Intractable diarrhoea in infancy in the 1990s: a survey in Italy.

UNLABELLED: A "quick" prevalence study of intractable diarrhoea (defined as diarrhoea lasting more than 3 weeks and dependent on parenteral nutrition [PN] for more than 50% of daily caloric intake) was conducted by FAX. All 35 paediatric gastroenterology services which had been contacted answered questionnaire sent by FAX. 20 cases of intractable diarrhoea were identified in 9 centres. In 12 cases PN was administered at home, the other 8 cases being treated as inpatients for an average duration of 9.5 months. A diagnosis had been established in 11 out of 20 cases. Auto-immune enteropathy was the most frequent diagnosis (5 cases); congenital microvillous atrophy (3 cases); chronic pseudo-obstruction (2 cases) and multiple food intolerance (1 case). Undefined 9/20 cases presented atrophy of intestinal mucosa. The age of the beginning of diarrhoea varied from 2 days to 12 years, but was more than 16 months only in some cases with auto-immune enteropathy. CONCLUSION: Intractable diarrhoea has a low prevalence in Italy and remains a rare but very intricating problem. Long-term PN is recommended in most cases: autoimmune enteropathy is the most frequent cause but in about half of the cases the aetiopathogenetic diagnosis is still not defined.

Human granulocyte colony-stimulating factor (rHuG-CSF) for treatment of neutropenia in Shwachman syndrome.

We report a boy affected by Shwachman syndrome (SS) who presented severe neutropenia and frequent suppurative infections, which we treated successfully with granulocyte colony-stimulating factor (rHuG-CSF). Daily injections of 7.5 micrograms/kg/day significantly increased the absolute neutrophil count and he was free from infections. In order to avoid the risk of side effects and to improve the child's quality of life, we intermittently administered lower doses of rHuG-CSF. A weekly dose of 2 micrograms/kg/day was able to maintain the absolute neutrophil count high enough (0.58-1.2 x 10(9)/L) to prevent suppurative infections. During the follow-up period (2.5 years) we tried suspending rHuG-CSF twice, but the absolute neutrophil count (0.18-0.31 x 10(9)/L) fell significantly and suppurative infections reoccurred (otitis, perianal abscess). rHuG-CSF may be a useful therapeutic agent in patients with symptomatic neutropenia in SS.

Signs of severe bacterial infection in neonates.

The objective of this study was to identify a short list of valid signs for the development of standard case management guidelines for severe bacterial infection (SBI) in newborn infants, an important cause of neonatal deaths in low-income countries. The reported and observed signs of 83 sick neonates admitted during 12 consecutive months were recorded. At discharge, 50 cases were classified, using predefined criteria, as SBI, mostly pneumonia, and 33 as other disease. The neonates with other diseases were significantly younger than those with SBI. None of the reported and observed signs, when used alone, had a high sensitivity, an important feature for a severe disease amenable to effective treatment. The best sensitivity (74 per cent) was obtained when a doctor observed severe chest indrawing or fast breathing or 'not looking well'; the specificity was 67 per cent and the positive predictive value 77 per cent. The sensitivity of reported difficult breathing and of observed severe chest indrawing, when measured only for the diagnosis of pneumonia, improved to 77 per cent, with a specificity of 84 per cent and 66 per cent, respectively. Reported fever and the observation that the neonate was 'not looking well' were the best independent predictors of SBI on logistic regression analysis. Simple standard case management (SCM) guidelines based only on reported and observed clinical signs would not identify the majority of neonates with SBI at primary health care level.

Thermal control of the newborn: knowledge and practice of health professional in seven countries.

Hypothermia is a common problem in neonates, particularly in developing countries where it is an important contributory factor to neonatal mortality and morbidity. An evaluation of the knowledge and practices of health professionals on the thermal control of newborns was carried out in seven countries: Brazil, India, Indonesia, Kazakhstan, Mozambique, Nepal and Zimbabwe. The evaluation, conceived as a preliminary phase for a one-day training course on thermal control, involved 28 health facilities and 260 health professionals (61 doctors and 199 nurses and midwives). It included an assessment of thermal control practices carried out in each health facility by external investigators and a questionnaire on knowledge about thermoregulation administered to health professionals involved in newborn care. The findings of the evaluation were consistent across countries and showed that thermal control practices were frequently inadequate in the following areas: ensuring a warm environment at the time of delivery; initiation of breastfeeding and contact with mother, bathing; checking the baby's temperature; thermal protection of low birth weight babies, and care during transport. Knowledge on thermal control was also insufficient, especially concerning the physiology of thermoregulation and criteria for defining hypothermia. During the one-day course that followed the evaluation, participants were able to recognize the existing gaps and to identify appropriate interventions. Knowledge and practice on the thermal control of the newborn are currently insufficient. However, awareness of the importance of thermal control and basic knowledge on thermal regulation and thermal protection can be easily acquired and on this basis motivation for improving thermal control practices can be developed.