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Is there a deficiency in the cellular brance of the patient\'s adaptive immune s

ID: 255651 • Letter: I

Question


Is there a deficiency in the cellular brance of the patient's adaptive immune system? Explain.

A 5-year-old girl was admitted to the hospital with a tender mass in the right upper quadrant of her abdomen, along with severe dyspnea, a persistent cough, and chest and abdominal pain She had a history of several bouts of pneumonia, starting at momths Chest X-ravs showed cloudy spots consistent with a lung infection. Sonography rerealed a liver abscess. The abscess was drained and a sample was cultured and examined by microscopy (seephoto). Note: the photo may look fuzzy when printed; be sure to look at the online version ] Blood tests revealed a white blood cell cmmt nf 12.1 × 109L (nonnal is 4-11 × 10,L). The ratio of neutrophils to lymphocytes was approximately 3:2 The serum IgG level was 16.5 mgmL, IgM was 2.5 mgmL, and IgA was 1.75 mgmL A sample of the patient's white blood cells was mixed with the fluorescent cye diavdrorhadamine (DHR) and with PNMA, asubstance that nonspecifically stimulates neutrophils (mimicking the response that occurs when a neutrophil encounters a pathogen). IfH.O, is produced in response to stimulation, DHR fluorescence increases. The white blood cells were analyzed before (Testing) and after the PMA stimulation Cell counts (number of cells) is plotted against DHR Auorescence for mhite blood cells obtained from a normal cantrol and from the patient Resting Stimulated with PMA Normal control 10 DHR 123 [Resting) DHR 123 (4) Patient HR 123 (Restingl

Explanation / Answer

As per the case details,I could conclude that the 5 year old girl admitted to the hospital is suffering from Chronic granulomatous diseases(CGD).This desorder is cause due to phagocytic cell deficiency.which comes under inherited primary immunodeficiency disorders.

Phagocytic deficency disorder-Due to the lack of phagocyted,bacterial infecton are common in the person.The phagocytes are unable to move,adhere and remove the harmful micro-organisms or fungi.

Chronic granulomatous diseases-It happense when neutrophils are unable to lyse harmful micro organisms because of defective NADPHoxidase enzyme.The defective enzyme leads to the formation of ROS(reactive oxygen species)H2O2 and SOD which promote the survival of harmful organisms resulting in formation of granuloma.

Major symptoms are- Persisting Pneumonia,Inflammation of lymph nodes,liver absesses.lung infection.

Diseases causing agent-Aspergillus,staphylococcus.

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