Dr. Atul A. Kulkarni, M.D.(Paed)*, Dr. Kiran B. Masal, Resident **
Department of Paediatrics, Ashwini Sahakari Rugnalaya & Research Centre, Solapur. *, Department of Paediatrics, Ashwini Sahakari Rugnalaya & Research Centre, Solapur. **
|Case History: 7 years female child presented with Polyuria, polydipsia, headache (mainly in occipital region) since 2 months. Drowsiness since 8 days, patient came in altered sensorium. |
- Hb - 7.5, TLC - 4800, Platelets - 1,50,000/μl
- CSF/BSL/ Urine examamination - normal
- Serum electrolytes: Na -192, K - 4.5, CI - 102 suggestive of diabetes insipidus
X-ray skull-Osteolytic lesion in occipital region CT head - Pituitary adenoma Biopsy - Type 1 histiocytosis.
Childhood histiocytosis constitutes a diverse group of disorders. Disease Cellular characteristics: Class 1 Langerhans Cell Histiocytosis, Langerhans Cells (CDI Positive) with Birbeck granules.
- Skeleton involvement in 80% cases. Osteolytic lesions - single or multiple mostly in skull.
- Seborrheic dermatitis, Hepatosplenomegaly, Lymphadenopathy (localized or disseminated) - Hematological disorders: Anemia, Leucopenia, Thrombocytopenia
- Pulmonary infiltration with respiratory distress and coughing
- Growth retardation (Pituitary or Hypothalamic dysfunction)
- Systemic manifestation in severely affected patient
Single system disease - Curettage or low dose local radiation therapy. Multisystem disease - Systemic multiagent chemotherapy (vinblastine or etoposide)
|How to Cite URL :|
|M.D.(Paed) K A A D, Resident M B K D.. Available From : http://www.pediatriconcall.com/fordoctor/ Conference_abstracts/report.aspx?reportid=244|