Evaluación de la atención hospitalaria de los pacientes fallecidos en el Hospital de Pediatría Juan.P Garrahan entre marzo y septiembre de 2001. Informe de la Comisión de Mortalidad / Evaluation of in-patient care of patients dying at the Hospital de Pediatría J. P Garrahan between March and September 2001

Objetivo:En el marco de un programa de evaluacíon de calidad de atención médica,se crea la comisión de Mortalidad con el objetivo de analizar,con una nueva propuesta metodológica,la atención de los pacientes fallecidos en el hospital.Este informe reporta los resultados de trabajo de dicha comisión.Pacientes y métodos:se realizó un análisis retrospectivo de todos los pacientes fallecidos en el hospital entre el 15 de marzo y el 30 de septiembre de 2001.Cada historia clínica tuvo dos instancias independientes de revisión:el grupo tratante y un miembro de la comisión.Se registraron;edad,lugar/res de internación,diagnostico de base,lugar de óbito,solicitud de necropsia,y concordancia entre el grupo tratante y la comisióm.La muerte se clasifico en inevitable,potencialmente evitable o evitable.Resultados:se registraron 7759 admisiones y 149 fallecimientos(1.9 por ciento)siete historias clínicas no pudieron ser evaluadas.La mediana de edad fue de 18.5 meses(r=1d-19a)El 17,6 por ciento eran menores de 1 mes y 43,4 por ciento menor de 1 año.la mediana del tiempo de internación de 9.5 días(r=3-215)Los diagnósticos más frecuentes de enfermedad de base fueron:enfermedad oncológica 36,cardiopatía congénita 15 e infección respiratoria 12.La principal causa de muerte de aquellas consideradas potencialemente evitables(n=10)fue la infección intrahospitalaria.Conclusiones:La mortalidad hospitalaria durante el período de estudio fue de 1.9 por ciento,El 80 por ciento de la muertes ocurrieron en el área de terapia intensiva(ucip,ucin y quemados)Se consideraron inevitables 97/107 muertes(90 por ciento)potencialmente evitables 10(9.3 por ciento)y una evitables(0.9 por ciento)Solo se realizó necropsia en el 14.7 por ciento de la población estudiada

Nasopharyngeal carcinoma in childhood and adolescence: a single-institution experience with combined therapy.

BACKGROUND: A high cure rate may be attained for locally advanced, undifferentiated nasopharyngeal carcinoma (NPC) in children, provided that a combined modality of treatment is employed. Both local and systemic therapies are necessary. Results at a single pediatric institution were analyzed. METHODS: From November 1988 to December 1997, 16 consecutive patients were treated with NPC at the Hospital Garrahan in Buenos Aires, Argentina. The authors were able to evaluate 11 patients (9 boys and 2 girls); their median age was 12 (range, 8-14) years. Chemotherapy consisted of 3 courses, every 3 weeks, of 5-fluorouracil (500 mg/m(2)) plus bleomycin (15 mg/m(2)) daily for 4 days, with cisplatin (100 mg/m(2)) added the last day. External beam radiotherapy was delivered over a median of 52 (range, 45-63) days, to a median cumulative dose to the primary site of 55 (range, 50-61.2) grays (Gy). The median dose for the lower neck area was 45 (range, 45-55.8) Gy. All patients received radiotherapy to the primary site and to the initially involved lymphoid areas, with daily single doses of 1.8 Gy (5 of 7 days per week). RESULTS: The main symptoms at onset were cervical mass (100%), epistaxis (54%), cephalalgia (36%), and trismus (36%). All cases were Stage IV (American Joint Committee on Cancer and International Union Against Cancer TNM system). Complete response was achieved in 45% of patients after initial chemotherapy. With a median follow-up of 63 (range, 23-119) months, disease free survival (with standard error [SE]) and overall survival estimates were 61% (16%) and 91% (9%), respectively, at 75 months. Acute toxicity due to therapy was tolerable. Chronic sinusitis (73%), hypothyroidism (73%), and mild (64%) or moderate (9%) neck fibrosis were detected at follow-up. CONCLUSIONS: Although this series is small, the authors concluded that NPC patients have a good chance of survival in the setting described, in spite of locally advanced disease. Chemotherapy might be useful in preventing the development of systemic metastases.

Phase II window of idarubicin in children with extraocular retinoblastoma.

PURPOSE: The aim of this study was to evaluate in an upfront phase II study the response to idarubicin in children with extraocular retinoblastoma. PATIENTS AND METHODS: The starting dose of idarubicin was 15 mg/m(2)/d (days 1 and 2) weeks 0 and 3. After an interim evaluation, the dose was reduced to 10 mg/m(2)/d (days 1 and 2) weeks 0 and 3 because of hematopoietic toxicity. Response was evaluated at week 6. RESULTS: At the Hospital JP Garrahan (Buenos Aires, Argentina), 10 patients (five bilateral) were entered onto the study from 1995 to 1998. A total of 19 cycles were administered. Extraocular sites included orbit (n = 10), bone marrow (n = 3), bone (n = 1), lymph node (n = 1), and CNS (n = 1). The response rate was 60% (95% confidence interval, 30% to 90%). One complete response was achieved, in addition to five partial responses, two cases of stable disease, and two cases of progressive disease. All patients with bone marrow involvement achieved complete clearance of tumor cells. The patient with CNS disease had progressive disease. All patients had severe hematopoietic toxicity (grade 4 neutropenia and grade 3/4 thrombocytopenia after most cycles). Other toxicities included grade 2 diarrhea in 30%. No echocardiographic changes were detected. CONCLUSION: Idarubicin is active in extraocular retinoblastoma. The activity of this drug should be explored in future phase III studies.

Retinoblastoma with low risk for extraocular relapse.

OBJECTIVE: To define a subgroup of patients with retino-blastoma and low risk of extraocular relapse through histopathological and clinical variables. PATIENTS AND METHODS: Inclusion criteria consisted of stage I (intraocular disease), stage IIb1 (without concomitant choroid and/or scleral invasion), and nonenucleated patients (according to the Grabowski-Abramson classification). A total of 112 consecutive patients admitted to Hospital JP Garrahan from 1987 to 1997 were evaluable. Treatment included enucleation or local therapy and no chemotherapy. RESULTS: Forty-one patients had stage Ia (intraretinal), 8 stage Ib (prelaminar optic nerve invasion), 40 stage Ic (uveal invasion), and 12 stage IIb1 (postlaminar optic nerve invasion and cut end free of tumor). Eleven patients had neither eye enucleated. Median follow-up was 60 months. Only two events occurred: one patient had progressive disease in the contralateral globe and died of CNS metastasis and another had an orbital relapse that was successfully treated. Both had choroidal invasion. Five-year pEFS and pOS were 0.97 and 0.98, respectively. Neither length of the optic nerve stump, tumor size, anterior chamber invasion, degree of differentiation, nor degree of ocular coat invasion correlated with increased risk of metastasis. CONCLUSIONS: A subset of patients with retinoblastoma with low risk of relapse can be determined using histopathological evaluation of the invasion of ocular coats. Adjuvant chemotherapy is not warranted for patients with intraretinal extension and prelaminar optic nerve invasion. It is also probable that those patients with isolated choroidal invasion and those with postlaminar optic nerve extension with surgical margins clear of tumor do not need chemotherapy.

Acute myelogenous leukemia in Down's syndrome: report of a single pediatric institution using a BFM treatment strategy.

Between July 1990 and December 1995, 111 new consecutive pediatric patients with acute myelogenous leukemia (AML) have been treated in our institution. Eleven of them (9.9%) had Down's syndrome (DS), 6 boys and 5 girls. The median age was 22.5 (range 10-40) months. FAB subtypes were the following: M7: 6, M4: 3, and M0: 2. Five of them had previously had myelodysplasia and in 3, all FAB M7, myelofibrosis was detected. This population was treated with two consecutive protocols. Nine patients were included in the AML-HPG-90 protocol and 2 patients in the AML-HPG-95 study, respectively. However, all DS patients in this series received the same treatment. Eight patients achieved complete remission: two patients received two cycles of intensification with high dose (HD) ara-C, and 1 patient, only one cycle; the other 5 were prevented from receiving such therapy because of unacceptable toxicity or death. At 45 months, event-free survival and overall survival estimates were 0.30, S.E. 0.16. Mortality was remarkably high. All deaths (7) were associated with sepsis (5) or pulmonary infection (2). Three deaths occurred before achieving complete remission, 3 patients died during the consolidation phase and 1 died whilst off treatment. No one presented leukemic relapse. We conclude that this AML-BFM treatment strategy is highly toxic to children with DS and AML in our setting. Efforts will be made to improve clinical support and to administer less intensive therapy to this particular pediatric AML subgroup, which, in fact, has a better prognosis than the same non-trisomic population.

Hodgkin disease in children: results of a prospective randomized trial in a single institution in Argentina.

PURPOSE: To compare prospective treatment strategies in childhood Hodgkin disease to the following subsets of patients: a) Favorable prognostic group: these patients were randomized to receive 6 vs. 3 CVPP chemotherapy cycles without radiotherapy (CVPP: cyclophosphamide, vinblastine, procarbazine, and prednisone. The scheme was repeated every 28 days). b) Intermediate prognostic group: these patients were randomized to receive 6 cycles of CVPP or AOPE (AOPE: Adriamycin, vincristine, prednisone, and etoposide). Between the third and fourth cycles, all patients in this group received radiotherapy (RT)(30-40 Gy to initially involved areas). c) Unfavorable prognostic group: those patients received a single arm regimen of 6 cycles of CCOPP/CAPTe (3 of each combination) every 28 days. All these patients received radiotherapy (30-40 Gy to initially involved areas). RESULTS: From October 1987 to December 1994, a total of 114 children and adolescents were evaluated. Mean age was 9 (range 2-17) years. There were 72 boys and 42 girls. With a median follow-up of 5 (range 1.5-8.7) years, at 80 months event-free survival (EFS) and overall survival (OS) for the whole cohort are 0.809 (SE: 0.04) and 0.873 (SE: 0.04), respectively (SE: Standard Error). Favorable prognostic group (n = 26) EFS is 0.831 (0.09) (Arm CVPP x 3:0.857 (0.13) and Arm CVPP x 6: 0.875 (0.08); p = non significant). Intermediate prognostic group (n = 64) EFS is 0.806 (0.05) (Arm CVPP x 6 + RT: 0.872 (0.05) and Arm AOPE x 6 + RT: 0.667 (0.10); p = 0.04). Unfavorable group (n = 24) EFS is 0.829 (0.07). CONCLUSIONS: Results of treatment for the whole group are satisfactory. However, 3 cycles of CVPP without radiotherapy obtain equal EFS than 6 cycles without radiotherapy in the favorable prognostic group. In the intermediate prognostic group, 6 cycles of CVPP plus radiotherapy obtain a superior EFS than 6 cycles of AOPE plus radiotherapy. With the success of treatment for Hodgkin disease in children, future research needs to be focused in reducing toxicity without altering the excellent actual outcome.

Efectos adversos y complicaciones agudas de la quimioterapia intratecal / Adverse effects and acute complicationsof intrathecal chemotherapy

Desde junio de 1991 a agosto de 1992, se evaluaron prospectivamente los efectos adversos y las complicaciones agudas en 83 pacientes (pts) que recibieron 141 punciones lumbares (PL) con QT IT, que se encontraban hospitalizados, en buen estado clínico previo al procedimiento y que pudieron ser evaluados sistemáticamente por médicos del Servicio de Hematooncología durante 48 horas siguientes al procedimiento. Cuatro fueron exluidos. Se analizaron los efectos secundarios de 137 PL en 82 pts (1,67 PL/pt, rango: 1-5). El promedio de edad fue de 6.75 (rango: 08-15) años: 33 niñas y 50 varones. Ciento seis PL correspondieron a Pts con leucemia linfoblástica aguda: 19 con administración de metotrexate intratecal (MTX) como monodroga y 87 asociado con citarabina (Ara-c) y dexametasona (DMT); 16 PL se realizaron en pts portadores de leucemia mieloide aguda administrándose Ara-c y DMT y 15 PL correspondieron a Pts con linfoma no Hodgkin asociando MTX, Ara-c y DMT. Las dosis de la AT IT se ajuntó a la edad de los pacientes. Los pts recibieron QT sitémica asociada en el 86.1 por ciento (118) de las PL. Un 3,6 por ciento de los procedimientos fueron traumáticos con líquido cefalorraquideo (LCR) hemorrágico. Se detectaron efectos adversos complicaciones durante las 48 hs siguientes a la PL en el 46 por ciento de los casos: vómitos 28,5 por ciento, náuseas 24,1 por ciento, cefaleas 13,9 por ciento, fiebre 4,4 por ciento, raquialgia 4,4 por ciento, dolores en miembros inferiores 3,6 por ciento, dolor en el sitio de la PL 2, 9 por ciento, parestesias 2,2 por ciento, ataxia 0.7 por ciento y paraplejía secundaria a hematoma intrarraquídeo en 1 pte. Las manifestaciones secundarias a la administración de medicación intratecal fueron habitualmente reversibles en el término de 48 hs y no dejaron secuela alguna. Concluimos, la administración de QT IT es un procedimiento seguro y con escasas complicaciones trascendentes en el marco del tratamiento de las enfermedades onco-...

Children with fever of unknown origin in Argentina: an analysis of 113 cases.

The aim of this study was to determine the causes of fever of unknown origin, to evaluate new diagnostic tests and to elucidate risk factors for chronic or life-threatening disorders. The medical records of 113 children who had undiagnosed fever for at least 3 weeks were reviewed. Infection (N = 41) was the most frequent cause of fever of unknown origin. Respiratory tract infections were the most common causes in infants and endocarditis and tuberculosis were more frequent in older children. Neoplastic disorders (N = 11) occurred in children older than one year. Juvenile rheumatoid arthritis (N = 9) was the most common collagen-vascular disorder (N = 15). Miscellaneous disorders and factitious fever occurred in 21 and 4 cases, respectively. Twenty-two patients remained undiagnosed. History and physical examination led to a final diagnosis in 81% of cases. Abdominal ultrasonography was performed in 71 patients (61%) and was helpful for diagnosis in 15%. Children with life-threatening or chronic disorders (N = 58) were older than those with self-limiting conditions (N = 55; P = 0.017). Cardiovascular and articular signs and symptoms were more frequent in the former group (P = 0.01).