Perfluorodecalin lavage of a longstanding lung atelectasis in a child with spinal muscle atrophy.

OBJECTIVE: Persistent lung atelectasis is difficult to treat and perfluorochemical (PFC) liquid may be an option for bronchioalveolar lavage (BAL). CASE REPORT: A 4-year-old girl with spinal muscle atrophy was admitted in respiratory failure. On admission, the X-ray confirmed the persistence of total right-sided lung atelectasis, which had been present for 14 months. She was endotracheally intubated and ventilated from the day of admission. BAL with normal saline was performed twice without improvement. Following failed extubation and being dependent on continuous respiratory support, a trial of BAL using PFC liquid (Perfluorodecalin HP) was carried out. The PFC was delivered through the endotracheal tube on three consecutive days. A loading dose of 3 ml/kg was administered, followed by a varying dose in order to more effectively lavage the lungs. She tolerated the procedure well the first 2 days, although there were no clinical signs of improvement in the atelectasis. Intentionally, higher inflation pressures were applied after PFC instillation on day 3. Chest X-ray then showed hazy infiltrates on her left lung and she required more ventilatory support. However, lung infiltrates cleared over the next 3 days. A tracheotomy was done 6 days after the last PFC instillation. She had a slow recovery and was successfully decanulated. Clinical improvement of lung function was seen including less need of BiPAP and oxygen. A chest CT scan showed then functional lung tissue appearing in the previous total atelectatic right lung. CONCLUSION: Lavage with PFC can safely be performed with a therapeutic effect in a child with unilateral total lung atelectasis.

Clinical aspects of a nationwide epidemic of severe haemolytic uremic syndrome (HUS) in children.

BACKGROUND: Report a nationwide epidemic of Shiga toxin-producing E. coli (STEC) O103:H25 causing hemolytic uremic syndrome (D+HUS) in children. METHODS: Description of clinical presentation, complications and outcome in a nationwide outbreak. RESULTS: Ten children (median age 4.3 years) developed HUS during the outbreak. One of these was presumed to be a part of the outbreak without microbiological proof. Eight of the patients were oligoanuric and in need of dialysis. Median need for dialysis was 15 days; one girl did not regain renal function and received a kidney transplant. Four patients had seizures and/or reduced consciousness. Cerebral oedema and herniation caused the death of a 4-year-old boy. Two patients developed necrosis of colon with perforation and one of them developed non-autoimmune diabetes. CONCLUSION: This outbreak of STEC was characterized by a high incidence of HUS among the infected children, and many developed severe renal disease and extrarenal complications. A likely explanation is that the O103:H25 (eae and stx2-positive) strain was highly pathogen, and we suggest that this serotype should be looked for in patients with HUS caused by STEC, especially in severe forms or outbreaks.

Skin conductance versus the modified COMFORT sedation score as a measure of discomfort in artificially ventilated children.

OBJECTIVE: We wanted to use skin conductance as a measure of increased stress in artificially ventilated children. The aim was to examine how changes in skin conductance, arterial blood pressure, and heart rate are associated with changes in the modified COMFORT sedation score during suction from the trachea. Nociceptive stimulation induces an outgoing sympathetic nervous burst to the skin and the palmar and plantar sweat glands are filled, which creates a skin conductance fluctuation. METHODS: Twenty children who were 1 day to 11 years of age were studied. All patients were artificially ventilated and circulatory stable. The data were obtained before, during, and 10 minutes after endotracheal suction. The number of skin conductance fluctuations, the amplitude of skin conductance fluctuations, the mean skin conductance level, arterial blood pressure, heart rate, and the modified COMFORT sedation score were recorded and tested from before to during and from during to after suction in the trachea. RESULTS. The number of skin conductance fluctuations, mean skin conductance level, arterial blood pressure, and the modified COMFORT sedation score increased during suction in the trachea, in contrast to heart rate and amplitude of skin conductance fluctuations. The number of skin conductance fluctuations from before to during and from during to after endotracheal suctioning correlated with changes in the modified COMFORT sedation score. This was in contrast to the other variables that did not. CONCLUSIONS: The number of skin conductance fluctuations during endotracheal suctioning showed better correlation with the increase in the modified COMFORT sedation score than heart rate and arterial blood pressure. Thus, the number of skin conductance fluctuations seems to be an objective supplement to the modified COMFORT sedation score for monitoring increased stress in artificially ventilated and circulatory stable children.

Bronchioalveolar lavage with perfluorochemical liquid during conventional ventilation.

OBJECTIVE: Therapeutic approaches with bronchioalveolar lavage are currently used in infants with severe alveolar space-occupying material. In many circumstances, bronchioalveolar lavage has been performed in conjunction with extracorporeal membrane oxygenation. CASE REPORT: A 2-month-old boy with severe respiratory failure requiring assisted ventilation did not respond to any conventional treatments, including surfactant. An open-lung biopsy showed intra-alveolar accumulation of proteinaceous material and foamy macrophages but was not diagnostically conclusive. Therapeutic trials with bronchioalveolar lavage using normal saline were unsuccessful, causing episodes of severe hypoxemia. Then, bronchioalveolar lavage during conventional mechanical ventilation was performed with the use of a medical-grade perfluorochemical liquid (perfluordecalin). After instillation of liquid (10 mL/kg), the lungs were refilled three times during the first 24 hrs and repeated 48 hrs later. During perfluorochemical liquid treatment, the infant's condition remained stable, with small improvements in pulmonary mechanics. Suction from the endotracheal cannula yielded only small amounts of gelatinous material. Considering the progression of the disease and just minimal pulmonary improvements by this intervention, further treatment was considered futile. Support was, thus, minimized, and the infant died a few days later. An autopsy revealed the diagnosis to be consistent with Niemann-Pick C2 disease. CONCLUSION: This study demonstrated that bronchioalveolar lavage with perfluorochemical liquid could be performed safely during conventional mechanical ventilation without the additional support of extracorporeal membrane oxygenation in infants with severe alveolar space-occupying material.