Enfermedad de Kawasaki: Un caso con pseudo-obstrucción intestinal y aneurisma gigante / Kawasaki disease: A case with intestinal pseudo-obstruction and giant aneurism

We present a rare association of Kawasaki disease in a two year old boy presenting with fever, a morbiliform rash and clinical signs of intestinal pseudo-obstruction. Our patient subsequently developed a giant aneurysm of the right coronary artery complicated with thrombosis. He presented fever again 48 hours after the first dose of Immunoglobulin and needed a second one with good results. These patients with giant coronary aneurysms have a greater risk of developing stenosis and myocardial infarction, and require anticoagulant therapy, frequent stress scans and coronary angiographies. Those who develope less severe disease need only antiplatelet therapy and less frequent cardiovascular tests. Patients with a normal echocardiogram after the acute phase of the disease are not treated. The long-term prognosis of this disease in uncertain, especially regarding the possible association with coronary disease in adult life.

Enfermedad de Kawasaki: a propósito de un caso atípico y con intususcepción / Kawasaki`s disease: atypical case with intussusception

We present a rare association of atypical Kawasaki disease and intussusception in a three month old male patient. it all began with high fever and an obstructive intestinal syndrome developed in the second day of hospitalization, diagnosed as a colo-colonic intussusception. Despite an adecuate postoperative antibiotic coverage the high fever continued, which made us pursue a diagnosis of a vasculitis syndrome among other entities. The echocardiogram showed dilatation of both coronary arteries. The currenty accepted clinical criteria for Kawasaki disease may not always allow its identification in patients who are younger than six months of age. A diagnosis of Kawasaki disease and an echocardiographic evaluation of the coronary arteries should be considered in young infants with prolongued fever of unkown origin. (Rev Med Hered 2001; 12: 37-41).

Sindrome de Poland: un caso con dextrocardia / Poland syndrome: a case report with detrocardia and review of the literature

Reportamos un caso de anomalía de Poland (ausencia del músculo pectoral mayor y braquisindactilia en la mano ipsilateral) con dextrocardia. La ausencia del músculo pectoral mayor sin anomalías del miembro superior puede ser una variantes de esta anomalía. En la mayoría de casos este sindrome ocurre en el hemicuerpo derecho, usualmente sin defectos costales. Se reporta este caso, debido a la asociación de dos rasgos que no son comunes en el sindrome de Poland, como es su presentación en el lado izquierdo y su asociación con dextrocardia. Asimismo, este paciente presenta defecto en la parrilla costal que usualmente está presente cuando la anomalía primaria está en el lado izquierdo.

Síndrome de Klinefelter con deficiencia parcial de hormona de crecimiento / Klinefelter sindrome with partial deficiency of growth hormone

We present a case of Klinefelterïs syndrome and short stature due to partial growth hormone deficiency. His height was below the third percentile for age and his bone age lagged behind four years. Cases like this are generally due to the presence of a an isochromosome Xq or to an isolated partial or total deficiency of growth hormone, or to partial or panhypopituitarism. We wish de emphasize the rare association between Klinefelter syndrome and growth hormone deficiency.

Toxoplasmosis y amigdalitis / Toxoplasmosis and tonsolitis

Acquired toxoplasmosis is a widespread, usually asymptomatic zoonotic infection caused by an intracellular protozoan parasite Toxoplasma gondii. Due to its predilection for parenchymal cells and those of the reticuloendothelial system, involvement of the lymphoid tissue is the most common clinical finding. This patient presented with abdominal pain predominantly in the right lower quadrant, which dissapeared promptly, painful cervical lymph node involvement and a severe case of acute exudative tonsilitis which is exceedingly unusual. Diagnostic levels of IgM against Toxoplasmosis highly suggests that the patient had acute or a very recent infection with the protozoan. It is difficult to determine whether the parasite was the cause of her illness or that she acquired a concomitant viral infection, like adenovirus. It would had been important and diagnostic to do a biopsy of the cervical lymph node involved, which was not done due to reasons out of our reach.

Esófago de Barret en pediatría: revisión de la literatura a propósito de un caso / Barret's esophagous in pediatrics: report of one case and review of the literature

This patient presented at one year of age with a dry and persistent cough prodominantly nocturnal, pneumonia on two occasions, and signs and symptoms compatible with bronchial asthma. Due to the high incidence of gastroesophageal reflux in patients with esophageal atresia, an entity that could be responsible for his symptoms, diagnostic procedures were done to confirm this diagnosis, and treatment was begun with cisapride during one month, but without improvement. It was then that a esophagogastroscopy was performed and Barret's esophagus was diagnosed. The patient was medicated with cisapride and omeprazole, and two months later a Thal Fundoplication was done. The patient has continued on cisapride and omeprazole with great improvement, being asymptomatic for a period of nine months, and having had only on one occasion a respiratory crisis due to bronchospasm. A repeat esophagogastroscopy has been planned one year after his operation.

Miositis aguda postinfecciosa / Acute post infection myositis

A seven year old patient is described with acute focal myositis, following an influenza-like infection, involving exclusively the gastroenemius and soleus muscles. The patient improved only with bed rest and symptomatic treatment and made a full recovery in three days. Laboratory studies showed a normal white blood count with an increase in the band forms, elevated SGOT, DHL, and CPK, all of which returned to normal after one week. The patient remains asymptomatic one month after his discharge

Incontinentia pigmenti: A propósito de un caso / Incontinentia pigmenti

Incontinentia Pigmenti is a rare X-linked multisystem neuroectodermic disorder with signs and symptonms related mainly to the dermatologic, dental, ocular and central nervous systems, and characterized by death in the majority of male embryos. Affected children do not appear sick, in spite of the skin eruption, the peripheral leucocytosis and marked eosinophilia. Most of the cases are reported in caucasians, although there are description in black children, orientals, north, central and south american indians, and in our mestizos. The name of Incontinentia Pigmeti describes the incontinence of the melain pigment from the basal layer of the epidemermis into the superficial dermis

Displasia renal fetal=reconocimiento prenatal y cirugía precoz / Fetal kidney displasia=prenatal diagnosis and early surgery

Se presenta un caso de displasia renal derecha (riñón multiquístico unilateral derecho) en un recién nacido, cuyo diagnóstico se efectuó prenatalmente por estudio ecográfico. Se confirmó el diagnóstico a las 12 horas del nacimiento demostrándose por ecografía presencia en el riñón derecho de múltiples quistes con ausencia total de corteza renal y mediante gammagrafía renal, la total falta de función en el riñón afectado con indemnidad total y normal funcionamiento del riñón izquierdo. No se encontró otra alteración siendo normales el resto de las investigaciones efectuadas al bebé. Al quinto día de vida se practica nefrectomía derecha con excelentes resultados y una evolución normal del paciente. Se efectúa una revisión de la literatura sobre el tema, con especial énfasis sobre los diferentes métodos y su importancia en el descubrimiento intrauterino de las malformaciones congénitas primordialmente del sistema genitourinario fetal