Accuracy of Le Fort I osteotomy in bimaxillary splint-based orthognathic surgery: focus on posterior maxillary movements.

This retrospective study was performed to verify the accuracy of horizontal and vertical repositioning of the maxilla in bimaxillary osteotomy with a focus on posterior vertical displacement. Data from 39 orthognathic patients undergoing bimaxillary surgery including a one-piece Le Fort I osteotomy with pitch rotation and advancement at the University Hospitals of Leuven (Belgium), between January 2015 and April 2016, were included in the study. Preoperative and 1-week postoperative lateral cephalograms were digitized and imported into cephalometric software. Horizontal and vertical measurements of dental landmarks were used to assess the accuracy of maxillary repositioning, and errors were reported in terms of the mean and absolute mean. The horizontal advancements were randomly under- and over-corrected an average of 1.4mm±1.2mm. Vertical repositioning of the anterior maxilla followed the planning. A tendency for under-correction was found for posterior vertical intrusion of the maxilla. The same tendency towards under-correction of posterior maxillary inferior repositioning was detected when planned movements were greater than 3mm. For all studied groups, no significant difference was found between the planning and the results achieved, validating the use of intermediate splints.

[A right-sided congenital diaphragmatic hernia masked by pneumonia]. / Een rechtszijdige congenitale hernia diaphragmatica gemaskeerd door een pneumonie.

A 1-day-old premature newborn (34 weeks and 6 days) presented with respiratory insufficiency due to a group B haemolytic streptococcal (GBS) pneumonia. She recovered after temporary treatment with mechanical ventilation and antibiotics. At the time of discharge there was a slight increased fogging on the right side of the chest X-ray, interpreted as residual pleural effusion. Three days later the patient was readmitted with respiratory failure and a need for respiratory support. A chest CT scan revealed a right-sided congenital diaphragmatic hernia (CDH) with a large part of the liver and intestine in the chest. The diaphragmatic defect was closed during a surgical procedure. After an uneventful recovery the patient was discharged in good clinical condition. The combination of delayed presentation of right-sided CDH and neonatal GBS infection occurs rarely but has been described. Its pathogenesis is still unclear.

Disturbed cerebral circulation during opening of the venoarterial bypass bridge in extracorporeal membrane oxygenation.

PURPOSE: To describe the effects on cerebral blood flow velocity (CBFV) of intermittent opening of the venoarterial bridge (VA bridge) during venoarterial extracorporeal membrane oxygenation (VA-ECMO). STUDY DESIGN: Prospective study in 22 newborns during VA ECMO. CBFV was measured in the pericallosal artery by Doppler ultrasound. Changes in peak systolic flow velocity (PSV), end diastolic flow velocity (EDV) and time-averaged mean flow velocity (TAM) on day 1, 2, 3, and 5 and at low ECMO flow (50- 150 ml/min) were analyzed (mean percentage+/-standard deviation (t-tests, p<0.05)). Changes >25% were considered relevant. The relationship between changes in CBFV and ECMO flow rate (Pearson correlation, p<0.01) was studied. RESULTS: Opening of the VA bridge resulted in statistically significant and relevant decreases in PSV (35 +/- 18%), EDV (93 +/- 15%) and TAM (68 +/- 13%), persisting during the consecutive days of treatment. Smaller changes in CBFV at low ECMO flow were statistically significant and mostly relevant: PSV (15 +/- 7%), EDV (76 +/- 21%) and TAM (40 +/- 12%). Changes in CBFV were positively correlated to the ECMO flow. CONCLUSION: Use of the VA bridge results in significant and relevant ECMO flow-dependent changes in CBFV, persisting during the treatment. The VA bridge should be used in such a way as to allow regular unclamping to be omitted.

Major sacrococcygeal teratoma in an extreme premature infant: a multidisciplinary approach.

Antenatally diagnosed, large sacrococcygeal teratomas in very premature infants are associated with a very poor outcome. We present an extreme premature infant with cardiac decompensation, diagnosed at 27 weeks and 1 day of gestational age. A positive outcome could be achieved with intensive multidisciplinary planning of the delivery, postnatal stabilization and surgical resection, as demonstrated in this case report.

[Central congenital hypothyroidism due to Graves' disease in the mother]. / Centrale congenitale hypothyreoïdie door ziekte van Graves bij de moeder.

Two male twins were born at a gestational age of 30 weeks. Five days after delivery, the mother was diagnosed with Graves' disease. The thyroid function in the neonates was therefore evaluated, which led to the detection of central congenital hypothyroidism (central CHT), even though the neonatal CHT-screening had been reported to be normal. Both boys were treated with thyroxine up to the age of nine months. It was then established that their development had been uneventful. Maternal Graves' disease can, due to the presence of anti-thyroid stimulating hormone (TSH) receptor antibodies and the maternal use of anti-thyroid drugs, result in thyroid dysfunction in the neonate. Neonates born to mothers with Graves' disease are at risk of developing central CHT. This occurs especially in children of mothers who are not treated or are inadequately treated during pregnancy. In view of the importance of thyroid hormone for brain development, children with central CHT are at risk for neurodevelopmental problems if thyroid dysfunction is not detected and treated early. The Dutch screening for congenital hypothyroidism is based on thyroxine (T4), TSH and thyroid-binding globulin. This makes it possible to detect central CHT. However, in prematurely born infants this disease may be missed because in this subgroup, referral is only based on increased TSH levels, which may not be present.

[The first newborn with congenital rubella syndrome during the rubella epidemic in The Netherlands in 2004/'05]. / De eerste pasgeborene met congenitaal rubellasyndroom tijdens de rubella-epidemie in Nederland in 2004/'05.

A newborn male was diagnosed with congenital rubella syndrome. His 31-year-old mother had had erythematous exanthema during a period of amenorrhea lasting 7 weeks; she was not vaccinated and had never had a rubella infection. The infection was confirmed serologically. The mother gave birth to an icteric, microcephalic, dysmature neonate with hepatosplenomegaly and exanthema with multiple, small purple-red spots. Ultrasound cardiography revealed a persistently open arterial duct and a small defect of the ventricular septum. Radiological evaluation of the long bones showed the characteristic longitudinal lucent strands ('celery stalk appearance'). Ultrasound of the cerebrum showed diffuse widespread calcifications in the white matter and basal ganglia, striatal vasculopathy and diffuse parenchymal disorders. Psychomotor development was impaired. The patient was completely deaf in the left ear and had severely poor hearing in the right ear. After the introduction of the rubella vaccine in the Netherlands in 1974 a substantial decrease was seen in the incidence of rubella infections as well as congenital rubella syndrome. An epidemic of rubella infections has been present within the non-vaccinated population since September 2004. Recognition of the clinical symptoms and confirmation of the clinical suspicion with proper viral diagnostic methods are needed to control the current epidemic and to prevent secundary spread. Infants born with congenital rubella syndrome remain infectious to non-vaccinated individuals for a prolonged period of time; the virus is excreted in the urine and faeces. Long-term medical follow-up is necessary because the congenital rubella infection can cause abnormalities after the neonatal period.

Recombinant human erythropoietin increases blood pressure, platelet aggregability and platelet free calcium mobilisation in uraemic children: a possible link?

Recombinant human erythropoietin was administered to 10 uraemic children on chronic haemodialysis, all of whom responded by correcting their haemoglobin. In addition, they showed an increase in blood pressure; platelet aggregations, subnormal before therapy, improved during treatment. The intracellular free calcium concentration in platelets after thrombin stimulation also increased significantly during erythropoietin administration. We hypothesize that the effect of erythropoietin on platelet aggregability and on blood pressure may be due to an increase in the intracellular free calcium mobilisation in platelets and possibly in smooth muscle cells respectively.

Enzymatic erythrocyte creatine determinations as an index for cell age.

Creatine concentration in red blood cells was determined after ammoniumsulfate precipitation on a clear hemoglobin-free filtrate with a new enzymatic assay making use of bacterial creatinase. The method described is more specific than Griffiths' method and can easily be mechanised and adapted for use in a routine laboratory using classical automated equipment. By contrast with Griffiths' method no significant interferences of amino acids and creatine-like molecules were found. Reference values for this method were 0.379 +/- 0.076 mmol/l. In patients with high turnover of erythrocytes, e.g. haemodialysis patients (0.529 +/- 0.122 mmol/l), and renal insufficiency patients (0.565 +/- 0.145 mmol/l), significantly increased creatine concentration in erythrocytes were observed. Low erythrocyte creatine concentrations were found in chronic ambulatory dialysis patients (0.311 +/- 0.042 mmol/l).

Mass concentration and activity concentration of creatine kinase isoenzyme MB compared in serum after acute myocardial infarction.

We compared three current methods (immunoinhibition, "Isomune-CK" immunoprecipitation, and the Tandem-E CKMB II immunoenzymometric assay) for determination of creatine kinase (CK; EC 2.7.3.2) isoenzyme MB in serum. Although results inter-correlated well, the immunoinhibition assay gave higher activity values. Atypical CK forms did not interfere with the immunoprecipitation and immunoenzymometric methods. In acute myocardial infarction the catalytic properties of CK decreased with the enzyme's age, as reflected by a steady increase in activation energy of the catalyzed reaction. In septicemia patients with very low CK and CK-MB catalytic activity, mean CK-MB mass concentration exceeded the upper reference limit, suggesting an increased rate of loss of activity concentration in these patients' sera. Because of the assay's lesser susceptibility to conformational changes at the active site of the enzyme, we suggest that measurement of CK-MB mass concentration is better suited for infarct sizing than measurement of catalytic activity.

Characterization of two simian virus 40-specific RNA molecules from infected BS-C-1 cells.

Two discrete simian virus 40 (SV40) RNA species sedimenting at 19 and 16S, respectively, that are present in infected BS-C-1 cells were characterized with respect to the base composition and the ribonuclease T1 fingerprints. The base composition of the 19S SV40 RNA was found to be cytidylic acid (C), 23.0; adenylic acid (A), 28.3; guanylic acid (G), 23.9; and uridylic acid (U), 24.8; that of the 16S SV40 RNA was C, 19.3; A, 34.0; G, 22.0; and U, 24.7 mol%. Analysis of the ribonuclease T1 fingerprints indicated a difference in the base sequence of the 19 and 16S SV40 RNA. The presence of long sequences of adenylic acid residues (poly A) in these viral RNAs was confirmed.