Multiple endocrine syndrome type IIb in early childhood

Article Abstract:

Multiple endocrine neoplasia (MEN) is a set of inherited disorders in which a genetic defect leads to the development of tumors in several different glands. MEN is divided into types based on the specific glands affected. In type II MEN, the patient develops thyroid cancer, pheochromocytoma, which is a cancer of the adrenal medulla, and hyperparathyroidism. Type IIb MEN is a particularly nasty form of MEN which develops early; the cancers can spread before a child reaches the age of four years. Generally, the disorder is diagnosed early in life only if a family history of MEN has already been established. Researchers now report the case of a girl born to a family without a history of MEN type IIb but in whom the disorder was first suspected at three weeks of age. The patient was born with a club foot. After birth, the infant failed to thrive, and examination revealed the presence of peripheral nerve tumors in the rectum. The combination of skeletal abnormalities, failure to thrive, and rectal neuromas first led to the suspicion of MEN. Measurements of the thyroid hormone calcitonin often reveal the presence of the thyroid medullary cancer, but in the present patient, calcitonin levels at five weeks of age were elevated above average, but not beyond the normal range. A thorough examination at three months of age uncovered further signs consistent with MEN type IIb, but the findings were inconclusive. Again, the calcitonin levels were not outside the normal range. Calcitonin levels, which trigger bone building, are higher in children than adults, and decrease with age. About 14 months after birth, it was determined that the calcitonin levels in this patient were rising, not falling. The patient's thyroid gland was removed, and microscopic examination confirmed the suspicion of thyroid medullary cancer. The patient's calcitonin levels have decreased, and she is now three years old with no evidence of recurrent cancer in her neck. She must be monitored closely, however, for signs of pheochromocytoma, another cancer for which this patient is at exceptionally high risk. (Consumer Summary produced by Reliance Medical Information, Inc.)

Case studies, Adenomatosis, Familial endocrine, Multiple endocrine neoplasia, Thyroid cancer

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Cancer and other causes of childhood mortality in Bombay, India

Article Abstract:

Cancer is less important as a cause of death among children in developing nations than it is in the West. However, as improvements are made in infant care and as progress is made in the control of infectious diseases, the importance of cancer as a cause of childhood mortality will rise in these countries. The contribution of cancer to deaths among children in India has been tabulated. Compared with Western nations and Africa, childhood cancer in India is rare. About 5 percent of male cancer patients in India are under the age of 15 years, as are 3 percent of female cancer patients. In India, between 19 and 38 percent of all deaths in children under 15 years are due to complications of prematurity. Pneumonia runs a close second, accounting for 19 to 27 percent of childhood deaths. Diarrhea and dysentery, always a serious threat in developing nations, accounted for 10 to 12 percent of the deaths in this study, which was conducted in Greater Bombay. The deaths of 0.8 to 1.1 percent of the boys resulted from cancer, compared with the deaths of 0.5 to 0.6 percent of the girls. The survival rates of childhood cancer victims are more difficult to determine, as it is common for these patients to be lost to long-term follow-up in Bombay. However, indications are that the rate of cancer deaths is declining for children under five years of age, and for some other groups of children as well. The cancer death rate is increasing, however, among boys from 10 to 14 years old. Although cancer is far from the most significant cause of childhood mortality in India, the data tabulated in the present study suggest that further spending to improve cancer care would be appropriate, even in a country with very limited health care resources. (Consumer Summary produced by Reliance Medical Information, Inc.)

Health aspects, Patient outcomes, Mortality, Children, India, Cancer in children, Childhood cancer, Child health

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