Urinary incontinence is one of the most common symptoms in children and adolescents. Due to numerous causes, a thorough examination is necessary. We present a case of a 6-year-old boy with diurnal enuresis, who was admitted to our Department for further treatment after the procedure of bilateral ureteroneocystostomy due to suspected bilateral ureter stenosis. With the persistence of the incontinence, our examination revealed a posterior urethral valve which was incised and the symptoms subsided.
The COVID-19 pandemic caused by coronavirus SARS-CoV-2 is currently a main public health problem worldwide. The clinical symptoms relate primarily to the respiratory system but may also involve multiple organs. The course of COVID-19 in children is usually mild, but in some cases may cause late complications, particularly the pediatric inflammatory multisystem syndrome (PIMS-TS). One of its symptoms may be acute kidney injury. We present a 10-year-old boy who developed nonspecific symptoms a few weeks after mild COVID-19, including weakness, weight loss, and polyuria. Clinical evaluation revealed acute renal failure secondary to acute tubulointerstitial nephritis (ATIN). Treatment with glucocorticoids resulted in rapid clinical and laboratory improvement. We hypothesize that the development of ATIN could be causally related to COVID-19 in an immune pathomechanism similar to PIMS-TS. The case provides new insights into possible complications of SARS-CoV-2 infection and indicates the need for renal follow-up after COVID-19.
Pierson syndrome (PIERSS) is a rare autosomal recessive disorder characterized by the combination of congenital nephrotic syndrome (CNS) and extrarenal symptoms including ocular malformations and neurodevelopmental deficits. PIERSS is caused by biallelic pathogenic variants in the LAMB2 gene leading to the defects of β2-laminin, the protein mainly expressed in the glomerular basement membrane, ocular structures, and neuromuscular junctions. Severe complications of PIERSS lead to the fatal outcome in early childhood in majority of the cases. We report a case of 5-year-old girl with severe phenotype of PIERSS caused by biallelic functional null variants of the LAMB2 gene. Due to consequences of CNS, the patient required bilateral nephrectomy and peritoneal dialysis since early infancy. The course was additionally complicated by tubulopathy, life-threatening infections, severe hypertension, erythropoietin-resistant anemia, generalized muscular hypotonia, neurogenic bladder, profound neurodevelopmental delay, epilepsy, gastrointestinal problems, secondary hypothyroidism, and necessity of repeated ocular surgery due to microcoria, cataract, and nystagmus. Due to multidisciplinary efforts, at the age of 4 years, the kidney transplantation was possible. Currently, the renal graft has an excellent function; however, the girl presents severe neurodevelopmental delay. The report presents a unique long-term follow-up of severe PIERSS with a few new phenotypical findings. It highlights the clinical problems and challenges in management of this rare condition.
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