I-Ackee Fruit (I-Hypoglycin Engavuthiwe I-Jamaican Vomiting Sickness) kanye ne-Lychee (MCPG) Ukuzila I-Hypoglycemic Encephalopathy Ezinganeni¶

Ukuhlanza ekuseni kungazelelwe, ukukhathala okujulile, ukuquleka okujwayelekile, noma ukukoma ngemva kokudla isithelo se-ackee esingavuthiwe (Blighia sapida) noma ukudla lychee (Litchi chinensis) esiswini esingenalutho ngaphandle kokudla kwakusihlwa kuyisimo esiphuthumayo esibucayi se-metabolic esibangelwa inhlekelele non-ketotic hypoglycemia (blood glucose often below 20 mg/dL or 1.1 mmol/L). Shayela inombolo yakho yasendaweni yezimo eziphuthumayo (911 e-United States nase-Canada, 112 ngaphesheya kwe-European Union, 999 e-United Kingdom) noma uxhumane nathi umkhombandlela wezinombolo eziphuthumayo zamazwe ngamazwe ngokushesha, futhi utshele umthumeli ukuthi isiguli sidinga ukuhlolwa okuphuthumayo kwe-glucose yegazi kanye nge-intravenous 10% Dextrose. Ngenkathi ulinde abezimo eziphuthumayo, lawula ukudlikizeka okusebenzayo usebenzisa eyethu isimo se-epilepticus prolonged seizure protocol, hlukanisa inkinga ye-metabolic naleyo ebangelwa imithi I-sulfonylurea hypoglycemic overdose, futhi usebenzise jikelele acute poisoning usizo lokuqala.

Kokubili i-ackee (isithelo sikazwelonke sase-Jamaica, esitshalwe kabanzi kulo lonke elase-Caribbean, eNtshonalanga Afrika, eFlorida, nase-Central America) kanye ne-lychee* (kanye ne-longan ne-rambutan, abazala bomndeni we-botanical Sapindaceae) aqukethe ama-protoxin anamandla e-cyclopropyl amino acid: Hypoglycin A (L-methylenecyclopropylalanine) kanye ne-homologue yayo ephansi ethi alpha-(methylenecyclopropyl)glycine (MCPG). Lapho ingane noma umuntu omdala edla ama- ackee arils angavuthiwe, avuleke ngenkani (noma ephuza amanzi obekubiliswe ngawo i- ackee), noma lapho ingane engondlekile idla ama-lychee angavuthiwe/angasevuthiwe emini futhi iyolala ngaphandle kokudla isidlo sakusihlwa, lobu buthi buhlala buthule 4 to 12 hours kuyilapho umzimba usagcine i-glycogen yesibindi. Ekuseni ngovivi (typically 3:00 AM to 8:00 AM), uma i-glycogen yesibindi isiphelile ebusuku, umzimba uzama ukushintshela ku-fatty acid beta-oxidation kanye ne-gluconeogenesis ukuze ugcine ubuchopho bunikezwa i-glucose. Ngoba MCPA-CoA (kusuka ku-Hypoglycin A) kanye ne-MCPF-CoA* (kusuka ku-MCPG) ivimba ngendlela engenakuhlehliswa i-mitochondrial medium-chain acyl-CoA dehydrogenase (MCAD) kanye nama-enzyme e-gluconeogenic, ushukela wegazi wehla ngokushesha ufinyelele kuqanda kuyilapho ama-acid e-organic chain short anoshevu egcwala ubuchopho, abangele ukuqubuka kobuchopho ngokushesha, isimo sokuwa, nokufa ngaphakathi. 12 to 48 hours ngaphandle uma ngokushesha ** 10% Dextrose (D10W) i-bolus kanye ne-glucose eqhubekayo yezinga eliphezulu** isetshenziswa.

+-------------------------------------------------------------------------+
|       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  |
+-------------------------------------------------------------------------+

Yenza lokhu manje¶

1. Shayela abezimo eziphuthumayo ngokushesha futhi ucele iyunithi yezimo eziphuthumayo ene-Dextrose efakwa emthanjeni. Dayela u-911 (US and Canada), 112 (European Union), 999 (United Kingdom), 119 / 911 (Jamaica and Caribbean), noma 108 / 112 (India). Tshela umthumeli ukuthi isiguli sidle isithelo se-ackee noma i-lychee futhi sihlushwa i-acute toxic hypoglycemia kanye ne-metabolic encephalopathy.
2. Uma umuntu ephapheme ngokuphelele futhi ekwazi ukugwinya ngokuphepha, mnike ushukela womlomo osebenza ngokushesha. Ncibilikisa 2 to 3 tablespoons (30 to 45 grams) ushukela wetafula, uju, isiraphu yommbila, noma impushana yeglucose engilazini yamanzi noma ijusi yezithelo bese ucela umuntu ukuthi ayiphuze ngokushesha. Ngisho noma othile eqaphela ukuthi usanda kudla i-ackee engavuthiwe noma ingane idle ama-lychees esiswini esingenalutho futhi isazizwa ijwayelekile ngokuphelele, ukunikeza ukudla okune-carbohydrate eningi nesiphuzo esinoshukela ngokushesha kugcina izitolo ze-glycogen zesibindi zigcwele futhi kuvimbela umzimba ekusebenziseni indlela evinjiwe ye-fatty-acid oxidation ngenkathi ushayela esibhedlela.
3. Uma umuntu ozela, edidekile, ehlanza, noma exhuzula, UNGALOKOTHI uthele uketshezi emlonyeni wakhe. Gingqa isiguli ohlangothini lwaso lwesobunxele (indawo yokululama) ukuze uvimbele ukuhlanza kungangeni emaphashini. Ungahlikihla kancane kancaneijeli yeglucose, uju, noma ushukela wetafula ngqo ngaphakathi esihlathini esingezansi nasezinsinini (buccal mucosa), kodwa ukuhlenga okuqinisekile kudinga i-intravenous 10% Dextrose (D10W) ilawulwa abezimo eziphuthumayo noma abasebenzi bomnyango wezimo eziphuthumayo.
4. Ukuqonda ukuthi kungani Ukujova iGlucagon kungasebenzi kubuthi be-Ackee noma i-Lychee. Imindeni noma abezimo eziphuthumayo abaphethe izimo eziphuthumayo izinto zokujova iglucagon kufanele bazi ukuthi iglucagon isebenza kuphela ngokushukumisa isibindi ukuba sephule i-glycogen egciniwe (glycogenolysisbese uhlanganisa i-glucose entsha (gluconeogenesis). Ku-ackee ne-lychee encephalopathy, i-glycogen yesibindi isivele isivele 100% depleted and gluconeogenesis is biochemically blocked by Hypoglycin A and MCPG, okusho ukuthi iglucagon ayisebenzi nhlobo. Ukuphathwa okuqondile kuphela kwe-dextrose (glucose)* yangaphandle kusindisa ubuchopho.
5. Londoloza noma yiziphi izigaxa ze-ackee ezisele ezingaphekiwe noma eziphekiwe, amanzi okupheka, noma isithelo se-lychee. Ungavumeli noma ubani omunye ekhaya adle ebhodweni elifanayo, ikakhulukazi amanzi okupheka ()pot water) noma umhluzi lapho ama-ackee arils abiliswa khona, njengoba i-Hypoglycin A encibilika emanzini ingena iqonde oketshezini.

Isihlahla sesinqumo: i-Ackee ne-Lychee hypoglycemic encephalopathy triage¶

flowchart TD
    A["I-Ackee Engakavuthwa Okusolwayo noma Ukuzila I-Lychee Ukungenisa"] --> B{"Hlola Isimo Somtholampilo Nokwazi"}
    B -->|"I-Asymptomatic (Ukungenisa Kwakamuva, Ukuphaphama Ngokugcwele)"| C["Nikeza Isidlo Esisheshayo Esine-carbohydrate Eningi + Isiphuzo Somlomo Seglucose Ukuze Ugcine I-Hepatic Glycogen"]
    C --> D["Yamukela Esibhedlela Ihora Le-24-48 Ngokuqhubekayo 10% I-Dextrose Infusion & Ukuhlolwa Kweglucose Ye-Fingerstick Ngehora"]
    B -->|"Ukuphalaza Kungazelelwe, Ukujuluka, Ukuqunjelwa, Ukuquleka, noma Ikhoma"| E["Hlola I-Stat Point-of-Care I-glucose Yegazi (Ngokuvamile ingaphansi kuka-20-30 mg/dL / 1.1-1.7 mmol/L) + Igesi Legazi/I-Lactate"]
    E --> F["I-IV Bolus esheshayo: 10% Dextrose (D10W) 2 kuya ku-5 mL/kg IV Push (Abadala: 50 mL ye-D50W noma 250 mL ye-D10W)"]
    F --> G["Qala i-IV eqhubekayo ngo-10% i-Dextrose (D10W ku-0.9% NaCl) ku-Glucose Infusion Rate (GIR) engu-6 kuya ku-8 mg/kg/min"]
    G --> H{"I-Active Status Epilepticus noma Izimpawu Ze-Cerebral Edema?"}
    H -->|"Yebo"| I["IV Lorazepam/Midazolam + Phakamisa Ikhanda Amadigri angu-30 + 3% I-Hypertonic Saline (Gwema I-Hypotonic Fluids)"]
    H -->|"Cha"| J["Gcina Ushukela Wegazi u-100-150 mg/dL (5.5-8.3 mmol/L); Engeza i-IV/Oral L-Carnitine + Glycine + Riboflavin (Vitamin B2)"]
    I --> J

I-Pathophysiology: I-Hypoglycin A ne-MCPG ivala kanjani i-metabolism yamandla omuntu¶

Ukuze uqonde ukuthi kungani ingane enempilo ingalala imamatheka ngemva kokudla ama-lychees noma i-ackee futhi ivuke 5:00 AM ku-coma ebulalayo, odokotela kufanele bahlole ukuvinjwa okuqondile kwe-enzyme ye-mitochondrial okubangelwa I-Hypoglycin A kanye ne-***** MCPG** (evame ukubizwa ngokuthi i-glutaric acidemia etholakala ngohlobo lwe-II noma okuthole ukushoda kwe-acyl-CoA dehydrogenase eminingi [MADD]).

1. Ukuguqulwa kwama-protoxin abe ama-thioester e-CoA abulalayo¶

Noma Hypoglycin A (L-alpha-amino-beta-[methylenecyclopropyl]propionic acid) noma MCPG (alpha-[methylenecyclopropyl]glycine) inobuthi obuqondile efomini layo le-amino acid eluhlaza. Uma esemuncwe emathunjini amancane, angena ku-mitochondrial branched-chain amino acid catabolic pathway: * I-Cytosolic kanye ne-mitochondrial ama-transaminase aguqula i-Hypoglycin A ibe yi-methylenecyclopropylpyruvic acid (MCPP), kanye ne-branched-chain alpha-keto acid dehydrogenase* oxidative decarboxylates ibe ubuthi obubulalayo obusebenzayo: methylenecyclopropylacetyl-CoA (MCPA-CoA). * Ngokufanayo, MCPG (echichima ngezinhlamvu ze-lychee ezingavuthiwe/ezivuthiwe kanye nama-arils, kanye nembewu ye-ackee) igaywa ibe yi-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. Kungani i-fatty acid beta-oxidation kanye ne-gluconeogenesis kokubili kwehluleka¶

1. ** Ukuvinjelwa kokuzibulala kukaFlavin (FAD) Ama-Acyl-CoA Dehydrogenases:** MCPA-CoA kanye ne-MCPF-CoA* asebenza njengama-inhibitor asekelwe kumshini ("ukuzibulala") we-flavin adenine dinucleotide ("ukuzibulala").FAD) iqembu lokufakelwa ku-mitochondrial short-chain acyl-CoA dehydrogenase (SCAD), medium-chain acyl-CoA dehydrogenase (MCAD), isovaleryl-CoA dehydrogenase (IVD), kanye ne-*glutaryl-CoA dehydrogenase (GCDH)**. Ngenxa yokuthi umzimba awusakwazi ukufaka i-oxidize ama-acids anamafutha ngesikhathi sokuzila, izicubu ziphoqeleka ukuthi zishise i-glucose ngesivinini esikhulu ngenkathi i-ketone body synthesis (acetoacetate futhi beta-hydroxybutyrate) ivinjwe ngokuphelele.
2. Ukuvalwa kwe-Hepatic Gluconeogenesis: Uma i-glycogen yesibindi isiphelile (4 to 10 hours ngemva kokudla kokugcina, kushesha kakhulu ezinganeni ezincane ezinezilinganiso eziphezulu zesisindo sobuchopho kuya esibindini noma izinqolobane ezinomsoco), isibindi sizama ukuhlanganisa i-glucose entsha ku-lactate, i-alanine, ne-glycerol nge-gluconeogenesis. Nokho, i-enzyme yokuqala enciphisa izinga le-gluconeogenesis, pyruvate carboxylase, idinga i-acetyl-CoA njengesishoshovu esiyisibopho se-allosteric kanye ne-ATP + NADH (ekhiqizwe i-fatty acid beta-oxidation) ukuze isebenze. Ngaphezu kwalokho, i-acyl-CoA esters engasetshenzisiwe kanye MCPA-CoA sequester yonke imitochondrial yamahhala Coenzyme A (CoA-SH) kanye ne-L-carnitine. Ngaphandle ATP, NADH, acetyl-CoA, noma mahhala CoA-SH, i-gluconeogenesis yesibindi iyagaya ize ime ngokuphelele.
3. Ukunqwabelana Kwe-Neurotoxic Branched kanye Ne-Short-Chain Organic Acids: I-beta-oxidation evinjiwe kanye ne-amino acid catabolism kubangela ukunqwabelana okukhulu kwama-acids ama-short-chain and medium-chain fatty acids (klzzwxh:0126). Lawa ma-asidaphilayo avimbela amaphampu esistimu yezinzwa emaphakathi avikelayo, aqala i-chemoreceptor igger zone** ku-brainstem (okubangela ukuhlanza okuzumayo), ahlanganise i-mitochondria ye-neuronal, futhi akhiqize i-cytotoxic i-cerebral edema ngaphandle koshukela wegazi ophansi ngokwawo.

Ukuqhathanisa Isifo Sokuphalaza sase-Jamaican (Ackee) vs. Fasting Lychee Encephalopathy¶

Isinyathelo ngesinyathelo somthetho wesibhedlela esiphuthumayo¶

Lapho ingane noma umuntu omdala efika enokuhlanza, isimo sengqondo esishintshile, noma ukuquleka ngemva kokudla i-ackee noma i-lychee, umzuzu nomzuzu we-hypoglycemia engelashiwe ne-organic acidemia yandisa ingozi yokulimala unomphela kobuchopho be-hypoxic-ischemic noma i-bratal stem herniation.

1. I-Intravenous 10% ye-Dextrose esheshayo (D10W) Ukuvuselela¶

Ungalindi ukuthi amaphaneli amakhemikhali aselabhorethri abuye ngaphambi kokunikeza i-dextrose; yenza ukuhlola ushukela wegazi oseduze komunwe ngokushesha (bese udweba ishubhu legazi elibomvu/eliluhlaza/elinsomi phezulu ukuze uthole iphrofayili ye-toxicology/acylcarnitine uma kungenzeka, ngaphandle kokulibazisa ukwelashwa 60 seconds).

• Kungani 10% Dextrose (D10W) ikhethwa ngaphezu kuka-25% noma u-50% we-Dextrose ezinganeni: Igxilile 50% i-dextrose (D50W, 2,525 mOsm/L) i-hyperosmolar enamandla kakhulu futhi ibangela i-venous sclerosis enzima, i-tissue necrosis uma i-extravased, kanye nokushintshashintsha okusheshayo kwe-osmotic okuyingozi kusithiyo sezingane esisengozini yegazi nobuchopho. Okujwayelekile 5% i-dextrose (D5W) ayilethi i-glucose eyanele ukuze icindezele ukuhlangana kwamafutha e-endogenous. 10% Dextrose (D10W, 100 mg of glucose per mL) indinganiso yegolide yakho kokubili ama-boluse okuqala kanye nokusetshenziswa okuqhubekayo kokulungisa.
• Umthamo wokuqala we-IV Bolus:
• Izingane: Phatha 2 to 5 milliliters per kilogram (2 to 5 mL/kg, ukuhlinzeka 0.2 to 0.5 grams/kg ushukela wegazi). 10% I-Dextrose (D10W) ngomthambo ngaphezulu 2 to 5 minutes.
• Abadala: Phatha 250 milliliters kwe 10% I-Dextrose (D10W) (noma 50 milliliters kwe 50% I-Dextrose [D50W]) ngomjovo.
• Isilinganiso Esiphezulu Esiqhubekayo Esiqhubekayo 10% Ukufakwa kwe-Dextrose (To Shut Down Lipolysis):
• Umthetho obalulekile wokuphila komzimba: I-bolus eyodwa ye-dextrose inyusa ushukela wegazi kuphela 20 to 30 minutes. Okubi nakakhulu, ku-Hypoglycin A kanye nobuthi be-MCPG, umgomo wokwelapha nge-dextrose awukona nje ukugcina ushukela wegazi unjalo. 70 mg/dL**, kodwa ukuvuselela ukukhululwa kwe-endo native insulin ukuze kuvale ngokuphelele i-lipase ezwela i-hormone kuzicubu ze-adipose. Uma nje umzimba uzama ukuhlanganisa ama-fatty acids mahhala ezitolo zamafutha, anobuthi MCPA-CoA, MCPF-CoA, kanye nama-organic chain short-chain ashintshashintshayo aqhubeka enqwabelana ngaphakathi kwe-mitochondria futhi abe nobuthi ebuchosheni.
• Isilinganiso sokufakwa (Glucose Infusion Rate [GIR]): Ngokushesha qala ukumnika okuqhubekayo emthanjeni we- 10% I-dextrose ngaphakathi 0.9% I-saline evamile (D10NS) (noma D10 1/2NS ne-potassium efanele uma ukusebenza kwezinso sekuqinisekisiwe) nge-Izinga Lokufakwa Kwe-Glucose (GIR) kwe 6 to 8 milligrams per kilogram per minute (roughly 3.6 to 4.8 mL/kg/hour kwe D10W), titrating ukugcina i-serum glucose phakathi 100 and 150 mg/dL (5.5 to 8.3 mmol/L) okungenani 24 to 48 hours.

2. I-Metabolic Cofactor and Conjugation Rescue (L-Carnitine, Glycine, futhi Riboflavin)¶

Ngoba MCPA-CoA futhi MCPF-CoA bamba yonke imitochondrial Coenzyme A yamahhala (CoA-SH) kanye ne-carnitine ngenkathi ibhubhisa i-flavin adenine dinucleotide (FAD) ama-cofactors, i-clinical toxicology protocols zisebenzisa izithasiselo ezintathu ezihlosiwe ze-metabolic ukuze kususwe okuphakathi okunobuthi:

1. I-Intravenous noma i-Oral L-Carnitine (Levocarnitine):
2. Indlela: Isengezo se-L-carnitine sibopha i-mitochondrial evalelekile MCPA-CoA, MCPF-CoA, i-isovaleryl-CoA, nama-molecule e-acyl-CoA e-medium-chain nge-carnitine acyltransferase, ewaguqulela ekubeni yi-acylcarnitine esters encibilikayo emanzini.MCPA-carnitine)* ezimpontshwa ku-mitochondria futhi zikhishwe emchamweni, zikhulule CoA-SH ngomjikelezo we-Krebs.
3. Umthamo: 100 milligrams per kilogram per day (100 mg/kg/day) ngomthambo (noma ngomlomo uma uphapheme) ihlukanise yonke into 6 to 8 hours (up to 2 to 3 grams/day).
4. I-Glycine yomlomo noma ye-Enteral:
5. Indlela: I-enzayimu ye-mitochondrial glycine N-acyltransferase ihlanganisa MCPA-CoA futhi MCPF-CoA ne-amino acid ethi glycine ukuze yakhe i-non-toxic MCPA-glycine kanye ne-MCPF-glycine, ehlanzwa ngokushesha yizinso.
6. Umthamo: 150 to 250 milligrams per kilogram per day ngomlomo noma nge-nasogastric tube ihlukaniswe njalo 6 to 8 hours.
7. I-Riboflavin enedosi ephezulu (Vitamin B2):
8. Indlela: Ngoba MCPA-CoA ngokuhlanganyela yenza i- FAD iqembu lokufakelwa le-acyl-CoA dehydrogenases, elihlinzeka ngedosi ephezulu Riboflavin (the direct precursor of FAD and FMN, **100 milligrams ngomlomo noma nge-NG tube 2 izikhathi ezingu-3 nsuku zonke **) isheshisa ukuvuselelwa kokusebenza MCAD futhi SCAD ama-enzyme.

3. Ukulawula Ukuquleka, I-Cerebral Edema, kanye ne-Acidosis¶

• Ukulawula ukubanjwa: Ukulungisa i-hypoglycemia nge 10% I-Dextrose imisa ngokushesha ukudlikizela ezigulini eziningi ezethula ngokushesha. Uma ukuqunjelwa kuqhubeka naphezu kokujwayelekile kweglucose yegazi (due to already-established cerebral edema or organic acid neurotoxicity), faka nge-intravenous lorazepam (0.1 mg/kg IV), midazolam (0.1 to 0.2 mg/kg IV/IM), noma 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).
• Ukulawulwa kwe-Cerebral edema: Phakamisa ikhanda lombhede ukuze 30 degrees, gcina i-normocarbia (PaCO2 35 to 40 mmHg) nge-endotracheal intubation yokuqala uma i-Glasgow Coma Scale (GCS) kube < 8, bese ufaka 3% Hypertonic Saline (3 to 5 mL/kg IV ngaphezulu 15 to 20 minutes) noma Mannitol (0.5 g/kg IV).
• I-Sodium Bicarbonate: Nge-acidosis enzima ye-metabolic (arterial pH < 7.15), faka umthambo oqaphile i-sodium bicarbonate ngenkathi ugcina ukumnika kweglucose okuqhubekayo.

Ukuvimbela: Ungayitshela kanjani i-Ackee evuthiwe ephephile ku-Ackee engavuthiwe enobuthi, kanye nemithetho ephephile yeLychee yezingane¶

1. Imithetho Emi-4 Egolide Yokulungiselela I-Ackee Ephephile¶

+-------------------------------------------------------------------------+
|         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. Vumela i-pod "izamule" ivuleke ngokwemvelo esihlahleni: Ungalokothi uvune noma upheke i-ackee pod engazange ihlukane yavuleka ngokwemvelo ngenkathi isalenga esihlahleni. Ungalokothi uphoqe i-pod evaliwe ivuleke, futhi ungalokothi ushiye ama-pods avaliwe efasiteleni elinelanga noma ophahleni elinde ukuthi avuleke esihlahleni (ama-pods ahlukanisiwe awayisusi kahle i-Hypoglycin A iphume e-arils iye embewini).
2. Susa yonke imbewu nayo yonke imikhondo yolwelwesi olupinki/obomvu (raphe): Imbewu emnyama ecwebezelayo kanye nolwelwesi olubomvu olubomvana olunamathisele i-aril ephuzi esitsheni sembewu luhlala lunobuthi unomphela (containing high levels of Hypoglycin A and B) ngisho nasezithelweni ezivuthwe ngokugcwele. Khipha lonke ulwelwesi olupinki bese ugeza ama-aril aphuzi kahle emanzini abandayo ahambayo ngaphambi kokupheka.
3. Bilisa ngokwehlukana futhi UHLALE ulahla amanzi okupheka: Bilisa ama-aril ahlanziwe emanzini alula kuqala, uwakhiphe ku-colander, futhi uthele amanzi abilayo aqonde phansi kusinki. Ungalokothi upheke irayisi, i-yam, ubhanana, amadombolo, noma isobho emanzini afanayo asetshenziselwe ukubilisa i-ackee.
4. Ukuphepha kwe-Ackee ekheniwe ekheniwe: E-United States, e-Canada, e-UK, nase-EU, ukuthengiswa kwe-ackee efakwe ethini kuqashwe ngokuqinile i-US FDA kanye neziphathimandla zokuphepha kokudla ukuze kuqinisekiswe ukuthi ukugxila kwe-Hypoglycin A kungaphansi 100 parts per million (ppm). Ngisho ne-ackee ekheniwe yokuhweba, khipha futhi ugeze amanzi ngaphambi kokuwososa nge-saltfish.

2. Imithetho Emi-3 Yezempilo Yomphakathi Eqede Ukuqubuka Kwe-Lychee Encephalopathy Ebuntwaneni¶

Kulandela uphenyo oluhlanganyelwe oluyingqopha-mlando lwe- Indian National Center for Disease Control (NCDC) kanye US CDC e-Muzaffarpur, Bihar (lapho amakhulu ezingane afa khona njalo ngoMeyi nangoJuni ngesikhathi sokuvuna i-lychee), iziphathimandla zezempilo zomphakathi zamisa imithetho emithathu elula yomndeni eyanciphisa ukufa 80%: 1. Ungalokothi uvumele izingane zilale zingadlanga ukudla kwakusihlwa okuphekiwe (isidlo sakusihlwa): Ukudla isidlo sakusihlwa esigcwele ama-carbohydrate kugcwalisa izitolo ze-hepatic glycogen ukuze isibindi sengane singalokothi sithembele ekuvimbeni kwe-asidi ye-beta-oxidation ngobusuku bonke. 2. Khawulela inani lama-lychees adliwa izingane ezincane, futhi ugweme izithelo ezingavuthiwe/eziwile: Ungavumeli izingane zidle ama-lychees angavuthiwe noma asele ingxenye engavuthwanga emhlabathini ezinsimini, njengoba ama-lychee angavuthiwe aqukethe okungaphezu kokuphindwe kabili kwe-MCPG kanye ne-Hypoglycin A etholakala esithelweni esibomvu esivuthwe ngokugcwele. 3. Nikeza ushukela ophuzwayo ngokushesha futhi uphuthume emtholampilo one-Dextrose engu-10% ekuqaleni kophawu lwasekuseni: Noma iyiphi ingane endaweni ye-lychee noma i-ackee evuka ihlanza, ikhathele, noma idikiza kufanele ithole **10% I-Dextrose ** phakathi nehora lokuqala (administering D10W within 4 hours of symptom onset reduces fatality from > 44% to < 5%).

Izimo eziphuthumayo zesifunda kanye nezinqubo zokulawula ushevu¶

• I-Jamaica ne-Caribbean: Shayela u-119* (I-Ambulensi / Amaphoyisa ase-Jamaica) noma 911 / 999 (kuzo zonke izindawo zase-Caribbean) futhi uphuthume ngokushesha emnyangweni wezimo eziphuthumayo wesibhedlela oseduze ukuze ufakwe emithanjeni. 10% I-Dextrose. I-Ministry of Health and Wellness of Jamaica igcina i-Ackee Poisoning (i-Jamaican Vomiting Sickness)* imile ngendlela eqinile** yokuvuselela izinqubo kuzo zonke izibhedlela zepherishi nezikhungo zezempilo.
• I-United States ne-Canada: Shayela u-911* ngokushesha bese udayela u-1-800-222-1222 (Usizo Lobuthi lwase-US) noma 1-844-POISON-X (1-844-764-7669 eCanada). I-ackee entsha ikhula eSouth Florida (where trees are occasionally harvested by residents unfamiliar with the "natural yawn" rule), kanye ne-ackee eqandisiwe/esemathinini ithengiswa ezimakethe zaseCaribbean kulo lonke elaseNyakatho Melika.
• I-India, Vietnam, Bangladesh, ne-Southeast Asia: Shayela u-108* noma 112 (India), 115 (Vietnam), noma 999 (Bangladesh/Malaysia). Phakathi noMeyi-kuya-Julayi lychee (litchi), longan, kanye rambutan inkathi yokuvuna, izikhungo zezempilo eziyisisekelo e-Bihar (India) kanye nama-glucometer aseduze nombhede wesitoko enyakatho yeVietnam kanye 10% Izikhwama ze-Dextrose eziqondene ngqo nokuhlenga ngokushesha kwezingane ze-hypoglycemic encephalopathy.
• I-United Kingdom, European Union, ne-West Africa: Shayela ku-999* (UK), 112 (EU), noma 112 / 199 (Nigeria, Ghana, Côte d'Ivoire, Burkina Faso, lapho i-Blighia sapida ikhula kakhulu futhi ibangela ubuthi bezingane besizini eyaziwa ngokuthi "ackee poisoning" noma "finsan" ubuthi). Odokotela base-UK/EU kufanele baxhumane ne-NPIS / TOXBASE noma izikhungo zezwe ezinobuthi ukuze bathole i-urinary acylcarnitine/organic acid diagnostic diagnostic and L-carnitine umthamo.

Imibuzo evame ukubuzwa¶

Kungani izingane ezinoshevu we-lychee noma i-acke engavuthiwe ngokuvamile zigula ekuseni kakhulu?¶

I-Hypoglycin A ne-MCPG ivimba ama-enzyme e-mitochondrial adingekayo ukuze kushiswe amafutha omzimba futhi kukhiqizwe ushukela omusha (i-gluconeogenesis), kodwa akuvimbi isibindi ekukhipheni i-glycogen esivele igciniwe. Lapho ingane idla i- ackee engavuthiwe noma idla ama-lychee futhi ilala ngaphandle kwesidlo sakusihlwa, i-glycogen yesibindi sayo egciniwe igcina ushukela wayo wegazi uvamile emahoreni angu-4 kuya kwangu-10 okuqala; phakathi kuka-3:00 AM kanye no-8:00 AM, i-glycogen yesibindi iyaphela ngokuphelele, okubangela ukuba ushukela wegazi uphahlazeke ngaphansi kuka-20 mg/dL kuyilapho amafutha anobuthi aphakathi nendawo egcwala ubuchopho.

Kungani umjovo we-Glucagon ophuthumayo ungasebenzi we-Ackee noma i-Lychee hypoglycemia?¶

I-Glucagon inyusa ushukela wegazi kuphela ngokuyalela isibindi ukuthi sephule i-glycogen egciniwe futhi sihlanganise ushukela omusha nge-gluconeogenesis. Ku-ackee kanye ne-lychee poisoning, i-glycogen yesibindi sesiguli isivele ingenalutho ngokuphelele futhi ama-enzyme abo e-gluconeogenic akhiywa nge-biochemically yi-MCPA-CoA ne-MCPF-CoA, okusho ukuthi i-glucagon ayinawo umphumela; isiguli kufanele sithole ushukela womlomo oqondile (uma siphapheme) noma nge-intravenous 10% Dextrose.

Kungani i-Valproic Acid (Depakote) iyingozi ekubanjweni okubangelwa i-Ackee noma i-Lychee poisoning?¶

I-Valproic acid iyi-branched-chain fatty acid yona ngokwayo edla i-mitochondrial L-carnitine, ivimbela ama-enzyme e-fatty acid beta-oxidation, futhi ikhulise i-ammonia yegazi. Ukunikeza i-valproic acid esigulini esindlela ye-beta-oxidation isivele inoshevu yi-Hypoglycin A noma i-MCPG yenza kube kubi kakhulu kwe-metabolic acidosis, i-hyperammonemia, kanye ne-cerebral edema ebulalayo; odokotela kufanele basebenzise i-benzodiazepines, i-levetiracetam (Keppra), noma i-phenobarbital eduze no-10% we-Dextrose esikhundleni salokho.

Ungabona kanjani ukuthi isithelo esisha se-Ackee siphephile ukuthi singasivuna sipheke?¶

I-ackee pod entsha iphephile kuphela uma isijike yababomvu ngokugqamile futhi iphuzi-orange futhi yavuleka yavuleka ngokwemvelo ("yazamula") ngenkathi isalenga esihlahleni elangeni, iveza ngokugcwele imbewu ezintathu ezimnyama ezicwebezelayo. Ungalokothi upheke i-pod eluhlaza engavuliwe, ungalokothi uphoqe i-pod ukuthi ivuleke ngommese, susa njalo imbewu emnyama kanye nolwelwesi lonke olubomvana olubomvana, futhi njalo uthele amanzi abilayo emseleni.

Kungani odokotela begcina i-10% ye-Dextrose IV drip isebenza amahora angama-24 kuye kwangama-48 ngisho nangemva kokuba ushukela wegazi usubuyele kwesijwayelekile?¶

Ukunikeza i-bolus eyodwa ye-glucose kulungisa ushukela wegazi ophansi imizuzu engama-20 ukuya kwengama-30 kuphela. Ukusebenzisa i-10% yezinga eliphezulu le-Dextrose (ku-6 kuya ku-8 mg/kg/ngomzuzu) kugcina ushukela wegazi uphakeme kancane (100 kuya ku-150 mg/dL), okukhuthaza i-insulin yesiguli ukuthi ivale ukuwohloka kwamafutha esicupheni se-adipose futhi kuvimbe umzimba ekukhiqizeni ama-enzyme e-neurotoxic short-chain organic organic acid.

Ingabe amahhashi nezinja nazo zifakwe ubuthi yi-Hypoglycin A ne-MCPG embewu yesihlahla?¶

Yebo, amahhashi adla emadlelweni ekwindla nasentwasahlobo avame ukuba nesimo esibulalayo esibizwa ngokuthi i-Equine Atypical Myopathy (e-Europe/UK) noma i-Seasonal Pasture Myopathy (eNyakatho Melika) ngemva kokudla imbewu enamaphiko ("helicopter samaras") kanye nezithombo ze-Sycamore Maple (Acer pseudoplatanus) kanye ne-Box Neguceaelder njenge-Apinda yomndeni ofanayo i-acke ne-lychee futhi iqukethe amazinga aphezulu e-Hypoglycin A.

Ukuze uphendule ngempumelelo ezimweni eziphuthumayo zezingane, ze-metabolic, kanye nezobuthi, cabanga ukuthatha usizo lokuqala lokuqala kanye nesifundo se-CPR esivela ku-Red Cross, i-St John Ambulance, noma umhlinzeki wendawo wokuqeqesha izimo eziphuthumayo.

Iziqondiso ezihlobene¶

• I-Sulfonylurea (Glipizide, Glyburide) Overdose, I-Hypoglycemia Ephindaphindiwe, kanye ne-Octreotide Triage
• I-Status Epilepticus, Ukuquleka Isikhathi Eside, kanye Nemithi Yokuhlenga
• Ukubanjwa: Usizo Lokuqala Nokuvikelwa Kwendlela Yomoya
• Ubuthi Bokudla, I-Acute Gastroenteritis, kanye Nokuphelelwa amanzi emzimbeni
• I-General Poisoning First Aid kanye Nokukhipha Ukungcola

Imithombo neziqondiso ezisemthethweni¶

• I-U.S. Centers for Disease Control and Prevention (CDC) & National Center for Disease Control (NCDC, India): Shrivastava A, et al.: *Inhlangano ye-acute toxic encephalopathy ne-litchi ukusetshenziswa ekuqubukeni e-Muzaffarpur, India, 2014: isifundo sokulawula icala * (The Lancet Global Health).
• I-Ministry of Health and Wellness of Jamaica & Caribbean Public Health Agency (CARPHA): Imihlahlandlela Yokuphathwa Komtholampilo ye-Ackee Poisoning (Isifo Sokuhlanza sase-Jamaica / I-Hypoglycin A Ukudakwa) (Kingston, Jamaica).
• I-U.S. Ukulawulwa Kokudla Nezidakamizwa (i-FDA): Isexwayiso Sokungenisa 21-11 & Nomhlahlandlela Wenqubomgomo Yokuthobela: Ukubopha I-Ackee Esethinini noma Eqandisiwe Equkethe Okungaphezu kuka-100 ppm Hypoglycin A (United States).
• Inhlangano Yezempilo Yomhlaba (i-WHO) / ​​IKomidi Elihlangene Lochwepheshe le-FAO Lezithako Zokudla: Ukuhlolwa Kokuphepha Kokungcoliswa Kokudla Okuthile: I-Hypoglycin A ku-Ackee Fruit (Blighia sapida) kanye ne-MCPG eSapindaceae Fruits (Geneva).
• I-Sherratt HSA / Tanaka K, Isselbacher KJ: I-Hypoglycin, ubuthi besithelo se-ackee esingavuthiwe: indlela yamakhemikhali ezinto eziphilayo evimbela i-beta-oxidation ne-gluconeogenesis (Biochemical Pharmacology / New England Journal of Medicine).