Hypereosinophilia, hyper-IgE syndrome, and atopic dermatitis in a toddler with food hypersensitivity.

We describe a 20-month-old girl with hypereosinophilia, hyper-immunoglobulin (Ig) E syndrome, and atopic dermatitis. Her peripheral eosinophil count and IgE plasma levels were 2.65 x 10(9)/L and 6702 IU/mL, respectively. Specific IgE levels for a variety of foods and inhalants were high and single-blind food challenges were positive for cow's milk, hen's egg, oat, wheat, and soy. When the patient received an extensively hydrolyzed milk formula, an exclusion diet, and 2 mg/kg of prednisone daily, the atopic dermatitis partially improved. Further improvement was observed with 1 mg/kg of azathioprine daily. Long-term clinical response was satisfactory. We suggest that food hypersensitivity should be ruled out in patients with hypereosinophilia, hyper-IgE syndrome, and atopic dermatitis. Azathioprine may be a good therapeutic alternative for treatment in such cases.

[Use of intravenous immunoglobulin in toxic epidermal necrolysis and Stevens-Johnson syndrome]. / Uso de inmunoglobulina intravenosa en el tratamiento de necrólisis epidérmica tóxica y síndrome de Stevens-Johnson.

Toxic epidermal necrolysis and Stevens-Johnson syndrome are described as variants of the same disease with distinct severity and constitute the most frequent cutaneous reactions in children, causing considerable morbidity. Several reports support the use of intravenous immunoglobulin therapy in these entities. We report the cases of two patients, one with toxic epidermal necrolysis and the other with Stevens-Johnson syndrome, in whom immunoglobulin treatment was successfully used. We also reviewed the outcomes of 13 patients with toxic epidermal necrolysis and Stevens-Johnson syndrome in the previous 10 years in the Hospital Infantil de Mexico, in whom conventional treatment was used.

[Fever of unknown origin. Presentation of 180 pediatric cases]. / Fiebre de origen obscuro. Presentación de 180 casos pediátricos.

Fever of unknown origin (FUO) is a frequent disorder in pediatric age. FUO is defined as the presence of fever over 38.4 centigrades in a patient for more than three weeks in which the etiology remains undetermined. From 30.736 consecutive admittances into our hospital, 180 patients with FUO were detected, and studied in a systematized way and according to a predetermined protocol. FUO was commonly found in children under six years of age. Our patients presented fever from three weeks to six and a half years of evolution; however, in the vast majority of the cases (n = 115) fever had a three weeks course. Several symptoms and physical manifestations other than fever were observed in our patients but they were widely variable and nonspecific. Infectious diseases were the commonest etiological factor encountered; among them, thyroid fever, and urinary tract infections were the most frequent infectious disorder found; in four children fever was associated to ampicillin administration; in 19 patients, fever was no demonstrated. A large number of laboratory investigations were done in the diagnosis of neoplastic diseases. We think that the study of a patient with FUO requires of a systematized approach.

[Congenital and neonatal varicella. A report on 9 cases]. / Varicela congénita y neonatal. Presentación de nueve casos.

Varicella infection in the first month of life is rare. Neonates may be infected through the placenta or in the perinatal period. In Mexico, the experience with neonatal varicella infection is limited; on the other hand this disease may be misdiagnosed in the neonate. The purpose of this work is to inform nine patients which presented varicella in the neonatal period; in six cases the disease was observed after the 10th but before the 30th day of life; in the remaining cases, the disease was present before the 10th day. Only one patient was premature. Five patients developed varicella in the hospital; in three cases, the mother presented the disease nine, eight, and one day prior delivery; one patient was exposed to varicella in his home. Four patients died but only in one case the death was attributed to varicella.

Development of West syndrome in a patient with Reye syndrome: a case study.

We report the case of a 3 month old female with a diagnosis of Reye Syndrome, confirmed with laboratory exams. Three months after apparently recovering from the acute clinical picture, the patient developed massive spasms and psychomotor delay with an EEG tracing typical of hypsarrythmia, the classic triade which suggests West Syndrome.

Rett's syndrome with Lennox-Gastaut pattern.

Three Latin American female patients studied in two different centers (Mexico City and Houston, Texas) had the clinical criteria of Rett's Syndrome, including progressive intellectual deterioration, stereotyped movements, loss of purposeful hand use, ataxia, and progressive disability. The three patients had normal laboratory studies including imaging studies (CT and MRI) but consistently abnormal EEGs with Lennox-Gastaut pattern. The usefulness of the EEG in the differential diagnosis and the association of this electrographic phenomenon are stressed.