Ackee Atikutsetse (Unripe Hypoglycin A Jamaican Vomiting Sickness) kple Lychee (MCPG) Nutsitsidɔ ƒe Suklidɔ ƒe Tagbɔdɔléle le Ðeviwo me¶

Ɣetrɔ me ʋuʋudedi kpata, kuviawɔwɔ vevie, alɔ̃madɔmadɔ si bɔ, alo alɔ̃madɔmadɔ le ackee-kutsetse si metsi o ɖuɖu vɔ megbe (Blighia sapida) alo lychee ɖuɖu ( .Litchi chinensis) le dɔgbo ƒuƒlu me fiẽnuɖuɖu manɔmee nye lãmenugbagbeviwo ƒe dɔwɔwɔ kpata si xɔa ɣeyiɣi vevi aɖe si tsoa afɔku gã aɖe non-ketotic hypoglycemia (blood glucose often below 20 mg/dL or 1.1 mmol/L). Ƒo ka na wò nutoa me xexlẽdzesi kpata (911 le United States kple Canada, 112 le Europa Dukɔwo ƒe Habɔbɔ bliboa me, 999 le United Kingdom) alo nàbia míaƒe... dukɔwo dome xexlẽdzesi siwo ku ɖe nudzɔdzɔ kpatawo ŋu ƒe nyatakakadzraɖoƒe enumake, eye nàgblɔ na amesi ɖoe ɖa be dɔnɔa hiã be woawɔ ʋumesukli ƒe dodokpɔ kpata kple Dextrose 10% si wodona ɖe ʋuka me. Esi nèle kpekpeɖeŋunalawo lalam la, tsɔ míaƒe... status epilepticus ɣeyiɣi didi aɖe ƒe alɔ̃dɔdɔ ƒe ɖoɖowɔɖi, de vovototo lãmenugbagbeviwo ƒe dɔwɔwɔ ƒe kuxia kple esi atikewo hena vɛ dome sulfonylurea suklidɔ ƒe agbɔsɔsɔ si gbɔ eme, eye nàtsɔe awɔ dɔ le mɔ gbadza nu aɖi si nu sẽ ƒe kpekpeɖeŋunana gbãtɔ.

ackee (Jamaica dukɔa ƒe atikutsetse, si wodona geɖe le Caribbean, Ɣetoɖoƒe Afrika, Florida, kple Titina Amerika) kple lychee (kple longan kple rambutan, siwo nye tɔɖiayɔvi siwo le numiemiewo ƒe ƒomea me Sapindaceae) siaa me cyclopropyl amino acid protoxins sesẽwo le: Hypoglycin A (L-methylenecyclopropylalanine) kple eƒe homologue si le ete alpha-(methylenecyclopropyl)glycine (MCPG). Ne ɖevi alo ame tsitsi aɖe ɖu ackee arils siwo metsi o, siwo wozi ɖe wo dzi wòʋu (alo no tsi si me woɖa ackee le), alo ne ɖevi si mekpɔa nu nyui ɖuna o ɖua lychee siwo metsi o/siwo tsi afã kple afã fũ le ŋkeke me eye wòmlɔa anyi fiẽnuɖuɖu maɖumaɖu la, aɖi siawo nɔa anyi kpoo hena 4 to 12 hours esime ŋutilãa gakpɔtɔ dzra aklã me glycogen ɖo. Le ŋdi kanya (typically 3:00 AM to 8:00 AM), ne wonya vɔ le aklã me glycogen le zã me ko la, ŋutilãa dzea agbagba be yeatrɔ ɖe fatty acid beta-oxidation kple gluconeogenesis ŋu be sukli nanɔ ahɔhɔ̃a me. Elabena MCPA-CoA (tso Hypoglycin A me) kple MCPF-CoA (tso MCPG me) xea mɔ na mitochondrial medium-chain acyl-CoA dehydrogenase ( .MCAD) kple suklidɔlékuiwutikewo, ʋumesukli dzina ɖe edzi kabakaba va ɖoa zero esime aɖi ƒe kɔsɔkɔsɔ kpui ƒe lãmenugbagbeviwo yɔa ahɔhɔ̃a me, si wɔnɛ be ahɔhɔ̃a ƒe ʋuʋu kabakaba, status epilepticus, kple ku le eme 12 to 48 hours negbe ɖe 10% Dextrose (D10W) bolus kple sukli si ƒe agbɔsɔsɔme sɔ gbɔ ƒe dodo atraɖii naa ame.

+-------------------------------------------------------------------------+
|       SAPINDACEAE FRUIT HYPOGLYCEMIC ENCEPHALOPATHY ACTION MATRIX       |
+-------------------------------------------------------------------------+
|  THE OVERNIGHT GLYCOGEN TRAP (WHY CHILDREN CRASH AT 4:00 TO 7:00 AM):   |
|  * Afternoon/Evening: Child eats unripe Ackee OR eats Lychees & skips   |
|    dinner -> Liver glycogen stores run completely dry by 3:00-6:00 AM   |
|  * Hypoglycin A (MCPA-CoA) & MCPG (MCPF-CoA) block fatty acid beta-     |
|    oxidation (MCAD/SCAD) and Pyruvate Carboxylase gluconeogenesis       |
|  * Result: Blood glucose drops < 20 mg/dL (< 1.1 mmol/L) + Toxic        |
|    organic acidemia -> Sudden vomiting, seizures, cerebral edema, coma  |
+-------------------------------------------------------------------------+
|  IMMEDIATE PRE-HOSPITAL & EMERGENCY DEPARTMENT RESCUE:                  |
|  1. Awake & Able to Swallow: Give 15-30 g fast oral sugar (sugar water, |
|     honey, fruit juice, glucose gel) immediately + complex carbs        |
|  2. Drowsy, Vomiting, or Seizuring: NEVER pour liquid in mouth; apply   |
|     buccal sugar/glucose gel inside cheek while awaiting EMS            |
|  3. Hospital IV Bolus: 2 to 5 mL/kg of 10% Dextrose (D10W) IV push      |
|  4. Continuous IV Infusion: 10% Dextrose at 6 to 8 mg/kg/minute (avoid  |
|     hypotonic fluids that worsen cerebral edema) + L-Carnitine/Glycine  |
+-------------------------------------------------------------------------+

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Wɔ esia fifia¶

1. Ƒo ka na dɔwɔƒe siwo kpɔa nudzɔdzɔ kpatawo gbɔ enumake eye nàbia kpekpeɖeŋunalawo ƒe dɔwɔƒe si wodo Dextrose ɖe ʋuka me. Ƒo ka na 911 (U.S. kple Canada), 112 (European Union), 999 (United Kingdom), 119 / 911 (Jamaica kple Caribbean), alo 108 / 112 (India). Gblɔ na amesi ɖoe ɖa be dɔnɔa ɖu ackee alo lychee atikutsetse eye wòle fu kpem le acute toxic hypoglycemia kple metabolic encephalopathy.
2. Ne amea nyɔ bliboe eye wòte ŋu mia nu dedie la, na sukli si wozãna kabakaba le nu me enumake. Tsɔe ƒu gbe 2 to 3 tablespoons (30 to 45 grams) ƒe kplɔ̃dzisukli, anyitsi, blitsi, alo sukli-mi de tsi alo atikutsetse me tsi kplu ɖeka me eye nàna amea nanoe enumake. Ne ame aɖe kpɔe dze sii be ɖeko yewoɖu ackee si metsi o alo ɖevi aɖe ɖu lychees le ƒodo ƒuƒlu me eye wògasena le eɖokui me kokoko be yeƒe lãme sẽ keŋkeŋ hã la, nuɖuɖu si me carbohydrate sɔ gbɔ ɖo kple aha si me sukli le nana enumake nana aklã me glycogen ƒe nudzraɖoƒewo yɔa fũ eye wòxea mɔ na ŋutilãa be wòagawɔ ami-acid oxidation mɔ si xe la ŋudɔ esime nèle ʋu kum yina kɔdzi o.
3. Ne amea le alɔ̃ dɔm, tɔtɔ, eƒe ʋu le ʋuʋum, alo eƒe lãme le ʋuʋum la, MÈGAKƆ tsi ɖe eƒe nu me GBEÐE o. Mia dɔnɔa ɖe woƒe miame (afisi wòle be wòahaya le) be ʋuʋudedi nagage ɖe lãkusi me o. Àte ŋu atsɔ glucose gel, anyitsi, alo kplɔ̃dzisukli sue aɖe asisi ɖe ŋgonu ƒe akpa si le ete kple aɖuɖɔtoe ƒe eme tẽ (buccal mucosa), gake xɔname si ŋu kakaɖedzi le bia Dextrose 10% si wodona ɖe ʋuka me (D10W) si kpekpeɖeŋunalawo alo dɔwɔƒe si kpɔa nudzɔdzɔ kpatawo gbɔ ƒe dɔwɔlawo kpɔa edzi.
4. Se nusitae Glucagon Dodo na ame MEWƆA dɔ le Ackee alo Lychee ƒe aɖi me o gɔme. Ele be ƒomewo alo kpekpeɖeŋunala siwo tsɔa glucagon injection kits kpata la nanya be glucagon wɔa dɔ ko to aklãa ƒe ʋuʋu be wòagbã glycogen si wodzra ɖo me (glycogenolysis) eye wowɔa sukli yeye ( .gluconeogenesis). Le ackee kple lychee tagbɔdɔléle me la, aklã me glycogen le xoxo 100% depleted eye gluconeogenesis nye nugbagbeŋusẽ si Hypoglycin A kple MCPG xe mɔ na, si fia be glucagon mewɔa dɔ kura o. Exogenous dextrose (glucose) dodo tẽ koe xɔa ahɔhɔ̃a ɖe agbe.
5. Dzra ackee-ku siwo susɔ alo esiwo woɖa, nuɖatsi, alo lychee-kutsetse ɖesiaɖe ɖo. Mègana ame bubu aɖeke le aƒea me naɖu nu tso ze ɖeka me o, vevietɔ nuɖatsi (pot water) alo nuɖuɖuvivinu si me woɖaa ackee arils le, esime Hypoglycin A si me tsi le la tsia tsia me tẽ.

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Nyametsotsoti: Ackee kple Lychee suklidɔ ƒe tagbɔdɔléle ƒe vovototodedeameme¶

flowchart TD
    A["Wosusu be Ackee si Metsi O alo Lychee si Wotsi Nutsitsidɔ ɖum"] --> B{"Assess Dɔnɔdzikpɔƒe ƒe Nɔnɔme & Sidzedze"}
    B -->|"Dzesi aɖeke mele eŋu o (Emie Nyitsɔ laa, Nyɔ Bliboe) ."| C["Na Nuɖuɖu Si Me Carbohydrate Le Enumake + Glucose Nono si Wono Be Aklã me Glycogen Nanɔ Asi"]
    C --> D["Woaxɔe ɖe Kɔdzi hena Gaƒoƒo 24-48 Madzudzɔmadzudzɔe 10% Dextrose Infusion & Gaƒoƒo sia Gaƒoƒo Fingerstick Glucose Dodokpɔ"]
    B -->|"Aɖuɖɔtoe Kpata, Fifia, Kuviawɔwɔ, Alɔ̃madɔmadɔ, alo Alɔ̃madɔmadɔ"| E["Kpɔ Stat Point-of-Care Ʋumesukli (Zi geɖe < 20-30 mg/dL / 1.1-1.7 mmol/L) + Ʋu me Gas/Lactate"]
    E --> F["IV Bolus Enumake: Dextrose 10% (D10W) 2 va ɖo 5 mL/kg IV Push (Ame tsitsiwo: D50W 50 mL alo D10W 250 mL)"]
    F --> G["Dze Dextrose 10% (D10W le 0.9% NaCl me) dodo ɖe lãme na ame si ayi edzi gɔme le Glucose Infusion Rate (GIR) si nye 6 va ɖo 8 mg/kg/min me"]
    G --> H{"Active Status Epilepticus alo Ahɔhɔ̃ me Ʋuʋudedi ƒe Dzesiwo?"}
    H -->|"Ɛ̃"| I["IV Lorazepam/Midazolam + Do Ta ɖe dzi Digri 30 + 3% Hypertonic Saline (Ƒo asa na Hypotonic Lluids)"]
    H -->|"Ao"| J["Lé Ʋumesukli ƒe Agbɔsɔsɔme me ɖe asi 100-150 mg/dL (5.5-8.3 mmol/L); Tsɔ L-Carnitine + Glycine + Riboflavin (Vitamin B2) si wodona ɖe lãme na ame la de eme."]
    I --> J

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Dɔlélenutsiŋutete: Alesi Hypoglycin A kple MCPG xea mɔ na amegbetɔ ƒe ŋusẽ ƒe asitɔtrɔ¶

Be woase nusita ɖevi si le lãmesẽ me ate ŋu amlɔ anyi alɔgbɔnukoko le lychees alo ackee ɖuɖu vɔ megbe eye wòanyɔ le... 5:00 AM le alɔ̃madɔmadɔ si wua ame me la, ele be atikewɔlawo nalé ŋku ɖe mitochondrial enzyme ƒe mɔxexe si tututu Hypoglycin A kple MCPG (si woyɔna zi geɖe be acquired glutaric acidemia type II alo acquired multiple acyl-CoA dehydrogenase deficiency [MADD]).

1. Protoxins ƒe tɔtrɔ zu CoA thioesters siwo wua ame¶

Hypoglycin A (L-alpha-amino-beta-[methylenecyclopropyl]propionic acid) alo MCPG (alpha-[methylenecyclopropyl]glycine) nye aɖi tẽ le eƒe amino acid ƒuƒu ƒe nɔnɔme me. Ne wonya xɔ wo tso dɔgbo suea me ko la, wogena ɖe mitochondrial branched-chain amino acid catabolic pathway me: * Cytosolic kple mitochondrial transaminases trɔa Hypoglycin A wòzua methylenecyclopropylpyruvic acid (MCPP), kple alɔdze-kɔsɔkɔsɔ alpha-keto acid dehydrogenase oxidative decarboxylates wòzua aɖi wuame si le dɔ wɔm: methylenecyclopropylacetyl-CoA (MCPA-CoA). * Nenema ke MCPG (si bɔ ɖe lychee nuku siwo metsi o/siwo tsi afã kple afã me, kpakple ackee nukuwo me) trɔna zua methylenecyclopropylformyl-CoA (MCPF-CoA).

+-------------------------------------------------------------------------+
|       MITOCHONDRIAL BLOCKADE BY HYPOGLYCIN A (MCPA-CoA) & MCPG          |
+-------------------------------------------------------------------------+
|                                                                         |
|   Unripe Ackee / Lychee  --->  Hypoglycin A & MCPG                      |
|                                       |                                 |
|                                       v  (Transamination & CoA binding) |
|                            MCPA-CoA  and  MCPF-CoA                      |
|                                       |                                 |
|         +-----------------------------+-----------------------------+   |
|         |                             |                             |   |
|         v                             v                             v   |
|  1. BLOCKS FATTY ACID          2. BLOCKS GLUCONEOGENESIS     3. TOXIC   |
|     BETA-OXIDATION:               * Depletes Free CoA-SH        ACIDS:  |
|     * Irreversibly inactivates      and Carnitine               * Accum.|
|       FAD cofactor of MCAD,       * Depletes Acetyl-CoA &         of C4 |
|       SCAD, IVD, and GCDH           NADH needed to activate       to C10|
|     * Zero ketones produced         Pyruvate Carboxylase          acids |
|                                                                         |
+-------------------------------------------------------------------------+
|   RESULT ONCE OVERNIGHT LIVER GLYCOGEN RUNS OUT (3:00 TO 7:00 AM):      |
|   * Profound Non-Ketotic Hypoglycemia (< 20 mg/dL / 1.1 mmol/L)         |
|   * Volatile short-chain fatty acids (isovaleric, butyric, glutaric,    |
|     hexanoic) cross blood-brain barrier -> Vomiting, Coma, Brain Edema  |
+-------------------------------------------------------------------------+

2. Nusitae ami ƒe beta-oxidation kple gluconeogenesis siaa do kpo nu¶

1. Ameɖokuiwuwu ƒe mɔxexe ɖe Flavin nu (FAD) Acyl-CoA Dehydrogenases: MCPA-CoA kple MCPF-CoA wɔa dɔ abe mɔnu si womate ŋu atrɔ o ("ameɖokuiwuwu") mɔxenu siwo xea mɔ na flavin adenine dinucleotide (FAD) ametia ƒe ƒuƒoƒo si le mitochondrial kɔsɔkɔsɔ kpui acyl-CoA dehydrogenase ( .SCAD), acyl-CoA dehydrogenase si ƒe kɔsɔkɔsɔ le titina (MCAD), isovaleryl-CoA dehydrogenase ƒe dɔlékuiwutike (IVD), kple glutaryl-CoA dehydrogenase (GCDH). Esi wònye be ŋutilãa megate ŋu wɔa oxidation na ami siwo le nutsitsidɔ me o ta la, wozia lãmekawo dzi be woatɔ dzo sukli le dzodzodzoetsitsi me esime ketone body synthesis (acetoacetate kple beta-hydroxybutyrate) nye esi woxe mɔ na keŋkeŋ.
2. Aklã me Gluconeogenesis ƒe nutsitsi: Ne aklã me glycogen vɔ ko (4 to 10 hours le nuɖuɖu mamlɛtɔ megbe, kabakaba ŋutɔ le ɖevi sue siwo ƒe ahɔhɔ̃ kple aklã ƒe kpekpeme sɔ gbɔ alo nunyiame si le wo si me mekɔ o me la, aklãa dzea agbagba be yeawɔ sukli yeye tso lactate, alanine, kple glycerol me to gluconeogenesis dzi. Ke hã, gluconeogenesis ƒe enzyme gbãtɔ si ɖoa seɖoƒe na agbɔsɔsɔ, pyruvate carboxylase, hiã acetyl-CoA abe allosteric activator si nye agbanɔamedzi kple ATP + NADH (si wowɔ to fatty acid beta-oxidation me) be wòate ŋu awɔ dɔ. Tsɔ kpe ɖe eŋu la, acyl-CoA esters siwo wometrɔ asi le o kple... MCPA-CoA sequester katã ablɔɖe mitochondrial Coenzyme A ( .CoA-SH) kple L-carnitine. Ne womewɔe o ATP, . NADH, . acetyl-CoA, alo femaxee CoA-SH, aklã me suklidɔ ƒe dzɔdzɔ nu tsona keŋkeŋ.
3. Neurotoxic Branched kple Short-Chain Organic Acids ƒe Ƒoƒu: Beta-oxidation kple amino acid catabolism si woxe mɔ na hea ami siwo ƒe kɔsɔkɔsɔ kpui kple titina siwo me dzoxɔxɔ le (butyric, isobutyric, isovaleric, 2-methylbutyric, glutaric, adipic, suberic, kple sebacic acids) ƒe ƒuƒoƒo gã aɖe vɛ. Organic acid siawo xea mɔ na ahɔhɔ̃a ƒe titina lãmekawo ƒe pɔmpi siwo kpɔa ame ta, woʋãa chemoreceptor trigger zone le ahɔhɔ̃a ƒe ke me (si hea ʋuʋudedi si wóna kpata vɛ), woɖea ahɔhɔ̃mekawo ƒe mitochondria ɖa, eye wowɔa cytotoxic ahɔhɔ̃ me ʋuʋu le wo ɖokui si le ʋumesukli si bɔbɔ ŋutɔ me.

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Jamaicatɔwo ƒe Ʋuʋudedi (Ackee) kple Nutsitsidɔ ƒe Lychee Tagbɔdɔléle tsɔtsɔ sɔ kple wo nɔewo¶

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Kɔdzi ƒe afɔɖeɖe kpata ƒe ɖoɖowɔɖi si wowɔna afɔɖeɖe ɖesiaɖe¶

Ne ɖevi alo ame tsitsi va ɖo kple ʋuʋudedi, susu ƒe nɔnɔme si trɔ, alo alɔ̃madɔmadɔ le ackee alo lychee ɖuɖu vɔ megbe la, aɖabaƒoƒo ɖesiaɖe si suklidɔ ƒe agbɔsɔsɔ si womewɔ atike na o kple organic acidemia dzia afɔku si le ahɔhɔ̃a ƒe gbegblẽ tegbee si nye hypoxic-ischemic alo ahɔhɔ̃mekawo ƒe ʋuʋu si wua ame la ɖe edzi.

1. Dextrose 10% Dodo Ðe Ʋuka Me Enumake (D10W) Gbugbɔgafɔ¶

Mègalala be nudokpɔƒe ƒe atikeŋutinunya ƒe akpawo natrɔ ava hafi ana dextrose o; wɔ asibidɛ ƒe asibidɛ ƒe ʋumesukli ƒe dodokpɔ enumake (eye nàta ʋuka si le etame dzĩ/amadede dzẽ/aɖabɛ dzĩ hena aɖiŋutinunya/acylcarnitine ƒe nɔnɔmetatawo wɔwɔ ne anya wɔ, evɔ màhe atikewɔwɔ ɖe megbe gɔ̃ hã o 60 seconds).

• Nukatae Dextrose 10% (D10W) nyo wu Dextrose 25% alo 50% le ɖeviwo me: Woƒe agbɔsɔsɔme 50% dextrose (D50W, . 2,525 mOsm/L) nye hyperosmolar vevie eye wòhea venous sclerosis sesẽ vɛ, lãmenugbagbeviwo ƒe necrosis ne woɖee do goe, kple osmotic ƒe tɔtrɔ kabakaba si me afɔku le to ɖeviwo ƒe ʋu kple ahɔhɔ̃ ƒe mɔxenu si ŋu woate ŋu adze afɔku le dzi. Dzidzenu 5% dextrose (D5W) metsɔa sukli si sɔ gbɔ be wòaxe mɔ ɖe ami siwo le lãme na ame ƒe ʋuʋu nu o. 10% Dextrose (D10W, . 100 mg of glucose per mL) nye sika ƒe dzidzenu na bolus gbãtɔwo kple beléle na wo atraɖii ƒe atikewo siaa.
• IV Bolus Dose Gbãtɔ:
• Ðeviwo: Na 2 to 5 milliliters per kilogram (2 to 5 mL/kg, si naa nu 0.2 to 0.5 grams/kg ƒe sukli) ƒe 10% Dextrose (D10W) si wodona ɖe ʋuka me dzi 2 to 5 minutes.
• Ame tsitsiwo: Na 250 milliliters ƒe 10% Dextrose (D10W) (alo 50 milliliters ƒe 50% Dextrose ƒe dɔlékuiwutike [ .D50W]) si wodona ɖe ʋuka me.
• Sedziwɔwɔ Madzudzɔmadzudzɔe High-Rate 10% Dextrose ƒe Dɔlékuiwutike (To Shut Down Lipolysis):
• Lãmesẽŋutinunya ƒe se vevi: Dextrose ƒe bolus ɖeka doa ʋumesukli ɖe dzi hena ko 20 to 30 minutes. Nusi gavloe wue nye be le Hypoglycin A kple MCPG aɖi me la, dextrose-dɔdamɔnu ƒe taɖodzinue nye menye be woana ʋumesukli nanɔ anyi ko o 70 mg/dL, gake be wòaʋã insulin ƒe dodo si sɔ gbɔ le lãmenugbagbeviwo me be atsi lipase si sea lãmetsiŋusẽ nu le ami me la keŋkeŋ. Zi alesi ŋutilãa dzea agbagba be yeaƒo ami siwo me ami mele o nu ƒu tso amidzraɖoƒewo ko la, aɖi le eme MCPA-CoA, . MCPF-CoA, eye kɔsɔkɔsɔ kpui siwo me lãmenugbagbeviwo le siwo meʋãna o yi edzi le ƒoƒom ɖe mitochondria me eye wole aɖi dem ahɔhɔ̃a me.
• Akɔntabubu ƒe Agbɔsɔsɔme (Glucose Infusion Rate [GIR]): Dze ** ƒe ʋuka me dodo atraɖii gɔme enumake.10% Dextrose le 0.9% Saline si sɔ (D10NS)** (alo D10 1/2NS kple potassium si sɔ ne wonya ɖo kpe ayiku ƒe dɔwɔwɔ dzi ko) le Glucose Infusion Rate (GIR) ƒe 6 to 8 milligrams per kilogram per minute (roughly 3.6 to 4.8 mL/kg/hour ƒe D10W), titrating be serum glucose nanɔ ** dome.100 and 150 mg/dL (5.5 to 8.3 mmol/L)** na ** ya teti.24 to 48 hours**.

2. Metabolic Cofactor kple Conjugation Xɔname (L-Carnitine, . Glycine, kple Riboflavin) .¶

Elabena MCPA-CoA kple MCPF-CoA mɔ̃ ɖe mitochondrial Coenzyme A si le ablɔɖe me katã (CoA-SH) kple carnitine esime wòle flavin adenine dinucleotide ( .FAD) cofactors, clinical toxicology protocols zãa metabolic adjunct etɔ̃ siwo woɖo taɖodzinu na tsɔ kɔa aɖi domedomenuwo ɖa:

1. L-Carnitine si wodona ɖe ʋuka me alo esi wodona ɖe nu me (Levocarnitine):
2. Mɔnu: L-carnitine kpeɖeŋutɔ blana mitochondrial si woxe MCPA-CoA, . MCPF-CoA, isovaleryl-CoA, kple acyl-CoA molecule siwo ƒe kɔsɔkɔsɔ le titina to carnitine acyltransferase dzi, si trɔa wo wozua acylcarnitine esters siwo me tsi le (MCPA-carnitine) siwo woɖena doa goe le mitochondria me eye woɖea wo doa goe le aɖuɖɔ me, si naa ablɔɖe veviwo CoA-SH na Krebs ƒe tsatsam.
3. Agbɔsɔsɔme: 100 milligrams per kilogram per day (100 mg/kg/day) to ʋuka me (alo to nu me ne wonyɔ) mama ɖesiaɖe 6 to 8 hours (up to 2 to 3 grams/day).
4. Glycine si wodona le nu me alo esi wodona le dɔgboa me:
5. Mɔnu: Mitochondrial enzyme glycine N-acyltransferase ƒoa ƒu MCPA-CoA kple MCPF-CoA kple amino acid glycine be woawɔ MCPA-glycine kple MCPF-glycine siwo me aɖi mele o, siwo ayikuwo kɔna kabakaba.
6. Agbɔsɔsɔme: 150 to 250 milligrams per kilogram per day to nu me alo to nasogastric tube dzi ma ɖesiaɖe 6 to 8 hours.
7. Riboflavin si ƒe agbɔsɔsɔme sɔ gbɔ (Vitamin B2):
8. Mɔnu: Elabena MCPA-CoA covalently nana be womewɔa dɔ o FAD acyl-CoA dehydrogenases ƒe ƒuƒoƒo si wotsɔ ŋutilã wɔe, si naa Riboflavin ƒe agbɔsɔsɔ gã (the direct precursor of FAD and FMN, ** .100 milligrams to nu me alo to NG tube dzi zi 2 va ɖo 3 gbesiagbe**) nana active ƒe gbugbɔgadzɔ kabakaba MCAD kple SCAD enzymewo ƒe dɔwɔwɔ.

3. Alɔ̃madɔmadɔ, Ahɔhɔ̃mekawo ƒe Ʋuʋudedi, Kple Acidosis Dzi Ðuɖu¶

• Alɔ̃madɔmadɔ dzi ɖuɖu: Suklidɔ ƒe dzidziɖedzi ɖɔɖɔɖo kple 10% Dextrose tɔa te ʋuʋudedi enumake le dɔnɔ geɖe siwo va kaba la ŋu. Ne alɔ̃madɔmadɔ gakpɔtɔ li togbɔ be sukli si le ʋu me va sɔ hã (due to already-established cerebral edema or organic acid neurotoxicity), do lorazepam ( .0.1 mg/kg IV), midazolam ( .0.1 to 0.2 mg/kg IV/IM), alo levetiracetam ( .Keppra, . 20 to 40 mg/kg IV) (strictly avoid Valproic Acid [Depakote], which itself sequesters carnitine, inhibits mitochondrial beta-oxidation, and worsens hyperammonemia and cerebral edema).
• Ahɔhɔ̃ me ʋuʋu dzikpɔkpɔ: Do aba ƒe ta ɖe dzi va ɖo 30 degrees, lé normocarbia (PaCO2 35 to 40 mmHg) kple endotracheal intubation kaba ne Glasgow Coma Scale (GCS) nye < 8, eye nàde 3% Hypertonic Saline (3 to 5 mL/kg IV wu enu 15 to 20 minutes) alo Mannitol ( .0.5 g/kg IV).
• Sodium Bicarbonate: Na lãmenugbagbeviwo ƒe acidosis sesẽ (arterial pH < 7.15), do sodium bicarbonate ɖe ʋuka me ŋuɖɔɖɔɖotɔe esime nèle sukli dodo ɖe lãme na ame atraɖii me.

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Mɔxexe ɖe enu: Alesi woawɔ anya Ackee si tsi nyuie si le dedie tso Ackee si metsi o si me aɖi le gbɔ, kple Lychee ƒe se siwo le dedie na ɖeviwo¶

1. Sikase 4 siwo ku ɖe Ackee Dzadzraɖo Dedie Ŋu¶

+-------------------------------------------------------------------------+
|         VISUAL GUIDE: TOXIC UNRIPE vs. SAFE RIPE ACKEE FRUIT            |
+-------------------------------------------------------------------------+
|  TOXIC UNRIPE ACKEE (DO NOT EAT - 1,000 ppm HYPOGLYCIN A):              |
|  [ (=======) ]  Pod is completely closed, green, or only slightly split |
|                 Forced open with a knife or cracked by hitting ground   |
|                 Arils are hard, pale cream, or small seeds are soft/red |
+-------------------------------------------------------------------------+
|  SAFE NATURALLY RIPE ACKEE (< 10 ppm HYPOGLYCIN A WHEN CLEANED):        |
|    /   |   \    Pod turned bright red/yellow-orange and YAWNED WIDE     |
|   | (O)|(O) |   OPEN naturally on the tree branch in full sunlight      |
|    \   |   /    3 large glossy black seeds are fully exposed to view    |
|                 Yellow aril flesh is firm-soft; remove all seeds & pink |
|                 membrane, wash thoroughly, and DISCARD BOILING WATER    |
+-------------------------------------------------------------------------+

1. Na pod la "yawn" naʋu le dzɔdzɔme nu le ati la dzi: Mègaŋe alo ɖa ackee pod si memã le dzɔdzɔme nu o esime wògakpɔtɔ le ati la dzi gbeɖe o. Mègazi atikutsetse si wotu dzi be wòaʋu gbeɖe o, eye mègagblẽ atikutsetse siwo wotu ɖe fesre alo xɔta si dzi ŋdɔ ʋuna le la dzi gbeɖe be woanɔ lalam be woaʋu tso ati la dzi o (aŋɔ siwo woɖe ɖe vovo meɖea Hypoglycin A tso arils me yia nukuawo me nyuie o).
2. Ðe nuku ɖesiaɖe kple pink/red membrane ƒe dzesi ɖesiaɖe ɖa (raphe): Nuku yibɔ siwo le keklẽm kple ka si le abe pink-dzẽ ene si léa aril ɣia ɖe nukuwo ƒe tsizea ŋu la gakpɔtɔ nye aɖi tegbee (containing high levels of Hypoglycin A and B) le atikutsetse siwo tsi bliboe gɔ̃ hã me. Tsɔ lãgbalẽ pink bliboa ƒu gbe eye nàklɔ aril ɣiawo nyuie le tsi fafɛ si le sisim me hafi nàɖae.
3. Ƒoe ɖe vovo eye nàtsɔ nuɖatsi la aƒu gbe ƔESIAƔI: Tsɔ ackee arils siwo ŋu wokɔ la ɖa le tsi dzro me gbã, nàɖe wo le colander me, eye kɔ tsi si le ƒoƒom la ɖe tsiléƒea tẽ. Mègaɖa bli, yam, akɔɖu, dumplings, alo mɔli le tsi ma ke si wozãna tsɔ ɖaa ackee la me gbeɖe o.
4. Ackee siwo wotsɔ tso gaze me ƒe dedienɔnɔ: Le United States, Canada, UK, kple EU la, US FDA kple nuɖuɖu ƒe dedienɔnɔ ŋuti dɔwɔƒewo léa ŋku ɖe ackee siwo wotsɔ gaze me siwo wotsɔ tso duta vɛ le asitsatsa me ŋu vevie be woakpɔe ɖa be Hypoglycin A ƒe agbɔsɔsɔme le sue wu 100 parts per million (ppm). Ne èzã ackee si wotsɔ gaze wɔe si wodzrana gɔ̃ hã la, ɖe tsi le tsi fafɛa me eye nàklɔe hafi nàtsɔ dzetsi atɔ dzoe.

2. Dukɔa ƒe Lãmesẽnyawo Ŋuti Se 3 Siwo Wu Ðeviwo ƒe Lychee Tagbɔdɔléle ƒe Dɔvɔ̃wo Nu¶

Le numekuku ɖedzesi siwo India Dukɔa ƒe Dɔlélenutsitsi Dɔwɔƒe (NCDC) kple U.S. CDC wɔ ɖekae le Muzaffarpur, Bihar (afisi ɖevi alafa geɖe kuna le May kple June ɖesiaɖe le lychee ŋeŋeɣi) megbe la, dukɔa ƒe lãmesẽnyawo gbɔ kpɔlawo de ƒome ƒe se bɔbɔe etɔ̃ siwo ɖe ku dzi kpɔtɔ wu 80%: 1. Mègana ɖeviwo namlɔ anyi gbeɖe ne mèɖu fiẽnuɖuɖu si woɖa (fiẽnuɖuɖu) o: Fiẽnuɖuɖu si sɔ si me carbohydrate sɔ gbɔ ɖo ɖuɖu nana aklã me glycogen ƒe nudzraɖoƒewo gayɔa aklã me ale be mehiã be ɖevia ƒe aklã naɖo ŋu ɖe ami si woxe mɔ na beta-oxidation ŋu le zã ɖeka me gbeɖe o. 2. Ðe seɖoƒe na lychee siwo ɖevi suewo ɖuna ƒe agbɔsɔsɔ, eye nàƒo asa na atikutsetse siwo metsi o/siwo ge dze anyi: Mègana ɖeviwo naɖu lychee siwo metsi o alo esiwo tsi afã kple afã tso anyigba le atikutsetsebɔwo me o, elabena MCPG kple Hypoglycin A si le atikutsetse dzĩ siwo tsi bliboe me la sɔ gbɔ wu zi gbɔ zi eve le lychees siwo metsi o me. 3. Na sukli si woano enumake eye nàƒu du ayi atikewɔƒe si me Dextrose 10% le le ŋdi ƒe dzesi gbãtɔ me: Ele be ɖevi ɖesiaɖe si le lychee alo ackee nuto me si nyɔ kple ʋuʋudedi, kuviatɔ, alo ʋuʋudedi la naxɔ 10% Dextrose le gaƒoƒo gbãtɔ me (administering D10W within 4 hours of symptom onset reduces fatality from > 44% to < 5%).

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Nutome ƒe nudzɔdzɔ kpata kple aɖi dzi ɖuɖu ƒe ɖoɖowo¶

• Jamaica kple Caribbean: Ƒo ka na 119 (Jamaica Ambulance / Police) alo 911 / 999 (le Caribbean-nyigbawo dzi) eye nàƒu du enumake ayi kɔdzi ƒe dɔwɔƒe si te ɖe ŋuwò kpata be woadoe ɖe ʋuka me 10% Dextrose ƒe atike. Jamaica ƒe Lãmesẽnyawo Kple Dzedzeme Dɔwɔƒe léa tsitretsitsi sesẽ Ackee Poisoning (Jamaican Vomiting Sickness) gbɔgbɔyɔyɔ ƒe ɖoɖowo me ɖe asi le sɔlemeha la ƒe kɔdziwo kple lãmesẽdɔwɔƒewo katã.
• United States kple Canada: Ƒo ka na 911 enumake eye nàƒo ka na 1-800-222-1222 (US Aɖi Kpekpeɖeŋunana) alo 1-844-POISON-X (1-844-764-7669 le Canada). Ackee yeyewo tsina le South Florida (where trees are occasionally harvested by residents unfamiliar with the "natural yawn" rule), eye wodzraa ackee si wofa tsikpe/gaze me le Caribbean ƒe asiwo me le Dziehe Amerika katã.
• India, Vietnam, Bangladesh, kple Anyieheɣedzeƒe Asia: Ƒo ka na 108 alo 112 (India), 115 (Vietnam), alo 999 (Bangladesh/Malaysia). Le May va se ɖe July lychee (litchi), longan, kple rambutan nuŋeɣi, lãmesẽdɔwɔƒe gbãtɔ siwo le Bihar (India) kple dziehe Vietnam dzraa suklidzidzenu siwo le aba dzi eye 10% Dextrose kotokuwo koŋ na ɖeviwo ƒe suklidɔ ƒe tagbɔdɔléle ɖeɖeɖa enumake.
• United Kingdom, Europa Dukɔwo ƒe Habɔbɔ, kple Ɣetoɖoƒe Afrika: Yɔ 999 (UK), 112 (EU), alo 112 / 199 (Nigeria, Ghana, Côte d'Ivoire, Burkina Faso, afisi Blighia sapida tsina le fũ eye wòhea aɖi na ɖeviwo le ɣeyiɣi aɖe me si woyɔna be "ackee poisoning" alo "finsan" aɖi si le ame ŋu). Ele be atikewɔla siwo le UK/EU nayi NPIS / TOXBASE alo dukɔa ƒe aɖi dɔwɔƒewo hena aɖuɖɔtoe me acylcarnitine/organic acid ƒe dɔléle ŋuti kpeɖodzi kple... L-carnitine agbɔsɔsɔme si wozãna.

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Nya siwo wobiaa amewo enuenu¶

Nukatae zi geɖe la, ɖevi siwo lychee alo ackee siwo metsi o de aɖi le la dzea dɔ ŋdi kanya?¶

Hypoglycin A kple MCPG xea mɔ na mitochondrial enzymes siwo hiã be woatsɔ atɔ dzo ŋutilã me ami ahawɔ sukli yeye (gluconeogenesis), gake womexea mɔ na aklã be wòagaɖe glycogen si wodzra ɖo xoxo la ɖa o. Ne ɖevi ɖu ackee si metsi o alo ɖu lychees eye wòmlɔ anyi fiẽnuɖuɖu manɔmee la, woƒe aklã me glycogen si wodzra ɖo la nana woƒe ʋumesukli nɔa ɖoɖo nu gaƒoƒo 4 va ɖo 10 gbãtɔwo; le ŋdi ga 3:00 kple ŋdi ga 8:00 dome la, aklã me glycogen nu yina keŋkeŋ, si wɔnɛ be sukli si le ʋu me la ɖiɖina ɖe anyi wu 20 mg/dL esime ami ƒe domedomenuwo yɔa ahɔhɔ̃a me.

Nukatae Glucagon dodo kpata mewɔa dɔ na Ackee alo Lychee ƒe suklidɔ o?¶

Glucagon naa sukli si le ʋu me dzina ɖe edzi to sedede na aklã be wòagbã glycogen si wodzra ɖo eye wòawɔ sukli yeye to gluconeogenesis me ko. Le ackee kple lychee ƒe aɖi me la, dɔnɔa ƒe aklã me glycogen ƒuƒlu keŋkeŋ xoxo eye woƒe gluconeogenic enzymes nye biochemically locked to MCPA-CoA kple MCPF-CoA, si fia be glucagon mekpɔa ŋusẽ aɖeke ɖe edzi o; ele be dɔnɔa naxɔ sukli si woano tẽ (ne enyɔ) alo Dextrose 10% si wodona ɖe ʋuka me.

Nukatae Valproic Acid (Depakote) nye afɔku na alɔ̃madɔmadɔ si Ackee alo Lychee ƒe aɖi hena vɛ?¶

Valproic acid nye ami si ƒe kɔsɔkɔsɔ le alɔdzewo me si ŋutɔ ɖua mitochondrial L-carnitine, xea mɔ na ami ƒe beta-oxidation enzymes, eye wòdoa ʋu me ammonia ɖe dzi. Valproic acid nana dɔnɔ si ƒe beta-oxidation mɔ dzi aɖi le xoxo le Hypoglycin A alo MCPG me la, egblẽa nu le lãmenugbagbeviwo ƒe acidosis, hyperammonemia, kple ahɔhɔ̃mekawo ƒe ʋuʋu si wua ame ŋu ŋutɔ; ele be atikewɔlawo nazã benzodiazepines, levetiracetam (Keppra), alo phenobarbital kpe ɖe 10% Dextrose ŋu ɖe eteƒe.

Aleke nàwɔ anya ne Ackee atikutsetse yeye aɖe le dedie be woafɔe ahaɖae?¶

Ne ackee ƒe aŋgba yeye trɔ zu dzĩ si klẽna eye wòzu ɣie-aŋutiɖiɖi eye wòma wòʋu le dzɔdzɔme nu ("yawned") esime wògakpɔtɔ le ati ŋu le ŋdɔ me, si na nuku yibɔ etɔ̃ siwo le keklẽm la dze keŋkeŋ. Mègaɖa aŋe dama si womeʋu o gbeɖe o, mèzi aŋe aɖe dzi be wòaʋu kple hɛ gbeɖe o, ɖe nuku yibɔawo kple lãkusi bliboa si le pink-dzẽ la ɖa ɣesiaɣi, eye nàkɔ tsi si le ƒoƒom la ɖe tsidzɔƒea ɣesiaɣi.

Nukatae ɖɔktawo naa Dextrose IV si me wodea tsii si nye 10% la nɔa dɔ wɔm gaƒoƒo 24 va ɖo 48 ne sukli si le ʋu me trɔ gbɔ va ɖo eƒe nɔnɔme nyuitɔ gɔ̃ hã?¶

Glucose ƒe bolus ɖeka nana nana sukli si le ʋu me la nɔa anyi aɖabaƒoƒo 20 va ɖo 30 pɛ ko. Dextrose 10% dodo ɖe lãme na ame atraɖii (le 6 va ɖo 8 mg/kg/aɖabaƒoƒo me) nana ʋumesukli dzina ɖe edzi vie (100 va ɖo 150 mg/dL), si ʋãa dɔnɔa ŋutɔ ƒe insulin be wòaxe mɔ na ami ƒe gbagbã le ami me eye wòxea mɔ na ŋutilãa be wòagawɔ ahɔhɔ̃mekawo ƒe aɖi kpui ƒe enzyme acid geɖe wu vaseɖe esime aklãawo gahaya.

Ðe Hypoglycin A kple MCPG si tso atikutsetsewo me dea aɖi sɔwo kple avuwo hãa?¶

Ẽ, sɔ siwo ɖua gbe le lãnyiƒewo le adame kple kele me la kpea fu zi geɖe le nɔnɔme aɖe si wua ame kabakaba si woyɔna be Equine Atypical Myopathy (le Europa/UK) alo Seasonal Pasture Myopathy (le Dziehe Amerika) me enuenu ne woɖu aʋala ƒe nukuwo ("helicopter samaras") kple Sycamore Maple (Acer pseudoplatanus) kple Box Elder (Acer negundo) ƒe nukuwo vɔ megbe . atiwo, siwo le Sapindaceae ƒome ɖeka me kple ackee kple lychee eye Hypoglycin A sɔ gbɔ ɖe wo me.

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Be nàwɔ nu nyuie ɖe ɖeviwo, lãmenugbagbeviwo ƒe dɔwɔwɔ, kple aɖi ƒe nɔnɔme kpatawo ŋu la, bu kpekpeɖeŋunana gbãtɔ kple CPR ƒe hehexɔxɔ si woawɔ kple asi tso Atitsoga Dzĩ Habɔbɔ, St John Ambulance, alo nutoa me ƒe hehenaƒe si naa hehe kpata le nutoa me ŋu.

Mɔfiame siwo do ƒome kplii¶

• Sulfonylurea (Glipizide, Glyburide) ƒe agbɔsɔsɔme si gbɔ eme, Suklidɔ si gbugbɔna, kple Octreotide ƒe vovototodedeameme
• Status Epilepticus, Alɔ̃madɔmadɔ Ɣeyiɣi Didi, Kple Xɔnametikewo
• Alɔ̃madɔmadɔ: Kpekpeɖeŋu Gbãtɔ Kple Yameʋuɖoɖo Takpɔkpɔ
• Nuɖuɖu ƒe Aɖi, Dɔgbo ƒe Dɔléle Sesẽ, Kple Tsikuɖiɖi
• Aɖi Siwo Wozãna le Amewo Dome Kpekpeɖeŋu Gbãtɔ Kple Ðiƒoƒo Ŋu

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Dzɔtsoƒewo kple dziɖuɖumegãwo ƒe mɔfiame¶

• U.S.A. Ƒe 1999 me. Dɔlélenutsitsi kple Mɔxexe Ðe Enu Dɔwɔƒe (CDC) & Dukɔa ƒe Dɔlélenutsitsi Dɔwɔƒe (NCDC, India): Shrivastava A, et al.: Kadodo si le tagbɔdɔléle sesẽ si me aɖi le kple litchi ɖuɖu dome le dɔvɔ̃ aɖe si do mo ɖa le Muzaffarpur, India, 2014: numekuku si wowɔ le dɔlélewo dzi ɖuɖu ŋu (The Lancet Global Health).
• Jamaica & Caribbean Dukɔa ƒe Lãmesẽnyawo Gbɔkpɔha ƒe Lãmesẽnyawo Kple Dzedzeme Dɔwɔƒe (CARPHA): Dɔwɔƒe ƒe Mɔfiamewo na Ackee Aɖi (Jamaicatɔwo ƒe Ʋuʋudedi Dɔléle / Hypoglycin A Amumumu) (Kingston, Jamaica).
• MÍ. Nuɖuɖu kple Atikewo Dzikpɔƒe (FDA): Import Alert 21-11 & Compliance Policy Guide: Ackee si le gaze me alo esi wofa tsikpe me si me Hypoglycin A si wu 100 ppm le la léle ɖe asi (United States).
• Xexeame ƒe Lãmesẽ Habɔbɔ (WHO) / ​​FAO ƒe Eŋutinunyalawo ƒe Habɔbɔ si Kpɔa Nuɖuɖu Kpekpeɖeŋunuwo Gbɔ: Nuɖuɖu Me Ðiƒoƒo Aɖewo ƒe Dedienɔnɔ Ŋuti Numekuku: Hypoglycin A le Ackee Atikutsetse (Blighia sapida) me kple MCPG le Sapindaceae Atikutsetsewo me (Geneva).
• Sherratt HSA / Tanaka K, Isselbacher KJ: Hypoglycin, aɖi si le ackee atikutsetse si metsi o me: nugbagbeŋusẽ ƒe mɔnu si xea mɔ na beta-oxidation kple gluconeogenesis (Biochemical Pharmacology / New England Journal of Medicine).