LETTERS TO THE EDITOR

3. Rolland-Cachera MF, Bellisle F. No relative weight. J Appl Behav Anal 1986; 16: correlation between adiposity and food intake: 371-378. why are working class children fatter? Am J 5. Robinson TN. Reducing children’s television Clin Nutr 1986; 44: 779-787. viewing to prevent obesity. A randomized 4. Klesges RC, Coates TJ, Brown G. Parental controlled trial. JAMA 1999; 282; 1561- influences on childrens’ eating behavior and 1567.

Diabetes Mellitus in no psychological or behavioral problems. Neurofibromatosis I: An Unusual Investigations revealed a random blood glucose 830 mg/dL, and fasting blood glucose Presentation 307 mg/dL. Urine examination showed specific gravity 1.040, glucosuria 4+, and traces of ketone bodies. No abnormality was detected on ultrasonography or CT scan of the Neurofibromatosis (NF) are a set of abdomen or duodenal endoscopy. MRI genetic disorders resulting from abnormalities abdomen could not be performed due to of neural crest development or ‘neurocristo- financial constraints of the patient. The patient pathy’. They can affect the development of was put on insulin therapy and normo- non-nervous tissue such as bones, soft tissue, glycemia was maintained on 0.8 units of endocrine glands and skin. We report a case of insulin / kg / day. NF I with diabetes mellitus. To the best of our knowledge diabetes mellitus in association The diagnosis of NF type I is based largely with NF 1, has not so far been reported in on clinical criteria set up by National Institute children. A 9-year-old male presented with of Health, 1987(1). The condition is general debility, fever off and on since birth, associated with protean manifestations as well polydipsia, polyuria, and polyphagia for the as some serious complications. In 1983 a last 3 months. There was no history of syndrome of multiple endocrine neoplasia diarrhea, steatorrhea, jaundice, abdominal was described as MEN type III which pain, headache or seizures. He was found to be included duodenal carcinoid (often producing both underweight and short statured. Multiple somatostatin) and NF type I or pheo- (>15) Cafe-au-lait spots measuring 2 mm to chromocytoma(2). Unlike other endocrinal 20 mm and axillary freckling was present. abnormalities, diabetes mellitus is rarely seen Two large naevi with underlying neuro- in association with NF I. It is attributed to fibromas covered the lower back and gluteal occurance of somatostatinomas in pancreas region. Eye examination revealed bilateral Iris and duodenum. These are rare gut-pancreatic Lisch nodules, and pigmentary changes in the endocrinomas that secrete somatostatin. fundus. The left lower limb was found to be Inhibition of insulin release produces the 6cm longer than the right. Systemic diabetic state which is easy to control because examination did not reveal any abnormality. of concomitant suppression of glucagon The child was normotensive, had a cheerful release. Most somatostatinomas in NF I isposition, average intelligence and showed are duodenal in location(3). Duodenal

INDIAN PEDIATRICS 185 VOLUME 42__FEBRUARY 17, 2005 LETTERS TO THE EDITOR somatostatinomas are rare neuroendocrine octreotide therapy is a newer modality for generally non- functioning well differentiated diagnosis and treatment. tumors with a low grade of malignancy and Zeeba Zaka-ur-Rab, are less frequently metastatic(4). These rare Kamlesh Chopra neoplasms have been reported only in adults and old patients. In contrast to pancreatic Correspondence to: tumors, duodenal tumors are seldom Dr. Zeeba Zaka-ur-Rab, associated with ‘somatostatin syndrome’ Assistant Professor, (diabetes mellitus, diarrhea, and chole- Department of Pediatrics, lithiasis). They are smaller in size, and give Himalayan Institute of Medical Sciences, rise to symptoms like abdominal pain or lump, Swami Ram Nagar, Dehradun 288 140, gastrointestinal hemorrhage and obstructive Uttaranchal, jaundice(3). A case has, however, been India. reported where partial somatostatinoma REFERENCES syndrome was found along with duodenal 1. Conference Report. Neurofibromatosis. carcinoid(5). In yet another case all the 3 Conference statement. National institute of features of somatostatin syndrome were health consensus development conference. Arch Neuro1, 1998; 45: 575-578. present in association with the same tumor. Most tumors located in the head of pancreas 2. Sauremann P, Binswanger R, Maurer R, are malignant with metastasis to liver. Stamm B, Hegglin J. Somatostatin- producing Pancreatic tumors and metastasis are equally endocrine pancreatic tumor in Reckling- hausen’s neurofibromatosis. Case report and well detected by CT and MRI. They are echo literature review. Schweiz Med Wochenschr poor and can sometimes be missed on USG 1987; 117: 11341139. (especially those located in the tail). 3. Cohen C, Heymann MF, Michenet P, Endoscopic evaluation is often required Memeteau F, Saint-Marc O, Emy P, Maitre F, for detection of duodenal carcinoids as they Le Bodic M F. Duodenal somatostatinomas can be missed on MRI or other radiographic associated with von Recklinghausen’s neurofibromatosis. Apropos of 2 cases. Ann techniques. Clinical and hormonal features of Pathol 2000; 2: 609-611. somatostatinoma syndrome are not requisite for diagnosis, analysis of plasma somatostatin 4. Green BT, Rocky DC. Duodenal somato- statinoma presenting with complete somato- immunoreactivity might lead to a higher statinoma syndrome. J Clin Gastroenterol detection rate of the tumor. Hyper- 2001; 33: 415-417. somatostatinemia demonstrated by Calcium- pentagastrin test or tolbutamide test is very 5. Swinbum BA, Yeong ML, Lane MR, Nicholson GI, Holdaway IM. Neuro- useful for diagnosis. Tumor is further fibromatosis associated with somatostatinoma: confirmed by immunohistochemical studies. a report of two patients. Clin Endocrinol (Oxf) Somatostatin receptor scintigraphy and 1998; 28: 353-359.

INDIAN PEDIATRICS 186 VOLUME 42__FEBRUARY 17, 2005