Dana Al-Nabhani

Posterior Reversible Encephalopathy Syndrome in Two Omani Children with Underlying Renal Diseases

Posterior reversible encephalopathy syndrome (PRES) is a neurological condition with a combination of clinical and radiological features. Clinical symptoms include headaches, confusion, seizures, disturbed vision or an altered level of consciousness. Classic magnetic resonance imaging (MRI) findings indicate subcortical and cortical oedema, affecting mainly the posterior cerebral region. We report two paediatric cases of PRES with underlying renal diseases presenting at the Sultan Qaboos University Hospital in Muscat, Oman, in April 2010 and August 2011. The first case was an 11-year-old girl diagnosed with systemic lupus erythematosus and the second was a six-and-a-half-year-old boy on peritoneal dialysis due to multi-drug-resistant nephrotic syndrome. Both patients were hypertensive and treated with blood pressure control medications. No residual neurological dysfunction was noted in the patients at a one-year follow-up and at discharge, respectively. The role of hypertension in paediatric PRES cases, among other important risk factors, is emphasised. Additionally, MRI is an important diagnostic and prognostic tool. Prompt diagnosis and aggressive management is fundamental to preventing permanent neurological damage.

Amlodipine: The double edged sword

Amlodipine, a calcium channel blocker became one of the most popular antihypertensive medications used in the paediatric population. This is mainly due to its long half‐life making it easy to use as a single daily dose. Amlodipine is associated with common side effects like flushing, headache and dizziness. The incidence of amlodipine‐related oedema was noted to be lower in the paediatric population compared to adults. We emphasise the importance of monitoring the development of oedema in patients treated with amlodipine.

Nocardia asteroides peritoneal dialysis-related peritonitis: First case in pediatrics, treated with protracted linezolid

Nocardia asteroides is a rare pathogen in peritoneal dialysis-related peritonitis. We report on a 13-year-old female with Nocardia asteroides peritonitis complicated by an intra-abdominal abscess. Linezolid was administered intravenously for 3 months and followed by oral therapy for an additional 5 months with close monitoring for adverse effects. The patient was discharged after 3 months of hospitalization on hemodialysis. The diagnosis and management of such cases can be problematic due to the slow growth and difficulty of identifying Nocardia species. The optimal duration of treatment for Nocardia peritonitis is not known. Linezolid can be used for prolonged periods in cases of trimethoprim/sulfamethoxazole-resistant cases with close monitoring for adverse effects.

Bilateral hydroureters and hydronephrosis in a neonate

A newborn boy was diagnosed antenatally with bilateral hydronephrosis. Postnatal renal ultrasound scan (USS) measured a renal pelvic anteroposterior diameter (APD) of 12 mm on the left side and 7 mm on the right side. The baby had good urine stream. Parents missed the repeat USS at the age of 1 week. An ultrasound done at 4 weeks revealed progressive hydronephrosis, bilateral hydroureters, with increased renal echogenicity. Normal bladder wall thickness was noted but two intravesical lesions were seen (figures 1 and 2). The APD was 13.5 and 11 mm on the left and right side, respectively. Figure 1 Renal ultrasound scan of (A) left kidney (LT) and (B) right kidney (RT) showing bilateral hydronephrosis (white arrows) and hydroureters (red arrow). Increased renal echogenicity is not shown in the figure. Figure 2 Showing normal bladder wall thickness but two intravesical lesions were seen (white arrows). Question How would you describe the intravesical lesions in figure 2? Bilateral ureteroceles Bilateral vesicoureteral reflux (VUR) Bilateral pelvi-ureteric junction obstruction Posterior urethral valves (PUVs) Which complication(s) may you expect in such cases? Urinary tract infection (UTI) Obstructive voiding symptoms Failure to thrive Ureteral calculus All of the above How would you treat this problem? Endoscopic puncture Deflux surgery Pyeloplasty Vesicostomy Answers are on page ▪▪▪

Quiz Page November 2015

CLINICAL PRESENTATION A 9-year-old boy known to have tuberous sclerosis complex (TSC) presented with gross hematuria. Three years earlier, a screening kidney ultrasound showed a left simple renal cyst and bilateral increased echogenicity of the corticomedullary regions. The child had no history of trauma, bleeding tendency, or other urinary symptoms. He was not taking any medications. His medical history is notable for retinal phakomas, cardiac rhabdomyoma, subependymal giant cell astrocytomas, and seizure disorder, as well as learning disabilities. Despite a family history of TSC in the mother and an elder sibling, no family member had kidney disease. On examination, the patient was afebrile with normal pulse and blood pressure. Abdominal examination findings were unremarkable, with no organomegaly. Laboratory investigation results were within the normal ranges, with serum creatinine level of 0.38 mg/dL (corresponding to estimated glomerular filtration rate of 135 mL/min/1.73 m as calculated by the Schwartz equation). Hemoglobin level was 11.8 g/dL; platelet count, 321 310/mL, and international normalized ratio, 1. Urinalysis showed albumin (11) and 4 squamous epithelial cells per high-power field (HPF), 12 white blood cells/HPF, and .60 red blood cells/HPF. Abdominal ultrasound and computed tomographic scan were obtained (Fig 1), and a left total nephrectomy was performed (Fig 2).

Hematuria in a Child With Tuberous Sclerosis

CLINICAL PRESENTATION A 9-year-old boy known to have tuberous sclerosis complex (TSC) presented with gross hematuria. Three years earlier, a screening kidney ultrasound showed a left simple renal cyst and bilateral increased echogenicity of the corticomedullary regions. The child had no history of trauma, bleeding tendency, or other urinary symptoms. He was not taking any medications. His medical history is notable for retinal phakomas, cardiac rhabdomyoma, subependymal giant cell astrocytomas, and seizure disorder, as well as learning disabilities. Despite a family history of TSC in the mother and an elder sibling, no family member had kidney disease. On examination, the patient was afebrile with normal pulse and blood pressure. Abdominal examination findings were unremarkable, with no organomegaly. Laboratory investigation results were within the normal ranges, with serum creatinine level of 0.38 mg/dL (corresponding to estimated glomerular filtration rate of 135 mL/min/1.73 m as calculated by the Schwartz equation). Hemoglobin level was 11.8 g/dL; platelet count, 321 310/mL, and international normalized ratio, 1. Urinalysis showed albumin (11) and 4 squamous epithelial cells per high-power field (HPF), 12 white blood cells/HPF, and .60 red blood cells/HPF. Abdominal ultrasound and computed tomographic scan were obtained (Fig 1), and a left total nephrectomy was performed (Fig 2).

Quiz page November 2015: hematuria in a child with tuberous sclerosis.

CLINICAL PRESENTATION A 9-year-old boy known to have tuberous sclerosis complex (TSC) presented with gross hematuria. Three years earlier, a screening kidney ultrasound showed a left simple renal cyst and bilateral increased echogenicity of the corticomedullary regions. The child had no history of trauma, bleeding tendency, or other urinary symptoms. He was not taking any medications. His medical history is notable for retinal phakomas, cardiac rhabdomyoma, subependymal giant cell astrocytomas, and seizure disorder, as well as learning disabilities. Despite a family history of TSC in the mother and an elder sibling, no family member had kidney disease. On examination, the patient was afebrile with normal pulse and blood pressure. Abdominal examination findings were unremarkable, with no organomegaly. Laboratory investigation results were within the normal ranges, with serum creatinine level of 0.38 mg/dL (corresponding to estimated glomerular filtration rate of 135 mL/min/1.73 m as calculated by the Schwartz equation). Hemoglobin level was 11.8 g/dL; platelet count, 321 310/mL, and international normalized ratio, 1. Urinalysis showed albumin (11) and 4 squamous epithelial cells per high-power field (HPF), 12 white blood cells/HPF, and .60 red blood cells/HPF. Abdominal ultrasound and computed tomographic scan were obtained (Fig 1), and a left total nephrectomy was performed (Fig 2).