" Many substances are potentially harmful to people with G6PD deficiency. Variation in response to these substances makes individual predictions difficult. Antimalarial drugs that can cause acute hemolysis in people with G6PD deficiency include primaquine, pamaquine, and chloroquine. There is evidence that other antimalarials may also exacerbate G6PD deficiency, but only at higher doses. Sulfonamides (such as sulfanilamide, sulfamethoxazole, and mafenide), thiazolesulfone, methylene blue, and naphthalene should also be avoided by people with G6PD deficiency as they antagonize folate synthesis, as should certain analgesics (such as phenazopyridine and acetanilide) and a few non-sulfa antibiotics (nalidixic acid, nitrofurantoin, isoniazid, dapsone, and furazolidone).[8][9][10] Henna has been known to cause hemolytic crisis in G6PD-deficient infants.[11] Rasburicase is also contraindicated in G6PD deficiency. High dose intravenous [vitamin C](https://en.wikipedia.o
... keep reading on reddit β‘Evaluation of effect of some corticosteroids on glucose-6-phosphate dehydrogenase and comparative study of antioxidant enzyme activities.
J Enzyme Inhib Med Chem. 2005 Feb;20(1):19-24.
Author: Ozmen I.
Thanks!
https://doi.org/10.4103/sja.sja_1041_20
https://pubmed.ncbi.nlm.nih.gov/34188645
Abstract
Medium-chain acyl-CoA dehydrogenase deficiency can cause symptoms resulting from Ξ²-oxidation disorder during preoperative fasting. Tight perioperative glucose monitoring is needed to avoid these symptoms. We report the first pediatric case using continuous tissue glucose monitoring devices. The patient was a 9-year-old boy with medium-chain acyl-CoA dehydrogenase deficiency for whom femoral osteotomy and selective muscle release of the hip and knee was planned to treat hip dislocation and joint contracture. To monitor tissue glucose levels continuously during preoperative fasting, a percutaneous sensor was attached to the right upper extremity, 2 days before the operation. Anesthetic management using Ringer's acetate containing 5% glucose, an inhalational agent, and epidural anesthesia without a muscle relaxant or propofol was performed without complications. The device achieved tight perioperative glucose monitoring. Continuous tissue glucose monitoring devices helped perioperative glucose monitoring of the pediatric patient with medium-chain acyl-CoA dehydrogenase deficiency.
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Open Access: True
Authors: Ito Hidekazu - Mizuno Shoji -
Additional links:
.
This is the best tl;dr I could make, original reduced by 27%. (I'm a bot)
> June 27 - Chinese scientists have discovered an enzyme that may prohibit lung cancer from creating secondary malignant tumors.
> Researchers from the Dalian Institute of Chemical Physics and the Shanghai Institute of Biochemistry and Cell Biology, both under the Chinese Academy of Sciences, found out that UDP-glucose 6-dehydrogenase impairs lung cancer metastasis, science journal Nature said in a report published yesterday.
> Metastasis is involved in 95 percent of all cancer-related deaths, the report said.
> China has over 730,000 new lung cancer cases each year, and the five-year survival rate is 16 percent.
> The study is a breakthrough in beginning to understand how lung cancer spreads.
> Previous research has pointed out the significance of abnormal cellular metabolisms in the human body as cancer cells proliferate but little is known about what catalyzes their growth.
Summary Source | FAQ | Feedback | Top keywords: cancer^#1 lung^#2 percent^#3 report^#4 metastasis^#5
Post found in /r/Futurology.
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https://doi.org/10.1016/j.braindev.2021.11.005
https://pubmed.ncbi.nlm.nih.gov/34863613
Abstract
BACKGROUND
Pyruvate dehydrogenase complex (PDHC) deficiency is an inborn error of metabolism that causes lactic acidosis and neurodevelopmental changes. Five causative genes have been identified: PDHA1, PDHB, DLAT, DLD, and PDHX. Four neurological phenotypes have been reported: neonatal encephalopathy with lactic acidosis, non-progressive infantile encephalopathy, Leigh syndrome, and relapsing ataxia. Of these, neonatal encephalopathy has the worst mortality and morbidity and there is no effective treatment.
SUBJECTS AND METHODS
We studied two girls who were clinically diagnosed with PDHC deficiency as neonates, they were subsequently found to have PDHA1 mutations. The clinical diagnosis was based on white matter loss and a lateral ventricular septum on fetal MRI, spasticity of the lower extremities, and lactic acidosis worsening after birth. Intravenous ketogenic diets were started within 24Β h after birth. The ketogenic ratio was increased until the blood lactate level was controlled, while monitoring for side effects.
RESULTS
In both cases, the lactic acidosis improved immediately with no apparent side effects. Both children had better developmental outcomes than previously reported cases, neither exhibited epilepsy.
CONCLUSIONS
Intravenous ketogenic diet therapy is a treatment option for neonatal-onset PDHC deficiency. Further studies are needed to optimize this therapy.
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Open Access: False
Authors: Takehiko Inui - Yoichi Wada - Moriei Shibuya - Natsuko Arai-Ichinoi - Yukimune Okubo - Wakaba Endo - Toshihiko Uchida - Noriko Togashi - Etsuo Naito - Kazuhiro Haginoya -
Additional links: None found
https://doi.org/10.1016/j.ymgmr.2021.100760
https://pubmed.ncbi.nlm.nih.gov/33996489
Abstract
Very long-chain acyl-coenzyme A dehydrogenase deficiency (VLCADD, OMIM 201475) is a congenital fatty acid oxidation disorder. Individuals with VLCADD should avoid catabolic states, including strenuous exercise and long-term fasting; however, such conditions are required when undergoing surgery. The perioperative management of VLCADD in infants has rarely been reported and details regarding the transition of serum biomarkers reflecting catabolic status have not been disclosed. Herein, we present the perioperative clinical and biological data of cryptorchidism in a 1.5-year-old boy with VLCADD. The patient was diagnosed through newborn screening and his clinical course was very stable. Genetic testing of ACADVL revealed compound heterozygous variants c.506 T > C (p.Met169Thr) and c.606-609delC (p.L216*). The enzyme activity of the patient with VLCAD was only 20% compared to that of healthy control. Left orchiopexy for the pediatric cryptorchidism was planned and performed at 1 and a half year of age. Induction anesthesia involved thiopental, fentanyl and rocuronium. The glucose infusion rate was maintained above 6.6 mg/kg/min starting the day before surgery until the operation was completed. Anesthesia was maintained with sevoflurane at approximately 2%. The serum concentration of tetradecenoylcarnitine were stable during the operation, ranging between 0.08 and 0.19 ΞΌM (cutoff <0.2 ΞΌM), and never deviated from the reference range. Concentration of other serum biomarkers including free fatty acid, 3-OH-butyrate, and creatine kinase, remained similarly unchanged. In this report, we describe the uneventful perioperative management of unilateral orchiopexy for left cryptorchidism in a 1.5-year-old boy with VLCADD using sufficient glucose infusion and volatile anesthesia.
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Open Access: True
Authors: Ryosuke Bo - Hiroyuki Awano - Kenji Yamada - Mayu Ooi - Yuichi Okata - Yuko Bitoh - Satoshi Mizobuchi - Kazumoto Iijima -
Additional links:
