Abstract
Langerhans cell histiocytosis is a multi system disorder with a certain predilection for involving hypothalamic pituitary axis. We hereby report a 7 year old girl presenting with polyuria, polydipsia and growth retardation. The girl had a past history of pain in right hip joint and nodular region over chest. Water deprivation test confirmed the diagnosis of central diabetes inspidus. Other investigations revealed Growth hormone deficiency and central hypothyroidism. X-ray and MRI hip revealed absent right inferior pubic ramus with bone marrow biopsy confirming the diagnosis of histiocytosis. Patient was treated with nasal Arginine Vasopressin spray, subcutaneous growth hormone and oral thyroxine. Key words: Histiocytosis; Diabetes inspidus; Growth hormone deficiency; Central hypothyroidism DOI: http://dx.doi.org/10.3126/jnps.v32i1.5343 J. Nepal Paediatr. Soc. Vol.32(1) 2012 81-84
Highlights
Langerhans’ cell histiocytosis (LCH) is a rare disease with an annual incidence of 4 per million for the age range median in children above 3 years of age[1]
A 7 year old girl presented with complaints of excessive thirst with thirst persisting during night, frequent urination including nocturia for six months
There was no associated altered mental status. She complained of lethargy, dry skin, cold intolerance and constipation. She had a history of painful nodular chest lesion on ribs and pain in hip joints following which X-ray pelvis (Figure 1) and MRI hip revealed absent right inferior pubic ramus
Summary
Langerhans’ cell histiocytosis (LCH) is a rare disease with an annual incidence of 4 per million for the age range median in children above 3 years of age[1]. The granulomatous deposits consisting of specific langerhan’s dendritic cells occur at multiple sites within the body and involve hypothalamic pituitary axis (HPA) in 5-50% cases[2]. Diabetes inspidus (DI) is the most common endocrine abnormality in 15-50% cases[3] with anterior pituitary dysfunction in 5-20% cases[4]. We hereby report a case of a girl child with histiocytosis presenting with DI and partial hypopituitarism.
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