Kikuchi-Fujimoto disease is a rare cause of lymphadenopathy and fever of unknown origin in children: report of two cases and review of the literature

Kikuchi-Fujimoto disease, a benign and unusual self-limiting histiocytic necrotizing lymphadenitis of unknown origin, should be included in the differential diagnosis of lymphadenopathy and fever of unknown origin. This disease mostly affects young Asian women and has rarely been reported in children, thus remaining a poorly recognized entity that is frequently confused with malignant lymphoma. The authors describe two children with Kikuchi-Fujimoto disease, with particular attention to diagnostic approach and clinical and histologic features of the disease.     

Difference of clinical features in childhood <Emphasis Type="Italic">Mycoplasma pneumoniae</Emphasis> pneumonia

Background  

M. pneumoniae pneumonia (MP) has been reported in 10-40% of community-acquired pneumonia cases. We aimed to evaluate the difference of clinical features in children with MP, according to their age and chest radiographic patterns.     

Pulmonary alveolar microlithiasis in pediatric patients — review of the world literature and two new observations

Report of two new pediatric cases of pulmonary alveolar microlithiasis (PAM). PAM is a rare disease of unknown etiology; not more than 38 cases have been reported aged less than 18 years. The essential features of this disease are (1) a characteristic radiographic appearance of a sand-like opacity of both lungs and linear densities like “beads on a string» along the heart, pleura and interlobar fissures; this is best visualized by fluoroscopy in combination with magnification technique; (2) extremely few clinical signs and almost no laboratory abnormalities.     

Condensing osteitis of the clavicle in childhood: A rare sclerotic bone lesion

Seven children and adolescents with sclerotic and periosteal alterations of the clavicle are reported, two of them in detail. Malignancies and bacterial inflammatory processes, which were first suspected, could be excluded. Clinical and radiographic features as well as differential diagnosis are discussed, as is the pertinent literature. The etiology of this uncommon bone lesion, which is described under different names, could not be clarified.     

Severe acute respiratory syndrome (SARS) in a paediatric cluster in Singapore

Background Severe acute respiratory syndrome (SARS) is a major infectious disease pandemic that occurred in early 2003, and one of the diagnostic criteria is the presence of chest radiographic findings.Objective To describe the radiographic features of SARS in a cluster of affected children.Materials and methods The chest radiographs of four related children ranging in age from 18 months to 9 years diagnosed as having SARS were reviewed for the presence of air-space shadowing, air bronchograms, peribronchial thickening, interstitial disease, pleural effusion, pneumothorax, hilar lymphadenopathy and mediastinal widening.Results Ill-defined air-space shadowing was the common finding in all the children. The distribution was unifocal or multifocal. No other findings were seen on the radiographs. None of the children developed radiographic findings consistent with acute respiratory distress syndrome. All four children showed significant resolution of the radiographic findings 4–6 days after the initial radiograph.Conclusions Early recognition of these features is important in implementing isolation and containment measures to prevent the spread of infection. SARS in children appears to manifest as a milder form of the disease as compared to adults.     

Primary bone tumours in infants

Ten cases of primary bone tumours in infants (1 osteosarcoma, 3 Ewing's sarcoma, 1 chondroblastoma and 5 angiomatosis) are reported. All cases of angiomatosis showed characteristic radiographic findings. In all the other tumours the X-ray appearances were different from those usually seen in older children and adolescents. In the authors' opinion the precise diagnosis of malignant bone tumours in infancy is very difficult as no characteristic X-ray features are present in this age period.     

Development of a life-threatening anaphylactoid reaction following administration of ioversol in a child

We report for the first time the case of a 3-year-old girl who developed a severe anaphylactoid reaction following the administration of the nonionic, low osmolar intravenous radiographic contrast agent ioversol. Severe reactions to ioversol have rarely been reported in adult patients and to our knowledge never in children. Health care providers managing children who may require radiologic imaging studies with ioversol should be aware of the potential complications of this radiographic contrast agent. Received: 13 August 1998 Accepted: 9 September 1998     

Radiographic features of appendiceal colic in children

The concept of appendiceal colic was introduced in 1980 to explain the common problem of recurrent crampy abdominal pain (RAP) in children. Children with appendiceal colic often have inspissated casts of stool as foreign bodies of the appendix. The radiographic findings of 115 children operated upon for appendiceal colic have been reviewed. The radiographic features of this syndrome have included: filling defects of the appendix (83% incidence of inspissated casts of stool within the appendix); partial filling of the appendix (44% incidence of fecal casts); retained barium behind 72 h (92% incidence of fecal casts blocking egress of barium); non-visualization of the appendix (42% incidence of fecal casts); and distention of the appendix (100% incidence of fecal casts). The correlation of radiographic and operative findings will be presented. A rational approach to the radiographic workup of a child with RAP will be presented.     

Idiopathic myelofibrosis with generalized periostitis in a 4-year-old girl

Idiopathic myelofibrosis, a chronic myeloproliferative disorder of unknown origin, is characterized by splenomegaly, extramedullary hematopoiesis, leukoerythroblastosis, teardrop erythrocytes, and myelofibrosis. It is a rare disorder in childhood. The authors describe a 4-year-old girl with features consistent with idiopathic myelofibrosis, who also had generalized solid laminated periosteal reaction involving all long bones. The presence of thrombocytopenia at the onset and lack of leukocytosis were in contrast to the reported features seen in children. Recent case reports describe a relatively indolent course in children. Spontaneous remissions have also been described in pediatric cases. The fulminant course of this patient without any features of malignant transformation was noteworthy in this regard.     

疑难病研究——遗传性巨十二指肠症

报道1例遗传性巨十二指肠症的病例,该病为一种以家族性空腔脏器肌病为特点的少见病,可能为常染色体显性遗传病。此病儿童发病罕见,临床表现为反复发作的假性消化道梗阻,以间断发生的呕吐、腹胀为主要症状,可造成营养障碍、发育迟缓。钡餐检查可见十二指肠扩张,食道测压显示食道蠕动功能受损。病理形态学上表现多样,可见肠壁的炎性浸润、平滑肌层变薄,纤维组织增生。确诊需结合家族史、病理检查及影像学和功能学检查方可作出。手术治疗可暂时缓解症状但无法治愈该病。     

Pneumococcal colitis: report of a case with radiologic and endoscopic findings

Gastrointestinal symptoms and signs have been reported in association with pneumococcal infection for many years. Only rarely has Streptococcus pneumoniae been reported as a bowel pathogen. We report the clinical, radiographic, colonoscopic, and pathologic features of a case of pneumococcal colitis in an otherwise healthy 3.5-year-old female.     

Outcomes of perinatal arterial ischemic stroke and cerebral sinovenous thrombosis

Perinatal stroke can result in cerebral palsy, cognitive impairment, epilepsy, and sensory deficits. While some children only experience one type of disability, severe disabilities often cluster together. Death associated with perinatal stroke is rare, and often associated with other comorbidities. Clinical and radiographic factors can help predict outcome, but additional as-yet unknown factors appear to contribute to outcome as well. Rehabilitation can ameliorate deficits, and advances in rehabilitation technology and in adult stroke rehabilitation offer promise to pediatric perinatal stroke patients.     

Tuberculosis in HIV-infected children

Tuberculosis (TB) is the most common opportunistic infection in human immunodeficiency virus (HIV)-infected people worldwide. HIV-positive children are at risk of diagnostic error as well as delayed diagnosis of TB because of overlapping clinical and radiographic features with other lung diseases. Acute pneumonias and chronic lung diseases such as bronchiectasis and lymphocytic interstitial pneumonitis are difficult to distinguish from TB. TB manifestations are more severe in HIV-positive children and progression to death is more rapid than in HIV-negative children. The response to standard short-course therapy in HIV-positive children is not as good as in HIV-negative children due to lower cure rates and higher mortality. TB hastens the progression of HIV disease by increasing viral replication and reducing CD4 counts further. Although Bacille Calmette-Guerin vaccination could lead to disseminated Mycobacterium bovis disease in the presence of immunosuppression, this has been rarely reported. More studies are required to assess the role of newer diagnostic tests, TB preventive therapy and co-administration of anti-retroviral therapy in the control of TB among HIV-infected children.     

DYGGVE DISEASE

A case of a 5 year old Polish girl with Dyggve disease is reported. The radiographic pattern of the vertebral column and pelvis is diagnostic in this syndrome. The course of the disease in infancy and in adult life is at present unknown.     

Benefits and unexpected artifacts of biplanar digital slot-scanning imaging in children

Biplanar digital slot-scanning allows for relatively low-dose orthopedic imaging, an advantage in imaging children given the growing concerns regarding radiosensitivity. We have used this system for approximately 1 year for orthopedic imaging of the spine and lower extremities. We have noted advantages of using the digital slot-scanning system when compared with computed radiographic and standard digital radiographic imaging systems, but we also found unexpected but common imaging artifacts that are the direct result of the imaging method and that have not been reported. This pictorial essay serves to familiarize radiologists with the advantages of the digital slot-scanning system as well as imaging artifacts common with this new technology.     

Evanescent colitis in a child

Pediatric Radiology - The clinical and radiographic features of evanescent colitis in a child are described. Previously reported cases have occurred in adults.     

腺病毒肺炎12例临床分析

目的 提高对腺病毒肺炎临床特点的认识.方法 回顾分析2010年10月 - 2011年5月12例确诊为腺病毒肺炎患儿的临床资料,对其临床、影像及纤维支气管镜(纤支镜)下特点,以及治疗进行总结.结果 12例患儿均有持续高热、热程长,喘鸣音顽固,肝肿大,感染中毒症状重,易出现合并症,其中低氧血症11例,呼吸衰竭5例,中毒性心肌炎5例,中毒性肝炎6例,脑病6例,典型川崎病改变3例,死亡2例.肺部由早期斑片状影迅速融合成大片影,病变部位以双下叶尤其是左下叶为甚.纤支镜镜下9例(75%)患儿有灰白色黏液栓致开口堵塞,吸出为支气管塑形样物,8例(66.6%)患儿有黏膜糜烂剥脱.结论 腺病毒肺炎纤支镜下黏膜糜烂剥脱可能预示其病情更重,治疗上除抗感染及常规对症治疗外,可酌情采用激素和静脉用丙种球蛋白以及纤维支气管镜灌洗等综合治疗.     

Mannosidosis: phenotype of a severely affected child and characterization of alpha-mannosidase activity in cultured fibroblasts from the patient and his parents.

A three-year-old boy has coarse facial features, upper respiratory congestion, profound mental retardation, hepatosplenomegaly, increased height and head circumference, cataracts, a gibbus deformity, radiographic changes of dysostosis multiplex, and vacuolized peripheral lymphocytes. These findings are the most commonly reported clinical features in the previously described patients with mannosidosis. Our patient has a severe deficiency, and his parents have intermediate levels, of the acidic component of alpha-mannosidase in their cultured fibroblasts.     

Pacman dysplasia: a lethal skeletal dysplasia with variable radiographic features

BACKGROUND: Punctate or stippled cartilaginous calcifications are associated with many conditions, including chromosomal, infectious, endocrine, and teratogenic etiologies. Some of these conditions are clinically mild, while others are lethal. Accurate diagnosis can prove instrumental in clinical management and in genetic counseling. OBJECTIVE: To describe the diagnostic radiographic features seen in Pacman dysplasia, a distinct autosomal recessive, lethal skeletal dysplasia. MATERIALS AND METHODS: We present the fourth reported case of Pacman dysplasia and compare the findings seen in our patient with the three previously described patients. RESULTS: Invariable and variable radiographic findings were seen in all four cases of histologically proven Pacman dysplasia. CONCLUSION: Pacman dysplasia presents both constant and variable diagnostic radiographic features.     

Melnick-Needles syndrome

Melnick-Needles syndrome is a rare connective tissue disorder producing somatic abnormalities with characteristic radiographic features. There are less than 35 documented cases reported to date. We present four new cases of Melnick-Needles Syndrome, one of which is the first reported Asian-ancestry patient. Two girls are daughters of one of Needle's original patients from family #2. Two others, from unrelated non-affected families, probably represent new mutations. All have characteristic radiographic features of the disorder.