An ultrasound scan of the abdomen was done. It showed a mass with alternating rings of hyperand hypo-echogenicity in the right upper abdomen on transverse scan, i.e., donut sign was positive (Figure 2). Pseudo-kidney appearance was seen on longitudinal scan (Figure 3). Thin rim ascites was found at hepatorenal area (Figure 4). Vascularity was shown within the intussusceptum (Figure 5). A diagnosis of intussusception was made and the patient was sent for pneumatic reduction immediately after ultrasound examination. Pre-pneumatic reduction image revealed prominent bowel loops in left abdomen and absence of bowel gas in right abdomen (Figure 6). Pneumatic reduction was performed with pressure around 80 mmHg. Under fluoroscopy, the soft tissue mass was seen to move from right upper abdomen to right lower abdomen. Its size decreased and then disappeared with reflux of air into small bowel loops
Poster: RANZCR-AOCR 2012 / R-0100 / Different Faces of Uncommon Childhood Encephalitis and Encephalopathy by: K. S. Tse, T. Wong, L. Chiu, T. Tsang, T. Fan
Mucopolysaccharidosis VI (Maroteaux-Lamy syndrome) is a very rare inherited lysosomal storage disease. We evaluated the efficacy and safety of weekly infusions of recombinant human arylsulfatase B as enzyme replacement therapy for two patients in whom this condition was advanced. The primary outcome variables were the distance walked in a 6-minute walk test, forced vital capacity, and ejection fraction. The secondary outcome variables were the number of stairs climbed in a 3-minute stair climbing test, joint mobility, urinary glycosaminoglycan excretion, auto-continuous positive airway pressure study and liver size. After 24 weeks of treatment, patient A walked 40 m (36%) and patient B walked 66 m (58%) more in the walk test than at baseline. After 48 weeks, in patient A the corresponding improvements were 142 m (129%) in the walk test and 33 stairs (60%) in the 3-minute stair climbing test, and in patient B the respective improvements were 198 m (174%) and 77 stairs (140%). There was a significant decline in urinary glycosaminoglycan excretion and improvement in range of motion of joints in both patients. The auto-continuous positive airway pressure study revealed improvements in patient A, while other efficacy variables remained static. There were no drug-related adverse events or allergic reactions reported during and after the infusions of recombinant human arylsulfatase B. Recombinant human arylsulfatase B significantly improves endurance and reduces urinary glycosaminoglycan excretion. The drug is generally safe and well tolerated.
Purpose: To review the association of occult spinal dysraphism in children with anorectal malformation. Methods: Fifty-eight children with anorectal malformation were reviewed. Twenty patients had high anomalies, whereas 6 had intermediate and 32 had low anomalies. Magnetic resonance imaging (MRI) of lumbosacral spine was performed on 10 children, because of abnormal preliminary ultrasonographic findings or persistent symptoms after surgery. Results: Four children had positive findings from the MRI. Tethered cord and intraspinal lipoma are the most common abnormalities. Lumbosacral spine X-ray was abnormal in only one patient. All patients had urodynamic assessment and abnormal cystometric finding was evident in two patients. Conclusions: Occult spinal dysraphism is common in children with anorectal malformation. A normal lumbosacral spine X-ray does not exclude the diagnosis. MRI of spine should be performed in suspicious cases. Urodynamic assessment is necessary to identify associated neurovesical dysfunction.
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