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Biomedical subjects

L Spitz

Publications and source records attributed to L Spitz.

At least 127 records · Page 7Linked to original sources
PubMed1996

Resection and primary anastomosis in necrotizing enterocolitis.

Necrotizing enterocolitis (NEC) is the most common surgical emergency in the newborn. Up to half of babies with NEC develop advanced disease requiring surgical intervention. Options include peritoneal drainage under local anaesthetic, enterostomy only, resection and enterostomies, and resection with primary anastomosis. Resection with enterostomies is favoured by many paediatric surgeons but management of neonatal enterostomies can be difficult. The outcome of 26 infants undergoing surgery for advanced NEC over a 2-year period is reviewed. Resection and primary anastomosis was possible in 18 infants of whom two (11%) died. Recurrent NEC developed in four (22%) and strictures in three (17%) of these infants. An initial enterostomy was fashioned in eight infants, three following resection of necrotic intestine and five as a proximal diverting stoma in infants with pan-intestinal involvement. Five of these eight infants died (63%), giving an overall mortality of 27%. Primary anastomosis is an effective procedure following resection of grossly involved intestine in infants with NEC. The mortality and morbidity in this series compared well with those reported for staged procedures.

Anastomosis, Surgical↗
PubMed1995

Expression of a high molecular weight form of insulin-like growth factor II in a Beckwith-Wiedemann syndrome associated adrenocortical adenoma.

Beckwith-Wiedemann syndrome is a rare condition (1/13,700 live births) occurring in both inherited and sporadic forms in the population. It is manifest as a fetal overgrowth syndrome, in which hypertrophy dominates the clinical picture. An additional complication is that these children are predisposed to a specific subset of childhood neoplasms, amongst which are Wilms' tumour and adrenocortical carcinoma. We report here the synthesis by an associated adrenal tumour of large quantities of a high molecular weight form of insulin-like growth factor II (IGF-II), associated with profound suppression of circulating IGFs in the patient's serum. As with other tumours of this type, the tumours showed loss of material on chromosome 11p.

Adenoma↗
PubMed1995

Management of alimentary tract duplication in children.

Duplication of the alimentary tract is rare but potentially dangerous. Five of 72 children with alimentary tract duplication treated between 1973 and 1992 died from postoperative complications; a further ten required more than one operation. Ileal duplications were the commonest, occurring in 16 patients (22 per cent). Thoracoabdominal duplications were the most complicated and responsible for much of the overall morbidity and mortality. Surgical complications were related to the size and location of the duplication, communication with the gastrointestinal tract or vertebral canal, presence of heterotopic gastric mucosa and involvement of mesenteric vessels. Complete excision of the duplication should be possible in most cases.

Adolescent↗
PubMed1995

Improved outcome for children with hepatoblastoma.

Between 1981 and 1993, 41 children were treated for hepatoblastoma. Clinical, radiological and pathological data were reviewed retrospectively, focusing on surgical aspects of treatment and outcome. Fourteen children underwent primary resection of the hepatic tumour. One infant with severe congenital anomalies received only palliative treatment. Of 26 with irresectable disease, pulsed cytotoxic chemotherapy (cisplatin and doxorubicin) enabled subsequent surgical excision in 22 and one child with persistent extensive intrahepatic disease was successfully treated by liver transplantation. Thus, with a policy of selective preoperative chemotherapy, 90 per cent of hepatoblastomas were resectable. There were no perioperative deaths from haemorrhage but one child died from an intraoperative tumour embolus. A total of 28 survivors, 27 of whom are disease-free, were followed for a median of 5 years. The cumulative probability of survival in patients treated with intent to cure was 67 per cent. Analysis of survival data suggested a favourable outcome for those with a pure fetal histological tumour subtype. These results demonstrate significant progress in the treatment of hepatoblastoma.

Child↗
PubMed1995

Klippel-Trenaunay syndrome: clinical features, complications and management in children.

Clinical features and management of 47 children with Klippel-Trenaunay syndrome treated since 1970 were reviewed. Haemangiomas and soft tissue and/or skeletal hypertrophy were present in all 47 patients; venous varicosities developed in 37 (79 per cent). There was no clinical evidence of macrofistulous arteriovenous communications in any patient. Thromboembolic episodes occurred in five children (11 per cent) and 25 (53 per cent) experienced thrombophlebitis. The Kasabach-Merritt syndrome was observed in 21 (45 per cent) and six (13 per cent) presented with high-output cardiac failure. Other manifestations included haematuria in five (11 per cent), rectal or colonic haemorrhage in six (13 per cent), and vaginal, vulval or penile bleeding in six (13 per cent) children with visceral and pelvic haemangiomas. In 26 patients (55 per cent) symptomatic treatment only was required. Surgery was undertaken in selected cases for complications of the haemangioma, for cosmetic reasons and for chronic venous insufficiency. Only one of four children who underwent resection of varicose veins improved. There was no death, but significant morbidity was associated with the treatment of Kasabach-Merritt syndrome and high-output cardiac failure.

Adolescent↗
PubMed1995

Psychosocial adjustment of children treated for anorectal anomalies.

The psychosocial adjustment of 160 children with anorectal malformations was assessed at 6 to 17 years of age in relation to levels of continence (Kelly score). Five measures of emotional and behavioural adjustment were used to assess a number of domains of child/adolescent functioning and to include measures from multiple perspectives. The psychiatric assessment of the child identified a disorder in 29% of the group overall. Based on parental assessments, behavioural maladjustment was shown in 27% of the children, and on the basis of a self-report questionnaire 24% of the children were depressed. Behavioural adjustment as rated by teachers was similar to the norms. The level of continence achieved (total Kelly score) did not appear to influence psychological adjustment, with the exception of the incontinent young girls (6 to 11 years) who were shown to be less well adjusted than the continent young girls. Differences between children showing positive versus negative adjustment were dependent on the perspective of the respondent and were also related to the child's age and gender, age of achieving continence, frequency of accidents, and parental factors.

Adaptation, Psychological↗
PubMed1995

Infantile hepatic hemangioendothelioma: the role of surgery.

Of 16 infants with infantile hepatic hemangioendothelioma, 14 (88%) presented before age 3 months. For seven cases (44%), the diagnosis was suspected from antenatal ultrasonographic findings. Two (13%) presented with asymptomatic hepatomegaly. The most common presenting features were high-output cardiac failure in 11 (69%), consumptive coagulopathy in 12 (75%), and anemia in 12(75%). Sixty-three percent of the children had associated cutaneous hemangiomata, and disseminated hemangiomatosis was noted in two (13%). Medical measures were effective in stabilizing seven (44%) cases with high-output congestive cardiac failure and/or consumptive coagulopathy. Partial right hepatectomy was successful for four patients; the only death occurred in a newborn, after intraperitoneal rupture of the hepatic hemangioma. Embolization was used in two children to induce involution. Spontaneous involution occurred in two patients. Initially, hepatic hemangiomas should be treated conservatively, with surgery reserved for intractable cardiac failure and/or refractory consumptive coagulopathy.

Beckwith-Wiedemann Syndrome↗
PubMed1995

Scattered radiation in a neonatal surgical unit.

We studied the doses of "scattered" received by neonates in a Neonatal Surgical Unit by placing thermoluminescent dosemeters in various positions expect the direct beam. Doses of "scattered" radiation received by neonates as a results of exposure of their neighbours to X-ray examinations were found to be negligible. Sick neonates need not be moved from their neighbours undergoing exposure to X-rays.

Humans↗
PubMed1995

Neonatal surgery.

Advances in the sophistication of operative and anaesthetic techniques and progress in pre- and postoperative care have contributed to the improved survival of infants undergoing surgery in the neonatal period. Neonatal surgery must be concentrated in designated regional centres where expertise of the full range of paediatric specialties is available to promote research and development of the specialty and to train future generations of paediatric surgeons.

Anesthesia↗
PubMed1994

Separation of brachio-thoraco-omphalo-ischiopagus bipus conjoined twins.

Separation of 3-year-old brachio-thoraco-omphalo-ishiopagus bipus conjoined twin girls is reported. Detailed preoperative assessment and a multidisciplinary approach were essential prerequistes to surgery. The difficulties of managing the extensive body wall defect resulting from separation are discussed. Despite the use of subcutaneous and intraperitoneal tissue expanders, a large area of prosthetic material was required to reconstruct the abdominal wall. Skin cover was achieved using a combination of meshed allogeneic skin, homograft split skin, and autologous cultured keratinocytes. One twin died in the early postoperative period, but the other has recovered well and is awaiting further rehabilitative treatment.

Abdominal Muscles↗
PubMed1994

Oesophageal atresia: at-risk groups for the 1990s.

The authors analyzed the outcome for 357 infants with oesophageal atresia and 15 with H-type tracheoesophageal fistula treated from 1980 through 1992. Survival according to Waterston risk categories was 99% for group A, 95% for group B, and 71% for group C. Presently, with optimal management, virtually all infants in groups A and B should survive. When examining the risk factors in the infants who died, two criteria were found to be important predictors of outcome: birth weight of less than 1,500 g and the presence of major congenital cardiac disease. A new classification for predicting outcome in oesophageal atresia is proposed: group I: birth weight > or = 1,500 g, without major cardiac disease, survival 97% (283 of 293); group II: birth weight < 1,500 g, or major cardiac disease, survival 59% (41 of 70); and group III: birth weight < 1,500 g, and major cardiac disease, survival 22% (2 of 9).

Abnormalities, Multiple↗
PubMed1994

Plasma interleukin-6 and tumour necrosis factor levels as predictors of disease severity and outcome in necrotizing enterocolitis.

Plasma tumour necrosis factor (TNF) and interleukin-6 (IL-6) levels of 18 infants with necrotizing enterocolitis (NEC) were measured at the time of diagnosis or on transfer to a paediatric surgical unit. Whereas TNF levels were similar for infants managed medically (stage II, n = 8) and surgically (stage III, n = 7), IL-6 levels were significantly higher in stage III cases (mean, 3,127 pg/mL [95% CI, 1,809 to 4,445 pg/mL]) than in stage II (mean, 127 pg/mL [95% CI, 10 to 329 pg/mL]; P = .001). Neither TNF nor IL-6 level predicted eventual outcome. Plasma IL-6 may be useful as an indication for operation in NEC.

Biomarkers↗
PubMed1994

Facilitated nuclear transport of calmodulin in tissue culture cells.

Calmodulin (CaM) potentiates Ca(2+)-dependent signaling pathways in both the cytoplasm and nucleus. We have investigated the mechanism of CaM nuclear transport using tissue culture cell microinjection and a permeabilized cell import assay. The inhibition of CaM import by the translocation inhibitor wheat germ agglutinin (WGA) and by chilling, indicates that CaM import is facilitated, but because ATP depletion does not affect CaM import, the mechanism does not appear to be active. Chilling and WGA arrest persist in ATP-depleted cells, indicating that CaM is not retained in the cytoplasm by an ATP-dependent mechanism. In permeabilized cells, both Ca(2+)-CaM and Ca(2+)-free CaM are sensitive to extract-dependent WGA and chilling import inhibition. Titration experiments in microinjected and permeabilized cells indicate that a saturable cytosolic factor(s) mediates chilling and WGA arrest.

Adenosine Triphosphate↗
PubMed1994

Gastric emptying in children with gastric transposition.

Using the noninvasive technique of electrical impedance tomography (EIT), gastric emptying was studied in 12 children, aged 9 months to 17 years, who had undergone gastric transposition (six with pyloroplasty) for oesophageal replacement (seven oesophageal atresia, five severe caustic or peptic damage). In two patients, gastric antral electrical control activity was also studied using surface electrogastrography. Nine patients had problems on oral feeds (respiratory symptoms, feeding difficulties, vomiting, abdominal pain, symptoms suggesting dumping), whilst three were asymptomatic. All 12 patients were tested with a milk meal; in addition four (two with and two without dumping symptoms) were tested with a hypertonic glucose drink; gastric emptying of the milk meal was expressed as the percentage of the meal remaining in the stomach at 60 min (R60). Mean (+/- 2 SD) R60 was 54.6% (+/- 17.4%) in 12 healthy controls and 59.8% (+/- 83.2%) in the 12 patients. Gastric emptying was normal in one patient (R60, 42.6%), delayed in seven (mean R60, 91.2%; range, 74.4-100%), and accelerated in four (R60, 0%). The emptying rate was unrelated to the presence or absence of pyloroplasty. Furthermore, the emptying pattern was extremely irregular, suggesting that gastroesophageal as well as duodenogastric reflux episodes occurred in all patients. The hypertonic glucose drink induced dumping (50% of the meal emptied at 1-3 min) in all four patients, two of whom had delayed emptying of the milk meal, but the gastric antral electrical control activity occurred at the normal frequency of 0.05 Hz.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1994

Plasma cytokine levels in necrotizing enterocolitis.

Plasma concentrations of tumour necrosis factor (TNF) and interleukin-6 (IL-6) were measured by ELISA in samples taken from 24 infants with necrotizing enterocolitis (NEC) between 0 and 306 h from diagnosis. TNF was detected (> 10 pg/ml) in 71% samples with a mean of 48 pg/ml (95% CI 42 to 55 pg/ml) and did not vary with either time from diagnosis or severity of disease. IL-6 was raised during the first 48 h with a significant difference between stage II (mean 127 pg/ml, 95% CI 10 to 329 pg/ml) and stage III (mean 3127 pg/ml, 95% CI 1809 to 4445 pg/ml, p = 0.001). Postoperative plasma IL-6 concentration fell to similar levels seen in stage II (mean 150 pg/ml, 95% CI 37 to 283 pg/ml, p = 0.79). We conclude that plasma concentration of IL-6 rather than TNF reflects the clinical severity of necrotizing enterocolitis and that the relative level of these cytokines has important implications for the use of anti-cytokine therapy in NEC.

Biomarkers↗
PubMed1994

Reduced nuclear import of human immunodeficiency virus type 1 preintegration complexes in the presence of a prototypic nuclear targeting signal.

Nuclear import of the retroviral preintegration complex and integration of retroviral with host cell DNA are essential steps for completion of the virus life cycle. The preintegration complex of the lentivirus human immunodeficiency virus type 1 (HIV-1) displays karyophilic properties and, as a consequence, is rapidly directed to the host cell nucleus by an energy-dependent transport pathway. The karyophilic properties of nuclear proteins are governed by a nuclear localization sequence, the targeting function of which can be inhibited in the presence of excess targeting signals. Here we present evidence that the nuclear import of a large karyophile--the preintegration complex of HIV-1--is inhibited in the presence of a prototypic nuclear targeting signal of simian virus 40 T antigen. This points to a novel strategy which prevents establishment of the provirus by interrupting nuclear localization of HIV-1 DNA.

Amino Acid Sequence↗
PubMed1994

Social and emotional impact of faecal incontinence after surgery for anorectal abnormalities.

A significant proportion of children with anorectal malformations have long term problems with faecal continence. The psychological consequences of this chronic disability was assessed in 160 children and adolescents. The prevalence of clinically significant emotional problems among the sample overall, as assessed by a diagnostic psychiatric interview (19%), parental assessment (27%), and child self report depressive scale (24%) was higher than expected relative to normative populations. With the exception of the young girls (6-11 years), the incontinent children and adolescents were not judged to be less well adjusted than those with good bowel control. Treatment for anorectal malformations appears to be associated with an increased risk for behavioural and social problems, but this was not related to the level of continence. Parental factors and gender were significantly associated with outcome. These children and families would benefit from psychological evaluation and support especially during early childhood.

Adolescent↗