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1.
C. Vermylen J. M. Scheiff J. Rodhain J. Ninane G. Cornu 《European journal of pediatrics》1986,145(3):232-235
Typical features of congenital dyserythropoietic anaemia (CDA) were found in a 13-year-old girl admitted for chronic recurrent multifocal osteomyelitis. The findings on light microscopy were in agreement with those described in CDA type II, whereas on electron microscopy, the ultrastructure findings were compatible with both types I and II. The acidified serum lysis test (Ham test) performed with eight normal sera was negative. The patient's red blood cells showed an increased agglutinability with anti-i antibodies. Morphological changes were also shown in the mature neutrophilic granulocyte suggesting that the primary disorder exists already in the multipotent stem cell.Abbreviations CDA
congenital dyserythropoietic anaemia
- BFU-E
erythroid burst-forming units
- CFU-E
erythroid colony-forming units
- CFU-GM
granulomonocytic colony-forming units
- CSA
colony-stimulating activity 相似文献
2.
Koturoğlu G Vardar F Ozkinay F Kurugöl Z Akalin T Ozkinay C 《The Turkish journal of pediatrics》2006,48(2):159-161
Pyoderma gangrenosum (PG) is an uncommon, chronic ulcerative condition of the skin that was first described in 1930. It can occur in any age group, but only 4% of the patients are infants or children. An underlying systemic disease is present in approximately 50% of the patients with PG. The most common associations include inflammatory bowel disease, arthritis, lymphoproliferative disorders and chronic recurrent multifocal osteomyelitis (CRMO). PG has been reported in association with CRMO in only a few children whose ages were between 18 months and 12 years. We report a six-month-old boy who was diagnosed as CRMO based on his clinical examination and histological findings. This is the youngest case reported in the literature (under 12 months of age) with PG associated with CRMO. 相似文献
3.
We describe the case of an 18-year-old girl with chronic recurrent multifocal osteomyelitis (CRMO) over a period of 10 years. She had suffered predominantly from very painful recurrent swelling of her cheeks. Various therapeutic regimens including nonsteroidal antiinflammatory drugs and steroids had shown only a partial or temporary response. Because tumor necrosis factor-alpha-blocking agents have been successfully applied in Crohn's-associated CRMO and the related SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome, tumor necrosis factor-alpha-blocking therapy with infliximab was initiated. Thereafter, apart from 1 mild episode, no additional recurrences were observed during 21 months of follow-up. Infliximab was well tolerated, and steroids were tapered off. Our observation indicates that infliximab may be an effective therapeutic option in CRMO. 相似文献
4.
M. Le Merrer R. Girot P. Parent V. Cormier-Daire P. Maroteaux 《European journal of pediatrics》1995,154(5):384-388
The term congenital dyserythropoietic anaemia (CDA) designates a group of rare but well defined erythrocytic disorders. Type I is defined by macrocytosis and megaloblastic changes of the bone marrow cells. Two unrelated children with CDA are described with associated defects: absence phalanges, polysyndactyly of the fourth metacarpal. One of them had also areas of depigmentation.Conclusion The association of congential dyserythropoietic anaemia with morphological defects of hands and feet is suggested to constitute a new syndrome caused by a single morphogenetic gene. 相似文献
5.
W. Handrick D. Hörmann A. Voppmann R. Schille P. Reichardt R. B. Tröbs R. P. Möritz M. Borte 《Pediatric surgery international》1998,14(3):195-198
The authors undertook a retrospective review of the clinical records and radiologic findings of eight children with chronic,
recurrent multifocal osteomyelitis (CRMO). This disease is a recognized clinicopathologic entity with typical radiographic
findings, mostly in the metaphyses of the long bones. The diagnosis is one of exclusion without pathognomonic findings. The
patients were treated with non-steroidal anti-inflammatory drugs. There was no evidence of altered bone growth or abnormal
joint development. It is concluded that CRMO is a distinct clinical entity that is different from acute or subacute bacterial
osteomyelitis. Recognition of this condition is important to avoid treatment with antibiotics and repeated operations.
Accepted: 8 April 1998 相似文献
6.
Paivi MH Miettunen Xingchang Wei Deepak Kaura Walid Abou Reslan Alberto Nettel Aguirre James D Kellner 《Pediatric rheumatology online journal》2009,7(1):2-14
Background
Chronic recurrent multifocal osteomyelitis (CRMO) is an inflammatory, non-infectious osteopathy that affects predominantly patients ≤ 18 years of age. There is no uniformly effective treatment. Our objective is to describe clinical, magnetic resonance imaging (MRI), and bone resorption response to intravenous pamidronate in pediatric CRMO. 相似文献7.
A. Jansson A. Golla K. Schneider V. Jansson B. H. Belohradsky 《Monatsschrift für Kinderheilkunde》2002,150(4):477-489
Chronic recurrent multifocal osteomyelitis (CRMO) is a nonbacterial osteomyelitis and most commonly occurs during childhood. Associations with palmoplantar pustulosis (PPP) have been reported in about 20%. Since the etiology of the disease is unknown it is suspected to be an autoimmune osteomyelitis. Own studies [27] demonstrate evidence for a genetic basis of CRMO for the first time. The clinical features, diagnostic procedures and therapeutic options are demonstrated. 相似文献
8.
J-F Lavis S Gigon I Gueit C Michot A Tardif E Mallet J-M Péron 《Archives de pédiatrie》2002,9(12):1252-1255
Chronic recurrent multifocal osteomyelitis (CRMO) is a disorder rarely localized to the lower jaw. CASE REPORT: A fourteen-year-old boy complained of a swollen of his lower jaw. After a CT Scan, a bone biopsy was performed and yielded S. oralis against which an adapted intravenous antibiotherapy was administered without efficacy. The absence of malignant process and the revelation of an other focus of fixation at the Tc bone scan localized on humerus called to mind the diagnosis of CRMO. CONCLUSION: The diagnosis of this disease is difficult and based on a number of concording arguments:clinical and radiological signs of osteomyelitis, multifocal presentation, recurrent relapses and remissions, inaction of antibiotics, elimination of the other differential diagnosis, in particular the infectious osteitis. 相似文献
9.
Chronic recurrent multifocal osteomyelitis (CRMO) is a rare disorder of unknown etiology. The main characteristics on plain X-rays are a lytic destruction in the metaphyseal region of the long bones followed by progressive sclerosis. The symmetrical pattern and the frequent involvement of the sternoclavicular joints and vertebrae are typical. An association with palmoplantar pustulosis has also been described. Laboratory findings are nonspecific. Although MRI is not necessary to make the diagnosis of chronic osteomyelitis, it is useful in assessing the extent and the evolution of the lesions. CRMO of the tibial and fibular bones is described in a 14-year-old girl, who suffered from pain and soft tissue swelling in both ankles. Initial plain X-rays and laboratory findings were normal. After relapsing clinically, progressive sclerosis of both fibular bones occurred. Lytic lesions in the left tibia and both fibular bones were visible. Scintigraphic examination showed pathologic tracer accumulation in both legs. The diagnosis of CRMO was suggested based on CT and MRI findings. CRMO was confirmed after curettage and bone biopsy. Histopathological findings were consistent with active chronic osteomyelitis. 相似文献
10.
Maus U Ihme N Schroeder S Andereya S Ohnsorge JA Hermanns B Deutz P Niedhart C 《Klinische P?diatrie》2007,219(5):277-281
BACKGROUND: Idiopathic tumorous calcinosis is a rare benign disease of the periarticular tissue near large joints. Deposits of hydroxyapatite in single or multiple pseudocysts lead to consecutive pain or complaints by attaching the surrounding tissues. The etiology of this disease is not definitively clear. CASE REPORT: We describe the case of an 11-year-old turkish girl with a well known chronic recurrent multifocal osteomyelitis (CRMO) and hyperphosphataemia. Furthermore, she developed a tumorous calcinosis around the left hip, which recurred after surgery, and around the ankle joint. CONCLUSIONS: CRMO and tumorous calcinosis can be associated diseases. The development of tumorous calcinosis in patients with CRMO and hyperphosphataemia should be excluded. 相似文献
11.
Chronic recurrent multifocal osteomyelitis: clinical outcomes after more than five years of follow-up 总被引:3,自引:0,他引:3
Huber AM Lam PY Duffy CM Yeung RS Ditchfield M Laxer D Cole WG Kerr Graham H Allen RC Laxer RM 《The Journal of pediatrics》2002,141(2):198-203
OBJECTIVE: To determine the clinical outcomes of children with chronic recurrent multifocal osteomyelitis (CRMO). STUDY DESIGN: Inception cohorts of children with CRMO were established at two tertiary pediatric centers. Outcome data were obtained through review of hospital charts, interview and examination of patients, and completion of questionnaires by patients. RESULTS: Of 45 eligible subjects, 23 (51%) were assessed. Median time since diagnosis was 13 years (range, 6-25). At evaluation, 6 (26%) had active disease; 18 (78%) had Health Assessment Questionnaire scores of 0 (no/minimal physical disability), and 5 had scores >0. Some impairment was seen in all domains of measurement of quality-of-life test, especially those concerning nonphysical aspects of health. Six (26%) subjects continued to have pain as a result of CRMO. Associated medical problems included arthritis in 6, sacroiliitis in 3, psoriasis in 5, recurrent pustular rashes in 2, and inflammatory bowel disease in 3. CONCLUSIONS: Long-term clinical outcomes for children with CRMO appear to be generally good, with most subjects having no evidence of disease activity or sequelae. However, a number of subjects had persistent disease and, therefore, remain at risk of physical and psychologic complications. Further research is required to identify patients at risk for persistent disease, and to determine therapies that may prevent morbidity. 相似文献
12.
Two cases of acrodysostosis syndrome in a brother and sister are reported. The parents and other siblings were unaffected. Features of this syndrome include extremely short, broad metacarpals, metatarsals, and phalanges, nasal hypoplasia, short stature, and mental deficiency. Previously published familial cases of acrodysostosis syndrome are consistent with autosomal dominant transmission, whereas the cases reported herein suggest other genetic mechanisms including recessive autosomal transmission, unstable mutation, and germ cell mosaicism, the latter being the most attractive hypothesis. 相似文献
13.
A Poujol S Toesca J N Di Marco Y Rimet H Tissot Dupont L Macé N Sudan M Panuel Y Turcat F Kelberine Y Brusquet 《Archives de pédiatrie》1998,5(3):291-294
Chronic recurrent multifocal osteomyelitis (CRMO) is a disorder of suspected--but unproved-infectious etiology. OBSERVATION: A girl presented with a typical CRMO involving successively the left fibula, radius, humerus and the right carpus. A Coxiella burnetii infection was indicated during the first attack. Two recurrences occurred in spite of suitable antibiotic treatment and with negative infectious investigation. Two months after stopping antibiotic treatment, a new recurrence associated with antibodies increase and positive bone culture occurred. CONCLUSION: Coxiella burnetii can initiate a CRMO. The mechanism involved is probably a delayed hypersensitivity. CRMO would therefore be the first type of reactive osteitis. 相似文献
14.
H. J. Girschick R. Krauspe A. Tschammler H. I. Huppertz 《European journal of pediatrics》1997,157(1):28-33
Chronic recurrent, uni- or multifocal osteomyelitis (CRMO), an inflammatory disorder of unknown origin, involves mk:/night/arul/4310946m.3dultiple
osseous sites and may affect the clavicle. We report on 6 children with clavicular involvement out of 11 children suffering
from CRMO. The major clinical symptoms were local swelling and pain. Five children had hyperostosis of the clavicle and synovitis
of adjacent joints. Histology showed chronic osteomyelitis with a predominance of lymphocytes in the inflammatory infiltrates.
Cultures of biopsy tissue specimens were sterile. The patients were followed for at least 3.5 years. Three patients had up
to six relapses. The most effective diagnostic tools to define CRMO were standard X-ray and bone scan in combination with
biopsy and cultures. In our patients CT and MRI were misleading as they suggested the presence of malignancy. However, the
sensitivity of MRI to detect involvement of bone, adjacent joints and soft tissues were better in comparison to X-ray or bone
scan. Non-steroidal anti-inflammatory drugs were effective in reducing pain, swelling and limitation of motion. Reconstructive
surgery was not indicated in any case. The long-term outcome of growth and function of affected bones was excellent.
Conclusion Diagnosis of chronic osteomyelitis of the clavicle should be made by history and physical examination and be confirmed by
standard X-ray, bone scan and open biopsy. In contrast MRI and CT can provide data on the involvement of adjacent joints,
soft tissue and muscles especially in the early process of disease, but do not add information relevant to the patient's management.
Treatment with non-steroidal anti-inflammatory drugs is rapidly beneficial in most patients.
Received: 24 January 1997 / Accepted: 26 May 1997 相似文献
15.
J. -F. Lavis S. Gigon I. Gueit C. Michot A. Tardif E. Mallet J. -M. Pron 《Archives de pédiatrie》2002,9(12):690
Chronic recurrent multifocal osteomyelitis (CRMO) is a disorder rarely localized to the lower jaw.Case report. – A fourteen-year-old boy complained of a swollen of his lower jaw. After a CT Scan, a bone biopsy was performed and yielded S. oralis against which an adapted intravenous antibiotherapy was administered without efficacy. The absence of malignant process and the revelation of an other focus of fixation at the Tc bone scan localized on humerus called to mind the diagnosis of CRMO.Conclusion. – The diagnosis of this disease is difficult and based on a number of concording arguments:clinical and radiological signs of osteomyelitis, multifocal presentation, recurrent relapses and remissions, inaction of antibiotics, elimination of the other differential diagnosis, in particular the infectious osteitis. 相似文献
16.
Chronic recurrent multifocal osteomyelitis is a recently recognized disease characterized by remissions and exacerbations of multiple bone lesions which radiographically and pathologically have the appearance of hematogenous osteomyelitis. No consistent etiology can be identified, and antimicrobial agents seem to have no beneficial effect. A review of the appearances of imaging modalities and the clinical and pathologic manifestations is undertaken in seven cases of CRMO. No therapeutic regimen resulted in consistent clinical or radiographic improvement. We suggest the sequelae of this process are not as benign as previously reported. 相似文献
17.
Chronic recurrent multifocal osteomyelitis (CRMO) primarily affects children and adolescents, and is characterized by episodic sterile osteomyelitis over several years. No definitive treatment is available. Non‐steroidal anti‐inflammatory drugs (NSAID) are common first‐line agents, but provide limited improvement in bone pain and do not affect disease duration. Several agents are utilized in the case of non‐response to NSAID, including corticosteroids, methotrexate, and tumor necrosis factor‐blocking agents. Bisphosphonates are increasingly being used. Most case series involve cyclic i.v. pamidronate, but this restricts the social lives of children and their families. Although oral medication has advantages over cyclic i.v. infusion because it does not require repeated hospital admissions, there have been no reports on treatment with oral bisphosphonates, such as alendronate, in pediatric CRMO patients. This case report describes the use of oral bisphosphonate as an alternative treatment in CRMO patients in whom standard therapy has failed. 相似文献
18.
R. C. M. Hennekam A. G. van de Meeberg J. M. van Doorne P. F. Dijkstra J. B. Bijlsma 《European journal of pediatrics》1988,147(5):539-543
A brother and sister with Martsolf syndrome are reported. The main characteristics of the syndrome are mental retardation, short stature, cataracts, hypogonadism and craniofacial anomalies including microcephaly, maxillary retrusion, pouting mouth, malaligned teeth and mildly dysplastic pinnae. The metacarpal and phalangeal bones are short. The occurrence of Martsolf syndrome in sibs of opposite sex suggests autosomal recessive inheritance.Abbreviations MCPPP
metacarpal phalangeal pattern profile
- FSH
follicle stimulating hormone
- LH
futeinizing hormone
- TORCH
toxoplasmosis, rubella, cytomegaly and herpes 相似文献
19.
Toni Hospach Micha Langendoerfer Tekla von Kalle Jan Maier Guenther E. Dannecker 《European journal of pediatrics》2010,169(9):1105-1111
There are only a few studies that address the frequency and type of spinal involvement in patients with chronic recurrent
multifocal osteomyelitis (CRMO) as well as the outcome of these patients treated with pamidronate (PAM). We performed a retrospective
study on patients with CRMO and analyzed clinical and pain assessments as well as regional and whole body MRI findings and
compared with posttreatment findings. Of 102 children and adolescents with CRMO, 27 (26%) had involvement of the spine. Vertebral
deformities were seen in 14 of these 27 patients, scoliosis or kyphosis in 6. After routine whole body MRI, 19 complained
of back pain, whereas eight were asymptomatic with spinal lesions detected incidentally. A total of 72 spinal lesions were
detected, thoracic vertebrae being the most commonly affected. Seven patients were treated with PAM; all of whom had vertebral
deformities and ongoing back pain. Pain resolution was achieved within 3 months of PAM treatment in every case. One patient
subsequently developed a pain amplification syndrome. Repeat MRI performed at a mean interval of 13 months revealed partial
or complete resolution of vertebral hyperintensities in every patient. Improvement of vertebral height was seen in a total
of three vertebrae in two patients. Severe side effects were not observed. In conclusion, we demonstrated that spinal involvement
and associated vertebral deformities with or without kyphoscoliosis are not rare in CRMO, and PAM appears to be an effective
and safe treatment for this condition. Although controlled studies are urgently needed, the use of PAM for refractory CRMO
with extended spinal involvement (vertebral deformities, kyphosis, and scoliosis) should be considered, especially after failing
of conventional therapy. 相似文献
20.
Guillén Martín S Belda Hofheinz S Rojo Conejo P Losada Pinedo B Ramos Amador JT Clemente Pollan J Ruiz Contreras J 《Anales de pediatría (Barcelona, Spain : 2003)》2005,62(6):573-578
Chronic recurrent multifocal osteomyelitis is a rare disease of unknown etiology that mainly affects children. We present a retrospective review of the medical records of five patients with a diagnosis of chronic recurrent multifocal osteomyelitis in an infectious diseases clinic at a tertiary center. We describe the epidemiological, clinical and radiological features, laboratory, microbiological and histological findings, treatment, course and prognosis in these patients. There is no specific treatment for chronic recurrent multifocal osteomyelitis. There are new treatment options such the combination of azithromycin and calcitonin in children without clinical improvement with non-steroidal anti-inflammatory drugs. 相似文献