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Biomedical subjects

J C Langer

Publications and source records attributed to J C Langer.

At least 91 records · Page 5Linked to original sources
PubMed1993

Fetal abdominal wall defects.

Abdominal wall defects are frequently detected prenatally. Optimal management requires identification of the type of lesion and a careful search for associated chromosomal and structural anomalies. For cases with lethal associated abnormalities, elective termination may be offered. Preterm delivery should be considered for fetuses with gastroschisis who have evidence of ongoing intestinal damage, as evidenced by bowel dilatation and thickening, although objective selection criteria are still being developed. There is currently no convincing evidence to support routine cesarean delivery in fetuses with abdominal wall defects. Most infants with abdominal wall defects should be born at a perinatal center, where neonatal and surgical expertise is immediately available. In every case, prenatal diagnosis should lead to parental counseling and decision-making by a multidisciplinary team consisting of obstetricians, neonatologists, and pediatric surgeons.

Abdominal Muscles↗
PubMed1993

Piperacillin versus cefazolin given perioperatively to high-risk patients who undergo open cholecystectomy: a double-blind, randomized trial.

OBJECTIVE: To study the efficacy, microbiologic features and toxicity of prophylactic cefazolin versus prophylactic piperacillin in high-risk patients who undergo open cholecystectomy. DESIGN: Double-blind randomized trial with follow-up for 6 weeks postoperatively. SETTING: An 850-bed community hospital, located in a major Canadian city. Patients admitted to hospital who satisfied published criteria for being at high-risk for infection after open cholecystectomy were entered into the protocol, and those who satisfied the criteria and provided consent were entered into the study. Eighty-one patients were randomly assigned by computer to receive either piperacillin or cefazolin as the prophylactic agent. INTERVENTIONS: Open cholecystectomy. MAIN OUTCOME: Provides detailed information on the organisms found in the biliary tree in patients with acute cholecystitis, assesses the in-vitro activity of cefazolin versus piperacillin against the isolated organisms, expecting that piperacillin would be much more active against isolated anaerobes and gram-negative bacteria. RESULTS: Bactobilia was documented in 42% of patients in the cefazolin group and 29% of patients in the piperacillin group. Piperacillin was active in vitro against 94% of all isolates versus 56% for cefazolin (p < 0.005, McNemar's test). Adverse effects and toxicities in both the piperacillin and cefazolin group were low and were not serious. CONCLUSIONS: Both piperacillin and cefazolin are safe and effective prophylactic antimicrobials for high-risk patients who undergo open cholecystectomy. However, piperacillin had a much wider spectrum of in-vitro activity against the isolated pathogens, especially Enterococcus sp., Enterobacter cloacae and the anaerobes.

Adult↗
PubMed1993

Late anastomotic ulceration after ileocolic resection in childhood [corrected].

Anastomotic ulcers are a rare late complication of ileocolic resection in children. The authors describe the cases of two patients who presented 10 and 8 years, respectively, after ileocolic resection. Both had suffered from iron-deficiency anemia caused by an anastomotic ulcer. In one patient the anemia improved after resection of the ulcer; in the second patient cholestyramine and a lactose-free diet resolved the problems of anemia and diarrhea. Anastomotic ulceration should be suspected in any child who has undergone an ileocolic anastomosis and subsequently presents with persistent iron-deficiency anemia and occult blood in the stool.

Anastomosis, Surgical↗
PubMed1993

Prenatal diagnosis of gastroschisis: development of objective sonographic criteria for predicting outcome.

OBJECTIVE: To determine which sonographic findings predict intestinal damage in fetuses with gastroschisis, and to develop objective criteria that may be useful in selecting candidates for preterm delivery. METHODS: Twenty-four consecutive fetuses at two perinatal centers were assessed retrospectively or prospectively. Maternal, perinatal, and sonographic data were recorded and correlated with postnatal outcome. RESULTS: Bowel diameter of at least 18 mm was associated with a significantly longer time to oral feeding and with significantly greater need for bowel resection. When gestational age was plotted against bowel diameter, a threshold curve was generated, above which all patients had prolonged hypoperistalsis and below which only 30% had prolonged hypoperistalsis. Two infants were delivered at 33 weeks' gestation, both of whom had complications potentially related to prematurity. Only one of 22 patients who delivered later than 33 weeks had similar complications. CONCLUSIONS: Bowel dilatation may be a marker of prenatal bowel damage in fetuses with gastroschisis, especially when it presents late in gestation. Prenatal sonography may be useful in selecting appropriate fetuses for preterm delivery.

Abdominal Muscles↗
PubMed1993

Prenatal diagnosis of congenital anomalies. What can and should be done?

Prenatal diagnosis of congenital anomalies provides an opportunity to prepare and counsel parents. It can lead to pregnancy termination; to a change in timing, mode, or location of delivery; or, in some cases, to prenatal intervention. Families with an affected fetus are best managed by a cohesive medical team. This article outlines the principles used to manage these cases.

Congenital Abnormalities↗
PubMed1992

Cloacal exstrophy: prenatal diagnosis before rupture of the cloacal membrane.

Embryologically, cloacal exstrophy is thought to result from persistence and subsequent rupture of the infraumbilical cloacal membrane during the fifth embryonic week. We report a case of cloacal exstrophy in which a prenatal diagnosis was made prior to rupture of the cloacal membrane. A routine ultrasound at 17 weeks' gestation demonstrated monoamniotic twins. One twin was normal, but the other was found to have a sacral myelomeningocele, "rocker-bottom" feet, splaying of the pubic rami, and a large cystic mass protruding from the infraumbilical anterior abdominal wall. A repeat ultrasound was performed at 22 weeks, with the same findings. At 26 weeks, further examination showed disappearance of the abdominal cyst, a small omphalocele, no demonstrable bladder, and the suggestion of prolapsed bowel inferior to the umbilical cord insertion. After delivery at 34 weeks, the abnormal twin was found to have the typical findings of cloacal exstrophy, myelomeningocele, bilateral lower limb anomalies, and extremely foreshortened small bowel. Rupture of the presumed cloacal membrane after 22 weeks in this case is inconsistent with our current understanding of the embryology of this anomaly, and should stimulate a reexamination of the current concepts. If the characteristic features are recognized, cloacal exstrophy can be diagnosed by prenatal ultrasound, permitting prenatal counseling and appropriate perinatal management.

Abnormalities, Multiple↗
PubMed1992

Increased mucosal permeability after intestinal ischemia-reperfusion injury is mediated by local tissue factors.

Subclinical intestinal ischemia-reperfusion injury (IRI) causes an increase in mucosal permeability and may represent an early event in the pathogenesis of necrotizing enterocolitis. The present study was undertaken to determine whether these changes are mediated by local or systemic factors. In 6-week-old weanling rats, the ileum was divided into two isolated loops with separate vascular supplies. The mesentery of the proximal loop was occluded for 30 minutes, following which the bowel was reperfused; permeability to 51Cr EDTA was then assessed in the distal loop 30 minutes after reperfusion. In control groups, the distal loop was subjected to 30-minute IRI ("positive" control) or 30-minute sham operation ("negative" control). Permeability in the distal loop was increased only with IRI to the distal bowel (15.4 +/- 3 counts/min/standard), and not with IRI to the proximal bowel (5.1 +/- 1) or with sham operation (8.5 +/- 2). To determine whether a mild "priming" injury might be necessary for systemic factors to have an effect, the distal loop was subjected to 2-minute IRI and the proximal to 30-minute IRI or sham. Permeability was not increased in the distal loop in either of these groups (5.7 +/- 1 and 7.8 +/- 2, respectively). Thirty-minute IRI in the proximal loop did not increase permeability in the distal loop, with or without a priming injury. Only direct IRI in the distal loop resulted in a significant increase in permeability. We conclude that the permeability changes in this model are mediated through local tissue effects, rather than by systemic factors.

Animals↗
PubMed1992

Management of prenatally diagnosed tracheal obstruction: access to the airway in utero prior to delivery.

A fetus of 23-weeks gestation presented with polyhydramnios, and was found on sonography to have a large anterior cervical teratoma. At 26 weeks the mother went into premature labor and the membranes ruptured; at this time the uterus was of 32-weeks size secondary to polyhydramnios. A cesarean section was performed through a low transverse uterine incision, and the fetal head and shoulders were delivered with the cord intact. An endotracheal tube was placed, and the cord was then divided. The baby was taken to the neonatal intensive care unit for stabilization prior to planned resection, but suffered pharyngeal hemorrhage followed by endotracheal tube dislodgement several hours after birth, and expired. Despite an unfortunate outcome, this case illustrates the feasibility and potential value of in utero airway access in cases of prenatally diagnosed tracheal obstruction.

Adult↗
PubMed1991

Transplantation of fetal cells.

Cellular transplantation is an attractive alternative to whole-organ transplantation when only a discrete function of the organ is deficient. Early fetal donor cells have an advantage because they engraft readily and do not cause graft-versus-host disease. Similarly, the fetus is an ideal recipient of allogeneic fetal cells as it is incapable of rejecting them early in gestation. This review presents the theoretical rationale, recent research advances, and clinical implications for adults with diabetes mellitus and Parkinson's disease; we also describe in utero transplantation of fetal hematopoietic stem cells and hepatocytes for the treatment of inherited hematologic and hepatic deficiencies, as well as the use of fetal islet cells and dopamine-producing cells to treat postnatal conditions.

Animals↗
PubMed1991

False diagnosis of intestinal obstruction in a fetus with congenital chloride diarrhea.

Intestinal obstruction is often diagnosed prenatally by ultrasound, providing an opportunity for prenatal counseling, genetic investigation, and planned delivery at a perinatal center. We describe a patient with typical features of fetal bowel obstruction, who was found at birth to have congenital chloride diarrhea. A 25-year-old white woman had marked polyhydramnios; multiple dilated, fluid-filled loops of intestine were seen in the fetal abdomen on prenatal ultrasound. However, postnatally, there was no evidence of bowel obstruction. The infant girl passed large amounts of watery stools, but tolerated feeds well. A rectal biopsy showed normal ganglion cells. On the fourth day of life her serum sodium and chloride were markedly decreased, and stool chloride levels were diagnostic of congenital chloride diarrhea. She was placed on sodium chloride and potassium chloride supplements, and her serum electrolytes normalized. Congenital chloride diarrhea is a rare, inherited condition caused by an abnormality of intestinal electrolyte transport. This case illustrates that it may present prenatally with a picture similar to that seen with intestinal obstruction.

Adult↗
PubMed1991

Heterotopic gastric mucosa in the gallbladder: a cause of chronic abdominal pain in a child.

A 12-year-old boy presented with a 7-year history of intermittent colicky epigastric pain. Serial abdominal ultrasound studies showed a contracted gallbladder, and single and double dose oral cholecystograms demonstrated nonfunction. All other investigation results were normal. At cholecystectomy, there was a fibrotic stricture at the junction of the gallbladder neck and cystic duct. The small, thin-walled gallbladder contained white bile. Histologically, the gallbladder was lined with normal mucous secreting columnar epithelium. The area of stricture showed diffuse fibrosis with islands of heterotopic gastric mucosa containing all gastric mucosal cell types. One year postoperatively, there have been no further episodes of abdominal pain. This is the seventh reported case of heterotopic gastric mucosa in the gallbladder of a child causing symptoms necessitating cholecystectomy. We recommend that a child with abdominal pain and a nonfunctioning gallbladder be considered for cholecystectomy even in the absence of cholelithiasis.

Abdominal Pain↗
PubMed1991

A prospective evaluation of the Button gastrostomy in children.

The Button gastrostomy has become popular for patients requiring long-term enteral feeding, because it is considered less irritating, more stable and more esthetically acceptable than the traditional tube gastrostomy. By a standardized questionnaire and personal interview, the authors evaluated prospectively the efficacy and complication rate in 19 children who had a Button gastrostomy inserted during a 6-month period. In 15 children the Button replaced a standard tube gastrostomy, and in 4 the Button was inserted surgically initially. Thirteen children had severe neurologic disabilities, and 6 required supplemental enteral feeding as part of the nutritional management of another chronic disease. In all 19 children, the Button was esthetically more acceptable and produced less skin irritation than the standard tube gastrostomy. All but one caregiver thought that the Button gastrostomy was preferable to a tube gastrostomy. This was especially apparent in the six neurologically normal children who were able to be more active and had an improved self-image. Cost analysis showed that, despite the higher initial cost of the Button, elimination of the need for frequent tube changes and hospital visits made it ultimately more cost effective than the standard tube gastrostomy. The authors conclude that the Button gastrostomy is a useful alternative to the standard tube gastrostomy in selected patients. Close long-term follow-up is extremely important to ensure a good result.

Adolescent↗
PubMed1990

Fetal intervention in obstructive uropathy: prognostic indicators and efficacy of intervention.

Management of the fetus with bilateral hydronephrosis is controversial; ability to predict outcome and efficacy of prenatal intervention are unknown. We studied 40 fetuses referred for ultrasonography, examination of fetal urine, and possible therapy. We retrospectively assigned fetuses to a good prognosis group if fetal urine was hypotonic (sodium less than 100 mEq/L, chloride less than 90 mEq/L, osmolarity less than 210 mOsm/L) and there was no ultrasonographic evidence of dysplasia; we assigned fetuses to a poor prognosis group if even one criterion was abnormal. Survival was greater in the good prognosis group than in the poor prognosis group (81% vs 12.5%; 87% vs 30%, excluding abortions) (p less than 0.005). We then attempted to assess the efficacy of prenatal urinary decompression by comparing outcome within the good and poor prognosis groups. Survival with intervention was greater in both the good prognosis group and the poor prognosis group (89% vs 70% and 30% vs 0%). In 6 of the 8 survivors in the good prognosis group, severe oligohydramnios was reversed by decompression. We conclude the fetal urine electrolyte levels and ultrasonographic appear helpful in predicting residual fetal renal function and neonatal outcome and that prenatal decompression may prevent the development of fatal pulmonary hypoplasia.

Amniotic Fluid↗
PubMed1990

Cyclic neutropenia with colonic perforation and nonhealing colocutaneous fistula.

Cyclic neutropenia is an hereditary disorder of white blood cells, characterized by profound neutropenic episodes approximately every three weeks. Septic complications are usually limited to cutaneous and oropharyngeal infections. A 4-year-old boy with known cyclic neutropenia was in shock with neutropenia, clostridial septicemia, and right lower quadrant peritonitis when he was admitted. At the time of laparotomy, inflammation of the cecum, with no gross perforation, was found; no resection or appendectomy was done. He subsequently developed a right lower quadrant abscess that was drained, resulting in a colocutaneous fistula. For the next 8 months his fistula persisted, with intermittent episodes of fever, increased fistula output, and abdominal pain during his neutropenic periods. Standard nonoperative approaches to healing the fistula failed (ie, elemental feeds, total parenteral nutrition, irrigations, antibiotics, and drains). Attempts to medically abolish his neutropenic episodes using lithium, gammaglobulin, and steroids also failed. Ultimately, he underwent an ileocecal resection with primary anastamosis; the operation was done immediately following a neutropenic episode, in order to allow adequate healing of his anastamosis before his next period of neutropenia. Postoperative course was satisfactory, and he remains well after 8 months follow-up. This case, and several similar previously reported cases, illustrate that cyclic neutropenia may present with serious surgical complications. They also underlines the important role that neutrophils play in the healing of enteric fistulae.

Agranulocytosis↗
PubMed1990

Complete heart block in fetal lambs. I. Technique and acute physiological response.

Prenatally diagnosed complete heart block (CHB), without associated cardiac anomalies, is often refractory to attempts to increase heart rate and cardiac output by medical therapy, and results in fetal death. We developed a model of CHB in fetal lambs in order to evaluate the acute physiological consequences of CHB and to assess the feasibility of fetal cardiac ventricular (V) and atrioventricular (AV) pacing. After hysterotomy and fetal sternotomy AV-sequential pacing leads were placed on the right atrium and ventricle (RV). Electromagnetic flow transducers were placed around the pulmonary artery and the ascending aorta to measure combined ventricular output (CVO) in normal sinus rhythm (NSR), CHB, AV, and V pacing. CHB block was successfully induced in 19 of 21 (90%) fetal lambs at 100 to 110 days' gestation by injection of formalin into the interventricular septum. Two fetuses were refractory to CHB despite multiple injections. The mean ventricular escape rate was 52 +/- 3 (+/- SEM) beats/min (range, 45 to 60 beats/min). CHB lasted for the period of study (1 to 8 hours), and spontaneously reverted to NSR in one fetus after 3 hours. Combined ventricular output in NSR was 470 +/- 31 mL/min (+/- SEM), which fell to 213 +/- 13 mL/min in CHB, 45% of normal CVO (P less than .0001). CVO during AV-sequential and V pacing was 77% (P = .02) and 62% (P less than .001), respectively, of combined ventricular output in NSR. Both RV (27%) and left ventricles (LV) (37%) showed compensatory increase in stroke volume during CHB.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗
PubMed1990

Nephron-sparing approach to bilateral Wilms' tumor: in situ or ex vivo surgery and radiation therapy.

Bilateral Wilms' tumor patients, who experience local recurrence after maximal multimodality therapy, present a difficult surgical problem. The role of surgery in the management of these patients has changed from ablation to preservation of renal tissue, with bilateral nephrectomy as a last resort. Two children who had recurrent tumors in the remaining kidney underwent nephron-sparing surgery and focal intraoperative radiation therapy. In one case, this nephron-sparing surgery and intraoperative radiation therapy were performed in situ, in the other kidney was removed and reimplanted (ex vivo bench surgery and irradiation). These techniques may allow complete obliteration of gross and microscopic disease, while maximizing residual renal function.

Child↗