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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The frequency of congenital anomalies in 39 cases of histiocytosis X collected over a 30 year period was 23%. Through a retrospective chart review of these cases and two control populations (children with bone tumors and children with suspected child abuse) we sought to assess the significance of this finding as well as any special characteristics of those histiocytic patients who had congenital anomalies. The frequency of congenital anomalies in the histiocytosis X group (23%) was greater than the frequency found in our control groups, the bone tumor group (13%), and the child advocacy control group (15%). Considering only major congenital anomalies, the histiocytic population had an increased frequency (18%) relative to both control groups (3% and 8%, respectively). Only one patient with unifocal eosinophilic granuloma had congenital anomalies. Patients with histiocytosis X and congenital anomalies were more likely to have histiocytosis X involving organ dysfunction (lung, liver, hematopoietic). Through this retrospective study we observe an increased frequency of congenital anomalies in patients with histiocytosis X. This observation does not seem to apply to patients with unifocal eosinophilic granuloma. The presence of congenital anomalies, especially multiple congenital anomalies, seemed to be a prognostic indicator of organ dysfunction in our histiocytic population.
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PMID:Frequency of congenital anomalies in patients with histiocytosis X. 266 May 47

We report a previously healthy 4-month-old who presented to the hospital with leg pain and swelling and no history of trauma. Radiographs demonstrated a comminuted left femur fracture. Given the concern for child abuse, skeletal survey was performed and revealed four vertebral compression deformities. Although abuse was suspected, the possibility of a lytic lesion associated with the femur fracture and multiple spinal abnormalities raised the possibility of an underlying process such as Langerhans cell histiocytosis. Subsequently 18F-NaF positron emission tomographic (PET) scintigraphy revealed increased tracer activity in the ischium, and MRI confirmed an ischial apophyseal fracture. Pelvic fractures, particularly ischial fractures, are extremely rare in the setting of child abuse. This case report describes the multimodality imaging findings of an ischial fracture in an abused infant.
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PMID:Ischial apophyseal fracture in an abused infant. 2468 21

An important mandate when imaging pediatric patients is the reduction of radiation exposure to the lowest possible level consistent with good quality diagnostic imaging, so individual selection of radiopharmaceutical dosage and imaging technique is essential. Although the logistics of image acquisition and the approach to interpretation mirror the more common use in adults, the challenges of imaging young children typically require greater attention to patient preparation, positioning, and supervision during imaging, with the use of parental or family engagement in the procedure, gentle restraint, and occasionally the need for sedation. Because the normal bone scan appearance varies dramatically with age as osseous structures mature, it is critical that the interpreting physician be well versed in the range of normal variability at each developmental stage. Although routinely employed in the evaluation of malignancy, radionuclide bone imaging in children is used for a much wider range of benign disorders than it is typical in adults. In many pediatric nuclear medicine departments, the evaluation of benign bone disorders represents most imaging procedures. Common indications include the evaluation of sports injuries, osteomyelitis, trauma, including suspected child abuse, growth disorders, such as mandibular condyle asymmetry, and noninfectious processes such as Langerhans cell histiocytosis. Both planar scintigraphy and SPECT imaging are widely employed, although the SPECT acquisition time presents a barrier in some age groups, making shorter planar spot views desirable when feasible. PET bone imaging with 18F-sodium fluoride has been shown useful in a number of pediatric disorders, but may present issues in young children, as it can require the use of sedation. Despite this challenge, the higher resolution and resulting better anatomical depiction of bone anatomy by PET techniques can be very valuable in evaluating small structures, and current experience suggests a large future role for fluoride PET imaging.
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PMID:Nuclear Medicine Techniques in Pediatric Bone Imaging. 2841 51

Langerhans cell histiocytosis is a rare non-malignant disease with clinical heterogeneity. The disease may present with various clinical findings and may imitate many other conditions. In this report we describe a 34-month-old girl who presented with chronic otitis and otorrhea, skull fracture, rash, vulvar edema, erythema and erosion in labia majors which initially suggested child abuse but the patient was diagnosed with Langerhans cell histiocytosis.
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PMID:A case of Langerhans cell histiocytosis mimicking child abuse. 2909 Aug 85