Renal Emergencies in Children

Kidney Injury ◽

Severity Of Illness ◽

Streptococcal Pharyngitis ◽

Acute Glomerulonephritis ◽

Wide Range ◽

History Of ◽

Uremic Syndrome ◽

Tract Infections ◽

Bloody Stools

Renal emergencies in pediatric patients range from more common conditions such as urinary tract infections to rarer conditions such as hemolytic uremic syndrome. This chapter reviews emergency conditions that are less commonly seen, with potentially significant sequelae, and with possible nonspecific or more subtle presentations. Acute kidney injury can be due to a wide range of causes, including benign gastroenteritis and post-streptococcal or toxin-related causes. Less common but more serious conditions, such as nephrotic syndrome or Henoch–Schönlein purpura, present with nonspecific signs such as edema or rash. Acute glomerulonephritis needs to be considered in a child with a history of streptococcal pharyngitis or skin infection. Hemolytic uremic syndrome is typically Shiga toxin-related and should be considered in any ill-appearing child, especially those presenting with bloody stools. Disposition of a child with a renal emergency will depend on the severity of illness, laboratory results, and the ability to follow-up with their primary care provider or specialist.

Atypical Hemolytic Uremic Syndrome Presenting as Acute Heart Failure—A Rare Presentation: Diagnosis Supported by Skin Biopsy

Journal of Investigative Medicine High Impact Case Reports ◽

10.1177/2324709619842905 ◽

2019 ◽

Vol 7 ◽

pp. 232470961984290

Author(s):

Asim Kichloo ◽

Savneek Singh Chugh ◽

Sanjeev Gupta ◽

Jay Pandav ◽

Praveen Chander

Keyword(s):

Heart Failure ◽

Skin Biopsy ◽

Thrombotic Microangiopathy ◽

Systolic Heart Failure ◽

Kidney Injury ◽

Rare Presentation ◽

History Of ◽

Atypical hemolytic uremic syndrome (aHUS) is a rare disorder of uncontrolled complement activation that manifests classically as anemia, thrombocytopenia, and renal failure, although extrarenal manifestations are observed in 20% of the patient most of which involving central nervous system, with relatively rare involvement of the heart. In this article, we report the case of a 24-year-old male with no history of heart disease presenting with acute systolic heart failure along with microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Given his presentation of thrombotic microangiopathy (TMA), along with laboratory results significant for low haptoglobin, platelets, hemoglobin, C3, C4, CH50, and normal ADAMTS13 levels, with no diarrhea and negative STEC polymerase chain reaction in stool, aHUS diagnosis was established with strong clinical suspicion, and immediate initiation of treatment was advised. Kidney biopsy to confirm diagnosis of aHUS was inadvisable because of thrombocytopenia, so the skin biopsy of a rash on his arm was done, which came to be consistent with thrombotic microangiopathy. Our case highlights a relatively rare association between aHUS and cardiac involvement, and the use of skin biopsy to support diagnosis of aHUS in patients who cannot undergo renal biopsy because of thrombocytopenia.

Angiotensinogen and Interleukin-18 as Markers of Chronic Kidney Damage in Children With a History of Hemolytic Uremic Syndrome

Physiological Research ◽

10.33549/physiolres.933340 ◽

2017 ◽

pp. 251-261 ◽

Cited By ~ 1

Author(s):

K. LIPIEC ◽

P. ADAMCZYK ◽

E. ŚWIĘTOCHOWSKA ◽

K. ZIORA ◽

M. SZCZEPAŃSKA

Keyword(s):

Serum Concentration ◽

Kidney Injury ◽

Control Group ◽

Healthy Children ◽

Protective Effects ◽

Interleukin 18 ◽

History Of ◽

Uremic Syndrome ◽

Serum And Urine

Hemolytic uremic syndrome (HUS) is a type of thrombotic microangiopathy, in the course of which some patients may develop chronic kidney disease (CKD). It is clinically important to investigate the markers of a poor prognosis. The levels of angiotensinogen (AGT) and interleukin-18 (IL-18) in serum and urine were evaluated. Study was conducted in 29 children with a history of HUS. Serum and urine AGT concentration was significantly higher in children after HUS as compared to the control group. No differences depending on the type of HUS and gender were noted. The serum concentration of IL-18 in children after HUS was significantly lower, whereas in urine did not differ significantly between the sick and healthy children. A negative correlation between the concentration of AGT in serum and albuminuria in patients after HUS was detected. The results indicate that the concentration of AGT in serum and urine in children after HUS increases, which may indicate the activation of the intrarenal renin-angiotensin-aldosterone system. The statement, that AGT may be a good biomarker of CKD after acute kidney injury due to HUS requires prospective studies with follow-up from the acute phase of the disease on a larger group of patients. Reduced IL-18 serum concentration in children after HUS with no difference in its urine concentration may indicate a loss of the protective effects of this cytokine on renal function due to previously occurred HUS.

Atypical hemolytic uremic syndrome in Brazil: clinical presentation, genetic findings and outcomes of a case series in adults and children treated with eculizumab

Clinical Kidney Journal ◽

10.1093/ckj/sfaa062 ◽

2020 ◽

Author(s):

Lilian Monteiro Pereira Palma ◽

Renato George Eick ◽

Gustavo Coelho Dantas ◽

Michele Káren dos Santos Tino ◽

Maria Izabel de Holanda

Keyword(s):

Clinical Laboratory ◽

Demographic Data ◽

Case Series ◽

Kidney Injury ◽

Adult Group ◽

Pregnancy Hypertension ◽

Wide Range ◽

Abstract Background Atypical hemolytic uremic syndrome (aHUS) is characterized by microangiopathic hemolytic anemia, thrombocytopenia and kidney injury caused by a dysregulation of the alternative complement pathway. Methods We conducted a multicenter nonregistry study aimed at collecting clinical, laboratory and genetic information of patients with aHUS in Brazil. Demographic data, genetic findings, treatments and outcomes are presented. Results Thirty-four patients were included, 62% were female and 67% were Caucasian. Half of the patients had the first manifestation of aHUS before the age of 18 years (pediatric group). Among the 17 patients who had the first manifestation after the age of 18 years (adult group), 6 were kidney transplant patients. Overall, 22 patients (65%) received plasma exchange/plasma infusion (PE/PI) and 31 patients (91%) received eculizumab. Eculizumab was started later in the adult group compared with the pediatric group. Two patients stopped dialysis after PE/PI and 19 patients stopped dialysis after eculizumab despite a late start. A pathogenic/likely pathogenic variant was found in 24.3% of patients. A coexisting condition or trigger was present in 59% of patients (infections, pregnancy, hypertension, autoimmune disease and transplant), especially in the adult group. There was a 30% relapse rate after stopping eculizumab, irrespective of genetic status. Conclusion This is the largest case series of aHUS in Brazil involving a wide range of patients for which eculizumab was the main treatment. Although eculizumab was started later than advised in the guidelines, most patients were able to stop dialysis at variable intervals. Discontinuation of eculizumab was associated with a 30% relapse of aHUS.

Tissue-resident macrophages mediate neutrophil recruitment and kidney injury in shiga toxin-induced hemolytic uremic syndrome.

Kidney International ◽

10.1016/j.kint.2021.03.039 ◽

2021 ◽

Author(s):

Julia K. Lill ◽

Stephanie Thiebes ◽

Judith-Mira Pohl ◽

Jenny Bottek ◽

Nirojah Subramaniam ◽

...

Keyword(s):

Shiga Toxin ◽

Kidney Injury ◽

Neutrophil Recruitment ◽

Eculizumab-Associated Moraxella lacunata Bacteremia and Systemic Inflammatory Response Syndrome in a Toddler with Atypical Hemolytic Uremic Syndrome

Clinical Medicine Insights Pediatrics ◽

10.1177/1179556521992367 ◽

2021 ◽

Vol 15 ◽

pp. 117955652199236

Author(s):

Paige S Bicoll ◽

Ashima Goyal ◽

Neal B Blatt ◽

Bishara J Freij

Keyword(s):

Systemic Inflammatory Response Syndrome ◽

Inflammatory Response ◽

Systemic Inflammatory Response ◽

Upper Respiratory Tract ◽

Invasive Bacterial Infection ◽

Uremic Syndrome ◽

Tract Infections ◽

Moraxella Lacunata

Moraxella lacunata, a low-virulence Gram-negative coccobacillus, is classically associated with conjunctivitis and upper respiratory tract infections; systemic infections such as sepsis have rarely been reported, especially in children. We describe a 28-month-old girl with atypical hemolytic uremic syndrome and stage II chronic kidney disease on long-term eculizumab therapy who presented with systemic inflammatory response syndrome and was found to have Moraxella lacunata bloodstream infection. Eculizumab, a humanized monoclonal anti-C5 antibody, has been associated with susceptibility to infections with encapsulated bacteria, especially Neisseria meningitidis. This is the first report of an invasive bacterial infection with Moraxella lacunata in a pediatric eculizumab recipient.

A history of uremic toxicity and of the European Uremic Toxin Work Group (EUTox)

Clinical Kidney Journal ◽

10.1093/ckj/sfab011 ◽

2021 ◽

Cited By ~ 1

Author(s):

Raymond Vanholder ◽

Angel Argiles ◽

Joachim Jankowski ◽

Keyword(s):

Work Group ◽

Target Identification ◽

Kidney Injury ◽

Uremic Toxins ◽

Uremic Toxin ◽

Post Translational Modifications ◽

History Of ◽

Advanced Stages ◽

Abstract The uremic syndrome is a complex clinical picture developing in the advanced stages of chronic kidney disease (CKD) resulting in a myriad of complications and a high early mortality. This picture is to a significant extent defined by retention of metabolites and peptides that with a preserved kidney function are excreted or degraded by the kidneys. In as far as those solutes have a negative biological/biochemical impact, they are called uremic toxins. Here, we describe the historical evolution of the scientific knowledge about uremic toxins and the role played in this process by the European Uremic Toxin Work Group (EUTox) during the last two decades. The earliest knowledge about a uremic toxin goes back to the early 17th century when the existence of what later would appear to be urea was recognized. It cost about two further centuries to better define the role of urea and its link to kidney failure and one more century to identify the relevance of post-translational modifications caused by urea such as carbamoylation. The knowledge progressively extended, especially from 1980 on, by the identification of more and more toxins and their adverse biological/biochemical impact. Progress of knowledge was paralleled and impacted by evolution of dialysis strategies. The last two decades, when Insights grew exponentially, coincides with the foundation and activity of EUTox. In the final section we summarize the role and accomplishments of EUTox and the part it is likely to play in future action, which should be organized around focus points like biomarker and potential target identification, intestinal generation, toxicity mechanisms and their correction, kidney and extracorporeal removal, patient-oriented outcomes, and toxin characteristics in acute kidney injury and transplantation.

Eculizumab discontinuation in atypical hemolytic uremic syndrome: TMA recurrence risk and renal outcomes

Clinical Kidney Journal ◽

10.1093/ckj/sfab005 ◽

2021 ◽

Author(s):

Gema Ariceta ◽

Fadi Fakhouri ◽

Lisa Sartz ◽

Benjamin Miller ◽

Vasilis Nikolaou ◽

...

Keyword(s):

Family History ◽

Univariate Analysis ◽

Renal Response ◽

Pathogenic Variants ◽

Increased Risk ◽

History Of ◽

ABSTRACT Background Eculizumab modifies the course of disease in patients with atypical hemolytic uremic syndrome (aHUS), but data evaluating whether eculizumab discontinuation is safe are limited. Methods Patients enrolled in the Global aHUS Registry who received ≥1 month of eculizumab before discontinuing, demonstrated hematologic or renal response prior to discontinuation and had ≥6 months of follow-up were analyzed. The primary endpoint was the proportion of patients suffering thrombotic microangiopathy (TMA) recurrence after eculizumab discontinuation. Additional endpoints included: eGFR changes following eculizumab discontinuation to last available follow-up; number of TMA recurrences; time to TMA recurrence; proportion of patients restarting eculizumab; and changes in renal function. Results We analyzed 151 patients with clinically diagnosed aHUS who had evidence of hematologic or renal response to eculizumab, before discontinuing. Thirty-three (22%) experienced a TMA recurrence. Univariate analysis revealed that patients with an increased risk of TMA recurrence after discontinuing eculizumab were those with a history of extrarenal manifestations prior to initiating eculizumab, pathogenic variants, or a family history of aHUS. Multivariate analysis showed an increased risk of TMA recurrence in patients with pathogenic variants and a family history of aHUS. Twelve (8%) patients progressed to end-stage renal disease after eculizumab discontinuation; 7 (5%) patients eventually received a kidney transplant. Forty (27%) patients experienced an extrarenal manifestation of aHUS after eculizumab discontinuation. Conclusions Eculizumab discontinuation in patients with aHUS is not without risk, potentially leading to TMA recurrence and renal failure. A thorough assessment of risk factors prior to the decision to discontinue eculizumab is essential.

Successful Remission of Hemolytic-Uremic Syndrome during the Third-line Weekly Gemcitabine for Metastatic Breast Cancer

Breast Cancer Basic and Clinical Research ◽

10.4137/bcbcr.s14920 ◽

2014 ◽

Vol 8 ◽

pp. BCBCR.S14920

Author(s):

Victor C. Kok ◽

Sheng-Chung Wu ◽

Chien-Kuang Lee

Keyword(s):

Breast Cancer ◽

Metastatic Breast Cancer ◽

Cancer Progression ◽

Palliative Chemotherapy ◽

Metastatic Breast ◽

Kidney Injury ◽

Peripheral Blood Smear ◽

Uremic Syndrome ◽

Third Line

Sequential palliative chemotherapy for metastatic breast cancer incorporating weekly gemcitabine administered as three-weeks-on, one-week-off schedule is widely adopted throughout the East Asia region. Hemolytic-uremic syndrome (HUS) associated with weekly gemcitabine for a breast cancer patient is extremely rare. We report here a case of 43-year-old woman with metastatic breast cancer who received weekly gemcitabine as a third-line palliative chemotherapy for her disease. She developed HUS after a cumulative dose of 11,000 mg/m2 gemcitabine, evidenced by microangiopathic hemolytic anemia (MAHA) with schistocytes seen in peripheral blood smear, decreased haptoglobin level (<0.29 mmol/L), thrombocytopenia, negative direct Coombs test, and acute kidney injury. Owing to the ease of administration of weekly gemcitabine, gemcitabine-induced thrombocytopenia, multifactorial anemia in metastatic breast cancer, and possibility of cancer progression, HUS could have gone unnoticed. Breast cancer oncologist should be cognizant of this rare HUS even during weekly gemcitabine treatment.

Familial Atypical Hemolytic Uremic Syndrome with Positive pS1191L (c.3572C> T) Mutation in CFH A Single-Center Experience

10.21203/rs.2.13028/v1 ◽

2019 ◽

Author(s):

Fadime ERSOY DURSUN ◽

Gözde YESIL ◽

Hasan DURSUN ◽

Gülşah SASAK

Keyword(s):

Renal Failure ◽

Chronic Renal Failure ◽

Family History ◽

Gene Mutations ◽

Factor H ◽

The Other ◽

History Of ◽

Abstract Background: Atypical hemolytic uremic syndrome is a condition characterized by thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney injury, which can exhibit a poor prognosis. Gene mutations play a key role in this disease, which may be sporadic or familial. Methods: We studied, 13 people from the same family were investigated retrospectively for gene mutations of familial atypical hemolytic uremic syndrome after a patient presented to our emergency clinic with atypical hemolytic uremic syndrome and reported a family history of chronic renal failure. Results: The pS1191L mutation in the complement factor H gene was heterozygous in 6 people from the family of the patient with atypical hemolytic uremic syndrome. One of these people was our patient with acute renal failure and the other two are followed up by the Nephrology Clinic due to chronic renal failure. The other 3 persons showed no evidence of renal failure. The index case had a history of 6 sibling deaths; two of them died of chronic renal failure. Plasmapheresis and fresh frozen plasma treatment was given to our patient. When patient showed no response to this treatment, eculizumab therapy was started. Conclusions: The study demonstrated that a thorough family history should be taken in patients with atypical hemolytic uremic syndrome. These patients may have familial type of the disease and they should be screened genetically. Eculizumab should be the first choice in the treatment with plasmapheresis. It should be kept in mind that the use of eculizumab as prophylaxis in post-transplant therapy is extremely important for prevention of rejection.

Ischemic Colitis and Stricture After Hemolytic-Uremic Syndrome

PEDIATRICS ◽

10.1542/peds.61.2.315 ◽

1978 ◽

Vol 61 (2) ◽

pp. 315-317

Author(s):

Hadi Sawaf ◽

Marcia J. Sharp ◽

Kum J. Youn ◽

Patrick A. Jewell ◽

Ali Rabbani

Keyword(s):

Renal Failure ◽

Sudden Onset ◽

Late Complications ◽

Surgical Removal ◽

Review Of The Literature ◽

Follow Up Studies ◽

History Of ◽

The hemolytic-uremic syndrome (HUS) was first described by Von Gasser et al.1 in 1955 as a syndrome of acute renal failure, hemolytic anemia, and thrombocytopenia in children. Follow-up studies on HUS have emphasized hypertension and uremia as late complications.2,3 A review of the literature has revealed no previously reported cases of persistent colitis and bowel stenosis after HUS. We present a child who continued to have intermittent intestinal obstruction and diarrhea until surgical removal of a segment of colon almost seven months after the onset of HUS. CASE REPORT A 26-month-old white boy who had no history of gastrointestinal disturbance had sudden onset of diarrhea with blood and mucus in the stool.