cs03- John Fitzgerald John Fitzgerald was well for the first 10 months of his li
ID: 151809 • Letter: C
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cs03- John Fitzgerald John Fitzgerald was well for the first 10 months of his life. In the next year he had pneumonia once, several episodes of otitis media, and on one occasiorn developed erysipelas (streptococcal skin infection) on his right cheek. These infections were all treated successfully with antibiotics. John's mom was a nurse in Norwalk OH, and to her it seemed like John was always on antibiotics. John's mom worried about such things, as she had had two brothers, each of whom died of pneumonia before they were two years old, and one sister, who lost one of her boys (Bobby, a 3-year-old male) to pneumonia just the previous year. John was a bright and active child who gained weight, grew, and developed normally, but continued to have ear and sinus infections, and twice again had pneumonia. At 2 years and 3 months his pediatrician tested his serum immunoglobulins. He found 80 mg/dl IgG (normal is 600-1500 mg/dl), no igA, and only 10 mg/dl lgM (normal is 75-150 mg/dl). John was started on monthly intramuscular injections of gamma globulin; his serum IgG level was maintained at 200 mg/di. He started school at age 5 and performed well At 9 years of age he was referred to the Children's Hospital because of atelectasis (collapse of a portion of a lung) and a chronic cough. Upon physical examination, he was found to be alert and weli-developed. He weighed 33.5 Kg and was 146 cm in height (normal for his age). The doctor noted that he had no visible tonsils (he had never had a tonsillectomy). With a stethoscope, the doctor also heard rales (moist crackles) at the base of each lung Further family history revealed that John had a younger brother, 7-year old Ted who nad also contracted pneumonia on three occasions. Ted had a serum IgG lever of 150 mg/di. Laboratory studies at the Cleveland Clinic gave a white blood count ot 5100 cells/pl (normal) of which 45% were neutrophils (normal), 43% lymphocytes (normal), 1 1% monocytes (elevated) and 2% eosinophils (normal). Further examination by flow cytometry showed that 85% of John's lymphocytes had the T-cell protein CD3 (normal), but only 2% had the B-cell protein CD19 (normal is 12%) T-cell function, measured by proliferation in response to tetanus toxoid was normal, but IgG levels remained lo 155 mg/dl), with IgM and IgA not etected John was started on a preparation of gamma globulin (purified from pooled human serum donors) rendered suitable for intravenous administration. He was given a dose so as to maintain his IgG level at 600 mg/dl. He improved remarkably The rales at his tung base disappeared. He continued to perform well in school and ultimately entered dental school at OSU Except for occasionat bouts of conjunctivitis or sinusitis. which respond well to oral antibiotic treatment, he remains in good health and leads an active life: He became adept at inserting a needle into a vein on the back of his hand and he infuses himself with 10 g gamma giobulin every weekendExplanation / Answer
Answer 1 John is suffering from X-linked agammaglobulinemia because following symptoms have appeared:
Answer 2 John was well for first 10 months of his life because till then the antibodies recieved from mother in fetal life and later on from breast milk worked well for him.
Answer 3 Both these are encapsulated bacteria. These bacteria are more pathogenic as the capsule present protects them from the action of macrophages. This is also revealed in blood report that monocyte level is elevated which is actually in response to infection due to innate immunity.
These bacteria can be acted upon only by antibodies specific for capsule which in the present case could not be formed.
Answer 4 Factors to be considered for maintaining suitable antibody dosages is the dody weight of the patient
Answer 5 John would be at greater risk because his body cannot produce enough antibodies. Also Dengue requires complete antibody mediated immunity to fight with the infection. So, he will be more prone to mosquito borne infections than a normal person.
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