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Biomedical subjects

R J Fitzgerald

Publications and source records attributed to R J Fitzgerald.

At least 19 recordsLinked to original sources

Primary hepatic tumours in children: a 26-year review.

Twenty-one children were admitted to a single paediatric institution between 1964-1990 with histologically proven primary liver tumours. The diagnosis was hepatoblastoma (HBL) in 15 patients, hepatocellular carcinoma (HCA) in 2, rhabdomyosarcoma (RMS) in 2, non-Hodgkin's lymphoma (NHL) in 1, and haemangioendothelioma (HE) in 1. The common presenting clinical features were anaemia, abdominal mass, and abdominal pain. Serum alpha-foetoprotein was useful in establishing a diagnosis in HBL and in monitoring disease activity. Computed tomographic (CT) scan, ultrasound, and angiography were useful preoperative investigations for assessing site and resectability of tumour. There were no survivors in patients with malignant hepatic tumours (n = 10) who had surgery alone prior to 1981. Of 7 patients with HBL diagnosed after 1981 who had adequate surgical resection and chemotherapy, 5(72%) are currently alive and disease free between 15 months and 8 years from diagnosis. We conclude that adequate surgical resection and adjuvant chemotherapy can improve disease free survival for children with HBL. Optimal treatment has yet to be devised for other malignant hepatic tumours.

Antineoplastic Agents

Delayed primary anastomosis for esophageal atresia: 18 months' to 11 years' follow-up.

In 1977 we started treating babies with isolated esophageal atresia by delayed primary anastomosis and in 1981 reported our early experience in five cases treated between 1977 and 1979. Since 1979, 11 further consecutive cases have been managed by initial gastrostomy followed by delayed primary esophageal anastomosis. Their mean gestation was 35 weeks (range, 28 to 40 weeks) and mean birth weight was 2,040 g (range, 1,140 to 2,720 g). The esophageal gap between the two ends when assessed initially at fluoroscopy ranged from 2.2 to 4.5 cm (mean, 3.2 cm). Age at delayed primary anastomosis ranged from 6 to 20 weeks. Anastomotic leak occurred in three babies in the immediate postoperative period and all were successfully managed conservatively. Eight of the 11 patients developed anastomotic strictures; seven cases required 1 to 5 esophageal dilatations. One patient who did not respond to multiple esophageal dilatations required resection of an esophageal stricture. One patient died at 15 months of age of unrelated causes. The 10 surviving patients have been followed-up from 18 months to 11 years. At follow-up, seven patients were eating normally. Three patients had swallowing difficulties and all three were found to have esophageal strictures on barium swallow, two of them also had gross esophageal reflux and hiatus hernia. The height and weight in the 10 patients varied from 3rd centile to 75th centile. Delayed primary anastomosis is feasible in cases of isolated esophageal atresia and the patient's own esophagus is the best. A more aggressive approach should be applied to gastroesophageal reflux in these patients.

Anastomosis, Surgical

The changing role of surgery in the management of rhabdomyosarcoma.

A retrospective analysis was performed of 56 patients presenting to Our Lady's Hospital for Sick Children, Crumlin, Dublin, Ireland, over a 25 year period with a diagnosis of rhabdomyosarcoma, in an effort to assess the changing role of surgery in the management of this disease. There were 38 males and 18 females; the age range at presentation was from birth to 13 years with a median of three years. Head and neck tumors occurred most frequently (23) followed by pelvic (14), paratesticular (6), extremities (6), gastrointestinal (4) and thoracic (3). Prior to 1971 there were no survivors while a doubling of survival has been observed within the past decade (31% to 63%). The minimum period of follow-up was four years. Surgical intervention ranged from total primary excision (20) to biopsy only (21) with total or subtotal resection performed electively in 15. Surgical evaluations, including examinations under anesthesia, staging laparotomy and second look laparotomy were also invaluable in assessing disease status. The incorporation of multiagent chemotherapy together with more selective use of radiotherapy has enabled a more conservative role to be adopted by the surgical oncologist thereby minimizing longterm sequelae.

Antineoplastic Combined Chemotherapy Protocols

Renal function following extracorporeal lithotripsy in children.

Although extracorporeal shockwave lithotripsy (ESWL) has revolutionised the management of urinary calculous disease, the long-term effects of ESWL on renal function are not known. This study of 18 children demonstrated a mild, statistically insignificant decrease of 15% in DTPA measured glomerular filtration rate following ESWL. In view of this, we recommend that pretherapy and posttherapy estimates of renal function be obtained in all patients undergoing ESWL. Long-term follow-up is necessary to further quantify the effects of ESWL on the developing kidney.

Adolescent

Are the selection criteria for the conservative management in spina bifida still applicable?

Management of spina bifida is still controversial. At one extreme is the policy of total care in all cases while some form of selection is practised in many centres. We reviewed 104 patients treated over the last five years. Forty-nine patients, who had one or more adverse criteria, were managed conservatively. Twenty-four of these 49 patients required VP shunt insertions subsequently. Four patients had delayed closure of back lesions, three of them were followed by shunts. There was an overall increased (57%) incidence of surgical intervention in conservatively managed patients over the last five years as compared with 34% from our previous report. We found no difference in the timing of insertion of shunts in actively and conservatively managed patients in this study. The mortality in the actively and conservatively managed patients was 7% and 47% respectively. Eighty-five per cent of the patients who had no surgery died at an average age of 23 days. Patients who died without surgical intervention did not survive long enough to warrant surgery, while the mortality was 20% and had among those who survived surgical intervention. Paraplegia alone was not a predictor of high mortality but the presence of two or more adverse criteria were more accurate indicators of poor prognosis. As 78% of all the deaths in conservatively managed patients occurred within three months, reappraisal of management policy is necessary in the infants surviving beyond three months of age as death is less likely after this time.

Humans

Ultrasonography to diagnose and exclude intussusception in Henoch-Schönlein purpura.

Abdominal pain is a frequent symptom in the child with Henoch-Schönlein purpura and raises the suspicion of intussusception or perforation. One hundred and fifty two children with a diagnosis of Henoch-Schönlein purpura over 11 years were reviewed. Of these 60 had abdominal pain, 19 gastrointestinal bleeding, and nine were suspected intussusception. Intussusception was confirmed in two of these cases with ultrasonography. Ultrasound is an important tool in the early diagnosis of intussusception complicating Henoch-Schönlein purpura. Where the intussusception appears loose an expectant policy, with careful monitoring, may allow spontaneous reduction. It may also be used in monitoring patients for postoperative recurrence of intussusception, mural haematoma, and uncomplicated intestinal vasculitis with oedema.

Child

Evaluation of conservative therapy for exomphalos.

Two management patterns were identified in 36 patients with exomphalos--primary surgical closure and initial topical therapy with delayed surgical closure. Primary surgical closure of minor exomphalos was well tolerated in 15 patients, but was associated with a high local and systemic morbidity rate in 14 patients with major defects. In contrast, initial topical therapy with silver sulphadiazine and delayed closure in seven matched patients with a major defect were well tolerated and did not prolong duration of hospitalization. Enteral feeding was more readily established and subsequent fascial closure facilitated in the conservatively treated group. It was suggested that this method should be more often considered in the management of all instances of major exomphalos.

Administration, Topical

An alternative approach to pyogenic hepatic abscess in childhood.

Childhood pyogenic hepatic abscess is rare and remains a difficult diagnostic problem. Seven cases in six children were treated over a 13 year period. There was a recurrence in one patient 7 years after the first presentation. Four patients had identified predisposing factors, namely, chronic granulomatous disease, acute appendicitis, previous abdominal surgery and umbilical vein catheterization, while the other two were cryptogenic. There were no specific symptoms and signs but the combination of unexplained pyrexia, upper abdominal tenderness, hepatomegaly and leucocytosis should raise the suspicion of hepatic abscess. Four cases were diagnosed by ultrasound, one by radionuclide scan and the other two at laparotomy. Treatment in the earlier years was by transabdominal drainage. In the later part of the series, percutaneous catheter drainage using ultrasound guidance was achieved with satisfactory result. Ultrasonography can provide an early diagnosis and effective treatment can be achieved with percutaneous catheter drainage in combination with appropriate antibiotic therapy.

Adolescent

Surgery for neonatal necrotising enterocolitis in Ireland 1980-1985.

Forty-seven infants were operated on for necrotising enterocolitis (NEC) in Ireland in a 6-year-period from 1980 to 1985. Incidence for surgical NEC in Ireland is 1/8733 live births. 44/47 patients were operated on during the acute phase and 3 patients had elective resection of strictures following conservative management. Indication for surgery in the acute phase was pneumoperitoneum in 37 infants. The initial procedure was: drainage alone in 10 patients, laparotomy and defunctioning enterostomy in 18, resection of the necrotic segment and proximal enterostomy in 15. 27/44 acute patients survived giving an overall mortality of 38.6% for acute NEC but mortality directly related to NEC was 31.8%. There was an inverse relationship between mortality and age at surgery. Medical treatment included total parenteral nutrition (TPN) in all patients for a mean time of 20.5 days.

Cohort Studies

Binding of ions and hydrophobic probes to alpha-lactalbumin and kappa-casein as determined by analytical affinity chromatography.

The technique of analytical affinity chromatography was extended to characterize binding of ions and hydrophobic probes to proteins. Using the immobilized protein mode of chromatography, alpha-lactalbumin and kappa-casein were covalently attached to 200-nm-pore-diameter controlled-pore glass beads and accommodated for high-performance liquid chromatography. The existence of a high affinity binding site (Kdiss = 0.16 microM) (site I) for calcium ion in alpha-lactalbumin was confirmed by chromatography of [45Ca2+]. In addition, chromatography of the hydrophobic probes, 1-(phenylamino)-8-naphthalene-sulfonate (ANS)2 and 4,4'-bis[1-(phenylamino)-8-naphthalenesulfonate (bis-ANS) indicated that Ca2+ bound to a second site (presumably the zinc site or site II) with weaker affinity. Dissociation constants obtained for apo-alpha-lactalbumin were about 80 microM for ANS and 4.7 microM for bis-ANS in the absence of sodium ion. Addition of Ca2+ initially caused a reduction in surface hydrophobicity (lowered affinity for the probe dyes) followed by an increase at higher Ca2+ concentrations (greater than 0.5 mM), suggesting that occupancy of site II restores an apo-like conformation to the protein. Moreover, the effect of Zn2+ was similar to that observed in the higher Ca2+ concentration range, whereas Na+ apparently bound to site I. A calcium binding site of moderate affinity also exists in kappa-casein (Kdiss = 15.6 microM). A cluster of negative charges, probably including the orthophosphate group, most likely comprise this binding site. By preventing self-association, analytical affinity chromatography permits microscale characterization of ligand equilibria in proteins that are unaffected by protein-protein interactions.

Animals

Cariogenicity of a lactate dehydrogenase-deficient mutant of Streptococcus mutans serotype c in gnotobiotic rats.

A lactate dehydrogenase-deficient (Ldh-) mutant of a human isolate of Streptococcus mutans serotype c was tested in a gnotobiotic rat caries model. Compared with the wild-type Ldh-positive (Ldh+) strains, it was significantly (alpha less than or equal to 0.005) less cariogenic in experiments with two different sublines of Sprague-Dawley rats. The Ldh- mutant strain 044 colonized the oral cavity of the test animals to the same extent as its parent strain 041, although its initial implantation was slightly but not significantly (P greater than or equal to 0.2) less. Multiple oral or fecal samples plated on 2,3,5-triphenyltetrazolium indicator medium revealed no evidence of back mutation from Ldh- to Ldh+ in vivo. Both Ldh+ strain 041 and Ldh- strain 044 demonstrated bacteriocinlike activity in vitro against a number of human strains of mutans streptococci representing serotype a (S. cricetus) and serotypes c and e (S. mutans). Serotypes b (S. rattus) and f (S. mutans) and strains of S. mitior, S. sanguis, and S. salivarius were not inhibited. Thus, Ldh mutant strain 044 possesses a number of desirable traits that suggest it should be investigated further as a possible effector strain for replacement therapy of dental caries. These traits include its stability and low cariogenicity in the sensitive gnotobiotic rat caries model, its bacteriocinlike activity against certain other cariogenic S. mutans (but not against more inocuous indigenous oral streptococci), and the fact that it is a member of the most prevalent human serotype of cariogenic streptococci.

Animals

Has vesicostomy a role in the neuropathic bladder?

This retrospective study aims to assess the efficacy of vesicostomy in the management of deteriorating upper renal tracts in patients with neuropathic bladders for whom intermittent self catheterization is not a clinical option. Between 1980-1987 vesicostomy was performed in 16 such patients (11 female, 5 male). Eleven had myelomeningocoele, 4 sacral anomalies and one cerebral palsy with scoliosis. All patients had urinary tract infections and deteriorating hydronephrosis and hydroureter (IVP, U/S); associated reflux in 13, impaired GFR in 4 and abnormal biochemistry in 8. The mean age at surgery was 3.3 years (8 mths. to 9 years). In all cases deterioration was arrested by vesicostomy. Fifteen patients showed structural or functional improvement and one stabilized. Late closure of the vesicostomy in two cases resulted in disimprovement of biochemical and radiological parameters. Acceptance of the vesicostomies was good. We advocate vesicostomy as an effective drainage procedure to preserve function in patients with neuropathic bladders and deteriorating renal tracts, in whom intermittent self catheterization is not a realistic alternative.

Child

An unusual case of complete urethral duplication in a female child.

A case is reported of a female child with complete urethral duplication draining a single bladder and with the accessory urethra communicating with the urachus. The clinical, operative and histological findings suggest that the urethral duplication may represent an abortive duplication of the bladder.

Female

Pledgets in ingrowing toenails.

Eighty children had cotton wool pledgets inserted under the nail corner for ingrowing toenails. Success was achieved in 44 (72%) of the 61 children followed up for a mean of 2.5 years. Appearance was excellent and compliance high. Initial insertion under anaesthesia improved the results, and chronicity did not adversely affect the outcome.

Adolescent

Corrosive injury of the oesophagus in childhood.

A 10-year retrospective review of 73 children who had ingested a corrosive agent was performed. Presentation, management, and eventual outcome were assessed, and comparisons were made between those who received early medical treatment and those who did not. Results suggested that immediate proper assessment of the oesophagus and institution of appropriate therapy may reduce the incidence of complications associated with treatment of subsequent stricture formation. The importance of oesophagoscopy in the direct visualization of the oesophagus within 24-48 h of the burn is stressed.

Anti-Bacterial Agents