Assessment of pressure injury risk in intensive care using the COMHON index: An interrater reliability study.

OBJECTIVES: To assess the interrater reliability of the COMHON (level of COnciousness, Mobility, Haemodynamics, Oxygenation, Nutrition) Index pressure injury risk assessment tool. DESIGN: Interrater reliability was tested. Twenty-five intensive care patients were each assessed by five different nurse-raters from a pool of intensive care nurses who were available on the days of assessment. In total, 25 nurses participated. SETTING: Two general and one cardiovascular surgery intensive care units in Istanbul, Turkey. MAIN OUTCOME MEASURES: Interrater reliability was analysed using intraclass correlations, and standard errors of measurement (SEM) were calculated for sum scores, risk level and item scores. Minimally detectable change (MDC) was also calculated for sum score. Consistency between paired raters was analysed using Pearson's Product Moment Correlation (r) for sum score and Spearman's rho (rs) for ordinal variables. RESULTS: All assessments were completed in ≤5 min. Interrater reliability was very high [ICC (1,1) = 0.998 (95 % CI 0.996 - 0.999)] with a SEM of 0.14 and MDC of 0.39. Consistency between paired raters was strong for sum and item scores and risk levels (coefficients >0.6). All scale items showed correlations of >.3 with the sum score. CONCLUSION: The results demonstrate near-perfect interrater reliability. Further research into the psychometric properties of the COMHON Index and its impact on preventative intervention use is warranted. IMPLICATIONS FOR CLINICAL PRACTICE: Pressure injury risk assessment within intensive care should be setting-specific due to the unique risk factors inherent to the patient population, which are not considered by general pressure injury risk assessment tools. An intensive care-specific pressure injury risk assessment tool was tested and demonstrated high reliability between intensive care nurses. Further research is needed to understand how its use in practice affects preventative intervention implementation and, in turn, how it impacts pressure injury outcomes.

Can Point Shear Wave Elastography Be Used as an Early Indicator of Involvement?: Evaluation of the Pancreas and Liver in Children With Cystic Fibrosis.

OBJECTIVES: The aim of this study was to determine the effect of cystic fibrosis (CF) on pancreas and liver elasticity in young children using point shear wave elastography and to determine the relationship with clinical findings. METHODS: Twenty-two patients with genetically proven CF, who were admitted to our pediatric gastroenterology clinic, and 22 healthy control participants were enrolled in the study. The shear wave velocity (SWV) of the liver and pancreas were measured with point shear wave elastography. RESULTS: The 22 patients with CF included 45.5% girls with a mean age ± SD of 35 ± 35.8 months (range, 5-123 months). The 22 healthy control participants included 41.2% girls with a mean age of 58.9 ± 44.4 months (range, 2-159 months). The mean SWV of the pancreas in the patients with CF (1.06 ± 0.26 m/s) was significantly higher than that of the healthy control participants (0.85 ± 0.23 m/s; P = .01). The mean SWV of the liver in the patients with CF (1.46 ± 0.24 m/s) was significantly higher than that of the healthy control participants (1.12 ± 0.21 m/s; P = .001). The SWV of the pancreas and liver did not show any significant differences depending on ursodeoxycholic acid use, malnutrition status, and the presence of the F508 deletion mutation. CONCLUSIONS: This study showed an increased SWV of the pancreas in children with CF, contrary to the literature. We also found an increased liver SWV even in the absence of CF-related liver disease. Ultrasound elastography may be a useful method of evaluating early changes in the pancreas and liver before the obvious clinical, laboratory, and B-mode ultrasound signs of CF-related involvement.

Novel MTTP Gene Mutation in a Case of Abetalipoproteinemia with Central Hypothyroidism

Abetalipoproteinaemia (ABL) is an autosomal recessive disorder characterized by very low plasma concentrations of total cholesterol and triglyceride (TG). It results from mutations in the gene encoding microsomal TG transfer protein (MTTP). A nine-month-old girl was admitted to hospital because of fever, cough, diarrhea and failure to thrive. She had low cholesterol and TG levels according to her age. The peripheral blood smear revealed acanthocytosis. Thyroid function test showed central hypothyroidism. Cranial magnetic resonance imaging revealed the retardation of myelination and pituitary gland height was 1.7 mm. A homozygous novel mutation [c.506A>T (p.D169V)] was detected in the MTTP gene. Vitamins A, D, E, and K and levothyroxine were started. The coexistence of ABL and central hypothyroidism has not previously been reported. A homozygous novel mutation [c.506A>T (p.D169V)] was detected in the MTTP gene.

Gallbladder Polyps: Rare Lesions in Childhood.

OBJECTIVE: Unlike adults, gallbladder polyps (GPs) are rare in childhood. The aim of this study was to evaluate patients with a GP diagnosis. METHODS: Patients who were diagnosed with GP via ultrasonography from October 2012 to October 2017 were retrospectively evaluated in terms of sociodemographic characteristics and laboratory findings. RESULTS: The study included 19 patients diagnosed with GP and followed up in our department. The patients comprised 14 (73.6%) girls with a mean age of 13.9 ±â€Š4.1 years and a mean follow-up period of 10.2 ±â€Š5.4 months (range, 3-26 months). The most common presenting symptom of the patients (n = 15, 78.9%) for ultrasonography was abdominal pain without biliary symptoms. Location of the polyps was in the corpus in 55% of patients, and either in the fundus (20%) or the neck of the gallbladder (25%). The average diameter of the polyps was 4.5 ±â€Š1.6 mm (range, 2-9 mm). Multiple polyps were observed in 3 patients. No significant change in the number or size of polyps was noted at the end of the follow-up periods. Cholecystectomy was applied to 1 patient who had >5 polyps with a rapid increase in size, and the pathology report was hamartomatous polyp. There was no remarkable change in the clinical or laboratory findings of other patients during the follow-up period. CONCLUSION: In this study, GPs could be seen in young children as young as 16 months of age and ultrasonography is sufficient for follow-up in stable and asymptomatic patients.

Efectos de la edad al momento del diagnóstico y cumplimiento de la dieta sobre los parámetros de crecimiento de los pacientes con celiaquía / Effects of age of diagnosis and dietary compliance on growth parameters of patients with celiac disease

Introducción. El objetivo fue evaluar la relación entre edad al diagnóstico y cumplimiento de dieta sin gluten (DSG) y su efecto sobre el crecimiento de niños celiácos y factores que influenciaron el cumplimiento de la DSG. Población y métodos. Se incluyeron pacientes celíacos con seguimiento en nuestro hospital entre enero 2015 a enero 2017. Se los clasificaron según edad al diagnóstico y cumplimiento de la DSG. Se compararon características antropométricas al diagnóstico y durante el seguimiento. Resultados. Participaron 73 pacientes con edad promedio de 10,4 ± 4,5 años; 35 (47,9%), los pacientes de talla baja al diagnóstico; eran mayores (7,8 ± 4,2 años) que los demás (5,1 ± 4,3 años de edad) (p= 0,005). Al diagnóstico, 33 (45,2%) pacientes tenían ≤6 años y 40 (54,8%) tenían >6 años. Los puntajes Z de estatura y peso a la edad >6 años eran significativamente menores que los diagnosticados a ≤6 años, en el diagnóstico (p= 0,01 y 0,04, respectivamente) como en el último control (p= 0,001 y 0,001, respectivamente). Tuvieron cumplimiento riguroso con DSG en 45 (61,6%) pacientes. Al comparar datos antropométricos , el aumento del índice de masa corporal (IMC) y del puntaje Z de peso en el grupo que cumplió la dieta fue significativamente mayor que en el otro grupo.Conclusiones. Demorar el diagnóstico de celiaquía afectó la estatura y peso. El cumplimiento de la DSG mejoró los parámetros de crecimiento, principalmente, el puntaje Z de peso y el IMC.

Introduction. The objective of this study was to evaluate the relation between age at diagnosis and compliance to gluten free diet (GFD) on growth in children with celiac disease and the factors that influenced compliance to GFD. Population and Methods. Celiac disease (CD) patients with villous atrophy followed in our hospital between January 2015 and January 2017, were included. They were classified according to diagnosis age and GFD compliance. Patients' anthropometric characteristics at diagnosis and follow-up were compared. Results. There were 73 patients with 10.4 ± 4.5 years of average age, 35 (47.9%) patients had a short stature at diagnosis, the ages of patients who had short stature (7.8 ± 4.2 years) were higher than those who did not (5.1 ± 4.3 years) (p= 0.005). At diagnosis, 33 (45.2%) patients were aged ≤6 years, 40 (54.8%) were aged >6 years. The height and weight z-scores of patients who were diagnosed at >6 years of age were significantly lower than those who were diagnosed ≤6 years of age both at diagnosis (p= 0.01 and 0.04) and at last control (p= 0.001 and 0.001), respectively. Forty-five (61.6%) patients were fully compliant with GFD. In comparison of anthropometric data in terms of GFD compliance, the increase in BMI and weightz-score in the fully compliant group was found to be significantly higher when compared with the other group. Conclusions. Delay in CD diagnosis negatively affected both the height and weight and other growth parameters. GFD compliance positively affected the patients' all growth parameters, especially weight and BMI z-score.

Retrospective analysis of children with α-1 antitrypsin deficiency.

BACKGROUND: α-1 Antitrypsin (AAT) deficiency is the most frequently occurring genetic liver disorder. The association among classical α-1 antitrypsin deficiency (AATD), chronic liver disease, and cirrhosis is common in adult patients but rare in children. AIM: To assess the clinical characteristics of children with AATD and to compare symptoms between homozygous and heterozygous children. MATERIALS AND METHODS: The study included 20 children who were found to have mutant Pi alleles. AAT phenotyping was conducted on patients with a low serum AAT level. The exclusion criteria included infectious, anatomic, and metabolic conditions. Symptoms on presentation, physical examination findings, laboratory values, liver biopsy results, and follow-up periods were recorded for each patient. RESULTS: The patients included six (30%) girls and 14 (70%) boys, with a mean age of 6.3±5.1 (1-16) years. The PiZZ phenotype was present in eight (40%) and PiMZ in 12 (60%) patients. The most frequent symptom was elevated liver function test results. Three patients were referred with neonatal cholestasis and one with compensated cirrhosis. Eight patients underwent liver biopsy; all patients except one had periodic acid-Schiff-positive diastase-resistant globules in the hepatocytes. The mean follow-up period was 34±33 (12-101) months. At the end of follow-up, all patients with PiZZ were found to have chronic hepatitis, and one with cirrhosis. On the contrary, two patients with PiMZ were found to have chronic hepatitis. CONCLUSION: Children with classical AATD commonly have chronic liver disease. In heterozygous (PiMZ) children with AATD, enzyme levels can normalize with occasional fluctuations, sometimes causing delayed diagnosis.

Role of Point Shear Wave Elastography in the Determination of the Severity of Fibrosis in Pediatric Liver Diseases With Pathologic Correlations.

OBJECTIVES: Our aims in this study were as follows: (1) to determine the cutoff value that can distinguish between advanced liver fibrosis and normal liver tissue for two different elastographic techniques; (2) to determine the cutoff value that can distinguish mild liver fibrosis from normal liver tissue for the techniques; and (3) to assess tissue stiffness in nonalcoholic fatty liver disease (NAFLD). METHODS: Seventy-five patients assessed for liver biopsy on the same day were evaluated by point shear wave elastography. Thirty-one healthy children and 11 children with NAFLD were also evaluated. A 9L4 transducer with Virtual Touch quantification (VTQ) and Virtual Touch imaging and quantification (VTIQ) modes (Siemens Medical Solutions, Mountain View, CA) was used for quantification. RESULTS: The shear wave speed of the patients with NAFLD was higher than that of the control group. The only predictive factor for VTQ and VTIQ was the histologic fibrosis score (model-adjusted R2 = 0.56 for VTQ and 0.75 for VTIQ). Shear wave speed cutoffs were 1.67 m/s for VTQ and 1.56 m/s for VTIQ in detecting fibrosis or inflammation and 2.09 m/s for VTQ and 2.17 m/s for VTIQ in discriminating children with low and high histologic liver fibrosis scores. CONCLUSIONS: The VTQ and VTIQ values reveal high-grade histopathologic fibrosis and have high success rates when distinguishing high- from low-grade fibrosis. However, they have limited success rates when differentiating low-grade fibrosis from normal liver tissue.

Vitamin D Deficiency and a Blunted Parathyroid Hormone Response in Children with Attention-Deficit/Hyperactivity Disorder.

BACKGROUND: Attention-deficit/hyperactivity disorder (ADHD) is the most frequently diagnosed neuropsychiatric disorder of childhood. The etiopathogenesis of ADHD has not been fully defined. Recent evidence has suggested a pathophysiological role of vitamin D deficiency in ADHD. In this study, we evaluated the serum levels of 25-hydroxy vitamin D (25(OH)D), parathyroid hormone (PTH), calcium (Ca), phosphate (P), and alkaline phosphatase (ALP) in children with ADHD. METHODS: The study group consisted of 105 children diagnosed with ADHD according to DSM-IV-TR criteria. A control group, matched for age and gender, was composed of 95 healthy children. Venous blood samples were collected, and 25(OH)D, PTH, Ca, P, and ALP levels were measured. RESULTS: The mean serum 25(OH)D, Ca, and P levels of the children with ADHD were significantly lower than those of the healthy controls. There were no significant differences between the groups regarding PTH and ALP. Serum PTH levels were found to be normal, but vitamin D deficiency, hypocalcemia, and hypophosphatemia were observed in children with ADHD. There was no correlation between serum PTH and Ca levels in children with ADHD, whereas, there was a negative correlation between serum PTH and Ca levels in healthy controls. There was no correlation between serum 25(OH)D and PTH levels in children with ADHD, whereas, there was a negative correlation between serum 25(OH)D and PTH levels in healthy controls. There were no significant differences in all parameters' levels among the subtypes of ADHD. CONCLUSIONS: The findings suggest that ADHD is associated with vitamin D deficiency, blunted PTH response, and impaired Ca homeostasis in children.

Lactose and Fructose Intolerance in Turkish Children with Chronic Abdominal Pain.

OBJECTIVE: To investigate the prevalence of lactose and fructose intolerance in children with chronic abdominal pain. METHODS: Hydrogen breath tests were done to detect lactose and fructose malabsorption in 86 children with chronic abdominal pain (44 irritable bowel syndrome, 24 functional abdominal pain and 17 functional abdominal pain syndrome as per Rome III criteria) presenting to a Pediatric Gastroentreology department. RESULTS: 14 (16.3%) of patients were diagnosed with lactose intolerance and 11 (12.8%) with fructose intolerance. CONCLUSION: Lactose and fructose intolerance in children can lead to chronic abdominal pain and symptoms improve with dietary modifications.

Different techniques for biliary diversion in progressive familial intrahepatic cholestasis.

BACKGROUND: Progressive familial intrahepatic cholestasis (PFIC) is a cholestatic liver disease of childhood. Pruritus resulting from increased bile salts in serum might not respond to medical treatment, and internal or external biliary drainage methods have been described. In this study, we aimed to evaluate different internal drainage techniques in patients with PFIC. PATIENTS AND METHODS: Between 2009 and 2014, seven children (4 male, 3 female, 3months-5years old), (median 2years of age) with PFIC were evaluated. The patients were reviewed according to age, gender, complaints, surgical technique, laboratory findings and outcome. In each two patients, cholecystoileocolonic anastomosis, cholecystojejunocolonic anastomosis and cholecystocolostomy were performed. Cholecysto-appendico-colonic anastomosis was the technique used in one patient. RESULTS: Jaundice and excessive pruritus were the main complaints. One of the patients with cholecystoileocolonic anastomosis died of comorbid pathologies (cirrhosis, adhesive obstruction and severe sepsis). Temporary rectal bleeding was observed in all the patients postoperatively. Regardless of the surgical technique, pruritus was dramatically decreased in all the patients in the postoperative period. CONCLUSION: Regardless of the technique, internal biliary diversion methods are beneficial for the relief of pruritus in PFIC patients. Selection of the surgical method might vary depending on the surgeon's preference and the surgical anatomy of the gastrointestinal system of the patient.

Low 25-hydroxyvitamin D level is associated with insulin sensitivity in obese adolescents with non-alcoholic fatty liver disease.

AIM: The aim of this study was to investigate the relationships between 25-hydroxy-vitamin D (25(OH)D) and insulin resistance in obese adolescents with non-alcoholic fatty liver disease (NAFLD). PATIENTS AND METHODS: Eighty-seven obese adolescents (45 girls and 42 boys, mean age: 12.7 ± 1.3 years, mean body mass index standard deviation score (BMI-SDS): 2.1 ± 0.3) and 30 lean subjects (15 girls and 15 boys, mean age: 12.3 ± 1.45 years, mean BMI-SDS: 0.5 ± 0.7) were enrolled for the study. The obese subjects were divided into two subgroups based on the presence or absence of fatty liver with high transaminases (NAFLD group and non-NAFLD group). Fasting blood samples were assayed for 25(OH)D, transaminases, glucose, and insulin levels. Insulin resistance was calculated by the homeostasis model assessment (HOMA-IR). RESULTS: 25(OH)D measurements were decreased in both obese groups (NAFLD and non-NAFLD) in comparison with the lean group (29.5 ± 18.4 vs. 41.0 ± 17.9 vs. 48.1 ± 22.2 ng/mL). However; the NAFLD group had significantly lower measurements of 25(OH)D than the non-NAFLD group (p < 0.001) and lean group (p < 0.001). 25(OH)D was negatively correlated with HOMA-IR (r = 0.158, p = 0.01) and with alanine aminotransferase (r = 0.794, p = 0.03) in the NAFLD obese group. There was no significant associations between fasting insulin, BMI-SDS and 25(OH)D in obese groups. CONCLUSION: We suggest that low 25(OH)D occurs commonly in obese adolescents with NAFLD and we demonstrated an association between insufficient vitamin D status and low insulin sensitivity in obese adolescents with NAFLD.