Invasive group A streptococcal infection after tonsillectomy.

Tonsillectomy is a very common procedure in childhood. Infectious complications after tonsillectomy are infrequently reported. We describe two children with severe group A beta-hemolytic streptococcal infection after tonsillectomy, and we review the literature about bacteremia and infectious complications after tonsillectomy.

Cellular and humoral defence mechanisms against bacteria.

Host defence to bacterial infection is a complex process of highly efficient effector systems that co-operate to eliminate microbial invaders. The systems not only co-operate, they are also interdependent, as deficiency of one system may cause dysfunction in another. As is so often the case in medicine, the discovery of the various effector systems and their components originated from studies of patients with unusually severe, frequently recurring infections.

Status asthmaticus treated by high-frequency oscillatory ventilation.

We present a 2.5-year-old girl in severe asthma crisis who clinically deteriorated on conventional mechanical ventilation, but was successfully ventilated with high-frequency oscillatory ventilation (HFOV). Although HFOV is accepted as a technique for managing pediatric respiratory failure, its use in obstructive airway disease is generally thought to be contraindicated because of the risk of dynamic air-trapping.

Congenital hepatic fibrosis in 3 siblings with phosphomannose isomerase deficiency.

Congenital hepatic fibrosis is a rare disorder of intrahepatic bile ducts with the persistence of embryological bile duct structures in ductal plate configuration. Three siblings aged 18, 17 and 14 years old were found to have congenital hepatic fibrosis associated with a deficiency of the enzyme phosphomannose isomerase. The clinical symptoms were recurrent attacks of persistent vomiting with diarrhea and mild hepatomegaly.

High-frequency oscillatory ventilation in pediatric patients.

Background: High-frequency oscillatory ventilation (HFOV) is a ventilatory mode using small tidal volumes with low phasic pressures at supraphysiological frequencies. Beyond the neonatal period there are distinct lung diseases for which HFOV is used. Data of 35 children who deteriorated on conventional ventilation were retrospectively analysed in two tertiary pediatric intensive care units.

Continuing education in neurometabolic disorders--serine deficiency disorders.

Serine deficiency disorders comprise a new group of inborn errors of serine metabolism. Patients affected with these disorders present with major neurological symptoms including congenital microcephaly, seizures, psychomotor retardation or polyneuropathy. The diagnosis of serine deficiency is based on the detection of low concentrations of the amino acids serine and glycine in fasted plasma and cerebrospinal fluid (CSF).

Peripheral muscle weakness and exercise capacity in children with cystic fibrosis.

Exercise intolerance in cystic fibrosis (CF) is attributed to diminished nutritional and pulmonary function. We studied the pathophysiology of such intolerance in relation to muscle force and fat-free mass (FFM), in 15 children with moderately severe symptoms of CF (FEV1 < 80% predicted and/or weight for age < -1 SD of reference median), 13 children with mild symptoms of CF (FEV1 and weight above these thresholds), and 13 healthy controls. Cycle maximal workload (Wmax) and V O2max were assessed.

Pain in childhood rheumatic arthritis.

Pain is a major symptom in chronic inflammatory arthropathies such as rheumatoid arthritis and affects the health status of arthritis patients negatively. There has been much debate about the role of pain in juvenile chronic arthritis and this review deals with the controversies about this subject. Pain in children is best understood as a multifactorial concept in which pain is the result of somatosensory, behavioural and environmental factors.

The success of dietary protein restriction in alkaptonuria patients is age-dependent.

Alkaptonuria is characterized by an increased urinary excretion of homogentisic acid, pigmentation of cartilage and connective tissues, and ultimately the development of inflammatory arthropathy. Various diets low in protein have been designed to decrease homogentisic acid excretion and to prevent the ochronotic pigmentation and arthritic lesions. However, limited information is available on the long-term beneficial effects of these diets.

Clinical heterogeneity and novel mutations in the glycerol kinase gene in three families with isolated glycerol kinase deficiency.

Isolated glycerol kinase deficiency (GKD) is an X linked recessive disorder. The clinical and biochemical picture may vary from a childhood metabolic crisis to asymptomatic adult "pseudohypertriglyceridaemia", the result of hyperglycerolaemia. We performed glycerol kinase (GK) gene analysis to study the molecular heterogeneity and genotype-phenotype correlation in eight males from three families with isolated GKD.

Beneficial effects of L-serine and glycine in the management of seizures in 3-phosphoglycerate dehydrogenase deficiency.

3-Phosphoglycerate dehydrogenase (3-PGDH) deficiency is an inborn error of serine biosynthesis. Patients are affected with congenital microcephaly, psychomotor retardation, and intractable seizures. The effects of oral treatment with amino acids were investigated in 2 siblings.

Antibody responses and opsonic activity in sera of preterm neonates with coagulase-negative staphylococcal septicemia and the effect of the administration of fresh frozen plasma.

Coagulase-negative staphylococcal septicemia is the most prominent nosocomial infection in neonatal intensive care units. Immaturity of host defenses in premature neonates is assumed to constitute an important risk factor. Opsonophagocytosis is considered to be the key host defense system against staphylococci with IgG antibodies as a major opsonin.

Total energy expenditure in infants with bronchopulmonary dysplasia is associated with respiratory status.

Unlabelled: Growth failure is a well-known problem in infants with bronchopulmonary dysplasia (BPD). We studied BPD infants' total daily energy expenditure (Ee), nutritional balance, and growth in relation to their past and current clinical status. Applying the doubly labelled water technique, Ee was measured in nine preterm infants with BPD receiving supplemental oxygen (postnatal age 61 +/- 13 days) and nine matched controls (36 +/- 21 days) during a 6-day period.

Ontogency of the responses of human peripheral blood T cells to glucocorticoids.

The inhibitory effect of steroids such as dexamethasone on the immune response of adults has been widely documented. Little is known, however, about the effect of these agents on the developing immune system in newborns. The present study describes the ontogeny of the sensitivity of peripheral blood T cells to dexamethasone during the first year of life.

Magnetic resonance imaging and spectroscopy of the brain in propionic acidemia: clinical and biochemical considerations.

Three patients with propionic acidemia were studied. The first patient was diagnosed at the age of 9 mo, 3 mo after he developed hypotonia and choreoathetoid movements after an upper respiratory tract infection. The second patient was diagnosed at the age of 1.

Physical adaptation of children to life at high altitude.

Children permanently exposed to hypoxia at altitudes of > 3000 m above sea level show a phenotypical form of adaptation. Under these environmental conditions, oxygen uptake in the lungs is enhanced by increases in ventilation, lung compliance, and pulmonary diffusion. Lung and thorax volumes in children growing up at high altitude are increased.

Treatment of giant omphalocele by enlargement of the abdominal cavity with a tissue expander.

Even in the absence of major associated anomalies, treatment of giant omphaloceles is difficult primarily because of the disproportion between the large volume of the omphalocele and the small volume of the intraabdominal cavity. The case of a child is presented in whom conservative treatment had to be abandoned. Reduction of the omphalocele contents and closure of the defect was successfully accomplished after a 19-day period of enlargement of the abdominal cavity by means of an intra-abdominally placed tissue expander.

Gas chromatography method for the separation of amino acids enantiomers in plasma and urine. Application in a case of short bowel syndrome.

Urinary amino acids were isolated from the urine of healthy controls and a patient with a short bowel syndrome. Following derivatization with isopropyl alcohol/HCl and trifluoroacetic anhydride the amino acid enantiomers were separated by gas chromatography on a Chirasil-L-Val column. All subjects excreted D-alanine (10-30% of total Ala).