Publications by authors named "Carolina Cordinhã"

10 Publications

  • Page 1 of 1

Severe acute anaemia in an infant: An unusual finding.

J Paediatr Child Health 2021 Mar 27. Epub 2021 Mar 27.

Infectious Diseases Unit and Emergency Service, Hospital Pediátrico de Coimbra, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

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http://dx.doi.org/10.1111/jpc.15432DOI Listing
March 2021

IgA vasculitis (Henoch-Schönlein purpura) nephritis and psoriasis in a child: is there a relationship?

J Bras Nefrol 2021 Feb 15. Epub 2021 Feb 15.

Centro Hospitalar e Universitário de Coimbra, Departamento de Pediatria, Serviço de Pediatria Ambulatória, Unidade de Nefrologia Pediátrica, Coimbra, Portugal.

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 disorders has scarcely been reported in pediatric patients. Henoch-Schönlein purpura (HSP) is a systemic vasculitis characterized by IgA deposits in the vessel walls of affected organs and in the mesangium of the kidney. HSP nephritis histopathology is identical to IgA nephropathy.

Case Report: A 6-year-old boy with recent onset of psoriasis developed HSP with kidney involvement, clinically manifested by nephrotic-range proteinuria and hematuria. Kidney biopsy revealed fibrocellular glomerular crescents and mesangial IgA deposits compatible with IgA nephropathy. Treatment with systemic corticosteroids led to the control of hematuria, but as nephrotic-range proteinuria persisted, cyclophosphamide was added, leading to a gradual decrease in proteinuria.

Conclusions: We propose an underlying common mechanism in the pathogenesis of both HSP and psoriasis, involving a dysregulation of the IgA-mediated immune response, which could predispose to both entities as well as to kidney damage and IgA nephropathy in these patients.
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http://dx.doi.org/10.1590/2175-8239-JBN-2020-0101DOI Listing
February 2021

[Diagnostic and Therapeutic Challenges in Atypical Hemolytic Uremic Syndrome: A Case Report].

Acta Med Port 2019 Oct;32(10):673-675

Unidade de Nefrologia Pediátrica. Hospital Pediátrico de Coimbra. Centro Hospitalar e Universitário de Coimbra. Coimbra.

The atypical hemolytic uremic syndrome comprises a thrombotic microangiopathy resulting from the complement alternate pathway hyperactivation. Its severity requires early diagnosis and treatment. The differential diagnosis includes typical hemolytic uremic syndrome (associated with Shiga toxin) and thrombotic thrombocytopenic purpura (associated with deficient activity of ADAMTS13). The only specific treatment currently available for atypical hemolytic uremic syndrome is eculizumab. We describe the case of a child with atypical hemolytic uremic syndrome diagnosed in the context of bloody diarrhea, complicated by neurological involvement that posed several diagnostic and therapeutic challenges.
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http://dx.doi.org/10.20344/amp.10021DOI Listing
October 2019

[Distal Renal Tubular Acidosis: Clinical Variability in the Same Family].

Acta Med Port 2019 Aug 1;32(7-8):542-545. Epub 2019 Aug 1.

Unidade de Nefrologia Pediátrica. Hospital Pediátrico de Coimbra. Centro Hospitalar e Universitário de Coimbra. Coimbra. Portugal.

Primary distal renal tubular acidosis is a genetic disorder characterized by the inability in acidification of urine. Symptoms are usually non-specific and highly variable. We described six cases in a family with four generations affected. The first case was diagnosed in a 3-year-old child presenting with hematuria and urolithiasis. Later, his sister, sons and two nephews were studied. Although asymptomatic, they all had nephrocalcinosis and hyperchloremic metabolic acidosis with normal anionic gap, except one case with normal arterial blood gas test but with nephrocalcinosis and inability of urinary acidification. At follow-up, they all maintained nephrocalcinosis, the index case had acute renal damage and developed hypertension, but none developed chronic renal disease. The diagnosis of autosomal dominant distal renal tubular acidosis is generally made later and patients tend to present with milder disease. But the condition may manifest early and have a variable phenotypic severity spectrum. Carrying out screening through assessment of family history enables an earlier diagnosis while also allowing treatment to start sooner.
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http://dx.doi.org/10.20344/amp.10758DOI Listing
August 2019

Pediatric Kidney Transplantation: Experience of a Center Over 4 Decades.

Transplant Proc 2019 Jun;51(5):1579-1584

Faculty of Medicine, Hospital and University Center of Coimbra, Coimbra, Portugal; Department of Urology and Renal Transplantation, Hospital and University Center of Coimbra, Coimbra, Portugal.

Introduction: Chronic kidney disease in the pediatric population is associated with numerous comorbidities and an increased risk of mortality. Kidney transplantation (KT) is considered to be the option of choice in children with end-stage renal disease.

Aim: To characterize a single center experience in pediatric KT in the last 35 years.

Methods: A retrospective analysis of epidemiologic and clinical data from KT pediatric patients from January 1981 to December 2016. For outcome analysis, cases were divided into decades (1981-89; 1990-99; 2000-09; 2010-16).

Results: One hundred and 4 children (KT mean age 13.7 ± 3.32 years; 57.7% male) underwent 111 renal transplants (13% with living donors). Congenital anomalies of the kidney and urinary tract (36.5%) and glomerular disease (29%) were the major causes of renal disease. Peritoneal dialysis was the predominant initial therapeutic modality in 69 children (62.2%). Mean dialysis time was 19.2 months, 9 patients (8.1%) having had preemptive KT. Median follow-up was 181 months. Uncensored graft survivals rates at 5, 10, 15, and 20 years were 79.7%, 74.3%, 59.5%, and 52.8%, respectively. Graft survival improved significantly over the decades (P = .03). Higher estimated glomerular filtration rate measured at 1 year, induction immunosuppressive therapy use (thymoglobulin/basiliximab), and lower incidence of acute rejection rates were associated with superior graft survival (P < .05).

Conclusions: Uronephropathies were the most frequent cause of renal failure and peritoneal dialysis, which was the main renal replacement therapy according to the literature. Graft survival improved significantly over the study period; we hypothesize that this was related to surgical advances and the use of more effective immunosuppressive drugs.
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http://dx.doi.org/10.1016/j.transproceed.2019.05.007DOI Listing
June 2019

Pneumonia-Associated Acute Glomerulonephritis: Report of 2 Cases.

Clin Pediatr (Phila) 2019 05 18;58(5):582-585. Epub 2019 Jan 18.

1 Pediatric Nephrology Unit, Hospital Pediátrico, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

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http://dx.doi.org/10.1177/0009922818825157DOI Listing
May 2019

Cerebral Venous Sinus Thrombosis in a Child with Idiopathic Nephrotic Syndrome: a case report.

J Bras Nefrol 2018 Oct-Dec;40(4):418-422. Epub 2018 Aug 2.

Centro Hospitalar e Universitário de Coimbra, Hospital Pediátrico, Unidade de Nefrologia Pediátrica, Coimbra, Portugal.

Complications are rare in pediatric cases of idiopathic nephrotic syndrome (NS). Thromboembolism ranks among the most uncommon and difficult complications to diagnose, particularly in the first episode of NS, since clinical signs might be unspecific. This report describes the case of a 5-year-old girl with NS for the first time presenting with severe hypoalbuminemia (< 2g/dL). The patient responded poorly to therapy with corticosteroids. On day 8 of hospitalization she started having headaches and vomiting; she did not present hemodynamic alterations, fever or exanthems, and her neurological parameters were normal. The patient was suspected for intracranial hypertension, and computed tomography scans revealed she had cerebral venous sinus thrombosis (CVST). She was started on anticoagulants and showed clinical signs of improvement. The patient had no evident prothrombotic risk factors. She had three other episodes since she was diagnosed, one in which her plasma antithrombin level was low. Although antithrombin levels were normal in her first episode, she was tested after the resolution of proteinuria. The low levels of antithrombin seen in the first recurrence might have mirrored the initial drop in plasma antithrombin levels, an idea supported by the severe hypoalbuminemia she had when diagnosed. This severe manifestation of acquired thrombophilia might be in the origin of CVST. This report presents a rare case of thromboembolic complication in a pediatric patient with NS. The patient progressed well since she was started on anticoagulants. Although she did not present any evident risk factors at first, the development of her case indicated that severe acquired thrombophilia might have worked as the pathophysiological mechanism leading to CVST.
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http://dx.doi.org/10.1590/2175-8239-jbn-2018-0009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6534000PMC
September 2019

Bilateral renal cell carcinoma in a paediatric patient with tuberous sclerosis complex.

BMJ Case Rep 2013 Jul 12;2013. Epub 2013 Jul 12.

Pediatric Nephrology Unit, Pediatric Hospital Carmona da Mota, Hospital Center and University of Coimbra, Coimbra, Portugal.

Tuberous sclerosis complex (TSC) is a genetic neurocutaneous disorder characterised by seizures, mental retardation and hamartoma formation in multiple organs, mainly in the brain, skin, kidney, liver, lung and heart. Renal manifestations occur in about 60-80% of all patients with TSC and their rate increases with age. We report the case of a 17-year-old boy with tuberous sclerosis who presented with abdominal pain associated with kidney failure. Investigation revealed bilateral renal lesions, suggesting angiomyolipomas. On further work-up, malignancy was suspected and the patient underwent bilateral partial nephrectomy with histological diagnosis of bilateral renal cell carcinoma. This is a rare complication of TSC, particularly in a paediatric setting. Adequate surveillance of kidney disorders in patients with TSC is warranted, to guarantee an early diagnosis and treatment.
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http://dx.doi.org/10.1136/bcr-2013-010015DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3736259PMC
July 2013

[Asymptomatic cardiac tumor in a child: an incidental diagnosis].

Rev Port Cardiol 2011 Oct;30(10):795-7

Serviço de Cardiologia Pediátrica, Hospital Pediátrico de Coimbra, Coimbra, Portugal.

Cardiac tumors in the pediatric population are rare, their incidence range between 0.001% and 0.003%. They are mostly benign, rhabdomyomas the most common type, followed by fibromas. The clinical features are being usually nonspecific and depend on the size and location of the tumor within the heart. We report the case of a previously healthy four-year-old boy referred for flu-like symptoms. A respiratory infection was suspected and a chest X-ray showed an increased cardiothoracic index. An echocardiogram revealed a single large heterogeneous mass in the left ventricle emerging from the lateral wall. Despite its size, the mass did not obstruct the left ventricular outflow tract or affect mitral valve function. Cardiac magnetic resonance imaging showed a large mass whose imaging features were suggestive of a fibroma. He became symptomatic during follow-up and was referred for surgical excision of the mass. Histological study confirmed a fibroma. At present the patient remains asymptomatic.
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http://dx.doi.org/10.1016/S0870-2551(11)70028-2DOI Listing
October 2011

Severe axillary lymphadenitis after BCG vaccination: alert for primary immunodeficiencies.

J Microbiol Immunol Infect 2010 Dec;43(6):530-7

Medical Inpatient Department, Coimbra Pediatric Hospital, Coimbra, Portugal.

The bacilli Calmette-Guérin (BCG) vaccine is administered to all newborns in countries where tuberculosis is endemic. Immunocompromised hosts, namely patients with human immunodeficiency virus infection or primary immunodeficiencies, are especially prone to serious complications from this vaccine. We report three cases of BCG disease in children with primary immunodeficiencies: one with a partial recessive interferon-γ receptor 1 deficiency, who developed BCG dissemination; and two relatives with ZAP70 deficiency, a severe combined immunodeficiency, both of whom presented with regional and distant BCG disease. All had severe axillary lymphadenitis. These clinical cases underline the importance of considering the diagnosis of immunodeficiency in a child with severe axillary lymphadenitis after BCG vaccination and of disseminated BCG disease in an immunodeficient child in the appropriate clinical setting. Moreover, BCG vaccination should be delayed in every newborn with a family history of primary immunodeficiency until the condition has been ruled out.
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http://dx.doi.org/10.1016/S1684-1182(10)60082-5DOI Listing
December 2010