Necrotising enterocolitis (NEC)--methods of treatment and outcome: a comparative analysis of Scottish (Glasgow) and Polish (Western Pomerania) cases.

AIM OF THE WORK: The aim of the work was to compare methods of treatment and outcome in newborns with necrotising enterocolitis (NEC) in two regional centres. METHODS: Medical files of 125 newborns with NEC, treated from 1990 to 1995 in two centres--Royal Hospital for Sick Children, Glasgow, Scotland, and Western Pomerania, Poland have been analysed retrospectively. The following criteria have been studied: birth weight, gestational age, sex, risk factors, initial NEC symptoms, time of onset of the disease, x-ray findings, NEC staging, treatment protocols, outcome and complications. RESULTS: The overall mortality was 32.8%; 34.4% in Glasgow vs. 31.3% in Pomorze. Higher incidence of NEC in the group of full-term babies was observed: 31.2%. Drainage under local anaesthesia was performed in 27 Scottish newborns; none of the Polish babies were treated by this method. CONCLUSIONS: The overall results of different treatment protocols in both centres are similar. However, Scottish newborns tended to be younger, smaller and more severely ill. Less aggressive treatment-- i.e. peritoneal drainage can be a successful alternative for these tiny, very sick babies.     

Pulmonary neuroendocrine cells (PNEC) and neuroepithelial bodies (NEB): chemoreceptors and regulators of lung development

The airway and alveolar epithelia contain pulmonary neuroendocrine cells whose structure indicates an endocrine function. They are also in contact with sensory nerve fibres. These cells often aggregate into distinct corpuscles-neuroepithelial bodies-and carry membrane receptors sensitive to a number of stimuli, including hypoxia and nicotine. They synthesise, store and release a number of bioactive substances such as serotonin, calcitonin gene-related peptide and the mitogen bombesin. When these are released they contribute to redistribution of pulmonary blood flow, regulation of bronchomotor tone, modulation of the immune response, stimulation of sensory nerve fibres and regulation of lung growth and development. Pulmonary neuroendocrine cells and neuroepithelial bodies seem to be most important in the fetal and neonatal lung as regulators of airway development and hypoxia-sensitive chemoreceptors. There is a link between these cells and specific types of lung cancer and their involvement in lung and paediatric pathology may be profound.     

Ureterocystoneostomy (UCN) and subureteral collagen injection (SCIN): combined one-stage correction of high-grade bilateral vesicoureteral reflux (VUR) in children.

The treatment of vesicoureteral reflux (VUR) is still a controversial issue. The efficacy of medical treatment appears to be equal to that of operative procedures in avoiding new formation of renal scars. However, there are generally accepted indications for operative procedures including bilateral high-grade VUR, especially in young patients. Ureteral reimplantation (UCN) is the operative treatment of choice in cases with high-grade VUR. Alternatively in cases with lower-grade VUR, injection of bulking agents under the refluxive orifice can be performed. It is also generally accepted that UCN with extravesical preparation of the ureter and the bladder should not be done bilaterally in a one-stage procedure. Postoperative bladder dysfunction may result due to detrimental neurogenic effects. In this study we report on our operative procedure in cases with bilateral high-grade VUR, during which we perform intra/extravesical UCN (mod. Leadbetter-Politano) of the higher-grade refluxive ureter, and (open) subureteral collagen injection (SCIN) of the lower-grade refluxive orifice as a combined one-stage procedure. In this study 50% of the patients had no VUR on either side after the first combined procedure. 15% of the patients showed significant down-grading of VUR of the injected side. These patients underwent a 2nd endoscopic SCIN. 35% of the patients showed no change of VUR of the injected side after the first procedure; these patients underwent reimplantation of this side in another operation. Accordingly, 50% of patients with bilateral high-grade VUR required a 2nd operative procedure under full anesthesia to achieve loss of VUR on both sides. None of the patients showed bladder dysfunction postoperatively. Mean follow-up after the last operative correction was 29.9 months (6 - 84 months).     

Hand-foot-uterus- (HFU) syndrome with hypospadias: The hand-foot-genital- (HFG) syndrome

Three brothers with the typical findings of the HFU-syndrome, but in addition with various degrees of hypospadias are reported. The family history otherwise was negative. The similarity of the pattern-profile of these cases with the mean pattern of the 11 previously reported ones is striking, as expressed by a product moment correlation of 0.83, 0.8 and 0.78 respectively. The widening of the concept of the HFU to that of a HFG-syndrome, as suggested by Poznanskiet al. in 1974, is discussed.Presented at the 12th annual meeting ESPR, Madrid, April 1975.     

Nephroblastoma (Wilms tumor): Tubule density and prognosis

Relative differences in tubule density of the primary tumors have been suggested to be prognostically useful in nephroblastoma (Wilms tumor). Forty cases from our institution were retrospectively graded according to tubule density. There were significant differences in disease-free survivals between histologic grades. When compared to other clinical and pathologic staging criteria, tubule density was not more useful prognostically than the staging systems tested. However, when used in conjunction with clinical or pathologic stage, tumor grade improved prognostic sensitivity. Regardless of grade or stage, patients less than 24-months-old at diagnosis had better disease-free survival.     

Metabolism: Galactosemia (Transferase Deficiency)

Galactose, a hexose Sugar, is a Constituent of many foodstuffs. It is present in most animals milks and represents a major source of calories for infants. Galactose provides a sources of energy through its converstion to glucose-1-containing compounds in the body. Inherited deficiency states have been described for all steps in the major pathway of galactose metabolism. Galatose-1-phosphate uridyl transferase (transferse) deficiency is the most commonly encontered. It has an incidence of approximately 1:60,000 and it is inherited as an autosomal recessive trait. The clinical consequence of the biochemical defecst appears early in life and usually is severe. Vomiting, diarrhea, jundice, hepatomegaly, renal tublar abnormalities, cataracts and mental retardation are some of the majour clinical features. Untreated, mortality rate is high. Treatment is readily accomplished and consists of elimination of galactose(lactose) from the diet. The acute manifestation are reversed; cataracts do not grogress and severs mental retardation prvented. The outcom, however, is not wholly satisfactory, even when treatment is intiated early. Growth and development usually are normal, but intellectual devlopment variable. Many children have visual-perceptual problem and I.Q.'s below average. School performance generally has been unsatisfactory. Ovarian failure frequently has been found in female galactosemics. The casuse has not been determined.     

Neuroactive neurosteroids: dehydroepiandrosterone (DHEA) and DHEA sulphate

Dehydroepiandrosterone (DHEA) and its sulphate ester (DHEAS) are neuroactive and are both imported into the brain from the circulation and produced in the nervous system. These neurosteroids have neurotrophic and excitatiory effets, and further study is needed to delineate their physiological and pathological functions.     

Insulin-like growth factor-I (lGF-l): safety and efficacy

Insulin-like growth factor I (IGF-I) is a peptide synthesized mainly in the liver by stimulation by pituitary growth hormone (GH). It circulates almost entirely bound to its binding proteins. It is the anabolic effector hormone of GH. It is the only treatment in states of GH resistance such as Laron syndrome and blocking antibodies to human GH. As it suppresses insulin and GH secretion it has been used in states of insulin resistance including Type II diabetes mellitus. IGF-I is administered by once or twice daily injections. Adverse effects are mostly caused by overdosage. The usual daily dose in children ranges from 100-200 microg/kg.     

Diphenoxylate-atropine (Lomotil) overdose in children: an update (report of eight cases and review of the literature)

Eight pediatric accidental overdoses of diphenoxylate-atropine (Lomotil) are reported, and 28 literature cases are reviewed. This overdose is primarily an opioid intoxication, occasionally associated with atropine toxicity. Only 6 of 36 children showed signs of atropine overdose (central nervous system excitement, hypertension, fever, flushed dry skin). Contrary to popular belief, atropine effects occur before, during, or after opioid effects. Opioid overdose (central nervous system and respiratory depression with miosis) predominated or occurred without any signs of atropine toxicity in 33 cases (92%). Diphenoxylate-induced hypoxia was the major problem and was associated with slow or fast respirations, hypotonia or rigidity, cardiac arrest, and in 3 cases cerebral edema and death. Respiratory depression recurred 13 to 24 hours after the ingestion in 7 cases and was probably due to accumulation of difenoxine, an active metabolite of diphenoxylate. Recommended treatment is intravenous naloxone for depressed or inadequate respirations, followed by continuous intravenous naloxone infusion, prompt gastric lavage, repeated administration of activated charcoal, and close monitoring for 24 hours.