Forty-Seven Children Suffering from Chronic Myeloid Leukemia at a Center Over a 25-Year Period

To determine the diagnostic characteristics, blast crisis status, appropriate treatment, overall patient survival, and prognostic factors pertaining to chronic myeloid leukemia (CML) for children, the authors reviewed 47 children under 18 years of age with CML who were admitted to the National Taiwan University Hospital in the period between 1976 and 2001 inclusively. Between 1976 and 1985, ten patients were diagnosed by clinical manifestation without the application of any cytogenetic study, while from 1986 to 2001, 37 patients were diagnosed as evidenced by the presence of a positive Philadelphia chromosome upon cytogenetic examination. The male-to-female ratio was 0.7 (14 boys/20 girls) for patients under 15 years of age. Abdominal fullness or pain appeared to be the most common presenting symptom, followed by body weight decline and bleeding. Marked leukocytosis, anemia, and thrombocytosis were common laboratory findings in these patients. The incidence of only lymphoid blast crisis was 38%, which appears to be the highest incidence when compared to the corresponding value as reported in other studies. Of 25 children treated with conventional chemotherapy including busulfan and hydroxyurea, the median survival period from diagnosis was 44 and 42 months, respectively. Between January 1984 and December 2001, 22 affected children underwent allogeneic stem-cell transplantation (SCT). The 5-year survival probability following hematopoietic SCT from matched sibling donors is 65%, and 22% following SCT from matched unrelated donors. This difference is statistically significant ( p = .01).     

Functional Abnormalities of Neutrophils in Childhood Acute Leukemia: Decreased Myeloperoxidase and 13-Glucuronidase Contents and Their Release

The myeloperoxidase and β-glucuronidase contents of neutrophils as total index (T index) and their release induced by formyl-methionyl-leucyl-phenylalanine (FMLP) as stimulation release index (SR index) and percent enzyme release index (%ER index) were assayed in children with acute leukemia at various stages. T indexes and SR indexes of these enzymes and %ER index of p-glucuronidase were significantly decreased at onset or relapse (P相似文献   

Intussusception in a premature neonate: A rare and often misdiagnosed clinical entity

Intussusception is a very rare cause of intestinal obstruction in neonates. It is of extremely rare occurrence among premature neonates. We present a case of 11-day-old premature neonate who presented with abdominal distension, intolerance to feeds, vomiting, significant bilious aspirate and bleeding per rectum. The initial diagnosis of necrotizing enterocolitis (NEC) led to a delay in the diagnosis. On exploratory laparotomy, it turned out to be a case of ileo-colic intussusception with Meckel''s diverticulum as a lead point. This site of intussusception (ileo-colic) and presence of a lead point among premature neonate is of exceedingly rare occurrence and very few such cases have been reported.In this article, the published work about clinical features and management on intussusceptions in premature neonates has been reviewed. The authors intend to highlight the difficulty in distinguishing the NEC and intussusception. Subtle clinical and radiological features which can help in differentiating the two conditions have been emphasized. This can avoid the delay in diagnosis and management which can prove critical. High index of suspicion with timely intervention is the key for optimizing outcome. A diagnosis of intussusception should always be considered in any preterm infant with suspected NEC.     

Hirschsprung's Disease: a Clinical and Pathologic Study in Iranian Constipated Children

Objective

Hirschsprung''s disease (HD) is a complex disorder resulting from absence of ganglion cells in the bowel wall leading to functional obstruction and bowel dilatation proximal to the affected segment. The aim of our study was to evaluate rectal biopsies from constipated children in different age groups to see in which age it is more likely to encounter HD to avoid unnecessary rectal biopsy.

Methods

Records of all children with chronic constipation undergoing a rectal biopsy to exclude HD were obtained from the files of Children''s Medical Center in Tehran, Iran. A detailed retrospective demographic review, including age of beginning of signs and symptoms was made of all cases.

Findings

Totally, 172 biopsies were taken from 168 children in a five year period, of which 127 cases (75%) had HD. The mean age of constipated patients at biopsy was 39 months and the mean age of patients with proven HD was 18 months. Males were affected more than females. Congenital anomalies associated with HD were found in 9.6%. In 85 (91%) cases constipation had begun in neonatal period.

Conclusion

Our data supports previous studies that if constipation begins after the neonatal period, the child is unlikely to have HD. In neonates delay in meconium passage is the most important clinical sign of HD.     

Treatment of childhood acute myelogenous leukemia with allogeneic and autologous stem cell transplantation during the first remission: a report from the Kyushu-Yamaguchi Children's Cancer Study group in Japan

A total of 64 newly diagnosed acute myelogenous leukemia patients (except FAB M3 and/or Down syndrome) under 18 years of age were consecutively enrolled into the study. Patients having an HLA-identical sibling (allo group) were assigned to undergo allogeneic bone marrow transplantation (allo BMT) in the first complete remission (CR). Others (non-allo group) were assigned to undergo autologous peripheral blood stem cell transplantation (PBSCT) or autologous BMT (auto BMT). Conditioning regimen was busulfan + melphalan for all transplantation. Of 64 patients (allo group 24; non-allo group 40), 59 (92.2%) achieved a CR. Eighteen relapses occurred (allo group 4; non-allo group 14) and 6 died during the first CR. The 5-year event-free survival (EFS) rate was 53.3 +/- 6.4% at a median follow-up period of 45 months. The 5-year EFS rates of allo and non-allo groups were 70.8 +/- 9.3% and 43.0 +/- 8.1%, respectively (p = .08). The EFS rates at 5 years post-transplant for allo BMT from an HLA-identical sibling (n = 18), PBSCT (11), and auto BMT (6) were 88.1 +/- 7.9%, 41.6 +/- 19.7%, and 83.3 +/- 15.2%, respectively. The outcome of allo BMT was superior to that of autograft. Auto BMT rather than PBSCT might contribute to a long-term survival in case of no available HLA-identical siblings.     

Trauma to the bones of small infants from passive exercise: a factor in the etiology of child abuse

Four infants, three premature and one term, developed serious bony injury from parent- or caretaker-administered passive exercises. In the three infants born prematurely, the exercise program was prescribed by neonatal intensive care unit staff before discharge; in the fourth infant, the babysitter initiated the program. In each case the passive exercise was begun to diminish actual or presumed muscle tightness. When these infants were presented to the physician with a serious traumatic injury between the ages of 4 and 10 months, the presumptive diagnosis of child abuse was made after radiologic assessment revealed multiple bone injuries. The several traumatic injuries to bones resulting from the home-administered physical therapy were most impressive. Passive exercise in three of the four infants was a significant factor in the cause of their injuries. Caution must be used in prescribing this form of "therapy" in small infants.