Abstract Introduction: Lupus nephritis (LN) in childhood usually presents after the age of 10 years and is more common in female children. Despite the availability of effective immunosuppressive therapies, treatment response in childhood‑onset LN remains suboptimal, with 50% to 78% patients in full remission at 24 months. Improvement in survivals have also plateaued in past decades, and up to 9% and 14% of chil...
Abstract Introduction: Multicystic dysplastic kidney (MCDK) is a severe form of cystic renal dysplasia that typically exhibits a favorable natural history. Though complications are low, systematic follow‑up is needed. Methods: Retrospective analysis covering children diagnosed with MCDK, undergoing follow‑up in a tertiary pediatric nephrology unit, from January/2011‑December/2023. Variables assessed included de...
Abstract Pediatric cystic kidney diseases include a diversity of hereditary or non‑hereditary conditions, whose phenotypic presentation can vary from asymptomatic to end‑stage renal disease. We report a case of a male with a prenatal diagnosis of pyelectasis. Throughout the first two years of life, serial renal ultrasound showed regression of pyelectasis, but increased renal echogenicity, first identified at th...
ABSTRACT Hypomagnesemia type I is a rare autosomal recessive disorder characterized by severe hypomagnesemia, often accompanied by hypocalcemia. This disease is caused by mutations in the TRPM6 gene (which encodes the respective channel), leading to reduced intestinal absorption of magnesium and increased renal excretion due to a defect in reabsorption in the distal convoluted tubule. It usually manifests itsel...
ABSTRACT Membranoproliferative glomerulonephritis is a rare chronic glomerulonephritis in pediatric age. It may be a primary disorder or secondary to other chronic diseases. Diagnose of membranoproliferative glomerulonephritis is based on specific kidney biopsy findings. The prognostic factors, the outcome and the most suitable treatment regimen are incompletely known in children. We report a case of a 3-year-o...
Abstract Background Psoriasis is a chronic immune-mediated disorder that primarily affects the skin in both adults and children but can also have systemic involvement, particularly with arthritis and kidney injury. IgA nephropathy is the most frequent kidney disorder associated with psoriasis. Approximately one third of all cases of psoriasis begin in childhood, but association between psoriasis and renal disor...
Resumo A cultura digital com as suas interfaces limitadas leva-nos a olhar fixamente, durante horas, para um ecrã; sentados, imóveis, batemos num teclado ou clicamos repetidamente num rato. O trauma físico resultante deste constrangimento é alimentado por ciclos tecnológicos pontuados pela invenção, desenvolvimento e obsolescência de novos gadgets. Estas são algumas das preocupações de que parte o projecto de I...
Introduction: Alport syndrome is a glomerular genetic disease progressing to chronic renal failure associated with deafness and ocular changes. Clinical presentation is usually in the first decade of life with microscopic haematuria and/or persistent proteinuria without hypertension or renal dysfunction. Case Report: Male, 4.5 years old, with an acute nephritic syndrome characterized by macroscopic haematuria, ...
Background: Prednisolone has been the first-line therapy in the treatment of idiopathic nephrotic syndrome in children. Recurrences are common, and the need for repeated high doses of prednisolone alone or in combination with other immunosuppressants may induce significant side effects. Deflazacort has been used in recent years as an alternative, but there are not many studies on its effectiveness in maintenanc...
Background: Most renal disorders affecting children (apart from simple urinary infections) are chronic and the majority of these children require additional and specific nephrological care throughout adulthood, with undeniable physical, psychological and social implications arising therefrom. Aim: The primary aim of the current study was to examine the clinical outcome of patients suffering from nephrotic syndr...