What is pulmonary alveolar proteinosis and lymphangioleiomyomatosis?

---

Pulmonary alveolar proteinosis is a rare condition and patients with this disease usually present with insidious dyspnoea that has progressed over months, sometimes with mild systemic symptoms such as low grade fever and weight loss.Usually, it has a benign course with eventual resolution. Rarely, rapid progression to respiratory failure is seen.
Death occurs due to progressive filling of alveoli or superimposed infection.
http://www.histopathology-india.net/PrAlPr.htm
In the rare condition of lymphangioleiomyomatosis (LAM) a bizarre proliferation of smooth muscle cells (LAM cells) in the lung involves the smooth muscle of lymphatics, arteries, veins, bronchioles, and alveolar walls.
Dyspnea is the usual presenting symptom.The condition is characterized clinically by slowly progressive breathlessness and basal shadowing, and three distinctive complications: multiple pneumothoraces, haemoptyses and chylous effusions.Oophorectomy has produced good results. Treatment is by oestrogen antagonists or progestogens but the response is unpredictable and does not always correlate with the presence of oestrogen receptors.
http://www.histopathology-india.net/lymphangioleiomyomatosis.htm  (+ info)

Pulmonary Alveolar Proteinosis?

---

I was just diagnosed with this today, and this is a very rare disease. Has anyone else had this disease or know anyone that has had this and what I can expect now that I know?
When my daughter first became ill, one of the diagnoses they considered for her was pulmonary alveolar proteinosis due to a partial Surfactant B deficiency. Due to the extent of her illness at that time, we were told that if that diagnosis was confirmed (due to the surfactant mutation, not the PAP), it would be fatal. Thankfully, that diagnosis was not confirmed (my daughter was diagnosed with interstitial lung disease, diffuse alveolar damage, and is alive and doing alright with chronic lung disease at age 19 months). I do regret that there are others not as fortunate as we are.

In an adult, since the cause is usually different, there are treatments that can be done, so the outcome can be positive. Whole lung lavage (washing the stuff out) can be performed, and this may lead to dramatic improvement in many. Lung transplant is also an option if other treatments do not work. The best thing you can do is to talk to your pulmonologist about your options and what he or she expects may happen.

I am sorry that you were diagnosed with this frightening condition, and I hope you do well with treatment. I do understand that the fact that it is a rare condition makes it that much more terrifying for you. As a mother to a child with a rare lung disease, I can tell you that there is a lot of information on the internet, and a whole lot of it will not apply to you. I reccomend reading the article below and contacting the PAP Foundation. They can put you into contact with others who have PAP. The third link is to the rare lung disease consortium. There is also some good info there. Best of luck to you.  (+ info)

what is the case in which the surface area of pulmonary alveolar are icreased?

---

the case in which surface area of pulmonary alveolar are increased that is lead to increasing in perfusion between them and blood supply
There is increasing evidence that hypercapnia improves oxygenation, although this is not predicted by the classical alveolar gas equation, which would suggest that at a given FIO2 and minute ventilation, increasing the FICO2 would reduce arterial O2. However, the effects appear to be mediated through improved V/Q matching, increased cardiac output and increased mixed venous oxygenation. Whereas beneficial effects in oxygenation are seen in multiple experimental models, these have not been mirrored in severely ill patients with ARDS. In this context, while hypercapnia per se may have effects on increasing V/Q matching and cardiac output, the effects of reduced tidal volume required to achieve hypercapnia may be counterproductive. Thus, there may be significant differences in oxygenation resulting from hypercapnia depending on how the hypercapnia is induced.  (+ info)

How does alveolar surface tension, compliance and airway resistance each affect pulmonary ventilation?

---

Very simply:
Alveolar surface tension: if too high, then alveoli have too much surfactant and do not transport oxygen well to the capillaries. If too low, then alveoli collapse and do not allow filling with air. Both will decrease ventilation

Airways resistance: if too high then airflow is reduced (think asthma). This leads to decreased absolute volume of air moving in and out of lungs, thereby reducing ventilation. If too low then the larger airways become floppy and tend to collapse with exhalation (called bronchomalacia), also decreases ventilation

Compliance: too low (noncompliant) = stiff lungs, harder to expand and contract = decreased ventilation. Too high (overly compliant) = flabby lungs = poor airflow even with full inhalation (you need some stiffness to create a venturi effect in the airways during inhalation) = decreased ventilation  (+ info)

How airway resistance, lung compliance, and alveolar surface tension influence pulmonary ventilation?

---

Help..any thing can help me out!
uhh, from what I remember about physiology:

Pulmonary ventilation - how much air exposure can you give your alveoli?

Airway resistance (eg. more resistance if bronchioles are narrowed in asthma).. the more resistance, the less pulmonary ventilation can be achieved.

Lung Compliance - how much "bounce-back" or recoil force there is from the lung (eg. emphysema.. have too little recoil force, and have problems exhaling). So there is a balance of forces here.

Alveolar surface tension - the little border between liquid and air in your alveoli. The oxygen in the air must get across the membrane to be absorbed by the blood vessels in your alveoli. The greater the surface tension, the harder it is for the oxygen to get into the blood. (example in some newborns, they don't have a certain chemical surfactant to reduce surface tension, and consequently can't breathe. They need treatment with a surfactant on the lung tissue) So the greater the surface tension, the lower the pulmonary ventilation.

Hope this helps  (+ info)

How long does it take to destroy your Alveolar sacs?

---

I've been a smoker for almost 3 years, but I'm quitting in 2 weeks. I want to know how long the Alveolar sacs can hold out and if mine can still recover.
Hey, congrats on being set to quit! Good luck with that. I think that once you destroy them, they can't come back. However, since you've only been smoking for 3 years, i don't think you'd have destroyed too many. On the good side, once you quit your lungs will gradually get into better condition.  (+ info)

What effects could pneumonia and pulmonary congestion have on gas exchange?

---

Both pneumonia (infectious inflammation of the lungs) and pulmonary congestion (left heart failure) can lead to fluid accumulation in the lungs.
Pneumonia and pulmonary congestion cause a fundamental change in the way gas is exchanged within the lungs. Gas is exchanged through structures called alveoli. They are microscopic sacs of epithelial tissue. When these sacs become filled with fluid (referred to as consildation) gas cannot pass from the alveoli into the blood. Thus, gas exchange is impeded and the patient typically must work harder to breathe.  (+ info)

What is involved in a typical pulmonary function test?

---

I have to get a typical pulmonary function test tomorrow. What exactly does this entail? I looked online and didn't find anything that really explained it. I'm 15, if age makes any difference. Thanks!
In a spirometry test, you breathe into a mouthpiece that is connected to an instrument called a spirometer. The spirometer records the amount and the rate of air that you breathe in and out over a period of time.  (+ info)

How does pregnancy increase the risk of pulmonary and other emboli?

---

How is pregnancy associated with an increased risk in pulmonary embolism? Does this explain the association between long term use of oral contraceptives and thrombosis, which could possibly lead to embolism?
Yes and no. The hormones that are increased in pregnancy and contained in the contraceptive Pill aren't the only risk factors for blot clots forming during pregnancy. What the Pill and pregnancy both have in common is that they both produce hormones that increase the bloods clotting ability. Oestrogen increases the risk of DVTs by increasing the production of certain chemicals necessary for the blood to clot. It also increases platelet numbers + stickiness of platelets, which increases clot formation (to prepare for blood loss during child birth). Progestogen in the Pill causes the blood vessels to relax and widen, allowing the blood to pool in the veins, increasing risk of clot formation.

Blood clots in pregnancy are generally the result of increased venous stasis- blood pooling in the lower deep veins. As I said above, balance of proteins of the coagulation + fibrinolytic systems also play a part. DVT can of course lead to a pulmonary embolism. Clots in pregnancy and from the Pill are generally always the results of clots that have formed in the deep veins of the legs, broken away and then embolised in the lungs.

Women who do have clots in pregnancy and whilst taking the contraceptive Pill, generally also have certain other risk factors such as protein C and S deficiency and most commonly Factor V Leiden mutation. Over all health of the woman also plays a part- obesity, poor diet/unhealthy lifestyle and a smoking history also increases the risk as does age.

During pregnancy the uterus also expands, putting pressure on the lower blood vessels which can restrict blood flow from the legs and pelvis back to the heart. Physiological structural changes during pregnancy are risk factors alone.  (+ info)

What are the multidisciplinary team responsibilities for the patient suffering a pulmonary contusion?

---

I have a case scenario which I am writing an assignment on. (Pulmonary contusion and Acute Respiratory Failure) part of this assignment requires me to discuss the multidisciplinary roles. The info on this is not very clear, I recognise there are roles played by the physiotherapist and nurse also the physician and have a general idea of what they are but need confirmation, Can anyone help?
let the nurses do everythng, cause they are the know-it-alls of the medical field.  (+ info)

---

---

• Home
• Our method
• Usage examples
• Index

• Statistics
• Contact

---