Pulmonary oedema following choking: report of two cases

Two children, aged 3 1/2 and 5 1/2 years, are described. Both developed pulmonary oedema (PE) following a short episode of choking on a sweet and an orange, respectively. On admission diagnosis was made by chest X-ray. One child was asymptomatic despite PE while the other showed only mild respiratory distress. Both children recovered spontaneously and chest X-rays showed a return to normal within 24 h. The mechanism of PE production is discussed. It is suggested that oedema formation occurs during the obstruction and that it is due to hypoxia and the severe negative pleural pressure resulting from attempts to inspire against the obstructed airway. both hypoxia and severe negative pleural pressure cause an increase in pulmonary capillary pressure and transduration of fluid across the capillary membrane.Abbreviations PE pulmonary oedema - UAO upper airway obstruction     

Meningioangiomatosis: MR imaging and pathological correlation in two cases

Meningioangiomatosis is a rare, benign neoplastic disorder involving the cortex and leptomeninges. The pathological findings are characterised by proliferation of meningothelial cells and leptomeningeal vessels and calcifications within the mass. We experienced two cases of pathologically confirmed meningioangiomatosis, one as a solitary cortical mass with calcification and the other as a cortical lesion manifested as extensive intracranial haemorrhage. On MRI, the first case showed an isointense cortical mass in the left frontal lobe and homogeneous enhancement on the contrast-enhanced study. The second case showed a target-like lesion with a peripheral dark signal rim on T2-weighted images accompanied by extensive haemorrhage in the adjacent frontal lobe and lateral ventricles.     

Primary sternal osteomyelitis: a report of two cases with literature review

Primary sternal osteomyelitis is a rare disease in children caused mainly by Staphylococcus aureus. We describe 2 cases resulting from Streptococcus pneumonia and Candida albicans. On the basis of these cases and other documented case reports, we discuss the pathogens, clinical course, and pathophysiology and suggest a management protocol based on early debridement to initiate appropriate antibiotic therapy and shorten hospitalization.     

Normal childhood developmental patterns in skull bone marrow by MR imaging

To establish the normal developmental pattern of skull bone marrow in children by MR imaging, sagittal T1-weighted MR skull images of 324 normal children (newborn to 18 years) were reviewed. Bone marrow intensity was assigned four gradations as compared with that of muscle and fat on the same image. Bone marrow became isointense with fat (yellow marrow) at a mean age ±S.E.M. (in years) of 8.5±0.24 in sphenoid, 9.1±0.29 in mandible, 9.3±0.28 in hard palate, 9.7±0.26 in frontal, 11.0±0.26 in squamous occiput, 11.5±0.28 in parietal, and 11.9±0.24 in basiocciput. There is a strong correlation between age and marrow intensity by Spearman analysis (p<0.001): hard palate 0.64, mandible 0.61, parietal 0.42, sphenoid 0.70, cervical spine 0.50, basi-occiput 0.58 and occiput 0.52. Two consistent overall patterns of red-yellow marrow conversion were observed. Bone marrow became isointense with fat prior to pneumatization of the paranasal sinuses. Marrow conversion in the bones of the face occurred before those of the calvarium in a specific pattern. There was no significant sex difference in the pattern or rate of marrow conversion. These normative data are necessary to evaluate the immature skull by MR imaging in disease states.     

MR appearances of bone marrow in children following bone marrow transplantation

Two cases are presented of children who demonstrated complete absence of bone marrow signal on MR imaging of the spine following bone marrow transplantation. The possible causes for these appearances are discussed.     

关节镜介入自体骨髓移植治疗单房性骨囊肿

目的：报道关节镜介入自体骨髓移植治疗15例单房性骨囊肿的疗效。方法：15例单房性骨囊肿患儿，男11例，女4例，平均年龄10岁1个月(7--18岁)。8例位于肱骨近端，5例位于股骨近端，1例位于股骨远端，1例位于胫骨近端。均在“C”型臂线机监视下骨囊肿置入关节镜，行囊肿壁搭刮处理后注入从髂骨吸取的骨髓。结果：15例患儿6个月后X线片示囊腔骨化，随访6～15个月骨愈合满意，未见并发症发生。结论：关节镜介入自体骨髓移植治疗单房性骨囊肿愈合快，疗效显著。     

Chronic recurrent multifocal osteomyelitis: report of five cases

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.     

Langerhans cell histiocytosis of bone: MR imaging

Magnetic resonance (MR) images of 12 pathologically proven lesions of Langerhans cell histiocytosis (LCH) of bone were reviewed retrospectively. MR identified all lesions, three of which were not identified on plain radiographs. In all cases, MR showed greater abnormality than did plain radiographs. With one exception, all lesions were hypointense on T1-weighted images and hyperintense on T2-weighted images. The lesions and associated soft tissue abnormalities were very conspicuous on short TI inversion sequences and T1-weighted post-contrast images. Follow-up MR studies in two patients after chemotherapy showed decreased size and enhancement of lesions compared with baseline studies.     

Chronic recurrent multifocal osteomyelitis with MR correlation: A case report

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.     

Allogeneic bone marrow transplantation in Fanconi anemia from Turkey: a report of four cases

Bone marrow transplantation (BMT) is currently the treatment of choice for patients with Fanconi anemia (FA) if a suitable donor is available. Four children with FA underwent allogeneic BMT from HLA-identical siblings during the period from 1995 to 1996. Pretransplant conditioning was Cyclophosphamide (Cy) (20 mg/kg) + Thoracoabdominal irradiation (TAI) (500 cGy) +/- Antithymocyte globulin (ATG) (2 mg/kg/day x 3). Cyclosporin A (CsA) was used as GvHD prophylaxis. The time of neutrophil (ANC>500) and platelet (>50,000) recovery were at 11-14 and 17-25 days, respectively. One patient with a pretransplant history of multiple transfusions experienced graft rejection and died at day +29 with infection and bleeding. Although three patients sustained engraftment one developed donor originated acute lymphoblastic leukemia (ALL) 18 months after BMT and died with CNS hemorrhage and infection at +25 months following 7 months of chemotherapy. None of the patients developed grade 3-4 acute GvHD. Cytotoxicity included grade II mucositis in all and severe gastroenteritis in one patient. During a follow-up period of 10 months and 2 years, two patients are well with normal blood count, recovering immune function and have a Karnofsky score of 90%.     

Diffusion-weighted MRI of bone marrow oedema, soft tissue oedema and synovitis in paediatric patients: feasibility and initial experience

ABSTRACT: BACKGROUND: MRI has become the mainstay of diagnostic imaging in paediatric rheumatology for lesion detection, differential diagnosis and therapy surveillance. MR imaging of synovitis, in particular, is indispensable for early diagnosis and follow-up in arthritis patients. We used diffusion-weighted MRI (DWI) as a new imaging modality in comparison to standard MRI sequences to study bone marrow oedema, soft-tissue oedema and synovitis in paediatric patients. METHODS: A total of 52 patients (mean age 11 +/- 5 years) with bone marrow oedema (n = 31), soft-tissue oedema (n = 20) and synovitis (n = 15) were examined with transversal diffusion-weighted single-shot echoplanar imaging in addition to standard MR sequences (T2W TIRM, T1W pre- and post-contrast). Diffusion-weighted images were used for lesion detection and apparent diffusion coefficient (ADC, unit x 10-3 mm2/s) values were measured with ROI technique on ADC maps. RESULTS: In 50 of 52 patients, DWI delineated the lesion of interest corresponding to pathological signal increase on standard sequences. Mean ADC was 1.60 +/- 0.14 (range 1.38 - 1.99) in osseous lesions, 1.72 +/- 0.31 (range 1.43 - 2.56) in soft tissue oedema and 2.82 +/- 0.24 (range 2.47 - 3.18) for joint effusion (ANOVA p < 0.001). No significant difference in mean ADC was seen for inflammatory vs. non-inflammatory lesions. Relative signal intensity of oedema was similar for DWI and T2W TIRM. DWI visualised synovial restricted diffusion with a mean ADC of 2.12 +/- 0.45 in 12 of 15 patients with synovitis. CONCLUSIONS: Diffusion-weighted MRI reliably visualises osseous and soft tissue oedema, as compared to standard sequences. DWI of synovitis is feasible in large joints and presents a novel approach to contrast-free imaging of synovitis. Whole-body DWI for chronic non-bacterial osteomyelitis should be evaluated in future studies.     

Iron overload following bone marrow transplantation in children: MR findings

Objective. The purpose of this study was to determine the incidence of post-transfusional iron overload in children after bone marrow transplantation by reviewing their magnetic resonance imaging (MR) findings. Materials and methods. We reviewed the abdominal MR studies of 13 children after autologous bone marrow transplantation. Nine of the children had also undergone MR prior to transplantation. Iron deposition in the liver, spleen and bone marrow was graded semi-quantitatively on both T1- and T2-weighted images. Serum ferritin levels and number of blood units given after bone marrow transplantation were recorded. Results. None of the pre-transplantation MR studies revealed iron overload. After bone marrow transplantation, three children showed normal liver and spleen. Iron overload in the liver was noted in ten patients (77 %), six of whom also showed iron overload in the spleen (46 %) and five in the bone marrow (38.5 %). The degree of hepatic iron overload was correlated significantly and splenic iron overload was correlated weakly with the number of blood transfusions (P = 0.01 and P > 0.01, respectively), but neither was correlated with the serum ferritin level. Conclusion. Iron overload commonly accompanies bone marrow transplantation. The observed pattern of iron deposition, in which the spleen was uninvolved in 40 % of patients demonstrating iron overload, is not typical of post-transfusional hemochromatosis. Received: 10 June 1997 Accepted: 10 June 1997     

Chronic recurrent multifocal osteomyelitis in children: report of 17 cases]

Chronic recurrent multifocal osteitis (OCRM) is a rare condition in children, of unknown aetiology, which may be misdiagnosed as osteomyelitis, arthritis or tumour. PATIENTS AND METHODS: We present a retrospective multicentric study of 17 patients (five boys and 12 girls) with an average follow-up of 7.5 years (six months-25 years). RESULTS: A spectrum of presenting features is possible, ranging from bone lesions alone to lesions combined with arthritis, palmoplantar pustulosis or psoriasis. The diagnosis was delayed from two weeks to five years. Roentgenographic evaluation was often normal at the beginning of the disease or showed nonspecific bone reactions. Radioisotope bone scans assisted in establishing the diagnosis and in identifying lesions that were initially clinically silent. Bone biopsies were performed in seven cases. Histopathological examination showed only mild inflammatory nonspecific changes. Microbiological cultures were always negative. Treatments were different according to the evolution of the disease and the hospital. There was no response to antibiotics in seven patients. The response to nonsteroidal anti-inflammatory agents and steroids was moderate and often transient. Salazopyrine and pamidronate treatment used in two patients allowed a durable remission. We lost sight of four patients, pain persisted in three in spite of treatment, it disappeared in two with treatment, mild pain persisted in five without treatment and remission occurred in three without treatment. CONCLUSION: This study clarifies the clinical and radiologic features of chronic recurrent multifocal osteomyelitis. The recognition of this rare entity is often delayed and difficulties in patient management sometimes emerge from its usual protracted course.     

Successful second bone marrow transplantation in Omenn's syndrome after bone marrow aplasia: a case report

Omenn's syndrome is a rare inherited variant of SCID. It is inevitably fatal, unless treated by bone marrow or stem cell transplantation. However, treatment-related complications and graft rejection are major obstacles to the success of transplantation. In this report, we describe an eight-month-old baby with Omenn's syndrome and disseminated BCGosis, who underwent allogenic BMT from his HLA-matched sister using anti-GVHD prophylaxis but without a conditioning regimen. Ten days after BMT, he developed acute GVHD involving the skin only (grade 1) with a flare of local BCGosis. Engraftment of 55% was demonstrated by FISH as early as the 11th day post-BMT. On day +48 post-BMT, he developed GVHD that was complicated by refractory pancytopenia, resistant to three doses of rituximab. Patient was re-transplanted (from the same donor) after receiving fludarabine and ATG, with successful engraftment and complete recovery from pancytopenia.     

外周血和骨髓微量神经母细胞瘤细胞检测的临床意义

目的 探讨CD45/CD56/GD2单克隆抗体(单抗)流式细胞仪分析法检测周围血和骨髓微量神经母细胞瘤(NB)细胞的临床意义。方法 11例腹部神经母细胞瘤(NB组)和23例其他肿瘤(对照组)患儿,年龄6个月至12岁,用单抗CD45/CD56/GD2流式细胞术检测外周血和骨髓穿刺标本微量NB细胞。结果 NB组入院时血和骨髓微量NB细胞阳性率分别为90.9％(10/11)和72.7％(8/11),对照组血和骨髓均为阴性(P<0.001)。NB组骨髓微量NB细胞检测与涂片细胞学检查阳性率有较好的相关性(P<0.05),但前者阳性率更高,不受骨髓稀释影响。骨髓NB细胞阳性率与临床分期有关,Ⅱ～Ⅳ期明显高于Ⅰ期(P<0.01)。化疗后血和骨髓NB细胞数均明显减少(P<0.05)。术后转移者血NB细胞重新出现阳性。结论 CD45/CD56/GD2单抗流式细胞术检测周围血和骨髓微量NB细胞具有高度敏感性和特异性,有助诊断、鉴别诊断、疗效观察和复发转移监测。     

Usefulness of bone marrow imaging in childhood malignancies

Two hundred six technetium-99m sulfur colloid bone marrow scans in 110 pediatrics patients were reviewed. The normal distribution of sulfur colloid in the lower extremities in various age groups was established. There was progressive loss of uptake with increasing age from less than two years to greater than ten years. Tumor replacement was seen as regions of decreased radioactivity, and the extent of the scan defect paralleled the response of the disease to therapy. Both chemotherapy and irradiation resulted in an extension of the Tc-99m SC to peripheral marrow sites. In irradiated areas, marrow scan defects were demonstrated and generally recovered normal activity by six months after the completion of therapy. Marrow scan abnormalities caused by tumor replacement were present in four patients despite normal bone scans and radiographs. Ultimate confirmation of tumor involvement was by needle aspiration or biopsy. Persistent marrow defects were seen in two patients with neuroblastoma who had remission of their disease: biopsy revealed myelofibrosis. Technetium-99m sulfur colloid bone marrow scanning is a sensitive monitor of altered marrow activity associated with pediatric hematologic or oncologic diseases.     

Flow cytometry and fluorescence in situ hybridization to detect residual neuroblastoma cells in bone marrow

BACKGROUND: In patients with neuroblastoma morphological assessment of BM for residual NB cells is not precise, particularly when the number of tumor cells is small. PROCEDURE: To develop a sensitive and rapid method of detecting NB cells in BM, we assessed the efficiency of flow cytometry (FCM) using markers CD9, CD56, and CD45. The percent of CD9+/CD56+/CD45- (NB phenotype) cells was determined by FCM in 41 samples (16 patients) at various time points. For confirmation fluorescence in situ hybridization (FISH) for 17q gain was performed. RESULTS: Nineteen of the 22 (86%) samples that were negative by morphology were positive by FCM (>0.006% CD9+/CD56+/CD45- cells). The longest time to complete the FCM study was 3 hr. In six FISH experiments the sorted CD9+/CD56+/CD45- population had a higher percentage of cells with 17q gain (11.5-95%) compared to a CD56-/CD45+ internal control population (2-8%). CONCLUSIONS: Our preliminary results suggest that FCM determination of the percent of CD9+/CD56+/CD45- cells is an effective method of rapidly detecting NB cells in BM.