The interaction between Sillence type and BMD in osteogenesis imperfecta.

Clinical studies with bisphosphonates in children with osteogenesis imperfecta (OI) show an increase in BMD and a decrease in fracture rate. Bone strength in children with OI is not only influenced by changes in BMD but also by changes in collagen I structure of the organic bone matrix. Therefore, we studied the interaction between these two factors in a cross-sectional, single center study including 54 children. We assumed that vertebral deformities in OI represent an unbalance between load and bone strength. Body weight was considered to be a well quantifiable load on vertebral bodies. BMD served as a marker, representing the amount of bone tissue available for vertebral load bearing, and the Sillence classification, either type I or III/IV, as a marker representing the quality of the organic bone matrix. Independent associations were observed between the prevalence of vertebral deformities and (1) Sillence type (OR: 5.7, 95%Cl:1.2-26.8), (2) BMD (OR: 0.003, 95%Cl: 0-0.25) and (3) body weight (OR: 1.15, 95%Cl: 1.05-1.25). Regarding the anthropometrical differences among the different types of OI, the BMD/body weight ratio was introduced to evaluate the BMD in relation to body size. Prevalent vertebral deformities were associated with low BMD/body weight ratios (OR: 0.04, 95%Cl: 0.008-0.2) in OI type I, but no association was found in type III/IV. It was concluded that BMD and Sillence type have independent relationships with vertebral deformities. The BMD/body weight ratio correlates with vertebral deformities in children with OI type I. Its meaning in types III/IV needs further research with larger samples because of the relatively high prevalence of vertebral deformities in this group.

The value of routine chest radiographs in a paediatric intensive care unit: a prospective study.

BACKGROUND: In many paediatric intensive care units (PI-CUs) chest X-ray films (CXRs) are required as part of the daily examination or after completion of invasive procedures. OBJECTIVE: First, to evaluate if the American College of Radiology (ACR) guidelines for adult patients are appropriate for paediatric patients. Second, to assess the diagnostic efficacy of the CXRs. MATERIALS AND METHODS: One-hundred-seventy-four CXRs acquired in 74 patients, either routinely or after invasive procedures, were analysed. The indication of the obtained CXRs, or the absence of indication in patients in whom no CXRs was taken, was compared with ACR guidelines. The position of medical devices was evaluated. Changes in cardiopulmonary status were noted. RESULTS: Sixty-seven percent of the CXRs were in accordance with the ACR guidelines, and in 74% of patients in whom no CXRs were taken this was also in accordance with these guidelines. Sixteen percent of the endotracheal tubes, 23% of central venous lines and 15% of nasogastric tubes were malpositioned. Changes in cardiopulmonary status, after the initial film, were noted in 63%. CONCLUSIONS: The indications for the majority of CXRs in our PICU appeared to be in accordance with ACR guidelines. The high percentage of malpositioned tubes and lines and the number of cardiopulmonary changes on CXRs in a PICU underline the value of these films. Adjustments of the ACR guidelines for particular groups of paediatric patients may limit the number of CXRs taken and may further increase diagnostic efficacy.

Treatment of adjuvant-induced arthritis by oral administration of mycobacterial Hsp65 during disease.

OBJECTIVE: Oral administration of antigen prior to disease induction has been shown to induce peripheral tolerance in several experimental autoimmune diseases. However, the clinical benefit of pretreatment with antigens is limited. The aim of this study was to investigate whether adjuvant-induced arthritis (AIA) could be treated by oral administration of mycobacterial heat-shock protein 65 (Hsp65) during ongoing disease. METHODS: AIA was induced in Lewis rats by immunization with Mycobacterium tuberculosis in Freund's incomplete adjuvant. Oral feeding of Hsp65 in the presence or absence of soybean trypsin inhibitor (SBTI) was started on day 11 after immunization. Arthritis was monitored visually, and joint pathology was examined radiologically. RESULTS: Oral treatment with Hsp65 during ongoing disease significantly reduced the activity of AIA. However, treatment with Hsp65 was only successful when SBTI was coadministered to prevent breakdown of the Hsp65. The beneficial effect of Hsp65/SBTI treatment during AIA was also represented by a clear reduction of articular destruction, as visualized by radiography. Moreover, feeding Hsp65/SBTI resulted in a lower number of both spleen and mesenteric lymph node (MLN) cells expressing the costimulatory molecule CD80 (B7-1). The number of cells expressing CD86 (B7-2) was not altered. Furthermore, MLN cells from AIA animals treated with Hsp65/SBTI contained a lower number of T cells expressing the activation marker CD134 (Ox-40). In addition, treatment with Hsp65/ SBTI was accompanied by an increased proliferative response of spleen cells to the Hsp65 antigen in vitro. Moreover, Hsp65/SBTI-treated rats showed less Hsp65-specific interferon-gamma and increased production of interleukin-10. CONCLUSION: Ongoing AIA activity can be reduced by oral administration of Hsp65 only when protein breakdown in the gastrointestinal tract is inhibited.

Displacement response of juvenile arthritic wrists during grasp.

OBJECTIVE: To analyze the displacement response of juvenile arthritic wrists during grasp in order to diagnose early ligamental laxity and facilitate early splinting. METHODS: X-rays of the wrists, made under standardized conditions, of 30 children with juvenile chronic arthritis (mean age 10.4 years, range 4.5-16.9) were analyzed after being digitalized. Osseous landmarks were identified, and coordinates were calculated from measured angles and lengths with an accuracy of 0.01'. Lunate and carpal-ulnar distance were obtained according to Youm, and ulnar variance according to Häfner. RESULTS: Overall, an increase in ulnar-lunate displacement and carpal narrowing and a decrease in ulnar variance were found. However, not all wrists responded to the same extent. Radial displacement of the lunate, though slight, was found in 2 wrists and the amount of ulnar displacement varied substantially (3.1% to 22.5%). The variance in amount of displacement could suggest that juvenile wrists do not respond to increased compressive forces to the same extent. CONCLUSION: The changes found are similar to those found in the healthy wrist. Furthermore, our findings suggest that the juvenile wrist acts in accordance with the generally accepted explanation for the development of malalignment in adult wrists. It seems that laxity of ligaments can be diagnosed early by the force grip maneuver during x-ray. It would have a significant impact on the moment of orthotic intervention as well as the design of the orthotic device. Further study along this line seems justified.

MRI of the pulmonary artery after arterial switch operation for transposition of the great arteries.

Twenty-six children who underwent an arterial switch operation for transposition of the great arteries were examined with MRI and Doppler ultrasound (US). The aim was to determine the capability of MRI for detecting a possible stenosis of the pulmonary arteries. In 10 children angiocardiography (ACG) was done after the MRI examination. Using ACG as the gold standard, MRI was sensitive in the detection of pulmonary artery stenosis. MRI was more precise in the localization of a stenosis than was Doppler US. Right ventricular hypertrophy was a sensitive indicator of pulmonary artery stenosis. These results suggest that MRI could serve as a screening examination for pulmonary artery stenosis after an arterial switch operation.

Epidurography in premature infants.

A caudal epidural catheter was inserted in 20 premature, high risk infants for abdominal or thoracic surgery under combined caudal epidural and general anaesthesia. Epidurography was used to confirm the position of the catheter which was found to be misplaced in three patients. The catheter penetrated the dura in one case, in another the tip was located in an epidural vessel and in the third the catheter was seen to be curled up within the epidural space. It was concluded that epidurographic control is essential with this method of anaesthesia in very small infants, in whom it was found to provide considerable advantages despite serious risks.

Ultrasound findings in post-operative jejunojejunal intussusception.

The case is reported of an 11-year-old boy, who developed bowel obstruction after surgical exploration of a kidney transplant. An ultrasound study showed a jejunojejunal intussusception.

Magnetic resonance appearance of adrenal hemorrhage in a neonate.

The Magnetic Resonance (MR) appearance of adrenal hemorrhage in a neonate is described and compared with Ultrasound (US). The value of US studies in adrenal neonatal hemorrhage is well known. We present the MR appearance of this common condition.

[Comment on the publication "Sonographic follow-up of hip dysplasia with expanding trouser therapy"]. / Stellungnahme zu der Publikation "Sonographische Verlaufskontrollen der Hüftdysplasie unter Spreizhosentherapie".

It should be pointed out that hipsonography by application of a flexible control procedure is not only suitable for introduction of therapy for CHD at an early stage, but also for avoiding superfluous treatment. Secondly, the authors are of the opinion that universal screening for CHD by means of hipsonography is for financial considerations not justifiable.

[The small left colon in newborn infants]. / Het kleine linker colon bij pasgeborenen.

We describe 4 patients with a neonatal small left colon. From these cases and from the literature we know that in 40 to 50% the mother has diabetes mellitus. We describe the differential diagnosis with morbus Hirschsprung, meconium plug and the occurrence of a microcolon in atresias and meconium ileus. Three of the four patients were prematures. From the literature we know this abnormality is found mostly in term infants.

Congenital hypothalamic hamartoma associated with severe midline defect: a developmental field defect. Report of a case.

We describe a median "cleft" face anomaly (MCFA) with congenital hypothalamic hamartoma in a newborn girl. The MCFA was associated with a frontal midline skull lipoma and a complex congenital heart defect. Possible pathogenetic mechanisms are discussed, and a review of the pertinent literature is given. It is concluded that probably all malformations in our patient are disturbances of a single developmental field defect, ie, the midline.