This article is specialized and is intended for medical professionals only
< h1 toutiao-origin="h2" > get the disease, these foods and drugs must be taken care of</h1>
That day, at work at the emergency room, suddenly came a child (boy, local), pale and looking listless.
There were many children queuing for treatment, and when registering, the nurse found that the children's mental state was poor, and brought them in to ask me if I could see it in advance. Combined with the parents' description, I understood the basic situation of the child:
The child, male, 4 years old and 7 months, this morning appeared skin yellow stain, side by side tea urine 1 time, the amount is large, no frequency of urination, urgency, no fever, no special treatment and medical treatment, symptoms did not alleviate, accompanied by pallor, dizziness, tinnitus, symptoms are progressively aggravated, so I took the child to our hospital for treatment.
Physical examination: P 145 times / min, R 52 times / min, BP 112 / 61 mmHg, mental tiredness. The color of the whole body skin and mucous membranes is mildly yellow, obviously pale, the sclera is yellow, the lips are pale, there are no obvious abnormalities in the cardiopulmonary examination, the nail bed is pale, the extremities are warm, and the CRT is 1 second.
Seeing this, many peers will consider diagnosis: 1. Acute hemolytic anemia; 2. Hemolytic tracing.
Pursuing the medical history, the family complained that the child had eaten several broad beans 3 days ago, the amount was not much, the specifics are unknown; and, 3 days ago, there was runny nose, sporadic cough, accompanied by fever, in a private clinic, amoxicillin clavulanate potassium granules and other "powder" (the specific composition is unknown). Considering that the child is seriously ill and hemolysis may still be underway, the child is received in the intensive care unit for further treatment.
pathogen? Glucose-6-phosphate dehydrogenase (G-6-PD) deficiency?
What is the basis for incomplete inspections?
▌ First: the child has acute hemolytic manifestations, sudden mental tiredness, pale face and tea-colored urine, the possible trigger before the onset of the disease is to eat broad beans, on the other hand, the child takes drugs due to illness, in addition to the clear amoxicillin clavulanate potassium granules, there is also a pack of powder, the composition of which is unknown.
Children with glucose-6-phosphate dehydrogenase deficiency, due to the defect of glucose-6-phosphate dehydrogenase in red blood cells, reduce the production of NADPH that maintains the stability of the red blood cell membrane, so that oxidative damage cannot be resisted, and eventually lead to the destruction of red blood cells and hemolysis. The child developed an acute manifestation of haemolysis after consumption of fava beans/suspicious drugs.
Therefore, we boldly speculate whether the powder contains antipyretic drugs, such as acetaminophen, but the family and doctors do not know that the child has G-6-PD deficiency.
Drugs can also cause hemolysis in children with G-6-PD deficiency, and common drugs are as follows:
1. Antipyretic analgesics: acetaminophen, aspirin, acetylphenylhydrazine, phenacetin, antipyridine.
2. Antimalarial drugs: primaquinine, paramatoquinine, adipine, quinine.
3. Sulfonamides: aminobenzenesulfame, N-acetylsulfonamide, sulfonamide acetyl, sulfamethoxazine, sulfasalazine, sulfa isoxazole, sulfapyridine.
4. Nitrofurans: furantandiine, furazolidone, furacillin.
5. Sulfone drugs.
6. Other drugs such as: dimercaptopropanol, methylene blue, water-soluble vitamin K (vitamin K3, K4), chloramphenicol, phenylhydrazine, carboxybenzene, sulfonamide (probenecid), quinidine, chloroquine, naphthalic acid, toluenesulfonurea, vitamin C (high dose) and some traditional Chinese medicines such as huanglian and other drugs.
▌ Second: The children are locals, among which the prevalence of people in guangdong, Guangxi, Sichuan, Hunan, Hainan and other southern regions is relatively high.
▌ Third: Children with G-6-PD deficiency have more genetic characteristics than women, because the G-6-PD gene is carried by the X chromosome and is an X-linked genetic disease, so in the same genetic background, boys are more likely to be affected than girls.
After admission, the relevant examinations will be completed:
Blood count: hemoglobin 68.0 g/L, neutrophil ratio 76.50%, erythrocyte count 2.20×1012/L, platelet count 460×109/L, white blood cell count 23.8×109/L; reticulocyte percentage 2.16%;
Urine routine: ketone body 1+, leukocyte lipase 3+, urine protein qualitative 2+, glucose qualitative 1+, urine occult blood 1+; yu did not see obvious abnormalities. No abnormalities were seen in the stool routine.
Biochemistry: aspartate aminotransferase 92U/L, total bilirubin 88.5 μmol/L, direct bilirubin 0.7 μmol/L, indirect bilirubin 77.8 μmol/L, lactate dehydrogenase 1171U/L; Yu no obvious abnormalities.
No significant abnormalities were seen in coagulation function; D-dimer 3.17 μg/ml; glucose-6-phosphate dehydrogenase 227.7U/L; Coombs' test (-); sucrose hemolysis test (+/-); acid hemolysis test (+/-); condensation test (-).
After symptomatic treatment, the child's condition is stable and there is no further hemolysis, and after giving the precautions for the disease, he will be discharged from the hospital.
Experience
In the treatment of this disease, it is first necessary to determine whether the child has hemolysis. If there is no hemolysis, no special treatment is required, just close observation and discontinuation of contact with drugs or foods that can cause hemolysis (i.e., blocking the trigger). If the child has an acute hemolysis, in addition to blocking the trigger, it is also necessary to provide symptomatic supportive treatment, children with severe anemia can be treated with blood transfusion, and drugs can be given to reduce hemolysis, such as severe hemolysis in newborn children, bilirubin is significantly elevated, and blood exchange therapy can also be performed to prevent the occurrence of bilirubin encephalopathy.
The prevention of fava bean disease is more important than the treatment after the onset of the disease, so increasing the publicity is conducive to reducing the incidence of children. Now, every newborn born will be screened for neonatal diseases, and G-6-PD deficiency is one of the screenings. If the screening results show that the values are lower than normal, the child is considered to have g-6-PD deficiency and should be informed to avoid drugs that may cause hemolysis each time they visit.
Like the common medications used by children, special attention is required:
Antipyretics require avoidance of acetaminophen, common drugs such as antipyretic suppositories (which may be acetaminophen-alone drugs or acetaminophen-containing combinations), pediatric aminophenol xanamin granules, phenolmemine, or phenolic coffee tablets. In this type of child, antipyretic may be considered with ibuprofen.
In terms of diarrhea and abdominal pain, it is necessary to pay attention to the use of berberine hydrochloride tablets. Berberine hydrochloride tablets can also cause hemolysis in children with G-6-PD.
Proprietary Chinese medicine, it is not recommended that such children use proprietary Chinese medicines containing drugs such as huanglian and honeysuckle, and the instructions for proprietary Chinese medicines do not specify whether children with G-6-PD are banned, so it is generally not recommended to use them, or to use preparations that have been taken by children in the past but are safe.
In daily life, it is necessary to avoid storing mothballs or odor pills, especially in children's clothing.
bibliography:
[1] Lu Xiaomei et al., Research status of glucose-6-phosphate dehydrogenase deficiency[J].International Journal of Pediatrics, 2014, (4) 373-375, 379
[2] Expert consensus on the screening, diagnosis and treatment of newborns with glucose-6-phosphate dehydrogenase deficiency in the Neonatal Screening, Diagnosis and Treatment of Birth Defect Prevention and Control Professional Committee of Chinese Preventive Medicine Association[J].Chinese Journal of Pediatrics,2017,55(6):411-414.
This article was first published: Pediatrics Channel of the Medical Professions
This article is written by Chen Peitian
Editor-in-Charge: CiCi
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This article is original Welcome to forward the circle of friends
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