Miraha Ackee (Hypoglycin aan bislaan A Jamaican Xanuun Matag ah) iyo Lychee (MCPG) Soonka Hypoglycemic Encephalopathy ee Carruurta¶
Nambarada deg-degga ahi way ku kala duwan yihiin waddan ahaan. Boggani wuxuu isticmaalaa 911, lambarka Maraykanka iyo Kanada. Wadamo badan oo kale ayaa isticmaala 112, 999, ama nambar kale. U hel lambarka wadankaaga.
Matag subax degdeg ah, caajisnimo qoto dheer, suuxdin guud, ama miyir-beel ka dib cunista ** midho ackee aan bislaan (Blighia sapida*** ama cunista lychee (Litchi chinensisCaloosha madhan adigoon cunin casho waa xaalad degdeg ah oo dheef-shiid kiimikaad ah oo ay keento masiibo ** aan ketotic hypoglycemiablood glucose often below 20 mg/dL or 1.1 mmol/L). Wac lambarkaaga deg-degga ah (911 gudaha Mareykanka iyo Kanada, 112 guud ahaan Midowga Yurub, 999 gudaha Boqortooyada Ingiriiska) ama la tasho annaga tusaha lambarada degdega ah ee caalamiga ah isla markiiba, oo u sheeg diraha in bukaanku u baahan yahay hubinta gulukoosta dhiiga ee degdega ah iyo xididka 10% Dextrose. Inta aad sugayso dhakhaatiirta caafimaadka, maamul gariir firfircoon adoo isticmaalaya our heerka suuxdinta borotokoolka suuxdinta daba dheeraatay, kala saar dhibaatada dheef-shiid kiimikaadka iyo ka dawooyinka sulfonylurea hypoglycemic xad dhaaf ah, oo codso guud sunta degdega ah.
Labada ackee *** (miraha qaranka ee Jamaica, oo si ballaaran uga koray Caribbean-ka, Galbeedka Afrika, Florida, iyo Bartamaha Ameerika) iyo ** lychee ** (oo ay la socdaan longan iyo rambutan, ilma adeer oo ka tirsan qoyska botanical ** Sapindaceae ) waxay ka kooban yihiin borotoxins cyclopropyl amino acid awood leh: ** Hypoglycin A (L-methylenecyclopropylalanine iyo homolog-keeda hoose ** alfa- (methylenecyclopropyl) glycine (MCPG). Marka ilmaha ama qofka weyni uu cuno cunnooyin aan bislaan, qasab ah oo furan (ama cabbo biyaha lagu kariyey), ama marka ilmo nafaqo darro ahi uu cuno xaddi badan oo liish ah oo aan bislaan/bislaan maalintii oo uu seexdo iyaga oo aan casho cunin, suntan waa iska aamusaan 4 to 12 hours halka jirku wali kaydsan yahay glycogen beerka. Saacadaha hore ee subaxdii (typically 3:00 AM to 8:00 AM), marka habeen dhaxa glycogen beerka uu daalan yahay, jirku waxa uu isku dayaa in uu u wareego fatty acid beta-oxidation iyo gluconeogenesis si uu maskaxda ugu hayo gulukoos. Sababtoo ah MCPA-CoA (oo ka yimid Hypoglycin A) iyo ** MCPF-CoA (oo ka yimid MCPG) waxay si aan leexleexad lahayn u xannibaysaa mitochondrial ** silsilad dhexdhexaad ah acyl-CoA dehydrogenase (MCAD iyo enzymes gluconeogenic, gulukoosta dhiigu si xawli ah ayey ugu socotaa eber halka asiidhyada silsilada gaaban ee sunta ah ay daadiyaan maskaxda, taasoo keenta barar maskaxeed oo degdeg ah, xaalad suuxdin, iyo dhimasho gudaha ah. 12 to 48 hours haddii aan degdeg ahayn ** 10% DextroseD10W) bolus iyo faleebo gulukoos oo joogto ah* ayaa la maamulaa.
+-------------------------------------------------------------------------+
| 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 |
+-------------------------------------------------------------------------+
Hadda samee tan¶
- ** Isla markiiba wac adeegyada degdegga ah oo codso unug caafimaad oo leh Dextrose xididka ah U sheeg diraha in bukaanku uu cunay miraha ackee ama lychee oo uu la ildaran yahay ** sun ah hypoglycemia oo daran iyo encephalopathy dheef-shiid kiimikaad**.
- Haddii qofku si buuxda u soo jeedo oo uu awoodo inuu si badbaado leh u liqo, sii sokor afka oo degdegaysa isla markaaba. milmi
2 to 3 tablespoons(30 to 45 grams) sonkorta miiska, malab, sharoobada galleyda, ama budada gulukooska gala galaas biyo ah ama casiir khudrad ah oo qofka isla markiiba cab. Xitaa haddii qof uu ogaaday in ay cuneen ackee aan bislaan ama ilmo uu cunay lichees calool madhan oo weli dareemaya caadi, ** bixinta cunto karbohaydrayt ku badan iyo cabitaan sonkor leh isla markiiba ** waxay ilaalisaa kaydka glycogen ee beerka oo buuxa waxayna ka hortagtaa jirka inuu dhaqaajiyo dariiqa oksaydhka dufanka-ashitada ee xannibmay markaad u sii socoto isbitaalka. - Haddii qofku hurdo, wareersan yahay, matagayo, ama suuxdin, WALIGAA ha ku shubin dareere afkooda. Waxa laga yaabaa inaad si tartiib ah u salaaxdo qadar yar oo ah glucose jel, malab, ama sokor miis si toos ah gudaha dhabanada hoose iyo ciridka (
buccal mucosa), laakiin samatabbixin qeexan waxay u baahan tahay ** xididka 10% Dextrose (D10W* oo ay maamulaan dhakhaatiirta caafimaadka ama shaqaalaha waaxda gurmadka. - ** Faham sababta Duridda Glucagon aysan uga shaqeynin sumowga Ackee ama Lychee
glycogenolysisiyo synthesize gulukoos cusubgluconeogenesis). Cudurka ackee iyo lychee encephalopathy, glycogen beerka ayaa hore u ahaa100%depleted iyo gluconeogenesis waxaa biochemically xannibay Hypoglycin A iyo MCPG, taasoo la micno ah ** glucagon gabi ahaanba waxtar lahayn ***. Kaliya maamulka tooska ah ee exogenous dextrose (glucose) ayaa badbaadiya maskaxda. - Keydi wixii soo haray cadeecada ceeriin ama karsan, biyaha karinta, ama miraha lichee. Ha u ogolaan qof kale oo guriga ka mid ah inuu wax ka cuno dheri isku mid ah, gaar ahaan biyaha karinta.
pot water) ama maraqa ay ku jiraan ackee arils la kariyey, sida Hypoglycin A biyo-milmi leh ayaa si toos ah ugu dareeraya dareeraha.
Waa maxay sababta loo karkariyo maraq ama biyo bariis ah oo aan bislaan waxay sun u siisaa dheriga oo dhan
Qulqulka ackee bislaaday ayaa si dabiici ah u kala dillaaca geedka ("yawns") qorraxda, oggolow 90% to 99% ee Hypoglycin A ee ku jira arils-jidhka huruudda ah si loogu beddelo abuurka madow ee adag ka hor goosashada. Haddii basal cagaar ah ama guduudan oo aan la furin goor hore la soo qaado oo lagu qasbo in la furo (or cracks open after falling on the ground), Jaallaha arils wali waxa ku jira 1,000 milligrams per kilogram (100 times normal levelsHypoglycin A. Sababtoo ah Hypoglycin A waa kuleyl-degan oo aad u-biyo-milmi kara, karkarinta ackee aan bislaan *ma baabi'inayso sunta; beddelkeeda, waxay u gudbisaa xaddi halis ah oo Hypoglycin A ah biyaha karinta. Xataa marka la karinayo ackee si buuxda u bislaaday, biyaha karkaraya waa in ** had iyo jeer lagu shubaa biya-mareenka oo la tuuraa ***, waligaa looma isticmaalin in lagu kariyo bariiska, bur burka, ama maraqa kalluunka cusbada leh.
Geedka go'aanka: Kala soocida Ackee iyo Lychee hypoglycemic encephalopathy¶
flowchart TD
A["Cake aan bislaan oo laga shakisan yahay ama cunista lychee ee sooman"] --> B{"Qiimee Xaaladda Caafimaad & Miyirka"}
B -->|"Asymptomatic (cunista dhawayd, si buuxda u soo jeedo)"| C["Si degdeg ah cunto karbohaydrayt sare leh + Cabbitaanka gulukooska afka si aad u ilaaliso Glycogen-ka Hepatic"]
C --> D["Geli Cisbitaalka 24-48 Saac oo Joogta ah 10% Faleebo Dextrose ah & Saacad kasta hubinta Gulukooska Faraha"]
B -->|"Matag kedis ah, dhidid, caajisnimo, suuxdin, ama miyir-beel"| E["Hubi Stat Point-of-Care Gulukoos Dhiiga (badanaa <20-30 mg/dL / 1.1-1.7 mmol/L) + Dhiigga Gaaska/Lactate"]
E --> F["Isla markiiba IV Bolus: 10% Dextrose (D10W) 2 ilaa 5 mL/kg IV riixida (dadka waaweyn: 50 mL oo D50W ama 250 ml D10W)"]
F --> G["Ku Bilow Joogto IV 10% Dextrose (D10W gudaha 0.9% NaCl) heerka faleebo Gulukoos (GIR) ee 6 ilaa 8 mg/kg/daqiiqo"]
G --> H{"Calaamadaha bararka maskaxda ee maskaxda?"}
H -->|"Haa"| I["IV Lorazepam/Midazolam + Sare 30 darajo + 3% Milixda Dhiig-karka ah (iska ilaali dareeraha Hypotonic)"]
H -->|"Maya"| J["Ilaali gulukooska dhiigga 100-150 mg/dL (5.5-8.3 mmol/L); Ku dar IV/Oral L-Carnitine + Glycine + Riboflavin (Vitamin B2)"]
I --> J
Pathophysiology: Sida Hypoglycin A iyo MCPG u xidhaan dheef-shiid kiimikaadka tamarta aadanaha¶
Si aad u fahamto sababta ilmaha caafimaadka qaba uu sariirta u seexan karo isagoo dhoola cadeynaya ka dib cunista lychees ama ackee oo uu ka soo tooso 5:00 AM miyir-beelka dilaaga ah ee dilaaga ah, takhaatiirtu waa inay baaraan xannibaadda saxda ah ee mitochondrial enzyme ee ay sababtay ** Hypoglycin A *** iyo ** MCPG** (badanaa loo yaqaan ** nooca glutaric acidemia nooca II ** ama ** la helay deficiency acyl-CoA dehydrogenase badan.MADD]**).
1. U beddelashada borotoksinnada oo loo beddelo thioesters-ka dilaaga ah ee CoA¶
Midna Hypoglycin A (L-alpha-amino-beta-[methylenecyclopropyl]propionic acidama MCPG (alpha-[methylenecyclopropyl]glycine) waxay si toos ah ugu suntan qaabka amino acid-ka ceeriin. Marka laga soo nuugo mindhicirka yar, waxay galaan dariiqa mitochondrial laamood ee amino acid catabolic:
* Cytosolic iyo mitochondrial ** transaminases ** waxay u beddelaan Hypoglycin A ** methylenecyclopropylpyruvic acid (MCPP, iyo ** silsilad laamood alfa-keto acid dehydrogenase oksijiinta decarboxylate waxay gelisaa sunta dilaaga ah ee firfircoon: ** methylenecyclopropylacetyl-CoA (MCPA-CoA)**.
* Sidoo kale, MCPG (ku badan yihiin iniinaha lychee ee aan bislaan/semi-bislaan iyo arils, iyo sidoo kale iniinaha ackee) ayaa la dheefshiido galay 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. Waa maxay sababta aysiidhka dufanka leh ee beta-oxidation iyo gluconeogenesis labaduba ay u fashilmaan¶
- ** Joojinta is-dilitaanka Flavin
FADAcyl-CoA Dehydrogenases:** MCPA-CoA iyo MCPF-CoA waxay u dhaqmaan sidii habab aan laga noqon karin oo ku salaysan ("is-dil") oo xannibaya flavin adenine dinucleotide.FAD) kooxda prosthetic on mitochondrial ** silsilad gaaban acyl-CoA dehydrogenase (SCAD, ** silsilad dhexdhexaad ah acyl-CoA dehydrogenase (MCAD, ** isovaleryl-CoA dehydrogenase (IVD, iyo glutaryl-CoA dehydrogenaseGCDH). Sababtoo ah jidhku ma sii dayn karo acids dufanka leh inta lagu jiro soonka, unugyadu waxay ku qasban yihiin inay gubo gulukooska si xad dhaaf ah halka ** isku-darka jirka ketone (acetoacetateiyobeta-hydroxybutyrate) gabi ahaanba waa xannibay. - ** Xiritaanka Gluconeogenesis-Cagaarshow:** Marka glycogen beerku daalo
4 to 10 hoursCunista ugu dambeysa ka dib, aad u dhaqso badan carruurta yaryar ee leh miisaanka miisaanka maskaxda iyo beerka ee sarreeya ama kaydka nafaqeynta ee hoos u dhacaya), beerku wuxuu isku dayaa inuu soo saaro gulukoos cusub oo laga helo lactate, alanine, iyo glycerol iyada oo loo marayo gluconeogenesis. Si kastaba ha noqotee, heerka ugu horreeya ee xaddidaya enzyme gluconeogenesis, ** pyruvate carboxylase , waxay u baahan tahay ** acetyl-CoA ** sida firfircoonida allosteric ee qasabka ah iyo ** ATP + NADH ** (oo ay soo saartay fatty acid beta-oxidation) si ay u shaqeyso. Intaa waxaa dheer, acyl-CoA esters aan dheef-shiid kiimikaad lahayn iyoMCPA-CoAsequester dhammaan mitochondrial bilaash ah ** Coenzyme A (CoA-SH) iyo L-carnitine. La'aanteedATP,NADH,acetyl-CoA, ama bilaashCoA-SH, Gluconeogenesis cagaarshow ayaa si buuxda u joogsanaysa. - Ururka Neurotoxic Branched iyo Silsilad-gaaban Acids Organic: xannibay beta-oxidation iyo catabolism amino acid waxay sababtaa dhismo weyn oo ah asiidh dufan ah oo gaaban iyo dhexdhexaad ah (**butyric, isobutyric, isovaleric, 2-methylbutyric, glutaric, adipic, suberic acid). Asiidhyadan organic-ga ah waxay horjoogsadaan matoorada hab-dhiska dhexe ee maskaxda ee difaaca, waxay kiciyaan ** aagga kiciya chemoreceptor ** ee jirridda maskaxda (sababta matag degdeg ah), mitochondria neuronal-ka oo aan lammaane lahayn, waxayna soo saaraan cytotoxic ** bararka maskaxda ** ka madax bannaan sonkorta dhiigga ee hoose lafteeda.
Isbarbardhigga Cudurka Matagga ee Jamaican (Ackee) vs. Soonka Lychee Encephalopathy¶
| Muuqaal | Cudurka Matagga ee Jamaica (Unripe Ackee - Blighia sapida) |
Soonka Lychee Encephalopathy (Litchi chinensis / "Chamki" Fever) |
|---|---|---|
| Isha dhirta & Qaybta halista ah | Ackee Arils Aan Furin/Aan Bislayn-1,000 ppm Hypoglycin A vs < 10 ppm marka si dabiici ah loo hamaansado geed dusheeda), ** iniin madow ***, xuub rafe casaan, iyo ** biyo karkaraya *** |
Lichee saxarka iyo iniinaha aan bislaan ama bislaan (highest MCPG and Hypoglycin A in green/unripe fruit fallen under orchard trees) |
| ** Amino Asiidhyada Sunta Asaasiga ah** | Inta badan Hypoglycin A (-> MCPA-CoA), oo leh heerka labaad ** Hypoglycin B *** (gamma-glutamyl conjugate) iyo MCPG |
Inta badan MCPG (-> MCPF-CoA) oo ay weheliso Hypoglycin A (-> MCPA-CoA) |
| ** Profile Dhibbanaha Caadiga ah & Kiciya Cudur-faafidda** | Carruurta iyo dadka waaweyn ee cuna ackee-ga khasabka ah ama maraq/bariis lagu kariyey dheri-biyoodka; daran/dhimasho u ah carruurta iyo dadka waaweyn ee nafaqo-xumada hayso | Carruurta yaryar (ages 1 to 10 yearsgobollada lychee-koritaankaMuzaffarpur in Bihar, India; Bac Giang in Vietnam; Bangladesh) kan calafsada lichees maalinta oo dhan ** hurdo adoon cunin casho fiidkii** |
| Muddada Latency & Waqtiga Bilawga | 2 to 12 hours cuntada kadib (frequently bimodal: abrupt vomiting, brief quiet sleep phase, then sudden morning seizures and coma) |
** Subaxdii hore ee caadiga ahayd3:00 AM to 8:00 AM)** hal mar habeen soonka waxa uu baabi'iyaa kaydka glycogen ee beerka |
| Saxiixa Soo Bandhigida Caafimaadka | Rabshado degdeg ah iyo matag (badanaa shuban ama qandho la'aan), raaxo la'aan epigastric, dhidid badan, daciifnimo xad dhaaf ah, ka dibna suuxdin guud ee tonic-clonic, miyir-beel, iyo neefsashada oo xirma. | Baraaruga lama filaanka ah ee waaberiga oo leh oohin dalool ah, matag, dheygag maran, gariir guud oo tonic-clonic ah, dhejis decerebral, bararka maskaxda, iyo miyir-beelka (often misdiagnosed as viral encephalitis) |
| ** Sawirada Faraha ee Shaybaarka *** | ** gulukoosta dhiiga < 20 to 30 mg/dL (< 1.1 to 1.7 mmol/L, ** maqan ama raad raac ketones kaadida, acidosis dheefshiidka (high anion gapSerum transaminases oo sarreeyaAST/ALT), hyperammonemia, iyo kaadida ** dicarboxylic aciduria + MCPA-glycine / MCPA-carnitine** |
** gulukoosta dhiiga < 20 to 30 mg/dL**, ** ketonuria maqan ***, tirada unugyada caadiga ah ee CSF (oo meesha ka saaraysa meningitis-ka faafa), iyo ogaanshaha kaadida/dhiigga MCPF-glycine, MCPA-glycine, iyo C4-C10 acylcarnitines |
| ** Daaweynta Isbitaalka Cornerstone *** | Isla markiiba 10% dextroseD10Wbolus (2 to 5 mL/kg + joogto ah ** faleebo D10W (GIR 6 to 8 mg/kg/min** + ** L-Carnitine, Glycine, iyo Riboflavin (Vitamin B2)** |
Isla markiiba 10% dextroseD10Wbolus (2 to 5 mL/kg* + joogto ah ** D10W gudaha 0.9% faleebo NaCl** + 3% Hypertonic Saline ee bararka maskaxda |
Tallaabo-tallaabo borotokoolka degdegga ah ee isbitaalka¶
Marka ilmo ama qof weyni la yimaaddo matag, xaalad maskaxeed oo beddelan, ama qalal ka dib cunista ackee ama lychee, ** daqiiqad kasta oo hypoglycemia aan la daweyn iyo aashitada organic waxay kordhisaa halista dhaawaca maskaxda ee hypoxic-ischemic joogto ah ama herniation dilaa ah maskaxda.
1. Isla markiiba xididka 10% Dextrose (D10W) Noolaynta¶
Ha sugin shaybaarada kimisteriga shaybaadhka inay soo noqdaan ka hor intaanad siin dextrose; Samee jeegareynta gulukoosta dhiigga ee farta dhinaceeda sariirta ah (oo sawir tuubada dhiigga ee casaanka/cagaaran/guduudka-korka ah si ay toxicology/acylcarnitine u sifeyso haddii ay suurtagal tahay, iyada oo aan dib loo dhigin daaweynta xitaa 60 seconds).
- Waa maxay sababta 10% dextrose
D10W) ayaa laga door bidaa in ka badan 25% ama 50% Dextrose carruurta: Ku urursan50%dextrose (D50W,2,525 mOsm/L) waa hyperosmolar aad u daran oo sababa sclerosis xidid oo daran, necrosis unug haddii ay xad dhaaf tahay, iyo osmotic degdeg ah oo khatar ah ayaa u wareegaysa xannibaad dhiig-maskaxda carruurta oo nugul. Heerka5%dextrose (D5W) ma keento gulukoos ku filan si loo xakameeyo abaabulka aashitada dufanka leh. ** 10% Dextrose (D10W,100 mg of glucose per mL*** waa halbeegga dahabiga ah ee labadaba boluses bilowga ah iyo faleebooyinka dayactirka joogtada ah. - ** Qiyaasta Bilowga IV ee Bilowga ah:**
- Carruurta: Maamule
2 to 5 milliliters per kilogram(2 to 5 mL/kg, bixinta0.2 to 0.5 grams/kggulukoos) ee10%Dextrose (D10W) xididka laga gooyey2 to 5 minutes. - Dadka: Maamule
250 millilitersee10%Dextrose (D10W) (ama50 millilitersee50%Dextrose [D50W]) xididka. - Qiimeyn Sare oo Joogto ah oo Waajib ah
10%Faleebo dextrose ah (To Shut Down Lipolysis): - Xeerka nafsiga ah ee muhiimka ah: Hal bolus oo dextrose ah ayaa kor u qaada sonkorta dhiiga oo keliya
20 to 30 minutes. Weli waxaa ka sii daran, Hypoglycin A iyo MCPG sumowga, hadafka daawaynta dextrose waa ** kaliya maaha in gulukoosta dhiigga lagu ilaaliyo70 mg/dL, laakiin si loo kiciyo endogenous ku filan insulin soo daynta si ** gabi ahaanba loo xidho lipase hormoonka xasaasiga ah ee unugyada adipose. Ilaa iyo inta uu jidhku isku dayo inuu abaabulo asiidh dufan ah oo bilaash ah oo laga helo dukaamada dufanka, sunta ahMCPA-CoA,MCPF-CoA, iyo asiidh-gaab-gaaban ee asiidhyada organic-ga ah waxay ku sii wadaan inay ku ururaan gudaha mitochondria oo sunta maskaxda. - ** Heerka faleebo (
Glucose Infusion Rate [GIR]):** Isla markiiba billow faleebo xidid oo joogto ah10%Dextrose gudaha0.9%Cusbada caadiga ah (D10NS)** (amaD10 1/2NSoo leh potassium ku habboon marka shaqada kelyaha la xaqiijiyo) ee ** Heerka faleebo gulukoos (GIR) ee6 to 8 milligrams per kilogram per minute(roughly 3.6 to 4.8 mL/kg/houreeD10W**, titrating si loo ilaaliyo gulukoosta serum inta u dhaxaysa100 and 150 mg/dL(5.5 to 8.3 mmol/Lugu yaraan24 to 48 hours.
Waligaa ha u isticmaalin Dheecaannada Hypotonic (Sida Cad D5W ama 0.225% cusbo) Carruurta Encephalopathic
Carruurta qaba ackee ama lychee encephalopathy waxay horeba u dhibtoonayeen cytotoxic iyo vasogenic ** bararka maskaxda *** ay dhaqaajiyaan asiidhka organic neurotoxic iyo tamar la'aanta. Ku shubista xaddi badan oo ah dheecaanka xididada hypotonic (such as plain 5% Dextrose in water [D5W] or 0.18% / 0.225% salineMarka gulukoosta dheefshiidku ay ka baxdo biyo lacag la'aan ah oo si toos ah ugu qulqula unugyada maskaxda ee barara, taasoo soo dedejisa dhimasho * herniation trans-tentorial brainstem . Had iyo jeer u diyaari 10% Faleebo dextrose ah oo ku jirta ** saldhigga sodium isotonic (0.9% sodium chloride) oo si dhow ula soco serum sodium si aad u ilaaliso normonatremia (138 to 145 mEq/L).
2. Isku-xidhaha dheef-shiid kiimikaadka iyo samatabbixinta isku xidhka (L-Carnitine, Glycineiyo Riboflavin)¶
Sababtoo ah MCPA-CoA iyo MCPF-CoA dabin dhammaan mitochondrial Coenzyme A (free)CoA-SH) iyo carnitine iyagoo burburinaya flavin adenine dinucleotide (FADCofactors, borotokoollada sunta kiliinikada ee kiliinikada waxay adeegsadaan saddex isku-dhafan oo dheef-shiid kiimikaad ah si ay u nadiifiyaan dhexdhexaadiyeyaasha sunta ah:
- ** Xididka ama afka L-Carnitine (
Levocarnitine):** - ** Farsamo:** Kaabista L-carnitine waxay xidhaa mitochondrial xanniban
MCPA-CoA,MCPF-CoA, isovaleryl-CoA, iyo silsilad dhexdhexaad ah acyl-CoA molecules iyada oo loo marayo carnitine acyltransferase, iyaga oo u beddelaya biyo-milmi leh ** acylcarnitine esters (MCPA-carnitine*** kuwaas oo laga soo saaro mitochondria oo laga soo saaro kaadida, oo sii daaya khatarCoA-SHloogu talagalay wareegga Krebs. - Qiyaas:
100 milligrams per kilogram per day(100 mg/kg/day)* xididka (ama afka haduu soo jeedo) mid walba waa loo qaybiyaa6 to 8 hours(up to 2 to 3 grams/day). - ** Glycine afka ama Enteral:**
- Mechanism: Enzyme mitochondrial ** glycine N-acyltransferase** isku xidha
MCPA-CoAiyoMCPF-CoAoo leh amino acid glycine si ay u sameeyaan aan sun ahayn MCPA-glycine iyo MCPF-glycine, kuwaas oo si degdeg ah kelyuhu u nadiifiyaan. - Qiyaas:
150 to 250 milligrams per kilogram per day* afka ama tuubada sanka ee sanka ayaa loo qaybiyaa mid kasta6 to 8 hours. - ** Qiyaasta Sare ee Riboflavin
Vitamin B2):** - Mechanism: Sababtoo ah
MCPA-CoAsi bareer ah u hawlgelisaFADkooxda prosthetic ee acyl-CoA dehydrogenases, oo bixiya qiyaas sare Riboflavin (the direct precursor of FAD and FMN, **100 milligramsafka ama tuubada NG 2 ilaa 3 jeer maalin kasta ***) waxay dedejisaa dib u soo kabashada firfircoonidaMCADiyoSCADenzymes.
3. Xakamaynta qalalka, bararka maskaxda, iyo acidosis¶
- ** Xakamaynta suuxdinta:** Ku saxida hypoglycemia
10%Dextrose waxay isla markiiba joojisaa gariir bukaanno badan oo hore u soo bandhigay. Haddii suuxdintu sii socoto in kasta oo gulukoosta dhiigga caadi laga dhigay (due to already-established cerebral edema or organic acid neurotoxicity), waxay maamulaan xididada ** lorazepam (0.1 mg/kgIV)**, midzolam (0.1 to 0.2 mg/kgIV/IM), ama levetiracetam (Keppra,20 to 40 mg/kgIV) (strictly avoid Valproic Acid [Depakote], which itself sequesters carnitine, inhibits mitochondrial beta-oxidation, and worsens hyperammonemia and cerebral edema). - Maareynta bararka maskaxda: Kor u qaad madaxa sariirta ilaa
30 degrees, ilaali normocarbia (PaCO2 35 to 40 mmHg) oo leh xiniinyaha endotracheal hore haddii Glasgow Coma Scale (GCS) waa< 8, oo ku shub 3% Cusbada Dhiig karka3 to 5 mL/kgIV dhaaftay15 to 20 minutes* ama ** Mannitol (0.5 g/kgIV)**. - Sodium Bicarbonate: Si loo helo asiidh dheef-shiid kiimikaad daran (
arterial pH < 7.15), si taxadir leh u bixi sodium bicarbonate iyadoo la ilaalinayo faleebo gulukoos oo joogto ah.
Kahortag: Sida loogu sheego Ackee bislaaday oo badbaado leh Ackee sun ah oo aan bislaan, iyo xeerarka badbaadada leh ee Lychee ee carruurta¶
1. 4ta Xeer ee Dahabka ah ee Diyaarinta Ackee Badbaadada ah¶
+-------------------------------------------------------------------------+
| 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 |
+-------------------------------------------------------------------------+
- Hamaansigu si dabiici ah ha u furmo geedka dushiisa: Weligaa ha goosan hana karsan basal ackee ah oo aan si dabiici ah u kala dillaacin adigoo weli geedka ka laadlaadsan. Waligaa ha ku qasbin in ay furto baal xidhan, hana uga tagin tubooyinka xidhan daaqadaha qoraxda leh ama saqafka iyaga oo sugaya in ay ka furaan geedka.
- ** Ka saar iniin kasta iyo raad kasta oo xuubka casaanka/casaanka ah (
raphe):** Miraha madow ee dhalaalaya iyo xuubka xuubka guduudan ee casaanka leh ee ku dheggan arilka jaalaha ah ee weelka abuurka ayaa weli ah suncontaining high levels of Hypoglycin A and B) xataa midho bislaaday. Ka soo saar dhammaan xuubka casaanka ah oo si fiican ugu maydh jaalaha jaalaha ah biyo qabow oo qabow ka hor intaadan karinin. - Si gooni ah u karkari oo MAR WALBA iska tuur biyaha karinta: Ku karkari geedaha ackee-ga ee la nadiifiyay biyo caadi ah marka hore, ku daadi qaboojiyaha, iyo ** biyaha karkarinta toos ugu shub saxanka**. Weligaa ha ku karsan bariis, yam, muus, bur bur, ama maraq isla biyaha la karkariyo
- ** Badbaadada Ackee la soo dhoofiyo ee qasacadaysan:** Gudaha Maraykanka, Kanada, UK, iyo Midowga Yurub, soo dejinta gasacadaysan ee ganacsiga waxaa si adag ula socda US FDA iyo mas'uuliyiinta badbaadada cuntada si loo xaqiijiyo in uruurinta Hypoglycin A ay ka hooseyso
100 parts per million (ppm). Xataa ackee qasacadaysan oo ganacsi ah, daadi oo raaci brine ka hor inta aanad ku karinin kalluunka milix.
2. 3-dii Xeer ee Caafimaadka Dadweynaha ee Dhammaatay Carruurnimada Cudurka Encephalopathy-ka ee Lychee¶
Ka dib baaritaanno taariikhi ah oo ay si wadajir ah u sameeyeen ** Xarunta Qaranka Hindiya ee Xakamaynta Cudurrada (NCDC)** iyo US CDC ee ku taal Muzaffarpur, Bihar (halkaas oo boqolaal carruur ah ay ku dhintaan Maajo iyo Juun inta lagu jiro goosashada lychee), mas'uuliyiinta caafimaadka dadwaynaha waxay dejiyeen saddex sharci oo qoys oo fudud oo hoos u dhigaya dhimashada in ka badan. 80%:
1. ** Waligaa ha u ogolaan caruurtu inay seexdaan iyagoon cunin cunto fiid ah oo la kariyey (casho):** Cunista cunto-caasheedka caadiga ah ee karbohaydraytku waxay buuxisaa bakhaarada glycogen ee beerka sidaa darteed beerka ilmuhu waligiis ma aha inuu ku tiirsanaado beta-oxidation fatty acid oo xiran.
2. Yaree tirada lychees-ka ee carruurta yaryari ay cunaan, kana fogaadaan midhaha aan bislaan/ dhicin: Ha u oggolaan carruurtu in ay ka daaqdo dhulka geedaha miraha aan bislaan ama bislaaday, sababtoo ah lychees-ka aan bislaan waxa ku jira in ka badan laban-laabka fiirsashada MCPG iyo Hypoglycin A oo laga helo midho guduudan oo si buuxda u bislaaday.
3. Si degdeg ah sokor afka ka bixi una degdeg rug caafimaad leh 10% Dextrose calamadaha subaxda hore: Ilmo kasta oo ku sugan gobolka lychee ama ackee ee ka kaca matag, caajisnimo, ama gariir waa in la helaa 10% Dextrose gudaha saacada ugu horeysa (administering D10W within 4 hours of symptom onset reduces fatality from > 44% to < 5%).
Degdegta gobolka iyo borotokoolka xakamaynta sunta¶
- Jamaica iyo Kariibiyaanka: Wac 119 (Jamaica Ambalaaska/Booliska) ama 911/999 (guud ahaan dhulalka Kariibiyaanka) isla markiiba ula degdeg qaybta degdega ah ee cisbitaalka kuugu dhow ee xididada
10%Dextrose Wasaaradda Caafimaadka iyo Fayo-qabka ee Jamaica waxay ilaalisaa si ba'an ** Sumowga Ackee ( Jirrada Matagga Jamaica)** borotokoolka dib-u-nooleynta ee dhammaan isbitaallada kaniisadda iyo xarumaha caafimaadka. - Mareykanka iyo Kanada: Wac 911 isla markiiba oo wac 1-800-222-1222 (Caawinta Sunta Mareykanka) ama 1-844-SUB-X
1-844-764-7669Kanada). Ackee cusub ayaa ka baxa Koonfurta Florida (where trees are occasionally harvested by residents unfamiliar with the "natural yawn" rule), iyo Ackee la qaboojiyey/gasacadaysan ayaa lagu iibiyaa suuqyada Kariibiyaanka ee Waqooyiga Ameerika. - Hindiya, Vietnam, Bangladesh, iyo Koonfur-bari Aasiya:** Wac 108 ama 112 (Hindiya), 115 (Vietnam), ama 999 (Bangladesh/Malaysia). Inta lagu jiro May-ilaa-July lychee (
litchi, longan, iyo rambutan xilliga goosashada, xarumaha caafimaadka aasaasiga ah ee Bihar (Hindiya) iyo waqooyiga Vietnam oo kaydiya glucometer-yada sariiraha iyo10%Bacaha dextrose si gaar ah loogu talagalay samatabbixin degdeg ah hypoglycemic encephalopathy carruurta carruurta. - United Kingdom, Midowga Yurub, iyo Galbeedka Afrika: Wac 999 (UK), 112 (EU), ama 112/199 (Nigeria, Ghana, Côte d'Ivoire, Burkina Faso, halkaas oo Blighia sapida ay aad u korto oo ay keento sumowga carruurta xilliyeed ee loo yaqaan
"ackee poisoning"ama"finsan"sunta). Dhakhaatiirta UK/EU waa in ay la tashadaan NPIS/TOXBASE ama xarumaha sunta qaranka si loo xaqiijiyo ogaanshaha cudurka acylcarnitine/organic acid iyoL-carnitineqaadashada.
Su'aalaha soo noqnoqda¶
Waa maxay sababta carruurta ay ku sumoobaan lychees ama ackee aan bislaan badanaa ay u bukoodaan aroortii hore?¶
Hypoglycin A iyo MCPG waxay xannibaan enzymes mitochondrial ee loo baahan yahay si ay u gubaan baruurta jidhka oo ay u soo saaraan gulukoos cusub (gluconeogenesis), laakiin kama joojiyaan beerka inuu sii daayo glycogen hore u kaydsan. Marka ilmuhu uu cuno ackee aan bislaan ama uu cuno lichees oo uu seexdo casho la'aan, glycogen-ka beerka ee kaydsan ayaa ilaalinaya sonkorta dhiigooda caadiga ah 4 ilaa 10 saacadood ee ugu horreeya; inta u dhaxaysa 3:00 subaxnimo iyo 8:00 subaxnimo, glycogen-ka beerku gabi ahaanba wuu baxayaa, taasoo keenta in gulukoosta dhiiga ay shil ka hooseyso 20 mg/dL halka aysiidhka dufanka sunta ahi dhex dhexaadintu ay daadiyaan maskaxda.
Waa maxay sababta cirbada degdega ah ee Glucagon ugu shaqayn wayday Ackee ama Lychee hypoglycemia?¶
Glucagon waxa ay kor u qaadaa sonkorta dhiiga kaliya iyada oo amar ku siinaysa beerka in uu jebiyo glycogen kaydsan oo uu ku sameeyo gulukoos cusub iyada oo loo marayo gluconeogenesis. Sumowga ackee iyo lychee, glycogen beerka bukaanku waa horeba gebi ahaanba madhan oo enzymes-kooda gluconeogenic waxaa biochemically quful MCPA-CoA iyo MCPF-CoA, taasoo la micno ah glucagon saameyn eber ah; Bukaanku waa inuu helaa sonkor afka ah oo toos ah (haddii uu soo jeedo) ama 10% Dextrose xididka ah.
Waa maxay sababta Valproic Acid (Depakote) ay khatar ugu tahay suuxdinta ay keento Ackee ama Lychee?¶
Valproic acid waa asiidh dufan leh oo laamo ah oo lafteedu cunta mitochondrial L-carnitine, waxay joojisaa enzymes beta-oxidation fatty acid, waxayna sare u qaadaa ammonia dhiigga. Siinta valproic acid bukaanka dariiqa beta-oxidation ay mar hore ku sumowday Hypoglycin A ama MCPG waxay si aad ah uga sii daraysaa acidosis-ka dheef-shiid kiimikaadka, hyperammonemia, iyo bararka maskaxda ee dilaaga ah; Dhakhaatiirtu waa inay isticmaalaan benzodiazepines, levetiracetam (Keppra), ama phenobarbital oo ay la socdaan 10% Dextrose.
Sideed ku ogaan kartaa in midho Ackee cusub ay badbaado u tahay in la soo qaado oo la kariyo?¶
Qulqulka ackee-ga cusub ayaa kaliya badbaado leh marka uu isu rogo casaan dhalaalaya iyo jaalle-oranji ah oo uu si dabiici ah u kala qaybsan yahay ("hamaantay") isagoo weli ku dheggan geedka qorraxda, isagoo si buuxda u soo bandhigaya saddexda miraha madow ee dhalaalaya. Weligaa ha karsan baal cagaaran oo aan furnayn, waligaa baal fur mindi, had iyo jeer ka saar abuurka madow iyo dhammaan xuubabka casaan-cas, oo had iyo jeer ku shub biyaha karkaraya qulqulka.
Maxay takhaatiirtu ugu hayaan faleebo 10% Dextrose IV ah oo soconaya 24 ilaa 48 saacadood xitaa ka dib marka sonkorta dhiigu caadi ku soo noqoto?¶
Siinta hal bolus oo gulukoos ah waxay hagaajinaysaa sonkorta dhiiga hoose 20 ilaa 30 daqiiqo oo kaliya. Socodka joogtada ah ee 10% faleebo Dextrose ah (6 ilaa 8 mg/kg/daqiiqo) waxa ay ilaalisaa gulukooska dhiiga wax yar kor u kaca (100 ilaa 150 mg/dL), taas oo kicisa insuliinka bukaanka si ay u xidho burburka dufanka ee unugyada adipose kana joojiya jidhka in uu soo saaro silsilado-gaaban ee neurotoxic acids organic ilaa beerka uu ka soo kabsado.
Fardaha iyo eyda sidoo kale ma ku sumoobeen Hypoglycin A iyo MCPG ee miraha geedka?¶
Haa, fardaha daaqaya daaqa inta lagu jiro xilliga dayrta iyo gu'ga waxay si joogta ah ula kulmaan xaalad degdeg ah oo dilaa ah oo loo yaqaan 'Equine Atypical Myopathy' (Europe/UK) ama daaqa xilliyeedka Myopathy (Waqooyiga Ameerika) ka dib markay cunaan miraha baalka leh ("helikobter samaras") iyo geedo of Sycamore Maple (Acer pseudoplatanus) iyo Box Elder oo isku mid ah qoyska Acer, Acer nepinda. ackee iyo lychee waxayna ka kooban yihiin heerar sare oo Hypoglycin A.
Si aad si wax ku ool ah uga jawaabto xaaladaha degdegga ah ee cudurrada carruurta, dheef-shiid kiimikaadka, iyo sumowga, tixgeli inaad ka qaadato gargaarka degdegga ah ee gacanta iyo koorsada CPR ee Laanqeyrta Cas, St John Ambulance, ama bixiye tababbarka degdegga ah ee deegaanka.
Tilmaamaha la xidhiidha¶
- Sulfonylurea (Glipizide, Glyburide) Qaadashada xad dhaafka ah, Hypoglycemia soo noqnoqda, iyo kala soocida Octreotide
- Xaaladda Epilepticus, Suuxdin Dheeraad ah, iyo Daawooyinka Samatabbixinta
- Suuxdin: Gargaarka Degdegga ah iyo Ilaalinta marin-haweedka
- Sumowga cuntada, Gastroenteritis-ka Ba'an, iyo fuuqbaxa
- Gargaarka Degdegga ah ee Sumowga Guud iyo Sumaynta
Ilaha iyo hanuuninta rasmiga ah¶
- ** U.S. Xarumaha Xakamaynta iyo Kahortagga Cudurrada (CDC) & Xarunta Qaranka ee Xakamaynta Cudurrada (NCDC, India): Shrivastava A, et al.:** *Ururka sunta encephalopathy sunta ba'an ee isticmaalka litchi ee dillaaca Muzaffarpur, India, 2014: daraasad-koontarool kiis * (Lancet Global Health).
- Wasaaradda Caafimaadka iyo Fayo-dhowrka Jamaica & Wakaaladda Caafimaadka Dadweynaha Kariibiyaanka (CARPHA): * Tilmaamaha Maareynta Caafimaadka ee Sumowga Ackee (Jirrada Matagga Jamaica / Hypoglycin A Sakhradda)* (Kingston, Jamaica).
- ** U.S. Maamulka Cuntada iyo Dawooyinka (FDA):** * Digniinta Soo Dejinta 21-11 & Tilmaamaha Siyaasadda U Hogaansanaan: Haynta Gasacadaysan ama Barafaysan oo ay ku jirto Wax ka badan 100 ppm Hypoglycin A* (Mareykanka).
- Ururka Caafimaadka Adduunka (WHO)/Guddiga Khabiirka Wadajirka ah ee FAO ee Cunnada lagu daro: Qiimaynta Badbaadada Wasakhowga Cunnada qaarkood: Hypoglycin A ee ku jirta Miraha Ackee (Blighia sapida) iyo MCPG ee Sapindaceae Miraha (Geneva).
- ** Sherratt HSA / Tanaka K, Isselbacher KJ:** * Hypoglycin, sunta miro ackee aan bislaan: habka biochemical ee ka hortagga beta-oxidation iyo gluconeogenesis*