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Biomedical subjects

J W Hanson

Publications and source records attributed to J W Hanson.

At least 19 recordsLinked to original sources
PubMed1979

Oculodentodigital dysplasia. Four new reports and a literature review.

Four new patients with oculodentodigital dysplasia (ODD) have been examined. The salient and fairly constant features of ODD appear to be (1) unique facial appearance, (2) microcornea with other inconstant ocular findings, (3) syndactyly of the hands with additional characteristic phalangeal aberrations, (4) diffuse skeletal dysplasia, (5) enamel dysplasia, and (6) trichosis. Echographic studies indicate that ODD globes have microcornea with otherwise normal dimensions. An increased number of vessels crossing the optic discs was observed in three patients from one family. The distance between the inner canthi and the medial orbital walls in three patients we studied suggests that previous reports of hypertelorism may have been illusions resulting from microcornea, small palpebral fissures, and variably present epicanthus.

Adolescent↗
PubMed1979

The Summitt syndrome: observations on a third case.

A 6 1/2 year old male presented with acrocephaly, brachydactyly, clinodactyly, mild syndactyly of the hands and feet, genu valgum, and marked obesity. Roentgenograms of the hands revealed hypoplasia or aplasia of the middle phalanges. Roentgenograms of the feet revealed hypoplasia of the middle phalanges and deformity of the proximal phalangeal epiphyses of the great toes. Chromosomes studies revealed a normal 46,XY karyotype, and psychological testing revealed low normal intelligence. Current data support autosomal recessive inheritance, although X-linkage cannot be excluded.

Child↗
PubMed1979

Pentasomy X with multiple dislocations.

We describe a pentasomy X (49,XXXXX) patient whose multiple dislocations led to a consideration of the Larsen syndrome. Review of the 11 reported cases of pentasomy X showed that elbow dislocations are known to occur in this syndrome. Our patient is the first to present hypoplasia of the glenoid process with consequent should dislocation. Clinical and radiologic findings of previously reported cases of pentasomy X are reviewed.

Abnormalities, Multiple↗
PubMed1979

Helmet treatment for plagiocephaly and congenital muscular torticollis.

Plagiocephaly usually originates in late fetal life through aberrant constraint of the fetal head and is often associated with sternocleidomastoid torticollis. In some patients the obligue head shape may become a permanent, cosmetic disability. Individually fitted plastic helmets, similar in style to football helmets, have been successfully used to remold the deformed heads of four infants. Each helmet is designed to fit snugly against the prominent aspects of the infants' cranium and to be loose fitting where the head is shallow. As the brain grows, the head is molded to fit the helmet and thus acquires a more usual shape. The treatment has been safe and effective. When torticollis is persistent after the initiation of treatment, rubber straps can be fixed to the outside of the helmet and attached to the side rails of the crib. While the infant sleeps, the straps can be tightened to stretch the shortened sternocleidomastoid muscle.

Casts, Surgical↗
PubMed1979

Fetal injury with midtrimester diagnostic amniocentesis.

One hundred seven infants born after midtrimester amniocentesis were examined. The frequency of fetal injury was 9%, and was directly related to the number of attempts at amniocentesis. All were minor cutaneous injuries except for 1 case of disruption of a patellar tendon. Suggestions are made regarding ways to reduce the incidence of fetal puncture.

Adult↗
PubMed1978

The effects of moderate alcohol consumption during pregnancy on fetal growth and morphogenesis.

Information on fetal hazards arising from moderate or low levels of maternal alcohol consumption is unavailable in man. In order to study this question an unselected group of pregnant women was interviewed during pregnancy regarding their alcohol intake. At the time of delivery pairs of high-risk and control infants were examined without knowledge of maternal drinking history. Of 163 infants examined, 11 were judged clinically to show signs compatible with a prenatal effect of alcohol on growth and morphogenesis. Nine of these 11 came from the high-risk drinking group. Only two of these infants were classified as having the fetal alcohol syndrome, and each of the mothers was a very heavy drinker. The other seven infants, who showed lesser alterations of growth and morphogenesis suggestive of fetal alcohol syndrome, were born to women who reported drinking an average of one ounce or more of absolute alcohol per day in the month prior to recognition of pregnancy. These results indicate that both moderate and high levels of alcohol intake during early pregnancy may result in alterations of growth and morphogenesis in the fetus.

Alcohol Drinking↗
PubMed1978

Trisomy 3 (p23-pter) resulting from maternal translocation, t (3 ; 4)(p23 ; q35).

We describe a patient with partial trisomy 3p resulting from maternal translocation, t(3:4)(p23;q35). The male newborn who died at the age of 22 hours presented with distinct facial features including a square-shaped face with prominent forehead and depressed temporal regions, prominent cheeks, short broad nose, left cleft lip and cleft palate, malformed ears, and a receding mandible. Further findings were flexion deformities of the fingers with finger-like thumbs and mild cutaneous syndactyly 2/3 and 4/5, hypoplastic penis and scrotum with no palpable testes. He probably had a congenital heart defect and situs inversus abdominalis. Many of these features have been reported in other patients with distal trisomy 3p.

Abnormalities, Multiple↗
PubMed1977

Subtotal neonatal calvariectomy for severe craniosynostosis.

This report sets forth an example of a new mode of management of severe craniosynostosis in the neonate: subtotal calvariectomy. An infant with synostosis of sagittal, coronal, and lambdoidal sutures who had signs of increased intracranial pressure with impending neurologic complications was operated on at 13 days of age. Wide excision of the bony calvarium from the underlying dura was accomplished from the supraorbital ridge to near the foramen magnum; the entire area of the coronal sutures was included. Regeneration of the calvarium occurred over two to 12 weeks and was accompanied by the presence of "sutures" in a usual location. These procedures have allowed for normal brain growth without reoperation up to the current age of two years. Neurologic and intellectual performance has been satisfactory, and cosmetic results appear to be preferable to those obtained by more limited surgery. The observations suggest that this approach may permit better craniofacila growth by minimizing secondary deformities in growth of the cranial base.

Child, Preschool↗
PubMed1977

Saethre-Chotzen syndrome: a broad and variable pattern of skeletal malformations.

A family is described in which 15 persons in five generations are affected with a complex of skeletal malformations which variably includes peculiar asymmetric facies, delayed closure of large fontanels, brachycephaly, acrocephaly, brachydactyly, cutaneous syndactyly, broad great toes, and mild shortness of stature. Although craniosynostosis is either lacking or relatively mild in the members of this family, their features are otherwise strikingly similar to those of patients with the Saethre-Chotzen syndrome. We believe the findings in this family indicate that the Saethre-Chotzen syndrome comprises a broad pattern of carniofacial and other skeletal malformations in which craniosynostosis may sometimes occur.

Acrocephalosyndactylia↗
PubMed1977

Alcohol and the fetus.

Current data clearly point to an association between chronic maternal alcoholism and serious morphological and developmental abnormalities in the fetus. When these abnormalities are severe the condition is recognizable as the fetal alcohol syndrome. However, this represents only the most severe end of a spectrum of abnormalities, some of which may be found in a proportion of infants exposed to lesser amounts of alcohol during pregnancy. Alcohol is an avoidable hazard for the fetus, but only through increased awareness by the medical community and improved education of the lay public may we hope to control this problem.

Abnormalities, Drug-Induced↗
PubMed1976

Fetal alcohol syndrome. Experience with 41 patients.

Forty-one patients with the fetal alcohol syndrome, including 11 whose cases were previously reported, were studied. These children have a pattern of defects including prenatal and postnatal growth deficiency, small head size with mental subnormality, and facial abnormalities allowing for recognition of the disorder in infancy. The same pattern of abnormalities has been independently reported from France in a series of 127 offspring of chronic alcoholics.

Abnormalities, Drug-Induced↗
PubMed1976

Risks to the offspring of women treated with hydantoin anticonvulsants, with emphasis on the fetal hydantoin syndrome.

The fetal hydantoin syndrome is a variable pattern of altered growth and performance which includes unusual facies, distal phalangeal hyoplasia, and other defects occurring in some infants exposed in utero to hydantoins. A prospective study of 35 infants exposed prenatally to this class of anticonvulsants showed that 11% had sufficient features to be classified as having the fetal hydantoin syndrome. An additional 31% displayed some features compatible with the prenatal effects of hydantoins. A case-control study of 104 infants whose mothers received hydantoins during pregnancy supports these conclusions. Reduction of intellectual ability in infants with the fetal hydantoin syndrome is the area of greatest concern. Women being treated with hydantoin anticonvulsants should be told of the nature and magnitude of risks to the developing fetus before considering a pregnancy.

Abnormalities, Drug-Induced↗
PubMed1976

Prominent lateral palatine ridges: developmental and clinical relevance.

Unusually prominent lateral palatine ridges have been found to be a nonspecific feature of a variety of disorders in which there is either neuromotor dysfunction or a malformation which prevents tongue thrust into the palatal vault. Observations of the lateral palatine ridges in 3 fetal specimens and in 260 normal individuals over a wide range of ages revealed that these structures are normally more prominent during prenatal life and infancy. With increasing age these ridges normally become progressively flattened and usually disappear by age 5 years. The observation of unusual prominence of these ridges in infants with neuromuscular dysfunction as well as in those with malformations which limit tongue thrust into the palatal vault suggests that a long-standing deficit of tongue thrust is the common pathogenetic mechanism. Prominent lateral palatine ridges may be misinterpreted as a true "narrow, high-arched palate", which is a much less common anomaly. This distinction is important clinically, since prominent lateral palatine ridges most commonly imply a long-term deficit of neuromuscular function and thus may be an important diagnostic clue to alterations dating back to early prenatal development.

Adolescent↗