Infant with Clinical Evidence of Pulmonary Hypoplasia: A Case Report.

Pulmonary hypoplasia is the incomplete development of lung tissue. A reduced number of lung cells, airways, and alveoli is the hallmark and can be seen unilaterally or in both lungs. The diagnosis, however, is usually made upon pathologic examination.

Brown adipose tissue in the buccal fat pad during infancy.

Background: The buccal fat pad (BFP) is an encapsulated mass of adipose tissue thought to enhance the sucking capabilities of the masticatory muscles during infancy. To date, no conclusive evidence has been provided as to the composition of the BFP in early postnatal life.

Objective: The purpose of this study was to examine whether the BFP of neonates and infants is primarily composed of white adipose tissue (WAT) or brown adipose tissue (BAT).

Glycosaminoglycan metabolism defects and atherosclerosis: frequent association of endothelial dysfunction in patients with Mucopolysaccharidosis.

Cardiovascular lesions, including coronary artery stenosis, are frequently associated and can cause sudden death in patients with genetic defects of glycosaminoglycan (GAG) metabolism. Early diagnosis of coronary artery lesions is difficult, although potentially lifesaving. Histopathological similarities between atherosclerotic changes in adults and in patients with genetic GAG metabolism defects have been known.

Human BAT possesses molecular signatures that resemble beige/brite cells.

Brown adipose tissue (BAT) dissipates chemical energy and generates heat to protect animals from cold and obesity. Rodents possess two types of UCP-1 positive brown adipocytes arising from distinct developmental lineages: "classical" brown adipocytes develop during the prenatal stage whereas "beige" or "brite" cells that reside in white adipose tissue (WAT) develop during the postnatal stage in response to chronic cold or PPARγ agonists. Beige cells' inducible characteristics make them a promising therapeutic target for obesity treatment, however, the relevance of this cell type in humans remains unknown.

Unequivocal identification of brown adipose tissue in a human infant.

We report the unique depiction of brown adipose tissue (BAT) by magnetic resonance imaging (MRI) and computed tomography (CT) in a human 3-month-old infant. Based on cellular differences between BAT and more lipid-rich white adipose tissue (WAT), chemical-shift MRI and CT were both capable of generating distinct signal contrasts between the two tissues and against surrounding anatomy, utilizing fat-signal fraction metrics in the former and x-ray attenuation values in the latter. While numerous BAT imaging experiments have been performed previously in rodents, the identification of BAT in humans has only recently been described with fusion positron emission and computed tomography in adults.

Developmental regulation of p66Shc is altered by bronchopulmonary dysplasia in baboons and humans.

Rationale: The p66(Shc) adapter protein antagonizes mitogen-activated protein, or MAP, kinase, mediates oxidative stress, and is developmentally regulated in fetal mouse lungs.

Objectives: To determine if p66(Shc) is similarly regulated in primates and in bronchopulmonary dysplasia (BPD), which results from oxidative injury to immature lungs.

Methods: Normal and injured lungs from humans and baboons were evaluated by Western analysis and immunohistochemistry.

Causes of nursery death beyond the neonatal period.

Objective: To investigate causes of death in infants who died after 28 days, beyond the neonatal period but before discharge from the nursery, to establish their clinical courses and causes of death and to attempt to find criteria for earlier identification of these infants.

Methods: We identified 30 such infants (12% of nursery deaths) from 1993 through 1998 and conducted a retrospective review of their records including placental pathology and autopsy reports when available. In all, 14 infants who weighed View Article and Find Full Text PDF

A rare presentation of Pompe disease with massive hypertrophic cardiomyopathy at birth.

We report a term infant with Pompe disease presenting in the immediate newborn period. The infant was born at 40 weeks' gestation, weighing 3600 g to a 32 year-old black female. Infant presented at delivery with massive hypertrophic cardiomyopathy and pulmonary hypertension.

Two-year outcome of infants weighing 600 grams or less at birth and born 1994 through 1998.

Objective: To assess the neurologic and developmental outcome at 2 years of age in preterm infants with birth weights 600 g or lower.

Method: We conducted a retrospective review from January 1994 through December 1998 for placental histopathology, maternal factors, neonatal intensive care unit course, growth, neurologic/special sense status, and development at 2 years of age corrected for prematurity.

Results: Of the 104 neonates weighing 600 g or less, 24 survived to nursery discharge (23%).