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OBJECTIVES: To describe our initial experience in the treatment of hyperparathyroidism (HPP) of renal cause using total or subtotal parathyroidectomy, autoimplant and cryopreservation in pediatric patients. Secondary HPP is the increased function of the parathyroid hormone (PTH) due to an abnormal phosphocalcic metabolism in patients with chronic renal failure (CRF). This situation produces increased bone resorption resulting in osteodystrophy and endovascular calcifications. Surgical treatment is aimed to diminish the level of PTH in CRF patients, to avoid HPP complications. METHODS AND MATERIALS: Descriptive, monocentric and retrospective study of a case series of patients with secondary and tertiary hyperparathyroidism, who went through total or subtotal parathyroidectomy, autoimplant and cryopreservation between 2009 and 2016. We analyzed the following variables: age, calcemia, PTH, phosphatemia, alkaline phosphatase (ALP), follow-up and complications. The continuous variables are expressed in median and interquartile range or in mean and SD, according to their distribution. The categorical variables were expressed in percentages and frequencies (repeated sentence). RESULTS: Number of patients included: 13. Mean age of the patients was 16.7 years old. Preoperative median calcium dosage was 9.1 mg/dl (IQR: 8.9-9.5). Median PTH was 2,600 pg/ml (IQR: 1,400 pg/ml to 2,785 pg/ml). Intraoperatory dosage of PTH reported a median drop of 86.6% in the first 15 minutes (IQR: 80.5-95.9). After the first 48 hours, median calcemia was 9 mg/dl (IQR: 7.7-9.4) and median PTH was 40 pg/ml (IQR: 20-113). We did not identify intraoperatory complications. In the immediate post operatory stage, mean IV calcium therapy was 4 days (SD: 2.39). Median time of follow-up was 18 months (IQR 9-36). Two patients had hungry bone syndrome and one patient had a recurrence of the pathology as remote post operatory complications. After a year, median calcemia, was 9 mg/dl (IQR: 7.6-9.3) and median PTH was 50 pg/ml (IQR: 28.5-108). The decrease in PTH and ALP were statistically significant with p value < 0.05. CONCLUSION: In our study, total parathyroidectomy with auto implant is a safe and effective option for the treatment of secondary and tertiary hyperparathyroidism in pediatric patients. This could also prevent bone complications.

OBJETIVOS: Describir la experiencia en el tratamiento del hiperparatiroidismo (HPP) de origen renal con la técnica de la paratiroidectomía total o subtotal, autoimplante y criopreservación en pacientes pediátricos y adultos jóvenes. El HPP secundario es el aumento de la función de las hormonas paratiroideas debido a una alteración en el metabolismo fosfocálcico a partir de la insuficiencia renal crónica (IRC). Esto produce una mayor resorción ósea, provocando alteraciones en los huesos y calcificaciones en el endotelio vascular. El tratamiento quirúrgico se indica para disminuir el nivel de parathormona (PTH), y así prevenir las complicaciones del hiperparatiroidismo en pacientes con IRC terminal. MATERIAL Y METODOS: Estudio descriptivo, retrospectivo, monocéntrico de una serie de pacientes con diagnóstico de HPP secundario y terciario sometidos a paratiroidectomía total, autoimplante y criopreservación entre 2009 y 2016. Se analizaron las siguientes variables: Edad, Calcemia, PTH, Fosfatemia, Fosfatasa alcalina (FAL), tiempo de seguimiento y complicaciones. Las variables continuas se expresan en mediana y rango intercuartil o en media y DS, según su distribución. Las variables categóricas se expresaron en porcentajes y frecuencias. RESULTADOS: Se incluyeron 13 pacientes. La edad media fue 16,7 años (R= 11:24). La mediana de calcemia preoperatoria fue 9,1 mg/dl (RIQ: 8,9-9,5). La mediana preoperatoria de PTH fue 2.600 pg/ml (RIQ: 1.400-2.785). La medición del descenso porcentual de PTHi ultrarrápida informó una mediana de 86,6% en los primeros 15 minutos (RIQ: 80,5-95,9). No se identificaron complicaciones intraoperatorias. A las 48 horas posquirúrgicas, la mediana de calcemia fue de 9 mg/dl (RIQ: 7,7-9,4) y la mediana de PTH de 40 pg/ml (RIQ: 20-113). La media de aporte de calcio endovenoso posquirúrgico fue de 4 días (DS 2,39). La mediana de seguimiento fue de 18 meses (RIQ 9-36). Como complicaciones posoperatorias tardías se identificaron dos pacientes con síndrome de hueso hambriento y un paciente con recidiva de la enfermedad. Al año postoperatorio la mediana de calcemia, fue de 9 mg/dl (RIQ: 7,6-9,3). La mediana de PTH fue de 50 pg/ml (RIQ: 28,5-108)y la de FAL fue de 116 UI/L (RIQ 102:273). El descenso de PTH y de FAL fueron estadísticamente significativos con p valor < 0,05. CONCLUSIONES: En nuestro estudio, la paratiroidectomía total con autoimplante es una alternativa segura y efectiva para el tratamiento del hiperparatiroidismo secundario y terciario en pediatría, permitiendo prevenir así complicaciones óseas y cardiovasculares.

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Paciente de 9 años, previamente sana, que ingresa en anasarca con síndrome nefrótico clínico y humoral, asociado a hipertensión arterial y microhematuria, con función renal normal y se comporta como corticorresistente. Se realiza 1º biopsia renal que informa glomerulonefritis proliferativa mesangial difusa con esclerosis focal y segmentaria. En tratamiento con ciclofosfamida y corticoides, presenta síndrome febril prolongado con anemia secundaria a crisis aplásica de la serie roja

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Paciente de 9 años, previamente sana, que ingresa en anasarca con síndrome nefrótico clínico y humoral, asociado a hipertensión arterial y microhematuria, con función renal normal y se comporta como corticorresistente. Se realiza 1° biopsia renal que informa glomerulonefritis proliferativa mesangial difusa con esclerosis focal y segmentaria. En tratamiento con ciclofosfamida y corticoides, presenta síndrome febril prolongado con anemia secundaria a crisis aplásica de la serie roja, asociada con una infección aguda por parvovirus B19, e insuficiencia renal aguda secundaria a nefritis tubulointersticial severa. La PCR para parvovirus B19 DNA fue positiva en tejido renal y médula ósea. La paciente evoluciona a insuficiencia renal crónica terminal. No se puede descartar que desde su inicio, el síndrome nefrótico estuviera asociado al daño glomerular por la infección viral, que comenzó como síndrome nefrótico con componentes nefríticos y que evoluciona inesperadamente a una nefritis tubulointersticial. Este sería el primer caso en el que se documenta como causa de insuficiencia renal crónica terminal, un daño tubulointersticial secundario a parvovirus B19.

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PURPOSE: This study (Childrens Cancer Group [CCG]-105) was designed in part to determine in a prospective randomized trial whether intrathecal methotrexate (IT MTX) administered during induction, consolidation, and maintenance could provide protection from CNS relapse equivalent to that provided by cranial radiation (CXRT) in children with acute lymphoblastic leukemia (ALL) and intermediate-risk features. PATIENTS AND METHODS: We randomized 1,388 children with intermediate-risk ALL to the two CNS regimens. They received either IT MTX at intervals throughout their course of therapy or CXRT (18 Gy) during consolidation with IT MTX during induction, consolidation, and delayed intensification. Systemic therapy was randomized to one of four treatment regimens derived from a regimen used by CCG in recent studies for this patient population and three more intensive regimens based on the Berlin-Frankfurt-Munster trials. RESULTS: Life-table estimates at 7 years show a 93% and 91% CNS relapse-free survival rate for the CXRT and IT MTX groups, respectively. The corresponding event-free survival (EFS) rates are 68% and 64%. The differences are not significant. Patients who received more intensive systemic therapy had a 94% CNS relapse-free survival rate on either CXRT or IT MTX, while patients who received standard systemic therapy had 90% and 80% rates for CXRT and IT MTX, respectively (P < .0001). Patients less than 10 years of age who received CXRT or IT MTX had 72% and 71% EFS rates if they received more intensive systemic therapy. Patients 10 years or older who received CXRT had an improved EFS (61% v 53%) with a more intensive systemic program. This was primarily due to fewer bone marrow relapses (P = .04). CONCLUSIONS: IT MTX during induction, consolidation, and maintenance provides protection from CNS relapse in patients with intermediate-risk ALL equivalent to that provided by CXRT if more intensive systemic therapy is given. The CNS relapse rate with either CXRT or IT MTX is in part dependent on the associated systemic therapy. For intermediate-risk patients less than 10 years of age, IT MTX with an intensified systemic regimen provided CNS prophylaxis comparable to that provided by CXRT, whereas older patients had fewer systemic relapses if they received CXRT.

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PURPOSE: The Berlin-Frankfurt-Munster (BFM) 76/79 trial of acute lymphoblastic leukemia (ALL) in children produced impressive disease-free survival (DFS) rates with a protocol that began with 8 weeks of intensive therapy, followed by 8 weeks of maintenance therapy, and then another 6 weeks of intensive treatment. The current study was conducted to determine the relative contributions of each of these periods of intense therapy on the DFS rates of ALL patients with intermediate presenting features. In addition, due to concerns regarding the toxicity of CNS irradiation, we compared cranial irradiation (CXRT) with intrathecal methotrexate (IT MTX) administered during induction and consolidation to IT MTX during all phases of the treatment program. PATIENTS AND METHODS: Between May 1983 and April 1989, more than 1,600 children with ALL and intermediate presenting features, as defined by the Childrens Cancer Group (CCG), were entered into a randomized trial that tested four systemic therapy regimens and two CNS programs. RESULTS: The results with a median follow-up of 57 months show that systemic regimens with a delayed intensification (Delint) phase of therapy had a 5-year event-free survival (EFS) rate of 73% compared with the control regimen EFS rate of 61% (p = .006). For children less than 10 years of age, standard three-drug induction and Delint produced a 77% 5-year EFS. IT MTX during all phases of therapy provided CNS protection comparable to the CXRT regimen in children less than 10 years of age. Children 10 years of age or older appear to have a better EFS rate with intensive induction, Delint, and CXRT. CONCLUSION: Delint improves the EFS rate of children with ALL and intermediate presenting features. Maintenance IT MTX can be safely substituted for CXRT for presymptomatic CNS therapy in children with intermediate-risk characteristics less than 10 years of age.

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Surface-mediated reactions of clotting were compared in 21 black children with homozygous sickle cell disease, 12 age-matched controls, and 15 adults. Both the coagulant and antigen titers of Hageman factor (factor XII) were decreased in asymptomatic patients compared with those in the control groups. These findings were associated with slight but significant reductions in the plasma titers of prekallikrein and high molecular weight kininogen. A further decrease from the initially low titers of these contact factors was observed during vaso-occlusive crises. Additionally, we observed a disparate relationship between Hageman factor coagulant activity and its antigen titers. These data provide evidence for reduction of the contact factors in patients with homozygous sickle cell disease.

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A group of children with acute lymphocytic leukemia was studied to investigate if a reduction in daily dose fraction of cranial radiation would reduce the incidence of somnolence syndrome. Thirty-one evaluable patients received 100 rad X 18 cranial radiation therapy. Sixty-six similar evaluable patients were given 180 rad X 10. Both groups received the same chemotherapy including intrathecal methotrexate. Clinically detectable somnolence appeared in 58% of ech group without significant differences in the overall frequency or severity of somnolence (p greater than 0.5). This study failed to substantiate a radiation dose fraction size dependence for somnolence syndrome in children with acute lymphocytic leukemia.

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L-Asparaginase therapy for childhood acute lymphoblastic leukemia causes deficiencies of plasma hemostatic proteins, especially antithrombin, plasminogen, and fibrinogen. Severe thromboses and hemorrhages occurred in 18 children receiving vincristine, prednisone, and asparaginase therapy for ALL. Thirteen children had intracranial thrombosis or hemorrhage, four had extremity thrombosis, and one had both an intracranial hemorrhage and an extremity thrombosis. These events occur characteristically in the third and fourth weeks of therapy during or just after a three-week course of L-asparaginase. Symptoms of headache, obtundation, hemiparesis, and seizure were common for the intracranial events: local pain, swelling, and discoloration were common for the extremity thromboses. These complications have been recognized in 1 to 2% of children undergoing induction therapy which includes asparaginase.

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