We consulted many services:
1) Dermatology who did a skin biopsy times two.
2) Immunology advised re immune work up
4) Infectious Disease
5) Genetics could not link his symptoms with any know genetic syndrome
6) Dietician added Resource just for kids as a supplement
7) Occupational Therapy assessed swallowing.
Determined from observation that he may occasional aspirate with feeds, general recommendations were to thicken feeds, place head of bed upright at 45 degrees.
COURSE IN HOSPITALHe was treated with intravenous Ceftazidine, Tobramycin, and Clindamycin for 14 days. He received supplemental oxygen. He received an initial course of oral prednisolone for five days. He spent a total of 6 weeks in our hospital and 11 days in a peripheral hospital.
DIAGNOSISThe diagnosis was Langerhans cell histiocytosis was confirmed from his scalp biopsy. He was transferred to the Oncology service. He was started on a regime of chemo therapy and steroids for six months to a year. He had an initial respiratory deterioration after starting his chemo therapy and steroid treatment.
CELL HISTIOCYTOSIS (LCH)
The incidence varies from 3-4 per million. A genetic link had not been proven and most cases are sporadic. There have been single reports of cases in twins, mother/daughter, and father/son. The pathogenesis is not well understood but may be linked to a viral, immunological, or malignant/neoplastic process.
The clinical course is unpredictable. In children there is a higher incidence in males 2:1. This differs from adults where the incidence is equal in males and females. This is presumably due to the increased incidence of smoking in adult females, which carries an increased risk in itself of pulmonary manifestations of histiocytosis. There is no known occupational risk factor. Smoking exposure in adults is thought to be a risk factor for pulmonary histiocytosis, if it is seen in other organs first. There is no geographic distribution. The median age varies depending on which study you read, however generally in there is a peak in children between 1 and 3 years of age. This is different from an adult peak of 32 years.
Langerhans cells are normally present in the skin, lymph bone, bronchial mucosa, and thymus. They have a role in immune surveillance changes. The pathological process is a proliferation of abnormal histiocytes. Langerhans cells function as antigen presenting cells.
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