Encephalopathy with retinitis due to cat-scratch disease.

Cat-scratch disease is one of several diseases known to be caused by Bartonella species. Some infections due to Bartonella resolve spontaneously without treatment with antibiotics, but in other cases the disease can be fatal without treatment. This case study reports a 7-year-old male who presented with an unexplained encephalopathy and unusual retinal findings associated with evidence supporting infection by B. henselae. The 7-year-old male presented with a 2-week history of general malaise and cervical lymphadenopathy progressing onto fever, headache, vomiting, and confusion associated with meningism. Lumbar puncture revealed a raised cerebrospinal fluid protein, low glucose, and raised white cell count. Abnormal retinal findings and raised antibodies titres to B. quintana indicated a diagnosis of cat-scratch disease. He was treated with azithromycin orally for 3 weeks and made a complete recovery.

The use of interferon beta in relapsing-remitting multiple sclerosis.

The use of interferon beta-1a to treat multiple sclerosis in a child of 7 years of age is discussed. To date, there is only one other published report of the use of interferon beta in a child as young as this. One year after commencing treatment she had shown significant clinical improvement, with a marked reduction in number of relapses. In her second year of treatment she suffered a major relapse from which she slowly recovered.

Ear nose and throat diseases in the Bedouin of the South Sinai Desert: a cross-sectional survey and discussion of healthcare needs.

In the summer of 2000, a team of medical and affiliated workers visited the South Sinai Desert to assess the medical problems and needs of the Bedouin population. This paper reports on the ENT diseases of this population, and discusses their healthcare needs.

Massive pulmonary embolus in a 14 year old boy.

Pulmonary embolus in children is rare. A case of massive pulmonary embolus, after surgery, in a child of 14 years is described. Accident and emergency doctors should be aware that pulmonary embolus can occur in children and exercise a high index of suspicion for the diagnosis in those patients with risk factors for the condition who present acutely with typical symptoms such as dyspnoea, chest pain, haemoptysis, or collapse.

Planning for the future: the experience of implementing a children's day assessment unit in a district general hospital.

A paediatric day assessment unit was opened in May 1995. An audit of the first year's activity showed that, despite an increase in emergency admissions of 8.4%, the number of children admitted as an emergency overnight was reduced by 3% in paediatrics and 7.2% in surgery. In the first year, 1731 children were assessed as an emergency of whom 658 did not require admission to the inpatient ward. Midnight occupancy fell 17.7% in paediatrics and 25.4% in surgery. The nurse staffing costs fell over Pounds 32,000 in the first year. No adverse events occurred during the first year of operation; this type of facility could lead to a more efficient use of resources in any children's unit.

Relationship of cranial ultrasonography, visual and auditory evoked responses with neurodevelopmental outcome.

Sixty-two preterm infants were followed up with flash visual evoked responses (VERs), brainstem auditory evoked responses (BAERs) and neurodevelopmental assessments for 18 months after term. Cranial ultrasonography showed that 18 infants developed intraventricular haemorrhage (IVH) during the newborn period and four of six infants with grade III or IV haemorrhage had absent VERs at term. The mean latency of the VER in the infants with IVH was significantly shorter than that of infants without IVH. There was no correlation between the degree of ventricular dilatation at term and the latency of the VER. Six months after term the four infants with previously absent VERs had normal responses, though there was still a significant difference between the latencies of infants with and without IVH. At 12 and 18 months of age these differences had disappeared. BAERs were not significantly different in the infants with and without IVH, and there was no difference in the VERs or BAERs of infants with neurodevelopmental delay and those developing normally. Neither flash VERs nor BAERs provide a good prognostic indicator of future neurodevelopmental disability or outcome.

Perinatal hemochromatosis. Clinical, morphologic, and quantitative iron studies.

Three sibling and two isolated-case perinates (4 newborn, 1 stillborn) died with siderotic cirrhosis and widespread parenchymal siderosis, the latter similar to that seen in both hereditary and secondary hemochromatosis. Reticuloendothelial siderosis was absent, as occurs in primary hemochromatosis. Studies of iron metabolism were performed antemortem in two of the siblings and ante-, post- and internatally in their mother, who showed hyperferremia antenatally. The only finding in the affected family suggestive of hereditary hemochromatosis was the commonly associated HLA haplotype (A3, B7) in the mother and an infant. Liver morphology, including immunocytochemistry and ultrastructure, was similar in the 5 infants and suggested that liver disease commenced as massive necrosis in midfetal life. Histologic grading and chemical assays for iron and copper on liver and spleen of the 5 index cases were compared with 26 controls; placentas were compared with 12 control placentas. Hepatic iron concentration, but not hepatic copper concentration, was significantly increased in index cases, compared with controls. Hepatic iron to copper ratio was significantly increased in index cases, compared with controls, but this ratio was unaltered in spleen and placenta. Total hepatic iron, but not total hepatic copper, was significantly increased in index cases, compared with a subgroup of 11 controls of low gestational age, similar to the fetal stage when liver disease commenced in utero. The results suggest that, irrespective of the fetal liver disease being genetic or acquired, hepatic iron overload was directly involved in pathogenesis.

Comparison of four pancreatic extracts in cystic fibrosis.

Four different pancreatin products, Pancrease, Creon, Pancrex V Forte, and Pancreatin Merck, were compared in a random crossover trial in children with cystic fibrosis. The results of our study showed that patients who received Creon and Pancrease had fewer gastrointestinal symptoms than patients who received Pancrex V Forte and Pancreatin Merck. Fat absorption was significantly improved with Pancrease when compared with Pancrex V forte and Pancreatin Merck. Also the fat absorption with Creon was superior to that with Pancrex V Forte. There was no significant difference in fat absorption between Pancrease and Creon. Pancrex V Forte and Pancreatin Merck, or Pancreatin Merck and Creon. Faecal nitrogen content was less with both Creon and Pancrease compared with Pancreatin Merck. Creon and Pancrease allow the patient with cystic fibrosis to take a high energy diet without any dietary restrictions.

Insulin dependent diabetes in Asians.

Type 1 diabetes is said to be extremely rare in children in India, where diabetes treated with insulin may be due to chronic pancreatic disease or malnutrition. To see whether typical type 1 diabetes occurred in Asian children in the United Kingdom, all known Asian children with diabetes in industrial West Yorkshire were ascertained. A total of 17 such children were studied; of these, seven were from three multiplex families and two fathers from these families had diabetes. All children were ketosis prone and developed diabetes while resident in the UK. There were significant increases in HLA-B8 and HLA-DR3 and increases in HLA-DR4 and HLA-DR3/DR4, while HLA-B15 was absent. Islet cell antibodies, either IgG or complement fixing, were present in four of 18 subjects tested, all of whom had disease of short duration. The prevalence of type 1 diabetes in Asian children aged 15 years or less in West Yorkshire was 36/100,000, assuming complete ascertainment. It is concluded that typical type 1 diabetes may occur in Asian children and this condition may be more common in families who have migrated to the UK.

Randomized controlled trial of very early continuous distending pressure in the management of preterm infants.

Application of continuous distending pressure at birth (very early CDP) should stabilize the immature airways and reduce the severity of respiratory distress syndrome (RDS) in preterm infants. Eighty-two preterm infants of less than 32 weeks gestation were randomly assigned at birth to early treatment group (TG), in which CDP of 6 cm water pressure was applied at birth by the nasopharyngeal route (NP-CDP), or to control group (CG), in which CDP was applied when indicated for established criteria (pO2 less than 50 mmHg in FiO2 greater than 0.5). Characteristics of the infants in the two groups were comparable. No statistically significant difference between the two groups was found in the incidence of RDS. The course of RDS, and oxygen and ventilatory requirements also did not appear to be changed. In blood gas parameters of most of the time frames, no significant difference was found between the two groups when the results were analyzed according to the assigned group. When the results were analyzed separately for the infants who developed RDS, infants in TG appear to have fared worse from the therapy in terms of oxygenation, as indicated by significantly higher FiO2 (P less than 0.01) and lower a/A (P less than 0.01) values on the third day of the course of RDS, as compared to infants in CG. The incidence of complications was comparable in the two groups. Four infants from TG (9.3%) and one from CG (2.6%) died (P = NS). We conclude that VECDP by nasopharyngeal route does not reduce the incidence of RDS and does not appear to improve the outcome and may worsen the severity of RDS when compared to application of CDP for established criteria.

High plasma urea concentrations in collodion babies.

We describe two infants born with a collodion membrane; both were treated with a product containing 10% urea and 5% lactic acid and as a consequence were found to have a raised plasma urea concentration.

Prevention of intraventricular haemorrhage by fresh frozen plasma.

Seventy three preterm infants weighing less than 1500 g or less than 32 weeks' gestation, or both, were allocated randomly to treatment (fresh frozen plasma 10 ml/kg on admission and at 24 hours of age) or control groups. Fifteen (41%) out of 37 control patients sustained intraventricular haemorrhage compared with five (14%) of 36 patients receiving treatment (X2 = 5.24, P = 0.022). No difference was found in coagulation factors measured at birth or at 48 hours of age in both groups. Fresh frozen plasma appears to have a beneficial effect in the prevention of intraventricular haemorrhage.

Intraventricular haemorrhage--timing of occurrence and relationship to perinatal events.

A total of 150 infants born at or before 34 weeks gestation had serial cranial ultrasound scans at age 8, 16, 24, 36, 48 h and 1 week. The overall incidence of IVH was 26%, but for infants less than 1500 g it was 51% and 50% of all haemorrhages were first detected in the first 8 h of life. Low-outlet forceps delivery and caesarean section offered some protection, but umbilical cord blood gas analysis did not support the hypothesis that hypoxia was causal. Respiratory distress and its complications were significantly associated with IVH. The more severe haemorrhages occurred in the less mature infants. The overall mortality in the study was 27% for the IVH group and 1.8% for the non-IVH group.

Cord blood gases, birth asphyxia, and intraventricular haemorrhage.

The relation between intraventricular haemorrhage, Apgar scores, and cord blood gases was determined prospectively in 150 infants of 34 weeks' gestation, or less. Although there was no significant relation between birth asphyxia and subsequent haemorrhage, a consistent trend for lower Apgar scores in infants who developed haemorrhage was shown.

Intraventricular haemorrhage and haemostasis defects.

Twenty five of 106 preterm infants of 34 weeks' gestation or less developed intraventricular haemorrhage within the first 48 hours of life. A comparison of infants with and without intraventricular haemorrhage showed no significant differences in their haemostatic parameters at birth. At age 48 hours the group with intraventricular haemorrhage showed a prolonged activated partial thromboplastin time and reduced factor II, VII, and X activity. There was a significant correlation between the severity of intraventricular haemorrhage and the degree of haemostasis abnormality both in cord blood and in blood obtained at age 48 hours. Those infants sustaining grade IV intraventricular haemorrhage had a significantly prolonged activated partial thromboplastin time, reduced factor II, VII, and X activity; and a decreased fibrinogen concentration at birth. At age 48 hours these defects were accompanied by reduced platelet counts and an increased megathrombocyte index. Although intraventricular haemorrhage is multifactorial, we postulate that correction of haemostasis abnormalities at birth may prevent progression to more severe grades of haemorrhage.