[Surgical treatment of vascular malformations in children and adolescents]. / Chirurgische Therapie vaskulärer Malformationen bei Kindern und Jugendlichen.

Vascular malformations are inborn errors of vasculogenesis in localised regions of the body. They are present at birth and grow proportionally with the child without ever showing any tendency to regress. This fact distinguishes them clearly from haemangiomas, which represent vascular tumours with high proliferation during the first year of life followed by a slow involution thereafter. The strategy for the treatment of vascular malformations differs substantially from the one for haemangiomas. According to the type of vascular malformation, the treatment may consist in laser therapy, sclerotherapy, selective embolisation, and/or surgical resection. Whereas systemic medication may save the life of children with life-threatening haemangiomas, such treatment would have no significant effect in children with vascular malformations. The aim of the surgical treatment is to perform a complete resection of the malformation in order to prevent its recurrence. However, since vascular malformations often have an infiltrative growth, frequently only subtotal resections can be performed to avoid unacceptable functional or cosmetic disfigurement of the body. Generally, an optimal management of children with vascular malformations includes a multidisciplinary approach with critical consideration of all potential treatment procedures.

[Medical treatment of children with hemangiomas and its limitations]. / Hämangiomtherapie im Kindesalter: medikamentöse Therapiemöglichkeiten und ihre Grenzen.

Children with disfiguring and/or life-threatening hemangiomas need medical treatment. Initial therapy comprises the oral administration of prednisolone in a dosage between 2-3 mg/kg/day. In cases of insufficient response to prednisolone the therapy may be extended by additional subcutaneous administration of interferon-alpha in a dosage between 1-3 million U/m(2)/day. However, due to the possible serious side effects of interferon-alpha, such as irreversible spastic diplegia, this therapy must be accompanied by close and meticulous neurological examinations of the treated children. The chemotherapeutic substance vincristine has nowadays become an alternative to interferon-alpha for life-threatening hemangiomas. The substance proved effective in a dosage of 0.05 mg/kg for children less than 10 kg and 1.5 mg/kg for children more than 10 kg given weekly strictly intravenously. In worst-case scenarios, a successful disease control has been achieved by intravenous administration of cyclophosphamide in a dosage of 10 mg/kg/day given on 3 consecutive days. Medical treatment of children with life-threatening hemangiomas still remains challenging for all involved persons and should always be performed in specialised centres.

Intestinal intraepithelial lymphocyte derived angiotensin converting enzyme modulates epithelial cell apoptosis.

BACKGROUND & AIMS: Intestinal adaptation in short bowel syndrome (SBS) consists of increased epithelial cell (EC) proliferation as well as apoptosis. Previous microarray analyses of intraepithelial lymphocytes (IEL) gene expression after SBS showed an increased expression of angiotensin converting enzyme (ACE). Because ACE has been shown to promote alveolar EC apoptosis, we examined if IEL-derived ACE plays a role in intestinal EC apoptosis. METHODS: Mice underwent either a 70% mid-intestinal resection (SBS group) or a transection (Sham group) and were studied at 7 days. ACE expression was measured, and ACE inhibition (ACE-I, enalaprilat) was used to assess ACE function. RESULTS: IEL-derived ACE was significantly elevated in SBS mice. The addition of an ACE-I to SBS mice resulted in a significant decline in EC apoptosis. To address a possible mechanism, tumor necrosis factor alpha (TNF-alpha) mRNA expression was measured. TNF-alpha was significantly increased in SBS mice, and decreased with ACE-I. Interestingly, ACE-I was not able to decrease EC apoptosis in TNF-alpha knockout mice. CONCLUSIONS: This study shows a previously undescribed expression of ACE by IEL. SBS was associated with an increase in IEL-derived ACE. ACE appears to be associated with an up-regulation of intestinal EC apoptosis. ACE-I significantly decreased EC apoptosis.

Lung contusion-lacerations after blunt thoracic trauma in children.

Chest trauma in children is an indicator of injury severity and is associated with a high mortality rate. The aim of this study was to investigate the impact of pulmonary contusion-laceration on short and long-term outcome of pediatric patients after blunt thoracic trauma. A retrospective analysis of records of 41 children aged 10 months to 17 years who were treated for pulmonary and associated injuries between 1986 and 2000 was done concerning mode of injury, types of injuries, management and outcome. In addition, a follow-up investigation was performed 4.5+/-1 years after injury. Of the patients 27 were involved in motor vehicle accidents (MVA group) and 14 patients suffered other types of accidents (others group). The mean injury severity score (ISS) was 30+/-2 (range 9-75) with no significant difference between the groups. Patients from the MVA group suffered more frequently bilateral pulmonary lesions and needed more often chest tube placement ( p<0.05), 5 patients died (12%) all from the MVA group. The follow-up investigation of 34 patients showed unremarkable chest x-rays and normal lung function in all but 1 patient with bronchial asthma. In conclusion, children who recover after a pulmonary contusion-laceration trauma do not suffer from significant late respiratory problems.

Reaction of term newborns with prolonged postnatal dyspnoea to early oxygen, mask continuous positive airway pressure, and volume expansion: a prospective, randomised, clinical trial.

UNLABELLED: In a prospective, randomised, open trial 103 term newborns with persisting dyspnoea, tachypnoea and/or cyanosis were treated with oxygen for 5-10 min and then with oxygen plus mask continuous positive airway pressure (CPAP) for another 5-15 min. Cases with overt prenatal or intrapartum obstetric pathology had been excluded from the study. Forty-one infants (40%) responded to this procedure within 10-25 min. The remaining 62 infants (60%) were randomly allocated to one of three forms of further treatment: continuation of mask CPAP for 20 min (group A, n = 24), volume expansion with 9 ml of 3 ml albumin, 3 ml glucose, and 3 mEq of sodium bicarbonate (group B, n = 24), or volume expansion with 4.5 ml albumin and 4.5 ml glucose (group C, n = 14). There was no statistical difference in birth weight, gestational age or Apgar scores at 1 and 5 min between the infants of the groups. Time to normalisation of symptoms was significantly shorter in the volume expansion groups (B: 45 +/- 41 min, range 20-180, and C: 80 +/- 72 min, range 20-210) than in the mask CPAP group (A; 224 +/- 256 min, range 30-1200, P = 0.02). There were statistical differences in umbilical cord and capillary pH values among the infants of the three groups, but the response to therapy was not related to the degree of acidaemia. Thirty-four infants (33%) who did not respond were admitted to a special care unit for further examination (group A: 21/24, group B: 7/24; group C: 6/14). Of these, 23 had no abnormal findings, 8 infants had radiological signs of transitory respiratory distress, and 1 had a non-tension pneumothorax. Septicaemia was found in two infants. No infant was intubated. At discharge all 103 infants did well. CONCLUSION: Incremental application of simple primary care procedures including volume expansion (with or without alkali) in term newborns with persisting postnatal tachypnoea and dyspnoea helps avoid overtreatment and unnecessary separation from the mothers in most cases and reliably selects infants who need close monitoring or special treatment.

Vagal response to feeding tube insertion in preterm infants: has the key been found?

We prospectively studied the vagal response to feeding tube insertion in eight healthy preterm infants, on three occasions in each infant during the first three weeks of life. Heart rate, oxygen saturation, respiration and cerebral blood flow velocities were assessed before, during and immediately after insertion of an orogastric feeding tube. The whole procedure was recorded on video. The duration and quality of tube insertion and the behaviour of the infant were evaluated from the recordings. Twenty-one measurements in eight infants were evaluated. The heart-rate decrease observed immediately after tube insertion correlated significantly with the duration of tube insertion, the quicker the manipulation, the greater the heart-rate decrease (P = 0.000). The maximal decrease of oxygen saturation after tube insertion correlated with the degree of heart rate deceleration (P = 0.009). Significant alterations of the flow velocities were observed only when the heart rate fell below 80 beats/min. We speculate that such episodes of bradycardia can be avoided by carefully inserting the feeding tube over a period of at least 15 s.