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PubMed1989

Pediatric AIDS/HIV infection: an emerging challenge to pediatric psychology.

The number of pediatric AIDS cases are increasing. This disease, with its social and political ramifications, as well as its biological consequences, presents a number of unique issues for psychologists. Some of the areas that present opportunities and problems for psychologists are briefly identified. These areas include prevention, clinical issues, public education, research, neuropsychological effects, psychoneuroimmunological issues, and ethical concerns. Current epidemiological projections and future directions for research are also discussed.

Acquired Immunodeficiency Syndrome↗
PubMed1992

An appraisal of pediatric liver transplantation from living relatives. Initial clinical experiences in 20 pediatric liver transplantations from living relatives as donors.

The authors performed 20 liver transplantations from living related donors between June 1990 and July 1991. The 20 pediatric patients (14 biliary atresia, two Budd-Chiari syndrome, one liver cirrhosis after hepatitis C viral infection (HCV hepatitis), 1 progressive intrahepatic cholestasis, 1 liver cirrhosis, 1 protoporphyria) were transplanted with 11 left lobes, eight left lateral segments, and one right lobe. The choice of donors was restricted to the parents of the recipients. The immunosuppressive treatment consisted of FK 506 and steroids. Seventeen recipients are alive, 15 of whom are well and at home. Two recipients, who underwent emergency transplantation, died of postoperative complications. Another recipient died of accidental asphyxia at 6 months after the transplantation. All 20 donors had uneventful postoperative courses and were able to resume their normal social lives. The arterial ketone body ratio (AKBR) increased to above 1.0 within 2 days after the transplantation in all cases. Relatively mild rejection episodes were encountered in only two cases transplanted with ABO-compatible grafts, and these were treated successfully with steroids and FK 506.

Adolescent↗
PubMed2000

Pharmacokinetic and metabolic investigations of mycophenolic acid in pediatric patients after renal transplantation: implications for therapeutic drug monitoring. German Study Group on Mycophenolate Mofetil Therapy in Pediatric Renal Transplant Recipients.

The need for mycophenolic acid (MPA) monitoring is still under discussion. Key issues for the PK/PD relationships of this drug are: the role of metabolites, the usefulness of AUC versus predose levels, and the need to monitor the free concentration of MPA (f-MPA). Recent advances have revealed that, in addition to 7-O-MPAG, three additional MPA metabolites are present in the plasma of transplant recipients. One of these metabolites (M-2), identified as an acyl glucuronide of MPA, was found to inhibit IMPDH-II in vitro. This active metabolite was also found to cross-react in the Emit assay for MPA. In an ongoing multicenter study, the authors are evaluating the relevance of monitoring total (t-MPA) and free mycophenolic acid (f-MPA) in pediatric renal transplant recipients. As in adults, a time-dependent increase of t-MPA-AUC(0-12h) within the first 3 months posttransplant (35 versus 64 mg x h/L, [corrected] 3 weeks versus 3 months respectively; daily dosage: 0.6 g/m2 bid) was seen. Receiver operating characteristics curve analyses were used to test the ability of predose levels or AUC(0-12h) to discriminate between cases with no complications and those with acute rejection, adverse events (severe infections, leukopenia), or gastrointestinal disorders observed during the early posttransplant course. In agreement with observations in adults, a significant (p = 0.001) association was observed between AUC(0-12h) and acute rejection. A t-MPA-AUC(0-12h) of approximately 30-60 mg x h/L [corrected], as determined by HPLC, seems to be a reasonable target for the early posttransplant period. It remains to be elucidated whether regular predose level monitoring may be of more practical value. A higher incidence of rejection was observed at predose MPA concentrations < or = 1 mg/L, as measured by HPLC. In contrast to t-MPA, f-MPA-AUC(0-12h) was significantly related to severe infections and leukopenia. The risk for severe adverse events was increased at f-MPA- AUC(0-12h) values > or =600 microg x h/L [corrected]. On the basis of these data and the observed variability in the pharmacokinetics of MPA, the development of monitoring strategies for this drug appears to be promising.

Child↗
PubMed1996

Clinical correlates of chronic rejection in pediatric renal transplantation. A report of the North American Pediatric Renal Transplant Cooperative Study.

A total of 1699 patients with living donor transplants and 1795 patients with cadaver donor transplants entered between January 1987 and November 1994 in the North American Pediatric Renal Transplant Cooperative Study registry form the cohort for this report. Failure from chronic rejection occurred in 76 (4.5%) of living donor transplants and 149 (8.3%) of cadaver transplants. Chronic rejection is the leading cause of graft failure, with 27% of living donor and 26.7% of cadaver donor graft failures attributed to chronic rejection. Univariate and multivariate analyses of various risk factors revealed acute rejection (relative risk [RR]=3.1, P<0.001) and greater than two acute rejections (RR=4.3, P<0.001)to be the most common correlates of chronic rejection. Additionally, late initial acute rejection (>365 days; RR=2.3, P<0.001) was also correlated. Cadaver donor source (RR=1.6, P=0.001) and African-American race (RR=1.6, P<0.003) were weaker but significant risk factors. An analysis of cyclosporine dosing revealed tht a dose of <5mg/kg/day at day 30 was also a risk factor (RR=1.5, P=0.027). Our study concludes that acute rejection is the single most critical element in the genesis of chronic rejection. Thus, measures to prevent the first episode of acute rejection could ameliorate the incidence of chronic rejection.

Adolescent↗
PubMed2001

Posttransplant diabetes mellitus in pediatric renal transplant recipients: a report of the North American Pediatric Renal Transplant Cooperative Study (NAPRTCS).

BACKGROUND: The incidence of renal post transplant diabetes mellitus (PTDM) in adults varies from 3-46%. METHODS: We did a retrospective analysis of 1365 children in The North American Pediatric Renal Transplant Cooperative Study with renal transplant (Tx) reported between January 92 and July 1997. PTDM, defined as >2 weeks of insulin therapy after Tx, developed in 36 patients. A control group of 153/1329 non-PTDM patients was selected and matched for age at Tx and primary diagnosis. RESULTS: African-Americans were overrepresented (36.1 vs. 17.6%, P=0.017) and Hispanics were underrepresented (5.6 vs. 26.1%, P=0.019) among cases. Although prednisone dose 30 days post-Tx was higher among cases (0.89 mg/kg/day) versus controls (0.71 mg/kg/day), P=0.019, cyclosporine dose was similar. No differences in prednisone or cyclosporine doses were observed at 6, 12, or 24 months post-Tx. Tacrolimus use in PTDM group was high (45%). The estimated incidence of first acute rejection at 1, 3, and 12 months was higher among cases, 0.41+/-0.08, 0.52+/-0.08, 0.61+/-0.08, compared to controls, 0.23+/-0.02, 0.37+/-0.02, and 47+/-0.02 (P=0.058). Crude graft failure rates of 13.5% (5/36) and 12.4% (19/153) were similar between the two groups, so was the calculated creatinine clearance at 12 and 24 months and post-Tx hospitalization days. CONCLUSION: PTDM occurs in <3% of children. African-Americans are at higher risk and Hispanics at lower risk for PTDM. Tacrolimus is a significant risk factor for PTDM. Children with PTDM had a higher incidence of acute rejection, but graft survival, kidney function, and hospitalization rates were similar to selected controls.

Adolescent↗
PubMedDate not supplied

Topotecan in pediatric patients with recurrent and progressive solid tumors: a Pediatric Oncology Group phase II study.

PURPOSE: A phase II study was designed to determine the efficacy of topotecan, an inhibitor of topoisomerase I, in the treatment of patients with progressive or recurrent pediatric extracranial solid tumors (STs). PATIENTS AND METHODS: Patients younger than 21 years at the time of initial diagnosis with refractory STs were treated with 2 mg/m2 topotecan given by 30-minute infusions for 5 days repeated every 3 weeks. Granulocyte colony stimulating factor (G-CSF) was added to the regimen only after occurrence of severe neutropenia or therapy delay due to sustained neutropenia. RESULTS: One hundred forty-one patients were treated with 539 courses of topotecan. Responses were seen in 34 patients (3 had complete responses [CRs], 2 had partial responses [PRs], and 24 had minor responses [MRs] or stable disease [SD]). The number of administered courses in patients with SD varied between 5 and 24, with a median of 10. The median time on the study for patients with SD was approximately 8.5 months. In patients without bone marrow involvement, the most frequent toxicity was myelosuppression: hemoglobin < 8 g/dl in 83 of 341 courses, absolute granulocyte count < 1,000/microl in 221 of 341 courses, and platelet count < 50,000/microl in 162 of 341 courses. Nausea and vomiting were infrequent; many patients were pretreated with ondansetron or granisetron. A recurrent rash developed in 16 patients and was usually well controlled with diphenhydramine and hydrocortisone. G-CSF was administered in 203 of 539 courses because of neutropenia. Therapy was delayed over 1 week in 33 instances. CONCLUSION: In previously treated patients, topotecan produced CRs and PRs in patients with neuroblastoma, Ewing's tumor, and retinoblastoma. In hepatoblastoma, rhabdomyosarcoma, and a few rare tumors, long-lasting MRs and SDs with excellent symptom control were seen. The toxicity of topotecan, predominantly myelosuppression, was tolerable.

Adolescent↗
PubMed2005

Pediatric urolithiasis: 15 years of local experience with minimally invasive endourological management of pediatric calculi.

PURPOSE: We sought to identify whether changes in technology and local practice have improved outcomes in the minimally invasive management of pediatric stone disease. MATERIALS AND METHODS: We reviewed retrospectively case notes and imaging from 1988 to 2003, noting treatment modality, stone-free rates, ancillary therapy and complications. RESULTS: A total of 122 children (140 renal units) with a mean age of 7.7 years underwent 209 extracorporeal shock wave lithotripsy (SWL) sessions. Stone size ranged from 6 to 110 mm. Stone-free rates were 84% for cases involving stones smaller than 20 mm, and 54% for those involving stones 20 mm or greater. For complex calculi 40% of patients were stone-free and 45% required ancillary procedures, with an overall complication rate of 26%. A total of 37 children (43 renal units) with a mean age of 6.4 years underwent 46 percutaneous nephrolithotomies (PCNLs). Stone size ranged from 8 to 155 mm. The overall stone-free rate was 79%. Of these patients 34% required ancillary procedures, with a major complication rate of 6%. A total of 35 children (35 renal units) with a mean age of 5.9 years underwent 53 ureteroscopies. Holmium laser was the most effective treatment modality in this group, with a 100% stone-free rate and no complications. CONCLUSIONS: For most renal stones smaller than 20 mm SWL was the most effective primary treatment modality. There was no statistical difference between the 2 lithotriptors for stone-free or ancillary procedure rate. The stone-free rate was dependent on stone size rather than type of lithotriptor. For renal stones 20 mm or greater and staghorn calculi we switched from SWL to PCNL as primary treatment, as stone-free rates were higher and the ancillary procedure and re-treatment rates were lower with PCNL. Electrohydraulic lithotripsy and pulse dye laser were initially used to treat ureteral stones. However, with the introduction of holmium laser technology we achieved higher stone-free rates and lower complication rates. Holmium laser lithotripsy is now used as a primary treatment modality for ureteral stones.

Adolescent↗
PubMed2006

The Pediatric AIDS Severity Score (PASS): a multidimensional AIDS-severity adjustment for pediatric HIV infection.

BACKGROUND: A severity staging system predictive of mortality for perinatally HIV-infected children is needed for clinical and research purposes. METHODS: A pediatric AIDS severity score (PASS) was developed using baseline sociodemographic, clinical, immunologic, and functional measures obtained from 786 perinatally HIV-infected children enrolled into a prospective cohort study (PACTG 219) in the pre-highly active antiretroviral therapy (HAART) era (pre-1996). PASS was then validated among 392 perinatally HIV-infected children randomly sampled from the original source population (n = 1178). Survival estimates and hazard ratios (HRs) were obtained using the Kaplan-Meier method and proportional hazards models, respectively. The most predictive models were determined using Harrell's "C" statistic. RESULTS: Overall survival was 95% and 90% at 1 and 2 years of follow-up, respectively. The most comprehensive model for predicting mortality, termed the "Full" PASS, included CD4% <15 (HR = 3.9), CDC category C (HR = 2.6), BMI <10% (HR = 2.4), a low (<70) neuropsychological score (HR = 2.6), a general health rating <5 (HR = 2.4), and an elevated symptoms score (HR = 1.9). These determinants were highly predictive of mortality (C statistic = 0.841). CONCLUSIONS: PASS will be helpful in assessing the effectiveness of ART among children with HIV infection, particularly when randomized clinical trials are not possible due to ethical and feasibility concerns.

Acquired Immunodeficiency Syndrome↗
PubMed2003

An analysis of pediatric gunshot wounds treated at a Level I pediatric trauma center.

BACKGROUND: We reviewed the incidence and injury severity of children with gunshot wounds (GSWs) at our hospital. METHODS: We compared trauma registry, emergency unit (EU), and admissions data from January 1993 to June 1996 (period I) and July 1996 to December 1999 (period II). Outcome measures included EU disposition (death, immediate operation, pediatric intensive care unit, ward), injury severity, mortality, and injury cause (accidental, intentional). RESULTS: We treated 437 children for GSWs in the EU, with 238 (54%) admissions and 199 discharges. Comparing period I versus period II, patients treated declined from 288 to 149 (-52%, p < 0.001), and admissions decreased from 159 to 79 (-50%, p < 0.001). Injury severity increased from 35% to 57% (p < 0.001). Patients requiring immediate operations increased from 20% to 42% (p < 0.001). Direct ward admissions declined from 65% to 43% (p < 0.001). Deaths occurred in 3% of patients in both time periods. Accidental and intentional GSWs were evenly divided. CONCLUSION: An alarming number of children, an average of 62 children annually, were treated for GSWs at our hospital. Despite a 52% reduction in GSWs, the percentage of severely injured patients increased by 63%. These data emphasize the importance of prevention, education, early assessment, and operative treatment.

Adolescent↗
PubMed2004

Studies of Pediatric Liver Transplantation: 2002 update. An overview of demographics, indications, timing, and immunosuppressive practices in pediatric liver transplantation in the United States and Canada.

Studies of Pediatric Liver Transplantation (SPLIT) was initiated in 1995 for the purpose of collecting comprehensive data from children undergoing liver transplantation. As of May 31, 2002, 1761 children were registered in SPLIT from 38 participating centers in the United States and Canada. This report focuses on the demographics, primary diagnoses, clinical indications for transplant, and probability of obtaining liver transplantation for the 1187 children receiving a liver transplant after registration in SPLIT. Demographic information is also provided for the 1092 children who received their first ever liver transplantation. For this cohort, we also describe immunosuppressive practices at the time of transplant, and how the use of different medications changes with time.

ABO Blood-Group System↗
PubMed2004

Pediatric en bloc dual kidney-pancreas transplantation into an adult recipient: a simplified technique. Benefits of the en bloc kidney-pancreas transplantation technique in pediatric donors.

The lower age limit for pancreas donors is not well defined. Fear of inadequate islet beta-cell mass and of technical complications has hampered the use of pediatric donors. A surgical technique of 'en bloc' kidney-pancreas is described. Both kidneys and pancreas were removed en bloc from a 13-kg, 31-month-old child. During bench preparation, one anastomosis was performed between the portal vein and the inferior vena cava. The proximal end of the aorta was closed. The bloc was transplanted into a 36-year-old type I diabetic patient intraperitoneally in the right iliac fossa. The kidneys functioned immediately. Pancreatic graft function resumed after POD 15 but insulin therapy was maintained until POD 112. Currently, the patient retains excellent kidney and pancreas graft functions. Very young donors can be accepted as pancreas donors for adult recipients, although slow recovery of pancreatic function can be expected. Use of the en bloc technique is well suited for very small children, as it prevents potential vascular complications.

Adult↗
PubMed1997

Study of use of cefdinir versus cephalexin for treatment of skin infections in pediatric patients. The Cefdinir Pediatric Skin Infection Study Group.

Three hundred ninety-four patients, aged 6 months to 12 years, entered a multicenter, randomized, controlled, investigator-blind study comparing cefdinir, 7 mg/kg of body weight twice a day, with cephalexin, 10 mg/kg four times a day, each given for 10 days. The most common infections treated were impetigo and secondary infection of preexisting dermatitis. The most common pathogens isolated were Staphylococcus aureus and Streptococcus pyogenes. Two hundred thirty-one patients were microbiologically evaluable. Microbiologic eradication rates were 164 of 165 pathogens (99.4%) in the cefdinir group and 152 of 156 pathogens (97.4%) in the cephalexin group (P = 0.14). Clinical cure rates were 116 of 118 patients (98.3%) in the cefdinir group and 106 of 113 patients (93.8%) in the cephalexin group (P = 0.056). Sixteen percent of cefdinir patients and 11% of cephalexin patients experienced adverse events (P = 0.11), the most common being diarrhea, which affected 8% of the cefdinir group and 4% of the cephalexin group. Cefdinir appears to be an effective and well-tolerated agent for the treatment of uncomplicated skin and skin structure infections in pediatric patients.

Cefdinir↗
PubMed1996

Inadvertent atrioventricular block during radiofrequency catheter ablation. Results of the Pediatric Radiofrequency Ablation Registry. Pediatric Electrophysiology Society.

BACKGROUND: Inadvertent atrioventricular block is a complication of radiofrequency ablation. The present study is an analysis of the incidence, significance, and factors associated with inadvertent atrioventricular block during radiofrequency catheter ablation in childhood and adolescence. METHODS AND RESULTS: The records of the Pediatric Radiofrequency Ablation Registry were reviewed. Between January 1, 1991, and April 1, 1994, atrioventricular block occurred in 23 of 1964 radiofrequency ablations (1.2%): 14 as third-degree block (3 transient) and 9 as second-degree block (5 transient). Atrioventricular block occurred from 5 seconds to 2 months (mean, 4.1 days; median, 15 seconds) after the onset of the energy application. Eight transient cases lasted 1 hour to 1 month (mean, 9.4 days; median, 7 days). Inadvertent atrioventricular block was related to the ablation anatomic site: 3 of 111 (2.7%) anteroseptal, 11 of 106 (10.4%) midseptal, and 2 of 197 (1.0%) right posteroseptal sites (P = .0007) for anteroseptal, P = .0001 for midseptal, and P = .17 for right posteroseptal versus nonright septal sites). Five of 314 (1.6%) ablations for atrioventricular nodal reentrant tachycardia resulted in atrioventricular block (P = .004 versus nonright septal sites). Compared with a matched subgroup, radiofrequency ablation experience was the only significant risk factor (32.7 versus 106.6, P = .002) for the occurrence of atrioventricular block. CONCLUSIONS: Inadvertent atrioventricular block may occur during or late after radiofrequency catheter ablation. It is associated with ablations for (1) anterior and midseptal accessory pathways and atrioventricular nodal reentry and (2) relative institutional inexperience.

Adolescent↗
PubMedDate not supplied

Nutritional assessment of pediatric patients admitted to an acute-care pediatric service utilizing anthropometric measurements.

Two hundred consecutive pediatric patients were given a nutritional assessment at the time of their admission to the hospital. A weight-for-height below the 5th percentile was an indication of acute depletion and a height-for-age below the 5th percentile a sign of chronic depletion. Measurement of weight-for-height and/or height-for-age was below the 5th percentile in 32% of those assessed. Seventy-five percent of the acutely depleted children and 60% of the acute and chronically depleted children had midarm circumferences below the 5th percentile. Midarm muscle circumference below the 5th percentile was significantly more prevalent in all the depleted children. This paper reports the results of our study of the objective (quantitative) elements of our nutritional assessment. These measurements indicate that a significant number of patients are at nutritional risk at the time they are admitted to the hospital. An adequate nutritional assessment cannot be limited to objective considerations, however. A complete medical evaluation of the patient is required, including objective and subjective considerations.

Anthropometry↗
PubMed1991

Intensive chemotherapy and low-dose radiotherapy for the treatment of advanced-stage Hodgkin's disease in pediatric patients: a Pediatric Oncology Group study.

Sixty-two patients with advanced-stage Hodgkin's disease and a median age of 12 years (range, 3 to 22 years) were treated with four cycles of mechlorethamine, vincristine, procarbazine, and prednisone (MOPP) alternating with four cycles of doxorubicin, vinblastine, bleomycin, and dacarbazine (ABVD) followed by low-dose radiotherapy (RT). We determined the feasibility, immediate safety, and rapidity of response of patients to this regimen, as well as the relationship between prognostic factors and the rate of complete remission (CR), event-free survival (EFS), and overall survival. Therapy was well tolerated, and the major toxicity was hematopoietic. At the end of chemotherapy, 54 of 62 patients (87%) were in CR by clinical restaging, with a biopsy of residual disease where necessary. The actuarial 3-year EFS is 77% (SE, 11%), with a median follow-up of 35 months, and the survival is 91% (SE, 7%). With respect to EFS, female patients and those with stage II or III disease fared statistically better than males and patients with stage IV disease, respectively. Six patients have died: three of progressive Hodgkin's disease, one of secondary acute myelocytic leukemia (AML), one of secondary non-Hodgkin's lymphoma (NHL), and one of overwhelming bacterial sepsis. The Pediatric Oncology Group (POG) is currently engaged in a randomized study of these eight cycles of chemotherapy with and without RT to assess the role of RT in achieving comparable results.

Adolescent↗
PubMed1993

With modern imaging techniques, is staging laparotomy necessary in pediatric Hodgkin's disease? A Pediatric Oncology Group study.

PURPOSE: To determine whether the information gained from staging laparotomy can be predicted by imaging and/or clinical factors in children with Hodgkin's disease. PATIENTS AND METHODS: Between 1986 and 1991, 216 consecutive pediatric patients with Hodgkin's disease underwent laparotomy and were treated on two concurrent protocols in a multiinstitutional cooperative group. All patients had computed tomography (CT) of the chest, abdomen, and pelvis. Clinical factors studied included sedimentation rate, B symptoms, histology, number and location of involved sites, sex, mediastinal involvement, and age. Pretreatment CTs were centrally reviewed in 88 cases for the presence and size of both supradiaphragmatic and infradiaphragmatic lymph nodes, intrinsic spleen lesions, and splenic size. Models were generated that were predictive of any abdominal disease, splenic involvement, extensive splenic involvement, and upstaging at the laparotomy. False-positive and false-negative rates were calculated. RESULTS: For the end point of any abdominal disease, a model based on B symptoms, histology, sedimentation rate, and number and location of involved sites was highly significant (P < .0001). However, the success in predicting abdominal disease in an individual patient was limited: false-negative rate, 26%; false-positive rate, 32%. Highly significant models based on clinical factors and/or radiographic findings were also generated to predict splenic involvement, extensive splenic involvement, and upstaging with laparotomy, but they also had high false-positive and false-negative rates. CONCLUSION: Laparotomy findings cannot be predicted accurately in the majority of patients based on knowledge of CT findings and clinical factors.

Adolescent↗
PubMed1994

The Pediatric Oncology Group experience with the international staging system criteria for neuroblastoma. Member Institutions of the Pediatric Oncology Group.

PURPOSE: An international consensus on the criteria for surgicopathologic staging (INSS) of patients with neuroblastoma has been published, but has not been validated. A retrospective study was conducted to assess if the INSS definitions identified prognostic subsets of patients with neuroblastoma. PATIENTS AND METHODS: The initial operative and pathology reports were reviewed from 675 patients on Pediatric Oncology Group (POG) #8104, a stage- and age-related treatment study that used the POG surgicopathologic staging system. RESULTS: Of 596 eligible cases, there was concordance between the POG and INSS stages for the 193 patients with localized, resected disease (POG stage A), the 202 with distant metastases, the 51 with POG stage Ds (IVs) tumors, and 40 of the cases with grossly unresected, localized tumor without lymph node involvement (POG stage B). Of the remaining 19 patients with POG stage B tumors, five were INSS stage 2B and 14 INSS stage 3. All of the 91 cases with nonadherent, regional lymph node metastases (POG stage C) conformed to the definitions for INSS stage 2B (n = 42) or 3 (n = 49). In infants, there was no difference in event-free survival (EFS) among INSS stages 2A, 2B, or 3. In contrast, older children with INSS stage 3 disease had inferior EFS compared with INSS stage 2A or 2B tumors. CONCLUSION: We conclude the following: (1) the INSS identifies distinct patient subsets, particularly in children; (2) infants remain a favorable group, regardless of INSS/POG stage; and (3) the INSS deserves further prospective study especially in the light of recent biologic prognostic variables.

Humans↗
PubMed1998

Prognostic significance of age, MYCN oncogene amplification, tumor cell ploidy, and histology in 110 infants with stage D(S) neuroblastoma: the pediatric oncology group experience--a pediatric oncology group study.

PURPOSE: Although a high rate of spontaneous regression is observed in infants with stage D(S) neuroblastoma (NB), survival is not uniform. To determine the prognostic relevance of age at diagnosis, therapy, and tumor biology in infants with stage D(S) NB, we reviewed the Pediatric Oncology Group (POG) experience. PATIENTS AND METHODS: A review of patients diagnosed with stage D(S) NB registered on POG protocols was performed. Survival according to age at diagnosis, treatment, and tumor biology was determined. RESULTS: Between 1987 and 1996, 110 infants with stage D(S) NB had an estimated 3-year survival rate of 85% +/- 4%; survival rate was 71% +/- 8% for infants 2 months of age or younger, and 68% +/- 12%, 44% +/- 33%, and 33% +/- 19% for patients with diploid, MYCN-amplified, and unfavorable histology tumors, respectively. Survival rates were similar for patients who received adjuvant chemotherapy versus those who did not (82% +/- 5% v 93% +/- 6%, respectively; P = .187). Furthermore, there was no statistical difference in survival rate for patients who underwent complete resection of their primary tumor compared with those who underwent partial resection or biopsy only (90% +/- 5% v 78% +/- 7%, respectively; P = .083). CONCLUSION: Our review confirmed that the survival of infants with stage D(S) NB is excellent. However, subsets of patients with poor prognosis can be identified by young age and unfavorable biologic factors. More effective therapy is needed for the group of stage D(S) infants who show unfavorable clinical and biologic features.

Age Factors↗