Aashima Dabas1, Atul Batra2, Rajesh Khadgawat1, Viveka P Jyotsna1, Sameer Bakhshi3. 1. Department of Endocrinology and Metabolism, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India. 2. Department of Medical Oncology, DR. B.R.A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, India. 3. Department of Medical Oncology, DR. B.R.A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, India. sambakh@hotmail.com.
Abstract
OBJECTIVE: To ascertain the growth and endocrinal disturbances associated with Pediatric Langerhans Cell Histiocytosis (LCH). METHODS: Retrospective analysis of hospital records of subjects with LCH, aged 1 mo to 18 y was performed. The diagnosis of LCH was made as per Histiocyte Society criteria. Subjects were classified as group A: multifocal bone disease; B: soft tissue involvement without organ dysfunction; and C: organ dysfunction and treated as per DAL-HX-83 protocol of the Histiocyte Society LCH treatment guidelines. Paired t-test was used to compare the baseline and follow-up data. RESULTS: Total 62 records (group A- 18, B-32 and C-12) were identified with median follow-up of 5.3 ± 3.3 y. Growth failure [measured as weight/ height Standard deviation score (SDS) ≤-2] was the commonest disorder seen in 27 (44 %) subjects. Central Diabetes Insipidus (DI) was seen in 12 (19 %) subjects. Subjects with group C of LCH had poorer weight and height at baseline and follow-up than subjects with group A or B. Height SDS were lower in subjects with concomitant DI than those without DI at baseline (-2.35 ± 1.9 and -1.69 ± 1.4; P 0.18). Subjects with DI did not show significant catch-up in their height (P 0.12) unlike those without DI who showed a catch-up in height (P 0.03) on follow-up. CONCLUSIONS: Growth monitoring and screening for DI should be essential part of follow-up in all subjects with LCH.
OBJECTIVE: To ascertain the growth and endocrinal disturbances associated with Pediatric Langerhans Cell Histiocytosis (LCH). METHODS: Retrospective analysis of hospital records of subjects with LCH, aged 1 mo to 18 y was performed. The diagnosis of LCH was made as per Histiocyte Society criteria. Subjects were classified as group A: multifocal bone disease; B: soft tissue involvement without organ dysfunction; and C: organ dysfunction and treated as per DAL-HX-83 protocol of the Histiocyte Society LCH treatment guidelines. Paired t-test was used to compare the baseline and follow-up data. RESULTS: Total 62 records (group A- 18, B-32 and C-12) were identified with median follow-up of 5.3 ± 3.3 y. Growth failure [measured as weight/ height Standard deviation score (SDS) ≤-2] was the commonest disorder seen in 27 (44 %) subjects. Central Diabetes Insipidus (DI) was seen in 12 (19 %) subjects. Subjects with group C of LCH had poorer weight and height at baseline and follow-up than subjects with group A or B. Height SDS were lower in subjects with concomitant DI than those without DI at baseline (-2.35 ± 1.9 and -1.69 ± 1.4; P 0.18). Subjects with DI did not show significant catch-up in their height (P 0.12) unlike those without DI who showed a catch-up in height (P 0.03) on follow-up. CONCLUSIONS: Growth monitoring and screening for DI should be essential part of follow-up in all subjects with LCH.
Authors: B E Favara; A C Feller; M Pauli; E S Jaffe; L M Weiss; M Arico; P Bucsky; R M Egeler; G Elinder; H Gadner; M Gresik; J I Henter; S Imashuku; G Janka-Schaub; R Jaffe; S Ladisch; C Nezelof; J Pritchard Journal: Med Pediatr Oncol Date: 1997-09
Authors: Tejinder Singh; C T Satheesh; L Appaji; B S Aruna Kumari; H S Mamatha; G V Giri; Clementina Rama Rao Journal: Indian J Med Paediatr Oncol Date: 2010-04
Authors: D B Dunger; V Broadbent; E Yeoman; J R Seckl; S L Lightman; D B Grant; J Pritchard Journal: N Engl J Med Date: 1989-10-26 Impact factor: 91.245