Hemoptysis Là Gì

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Home ▶ Professionals ▶ Clinical Resources ▶ Clinical Cases ▶ A Cystic Fibrosis Patient with Hemoptysis From an Unusual Cause

Sugeet Jagpal, MD

Fellow

Division of Pulmonary và Critical Care Medicine

Rutgers University Robert Wood Johnson Medical School

New Brunswiông chồng, New Jersey


Vyacheslav Gendel, MD

Division of Interventional Radiology

Rutgers University Robert Wood Johnson Medical School

New Brunswichồng, New Jersey


John L. Nosher, MD

Division of Interventional Radiology

Rutgers University Robert Wood Johnson Medical School

New Brunswick, New Jersey


Lakshmi Uppaluri, MBBS

Assistant Professor

Department of Pediatrics

Rutgers University Robert Wood Johnson Medical School

New Brunswichồng, New Jersey


A Caucasian child had Cystic Fibrosis (CF) diagnosed at birth with a positive sweat chloride kiểm tra, & required surgery as a neonate for meconium ileus.  A liver biopsy at three months of age demonstrated focal biliary cirrhosis, and the patient suffered from esophageal varices that required sclerotherapy until eighteen months of age.  From the age of two onwards the patient did not have sầu further clinical manifestations of cirrhosis.  Her pulmonary symptoms were not significant, & imaging demonstrated mild bronchiectasis of the right middle và lower lobes.  Genotyping was done during adolescence, and she was found to lớn have sầu one copy of ΔF508 while the second mutation remained unknown.

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After being lost to lớn follow up for several years, the patient presented to our emergency room at the age of seventeen with nausea & dizziness.


Physical Exam


The vitals on admission revealed mild sinus tachycardia with a pulse of 90/minute, systolic blood pressure between 90-100 milimet Hg range, but no tachypnea, pain or fever. Exam demonstrated a thin female who was developmentally normal for her age. She did not have any overt manifestations of cirrhosis, with no spider angiomas, palmer erythema, or asterexis noted. Her lung exam was positive for mild crackles at the right lung base, and she did not have clubbing. Abdominal exam was benign with normoactive bowel sounds.

Orthostatics were attempted & rapidly aborted when the patient vomited a moderate amount of bright red blood.  Blood work demonstrated that her hemoglobin and platelets had decreased compared to prior labs (9 gm/dl Hg and platelets 100,000/ µL), and she was admitted to lớn the pediatric intensive care unit. She was given packed red blood cells, esomeprazole và octreotide drips were administered, & endoscopy was planned.


Question 1

What is the underlying mechanism by which cystic fibrosis patients develop cirrhosis?

A.Agenesis of the liver Incorrect!
B.Dietary deficiency from lack of absorption of vitamins Incorrect!
C.Loss of function of transmembrane receptor on cholangiocyte Correct!
D.Medication side effect from CF medications Incorrect!

Answer: C

The cystic fibrosis transmembrane receptor (CFTR) is located on the apical membrane of the cholangiocyte, và with loss of function in the receptor there is decreased bile flow and inspissation of bile. This leads to lớn an obstructive sầu process, & eventually to lớn bile plugs, bridging fibrosis, and obliteration of bile ducts.  Fibrosis of the liver leads to the development of portal hypertension, which can cause esophageal varices, portosystemic shunts, và pulmonary complications such as portopulmonary hypertension or hepatopulmonary syndrome (1, 2).

Despite the loss of function of CFTR, only 10% of CF patients develop clinically significant liver disease (1).  It is thought there is a second, & possibly third, hit that is required prior lớn progression.  Exposure lớn a hepatotoxin, or presence of other genes damaging the liver in combination with loss of CFTR function leads to release of proinflammatory and fibrogenic mediators và activation of inflammatory cells, including portal fibroblasts, which lead khổng lồ formation of peribiliary fibrosis.

History Continued:

The patient was taken khổng lồ the endoscopy suite the next morning, và was intubated for an upper endoscopy. Three esophageal varices were banded, và subsequently the patient was noted to have frank hemoptysis from the endotracheal tube.

Question 2

What is the most likely vascular bleeding source for this cystic fibrosis patient?

A.Bronchial artery Correct!
B.Pulmonary artery Incorrect!
C.GI tract Incorrect!
D.

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AV malformation Incorrect!

Answer: A  

Ninety percent of hemoptysis originates from the bronchial artery, và the pulmonary artery accounts for 5% (3). Tuberculosis remains the leading cause of hemoptysis worldwide. Other conditions include bronchiectasis, especially in cystic fibrosis patients, và bronchitis.

The patient was taken from the endoscopy suite khổng lồ the interventional radiology suite for a bronchial arteriogram, with the assumption that her hemoptysis was secondary khổng lồ bronchiectasis. Bronchoscopy could have been considered for this patient, but given the non-focal computed tomography of the chest in the past & the belief that there would not be an endobronchial source for intervention it was not considered to be high yield.

Question 3

What is the most effective sầu non-surgical management for hemoptysis?

A.Lateral decubitus position Incorrect!
B.Endobronchial tamponade Incorrect!
C.Conservative sầu management Incorrect!
D.Bronchial artery embolization Correct!

Answer: D

Bronchial artery embolization (BAE) is considered the most effective sầu non-surgical treatment in massive sầu hemoptysis because of immediate & long-term results (3, 4).  Selective sầu angiography should be performed initially lớn locate the bleeding bronchial artery before the injection of particles (polyvinyl alcohol foam, isobutyl-2-cyanoacrylate, absorbable gelatin pledgets, or Gianturco steel coils) (4).  

For our patient, the bronchial angiogram demonstrated evidence of peripheral arteriovenous shunts – findings that are suggestive of hepatopulmonary syndrome (Figure 1a) as opposed to lớn bleeding from bronchiectasis. Hemoptysis is not a comtháng finding for hepatopulmonary syndrome, but when it occurs it is thought that the vascular malformations are a result of the hypertensive portal system (5). The patient had cross-sectional imaging after her procedure, which verified that she had cirrhosis with portal hypertension as well as areas of arteriovenous shunting (Figure 1b). She also demonstrated evidence of hypoxia, with a PaO2 of 47 on 50% FiO2.  

History Continued:

She was extubated two days post procedure, but required approximately a week of supplemental oxygene prior khổng lồ being discharged home on room air. She did not require oxygene with exertion.

Figure 1

*

A (left): Right bronchial arteriogram demonstrating atypical dilatation of the peripheral arterial branches and significant arteriovenous shunting.

B (right): Axial CT slice of the chest demonstrating bilateral peripheral nodularities, consistent with pulmonary arterial branches with areas of arteriovenous shunting. Also noted is a relative lachồng of bronchiectasis typically seen in patients with cystic fibrosis.

Question 4

What is the most conspicuous pathoxúc tích và ngắn gọn finding in hepatopulmonary syndrome?
A.Bronchial wall thickening Incorrect!
B.Pronounced vascular dilatation of blood vessels Correct!
C.Pulmonary hypertension Incorrect!
D.Epithelial cell destruction Incorrect!

Answer: B  

Hepatopulmonary syndrome is a complication of cirrhosis that is defined by advanced chronic liver disease with increased portal pressures, an arterial oxygenation defect, và widespread pulmonary vascular dilations (6). The most conspicuous pathosúc tích & radiologic finding is pronounced vascular dilatation of peripheral vessels. Clinically, patients can present with clubbing, spider angiomas, platypnea (shortness of breath that worsens with sitting) and orthodeoxia (accentuation of arterial hypoxemia in an erect position). The precise pathogenesis is under study, with the nitric oxide pathway at the forefront of research (6).  When hepatopulmonary syndrome is mild to lớn moderate, the predominant mechanism for hypoxemia is a ventilation perfusion abnormality. When hepatopulmonary syndrome is severe, a shunt mechanism is involved as well.  Diffusion is also impacted as the diameter of peripheral vessels increases, which leads to exertional hypoxia.  

The patient initially did well for several months; however, she returned six months later with abdominal pain. An extensive workup initially failed lớn reveal the cause.  She eventually had a pelvic arteriogram, which revealed right lower quadrant varices from a portosystemic shunt.  Given the presence of multiple portosystemic shunts, the patient was referred once again khổng lồ interventional radiology for a transjugular intrahepatic portosystemic shunt (TIPS) procedure, which was performed without ostensible difficulty or complications.

Since her TIPS, the patient has not had variceal bleeding or further episodes of hemoptysis. Her imaging continues to show a nodular liver with functional TIPS 4-years after the procedure.


References

Rowl& M, Bourke B. Liver disease in cystic fibrosis. Current Opinion in Pulmonary Medicine 2011; 17:461-466Cheng K, Ashby D, Smyth RL. Ursodeoxycholic acid for cystic fibrosis-related liver disease. Cochrane Database Systemic reviews 2012; Issue 10Jean-Baptiste E. Clinical assessment & management of massive sầu hemoptysis. Crit Care Med 2000;28:1642-1647Funaki B. Systemic arterial embolization for hemoptysis. Seminars in Interventional Radiology 2010; 314-319.Schnader J, Smith R, Britt EJ, Katz A, Kelley M, Schraufnagel D. Hemoptysis, Hepatopulmonary Syndrome, và Respiratory Failure: Clinical Conference on Management Dilemmas. Chest 1997; 111:1724-32Fritz J, Fallon M, Kawut, S. Pulmonary vascular complications of liver disease. Am J Respir Crit Care Med 2013;187: 133–143

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