Cranial ultrasound performance assessment tool: development and validation.

AIM: A standard assessment tool for direct evaluation of procedural skills to ensure proficiency of trainees is necessary for cranial ultrasound (US) in clinical practice. This study created and validated an assessment tool for cranial US performance by radiologists. MATERIAL AND METHODS: An initial evaluation tool for cranial US using criteria was developed based on existing literature. The assessment form was modified using a three-round Delphi process by an expert panel, conducted between January 2021 and April 2021. Rubric scales for grading were added once consensus regarding generated items was reached. Experts confirmed the final assessment tool using a rubric scale. Two raters evaluated cranial US performance of 27 residents in video clips using the tool. Reliability and percent agreement were assessed. RESULTS: Seventeen pediatric radiologists working in different settings participated in the expert panel. The content validation of the proposed evaluation tool was enabled by expert pediatric radiologists. Following three rounds of the Delphi process, the initial 14-item assessment form became a final 15-item form. A three-part rubric scale was used in the final form (preparation, US machine operation, and cranial US performance). Interrater reliability was evaluated with Cohen's Kappa. The Kappa value and percent interrater agreement for most items was moderate to almost perfect (0.42-0.93 and 77.8-100%, respectively). The Cronbach's alpha values for both raters were 0.856 and 0.891. CONCLUSIONS: This study produced the first validated cranial US assessment tool using a modified Delphi method. The final assessment form is a simple and reliable tool.

Accuracy of chest radiography for evaluating significantly abnormal pulmonary vascularity in children with congenital heart disease.

The purpose is to determine the accuracy of chest radiography for evaluating significantly abnormal pulmonary vascularity in children with congenital heart disease. This retrospective study included 120 children. Forty pediatric congenital heart disease patients with a ratio of pulmonary to systemic blood flow (Qp:Qs) lower than 0.8 by cardiac catheterization were enrolled as the decreased pulmonary vascularity group. Another forty pediatric congenital heart disease patients with a Qp:Qs higher than 1.5 were enrolled as the increased pulmonary vascularity group. Forty pediatric patients who had no cardiopulmonary problems were enrolled as the normal control group. All chest radiographs were reviewed by three readers. The results were compared to cardiac catheterization as a gold standard. Linear weighted kappa test was used to determine intra- and inter-observer agreements. The accuracy, specificity, positive predictive value, and negative predictive value of chest radiography to characterize pulmonary vascularity patterns in the three groups were moderate to high, falling between 73 and 92 %, 61 and 96 %, 71 and 94 %, and 71 and 98 %, respectively. The sensitivity of chest radiography to interpret decreased pulmonary vascularity patterns was low (24-68 %), whereas the sensitivity to interpret normal and increased pulmonary vascularity patterns were high (84-94 %). The inter-observer agreement was moderate to good (k = 0.53-0.67). The intra-observer reliability was good (k = 0.71-0.79). Pediatric chest radiography exhibits good accuracy and reproducibility to identify significantly abnormal pulmonary vascularity in children with congenital heart disease. However, the sensitivity to detect decreased pulmonary vascularity pattern is low.

Two siblings with a novel nonsense mutation, p.R50X, in the vitamin D receptor gene.

Hereditary vitamin D-resistant rickets (HVDRR), an autosomal recessive disorder caused by inactivating mutations in the vitamin D receptor (VDR) gene. We identified two affected children from the same family, one at the age of 10 years and 9 months and the other at 9 months old. Mutation analysis by PCR-sequencing the entire coding region of the VDR gene revealed a homozygous C to T transition in exon 2 of the VDR gene (c.148C>T) resulting in a stop codon at amino acid position 50 (p.R50X) in the proband and his younger sister. The p.R50X has never been previously described. Both asymptomatic parents were heterozygous for the mutation. In addition to most of the clinical features of HVDRR including total alopecia, symptoms of hypocalcemia at a later onset and normophosphatemia, rarely found in HVDRR were present in the proband. This study also emphasizes an important role of genetic testing for early diagnosis and genetic counseling.

Effect of a whey-predominant starter formula containing LCPUFAs and oligosaccharides (FOS/GOS) on gastrointestinal comfort in infants.

Development of new infant formulas aims to replicate the benefits of breast milk. One benefit of breast milk over infant formulas is greater gastrointestinal comfort. We compared indicators of gastrointestinal comfort in infants fed a whey-predominant formula containing long-chain polyunsaturated fatty acids, galacto-oligo-saccharides and fructo-oligosaccharides, and infants fed a control casein-predominant formula without additional ingredients. The single-centre, prospective, double-blind, controlled trial randomly assigned healthy, full-term infants (n=144) to receive exclusively either experimental or control formula from 30 days to 4 months of age. A group of exclusively breast-fed infants served as reference (n=80). At 1, 2, 3, and 4 months, infants' growth parameters were measured and their health assessed. Parents recorded frequency and physical characteristics of infants' stool, frequency of regurgitation, vomiting, crying and colic. At 2-months, gastric emptying (ultrasound) and intestinal transit time (H2 breath test) were measured, and stool samples collected for bacterial analysis. Compared to the control (n=69), fewer of the experimental group (n=67) had hard stools (0.7 vs 7.5%, p<0.001) and more had soft stools (90.8 vs 82.3%, p<0.05). Also compared to the control, the experimental group's stool microbiota composition (mean % bifidobacteria: 78.1 (experimental, n=17), 63.7 (control, n=16), 74.3 (breast-fed, n=20), gastric transit times (59.6 (experimental, n=53), 61.4 (control, n=62), 55.9 (breast-fed, n=67) minutes) and intestinal transit times (data not shown) were closer to that of the breast-fed group. Growth parameter values were similar for all groups. The data suggest that, in infants, the prebiotic-containing whey-based formula provides superior gastrointestinal comfort than a control formula.

Fibrolamellar carcinoma presenting as a pancreatic mass: case report and review of the literature.

Fibrolamellar carcinoma is a subtype of hepatocellular carcinoma with distinct clinicopathologic features including presentation at a younger age. Although early studies suggested that fibrolamellar carcinoma had a better prognosis than conventional hepatocellular carcinoma, most later studies have found no difference. Patients often have lymph node metastases at presentation in addition to the hepatic primary. We describe an unusual case in a Thai boy who presented with a pancreatic mass that was clinically suspected to be a primary pancreatic tumor, but on biopsy was found to be metastatic fibrolamellar carcinoma. To our knowledge, this manner of presentation has not been previously reported for fibrolamellar carcinoma, nor has metastatic spread to the pancreas.

Gastric volvulus caused by percutaneous endoscopic gastrostomy: a case report.

Percutaneous endoscopic gastrostomy is commonly performed for enteric feeding in children with neurologic impairment because of its low rates of complication. We report a case of gastric volvulus, an unusual complication, after percutaneous endoscopic gastrostomy procedure in our institution.

Capacity of the unused bladder in end stage renal disease.

OBJECTIVES: To evaluate the bladder capacity in patients diagnosed with end stage renal disease (ESRD). MATERIAL AND METHOD: A six-month prospective study was conducted at the outpatient X-ray unit, Department of Radiology, King Chulalongkorn Memorial Hospital from July 2002 to January 2003. Datas from adult patients sent to the unit for voiding cystourethrography (VCUG) were collected. Bladder capacity was obtained by calculating the amount of contrast material introduced into the urinary bladder in each VCUG. The urinary bladder was assumed to be at the maximum capacity when the patient had a strong desire to void during contrast instillation. Patients with voiding dysfunction or neurogenic bladder were excluded from the present study. RESULTS: There were 11 patients with ESRD (male=7, female=4, mean age=41 years) and 7 patients without ESRD (male=1, female=6, mean age=41 years). The maximum bladder capacity was 400 ml for the ESRD patients and 500 ml for the non-ESRD patients. The smallest bladder capacity was 100 ml for the ESRD patients and 250 ml for the non-ESRD patients. The mean bladder capacity was 254.5 ml (+/-SD) for the patients with ESRD and was 364.28 ml for the patients without ESRD. The P-value was 0.043. In conclusion, the mean bladder capcity of the patients with ESRD was statistically smaller than that of patients without ESRD.

Extravasation of the contrast in the unused urinary bladder.

Ruptured bladder or extravasation from the bladder is almost always associated with trauma. Spontaneous extravasation is extremely rare with only a few reported cases. All those reported extravasations occurred in the patients diagnosed with end stage renal disease (ESRD) and were self limiting conditions. During the one-year period of 2001, voiding cystourethrography (VCUG) of 115 patients were performed in our institution with various indications. The authors found 3 cases with extravasation of contrast from the bladder during VCUG. All extravasations were occurred in ESRD patients and the VCUG was performed as part of the routine investigation prior to renal transplantation. The presented cases shared a similar observation of 1) no traumatic catheterization, 2) only minimal and self limiting extravasation and 3) no immediate or delayed symptom associated with extravasation. The cause and mechanism of leakage remain uncertain. Hypertonicity and mucosal changes within the unused bladder were purposed as probably the two main causes of extravasation.

Hysterosalpingographic accuracy of peritubal adhesion.

The purpose of this study was to determine diagnostic accuracy of hysterosalpingography by using different diagnostic criteria in peritubal adhesion diagnosis. The authors retrospectively reviewed cases in which both hysterosalpingography and laparoscopy were performed. Fifty-nine of 84 cases had laparoscopy proved peritubal adhesion. Five hysterosalpingographic signs (convoluted tube, vertical tube, ampullary dilatation, peritubal halo and loculation of the spillage of contrast material) defined by Karasick and Goldfarb were used to diagnose peritubal adhesion. All cases were analyzed by two different diagnostic criteria: first diagnostic criterion, presence of one or more signs means abnormal; second diagnostic criterion, presence of two or more signs means abnormal. Peritubal adhesion was diagnosed in 70 of 84 cases by using the first diagnostic criterion, 53 of 84 cases by using the second diagnostic criterion. The first diagnostic criterion displayed 94.9 per cent sensitivity, 44 per cent specificity, 80 per cent positive predictive value, 79.76 per cent accuracy and the likelihood ratio of 1.69. The second diagnostic criterion showed 74.6 per cent sensitivity, 64 per cent specificity, 83 per cent positive predictive value, 71.43 per cent accuracy and the likelihood ratio of 2.07. The authors conclude that using the 2nd diagnostic criterion is more appropriate than using the 1st diagnostic criterion in diagnosing peritubal adhesion.