Biomedical subjects

J L Grosfeld

Publications and source records attributed to J L Grosfeld.

At least 91 records · Page 5Linked to original sources

PubMed1993

Familial Hirschsprung's disease: 20 cases in 12 kindreds.

This report describes 20 infants and children with a family history of Hirschsprung's disease in 12 kindreds. A total of 260 patients were treated for Hirschsprung's disease (1972 to 1991), yielding a familial incidence of 8%. There were no families with consanguineous marriage. Sixteen patients were male and four were female. The mean age at diagnosis was 18 days. Clinical presentation included delayed passage of meconium in 15, abdominal distention in 11, vomiting in 9, feeding abnormalities in 3, and complete bowel obstruction in 1. Associated congenital anomalies occurred in 25% of the patients. The extent of aganglionosis was rectal in 4, sigmoid in 4, left colon in 2, transverse or right colon in 2, and total colonic in 8. Enterocolitis occurred in 7 patients (35%); 2 at diagnosis, 2 after an ostomy, and 3 after a pull-through procedure. There were no deaths associated with enterocolitis. All patients had a proximal diverting colostomy or ileostomy, and 19 of 20 underwent a definitive pull-through procedure. Three patients were lost to follow-up and one patient died of complications of multiple congenital anomalies unassociated with Hirschsprung's disease. Of the remaining 16 patients, all of whom have undergone a pull-through procedure, 11 are fully continent, 2 have nighttime soiling, 2 are too young to evaluate bowel function, and 1 still has an ostomy.(ABSTRACT TRUNCATED AT 250 WORDS)

Age Distribution↗

PubMed1993

Inguinal hernia and umbilical anomalies.

Inguinal hernias and umbilical anomalies remain the most common congenital anomalies. The loss of testis, ovary, or a portion of bowel from an irreducible hernia and the infectious complications of umbilical anomalies continue to be a threat to infants and young children. This article reviews the embryology, clinical features, and treatments of these anomalies and discusses some of the unusual and special considerations of these children.

Child↗

PubMed1993

Inguinal and umbilical hernia repair in infants and children.

Inguinal hernia repair is the most common operation performed in infants and children. In this article, the embryology, clinical presentation, and treatment of congenital inguinal hernias and umbilical hernias in children are reviewed. Specific attention is directed to such topics as the incarcerated hernia, hernias in girls, hernia repair in premature infants, and anesthetic considerations.

Child↗

PubMed1993

Changes of hepatoma echo patterns with tumor growth. A study of the microanatomic basis in a rat model.

OBJECTIVES: The echo patterns of human hepatoma vary considerably as the tumors grow. This sonographic study investigates the microanatomic basis of the echo-pattern changes in an animal model. METHODS: ACI rats (n = 35) with intrahepatically implanted Morris hepatoma 3924A were imaged with 10-MHz ultrasound from days 7 to 28 after tumor implantation. The sonograms were precisely correlated with the histologic sections and microangiograms obtained from the same section plane. RESULTS: Small hepatomas (size = 6.6 +/- 1.6 mm) without necrosis demonstrated a low-echo pattern. Medium-sized tumors (size = 11.7 +/- 3.3 mm) had a low-echo periphery and a high-echo center. The low-echo periphery corresponded to viable tumor and the high-echo core corresponded to central coagulation necrosis. Large tumors (size = 20.9 +/- 7.5 mm) with extensive necrosis revealed an irregular mixed-echo pattern. CONCLUSIONS: The trend of increasing echogenicity of the hepatomas with tumor growth is due to the progression of coagulation necrosis.

Animals↗

PubMed1993

Liver cancer ablation with extracorporeal high-intensity focused ultrasound.

Recent animal studies have demonstrated the capacity of high-intensity focused ultrasound (HIFU) to extracorporeally ablate selective tissue targets in the liver without requiring surgical exposure of the liver or insertion of instruments into the liver. The potential value of HIFU as a noninvasive local treatment for human hepatic cancers has attracted considerable interest. This report reviews the current status of HIFU research and sets forth questions for future study.

Animals↗

PubMed1993

Neuroblastoma in the first year of life: clinical and biologic factors influencing outcome.

The survival for infants less than 1 year of age with neuroblastoma is twice that of older children. This report describes 70 babies with neuroblastoma who experienced an 84% survival. Survival was 100% for stages I, II, and III, 81% for stage IV-S, and 50% for stage IV. Most survivors had favorable biologic characteristics including spontaneous regression, favorable Shimada histology, normal first chromosome, aneuploid tumors, absent or low N-myc oncogene expression, and normal serum ferritin levels.

Biomarkers, Tumor↗

PubMed1993

Sonographic and computed tomography characteristics of liver ablation lesions induced by high-intensity focussed ultrasound.

RATIONALE AND OBJECTIVES: The authors have previously demonstrated the ability of high-intensity focused ultrasound (HIFU) to extracorporeally induce selective tissue destruction in the liver without causing damage to the intervening abdominal wall. The potential usefulness of HIFU as a noninvasive therapy for liver cancer has been suggested. This study observes sonographic and computed tomography (CT) characteristics of HIFU-ablated liver tissue in an attempt to assess the possibility of using these imaging methods to monitor the therapeutic results. METHODS: A sonoablated lesion was induced in the liver in each of 20 rabbits with a HIFU therapeutic system. Sequential imaging of the hepatic sonolesions with sonography and CT was performed up to 8 days after treatment, and the imaging patterns were correlated with the histopathology. RESULTS: Hepatic sonoablated tissue could be clearly visualized by sonography as a hypoechoic lesion. On contrast-enhanced CT, the sonolesions were depicted as nonenhanced low-density regions. There was good correlation among the sizes of sonography- and CT-depicted lesions and pathologic specimens. CONCLUSION: In this model, sonography and contrast-enhanced CT were useful imaging modalities for monitoring sonolesion evolution after HIFU treatment.

Animals↗

PubMed1992

Hirschsprung's disease. Evaluation of mortality and long-term function in 260 cases.

This report describes 260 patients treated for Hirschsprung's disease. There were 213 boys (82%) and 47 girls (18%). Age at diagnosis was younger than 30 days in 106 patients (41%), 1 month to 1 year in 90 patients (35%), and older than 1 year in 64 patients (25%). Diagnosis was achieved with barium enema and rectal biopsy. Aganglionosis involved the rectum or rectosigmoid in 174 patients (67%), the left colon in 38 patients (15%), and the proximal colon in 23 patients (9%); 25 patients (9%) had total colonic aganglionosis. Enterocolitis occurred in 47 cases (18%). Following an initial colostomy or ileostomy, a definitive pull-through procedure was performed in 247 patients (95%) (modified Duhamel in 185, Soave in 25, Swenson procedure in 15, and anomyectomy/sphincterotomy in 22); the overall survival rate was 93.8% (244 of 260 patients). An increased mortality was associated with Down syndrome, total colonic aganglionosis, and enterocolitis. Long-term follow-up (mean, 6 years 10 months) was available in 103 patients who underwent a Duhamel procedure. Sixty-seven (65%) had normal bowel function, 28 (27%) occasionally used enemas or stool softeners, and eight (8%) had severe constipation or soiling. Bowel habits improved with time and were considered normal in 58% of patients at less than 5 years of follow-up and in 88% of patients at more than 15 years of follow-up. The Duhamel operation is a very effective definitive procedure for Hirschsprung's disease. Long-term follow-up is an important component of patient care.

Anastomosis, Surgical↗

PubMed1992

Effects of high-intensity focused ultrasound in the treatment of experimental neuroblastoma.

This report evaluates the effect of high-intensity focused ultrasound (HIFU) on subcutaneous murine neuroblastoma C1300. HIFU treatment was administered with a focused 4-MHz quartz transducer with a peak intensity of 550 W/cm2. In experiment 1, 60 animals with tumor were divided into four groups. Group I (n = 15) were controls; group II (n = 15) received adriamycin, 5 mg/kg intraperitoneally; group III (n = 15) received HIFU; and group IV (n = 15) received both adriamycin and HIFU. All the animals in groups I and II died of tumor by 35 days. Fifty-three percent (8/15) of mice in group III and 80% (12/15) in group IV were cured with no evidence of tumor (NET) at 200 days. Log-rank statistics showed significant prolongation of survival in the groups III and IV as compared with groups I or II (P less than .05). In experiment 2, 45 animals with tumor were divided into three groups. Group I (n = 15) were controls; group II (n = 15) received HIFU; and group III (n = 15) received repeated HIFU. The results showed 47% (7/15) of mice in group II and 67% (10/15) in group III were NET at 200 days. Significant survival prolongation was achieved in groups II and III in comparison with group I (P less than .05). In experiment 3, 90 mice received either tumor (n = 60) or saline (n = 30) inoculation in the left flank. On day 5, 45 mice with tumor were treated with HIFU (group I), while the other 15 mice with tumor (group II) had a sham procedure.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

PubMed1992

Persistence of Barrett's esophagus in children after antireflux surgery: influence on follow-up care.

Adenocarcinoma arising in Barrett's esophagus has recently been described in two children aged 11 and 14 years. The long-term follow-up of Barrett's esophagus in children is not well described. We evaluated 16 cases of Barrett's esophagus in children treated at this institution during the last 16 years. Ages ranged from 1.2 to 16 years (mean, 10.3 years). There were 11 boys and 5 girls. Barrett's esophagus was documented by endoscopy in 14 instances and at autopsy in 2 patients with secretory diarrhea and tetralogy of Fallot who died of sepsis. Two children had cancer (neuroblastoma, leukemia) and died of their malignant disease. Five patients had cerebral palsy, 1 esophageal atresia, 1 Fanconi's anemia, and 5 were otherwise normal children. Six were treated medically. Eight patients underwent Nissen fundoplication for complications of gastroesophageal reflux (GER). Five patients were available for follow-up endoscopy (mean, 2 years; range, 1.1 to 5.4 years). Endoscopy was performed on a yearly basis, obtaining biopsy specimens from multiple levels of the esophagus. Four children had satisfactory clinical response to an antireflux procedure including the resolution of a stricture in one case. However, in all 5 cases persistent metaplastic epithelium was documented and showed no evidence of regression. Although there has been speculation that Barrett's esophagus in children may be more likely to revert to normal squamous epithelium than in the adult, there has been only one case of regression in 180 cases of Barrett's esophagus occurring in children described in 37 reports in the literature.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

PubMed1992

The atonic baggy rectum: a cause of intractable obstipation after imperforate anus repair.

Three children with successful imperforate anus repairs had chronic, intractable obstipation and recurrent fecal impaction refractory to diligent medical therapy. All had massive stool-filled rectums up to 14 cm in diameter on abdominal radiographs. They responded well to resection of the baggy atonic rectum with low anterior anastomosis. The massively dilated atonic rectal pouch in some children with repaired imperforate anus can demonstrate abnormal motility characterized by poor peristalsis and propulsion. This results in recurrent obstipation, impaction, and soiling refractory to the usually successful medical management.

Anus, Imperforate↗

PubMed1992

A reproducible rat liver cancer model for experimental therapy: introducing a technique of intrahepatic tumor implantation.

To investigate therapeutic strategies for hepatoma, it is necessary to have a reproducible animal model with a tumor growth pattern allowing accurate assessment of results. Many techniques of intrahepatic tumor implantation (IHTI) have been devised for intrahepatic tumor models. Most of them, however, have the disadvantage of high rates of artificial tumor dissemination during tumor implantation, which interferes with the evaluation of therapy. To overcome this problem, we have developed a technique of IHTI in which a piece of Gelfoam is placed into a small incision in the liver for the purpose of both hemostasis and formation of a tension-free pocket to accept the tumor implant. In 583 ACI rats receiving IHTI with Morris hepatoma 3924A, the tumor take rate was 100%. Resembling the natural course of human hepatoma, the implanted tumor grows locally early in the course of disease and eventually invades the surrounding organs causing ascites and also metastasizes to the lung. Liver microangiography demonstrated that the tumor received blood supply mainly from the hepatic artery. This IHTI technique was also compared to two other methods of IHTI: insertion of fragments without using Gelfoam and implantation with a tumor cell suspension. A significantly lower rate of early lung metastases was achieved with our technique (0%) in comparison with other two techniques (41 and 80%). We conclude that this rat liver cancer model is reproducible and allows efficient evaluation of treatment modalities for liver cancer without interference from tumor at undesirable sites.

Animals↗

PubMed1992

Hypothyroidism impairs colonic motility and function. An experimental study in the rat.

Hypothyroidism (HT) may be associated with either megacolon or severe constipation that mimics Hirschsprung's disease. The exact nature of this relationship is unclear. This report evaluates colonic motility in experimental HT. HT was produced by a total thyroidectomy in 20 male Sprague-Dawley rats (200-300 g). Ten of the HT rats were given thyroxin (30 micrograms/kg/day i.p. for 4 weeks) for a replacement study (RS). Ten sham operated rats (SH) and 50 unoperated rats (UN) were studied as controls. HT was documented at 4 weeks following total thyroidectomy by measuring serum T3, T4 uptake, and TSH. At four to 6 weeks the animals were evaluated for several studies concerning colonic motility. Normal weight gain was significantly impaired under HT. In addition, daily stool volume as well as the number of fecal pellets were significantly reduced in HT. A barium enema showed a dilated colon in HT with an increase of recto-colonic ratio. Anal canal pressure was relatively low in HT. HT rats had a decreased frequency of rhythmic colonic activity (cycle/min) (HT: 10.22 +/- 2.16 vs. UN: 18.09 +/- 4.79, SH: 15.88 +/- 3.92, p less than 0.001). RS rats showed a recovery of rhythmic frequency (15.56 +/- 4.46, p less than 0.001). A positive recto-anal reflex was seen in all UN and SH rats, but only 40% in HT and 80% in RS. The effect of isoproterenol on anal canal pressure was significantly less in HT. Colonic transit at 4 hours was considerably slower in HT (HT: 29.0 +/- 21.0% vs. US: 60.4 +/- 18.3%, SH: 58.7 +/- 14.8%, p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

PubMed1992

The effects of elemental diet following ileo-endorectal pull-through in rats.

Total colectomy, mucosal proctectomy and ileo-endorectal pull-through (IEP) have been carried out mostly in dogs, because of high mortality after IEP in rats. This report evaluates the effects of elemental diet on bodily metabolism and survival following IEP in the rat. Survival at 4 weeks was 100% in the sham operated group used as controls, 80% in IEP group given laboratory food + 10% Vivonex ten (group 2), 30% in IEP group given food + 1/2 saline (group 3), and only 10% in IEP group given food + water (group 1). The serum Na, Cl, HCO3 were significantly lower from day 3-7 to day 28 in the group 1. On the contrary, both serum K and osmolality were extremely elevated in the group 1. The total protein level also increased gradually in the group 1 due to extracellular fluid loss. Groups 2 and 3 maintained bodily metabolism related to water and electrolytes well. Our data support the suggestion that severe water and electrolytes imbalance may occur following total colectomy. A supplementary elemental diet prevents water and electrolyte depletion and improves survival following total colectomy and IEP in the rat.

Anastomosis, Surgical↗

PubMed1992

Delayed surgical repair and ECMO improves survival in congenital diaphragmatic hernia.

One hundred ten infants with congenital diaphragmatic hernia (CDH) developed life-threatening respiratory distress in the first 6 hours of life. Associated anomalies were present in 33%. Twenty-eight of 65 infants (43%) treated before 1987 (pre-extracorporeal membrane oxygenation [ECMO] era) survived after immediate CDH repair, and mechanical ventilation with or without pharmacologic support. Only two of 16 (12.5%) infants requiring a prosthetic diaphragmatic patch survived. Since 1987, 31 of 46 (67.4%) infants with birth weight, gestational age, and severity of illness similar to the pre-1987 group survived. All patients were immediately intubated and ventilated. Seven (four with lethal chromosomal anomalies) infants died before treatment, and 30 stabilized (partial pressure of carbon dioxide [PCO2] < 50; partial pressure of oxygen [PO2] > 100; pH > 7.3) and underwent delayed CDH repair at 5 to 72 hours. Fifteen did well on conventional support and survived. Fifteen infants deteriorated after operation: 11 were placed on ECMO with eight survivors, and four infants were not considered ECMO candidates. Nine babies failed to stabilize initially and were placed on ECMO before CDH repair (alveolar-arterial gradient > 600 and oxygenation index > 40), and seven survived. The overall survival rate was 80% at 3 months in this ECMO-treated group. Early mortality was due to inability to wean from ECMO (one), intracranial hemorrhage (one), liver injury (one), and pulmonary hypoplasia (one). Nine of 11 babies requiring a prosthetic patch in the post-1987 ECMO group survived (81.8%). There were three late post-ECMO deaths (3 to 18 months) of right heart failure (two) and sepsis (one). Symptomatic gastroesophageal reflux occurred in nine cases, six requiring a fundoplication in the bypass babies. Recurrent diaphragmatic hernia occurred in nine cases (five ECMO). The overall survival rate was significantly improved in the delayed repair/ECMO group (67% versus 43%; p < 0.05) and was most noticeable in infants requiring a prosthetic diaphragm (81.2% versus 12.5%; p < 0.005). These data indicate that early stabilization, delayed repair, and ECMO improve survival in high-risk CDH. Early deaths are related to pulmonary hypertension and can be reversed by ECMO.

Extracorporeal Membrane Oxygenation↗

PubMed1992

Extracorporeal liver ablation using sonography-guided high-intensity focused ultrasound.

RATIONALE AND OBJECTIVES: High-intensity focused ultrasound (HIFU) is the only radiation beam that can remotely destroy deep-seated tissue targets without causing damage to the intervening tissues. This study evaluates the ability of sonography-guided HIFU to extracorporeally induce liver ablation in a rabbit model. METHODS: Under sonographic guidance, the HIFU beam was transcutaneously focused at the target tissue in the liver through a subcostal approach. A computer controlled the HIFU exposure and transducer movement to destroy a preselected tissue volume. Simultaneous sonography monitored the tissue response. Ten insonated rabbits were killed from days 0 to 10, and the liver and intervening tissues were examined histologically. RESULTS: A sharply demarcated sonolesion of coagulation necrosis was produced in the liver in 9 of 10 animals. No damage was found in the intervening tissues (n = 6) when adequate acoustic coupling and proper beam path was applied. CONCLUSION: Sonography-guided HIFU might be a potential new modality for extracorporeal inducement of liver cancer ablation without resorting to laparatomy.

Animals↗

PubMed1992

Initial organ localisation of blood-borne Candida albicans in a rat model of disseminated candidosis.

The rat was evaluated as an experimental model for disseminated candidosis by quantitating blood clearance and initial organ localisation of 3H-leucine-labelled Candida albicans after intravenous injection into the tail or portal vein. Viable or formalin-killed blastoconidia or viable blastoconidia with germ tubes were injected into experimental animals. Blood and tissue samples were obtained up to 24 h after injection and processed for liquid scintillation counting (to determine the distribution of labelled yeasts) and quantitation of viable organisms. Yeasts were cleared rapidly after intravenous (i.v.) injection by either route, i.e., < 5% of the radioactivity was detected in the blood after 5 min. The liver and lung were the major organs that sequestered blood-borne yeasts 1 h after tail vein injection (42.5 SD 15% and 41.4 SD 6.4% of labelled yeasts injected, respectively). However, injections via the portal vein resulted in trapping of the yeasts predominantly by the liver. Recovery of radioactivity and viable yeasts from all organs except the kidneys decreased with time. Overall, the results indicated that the rat might serve as a reliable model for short-term studies on organ distribution and thus contribute to our understanding of tissue trophism in candidosis.

Animals↗

PubMed1992

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↗