Hua Ackee (Hypoglycin Kore i maoa He mate ruaki a Jamaica) me te Lychee (MCPG) Te nohopuku Hypoglycemic Encephalopathy i roto i nga tamariki¶

Ka ruaki ohorere i te ata, te ngenge nui, te hopukanga whanui, te koma ranei i muri i te kai ** hua ackee kare i maoa (Blighia sapida)** kai ranei raihi (Litchi chinensis) i runga i te puku kau karekau he kai i te ahiahi he ohotata mo te wa tino nui na te mate kino kaore i te ketotic hypoglycemia (blood glucose often below 20 mg/dL or 1.1 mmol/L). Waea atu ki to nama whawhati tata (911 i te United States me Kanata, 112 puta noa i te Kotahitanga o Europi, 999 i te United Kingdom) korero ranei ki a maatau whaiaronga tau ohorere o te ao i te wa tonu, me korero ki te kaikawe ka hiahia te manawanui ki te tirotiro tere i te huka toto me te whakaroto 10% Dextrose. I a koe e tatari ana mo nga paramedics, whakahaere i nga pupuhi kaha ma te whakamahi i to maatau status epilepticus kawa hopu roa, te wehewehe i te raruraru metabolic i te rongoa-whakaawe sulfonylurea hypoglycemic overdose, me te whakamahi whanui paihana whakapeka awhina tuatahi.

Ko te ackee (te hua o te motu o Jamaica, i tipu whanui puta noa i te Karipiana, Awherika ki te Hauauru, Florida, me Amerika Waenga) me te lychee (me te longan me te rambutan, he whanaunga kei roto i te whanau huaota Sapindaceae) kei roto i nga protoxins waikawa amino cyclopropyl kaha: Hypoglycin A (L-methylenecyclopropylalanine) me tona homologue raro alpha-(methylenecyclopropyl)glycine (MCPG). Ki te kai te tamaiti, pakeke ranei i te ackee kare i maoa, ka whakatuwheratia (ka inu ranei i te wai i kohuatia ai te ackee), ka kai ranei te tamaiti ngoikore i te maha o nga raihi kare i maoa i te awatea ka moe kare i kai i te hapa, ka noho wahangu enei paitini mo 4 to 12 hours i te mea kei te pupuri tonu te tinana i te glycogen ate. I nga haora o te ata (typically 3:00 AM to 8:00 AM), i te paunga o te glycogen ate mo te po, ka ngana te tinana ki te huri ki te fatty acid beta-oxidation me gluconeogenesis kia mau ai te roro ki te hukahuka. Na te mea ko te MCPA-CoA (mai i te Hypoglycin A) me te MCPF-CoA (mai i te MCPG) ka aukati i te mitochondrial medium-chain acyl-CoA dehydrogenase (MCAD) me nga enzyme gluconeogenic, ka tino heke te huka toto ki te kore i te wa e waipuke ana nga waikawa waro mekameka poto paitini i te roro, ka tere te edema cerebral, te mate epilepticus me te mate i roto. 12 to 48 hours ki te kore 10% Dextrose (D10W) ka tukuna he bolus me te whaowhia o te huka tere tere.

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

Mahia tenei inaianei¶

1. Waea wawetia nga ratonga whawhati tata me te tono i tetahi waahanga paramedic me te Dextrose intravenous. Waea 911 (US me Canada), 112 (Uniana Pakeha), 999 (United Kingdom), 119 / 911 (Jamaica me Caribbean), ranei 108 / 112 (India). Korero atu ki te kaikawe kua kai te manawanui i nga hua ackee, raiti ranei, kei te pangia e te acute toxic hypoglycemic and metabolic encephalopathy.
2. Mena kei te oho te tangata, ka taea e ia te horomia marie, hoatu te huka-waha tere tere. Whakarewa 2 to 3 tablespoons (30 to 45 grams) o te huka tepu, te honi, te tirikara witi, te paura huka ranei ki roto i te karaihe wai, wai huarākau ranei ka inu tonu te tangata. Ahakoa ka mohio tetahi ka kai noa ia i te ackee kare i maoa, ka kai ranei te tamaiti i te raihiihi i runga i te puku kau ka ahua noa tonu, ka hoatu he kai warowaihā me te inu huka tonu ka noho ki tonu nga toa glycogen ate me te aukati i te tinana ki te whakahohe i te ara whakahiato waikawa ngako i a koe e peia ana ki te hohipera.
3. Mena kei te momoe te tangata, kei te rangirua, kei te ruaki, kei te paopao ranei, KAUA rawa e ringihia he wai ki roto i te waha. Hurihia te turoro ki tona taha maui (te tuunga whakaora) kia kore ai e uru te ruaki ki roto ki nga puku. Ka taea e koe te mirimiri marie i tetahi wahi iti o te glucose gel, honi, te huka tepu ranei tika ki te roto o te paparinga o raro me te kapia (buccal mucosa), engari ko te whakaoranga tino ka hiahiatia whakaurua 10% Dextrose (D10W) e whakahaeretia ana e nga paramedic, nga kaimahi o te tari ohorere ranei.
4. Kia mohio he aha te Injecting Glucagon KORE e mahi i roto i te Ackee, Lychee poisoning ranei. Ko nga whanau me nga paramedic e kawe ohorere ana kete werohanga glucagon me mohio kei te mahi te glucagon anake ma te whakaihiihi i te ate ki te wawahi i te glycogen kua penapena (glycogenolysis) me te whakahiato i te huka hou (gluconeogenesis). I roto i te ackee me te lychee encephalopathy, kua noho kē te glycogen ate 100% kua pau me te gluconeogenesis kua aukatihia e te Hypoglycin A me te MCPG, ko te tikanga ko te glucagon he tino koretake. Ko te whakahaere tika anake o te dextrose (glucose) o waho ka whakaora i te roro.
5. Whakaorangia nga toenga o te ackee mata, ka tunua ranei nga ackee pods, te wai tunu, te hua raihi ranei. Kaua e tukua tetahi atu o te whare ki te kai i te kohua kotahi, ina koa ko te wai tunu (pot water) he kirikiri ranei i kohuatia ai nga kiri ackee, i te mea ka rehu totika te Hypoglycin A wairewa ki roto i te wai.

Rakau whakatau: Ackee and Lychee hypoglycemic encephalopathy triage¶

flowchart TD
    A["E whakapaetia ana ko te Ackee kare i maoa, ka horomia ranei te Raikiti nohopuku"] --> B{"Te Aromatawai i te Mana Haumanu me te Maaramatanga"}
    B -->|"Asymptomatic (Te horomitanga o tata nei, ka oho katoa)"| C["Hoatu Inamata te Kai Waowao-Teitei + Te Inu Huka Waa-waha hei pupuri i te Glycogen ate"]
    C --> D["Whakauruhia ki te Hohipera mo te 24-48 Hour Tonu 10% Dextrose whaowhia me te Tirohanga huka huka ia haora"]
    B -->|"Ruaki ohorere, te werawera, te ngenge, te hopu, te koma ranei"| E["Tirohia te Tatauranga Tohu-o-Tiaki Toto huka (I te nuinga o te waa <20-30 mg/dL / 1.1-1.7 mmol/L) + Toto Gas/Lactate"]
    E --> F["Tona IV Tonu: 10% Dextrose (D10W) 2 ki te 5 mL/kg IV Push (Pakeke: 50 mL o D50W ranei 250 mL o D10W)"]
    F --> G["Tīmatahia te IV Tonu 10% Dextrose (D10W i roto i te 0.9% NaCl) i te Reiti Whakawhanake Glucose (GIR) o 6 ki te 8 mg/kg/min"]
    G --> H{"Nga Tohu Mahinga Epilepticus, Nga Tohu Edema Cerebral ranei?"}
    H -->|"Ae"| I["IV Lorazepam/Midazolam + Whakanuia te Upoko 30 Degrees + 3% Hypertonic Saline (Ka karohia nga Wai Hypotonic)"]
    H -->|"Kao"| J["Kia mau tonu te Huka Toto 100-150 mg/dL (5.5-8.3 mmol/L); Tāpirihia te IV/Oral L-Carnitine + Glycine + Riboflavin (Vitamin B2)"]
    I --> J

Pathophysiology: Me pehea te Hypoglycin A me te MCPG i kati i te paopaotanga kaha o te tangata¶

Kia mohio ai koe he aha te tamaiti hauora ka moe me te ataata i muri i te kai raihi, ackee ranei ka oho ake 5:00 AM i roto i te coma convulsive mate, me tirotirohia e nga kairoro te tino aukati i te hakihaki mitochondrial na te Hypoglycin A me MCPG (e kiia ana ko i whiwhi glutaric acidemia momo II ranei ** kua whiwhi te acyl-CoA dehydrogenase deficiency maha [MADD]**).

1. Te hurihanga o nga protoxins ki nga CoA thioesters whakamate¶

Kaore hoki Hypoglycin A (L-alpha-amino-beta-[methylenecyclopropyl]propionic acid) ranei MCPG (alpha-[methylenecyclopropyl]glycine) he paitini i roto i tona ahua waikawa amino mata. Ka uru mai i roto i te whekau iti, ka uru ki te ara mitochondrial branched-chain amino acid catabolic: * Ko te cytosolic me te mitochondrial transaminases ka huri i te Hypoglycin A ki te methylenecyclopropylpyruvic acid (MCPP), me te waina peka alpha-keto acid dehydrogenase oxidative decarboxylate ki roto i te paitini whakamate kaha: methylenecyclopropylacetyl-CoA (MCPA-CoA). * Waihoki, ko te MCPG (he nui i roto i nga kakano reihi kare i maoa/haehae-maoa me nga aril, tae atu ki nga kakano ackee) ka whakaurua ki te 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. He aha te take i kore ai te beta-oxidation waikawa ngako me te gluconeogenesis¶

1. Te whakamomori a Flavin (FAD) Acyl-CoA Dehydrogenases: MCPA-CoA me MCPF-CoA te mahi hei aukati i runga i te tikanga ("whakamomori") o te flavin adenine dinucleotide (FAD) rōpū prosthetic i runga mitochondrial ** mekameka poto acyl-CoA dehydrogenase (SCAD)**, mekameka-waewae acyl-CoA dehydrogenase (MCAD), isovaleryl-CoA dehydrogenase (IVD), me glutaryl-CoA dehydrogenase (GCDH). Na te mea kua kore e taea e te tinana te whakahaora i nga waikawa ngako i te wa e nohopuku ana, ka kaha te wera o nga kopa i te hukahuka i te wa o te ketone body synthesis (acetoacetate a beta-hydroxybutyrate) kua tino aukatia.
2. Kati te Gluconeogenesis Hepatic: Kia pau te ate glycogen (4 to 10 hours i muri i te kai whakamutunga, he tere ake i roto i nga tamariki nohinohi he nui te taumaha o te roro-ki-te-ata, he rahui kai maana ranei), ka ngana te ate ki te whakahiato i te huka hou mai i te lactate, alanine, me te glycerol ma te gluconeogenesis. Heoi ano, ko te whaariki whakatiki reiti tuatahi o te gluconeogenesis, pyruvate carboxylase, e hiahia ana ki te acetyl-CoA hei kaiwhakaora allosteric me te ATP + NADH (he mea hanga e te waikawa ngako beta-oxidation) kia rere. I tua atu, ko nga hākawa acyl-CoA kore-metabolized me MCPA-CoA whakawhaiti i te mitochondrial kore utu katoa Coenzyme A (CoA-SH) me L-carnitine. Kore ATP, NADH, acetyl-CoA, kore utu ranei CoA-SH, ko te gluconeogenesis hepatic e huri ana ki te mutu.
3. Ko te Whakaeminga o nga Waikawa Organic Peka me te Waena Poto-poto: Ko te beta-oxidation me te catabolism waikawa amino ka nui te hanga o nga waikawa ngako mekameka poto me te waenga-waewae (butyric, isobutyric, isovaleric, 2-methylbutyric, glutaric, adipic, suberic, me te waikawa sebacic). Ko enei waikawa pararopi ka aukati i nga papua o te roro o te roro, ka whakaoho i te ** chemoreceptor trigger zone** i roto i te roro (ka puta te ruaki pahū ohorere), te wehe i te mitochondria neuronal, me te whakaputa cytotoxic cerebral edema motuhake ake i te iti o te huka toto.

Te whakatairite i te mate ruaki a Jamaica (Ackee) ki te nohopuku Lychee Encephalopathy¶

Te kawa whawhati tata o te hohipera¶

Ka tae mai te tamaiti, pakeke ranei me te ruaki, te whakarereketanga o te mana hinengaro, te hopu ranei i muri i te kai i te ackee, i te raihi ranei, i nga meneti katoa o te hypoglycemia kaore i te rongoa me te waikawa waro ka nui ake te tupono o te pakaru o te roro hypoxic-ischemic pumau, te mate roro mate ranei.

1. Tonu Inuata 10% Dextrose (D10W) Whakaora¶

Kaua e tatari kia hoki mai nga panui matū taiwhanga i mua i te tuku dextrose; mahia tonutia he tirotiro huka toto i te taha o te maihao (me te tuhi i te ngongo toto whero/matomato/papura-runga mo te whakamaaramatanga paitini/acylcarnitine mena ka taea, me te kore e whakaroa i te rongoa ma te ara noa. 60 seconds).

• He aha te 10% Dextrose (D10W) he pai ake i te 25%, 50% ranei te Dextrose i roto i nga tamariki: Kua kaha 50% dextrose (D50W, 2,525 mOsm/L) he tino hyperosmolar, ka puta he sclerosis venous nui, te necrosis kiko ki te toha, me te huringa osmotic tere kino puta noa i te arai toto-roro tamariki whakaraerae. Paerewa 5% dextrose (D5W) kare e nui te huka ki te peehi i te whakakorikori o te waikawa ngako. 10% Dextrose (D10W, 100 mg of glucose per mL) Ko te paerewa koura mo nga boluses tuatahi me nga whaowhanga tiaki tonu.
• Te Inenga IV Bolus Tuatahi:
• Tamariki: Whakahaere 2 to 5 milliliters per kilogram (2 to 5 mL/kg, whakarato 0.2 to 0.5 grams/kg o te hukahuka) o 10% Dextrose (D10W) ka mutu ki roto 2 to 5 minutes.
• Pakeke: Whakahaere 250 milliliters o 10% Dextrose (D10W) (ranei 50 milliliters o 50% Dextrose [D50W]) whakaroto.
• Te Reiti Teitei Tonu 10% Dextrose Infusion (To Shut Down Lipolysis):
• Ture whaiaroaro tino nui: Ko te bolus kotahi o te dextrose ka piki ake te huka toto mo anake 20 to 30 minutes. Ko te mea kino rawa atu, i roto i te paihana o te Hypoglycin A me te MCPG, ko te whainga o te rongoa dextrose he ehara i te pupuri noa i te huka toto 70 mg/dL, engari ki te whakaihiihi i te nui o te tukunga o te insulin i roto i te kopa adipose. I te wa e ngana ana te tinana ki te whakakorikori i nga waikawa ngako koreutu mai i nga toa ngako, he paitini MCPA-CoA, MCPF-CoA, me nga waikawa pararopi-poto-poto e putu tonu ana ki roto i te mitochondria me te paihana i te roro.
• Karei whaowhia (Glucose Infusion Rate [GIR]): Me timata tonu te whaowhia ki roto i te toto o 10% Dextrose ki roto 0.9% Saline Pūnoa (D10NS) (ranei D10 1/2NS me te pāhare pāporo e tika ana kia manatokohia te mahi whatukuhu) i runga i te Reiti Whakawhowhanga Glucose (GIR) o 6 to 8 milligrams per kilogram per minute (roughly 3.6 to 4.8 mL/kg/hour o D10W), te titrating ki te pupuri i te huka toto i waenga 100 and 150 mg/dL (5.5 to 8.3 mmol/L) mo te iti rawa o 24 to 48 hours.

2. Whakaora Metabolic Cofactor and Conjugation (L-Carnitine, Glycine, a Riboflavin)¶

No te mea MCPA-CoA a MCPF-CoA mahanga katoa te mitochondrial Coenzyme A (CoA-SH) me te carnitine i te wa e whakangaro ana i te flavin adenine dinucleotide (FAD) ko nga kawa, ko nga kawa paitini haumanu e whakamahi ana i nga taapiri taapiri e toru hei whakaea i nga takawaenga paitini:

1. L-Carnitine i roto i te toto, i te waha ranei (Levocarnitine):
2. Mahanga: Ko te taapiri L-carnitine ka herea te mitochondrial kua mau MCPA-CoA, MCPF-CoA, isovaleryl-CoA, me nga ngota ngota acyl-CoA mekameka waenga ma te carnitine acyltransferase, ka huri ki te wai-wairewa acylcarnitine esters (MCPA-carnitine) ka panaia ki waho o te mitochondria ka peia ki roto i te mimi, ka whakawātea i te mate kino CoA-SH mo te huringa Krebs.
3. Inenga: 100 milligrams per kilogram per day (100 mg/kg/day) whakaroto (ma te waha ranei ki te oho) wehea ia 6 to 8 hours (up to 2 to 3 grams/day).
4. Glycine Oral or Enteral Glycine:
5. Meahanga: Ka hono te enzyme mitochondrial glycine N-acyltransferase MCPA-CoA a MCPF-CoA me te waikawa amino glycine hei hanga i te MCPA-glycine me te MCPF-glycine, ka tere te whakakorehia e nga whatukuhu.
6. Inenga: 150 to 250 milligrams per kilogram per day ma te waha, ma te ngongo nasogastric ka wehea ia 6 to 8 hours.
7. Riboflavin Inenga-Teitei (Vitamin B2):
8. Mahanga: Na te mea MCPA-CoA kovalently inactivates te FAD rōpū prosthetic o acyl-CoA dehydrogenases, whakarato horopeta teitei Riboflavin (the direct precursor of FAD and FMN, 100 milligrams ma te waha, ma te ngongo NG 2 ki te 3 nga wa ia ra) ka whakatere i te whakaoranga o te hohe MCAD a SCAD whākōkī.

3. Te Whakahaere i nga Whakaaetanga, te Cerebral Edema, me te Acidosis¶

• Te mana hopu: Te whakatikatika i te hypoglycemia me te 10% Ka whakamutua e te Dextrose te ngaru i roto i te maha o nga turoro e whakaatu wawe ana. Mena kei te mau tonu te marutanga ahakoa te whakaheke o te huka toto (due to already-established cerebral edema or organic acid neurotoxicity), tukuna ki roto i te toto lorazepam (0.1 mg/kg IV), midazolam (0.1 to 0.2 mg/kg IV/IM), levetiracetam ranei (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).
• Te whakahaere o te edema cerebral: Whakanuia te upoko o te moenga ki 30 degrees, pupuri normocarbia (PaCO2 35 to 40 mmHg) me te whakaurunga endotracheal moata ki te Glasgow Coma Scale (GCS) ko < 8, ka whaowhia 3% Hypertonic Saline (3 to 5 mL/kg IV mutu 15 to 20 minutes) ranei Mannitol (0.5 g/kg IV).
• Konutai Bicarbonate: Mo te mate waikawa nui (arterial pH < 7.15), tukuna kia tupato ** konutai bicarbonate** i te wa e mau tonu ana te whaowhia hukahuka.

Aukati: Me pehea te korero i te Ackee maoa haumaru mai i te Ackee kare i maoa paitini, me nga ture Lychee haumaru mo nga tamariki¶

1. Ko nga Ture koura e 4 mo te Whakaritea Haumaru Ackee¶

+-------------------------------------------------------------------------+
|         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. Kia tuwhera noa te peera ki runga i te rakau: Kaua rawa e hauhake, e tunu ranei i te ackee pod karekau i wehe whanui i te wa e iri tonu ana ki runga i te rakau. Kaua rawa e tohe kia puare te peera kua kati, kaua hoki e waiho nga peera kati i runga i te matapihi paki, i te tuanui ranei e tatari ana kia puare mai i te rakau (kaore e tika te whakawhiti i te Hypoglycin A mai i nga aril ki nga kakano).
2. Tangohia nga kakano me nga tohu katoa o te kiripi mawhero/whero (raphe): Ko nga kakano pango mohinuhinu me te kiriuhi kikorangi mawhero-whero e piri ana te kiri kowhai ki te ipu kakano ka noho paitini tonu (containing high levels of Hypoglycin A and B) ahakoa i roto i nga hua tino maoa. Katihia te kiriuhi mawhero katoa ka horoi i nga kiri kowhai ki te wai rere matao i mua i te tunu kai.
3. Kohuatia wehea ka maka tonutia te wai tunu: Kohuatia nga ackee kua horoia ki te wai totika i te tuatahi, ka whakamakuku ki roto i te colander, ka ringihia te wai kohua ki raro i te totohu. Kaua rawa e tunua te raihi, te uwhi, te panana, te dumplings, te hupa ranei i roto i te wai kotahi hei kohua i te ackee.
4. Te haumaru Ackee kua kawemai ki te kene: I te United States, Canada, UK, me te EU, ka tino aro turukihia nga kawemai ackee ackee arumoni e te US FDA me nga mana haumaru kai hei manatoko kei raro iho nga kohinga Hypoglycin A 100 parts per million (ppm). Ahakoa me te ackee kēne arumoni, whakamakuku me te horoi i te tote i mua i te tunu ki te tote.

2. Ko nga Ture Hauora Tumatanui e 3 I Whakamutua nga Ngarara o te Tamariki Lychee Encephalopathy¶

Whai muri i nga tirotirohanga nui a te Indian National Center for Disease Control (NCDC) me te US CDC i Muzaffarpur, Bihar (i reira nga rau o nga tamariki i mate ia Mei me Hune i te wa o te hauhake raihi), ka whakatauhia e nga mana hauora tūmatanui e toru nga ture ngawari o te whanau e whakaiti ana i te mate mate. 80%: 1. Kaua rawa e tukua nga tamariki ki te moe me te kore e kai i te kai maoa o te ahiahi (kai): Ko te kai i te kai o te ahiahi ka nui rawa te warowaiha ka whakakiia nga toa glycogen ate kia kore rawa te ate o te tamaiti e whakawhirinaki ki te waikawa ngako kua aukatihia te beta-oxidation mo te po. 2. Whakawhāitihia te nui o te riihi kai a nga tamariki iti, ka karo i nga hua kare i maoa/kua hinga: Kaua e tukua nga tamariki ki te kai i nga reihi kare i maoa, i te haurua-maoa ranei i te whenua i roto i nga kari, i te mea kei roto i nga reihi kare i maoa te nui atu i te rua o te kukū o MCPG me Hypoglycin A kei roto i nga hua whero tino maoa. 3. Hoatu wawetia te huka-waha me te tere ki te whare haumanu me te 10% Dextrose i te ata tuatahi o te tohu: Ko te tamaiti kei roto i te rohe riiki, ackee ranei ka oho ki te ruaki, ki te ngenge, ki te wiri ranei me whiwhi 10% Dextrose i roto i te haora tuatahi (administering D10W within 4 hours of symptom onset reduces fatality from > 44% to < 5%).

Nga tikanga ohotata a rohe me te whakahaere paihana¶

• Jamaica me te Karipiana: Waea 119 (Jamaica Ambulance / Pirihimana) ranei 911 / 999 (puta noa i nga rohe o Karipiana) ka tere tere ki te tari whawhati tata o te hōhipera mo te werohia. 10% Dextrose. Ko te Te Manatū Hauora me te Ora o Jamaica kei te pupuri i nga tikanga whakaora i nga whare hauora katoa me nga whare hauora.
• Te Hononga o Amerika me Kanata: Waea wawe ki a 911 ka waea atu ki te 1-800-222-1222 (Awhina Poison US) ranei 1-844-POISON-X (1-844-764-7669 i Kanata). Ka tipu te ackee hou i te tonga o Florida (where trees are occasionally harvested by residents unfamiliar with the "natural yawn" rule), a ka hokona te ackee tio/kene ki nga maakete Karipiana puta noa i Amerika Te Tai Tokerau.
• India, Vietnam, Bangladesh, me te tonga-tonga o Ahia: Waea 108 ranei 112 (India), 115 (Vietnam), 999 ranei (Bangladesh/Malaysia). I te wa o Mei-ki-Hurae lychee (litchi), longan, me te rambutan te wa hauhake, nga pokapu hauora tuatahi i Bihar (India) me te raki o Vietnam nga taputapu huka i te taha o te moenga me 10% Ko nga peke Dextrose mo te whakaora tonu i nga tamariki hypoglycemic encephalopathy.
• United Kingdom, European Union, and West Africa: Waea 999 (UK), 112 (EU), ranei 112 / 199 (Nigeria, Ghana, Côte d'Ivoire, Burkina Faso, kei reira a Blighia sapida e tipu nui ana, e pa ana ki nga tamariki o te tau. "ackee poisoning" ranei "finsan" paitini). Me korero nga tohunga haumanu i te UK/EU ki te NPIS / TOXBASE, ki nga pokapu paihana o te motu mo te whakapumautanga mate acylcarnitine/waikawa waro mimi me L-carnitine horopeta.

Pātai auau¶

He aha nga tamariki kua paihana e te raihi me te ackee kare i maoa ka mate moata i te ata?¶

Ko te Hypoglycin A me te MCPG te aukati i nga enzyme mitochondrial e hiahiatia ana hei tahu ngako tinana me te hanga huka hou (gluconeogenesis), engari kaore e aukati i te ate ki te tuku i te glycogen kua rongoa. Ka kai te tamaiti i te ackee kare i maoa, ka kai ranei i te reihi ka haere ki te moe me te kore kai, ka noho tonu te huka toto mo te 4 ki te 10 haora tuatahi; i waenganui i te 3:00 AM me te 8:00 AM, ka pau katoa te ate glycogen, ka heke te huka toto ki raro i te 20 mg/dL i te wa e waipuke ana te roro o te waikawa ngako paitini.

He aha te take i kore ai te werohanga Glucagon ohorere e mahi mo Ackee, Lychee hypoglycemia ranei?¶

Ko te Glucagon e whakanui ana i te huka toto ma te whakahau i te ate ki te wawahi i te glycogen kua penapena me te whakahiato i te huka hou ma te gluconeogenesis. I roto i te paihana ackee me te raihi, kua tino kau te glycogen ate o te manawanui, a ko o ratou enzyme gluconeogenic kua maukatihia e te MCPA-CoA me te MCPF-CoA, ko te tikanga kaore he painga o te glucagon; me whiwhi te manawanui i te huka-waha tika (mehemea e oho ana) he 10% Dextrose ranei.

He aha te take he kino te Waikawa Valproic (Depakote) mo te hopukina na te paihana Ackee, Lychee ranei?¶

Ko te waikawa valproic he waikawa ngako mekameka-manga e kai ana i te mitochondrial L-carnitine, ka aukati i te waikawa ngako beta-oxidation enzyme, me te whakanui i te haukini toto. Ko te hoatu waikawa valproic ki tetahi turoro kua paihana te ara beta-waikura e te Hypoglycin A, MCPG ranei, ka tino kino te waikawa metabolic, hyperammonemia, me te edema cerebral mate; Me whakamahi e nga kairoro te benzodiazepines, te levetiracetam (Keppra), te phenobarbital ranei i te taha o te 10% Dextrose.

Me pehea e mohio ai mena he pai te kohi me te tunu i tetahi hua hou Ackee?¶

Ka noho haumaru te peera ackee hou ina huri whero kanapa me te kowhai-karaka, ka wehea whanui noa ("kua oho") i te wa e iri tonu ana i runga i te rakau i te ra, ka tino kitea nga kakano pango mohinuhinu e toru. Kaua rawa e tunu i te pākākano kaakaariki karekau i whakatuwherahia, kaua e tohe i te peera kia tuwhera ki te maripi, tangohia nga kakano pango me te kiriuhi mawhero-whero katoa, ka riringi tonu i te wai kohua ki raro i te awaawa.

He aha nga taote ka pupuri i te 10% Dextrose IV drip mo te 24 ki te 48 haora ahakoa i muri i te hokinga o te huka toto ki te tikanga?¶

Ko te hoatu i te bolus kotahi o te hukahuka ka whakatika i te iti o te huka toto mo te 20 ki te 30 meneti noa iho. Ko te whakahaere i te whaowhia 10% Dextrose teitei tonu (i te 6 ki te 8 mg/kg/meneti) ka piki ake te huka toto (100 ki te 150 mg/dL), e whakaihiihi ana i te insulin o te manawanui ki te kati i te pakaru o te ngako i roto i te kiko adipose me te aukati i te tinana ki te whakaputa i etahi atu neurotoxic waikawa waro mekameka poto kia ora ai te ate.

Kei te paihana ano nga hoiho me nga kuri e te Hypoglycin A me te MCPG mai i nga kakano rakau?¶

Ae, ko nga hoiho e whangai ana i nga ngahere i te wa o te ngahuru me te puna ka pa te mate tere e kiia nei ko Equine Atypical Myopathy (i Uropi/UK) ranei Seasonal Pasture Myopathy (i Amerika ki te Raki) i muri i te kai i nga kakano pakau ("helicopter samaras") me nga tipu o Sycamore Maple (Acer pseudoplatanus) me Boxedo Elder (Acer pseudoplatanus) no te whanau o Sapindo (Acer negundo) ackee me te raikini me nga taumata tiketike o te Hypoglycin A.

Kia pai ai te whakautu ki nga mate ohorere a te tamaiti, te metabolic, me te paihana, whakaarohia te tango i te awhina tuatahi me te akoranga CPR mai i te Red Cross, St John Ambulance, tetahi kaiwhakarato whakangungu ohorere a rohe ranei.

Nga kaiarahi e pa ana¶

• Sulfonylurea (Glipizide, Glyburide) He nui te horopeta, Hipoglycemia Recurrent, me te Triage Octreotide
• Tūnga Epilepticus, Mau Roopu Roa, me nga Rongoa Whakaora
• Ka mau: Awhina Tuatahi me te Tiaki Rererangi
• Poisoning Kai, Acute Gastroenteritis, me te mate wai
• Paihana Whanui Awhina Tuatahi me te Whakamate

Nga puna me nga aratohu whai mana¶

• U.S. Centers for Disease Control and Prevention (CDC) & National Center for Disease Control (NCDC, India): Shrivastava A, et al.: Association of acute toxic encephalopathy with litchi consumption in an outbreak in Muzaffarpur, India, 2014: a case-control study (The Lancet Global Health).
• Te Manatū Hauora me te Waiora o Jamaica & Caribbean Public Health Agency (CARPHA): Nga Aratohu Whakahaere Hauora mo te Paawaa Ackee (Mai Ruaki Tiaki / Hypoglycin A Intoxication) (Kingston, Jamaica).
• U.S. Whakahaere Kai me te Taakaro (FDA): Mahiti Kawemai 21-11 & Aratohu Kaupapa Tautukunga: Te pupuri i te Ackee Keena, Whakatio ranei kei roto i te 100 ppm Hypoglycin A (United States).
• World Health Organisation (WHO) / ​​FAO Joint Expert Committee on Food Additives: Aromātai Haumarutanga o etahi Whakakino Kai: Hypoglycin A i Ackee Fruit (Blighia sapida) me MCPG i Sapindaceae Fruits (Geneva).
• Sherratt HSA / Tanaka K, Isselbacher KJ: Hypoglycin, te paitini o te hua ackee kare i maoa: te tikanga matū koiora hei aukati i te beta-oxidation me te gluconeogenesis (Biochemical Pharmacology / New England Journal of Medicine).