Hemangioendotelioma hepático infantil multifocal / Multifocal hepatic hemangioendothelioma

INTRODUCCIÓN: El hemangioendotelioma hepático (HEH) es un tumor benigno, raro en niños, que se presenta frecuentemente en el primer año de vida. La presentación clínica es variable y su diagnóstico se realiza en base a la sospecha clínica, estudios de laboratorio y de imagen. El objetivo fue describir un caso de hemangioendotelioma hepático multifocal. CASO CLÍNICO: Niña de 3 meses que presentó hepatomegalia sin elementos de falla hepática ni cardiaca. Se realizó ecografía y tomografía de ab domen que orientaron al diagnóstico de HEH que se confirmó con la angioresonancia de abdomen. Recibió glucocorticoides a altas dosis en forma prolongada. Al año y medio de iniciado el tratamiento se evidenció remisión del tumor. Presentó efectos secundarios por el tratamiento instaurado. CONCLUSIONES: La presencia de una hepatomegalia aislada en un lactante asintomático debe hacer pensar en una probable patología tumoral, orientándonos por la clínica e imagenología al diagnóstico nosológico de la misma. Siempre se debe sospesar las posibles complicaciones con los riesgos de los tratamientos a realizar. En este caso la extensión del tumor y sus probables complicaciones justificó el uso de corticoterapia prologada a altas dosis a pesar de sus efectos adversos.

INTRODUCTION: Hepatic hemangioendothelioma is a rare benign tumor in children, which frequently occurs in the first year of life. The clinical presentation is variable and the diagnosis is based on clinical suspicion, and laboratory and imaging studies. The objective was to describe a case of multifocal hepa tic hemangioendothelioma. CLINICAL REPORT: 3-month-old girl who presented hepatomegaly without elements of hepatic or heart failure. Abdominal ultrasound and CT scan were used to diagnose hepatic hemangioendothelioma, which was confirmed by CT abdominal angiography. The patient received glucocorticoid treatment at high doses for a prolonged period. A year and a half after treatment, there was evidence of tumor remission. She had side effects from the established treatment. CONCLUSIONS: In asymptomatic patients with isolated hepatomegaly, it should be considered a probable tumor patho logy, considering the clinic and imaging studies. Possible complications and treatments risks must always be assessed. In this case, the tumor extension and its probable complications justified the use of prolonged corticosteroid therapy at high doses despite its adverse effects.

Bone mineral density in children and adolescents with hepatosplenic mansonic schistosomiasis and esophageal varices who underwent splenectomy and ligature of the left gastric vein

Twenty eight children and adolescents from 7 to 19 years of age, suffering from hepatosplenic mansonic schistosomiasis and bleeding esophageal varices were evaluated for bone mineral density (BMD), before undergoing medical and surgical treatment. The surgical protocol was splenectomy, autoimplantation of spleen tissue into a pouch of the greater omentum and ligature of the left gastric vein. Twenty one patients were evaluated after a follow up from two to nine years post surgical treatment. The BMD was measured at the lumbar spine (L2 - L4) through the dual energy absorptionmetry X-ray (DEXA), using a LUNAR DPX-L densitometer. Preoperatively, all patients showed deficit of the BMD varying from 1 to 7.07 standard deviations (Mean +/- SEM - 2.64 +/- 0.28), considering the mean line of the control curve for healthy children accepted as normal. The BMD deficit was more evident among the females than the males. After treatment there was a significant increment (C2 = 9.19 - p =0.01) of the BMD and 29 percent of the patients (six out of twenty one) were considered without bone mineral deficit. It was concluded that the patients included in this series, who suffer from hepatosplenic mansonic schistosomiasis, showed an important BMD deficit, specially among the females which has had a significant improvement after medical and surgical treatment

Specific treatment of advanced Schistosomiasis liver disease in man: favourable results

One hundred eighty-four patients with hepatosplenic schistosomiasis mansoni from the northeast of Brazil were studied. All were treated with a single dose of Oxamniquine or Praziquantel, and were observed over 6 to 12 months. Special attention was given to the evolution of severe hepatopathy. Favourable results were obtained, particularly with the compensated hepatosplenic form. Hepatic function showed great improvement. Hepatomegaly and splenomegaly were significantly reduced in size, to a greater or lesser extent, in the great majority of patients. The implications of the results obtained are considered below.

The impact of schistosomiasis mass treatment on hepato-splenomegaly in Napu Valley, Central Sulawesi, Indonesia.

The impact of mass treatment with praziquantel on 241 individuals, 131 males and 110 females in Napu Valley, Indonesia was studied. The pre-treatment overall stool positive cases ranged from 43% to 79%, the highest in the 20-29 age group. Post-treatment follow-up study showed that the cases dropped to 2.5%. Significant reduction was seen in liver rate from 68% to 51% and in the age groups 0-9, 10-19, there was a significant decrease in liver enlargement after treatment, but not significant differences were seen in spleen rate in all age groups. This might be due to the early stage of Schistosoma infection in younger age groups where complete resolution of fibrosis might occur.