Mild COVID-19 in a pediatric renal transplant recipient.

As of mid-April 2020, the coronavirus disease of 2019 (COVID-19) pandemic has affected more than 2 million people and caused 135 000 deaths worldwide. Not much is known about the effect of this disease in immunosuppressed children with renal transplantation (RT). Here we report a 13-year-old child with multiple comorbidities who acquired COVID-19 5 years post-RT in the United States. Maintenance immunosuppression (IS) consisted of sirolimus and mycophenolate. There was no history of travel or exposure to sick contacts. The presenting features were fever, cough, rhinorrhea, and hypoxemia. Diarrhea was the only extrapulmonary manifestation. Chest X-ray was normal. He did not require intensive care unit care or ventilation. There was a transient rise in his serum creatinine without change in urine output; dialysis was not required. Slight reduction in IS was done. He had an excellent clinical recovery within 4 days and was able to be discharged home. His respiratory symptoms resolved but the diarrhea persisted during a 4-week follow-up period. This report provides a brief perspective on the short-term COVID-19 clinical course in an immunosuppressed child. More reports will add valuable information on the potential variety of spectrum of the illness in this subset of children.

The use of internet sources for nutritional information is linked to weight perception and disordered eating in young adolescents.

The internet serves as an accessible and confidential resource for young adolescents seeking nutritional information. However, the quality of information retrieved online is mixed and could have serious implications for users. Young adolescents who perceive themselves as overweight may be disproportionately affected as they are at greater risk for disordered eating. The current study aimed to (1) assess whether the frequency of use of different internet sources to obtain nutritional information differs between healthy weight young adolescents and those with overweight/obesity based on both objective and perceived weight status and (2) evaluate the relationships between different internet sources utilized for nutritional information and disordered eating. Young adolescents (n = 167; 10-15 years) completed the Children's Eating Attitudes Test (ChEAT; total disordered eating), indicated their perceived weight status, and reported how often they obtained nutritional information from the following internet sources: professional websites, personal websites, social media, commercial weight loss websites, and forums. Objective height and weight measurements were obtained. Young adolescents that perceived themselves to be a little overweight or overweight reported greater use of personal websites (p = .012), commercial weight loss websites (p = .011), and social media (p = .019) for nutritional information than those that did not perceive themselves to be a little overweight or overweight. The frequency of use of internet sources for nutritional information did not differ based on objective weight status. Greater use of each of the internet sources for nutritional information was related to greater disordered eating (p's < 0.05). While longitudinal research is needed to further examine these relationships, healthcare providers and teachers should provide young adolescents with guidance for interpreting and using online nutritional information to encourage valid and reliable health recommendations.

Isolated abdominal nocardiosis in a pediatric renal transplant recipient.

Nocardia infection after RT is uncommon. The most common modes of exposure are inhalation of dust containing nocardia or contaminated soil/water and surgical instruments. Isolated abdominal nocardiosis following RT has not been reported. We report an 11-year-old female who developed nocardia abscesses of the abdomen post-RT. ESRD was secondary to FSGS and she had received multiple immunosuppressive agents prior to transplant. Induction immunosuppression consisted of thymoglobulin and maintenance was with tacrolimus, mycophenolate, and prednisone. There were construction activities in the hospital ward during her hospital stay. Due to immediate recurrence of FSGS in the allograft, she received plasma exchange, rituximab, and IVIG. Anti-infective prophylaxis consisted of TMP-SMX, valganciclovir, and nystatin. She developed multiple loculated fluid collections in the abdomen 6 weeks later. Histology of lesions demonstrated suppurative caseating granulomatous inflammation and the AFB culture showed Nocardia farcinica. With the reduction of immunosuppressive agents along with usage of TMP-SMX, imipenem-cilastatin, and linezolid, she had a partial recovery after 9 months with persistent small abscesses but remained asymptomatic clinically. There was no evidence of nocardia infection in lungs and brain. Repeat AFB culture from the lesions was negative. Allograft function remained stable throughout. She remains on oral TMP-SMX therapy. We postulated that she could have acquired nocardia either from the contaminated air particles in the hospital from the construction activities or reactivation of nocardia from prior colonization. Nocardia infection should be suspected in immunocompromised patients with unexplained fever and abdominal pain.

Novel Interventions and Assessments Using Patient Portals in Adolescent Research: Confidential Survey Study.

BACKGROUND: While adolescents can receive confidential health care without parental or guardian notification, they are rarely asked about their experiences and opinions regarding their care because participation in research often requires parental consent. Anonymous research with adolescents via confidential patient portals may ameliorate this research gap. OBJECTIVE: Because use of a confidential online adolescent patient portal is high at our academic institution, we hypothesized that adolescents would also respond to survey-based research via the portal, especially if asked anonymously and without parental consent. We used a clinical scenario of needing to better understanding adolescent and young adults' views about their health and health care, including information on a long-acting reversible contraceptive (LARC) to test if and how they will use a portal for research. METHODS: Upon receiving Institutional Review Board approval, we sent 2 portal-based surveys about confidential services to 2 groups of females, ages 14 to 25 years, who had attended an adolescent clinic in the past 3 years. This clinic mostly serves Medicaid recipients (80%) and is racially and ethnically diverse with half of patients identifying as African American and roughly 10% Hispanic. The control group was a random sample of female patients who never received a LARC (n=150) and the intervention group included all female adolescents who had received a LARC from the same clinic (n=107). This second sample was manually cross-checked to confirm they had an office visit for this reason. Consenting for themselves, the control group received an email through the patient portal with a link and a request to perform an assessment. The survey for the control group included items assessing health literacy and health communication preferences. The survey for the intervention group included health literacy items as well as items to assess their opinions and perceptions regarding LARCs. We tracked click-through rates and opened messages; each participant received 4 reminders. RESULTS: While only 3 participants fully completed either survey, email read rates (29/107 [27.1%] of LARC recipients and 39/150 [26.0%] of controls) were encouraging. Additionally, of those who opened the messages, almost twice as many of the LARC recipients (10/107 [9.3%]) read through the entire survey, while less than half read the entire survey as compared to those who received the survey asking about health literacy and health care preferences (6/150 [4.0%]). CONCLUSIONS: The methodology of using adolescent portals for online surveys provides a new avenue for research even though the study did not yield sufficient participation to understand these adolescents' preferences. Future studies need to test if a different survey topic would engage adolescents or if other methods like text-based reminders would improve participation.

Safety and Efficacy of Very Early Conversion to Belatacept in Pediatric Kidney Transplantation with Transplant-Associated Thrombotic Microangiopathy: Case Study and Review of Literature.

The inhibition of co-stimulation during T-cell activation has been shown to provide effective immunosuppression in kidney transplantation (KT). Hence, the conversion from calcineurin inhibitor (CNI) to belatacept is emerging as a potential alternate maintenance immunosuppressive therapy in those with transplant-associated thrombotic microangiopathy (TA-TMA) or in the prevention of TA-TMA. We present a 17-year-old male who presented with biopsy-proven CNI-associated TA-TMA immediately post-KT. The administration of eculizumab led to the reversal of TMA. Tacrolimus was converted to belatacept with excellent efficacy and safety during a short-term follow-up of one year. Further larger controlled studies are required to demonstrate the efficacy of this approach in children who present with early-onset TMA post-KT.

Efficacy and Safety of Eculizumab in Enteroaggregative E. coli Associated Hemolytic Uremic Syndrome.

BACKGROUND: Hemolytic uremic syndrome (HUS) may present atypically without the full triad of classical HUS. Eculizumab has been shown to be efficacious in complement-mediated atypical HUS and some cases of Shiga-toxin (ST) associated HUS. We report the utility of eculizumab in enteroaggregative E. coli (EAEC) associated HUS. CASE SUMMARY: A female toddler presented with hemolytic anemia, oliguric acute kidney injury (AKI) without thrombocytopenia, and peripheral schistocytes. The stool examination for ST was negative but positive for EAEC. She required several hemodialysis sessions and received one dosage of eculizumab with rapid reversal of AKI and hemolytic markers. A kidney biopsy revealed acute tubular injury and segmental glomerular basement membrane splitting. Genetic testing was negative for complement mutations or deficiencies. A follow-up six months later showed persistently normal renal function and hematological markers. CONCLUSION: The clinical and histological manifestations of non-ST-associated diarrheal HUS and the role of eculizumab in this condition warrant future larger studies.

Early recurrence of focal segmental glomerulosclerosis in a kidney transplant recipient with APOL1 one risk variant.

Apolipoprotein 1 (APOL1) risk variants (G1 and G2) are associated with focal segmental glomerulosclerosis (FSGS) in patients of African ancestry. The prevalence of APOL1 two risk variants is lower in Hispanics and very rare in European and Asian populations. APOL1 two risk variants in donor kidneys is associated with recipient kidney graft loss, however the effect of recipient risk variant in the kidney transplant outcome is unclear. Here, we present a late adolescent male with FSGS and end stage renal disease with one APOL1 risk variant (G2) who had immediate recurrence of FSGS in the post-KT period. There was an excellent response to few sessions of plasmapheresis and Rituximab with no further recurrence of FSGS in the 1 year follow-up period. It needs to be seen whether the recipient APOL1 single risk variant causes increased susceptibility to kidney graft loss on a long run via recurrent or de novo pathologies.

Short-Term Outcome of Isolated Kidney Transplantation in Children with Autosomal Recessive Polycystic Kidney Disease: A Case Series and Literature Review.

Autosomal recessive polycystic kidney disease (ARPKD) is often associated with hepatobiliary disease in the form of hepatic fibrosis and/or Caroli disease. Combined liver-kidney transplantation (CLKT) is a transplant modality of choice in children with both end-stage renal disease (ESRD) and severe hepatic disease. However, there is no consensus on whether children with ARPKD-associated ESRD without severe hepatic disease can be treated with isolated kidney transplantation (KT) without the need for CLKT. We retrospectively studied the efficacy of isolated KT in children with ARPKD without severe hepatic disease, and followed the course of hepatic disease post KT. This is a single-center study of three children with ARPKD and ESRD who underwent isolated KT. None of them had severe hepatic disease at the time of KT. All children were clinically diagnosed with ARPKD in the immediate postnatal period. All had hepatic fibrosis of varying degrees and two had intrahepatic biliary duct (IHBD) dilatation. None had gastrointestinal (GI) bleed, portal hypertension or cholangitis. Two children had preemptive KT. Pre-transplant unilateral or bilateral native nephrectomy were performed for two children, and one underwent unilateral native nephrectomy at the time of KT. The median creatinine clearance at a median post-KT follow-up of 24 months was 60.3 mL/min/1.73 m2. The two-year graft and patient survival were both 100%. Post KT, all three patients continued to demonstrate evidence of hepatic fibrosis and IHBD on sonogram; however, none of them were either evaluated for or required liver transplantation given normal synthetic liver function and absence of portal hypertension or other severe hepatobiliary disease. There were no adverse events observed such as cholangitis, GI bleed, or multiorgan failure. Hence, an excellent short-term graft and patient survival was demonstrated in this study of children with ARPKD and mild to moderate hepatic disease who received isolated KT. Long-term follow-up and larger studies are important to assess the efficacy of isolated KT in this subset of children with ARPKD.

Synthetic cannabinoid-associated acute interstitial nephritis: An emerging cause of pediatric acute kidney injury?

Synthetic cannabinoid (SCB) usage among children is a rapidly emerging public health concern in the United States. Acute kidney injury (AKI) is an uncommon manifestation of SCB usage, with acute tubular necrosis (ATN) as the predominant histology. Here we describe a 16-year-old adolescent who sustained severe non-oliguric AKI in association with SCB usage. Emesis, right flank pain, and hypertension were the presenting clinical features. There was no uveitis, skin rash, joint pains, or eosinophilia. Urinalysis showed absence of proteinuria or hematuria. Urine toxicology was negative. Renal sonogram showed bilateral echogenic kidneys. Renal biopsy demonstrated severe acute interstitial nephritis (AIN), mild tubulitis, and absence of ATN. AIN responded with pulse steroid followed by oral steroid. Renal replacement therapy was not required. Although the exact pathophysiology of SCB-associated AIN is not known, immune response elicited by the renal tubulointerstitial cells against the antigens present in the SCB is the most likely mechanism. A high index of suspicion for SCB-induced AKI is necessary in adolescents who present with AKI of unclear etiology.

Situs inversus totalis in an asymptomatic adolescent - importance of patient education: A case report.

BACKGROUND: Situs inversus totalis (SIT) may be an incidental finding in asymptomatic children. Patients may not understand the implications of this condition and the importance of relaying the diagnosis to their healthcare providers. CASE SUMMARY: We report an asymptomatic seventeen-year-old adolescent with previously-diagnosed SIT who presented for a routine well-child visit. During history taking, he denied any past medical conditions, including cardiovascular conditions. Only when physical exam revealed point of maximal impulse and heart sounds on the right side, did he convey that he had been diagnosed with SIT incidentally at age of 12 years. He was not aware of associated conditions or the potential implications of his diagnosis, nor did he realize it is pertinent medical history to be relayed to healthcare providers. Chest X-ray confirmed dextrocardia and abdominal X-ray showed right-sided stomach. Abdomen sonogram showed left-sided liver and right-sided spleen. Echocardiogram showed normal valvular structure and function. A comprehensive discussion was provided to address the patient's lack of understanding that SIT is a medical diagnosis with potential implications. CONCLUSION: While SIT is rare and mostly asymptomatic, affected patients may not comprehend the importance of the diagnosis and its potential ramifications. Recognition of the patient's lack of awareness allows the healthcare provider to educate the patient and hopefully can prevent potential medical and surgical complications.

Severe hypertriglyceridemia in an infant on chronic hemodialysis.

Severe hyperlipidemia is a risk factor for cardiovascular disease. Children with chronic kidney disease and end stage renal disease are at risk for development of hyperlipidemia. In this report, we describe a 7-month-old male infant with Denys-Drash syndrome who was found to have a "milky-layer" floating on the deaerator of the hemodialysis machine. Investigations showed severe hypertriglyceridemia of >1000 mg/dl. The patient had been on chronic continuous manual peritoneal dialysis until 6 months of age and recently had been switched to hemodialysis. Management included lowering of caloric intake and addition of medium chain triglyceride with reduction of the serum triglyceride levels to 300-400 mg/dl. Close monitoring of serum lipids and timely intervention is important to prevent serious complications associated with dyslipidemia. Observation of the "milky layer" in the deaerator of the hemodialysis machine may be an interesting visual clue of underlying severe hypertriglyceridemia.

Efficacy and safety of local candida immunotherapy in recalcitrant warts in pediatric kidney transplantation: A case report.

BACKGROUND: Warts are common in recipients of kidney transplantation (KT). Resistant warts which are not amenable to conventional therapies may lead to significant mor bidity. Limited data exists on safety and efficacy of local immunotherapy among immunocompromised KT recipients. CASE SUMMARY: We report a seven-year-old child who presented with recalcitrant plantar per iungual warts in the early KT period. Immunosuppression consisted of tacrolimus, mycophenolate and steroid. Due to failure of conventional anti-wart therapies, he was treated with two sessions of intralesional (IL) candida immunotherapy along with liquid nitrogen cryotherapy leading to complete resolution of the warts. Interestingly, de novo BK viremia was seen about three weeks following the last candida immunotherapy. This required reduction of immu nosuppression and other anti-BK viral therapies. Allograft function remained stable but there were donor specific antibodies detected. There also was elevated level of plasma donor derived cell-free DNA. A pneumocystis jirovecii pneumonia occurred ten months following completion of immunotherapy that was successfully treated with trimethoprim-sulfamethoxazole. During this ten-month follow-up period, there have been no recurrence of warts, and transplant kidney function has remained stable. CONCLUSION: Stimulation of cell-mediated immunity against the human papilloma virus induced by the IL candida immunotherapy is thought to be a cause for wart resolution. With this therapy, whether it is necessary to augment the immunosuppression to prevent rejection is unclear as that may come with a risk of infectious complications. Larger, prospective studies in pediatric KT recipients are needed to explore these important issues.

Hyperinsulinemic Hypoglycemia Due to PMM2 Mutation in Two Siblings with Autosomal Recessive Polycystic Kidney Disease.

Background: Hyperinsulinemic hypoglycemia (HH) is an important cause of persistent hypoglycemia in newborns and infants. Recently, PMM2 (phosphomannomutase 2) mutation has been associated with HH, especially in conjunction with polycystic kidney disease (PKD). PMM2 deficiency is one of the most common causes of congenital disorder of glycosylation (CDG). Renal involvement in PMM2-CDG manifests as cystic kidney disease, echogenic kidneys, nephrotic syndrome or mild proteinuria. Case Summary: Here, we describe a pair of siblings with HH associated with autosomal recessive polycystic kidney disease (ARPKD) and PMM2 mutation. Two siblings with ARPKD presented during infancy and early toddler years with severe hypoglycemia. Both had inappropriately elevated serum insulin, low ß-hydroxybutyrate, a need for a high glucose infusion rate, positive glycemic response to glucagon, positive diazoxide response and PMM2 mutation. Conclusions: Although this combination of HH and PKD was recently described in patients of European descent who also had PMM2 mutation, our report is unique given that these non-consanguineous siblings were not exclusively of European descent. PMM2 mutation leading to abnormal glycosylation and causing cystic kidneys and the alteration of insulin secretion is the most likely pathogenesis of this clinical spectrum.

Polyethylene Glycol 3350 Crystal Nephropathy in Association With Glomerular Mesangial Immunoglobin A Deposition.

Polyethylene glycol (PEG) 3350, an active ingredient of over-the-counter MiraLAX, is a commonly used laxative in children and is produced by polymerization of ethylene glycol (EG). Masked EG toxicity secondary to contamination of PEG 3350 could occur. We present a 7-year-old child with developmental delay who presented with altered mental status and acute kidney injury (AKI) following intake of generic PEG 3350 for few days prior to presentation. There was high anion gap metabolic acidosis, hypernatremia, elevated osmolar gap, lactic acidosis, and AKI. Urinalysis showed tubular proteinuria, microscopic hematuria, and calcium oxalate crystals. Prior urinalyses were normal without hematuria or proteinuria. Renal biopsy revealed evidence of mesangial dominant immunoglobulin A (IgA) and complement 3 (C3) deposits along with dense tubular deposition of calcium oxalate crystals. He subsequently developed worsening oliguric AKI and required hemodialysis (HD) for several sessions. The AKI resolved within 2 weeks and further HD was not required. Mental status improved in few days. Follow-up urinalyses showed resolution of microscopic hematuria and crystalluria. We hypothesized that the generic PEG 3350 most likely was contaminated with EG leading to the presentation. A high index of suspicion of contamination of PEG 3350 with EG is required in patients presenting with unexplained high anion gap metabolic acidosis, elevated osmolar gap, lactic acidosis, AKI, calcium oxalate crystalluria, and oxalate crystals on renal biopsy. Further studies are needed to determine whether there is an association between transient glomerular mesangial IgA deposition and crystal nephropathy.

An Unusual Occurrence of Erythrocytosis in a Child with Nephrotic Syndrome and Advanced Chronic Kidney Disease.

Background: Anemia is common in patients with nephrotic syndrome (NS) for various reasons. Furthermore, anemia can occur in patients with chronic kidney disease (CKD) predominantly owing to inappropriately low erythropoietin (EPO) production relative to the degree of anemia. However, erythrocytosis is uncommon in patients with NS and advanced CKD who are not treated with exogenous erythropoietin stimulating agents, and when present, will necessitate exploration of the other etiologies. Case summary: Here, we describe an 8-year-old girl with erythrocytosis in association with NS and advanced CKD. The patient was found to have erythrocytosis during the evaluation for hypertensive urgency. She also had nephrotic range proteinuria without edema. Serum hemoglobin and hematocrit were 17 gm/dL and 51%, respectively, despite hydration. Renal function test showed an estimated glomerular filtration rate of 30 mL/min/1.73 m2. There was mild iron deficiency anemia with serum iron saturation of 18%. Serum EPO level was normal. Urine EPO was not measured. Renal biopsy showed evidence of focal segmental glomerulosclerosis. Genetic testing for NS showed mutations in podocyte genes: NUP93, INF2, KANK1, and ACTN4. Gene sequence analysis of genes associated with erythrocytosis showed no variants in any of these genes. She required chronic dialysis ten months later and, subsequently, a renal transplantation 14 months after the initial presentation. Conclusion: Since the serum EPO level was normal, an increased sensitivity to EPO is the most probable mechanism of erythrocytosis. The unusual association of erythrocytosis in patients with NS and advanced CKD needs to be studied further in larger studies.

Unmasking of Gitelman Syndrome during Pregnancy in an Adolescent with Thyrotoxic Crisis.

BACKGROUND: Gitelman syndrome (GS) is an inherited salt-losing renal tubulopathy characterized by hypokalemic metabolic alkalosis with hypomagnesemia and hypocalciuria. Patients can be asymptomatic until late adolescence or adulthood, and hence may be discovered incidentally during presentation with other illnesses. GS has been described in association with thyroid disorders and should be considered in patients with hyperthyroidism and persistent hypokalemia, especially in those with associated hypomagnesemia and hypocalciuria. CASE SUMMARY: In this report, we describe an 18-year-old female who presented with hyperemesis gravidarum and thyrotoxicosis, and was incidentally found to have GS, confirmed by the sequence analysis of SLC12A3. Conclusions. Thyroid dysfunctions, such as hypothyroidism, thyrotoxicosis, and thyroid nodules, may develop during pregnancy. A structural homology between the beta-human chorionic gonadotropin and thyroid stimulating hormone molecules, as well as their receptors is probably the basis for the transient thyrotoxicosis crisis during pregnancy. Since hyperemesis in pregnancy can also lead to hypokalemia and alkalosis, a high index of suspicion for GS during pregnancy is required for timely diagnosis and management.

End-stage renal disease in a child with focal segmental glomerulosclerosis associated with a homozygous NUP93 variant.

This report highlights that the genetic causes of FSGS, including NUP93 gene variant, such as the one described in this report, progress to end-stage renal disease rapidly and that the risk of recurrence post-renal transplantation is less likely.

Concomitant nephrotic syndrome and tubulointerstitial nephritis in a child with Epstein-Barr virus mononucleosis.

Acute kidney injury (AKI) and nephrotic syndrome (NS) are uncommon manifestations of Epstein-Barr virus (EBV) mononucleosis. We report a 4-year-old boy with Infectious mononucleosis (IM) who presented with dialysis-requiring AKI and NS. Renal biopsy showed severe acute tubular necrosis, mild chronic interstitial nephritis and focal podocyte foot processes effacement. EBV early RNA was not detected in the renal tissue. However, immunophenotyping of peripheral lymphocytes showed increased cytotoxic T cell activity and increased memory B cells. Treatment with steroid led to rapid resolution of NS within 3 weeks. Renal function stabilised. EBV viral capsid antigen (VCA) IgM remained elevated until 4 months before starting to decline when VCA IgG and nuclear antigen started appearing. B lymphocytes are the predominant target cells in EBV infection and additionally may also act as antigen presenting cells to T lymphocytes, thereby eliciting the strong immune response and leading to podocyte and tubulointerstitial injury.

Hereditary hemorrhagic telangiectasia presenting as a recurrent epistaxis in an adolescent: A case report.

BACKGROUND: Epistaxis can be an isolated finding or a manifestation of a systemic disease. Some of the potential etiologies are usage of anticoagulants, bleeding disorders, vascular aneurysms, nasal neoplasm, hypertension and nasal steroids. Hereditary hemorrhagic telangiectasia (HHT) as a cause of recurrent epistaxis is uncommon. CASE SUMMARY: In this report, we describe an 18-year-old adolescent with recurrent epistaxis, mucocutaneous telangiectasia and family history of HHT, consistent with HHT. CONCLUSION: Timely diagnosis is needed not only to treat the epistaxis but also to be vigilant for other serious manifestations of this condition.