Cervical Lymphadenopathy in Two Young Women The Same Rare Diagnosis with Different Presentations.

Kikuchi-Fujimoto disease (KFD) is a rare, benign and usually self-limiting disorder that more often affects young women, which is characterized by cervical lymphadenopathy and fever. Clinical presentation may be indistinguishable from other diseases, and its inclusion in the differential diagnosis of lymphoproliferative, infective and autoimmune diseases is essential. An association with systemic lupus erythematosus is acknowledged. We present 2 different cases of 2 young women with KFD; the first case highlights the classic diagnostic features of this rare entity, and the second, the findings when KFD occurs in association with systemic lupus erythematosus. LEARNING POINTS: Fever and cervical lymphadenopathy presenting in a young woman are the main features of KFD.A timely excisional lymph node biopsy is of the utmost importance in establishing a correct diagnosis and in the management of this condition.In most patients with KFD the course of the disease is benign and self-limiting; however, when associated with other conditions (mainly systemic lupus erythematosus) it can follow a more severe evolution.

Cardiac Tamponade after Thrombolysis for Acute Ischemic Stroke A Case with an Excellent Outcome.

Intravenous thrombolysis with recombinant tissue plasminogen activator (rtPA) is the established treatment for acute ischemic stroke and has been highly effective in reducing the neurological deficit. Serious adverse events are not uncommon, with hemorrhage being the major complication. We describe the case of a patient with acute ischemic stroke that also presented with vague cardiac symptoms and was treated with rtPA, which was complicated by a hemopericardium causing cardiac tamponade. Pericardiocentesis was promptly performed, which resulted in rapid resolution of the cardiogenic shock. The patient recovered consciousness within a few minutes. A search of the MEDLINE database shows that this is the first report of cardiac tamponade after rtPA thrombolysis occurring in a patient with no history of recent myocardial infarction or aortic dissection. LEARNING POINTS: Cardiac tamponade is a medical emergency and if not recognized and treated quickly results in cardiogenic shock and death. Hence, it is necessary to be aware that the established treatment for acute ischemic stroke can lead to cardiac tamponade.Cardiac tamponade after rtPA thrombolysis can occur in patients with no history of recent myocardial infarction or aortic dissection.

A Mesothelial Inclusion Cyst Presenting in a 40-Year-Old Woman as Abdominal Pain and Bloating - A Rare Diagnosis.

Cystic mesotheliomas (also called mesothelial inclusion cysts) are rare benign neoplasms that occur more often in young women. Symptoms are usually non-specific, demanding a thorough work-up. We report a case of a 40-year-old female patient with 2 prior caesarean sections presenting with a 3-month history of abdominal pain. Laboratory tests revealed microcytic hypochromic anaemia and an elevated tumour marker CA-125. An investigation identified a large cystic abdominal mass, mostly in the left side of the abdomen. A laparotomy was performed with total resection of the lesion. Histological assessment resulted in a diagnosis of a mesothelial inclusion cyst. The patient had no recurrence after 3 years. LEARNING POINTS: Peritoneal mesotheliomas are rare conditions, and with benign features are even rarer.Mesothelial inclusion cysts, also known as benign cystic mesothelioma, occur more often in young to middle-aged women with excellent prognosis, although there are high rates of local recurrence.Investigation unmasks large abdominal lesions. A timely diagnosis is of the utmost importance, to prevent a dramatic change in outcome if severe complications develop.

Long-term evolution after in-hospital cardiac arrest in children: Prospective multicenter multinational study.

OBJECTIVE: The main objective was to study survival and neurologic evolution of children who suffered in-hospital pediatric cardiac arrest (CA). The secondary objective was to analyze the influence of risk factors on the long term outcome after CA. METHODS: prospective, international, observational, multicentric study in 48 hospitals of 12 countries. CA in children between 1 month and 18 years were analyzed using the Utstein template. Survival and neurological state measured by Pediatric Cerebral Performance Category (PCPC) scale one year after hospital discharge was evaluated. RESULTS: 502 patients with in-hospital CA were evaluated. 197 of them (39.2%) survived to hospital discharge. PCPC at hospital discharge was available in 156 of survivors (79.2%). 76.9% had good neurologic state (PCPC 1-2) and 23.1% poor PCPC values (3-6). One year after cardiac arrest we could obtain data from 144 patients (28.6%). PCPC was available in 116 patients. 88 (75.9%) had a good neurologic evaluation and 28 (24.1%) a poor one. A neurological deterioration evaluated by PCPC scale was observed in 40 patients (7.9%). One year after cardiac arrest PCPC scores compared to hospital discharge had worsen in 7 patients (6%), remained constant in 103 patients (88.8%) and had improved in 6 patients (5.2%). CONCLUSION: Survival one year after cardiac arrest in children after in-hospital cardiac arrest is high. Neurologic outcome of these children a year after cardiac arrest is mostly the same as after hospital discharge. The factors associated with a worst long-term neurological outcome are the etiology of arrest being a traumatic or neurologic illness, and the persistency of higher lactic acid values 24h after ROSC. A standardised basic protocol even practicable for lower developed countries would be a first step for the new multicenter studies.

Cardiac arrest and resuscitation in the pediatric intensive care unit: a prospective multicenter multinational study.

OBJECTIVE: The aim of the study was to analyze the mortality and neurological outcome factors of in-pediatric intensive care unit (in-PICU) cardiac arrest (CA) in a multicenter international study. PATIENTS AND METHODS: It was a prospective observational multicenter study in Latin-American countries, Spain, Portugal, and Italy. A total of 250 children aged from 1 month to 18 years who suffered in-PICU CA were studied. Countries and patient-related variables, arrest life, support-related variables, procedures, and clinical and neurological status at hospital discharge according to the Pediatric Cerebral Performance Category (PCPC) scale were registered. The primary endpoint was survival at hospital discharge and neurological outcome at the same time was the secondary endpoint. Univariate and multivariate logistic regression analyses were performed. RESULTS: Return of spontaneous circulation maintained longer than 20 min was achieved in 172 patients (69.1%) and 101 (40.4%) survived to hospital discharge. In the univariate analysis, oncohematologic diseases, inotropic infusion at the time of CA, sepsis and neurologic causes of CA, primary cardiac arrest, need of adrenaline, bicarbonate or volume expansion during resuscitation, and long duration of resuscitation were related with mortality. In the multivariate logistic regression analysis, factors related to mortality were hemato-oncologic illness and previous treatment with vasoactive drugs at the time of CA event, neurological etiology of CA, and cardiopulmonary resuscitation (CPR) duration for more than 10 min. One year after CA, neurological status was assessed in 65 patients; among them, 81.5% had mild disabilities or none. CONCLUSIONS: Survival with good neurological outcome of CA in the PICU is improving. The most important prognostic indicator is the duration of resuscitation.

In-hospital pediatric cardiac arrest in Spain.

INTRODUCTION AND OBJECTIVES: The objective was to analyze the characteristics and prognostic factors of in-hospital pediatric cardiac arrest in Spain. METHODS: A prospective observational study was performed to examine in-hospital pediatric cardiac arrest. Two hundred children were studied, aged between 1 month and 18 years, with in-hospital cardiac arrest. Univariate and multivariate logistic regression analyses were performed to assess the influence of each factor on survival to hospital discharge. RESULTS: Return of spontaneous circulation was achieved in 74% of the patients and 41% survived to hospital discharge. The survival rate was significantly higher than that reported in a previous Spanish study 10 years earlier (25.9%). In the univariate analysis, the factors related to mortality were body weight higher than 10 kg; continuous infusion of vasoactive drugs prior to cardiac arrest; sepsis and neurological disorders as causes of cardiac arrest, the need for treatment with adrenaline, bicarbonate, and volume expansion, and prolonged cardiopulmonary resuscitation. In the multivariate analysis, the factors related to mortality were hematologic/oncologic diseases, continuous infusion of vasoactive drugs prior to cardiac arrest, cardiopulmonary resuscitation for more than 20 min, and treatment with bicarbonate and volume expansion. CONCLUSIONS: Survival after in-hospital cardiac arrest in children has significantly improved in recent years. The factors related to in-hospital mortality were hematologic/oncologic diseases, continuous infusion of vasoactive drugs prior to cardiac arrest, the duration of cardiopulmonary resuscitation, and treatment with bicarbonate and volume expansion.

Parada cardiaca pediátrica intrahospitalaria en España / In-hospital Pediatric Cardiac Arrest in Spain

Introducción y objetivos: Analizar las características y los factores pronósticos de la parada cardiaca intrahospitalaria en España. Métodos: Se realizó un estudio prospectivo observacional en el que se incluyó a 200 niños, de edades entre 1 mes y 18 años, con parada cardiaca intrahospitalaria. Se realizó un estudio univariable y multivariable para analizar la influencia de los factores en la supervivencia al alta del hospital. Resultados: En un 74% de los pacientes se logró la recuperación de la circulación espontánea y el 41% sobrevivía al alta del hospital. La supervivencia fue mayor que la del estudio realizado 10 años antes (25,9%). En el estudio univariable, los factores relacionados con la mortalidad fueron el peso superior a 10 kg, el tratamiento con fármacos vasoactivos en perfusión continua antes de la parada, la sepsis y la enfermedad neurológica como causas de la parada cardiaca, la necesidad de tratamiento con adrenalina, bicarbonato y expansión de volumen, y un tiempo de reanimación cardiopulmonar largo. En el estudio multivariable, los antecedentes hematooncológicos, el tratamiento previo con fármacos vasoactivos, la duración de la reanimación cardiopulmonar superior a 20 min, el tratamiento con bicarbonato y la expansión de fluidos fueron los factores relacionados con la mortalidad. Conclusiones: La supervivencia a la parada cardiaca intrahospitalaria en la infancia ha mejorado significativamente en los últimos años. Las enfermedades hematooncológicas, el tratamiento previo con fármacos vasoactivos, la duración de la reanimación cardiopulmonar y el tratamiento con bicarbonato y expansión de líquidos son los factores asociados con la mortalidad al alta hospitalaria (AU)

Introduction and objectives: The objective was to analyze the characteristics and prognostic factors of in-hospital pediatric cardiac arrest in Spain. Methods: A prospective observational study was performed to examine in-hospital pediatric cardiac arrest. Two hundred children were studied, aged between 1 month and 18 years, with in-hospital cardiac arrest. Univariate and multivariate logistic regression analyses were performed to assess the influence of each factor on survival to hospital discharge. Results: Return of spontaneous circulation was achieved in 74% of the patients and 41% survived to hospital discharge. The survival rate was significantly higher than that reported in a previous Spanish study 10 years earlier (25.9%). In the univariate analysis, the factors related to mortality were body weight higher than 10 kg; continuous infusion of vasoactive drugs prior to cardiac arrest; sepsis and neurological disorders as causes of cardiac arrest, the need for treatment with adrenaline, bicarbonate, and volume expansion, and prolonged cardiopulmonary resuscitation. In the multivariate analysis, the factors related to mortality were hematologic/oncologic diseases, continuous infusion of vasoactive drugs prior to cardiac arrest, cardiopulmonary resuscitation for more than 20 min, and treatment with bicarbonate and volume expansion. Conclusions: Survival after in-hospital cardiac arrest in children has significantly improved in recent years. The factors related to in-hospital mortality were hematologic/oncologic diseases, continuous infusion of vasoactive drugs prior to cardiac arrest, the duration of cardiopulmonary resuscitation, and treatment with bicarbonate and volume expansion (AU)

Post return of spontaneous circulation factors associated with mortality in pediatric in-hospital cardiac arrest: a prospective multicenter multinational observational study.

INTRODUCTION: Most studies have analyzed pre-arrest and resuscitation factors associated with mortality after cardiac arrest (CA) in children, but many patients that reach return of spontaneous circulation die within the next days or weeks. The objective of our study was to analyze post-return of spontaneous circulation factors associated with in-hospital mortality after cardiac arrest in children. METHODS: A prospective multicenter, multinational, observational study in 48 hospitals from 12 countries was performed. A total of 502 children aged between 1 month and 18 years with in-hospital cardiac arrest were analyzed. The primary endpoint was survival to hospital discharge. Univariate and multivariate logistic regression analyses were performed to assess the influence of each post-return of spontaneous circulation factor on mortality. RESULTS: Return of spontaneous circulation was achieved in 69.5% of patients; 39.2% survived to hospital discharge and 88.9% of survivors had good neurological outcome. In the univariate analysis, post- return of spontaneous circulation factors related with mortality were pH, base deficit, lactic acid, bicarbonate, FiO2, need for inotropic support, inotropic index, dose of dopamine and dobutamine at 1 hour and at 24 hours after return of spontaneous circulation as well as Pediatric Intensive Care Unit and total hospital length of stay. In the multivariate analysis factors associated with mortality at 1 hour after return of spontaneous circulation were PaCO2 < 30 mmHg and >50 mmHg, inotropic index >14 and lactic acid >5 mmol/L. Factors associated with mortality at 24 hours after return of spontaneous circulation were PaCO2 > 50 mmHg, inotropic index >14 and FiO2 ≥ 0.80. CONCLUSIONS: Secondary in-hospital mortality among the initial survivors of CA is high. Hypoventilation, hyperventilation, FiO2 ≥ 0.80, the need for high doses of inotropic support, and high levels of lactic acid were the most important post-return of spontaneous circulation factors associated with in-hospital mortality in children in our population.

Factors associated with mortality in pediatric in-hospital cardiac arrest: a prospective multicenter multinational observational study.

PURPOSE: To analyze prognostic factors associated with in-hospital cardiac arrest (CA) in children. METHODS: A prospective, multicenter, multinational, observational study was performed on pediatric in-hospital CA in 12 countries and included 502 children between 1 month and 18 years. The primary endpoint was survival at hospital discharge. Univariate and multivariate logistic regression analyses were performed to assess the influence of each factor on mortality. RESULTS: Return of spontaneous circulation was achieved in 69.5 % of patients; 39.2 % survived to hospital discharge and 88.9 % of survivors had good neurological outcome. The pre-arrest factors related to mortality were lower Human Development Index [odds ratio (OR) 2.32, 95 % confidence interval (CI) 1.28-4.21], oncohematologic disease (OR 3.33, 95 % CI 1.60-6.98), and treatment with inotropic drugs at the time of CA (OR 2.35, 95 % CI 1.55-3.56). CA and resuscitation factors related to mortality were CA due to neurological disease (OR 5.19, 95 % CI 1.49-18.73) and duration of cardiopulmonary resuscitation greater than 10 min (OR 4.00, 95 % CI 1.49-18.73). Factors related to survival were CA occurring in the pediatric intensive care unit (PICU) (OR 0.38, 95 % CI 0.16-0.86) and shockable rhythm (OR 0.26, 95 % CI 0.09-0.73). CONCLUSIONS: In-hospital CA in children has a low survival but most of the survivors have a good neurological outcome. Some prognostic risk factors cannot be modified, making it important to focus efforts on improving hospital organization to care for children at risk of CA in the PICU and, in particular, in other hospital areas.

Hyperoxia, hypocapnia and hypercapnia as outcome factors after cardiac arrest in children.

PURPOSE: Arterial hyperoxia after resuscitation has been associated with increased mortality in adults. The aim of this study was to test the hypothesis that post-resuscitation hyperoxia and hypocapnia are associated with increased mortality after resuscitation in pediatric patients. METHODS: We performed a prospective observational multicenter hospital-based study including 223 children aged between 1 month and 18 years who achieved return of spontaneous circulation after in-hospital cardiac arrest and for whom arterial blood gas analysis data were available. RESULTS: After return of spontaneous circulation, 8.5% of patients had hyperoxia (defined as PaO(2)>300 mm Hg) and 26.5% hypoxia (defined as PaO(2)<60 mm Hg). No statistical differences in mortality were observed when patients with hyperoxia (52.6%), hypoxia (42.4%), or normoxia (40.7%) (p=0.61). Hypocapnia (defined as PaCO(2)<30 mm Hg) was observed in 13.5% of patients and hypercapnia (defined as PaCO(2)>50 mm Hg) in 27.6%. Patients with hypercapnia or hypocapnia had significantly higher mortality (59.0% and 50.0%, respectively) than patients with normocapnia (33.1%) (p=0.002). At 24h after return of spontaneous circulation, neither PaO(2) nor PaCO(2) values were associated with mortality. Multiple logistic regression analysis showed that hypercapnia (OR, 3.27; 95% CI, 1.62-6.61; p=0.001) and hypocapnia (OR, 2.71; 95% CI, 1.04-7.05; p=0.04) after return of spontaneous circulation were significant mortality factors. CONCLUSIONS: In children resuscitated from cardiac arrest, hyperoxemia after return of spontaneous circulation or 24h later was not associated with mortality. On the other hand, hypercapnia and hypocapnia were associated with higher mortality than normocapnia.

Galactosèmia com a causa d’ascites i colèstasi neonatal / Galactosemia as a cause of neonatal ascitis and cholestasis

Introducció. La galactosèmia és un trastorn del metabolismed’afectació multisistèmica secundari a la impossibilitatde metabolitzar correctament la galactosa, amb la conseqüentacumulació d’aquesta en forma de galactosa 1-fosfata les cèl·lules del parènquima hepàtic, renal i cerebral.Observació clínica. Exposem el cas d’un nadó de 32 diesde vida que presenta icterícia colestàtica, distensió abdominalimportant i trastorn de síntesi hepàtica. L’ecografiaabdominal mostra ascites a tensió sense causa extrahepàticaresponsable. S’inicia estudi de trastorn metabòlic i esdescarta dèficit d’alfa-1 antitripsina, tirosinèmia o fibrosiquística, entre d’altres, alhora que s’obtenen cossos reductorsen orina suggestius de galactosèmia. L’estudi intraeritrocitariva permetre el diagnòstic evidenciant augment degalactosa 1-fosfat i manca d’activitat de l’enzim GALT (galactosa1-fosfat uridiltransferasa). L’exclusió de galactosade la dieta va permetre frenar la progressió cap a la fallidahepàtica i la recuperació completa del pacient.Comentaris. La particularitat d’aquest cas rau en l’ascitesa tensió com a debut de la malaltia en absència de capaltra simptomatologia molt més característica, i en la greuevolució potencial sense l’exclusió de galactosa precoç

Introduction. Galactosemia is a metabolic disease withsystemic manifestations secondary to the inability to metabolizegalactose, resulting in accumulation of galactose-1-phosphate in liver, kidneys and brain.Case Report. We describe the case of a 32-day old infantwho presented with cholestatic jaundice, abdominaldistension and deficits in hepatic synthetic function. Abdominalultrasound showed tension ascitis without an obviousextra-hepatic cause. A metabolic work-up was performed,and alpha-1-antitrypsine, tyrosinemia, and cysticfibrosis were ruled out. The presence of reducing substancesin the urine suggested the diagnosis of galactosemia.Increased concentration of galactose-1-phosphate, and deficitof galactose-1-phosphate uridyl transferase werefound in red blood cells, thus confirming the diagnosis. Eliminationof galactose from the diet reversed the liver damageand the patient recovered completely.Comments. The unique feature of this case was thepresentation with tension ascitis in the absence of themore characteristic symptoms of the disease. The early eliminationof galactose from the diet prevented furtherprogression

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