Imbuto za Ackee (Hypoglycin idahiye Indwara yo kuruka yo muri Jamayike) na Lychee (MCPG) Kwiyiriza ubusa Hypoglycemic Encephalopathie mu bana¶
Imibare yihutirwa itandukanye nigihugu. Uru rupapuro rukoresha 911, umubare kuri Amerika na Kanada. Ibindi bihugu byinshi bikoresha 112, 999, cyangwa undi mubare. Shakisha umubare wigihugu cyawe.
Kuruka gitunguranye mugitondo, ubunebwe bukabije, gufatwa muri rusange, cyangwa koma nyuma yo kurya ** imbuto za ackee zidahiye (Blighia sapida) ** cyangwa kurya ** lychee (Litchi chinensis) ** ku gifu kitarimo ifunguro rya nimugoroba ni ibihe byihutirwa byo guhindagurika biterwa na catastrophique ** non-ketotic hypoglycemia (blood glucose often below 20 mg/dL or 1.1 mmol/L) **. Hamagara nimero yawe yihutirwa (911 muri Amerika na Kanada, 112 mu bihugu by’Uburayi, 999 mu Bwongereza) cyangwa ubaze inama zacu ububiko mpuzamahanga bwihutirwa ako kanya, hanyuma ubwire abohereje ko umurwayi akeneye kwisuzumisha byihutirwa glucose na ** imitsi 10% Dextrose **. Mugihe utegereje inkeragutabara, koresha guhungabana ukoresheje ibyacu imiterere epilepticus protokol igihe kirekire, gutandukanya ikibazo cya metabolike n'imiti iterwa n'imiti sulfonylurea hypoglycemic irenze urugero, hanyuma ukoreshe rusange uburozi bukabije ubufasha bwambere.
Byombi ** ackee ** (imbuto zigihugu cya Jamayike, zikura cyane muri Karayibe, Afrika yuburengerazuba, Floride, na Amerika yo Hagati) na ** lychee ** (hamwe na longan na rambutan, mubyara mumuryango wibimera ** Sapindaceae **) irimo protokine ya cyclopropyl amino acide: ** Hypoglycin A (L-methylenecyclopropylalanine) ** na homologue yo hepfo ** alpha- (methylenecyclopropyl) glycine (MCPG) **. Iyo umwana cyangwa umuntu mukuru anyoye arile idahiye, ifunguye-ifungura ackee aril (cyangwa anywa amazi yatekeshejwe ackee), cyangwa mugihe umwana udafite imirire mibi arya lisiti nyinshi zidahiye / zumye-ku manywa akajya kuryama ** atarya ifunguro rya nimugoroba **, ubwo burozi buraceceka 4 to 12 hours mugihe umubiri uracyafite umwijima glycogene. Mu gitondo cya kare (typically 3:00 AM to 8:00 AM), iyo ijoro ryose umwijima glycogene urangiye, umubiri ugerageza kwimukira kuri ** fatty acide beta-okiside ** na ** gluconeogenesis ** kugirango ubwonko butange glucose. Kuberako ** MCPA-CoA ** (kuva Hypoglycin A) na ** MCPF-CoA ** (kuva MCPG) guhagarika bidasubirwaho mitochondrial ** urunigi ruciriritse acyl-CoA dehydrogenase (MCAD. 12 to 48 hours keretse ako kanya ** 10% Dextrose (D10W) bolus hamwe no gukomeza umuvuduko mwinshi glucose infusion ** itangwa.
+-------------------------------------------------------------------------+
| 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 |
+-------------------------------------------------------------------------+
Kora ubu¶
- ** Hamagara ubutabazi bwihuse hanyuma usabe ishami ryinkeragutabara rifite Dextrose yimitsi. Bwira abatumwe ko umurwayi yariye imbuto za ackee cyangwa lychee kandi ko arwaye ** acute toxic hypoglycemia na metabolic encephalopathie **.
- ** Niba umuntu akangutse rwose kandi akaba ashobora kumira neza, tanga isukari yo mu kanwa yihuta. ** Dissolve
2 to 3 tablespoons(30 to 45 grams) by'isukari yo ku meza, ubuki, umutobe w'ibigori, cyangwa ifu ya glucose mu kirahure cy'amazi cyangwa umutobe w'imbuto hanyuma usabe umuntu kuyinywa ako kanya. Nubwo umuntu yamenya ko yariye gusa ackee idahiye cyangwa umwana yariye lychees ku gifu cyuzuye kandi akumva ko ari ibisanzwe rwose, ** gutanga ifunguro ryinshi rya karubone-hydrata hamwe n’ibinyobwa birimo isukari ako kanya ** ituma ububiko bwumwijima glycogene bwuzuye kandi bikabuza umubiri gukora inzira ya okiside ya aside irike mugihe utwaye imodoka ujya mubitaro. - ** Niba umuntu asinziriye, arangaye, aruka, cyangwa afite igicuri, NTUKIGERE usuka amazi mumunwa. Urashobora korohereza buhoro buhoro geli ya glucose ya glucose, ubuki, cyangwa isukari yo kumeza ** imbere yimbere mumatama yo hepfo hamwe namenyo (
buccal mucosa), ariko gutabarwa byuzuye bisaba ** imitsi 10% Dextrose (D10W) ** iyobowe n'inkeragutabara cyangwa abakozi b'ishami ryihutirwa. - ** Sobanukirwa n'impamvu gutera inshinge Glucagon bidakora muburozi bwa Ackee cyangwa Lychee.
glycogenolysis) no guhuza glucose nshya (gluconeogenesis). Muri ackee na lychee encephalopathie, umwijima glycogene umaze100%depleted na gluconeogenezi bihagarikwa na biohimiki na Hypoglycin A na MCPG, bivuze ko ** glucagon idakora neza **. Gusa ubuyobozi butaziguye bwa exogenous ** dextrose (glucose) ** ikiza ubwonko. - ** Uzigame ibisigazwa bisigaye cyangwa bitetse bya ackee, amazi yo guteka, cyangwa imbuto za lychee. ** Ntukemere ko hagira undi muntu murugo urya inkono imwe, cyane cyane amazi yo guteka (
pot water) cyangwa gravy yashizwemo ackee arils, nkuko Hypoglycin ikabura amazi-amazi yinjira mu mazi.
Kuki Guteka Ackee idahiye muri Soup cyangwa Amazi yumuceri Uburozi Inkono yose
Inkono yeze ackee igabanije gufungura bisanzwe kubiti ("yawns") ku zuba, kwemerera 90% to 99% ya ** Hypoglycine A ** muri arile yinyama yumuhondo kugirango ihindurwe mu mbuto zirabura zikomeye mbere yo gusarura. Niba icyatsi kidakinguwe cyangwa icyatsi gitukura cyatoranijwe hakiri kare kandi kigakingurwa (or cracks open after falling on the ground), arile yumuhondo iracyafite **1,000 milligrams per kilogram (100 times normal levels) ya Hypoglycine A **. Kuberako Hypoglycin A ari ** ubushyuhe butajegajega kandi bukabura amazi menshi **, guteka ackee idatetse ntabwo * isenya uburozi; ahubwo, ihererekanya urugero rwica Hypoglycine A mumazi yo guteka. Ndetse iyo utetse ackee yeze neza, amazi abira agomba ** guhora asukwa kumazi hanyuma akajugunywa **, ntabwo yigeze akoreshwa muguteka umuceri, amase, cyangwa umunyu mwinshi.
Igiti cyemezo: Ackee na Lychee hypoglycemic encephalopathie triage¶
flowchart TD
A["Ukekwaho Ackee idahiye cyangwa Kwiyiriza ubusa kwa Lychee"] --> B{"Suzuma Imiterere ya Clinical & Umutimanama"}
B -->|"Asimptomatic (Ingestion iheruka, Kanguka byuzuye)"| C["Tanga ako kanya Ifunguro Ryinshi rya Carbohydrate + Ikinyobwa cya Glucose yo mu kanwa kugirango ukomeze Hepatike Glycogene"]
C --> D["Injira mubitaro kumasaha 24-48 Gukomeza 10% Dextrose Infusion & Isaha ya Fingerstick Glucose Kugenzura"]
B -->|"Kuruka gitunguranye, Kubira ibyuya, Ubunebwe, Gufata, cyangwa Koma"| E["Reba Imiterere Ingingo-yo-Kwitaho Amaraso Glucose (Akenshi <20-30 mg / dL / 1.1-1.7 mmol / L) + Gazi Yamaraso / Lactate"]
E --> F["Ako kanya IV Bolus: 10% Dextrose (D10W) 2 kugeza 5 mL / kg IV Gusunika (Abakuze: 50 mL ya D50W cyangwa 250 mL ya D10W)"]
F --> G["Tangira Gukomeza IV 10% Dextrose (D10W muri 0.9% NaCl) kuri Glucose Infusion Rate (GIR) ya 6 kugeza 8 mg / kg / min"]
G --> H{"Ibimenyetso bifatika Epilepticus cyangwa ibimenyetso byubwonko bwubwonko?"}
H -->|"Yego"| I["IV Lorazepam / Midazolam + Uzamure Umutwe 30 Impamyabumenyi + 3% Saline Hypertonic (Irinde Amazi ya Hypotonic)"]
H -->|"Oya"| J["Komeza Glucose Yamaraso 100-150 mg / dL (5.5-8.3 mmol / L); Ongeramo IV / Umunwa L-Carnitine + Glycine + Riboflavin (Vitamine B2)"]
I --> J
Pathophysiology: Uburyo Hypoglycine A na MCPG byahagaritse imbaraga za metabolism zabantu¶
Kumva impamvu umwana muzima ashobora kuryama amwenyura nyuma yo kurya lychees cyangwa ackee hanyuma akanguka 5:00 AM muri koma ihitana abantu benshi, abaganga bagomba gusuzuma neza inzitizi ya mitochondrial enzyme yatewe na ** Hypoglycin A ** na ** MCPG ** (bakunze kwita ** baguze glutaric acideemia yo mu bwoko bwa II ** cyangwa ** babonye acile-CoA dehydrogenase nyinshi [MADD] **).
1. Guhindura protoxine muri thioester yica¶
Ntabwo ** Hypoglycin A ** (L-alpha-amino-beta-[methylenecyclopropyl]propionic acid) cyangwa ** MCPG ** (alpha-[methylenecyclopropyl]glycine) ni uburozi muburyo bwa aside amine mbisi. Iyo zimaze kwinjizwa mu mara mato, zinjira muri mitochondrial ishami ryumunyururu-aminide acide catabolika:
* Cytosolike na mitochondrial ** transaminase ** ihindura Hypoglycine A muri ** methylenecyclopropylpyruvic aside (MCPP) **, na ** ishami ryumunyururu alpha-keto acide dehydrogenase ** okiside decarboxylates iyitera uburozi bwica: ** methylenecyclopropylacetyl-CoA (MCPA-CoA) **.
* Mu buryo nk'ubwo, ** MCPG ** (nyinshi mu mbuto zidahiye / igice cyeze cya lychee na arile, ndetse n'imbuto za ackee) ihindurwamo ** 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. Kuki aside irike beta-okiside na gluconeogenezi byombi binanirwa¶
- ** Kubuza kwiyahura kwa Flavin (
FAD) Acyl-CoA Dehydrogenase: ** ** MCPA-CoA ** na ** MCPF-CoA ** ikora nkuburyo budasubirwaho bushingiye ("kwiyahura") ibuza flavin adenine dinucleotide (FAD) itsinda rya prostate kuri mitochondrial ** umunyururu mugufi acyl-CoA dehydrogenase (SCAD) **, ** urunigi ruciriritse acyl-CoA dehydrogenase (MCAD) **, ** isovaleryl-CoA dehydrogenase (IVD) **, na ** glutaryl-CoA dehydrogenase (GCDH) **. Kubera ko umubiri udashobora kongera okiside ya aside irike mugihe cyo kwiyiriza ubusa, tissue zitegekwa gutwika glucose kumuvuduko mwinshi mugihe ** ketone yumubiri wa ketone (acetoacetatenabeta-hydroxybutyrate) irahagaritswe rwose **. - ** Guhagarika Gluconeogenezi ya Hepatique: ** Umwijima glycogene umaze gushira (
4 to 10 hoursnyuma yibyo kurya byanyuma, byihuse cyane mubana bato bafite igipimo cyinshi cyubwonko-bwumwijima cyangwa ibigega byintungamubiri), umwijima ugerageza guhuza glucose nshya iva muri lakate, alanine, na glycerol ikoresheje ** gluconeogenez **. Nyamara, enzyme yambere igabanya igipimo cya gluconeogenezesi, ** pyruvate carboxylase **, isaba ** acetyl-CoA ** nkumushinga uteganijwe wa allosteric na ** ATP + NADH ** (byakozwe na aside irike beta-okiside) kugirango ikore. Mubyongeyeho, est-metabolized acyl-CoA esters naMCPA-CoAsequester byose byubusa mitochondrial ** Coenzyme A (CoA-SH) ** na ** L-karnitine **. NtaATP,NADH,acetyl-CoA, cyangwa ubuntuCoA-SHhepatike gluconeogenezi irasya kugirango ihagarare burundu. - ** Kwiyongera kwa Neurotoxic Amashami na Acide Organic Acide Organic: ** Guhagarika beta-okiside hamwe na catabolisme ya amino acide itera kwiyongera kwinshi kwa acide fatty acide na acide (** butyric, isobutyric, isovaleric, 2-methylbutyric, glutaric, adipic, suberic, na sebacic **). Izi acide kama ibuza ubwonko kurinda ubwonko bwa sisitemu yo mu bwoko bwa pompe, bikurura ** chemoreceptor trigger zone ** mu bwonko (bitera kuruka gitunguranye), mitochondriya idakira, kandi ikabyara cytotoxic ** cerebral edema ** idashingiye ku isukari nkeya mu maraso ubwayo.
Kugereranya uburwayi bwo kuruka muri Jamayike (Ackee) na Kwiyiriza ubusa Lychee Encephalopathie¶
| Ikiranga | Kurwara Jamayike Kurwara (Unripe Ackee - Blighia sapida) |
Kwiyiriza ubusa Lychee Encephalopathie (Litchi chinensis / "Chamki" Fever) |
|---|---|---|
| ** Inkomoko y'Ibimera & Akaga Igice ** | ** Gufungura / Ackee Ackee arils ** (-1,000 ppm Hypoglycin A vs. < 10 ppm iyo bisanzwe byafunguye ku giti), ** imbuto z'umukara **, ibara ryijimye ryijimye, na ** amazi abira ** |
** Imbuto idahiye cyangwa igice cyeze Lychee pulp n'imbuto ** (highest MCPG and Hypoglycin A in green/unripe fruit fallen under orchard trees) |
| ** Uburozi bwibanze bwa Amino Acide ** | Ahanini ** Hypoglycine A ** (-> MCPA-CoA), hamwe na kabiri ** Hypoglycin B ** (gamma-glutamyl conjugate) na ** MCPG ** |
Ahanini ** MCPG ** (-> MCPF-CoA) kuruhande rwa ** Hypoglycin A ** (-> MCPA-CoA) |
| ** Umwirondoro Wabahohotewe & Epidemiologic Trigger ** | Abana n'abantu bakuru barya ackee ku gahato cyangwa isupu / umuceri utetse muri ackee-amazi; bikabije / byica abana hamwe nabakuze bafite imirire mibi | Abana bato (ages 1 to 10 years) mu turere dukura lychee (Muzaffarpur in Bihar, India; Bac Giang in Vietnam; Bangladesh) ninde urisha lychees umunsi wose na ** akajya kuryama atarya ifunguro rya nimugoroba ** |
| ** Igihe cyihuta & Igihe cyo Gutangira ** | **2 to 12 hours nyuma yo kurya ** (frequently bimodal: abrupt vomiting, brief quiet sleep phase, then sudden morning seizures and coma) |
** Amateka ya kare-kare kare ()3:00 AM to 8:00 AM) ** rimwe nijoro kwiyiriza ubusa bigabanya umwijima glycogene |
| ** Umukono wa Clinical Yerekana ** | Mu buryo butunguranye gusubiramo no kuruka (akenshi nta mpiswi cyangwa umuriro), kutamererwa neza na epigastrica, kubira ibyuya byinshi, intege nke zikabije, hanyuma muri rusange gufatwa na tonic-clonic, koma, no gufatwa nubuhumekero | Kubyuka gitunguranye mugitondo hamwe no gutaka, kuruka, kutareba neza, guhindagurika kwa tonic-clonic, guhungabana kwifata, kurwara ubwonko, na koma (often misdiagnosed as viral encephalitis) |
| ** Urutoki rwa Laboratoire ** | ** Amaraso glucose < 20 to 30 mg/dL (< 1.1 to 1.7 mmol/L) **, ** ketone idahari cyangwa ikurikirana inkari **, aside aside metabolike (high anion gap), hejuru ya serumu transaminase (AST/ALT), hyperammonemia, hamwe ninkari ** dicarboxylic asideuria + MCPA-glycine / MCPA-karnitine ** |
** Amaraso glucose < 20 to 30 mg/dL**. |
| ** Ubuvuzi bwibitaro bya Cornerstone ** | Ako kanya ** 10% Dextrose (D10W) bolus (2 to 5 mL/kg) ** + ikomeza ** D10W gushiramo (GIR 6 to 8 mg/kg/min) ** + ** L-Carnitine, Glycine, na Riboflavin (Vitamine B2) ** |
Ako kanya ** 10% Dextrose (D10W) bolus (2 to 5 mL/kg) ** + ikomeza ** D10W muri 0.9% NaCl infusion ** + ** 3% Hypertonic Saline ** kubwonko bwubwonko |
Intambwe ku yindi ibitaro byihutirwa protocole¶
Iyo umwana cyangwa umuntu mukuru ageze afite kuruka, guhindura imitekerereze, cyangwa gufatwa nyuma yo kurya ackee cyangwa lychee, ** buri munota wa hypoglycemia itavuwe hamwe na acide acide organique byongera ibyago byo kwangirika kwa hypoxic-ischemic ubwonko cyangwa kwica ubwonko bwica. **
1. Ako kanya imitsi 10% Dextrose (D10W) Kuzura¶
Ntutegereze ko laboratoire ya chimie yagaruka mbere yo gutanga dextrose; kora ako kanya uburiri bwurutoki rwamaraso glucose (hanyuma ushushanye umuyoboro wamaraso utukura / icyatsi / umutuku-hejuru wamaraso ya toxicology / acylcarnitine umwirondoro niba bishoboka, udatinze kuvura na 60 seconds).
- ** Kuki 10% Dextrose (
D10W) bikunzwe hejuru ya 25% cyangwa 50% Dextrose mubana: ** Kwibanda50%dextrose (D50W,2,525 mOsm/L) ni hyperosmolar cyane kandi itera sclerose ikabije y'amaraso, tissue necrosis iyo ikabije, kandi ihinduka ryihuse rya osmotic ryambukiranya inzitizi zamaraso yubwonko bwabana bato. Bisanzwe5%dextrose (D5W) ntabwo itanga glucose ihagije kugirango ihagarike ubukangurambaga bwa aside irike. ** 10% Dextrose (D10W,100 mg of glucose per mL) ** nigipimo cya zahabu kubintu byombi byambere no gukomeza kubungabunga. - ** Intangiriro ya IV Bolus Dose: **
- ** Abana: ** Umuyobozi **
2 to 5 milliliters per kilogram(2 to 5 mL/kg, gutanga0.2 to 0.5 grams/kgya glucose) ya10%Dextrose (D10W) ** imitsi irangiye2 to 5 minutes. - ** Abakuze: ** Umuyobozi **
250 millilitersBya10%Dextrose (D10W) ** (cyangwa50 millilitersBya50%Dextrose [D50W]). - ** Gutegekwa Gukomeza Hejuru-Igipimo
10%Dextrose Kwinjiza (To Shut Down Lipolysis): ** - ** Amategeko akomeye ya physiologiya: ** Bolus imwe ya dextrose izamura isukari yamaraso gusa
20 to 30 minutes. Ikibabaje kurushaho, muri Hypoglycin A na MCPG uburozi, intego yo kuvura dextrose ni ** ntabwo ari ukugumisha glucose yamaraso gusa70 mg/dL**, ariko kugirango ushishikarize endogenous ihagije ya ** insuline irekura ** kugeza ** kuzimya burundu lipase yangiza imisemburo mumyanya ya adipose **. Igihe cyose umubiri ugerageza gukangurira aside irike kububiko bwibinure, uburoziMCPA-CoA,MCPF-CoA, hamwe na acide organic organique ihindagurika ikomeza kurundarunda imbere muri mitochondria no kwangiza ubwonko. - ** Igipimo cyo Kwinjiza (
Glucose Infusion Rate [GIR]): ** Ako kanya utangire kwinjiza imitsi ikomeza ya **10%Dextrose in0.9%Saline isanzwe (D10NS) ** (cyangwaD10 1/2NShamwe na potasiyumu ikwiye iyo imikorere yimpyiko imaze kugenzurwa) kuri ** Glucose Infusion Rate (GIR) ya6 to 8 milligrams per kilogram per minute(roughly 3.6 to 4.8 mL/kg/hourByaD10W) **, kwitirirwa kubungabunga serumu glucose hagati ya **100 and 150 mg/dL(5.5 to 8.3 mmol/L) ** byibuze24 to 48 hours.
Ntuzigere ukoresha Amazi ya Hypotonic (Nka Kibaya D5W cyangwa 0.225% Saline) mubana ba Encephalopathic
Abana barwaye ackee cyangwa lychee encephalopathie basanzwe barwaye cytotoxic na vasogenic ** cerebral edema ** itwarwa na acide neurotoxic organic acide no kunanirwa kwingufu. Gutera umubyimba munini wa hypotonic intravenous fluid (such as plain 5% Dextrose in water [D5W] or 0.18% / 0.225% saline) iyo glucose imaze guhindurwa amababi inyuma yamazi yubusa yihuta mu ngirabuzimafatizo zabyimbye, bikagwa byica ** trans-tentorial brainstem herniation **. Buri gihe utegure 10% Dextrose gushiramo muri ** isotonic sodium base (0.9% sodium chloride) ** hanyuma ukurikirane hafi ya sodium ya sodium kugirango ukomeze normonatremia (138 to 145 mEq/L).
2. Metabolic Cofactor hamwe nubutabazi bwa Conjugation (L-Carnitine, Glycine, na Riboflavin)¶
Kuberako MCPA-CoA na MCPF-CoA umutego wose wa mitochondrial Coenzyme A (CoA-SH) na karnitine mugihe usenya flavin adenine dinucleotide (FAD) cofactors, protocole ya toxicology ivura ikoresha ibice bitatu bigamije guhuza metabolike kugirango ikureho abahuza uburozi:
- ** Imitsi yinjira cyangwa umunwa L-Carnitine (
Levocarnitine): ** - ** Mechanism: ** L-karnitine yinyongera ihuza mitochondrial yafashwe
MCPA-CoA,MCPF-CoA.MCPA-carnitine) ** zisohoka muri mitochondriya zigasohoka mu nkari, zikarekura zikomeyeCoA-SHKuri Krebs. - ** Igipimo: ** **
100 milligrams per kilogram per day(100 mg/kg/day) ** imitsi (cyangwa umunwa niba ikangutse) igabanije buri6 to 8 hours(up to 2 to 3 grams/day). - ** Glycine yo mu kanwa cyangwa mu nda: **
- ** Mechanism: ** Enzyme ya mitochondrial ** glycine N-acyltransferase ** conjugates
MCPA-CoAnaMCPF-CoAhamwe na aside amine ** glycine ** kugirango itagira uburozi ** MCPA-glycine ** na ** MCPF-glycine **, isukurwa vuba nimpyiko. - ** Igipimo: **
150 to 250 milligrams per kilogram per daykumunwa cyangwa ukoresheje nasogastric tube yagabanijwe buri6 to 8 hours. - ** Riboflavin Yuzuye (
Vitamin B2): ** - ** Mechanism: ** Kuberako
MCPA-CoAku buryo budasubirwahoFADitsinda rya prostate ya acyl-CoA dehydrogenase, itanga urugero rwinshi ** Riboflavin ** (the direct precursor of FAD and FMN, **100 milligramsmunwa cyangwa ukoresheje NG tube inshuro 2 kugeza kuri 3 kumunsi **) byihutisha kuvugurura ibikorwaMCADnaSCADenzymes.
3. Kugenzura Indwara, Indwara yubwonko, na Acide¶
- ** Igenzura ryo gufatwa: ** Gukosora hypoglycemia hamwe
10%Dextrose ihita ihagarika guhungabana mubarwayi benshi-berekana kare. Niba gufatwa bikomeje nubwo glucose yamaraso isanzwe (due to already-established cerebral edema or organic acid neurotoxicity), gutanga imiyoboro y'amaraso ** lorazepam (0.1 mg/kgIV) **, ** midazolam (0.1 to 0.2 mg/kgIV / IM) **, cyangwa ** 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). - ** Gucunga ubwonko bwubwonko: ** Uzamure umutwe wigitanda kuri
30 degrees, kubungabunga normocarbia (PaCO2 35 to 40 mmHg) hamwe na endotracheal intubation kare niba Glasgow Coma Igipimo (GCS) ni< 8, no gushiramo ** 3% Saline Hypertonic (3 to 5 mL/kgIV birangiye15 to 20 minutes) ** cyangwa ** Mannitol (0.5 g/kgIV) **. - ** Sodium Bicarbonate: ** Kuri acide metabolike ikabije (
arterial pH < 7.15), koresha ubwitonzi bwimitsi ** sodium bicarbonate ** mugihe ukomeje glucose ikomeza.
Kwirinda: Nigute wabwira Ackee itekanye neza Ackee idafite uburozi, hamwe n amategeko ya Lychee atekanye kubana¶
1. Amategeko 4 ya Zahabu yo Gutegura Ackee Yizewe¶
+-------------------------------------------------------------------------+
| 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 |
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| 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 |
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- ** Reka reka pod "yawn" ifungure bisanzwe mubiti: ** Ntuzigere usarura cyangwa ngo uteke pode ya ackee itagabanije kwaguka muburyo busanzwe mugihe ikimanitse kumiti. Ntuzigere uhatira icyuma gifunze, kandi ntuzigere usiga ibishishwa bifunze kumadirishya yizuba cyangwa igisenge utegereje ko bakingura igiti (ibishishwa bitandukanijwe ntibisobanura neza Hypoglycine A muri arils mu mbuto).
- ** Kuraho imbuto zose hamwe nibisobanuro byose byijimye / umutuku (
raphe).containing high levels of Hypoglycin A and B) ndetse no mu mbuto zeze neza. Kuramo ibibabi byose byijimye hanyuma ukarabe neza yumuhondo mumazi akonje mbere yo guteka. - ** Guteka ukundi kandi BURUNDI uta amazi yo guteka: ** Guteka mbere ya arile isukuye mumazi asanzwe, ubanza kuyakuramo muri colander, hanyuma ** usuke amazi abira ahita umanuka **. Ntuzigere uteka umuceri, yam, igitoki, amase, cyangwa isupu mumazi amwe yakoreshejwe guteka ackee.
- ** Umutekano wa Ackee watumijwe mu mahanga: ** Muri Amerika, Kanada, Ubwongereza, n’Ubumwe bw’ibihugu by’Uburayi, ibicuruzwa biva mu mahanga byinjira mu bucuruzi bikurikiranwa cyane na ** US FDA ** hamwe n’ubuyobozi bushinzwe umutekano mu biribwa kugira ngo barebe ko Hypoglycine A yibanda munsi
100 parts per million (ppm). Ndetse hamwe nubucuruzi bwa ackee yubucuruzi, kura hanyuma woge brine mbere yo gutekesha amafi yumunyu.
2. Amategeko 3 yubuzima rusange yarangije ubwana Lychee Encephalopathie¶
Nyuma y’iperereza ryibanze ryakozwe n’ikigo cy’igihugu cy’Ubuhinde gishinzwe kurwanya indwara (NCDC) ** na ** CDC ** yo muri Amerika i Muzaffarpur, muri Bihar (aho abana babarirwa mu magana bapfaga buri Gicurasi na Kamena mu gihe cyo gusarura lychee), abayobozi b’ubuzima rusange bashyizeho amategeko atatu yoroshye y’umuryango agabanya impfu zirenga 80%:
1. ** Ntuzigere ureka abana ngo baryame batiriwe barya ifunguro rya nimugoroba (ifunguro rya nimugoroba): ** Kurya ibiryo bisanzwe bikungahaye kuri karubone-hydrata byuzuza ububiko bwa hepatike glycogene kuburyo umwijima wumwana utagomba na rimwe kwishingikiriza kuri aside irike ya beta-okiside ijoro ryose.
2. ** Gabanya ingano ya lychees abana bato barya, kandi wirinde imbuto zidahiye / zaguye: ** Ntukemere ko abana barisha lychees zidahiye cyangwa igice cyeze cyera hasi mu busitani, kuko lychees idahiye irimo inshuro zirenze ebyiri kwibanda kuri ** MCPG ** na ** Hypoglycin A ** iboneka mu mbuto zitukura zeze neza.
3. ** Tanga isukari yo mu kanwa uhite wihutira kujya ku ivuriro rifite 10% Dextrose mu kimenyetso cya mbere cya mugitondo: ** Umwana wese wo mu karere ka lychee cyangwa ackee akangutse kuruka, ubunebwe, cyangwa kwikuramo agomba kwakira **10% Dextrose ** mu isaha yambere (administering D10W within 4 hours of symptom onset reduces fatality from > 44% to < 5%).
Porotokole yihutirwa yo kurwanya no kurwanya uburozi¶
- ** Jamayike na Karayibe: ** Hamagara ** 119 ** (Ambulance ya Jamaica / Polisi) cyangwa ** 911/999 ** (hirya no hino mu turere twa Karayibe) hanyuma uhite wihutira kujya mu ishami ryihutirwa ry’ibitaro ryegereye kugira ngo winjire.
10%Dextrose. Minisiteri y’ubuzima n’ubuzima bwiza ya Jamayike ** ikomeje guhagarara ** Uburozi bwa Ackee (Indwara yo kuruka muri Jamayike) ** protocole yubuzima ku bitaro byose bya paruwasi n’ibigo nderabuzima. - ** Amerika na Kanada: ** Hamagara ** 911 ** ako kanya hanyuma uhamagare ** 1-800-222-1222 ** (Ubufasha bwuburozi bwa Amerika) cyangwa ** 1-844-POISON-X ** (
1-844-764-7669muri Kanada). Ackee nshya ikura muri Floride yepfo (where trees are occasionally harvested by residents unfamiliar with the "natural yawn" rule), hamwe na ackee ikonje / ikonjeshwa igurishwa mumasoko ya Karayibe muri Amerika ya ruguru. - ** Ubuhinde, Vietnam, Bangladesh, na Aziya y Amajyepfo yAmajyepfo: ** Hamagara ** 108 ** cyangwa ** 112 ** (Ubuhinde), ** 115 ** (Vietnam), cyangwa ** 999 ** (Bangladesh / Maleziya). Mugihe cya Gicurasi-Nyakanga Nyakanga lychee (
litchi) **, ** longan **, na ** rambutan ** igihe cyo gusarura, ibigo nderabuzima byibanze muri Bihar (Ubuhinde) hamwe na Vietnam ya ruguru ububiko bwa glucometero na10%Dextrose imifuka byumwihariko kubana bato hypoglycemic encephalopathie gutabara. - ** Ubwongereza, Umuryango w’ubumwe bw’ibihugu by’Uburayi, na Afurika y’iburengerazuba: ** Hamagara ** 999 ** (UK), ** 112 ** (EU), cyangwa ** 112/199 ** (Nijeriya, Gana, Côte d'Ivoire, Burkina Faso, aho * Blighia sapida * ikura cyane kandi igatera uburozi bw’abana ibihe bizwi ku izina rya
"ackee poisoning"cyangwa"finsan"uburozi). Abaganga b’amavuriro mu Bwongereza / Umuryango w’ubumwe bw’ibihugu by’Uburayi bagomba kugisha inama ** NPIS / TOXBASE ** cyangwa ibigo by’uburozi by’igihugu by’inkari acylcarnitine / kwemeza aside isuzuma kandiL-carnitineikinini.
Ibibazo bikunze kubazwa¶
Kuki abana bafite uburozi bwa lychees cyangwa ackee idahiye bakunze kurwara kare mugitondo?¶
Hypoglycine A na MCPG bibuza imisemburo ya mitochondrial ikenewe mu gutwika amavuta yumubiri no gukora glucose nshya (gluconeogenez), ariko ntibibuza umwijima kurekura glycogene yari imaze kubikwa. Iyo umwana arya ackee idahiye cyangwa akarya lychees akajya kuryama nta kurya, umwijima wabitswe glycogene utuma isukari yamaraso yabo iba isanzwe mumasaha 4 kugeza 10; hagati ya 3h00 AM na 8h00 AM, umwijima glycogene urashira burundu, bigatuma glucose yamaraso igwa munsi ya 20 mg / dL mugihe abahuza aside irike yubumara yuzuye ubwonko.
Kuki inshinge yihutirwa ya Glucagon idakora kuri Ackee cyangwa Lychee hypoglycemia?¶
Glucagon izamura isukari mu maraso gusa itegeka umwijima kumena glycogene yabitswe no guhuza glucose nshya binyuze muri gluconeogenez. Mu burozi bwa ackee na lychee, umwijima w'umwijima glycogene umaze kuba ubusa rwose kandi imisemburo ya gluconeogenic ifunga ibinyabuzima na MCPA-CoA na MCPF-CoA, bivuze ko glucagon igira ingaruka zeru; umurwayi agomba kwakira isukari yo mu kanwa (niba ikangutse) cyangwa imitsi ya 10% Dextrose.
Kuki Acide Valproic (Depakote) iteje akaga kubwo gufatwa biterwa n'uburozi bwa Ackee cyangwa Lychee?¶
Acide ya Valproic ni acide ishami ryumunyu wa aside irike ubwayo ikoresha mitochondrial L-karnitine, ikabuza imisemburo ya aside irike beta-okiside, kandi ikazamura amoniya yamaraso. Guha aside aside ya valproic kumurwayi ufite inzira ya beta-okiside yamaze kwangizwa na Hypoglycin A cyangwa MCPG byangiza cyane aside aside metabolike, hyperammonemia, na edema yica ubwonko; abaganga bagomba gukoresha benzodiazepine, levetiracetam (Keppra), cyangwa phenobarbital hamwe na 10% Dextrose.
Nigute ushobora kumenya niba imbuto nshya ya Ackee ifite umutekano guhitamo no guteka?¶
Ikibabi gishya cya ackee gifite umutekano gusa iyo cyahindutse umutuku n'umuhondo-orange hanyuma kigacamo ibice bisanzwe ("yawned") mugihe ukimanitse ku giti izuba, bikerekana neza imbuto eshatu z'umukara zirabagirana. Ntuzigere uteka icyatsi kidakinguye, ntuzigere uhatira icyuma gifunguye ukoresheje icyuma, burigihe ukureho imbuto yumukara hamwe nibice byose byijimye-umutuku, kandi uhore usuka amazi abira kumugezi.
Kuki abaganga bakomeza gutonyanga 10% ya Dextrose IV ikora amasaha 24 kugeza 48 na nyuma yisukari yamaraso igarutse mubisanzwe?¶
Gutanga bolus imwe ya glucose ikosora isukari nke mumaraso muminota 20 kugeza 30. Gukoresha umuvuduko ukabije wa 10% Dextrose (kuri 6 kugeza 8 mg / kg / kumunota) ituma glucose yamaraso izamuka gato (100 kugeza 150 mg / dL), itera insuline yumurwayi ubwayo guhagarika ibinure byamavuta ya adipose kandi bikabuza umubiri gukora aside irike ya neurotoxic ngufi kugeza igihe imisemburo yumwijima ikize.
Ese amafarasi n'imbwa nabyo byarozwe na Hypoglycin A na MCPG biva mu mbuto z'ibiti?¶
Nibyo, amafarashi arisha mu rwuri mu gihe cyizuba n'itumba bikunze guhura n'ikibazo cyica cyane cyitwa Equine Atypical Myopathie (mu Burayi / Ubwongereza) cyangwa Myopathie Pasture (muri Amerika y'Amajyaruguru) nyuma yo kurya imbuto zifite amababa ("kajugujugu ya kajugujugu") hamwe n'ingemwe za Sycamore Maple (Acer pseudoplatanus) hamwe na Box Elder (Acer negoe). lychee kandi irimo urwego rwo hejuru rwa Hypoglycine A.
Kugira ngo usubize neza ibibazo byihutirwa by’abana, metabolike, n’uburozi, tekereza gufata ubufasha bwambere n’amasomo ya CPR muri Croix-Rouge, Ambulance ya St John, cyangwa utanga amahugurwa yihutirwa.
Kuyobora¶
- Sulfonylurea (Glipizide, Glyburide) Kurenza urugero, Hypoglycemia isubirwamo, hamwe na Octreotide
- Imiterere Epilepticus, Gufata igihe kirekire, n'imiti yo gutabara
- Gufata: Imfashanyo Yambere no Kurinda Umuyaga
- Uburozi bwibiryo, Gastroenteritis ikaze, hamwe no kubura amazi
- Uburozi Rusange Imfashanyo Yambere no Kwanduza
Inkomoko nubuyobozi¶
- ** Amerika. Ibigo bishinzwe kurwanya no gukumira indwara (CDC) & Ikigo cy’igihugu gishinzwe kurwanya indwara (NCDC, Ubuhinde): Shrivastava A, n’abandi:
- ** Minisiteri y’ubuzima n’ubuzima bwiza bwa Jamayike & Ikigo cy’ubuzima rusange cya Karayibe (CARPHA): ** * Amabwiriza yo gucunga amavuriro y’uburozi bwa Ackee (Indwara yo kuruka muri Jamayike / Hypoglycine Ubusinzi) * (Kingston, Jamayike).
- ** Amerika. Ubuyobozi bushinzwe ibiryo nibiyobyabwenge (FDA): ** * Kumenyekanisha Kumenyesha 21-11 & Amabwiriza ya Politiki yo kubahiriza: Gufunga Ackee yafunzwe cyangwa ikonje irimo ibirenze 100 ppm Hypoglycine A * (Amerika).
- ** Ishami ry'Umuryango w'Abibumbye ryita ku Buzima (OMS) / Komite y’impuguke ihuriweho na FAO ku byongeweho ibiryo: ** * Isuzuma ry’umutekano ry’ibintu bimwe na bimwe byangiza ibiryo: Hypoglycine A mu mbuto za Ackee (Blighia sapida) na MCPG mu mbuto za Sapindaceae * (Geneve).
- ** Sherratt HSA / Tanaka K, Isselbacher KJ: ** * Hypoglycin, uburozi bwimbuto za ackee zidahiye: uburyo bwa biohimiki bwo kubuza beta-okiside na gluconeogenezi * (Biochemical Pharmacology / New England Journal of Medicine).