Jan Watterson

Looking beyond where children die : Determinants and effects of planning a child's location of death.

While dying at home may be the choice of many, where people die may be less important than argued. We examined factors associated with parental planning of a childs location of death (LOD) and its effects on patterns of care and parents experience. In a cross-sectional study of 140 parents who lost a child to cancer at one of two tertiary-level U.S. pediatric hospitals, 88 (63%) planned the childs LOD and 97% accomplished their plan. After adjusting for disease and family characteristics, families whose primary oncologist clearly explained treatment options during the childs end of life and who had home care involved were more likely to plan LOD. Planning LOD was associated with more home deaths (72% vs. 8% among those who did not plan, P<0.001) and fewer hospital admissions (54% vs. 98%, P<0.001). Parents who planned were more likely to feel very prepared for the childs end of life (33% vs. 12%, P=0.007) and very comfortable with LOD (84% vs. 40%, P<0.001), and less likely to have preferred a different LOD (2% vs. 46%, P<0.001). Among the 73 nonhome deaths, planning was associated with more deaths occurring in the ward than in the intensive care unit or other hospital (92% vs. 33%, P<0.001), and fewer children being intubated (21% vs. 48%, P=0.029). Comprehensive physician communication and home care involvement increase the likelihood of planning a childs LOD. Opportunity to plan LOD is associated with outcomes consistent with high-quality palliative care, even among nonhome deaths, and thus may represent a more relevant outcome than actual LOD.

Excessive spinal cord toxicity from intensive central nervous system- directed therapies

Background. Intrathecal chemotherapy, radiation therapy, and systemic chemotherapy are used for both prophylaxis and treatment of central nervous system (CNS) disease in hematologic malignancies. Twenty‐three cases of myelopathy that occurred in patients who received intensive CNS‐directed therapy were evaluated to identify the determinants of this severe CNS toxicity.

Central nervous system atypical teratoid tumor/rhabdoid tumor: response to intensive therapy and review of the literature.

Central nervous system atypical teratoid/rhabdoid tumor (ATT/RT) of infancy and childhood is a unique histologic entity with an extremely aggressive natural history. Standard therapy for infant and childhood medulloblastoma, for which this entity is often mistaken, has been ineffective; most children survive less than 12 months after diagnosis. Intensified therapy has been recently used for children with this disease, with promising results [1,2].We report four cases of ATT/RT in young children; all had subtotal resections and localized disease at diagnosis. One child treated prior to bone marrow transplant availability died of progressive disease 9 months after diagnosis. Another child, treated with high-dose chemotherapy and radiotherapy in preparation for bone marrow transplant, had a recurrence and died 20 months after diagnosis, without undergoing the transplant. Two children received high-dose chemotherapy and autologous bone-marrow transplant and had a good response to treatment; one survived 19 months, the other child is free of disease 46 months from diagnosis. Intensified therapy has altered the natural history of central nervous system ATT/RT.

Jaundice as a paraneoplastic phenomenon in a T-cell lymphoma.

An adolescent male developed severe unexplained cholestatic jaundice 3 mo before diagnosis of mediastinal non-Hodgkins lymphoma (T-cell, late thymic phenotype). There was no anatomic obstruction to bile flow, no evidence for an infectious etiology, and no neoplastic involvement of the liver or bile ducts. A paraneoplastic phenomenon is postulated because the jaundice resolved after treatment of the lymphoma. We suggest that occult lymphoma must be added to the differential diagnosis of unexplained intrahepatic cholestasis.

Retinal toxicity associated with cisplatin and etoposide in pediatric patients

Cisplatin is an effective chemotherapeutic agent used in the treatment of many pediatric solid tumors. Retinal toxicity is a side effect of the drug reported in adults, but is not well described in pediatric patients. We present the cases of two children treated with cisplatin and etoposide who experienced retinal toxicity documented by visual evoked response (VER) and electroretinogram (ERG). significantly, both patients had abnormal renal function. The mechanism of visual toxicity induced by cisplatin is unknown but may result from central nervous system (CNS) accumulation of drug after repeated doses, especially with high-dose platinum (HDP) containing regimens. Because clearance of platinum is related to adequate renal-function, patients with any decrease in glomerular filtration rate (GFR) may have delayed platinum excretion. We propose that the patients at greatest risk of cisplatin-induced toxicity are those pretreated with nephrotoxic therapy or those with impairment of renal function from other causes. These patients should have prospective ophthalmologic evaluation especially when treated with HDP containing regimens.

Autoimmune Neutropenia of Infancy and Early Childhood

Forty-one children were identified with autoimmune neutropenia of infancy and early childhood (absolute neutrophil count [ANC] less than 500/microliters and demonstrable serum antineutrophil antibodies). There were 21 boys and 20 girls; the median age at diagnosis was 11 months (range 5-38 months). No life-threatening infections occurred. There was a gradual upward trend in ANC in all patients over many months, with 87% having an ANC > 1000/microliters by 24 months from diagnosis. Among various clinical and laboratory parameters analyzed statistically, only younger age at diagnosis was associated with earlier neutrophil recovery. There was no association between degree or duration of neutropenia and sex, race, antibody reactivity, or presence of serious illness at diagnosis.

A complete ileo-tumor-colic fistula in a child with Burkitt's lymphoma

A boy with abdominal Burkitts lymphoma developed rectal bleeding, abdominal distension, pain, and fever three weeks after diagnosis. Imaging studies revealed a necrotic tumor mass allowing a fistulous pathway from the ileum to the proximal colon. A laparotomy was performed, with resection of the large necrotic tumor. The child recovered, and has had no further evidence of Burkitts disease. The radiological evaluation performed in this case ensured proper medical management and surgical intervention during a life-threatening event. the possibility of bowel perforation as a complication of therapy for abdominal lymphoma must be recognized.