Vitamin B1 Deficiency Identified from Incidental Detection of Hyperlactatemia: A Case Report.

Introduction: Vitamin B1 deficiency poses a significant risk of impaired consciousness, with manifestations ranging from anorexia and fatigue to severe neurological and cardiovascular disturbances. Wernicke's encephalopathy, a neurological disorder stemming from vitamin B1 deficiency, presents as the triad of ophthalmoplegia, altered mental state, and cerebellar ataxia. However, these symptoms are not consistently present, complicating the diagnosis. In addition, subclinical vitamin B1 deficiency can progress unnoticed until severe complications arise. Studies indicate a high rate of undiagnosed cases, emphasizing the need for early detection and intervention. Case presentation: We present the case of a 65-year-old man in whom hyperlactatemia was incidentally detected, leading to the diagnosis of vitamin B1 deficiency. The patient, presenting with vertigo and vomiting, had been eating boxed lunches bought from convenience stores following the death of his wife 3 years earlier. Vertigo gradually improved with rest, but the persistence of hyperlactatemia prompted further investigation, revealing low vitamin B1 levels and high pyruvate levels. Treatment with dietary adjustments and supplements significantly improved his symptoms. Discussion: In this case, hyperlactatemia was found in a vertigo patient, revealing asymptomatic vitamin B1 deficiency. Elevated lactate is often linked with conditions like sepsis but can also stem from overlooked factors such as low vitamin B1 levels due to poor diet habits like consuming fried foods. Conclusion: This case highlights the importance of considering vitamin B1 deficiency in patients with unexplained hyperlactatemia, even in high-income countries. Early detection can prevent progression to the severe complications associated with Wernicke's encephalopathy. Proactive measurement of lactate levels in at-risk populations may facilitate early diagnosis and intervention, ultimately improving patient outcomes.

Relationship between peripheral ischemic microvascular reserve, persistent hyperlactatemia, and its temporal dynamics in sepsis: a post hoc study.

OBJECTIVE: To measure the prognostic value of peripheral ischemic microvascular reserve in the context of persistent sepsis-induced hyperlactatemia and measure its influence on the temporal dynamics of lactate and the strength of association between these variables. METHODS: This post hoc analysis of the peripheral perfusion index/postocclusive reactive hyperemia trial, an observational cohort study that enrolled patients with sepsis who persisted with lactate levels ≥ 2mmol/L after fluid resuscitation (with or without shock). Peripheral ischemic microvascular reserve was evaluated using the association of the peripheral perfusion index and postocclusive reactive hyperemia techniques. The cutoff point of ∆ peripheral perfusion index peak values (%) defined the groups with low (≤ 62%) and high peripheral ischemic microvascular reserve (> 62%). RESULTS: A total of 108 consecutive patients with persistent sepsis-induced hyperlactatemia were studied. The high peripheral ischemic microvascular reserve group showed higher 28-day mortality than the low peripheral ischemic microvascular reserve group (p < 0.01). The temporal dynamics of lactate within the first 48 hours showed a rapid decrease in lactate levels in the low peripheral ischemic microvascular reserve group (p < 0.01). However, this result was not reproduced in the linear mixed effects model. A weak correlation between peripheral ischemic microvascular reserve (%) and lactate level (mmol/L) was observed within the first 24 hours (r = 0.23; p < 0.05). CONCLUSION: The prognostic value of high peripheral ischemic microvascular reserve was confirmed in the context of persistent sepsis-induced hyperlactatemia. Although there was a weak positive correlation between peripheral ischemic microvascular reserve value and lactate level within the first 24 hours of sepsis diagnosis, the low peripheral ischemic microvascular reserve group appeared to have a faster decrease in lactate over the 48 hours of follow-up.

Hyperlactatemia and altered lactate kinetics are associated with excess mortality in sepsis : A multicenter retrospective observational study.

Severe hyperlactatemia (>10mmol/L) or impaired lactate metabolism are known to correlate with increased mortality. The maximum lactate concentration on day 1 of 10,724 septic patients from the eICU Collaborative Research Database was analyzed and patients were divided into three groups based on maximum lactate in the first 24 h (<5mmol/l; ≥5mmol/l & <10mmol/l; ≥10mmol/l). In addition, delta lactate was calculated using the following formula: (maximum lactate day 1 minus maximum lactate day 2) divided by maximum lactate day 1. A multilevel regression analysis was performed, with hospital mortality serving as the primary study end point. Significant differences in hospital mortality were found in patients with hyperlactatemia (lactate ≥10mmol/l: 79%, ≥5mmol/l & <10mmol/l: 43%, <5mmol/l, 13%; p<0.001). The sensitivity of severe hyperlactatemia (≥10mmol/l) for hospital mortality was 17%, the specificity was 99%. In patients with negative delta lactate in the first 24 h, hospital mortality was excessive (92%). In conclusion, mortality in patients with severe hyperlactatemia is very high, especially if it persists for more than 24 h. Severe hyperlactatemia, together with clinical parameters, could therefore provide a basis for setting treatment limits.

Hyperlactatemia and other perioperative metabolic disturbances in neuroanesthesia.

PURPOSE OF REVIEW: The concept of 'brain-body cross-talking' has gained growing interest in the last years. The understanding of the metabolic disturbances (e.g., hypernatraemia/hyponatraemia and hyperlactatemia) in neurosurgical patients has improved during the last years. RECENT FINDINGS: The impact of elevated lactate without acidosis in neurosurgical patients remains controversial. The pathophysiology of inappropriate secretion of antidiuretic hormone (SIADH) has become clearer, whereas the diagnosis of cerebral salt wasting should be used more carefully. SUMMARY: These findings will contribute to a better understanding of the pathophysiology involved and enable better prevention and therapy where possible in clinical practice.

Risk Factors and Consequences of Late-Onset Hyperlactatemia After Cardiac Surgery With Cardiopulmonary Bypass: A Single-Center Retrospective Study.

OBJECTIVE: Mechanisms and consequences of late-onset hyperlactatemia after cardiac surgery remain unclear. The aim of this study was to identify risk factors and outcomes of late hyperlactatemia, defined as a lactate value ≥3 mmol/L developing in the intensive care unit (ICU) after not being elevated on admission after cardiac surgery with cardiopulmonary bypass. DESIGN: A retrospective analysis of prospectively collected data. SETTING: A single-center University Hospital. PARTICIPANTS: Patients who underwent elective cardiac surgery with cardiopulmonary bypass in 2019 and who had normal lactatemia at ICU admission. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Patients were divided in 2 groups according to their lactatemia on postoperative day 1 (14.0 ± 3.0 hours after ICU admission): normal lactatemia (control group) and late hyperlactatemia (HL group). Risk factors for late hyperlactatemia were identified using a multivariate analysis, and postoperative outcomes were compared using a composite criterion of severe outcomes. Of the 432 included patients, 37 (8.5%) presented with late hyperlactatemia. Risk factors independently associated with hyperlactatemia were afternoon surgery (odds ratio [OR] 4.24, 95% CI 2.00-9.35), a bleeding >300 mL within the 6 hours after surgery (H6) (OR 3.77, 95% CI 1.71-8.30), and H6 fluid loading >250 mL (OR 2.64, 95% CI 1.22-5.55). Patients with hyperlactatemia presented more frequently with major postoperative complications, including acute kidney injury, and received more frequent red-cell transfusion. CONCLUSION: The strongest risk factors associated with late-onset hyperlactatemia in the authors' population were afternoon surgery and H6 bleeding >300 mL. Poor postoperative outcomes were more frequent in patients with late hyperlactatemia, even in the absence of early hyperlactatemia or severe obvious condition.

Association Between Hyperlactatemia, Perfusional Parameters, and Lymphocyte Mitochondrial Dysfunction in Septic Shock Patients.

INTRODUCTION: In septic shock, mitochondrial dysfunction, and hypoperfusion are the main triggers of multi-organ failure. Little is known about the crosstalk between mitochondrial dysfunction and hemodynamic alterations, especially in the post-resuscitation phase. Here, we assess whether hypoperfusion and lactate levels are associated with oxygen consumption linked to mitochondrial bioenergetic activity in lymphocytes of patients admitted with septic shock. PATIENTS AND METHODS: Prospective cohort study in patients with septic shock defined as the requirement of vasopressors to maintain a mean arterial pressure 65 mm Hg after initial fluid administration. Basal mitochondrial and Complex I respiration was measured to evaluate mitochondrial activity. Both variables and capillary refill time were compared with arterial lactate post-fluid resuscitation. We also compared mitochondrial activity measurements between patients with and without hypoperfusion status. RESULTS: A total of 90 patients were included in analysis. The median arterial lactate at the time of septic shock diagnosis was 2.0 mmol/Dl (IQR 1.3-3.0). Baseline respiration at the time of septic shock diagnosis was correlated with lactate (Spearman -0.388, 95% CI -0.4893 to -0.1021; P = 0.003), as well as Complex I respiration (Spearman -0.403, 95% CI -0.567 to -0.208; P < 0.001). Patients with hypoperfusion status had no difference in basal respiration when compared with patients who did not have hypoperfusion status (P = 0.22) nor in Complex I respiration (P = 0.09). CONCLUSION: Changes in lymphocytic mitochondrial metabolism are associated with post-resuscitation arterial lactate in septic shock; however, they are not associated with the presence of a hypoperfusional status. In this scenario, it is therefore suggested that systemic perfusion and mitochondrial metabolism have different courses.

What Is the Utility of Measuring Lactate Levels in Patients with Sepsis and Septic Shock?

Elevations in blood lactate concentrations have been studied in sepsis and other disease states for decades and are well known to be associated with increased mortality. Many studies have also demonstrated the prognostic accuracy of serial lactate levels, and some have suggested that lactate clearance may be a useful therapeutic target for resuscitation. Lactate measurements have therefore gained an increasingly prominent role in sepsis definitions, screening protocols, management guidelines, and quality measures over the past two decades. The heavy emphasis on lactate monitoring, however, has also generated controversy and concerns. Lactate is not specific to infection and its frequent use for sepsis screening and diagnosis may therefore trigger unnecessary broad-spectrum antibiotic use in some patients. Because hyperlactatemia does not always reflect fluid-responsive hypoperfusion, titrating resuscitation to lactate clearance can also lead to unnecessary fluid and volume overload. More broadly, there is a lack of high-quality evidence demonstrating that initial and serial lactate monitoring leads to better patient-centered outcomes. Indeed, a recent randomized controlled trial comparing resuscitation strategies based on lactate clearance versus normalizing capillary refill time showed no benefit and potential harm with lactate-guided therapy. In this article, we review the basic pathobiology of lactate metabolism and delineate why the traditional paradigm that hyperlactatemia reflects tissue hypoxia is overly simplistic and incomplete. We then review the evidence behind the diagnostic, prognostic, and therapeutic uses of lactate monitoring and place this in the context of evolving sepsis diagnosis and management guidelines.

Hyperlactacidemia as a risk factor for intensive care unit-acquired weakness in critically ill adult patients.

INTRODUCTION/AIMS: Intensive care unit-acquired weakness (ICUAW) is a severe neuromuscular complication of critical illness. Serum lactate is a useful biomarker in critically ill patients. The relationship between serum lactate level and ICUAW remains controversial. This study evaluated whether hyperlactacidemia (lactate level >2 mmol/L) was an independent risk factor for ICUAW in critically ill adult patients. METHODS: An observational cohort study was performed in a general multidisciplinary intensive care unit (ICU). Sixty-eight consecutive adult critically ill patients without preexisting neuromuscular disease or a poor pre-ICU functional status whose length of ICU stay was 7 or more days were evaluated. Patients were screened daily for signs of awakening. Muscle strength assessment using the Medical Research Council score was performed on the first day a patient was considered awake. Patients with clinical muscle weakness were considered to have ICUAW. RESULTS: Among the 68 patients who achieved a satisfactory state of consciousness, the diagnosis of ICUAW was made in 30 patients (44.1%). After multivariate analysis, hyperlactacidemia (P = .02), Acute Physiology and Chronic Health Evaluation II score (P = .04), duration of mechanical ventilation (P = .02), and the use of norepinephrine (P = .04) were found to be significantly associated with the development of ICUAW in critically ill patients. DISCUSSION: This study shows a number of risk factors to be significantly associated with the development of ICUAW in critically ill adults. These factors should be considered when building early prediction models or designing prevention strategies for ICUAW in future studies.

Prognostic role of perioperative acid-base disturbances on the risk of Clostridioides difficile infection in patients undergoing cardiac surgery.

BACKGROUND: It is unclear whether acid-base balance disturbances during the perioperative period may impact Clostridium difficile infection (CDI), which is the third most common major infection following cardiac surgery. We hypothesized that perioperative acid-base abnormalities including lactate disturbances may predict the probability of incidence of CDI in patients after cardiac procedures. METHODS: Of the 12,235 analyzed patients following cardiac surgery, 143 (1.2%) developed CDI. The control group included 200 consecutive patients without diarrhea, who underwent cardiac procedure within the same period of observation. Pre-, intra and post-operative levels of blood gases, as well as lactate and glucose concentrations were determined. Postoperatively, arterial blood was drawn four times: immediately after surgery and successively; 4, 8 and 12 h following the procedure. RESULTS: Baseline pH was lower and PaO2 was higher in CDI patients (p < 0.001 and p = 0.001, respectively). Additionally, these patients had greater base deficiency at each of the analyzed time points (p < 0.001, p = 0.004, p = 0.012, p = 0.001, p = 0.016 and p = 0.001, respectively). Severe hyperlactatemia was also more common in CDI patients; during the cardiac procedure, 4 h and 12 h after surgery (p = 0.027, p = 0.004 and p = 0.001, respectively). Multivariate logistic regression analysis revealed that independent risk factors for CDI following cardiac surgery were as follows: intraoperative severe hyperlactatemia (OR 2.387, 95% CI 1.155-4.933, p = 0.019), decreased lactate clearance between values immediately and 12 h after procedure (OR 0.996, 95% CI 0.994-0.999, p = 0.013), increased age (OR 1.045, 95% CI 1.020-1.070, p < 0.001), emergent surgery (OR 2.755, 95% CI 1.565-4.848, p < 0.001) and use of antibiotics other than periprocedural prophylaxis (OR 2.778, 95% CI 1.690-4.565, p < 0.001). CONCLUSION: This study is the first to show that perioperative hyperlactatemia and decreased lactate clearance may be predictors for occurrence of CDI after cardiac surgery.

Semiquantitative acid-base analysis in dogs with typical hypoadrenocorticism.

OBJECTIVE: To describe the semiquantitative acid-base status of dogs with untreated naturally occurring typical hypoadrenocorticism and to compare this to the status determined by traditional acid-base analysis. DESIGN: Retrospective study. SETTING: University teaching hospital. ANIMALS: Thirty-three dogs with newly diagnosed typical hypoadrenocorticism between 2000 and 2017. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Dogs were included if they had newly diagnosed hypoadrenocorticism, post-ACTH stimulation serum cortisol concentration <2 µg/dL, and blood collected within 6 hours of presentation for acid-base, electrolyte, and serum biochemical assays. Dogs were excluded if the Na+ :K+ ratio was ≥28 or the dog had received a mineralocorticoid-containing corticosteroid medication within the preceding month. Traditional acid-base analysis identified normal acid-base status in 1 dog, simple respiratory acid-base abnormalities in 2 of 33 dogs, and simple metabolic acidosis in 14 of 33 dogs. A mixed disorder was most common, noted in 16 of 33 dogs. The semiquantitative approach identified metabolic abnormalities in all cases. All dogs had ≥1 acidifying process, and 29 of 33 had both acidifying and alkalinizing processes. Acidosis attributable to excess free water was present in all dogs, and an acidifying phosphate effect was present in 27 of 33. Hyperlactatemia contributed to the acidosis in 8 of 33 dogs, with a median (range) lactate concentration of 1.5 mmol/L (13.5 mg/dL) (0.3-4.2 mmol/L [2.7-37.8 mg/dL]). CONCLUSIONS: Dogs with untreated Addison's disease have complex acid-base derangements. The semiquantitative approach to acid-base analysis provides greater insight into the underlying mechanisms of metabolic acid-base abnormalities in these dogs, particularly because lactic acidosis appears to be a minor influence in most cases.

Predictors of Increased Lactate in Neonatal Cardiac Surgery: The Impact of Cardiopulmonary Bypass.

OBJECTIVE: Hyperlactatemia develops intraoperatively during cardiac surgery and is associated with postoperative mortality. This study aimed to determine the factors that lead to an increase in lactate during cardiopulmonary bypass (CPB) in neonates undergoing cardiac surgery. DESIGN: Retrospective study from July 2015 to December 2018. SETTING: Academic tertiary children's hospital. PARTICIPANTS: The study comprised 376 neonates. INTERVENTIONS: No interventions were performed. MEASUREMENTS AND MAIN RESULTS: Lactate measurements at prebypass, upon initiation of CPB and before coming off CPB, last in the operating room, and first in the cardiac intensive care unit were collected. The changes in lactate levels were compared using the nonparametric Wilcoxon signed rank test for paired data. Univariate and multivariate median regression models of the change during CPB were determined. The cohort characteristics were male (60%), median age 5 days (range 1-30), and weight 3.2 kg (range 1.5-4.7). Most patients had a STAT score of 4 (45%) or 5 (23%). Significant increases in lactate were observed from pre-CPB to start of CPB (p < 0.001) and from start to end of CPB (p < 0.001). In the multivariate regression analysis, duration of circulatory arrest (coefficient = 1.216; 95% confidence interval [CI] 0.754-1.678; p < 0.001), duration of mean arterial pressure < 25 mmHg (coefficient = 0.423; 95% CI 0.196-to- 0.651; p < 0.001), and duration of mean arterial pressure between 35 and 39 mmHg (coefficient = -0.246; 95% CI -0.397 to -0.095; p = 0.001) were identified as significant independent predictors of the lactate change per 30- minutes duration. CONCLUSION: These results emphasized the importance of blood pressure management during CPB and the importance of the duration of circulatory arrest.

Relación entre el tiempo de circulación extracorpórea y el pinzamiento aórtico con diferentes variables de evolución postquirúrgicas / Relationship between cardiopulmonary bypass time and aortic cross clamping time with different variables of post-surgical evolution

Resumen Justificación: La mayor cantidad de cirugías cardiacas realizadas a nivel mundial se efectúa con circulación extracorpórea y pinzamiento de la aorta, lo que conlleva una serie de alteraciones fisiopatológicas que deben ser reconocidas por el personal de salud que participa en la atención de estos pacientes. Objetivo: Describir el perfil y los factores de riesgo presentes en los pacientes sometidos a cirugía cardiaca con circulación extracorpórea, y analizar la existencia de una potencial relación entre el tiempo de circulación extracorpórea y el pinzamiento aórtico, con la aplicación de desfibrilaciones tras al pinzado de la aorta, la necesidad de soporte cardiovascular farmacológico, el comportamiento del nivel de lactato plasmático y la mortalidad. Métodos: Se desarrolló un estudio observacional y descriptivo con una muestra de 104 pacientes electivos, sometidos a intervención quirúrgica y circulación extracorpórea, en el Hospital México, desde octubre de 2016 a noviembre de 2017. Se caracterizó la población en estudio, se analizaron los factores de riesgo incluido el EuroSCORE I y II, el tiempo de circulación extracorpórea, el tiempo de pinzamiento aórtico, las desfibrilaciones posteriores al pinzamiento aórtico, e lactato inmediatamente postcirculación extracorpórea, y a las 2, 6, 24 h postquirúrgicas, el uso de soporte cardiovascular farmacológico en infusión continua posterior a la circulación extracorpórea y mortalidad a los 30 días. Resultados: La edad media fue 56,4 años, predominó el sexo masculino (69 %) y la hipertensión arterial fue el factor de riesgo más frecuente (76,07 %). Se registró un tiempo de pinzado aórtico menor a 100 min en 61 pacientes (58,65 %) y superior a ese tiempo en 43 pacientes (41,35 %). El EuroSCORE I promedio fue del 4,21 % (DE: 4,80), mientras que el EuroSCORE II fue del 2,37 % (DE: 2,41). El tiempo promedio de circulación extracorpórea fue de 129 minutos (DE: 36,88) y el de pinzado aórtico, de 94 minutos (DE:32,04). Hubo un pico de lactato a las 6 horas postquirúrgicas (5,13 mmol/L, DE:2,89); un 8,65 % de los pacientes fueron desfibrilados después del retiro de la pinza en la aorta; se utilizó soporte cardiovascular en el 16,35 % y la mortalidad quirúrgica fue del 1,92 %. Conclusiones: En el estudio, el tiempo de circulación extracorpórea y el pinzado aórtico junto con el uso de inotrópicos, vasoconstrictores, hiperlactatemia y mortalidad quirúrgica, no alcanzó una relación significativa.

Abstract Justification: Currently, the largest number of cardiac surgeries performed worldwide are performed with cardiopulmonary bypass and aortic cross clamp, which leads to a series of pathophysiological alterations that are important for health personnel involved in the care of these patients. Objective: To describe the profile and risk factors present in patients undergoing cardiac surgery with cardiopulmonary bypass and the existence of a potential relationship between the cardiopulmonary bypass time and aortic cross clamping time, with the use of post clamp defibrillations, pharmacological cardiovascular support, plasma lactate behavior and mortality. Methods: An observational and descriptive study was carried out with a sample of 104 elective patients, undergoing surgical intervention and cardiopulmonary bypass at Hospital México, from October 2016 to November 2017. The study population was characterized, risk factors were analyzed including EuroSCORE I and II, CPB time, aortic cross clamping time, post-aortic clamping defibrillation, lactate immediately after extracorporeal circulation and at 2, 6, 24 hours postoperatively, use of pharmacological cardiovascular support in continuous infusion after extracorporeal circulation and mortality at 30 days. Results: The mean age was 56.4 years, the male sex predominated (69%) and arterial hypertension was the most frequent risk factor (76.07%). Aortic cross clamp time of less than 100 min was recorded in 61 patients (58.65%) and greater than that time in 43 patients (41.35%). The average EuroSCORE I was 4.21% (SD: 4.80), while the EuroSCORE II was 2.37% (SD: 2.41). The average cardiopulmonary bypass time was 129 minutes (SD: 36.88) and aortic cross clamp time was 94 minutes (SD: 32.04). There was a lactate peak at 6 postoperative hours (5.13 mmol/L, SD: 2.89); 8.65% of patients were defibrillated after removal the clamp in the aorta; pharmacological cardiovascular support was used in 16.35% and surgical mortality was 1.92%. Conclusions: In this study, cardiopulmonary bypass time and aortic cross clamp time together with the use of inotropics, vasoconstrictors, hyperlactatemia and surgical mortality did not reach a significant relationship.

Shock Index As A Predictor Of Hyperlactatemia For Early Detection Of Severe Sepsis In Patients Presenting To The Emergency Department Of A Low To Middle Income Country.

BACKGROUND: Early detection of sepsis in the emergency department is of prime importance and requires tools that are time and cost-effective. The Systemic Inflammatory Response Syndrome (SIRS) has been poorly associated with sepsis. Timothy et al in a retrospective analysis of Emergency Department (ED) visit stated estimate of SIRS at 17.8% accounting to an annual yield of 16.6 million adult visits with SIRS per year, among these only 26% accounted as an infectious aetiology of SIRS, trauma being 10% and other causes being rare. Shock index is found to be independently associated with 30-day mortality in a broad population of ED patients including sepsis. With limited health resources in a low to middle income country, focused utilization is important and so is the need for markers that are non-invasive, readily available, cost effective, and easy to interpret. Shock index can serve this purpose as a surrogate marker of disease severity in patients with severe sepsis and thus resulting in early detection of such patients. METHODS: This cross-sectional study was conducted from December 2014 to May 2015 at a tertiary care setup (Aga Khan University Hospital) in Karachi consisting of all septic patients received at the emergency department. Non-probability sampling technique was used. p-value <0.05 was taken as significant. RESULTS: Out of 180 study participants 94 (52.22%) were males while 86 (47.78%) were females. The mean age was 57.48±18.8 years. Cohen's κ was used to determine an agreement between the Shock index and Lactate levels. Shock index with cut off value of > 0.7 was used and moderate to the strong agreement between the two was found with kappa κ = 0.786 which was statistically significant (p=<0.001). Sensitivity was found to be 0.99, specificity 0.75, NPV 0.98, PPV 0.87. CONCLUSIONS: To conclude the shock index has some very favourable features, including availability, low cost, and direct relevance to sepsis in terms of its high validity. A high SI predicts elevated lactate levels in patients with sepsis.

[Menkes disease, a diagnosis to consider in case of severe epilepsy with hyperlactacidemia: a case report]. / Epilepsie réfractaire et hyperlactacidémie chez un nourrisson : la maladie de Menkès, un diagnostic à envisager. A propos d'un cas.

Menkes disease is an X-linked recessive disorder affecting copper metabolism due to an inactivating mutation of ATP7A gene. This result in loss of copper intestinal absorption, tissue deficiency and failure in multiple essential copper-enzyme systems such as the cytochrome c oxidase. Symptoms usually occur during the first months of life with neurological signs such as epilepsy associated to other signs among them typical hair appearance. We report the case of a 3 month-old infant hospitalized due to partial tonic-clonic seizures. Laboratory findings showed increased of lactates in blood and in cerebrospinal fluid. First screenings for infectious, metabolic and genetic causes were negative. After recurrence of multifocal seizures further investigations are made according to the presence of thick and tortuous hair. Low levels of ceruloplasmin and copper in plasma are in agreement with the suspected diagnosis of Menkes disease. Molecular analysis of the ATP7A gene confirmed the diagnosis with a non-sens mutation.

Hyperlactatemia: An Update on Postoperative Lactate.

While hyperlactatemia in postoperative cardiac surgery patients was once believed to solely reflect hypoperfusion, either from the accumulated "oxygen debt" during bypass or ongoing inadequate perfusion, our understanding of lactate generation, clearance, and management has evolved. A contemporary understanding of lactate balance is critical to the management of the postoperative patient with hyperlactatemia. In this review, we summarize the current understanding of lactate metabolism in pediatric patients following cardiac surgery and highlight two types of hyperlactatemia: type A, which is secondary to inadequate oxygen delivery and tissue hypoxia, and type B, which in postoperative pediatric cardiac surgery patients largely reflects increased glycolysis driven by the stress response. Both types may coexist; thus, it is imperative that providers first assess the patient for evidence of hypoperfusion. In patients with evidence of adequate perfusion, a type B component is often associated with a concomitant balanced (normal anion gap) metabolic acidosis and hyperglycemia. These patients will benefit from a more nuanced approach to their type B hyperlactatemia, as many will have a benign course and may be managed expectantly.

Myasthenic crisis and late deep vein thrombosis following thymectomy in a patient with myasthenia gravis: A case report.

INTRODUCTION: Surgical stress and pain are potential provoking factors for postoperative myasthenic crisis (POMC). We report the occurrence of early POMC and late deep vein thrombosis (DVT) in a man with myasthenia gravis (MG) undergoing thymectomy, addressing possible link between reversal of opioid overdose with naloxone and the triggering of POMC. PATIENT CONCERNS: A 71-year-old man with impaired renal function (ie, estimated glomerular filtration rate [egfr]: 49.1 mL/min/1.73 m) with diagnosis of MG made 2 months ago was scheduled for thymectomy. After uncomplicated surgery, he experienced opioid overdose that was treated with naloxone. Hyperlactatemia then developed with a concomitant episode of hypertension. Three hours after reversal, he suffered from myasthenic crisis presenting with respiratory failure and difficult weaning from mechanical ventilation. DIAGNOSIS: Stress-induced hyperlactatemia and subsequent myasthenic crisis INTERVENTIONS:: Pyridostigmine and immunosuppressive therapy with prednisolone were initiated. Hyperlactatemia subsided on postoperative day (POD) 5. Tracheal extubation was performed successfully on POD 6. OUTCOMES: During the course of hospitalization, his eGFR (ie, 88.9 mL/min/1.73 m) was found to improve postoperatively. After discharge from hospital, he developed DVT in the left femoral and popliteal veins on POD 24 when he was readmitted for immediate treatment with low-molecular-weight heparin. He was discharged without sequelae on POD 31. There was no recurrence of myasthenic crisis or DVT at 3-month follow-up. CONCLUSIONS: Following naloxone administration, hyperlactatemia may be an indicator of pain-related stress response, which is a potential provoking factor for myasthenic crisis. Additionally, patients with MG may have an increased risk of DVT possibly attributable to immune-mediated inflammation. These findings highlight the importance of perioperative avoidance of provoking factors including monitoring of stress-induced elevations in serum lactate concentration, close postoperative surveying for myasthenic crisis, and early recognition of possible thromboembolic complications in this patient population.

Prognostic relevance of pre- and postoperative plasma l-lactate measurements in calves with acute abdominal emergencies.

In both human and veterinary medicine, l-lactate is a well-established prognostic biomarker of disease severity and mortality and has also attracted increasing attention in bovine medicine due to the availability and validation of cheap and portable l-lactate analyzers. The aim of the present study was to evaluate the prognostic accuracy of plasma L-lactate measurements in calves with acute abdominal emergencies before and during the initial therapeutic period after surgical intervention. A prospective observational study was carried out involving 83 hospitalized calves up to an age of 7 mo, which required surgical intervention for reasons of an acute abdominal emergency such as gastrointestinal ileus or peritonitis. Plasma l-lactate (L-LAC) concentrations were determined immediately before initiation of surgery and 6, 12, 24, 48, and 72 h later. The outcome of calves was evaluated 3 mo after discharge by a phone call to the farmer, and a positive outcome was defined if the calf was still alive and the owner was satisfied with the animal's postsurgical progress. A total of 29% of calves were discharged from the hospital and the proportion of calves with a positive outcome after the 3-mo period was 24%. At all sampling times during the first 48 h after initiation of surgical intervention, calves with a negative outcome had significantly higher L-LAC than calves with a positive outcome. A binary logistic regression analysis indicated that the odds for a negative outcome during the 3-mo observation period increased by a factor of 1.23 [95% confidence interval (CI): 1.04-1.44] for every mmol/L increase of L-LAC before initiation of surgical intervention, but by a factor of 5.29 (95% CI: 1.69-16.6) and 5.92 (95% CI: 1.29-27.3) at 12 and 24 h, respectively. The largest area under the receiver operating characteristic curve for L-LAC was observed at 12 h (0.91; 95% CI: 0.83-0.99), and a cut-point of 2.75 mmol/L was identified that had a sensitivity and specificity for predicting a negative outcome of 68 and 100%, respectively. In conclusion, persistent hyper-l-lactatemia during the early postoperative period is a more reliable indicator for a negative outcome in calves with acute surgical abdominal emergencies than hyper-l-lactatemia before initiation of surgical intervention. Postoperative measurements of L-LAC are therefore a clinically useful tool to identify patients with an increased risk for a negative outcome at an early stage after surgical intervention was carried out.