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Introduction: Prior analysis of children with grade 3 and 4 congenital hydronephrosis demonstrated that renal medullary pyramidal thickness (PT) is predictive of subsequent pyeloplasty (area under the curve [AUC] = 0.78). The objective of this study was to further analyze the utility of sonographic measurements including PT, anteroposterior pelvic diameter (APD), and renal length with an expansion of the number of infants with hydronephrotic kidneys including grades 2, 3, and 4 hydronephrosis.

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Anterior external arcuate fibers (AEAF) are efferents of the arcuate nuclei, which are located on the ventral surface of pyramids. Several types of fibre bundles superficial to the pyramids have been described in early and mid 20th century. Recently, few of these have been studied in detail.

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Background: Opalski syndrome is a rare variant of Wallenberg syndrome (lateral medullary syndrome) that demonstrates concomitant ipsilateral hemiplegia due to infarctions within the lateral medulla and the cervical spinal cord, which also extend to the post-pyramidal decussation to affect the corticospinal tract.

Case Presentation: A 56-year-old man initially presented with a unilateral headache with right cervical pain. Consequently, he developed symptoms that indicate Wallenberg syndrome: vertigo, dysphonia, dysarthria, right limb ataxia with a tendency to fall, and ptosis, in addition to ipsilateral hemiparesis.

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Article Synopsis
  • The corticospinal tract (CST) is crucial for voluntary movement in vertebrates, and Netrin-1 serves as a key guidance molecule for axons crossing the midline during embryonic development.
  • Research using conditional knockout (CKO) mice revealed that the lack of Netrin-1 resulted in CST axons failing to cross over correctly and instead descending on the same side of the spinal cord.
  • These findings suggest a significant role of Netrin-1 in CST formation and provide new insights into its involvement in congenital mirror movements (CMM) due to abnormal CST trajectories.
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A case of diffuse kidney hyperechogenicity in early childhood associated with biallelic PKHD1 variants.

Pediatr Nephrol

September 2024

Departamento de Pediatría, Clínica Alemana de Santiago, Santiago, Chile.

Article Synopsis
  • * A 3-month-old girl with suspected milk protein allergy showed signs of medullary NC during an ultrasound, and follow-up imaging at 18 months indicated progression of the condition along with genetic analysis revealing PKHD1 variants.
  • * While kidney function remained normal until age 7, the increasing incidence of NC in newborns emphasizes the need for early diagnosis and careful clinical monitoring to prevent complications such as kidney failure.
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