Single-staged anterior and posterior spinal fusion: A safe and effective alternative for severe and rigid adolescent idiopathic scoliosis in China

Aim:   Delayed treatment of adolescent idiopathic scoliosis (AIS) is common in Mainland China because of the lack of public education about health care resulting in the reluctance to undergo surgery. This leads to a high incidence of complex cases where surgeons may not be trained in advanced procedures. We report the efficacy of single-staged anterior and posterior spinal fusion for correction of severe AIS in China.
Methods:   A retrospective review was performed of 31 consecutive cases in which patients were treated at the Orthopaedic Department of Changhai Hospital in Shanghai between 2001 and 2004 with a combined anterior and posterior spinal fusion with screws, hooks, sublaminar wires or cables.
Results:   Thirty-one patients with AIS with Lenke type 1, 2, 3 and 4 curves were included for analysis. At least one of the curves was ≥90° in each patient. The mean coronal and sagittal Cobb angles of the main thoracic curve were 98° and 22° before surgery, 50.5° and 21° after surgery, and 53.7° and 24° at follow-up, respectively. No neurological deficits or deaths occurred. Solid arthrodesis with coronal and sagittal balance was achieved in all patients.
Conclusions:   A single-stage anterior release and fusion and posterior fusion for treatment of severe AIS is good alternative to pedicle screws/vertebrectomy on the basis of risk–benefit balance, and can be performed by surgeons not experienced in more complex procedures. The risk of pulmonary complications may be preferable to the risk of severe neurological complications when thoracic pedicle screws are applied, especially when surgeons are not adequately trained in their use.     

Glossopharyngeal pistoning for lung insufflation in children with spinal muscular atrophy type II

Aim:  To evaluate whether children with spinal muscular atrophy (SMA) type II were able to learn glossopharyngeal pistoning for lung insufflation (GI), and to evaluate the effects of GI on pulmonary function and chest expansion.
Methods:  Eleven children with SMA type II were recruited. They performed 10 cycles of GI, four times per week, for 8 weeks. Lung function and chest expansion were measured before and after the 8-week period.
Results:  Five of the 11 children learned the technique. The median GI volume was 0.28 (range 0.15–0.98) L. Four of the children who completed the study showed a mean increase in inspiratory vital capacity (IVC) of 0.13 L (95% confidence interval (CI) 0.03–0.23) and peak expiratory flow (PEF) of 116 L/min (95% CI 60–173). They also had an increased chest expansion with GI at the level of the xiphoid process of 1.50 cm (95% CI 0.16–2.84) and at the level of the fourth costa of 1.79 cm (95% CI 0.85–2.73). The children reported temporary symptoms of dizziness and tension in the chest.
Conclusion:  Five of the 11 children were able to learn the technique of GI and for the four who fulfilled the training, it had positive effects on IVC, PEF and chest expansion. GI did not cause major discomfort.     

Molecular analysis of the SMN and NAIP genes in Iranian spinal muscular atrophy patients

Background:  Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disorder characterized by degeneration of spinal cord anterior horn cells, leading to muscular atrophy. SMA is clinically classified into three subgroups based on the age of onset and severity. The majority of patients with SMA have homozygous deletions of exons 7 and 8 of the survival motor neuron ( SMN ) gene. The purpose of the present study was to determine the frequency of SMN and neuronal apoptosis inhibitory protein ( NAIP ) gene deletions in Iranian SMA patients. Experience in prenatal diagnosis of SMA in this population is also reported.
Methods:  To study the frequency of deletions of SMN and NAIP genes in an Iranian sample group, 75 unrelated SMA patients (54 type I, eight type II and 13 type III) were analyzed according to the methods described by van der Steege et al and Roy et al .
Results:  Homozygous deletion of SMN1 exons 7 and/or 8 were identified in 68 out of 75 patients (90%). Deletion of exon 5 of the NAIP gene was found in 40/54 of type I, 2/8 of type II and 1/13 of type III patients.
Conclusions:  Deletion of the SMN1 gene is a major cause of SMA in Iran, and NAIP gene deletions were common in the present patients with type I SMA. Also, the incidence of NAIP deletion is higher in more severe SMA.     

One-year glargine treatment can improve the course of lung disease in children and adolescents with cystic fibrosis and early glucose derangements

Background:  Diabetes increases morbidity and mortality in cystic fibrosis (CF) patients, but several studies indicate that also prediabetic status may have a potential impact on both nutrition and lung function.
Objective:  To evaluate the effect of glargine on the clinical course in CF patients with early glucose derangements.
Methods:  CF population was screened for glucose tolerance. CF patients with age >10 yr were screened with fasting hyperglycemia (FH). CF patients with age >10 yr without FH and those with age <10 yr with occasional FH were evaluated for glucose abnormalities on the basis of oral glucose tolerance test and/or continuous glucose monitoring system. All CF patients with glucose derangements were enrolled in an open clinical trial with glargine. Body mass index (BMI) z-score, forced expiratory volume in the first second (FEV₁), number of acute pulmonary exacerbations and hemoglobin A1c, were as outcome measures at baseline and after 1 yr of treatment.
Results:  After 12 months of therapy, BMI z-score improved only in patients with baseline BMI z-score less than −1 (p = 0.017). An 8.8% increase in FEV₁ (p = 0.01) and 42% decrease in the number of pulmonary exacerbations (p = 0.003) were found in the whole group compared with previous 12 months of therapy.
Conclusion:  Glargine could represent an innovative strategy to prevent lung disease progression in CF patients with early glucose derangements. Larger controlled trials are needed to better clarify the effects of insulin on clinical status in CF patients with early glucose derangements.     

Head circumference and development in young children after renal transplantation

Background:  Growth impairment, microcephaly and developmental delay in young children with chronic renal failure improve after successful renal transplantation. There have been few reports on head circumference (HC) and development after transplantation.
Method:  Standard deviation scores (SDS) of height and HC and developmental quotient (DQ) after successful renal transplantation were evaluated in 12 recipients under 5 years of age. At the time of transplantation their mean age was 2.5 years and mean bodyweight was 9.0 kg.
Results:  Mean height SDS was −3.0 at transplantation and increased to −2.3 at 1 year after transplant (P = 0.002). Mean HC-SDS increased from −1.4 to −0.9 at 1 year after transplant (P = 0.02). As for each category of DQ examined 1 year after transplant, mean scores of gross motor function, basic practice, personal relations, speech and recognition increased from 69 to 90 (P = 0.007), from 77 to 102 (P = 0.02), from 87 to 103 (P = 0.04), from 71 to 90 (P = 0.0006), and from 88 to 101 (P = 0.03), respectively.
Conclusion:  In young children, physical growth, HC growth and DQ scores increased 1 year after transplantation. Dialysis and transplantation program should be planned in young children with end-stage renal failure in anticipation of growth and development of each patient.     

Monitoring of Epstein–Barr virus load and antibody in pediatric renal transplant patients

Background: Epstein–Barr virus (EBV) infection can lead to life-threatening post-transplant lymphoproliferative disorder (PTLD). The aim of the present study was to establish EBV monitoring methods to prevent PTLD.
Methods: EBV-DNA load was investigated, using real-time polymerase chain reaction (PCR) and anti-EBV antibody titers, in peripheral blood mononuclear cells of 21 renal transplant patients (seven recipients who were EBV-seronegative, R[−]; 14 who were EBV-seropositive, R[+]) before grafting. The mean age at entry and the mean follow-up period was 7.8 years of age (range, 3.3–12.0 years) and 1.8 years (range, 0.4–4.0 years), respectively, in the R(−) group, and 12.5 years of age (range, 3.9–17.7 years) and 3.8 years (range, 0.8–8.2 years) in the R(+) group, respectively.
Results: The mean maximum load of the EBV genome was 1071 copies/μg DNA (range, 106–20700 copies/μg DNA) in the R(−) group, and 61 copies/μg DNA (range, <50–552 copies/μg DNA) in the R(+) group. During follow up no patient in the R(+) group had any noticeable symptoms that could be related to EBV, but three recipients in the R(−) group developed EBV-related symptoms including adenoid hypertrophy, cervical lymphadenopathy, and PTLD (B cell lymphoma), in one patient each. In the R(−) group the first leukocyte-associated viremia was detected at 30–180 days, and seroconversion at 43–266 days after transplantation.
Conclusions: Viral DNA detection using PCR is a useful tool for EBV surveillance, but the maximum EBV load was not markedly elevated (2474 copies/μg DNA) in a patient with PTLD. Therefore, EBV surveillance using only monitoring of EBV load in peripheral leukocyte may be insufficient. Histology may therefore be necessary to accurately diagnose PTLD.     

Nephrocalcinosis in children: a retrospective multi-centre study

Aim:  To review the data of children with NC and to analyse aetiology, clinical manifestations, growth and renal function at presentation; to relate growth and renal function to changes in NC in patients with a follow-up of at least 12 months.
Methods:  Data of 41 children from four institutions were gathered retrospectively.
Results:  Presenting symptoms were failure to thrive in the first year of life (41%), urinary tract infections, bladder voiding dysfunction or abdominal pain (17%) and psychomotor delay (10%). In 24% of cases NC was detected incidentally. Glomerular function at diagnosis was normal in 83% of children. During a median follow-up of 4 yrs and 5 months in 28 patients, growth standard deviation score improved from a median of −2.2 to −1.0 and glomerular function remained stable in 89% of patients, in spite of worsening of the degree of NC in 62% of cases. The most frequent causes of NC were hereditary tubulopathies and vitamin D intoxication.
Conclusion:  Our results show that the treatment of the underlying conditions is associated with catch-up growth and stabilization of glomerular function in many children, but not with the reduction in the degree of NC in the majority of cases. We believe that early recognition of conditions leading to NC is clinically useful and suggest a diagnostic flowchart, which may be helpful in the approach to NC.     

Impact of prenatal diagnosis on choledochal cysts and the benefits of early excision

Aim:   To evaluate the clinical outcomes of patients with prenatally diagnosed choledochal cysts compared with those diagnosed after birth and the optimal timing of definitive treatment.
Methods:   Retrospective review of all patients who underwent primary choledochal cyst excision and Roux-en-Y hepaticojejunostomy from 1996 to 2006 at a single institution.
Results:   A total of 45 patients were included. Ten (22.2%) of the patients had prior prenatal diagnosis. The mean age at operation for this group was 4.4 months and mean follow up was 55.9 months. There was no operative complication or late morbidity. For the post-natal diagnosis group, the mean age at operation was 5.7 years ( P  < 0.000) and mean follow up period was 69 months. The most common presentation in this group was abdominal pain (31.4%), followed by pancreatitis (28.6%) and symptoms of cholestasis (25.7%). Early post-operative morbidities occurred in two (5.7%) patients. On long-term follow up, two (5.7%) further patients in the post-natal group developed complications.
Conclusion:   Prenatal diagnosis of choledochal cysts results in earlier definitive surgery. More adverse complications were seen in those who had surgery at an older age. We therefore recommend early excision of choledochal cysts.     

Childhood cardiac function after twin-to-twin transfusion syndrome – a 10-year follow up

Aim:  To perform a 10-year follow up of cardiac structure and function after twin-to-twin transfusion syndrome (TTTS) – a severe foetal circulatory complication associated with myocardial hypertrophy in the recipient twin.
Methods:  Cardiac dimensions, systolic and diastolic function as assessed by echocardiography including flow and tissue Doppler velocimetry in 22 healthy survivors of TTTS with a mean age of 9.6 (7.2–11.8) years.
Results:  The donor and recipient twin did not show any differences in end-diastolic ventricular size, interventricular septum thickness, diameter of right ventricular outflow tract, cardiac valves, coronary arteries or in systolic blood flow velocities. However, compared with the donors, the recipients had significantly lower E/A ratios because of lower E-waves in both mitral (−0.15 ± 0.10, p < 0.01) and tricuspid (−0.09 ± 0.07, p < 0.01) valves, indicating reduced early diastolic ventricular fillings compared with donors.
Conclusion:  At school age, twins surviving TTTS had a cardiac structure and function within normal range. There were no differences in heart structure or systolic ventricular function between twins but, compared with the donor twin, we found a reduced early diastolic function in the recipient.     

Continuous subcutaneous insulin infusion vs. multiple daily injections in children with type 1 diabetes: a systematic review and meta-analysis of randomized control trials

Objective:  To investigate potential effects of continuous subcutaneous insulin infusion (CSII) compared with multiple daily injections (MDI) on glycemic control in children with type 1 diabetes mellitus (T1DM).
Study design:  Meta-analysis and systematic review of randomized control studies (RCTs). The electronic databases MEDLINE, Cochrane Library, and EMBASE were searched through October 2007.
Results:  Six RCTs involving 165 participants with T1DM met our predefined inclusion criteria. Combined data from all trials showed that the CSII group compared with the MDI group experienced a significant reduction in the level of glycosylated hemoglobin. The pooled weighted mean difference (WMD) was −0.24% [95% confidence interval (95% CI) −0.41 to −0.07, p < 0.001] with a fixed model and remained significant in the random effect model. This effect was reached by slightly decreasing insulin requirement [three RCTs, n = 74, WMD −0.22 IU/kg/d (95% CI −0.31 to −0.14, p < 0.001)]. No differences in the incidences of ketoacidosis and severe hypoglycemic events were found.
Conclusions:  In short-term insulin therapy, CSII compared with MDI is a more effective form of metabolic control and allows reducing the daily insulin requirement. Yet, no conclusions have been made so far whether this effect holds in later years. These results should be approached with caution because of the methodological limitations of the analyzed studies.     

Cardiac outcomes of hydrops as a result of twin–twin transfusion syndrome treated with laser surgery

Aim:   To determine cardiac outcomes of foetal hydrops as a result of twin–twin transfusion syndrome treated with laser surgery.
Methods:   Hydrops identified in 16 recipient foetuses with twin–twin transfusion syndrome was treated with laser ablation surgery to anastomotic vessels. Prior to laser surgery, the foetuses were assessed by echocardiography for cardiac abnormalities and ventricular and valvular dysfunction. After delivery, echocardiography was performed on 15 of the 16 newborn infants.
Results:   Foetal echocardiography indicated impaired biventricular function in the 16 hydropic foetuses. Five foetuses had little or no forward flow through the pulmonary valve, while four had pulmonary regurgitation. Following laser surgery performed at a mean of 22.9 weeks gestation, hydrops resolved in all cases. Delivery occurred at a mean of 33.6 weeks gestation. Post-natal echocardiography revealed cardiac abnormalities in five neonates, of whom three had right ventricular outflow tract obstruction. One preterm infant with severe pulmonary stenosis died with intractable cardiac failure.
Conclusion:   The majority of hydropic infants with twin–win transfusion syndrome have normal cardiac outcomes following intrauterine laser surgery. As up to one-third may have cardiac abnormalities, cardiological monitoring is recommended during the first year of life.     

Milk formulas in acute gastroenteritis and malnutrition: a randomized trial

Objective:   To compare three low-lactose milk formulas differing in osmolality and degree of protein hydrolysis in the treatment of diarrhoea and malnutrition in subjects with high rates of lactose intolerance, osmotic diarrhoea and a tropical/environmental enteropathy.
Methods:   A randomized double-blind trial of 180 Aboriginal children under 3 years of age admitted with acute diarrhoea and/or malnutrition was carried out. The intervention milk formulas were: (i) De-Lact, a low-osmolality lactose-free formula; (ii) O-Lac, a lactose-free formula; and (iii) Alfaré, a partially hydrolysed formula. Outcome measures were diarrhoeal severity, weight gain, formula palatability and changes in intestinal permeability (L/R ratios).
Results:   The duration of diarrhoea in days (mean; 95% confidence interval) was significantly longer on Alfaré (8.5; 7.0−10.0) compared to De-Lact (6.1; 5.0−7.2) and O-Lac (6.9; 5.6−8.1; P  = 0.04). There were no differences in mean intake between formulas, but palatability of Alfaré was significantly worse ( P  < 0.01) than the other formulas. Over the trial 5 days, improvement in L/R ratios was significantly greater ( P  = 0.05) for De-Lact (18.6; 10.6−26.6) than for Alfaré (8.5; 2.1−14.9). Weight gain was not significantly different between the three formulas, except in a malnourished subgroup who had better weight gain on De-Lact ( P  = 0.05).
Conclusions:   In these Aboriginal children with diarrhoea and growth failure, a low osmolality milk was associated with better outcomes and a partially hydrolysed formula with less improvement in mucosal recovery, suggesting that cow's milk protein intolerance is not contributing to greater diarrhoeal severity or enteropathy in Aboriginal children.     

Basal insulin switch from NPH to glargine in children and adolescents with type 1 diabetes

Background:  Insulin glargine is a long-acting insulin analogue increasingly used instead of neutral protamine Hagedorn (NPH) insulin in young subjects with type 1 diabetes.
Objective:  We evaluated the clinical course of diabetes in children and adolescents who were switched from NPH to insulin glargine.
Methods:  Between August 2003 and November 2004, a total of 76 subjects were switched to glargine in our clinic, treating 340 children with type 1 diabetes. All the subjects had been receiving insulin NPH, and their serum C-peptide levels had been non-detectable for at least 1 yr. Data were collected retrospectively, and 12–18 months after the change, experiences with glargine were inquired using a questionnaire. Seven subjects (9.2%) discontinued glargine before 12 months, and seven refused to participate.
Results:  Data for 62 subjects were analyzed. At the switch (0 months), their mean age was 12.7 yr (range 5.1–17.5), mean duration of diabetes was 6.7 yr (range 1.8–14.3), and mean hemoglobin A1c was (HbA1c) 9.2%. Twelve months later (+12 months), the mean HbA1c remained similar (9.2%), the proportion of long-acting insulin was smaller (47.7 vs. 58.1%; p < 0.001), and the daily insulin dose was lower (0.97 vs. 1.05 IU/kg; p < 0.001). The number of injections was lower at +12 months (17.7% with more than five injections vs. 64.5%; p < 0.001). No differences were seen in weight for height or the number of severe hypoglycemias. Most subjects who continued with glargine for ≥12 months considered glargine better than NPH.
Conclusions:  A switch to insulin glargine retains a similar glycemic control and does not change the number of severe hypoglycemias.     

The development and evaluation of a simple method of gestational age estimation

Aims:   The aims of this study were to develop and evaluate a shorter gestational age estimation method based on the Dubowitz scoring system (DSS) that could be taught easily to untrained health-care workers.
Methods:   Using the DSS, seven criteria were identified that explained 90% of the variance in gestational age estimation. Ventral suspension was excluded, skin texture was substituted for skin colour and ear bending was for ear firmness. The final six criteria comprise the short DSS (SDSS). The study population was 100 babies, 51 Caucasian, 34 Aboriginal. Birth weight ranged from 1351 to 5430 g. The gestational age range was 29.6–41.7 weeks. Three researchers (medical student 1 (MS1), medical student 2 (MS2) and a paediatrician) who were blind to other available gestational age estimations assessed babies within 72 h of birth using the DSS and SDSS.
Results:   MS1 found the 95% limits of agreement (LOA) between SDSS and DSS to be −1.1 to 1.7 weeks. In the 27 babies who had first trimester foetal ultrasound (USS), the LOA between USS and SDSS were −1.9 to 1.4 weeks and −2.3 to 1.9 weeks, respectively. Using the SDSS, the LOA for the three researchers were within ±2 weeks.
Conclusion:   The data presented suggest that the SDSS is approximately equivalent to the DSS in the relatively limited population studied, and within these limitations appears to be a simple alternative to the DSS. Further evaluation is needed before firm conclusions about the accuracy and clinical utility of the SDSS can be made.     

Prospective cohort study of definite spider bites in Australian children

Objectives:   To describe the clinical effects of spider bites in a paediatric population and compare to bites in an adult population.
Methods:   Prospective follow up study of children with definite spider bites where the spiders were immediately collected and expertly identified. Cases were recruited from Australian emergency departments and poison information centres and followed up. Data were collected on circumstances of bites and clinical effects, and compared with similar data from adult spider bites.
Results:   There were 163 cases (62% male, 38% female; age <16, median age 7 years, interquartile range (IQR): 3−11 years). The median duration of effects was 2 h (IQR 0.25−12 h). The commonest spiders were Huntsman spiders ( Sparassidae ) and Orb weavers ( Araneidae ), and comparatively few bites by theridiid spiders, including Red-back spiders. Pain or discomfort occurred in all bites and was severe in 20%. Local effects included puncture marks/bleeding (36%) and red mark/redness (73%). Systemic effects occurred in only 6% of cases. There were no necrotic lesions or ulcers as a consequence of any spider bites (0%; 97.5% CI 0.0−2.2%) and no early allergic reactions or secondary infections. The median duration of clinical effects was shorter than adults, the frequency of severe pain was less than adults and systemic effects were less common in children.
Conclusions:   Paediatric spider bite causes minor effects in the majority of cases, and is unlikely to cause necrotic ulcers, allergic reactions or infection. The severity and duration of effects differ from adults.     

Increasing ambient operating theatre temperature and wrapping in polyethylene improves admission temperature in premature infants

Aim:   To improve admission temperatures of preterm infants ≤31 weeks gestation by increasing the ambient temperature in the operating theatre and wrapping in polyethylene wrap at caesarean section.
Methods:   A review of admission temperature of infants with gestational age ≤31 weeks from January 2000 to July 2002 was performed. Between October 2002 and 2003 the ambient operating theatre temperature was increased to 26–28°C for deliveries ≤27 weeks gestation and to 25°C for deliveries ≥28 weeks gestation. From September 2004 to December 2005 the ambient theatre temperature was increased along with wrapping infants in polyethylene. A clinical audit cycle review of admission temperatures and early morbidity and mortality was undertaken.
Results:   156 premature infants were included, 42 <28 weeks and 114 28–31 weeks gestation. The mean admission temperature in <28 weeks infants prior to intervention was 35.3°C, after increasing ambient theatre temperature 35.9°C, and after increasing ambient temperature and using polyethylene wrap 37.0°C ( P  < 0.0001). For infants 28–31 weeks the mean admission temperatures in the three epochs were 36.3°C, 36.5°C and 36.6°C, respectively ( P  = 0.002). There was no statistically significant difference in: total days of ventilation or oxygen, definite necrotising enterocolitis, intraventricular haemorrhage grade 3 or 4 or survival.
Conclusions:   Increasing the ambient temperature in the operating theatre and wrapping premature infants in polyethylene wrap improves admission temperature. Further studies are required to determine whether these interventions are associated with improved outcome in the premature neonate.     

SMN2 and NAIP gene dosages in Vietnamese patients with spinal muscular atrophy

Background: The SMN1 gene is now recognized as a spinal muscular atrophy (SMA)-causing gene, while SMN2 and NAIP have been characterized as a modifying factor of the clinical severity of SMA. Gene dosage of SMN2 is associated with clinical severity of SMA. But the relationship between gene dosage of NAIP and clinical severity of SMA remains to be clarified, although complete deletion of NAIP is frequent in type I patients.
Methods: To evaluate the contribution of the SMN2 and NAIP gene dosages to SMA, quantitative real-time polymerase chain reaction was used to measure copy numbers of SMN2 and NAIP in 34 Vietnamese SMA patients lacking SMN1 (13 type I, 11 type II and 10 type III patients).
Results: The SMN2 copy number in type I patients was significantly lower than that in type II–III patients, which was compatible with the previous reports. In contrast, 25 out of 34 patients had only zero or one copy of NAIP , while 50 out of 52 controls had two or more copies. For NAIP (+) genotype, six out of 13 type I patients, eight out of 11 type II patients and six out of 10 type III patients carried one NAIP copy.
Conclusions: The SMN2 copy number was related to the clinical severity of SMA among Vietnamese patients. The presence of one NAIP copy, that is, heterozygous NAIP deletion, was common in Vietnamese SMA, regardless of clinical phenotype.     

Seropositivity to celiac antigens in asymptomatic children with type 1 diabetes mellitus: association with weight, height, and bone mineralization

Background:  Screening for celiac disease (CD) in children with diabetes is controversial because no studies have demonstrated metabolic complications in asymptomatic, seropositive subjects or beneficial effects of dietary intervention.
Objective:  We hypothesized that seropositivity to celiac antigens is associated with decreased growth and bone mineralization in asymptomatic diabetic children.
Design/Methods:  Asymptomatic diabetic children were screened for seropositivity to tissue transglutaminase. Villous atrophy was assessed by small bowel biopsy in a subset of seropositive subjects. We compared measures of growth and bone mineralization in 30 seropositive subjects, and 34 matched seronegative controls.
Results:  Relative to seronegative controls, the seropositive subjects had reductions in insulin-like growth factor (IGF) binding protein 3 z scores (p < 0.05) and bone mineral density (BMD) z scores (p = 0.05). Weight, body mass index, IGF-I, and bone mineral apparent density (BMAD) z scores were marginally lower, but height z scores were comparable. Seropositive patients with severe villous atrophy had lower weight (−0.91 SDs), height (−1.1 SDs), BMD (−2.0 SDs), and BMAD (−2.0 SDs) z scores and significant increases in parathyroid hormone (all p < 0.05). Four patients with severe villous atrophy maintained strict gluten restriction for at least 12 months. Gluten restriction increased BMD and BMAD z scores.
Conclusions:  High-titer seropositivity to celiac antigens is associated with reductions in weight and BMD in diabetic children, justifying screening of high-risk patients. Results suggest that biopsy is required to confirm the diagnosis and assess the severity of CD; those with severe villous atrophy are more likely to have growth failure and osteopenia. Gluten restriction may reverse these complications.     

Thyroid autoimmunity in children with features of both type 1 and type 2 diabetes

Aim/hypothesis:  To assess the prevalence of autoimmune thyroid disease (ATD) in insulin-treated youth with clinical features of type 2 diabetes mellitus (T2DM).
Methods:  We evaluated prevalence of thyroid peroxidase (TPO) and thyroglobulin (TGA) antibodies at onset of insulin-treated diabetes and follow-up in 183 White and Black children. Of these, 136 had a body mass index (BMI) <85th percentile with 122 (89%) positive for β-cell autoimmunity [type 1 diabetes mellitus (T1DM)/group I], 25 were overweight (BMI ≥85thpercentile) with or without acanthosis nigricans with β-cell autoimmunity ['double' diabetes (DD)/group II], and 22 were overweight with no conventional β-cell autoantibodies (group III).
Results:  The prevalence of TPO and/or TGA was 39 and 29% (p = 0.19) in White and Black children and 39, 32, and 0% (p = 0.007) in groups I, II, and III, respectively. After a median follow-up of 60 months, 3.7, 4.3, and 0% developed hypothyroidism (increased thyroid-stimulating hormone with or without decreased free T4) in groups I, II, and III, respectively (p = 0.6). In subjects with TPO and/or TGA, hypothyroidism developed in 10 and 14% of groups I and II, respectively (p = 0.7). No child without thyroid antibodies developed hypothyroidism.
Conclusions:  In patients with clinical features of T2DM who have evidence of β-cell autoimmunity (DD), the frequency of thyroid antibodies and ATD is similar to that in classical T1DM. This suggests that TIDM comorbidities may be common in clinical T2DM patients who have β-cell autoimmunity. Despite their obesity, youth with insulin-requiring diabetes should be screened for thyroid and possibly other T1DM-associated autoimmune diseases.     

Effect of single loading dose of intravenous caffeine infusion on superior mesenteric artery blood flow velocities in preterm infants

Aim:   To evaluate the effects of a single loading dose of caffeine base (10 mg/kg) on superior mesenteric artery (SMA) blood flow velocities (BFV).
Methods:   Eighteen preterm infants of gestational age ≤32 weeks gestation were investigated prospectively. SMA BFV before infusion, 1 h, 2 h and 6 h after a single loading dose of caffeine were measured using Doppler ultrasonography.
Results:   The peak systolic velocity in SMA decreased by 18% from baseline at 1 h after caffeine infusion and improved towards the baseline by 6 h after the infusion. The reduction in velocity after caffeine infusion was not statistically significant. No significant changes were observed in heart rate, blood pressure and incidence of necrotising enterocolitis.
Conclusion:   A single 10 mg/kg intravenous loading dose of caffeine does not cause a significant reduction in SMA BFV and therefore does not place the preterm intestine at increased risk of ischemic injury.