COVID-19 in Pediatric Cancer Patients and Hematopoietic Stem Cell Transplant Recipients in a Tertiary Care Hospital in Argentina.

The outcome of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in cancer pediatric patients was initially uncertain. The objective of this study was to describe the characteristics and outcome of cancer patients and hematopoietic stem cell transplant recipients from 0 to 19 years with detectable SARS-CoV-2 from April 23, 2020, to April 30, 2022, treated in a tertiary-level hospital in Argentina. A total of 348 cases were registered in 339 patients. The median age was 89.5 (3 to 224) months. The sex was predominantly male: 193 (55.5%). The most common malignant disease was leukemia (42.8%). One hundred four cases (29.9%) had comorbidities. Of the 346 cases with an available blood count, 17.6% had a lymphocyte count <300/mm 3 . Fever was the most common symptom. In most cases (93.1%) presented asymptomatic or mild disease. Twenty-one cases (6%) presented severe or critical status. Eleven of 24 admissions to the intensive care unit were due to COVID-19 (coronavirus disease 2019). Eight patients (2.3%) died. Two deaths were attributable to SARS-CoV-2 (0.6%). Being older, having fever, lymphopenia at diagnosis, and having received hematopoietic stem cell transplant were associated with a more severe disease. Around 90% of the children continued their cancer treatment without any change.

Síndrome de cascanueces: una causa infrecuente de hematuria / Nutcracker Syndrome: a Rare Cause of Hematuria

La hematuria en pediatría responde habitualmente a etiologías benignas. Una causa poco frecuente es el síndrome de cascanueces, que se define como la compresión de la vena renal izquierda entre la aorta y la arteria mesentérica superior, que deriva en una presión elevada en la vena renal izquierda con el desarrollo de venas colaterales y dilataciones varicosas. La prevalencia de este síndrome se desconoce. Cuando es sintomático ocasiona hematuria, proteinuria y dolor pélvico crónico. En la pubertad, el crecimiento rápido y el desarrollo de los cuerpos vertebrales pueden producir un estrechamiento del ángulo entre la aorta y la arteria mesentérica superior. Se describe el caso de una adolescente con diagnóstico de carcinoma mucoepitelial metastásico óseo múltiple que presenta hematuria anemizante. Se arribó al diagnóstico de síndrome de cascanueces y se discutieron las opciones de tratamiento. Finalmente, con el uso de realce del calzado para corrección de la escoliosis, se atenuó significativamente la hematuria. (AU)

In pediatrics, hematuria usually responds to benign etiologies. A rare cause is nutcracker syndrome, defined as compression of the left renal vein between the aorta and the superior mesenteric artery, resulting in elevated pressure in the left renal vein with the development of collateral veins and varicose dilatation. The prevalence of this syndrome is unknown. When symptomatic, it causes hematuria, proteinuria, and chronic pelvic pain. At puberty, the rapid growth and development of the vertebral bodies can produce a narrowing of the angle between the aorta and the superior mesenteric artery.We describe the case of a teenage girl diagnosed with multiple metastatic mucoepithelial carcinoma of bone who presented anemia-producing hematuria. The diagnosis of nutcracker syndrome was arrived at with consideration of the therapeutic options. Finally, with shoe enhancement for scoliosis correction, hematuria was significantly lessened. (AU)

Síndrome hipotalámico como presentación inusual de recaída en el sistema nervioso central de leucemia linfoblástica aguda en niños: reporte de 4 casos / Hypothalamic syndrome as an unusual presentation of CNS relapse of acute lymphoblastic leukemia in children: report of 4 cases

La leucemia linfoblástica aguda (LLA) es la patología oncológica más frecuente en pediatría, y corresponde al 23% de las neoplasias en menores de 15 años. Alrededor del 20% de los pacientes con LLA presentan recaídas, en la mayoría de los casos, en la médula ósea. Las recaídas extramedulares son inusuales y las dos localizaciones más frecuentes son el sistema nervioso central (SNC) y los testículos. Cuando las recaídas ocurren en el SNC, suelen manifestarse con un síndrome meníngeo. El síndrome hipotalámico se define como la presencia de hiperfagia, obesidad y/o cambios en el estado de ánimo, y es una forma de presentación clínica inusual de las recaídas en el SNC y debe alertar al pediatra para mantener un alto índice de sospecha.Se describen cuatro casos que se presentaron con síndrome hipotalámico al momento de desarrollar una recaída de LLA en el SNC

Acute lymphoblastic leukemia (ALL) is the most common malignancy in childhood, corresponding to 23% of cancer in children younger than 15 years old. About 20% of ALL cases will relapse, commonly in the bone marrow. Extramedullar relapses are unusual, and the two most frequent locations are CNS and testicles. ALL relapses, when diagnosed in the CNS, frequently present with clinical features of a meningeal syndrome. The hypothalamic syndrome, consisting of hyperphagia, obesity and / or behavior disturbances, corresponds to an unusual presentation of relapses in this location and should alert pediatricians to suspect it.We describe 4 ALL cases of hypothalamic syndrome at the time of CNS relapse

[Hypothalamic syndrome as an unusual presentation of CNS relapse of acute lymphoblastic leukemia in children: report of 4 cases]. / Síndrome hipotalámico como presentación inusual de recaída en el sistema nervioso central de leucemia linfoblástica aguda en niños: reporte de 4 casos.

Acute lymphoblastic leukemia (ALL) is the most common malignancy in childhood, corresponding to 23 % of cancer in children younger than 15 years old. About 20 % of ALL cases will relapse, commonly in the bone marrow. Extramedullar relapses are unusual, and the two most frequent locations are CNS and testicles. ALL relapses, when diagnosed in the CNS, frequently present with clinical features of a meningeal syndrome. The hypothalamic syndrome, consisting of hyperphagia, obesity and / or behavior disturbances, corresponds to an unusual presentation of relapses in this location and should alert pediatricians to suspect it. We describe 4 ALL cases of hypothalamic syndrome at the time of CNS relapse.

La leucemia linfoblástica aguda (LLA) es la patología oncológica más frecuente en pediatría, y corresponde al 23 % de las neoplasias en menores de 15 años. Alrededor del 20 % de los pacientes con LLA presentan recaídas, en la mayoría de los casos, en la médula ósea. Las recaídas extramedulares son inusuales y las dos localizaciones más frecuentes son el sistema nervioso central (SNC) y los testículos. Cuando las recaídas ocurren en el SNC, suelen manifestarse con un síndrome meníngeo. El síndrome hipotalámico se define como la presencia de hiperfagia, obesidad y/o cambios en el estado de ánimo, y es una forma de presentación clínica inusual de las recaídas en el SNC y debe alertar al pediatra para mantener un alto índice de sospecha. Se describen cuatro casos que se presentaron con síndrome hipotalámico al momento de desarrollar una recaída de LLA en el SNC.

Actinomicosis torácica: presentación de un caso / Thoracic actinomycosis: report of a case

La actinomicosis es una enfermedad producida por bacterias del género Actinomyces. La forma torácica representa el 30% de los casos. La evolución es habitualmente crónica, con clínica variable. Se presenta una nina de 11 años de edad con tumefacción subescapular derecha de un mes de evolución, asociada a fiebre, hiporexia y pérdida de peso. Se diagnosticó actinomicosis torácica producida por Actinomyces meyeri. Recibió tratamiento antibiótico durante un año, con resolución completa. Aunque es poco frecuente, debe ser tenida en cuenta como diagnóstico diferencial en cuadros de tumoración torácica de larga evolución con compromiso del estado general. Debe informarse al microbiólogo la sospecha diagnóstica por lo difícil de su desarrollo en los medios de cultivo habitual.

Actinomycosis is a disease produced by bacteria of the genus Actinomyces. The thoracic form represents 30% of the cases. Signs and symptoms are generally unspecific. A previously healthy 11 year old girl was admitted with a chest mass of a month of evolution associated with fever, hyporexia and weight loss. Thoracic actinomycosis was diagnosed with the isolation of Actinomyces meyeri in the mass drainage. She received antibiotic treatment for a year with complete resolution. Although rare, it should be taken into account as a differential diagnosis in long evolution thoracic tumor affecting the general condition. The microbiologist must be warned about the suspected diagnosis because it is difficult to find it in routinely used culture media.

[Thoracic actinomycosis: report of a case]. / Actinomicosis torácica: presentación de un caso.

Actinomycosis is a disease produced by bacteria of the genus Actinomyces. The thoracic form represents 30% of the cases. Signs and symptoms are generally unspecific. A previously healthy 11 year old girl was admitted with a chest mass of a month of evolution associated with fever, hyporexia and weight loss. Thoracic actinomycosis was diagnosed with the isolation of Actinomyces meyeri in the mass drainage. She received antibiotic treatment for a year with complete resolution. Although rare, it should be taken into account as a differential diagnosis in long evolution thoracic tumor affecting the general condition. The microbiologist must be warned about the suspected diagnosis because it is difficult to find it in routinely used culture media.

Symptomatic bone langerhans cell histiocytosis treated at diagnosis or after reactivation with indomethacin alone.

This study evaluated the outcome of patients with symptomatic bone Langerhans cell histiocytosis (LCH) treated with indomethacin alone, either at diagnosis or after reactivation (after recurrence with previous therapies). We evaluated the nonrandomized use of oral indomethacin (2 mg/kg/d) in patients with symptomatic single-system bone LCH. From 1997 to 2012, 38 sequential patients were treated for a median of 4 months. Criteria of nonactive disease (NAD) after initial treatment (8 wk) were: no pain, no soft tissue involvement, no increase of size, or no new bone lesions. Twenty-two patients were treated at diagnosis: 18 showed NAD after initial treatment (2 patients who had bone reactivations were retreated with indomethacin and remain with NAD). Three patients improved and they are with NAD after treatment with indomethacin, steroids, or radiotherapy. One patient developed progressive bone disease and he is with NAD after treatment with steroids and chemotherapy. Sixteen patients were treated after reactivation, and all were with NAD after initial treatment: 5 reactivated and 4 remain with NAD after retreatment with indomethacin. Toxicity was not significant. We conclude that indomethacin is a well tolerated and active drug in patients with symptomatic bone disease. The results support the concept that chemotherapy may not be necessary for limited bone disease.

Importance of multi-lineage hematologic involvement and hypoalbuminemia at diagnosis in patients with "risk-organ" multi-system Langerhans cell histiocytosis.

AIM: To perform a risk factor analysis in patients with "risk organ" multi-system Langerhans cell histiocytosis at diagnosis. METHODS: From 1987 to 2007, 77 patients were analyzed. A univariate analysis of the variables, age <2 years, lungs, spleen and hepatic involvement, presence of >or=2 risk involved organs, hypoalbuminemia and the presence of isolated anemia, anemia with thrombocytopenia with or without leukopenia at diagnosis was performed. Statistically significant variables were combined and entered into a multivariate analysis. RESULTS: Fifty-six and 66 evaluable patients had hematologic and hepatic involvement at diagnosis, respectively. Among the hematologic patients, the subgroup of anemia with thrombocytopenia with or without leukopenia showed a significantly lower 5-year survival than the subgroup of isolated anemia (0.19 vs. 0.87, respectively; P=0.0001). Of all the patients, those with hypoalbuminemia had a 5-year survival of 0.16 compared with those with normal albumin levels, who had a 5-year survival of 0.65 (P<0.0001). In multivariate analysis, only anemia with thrombocytopenia with or without leukopenia and hypoalbuminemia were the independent risk factors (relative risk 3.77; confidence interval, 1.7-8.4; P<0.0011 and relative risk 2.59; confidence interval, 1.24-5.4; P<0.0112). CONCLUSIONS: Anemia with thrombocytopenia with or without leukopenia and hypoalbuminemia, were associated with worse prognosis in multi-system Langerhans cell histiocytosis. Other therapeutic strategies should be considered at diagnosis or early during the initial treatment for this high risk subgroup of patients.

Reactivation and risk of sequelae in Langerhans cell histiocytosis.

OBJECTIVE: To evaluate disease reactivation in patients with Langerhans cell histiocytosis (LCH) and its impact on adverse sequelae. MATERIALS AND METHODS: A retrospective evaluation of 300 patients diagnosed with LCH between 1987 and 2002 with complete response to initial treatment was performed. RESULTS: Mean age at diagnosis was 5.3 years. With a mean follow-up of 4.8 years, reactivation of the disease occurred in 29.7% (89/300) of the patients, with two or more reactivations in 34.8% (31/89) of those. Reactivation occurred in 17.4, 36.8, 46.5, and 53.5% of the patients with single-system unifocal disease (Group A: 161 patients), single-system multifocal disease (Group B: 53 patients), multi-system disease without (Group C: 58 patients), and with (Group D: 28 patients) risk-organ involvement, respectively. The differences between the incidence rates of Groups A and B (P < 0.0004), A and C (P < 0.0001), and A and D (P < 0.0001) were highly significant. The most common reactivation sites involved were bone, middle ear, and skin; reactivation was rare in risk organs (9.5%). The median time between initial complete response and the first reactivation episode was 1 year for Group A, 1.3 years for Group B, and 9 months for Groups C and D. Most reactivation episodes (88%) occurred within the first 2 years of follow-up. Adverse sequelae were recognized in 242/300 patients: 71% (49/69) of patients with and 25.4% (44/173) without reactivations developed these adverse sequelae (P < 0.0001), respectively. Sites most commonly showing sequelae were bone, middle ear, and hypothalamus (Diabetes Insipidus). CONCLUSIONS: Incidence of reactivation correlates with the stage of the disease at diagnosis. Incidence of sequelae correlates with the occurrence of reactivations.

Isolated pulmonary Langerhans cell histiocytosis presenting with recurrent pneumothorax.

We describe the outcome of a 20-month-old female and a 6-year-old male, both of whom had acutely developed severe respiratory distress with tachypnea, cyanosis and, in Patient 2, thoracic pain. Chest X-ray and CT scan showed interstitial pulmonary involvement and a bullous process with bilateral pneumothoraces for both children. Pulmonary biopsy confirmed the diagnosis of Langerhans cell histiocytosis (LCH). Laboratory testing and skeletal radiography did not reveal any other involvement of LCH. The patients received chemotherapy (prednisone, vinblastine, 6-mercaptopurine). They had recurrent episodes of pneumothorax during follow-up and placement of chest tubes was the treatment chosen. They were asymptomatic, with regression of bullae and disappearance of pneumothorax at 58 and 63 months of follow-up, respectively. Pulmonary function tests done during follow-up were normal in both patients. Despite severe pulmonary involvement, conservative surgical treatment and moderate chemotherapy produced good results in these two rare cases.

Outcome in children with pulmonary Langerhans cell Histiocytosis.

BACKGROUND: The aim of this study was to evaluate features and outcome of children with Langerhans cell Histiocytosis (LCH) and pulmonary involvement. PROCEDURE: Retrospective evaluation of LCH patients was performed from 1987 to 2001. Multisystem patients were classified according to the pattern of organ system involvement into Groups: A (no pulmonary, hematologic, or hepatic involvement), B (pulmonary involvement), C (pulmonary and hematologic or hepatic involvement), and D (hematologic or hepatic involvement). All had skin, bone, or lymph node involvement. Chest X-ray was performed in all patients and computed tomography (CT) in 21. Diagnostic lung biopsy was performed in five patients. RESULTS: Pulmonary involvement was found in 36/220 patients studied. Two patients had isolated pulmonary involvement. Multisystem involvement was present in 83 patients, 34 of whom had pulmonary involvement. In 20/36 patients, tachypnea, cough, and thoracic pain occurred. Diffuse interstitial involvement was found in all cases. Pulmonary function tests were performed in nine patients, six of whom revealed mild to moderate restrictive respiratory involvement. The two patients having isolated pulmonary involvement survived 2 and 2.7 years after diagnosis. Median follow-up of all multisystem patients was 2.1 years, with a 5-year survival probability of 0.59. The 5-year survival probability of Groups A-D was 0.94, 0.83, 0.23, and 0.40, respectively. The survival difference between Groups B and C was statistically different (P < 0.0071). CONCLUSIONS: According to our data, pulmonary compromise without other risk organ involvement does not appear to be a negative prognostic factor in our study.

Varicela en el niño inmunocomprometido en la era del aciclovir / Varicella in the imnunosupressed child in the acyclovir age

ResumenIntroducción. La varicela es una infección que sepresenta frecuentemente en los niños y cursa en lamayoría de los casos en forma benigna. En lospacientes inmunocomprometidos puede producir complicaciones con mayor frecuencia. El tratamiento con aciclovir ha logrado disminuir esta tasa de complicaciones y la mortalidad en lospacientes inmunocomprometidos. La incidencia de complicaciones varía de acuerdo con el tipo de alte-ración de la inmunidad que presente cada paciente. Objetivo. Comparar las características clínicas y deevolución de los niños con distintos tipos de inmunocompromiso y varicela.Población, material y métodos. Se llevó a cabo unestudio retrospectivo, descriptivo y comparativo detodos los pacientes inmunocomprometidos con va-ricela que se internaron en el hospital. Resultados. Entre el mes de enero de 1993 y el dediciembre de 1999 se diagnosticaron 157 pacientes inmunocomprometidos con varicela. La medianade edad de los niños fue de 4 años (rango: 4 meses a20 años). El 53% fueron varones y en el 94% de los casos la adquisición de la enfermedad fue extrahos-pitalaria. En el 22% de los pacientes se detectó algúntipo de complicación; las infecciones de piel y partes blandas (21 pacientes), la sepsis (7 pacientes) y la neumonitis (5 pacientes) fueron las más frecuente-mente halladas. El 4,5% de los niños tuvo bacterie-mia. De la población estudiada, 77 fueron pacientes con patología hemato-oncológica, 40 tenían infec-ción causada por el VIH, 24 recibían tratamiento concorticoides y 15 niños habían recibido trasplantes.Todos los pacientes recibieron tratamiento conaciclovir.

Varicella is a benign infection in children. Immu-nocompromised patients frequently have complications. The treatment with acyclovir had led to adecrease in the rate of complications and the mortality in this group of patients. The incidence of complications varies according to the type of immunodeficiency. Between January of 1993 and De-cember of 1999 we compared the clinical characteristics and the outcome of immunocompromised children with varicella. One hundred fifty sevenimmuno-compromised children with varicellawere admitted. The average age of patients was 4 years (range: 4 months to 20 years). Fifty-three percent (53%) was male and 94% of the cases were acquired in the community. In 22% (35 patients) some complication was detected. Celullitis (21patients) sepsis (7 patients) and pneumonitis (5patients) were the most frequent. Five percent (7patients) of the children had bacteriemia. Of thestudied population, 77 had hemato-oncologic diseases, 40 had HIV infection, 24 received prolonged treatment with steroids and 15 have been subjected to transplantations. All the patients received treatment with acyclovir. There were no statistical differences in age, bacteriemia presence, incidence and type of complications between the children. Alonger rash period, as well as a more frequent monomorphic rash, were observed in children with steroid treatment and transplantation patients (p<0.05). The global mortality was 1.9%. Children with hemato-oncologic diseases and transplantation recipients had higher mortality rates (p <0.05). Children with prolonged treatment with steroidsand transplanted with varicella had longer duration of rash as well as a higher frequency of monomorphic rash. Children with organ transplantations and those with hemato-oncologic illnesses had higer mortality rates.

Varicela en el niño inmunocomprometido en la era del aciclovir / Varicella in the imnunosupressed child in the acyclovir age

ResumenIntroducción. La varicela es una infección que sepresenta frecuentemente en los niños y cursa en lamayoría de los casos en forma benigna. En lospacientes inmunocomprometidos puede producir complicaciones con mayor frecuencia. El tratamiento con aciclovir ha logrado disminuir esta tasa de complicaciones y la mortalidad en lospacientes inmunocomprometidos. La incidencia de complicaciones varía de acuerdo con el tipo de alte-ración de la inmunidad que presente cada paciente. Objetivo. Comparar las características clínicas y deevolución de los niños con distintos tipos de inmunocompromiso y varicela.Población, material y métodos. Se llevó a cabo unestudio retrospectivo, descriptivo y comparativo detodos los pacientes inmunocomprometidos con va-ricela que se internaron en el hospital. Resultados. Entre el mes de enero de 1993 y el dediciembre de 1999 se diagnosticaron 157 pacientes inmunocomprometidos con varicela. La medianade edad de los niños fue de 4 años (rango: 4 meses a20 años). El 53% fueron varones y en el 94% de los casos la adquisición de la enfermedad fue extrahos-pitalaria. En el 22% de los pacientes se detectó algúntipo de complicación; las infecciones de piel y partes blandas (21 pacientes), la sepsis (7 pacientes) y la neumonitis (5 pacientes) fueron las más frecuente-mente halladas. El 4,5% de los niños tuvo bacterie-mia. De la población estudiada, 77 fueron pacientes con patología hemato-oncológica, 40 tenían infec-ción causada por el VIH, 24 recibían tratamiento concorticoides y 15 niños habían recibido trasplantes.Todos los pacientes recibieron tratamiento conaciclovir.(AU)

Varicella is a benign infection in children. Immu-nocompromised patients frequently have complications. The treatment with acyclovir had led to adecrease in the rate of complications and the mortality in this group of patients. The incidence of complications varies according to the type of immunodeficiency. Between January of 1993 and De-cember of 1999 we compared the clinical characteristics and the outcome of immunocompromised children with varicella. One hundred fifty sevenimmuno-compromised children with varicellawere admitted. The average age of patients was 4 years (range: 4 months to 20 years). Fifty-three percent (53%) was male and 94% of the cases were acquired in the community. In 22% (35 patients) some complication was detected. Celullitis (21patients) sepsis (7 patients) and pneumonitis (5patients) were the most frequent. Five percent (7patients) of the children had bacteriemia. Of thestudied population, 77 had hemato-oncologic diseases, 40 had HIV infection, 24 received prolonged treatment with steroids and 15 have been subjected to transplantations. All the patients received treatment with acyclovir. There were no statistical differences in age, bacteriemia presence, incidence and type of complications between the children. Alonger rash period, as well as a more frequent monomorphic rash, were observed in children with steroid treatment and transplantation patients (p<0.05). The global mortality was 1.9%. Children with hemato-oncologic diseases and transplantation recipients had higher mortality rates (p <0.05). Children with prolonged treatment with steroidsand transplanted with varicella had longer duration of rash as well as a higher frequency of monomorphic rash. Children with organ transplantations and those with hemato-oncologic illnesses had higer mortality rates.(AU)

Outpatient, sequential, parenteral-oral antibiotic therapy for lower risk febrile neutropenia in children with malignant disease: a single-center, randomized, controlled trial in Argentina.

BACKGROUND: Recent reports and previous randomized trials conducted at the authors' institution suggested that children with lower risk febrile neutropenic (LRFN) may benefit from substitution of oral antibiotic therapy for parenteral therapy. The objective of this study was to determine the efficacy of parenteral-oral outpatient therapy in the management of children with LRFN who were receiving treatment for malignant disease. METHODS: From August 2000 to April 2002, 135 children with a median age of 7.5 years (range, 1.6-15.8 years) who had a total of 177 episodes of LRFN were included in a prospective, randomized, single-institution trial. Children with LRFN received a single dose of ceftriaxone and amikacin and completed a risk-assessment work-up. All patients were discharged immediately and, at 24 hours, were allocated randomly to two groups: Group A (89 episodes) received oral ciprofloxacin, and Group B (88 episodes) received intravenous ceftriaxone. RESULTS: Most patients (61% in Group A and 51% in Group B) were receiving treatment for leukemia (P value not significant [NS]). Twenty-eight children (31%) in Group A and 22 children (25%) in Group B displayed unexplained fever (P value NS). No significant differences in sites of initial infection were found between the two groups. The median duration of neutropenia was 4.2 days and 4.7 days for Group A and Group B, respectively (P value NS); the median duration of fever was 2.3 days and 2.6 days, respectively (P value NS); and the median duration of antibiotic treatment was 4.5 days and 4.8 days, respectively (P value NS). The overall results of the study were excellent. Only four treatment failures in Group A (5%) and 6 treatment failures in Group B (7%) were observed. These patients were readmitted to the hospital and did well with appropriate treatment. CONCLUSIONS: In children with LRFN who are receiving treatment for malignant disease, outpatient oral ciprofloxacin after 24 hours of a single dose of intravenous ceftriaxone and amikacin was as safe and efficacious as parenteral ceftriaxone. Outpatient management and early antibiotic withdrawal were safe for both groups.

Cholestasis, sclerosing cholangitis, and liver transplantation in Langerhans cell Histiocytosis.

OBJECTIVE: To analyze features and outcomes of cholestasis, sclerosing cholangitis (SC), and liver transplantation (LTx) in patients with Langerhans cell Histiocytosis (LCH) between October 1987 and June 1999. STUDY DESIGN: Of 182 cases with LCH, 36 had hepatic involvement and 12 of those presented with cholestasis. These 12 were the focus of our study. Their median age was 23 months (range: 3-36). Hepatomegaly or hepatosplenomegaly was found in 11 of the 12; elevations of alkaline phosphatase, transaminases, gamma glutamyl transpeptidase (GGT), and less frequently direct bilirubin were detected. Sonography, liver biopsy, and cholangiography were consistent with the diagnosis of SC in 11 patients. None of the biopsies revealed Langerhans cells (LC). Frequently associated lesions of skin, bone, and ear were noted. Early patients were treated with Vinblastine/prednisone for 8 weeks, later patients with the LCH I and LCH II protocols of the Histiocyte Society (HS). RESULTS: Median follow-up was 28 months (range: 10-86). Three patients improved and remained without signs of progressive SC at 27, 32, and 86 months. Nine had progressive liver sequelae resistant to chemotherapy. Of these nine, five received LTx, three died before LTx with progressive SC, and one awaits LTx. Three LTx patients survive without disease reactivation 14, 25, and 37 months post-transplant. Two patients died less than one month after LTx, due to renal failure and sepsis in the first patient and bowel volvulus with perforation followed by sepsis in the second one. CONCLUSIONS: SC is a frequent and usually progressive sequela of multisystem LCH in our institution. LTx has become the treatment of choice for the majority of patients and should be considered early in cases with severe hepatic involvement.

Criterios de bajo riesgo de mortalidad en niños con neutropenia y fiebre durante la quimioterapia por cancer / Criteria of low risk of mortality in children with neutropenia and fever during cancer chemotherapy

Con el objetivo de validar el uso de un perfil de bajo riesgo de mortalidad, y evaluar la eficacia de un esquema de tratamiento secuencial parenteral-oral en niños con neutropenia y fiebre durante la terapia de enfermedades malignas, se llevó a cabo un estudio prospectivo entre mayo de 1997 y diciembre de 1999. En el período de estudio fueron incluídos 247 episodios de neutropenia y fiebre en 215 pacientes. Los niños en tratamiento por enfermedades malignas que presentaban: neutropia (recuento absoluta de neutrófilos<500/mm3), fiebre (> 38§C) buen estado general, que no presentaban un foco clínico de riesgo, que no tenían factores comorbilidad severos asociados, sin bacteriemia y familias continente, fueron elegidos para recibir un tratamiento inicial con ceftrixona y amikacina en el hospital seguido de cefixima o ciprofloxacina por vía oral en forma ambulatoria hasta completar 7 días. La edad media de los niños fue de 64 meses (r: 8-200). El 48 porciento (118) tuvo leucemia y el 57 porciento (141) catéteres endovasculares. El 47 porciento (122) tuvo foco clínico de infección, donde predominó la infección respiratoria alta (81 porciento). El tiempo medio de fiebre fue de 1.1 días (r: 1-8) y de neutropenia 3.9 días (r: 1-9). El 61 porciento (150) de los niños fue dado de alta con neutropenia. La media de internación fue de 1.5 días. Se registraron 4 fallos (1.6 porciento), los cuáles fueron tratados satisfactoriamente y ninguno presentó complicaciones mayores, falleció o abandonó el tratamiento. El perfil de riesgo utilizado fue seguro, y el tratamiento secuencial fue eficaz en el manejo de los niños con neutropenia y fiebre de origen hemato-oncológico y bajo riesgo de mortalida.

Criterios de bajo riesgo de mortalidad en niños con neutropenia y fiebre durante la quimioterapia por cancer / Criteria of low risk of mortality in children with neutropenia and fever during cancer chemotherapy

Con el objetivo de validar el uso de un perfil de bajo riesgo de mortalidad, y evaluar la eficacia de un esquema de tratamiento secuencial parenteral-oral en niños con neutropenia y fiebre durante la terapia de enfermedades malignas, se llevó a cabo un estudio prospectivo entre mayo de 1997 y diciembre de 1999. En el período de estudio fueron incluídos 247 episodios de neutropenia y fiebre en 215 pacientes. Los niños en tratamiento por enfermedades malignas que presentaban: neutropia (recuento absoluta de neutrófilos<500/mm3), fiebre (> 38ºC) buen estado general, que no presentaban un foco clínico de riesgo, que no tenían factores comorbilidad severos asociados, sin bacteriemia y familias continente, fueron elegidos para recibir un tratamiento inicial con ceftrixona y amikacina en el hospit