h*****g
发帖数: 1523 | 1 一个朋友的孩子（女孩）生下来之后身体就比较弱，当时上海儿童医院诊断是巨细胞感
染造成的血小板
下降。
中西医治疗了一阵子后有所稳定，但是现在又开始严重，主要症状是脾肿大（已经进入
盆腔）. 其他伴
随的症状还有：
经常鼻子出血（特别是血小板水平低的时候）；
脸上有小紫点（应该是血斑）；
饭量不好（现在三岁半，身高只有92cm）；
国内南京和连云港，还有苏州的医院的医生倾向于认为是母亲怀孕时通过脐带造成的病
毒感染，导致现
在的脾肿大。有的医生建议进行脾切除手术，但是这么小的小孩可能扛不住这样的大手
术。
我觉得国内的医生没有把病因搞清楚，大家对这种病有什么看法？
或者有没有别的建议，比如只把脾部分切除等等。孩子家里足够的钱到国外治病，比如
到香港地区，或
者美国，日本这些国家做西医手术治疗。
先谢谢大家了！孩子的妈妈哭着打电话给我让我问问在美国学医的朋友，所以先到未名
上来请教大家。
希望大家能提供看法或者信息。 | c****i
发帖数: 40 | 2 不忍心看着你焦急的心情，其实这里很多人都愿意帮忙，但是由于种种原因，无法给你
回复。
就以一般性的回复几句，首先你给的非常有限的病史体检化验结果等等，所以基本上没
人能在这里给你一个诊断。我search了一下，下面是链接以及原文，希望对你能有帮助
。另外，也不要小看国内的医生，有很多是不错的。
点击链接可以发现更多的内容。
http://emedicine.medscape.com/article/958739-overview
History
Despite the extensive differential diagnoses of splenomegaly, careful
history taking and physical examination, along with a CBC count and liver
function tests, often help in narrowing the list of possible causes. The
history should include attention to the following important areas, as listed
below.15
* Chief symptoms
o Acute or chronic nature
o Painful or asymptomatic manifestation
o Ingestion of hepatotoxic agents resulting in hepatitis or portal
hypertension
o Abdominal trauma that may cause splenic hematoma
o Acute illness such as hepatitis, mononucleosis, or malaria
o Diarrhea (eg, salmonellosis, inflammatory bowel disease)
o Bone pain, fever, malaise, lethargy, or bruising (eg, associated
with leukemia)
o Weight loss, fevers, night sweats (eg, associated with Hodgkin
disease)
o Jaundice
* Medical history
o Complicated neonatal period (eg, sepsis, hypotension)
o Umbilical catheter thrombosis
o Hyperbilirubinemia, anemia (eg, due to hereditary spherocytosis
or hemolysis)
o Heart disease (eg, congestive heart failure)
o Past surgeries (eg, leading to infection, thrombosis, portal
hypertension)
o Transfusions (eg, resulting in hepatitis)
o Hepatitis
o Abdominal trauma (possibly resulting in splenic pseudocyst)
o Travel (possible presence of malaria, leishmaniasis,
schistosomiasis, or trypanosomiasis)
o Sexual behavior (possible presence of hepatitis, cytomegalovirus
[CMV], or human immunodeficiency virus [HIV])
o Known blood disorder (eg, sickle cell disease, hereditary
spherocytosis)
* Family history
o Anemia, cholecystectomy (eg, due to hemolytic anemia–associated
gallstones)
o Splenectomy (eg, due to hemolytic anemia)
o Mediterranean ethnicity (increased incidence of thalassemia and
glucose-6-phosphate dehydrogenase [G6PD] deficiency)
o African ethnicity (increased incidence of sickle cell anemia,
G6PD deficiency, and hereditary pyropoikilocytosis)
o Ashkenazi Jewish ethnicity (increased incidence of Gaucher
disease and Niemann-Pick disease)
o Northern European ethnicity (increased incidence of pyruvate
kinase deficiency and hereditary spherocytosis)
o Asian ethnicity (increased incidence of G6PD)
o South Asian ethnicity (increased incidence of portal
hypertension secondary to noncirrhotic portal fibrosis)
Physical
The patient should be examined in the supine or right lateral decubitus
position. The spleen is best palpated with the clinician kneeling on the
patient's right side and by palpating the left upper quadrant of the abdomen
with the right hand.
Palpation should start just above the pubis and move toward the left upper
quadrant to find the medial border of the spleen. The examiner usually
palpates the spleen by feeling its inferolateral margins. If the enlarged
tip of the spleen is below the examiner's hands, he or she often misses it.
Likewise, light pressure should be used with small children, because the
spleen can easily be pushed out of the way without the clinician feeling its
edge. At times, the superior medial edge of the spleen is more readily
palpated than the inferior margin.
Percussion over the left lateral areas of the lower ribs may reveal
splenomegaly that is not evident upon palpation.16,17,18 This procedure is
particularly helpful in a crying child in whom splenic palpation is
difficult. It is similarly helpful in children and adolescents who are obese
. A normal-sized liver and spleen may also become palpable if pulmonary
pathology is causing hyperinflation of the lungs (pseudosplenomegaly). The
spleen is occasionally confused with the left lobe of the liver or with a
tumor in the left upper quadrant (eg, Wilms tumor, neuroblastoma). The
characteristic downward movement of the spleen with inspiration and its
relatively flat surface and shape can help in differentiating it from other
masses of the left upper quadrant. Important features on physical
examination are as follows:15
* General findings - Failure to thrive, ill-appearing (eg, in the
presence of malignancy, chronic hemolysis, chronic infection, metabolic
disease, liver disease, or inflammatory disease)
* Dermal findings
o Pallor (eg, due to anemia, which may indicate hemolysis, bone
marrow infiltration, or hypersplenism)
o Petechiae, purpura (eg, due to thrombocytopenia, which may
indicate bone marrow failure, autoimmune disorder, or hypersplenism)
o Jaundice (eg, due to hemolytic anemia or liver disease)
o Itching, pruritus (eg, due to liver dysfunction, Hodgkin
lymphoma)
o Rashes (eg, due to acute and chronic infections, systemic lupus
erythematosus, rheumatoid arthritis, infective endocarditis, histiocytoses,
or hemangiomata)
o Eczematous rash (eg, due to Langerhans cell histiocytosis or
immunodeficiency)
* Head, eyes, ears, nose, and throat findings
o Icterus (eg, due to hemolytic anemia or liver dysfunction)
o Cherry red retinal spots, cloudy corneas (eg, due to lipid
storage diseases)
* Respiratory and cardiovascular findings
o Dyspnea, fatigue (eg, due to anemia or congestive heart failure)
o New murmur (eg, due to infective endocarditis)
* GI findings
o Abdominal tenderness (eg, due to gallstones, hepatitis, trauma,
or acute splenomegaly)
o Distention, prominent abdominal veins, ascites (eg, due to liver
disease)
o Enlarged liver with a firm, knobby texture
* Musculoskeletal findings
o Joint pain (eg, due to systemic lupus erythematosus, rheumatoid
arthritis, or autoimmune inflammatory diseases)
o Poor bone growth (eg, due to storage diseases or osteopetrosis)
o Bone pain (eg, due to leukemia or Gaucher disease)
* Neurologic findings
o Poor vision (eg, due to osteopetrosis)
o Uveitis, iritis (eg, due to sarcoidosis or rheumatoid arthritis)
o Loss of developmental milestones (eg, due to storage diseases,
chronic infection, or immunodeficiency)
Causes
Despite the numerous causes of splenomegaly (see Differentials), the spleen
is rarely the primary site of disease.
* The most common mechanism of splenomegaly in children is hyperplasia
of the MPS, which can be categorized as excessive antigenic stimulation (ie,
infection), disorders of immunoregulation (ie, autoimmune disorders), or
excessive destruction of abnormal blood cells (ie, hemolysis).2,19
o Excessive antigenic stimulation due to infection is the cause of
most cases of splenomegaly in children. Viral infections are the most
frequent culprits, and the associated splenomegaly is usually transient and
only mild to moderate in severity. Although Epstein-Barr virus (EBV) and CMV
are well known causes of splenomegaly, the most typical viral illnesses of
childhood are the most frequent causes.
o Other common infectious etiologies include bacterial, protozoal,
and fungal infections. In endemic areas, malaria and schistosomiasis are
routine causes of splenomegaly. Concomitant generalized lymphadenopathy is
common in many of these infectious etiologies.
o Inflammation due to collagen vascular diseases, such as juvenile
rheumatoid arthritis, and increased destruction of blood cells from
hemolytic anemias are relatively uncommon, but clinically significant,
causes of splenomegaly.
o Splenomegaly can be a presenting sign of neoplasia. One half of
children with acute lymphoblastic leukemia have splenomegaly. Splenomegaly
is also a frequent finding in non-Hodgkin lymphoma, Hodgkin disease, and
acute or chronic myeloblastic leukemia. Metastatic involvement of the spleen
, which is uncommon in children, is most often caused by neuroblastoma.
Histiocytes can infiltrate the spleen; Langerhans cell histiocytosis and
other rare histiocytic disorders are usually the cause of this condition.
o Obstructed venous blood flow of intrahepatic or extrahepatic
etiology can cause splenomegaly. The most common causes include portal vein
thrombosis, hepatic cirrhosis, and congestive heart failure. Children with
extrahepatic portal venous obstruction, such as cavernous transformation,
often present with splenomegaly as the primary manifestation of their
disease.
o Many storage diseases result in splenomegaly. In Gaucher or
Niemann-Pick disease, splenomegaly is often the first clinical manifestation
. Splenomegaly is the result of the accumulation of abnormal lipids in
splenic macrophages.
o After trauma, palpable subcapsular hematomas may develop in the
spleen, which may eventually develop into clinically palpable pseudocysts.
Patients with congenital splenic cysts usually present with asymptomatic
splenomegaly.
o Although normally found only during the first 6 months of life,
extramedullary hematopoiesis may occur in diseases associated with intense
demand on the bone marrow for cell production. Thalassemia major,
osteopetrosis, and idiopathic myelofibrosis are examples of this rare cause
of splenomegaly.
* Hypersplenism is a clinical syndrome in which cytopenias result from
excessive splenic function, which occurs as the spleen and its MPS tissues
hypertrophy. The pathologic action of the spleen, ie, the reduction of
circulating blood elements, has been attributed to 4 possible mechanisms:
excessive splenic phagocytic activity, splenic production of an antibody
that results in the destruction of hematopoietic cells, overactivity of
splenic function, and sequestration.20 In patients with cirrhosis,
abnormalities of cytokine production may contribute to the cytopenias noted.
13
o As the spleen enlarges, it can sequester erythrocytes,
leukocytes, and platelets, resulting in mild-to-moderate decreases in some
or all of these cell lines. Severe reductions in cell counts are unusual and
should prompt a search for alternative etiologies.
o Venous obstruction is the most common cause of hypersplenism.
Any increase in portal pressure is reflected in the splenic venous sinuses.
This impairs blood flow out of the cords and results in the sequestration of
blood cells and hypersplenism. Hypersplenism in children is most frequently
caused by portal hypertension. Extrahepatic venous obstruction from portal
vein thrombosis is the most common cause of increased portal pressures. In
extrahepatic venous obstruction, hepatic function is normal. Intrahepatic
venous obstruction is usually due to cirrhosis.
o Portal hypertension usually increases flow through minor
collateral vessels between the portal circulation and the systemic
circulation. Portal hypertension can result in recognizable dilatation of
the superficial abdominal veins and esophageal varices. Patients with these
varices may present with sudden and catastrophic GI hemorrhage.
* Splenic sequestration crisis is a specific form of acute hypersplenism
in young children with sickle cell anemia.20
o Children less than 6 years old can develop rapid splenic
sequestration and splenomegaly with the consumption of large volumes of
erythrocytes. They present with sudden weakness, dyspnea, and left-sided
abdominal pain in addition to splenomegaly.
o Splenic sequestration is an emergency. Rapid death from
hypovolemic shock can result.
o Treatment consists of fluids and erythrocyte transfusions. To
prevent recurrences, splenectomy may be indicated. In most patients with
sickle cell disease, the spleen eventually involutes, and sequestration is
no longer possible.
【在 h*****g 的大作中提到】
: 一个朋友的孩子（女孩）生下来之后身体就比较弱，当时上海儿童医院诊断是巨细胞感
: 染造成的血小板
: 下降。
: 中西医治疗了一阵子后有所稳定，但是现在又开始严重，主要症状是脾肿大（已经进入
: 盆腔）. 其他伴
: 随的症状还有：
: 经常鼻子出血（特别是血小板水平低的时候）；
: 脸上有小紫点（应该是血斑）；
: 饭量不好（现在三岁半，身高只有92cm）；
: 国内南京和连云港，还有苏州的医院的医生倾向于认为是母亲怀孕时通过脐带造成的病
| h*****g
发帖数: 1523 | 3 谢谢，现在国内能找到一个认真负责，而且有水平的医生真的很难。
文章我先看看！ |
|
