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Congenital Zika infection has been linked with a characteristic phenotype including neurologic sequelae. However, West syndrome has not been previously well described as a consequence. We aim to show this association through a retrospective descriptive study performed in Ecuador. Among 147 infants with congenital Zika infection, 7.5% suffered from West syndrome. Vigabatrin seems to be effective to control the spasms.

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BACKGROUND: Few congenital Zika syndrome (CZS) cases have been notified in Ecuador and, to our knowledge, there are no significant published studies dealing with their clinical evolution. We present a detailed clinical characterization of 21 children with congenital Zika virus (ZIKV) infection born in Ecuador who were followed up until September 2019. METHODS: We did a retrospective longitudinal study of children attended by the infectious disease specialists of Francisco Icaza Bustamante Children's Hospital (Guayaquil) due to congenital ZIKV infection suspicion. The inclusion criteria consisted of laboratory confirmed diagnosis of congenital ZIKV infection. RESULTS: Sixteen of these 21 cases of congenital ZIKV infection showed clinical, neuroimaging and laboratory findings strongly suggestive of CZS and 5 children showed laboratory findings compatible with congenital ZIKV infection without congenital manifestations associated to CZS. All children with CZS showed neurodevelopmental delay, spasticity and hyperreflexia during follow-up, whereas the majority of them (14/15) experienced recurrent epileptic seizures and dysphagia (12/13). Two CZS cases died during follow-up. Visual evoked potential and hearing screening with acoustically evoked auditory brainstem response were abnormal in 50% and 37.5% of CZS cases, respectively. Congenital ZIKV infection without findings consistent with CZS at birth was not clinically relevant at 23 months of age in the five cases of our cohort. CONCLUSIONS: Severe neurodevelopmental delay, severe microcephaly, epileptic seizures and dysphagia were present at 2 years of age in most CZS cases of our cohort.

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El presente documento contiene una apuesta de largo plazo, que se presenta a través de la revisión de los conceptos y teorías del modelo, además contiene un resumen de las experiencias de operativización y lecciones aprendidas, las cuales son aplicables en el corto plazo. Puede ser tomado como un documento orientador y motivador para otros que estén iniciando un camino similar. El trabajo está estructurado de la siguiente manera: Primero, una revisión del marco teórico de la intervención, luego se explica el contexto general de la provincia, a continuación se presentan los principales avances, métodos e instrumentos usados, y finalmente se realiza un repaso a las lecciones aprendidas hasta la fecha(AU)

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El documento contiene una apuesta de largo plazo, que se presenta a través de la revisión de los conceptos y teorías del modelo, además contiene un resumen de las experiencias de operativización y lecciones aprendidas, las cuales son aplicables en el corto plazo. Puede ser tomado como un documento orientador y motivador para otros que estén iniciando un camino similar. El trabajo está estructurado de la siguiente manera: Primero, una revisión del marco teórico de la intervención, luego se explica el contexto general de la provincia, a continuación se presentan los principales avances, métodos e instrumentos usados, y finalmente se realiza un repaso a las lecciones aprendidas hasta la fecha

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Flutamide is a non-steroidal anti-androgenic drug, commonly used in the treatment of advanced prostate cancer, acne and hirsutism. This drug may induce various degrees of liver injury, including acute liver failure (ALF), with further need for liver transplantation. Here, we present a report of 10 consecutive patients seen in a period of 14 years, with acute liver toxicity induced by flutamide (in most cases severe hepatotoxicity): 3 men and 7 women, with a mean age of 75 and 29 years old, respectively. All men received flutamide as treatment of advanced prostate carcinoma and they developed hepatotoxicity without ALF, and three months after withdrawal of the drug, they recovered completely. In contrast, in 7 young female with liver toxicity caused by flutamide as treatment of various hyperandrogenic conditions (acne and hirsutism), ALF was observed in 5 patients, all of them requiring urgent liver transplantation, with excellent outcome and survival in 4 of them. Based on the above, we believe that flutamide treatment should be preferentially avoided in young female patients with benign pathologies, or if it is used, patients should be warned of its potential severe complications. Also, serial liver tests should be closely monitored and, in case of elevations, the drug should be immediately withdrawn.

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We report a 33 year-old female with a diagnosis of halothane-induce fulminant hepatic failure who was subjected to a liver transplant with an ABO-incompatible graft. The patient received a therapeutic protocol that included total plasma exchange, splenectomy and quadruple immunosuppression. After 5 years, the patient remains asymptomatic and with normal liver enzymes, while she has been treated with low dose of immunosuppressive drugs. This case demonstrates an example of how the immunological process of accommodation opens the possibility of using ABO-incompatible organs as a definitive grafts.

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Although the use of cadaveric split or living donor liver transplantation is a valid option for liver transplants, they have several complications, being the "small-for-size syndrome" one of the most frequent. This entity is mainly due to the incapacity that the graft has to meet the blood drainage demands. We report a 61 year-old patient with sub-acute liver failure, transplanted with a partial liver graft that developed hyperbilirubinemia, ascites and liver function deterioration. A meso-caval shunt was performed, after which the ascites resolved, serum bilirubin normalized and the synthetic function of the liver improved. After one month, a follow-up CT seen showed the absence of blood flow in the shunt, possible due to the reduction of the hyper-perfusion of the liver. The clinical and biochemical condition of the patient continued improving despite the lack of flow through the shunt.

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We report a 33 year-old female with a diagnosis of halothane-induce fulminant hepatic failure who was subjected to a liver transplant with an ABO-incompatible graft. The patient received a therapeutic protocol that included total plasma exchange, splenectomy and quadruple immunosuppression. After 5 years, the patient remains asymptomatic and with normal liver enzymes, while she has been treated with low dose of immunosuppressive drugs. This case demonstrates an example of how the immunological process of accomodation opens the possibility of using ABO-incompatible organs as a definitive grafts.

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Although the use of cadaveric split or living donor liver transplantation is a valid option for liver transplants, they have several complications, being the "small-for-size syndrome" one of the most frequent. This entity is mainly due to the incapacity that the graft has to meet the blood drainage demands. We report a 61 year-old patient with sub-acute liver failure, transplanted with a partial liver graft that developed hyperbilirubinemia, ascites and liver function deterioration. A meso-caval shunt was performed, after which the ascites resolved, serum bilirubin normalized and the synthetic function of the liver improved. After one month, a follow-up CT seen showed the absence of blood flow in the shunt, possible due to the reduction of the hyper-perfusion of the liver. The clinical and biochemical condition of the patient continued improving despite the lack of flow through the shunt.

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BACKGROUND: Octreotide is used in the treatment of acute variceal bleeding, based on its inhibitory effects of post-prandial splanchnic hyperemia and splanchnic venoconstriction. The consequences of these haemodynamic changes on renal circulation are not well known in cirrhotic patients. AIM: To evaluate the effects of acute octreotide administration on several parameters of renal function, including free water clearance, in patients with cirrhosis with or without ascites. PATIENTS AND METHODS: Twenty cirrhotic patients, Child-Pugh A orB, with or without ascites, with esophageal varices, normal renal function and free of medications (vasoactive drugs or diuretics) were assigned to 2 different protocols. Protocol 1: 10 patients were randomized to receive octreotide or placebo, as a bolus followed by a continuous infusion. Glomerular filtration rate (GFR) and renal plasma flow (PRF) were measured, in basal conditions and during the drug or placebo administration. Protocol 2: 10 additional patients were randomized in the same way and free water clearance and urinary sodium excretion were again measured in the basal period and during the drug or placebo infusion. RESULTS: After octreotide or placebo administration no significant changes were observed neither in GFR nor in PRF. The free water clearance decreased significantly during octreotide administration (3.12 ml/min+/-1.04 SE vs 0.88+/-0.39, p<.03). In both protocols no changes in mean arterial pressure were observed. CONCLUSIONS: Acute administration of octreotide to cirrhotic patients with portal hypertension, with or without ascites, did not produce any change in glomerular filtration rate or in estimated renal plasm blood flow. However the free water clearance decreased significantly. This effect, under chronic administration, could be clinically important and deserves further studies.

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BACKGROUND: Acute variceal bleeding in cirrhotic patients is an emergency with a high risk of rebleeding and death. Endoscopic procedures such as sclerotherapy or banding, combined or not with drugs such as octreotide could be considered. AIM: To assess the value of octreotide in the control of acute variceal bleeding. PATIENTS AND METHODS: Ninety-two patients were randomized into three groups: endoscopic therapy plus octreotide 50 microg/h bolus and continuous infusion for 5 days (n=36); octreotide (same dosage) (n=13) and endoscopic therapy only (banding and/or sclerotherapy) (n=43). RESULTS: Haemostasis at 24 hours was achieved in 97% of patients with combined treatment, 69% of patients receiving octreotide, and 93% of patients with endoscopic therapy (p=0.2). Three patients with combined treatment, four patients receiving octreotide and eight patients with endoscopic therapy, rebled during the first five days (p=0.15). The mean of blood units transfused was similar in the three groups. No differences were observed in hospital days and side effects. At 42 days of follow up, eight patients with endoscopic therapy, one patients with combined therapy and 2 patients receiving octreotide, died (p=NS). CONCLUSIONS: Octreotide is useful in the management of acute variceal bleeding. The absence of important side effects, renders it as a safe adjuvant treatment associated with endoscopic treatment.

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Se describe el caso de una paciente de quince años de edad, que presentó cefalea, disminución de la agudeza visual, de tres años de evolución con carácter progresivo. Se realizó una Topografía de Cerebro computada que mostró un gran Meningioma, Hidrocefalia y edema vasogénico. Realizada la exéresis total del tumor, la hidrocefalia se resolvió espontáneamente y el edema con tratamiento farmacológico. Se revisa la bibliografía.

A case of a fifteen year-old patient that who has been experiencing migraine for three years, a decrease of the visual sharpness of progressive character. A PET scan was done we could visualize that the patient had a great on Meningioma., hydrocephalus, and vasogenic edema. Removing the tumor the hydrocephalus was resolved and the edema received pharmacological treatment.

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El primer embarazo después de un transplante conocido ocurrió en 1958, en una destinataria de un transplante renal donado por su hermana gemela idéntica. El primer embarazo posterior a un transplante hepático conocido fue en 1978. Durante los últimos 20 años, los problemas específicos del transplante hepático y embarazo se han comenzado a identificar gracias al conocimiento del comportamiento similar al transplante renal y embarazo. Se conoce por evidencia que el embarazo después de un transplante hepático es bien tolerado; las drogas inmunosupresoras no han demostrado ser teratógenas, y en estas pacientes existe un riesgo aumentado para realizar en el transcurso de su gestación, hipertensión y preeclampsia, restricción de crecimiento intrauterino, anemia y síntoma de parto prematuro. En el puerperio presenta una mayor incidencia de infecciones secundaria a su condición de inmunosupresión. El embarazo debe ser considerado de alto riesgo y compromete a un equipo médico multidisciplinario para llevar un buen control clínico y de laboratorio (control fisiológico materno, fetal y niveles plasmáticos de drogas inmunosupresoras). El resultado perinatal ha sido exitoso, con tendencia a presentar niños de bajo peso sin complicaciones. Se presenta un caso clínico de embarazo posterior a transplante hepático. Ocurrió 55 meses posteriores al transplante, primigesta, él término del embarazo fue a las 33+5 semanas. Presentó complicaciones durante la gestación como anemia severa, preeclampsia moderada a severa, (de inicio precoz), y colestasia intrahepática. En el puerperio realizó un absceso subaponeurótico, con buen resultado perinatal.

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BACKGROUND/AIMS: In patients with cirrhosis pharmacological treatment of portal hypertension using beta-blockers and vasodilators has raised concerns for its potential deleterious effects on renal function and encephalopathy. To clarify this issue we evaluated the effects of propranolol and 5-isosorbide mononitrate or both on subclinical hepatic encephalopathy and renal function in a prospective randomized double-blinded study. METHODOLOGY: Thirty patients Child-Pugh A or B, with esophageal varices, normal renal function and non-previous pharmacological treatment were studied. After a basal period, patients received during 4 weeks 5-isosorbide mononitrate (80 mg/day) or placebo. In the next 4 weeks, propranolol was added to both groups. At baseline and at the end of each study period we assessed: renal function tests; plasma renin activity and aldosterone; subclinical hepatic encephalopathy (electroencephalograms, visual evoked potentials and psychometric studies). Mean arterial pressure, cardiac output (echo-Doppler) and indocyanine green retention were also measured. RESULTS: The most common alterations at baseline were increased arterial ammonia levels (85%), abnormal indocyanine green retention (75%), abnormal trail making B (44%), decreased inulin clearance (30%) and high plasma renin activity (27%). After 4 weeks of 5-isosorbide mononitrate or placebo no significant changes were observed in any variable. Five out of 14 patients receiving 5-isosorbide mononitrate were withdrawn due to side effects. The addition of propranolol decreased significantly plasma renin activity in both groups and cardiac output in those receiving 5-isosorbide mononitrate but did not change other variables. CONCLUSIONS: In patients with compensated or slightly decompensated liver cirrhosis 5-isosorbide mononitrate, propranolol or the association of both did not produce detectable worsening of subclinical hepatic encephalopathy or renal function.

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