What killed the life of a 14-year-old teenager? Cases in action

Author: Shao Ming Xiao Yuzhen Hepatobiliary and Gastroenterology Specialist Hospital of Yongji City, Shanxi Province

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Patient Yang, male, 14 years old, Shanxi nationality
.
The patient was treated in our hospital
on April 22, 2009 due to "abdominal distension, intermittent fever, and edema of both lower limbs for 40 days".

Present history: the patient had abdominal distention, intermittent fever, body temperature up to 39°C 40 days ago, infusion therapy was given without a clear local diagnosis, and both lower extremities had subsided, and abdominal distension
was still felt.
On April 9, 2009, he was diagnosed in a hospital, and was diagnosed as: liver cirrhosis, sepsis, hypoproteinemia, blood examination routine: WBC: 15.
53×10 9/L, HGB: 62g/L, PLT: 197.
00×10⁹/L; Liver function TB: 132.
30 μmol/L, ALT: 167.
0U/L, AST: 322.
0 U/L, Alb: 29.
1 g/L
.
Electrolyte: sodium: 127mmol/L; Coagulation series: PT: 20.
3Sec
.
Ultrasound: large abdominal effusion
.
Bone marrow aspiration is performed with unknown results and unclear diagnosis
.
No chest tightness, diarrhea, come to our hospital for treatment, outpatient admission with "cause of ascites to be investigated"
.
The patient has clear consciousness, poor spirit, 1/2 less diet than normal, acceptable sleep, basically normal stool, and no clay-colored feces
.
Yellow urine with normal
amount.

Epidemiological history: denial of
close contact with a person with hepatitis.
No history
of blood transfusion.
Vaccination history is unknown
.

Past medical history: denial of history of hypertension, diabetes, heart disease, history of infectious diseases such as typhoid fever and tuberculosis, history of trauma, history of poisoning, and history of drug allergy
.

Personal history: born in the local area, has not been to the epidemic area, no bad habits
.

Family history: denies a family history
of related diseases.

physical examination

Body temperature: 37.
4°C Pulse: 120 beats/min Breathing: 21 times/min Blood pressure: 100/60mmHg Weight: 38kg
.
Clear mind, normal development, clear consciousness, poor spirit, anemia appearance, moderate nutrition, physical examination cooperation
.
Mild xanthochroma of the sclera, spider angiomata (-), palmar hepatocarp (-).

Superficial lymph nodes throughout the body are not palpable
.
Lung (-), fast and uniform heart rate, no murmur heard in the auscultation area of each valve
.
Varicose veins of the chest wall may be seen
.
Abdominal distension, varicose veins of the abdominal wall, and the direction of blood flow is bottom-up
when examined.
Tenderness and rebound pain (+), Murphy sign (-).

The liver is palpable 5 cm under the right rib and 4 cm under the xiphoid process, hard, blunt at the edge, tender (-).

The spleen is palpable 5 cm below the ribs, hard, tender (-).

Liquid wave tremor (-).

Abdominal percussion shows mobile dullness (+), hepatic percussion (+), and renal percussion (-).

Auscultated bowel sounds 5 times/minute, and no vibrating sounds and vascular bruits were
heard.
Mild edema of both lower extremities, without varicose veins and pigmentation
.
Both lower extremities have rough skin
.

Ancillary examination

Liver function: TBil 108.
0umol/L, DBil 54.
40umol/L, IBil 53.
60umol/L, ALT 214.
0U/L, AST 417.
0U/L, ALP 129.
0U/L, GGT 143.
0U/L, ChE 2868.
3U/L, TP 56.
4g/L, Alb 29.
4g/L, TBA 97.
0umol/L, BS 4.
26mmol/L
。 Renal function, blood lipids: normal
.
Electrolyte: calcium: 2.
01mmol/L, Yu normal
.
Ceruloplasmin: 151.
8 mg/L
.
Blood copper: 16.
6 umol/L
.
Blood routine: WBC: 11.
3×10 9/L, RBC: 3.
45×10 12/L, Hb: 91g/L, PLT 163×10 9/L.

BG: O type, Rh(D): positive; AFP＞400ng/ml，CEA 21ng/ml
。 Anti-HAV-IgM (-), hepatitis B series: total yin
.
Anti-HCV(-), anti-HDV(-), anti-HEV(-)
.
Coagulation series: PT: 18.
6Sec
.
RF, ASO, ESR: normal
.
Thyroid function: normal
.
Urine 11 items: bilirubin: 2+, protein: 1+, Yu normal
.

ECG: sinus rhythm 79 beats/minute Normal ECG
.

Chest radiograph: slightly larger
heart.

Abdominal ultrasound: 1.
Large liver (maximum oblique diameter 15.
3cm, caudate lobe size 7.
8×3.
9cm), dense light spots of liver parenchyma; 2.
Splenomegaly (intercostal thickness 4.
5cm); 3.
Edema of the gallbladder wall; 4.
A small amount of ascites
.

Initial analysis of diagnosis

There are many clinical causes of jaundice and abnormal liver function, such as: viruses, infections, chemical poisoning, immune dysfunction, nutritional insufficiency, biliary obstruction, blood circulation disorders, tumors, genetic defects, etc
.
According to the analysis of test results: viral hepatitis (A, B, C, D, E), drug-induced hepatitis, fatty liver, toxic hepatitis, liver tumors, etc.
can cause abnormal liver function, which can be basically excluded
.
The patient has jaundice, no skin itching, no clay-colored stool, and no intrahepatic bile duct dilation combined with abdominal ultrasound
.
Let's look for clues, what causes jaundice, enlarged liver, and enlarged spleen, which is worth thinking about clinicians? In clinical practice, when we analyze and consider the diagnosis of diseases, we should first consider common diseases, and then consider rare diseases and rare diseases, and pay attention to "monism" in order to diagnose diseases on a basis
.
Complete a CT examination of the abdomen for unexpected findings
.

Liver CT non-contrast scan + enhanced scan: plain scan: large liver volume, liver surface under-polished, left lobe and caudate lobe significantly enlarged, liver parenchymal density is normal
.
The spleen is full in shape and normal in density
.
The gallbladder is large in size and slightly thicker in the wall, and no abnormal density is seen in it
.
Pancreas bulk density is normal
.
Fluid density opacities
may be seen in the abdominal cavity.
Enhancement: Arterial phase: uneven strengthening of the liver parenchyma, poor portal vein, narrow inferior vena cava behind the liver
.
The spleen did not show abnormal strengthening
.
Portal vein phase: uniform strengthening of the liver parenchyma, poor hepatic vein, open development of portal communicating branches, no abnormality
in the pancreas.
CT diagnosis: 1.
Cirrhosis, splenomegaly, ascites, esophageal and gastric veins, splenic portal vein tortuous expansion, gallbladder volume
.
2
.
Poor development of inferior vena cava and hepatic vein in the liver segment, combined with clinical, further examination of hepatic vein and inferior vena cava angiography can be carried out if necessary.

Initial diagnosis:

1.
Decompensated cirrhosis 2.
Hypoproteinemia 3.
Peritoneal effusion 4.
Esophageal and gastric varices

Treatment: give liver protection, yellowing, anti-infection, correction of hypoproteinemia (albumin, plasma), correction of anemia, diuresis, nutritional support and other symptomatic treatment, continue to improve relevant examination items
.
A liver biopsy was performed, and the family agreed
.

Result return: Autoimmune liver disease antibody combination: negative
.
CMV-IgM antibody, CMV-IgG antibody: negative, CMV-DNA quantification: negative; Epstein-Barr virus shell antigen, IgM antibody: negative; Epstein-Barr virus DNA quantification: normal
.
Autoimmune liver disease, Epstein-Barr virus infection, and cytomegalovirus infection can be ruled out
in combination with test results.

Liver aspiration is liver tissue biopsy, which has reliable practical value
for the diagnosis of difficult liver diseases.
Liver puncture has become one of the
simple, safe and effective diagnostic methods for liver disease.
The role of liver puncture examination: 1.
It is conducive to the differential diagnosis
of a variety of liver diseases.
2.
Understand the extent and activity of liver lesions
.
3.
Provide etiological diagnosis
of various types of viral hepatitis.
4.
Detection of early, quiescent or compensated cirrhosis
.
5
.
It is conducive to the selection of drugs and the judgment of the efficacy of drugs.
6.
Identify the nature and causes
of jaundice.
7.
As an evaluation index
for chronic hepatitis and prognosis.
8.
Diagnostic treatment
can be performed.

On 23 April 2009, liver tissue biopsy was performed on the patient under ultrasound guidance
.
One liver tissue specimen was taken, fixed with 10% formalin solution, and sent to the Department of Pathology of Beijing China-Japan Friendship Hospital for examination
.
The results of liver puncture are as follows:

Microscopic examination: the liver penetrating tissue was 1.
5 cm long, the liver parenchyma was diffusely destroyed, except for a few hepatocyte nodules scattered around the duct area, the remaining hepatic sinuses have collapsed fibrosis, and most dilated blood vessels (immunohistochemistry: CD34+, see right figure) can be seen in the fibrotic tissue in the center of the lobule (immunohistochemistry: CD34+, see the figure on the right), surrounded by a large number of fine bile duct hyperplasia (indicating that stem cells around the ductal area are actively proliferated
after the necrosis of large liver cells.
Immunohistochemistry: CK7+, see left figure), with scattered infiltration in mononuclear cells
.

Pathological diagnosis: (liver puncture) obstruction of hepatic venous return, early cirrhosis, combined with clinical considerations Budd-chiari syndrome
.

Gastroscopic results on May 10, 2009: esophageal and gastric varices
.

Cirrhosis in this patient is due to
Boo-Chia syndrome.

Final diagnosis

1.
Budd-Chia syndrome 2.
Hypoproteinemia 3.
Peritoneal effusion 4.
Esophageal and gastric varices

Follow-up: The patient was discharged for financial reasons and was recommended to be transferred to a higher-level hospital for further diagnosis and treatment
.
After discharge, the patient developed fever at home and died
after 1 month.

Budd-Chiari syndrome (BCS), also known as hepatic vein obstruction syndrome, refers to a group of diseases characterized by partial or complete obstruction of the hepatic vein or inferior vena cava due to various reasons, obstructing the return of hepatic venous and causing portal hypertension and/or inferior vena cava hypertension.

The most common are inferior vena cava septum above the hepatic venous opening and intrahepatic thrombosis
.
At present, the more unified classification method is: A.
localized inferior vena cava obstruction
.
B.
Stenosis or obstruction
of the long segment of the inferior vena cava.
C.
Hepatic vein obstruction
.
Hepatic vein obstruction or inferior vena cava obstruction is mostly due to the following reasons:(1) hepatic vein thrombosis caused by blood hypercoagulable states (such as: oral contraceptives, polycythemia); (2) The veins are externally compressed by the tumor; (3) Cancer invades the hepatic vein (such as liver cancer) or inferior vena cava (such as kidney cancer, adrenal cancer); (4) Congenital dysplasia of inferior vena cava (diaphragm formation, stenosis, atresia).

BCS is a rare and difficult disease, because it has no obvious specific symptoms, it is often misdiagnosed and mistreated, because its clinical symptoms and its prognosis resemble cirrhosis after hepatitis, so some people call the disease a "sister" disease
of hepatitis.
The misdiagnosis and mistreatment rate of this disease is quite high, according to statistics, the misdiagnosis rate of this disease is as high as 83.
6%.

The onset of this disease is mostly young and middle-aged, mostly between 20~45 years old, more men than women, and most of them have slow
onset.
Imaging of BCS is the most important diagnostic method
.
Non-invasive tests are mainly ultrasound, CT and MRI
.
Ultrasound of the liver is a non-invasive and earliest and fastest way to detect the disease, so it is called "sentinel examination"
.
Hepatic caudate lobe hypertrophy has a suggestive effect
on this disease.
Abdominal ultrasound can make a preliminary and correct diagnosis in most cases, and its compliance rate can reach more than
95%.
Inferior vena cava angiography is called the "gold standard"
because it can not only confirm the diagnosis, distinguish types, and provide a good basis for designing treatment options.
After the disease, it can be 2-fold
higher than normal due to increased pressure in both the liver and inferior vena cava.
Therefore, the key points of diagnosis are: "one black" - skin pigmentation of the lower extremities, "two large" - bloody enlargement of the liver and spleen, "three varicose veins" - chest and abdominal wall veins, spermatic veins, and saphenous veins
.
"Two more" - young and middle-aged people have more diseases, and men have more
cases.

BCS can be clinically divided into the following types: 1.
Asymptomatic type: although there is hepatic vein thrombosis, there is no obvious circulatory disorder, only incidentally found
during hepatic venography and B-ultrasound examination.
2.
Acute type: acute onset, severe epigastric pain, nausea, vomiting, ascites, jaundice and hepatomegaly, death can occur in the short term
.
3.
Chronic type: slow onset and development, gradually abdominal distension, liver discomfort, dull pain and hepatomegaly, resulting in liver cirrhosis after many years, splenomegaly, ascites, gastrointestinal bleeding, etc
.

This patient was analyzed as type 3, that is, chronic type, because abdominal CT and liver puncture results showed cirrhosis, indicating that the course of the disease has been very long, if the course of the disease is short, cirrhosis
will not form.

This patient reminds us that clinicians should have knowledge of BCS and not misdiagnose.