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Biomedical subjects

J C Adkins

Publications and source records attributed to J C Adkins.

5 recordsLinked to original sources
PubMed1989

Familial abdominal wall defects.

We report 2 families, each having multiple sibs with abdominal wall defects. In family 1, normal parents gave birth to identical (monochorionic, diamniotic) twins. This is the first reported case of gastroschisis occurring in monozygotic twins. In family 2, a normal mother gave birth to a son with omphalocele. Two subsequent pregnancies with a different husband resulted in a stillborn girl with partial atresia of the colon and a liveborn girl with gastroschisis. In neither case were there any associated anomalies. In neither of the 2 families was there consanguinity or history of other abdominal wall defects. The familial occurrence of these defects suggests that 1) multifactorial determination should be considered in at least some cases of abdominal wall defects, 2) the bowel atresias that occasionally accompany gastroschisis may also have a genetic (multifactorial) cause, and 3) some cases of gastroschisis and omphalocele may have the same genetic cause.

Abdominal Muscles↗
PubMed1986

Neurofibromatosis and childhood tumors.

Although tumors are a known complication of von Recklinghausen's neurofibromatosis (NF), they often develop after the second decade and their characteristics in the pediatric population are unclear. To define the frequency and distribution of tumor types in children with NF, the authors reviewed the experience at Children's Hospital of Pittsburgh between 1953 and 1984. During that time, 121 children younger than 18 years with documented NF were seen. Three patients (2.5%) developed locally extensive, unresectable sarcomas, two of which were clearly neural in origin. Two other children died from complications of highly cellular but not histologically malignant neurofibromas. Seventeen patients (14%) had brain tumors, of which 3 (2.5%) were malignant astrocytomas and 9 were optic gliomas. Two children had acute myelogenous leukemia. Although it has been said that patients with NF are particularly susceptible to the oncogenic effects of radiation, 12 children were treated with a median of 5000 rad to their tumor bed. Follow-up after radiation in that small cohort of patients ranged from 6 months to 12 years (mean, 6 years). Eleven of 12 patients experienced adequate local control. During the follow-up period, none developed a second tumor outside or within the radiation port that could be related to radiation. The authors conclude that clinically significant tumors in children with NF, not all of which are histologically malignant, are not rare. Although more prospective information is needed on response to and sequelae from therapy, the data in this report suggest no contraindication to aggressive therapy.

Adolescent↗
PubMed1977

The operative management of von Recklinghausen's neurofibromatosis in children, with special reference to lesions of the head and neck.

Eighty-five patients with von Recklinghausen's neurofibromatosis were seen at the Children's Hospital of Pittsburgh from 1952 through 1976. Nineteen of these patients had significant head or neck extracranial lesions. Based upon these and a personal series of seven other head and neck patients, we emphasize several critical points in the care of these patients: (1) large, painful, conspicuous, or strategically placed tumors should be removed: (2) early removal is easier and improves cosmetic results; (3) neck and chest tumors carry a high risk of subsequent rapid vertebral angulation with possible paralysis; (4) close observation allows timely application of appropriate surgical procedures.

Adolescent↗
PubMed1976

Imperforate anus.

Sucessful treatment of the child with imperforate anus yields an individual with satisfactory continence and a healthy urinary tract. These goals are achieved by accurate evaluation of the newborn, optimal technical results, and conscientious follow-up. The infant born with one of these malformations frequently has a serious associated anomaly which also requires early specific diagnosis and management. When the bowel has passed through the puborectalis in utero, the deformity can often be definitively treated in the neonate by dilations or anoplasty. However, if a urinary or high vaginal fistula is present, or if the anomaly is of the supralevator type, definitive surgery is best postponed some months and the neonate given a colostomy. We have found the sacroabdominoperitoneal approach to be the most satisfactory pullthrough procedure for the supralevator lesion. The children with high, supralevator anomalies have higher mortality rates and, in one out of four cases, have suboptimal fecal continence. They require the utmost in technical skill and continuing care if they are to lives free of social stigma. The mortality rate in all other types is low and the functional continence uniformly acceptable.

Abnormalities, Multiple↗