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Biomedical subjects

J L Grosfeld

Publications and source records attributed to J L Grosfeld.

At least 19 recordsLinked to original sources

Cholecystitis and cholelithiasis in children.

Cholecystitis and cholelithiasis are being recognized with increasing frequency in infancy and childhood. Hematologic disorders continue to account for a large proportion of cases; however, many children are noted with cholelithiasis in association with total parenteral nutrition, ileal disorders, and prolonged fasting. Spontaneous stone resolution has been noted in many cases in infancy and a period of observation is indicated in the absence of obstructive symptoms. Cholecystectomy remains the procedure of choice for symptomatic cholelithiasis and cholecystitis and a laparoscopic procedure is possible in most cases after infancy.

Adolescent

Gastroesophageal reflux in infants and children. Diagnosis and management.

Eighty-three infants and children underwent surgical correction of gastroesophageal reflux (GER) from 1973 to 1978. Fifty-four patients had coexistent brain damage (most commonly due to cerebral palsy), eight were previously treated for esophageal atresia, and four had gastroschisis or omphalocele repair. Clinical presentation included failure to thrive in 64 patients, vomiting in 59, and recurrent bouts of aspiration pneumonitis in 43. Barium roentgenography showed GER in 61 patients, whereas additional tests (particularly pH monitoring) were required for detection of GER in 22 patients. After failure of medical management, transabdominal Nissen fundoplication was performed in 80 cases and a Hill repair in three cases. The surgical mortality was zero, but there were five late deaths. Results were considered excellent in 54 patients, good in 22 patients, and poor in seven. Ten of 12 patients with preoperative stricture responded to dilation after fundoplication. Nissen fundoplication was a safe and effective antireflux procedure in 76 of the 83 cases.

Adolescent

Multiple pulmonary fibroleiomyomatous hamartomata in childhood.

A 9-yr-old girl with a history of rhabdomyosarcoma developed multiple pulmonary fibroleiomyomatous hamartomata. This clinical setting illustrates the tendency of these hamartomata to clinically mimic metastatic tumors. Further, this case represents the first description of multiple pulmonary fibroleiomyomatous hamartomata in a pediatric age group, and finally, in this clinical setting, supports the concept that these hamartomata represent a distinct entity apart from benign metastasizing uterine leiomyomata.

Abdominal Neoplasms

Adverse effects of endotoxin following massive distal bowel resection.

Sepsis is a frequent cause of morbidity following extensive bowel resection. It has been suggested that the lymphoid tissues of the gut may be essential to normal humoral immunity. This study evaluates: (1) the effect of endotoxin on mortality following selective massive bowel resection and jejunoileal bypass; (2) cellular immunity by skin allograft rejection and bypass, and (3) T and B cell lymphocyte subpopulations is mesenteric lymph nodes, intestine and appendix. Endotoxin increased mortality in rats with more distal bowel resection but not following bypass. Skin allograft rejection was similar in each group. Peyer's patches, mesenteric lymph nodes and appendices were evaluated for T & B cell subpopulations. These tissues had a greater percentage of B cells (53% lymph nodes, 63% appendix) with IgM the predominant immunoglobulin. Cellular immunity was not a factor. Lymphoid tissues of the distal bowel and mesentery contain abundant B cells and IgM that may contribute to humoral immunity. Massive bowel resection may increase the risk of morbidity from gram negative sepsis and/or endotoxin presumably due to decreased humoral immunity.

Animals

Respiratory paralysis to improve oxygenation and mortality in large newborn infants with respiratory distress.

The nonsynchronous respiratory efforts of neonates with surgically correctable disorders may inhibit effective mechanical ventilation. The records of 25 infants treated with metocurine for muscular paralysis to improve mechanical ventilation were reviewed. All patients were greater than 35 (37.6 +/- 2.1) weeks gestation and 2.27 (2.98 +/- .47) kg. All required ventilatory support with an FiO2 of 100%. The mortality rate of this group of infants was 20% as compared with 73% (p < .001) in a similar group of 26 infants managed without paralysis. In 10 of the 25 infants treated with metocurine, pre- and 1 hr postparalysis paO2 values were available. The mean paO2 prior to paralysis was 62 (45--111) mm Hg and the mean post-paralysis paO2 was 144 (75--227) mm Hg, representing at 132% increase in paO2 (p < .001). The mean dosage for metocurine was 3.5 (1.45--6.79) mg/kg/day; however, those requiring paralysis for greater than 7 days showed a dramatically increasing requirement. These preliminary data suggest that respiratory paralysis reduces right-to-left shunting, improves paO2 and decreases mortality in large infants with severe respiratory distress requiring ventilatory support.

Diaphragm

Reversal of protein-energy malnutrition in children during treatment of advanced neoplastic disease.

The effectiveness of enteral and parenteral feeding in supporting a satisfactory nutritional status and/or reversing protein-energy malnutrition was evaluated in 28 children, ages 1-19 (14 female) with advanced malignant disease (21 solid tumors, 7 leukemia-lymphoma). At the onset of treatment, 21 patients received intensive nutritional counseling (INC) and oral supplementation while seven received total parenteral nutrition (TPN). Sixteen of 21 patients who received INC had a decreased intake (x 48 +/- 24%) Recommended Dietary Allowances (RDA) for kilocalories and dramatic weight loss (x 16.4 +/- 12.4%). A total of 18 patients received TPN for a mean of 24 days (7-60); kcal averaged 90 +/- 26% RDA during weight gain. At onset of TPN, the mean serum albumin, transferrin and total lymphocyte counts were 3.06 +/- 0.38 g/dl, 175 +/- 62 mg/dl, and 1102 +/- 966/mm3 respectively, 15/18 children had subnormal anthropometric measurements and 17/18 patients were anergic to recall skin test antigens. TPN for less than 9-14 days neither repleted weight, skinfold reserves, nor serum albumin concentrations (greater than 3.2 g/dl) although an early increase (p less than .02) in transferrin concentration was observed. However, TPN for 28 days supported weight gain (3.27 kg, 16 +/- 6%), increased serum albumin (0.62 +/- 0.43 g/dl, p less than .001) and transferrin (62 +/- 42, p less than .002) to normal concentrations and reversed anergy in 7/11 patients retested. This study documents the severity of protein energy malnutrition which accompanies intense treatment of children with cancer and the nutritional and immunological benefits of a 28 day course of TPN.

Adolescent

Disseminated varicella complicating ulcerative colitis.

Disseminated varicella is an uncommon complication of ulcerative colitis, despite the frequent use of steroid therapy in this disorder. Fatal varicella infection occurred in a patient with ulcerative colitis who was receiving steroid therapy. This observation suggests that zoster-immunoglobulin should be administered to patients exposed to varicella with ulcerative colitis who are receiving steroid therapy.

Adolescent

A critical evaluation of the Duhamel operation for Hirschsprung's disease.

Eighty-nine patients with Hirschsprung's disease were treated from 1972 to 1977. Of the patients, 70 were boys, 85 were the result of full-term pregnancy, and four had Down's syndrome. There were 23 children, 32 infants, and 34 neonates including seven (20%) with enterocolitis. Early mortality was 8.8% in neonates and 3% in infants. After initial diversion (colostomy or enterostomy), 66 patients had "classic" (ten), modified (52), or "long" (four) Duhamel pull-through procedures at age 1 year. Operative mortality was zero. Three late deaths occurred; two of them were mongols. Fecaloma occurred in eight of ten classic cases that required revision. Postoperative enterocolitis occurred in three of four long procedures done for total colonic aganglionosis. The modified Duhamel procedure was associated with a low complication rate and good function. There were no instances of anastomotic leak, anal stricture, or genitourinary problems. These data indicate that the modified Duhamel operation is a highly acceptable procedure in the management of Hirsch sprung's disease.

Adolescent

A critical assessment of fluid requirements in gastroschisis.

This report critically evaluates the fluid requirements of 30 infants with gastroschisis. Fluid requirements were unrelated to gestational age, birth weight, or type of repair. Twenty-five percent of the fluid was colloid needed to restore intravascular volume and oncotic pressure, the balance consisting of Ringer's lactate solution. These data support the conclusion that the fluid requirements of the newborn with gastroschisis are significantly increased over those of the normal neonate. This is a reflection of antenatal peritonitis, third space fluid shifts and loss of water and electrolytes during transport to the pediatric surgical center. Recognition of these needs and appropriate replacement should reduce the perioperative morbidity and mortality in these seriously ill neonates.

Abdominal Muscles

Experience with "second-look" operations in pediatric solid tumors.

"Second-look" operations were performed in 32 infants and children with initially unresectable or recurrent solid tumors treated with combination chemotherapy and/or irradiation. Tumors were resectable in 26 of 32 cases (81%). These procedures often yielded information affecting staging and treatment. Disease-free survival was achieved in 18 of 32 patients (56%). Mortality was related to progressive disease in seven cases and opportunistic infections due to immunosuppression in three. Four additional patients are alive with evidence of persistent tumor. Second-look procedures were beneficial in patients with Wilms' tumor previously operated upon by a flank approach and in children with bilateral tumors. These procedures were particularly useful in children with stage III localized neuroblastoma and cases of metastatic neuroblastoma that respond to chemotherapy. Second-look operations were also useful in selected cases of rhabdomyosarcoma, teratoma, and Ewing's sarcoma. These observations suggest that combination chemotherapy has increased the use of second-look operations in a variety of less favorable (e.g. initially unresectable or recurrent) pediatric solid tumors.

Antineoplastic Agents