NEET MDS Lessons
General Pathology
Achrondroplasia
is a major cause of dwarfism. The underlying etiology is a point mutation in the fibroblast growth factor receptor, which causes inhibition of chondrocyte proliferation, which is associated with suppression of the normal epiphyseal growth plate expansion. Thus, long bone growth is markedly shortened.
The most conspicuous changes include disproportionate shortening of the proximal extremities, bowing of the legs, and a lordotic posture.
Microscopically, the cartilage growth plates are disorganized and hypoplastic.
Cryptococcosis
An infection acquired by inhalation of soil contaminated with the encapsulated yeast Cryptococcus neoformans, which may cause a self-limited pulmonary infection or disseminate, especially to the meninges, but sometimes to the skin, bones, viscera, or other sites.
Cryptococcosis is a defining opportunistic infection for AIDS, although patients with Hodgkin's or other lymphomas or sarcoidosis or those receiving long-term corticosteroid therapy are also at increased risk.
AIDS-associated cryptococcal infection may present with severe, progressive pneumonia with acute dyspnea and an x-ray pattern suggestive of Pneumocystis infection.
Primary lesions in the lungs are usually asymptomatic and self-limited
Pneumonia usually causes cough and other nonspecific respiratory symptoms. Rarely, pyelonephritis occurs with renal papillary necrosis development.
Most symptoms of cryptococcal meningitis are attributable to brain swelling and are usually nonspecific, including headache, blurred vision, confusion, depression, agitation, or other behavioral changes. Except for ocular or facial palsies, focal signs are rare until relatively late in the course of infections. Blindness may develop due to brain swelling or direct involvement of the optic tracts. Fever is usually low-grade and frequently absent.
Bronchitis
Bronchitis is an obstructive pulmonary disease characterized by inflammation of the bronchi of the lungs
Signs and symptoms
persistent cough that produces sputum
shortness of breath (dyspnea) on exertion
hypercapnia
insufficient oxygenation of the blood hypoxemia leading to cynosis
Severe chronic bronchitis will commonly lead to cor pulmonale and heart failure.
Pathology
an increase in the number of goblet cells with mucus blocking the airway clusters of pigmented alveolar macrophages
the presence of inflammatory cells (e.g. neutrophils) scarring (fibrosis) of the walls of the bronchioles
Diagnosis
- decreased intensity of breath sounds (rhonchi) and extended expiration.
- a sputum culture has pathogenic microorganisms
- a chest x-ray that reveals hyperinflation and increased bronchovascular markings
- a pulmonary function test that shows an increase in the lung's residual volume and a decreased vital capacity
Pathophysiology
- The initiating event in developing bronchitis appears to be chronic irritation due to inhalation of certain chemicals
- earliest clinical feature of bronchitis is increased secretion of mucus by submucousal glands of the trachea and bronchi
- Damage caused by irritation of the airways leads to inflammation and infiltration of the lung tissue by neutrophils
- The neutrophils release substances that promote mucousal hypersecretion
- As bronchitis persists to become chronic bronchitis, a substantial increase in the number of goblet cells in the small airways is seen
- The role of infection in the pathogenesis of chronic bronchitis appears to be secondary.
Treatment
Quit smoking, Oxygen therapy, bronchodilator drugs
Prognosis
Pulmonary hypertension, cor pulmonale, and chronic respiratory failure are possible complications of chronic bronchitis
In severe chronic bronchitis is poor
Lymphomas
A. Hodgkin’s disease
1. Characterized by enlarged lymph nodes and the presence of Reed-Sternberg cells (multinucleated giant cells) in lymphoid tissues.
2. Disease spreads from lymph node to lymph node in a contiguous manner.
3. Enlarged cervical lymph nodes are most commonly the first lymphadenopathy observed.
4. The cause is unknown.
5. Occurs before age 30.
6. Prognosis of disease depends largely on the extent of lymph node spread and systemic involvement.
B. Non-Hodgkin’s lymphoma
1. Characterized by tumor formation in the lymph nodes.
2. Tumors do not spread in a contiguous manner.
3. Most often caused by the proliferation of abnormal B cells.
4. Occurs after age 40.
5. Example: Burkitt’s lymphoma
a. Commonly associated with an EpsteinBarr virus (EBV) infection and a genetic mutation resulting from the translocation of the C-myc gene from chromosome 8 to 14.
b. The African type occurs in African children and commonly affects the mandible or maxilla.
c. In the United States, it most commonly affects the abdomen.
d. Histologically, the tumor displays a characteristic “starry-sky” appearance.
Str. Pneumoniae
Probably the most important streptococci. Primary cause of pneumonia. Usually are diplococci. Ste. pneumoniae are α-hemolytic and nutritionally fastidious. Often are normal flora.
Key virulence factor is the capsule polysaccharide which prevents phagocytosis. Other virulence factors include pneumococcal surface protein and α-hemolysin.
Major disease is pneumonia, usually following a viral respiratory infection. Characterized by fever, cough, purulent sputum. Bacteria infiltrates alveoli. PMN’s fill alveoli, but don’t cause necrosis. Also can cause meningitis, otitis, sinusitis.
There are vaccines against the capsule polysaccharide. Resistance to penicillin, cephalosporins, erythromycins, and fluoroquinalones is increasing.
Autoimmune(acquired) Haemolytic anaemia
Auto antibodies are usually Ig g type (may be Ig M or Ig A). They may or may not bind complement and may be active in warm or cold temperature They may be complete (agggIutinating) or incomplete. Haemolysis s may be intravascular due to destruction of the antibody coated cells by RE system.
Causes:
a. Idiopathic
b. Secondary to
o Drugs - Methyldopa, Mefanamic acid
o Disease like
-> Infections especially viral.
-> Autoimmune disease especially SLE.
-> Lymphomas and chronic lymphatic leukaemia.
-> Tumours.
Diagnosis : is based on
• Evidences of haemolytic anaemia.
• Demonstration of antibodies
- On red cell surface by direct Coomb’s test
- In serum by indirect Coomb’s test.
Chronic lymphocytic leukaemia
Commoner in middle age. It starts insidiously and often runs a long chronic course
Features:
- Lymphnode enlargement.
- Anaemia (with haemolytic element).
- Moderate splenomegaly.
- Haemorrhagic tendency in late stages.
- Infection.
Blood picture:
- Anaemia with features of haemolytic anaemia
- Total leucocytic count of 50-100,OOO/cu.mm.
- Upto 90-95% cells are lymphocytes and prolymphocytes.
- Thrombocytopenia may be seen.
Bone marrow. Lymphocytic series cells-are seen. Cells of other series are reduced,