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1.
J Med Case Rep ; 18(1): 439, 2024 Sep 11.
Artículo en Inglés | MEDLINE | ID: mdl-39256769

RESUMEN

BACKGROUND: Potassium nitrate poisoning is a rare but potentially serious condition that can result in methemoglobinemia and subsequent cyanosis. This case report presents a unique instance of rapid-onset methemoglobinemia resulting from the ingestion of a traditional medicine preparation containing potassium nitrate, known as "kalmi shora." CASE PRESENTATION: A 14-year-old Pakistani boy reported to the emergency department with a history of sudden-onset headache, drowsiness agitation, irritability, and generalized cyanosis. Pulse oximetry showed a concerning oxygen saturation level of 58%, whereas arterial blood gas analysis revealed a normal partial pressure of oxygen (90 mmHg). The profile of abrupt onset of symptoms, generalized cyanosis, and the discrepancy between the partial pressure of oxygen and oxygen saturation readings necessitated a comprehensive assessment including inquiries into potential toxins. The peculiar appearance of the blood, resembling chocolate in color, further indicated the possibility of methemoglobinemia. The patient was successfully treated with methylene blue, leading to a prompt resolution of symptoms. CONCLUSION: This case highlights the significance of considering toxin exposures, such as traditional-medicine-induced poisoning, in emergency settings. The report contributes to the medical literature by highlighting the potential risks associated with traditional remedies and emphasizes the critical role of prompt diagnosis and intervention in optimizing patient outcomes. Recognition of the specific etiology of methemoglobinemia, in this case, traditional medicine ingestion, is essential for effective management in emergency medicine.


Asunto(s)
Metahemoglobinemia , Azul de Metileno , Humanos , Azul de Metileno/uso terapéutico , Metahemoglobinemia/inducido químicamente , Metahemoglobinemia/tratamiento farmacológico , Masculino , Adolescente , Nitratos/envenenamiento , Resultado del Tratamiento , Cianosis/inducido químicamente
2.
Cells ; 13(17)2024 Sep 05.
Artículo en Inglés | MEDLINE | ID: mdl-39273064

RESUMEN

Background and Aim: Patients with cyanosis secondary to congenital heart disease (CHD) are characterized by erythrocytosis and increased blood viscosity, which contribute to endothelial dysfunction, increased arterial stiffness, and impaired vascular function, which may affect the final clinical presentation. Asymmetric dimethylarginine (ADMA) and e-selectin (e-sel) are valuable biomarkers for endothelial and vascular dysfunction. Their concentration levels in blood serum have the potential to be an accessible tool that reflects the severity of the disease. We aimed to assess e-sel and ADMA levels and their relationship with the clinical status and endothelial and vascular function. Methods: A cross-sectional study, including 36 adult CHD cyanotic patients [(17 males) (42.3 ± 16.3 years)] with an arterial blood oxygen saturation less than 92% and 20 healthy controls [(10 males) (38.2 ± 8.5 years)], was performed. All the patients underwent a clinical examination, blood testing, and cardiopulmonary tests. Their endothelial function was assessed using the intima media thickness and flow-mediated dilatation. Vascular function, using applanation tonometry methods, was determined using the aortic systolic pressure, aortic pulse pressure, augmentation pressure, augmentation index, pulse pressure amplification, and pulse wave velocity. Results: The concentrations of e-sel and ADMA were significantly higher in the patients with CHD. The E-sel levels correlated positively with red blood cells, hemoglobin concentration, hematocrit, and augmentation pressure; they correlated negatively with blood oxygen saturation, the forced expiratory one-second volume, forced vital capacity, and oxygen uptake. The ADMA levels were found to correlate only with age. Conclusions: The E-sel level, unlike ADMA concentration, reflects the severity of erythrocytosis and hypoxia and, thus, the physical status of patients with cyanotic CHD.


Asunto(s)
Arginina , Cianosis , Selectina E , Cardiopatías Congénitas , Humanos , Masculino , Adulto , Femenino , Arginina/análogos & derivados , Arginina/sangre , Cardiopatías Congénitas/sangre , Cardiopatías Congénitas/fisiopatología , Cardiopatías Congénitas/complicaciones , Cianosis/sangre , Cianosis/fisiopatología , Selectina E/sangre , Estudios Transversales , Persona de Mediana Edad , Biomarcadores/sangre , Endotelio Vascular/fisiopatología , Endotelio Vascular/metabolismo , Estudios de Casos y Controles
3.
Bol Med Hosp Infant Mex ; 81(4): 250-252, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-39236666

RESUMEN

BACKGROUND: Cor triatriatum dexter (CTD) is an extremely rare pathology, with an incidence of < 0.4%. Its main characteristic is a partitioning of the right atrium by the persistence of the embryonic valve of the right sinus venosus. CLINICAL CASE: In this report, we describe the case of a 7-day-old newborn who presented with persistent cyanosis associated with feeding and crying. The diagnosis of CTD was made after an echocardiogram and confirmed using cardiac magnetic resonance imaging. The patient underwent successful surgery on day 14 with a favorable outcome and without complications. CONCLUSION: The importance of our case lies in the identification of rare heart disease as a cause of cyanosis and desaturation in a neonatal patient in the first days of life who did not present signs of heart failure and whose condition improved with supplemental oxygen. We also demonstrate that early diagnosis with echocardiography and surgical resolution resulted in clear clinical improvement and avoided future complications.


INTRODUCCIÓN: El cor triatriatum dexter es una cardiopatía muy rara, caracterizada por la división parcial del atrio derecho en dos cavidades por la persistencia de una membrana que embriológicamente representa la valva derecha del seno venoso. CASO CLÍNICO: En este reporte de caso, presentamos el caso de un neonato en su día 7 de vida que acude a valoración por presentar desaturación persistente con cianosis al llanto. El diagnóstico se realizó con ecocardiograma posterior al cual se decidió la resección quirúrgica de la membrana, procedimiento que fue llevado a cabo el día 14 de vida con éxito sin complicaciones. CONCLUSIONES: La importancia de este caso clínico radica en la identificación de una cardiopatía rara como causa de cianosis y desaturación en un paciente en etapa neonatal, el cual no presentaba datos de compromiso hemodinámico. También se muestra como un diagnóstico y tratamiento quirúrgico oportuno permitieron una resolución de los síntomas sin complicaciones futuras.


Asunto(s)
Corazón Triatrial , Cianosis , Ecocardiografía , Imagen por Resonancia Magnética , Humanos , Corazón Triatrial/diagnóstico , Corazón Triatrial/cirugía , Corazón Triatrial/complicaciones , Cianosis/etiología , Recién Nacido , Masculino , Femenino
4.
Chest ; 166(2): e29-e33, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-39122304

RESUMEN

CASE PRESENTATION: A 36-year-old woman with a medical history of opioid use disorder and frequent urinary tract infections presented to the ED from her opioid use disorder clinic, where she was found to have an oxygen saturation by pulse oximetry (Spo2) of 82% on room air. Starting 3 days before presentation, the patient's family noted worsening pale complexion and blue lips at rest. These findings of cyanosis had occurred a few times before and always resolved within a couple days without any medical intervention. She had no pulmonary symptoms outside of long-standing dyspnea with moderate exertion when at work or doing chores around the house. Her medications included methadone 160 mg daily, acetaminophen 650 mg nightly as needed, and phenazopyridine 199 mg three times daily as needed for increased urinary frequency and urethral discomfort that lasted a maximum of 4 days at a time. She confirmed she had started taking a new course of phenazopyridine 4 days before presenting to the ED. She had no dietary restrictions, had been eating her normal diet, and lived in a mobile home with her family, two dogs, and a gerbil. The patient reported using less than 10 tobacco cigarettes per day, one marijuana cigarette nightly, and no alcohol or other drugs. She worked in a warehouse stacking prepackaged bread.


Asunto(s)
Cianosis , Humanos , Femenino , Adulto , Cianosis/etiología , Cianosis/diagnóstico , Diagnóstico Diferencial
5.
BMC Pediatr ; 24(1): 541, 2024 Aug 23.
Artículo en Inglés | MEDLINE | ID: mdl-39174917

RESUMEN

BACKGROUND: In light of prolonged hypoxia, children with cyanotic heart disase (CHD) are at a high risk of developing iron deficiency iron deficiency (ID) and iron deficiency anemia (IDA). Reticulocyte hemoglobin equivalent (Ret-He) is a novel and dependable indicator for assessing iron status. However, there has been no previous study regarding cut-off value in pediatric CHD group. The purpose of this study is to assess the role of Ret-He and to establish cut-off points in the diagnosis of iron deficiency and IDA in pediatric cyanotic heart disease. METHOD: This study was conducted in two tertiary hospitals in Jakarta, Indonesia. 59 children with CHD, aged 3 months to 18 years, were enrolled consecutively. To determine iron status, hematological parameters (hemoglobin, hematocrit, mean corpuscular volume, mean corpuscular hemoglobin) and biochemical parameters for iron status (serum ferritin, transferrin saturation) were analysed and compared to Ret-He levels. The receiver operating characteristic (ROC) analysis was performed for the Ret-He cut-off points for ID and IDA. Sensitivity, specificity, positive and negative predictive value were calculated for each cut-off point. RESULT: Normal iron status was identified in 27 (45.8%) subjects, ID in 8 (13.5%) subjects, and IDA 24 (40.7%) subjects. The ID cut-off value for Ret-He is 28.8 pg (sensitivity 75%, specificity 85.2%, PPV 60%, NPV 92%, and AUC 0.828) and the Ret-He cut-off point for IDA is 28.15 pg (sensitivity 75%, specificity 88.9%, PPV 85.7%, NPV 80%, and AUC 0.824). Hemoglobin should be used in conjunction with Ret-He. ID might be detected in this cohort with Ret-He 28.8 pg and hemoglobin > 16,5 g/dL. While Ret-He 28.15 pg or Ret-He 28.15-28.8 pg with hemoglobin 16.5 g/dL could be used to diagnose IDA. CONCLUSION: The reticulocyte hemolgobin equivalent could be utilised as an iron status parameter in pediatric CHD, with a cut-off value of 28.8 pg for ID and 28.15 pg for IDA.


Asunto(s)
Anemia Ferropénica , Cardiopatías Congénitas , Hemoglobinas , Deficiencias de Hierro , Reticulocitos , Humanos , Anemia Ferropénica/diagnóstico , Anemia Ferropénica/sangre , Anemia Ferropénica/etiología , Preescolar , Masculino , Indonesia , Femenino , Lactante , Niño , Hemoglobinas/análisis , Reticulocitos/metabolismo , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/sangre , Cardiopatías Congénitas/diagnóstico , Adolescente , Cianosis/sangre , Cianosis/etiología , Cianosis/diagnóstico , Curva ROC , Sensibilidad y Especificidad , Biomarcadores/sangre , Hierro/sangre , Ferritinas/sangre
6.
BMJ Case Rep ; 17(7)2024 Jul 24.
Artículo en Inglés | MEDLINE | ID: mdl-39053919

RESUMEN

A pregnant female in her early 30s presented with cyanosis and oxygen saturation of 78%. She ingested isopropyl nitrate mistaking it for cannabidiol. Her arterial blood gas showed a methaemoglobin of >30% (outside the measuring range). She was treated with 120 mg of methylthioninium chloride (2 mg/kg) and symptoms improved. Her pregnancy progressed but was induced at 36 weeks because her child was small for gestational age. Methaemoglobinaemia is a rare presentation in pregnancy. There have been no reported cases of isopropyl nitrate-induced methaemoglobinaemia in pregnancy. Historically, intra-amniotic methylthioninium chloride was used in amniocentesis but use stopped after links to fetal malformations and neonatal death were made. There is no evidence outlining the risks of isopropyl nitrate in pregnancy and limited data on fetal effects from maternal exposure to intravenous methylthioninium chloride. This case adds to the evidence that treating methaemoglobinaemia may outweigh the risks of maternal exposure to methylthioninium chloride.


Asunto(s)
Metahemoglobinemia , Humanos , Femenino , Embarazo , Metahemoglobinemia/inducido químicamente , Metahemoglobinemia/tratamiento farmacológico , Metahemoglobinemia/diagnóstico , Adulto , Nitratos , Azul de Metileno/uso terapéutico , Azul de Metileno/administración & dosificación , Cianosis/inducido químicamente , Cianosis/tratamiento farmacológico
8.
Neoreviews ; 25(7): e443-e446, 2024 Jul 01.
Artículo en Inglés | MEDLINE | ID: mdl-38945964
11.
Pan Afr Med J ; 47: 92, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38799189

RESUMEN

Indoxacarb, a large-spectrum non-organophosphorus oxadiazine insecticide, is broadly used in farming whose mechanism of action is the blockage of voltage-gated sodium channels of insects. There is restricted data on human poisoning. We report a case of an 18-year-old male patient without comorbidities presented with unconsciousness and cyanosis after the intentional ingestion of indoxacarb in a suicide attempt. Methemoglobinemia was clinically suspected and was successfully treated after methylene blue injection, associated with supportive and symptomatic management. This case emphasizes the importance of considering methemoglobinemia after indoxacarb ingestion in addition to its early recognition and timely injection of methylene blue which led to complete recovery without sequelae.


Asunto(s)
Insecticidas , Metahemoglobinemia , Azul de Metileno , Oxazinas , Intento de Suicidio , Humanos , Metahemoglobinemia/inducido químicamente , Metahemoglobinemia/diagnóstico , Masculino , Azul de Metileno/administración & dosificación , Adolescente , Insecticidas/envenenamiento , Oxazinas/envenenamiento , Oxazinas/administración & dosificación , Cianosis/inducido químicamente
12.
Pediatr Pulmonol ; 59(6): 1757-1764, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38695627

RESUMEN

BACKGROUND: Tracheal agenesis, or tracheal atresia, is a rare congenital anomaly. The presence of a tracheoesophageal fistula (TEF) can help with breathing for newborns with tracheal agenesis. In this article, we presented three unique cases and outcomes of neonates with tracheal agenesis along with a review of the literature. METHODS: This study consisted of a single center case series followed by a review of literature. Case reports were generated using both written and electronic medical records from a single hospital. We summarized three unique cases and outcomes of neonates with tracheal agenesis and performed a review of the literature. RESULTS: We identified three cases of tracheal agenesis presented with severe cyanosis without spontaneous crying upon birth. Experienced pediatricians attempted to intubate the babies but were unsuccessful. Endotracheal tubes were subsequently either accidentally or purposely placed into the esophagus, and oxygen saturation levels improved. This suggested tracheal agenesis with TEF. Two cases underwent surgical intervention after resuscitation with esophageal intubation. CONCLUSION: Esophageal intubation may be a life-sustaining ventilation support for patients with tracheal agenesis and TEF at initial resuscitation. Clinicians should suspect tracheal agenesis when a newborn presents with severe cyanosis and voiceless crying upon birth, and esophageal intubation should be immediately attempted.


Asunto(s)
Intubación Intratraqueal , Tráquea , Fístula Traqueoesofágica , Humanos , Recién Nacido , Tráquea/anomalías , Tráquea/diagnóstico por imagen , Masculino , Intubación Intratraqueal/métodos , Femenino , Fístula Traqueoesofágica/complicaciones , Fístula Traqueoesofágica/cirugía , Esófago/anomalías , Esófago/diagnóstico por imagen , Resucitación/métodos , Cianosis/etiología , Constricción Patológica
13.
Braz J Cardiovasc Surg ; 39(3): e20230376, 2024 May 15.
Artículo en Inglés | MEDLINE | ID: mdl-38748885

RESUMEN

INTRODUCTION: Cyanotic congenital heart diseases constitute 40-45% of all congenital heart diseases. In patients who are not suitable for primary repair, modified BT (MBT) shunt and central shunt (CS) procedures are still frequently used. METHODS: This study included 62 pediatric patients who underwent MBT shunt or CS via median sternotomy. Patients' demographic, echocardiographic, operative, and postoperative data were collected retrospectively. The patients were classified as single ventricle and bi-ventricle according to their cardiac anatomy, and the presence of prematurity and heterotaxy was noted. Procedure details of the patients who underwent endovascular intervention prior to the surgery were investigated, and operation data were accessed from the surgery notes. Data regarding postoperative follow-ups were obtained and comparatively analyzed. RESULTS: Of the total 62 patients, 32 (51.6%) were newborns and 16 (25.8%) had a body weight < 3 kg. MBT shunt was applied to 48 patients (77.4%), while CS was applied to 14 patients (22.6%). There was no significant difference between the two surgical procedures in terms of requirement for urgent shunt or cardiopulmonary bypass, additional simultaneous surgical intervention, need for high postoperative inotropes, and in-hospital mortality (P>0.05). The rate of congestive heart failure in patients with in-hospital mortality was determined as 66.7% and it was significantly higher than in patients without heart failure (P<0.001). CONCLUSION: MBT shunt and CS are still frequently used in cyanotic patients. The use of small-diameter shunts, particularly when centrally located, can prevent the onset of congestive heart failure and lower mortality.


Asunto(s)
Cardiopatías Congénitas , Humanos , Cardiopatías Congénitas/cirugía , Estudios Retrospectivos , Masculino , Femenino , Lactante , Recién Nacido , Preescolar , Resultado del Tratamiento , Niño , Mortalidad Hospitalaria , Cianosis/etiología , Cianosis/cirugía , Ecocardiografía
14.
Int J Legal Med ; 138(5): 2049-2055, 2024 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-38664248

RESUMEN

Sudden unexpected postnatal collapse (SUPC) is a sudden collapse of the clinical conditions of a full-term or near-term newborn, within the first 7 days of life, that requires resuscitation with positive ventilation and who either dies, has hypoxic-ischemic encephalopathy, or requires intensive care. The incidence of SUPC is very low, and most often presents a negative prognosis. The BUB1B gene is a mitotic checkpoint of serine/threonine kinase B that encodes a protein crucial for maintaining the correct number of chromosomes during cell division. Mutations in the BUB1B gene are linked to mosaic variegated aneuploidy syndrome 1 (MVA1), a rare autosomal recessive disorder characterized by diffuse mosaic aneuploidies involving several chromosomes and tissues. This paper discusses a case of a newborn who had a spontaneous delivery. After 2 h and 10 min, the infant showed generalized hypotonia and cyanosis, and his doctors performed orotracheal intubation, cardiac massage, pharmacological hemodynamic therapy, mechanical ventilation, antibiotic therapy, and hypothermic treatment. The newborn was discharged after 5 months with the diagnosis of hypoxic-ischemic encephalopathy. Suspecting an SUPC, a complete genetic analysis was performed demonstrating a compound heterozygous mutations in the BUB1B gene. The newborn died at 6 months of life, 1 month after discharge. A complete autopsy was performed, determining that the cause of death was due to sepsis starting from a brocopneumonic process, with outcomes of hypoxic-ischemic encephalopathy (HIE). In this scenario, it is not possible to demonstrate the causal effect of this mutation, considering that it could play a causal or concausal role in the onset of SUPC. Further research based on multicenter studies, as well as on animal models, could be very useful to clarify the pathological effect of this mutation.


Asunto(s)
Mutación , Proteínas Serina-Treonina Quinasas , Humanos , Recién Nacido , Proteínas Serina-Treonina Quinasas/genética , Masculino , Proteínas de Ciclo Celular/genética , Hipoxia-Isquemia Encefálica/genética , Muerte Súbita/etiología , Trastornos de los Cromosomas/genética , Hipotonía Muscular/genética , Mosaicismo , Cianosis/genética
16.
Clin Respir J ; 18(5): e13767, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38685746

RESUMEN

OBJECTIVE: The aim of this study is to investigate the clinical characteristics and pathogens involved in persistent or recurrent pneumonia combined with airway malacia in children. METHODS: We retrospectively reviewed the information of children hospitalised with persistent or recurrent pneumonia, including clinical presentations, laboratory examination results and pathogens. RESULTS: A total of 554 patients were admitted, 285 (51.44%) of whom were found to have airway malacia. There were 78 (27.37%), 166 (58.25%) and 41 (14.39%) patients with mild, moderate and severe malacia, respectively. Patients with airway malacia were younger than those without malacia (6.0 vs. 12.0 months, p < 0.01) and were more likely to present with wheezing (75.07%), fever (34.39%), dyspnoea (28.77%), cyanosis (13.68%) and wheezing in the lungs (78.95%). The incidence of preterm delivery, oxygen therapy, paediatric intensive care unit (PICU) admission and mechanical ventilation was higher, and the hospital stay (11.0 vs. 10.0 days, p = 0.04) was longer in these patients than in those without malacia. Patients with severe airway malacia were more likely to undergo oxygen therapy, PICU admission, mechanical ventilation and have multiple malacia than were those with mild or moderate malacia. Mycoplasma pneumoniae (30.18%) was the most common pathogen. CONCLUSION: Severe airway malacia likely aggravates conditions combined with pneumonia. The proportion of multisite malacia was greater in severe airway malacia patients.


Asunto(s)
Recurrencia , Humanos , Femenino , Masculino , Estudios Retrospectivos , Lactante , Preescolar , Neumonía/epidemiología , Neumonía/complicaciones , Neumonía/microbiología , Neumonía/diagnóstico , Niño , Ruidos Respiratorios/etiología , Neumonía por Mycoplasma/complicaciones , Neumonía por Mycoplasma/epidemiología , Neumonía por Mycoplasma/diagnóstico , Respiración Artificial/estadística & datos numéricos , Tiempo de Internación/estadística & datos numéricos , Disnea/diagnóstico , Disnea/etiología , Disnea/epidemiología , Unidades de Cuidado Intensivo Pediátrico/estadística & datos numéricos , Índice de Severidad de la Enfermedad , Hospitalización/estadística & datos numéricos , Cianosis/etiología
17.
J Neonatal Perinatal Med ; 17(2): 261-264, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38640175

RESUMEN

 Cyanosis is a bluish discoloration of the tissues due to increased levels of deoxygenated hemoglobin in capillaries. It is a common finding in newborn infants that can be caused by different diseases, including pulmonary, cardiac, infectious, and hematological disorders. Methemoglobinemia is a rare cause of cyanosis, in which hemoglobin is oxidized, changing its heme iron configuration from the ferrous (Fe2 +) to the ferric (Fe3 +) state, creating methemoglobin (Met-Hb), a form that does not bind oxygen, leading to decreased oxygen delivery to the tissues and cyanosis. We report a rare case of a preterm newborn, who developed cyanosis and worsening hypoxemia on day ten of life, she was found to have elevated Met-Hb percentage in blood gas analysis that required treatment with intravenous methylene blue. Her symptoms resolved after a period of maintenance treatment with oral methylene blue and ascorbic acid, and the etiology of her disease remains unclear.


Asunto(s)
Ácido Ascórbico , Cianosis , Recien Nacido Prematuro , Metahemoglobinemia , Azul de Metileno , Humanos , Metahemoglobinemia/diagnóstico , Metahemoglobinemia/etiología , Recién Nacido , Femenino , Azul de Metileno/uso terapéutico , Cianosis/etiología , Ácido Ascórbico/uso terapéutico , Ácido Ascórbico/administración & dosificación , Análisis de los Gases de la Sangre , Hipoxia/etiología
19.
Kidney Blood Press Res ; 49(1): 211-217, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38447536

RESUMEN

INTRODUCTION: Cyanotic nephropathy, a rare disease characterized by proteinuria, decreased estimated glomerular filtration rate, thrombocytopenia, polycythemia, and hyperuricemia, may occasionally be secondary to cyanotic congenital heart disease (CHD). There are currently no detailed diagnostic criteria or treatments for cyanotic nephropathy, owing to its extremely low incidence. Eisenmenger syndrome (ES) was initially defined by Paul Wood in pathophysiologic terms as "pulmonary hypertension (PH) at the systemic level, caused by a high pulmonary vascular resistance, with a reversed or bidirectional shunt at the aorto-pulmonary, ventricular, or atrial level." It typically develops in the presence of large, unrepaired atrial or ventricular septal defects, arterial shunts, or complex forms of CHD and is the most severe hemodynamic phenotype of pulmonary arterial hypertension associated with CHD. This study aimed to outline the case of an ES patient who developed cyanotic nephropathy and successfully achieved clinical remission through primary disease treatment and symptomatic management. Overall, this case expands our understanding of cyanotic nephropathy and lays a theoretical reference for the treatment of ES. CASE PRESENTATION: A 33-year-old Chinese female attended the outpatient department with abnormal urine test results over the past two and a half years. Following a comprehensive medical history collection, she underwent the necessary tests. Cardiac color ultrasound displayed a significant widening of the pulmonary artery and PH (severe), as well as mild tricuspid regurgitation and patent ductus arteriosus. The results of the kidney biopsy, combined with clinical findings, suggested a high risk of polycythemia-related kidney disease. She was eventually diagnosed with cyanotic nephropathy and ES. Her symptoms were relieved following symptomatic treatment, such as the administration of ambrisentan, febuxostat, and home oxygen therapy. Her follow-up visit at 6 months demonstrated improvements in hyperuricemia and a significant increase in physical strength. CONCLUSION: Cyanotic nephropathy is a rare condition in adults. Kidney biopsy remains the gold standard of diagnosis for various nephropathies. Active treatment of CHD and alleviating hypoxia may be pivotal for the treatment of cyanotic nephropathy.


Asunto(s)
Complejo de Eisenmenger , Humanos , Femenino , Adulto , Complejo de Eisenmenger/complicaciones , Complejo de Eisenmenger/terapia , Enfermedades Renales/etiología , Cianosis/etiología , Policitemia/complicaciones , Policitemia/terapia
20.
Heart Fail Clin ; 20(2): 223-236, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38462326

RESUMEN

Heart failure in cyanotic congenital heart disease (CHD) is diagnosed clinically rather than relying solely on ventricular function assessments. Patients with cyanosis often present with clinical features indicative of heart failure. Although myocardial injury and dysfunction likely contribute to cyanotic CHD, the primary concern is the reduced delivery of oxygen to tissues. Symptoms such as fatigue, lassitude, dyspnea, headaches, myalgias, and a cold sensation underscore inadequate tissue oxygen delivery, forming the basis for defining heart failure in cyanotic CHD. Thus, it is pertinent to delve into the components of oxygen delivery in this context.


Asunto(s)
Cardiopatías Congénitas , Insuficiencia Cardíaca , Humanos , Cardiopatías Congénitas/complicaciones , Insuficiencia Cardíaca/complicaciones , Insuficiencia Cardíaca/terapia , Cianosis/etiología , Oxígeno , Función Ventricular
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