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Biomedical subjects

A J Ross

Publications and source records attributed to A J Ross.

At least 73 records · Page 4Linked to original sources

Massive gastrointestinal hemorrhage in children with posterior fossa tumors.

Over a 12-month period (September 1984 to September 1985), 64 children with newly diagnosed brain tumors were admitted to the Neurosurgical Service at The Children's Hospital of Philadelphia. Of these children, 29 had posterior fossa tumors. Of this population of children with posterior fossa tumors, three patients aged 4 months, 22 months, and 4 years old developed massive exsanguinating upper gastrointestinal hemorrhage within seven days of their primary neurosurgical procedure. In each instance, large posterior duodenal ulcers were encountered and were treated with oversewing of the duodenal ulcer and vagotomy-pyloroplasty. Follow-up currently ranges from 18 to 26 months. All three children have survived and none have had any gastrointestinal bleeding since then. Massive exsanguinating hemorrhage was not seen in children with brain tumors in locations other than the posterior fossa. In this population of patients, we advocate the use of prophylactic cimetidine and titration of gastric acidity with antacids.

Antacids↗

Microdiverticulitis: a rare but distinct cause of small bowel obstruction in early infancy.

During a 20-year period, eight term infants admitted to a large children's hospital for treatment of dehydration associated with diarrhea (6) and vomiting (2), and who ranged in age from 10 to 60 days at onset of symptoms and 18 to 75 days at presentation, developed distal small bowel obstruction following apparently successful rehydration. With the exception of a single infant who was dead on arrival, and another whose obstruction went unrecognized, all infants came to operation shortly after obstruction was confirmed. In each case, a severely inflamed area of distal ileum (7) or proximal colon (1) was found at autopsy (2) or operation (6) to be the cause; perforation was present in four of the cases. Resection of the diseased segments of intestine, and primary anastomosis, were performed in all six operated cases; reoperation was required in four of the six for leaks (3) and adhesions (1). All but two survived. Pathologically, the resected intestinal segments showed a unique pattern of injury: numerous punctate ulcers were apparent, which undermined the muscularis mucosae, without evidence of necrosis. Regenerating epithelium extended through these defects, resulting in the presence of glandular invaginations, which were surrounded by a brisk inflammatory response: hence the term "microdiverticulitis." We believe this lesion represents a beginning or furtive attempt at repair of severely inflamed, but viable intestine, and that it is a rare but true cause of small bowel obstruction in early infancy, separate and distinct from necrotizing enterocolitis.

Diverticulitis↗

Recent experience with choledochal cyst.

This report details an 11-year experience with 17 patients ranging from newborn to 17 years with choledochal cyst. Two distinct groups were noted: an infantile group (mean age: 3 months) with obstructive jaundice identical to biliary atresia and a late onset group (mean age: 9 years) with various combinations of pain, mass, and jaundice. Two patients had cystoduodenostomy performed and both required revision. One of six patients who had Roux-Y cystojejunostomy required revision. All seven patients who had primary cyst excision and two patients who had secondary cyst excision with Roux-Y hepaticojejunostomy have been followed prospectively and have done well. The follow-up period ranges from 1-11 years with an average of 5.8 years. Cyst excision should be performed as a primary or secondary procedure whenever feasible. The rare patients with intrahepatic ductal dilatation (Caroli's disease) are best approached by hepatic lobectomy when possible, and those with choledochocele should be treated by unroofing the cyst as indicated by the anatomy encountered.

Adolescent↗

Primary hyperparathyroidism in infancy.

Primary hyperparathyroidism in the neonate is a rare and often fatal disorder. These infants typically display severe hypercalcemia, respiratory distress, muscular hypotonia, and skeletal demineralization. They are usually diagnosed within the first three months of life and have hyperplasia of the four parathyroid glands. Twenty-nine infants with primary hyperparathyroidism are reported in the literature. Mortality is 87.5% in medically managed patients and 24% in surgically managed patients. Surgical management has not been satisfactory, in that recurrent hypercalcemia has been encountered in most patients undergoing subtotal parathyroidectomy, and total parathyroidectomy has resulted in the need for lifelong calcium and vitamin D supplementation. We have recently cared for a term newborn female in whom the diagnosis of primary hyperparathyroidism was made clinically on the second day of life, and later was confirmed biochemically. The baby underwent neck exploration on the 11th day of life and was successfully treated with total parathyroidectomy and parathyroid autotransplantation. Although initially rendered eucalcemic, the infant subsequently developed recurrent hypercalcemia requiring the removal of some of the autograft. Currently, the child is more than 2 years following surgery, growing well, and off all medication. The world literature is reviewed in this report of one of the first and the youngest infants, to our knowledge, to undergo parathyroid autotransplantation. In view of its success in avoiding the complication of repeated neck exploration for recurrent hyperparathyroidism or the creation of permanent hypoparathyroidism, we recommend this surgical approach for the rare neonate with primary hyperparathyroidism.

Biopsy↗

A new technique for evaluating cutaneous vascularity in complicated conjoined twins.

Ischiopagus tripus conjoined twins were recently encountered. All organ systems were thoroughly evaluated using radiographic, angiographic, radionuclide, and CT imaging techniques. None of these studies were capable of accurately assessing the vascular territories of the skin at the level of the pelvis, the most critical area in terms of separation. Qualitative visual assessment of tissue fluorescein delivery under ultraviolet illumination is subject to subjective errors. Perfusion fluorometry is a recently developed technique whereby tissue fluorescence can be quantitated over time in very small amounts, even with repeated injections. In these conjoined twins, using sequential fluorescein injections in each twin, it was possible to accurately determine which portions of the pelvis and the shared leg belonged to which twin and exactly where the skin incisions should be made. Additionally, during operation it was possible to accurately predict the viability of skin flaps used for closure. Both twins survived. This is the first time it has been possible to accurately assess vascular territories of the skin in a complicated form of conjoined twinning. The technique is also useful in the evaluation of flow patterns in various other parts of the body.

Aortography↗

Resolution of intractable cholestasis associated with total parenteral nutrition following biliary irrigation.

Although the protracted use of total parenteral nutrition (TPN) in infants is known to predispose to cholestasis, which in certain instances may not be reversible, failure to understand the pathogenesis of this condition has hindered the development of an effective medical treatment. That surgical treatment may reverse this process in selected patients is the subject of the present report. Two premature infants and one term infant, with short bowel syndrome acquired in infancy, developed conjugated hyperbilirubinemia following institution of balanced TPN. Infectious and metabolic etiologies for the jaundice were ruled out. No excretion of 99mTc-labeled DISIDA from the biliary tree was demonstrable 24 hours following injection by nuclear scintigraphy, despite the absence of extrahepatic biliary obstruction by abdominal sonography. When all conventional medical methods of managing the problem failed, exploration was performed. Intraoperative cholangiography showed normal intrahepatic and extrahepatic biliary ducts in each instance. Irrigation of the biliary tree with normal saline solution was then performed. In all cases, liver biopsies were obtained that were consistent with TPN-associated cholestasis and in all cases, jaundice resolved promptly following operation. We conclude that intractable cholestasis associated with TPN, regardless of its etiology, is a functional abnormality of biliary excretion that may respond in selected patients to irrigation of the biliary tree with radiographic dye and/or normal saline solution.

Biliary Tract↗

Morbidity and mortality of short-bowel syndrome acquired in infancy: an update.

The advent of total parenteral nutrition (TPN) has made survival beyond infancy possible for large numbers of patients who have sustained massive small intestinal loss due to a variety of intraabdominal catastrophes. However, the quantity and quality of life have been limited by the development of late sequelae due both to the protracted use of TPN and the long-term complications of foreshortening of the gut. To determine to what extent the morbidity and mortality of short-bowel syndrome (SBS) may have improved over the last 10 years, we reviewed our experience since 1973 with patients losing more than 50% of total small intestinal mass in infancy. The etiologies of SBS in the 16 study patients were necrotizing enterocolitis (6), midgut volvulus (5), multiple atresias (3), gastroschisis (1), and congenital SBS (1). Overall survival was 81%; total small intestinal length (SIL) at the time of diagnosis was 44.2 +/- 7.9 cm in survivors and 30.3 +/- 7.8 cm in nonsurvivors, probability values not significant. Although no patient survived without an ileocecal valve whose total SIL was greater than 20 cm, the three deaths in this series were not related directly to the SIL, but to end-stage liver disease resulting from TPN-associated cholestasis. Among the survivors, adaptation to enteral feedings required 13.8 +/- 2.5 mo, during which time weaning from TPN occurred; weight at adaptation was 6.87 +/- 1.32 kg.(ABSTRACT TRUNCATED AT 250 WORDS)

Child↗

Ciliated cutaneous cyst of the foot.

We report a 36-year-old woman with a cutaneous cyst of the dorsal aspect of the left foot. Because of the symptoms of pain and paresthesias attributed to the lesion, it was clinically felt to be a ganglion. After excision, these symptoms abated. Yet histologic examination showed this lesion to be a rare tumor, the ciliated cutaneous cyst. This is the third patient with this distinctive lesion on the foot or ankle.

Adult↗

Signal statistics in objective auditory evoked potential (AEP) detection by the phase spectral method.

This paper reports on statistical aspects relevant to the use of the phase spectrum of post-stimulus EEG, in objective detection of the auditory evoked potential. The sampling statistics of two statistical estimators are discussed: the mean phase vector magnitude, and the standard deviation of an ensemble of post-stimulus EEG phases. These two estimators are circular statistics, and subject to strong sample size bias. Their confidence intervals have been derived empirically for sample sizes routinely used in clinical audiometry. A trial example illustrates the use of the objective phase statistics developed here; it is noted that the method may also be more efficient than the visual scoring of averaged responses.

Adult↗

Effects of calcium and cyclic nucleotides on rat calcitonin and parathyroid hormone secretion.

Recently we developed a system for studying concurrent secretion of calcitonin (CT)and parathyroid hormone(PTH)in vitro from single rat thyroparathyroid gland complexes. In the present study, mechanisms involved in secretion of CT and PTH were explored by altering the medium [Ca ] and by using the Ca antagonist, verapamil. We also re-examined the idea that cyclic nucleotides may help regulate secretion of these hormones and attempted to determine if effects of cyclic nucleotides might be altered by changes in medium [Ca]. Thyroparathyroid glands from 8-day-old rats were incubated in serum-free medium for 8h, and CT and PTH levels in the medium were measured by radioimmunoassays. We show for the first time that: (1) although low [Ca] is well known to promote PTH release, some extracellular Ca is needed for PTH secretion to occur at a maximal rate; (2) inhibition of Ca entry into cells with verapamil mimics the effects of low medium Ca on both CT and PTH release; and (3) cyclic nucleotides may exert their effects on secretion of CT and PTH at least in part via effects on Ca entry into cells.

Animals↗