Leungo la Ackee (Hypoglycin e e sa Butswang Bolwetse jwa go Kgwa jwa Jamaica) le Lychee (MCPG) Bolwetse jwa Boboko jwa go Itima dijo jwa Sukiri mo Baneng¶

Go kgwa ka tshoganyetso mo mosong, go lapa thata, go thuthumela ka kakaretso, kgotsa go idibala morago ga go ja leungo la ackee le le sa buduleng (Blighia sapida) kgotsa go ja litchi (Litchi chinensis) fa o sa je sepe kwantle ga dijo tsa maitseboa ke boemo jo bo masisi jwa metaboliki jo bo bakiwang ke hypoglycemia e e seng ya ketotic (blood glucose often below 20 mg/dL or 1.1 mmol/L). Leletsa nomoro ya tshoganyetso ya lefelo la lona (911 kwa United States le Canada, 112 go ralala Kopano ya Yuropa, 999 kwa United Kingdom) kgotsa ikgolaganye le dinomoro tsa maemo a tshoganyetso tsa boditšhabatšhaba ka bonako, mme o bolelele moromedi gore molwetse o tlhoka go tlhatlhobiwa ka bonako ga sukiri mo mading le 10% Dextrose e e tsenngwang mo tshikeng. Fa o ntse o emetse bathusi ba kalafi, laola go thunya go go matlhagatlhaga o dirisa Porothokholo ya boemo jwa sethoathoa nako e telele, go farologanya mathata a metaboliki le a a bakiwang ke melemo selekanyo se se feteletseng sa sulfonylurea, le go dirisa kakaretso go tsenwa ke botlhole jo bo tseneletseng.

Bobedi ackee (leungo la bosetšhaba la Jamaica, le le jalwang thata go kgabaganya Caribbean, Afrika Bophirima, Florida, le Amerika Bogare) le lychee (mmogo le longan le rambutan, ditsala tsa lelapa la dimela Sapindaceae) di na le di-amino acid tse di maatla tsa cyclopropyl promoL-methylenecyclopropylalanine) le setshwani sa yone se se kwa tlase alfa-(methylenecyclopropil)glycine (MCPG). Fa ngwana kgotsa mogolo a ja di-ackee arils tse di sa butswang, tse di butsweng ka pateletso (kgotsa a nwa metsi a ackee e neng e bedisitswe mo go one), kgotsa fa ngwana yo o sa fepiwang sentle a ja di-lychee tse dintsi tse di sa buduleng/tse di buduleng go sekae motshegare mme a robala ntle le go ja dijo tsa maitseboa remains for . 4 to 12 hours fa mmele o sa ntse o na le glycogen ya sebete e e bolokilweng. Mo diureng tsa makuku (typically 3:00 AM to 8:00 AM), fa glycogen ya sebete e setse e fedile bosigo jotlhe, mmele o leka go fetogela kwa beta-oxidation ya diesiti tsa mafura le gluconeogenesis go boloka boboko bo tlamelwa ka sukiri. Ka gonne MCPA-CoA (go tswa mo Hypoglycin A) le MCPF-CoA (go tswa mo MCPG) di thibela ka tsela e e ka se busediweng moragoMCAD)** le diensaeme tsa gluconeogenic, sukiri mo mading e wela tlase ka bonako go ya kwa lefelong fa diesiti tse di botlhole tsa ketane e khutshwane di tlala mo bobokong, di baka go ruruga ka bonako ga boboko, status epilepticus, le loso mo teng ga . 12 to 48 hours ntle le fa e le ka bonako 10% ya Dekestrose (D10W) bolus le go tshelwa ga glucose e e tswelelang pele ya selekanyo se se kwa godimo di a tsenngwa.

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

Dira se jaanong¶

1. Leletsa ditirelo tsa tshoganyetso ka bonako mme o kope yuniti ya kalafi e e nang le Dextrose e e tsenngwang mo ditshikaneng. Letsa 911 (US le Canada), 112 (Kopano ya Yuropa), 999 (Bogosi jo bo Kopaneng), 119 / 911 (Jamaica le Caribbean), kgotsa 108 / 112 (India). Bolelela moromedi gore molwetse o jele leungo la ackee kgotsa lychee mme o na le go fokotsega ga sukiri mo mading a a botlhole le bolwetse jwa boboko jwa metaboliki.
2. Fa motho a tsogile ka botlalo mme a kgona go kometsa ka pabalesego, mo neye sukiri e e dirang ka bonako ya molomo ka bonako. Tlhatswa 2 to 3 tablespoons (30 to 45 grams) ya sukiri ya tafole, tswina ya dinotshe, sirapo ya mmidi kgotsa glucose powder mo galaseng ya metsi kgotsa matute a maungo mme o dire gore motho yoo a e nwe gone fela foo. Le fa mongwe a ka lemoga gore ba sa tswa go ja ackee e e sa buduleng kgotsa ngwana a ne a ja di-lychee a sa ja sepe mme a sa ntse a ikutlwa a tlwaelegile gotlhelele, go naya dijo tse di nang le dikhabohaetereite tse dintsi le seno se se nang le sukiri ka bonako go dira gore mabenkele a glycogen a sebete a nne a tletse mme go thibela mmele go dira gore tsela e e thibetsweng ya go tsamaisa mafura e dire.
3. Fa motho a otsela, a tlhakane tlhogo, a tlhatsa, kgotsa a na le seterouku, O SE KA WA tshela diedi mo molomong wa gagwe. Pitikolola molwetse mo letlhakoreng la gagwe la molema (boemo jwa go fola) go dira gore matlhatsa a se ka a tsena mo mafatlheng. O ka nna wa itlotsa ka bonolo selekanyo se sennye sa gel ya glucose, tswina ya dinotshe, kgotsa sukiri ya mo tafoleng ka tlhamalalo mo teng ga lerama le le kwa tlase le marinini (buccal mucosa), mme go namola ka botlalo go tlhoka Dextrose ya 10% e e tsenngwang mo ditshikaneng (D10W) e e tsamaisiwang ke bathusi ba kalafi kgotsa badiri ba lefapha la maemo a tshoganyetso.
4. Tlhaloganya gore ke eng fa go tlhaba Glucagon go SA bereke mo go tsenweng ke botlhole jwa Ackee kgotsa Lychee. Malapa kgotsa bathusi ba kalafi ba ba tshotseng didirisiwa tsa go tlhaba glucagon tsa tshoganyetso ba tshwanetse go itse gore glucagon e dira fela ka go tlhotlheletsa sebete go thuba glycogen e e bolokilweng (glycogenolysis) le go dira sukiri e ntšha (gluconeogenesis). Mo bolwetseng jwa boboko jwa ackee le lychee, glycogen ya sebete e setse e 100% e fedile mme go tlhamiwa ga sukiri go thibelwa ke Hypoglycin A le MCPG ka dikhemikhale tsa ditshedi, se se rayang gore glucagon ga e dire gotlhelele. Ke fela tiriso e e tlhamaletseng ya dextrose (sukiri) e e tswang kwa ntle e e bolokang boboko.
5. Boloka diphotlwa dipe tse di setseng tsa ackee tse di tala kgotsa tse di apeilweng, metsi a go apaya, kgotsa leungo la lychee. O se ka wa letla ope yo mongwe mo lelapeng go ja mo pitseng e le nngwe, bogolo jang metsi a go apaya (pot water) kgotsa moro o mo go one go neng go bedisiwa di-ackee aril, jaaka fa Hypoglycin A e e nwelelang mo metsing e elela ka tlhamalalo mo seeding.

Setlhare sa tshwetso: Tlhatlhobo ya bolwetse jwa boboko jwa Ackee le Lychee¶

flowchart TD
    A["Go Belaelwa Go Metsa Ackee e e sa Butswang kgotsa Lychee e e sa Butswang"] --> B{"Tlhatlhoba Boemo jwa Tleliniki le Boitemogo"}
    B -->|"Asymptomatic (Go Metsa Bosheng, Go Tsoga ka Botlalo)"| C["Naya Dijo tse di Nang le Dikhabohaetereite tse dintsi ka Bofefo + Seno sa Sukiri sa Molomo go Boloka Sukiri ya Sebete"]
    C --> D["Amogelwa kwa Bookelong Diura di le 24-48 Tshelo ya Dextrose ya 10% e e Tswelelang le Ditshekatsheko tsa Sukiri ka Menwana ya Ura le Ura"]
    B -->|"Go kgwa ka tshoganyetso, go fufula, go lapa, go thuthumela, kgotsa go idibala"| E["Tlhola Dipalopalo tsa Sukiri mo Mading (Gantsi < 20-30 mg/dL / 1.1-1.7 mmol/L) + Gase ya Madi/Laktate"]
    E --> F["Bolus ya IV ya ka bonako: 10% ya Dekestrose (D10W) 2 go ya go 5 mL/kg Kgarametso ya IV (Bagolo: 50 mL ya D50W kgotsa 250 mL ya D10W)"]
    F --> G["Simolola IV e e Tswelelang 10% Dekestrose (D10W mo 0.9% NaCl) ka Seelo sa Tshelo ya Sukiri (GIR) sa 6 go ya go 8 mg/kg/motsotso"]
    G --> H{"Matshwao a Seemo se se Dirang sa Sethoathoa kgotsa sa Edema ya Boboko?"}
    H -->|"Ee"| I["IV Lorazepam/Midazolam + Tsholetsa Tlhogo ka Didikirii di le 30 + 3% ya Letswai le le Feteletseng (Tila Diedi tse di Feteletseng)"]
    H -->|"Nnyaa"| J["Boloka Sukiri ya Madi e le 100-150 mg/dL (5,5-8,3 mmol/L); Tsenya L-Carnitine + Glycine + Riboflabine (Bithamini B2)"]
    I --> J

Bolwetse: Kafa Hypoglycin A le MCPG di thibelang go fetoga ga maatla a motho ka gone¶

Go tlhaloganya gore ke eng ngwana yo o itekanetseng a ka robala a nyenya fa a sena go ja di-lychee kgotsa di-ackee mme a tsoge ka 10.00. 5:00 AM mo koma e e bolayang ya go thunya, dingaka di tshwanetse go sekaseka thibelo e e tlhomameng ya ensaeme ya mitochondrial e e bakiwang ke Hypoglycin A le MCPG (e gantsi e bidiwang **mofuta wa II wa glutaric acidemia e e bonweng kgotsa go tlhaela ga acyl-CoA dehydrogenase e e bonweng [ 1 ].MADD]).

1. Go fetola diporotokisine go nna di-thioester tsa CoA tse di bolayang¶

Le fa e le Haipoglycin A (L-alpha-amino-beta-[methylenecyclopropyl]propionic acid) kgotsa MCPG (alpha-[methylenecyclopropyl]glycine) e botlhole ka tlhamalalo ka sebopego sa yone sa amino acid e e tala. Fa di sena go monyelwa go tswa mo maleng a mannye, di tsena mo tseleng ya catabolic ya amino acid ya ketane e e nang le dikala tsa mitochondrial: * Ditransaminase tsa saetesoli le tsa maithokondria di fetola Hypoglycin A go nna esiti ya methylenecyclopropilpyruvic (MCPP), le esiti ya alfa-keto ya ketane e e nang le dikala e e ntsha dikhaboksiele go nna botlhole jo bo bolayang jo bo matlhagatlhaga: methylenecyclopropylacetyl-CoA (MCPA-CoA). * Fela jalo, MCPG (e e ntsi mo dipeong tsa litšhi le di-aril tse di sa butswang/tse di buduleng go sekae, mmogo le dipeo tsa ackee) e fetolwa go nna methylenecyclopropilformyl-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. Ke ka ntlha yang fa beta-oksijene ya diesiti tsa mafura le gluconeogenesis ka bobedi di palelwa¶

1. Thibelo ya go ipolaya ya Flavin (FAD) Di-Dehydrogenase tsa Acyl-CoA: MCPA-CoA le MCPF-CoA di dira jaaka dithibela-dinyukleotaete tsa flavin adenine (FAD) setlhopha sa maitirelo mo maitokhondrialeng ketane e khutshwane ya asile-CoA dehaeterojenase (SCAD), dehaeterojenase ya asile-CoA ya ketane e e magareng (MCAD), isovalerile-CoA dehaeterojenase (IVD), le glutarale-CoA dehaeterojenase (GCDH). Ka gonne mmele o sa tlhole o kgona go oksidisa diesiti tsa mafura ka nako ya go itima dijo, dithishu di patelesega go fisa sukiri ka lobelo lo logolo fa ketone body synthesis (acetoacetate le beta-hydroxybutyrate) e thibetswe gotlhelele.
2. Go Kgaolwa ga Gluconeogenesis ya Sebete: Fa glycogen ya sebete e fedile (4 to 10 hours morago ga dijo tsa bofelo, ka bonako thata mo baneng ba ba botlana ba ba nang le ditekanyetso tse di kwa godimo tsa bokete jwa boboko le sebete kgotsa dipolokelo tse di sa tlhomamang tsa dikotla), sebete se leka go dira sukiri e ntšha go tswa mo lactate, alanine, le glycerol ka gluconeogenesis. Le fa go ntse jalo, ensaeme ya ntlha e e lekanyetsang seelo sa go tlhamiwa ga sukiri, pyruvate carboxylase, e tlhoka acetyl-CoA jaaka setlhotlheletsi sa allosteric se se tlamang le ATP + NADH (e e tlhagisiwang ke beta-oksijene ya esiti ya mafura) gore e tsamaye. Mo godimo ga moo, diester tsa acyl-CoA tse di sa fetolwang le MCPA-CoA Khonsaeme A (CoA-SH) le L-khanitine. Kwa ntle ga ATP, NADH, acetyl-CoA, kgotsa mahala CoA-SH, gluconeogenesis ya sebete e a ema gotlhelele.
3. Go kgobokana ga Diesiti tsa Ditshedi tse di nang le Makala le tsa Ketane e Khutshwane tse di Botlhole jwa Methapo: Go thibelwa ga beta-oksijene le go fetoga ga diesiti tsa amino go baka go kokoana go gogolo ga diesiti tsa mafura tsa ketane e khutshwane le e e magareng tse di sa tlhomamang (**butyric, isobutyric, isovaleric, 2-methylbutyric, 2-methylbutyric,). Diesiti tseno tsa ditshedi di thibela dipompo tsa boboko tse di sireletsang tsa thulaganyo ya methapo ya bogare, di tsosa kgaolo ya go tsosa di-chemoreceptor mo kutung ya boboko (di baka go kgwa go go thunyang ka tshoganyetso), di kgaoganya di-mitochondria tsa di-neuron, mme di ntsha edema ya boboko e e nang le botlhole jwa disele e e ikemetseng ka nosi mo sukiri e e kwa tlase mo mading ka boyone.

Go bapisa Bolwetse jwa go Kgwa jwa Jamaica (Ackee) kgatlhanong le Bolwetse jwa Boboko jwa Lychee jwa go Itima dijo¶

Porothokholo ya tshoganyetso ya bookelo ya kgato ka kgato¶

Fa ngwana kgotsa mogolo a goroga a kgwa, a fetogile seemo sa tlhaloganyo, kgotsa a thuthumela morago ga go ja ackee kgotsa lychee, motsotso mongwe le mongwe wa hypoglycemia e e sa alafiwang le organic acidemia o oketsa kotsi ya tshenyo ya boboko ya leruri ya hypoxic-ischemic kgotsa go tswa ga boboko go go bolayang.

1. Dekestrose ya 10% e e Tshelwang ka bonako mo Tshikeng (D10W) Go tsosolosa¶

O se ke wa emela diphanele tsa khemiseteri ya laboratori ho kgutla pele o fana ka dextrose; dira tlhatlhobo ya sukiri mo mading ka monwana ka bonako fa thoko ga bolao (mme o thale tšhupu ya madi e e khibidu/tala/phepole e e kwa godimo go dira porofaele ya botlhole/acylcarnitine fa go kgonega, kwantle ga go diegisa kalafi ka 60 seconds).

• Goreng 10% ya Dekestrose (D10W) e ratwa go feta 25% kgotsa 50% ya Dextrose mo baneng: E e tseneletseng 50% deketerose (D50W, 2,525 mOsm/L) e na le hyperosmolar e e tseneletseng mme e baka bolwetse jo bo masisi jwa go phatloga ga ditshika, go bola ga dithishu fa di ka tsenngwa kwa ntle ga madi, le go fetoga ga osmotic ka bonako go go kotsi go kgabaganya sekgoreletsi se se kotsi sa madi le boboko sa bana. Tlwaelo 5% deketerose (D5W) ga e ntshe sukiri e e lekaneng go gatelela go kokoanngwa ga diesiti tsa mafura tse di mo mmeleng. 10% Dekestrose (D10W, 100 mg of glucose per mL) ke tekanyetso ya gauta ya di-bolus tsa ntlha le di-infusion tsa tlhokomelo e e tswelelang.
• Selekanyo sa ntlha sa IV Bolus:
• Bana: Tsamaisa 2 to 5 milliliters per kilogram (2 to 5 mL/kg, go tlamela ka 0.2 to 0.5 grams/kg ya sukiri) ya 10% Dekestrose (D10W) ka ditshika mo godimo ga 2 to 5 minutes.
• Bagolo: Tsamaisa 250 milliliters ya 10% Dekestrose (D10W) (kgotsa 50 milliliters ya 50% Dekestrose [D50W]) ka ditshika.
• Kelo e e kwa Godimo e e Tswelelang e e Patelediwang 10% Tshelo ya Deketerose (To Shut Down Lipolysis):
• Molao o o botlhokwa wa fisioloji: Bolus e le nngwe ya dextrose e tlhatlosa sukiri mo mading ka 20 to 30 minutes. Se se maswe le go feta, mo go tsenweng ke botlhole jwa Hypoglycin A le MCPG, boikaelelo jwa kalafi ya dextrose ga se fela go boloka sukiri mo mading e le kwa 70 mg/dL**, mme go tlhotlheletsa go gololwa ga insulin go go lekaneng ga endogenous go tswala lipase e e bosisi mo dihomoneng ka botlalo mo dithishung tsa mafura. Fa fela mmele o leka go kokoanya diesiti tsa mafura tse di sa duelelweng go tswa mo mabenkeleng a mafura, botlhole . MCPA-CoA, MCPF-CoA, mme diesiti tsa ditshedi tse di nang le ketane e khutshwane tse di sa tlhomamang di tswelela di kgobokana mo teng ga di- mitochondria mme di tsenya boboko botlhole.
• Seelo sa Tshelo (Glucose Infusion Rate [GIR]): Ka bonako fela simolola go tshelwa 10% Dekestrose mo 0.9% Letswai le le Tlwaelegileng (D10NS) (kgotsa D10 1/2NS ka potasiamo e e tshwanetseng fa tiro ya diphilo e netefaditswe) ka Seelo sa Tshelo ya Sukiri (GIR) ya 6 to 8 milligrams per kilogram per minute (roughly 3.6 to 4.8 mL/kg/hour ya D10W), go lekanyetsa go boloka sukiri mo serumong fa gare ga 100 and 150 mg/dL (5.5 to 8.3 mmol/L) bonnye jwa 24 to 48 hours.

2. Sedirisiwa sa Metaboliki le Phalotso ya go Kopanya Dijo (L-Carnitine, Glycine, le Riboflavin)¶

Ka gonne MCPA-CoA le MCPF-CoA go tshwara Khoensaeme A yotlhe e e sa duelelweng ya maithokhondria (CoA-SH) le carnitine fa di ntse di senya dinyukleotaete tsa flavin adenine (FAD) di-cofactor, diporothokholo tsa botlhole jwa kalafi di dirisa ditlaleletsi tse tharo tse di lebilweng tsa metaboliki go tlosa dilo tse di fa gare tse di botlhole:

1. L-Carnitine e e tsenngwang mo tshikeng kgotsa e e nowang (Levocarnitine):
2. Mokgwa wa go dira: L-carnitine ya tlaleletso e bofa dimaekhondria tse di tshwaregileng MCPA-CoA, MCPF-CoA, isovaleryl-CoA, le dimolekhule tsa acyl-CoA tsa ketane e e magareng ka go dirisa asyltransferase ya carnitine, e di fetola go nna diester tsa asilcarnitine tse di nwelelang mo metsing (MCPA-carnitine) tse di pompiwang go tswa mo di-mitochondria di bo di ntshiwa mo motlhapong, di golola dikarolo tse di botlhokwa CoA-SH bakeng sa potoloho ya Krebs.
3. Selekanyo: 100 milligrams per kilogram per day (100 mg/kg/day) ka tshika (kgotsa ka molomo fa e tsogile) e kgaogantswe nngwe le nngwe 6 to 8 hours (up to 2 to 3 grams/day).
4. Glycine e e nowang kgotsa e e tsenngwang mo mpeng:
5. Mokgwa wa go dira: Ensaeme ya maetokhondria galasine N-asiletransferase e e kopantsweng MCPA-CoA le MCPF-CoA ka esiti ya amino glycine go bopa MCPA-glycine le MCPF-glycine tse di seng botlhole, tse di phepafadiwang ka bonako ke diphilo.
6. Selekanyo: 150 to 250 milligrams per kilogram per day ka molomo kgotsa ka tšhupu ya nasogastric e e kgaogantsweng nako le nako 6 to 8 hours.
7. Riboflavin ya Selekanyo se se Kwa Godimo (Vitamin B2):
8. Mokgwa: Ka gonne MCPA-CoA e dira gore FAD setlhopha sa maitirelo sa di-dehydrogenase tsa acyl-CoA, tse di tlamelang ka selekanyo se se kwa godimo sa Riboflavin (the direct precursor of FAD and FMN, 100 milligrams ka molomo kgotsa ka tšhupu ya NG makgetlo a le 2 go ya go a le 3 ka letsatsi) e itlhaganedisa go tsosolosiwa ga MCAD le SCAD diensaeme.

3. Go Laola go Thuthumela, go Ruruga ga Boboko le go Nna le Acidosis¶

• Taolo ya go thuthumela: Go baakanya hypoglycemia ka 10% Dextrose e emisa go thunya ka bonako mo balwetseng ba le bantsi ba ba tlhagelelang go sa le gale. Fa go thuthumela go tswelela le fa sukiri mo mading e tlwaelegile (due to already-established cerebral edema or organic acid neurotoxicity), tsenya lorazepam (0.1 mg/kg IV), midazolamo (0.1 to 0.2 mg/kg IV/IM), kgotsa lefetirasetamo (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).
• Taolo ya edema ya boboko: Tsholetsa tlhogo ya bolao go 30 degrees, go boloka nomokhabia (PaCO2 35 to 40 mmHg) ka go tsenngwa ga tšhupu mo mohelong go sa le gale fa e le Sekale sa Glasgow Coma (GCS) ke < 8, le go tshela 3% ya Letswai le le Feteletseng (3 to 5 mL/kg IV mo godimo ga 15 to 20 minutes) kgotsa Manitole (0.5 g/kg IV).
• Sodiamobaekhaboneite: Mo bolwetseng jo bo tseneletseng jwa asitose ya metaboliki (arterial pH < 7.15), go tsenya sodium bicarbonate ka kelotlhoko mo ditshikaneng fa o ntse o boloka go tsenngwa ga sukiri ka metlha.

Thibelo: Tsela ya go lemoga Ackee e e buduleng e e babalesegileng go tswa mo Ackee e e sa butswang e e botlhole, le melao e e babalesegileng ya Lychee ya bana¶

1. Melao e 4 ya Gauta ya go Baakanyetsa Ackee e e Babalesegileng¶

+-------------------------------------------------------------------------+
|         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. A lephutshe le "yawn" le bulege ka tlhago mo setlhareng: O se ka wa roba kgotsa wa apaya lephutshe la ackee le le sa kgaoganang ka tlhago fa le santse le lepeletse mo setlhareng. Le ka motlha o se ke wa pateletsa lephutshe le le tswetsweng go bulega, mme le ka motlha o se ke wa tlogela diphotlwa tse di tswetsweng mo fensetereng e e nang le letsatsi kgotsa marulelo o emetse gore di bulege go tswa mo setlhareng (diphotlwa tse di kgaoganeng ga di sutise Hypoglycin A sentle go tswa mo di-arils go tsena mo dipeong).
2. Tlosa peo nngwe le nngwe le motlhala mongwe le mongwe wa lera le le pinki/lihibidu (raphe): Dipeo tse dintsho tse di phatsimang le lera le le bohibidu jo bo pinki le le gokelelang aril e e serolwana mo sejaneng sa dipeo di nna di le botlhole go ya go ile (containing high levels of Hypoglycin A and B) le mo maungong a a boduleng ka botlalo. Gouge tsoa lera lohle le pinki mme o hlatswe arils tse tshehla hantle metsing a batang a phallang pele o pheha.
3. Bedisa ka go kgaogana mme KA METLHA o latlhele metsi a go apaya: Bedisa di-ackee arils tse di phepafaditsweng mo metsing a a tlwaelegileng pele, di tlhotlhore mo colander, mme o tshele metsi a a belang ka tlhamalalo mo sinking. Le ka motlha o se ke wa apaya raese, yam, panana, di-dumpling kgotsa sopo mo metsing a a dirisiwang go bedisa ackee.
4. Pabalesego ya Ackee e e rekwang mo makotikoting: Kwa United States, Canada, UK, le EU, dithomeloteng tsa ackee tse di mo makotikoting tsa kgwebo di bewa leitlho thata ke US FDA le balaodi ba pabalesego ya dijo go netefatsa gore dilekanyo tsa Hypoglycin A di kwa tlase ga . 100 parts per million (ppm). Le ka ackee e e rekisiwang e e mo makotikoting, ntsha metsi le go tlhatswa letswai pele ga o gadika ka tlhapi ya letswai.

2. Melao e 3 ya Pholo ya Setšhaba e e Fedisitseng go Runya ga Bolwetse jwa Boboko jwa Lychee jwa Bana¶

Morago ga dipatlisiso tse di kopanetsweng tse di botlhokwa tse di dirilweng ke Setheo sa Bosetšhaba sa India sa Taolo ya Malwetse (NCDC) le US CDC kwa Muzaffarpur, Bihar (kwa makgolokgolo a bana ba neng ba swa ka Motsheganong mongwe le mongwe le Seetebosigo ka nako ya thobo ya lychee), balaodi ba boitekanelo jwa setšhaba ba ne ba tlhoma melao e meraro e e bonolo ya lelapa e e neng ya fokotsa dintsho ka go feta 100. 80%: 1. O se ka wa letlelela bana go robala ba sa ja dijo tsa maitseboa tse di apeilweng (dijo tsa maitseboa): Go ja dijo tsa maitseboa tse di tlwaelegileng tse di nang le dikhabohaetereite tse dintsi go tlatsa mabolokelo a glycogen a sebete ka jalo sebete sa ngwana ga se ke se tshwanelwa ke go ikaega ka beta-oxidation ya fatty acid e e thibegileng bosigo jotlhe. 2. Fokotsa palo ya di-litchi tse bana ba bannye ba di jang, mme o tile maungo a a sa butswang/a a wetseng fa fatshe: O se ka wa letla bana go ja di-litchi tse di sa butswang kgotsa tse di buduleng ka sephatlo go tswa mo mmung mo masimong a maungo, ka gonne di-litchi tse di sa butswang di na le selekanyo se se fetang gabedi sa MCPG le Hypoglycin red A se se fitlhelwang mo 3. Naya sukiri ya molomo ka bonako mme o itlhaganele kwa tleliniking e e nang le 10% Dextrose ka letshwao la ntlha la mo mosong: Ngwana mongwe le mongwe mo kgaolong ya lychee kgotsa ackee yo o tsogang a kgwa, a lapile, kgotsa a roroma o tshwanetse go amogela 10% Dekestrose mo lobakeng lwa ura ya ntlha (administering D10W within 4 hours of symptom onset reduces fatality from > 44% to < 5%).

Diporothokholo tsa kgaolo tsa maemo a tshoganyetso le tsa taolo ya botlhole¶

• Jamaica le Caribbean: Leletsa 119 (Ambulense / Sepodisi sa Jamaica) kgotsa 911 / 999 (go kgabaganya mafelo a Caribbean) mme o itlhaganele ka bonako kwa lephateng la tshoganyetso la bookelo le le gaufi go ya go tsenngwa madi mo ditshikaneng 10% Dekestrose. Lefapha la Boitekanelo le Boitekanelo la Jamaica le boloka diporothokholo tsa go tsosolosa Ackee Poisoning (Bolwetse jwa go Kgwa ga Jamaica) tse di emeng kwa dikokelong tsotlhe tsa pharishe le kwa mafelong a boitekanelo.
• Dinaga tse di Kopaneng le Canada: Leletsa 911 ka bonako mme o leletsa 1-800-222-1222 (Thuso ya Botlhole ya Amerika) kgotsa 1-844-BOJHELE-X (1-844-764-7669 kwa Canada). Ackee e e foreše e gola kwa Florida Borwa (where trees are occasionally harvested by residents unfamiliar with the "natural yawn" rule), mme ackee e e gatseditsweng/e e mo makotikoting e rekisiwa mo mebarakeng ya Caribbean go ralala Amerika Bokone.
• India, Vietnam, Bangladesh, le Borwabotlhaba jwa Asia: Leletsa 108 kgotsa 112 (India), 115 (Vietnam), kgotsa 999 (Bangladesh/Malaysia). Ka nako ya Motsheganong go ya go Phukwi litchi (litchi), longan, le rambutan paka ya thobo, mafelo a motheo a boitekanelo kwa Bihar (India) le kwa bokone jwa Vietnam a na le di- glucometer tse di fa thoko ga malao le 10% Dikgetsi tsa Dextrose ka tlhamalalo tsa go namola bolwetse jwa boboko jwa hypoglycemic jwa bana ka bonako.
• Bogosi jo bo Kopaneng, Kopano ya Yuropa, le Aforika Bophirima: Leletsa 999 (UK), 112 (EU), kgotsa 112 / 199 (Nigeria, Ghana, Côte d’Ivoire, Burkina Faso, kwa Blighia sapida e golang ka bontsi gone mme e baka botlhole jo bo itsiweng jwa ngwana wa paka "ackee poisoning" kgotsa "finsan" botlhole). Dingaka kwa UK/EU di tshwanetse go buisana le NPIS / TOXBASE kgotsa kwa mafelong a bosetšhaba a botlhole go bona netefatso ya go tlhatlhoba acylcarnitine/esiti ya dibodi mo motlhapong le L-carnitine go lekanyetsa.

Dipotso tse di bodiwang kgapetsakgapetsa¶

Ke ka ntlha yang fa bana ba ba tsentsweng botlhole ke di- lychee kgotsa di- ackee tse di sa butswang gantsi ba lwala go sa le phakela?¶

Hypoglycin A le MCPG di thibela diensaeme tsa mitochondria tse di tlhokegang go fisa mafura a mmele le go dira sukiri e ntšha (gluconeogenesis), mme ga di thibele sebete go golola glycogen e e setseng e bolokilwe. Fa ngwana a ja ackee e e sa buduleng kgotsa a ja di- lychee mme a robala a sa ja dijo tsa maitseboa, glycogen ya gagwe e e bolokilweng ya sebete e dira gore sukiri ya gagwe mo mading e nne e le mo maemong a a siameng mo diureng tsa ntlha tse 4 go ya go tse 10; fa gare ga 3:00 AM le 8:00 AM, glycogen ya sebete e a fela gotlhelele, mme seno se dira gore sukiri mo mading e wele kwa tlase ga 20 mg/dL fa diesiti tse di mafura tse di botlhole di tlala mo bobokong.

Ke ka ntlha yang fa tlhabelo ya Glucagon ya tshoganyetso e sa bereke mo bolwetseng jwa go fokotsega ga sukiri mo mading jwa Ackee kgotsa Lychee?¶

Glucagon e tlhatlosa sukiri mo mading fela ka go laela sebete go thuba glycogen e e bolokilweng le go dira sukiri e ntšha ka gluconeogenesis. Mo go tsenweng ke botlhole jwa ackee le lychee, glycogen ya sebete sa molwetse e setse e se na sepe gotlhelele mme diensaeme tsa tsona tsa gluconeogenic di notletswe ka baeyokhemikhale ke MCPA-CoA le MCPF-CoA, go kaya gore glucagon ga e na phelelo ya lefela; molwetse o tshwanetse go amogela sukiri e e tlhamaletseng ya molomo (fa a tsogile) kgotsa 10% Dextrose e e tsenngwang mo tshikeng.

Ke ka ntlha yang fa Esiti ya Valproic (Depakote) e le kotsi mo go thuthumeng go go bakiwang ke go tsenwa ke botlhole jwa Ackee kgotsa Lychee?¶

Esiti ya Valproic ke esiti ya mafura e e nang le ketane e e nang le dikala e ka boyone e jang L-carnitine ya mitochondrial, e thibela diensaeme tsa beta-oksijene ya esiti ya mafura, le go tsholetsa ammonia mo mading. Go naya molwetse yo tsela ya gagwe ya beta-oxidation e setseng e tsentswe botlhole ke Hypoglycin A kgotsa MCPG esiti ya valproic go gakatsa thata go nna le asiti ya metaboliki, go nna le madi a mantsi thata mo mmeleng le go ruruga ga boboko go go bolayang; dingaka di tshwanetse go dirisa diokobatsi tsa diokobatsi, levetiracetam (Keppra), kgotsa phenobarbital mmogo le 10% ya Dextrose boemong jwa tsone.

O ka bona jang gore leungo le le foreshe la Ackee le babalesegile go le kgetla le go le apaya?¶

Lephutshe le le foreshe la ackee le babalesegile fela fa le fetogile bohibidu jo bo galotseng le bosetlha-lamune mme le kgaogane ka bophara ka tlhago ("yawned") fa le santse le lepelela mo setlhareng mo letsatsing, le senola ka botlalo dipeo tse tharo tse dintsho tse di phatsimang. Le ka motlha o se ke wa apaya lephutshe le letala le le sa bulwang, le ka motlha o se ke wa pateletsa lephutshe go bulega ka thipa, ka metlha tlosa dipeo tse dintsho le lera lotlhe le le bohibidu jo bo pinki, mme ka metlha o tshele metsi a a belang mo moseleng.

Ke ka ntlha yang fa dingaka di boloka 10% Dextrose IV drip e tsamaya diura di le 24 go ya go di le 48 le fa sukiri mo mading e sena go boela kwa maemong a a tlwaelegileng?¶

Go naya bolus e le nngwe ya glucose go baakanya sukiri e e kwa tlase mo mading metsotso e le 20 go ya go e le 30 fela. Go tshelwa ga Dextrose ya 10% ka selekanyo se se kwa godimo (ka 6 go ya go 8 mg/kg/motsotso) go dira gore sukiri mo mading e nne kwa godimo go sekae (100 go ya go 150 mg/dL), e leng se se tlhotlheletsang insulin ya molwetse go thibela go thubega ga mafura mo ditogweng tsa mafura le go thibela mmele go ntsha enzyme e ntsi ya neurototil fola.

A dipitse le dintša le tsone di tsentswe botlhole ke Hypoglycin A le MCPG go tswa mo dipeong tsa ditlhare?¶

Ee, dipitse tse di fulang mo mafulong ka nako ya letlhafula le dikgakologo gantsi di nna le boemo jo bo bolayang ka bonako jo bo bidiwang Equine Atypical Myopathy (kwa Yuropa/UK) kgotsa Seasonal Pasture Myopathy (kwa Amerika Bokone) morago ga go ja dipeo tse di nang le diphuka ( le ditlhare tsa Box Elder (Acer negundo), tse di leng tsa lelapa le le tshwanang la Sapindaceae jaaka ackee le litchi mme di na le maemo a a kwa godimo a Hypoglycin A.

Go tsibogela ka katlego maemo a tshoganyetso a bana, a metaboliki, le a go tsenwa ke botlhole, akanyetsa go tsaya khoso ya thuso ya potlako le CPR go tswa go Sefapaano se Sehibidu, St John Ambulance, kgotsa moabi wa katiso ya maemo a tshoganyetso wa lefelo leo.

Dikaedi tse di tsamaelanang¶

• Go dirisa Sulfonylurea (Glipizide, Glyburide) go feta selekanyo, go fokotsega ga sukiri mo mading gangwe le gape, le go Tlhaola Octreotide
• Boemo jwa Sethoathoa, Go Thuthumela ka Nako e Telele, le Melemo ya Phalotso
• Go thuthumela: Thuso ya Ntlha le Tshireletso ya Tsela ya Mowa
• Botlhole jwa Dijo, Gastroenteritis e e Bogale, le go Felelwa ke Metsi mo Metsi
• Thuso ya Ntlha ya Botlhole ka Kakaretso le go Tlhatswa Kgotlelego

Metswedi le kaelo ya semmuso¶

• U.S. Ditsi tsa Taolo le Thibelo ya Malwetse (CDC) & Setsi sa Bosetšhaba sa Taolo ya Malwetse (NCDC, India): Shrivastava A, le ba bangwe: Kamano ya bolwetse jo bo botlhole jo bo botlhoko jwa boboko le tiriso ya litchi mo go runyeng ga bolwetse kwa Muzaffarpur, India, 2014: thutopatlisiso ya taolo ya dikgetse (The Lancet Global Health).
• Lefapha la Boitekanelo le Boitekanelo la Jamaica & Setheo sa Boitekanelo jwa Setšhaba sa Caribbean (CARPHA): Dikaelo tsa Taolo ya Kalafi ya Botlhole jwa Ackee (Bolwetse jwa go Kgwa jwa Jamaica / Botagwa jwa Hypoglycin A) (Kingston, Jamaica).
• RONA. Tsamaiso ya Dijo le Melemo (FDA): Tlhagiso ya Thomeloteng 21-11 & Kaedi ya Pholisi ya Kobamelo: Go Tshwara Ackee e e mo Makotikoting kgotsa e e Gatseditsweng e e Nang le Hypoglycin A e e Fetang 100 ppm (United States).
• Mokgatlho wa Lefatshe wa Boitekanelo (WHO) / ​​Komiti e e Kopanetsweng ya Baitseanape ya FAO ka ga Ditlaleletsi tsa Dijo: Tlhatlhobo ya Pabalesego ya Dikgotleletsi Tse di rileng tsa Dijo: Hypoglycin A mo Maungo a Ackee (Blighia sapida) le MCPG mo Maungo a Sapindaceae (Geneva).
• *Hypoglycin, botlhole jwa leungo la ackee le le sa butswang: mokgwa wa baeyokhemikhale wa go thibela beta-oksijene le go tlhamiwa ga sukiri (Khemikale ya ditshedi / Lekwalodikgang la Kalafi la New England).