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SAGE Open Medical Case Reports 2024FLAMES, or fluid-attenuated inversion recovery-hyperintense lesions in anti-myelin oligodendrocyte glycoprotein (anti-myelin oligodendrocyte glycoprotein)-associated...
A burning encephalitis: Fluid-attenuated inversion recovery-hyperintense lesions in Anti-myelin oligodendrocyte glycoprotein-associated encephalitis with seizures in anti-myelin oligodendrocyte glycoprotein-associated encephalitis with seizures-A case report and review of the literature.
FLAMES, or fluid-attenuated inversion recovery-hyperintense lesions in anti-myelin oligodendrocyte glycoprotein (anti-myelin oligodendrocyte glycoprotein)-associated encephalitis with seizures, represents a rarely documented syndrome characterized by ambiguous features. Positioned within the spectrum of inflammatory demyelinating diseases of the central nervous system, it is regarded as a distinct subset of myelin oligodendrocyte glycoprotein antibody-associated disease, the latest classification in this domain. Myelin oligodendrocyte glycoprotein antibody-associated disease exhibits a diverse clinical spectrum, spanning from solitary optic neuritis or myelitis to multifocal central nervous system demyelination, manifesting as acute disseminated encephalomyelitis, or cortical encephalitis accompanied by seizures, delineating the fluid-attenuated inversion recovery-hyperintense lesions in anti-myelin oligodendrocyte glycoprotein-associated encephalitis with seizures syndrome. We present a compelling case study of a 30-year-old individual with a history of recurrent seizures initially diagnosed with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes. However, the disease's progression more closely resembled self-resolving cerebral cortical encephalitis linked with myelin oligodendrocyte glycoprotein antibodies. In addition, we undertake a systematic review of literature cases to explore the diagnostic significance of magnetic resonance angiography, fluid-attenuated inversion recovery, and specialized markers such as diffusion-weighted imaging and perfusion in discerning fluid-attenuated inversion recovery-hyperintense lesions in anti-myelin oligodendrocyte glycoprotein-associated encephalitis with seizures syndrome and elucidating its distinctive characteristics.
PubMed: 38881971
DOI: 10.1177/2050313X241261021 -
Frontiers in Genetics 2024Patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) usually present with multisystemic dysfunction with a wide range of...
BACKGROUND
Patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) usually present with multisystemic dysfunction with a wide range of clinical manifestations. When the tests for common mitochondrial DNA (mtDNA) point mutations are negative and the mtDNA defects hypothesis remains, urine epithelial cells can be used to screen the mitochondrial genome for unknown mutations to confirm the diagnosis.
CASE PRESENTATION
A 66-year-old Chinese woman presented with symptoms of MELAS and was initially misdiagnosed with acute encephalitis at another institution. Although genetic analysis of blood lymphocyte DNA was negative, brain imaging, including magnetic resonance imaging, magnetic resonance spectroscopy, and clinical and laboratory findings, were all suggestive of MELAS. Finally, the patient was eventually diagnosed with MELAS with the mtDNA 5783G>A mutation in the MT-TC gene with a urinary sediment genetic test.
CONCLUSION
This case report expands the genetic repertoire associated with MELAS syndrome and highlights the importance that full mtDNA sequencing should be warranted beside the analysis of classical variants when a mitochondrial disorder is highly suspected. Furthermore, urine sediment genetic testing has played a crucial role in the diagnosis of MELAS.
PubMed: 38881794
DOI: 10.3389/fgene.2024.1367716 -
Canadian Journal of Anaesthesia =... Jun 2024The benefits of intraoperative dialysis during orthotopic liver transplantation remain controversial. In patients with anuric renal failure and portopulmonary...
PURPOSE
The benefits of intraoperative dialysis during orthotopic liver transplantation remain controversial. In patients with anuric renal failure and portopulmonary hypertension, maintaining venous return during caval clamping and unclamping along with minimizing fluid overload is critical to avoiding right ventricular strain and failure.
CLINICAL FEATURES
We present the case of a 54-yr-old female who underwent orthotopic liver transplantation for alcohol-related liver disease with acute decompensation including severe hepatorenal syndrome (anuric requiring dialysis), probable hepatopulmonary syndrome, moderate pulmonary hypertension (right ventricular systolic pressure, 44 mm Hg), hepatic encephalopathy (grade 2), and esophageal varices. Prior to incision, pulmonary arterial pressures were 48/28 (mean, 35) mm Hg with a central venous pressure of 30 mm Hg, cardiac output of 7.4 L·min, and pulmonary vascular resistance of 98 dynes·sec·cm. In the context of right ventricular strain and volume overload observed on transthoracic echocardiography, we inserted an additional dialysis catheter into the right femoral vein. We initiated dialysis using the two catheters as a circuit (femoral line to the dialysis machine; blood was reinjected via the subclavian line) acting as a limited venovenous bypass, allowing right ventricular offloading and hemodialysis throughout the case. We removed 4.5 L via hemodialysis during the surgery, while avoiding acidosis, hyperkalemia, and sodium shifts. The patient tolerated reperfusion adequately despite pre-existing right ventricular dilation and dysfunction.
CONCLUSION
We report on the use two hemodialysis catheters in a patient undergoing orthotopic liver transplantation as a circuit for simultaneous anuric hepatorenal syndrome and moderate pulmonary hypertension with right ventricular dilation and dysfunction. We believe this technique was instrumental in the patient's successful transplant.
PubMed: 38877135
DOI: 10.1007/s12630-024-02777-3 -
Archives of Gynecology and Obstetrics Jun 2024This study aimed to address the increasing prevalence of cesarean section and the importance of evaluating newborn health through arterial blood gas analysis. Its...
BACKGROUND
This study aimed to address the increasing prevalence of cesarean section and the importance of evaluating newborn health through arterial blood gas analysis. Its primary objective was to compare the umbilical cord blood gas levels in newborns delivered through different delivery methods.
METHOD
This retrospective descriptive cross-sectional study included singleton pregnancies with a gestational age between 37 and 42 weeks and infants weighing between 2500 and 4000 g. Newborns with an Apgar score of 7 or higher at 1 and 5 min were included. Umbilical cord blood samples were collected from each newborn for blood gas analysis within 60 min after birth.
RESULT
The study included 340 neonates, with 170 born via caesarean section and 170 born through vaginal delivery. No significant differences were observed in Apgar scores between two groups. ABG analysis showed that vaginally born neonates had lower pH (7.24 ± 0.08 vs. 7.27 ± 0.07, P < 0.001), PCO2 (P = 0.015), and HCO3 (P < 0.001). Cesarean section neonates had higher oxygen saturation (P = 0.007) and pressure of oxygen (P < 0.001), and less negative base excess (P < 0.001). In the subgroup analysis, neonates whose mothers received epidural anesthesia had lower pH (7.23 ± 0.07 vs. 7.25 ± 0.08, P = 0.021) and more negative base excess (P = 0.026). Other parameters of ABG did not differ significantly between the groups (P > 0.05).
CONCLUSION
It has been proven that the mode of delivery, whether it is vaginal or cesarean, as well as the administration of epidural anesthesia during vaginal delivery, have a significant impact on newborns at birth. Newborns delivered vaginally exhibit metabolic acidosis compared to those delivered via cesarean section. Although these differences are statistically significant, they do not have a notable clinical significance, as the average values of the evaluated parameters in both groups fall within the normal range.
PubMed: 38874777
DOI: 10.1007/s00404-024-07594-z -
Dalton Transactions (Cambridge, England... Jun 2024Physiological or pathophysiological changes lead to posttranslational changes in the sialic acid content of human serum transferrin (hTf), an essential mediator of iron...
Physiological or pathophysiological changes lead to posttranslational changes in the sialic acid content of human serum transferrin (hTf), an essential mediator of iron transport in the human body, resulting in a significantly increased concentration of desialylated hTf. The intrinsic fluorescence quenching upon binding of iron to hTf was successfully modeled using the binding polynomial for two iron-binding sites, allowing measurements in a high-throughput format. Removal of sialic acid residues resulted in a 3-fold increase in iron binding affinity for both sites of hTf at pH 7.4. The pH-dependence of iron binding showed significant differences in equilibrium constants, resulting in a 10-fold increase in binding affinity for desialylated hTf at pH 5.9. The changes in hTf sialylation apparently result in tuning of the stability of the conformational state, which in turn contributes to the stability of the diferric hTf. The observed differences in the conditional thermodynamic equilibrium constants suggest that the desialylated protein has a higher preference for diferric hTf over monoferric hTf species down to pH 6.5, which may also influence the interaction with transferrin receptors that preferentially bind to diferric hTf. The results suggest a link between changes in hTf glycan structure and alterations in iron binding equilibrium associated with tissue acidosis.
Topics: Transferrin; Humans; Hydrogen-Ion Concentration; Protein Binding; Iron; N-Acetylneuraminic Acid; Ferric Compounds; Binding Sites; Thermodynamics
PubMed: 38873789
DOI: 10.1039/d4dt01311e -
Journal of Paediatrics and Child Health Jun 2024
PubMed: 38873727
DOI: 10.1111/jpc.16597 -
Cureus May 2024Diabetic ketoacidosis (DKA) is a severe complication of diabetes mellitus characterized by hyperglycemia, metabolic acidosis, and ketosis. We present a challenging case...
Diabetic ketoacidosis (DKA) is a severe complication of diabetes mellitus characterized by hyperglycemia, metabolic acidosis, and ketosis. We present a challenging case of euglycemic DKA secondary to fasting and urinary tract infection with acute renal failure in a 50-year-old woman. Despite normal random blood sugar levels, the patient exhibited clinical signs of DKA, leading to further investigation. High anion gap metabolic acidosis with hyperkalemia and abnormal renal function tests were identified. After hemodialysis, serum ketones were found to be highly positive, confirming the diagnosis. Prompt management led to a complete clinical and laboratory resolution. This case underscores the importance of considering DKA in patients with suggestive symptoms, even with normal blood sugar levels.
PubMed: 38872637
DOI: 10.7759/cureus.60171 -
Deutsches Arzteblatt International Apr 2024
Topics: Humans; Diabetic Ketoacidosis; Germany; Diabetes Mellitus, Type 1; Child; Male; Female; Child, Preschool; Adolescent; Risk Factors; Infant; Incidence; Prevalence
PubMed: 38872431
DOI: 10.3238/arztebl.m2024.0021 -
Clinical and Experimental Nephrology Jun 2024The beneficial effects of oral supplements with alkalinizing agents in patients with chronic kidney disease (CKD) have been limited to the severe stages. We investigated...
BACKGROUND
The beneficial effects of oral supplements with alkalinizing agents in patients with chronic kidney disease (CKD) have been limited to the severe stages. We investigated whether two types of supplements, sodium bicarbonate (SB) and potassium citrate/sodium citrate (PCSC), could maintain renal function in patients with mild-stage CKD.
METHODS
This was a single-center, open-labeled, randomized cohort trial. Study participants with CKD stages G2, G3a, and G3b were enrolled between March 2013 and January 2019 and randomly assigned by stratification according to age, sex, estimated glomerular filtration rate (eGFR), and diabetes. They were followed up for 6 months (short-term study) for the primary endpoints and extended to 2 years (long-term study) for the secondary endpoints. Supplementary doses were adjusted to achieve an early morning urinary pH of 6.8-7.2. We observed renal dysfunction or new-onset cerebrovascular disease and evaluated urinary surrogate markers for renal injury.
RESULTS
Overall, 101 participants were registered and allocated to three groups: standard (n = 32), SB (n = 34), and PCSC (n = 35). Two patients in the standard group attained the primary endpoints (renal stones and overt proteinuria) but were not statistically significant. There was one patient in the standard reduced eGFR during the long-term study (p = 0.042 by ANOVA). SB increased proteinuria (p = 0.0139, baseline vs. 6 months), whereas PCSC significantly reduced proteinuria (p = 0.0061, baseline vs. 1 year, or p = 0.0186, vs. 2 years) and urinary excretion of 8-hydroxy-2'-deoxyguanosine (p = 0.0481, baseline vs. 6 months).
CONCLUSION
This study is the first to report supplementation of PCSC reduced intrarenal oxidative stress in patients with mild-stage CKD.
PubMed: 38872014
DOI: 10.1007/s10157-024-02517-3 -
Zhonghua Yi Xue Za Zhi Jun 2024To investigate the impact of intraoperative hypothermia on postoperative outcome in neonatal patients undergoing non-cardiac surgery. The data of 1 008 neonates...
To investigate the impact of intraoperative hypothermia on postoperative outcome in neonatal patients undergoing non-cardiac surgery. The data of 1 008 neonates undergoing non-cardiac surgery in Children's Hospital, Zhejiang University School of Medicine from January 2020 to October 2022 were retrospectively collected,which included 558 males and 450 females, with a midian age [ (, )] of 6 (2, 14) days. Neonates were divided into 4 groups according to whether hypothermia (below 36 ℃) occurred and the lowest body temperature during the surgery: normal temperature group (=246), mild hypothermia group (the lowest temperature ranged 35.0-35.9 ℃, =434), moderate hypothermia group (the lowest temperature ranged 34.0-34.9 ℃, =232) and severe hypothermia group (the lowest temperature<34 ℃, =96). The primary outcome was the incidence of intraoperative hypothermia. The four groups' difference of postoperative hospital stay, postoperative mortality within 30 days, postoperative pulmonary complications, postoperative hemorrhage/blood transfusion and acidosis were compared. Multivariate logistic regression was used to analyze the relationship between intraoperative hypothermia and prolonged postoperative hospital stay (>14 d), 30 d-mortality and other complications. In the 1 008 neonatal patients, 762 (75.6%) cases suffered intraoperative hypothermia, among which the incidence of mild, moderate and severe hypothermia was 43.1% (434/1008), 23.0% (232/1008) and 9.5% (96/1008), respectively. The postoperative hospital stay in normal, mild, moderate and severe hypothermia groups was 9.0 (5.8, 18.0), 12.0 (7.0, 21.0), 17.0 (10.0, 34.5) and 31.5 (12.5, 55.8) days. The mortality rate with 30 days after surgery was 2.9% (7/246), 4.4% (19/434), 6.9% (16/232) and 14.7% (14/96), the incidence of postoperative pulmonary complications was 31.7%(78/246), 39.9%(173/434), 44.8%(104/232) and 67.4%(64/96), the rate of postoperative hemorrhage/blood transfusion was 19.9%(49/246), 32.3%(140/434), 49.1%(114/232) and 79.0%(75/96), and the incidence of acidosis was 26.8%(66/246), 35.7%(155/434), 44.4%(103/232) and 46.3%(44/96), respectively. All differences were statistically significant (all <0.05). According to the adjusted logistic regression analysis, compared with the normal body temperature group, severe hypothermia was associated with prolonged postoperative hospital stay (=1.962, 95%: 1.063-3.619) and postoperative pulmonary complications (=2.020, 95%: 1.149-3.553). The mild, moderate and severe hypothermia group could increase the risk of postoperative blood/transfusion rate (mild: =1.690, 95%: 1.080-2.644; Moderate: =2.382, 95%: 1.444-3.927; Severe: =8.334, 95%: 3.123-8.929). The mild and moderate hypothermia could raise the risk of acidosis (mild: =1.458, 95%: 1.009-2.107; Moderate: =1.949, 95%: 1.279-2.972). Intraoperative hypothermia can prolong the postoperative hospital stay, and increase the risk of postoperative mortality, postoperative pulmonary complications, postoperative hemorrhage/transfusion, and acidosis.
Topics: Humans; Male; Female; Retrospective Studies; Hypothermia; Infant, Newborn; Prognosis; Postoperative Complications; Intraoperative Complications; Body Temperature; Incidence
PubMed: 38871472
DOI: 10.3760/cma.j.cn112137-20231120-01139