Pediatric oncology situation analysis (Jordan).

This article highlights the current situation of pediatric oncology in Jordan by reviewing the available population based data from the surveillance. Cancer among children aged less than 15 years make up 4 to 6% of all registered new cancer cases in all ages each year. The major types of cancer in females for the year 2008 are leukemia 32.2%, central nervous system 18.8%, skeletal and soft tissue 8.8%, lymphomas 5.5%, and sympathetic 5.5%, whereas in males, they are leukemia 28.1%, central nervous system 18.8%, lymphomas 16.2%, skeletal and soft tissue 7% and sympathetic 6.3%. Children with cancer receive treatment through pediatric oncology wards in King Hussein Cancer Center (KHCC) and Royal Medical Services hospital or through pediatric departments in King Abdullah university Hospital and Princes Rahma hospital with full government coverage of the treatment expenses. Currently the pediatric palliative care program is limited to KHCC which provides inpatient care, and out-patients and drop-in care on a daily basis to provide symptom management, counseling, and other services as appropriate. The lack of palliative care in other hospital and pediatric home care coverage stress the need to develop special plan of action to initiate these services.

Mediterranean glucose-6-phosphate dehydrogenase (G6PD(C563T)) mutation among Jordanian females with acute hemolytic crisis.

OBJECTIVE: To evaluate the G6PD(C563T) Mediterranean mutation among Jordanian females who were admitted to Princess Rahma Teaching Hospital (PRTH) with/or previous history of favism. STUDY DESIGN: A descriptive study. PLACE AND DURATION OF STUDY: Jordanian University of Science and Technology and PRTH, from October 2003 to October 2004. METHODOLOGY: After obtaining approval from the Ethics Committee of Jordanian University of Science and Technology, a total of 32 females were included in this study. Samples from 15 healthy individual females were used as a negative control. Blood samples from these patients were collected and analyzed by allele-specific polymerase chain reaction (AS-PCR) to determine the G6PD(C563T) mutation. RESULTS: Twenty one out of 32 patients were found to be G6PD(C563T) Mediterranean mutation (65.6%) positive. Three out of 21 patients were homozygous and remaining 18 were heterozygous for G6PD(C563T) Mediterranean mutation. Eleven (34.4%) out of 32 patients were found to be negative for G6PD(C563T) mutation indicating the presence of other G6PD mutations in the study sample. CONCLUSION: G6PD(C563T) Mediterranean mutation accounted for 65.6% of the study sample with favism in the North of Jordan. There is likely to be another G6PD deficiency variant implicated in acute hemolytic crisis (favism).

Pubertal evaluation of adolescent boys with beta-thalassemia major and delayed puberty.

OBJECTIVE: To examine the hormonal status of the hypothalamic-pituitary-gonadal axis in adolescent males with beta-thalassemia major. DESIGN: Controlled clinical study. SETTING: Tertiary referral teaching hospital. PATIENT(S): Thirty-three adolescent males with beta-thalassemia major. INTERVENTION(S): Basal LH, FSH, and T were examined. All individuals received 100 microg GnRH analogue. Four hours later the hormone levels were retested. Patients with beta-thalassemia and low T levels received hCG. MAIN OUTCOME MEASURE(S): The preintervention and postintervention levels of FSH, LH, and T were examined. RESULT(S): Of the 33 beta-thalassemia major adolescents, 17 had delayed puberty. The difference in basal LH, FSH, and T levels between delayed and normal puberty beta-thalassemia groups were statistically significant. These levels were significantly lower compared with the constitutional delayed puberty group and become even more significant after GnRH analogue administration. The T levels in the beta-thalassemia group were significantly lower than in the control group. After hCG administration, the T levels remained significantly lower in the delayed-puberty beta-thalassemia compared to the normal-puberty beta-thalassemia group. CONCLUSION(S): Despite recent therapeutic advances in the management of beta-thalassemia major, the risk of secondary endocrine dysfunction remains high. Hypogonadism is one of the most frequent endocrine complications.