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Biomedical subjects

M T Gyepes

Publications and source records attributed to M T Gyepes.

At least 19 recordsLinked to original sources

Treatment of overt isolated testicular relapse in children on therapy for acute lymphoblastic leukemia. A report from the Childrens Cancer Group.

BACKGROUND: Fifty-seven children with acute lymphoblastic leukemia (ALL) receiving therapy who experienced overt isolated testicular relapse while in bone marrow remission were entered into a study that featured an intensive retreatment regimen. METHODS: The objective was to determine whether a change in chemotherapy and local irradiation would prevent subsequent marrow relapse and increase the survival rate. The regimens used (modified Berlin-Frankfurt-Munster or modified New York) delivered acceptable therapy based on analyses of toxicity data. RESULTS: Overall survival at 5 years from the time of testicular relapse was 47%, with an event-free survival of 43%. Events have been documented in 28 of 55 evaluable children. Analysis of these patients revealed that 23 children had bone marrow relapse, 4 children had central nervous system relapse, and 1 child had testicular relapse. In addition, two patients were removed from the study for toxicity, one child for infection (mucormycosis), and five children had a bone marrow transplantation while in remission and became ineligible to continue in the study. Two children were removed at the request of their parents, and nine children were lost to follow-up. CONCLUSION: Because of their high risk of developing systemic relapse, boys with ALL who experience isolated overt testicular relapse during active therapy should be retreated with intensive treatment.

Adolescent

Beckwith-Wiedemann syndrome: development of nephroblastoma during the surveillance period.

Two children, with Beckwith-Wiedemann syndrome and hemihypertrophy who were followed by ultrasonography, developed a large nephroblastoma in the interval between two abdominal ultrasound examinations. The customarily suggested bi-annual examinations are not frequent enough and may give physicians and parents a false sense of security.

Beckwith-Wiedemann Syndrome

Jejunal atresia with 'apple peel' deformity. A report of eight survivors.

Jejunal atresia with an "apple peel" deformity is one of the most severe forms of intestinal atresia. Until quite recently, neonatal mortality rates as a result of this anomaly were high. Early diagnosis, appropriate surgery, and prolonged parenteral nutrition have led to a spectacular improvement of the survival rates. We are reporting eight cases of apple peel atresia--all eight patients survived. The pathophysiology, diagnostic aspects, surgical approaches, and postoperative management are discussed.

Female

Radiographic-endoscopic correlations in the examination of airway disease in children.

The results of radiographic examinations and flexible fiberoptic endoscopy (FFE) of the airways were correlated in 100 pediatric patients. For the diagnosis of laryngomalacia, tracheal stenosis and tracheal granulomas. FFE proved to be more accurate than radiography. In epiglottitis and croup, the two methods were comparable. In bronchial and resulting parenchymal disease, the chest films served as the endoscopist's guide because the consequences of bronchial obstruction manifested rapidly on chest roentgenograms. FFE was particularly useful when the chest films did not reveal or lateralize a clinically suspected foreign body. FFE was not error-free; in a small number of cases, radiologic methods provided critical diagnoses, which were not arrived at by endoscopy.

Adolescent

Chronic idiopathic intestinal pseudo-obstruction syndrome. Radiologic signs in children with emphasis on differentiation from mechanical obstruction.

The radiographic pattern of chronic idiopathic intestinal pseudoobstruction syndrome (CIIPS) in children includes: (a) manifestation of motility disorder, and (b) signs of bowel occlusion. The findings on plain films are identical to those of mechanical obstruction. However, when there is a clinical history of chronic symptoms of a disparity between the patient's clinical condition and the radiographic findings, opaque studies are indicated. Signs suggestive of CIIPS are: (a) an absence of stricture; (b) disorganized, decreased or absent motility in any segment of the gastrointestinal tract; (c) dilated loops of bowel, particularly the proximal portion of the duodenum (so-called superior mesenteric artery syndrome), and (d) decreased or absent haustrations and/or redundancy in the colon.

Adolescent

Wilms tumor, misdiagnosed preoperatively: a review of 19 National Wilms Tumor Study I cases.

This study establishes the difficulty in making specific diagnoses in children with retroperitoneal tumors when only excretory urograms are used in diagnosis. Complementary use of excretory urography and ultrasonography should make the number of preoperative errors less by separating cases of Wilms tumor from those with cystic disease. This investigation underscores the hazards inherent in the use of preoperative irradiation and chemotherapy, since some children with benign disease are likely to receive inappropriate therapy.

Child

Radiographic manifestations of bone marrow transplantation in children.

Radiographically detectable complications in 35 children after bone marrow transplant are reviewed. These complications are most frequently due to infection, chemoradiotherapeutic toxicity, and graft versus host disease (a transplant rejection phenomenon peculiar to bone marrow transplant patients). The pulmonary complications within the first 2 months are secondary to a form of interstitial lung disease. Interstitial lung disease has a strong correlation with graft versus host disease. Extrapulmonary visceral complications include hepatosplenomegaly, nephromegaly, and hemorrhagic cystitis. These are due to graft versus host disease, radiation, and chemotherapeutic toxicities, respectively. Sinusitis, cerebral atrophy, and intracerebral hematomas are less frequent complications. Osteoporosis due to steroids is the single most important osseous complication.

Adolescent

"Contrast buttle": a sign indicating perforation of the digestive tract.

Bubble formation outside the digestive tract that correlated with small perforations was observed in two infants during water-soluble contrast material studies using a 50% solution of Gastrografin in water. The causes of extraluminal bubbles are discussed; in vitro experiments demonstrated the mechanism of formation and the radiographic characteristics of the various types of bubbles. Extraluminal bubbles appearing during a water-soluble contrast material examination actually represent contrast bubbles. The radiographic density of the bubble walls varies. When the bubbles are small and diluted contrast material is used, it is not possible to detect the presence of contrast material radiographically. Contrast material bubbles are likely to be observed only if a low surface tension contrast agent is used, such as Gastrografin full- or half-strength in water). Extraluminal contrast material bubble formation may identify and localize a small perforation of the gut when other findings are inconclusive.

Contrast Media

Complete obstruction of the gastric antrum in children following acid ingestion.

We report on two children who experienced delayed complete obstruction of the gastric antrum following concentrated acid ingestion. Both patients required initial tube gastrostomy and subsequent antrectomy with intestinal reconstruction. Unlike the more common alkaline corrosives, ingested acids tend to spare the esophagus and gastric fundus. While gastric perforation and vascular collapse may occur immediately following overwhelming acid ingestion, the more common course is chronic gastric antral inflammation with subsequent fibrosis and, in some cases, complete stricture. Delayed surgical reconstruction is recommended to permit the acute inflammation and edema to subside.

Adolescent

Renovascular hypertension in children and adolescents.

Renovascular disease often leads to hypertension in children. The most frequent cause is fibromuscular dysplasia of focal type affecting main and peripheral arteries. Diastolic readings in excess of 110 mm Hg with normal serum creatinine and urinalysis are suggestive of renovascular disease. Excretory urography was positive in 65% of patients with unilateral disease. Radionuclide scans complement a positive excretory urogram but may be positive when the urogram is negative. Plasma renin activity was raised in the majority of patients; if the patient does not have peripheral branch stenosis, the renal vein renin ratio will lateralize in unilateral renal disease. The overall results of surgery are encouraging: 86% of surgical procedures alleviated hypertension in unilateral disease.

Adolescent

Obstruction of the airways by the heart and pulmonary vessels in infants.

It is well known that certain cardiovascular abnormalities, frequently compress the airways in infants [1]. Two additional cardiac mechanisms of airway obstruction are described in this paper: progressive compression of the bronchial tree by left sided cardiac enlargement and infantile cardiac asthma due to compression of the small airways by pulmonary venous congestion. Functional and reversible collapse of the trachea is a frequent finding in cardiac asthma. Infants with these entities often appear to have primary pulmonary disease when in fact the underlying cause of their distress is cardiac in origin.

Airway Obstruction