Ebstein's anomaly and extracardiac defects.

Ebstein's anomaly of the tricuspid valve occurs as an isolated defect with other forms of congenital heart disease such as transposition of the great arteries or tetralogy of Fallot or, rarely, in association with extracardiac malformations. Because so little is known about this latter group, we studied four cases clinically, at autopsy, and by means of a retrospective chart review. Major extracardiac changes most often involved the craniofacial region, central nervous system, and limbs.

Clinical efficacy and safety of teicoplanin in the treatment of gram-positive infections.

This study was an open efficacy and safety study of teicoplanin in hospitalized patients with gram-positive infections. 26 patients entered the study. Teicoplanin was administered by intravenous bolus injection at a dose of 200 mg or 400 mg every 24 h, and in all cases an initial loading dose of 400 mg was given.

Megalencephaly in sudden infant death syndrome.

The fresh brain weights of 79 infants with sudden infant death syndrome (SIDS) were tabulated and compared with expected "normal" brain weights. The series included 47 males and 32 females. Their ages ranged from 8 days to 16 months with a median of 2.

Congenital posterolateral diaphragmatic hernia: associated malformations.

Congenital posterolateral diaphragmatic hernia (CDH) is widely regarded as an isolated defect, but this view is incomplete. We reviewed our clinical and autopsy experience and the literature from the past 25 years in order to catalogue the frequency and clinical importance of additional malformations in patients with CDH. The study showed two broad categories of patients.

Lower lid blepharoplasty with skin flap and muscle split.

A skin flap, muscle split lower eyelidplasty is recommended for the management of excess skin of the lower eyelids associated with herniated infraorbital fat pads. Precise fat pad removal and skin excision are necessary.

Characterization of 15 selected coccal bacteria isolated from Antarctic rock and soil samples from the McMurdo-Dry Valleys (South-Victoria Land).

Approximately 1500 cultures of microorganisms were isolated from rocks and soils of the Ross Desert (McMurdo-Dry Valleys). From these, 15 coccoid strains were chosen for more detailed investigation. They were characterized by morphological, physiological and chemotaxonomical properties.

Atelencephalic microcephaly: craniofacial anatomy and morphologic comparisons with holoprosencephaly and anencephaly.

This is the first detailed report of the craniofacial anatomy of atelencephaly. Comparisons were made with a control specimen and others with holoprosencephaly, anencephaly, and anencephaly with holoprosencephalic facial features. In the atelencephalic fetus, severe microcephaly, flattened calvaria, and partial synostosis of the metopic suture were apparent.

Duplication 7p in a family with t(7;11): association with anomalies of the anterior cranial base.

Duplication 7p has been observed in association with several balanced translocations. Of eight previously recorded cases, only one exhibited duplication of the entire short arm of chromosome 7. We report on a newborn infant with multiple congenital anomalies and an abnormal chromosome constitution: 46,XX,-11,+der(11),t(7;11) (p11.

[Chronic lichenoid dermatitis in sensitization to alpha-amylase in a baker].

We report on the sensitization of a baker against alpha-amylase contained in a dough additive. Positive test reactions to alpha-amylase were observed after 20 min., 6 h, and 72 h, even following heat inactivation.

Chest size and symmetry in congenital diaphragmatic hernia.

Infants with congenital diaphragmatic hernia have significantly increased chest circumferences. This implies that intrathoracic volumes are increased as well. Forces produced by the herniated abdominal viscera seem to provide the chief impetus for this change.

Prenatal growth of the median face.

The growth and development of the median facial region remain incompletely understood. Data concerning the prenatal size and growth rates of facial components are especially lacking. A group of fetuses and newborn infants with apparently normal craniofacial development and body size was therefore studied at autopsy.

Atelencephalic microcephaly in a 21-week human fetus.

Atelencephalic microcephaly, a rare and extreme disorder, is known morphologically by only six cases. Derivatives of the telencephalon are absent or dysplastic, while more caudal structures are normal or mildly deformed. A more extensive form, aprosencephaly, involves structures of the diencephalon and may be associated with holoprosencephalic facies.

Relationships of cardiac and somatic growth in infancy and childhood.

Recent advances at clinical and experimental levels bespeak the need for a more complete understanding of cardiac growth and its relationship to somatic growth. The size of the heart is integral to several physiologic processes, including aerobic and work capacity, athletic performance, and reproduction. Because the morphologic foundations for cardiac size are laid in large part before adulthood, cardiac growth and the factors controlling it are important in achieving optimal cardiovascular fitness.

Pathologic features of the CHARGE association: support for involvement of the neural crest.

Defects associated with choanal atresia include coloboma, cardiac anomalies (usually involving the conotruncal region), physical or mental retardation, genital hypoplasia, and abnormalities of the ear. This constellation of defects is known as the "CHARGE" association and may be accompanied by other anomalies. Many of these defects seem to result from abnormalities in the development, migration, or interaction of cells of the cephalic neural crest.

A morphometric study of normal and abnormal fetal to childhood tongue size.

The weight, length, width and thickness of 83 normal tongues from patients aged 25 weeks of gestation to 10.5 years were compared statistically with several measures of body and head size and changes described by linear regression; the relationships between measures were examined by partial and least-squares correlation. Sex differences in tongue size were not evident.

Left ventricular hypoplasia in congenital diaphragmatic hernia.

Quantitative anatomic study of the hearts of eight infants with left-sided congenital diaphragmatic hernia (CDH) has revealed significantly decreased cardiac mass, due to hypoplasia of the left atrium and ventricle and interventricular septum. These morphologic deficiencies may be a result of compression of mediastinal structures by herniated abdominal viscera during prenatal life. Left ventricular hypoplasia is likely to be an important factor in the pathogenesis of cardiac insufficiency in patients with left CDH.

Self-reported physical stress reactions: first- and second-order factors.

Stress Inventory-6, a 72-item self-report questionnaire designed to tap 18 factors of physical stress reactions, was subjected to factor analysis. The instrument was given to 474 students (254 females, 206 males, and 14 who failed to indicate their gender). The final factor solution identified 16 first-order and 4 second-order factors.

Unilateral skin lesions associated with multiple neoplasms.

A girl with unilateral partly depigmented, partly hyperpigmented atrophic skin lesions, hypertrichosis, postauricular nodules, cataracts, and mild mental retardation is presented. Additionally, she developed multiple neoplasms as optic glaucoma, a psammomatous meningioma and an ameloblastoma. It remains unclear whether our patient suffers from a variant of the epidermal nevus syndrome or a new neuro-ectodermal dysplasia.

Determinants in the morphogenesis of muscle tendon insertions.

The factors that normally determine the location and insertion of a muscle were explored in human experiments of nature with early problems in morphogenesis. Monozygotic conjoined twins for whom there could be no genetic determinants for muscle attachments at the sites of juncture served as one model; these attachments had to follow general principles of morphogenesis. A second type of problem involved absence of bone that presumably antedated muscle and tendon development (e.

Parasitic conjoined twins, two cases, and their relation to limb morphogenesis.

Reports of parasitic conjoined twins have appeared rarely in the scientific literature and only one study has included a detailed description of the parasitic extremities. We report here two specimens consisting for the most part of normal hosts with ectopic legs attached to the thorax (case 1, thoracoparasitus) or the abdomen (case 2, gastroparasitus). These two specimens are in agreement with several others reported in the literature in having no sign of vertebral columns.

Cyclopia: an anatomic and histologic study of two specimens.

The craniofacial complex of two neonatal, human, cyclopic specimens was studied in detail. Both specimens exhibited a single ocular opening and one had a prominent probosics positioned in the midline directly superior to the ocular aperture. No external nasal development was noted in the other specimen.