In the current studies, a 9-yr-old boy with a history of recurrent infections and specific granule deficiency (absent lactoferrin, B-12 binding proteins, and characteristic specific granules on sucrose gradient centrifugation of cell homogenates) was [bloodjournal.org]

"Lactoferrin deficiency as a consequence of a lack of specific granules in neutrophils from a patient with recurrent infections. Detection by immunoperoxidase staining for lactoferrin and cytochemical electron microscopy". Am. J. [en.wikipedia.org]

Anatomic lesions, whether congenital or acquired, are often responsible for recurrent infections. The initial approach to an adult patient with recurrent infections is discussed here. [bcervantes-primarycare.blogspot.com]

Keywords: Antibody deficiency, Primary immunodeficiency, Recurrent infection, Severe combined immunodeficiency How to cite this article: AlKhater SA. Approach to the child with recurrent infections. [jfcmonline.com]

Most patients who have recurrent infections do not have an identifiable phagocyte defect or immunodeficiency. [pedsinreview.aappublications.org]

In contrast, serum IL-8 levels were markedly elevated in the SGD individual compared with those of non-SGD individuals in sepsis. [ncbi.nlm.nih.gov]

Patients may also develop sepsis, mastoiditis, otitis media, and lymphadenopathy. [en.wikipedia.org]

[…] in Patients Who Have No Primary Immunodeficiency Syndromes In general, evaluations should be initiated for those who have had at least one of the following clinical features within a 1-year period: 1) more than two systemic bacterial infections (eg, sepsis [pedsinreview.aappublications.org]

Bacteremia and sepsis — Patients with deficiency or dysfunction of mannose binding lectin, a component of the innate immune system that is involved in complement activation, may be at higher risk for bacteremia and sepsis [58,59]. [bcervantes-primarycare.blogspot.com]

Abstract Neutrophil-specific granule deficiency (SGD) is a rare, congenital disease characterized by atypical neutrophil structure and function, resulting in recurrent bacterial infections from early infancy. [ncbi.nlm.nih.gov]

bacterial infections from early infancy [ 1, 2 ]. [link.springer.com]

[…] actin Neutrophilia, recurrent bacterial infection without pus 45. [slideshare.net]

Phagocytic cell deficiencies Most of the defects of phagocytic cells present with recurrent bacterial infections. [theasthmacenter.org]

bacterial infections; hemolytic anemia Reduced levels of glucose-6-phosphate dehydrogenase Clinical testing available* Antibiotics Myeloperoxidase deficiency AR Mild, if any, susceptibility to infection Reduced levels of myeloperoxidase Generally none [what-when-how.com]

Patients may also develop sepsis, mastoiditis, otitis media, and lymphadenopathy. [en.wikipedia.org]

Sinus Histiocytosis with Massive Lymphadenopathy Sinus histiocytosis with massive lymphadenopathy, or Ro-sai-Dorfman disease, is characterized by chronic, painless, massive lymphadenopathy that usually involves the cervical nodes and less frequently involves [what-when-how.com]

Lymph node biopsy For some patients with lymphadenopathy, to determine whether germinal centers are normal and to exclude cancer and infection Interpretation varies by histology. [merckmanuals.com]

[…] dinucleotide phosphate ( NADPH ) oxidase ( X-linked or autosomal recessive inheritance (2:1) ) Clinical features Recurrent, severe infections (chronic skin, lymph node, bone, respiratory, GI, and urinary tract) by catalase-positive organisms Anemia and lymphadenopathy [amboss.com]

General manifestations Hepatospleenomegaly and generalized lymphadenopathy. Chronic diarrhea. Lymphoma may develop in some cases. Eosinophilia and lymphocytosis. [drmhijazy.com]

This book is a comprehensive yet manageable resource for physicians and nurses wishing to learn more about PID, as well as a useful tool for both doctors-in-training and specialists in clinical decision-making and treatment planning. [books.google.com]

C 3 Physicians should suspect a primary immunodeficiency disease in children who have unusually severe and recurrent infections with common pathogens, or infections with unusual pathogens. [aafp.org]

Similarities in the clinical presentation of neutrophil, antibody, and complement disorders can prove difficult for the physician attempting to establish a diagnosis. [pedsinreview.aappublications.org]

Therefore, physicians should be aware of recurrent infections caused by non-immunologic conditions (Table 1) and clinical clues suggestive of immunologic disorders (Table 2). [hawaii.edu]

Many physicians suggest that swimming should be confined to well-chlorinated pools. Brackish water in particular may expose patients to organisms that are specifically dangerous in CGD ( Francisella philomiragia, Chromobacterium violaceum ). [primaryimmune.org]

Most patients with an Ig or a complement deficiency have a good prognosis with a near-normal life expectancy if they are diagnosed early, are treated appropriately, and have no coexisting chronic disorders (eg, pulmonary disorders such as bronchiectasis [merckmanuals.com]

Infants may present with vomiting, diarrhea, and failure to thrive. [2] Diagnosis can be made based upon CEBPE gene mutation or a pathognomonic finding of a blood smear showing lack of specific granules. [en.wikipedia.org]

Diarrhea, failure to thrive, opportunistic infections, and severe routine infections in a child younger than three months should raise suspicion for SCID. [aafp.org]

Possible poor growth or failure to thrive. T-Cell and Combined Immunodeficiency:. .. .. Presentation in early infancy.. .. .. Poor growth or failure to thrive.. .. .. Persistent oral thrush.. .. .. Opportunistic infection. Phagocytic Defects:. .. .. [hawaii.edu]

[…] to thrive Diagnosis IgG, IgA, IgE Normal or IgM Treatment Ataxia telangiectasia See ataxia telangiectasia. [amboss.com]

A combination of erythroderma of early onset with failure to thrive is highly suggestive of immunodeficiency .It is a rather non-specific feature of immunodeficiency in infancy. Eczema. [drmhijazy.com]

New Third Edition chapters cover bioterrorism, hospital infections, emerging infections, Kawasaki syndrome, transmissible spongiform encephalopathies, diabetic foot ulcers, decubitus ulcers, staphylococcal and streptococcal toxic shock syndrome, food [books.google.de]

Cutaneous ulcers or abscesses and pneumonia and chronic lung disease are common. Patients may also develop sepsis, mastoiditis, otitis media, and lymphadenopathy. [en.wikipedia.org]

Nodular lesions and necrotic ulcers. Subcutaneous nodules may develop at immunization sites, and these also tend in time to ulcerate. [drmhijazy.com]

[…] infections caused by unusual pathogens (eg, Aspergillus pneumonia, disseminated candidiasis, infection with Serratia marcescens, Nocardia sp, Burkholderia cepacia) ; 6) infections of unusual severity; and 7) chronic gingivitis and recurring aphthous ulcers [pedsinreview.aappublications.org]

Other signs include skin lesions (eg, eczema, warts, abscesses, pyoderma, alopecia), oral or esophageal thrush, oral ulcers, and periodontitis. [merckmanuals.com]

Viral infections manifesting with skin ulcerations particularly in the perineal area, the oral mucosa or the tongue. Severe varicella-zoster infections may also occur. [drmhijazy.com]

Chronic skin ulcers without pus can form and there is delayed wound healing. [jcp.bmj.com]

New Third Edition chapters cover bioterrorism, hospital infections, emerging infections, Kawasaki syndrome, transmissible spongiform encephalopathies, diabetic foot ulcers, decubitus ulcers, staphylococcal and streptococcal toxic shock syndrome, food [books.google.de]

These phenotypic and functional alterations of PB monocytes in the SGD individual suggest that C/EBPepsilon plays a critical role in monocyte/macrophage development of humans and is consistent with observations in the murine system. [ncbi.nlm.nih.gov]

Abstract It has been suggested that neutrophil (PMN) specific granules are important in cell aggregation, locomotion, hydroxyl radical formation, and in extracellular functions such as the generation of complement- related inflammatory mediators (C5a) [bloodjournal.org]

[…] majority of patients with SGD have been found to have mutations in the CEBPE (CCAAT/enhancer-binding protein epsilon) gene, a transcription factor primarily active in myeloid cells. [3] Almost all patients have been found to be homozygous for the mutation, suggesting [en.wikipedia.org]

[…] of NF–kappa B regulation, and abnormalities in IL-2RG suggest SCID. [merckmanuals.com]

The presence of family members with a similar disease, recurrent infections, unexplained death, malignancy, or autoimmune disease suggests the possibility of a genetic illness. [jfcmonline.com]

New Third Edition chapters cover bioterrorism, hospital infections, emerging infections, Kawasaki syndrome, transmissible spongiform encephalopathies, diabetic foot ulcers, decubitus ulcers, staphylococcal and streptococcal toxic shock syndrome, food [books.google.de]

Eosinophilic fasciitis, pneumonitis, and myocarditis; neuropathy culminating in respiratory failure; and encephalopathy may occur. The disease is caused by ingesting contaminants in L-tryptophan–containing products. [benhhoc.com]

Neutrophils and eosinophils will contain hyposegmented nuclei (a pseudo- Pelger–Huet anomaly ). [en.wikipedia.org]

(b) Pelger-Huet anomaly with a bilobed nucleus. (c) Alder-ReiHy anomaly with prominent purple granules (also in monocytes and lymphocytes). (d) May-Hegglin anomaly with Döhle bodies in the cytoplasm. (e) Toxic granulation. [europeanmedical.info]

Acquired bilobed nuclei, pseudo Pelger-Huet anomaly, can occur with acute infections or in myelodysplastic syndromes. [benhhoc.com]

Symptoms [ edit ] Atypical infections are the key clinical manifestation of SGD. [1] Within the first few years of life, patients will experience repeated pyogenic infections by species such as Staphylococcus aureus, Pseudomonas aeruginosa or other Enterobacteriaceae [en.wikipedia.org]

Staphylococcus aureus causes most liver abscesses in patients with CGD. Often the liver abscesses are hard to drain and may need surgery. [primaryimmune.org]

These are usually caused by staphylococcus aureus and less common by Gram-negative organisms such as Escherichia coli and pseudomonas species. [drmhijazy.com]

Hepatic involvement by Staphylococcus aureus and Pseudomonas cepacia can manifest as granuloma or abscess formation [ 113 ]. [clinicalmolecularallergy.biomedcentral.com]

This edition provides complete coverage of newer viruses such as human herpesvirus 6 and 8, West Nile virus, hepatitis C, D, and E, hantaviruses, caliciviruses, and astroviruses, as well as recently discovered microorganisms such as Escherichia coli 0157 [books.google.de]

Human herpesvirus 6 (HHV-6) disease in the setting of transplantation. Curr Opin Infect Dis. 2012 Aug. 25(4):438-44. [Medline]. Özkan H, Köksal N, Çetinkaya M, Kiliç S, Çelebi S, Oral B, et al. [emedicine.medscape.com]

pylori, Chlamydia pneumoniae, Bartonella, and microsporidia. [books.google.de]

Pylori Infection Hematopoietic Stem Cell Transplantation Hemochromatosis, Neonatal Hemorrhagic Fever With Renal Failure Syndrome Hemosiderosis Hepatitis A Hepatitis B Hepatitis C Hepatoblastoma Hepatocellular Carcinoma Hepatorenal Syndrome Herpes Simplex [emedicine.medscape.com]

[…] complete coverage of newer viruses such as human herpesvirus 6 and 8, West Nile virus, hepatitis C, D, and E, hantaviruses, caliciviruses, and astroviruses, as well as recently discovered microorganisms such as Escherichia coli 0157, Helicobacter pylori, Chlamydia [books.google.de]

Variants in toll-like receptor 1 and 4 genes are associated with Chlamydia trachomatis among women with pelvic inflammatory disease. J Infect Dis. 2012 Feb 15. 205(4):603-9. [Medline]. [Full Text]. [emedicine.medscape.com]