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پاسخ :

تشخيص هاي افتراقي:

تشخيص نهايي :

Low grade mucoepidermoid tumor

معرفی کیس:

دکتر صدر

تشخیص های افتراقی:

خانم دکتر ناهید رادمنش

رزیدنت بیمارستان مفید

خانم دکتر لیلا بنی آدم

رزیدنت بیمارستان امام حسین

خانم دکتر صدیقه تهرانچی

رزیدنت بیمارستان لقمان

خانم دکتر ندا قیام

رزیدنت بیمارستان شهدا

دکتر غفاری پور

فلو ریه بیمارستان مفید

دکتر کاکوئی

رادیولوژیست بیمارستان مسیح دانشوری

خانم دکتر ناهید رادمنش

رزیدنت بیمارستان مفید

خانم دکتر لیلا بنی آدم

رزیدنت بیمارستان امام حسین

خانم دکتر صدیقه تهرانچی

رزیدنت بیمارستان لقمان

Problem list

Chronic cough

Acute respraitory distress

Anemia

Collapsed lung

Bronchiectasis&wheezing

hepatomegaly

Bronchiectasis

PATHOPHYSIOLOGY — In general, induction of bronchiectasis requires two factors

An infectious insult

mpaired mucus clearance, airway obstruction, or a defect in host defense

BRONCHIECTASIS

Definition:  Abnormal and permanent dilation of bronchi.

Focal or diffuse distribution

Clinical consequences – chronic and recurrent infection and

Pooling of secretions in dilated airways.

Classification: 

Cylindrical (fusiform)

Saccular

Varicose

The correlation of these patterns with clinical status, etiology, or pathophysiology is not well established .However, studies using high-resolution CT scan suggest that bronchiectasis may be a dynamic process, and that cylindrical bronchiectasis can be reversible if the underlying cause is successfully treated (eg, patients with atelectasis, infection, or a retained foreign body)

Etiology:

Acquired bronchial obstruction

Congenital anatomic defects

Immunodeficiency states

Abnormal secretion clearance

Infection

Other disorders (including alpha-1 antitrypsin, bronchiolitis obliterans, and connective tissue diseases)

in 25 to 38 percent of pediatric cases, an underlying cause could not be identified despite a thorough evaluation

Causes of bronchiectasis in children, based on distribution

Localized

Foreign body aspiration

Intraluminal obstruction (granuloma, tumor)

Right middle lobe syndrome

Congenital abnormality (intralobar bronchopulmonary sequestration, bronchial stenosis, bronchomalacia, tracheal bronchus)

Extraluminal compression (lymphadenopathy ie, tuberculosis or vascular compression)

Foreign body aspiration

most common .a history of choking may not be recalled. pneumonia may improve with antibiotic therapy. However, the infiltrate on chest radiograph usually does not resolve, and recurrence of pneumonia is common

TB

 Pulmonary disease and associated intrathoracic adenopathy are the most frequent presentations of tuberculosis in children

classic triad: (1) recent close contact with an infectious case, (2) a positive TST, and (3) suggestive findings on chest radiograph or physical examination

A negative TST does NOT rule out tuberculosis disease. 15 percent or less for smear and 30 percent or less for culture.The most common chest radiograph finding is a primary complex, which consists of opacification with hilar or subcarinal lymphadenopathy, in the absence of notable parenchymal involvement re.

Connective tissue disorders&lung cancer

particularly systemic lupus erythematosus (SLE), can be complicated by bronchiectasis

Wegener,sarcoidosis,churg-strauss syndrome

Congenital

Williams-Campbell syndrome is a rare congenital disorder characterized by deficient cartilage in the bronchial tree, causing generalized tracheobronchomalacia .The bronchial cartilage is absent or deficient; the segmental and subsegmental bronchi are dilated and collapse easily.

 Children typically present before three years of age with cough, wheezing, and recurrent febrile illness

Congenital tracheobronchomegaly (Mounier-Kuhn syndrome) :a congenital disorder that is characterized by markedly dilated trachea and main bronchi, resulting in dynamic dilation and collapse during inspiration and exhalation. On pathologic examination, there is atrophy or absence of elastic tissue, and thinning of the muscular components of the airway. Outpouching of redundant mucosal tissue results in pooling of secretions and recurrent infection, leading to bronchiectasis. The clinical manifestations range from minimal disease to respiratory failure and death

sequestrations

On a chest radiograph, sequestrations typically appear as a uniformly dense mass within the thoracic cavity or pulmonary parenchyma .Recurrent infection can lead to the development of cystic areas within the mass disorder.

CT: solid mass that may be homogeneous or heterogeneous, sometimes with cystic changes

Immunodeficiency

Bronchiectasis has occasionally been reported in patients with selective IgA deficiency, which is the most common immune deficiency

Abnl secretion clearance

Cystic fibrosis (CF) is the most common cause of bronchiectasis in industrialized countries. a persistent, productive cough, hyperinflation of the lung fields on chest radiograph,

types:Classic, Nonclassic CF 

situs inversus (Kartagener syndrome). Children typically present with recurrent sinusitis, otitis media, and recurrent pneumonia due to impaired ciliary function, and bronchiectasis

Young's syndrome is characterized by obstructive azoospermia and sinopulmonary infections, but no identifiable ciliary dysfunction or structural defec

Alpha-1 antitrypsin

Alpha-1 antitrypsin (AAT) is a protease inhibitor that protects tissues from degradation. Severe deficiency of AAT is most often associated with emphysema and cirrhosis. Bronchiectasis is an uncommon complication of long-standing pulmonary disease; it may occur in older children and adults with AAT deficiency

The main clinical manifestations relate to three separate organs: the lung, the liver, and much less often the skin.

severe deficiency of AAT predisposes to chronic obstructive pulmonary disease, especially panacinar emphysema. Within the first two decades of life, liver dysfunction is the major threat to the health of affected individuals, and pulmonary dysfunction is not a major concern

Persistent bacterial bronchitis

(PBB) is increasingly recognized as a cause of chronic wet cough, particularly in young children. Identification and treatment of PBB is important because it may be a precursor to chronic suppurative lung disease, including bronchiectasis

Childhood infections with pertussis and measles have been associated with the development of bronchiectasis .However, since the advent of routine vaccinations, the incidence of these childhood infections has markedly decreased.

adenovirus has been associated with the development of Swyer James syndrome, which is characterized by unilateral small hyperlucent lung with bronchiectasis and diminished arterial supply

missing

Developmental hx.

H.x of close contact for TB

Liver span

Bronchoscopy findings

DIAGNOSIS

CF&TB

FBA&A1AT

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