cyanotic group 1.24 ± 0.12 vs. acyanotic group 1.12 ± 0.27, p = 0.01) were significantly greater in the cyanotic group. CONCLUSION: There were no differences in thromboelastographic profiles between the patients with or without cyanosis, regardless of age. The Hct was higher in the cyanotic group in patients under 12 months, while the PT was prolonged and the INR was increased in the cyanotic group in patients under 3 months.


Assuntos
Cardiopatias Congênitas , Humanos , Criança , Cardiopatias Congênitas/cirurgia , Cianose/complicações , Cianose/cirurgia , Tromboelastografia , Testes de Coagulação Sanguínea , Hipóxia/complicações
13.
Braz J Cardiovasc Surg ; 38(2): 300-304, 2023 04 23.
Artigo em Inglês | MEDLINE | ID: mdl-36259992

RESUMO

CLINICAL DATA: Infant, nine months of age, female, diagnosed with congenital heart disease, with signs of heart failure associated with cyanosis and difficulty in gaining weight. CHEST RADIOGRAPHY: Cardiomegaly with prevalence of pulmonary vascular network. ELECTROCARDIOGRAM: Ectopic atrial rhythm with right ventricular overload and left anterosuperior divisional block. ECHOCARDIOGRAM: Single atrium with absent interatrial septum, atrioventricular connection with a single valve and two orifices, with increased pulmonary pressure and high Qp/Qs. COMPUTED TOMOGRAPHY: Absence of portal vein and intrahepatic segment of the inferior vena cava. Infrahepatic portion continuing with the azygos system at the level of the thoracic cavity, presence of mesenteric-caval communication associated with signs suggestive of hepatic peribiliary fibrosis. DIAGNOSIS: Abernethy malformation is a rare condition and represents an extrahepatic portosystemic shunt that develops between the mesenteric-portal vasculature and the systemic veins. It may be associated with cardiac malformations and advance with pulmonary hypertension and even the need for liver transplantation. Persistent cyanosis after corrective surgery led to a deeper investigation and correct diagnosis of this malformation. OPERATION: Sternotomy with 68 minutes of cardiopulmonary bypass and nine minutes of total circulatory arrest. In the postoperative period, persistence of cyanosis was evident, even though there were no immediate complications. Patient was discharged on the 10th postoperative day. An abdominal computed tomography angiography confirmed the diagnosis of Abernethy type I malformation, and the patient was transferred for liver transplantation after congenital heart disease treatment.


Assuntos
Cardiopatias Congênitas , Malformações Vasculares , Lactente , Humanos , Feminino , Veia Porta/diagnóstico por imagem , Veia Porta/anormalidades , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Veia Cava Inferior/diagnóstico por imagem , Cianose/complicações , Angiografia por Tomografia Computadorizada , Malformações Vasculares/diagnóstico , Malformações Vasculares/diagnóstico por imagem
14.
Turk J Pediatr ; 64(5): 932-934, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36305445

RESUMO

BACKGROUND: Anomalies in systemic venous return most commonly involve a persistent left supe-rior vena cava draining into the left atrium. Anomalous drainage of the inferior vena cava (IVC) into the left atrium is a rare congenital vascular disorder. The diagnosis was confirmed as anoma-lous drainage of the right superior pulmonary vein and large atrial septal defect following echo-cardiography. Anomalous drainage of the inferior vena cave was confirmed with computed tomog-raphy (CT). We report a rare combination of drainage of the inferior vena cava associated with atrial septal defect (ASD) and partial anomalous pulmonary venous return. CASE: A 14-year-old girl was referred to our hospital for the evaluation of palpitations, hypoxia, exertional dyspnea, and cyanosis. Transthoracic echocardiography (TTE) revealed a large sinus venosus ASD and anomalous right superior pulmonary venous return. A cardiac CT demonstrated IVC drainage to the left atrium and an anomalous right superior pulmonary vein draining into the right atrium. CONCLUSIONS: In older patients with cyanosis, further imaging methods together with TTE will be useful in detecting additional cardiac anomalies. Patients with inferior vena cava opening to the left atrium are different from caval type ASD`s and should be surgically repaired using a patch. Corrective surgery involves repositioning of the interatrial septum via a patch.


Assuntos
Comunicação Interatrial , Veias Pulmonares , Veia Cava Inferior , Adolescente , Feminino , Humanos , Cianose/complicações , Átrios do Coração/diagnóstico por imagem , Átrios do Coração/anormalidades , Comunicação Interatrial/diagnóstico por imagem , Comunicação Interatrial/cirurgia , Comunicação Interatrial/complicações , Veias Pulmonares/diagnóstico por imagem , Veias Pulmonares/cirurgia , Veias Pulmonares/anormalidades , Veia Cava Inferior/diagnóstico por imagem , Veia Cava Inferior/cirurgia , Veia Cava Inferior/anormalidades , Veia Cava Superior/anormalidades , Veia Cava Superior/cirurgia
15.
Rev. med. Chile ; 150(10): 1401-1406, oct. 2022. ilus, tab
Artigo em Espanhol | LILACS | ID: biblio-1431842

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
Humanos , Feminino , Sulfemoglobinemia/complicações , Metemoglobinemia/diagnóstico , Metemoglobinemia/induzido quimicamente , Oxigênio , Oximetria/efeitos adversos , Cianose/complicações
16.
Rev Med Liege ; 77(7-8): 435-439, 2022 Jul.
Artigo em Francês | MEDLINE | ID: mdl-35924498

RESUMO

We report the case of a 29-year-old man admitted to the emergency department for dyspnea and changes in mental status during a festivity. Clinically the patient presented a central cyanosis refractory to the administration of high concentration of oxygen. The consumption of poppers is increasingly used by young people for recreational purposes because they are inexpensive and easy to acquire. Methemoglobinemia is a potentially serious and little known complication of popper intoxication. This condition, known as «methemoglobinemia¼, was suspected by the emergency physician and confirmed through non-invasive measurement of methemoglobinemia in arterial blood gases. The early recognition of methemoglobinemia and prompt treatment allowed a favourable evolution of our patient avoiding the development of multi-systemic organ failure or even death.


Nous rapportons le cas d'un homme de 29 ans admis pour dyspnée et altération de l'état de conscience survenue dans le décours d'une soirée festive. Le tableau clinique est marqué par une cyanose centrale réfractaire à l'administration d'oxygène au masque à haute concentration. La consommation de poppers à usage récréatif est de plus en plus fréquente chez les jeunes adultes. C'est une substance peu coûteuse et facile d'accès, consommée, notamment, pour ses propriétés euphorisantes. La méthémoglobinémie est une complication potentiellement grave et peu connue de l'intoxication par poppers. Dans le cas présenté, la méthémoglobinémie, suspectée par le médecin urgentiste, a pu être confirmée rapidement par une mesure non invasive à la gazométrie artérielle. La reconnaissance précoce de la méthémoglobinémie et l'initiation d'un traitement efficace ont permis une évolution rapidement favorable et d'éviter une défaillance multi-systémique pouvant conduire au décès du patient.


Assuntos
Cianose/etiologia , Metemoglobinemia/diagnóstico , Adulto , Gasometria , Cianose/complicações , Diagnóstico Diferencial , Dispneia/etiologia , Serviços Médicos de Emergência , Humanos , Hipóxia , Drogas Ilícitas/efeitos adversos , Masculino , Metemoglobinemia/induzido quimicamente , Metemoglobinemia/complicações , Metemoglobinemia/terapia
17.
Inn Med (Heidelb) ; 63(6): 591-600, 2022 Jun.
Artigo em Alemão | MEDLINE | ID: mdl-35925129

RESUMO

Vascular acrosyndromes are characterized by sparse, uniform clinical manifestations and a variety of possible pathomechanisms. The present article focuses on the functional entities. Raynaud phenomenon is based on cold- or stress-induced vasospasms of acral arteries. It is defined by the color changes of the skin, in the typical case white-blue-red (tricolore). The long fingers are most commonly affected. The etiology is unknown, and the pathophysiology is poorly understood. A distinction is made between primary and a secondary Raynaud phenomenon. The most important underlying diseases include collagenosis, primarily systemic sclerosis, and malignancies; furthermore, medications and drugs may promote vasospasm. Treatment is aimed at preventing or breaking the vasospasm, but has been only partially effective in doing so. Acrocyanosis is a vasospastic dystonic acral disorder that results in permanent reddish-livid discoloration, especially of the hands and feet. Secondary forms occur in collagenosis, malignancies, and myelodysplastic syndromes. The etiology and pathophysiology are virtually unknown. Targeted pharmacological intervention is not possible. Unlike all other vascular acrosyndromes, erythromelalgia is characterized by hyperemia. The primary form is a genetic sodium channelopathy, while secondary forms include malignancies, connective tissue diseases, and myelodysplastic syndromes. The symptoms are often distressing and disabling. Therapy requires a multimodal approach that includes both nonpharmacological and pharmacological strategies. Close interdisciplinary collaboration is essential for the management of this disease.


Assuntos
Eritromelalgia , Síndromes Mielodisplásicas , Doença de Raynaud , Doenças Vasculares , Cianose/complicações , Eritromelalgia/complicações , Humanos , Síndromes Mielodisplásicas/complicações , Doença de Raynaud/diagnóstico , Doenças Vasculares/complicações
18.
PLoS One ; 17(7): e0263948, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35901057

RESUMO

BACKGROUND: Coagulation abnormality is a significant complication and cause of mortality in children with uncorrected congenital heart defects (CHD). The aim of this study was to determine the prevalence of coagulation abnormalities and the associated factors in children with uncorrected CHD. METHOD: A cross sectional study conducted to determine the prevalence of coagulation abnormalities among 70 children with uncorrected CHD aged six months to 17 years and 70 age and sex matched apparently healthy controls. Coagulation abnormalities was determined using complete blood count, prothrombin time, activated partial thromboplastin time and D-dimer assay. RESULTS: The prevalence of coagulation abnormalities among children with CHD and controls was 37.1% and 7.1% respectively. Children with Cyanotic CHD had a significantly higher prevalence of coagulation abnormalities compared to children with Acyanotic CHD (57.1% versus 17.1%). Haematocrit and oxygen saturation levels were significantly associated with coagulation abnormalities. CONCLUSION: This study affirms that coagulation abnormalities are frequent in children with uncorrected CHD. Oxygen saturation and haematocrit are risk factors of coagulation abnormalities. Routine coagulation screen is recommended especially in children with cyanotic congenital heart defects to improve their quality of life and reduce morbidity and mortality while awaiting definitive surgeries.


Assuntos
Transtornos da Coagulação Sanguínea , Cardiopatias Congênitas , Transtornos da Coagulação Sanguínea/complicações , Criança , Estudos Transversais , Cianose/complicações , Países em Desenvolvimento , Cardiopatias Congênitas/cirurgia , Hospitais de Ensino , Humanos , Lactente , Qualidade de Vida
19.
Medicine (Baltimore) ; 101(28): e29355, 2022 Jul 15.
Artigo em Inglês | MEDLINE | ID: mdl-35839015

RESUMO

RATIONALE: Sodium nitrite is a potent oxidizing agent that impairs oxygen transport and delivery through methemoglobin formation. Clinical manifestations are known to induce methemoglobinemia, dysrhythmia, hypotension, and even death. While accidental intoxication of sodium nitrite by contaminated water and food has previously occurred, there has been a substantial upsurge in suicide intoxication in recent years. PATIENT CONCERNS: We present case reports of 2 patients who attempted suicide by sodium nitrite after ordering a "suicide powder" on the internet market. They were brought to the emergency department after attempting suicide by ingesting sodium nitrite. They experienced dyspnea, cyanosis, and mild nausea. DIAGNOSIS: Based on their history and blood tests, methemoglobinemia was initially diagnosed. INTERVENTIONS AND OUTCOMES: The patients received methylene blue antidotal therapy in the emergency department. The patients were discharged after neuropsychiatric evaluation and treatment for mental illness, suicidal ideation, and suicide attempts. They informed us of how simple and easy it was for them to buy sodium nitrite for suicidal purposes. LESSONS: With widely shared information on the usage of sodium nitrite for suicide and the absence of proper regulation, the incidence of acute poisoning will increase. This increases physicians' chances of encountering unexplained cyanosis and methemoglobinemia. Clinical suspicion of sodium nitrite intoxication is warranted in cases of unexplained cyanosis or methemoglobinemia. We want to highlight how simple and easy it is to buy sodium nitrite for suicidal purposes.


Assuntos
Metemoglobinemia , Nitrito de Sódio , Cianose/complicações , Humanos , Internet , Metemoglobinemia/induzido quimicamente , Metemoglobinemia/diagnóstico , Metemoglobinemia/tratamento farmacológico , Azul de Metileno/uso terapêutico , Tentativa de Suicídio
20.
J UOEH ; 44(2): 185-190, 2022.
Artigo em Japonês | MEDLINE | ID: mdl-35660684

RESUMO

Toluidine is a known cause of bladder cancer, but it is less widely recognized as a cause of methemoglobinemia because methemoglobinemia is rare. We herein report a case of methemoglobinemia caused by toluidine in a 50-year-old man. A solution of toluidine overflowed from its container during transportation and adhered to the man's clothes, but he drove to his workplace 100 km away without changing his clothes or undergoing decontamination. Before arriving at his workplace, he developed dyspnea and called emergency services, and he was then transported to a local hospital. He had significant cyanosis upon arrival, and arterial blood gas analysis revealed a high methemoglobin level of 44%. He was diagnosed with toluidine-induced methemoglobinemia and was transported to our hospital, where he was admitted to the intensive care unit. Treatment for methemoglobinemia was started immediately after hospitalization, and the patient's symptoms and methemoglobin level improved. Methemoglobinemia should be considered in workers who handle toluidine and develop cyanosis and dyspnea.


Assuntos
Metemoglobinemia , Cianose/induzido quimicamente , Cianose/complicações , Dispneia/complicações , Humanos , Masculino , Metemoglobina/efeitos adversos , Metemoglobina/análise , Metemoglobinemia/induzido quimicamente , Metemoglobinemia/diagnóstico , Pessoa de Meia-Idade , Toluidinas/efeitos adversos
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