Hepatopulmonary Syndrome

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Hepatopulmonary Syndrome is an uncommon severe lung abnormality, due to triad of liver condition, in the blood, it lessens the quantity of oxygen (hypoxemia) and presence of intrapulmonary vascular dilation rises. WBC or white blood cells function to protect the body’s immune system against infection and eosinophils comes in the category of WBC. Hypereosinophilic syndrome […] Read More

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Hepatopulmonary Syndrome

Hepatopulmonary Syndrome is an uncommon severe lung abnormality, due to triad of liver condition, in the blood, it lessens the quantity of oxygen (hypoxemia) and presence of intrapulmonary vascular dilation rises.

WBC or white blood cells function to protect the body’s immune system against infection and eosinophils comes in the category of WBC. Hypereosinophilic syndrome predominantly affects skin, heart, bone marrow, nervous system and lungs. Peripheral eosinophils typically elevate so disorder is idiopathic. This syndrome largely affects men between the ages of 20-60. In a healthy person count of eosinophil is around 500-600 per microlitre. Broad names of this syndrome are lymphocytic and myeloproliferative.

Children and women have negligible (very less) chances of this disorder. The chronic hypereosinophilic syndrome can end up in myocardial fibrosis, heart failure and death. Ethnicity isn’t involved in this disease so any men are it black white or Asian, have equal chances of getting trapped in this. 


Complications Of Hypereosinophilic Syndrome

  • Parasitic infection
  • Thrombus formation
  • Chromosomal Abnormalities
  • Heart valve thickens
  • Endomyocardial fibrosis
  • Alkaline phosphatase level increases

Risk Factors Of Hypereosinophilic Syndrome

  • A person whose body doesn’t fight against infection
  • A person whose gender is male
  • Age is 20-60
  • Past history of blood cancer
  • Past history of rheumatic diseases
  • Repetitive skin diseases
  • Family history

Hypereosinophilic Syndrome’s Treatment

The purpose of treatment is to normalize the eosinophil count, reduce the skin disease and to arrange chromosomes in order. Type of the treatment relies on symptoms of the patient, its cause, the organ/organs damage level and on conducted tests.

If no serious symptoms are there then monitoring and medicines are considered to be excellent options. Medicines like:

  • Gleevec
  • Siklos
  • Vincristine
  • Hydrea
  • Droxia 

A complete examination is done to vanish the chances of organ damage.

If the heart or nervous system aren’t functioning in the way it should then Prednisone will be prescribed by a doctor. Usually, this syndrome thickens the blood so to neutralize it warfarin or heparin is given most of the time. 

Other medications are:

  • Etoposide
  • Arevincristine

Surgery For Hypereosinophilic Syndrome

When nothing works, and damaged organs begin to damage other organs of the body, surgery becomes essential. There are multiple options for surgery which doctor selects according to the patient’s health and the organ which is damaged.

  • Valve Replacement Surgery

This surgery increases the life expectancy of patients by 90-95%.  When single or all the four valves stop responding due to this syndrome. The huge cut will be made from the neck to belly button, cut can be shorter and also depends upon the severity, then another cut will be made in the aorta to remove the damaged valve and to add a new valve. The surgery has minimal chances of death about 1-2% and also this surgery regulates accurate blood circulation. 

  • Thrombectomy

When syndrome enters in your body, it starts interrupting the consistency of blood by making it thicker and eventually blood clotting starts. During this procedure, the surgeon puts intravenous in your hand. Warfarin or heaprin will be given. Incisions will be made around blood vessels will remove the clotted blood then the area will be closed with sutures and normal blood flow starts.

  • Bone Marrow Transplant

Damaged bone marrow will be replaced with a new one by adding new and working stm cells. This surgery is the most chosen one.

  • Splenectomy – When the spleen is causing blood-related disorders then the doctor either treats the particular area or removes a portion or entire spleen.

DIAGNOSIS OF HYPEREOSINOPHILIC SYNDROME

Patients should clear medical history in order to pursue the diagnosis. 

Tests which are performed during analysing this disorder are:

  • Electrocardiogram
  • Spirometry – to check the health of lung
  • Computed Tomography – It thoroughly monitors spleen, bone marrow and chest
  • Blood tests – To know about infections 
  • Chest X-ray
  • Cytogenetic Test
  • CT scan
  • To detect the gene mutation – genetic tests are available
  • Pulmonary function test
  • MRI Scan

Symptoms

  • Skin itching and redness
  • Abdominal pain
  • Joint and muscle pain
  • Fever
  • Chest pain
  • Fatigue
  • Rhinitis
  • Shortness of breath
  • Cough
  • Blurry vision
  • Dizziness
  • Night sweats
  • Irregular bowel movements
  • Nausea

Symptoms totally depend upon the age and which part of the body is connected with this syndrome.

Causes

Actual cause is still hidden but assumptions are made, like:

  • Drug/medicine allergy
  • Mutation of genes generally called familial hypereosinophilic syndrome, results due to some family history.
  • Clonal Myeloid malignancy
  • Elevation of protein
  • Addison’s disease
  • Skin diseases like dermatitis
  • Helminthic disease
  • Sarcoidosis
  • Leukaemia
  • Bone Marrow disorders

However it is not sure that all these things are causing this syndrome but still research is saying that this can worsen eosinophils numb