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Biomedical subjects

A J Mares

Publications and source records attributed to A J Mares.

At least 55 records · Page 3Linked to original sources

Anterolateral diaphragmatic hernia: is it a Morgagni hernia?

Three unusual cases of large anterolateral congenital diaphragmatic hernia are described with emphasis on its embryogenesis. This type of diaphragmatic defect is rare and underemphasized. Its clinical spectrum ranges from the asymptomatic case to those with acute respiratory distress. It may present itself as a solitary defect or as part of a more complex association such as the thoracoabdominal syndrome. This defect should be differentiated from Morgagni (and other anterior) diaphragmatic hernias by its size and lateral extension and the presence of left pulmonary hypoplasia. Treatment is by primary operative repair, and prognosis depends on the presence and severity of associated malformations.

Abnormalities, Multiple↗

Acute acalculous cholecystitis caused by Salmonella typhi in a 6-year-old child.

A rare case of acute acalculous cholecystitis caused by Salmonella typhi in a 6-year-old child is presented. The clinical signs were fulminant, with diffuse peritonitis being suspected. Cholecystostomy and i.v. ceftriaxone proved efficacious and the girl was discharged in less than two weeks. The appropriate literature is reviewed.

Ceftriaxone↗

Torsion of uterine adnexa in neonates and children: a report of 20 cases.

Twenty children under the age of 14 years with torsion of the uterine adnexa are discussed. Of the 14 postnatal cases, 8 were associated with additional ovarian pathology whereas in 6 cases the torsion was of normal uterine adnexa. Six additional cases had antenatal torsion with "pseudocyst" formation. All 14 postnatal cases presented as acute abdominal emergency requiring immediate surgery. Only 6 of the 14 had been correctly diagnosed preoperatively, the rest being mostly diagnosed as acute appendicitis. Sonographic studies can improve the preoperative diagnosis. Hemorrhagic necrosis of the adnexa (secondary to the torsion) was found in all cases except one, and necessitated adnexal resection. In only one case preservation of the adnexa was possible. Fixation of the contralateral ovary is recommended in cases of torsion of normal uterine adnexa. All six cases of antenatal torsion were diagnosed by routine ultrasonography during pregnancy between the 29th and 40th week of gestation. The "fluid-fluid" level sign is characteristic of this lesion. These neonates were born in no acute distress and were found to be completely asymptomatic. Hence, they did not require urgent surgery and underwent operation electively at the age of 2 weeks. Resection of the pseudocyst with fixation of the contralateral ovary is the treatment of choice in these cases.

Abdomen, Acute↗

Erythrocyte acetylcholinesterase activity in Hirschsprung's disease in Israel.

Hirschsprung's disease is a relatively prevalent disorder in newborn babies and young children. Acetylcholinesterase (AChE) activity is augmented locally, in the neural plexus of the affected gut segment. It has been suggested that this enzyme will also be increased in red blood cells (RBCs) of affected children. Thus, we studied AChE activity in newborn babies and children, as well as age-adjusted data. This Israeli study consisted of healthy and affected Bedouins as well as Ashkenazi and Sephardic Jews. This study supports the Japanese study in that the RBC AChE is not augmented in Hirschsprung's disease. However, final conclusions cannot yet be drawn, because of the small sample.

Acetylcholinesterase↗

Pitfall in diagnosis of Crohn's disease in a cystic fibrosis patient.

Cystic fibrosis (CF) and Crohn's disease may both present as failure to thrive and recurrent intestinal obstruction. Proper treatment and adequate nutrition may reverse these manifestations and improve the patient's quality of life. We describe a girl with CF who, despite appropriate management, failed to grow and had several episodes of bowel obstruction. After the additional diagnosis of Crohn's disease was reached, the patient improved on antiinflammatory and nutritional therapy. This patient illustrates the pitfall in the diagnosis of Crohn's disease in a CF patient due to the clinical overlap between the two conditions. We suggest that therapeutic failure in a chronic disease justifies additional diagnostic efforts resulting in a completion of diagnosis and significant changes in management.

Child↗

The thoracoabdominal syndrome (TAS): a new X-linked dominant disorder.

We report on a family with a previously undescribed malformation syndrome. The syndrome is composed of diaphragmatic and ventral herniae, hypoplastic lung, and associated cardiac anomalies. The relative number of affected males and females and appreciably severer manifestations in males suggest an X-linked dominant inheritance.

Abdominal Muscles↗

Pulmonary plasma-cell granuloma.

A large pulmonary plasma-cell granuloma (PCG) mimicking a mediastinal germinal-cell tumor in a 9-year-old boy is presented. The nonspecificity of the tumoral calcifications and of the radiographic and CT findings in pulmonary PCG is demonstrated.

Calcinosis↗

Surgical management of the cleft sternum.

Cleft sternum is a rare disturbance of unknown origin in the normal central midline fusion. Three cases of upper cleft sternum are reported here, one associated with a pericardium to the skin sinus tract. Surgical correction of this anomaly in the neonatal period is advised, whether the infant is symptomatic or not. Simple closure of the defect during the first month of life avoids the more complex reconstruction necessary in older children.

Humans↗

Congenital disseminated neurofibromatosis: a "benign" diagnosis with malignant prognosis.

A neonate with congenital disseminated neurofibromatosis involving skin, subcutaneous tissues retropharyngeal, retroperitoneal and urinary bladder is presented. The massive presentation precluded radical surgical approach. This seems to be the first report of congenital widely disseminated neonatal neurofibromatosis in the English literature.

Abdominal Neoplasms↗

Antenatal torsion of the uterine adnexa in a neonate.

An antenatal detection of unilateral torsion of uterine adnexa was made by means of ultrasonography on the 32nd gestational week. This presented as an asymptomatic large "pseudo-cystic" mass, and was resected electively at the age of two weeks. A "fluid-fluid" level on ultrasonography is highly suggestive of this rare entity. This is the fourth case in the English literature to have been detected antenatally, and among the first dozen to be reported in the neonatal period.

Adult↗

Congenital esophageal stenosis. Transendoscopic web incision.

We treated a case of congenital membranous stenosis of the esophagus successfully by transendoscopic incision. We discuss various types of true congenital esophageal stenosis and their treatment, to distinguish these rare anomalies from the more frequently encountered acquired variety.

Endoscopy↗