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• W2021746629 abstract "A 7.5-month-old boy presented to our emergency department with shortness of breath. He was the product of an in vitro fertilization pregnancy born to unrelated parents. Antenatal ultrasound did not reveal any abnormalities except mild hydronephrosis. He was delivered at 30 weeks of gestation by cesarean section because of prolonged preterm rupture of membranes. He was intubated on the first day of life because of respiratory distress syndrome, was ventilated for 3 days in the neonatal intensive care unit and required supplemental oxygen for a total of 6 days. At 2 weeks of age he had apnea without other signs of sepsis. A blood culture grew Candida spp. He was treated with amphotericin B with no further complications and was discharged after 2 months, in good general condition without severe complications of prematurity. At home, he continued to develop and grow well. At age 4 months, he was seen by a pediatric pulmonologist because of mild respiratory distress and wheezes on clinical examination and received budesonide and albuterol nebulizers, with rapid improvement. At age 6 months, he presented to our emergency room with a 2-week history of worsening tachypnea, without fever or other symptoms. He was unresponsive to nebulizer therapy. On examination he was alert, afebrile, tachypneic at 50 breaths/min and dyspneic, with oxygen saturations of 88% in room air and bilateral inspiratory crepitations without wheezes. Heart sounds were normal; the abdomen was soft and nontender without organomegaly. The rest of the physical examination was normal. Computerized white blood count (WBC) was 42,800/mm3 with 28% eosinophils, 58% lymphocytes, 7.5% neutrophils, 5.8% monocytes and 0.4% basophils. Hemoglobin was 14.6 g/dL, and platelet count was 390,000/mm3. Total protein was 6.2 g/dL, and albumin 4.0 g/dL. The rest of the chemistry panel was unremarkable. The initial chest radiograph showed bilateral patchy infiltrates. He was given a presumptive diagnosis of eosinophilic pneumonia and was treated with 2 mg/kg iv methylprednisolone in addition to clarithromycin. After 2 days of treatment, he improved clinically, oxygen saturations returned to normal and his total WBC decreased to 26,900/mm3 with only 1% eosinophils. Other investigations revealed no evidence of parasites in the stool, and he had negative serology for Chlamydia, Mycoplasma and Legionella. The sweat chloride test was normal. Immunoglobulins showed IgG 39 mg/dL; IgM 27 mg/dL; IgA < 0.1 mg/dL and IgE < 4.5 IU/mL. Repeat immunoglobulins showed persistent low levels: IgG 26 mg/dL; IgM 35 mg/dL; and IgA < 5.5 mg/dL. Other immunologic investigations were unremarkable; total lymphocyte count was 11,520/mm3, of which 4147 were B cells (CD19+) and 7027 were T cells (CD3+). The distribution among B cells showed CD19/CD5+ 2304/mm3, and that among T cells showed CD4+ 5379/mm3 and CD8+ 1486/mm3; with natural killer cells 207/mm3 (CD16/CD56+). The total number of κ cells was 2102, and the total number of λ cells was 1853/mm3. Response to mitogens, including phytohemagglutinin and pokeweed antigens, was normal. After 9 days of hospitalization, he was discharged, but 4 days later he returned with cellulitis in his middle finger that developed into an abscess despite treatment with oral antibiotics. He received intravenous immunoglobulins (IVIG) 400 mg/kg, intravenous cefazolin and gentamicin. Three weeks later, his WBC was 38,200/mm3 with 18% eosinophils. Repeat immunoglobulins showed IgG 754 mg/dL; IgM 35 mg/dL and IgA < 5.5 mg/dL. Clinical examination revealed mild tachypnea, oxygen saturation of 92% in room air but no additional findings on auscultation. Chest radiograph showed bilateral patchy infiltrates. Elective bronchoscopy was performed. After the procedure, he became more tachypneic and hypoxic and was admitted and treated with intravenous ampicillin-sulbactam and supplemental oxygen. He was also given a second dose of IVIG. The routine bacterial culture from the bronchoalveolar lavage (BAL) showed Haemophilus influenzae susceptible to ampicillin-sulbactam. It was negative for acid-fast bacilli and fungi. Polymerase chain reaction for cytomegalovirus and silver stain and immunofluorescence for Pneumocystis jiroveci were negative. There was no increase in eosinophils in BAL fluid. Despite antibiotic treatment, the child's respiratory status deteriorated, and on day 6 he was intubated. He received a third dose of IVIG, and a second BAL was performed which was also negative for all of the aforementioned pathogens. Another procedure revealed the diagnosis. For denouement, see p. 853. Denouement Continued from p. 848 An open lung biopsy was performed because of continuing clinical deterioration. Pathology revealed strongly positive silver stain for Pneumocystis jiroveci. Treatment with steroids and trimethoprim-sulfamethoxazole was started, and his condition improved. Hewas extubated after 9 days and discharged home after 3 weeks of treatment. To further investigate the hypogammaglobulinemia and the presence of P. jiroveci infection, despite the low normal level of IgM, a diagnosis of hyper-IgM syndrome was suspected. A sample of heparinized blood was stimulated with phorbol myristate acetate and calcium ionophore.1 Despite stimulation and activation of CD4 T cells as measured by CD69, the patient's activated T-cells did not express CD40L. Three normal controls showed up-regulation of both CD69 and CD40L. The diagnosis of hyper-IgM syndrome was established, and the patient is currently awaiting bone marrow transplantation. Current therapy includes IVIG and continued trimethoprim-sulfamethoxazole. The hyper-IgM syndrome is a group of B cell defects characterized by an inability to perform class switch from IgM to IgG, IgE and IgA.2 There is also an inability to activate T cells, Kupffer cells and pulmonary macrophages, leading to an increased incidence of P. jiroveci, bacterial sinopulmonary and opportunistic and disseminated viral infections.2 Seventy-five percent of patients succumb to sclerosing cholangitis secondary to a chronic Cryptosporidium parvum enteritis.2 This, not uncommonly, leads to cirrhosis and/or malignancy.3,4 The disease is typified by low IgG, IgA and IgE with high IgM; however, as in our case, IgM levels are not high in 30% of cases.4 Approximately two-thirds of patients have neutropenia, but >90% have normal T cell and B cell quantity and function.4 There is an association with autoimmune disease, butthe clinical relevance of this is uncertain.3,4 Common characteristic infectious complications include persistent C. parvum enteritis, bacterial sinopulmonary infections and P. jiroveci pneumonia. Enteroviral meningoencephalitis and disseminated cytomegalovirus are rarer complications.5,6 Previously treatment relied on prophylactic antibacterials (trimethoprim-sulfamethoxazole) and IVIG, but since 1993 bone marrow transplantation has become recognized as a potentially curative procedure.3 A major obstacle is reactivation of previously quiescent cryptosporidial disease causing worsening liver function postoperatively, and there is no highly effective treatment of this protozoan. In a recent article,3 a 50% survival rate at 1 year post-bone marrow transplant was reported, and risk factors for poor outcome included older age at the time of transplant, chronic lung disease (bronchiectasis), abnormal liver histology and previous cryptosporidial infection. Recently reports of increased expression of CD40 and CD40L in autoimmune diseases including systemic lupus erythematosus and rheumatoid arthritis has been published.7 There has been some success in animal models using anti-CD40 ligand monoclonal antibodies, but this has not been the case with the few trials in humans. The importance of P. jiroveci pneumonia in immunocompromised patients is well-known, and a low threshold for investigation is warranted. This case report stresses the importance of considering the diagnosis of hyper-IgM syndrome in patients with P. jiroveci pneumonia and panhypogammaglobulinemia, even with low concentrations of IgM. Furthermore in cases where P. jiroveci is suspected but not demonstrated on BAL, a lung biopsy should be considered.8" @default.
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• W2021746629 title "Eosinophilia, Pneumonia and Hypogammaglobulinemia" @default.
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