Cases reported "Neutropenia"

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1/22. microbiology of destructive periodontal disease in adolescent patients with congenital neutropenia. A report of 3 cases.

    BACKGROUND, AIMS: Congenital neutropenia is one condition that may predispose for destructive periodontal disease at a young age. In this report, we describe the microbiology of 3 adolescent patients with congenital neutropenia two of whom suffered from severe periodontitis. METHOD: Microbiological testing of the parents was also performed in 1 case. dna fingerprinting was used to study transmission of putative periodontal pathogens in this case. From 1 patient with periodontitis, actinobacillus actinomycetemcomitans and porphyromonas gingivalis were isolated; a 2nd periodontitis patient was infected with P. gingivalis. A 3rd patient had gingivitis only and no A. actinomycetemcomitans or P. gingivalis were found. RESULTS: Using the amplified fragment length polymorphism dna fingerprinting technique, bacterial transmission between the father and a patient was shown for A. actinomycetemcomitans but not for P. gingivalis.
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2/22. Chronic idiopathic neutropenia associated with gingival enlargement.

    A girl with chronic idiopathic neutropenia who developed gingival enlargement at seven years of age is presented. Intraoral examination revealed generalized gingival inflammation with a tendency to bleeding and inflammatory gingival enlargement localized to the anterior region. A considerable amount of bacterial plaque was noted on the teeth. There were also 4-5 mm pocket depths around the first molars. Radiographic examination also indicated the presence of incipient bone loss around the first molars in both jaws. The patient, who was diagnosed as localized prepubertal periodontitis with generalized gingival inflammation and anterior gingival enlargement, accentuates the importance of evaluation of periodontal status in patients with chronic idiopathic neutropenia, to avoid the destruction of supporting structures of the dentition.
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3/22. Infantile genetic agranulocytosis, morbus Kostmann: presentation of six cases from the original "Kostmann family" and a review.

    In 1956 Rolf Kostmann reported on six children with severe neutropenia associated with a block in myelopoiesis at the promyelocyte/myelocyte stage and an autosomal recessive inheritance. He named the new syndrome infantile genetic agranulocytosis. Today it is known as Kostmann's syndrome or severe congenital neutropenia. In 1975 an additional 10 cases from northern sweden were published. This article reports on the only long-term survivor from the 1975 report plus another five patients born after 1975 who belong to the original "Kostmann family". Treatment and survival have changed dramatically since Kostmann's first publication. In the pre-antibiotic era, Kostmann's syndrome was inevitably fatal during the first year of life. CONCLUSION: Since the introduction of recombinant human granulocyte colony-stimulating factor (G-CSF) about 10 y ago, most patients now enjoy a normal life span and a greatly improved quality of life. Although the threat of death has disappeared, patients still have problems with infections, especially chronic gingivitis and periodontitis. In other groups of severe neutropenia, not related to the original "Kostmann family", an increased incidence of myeloid leukaemia has been observed. However, in this small cohort none of the children on chronic G-CSF therapy have developed malignancies.
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4/22. oral manifestations of congenital neutropenia or Kostmann syndrome.

    Infantile congenital agranulocytosis or Kostmann syndrome is a rare hereditary kind of severe neutropenia. The typical symptoms, which appear since the first days of life, are abscesses located on various parts of the body: ear, cutis, lung and oral cavity. These abscesses are due to an almost total disimmunity typical of the neutropenia. The aim of this article is to describe the most typical signs of this pathology in the oral cavity, reporting a case observed in our department in Florence, italy. On the basis of the personally observed case and of the review of the literature, it is possible to consider, as a characteristic finding in Kostmann syndrome, a typical very serious periodontal pathology, which is similar to the prepubertal periodontitis in deciduous dentition. At the age of 19 years the patient showed a dramatic compromise of the masticatory function. It is obvious that the lack of response of the host can obstruct the interaction between the host and the microbic flora, because the lack of neutrophils increases the susceptibility of the patient to every kind of infection, even to periodontitis. A periodontal prophylaxis, since the very first observations, followed by a rigorous maintenance with frequent and regular professional hygienic treatments could be effective in controlling the effects of periodontal disease and could reduce the tragic evolution. We need to recognise that it could be hard to monitor the oral situation correctly in these patients, as they have a continuously poor systemic condition. Finally in these cases the rehabilitative therapy is very problematical.
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5/22. oral manifestations of cyclic neutropenia in a Japanese child: case report with a 5-year follow-up.

    Cyclic neutropenia is an uncommon hematologic disorder characterized by a marked decrease in the number of neutrophils in the peripheral blood occurring at regular intervals. The neutropenic phase is characteristically associated with clinical symptoms such as recurrent fever, malaise, headaches, anorexia, pharyngitis, ulcers of the oral mucous membrane, and gingival inflammation. This case report describes a Japanese girl who has this disease and suffers from periodontitis and oral ulceration. Her case has been followed up for the past 5 years from age 7 to 12. The importance of regular oral hygiene, careful removal of subgingival plaque and calculus, and periodic and thorough professional mechanical tooth cleaning was emphasized to arrest the progress of periodontal breakdown. Local antibiotic application with minocycline ointment in periodontal pockets was beneficial as an ancillary treatment, especially during neutropenic periods.
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6/22. Detection and quantification of herpesviruses in Kostmann syndrome periodontitis using real-time polymerase chain reaction: a case report.

    BACKGROUND/AIMS: Kostmann syndrome, or severe congenital neutropenia, is an autosomal recessive disease of neutrophil production and is associated with severe periodontal pathology. The aim of this study was to determine whether human cytomegalovirus (HCMV) and Epstein-Barr virus (EBV) contribute to the pathogenesis of Kostmann syndrome periodontitis. methods: Supragingival plaque and saliva samples were taken from a 6-year-old boy and his 3-year-old sister suffering from Kostmann syndrome, and from two age- and gender-matched healthy children serving as controls. The samples were taken before and 24 months after periodontal treatment. real-time polymerase chain reaction (TaqMan Real-Time PCR) assay was used to quantify HCMV and EBV DNA. RESULTS: EBV was detected in baseline samples from the Kostmann syndrome patients but not in samples from the healthy control subjects. HCMV was only detected in the saliva of the boy with Kostman syndrome at baseline. Herpesviruses numbers decreased dramatically in the post-treatment samples. CONCLUSION: EBV and HCMV were detected in the two subjects with Kostmann syndrome periodontitis. The results of the study indicate that nonsurgical treatment of Kostmann syndrome periodontitis can reduce supragingival and salivary herpes viral loads.
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7/22. Periodontal disease in patients from the original Kostmann family with severe congenital neutropenia.

    BACKGROUND: patients with Kostmann syndrome (severe congenital neutropenia [SCN]) typically normalize their absolute neutrophil count (ANC) upon granulocyte colony-stimulating factor (G-CSF) therapy. However, although they no longer experience life-threatening bacterial infections, they frequently still have recurrent gingivitis and even severe periodontitis, often starting in early childhood. methods: We studied the periodontal disease in the four surviving patients belonging to the family originally described by Kostmann. Their odontological records, x-rays, color photos, bacterial cultures, serum antibodies to oral bacteria, and histopathological examinations were reviewed. The data were also correlated to previous investigations on their antibacterial peptides and molecular biology. RESULTS: Three patients had periodontal disease, despite normal ANC and professional dental care, and had neutrophils deficient in antibacterial peptides. One of these patients also had a heterozygous mutation in the neutrophil elastase gene, had severe periodontal disease and overgrowth of the periodontal pathogen actinobacillus actinomycetemcomitans in the dental flora, and 15 permanent teeth had been extracted by the age of 27. One bone marrow-transplanted patient had no periodontal disease. CONCLUSIONS: Normalized ANC levels are not sufficient to maintain normal oral health in SCN patients, and because neutrophils are important for first-line defense and innate immunity, the deficiency of the antibacterial peptide LL-37 probably explains their chronic periodontal disease. Professional dental care is still important for SCN patients, despite treatment with G-CSF and normal ANC levels. Whether antibacterial peptides play a role in the pathogenesis of periodontitis in other patients remains to be elucidated.
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8/22. periodontitis as a manifestation of chronic benign neutropenia.

    BACKGROUND: A subcategory of chronic neutropenia is chronic benign neutropenia, which is characterized by a prolonged non-cyclic neutropenia as the sole abnormality, with no underlying disease to which the neutropenia can be attributed. Chronic neutropenia is defined as a low absolute neutrophil count for >6 months. In this presentation, periodontitis seems to be the sole manifestation of a juvenile patient with chronic benign neutropenia. A 7-year-old white male presented with periodontitis of the primary dentition and early tooth loss. His medical and dental history was otherwise unremarkable. Suspecting some systemic illness as the underlying cause, the patient was referred for a medical consultation and a series of blood tests. methods: Blood analyses included a complete blood count (CBC), sequential multiple analyzer 24 (SMA 24), glycated hemoglobin levels, and screening for anti-white blood cell antibodies. Blood levels of calcium, vitamin d, dihydroxyvitamin-D, phosphorus, and alkaline phosphatase were also measured. liver function tests were performed. RESULTS: Following analysis of recent and previous blood test results, a diagnosis of chronic benign neutropenia was assigned. The patient's periodontal condition was treated with scaling and root planing, oral hygiene instruction, and antimicrobial mouthrinses. Three-month recall visits were recommended as a follow-up protocol. CONCLUSIONS: This case represents the importance of diagnosing periodontal disease as a possible indicator of underlying systemic disease. When a patient presents with an unusual, generalized form of periodontal disease, screening for systemic disorders is required, as the oral condition may be the first or only manifestation of a systemic abnormality. This case also illustrates the reason for the change in classification of such a condition to periodontitis as a manifestation of systemic disease. This condition was previously classified as prepubertal periodontitis, a disease diagnosis that focused on the patient's age at the onset of the disease rather than the etiology.
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9/22. Capnophilic and anaerobic bacteremia in neutropenic patients: an oral source.

    The currently accepted empiric antibiotic therapy for bacterial infections in neutropenic patients may not cover the possibility of capnophilic and anaerobic bacteremia. Many of these infections develop in patients with severe mucositis or periodontitis, and the type of organisms recovered also suggests an oral source of infection. We present two cases of bacteremia in neutropenic patients who had been empirically treated with ceftazidime and piperacillin plus amikacin. In the first case a beta-lactamase-producing strain of capnocytophaga ochracea was isolated; in the second case bacteremia was due to a mixture of leptotrichia buccalis and fusobacterium nucleatum. These observations emphasize the necessity for a reevaluation of the possible use of antimicrobial agents active against beta-lactamase-producing capnophilic organisms and anaerobic bacteria during empiric therapy in neutropenic patients with an oral source of infection.
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10/22. A case report of chronic neutropenia: clinical and ultrastructural findings.

    This case report of severe periodontitis concerns a young male patient with chronic idiopathic neutropenia seen between 1981 and 1983 by the School of Dental medicine of Geneva and from 1984 to 1988 by the Eastman Dental Hospital of london. The patient was maintained by intermittent systemic antibiotics, subgingival chlorhexidine irrigation, conventional debridement, brushing, and wire-mesh and composite splinting of loose teeth. After leaving school, at the patient's express wish, the extreme downhill pattern of the periodontitis resulted in removal of affected teeth and overdenture construction. The extracted teeth and associated soft tissues were examined for relevant plaque and host response features. The intact morphotypes associated with the advancing front of the lesion were invariably Gram-negative or positive coccoid cells, or less commonly short rods, as in all documented forms of periodotitis. connective tissue destruction was associated with a leucocyte accumulation comprising mainly polymorphonuclear neutrophils (PMN) and plasma cells. Both were observed degenerated more deeply than in routine adult periodontitis, including PMN lysosome loss and lymphocyte maturation within peripheral blood vessels. Only in one instance, one part of the superficial connective tissue of one block contained bacteria. It was concluded that the features of plaque and the lesion suggest a typical first line of defence response as in other forms of periodontitis. From the consideration of the 10 years clinical history of the patient, it is clear that non-surgical management is possible even in extreme cases, without any compromise of the patient's oral or systemic health, and in accord with the patient's wishes.
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