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Catheter-Directed Thrombolysis for Neonatal IVC and Bilateral Renal Vein Thrombosis: A Case Report

Guichet, Phillip L; Jasinski, Sylwia; Malaga-Dieguez, Laura; De Los Reyes, Francis A; Ahuja, Tania; Bride, Karen L; Patel, Amish
Renal vein thrombosis is the most common non-catheter-associated venous thromboembolism event in neonates, accounting for up to 20% of cases. Although mortality rates are lower than a variety of other forms of pediatric thrombosis, renal vein thrombi are associated with significant short-term and long-term sequelae. This report presents the case of a full-term neonate presenting with bilateral renal vein thrombosis with inferior vena cava involvement treated with catheter-directed thrombolysis. This case report intends to highlight the value of a multidisciplinary approach to pediatric venous thromboembolism and to outline relevant procedural details and current laboratory and imaging monitoring of catheter-directed thrombolysis.
PMID: 32569035
ISSN: 1536-3678
CID: 4492822

Pulmonary Manifestations of Renal Disorders in Children

Malaga-Dieguez, Laura; Trachtman, Howard; Giusti, Robert
The causes of kidney disease in pediatric patients are evenly divided between congenital abnormalities of the kidney and urinary tract and acquired disorders. Nearly 10% to 15% of adults in the United States have chronic kidney disease (CKD); there are no comparable data in children. Regardless of patient age, CKD is a systemic problem that affects every organ system, including the lung. We review the tests used to diagnose and evaluate kidney disease and the main clinical syndromes that are likely to be encountered to aid the pulmonology consultant who is asked to evaluate patients with kidney disease.
PMID: 33228933
ISSN: 1557-8240
CID: 4680392

Center Volume and Kidney Transplant Outcomes in Pediatric Patients

Contento, Marissa N; Vercillo, Rachel N; Malaga-Dieguez, Laura; Pehrson, Laura Jane; Wang, Yuyan; Liu, Mengling; Stewart, Zoe; Montgomery, Robert; Trachtman, Howard
Rationale & Objectives/UNASSIGNED:Recent data demonstrate that center volume is not a factor in the outcomes of adult kidney transplant recipients. This study assessed whether center volume affects graft survival in pediatric patients who received a kidney transplant. Study Design/UNASSIGNED:Case-cohort study. Setting & Participants/UNASSIGNED:Kidney transplantation centers, recipients younger than 18 years. Results/UNASSIGNED: = 0.02. Although outcomes for deceased donor kidney recipients were similar in the 3 volume categories, outcomes in patients who received a living kidney donation were better in the high-volume centers. Low household income was associated with poorer outcomes. However, 3-year graft survival was similar in the 3 center volume categories in high and low mean household income states. Limitations/UNASSIGNED:Lack of information for complications and individual family household income of recipients. Conclusions/UNASSIGNED:Transplantation outcomes are worse in pediatric patients treated at lower-volume centers. The difference was more pronounced for patients receiving living versus deceased donor kidneys. The distribution of household income in pediatric transplant recipients may also be a factor that contributes to lower 3-year graft survival in low-volume centers.
PMID: 32734249
ISSN: 2590-0595
CID: 4540722

The Psychosocial Impact of a Diagnosis of Hypertension in Pediatric Patients

Bieber, Amy Kalowitz; Pehrson, Laura Jane; Vento, Suzanne; Malaga-Dieguez, Laura; Spruill, Tanya M; Trachtman, Howard
PMID: 32043037
ISSN: 2468-0249
CID: 4303892

Hypokalemia Associated With a Claudin 10 Mutation: A Case Report

Meyers, Nicole; Nelson-Williams, Carol; Malaga-Dieguez, Laura; Kaufmann, Horacio; Loring, Erin; Knight, James; Lifton, Richard P; Trachtman, Howard
Hypokalemia of renal origin can arise from genetic abnormalities in a variety of transporters or channel proteins that mediate tubular handling of potassium. Recently, mutations in claudin 10 have been documented in patients with hypokalemia in association with a range of other electrolyte abnormalities and skin and sweat gland manifestations. We report a 12-year-old Hispanic boy who presented with anhydrosis, aptyalism, alacrima, hypokalemia, and hypocalciuria, in whom we detected a homozygous mutation in the claudin 10 gene. During the 4-year follow-up period, he developed hypermagnesemia and a decline in estimated glomerular filtration rate to 59mL/min/1.73m2. His unaffected parents and siblings were heterozygous for the mutation. We summarize the clinical phenotype encountered in patients with claudin 10 mutations. It is characterized by significant heterogeneity in electrolyte and extrarenal abnormalities and is associated with a risk for progressive loss of kidney function in up to 33% of cases. Awareness of this association between claudin 10 mutations and electrolyte abnormalities, namely hypokalemia and hypermagnesemia, sheds new light on the physiology of potassium and magnesium handling along the nephron and increases the likelihood of identifying the underlying tubular mechanism in patients with newly diagnosed hypokalemia with or without concomitant hypermagnesemia.
PMID: 30482581
ISSN: 1523-6838
CID: 3657872

Infection-associated glomerulonephritis

Chapter by: Malaga-Dieguez, Laura
in: Glomerulonephritis by
[S.l. : s.n.], 2019
pp. 437-450
ISBN: 9783319493787
CID: 3857182

Immune cell profiles in children with essential hypertension (EH) [Meeting Abstract]

Trachtman, H; Pehrson, L J; Malaga-Dieguez, L; Alexandre, J M; Chattopadhyay, P K
Background: There is growing evidence that sodium is stored in a non-osmotic form in the interstitial compartment of skin and muscle. In these sites, sodium may contribute to the development of EH by altering the immune system. These effects may be reflected in peripheral blood (PB). We tested PB of children with EH to examine the diversity of immunophenotypes, and to test whether disease treatment changed circulating cells. We deployed high parameter flow cytometry, which allowed detailed characterization of T-cell subsets.
Method(s): Eight pediatric patients with EH were enrolled. PB was collected at baseline for all patients, and after 4 and 16 weeks of anti-hypertensive treatment for 3 patients. We designed a 24-parameter flow cytometry panel to enumerate various T-cell subsets, including naive, memory, dividing, exhausted, regulatory, and suppressive cells. We analyzed data using t-sne, a dimension reduction algorithm that provides a broad overview of the landscape of T-cell immunophenotypes, and applied bivariate difference gating to identify the cell populations uniquely altered with treatment.
Result(s): At baseline, 7 of 8 patients showed the expected diversity in T-cells subsets. There were dominant populations that differed by patient, suggesting heterogeneity that might be linked to clinical outcome. The 8th patient had a striking polarization in T-cell phenotype at baseline, with two major subpopulations and very few other cells. Her "skewed" T-cell landscape resolved with treatment, and we could precisely identify the cells lost. Cells were uniformly CD4+CD45RA+CD127+CD25-CD38+CCR4-Ki67-LAG3+CTLA4-CD39-IDO-HELIOS-FoxP 3-CXCR3-GITR+. This phenotype represents a class of naive, non-classical regulatory (i.e., suppressive) T-cells. Subsets also expressed other suppressive markers like LAP, GARP, and CD73. Interestingly, the other two patients also showed loss of cells expressing LAG3, CD73, LAP, and/or GARP with treatment.
Conclusion(s): Children with EH have heterogeneous regulatory T-cell subsets. Successful control of blood pressure with anti-hypertensive drugs re-shapes the T-cell landscape in PB, reducing the number of suppressive T-cells. Our approach-to precisely identify specific cell types altered with disease-is well-suited to identifying biomarkers, and can provide detailed mechanistic information that informs treatment approaches
ISSN: 1533-3450
CID: 4755042

Efficacy of a gluten-free diet (GFD) in children with difficult-to-manage nephrotic syndrome (NS) [Meeting Abstract]

Trachtman, H; Pehrson, L J; Vento, S M; Malaga-Dieguez, L; Gipson, D S; Lemley, K V; Dell, K M; Srivastava, T; Kaskel, F J; Meyers, K E; Faul, C
Background: Zonulin (ZON) increases gut permeability after exposure to gliadin in children with celiac disease. Plasma zonulin levels are increased in children with NS. Protease activated receptor-2, which mediates ZON effect in enterocytes, is present on podocytes. Thus, gluten-induced elevations in ZON may affect glomerular permeability and mediate proteinuria in children with NS. We conducted this study to assess the efficacy of a GFD in controlling disease in children with difficult-to-manage NS.
Method(s): This multicenter, open-label trial tested the efficacy of a GFD in children with steroid-responsive, difficult-to-manage NS. The Treatment Period was 6 months. A positive response was defined as >=50% reduction in relapse rate versus the prior 6 months or discontinuation of >=1 immunosuppressive medication. The following data were tabulated: age, gender, race/ethnicity, serum creatinine, proteinuria, histopathology if available, and treatment. Serum was collected prior to and at completion of the Treatment Period to assess the effect on the glomerular cytoskeleton in vitro. Data are provided as mean+/-SD.
Result(s): 14 children (8F:6M) were enrolled, age 7.8+/-4.6 yr, baseline serum creatinine 0.46+/-0.12 mg/dl, and Up/c 0.45+/-0.49 (mg:mg). There were 11 Whites, 1 Black and 3 other racial groups and 2 children were Hispanic/Latino. The underlying disease was MCD in 10 and FSGS in 4 cases. At the end of the Treatment Period, 4 participants had a positive response (2 reduced relapse rate and 2 reduced medication burden), 5 had no benefit (2 withdrew before 6 months), 3 patients are in the 6-month Treatment Period, and 1 child was lost to follow-up. One adolescent had no change in relapse rate but responded to corticosteroids more rapidly on the GFD. Baseline plasma zonulin concentration was 19.4+/-1.7 vs 13.4+/-0.9 pg/mL in non-responders (n=4) vs GFD responders (n=2), respectively, P=0.01.
Conclusion(s): Up to a third of patients with difficult-to-manage NS have a favorable response to implementation of a GFD. An elevated plasma zonulin level may predict a poor response to the maneuver. A trial of this dietary intervention may be warranted in children with frequently relapsing or steroid dependent NS to minimize the need for immunosuppressive agents
ISSN: 1533-3450
CID: 4755162

Interstitial nephritis: Two pediatric cases with atypical radiological features

Connors, Joseph; Aronov, Rachel; Malaga-Dieguez, Laura; Vento, Suzanne; Pehrson, Laura Jane; Wu, Ming; Lala, Shailee; Trachtman, Howard
Interstitial nephritis (IN) is a relatively rare entity in children and adolescents that can be caused by a range of disorders including infection, medications, inflammatory bowel disease, and sarcoid. There is no proven therapy for this condition. We present 2 cases of biopsy-proven interstitial nephritis, of which 1 case was with granulomatous features that presented with unusual sonographic findings of discrete mass lesions in the kidney parenchyma bilaterally. Although a precise cause could not be identified in either case, 1 patient progressed to end-stage kidney disease (ESKD) and the other is in the early stages of treatment. We suggest that recognition of the atypical imaging features of interstitial nephritis may enable early recognition of this condition and avoid confusion with neoplastic or infectious processes.
PMID: 30116463
ISSN: 1930-0433
CID: 3241102

Impact of chronic illness in children on families: Kidney disease (KD) versus diabetes mellitus (DM) [Meeting Abstract]

Trachtman, H; Malaga-Dieguez, L; Vento, S M; Jane, Pehrson L; Rodgin, S L; Adkisson, H Y; Brodzinsky, L; Lois, R; Ilkowitz, J; Gallagher, M P
Background: Chronic illness in children has adverse effects on family members besides the patient and can impact the integrity and function of the family unit. Most previous studies have examined a single disease entity. However, there has been limited assessment comparing the effect of different illnesses on family function.
Method(s): Established patients treated in the pediatric ambulatory Nephrology or DM clinics were included in the study. Their parents were asked to complete the 2-page Pediatric Quality-of-Life Family Impact Module (PedsQL-FIM), version 2.0, a validated survey instrument. Clinical and laboratory data were retrieved from the electronic health record. Data were summarized as mean+/-SD. Disease group and child age were entered as predictors in linear regression analyses with FIM total and subscale scores as outcome variables. Comparisons between groups were assessed using paired t-tests.
Result(s): 96 patients (43 F: 53 M) were evaluated in the Nephrology Clinic and 55 (30 F: 25 M) in the DM Clinic. The mean age of the patients was 13.0+/-3.9 and 10.4+/- 6.3 yr, respectively. Within the KD sample, older age was significantly associated with lower scores on all FIM subscale scores. Gender was not a significant predicator for FIM scores in either disease group. Controlling for age, chronic illness group was a significant predictor of the FIM total and subscale scores. Parents of D patients endorsed significantly lower total FIM scores compared to the KD patients (D 58+/-16; KD 79+/-17 p <0.001) as well as on subscales of physical, emotional, social, and cognitive functioning, communication, worry, daily activities, family relationships, and reports of health-related quality of life (P<0.01).
Conclusion(s): Our findings confirm that chronic illness in childhood adversely affects a wide range of aspects of family function. The impact is greater in older children with KD and varies depending on the disease context. Families with children who have DM manifested greater disturbances than those with children who have isolated KD. Further study is warranted to assess the effects of the underlying renal disease and intensity of medical care and whether there are specific features can be used to identify vulnerable families
ISSN: 1533-3450
CID: 4758082