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The mainstay of treatment in severe asthma is β2 agonist therapy. During its treatment, toxicity may occur especially with the use of oral albuterol or its prolonged nebulization. The clinical manifestations of albuterol toxicity include tachycardia, hypokalemia, tremors, and hyperglycemia. Toxicity occurring due to the use of metered-dose inhaler is less common. Moreover, levalbuterol is believed to have a better safety profile than albuterol, making it a very unusual culprit for toxicity. We report an 8-year-old asthmatic boy who developed severe tachycardia, and agitation and exhibited signs of levalbuterol toxicity. He had been inadvertently administered 50 puffs of levalbuterol via metered drug inhaler at home for an acute exacerbation of asthma. With supportive management, his condition stabilized in 36 hours. This case illustrates that a large dose of levalbuterol administered with a metered-dose inhaler can lead to toxicity, which resolves with discontinuation of β2 agonist therapy and supportive treatment.

Background Mulibrey-Nanism (Muscle-liver-brain-eye Nanism = dwarfism; MUL) is a rare genetic syndrome. The underlying TRIM37 mutation predisposes these children to develop tumors frequently. In the largest published series of MUL, 8% patients were reported to develop Wilms tumor (WT). The published literature lacks data regarding the best treatment protocol and outcome of this cohort of children with WT and MUL. We report here a 2-year-old boy with WT and MUL and present a review of literature on WT in MUL. Case Our patient had associated cardiac problems of atrial septal defect, atrial flutter and an episode of sudden cardiac arrest. We managed him successfully with chemotherapy, surgery and multi-speciality care. He is alive and in remission at follow-up of 6 months. Conclusion A total of 14 cases (including present case) of WT have been reported in MUL and treatment details were available for six cases. They were managed primarily with surgery, chemotherapy with/without radiotherapy, and all achieved remission. The outcome data is available only for two cases, one has been followed up till 15 years post treatment for WT and other is our patient.

Scrub typhus is a vector-borne disease caused by Orientia tsutsugamushi. Clinical manifestations generally occur due to vasculitis and inflammation and can have variable degrees of systemic involvement. Meningoencephalitis and cerebellitis are well-known neurological manifestations of scrub typhus, but the occurrence of Guillain-Barré syndrome is extremely rare. The authors report a 7-y-old boy who developed fever followed by rapidly progressive ascending quadriparesis with areflexia and whose etiological workup revealed positive IgM scrub typhus antibody, as well as, a high OXK titer (1:80). Nerve-conduction studies in all four limbs were suggestive of demyelinating neuropathy. He showed complete recovery after treatment with intravenous immunoglobulin (2 g/kg) and azithromycin.

Dengue induced-hemophagocytic lymphohistiocytosis (HLH) is increasingly recognized as an important cause of secondary HLH. Early identification of dengue HLH and directed therapy for HLH may help to alter the outcomes in critically ill patients. Soluble interleukin-2 receptor (IL2R) is a useful inflammatory marker and is seen to correlate with HLH disease activity. There is scarcity of data on IL2R in pediatric dengue patients with HLH. All patients (age < 18 years) with severe dengue confirmed by positive dengue IgM ELISA admitted to PICU were retrospectively enrolled. Patientswere screened for presence of HLH according to HLH 2004 criteria. Hemogram, ferritin, fibrinogen, liver, and renal function tests were noted. Patients who met four or more HLH criteria were treated with steroids and IL2R levels were sent to confirm the diagnosis of HLH. Out of 15 patients, nine patients met the criteria of HLH. IL2R levels were high in all HLH patients (mean 51,711, range 18,000–98,715 pg/mL). Mean ferritin levels were high in the HLH group as compared to non-HLH group (mean ferritin 34,593 vs. 3,206 ng/mL; p-value 0.004). Liver dysfunction was notably higher in the HLH group compared to non-HLH group (mean alanine aminotransferase 6,621 U/L vs. 165.6 U/L; p-value 0.04, mean aspartate aminotransferase 2,145 U/L vs. 104.2 U/L; 0.04, bilirubin level 4.2 mg/dL vs. 0.7 mg/dL; p-value 0.03). Four patients in the HLH group had acute kidney injury (AKI) and two required renal replacement therapy in the form of sustained low efficiency dialysis (SLED). Requirement for invasive ventilation was exclusively seen in HLH group and three patients developed ARDS. Two patients each in HLH and non-HLH group had shock requiring vasoactive therapy in addition to fluids. Mean days of ICU and hospital stay were higher in HLH group vs. non-HLH group but not statistically significant (6.4 vs. 4.4; p-value 0.32 and 8.44 vs. 5.6; p-value 0.18 days, respectively). All children in HLH group received steroids as per HLH protocol. In the HLH group, seven survived while two died. In the non-HLH group, all five patients survived. We concluded that IL2R levels are high in dengue HLH and useful for definitive diagnosis. Early recognition of this condition in severe dengue and prompt steroid therapy improves chances of better outcome.

We present our experience with pediatric ABO-incompatible liver transplantation in India. Data of patients18 years of age undergoing ABO-incompatible liver transplantation our hospital between January, 2011 and November, 2018 were analyzed. Plasmapheresis was done pre-transplant till antibody titer was16 units. Rituximab/Intravenous immunoglobulin was used for immunosuppression, in addition to standard drugs (mycophenolate mofetil, steroids, and tacrolimus). Out of 203 patients that underwent liver transplant during this period, 8 underwent ABO-incompatible liver transplantation; 4 (3 boys) had blood group O+ve. Median (range) age was 28 (7-91) mo, PELD score was 24.5 (14-42), and pre-transplant antibody titer range was 1:32-1024. Number of plasmapheresis sessions required ranged from 1-6. Post-operatively two patients had rise in antibody titer64 requiring plasmapheresis. All 8 patients survived without rejection/biliary issues. Mean (range) of post-transplant hospital stay was 19.1 (13-22) d and follow-up period was 38.1 (7.1-84.4) mo. Pediatric ABO-incompatible liver transplantation can be successfully performed using plasmapheresis with optimal immune-suppression and vigilant post-op monitoring.

Data on pediatric patients with HPS undergoing LT are limited. Our aim was to study the spectrum and outcomes of pediatric patients with HPS undergoing LDLT. The role ofiNO for post-LDLT refractory hypoxemia was also assessed. Patients (aged < 18 years) undergoing LT were retrospectively studied. HPS was diagnosed based on European Respiratory Society Taskforce 2004 criteria. HPS was graded based on oxygenation criteria and contrast-enhanced echocardiogram. Post-operative course was studied. Refractory post-operative hypoxemia was treated with iNO by institutionally developed protocol. 23/150 pediatric patients undergoing LDLT had HPS. BA was the most common underlying cause (52.2%). By oxygenation criteria, 6 (26.1%) had VS-HPS. VS-HPS was associated with longer LOS (p = .031) and prolonged oxygen requirement (p = .001) compared with other HPS patients. 4/6 patients with VS-HPS had pO2 < 45 mm Hg. Among these, 2 developed ICH post-operatively and 1 died. 3 developed refractory post-operative hypoxemia, successfully treated with iNO. Mean duration of iNO was 26.3 days. In the group of patients with HPS, the incidence of HAT and portal vein thrombosis was 17.3% and 4.3%, respectively. One year post-LDLT survival of patients with HPS was similar to non-HPS patients (86.9% vs 94.4%; p = .88). We concluded that, pediatric patients with VS-HPS, especially those with pre-operative pO2 < 45 mm Hg, have long and difficult post-LT course. Refractory postoperative hypoxemia can be successfully overcome with strategic use of iNO. Vigilant monitoring and good intensive care support are essential.

We present our experience with pediatric ABO-incompatible liver transplantation in India. Data of patients 64 requiring plasmapheresis. All 8 patients survived without rejection/biliary issues. Mean (range) of post-transplant hospital stay was 19.1 (13-22) d and follow-up period was 38.1 (7.1-84.4) mo. Pediatric ABO-incompatible liver transplantation can be successfully performed using plasmapheresis with optimal immune-suppression and vigilant post-op monitoring.

Introduction: Sustained low-efficiency dialysis (SLED) has emerged as a cost effective alternative to Continuous Renal Replacement Therapy in the management of hemodynamically unstable adult patients with acute kidney injury. The objective of the study was to document the SLED practices in these centers, and to look at the feasibility, and tolerability of SLED in critically sick pediatric patients. Methods: It was a retrospective record review from January 2010 to June 2016 done in four tertiary pediatric nephrology centers in India. All pediatric patients undergoing SLED in the collaborating centers were included in the study. Basic demographic data, prescription parameters and outcomes of patients were recorded. Findings: During the study period a total of 68 children received 211 sessions of SLED. PRISM score at admission in patients was 13.33 ± 9.15. Fifty-seven patients were ventilated (84%). Most of the patients had one or more organ system involved in addition to renal (n = 64; 94%). Heparin free sessions were achievable in 153 sessions (72%). Out of 211 sessions, 148 sessions were on at least one inotrope (70.1%). Overall premature terminations had to be done in 27 sessions (13% of all sessions), out of which 7 sessions had to be terminated due to circuit clotting (3.3%). Intradialytic hypotension or need for inotrope escalation was seen in 31 (15%) sessions but termination of the session for drop in BP was required in only 20 (9%) sessions. Conclusion: SLED is a feasible method of providing renal replacement in critically ill pediatric patients.

Hypertension is common in hemolytic uremic syndrome (HUS) and often difficult to control. Local renin-angiotensin activation is believed to be an important part of thrombotic microangiopathy, leading to a vicious cycle of progressive renal injury and intractable hypertension. This has been demonstrated in vitro via enhanced tissue factor expression on glomerular endothelial cells which is enhanced by angiotensin II. We report two pediatric cases of atypical HUS with severe refractory malignant hypertension, in which we targeted the renin-angiotensin system by using intravenous (IV) enalaprilat, oral aliskiren, and oral enalapril with quick and dramatic response of blood pressure. Both drugs, aliskiren and IV enalaprilat, were effective in controlling hypertension refractory to multiple antihypertensive medications. These appear to be promising alternatives in the treatment of severe atypical HUS-induced hypertension and hypertensive emergency.

Much progress has been made in understanding the pathophysiology and treatment of atypical hemolytic uremic syndrome (aHUS). Plasma therapy is the mainstay of treatment for aHUS. The availability of the first effective anti-complement therapeutic agent, eculizumab, has dramatically changed the outlook of this disease. However, its use in clinical practice raises important questions, such as who should receive the drug, when to start such therapy, and is it safe to stop treatment once the disease is controlled. We describe here for the 1st time in India, use of eculizumab in a 12-year-old boy with aHUS. We also describe in this report challenges faced in procuring the drug, and an ideal, evidence-based method of treating aHUS in children.

The incidence of acute pancreatitis (AP) in children is slowly increasing. Etiological pattern of AP in children is varied and unlike adults; it mainly includes trauma, infection, medications and idiopathic causes. AP is characterised by severe abdominal pain, 3 fold elevation in amylase and/or lipase & characteristic findings on imaging. Early diagnosis is essential for appropriate management; however this is challenging especially in young and sick children where symptoms may not be forthcoming. Also overlapping symptoms and laboratory investigations in certain scenarios such as diabetic ketoacidosis and head trauma may lead to diagnostic difficulties. Regardless of etiology, the evolution of AP has 2 common phases which need to be recognised by the intensivist. These include initial phase of SIRS and subsequent phase of infectious complications; both of which can be potentially lethal. Clinical severity scoring systems used in adults are not applicable to children with AP. 7 fold rise in serum lipase appears to be an accurate predictor of severity in children. Essential to management is early aggressive fluid resuscitation (within 6-12 hours of admission) which should be guided by monitoring of various hemodynamic parameters in PICU rather than fixed fluid guidelines. Intravenous contrast enhanced CT of the abdomen is required to assess the severity of AP and extent of regional complications. It is however best delayed until hemodynamic stabilisation or for at least 48-72 hours after onset of symptoms. Pain management and early nutrition are important aspects of care along with close monitoring for development of multi-organ dysfunction. This review attempts to address AP in children from a pediatric intensivist’s perspective.

Pediatric acute liver failure (ALF) is a rare complex clinical syndrome with a fatal outcome, if not diagnosed and treated at the right time. ALF in children can be difficult to recognize and is very different from ALF in adults; in terms of definition, etiology, symptomatology and management. The causes of ALF in children represent a large heterogeneous list, which vary by age and geographical location. The management of ALF mandates multidisciplinary approach with comprehensive intensive and supportive care; which at times is complex and can be challenging to a pediatric intensivist. The key steps in management are to monitor and support affected organ systems; anticipate, identify and treat complications; and maintain optimal clinical condition till spontaneous recovery or liver transplantation. This review describes definitions, etiopathogenesis, clinical features and the most recent management strategies, including emergency liver transplantation considerations in pediatric ALF.

Objectives: We investigated the association of fluid overload and oxygenation in critically sick children, and their correlation with various outcomes (duration of ventilation, ICU stay, and mortality). We also assessed whether renal angina index (RAI) at admission can predict mortality or acute kidney injury (AKI) on day 3 after admission.Design and setting: Prospective study, pediatric intensive care in a tertiary hospital.Duration: June 2013-June 2014.Patients: Patients were included if they needed invasive mechanical ventilation for >24 h and had an indwelling arterial catheter. Patients with congenital heart disease or those who received renal replacement therapy (RRT) were excluded.Methods: Oxygenation index, fluid overload percent (daily, cumulative), RAI at admission and pediatric logistic organ dysfunction (PELOD) score were obtained in all critically ill children. KDIGO classification was used to define AKI, using both creatinine and urine output criteria. Admission data for determination of RAI included the use of vasopressors, invasive mechanical ventilation, percent fluid overload, and change in kidney function (estimated creatinine clearance). Univariable and multivariable approaches were used to assess the relations between fluid overload, oxygenation index and clinical outcomes. An RAI cutoff >8 was used to predict AKI on day 3 of admission and mortality.Results: One hundred and two patients were recruited. Fluid overload predicted oxygenation index in all patients, independent of age, gender and PELOD score (p < 0.05). Fluid overload was associated with longer duration of ventilation (p < 0.05), controlled for age, gender, and PELOD score. Day-3 AKI rates were higher in patients with a RAI of 8 or more, and higher areas under the RAI curve had better prediction rates for Day-3 AKI. An RAI

The incidence of Staphylococcus aureus infections is increasing worldwide. Apart from localized infections, it can cause life-threatening disseminated disease. Community-associated staphylococcal infections are difficult to recognize early unless there is awareness of its varied presentation. We present two previously healthy adolescent boys with methicillin-sensitive S. aureus sepsis, highlighting its invasive nature and specific treatment considerations.

Recent literature has endorsed favorable outcomes following ABOi kidney transplantation in pediatric population. Nevertheless, reluctance to pursue an ABOi still remains pervasive. This could be ascribed to various legitimate reasons, namely less extensive pediatric ABOi data, technical difficulties encountered during PP, cost restraints, and concerns regarding higher rates of antibody-mediated rejection, infectious complications, and post-transplant lymphoproliferative disorder as compared to adults. However, given the similar excellent outcomes of both ABOi and ABOc kidney transplantation, clinicians should consider this option sooner if a compatible donor or swap is not available. Here, we describe the outcomes of three pediatric ABOi performed at our institute in India (from 2014 till now), wherein distinct apheresis modalities had been employed in each desensitization protocol, and our techniques evolved with advancing science in apheresis. This case series includes India's first published pediatric ABO-incompatible transplant (Case 2) and the youngest child to undergo ABO-incompatible renal transplant in SAARC nations (Case 3).

Sustained low-efficiency dialysis (SLED) has emerged as a cost effective alternative to Continuous Renal Replacement Therapy in the management of hemodynamically unstable adult patients with acute kidney injury. The objective of the study was to document the SLED practices in these centers, and to look at the feasibility, and tolerability of SLED in critically sick pediatric patients.It was a retrospective record review from January 2010 to June 2016 done in four tertiary pediatric nephrology centers in India. All pediatric patients undergoing SLED in the collaborating centers were included in the study. Basic demographic data, prescription parameters and outcomes of patients were recorded.During the study period a total of 68 children received 211 sessions of SLED. PRISM score at admission in patients was 13.33 ± 9.15. Fifty-seven patients were ventilated (84%). Most of the patients had one or more organ system involved in addition to renal (n = 64; 94%). Heparin free sessions were achievable in 153 sessions (72%). Out of 211 sessions, 148 sessions were on at least one inotrope (70.1%). Overall premature terminations had to be done in 27 sessions (13% of all sessions), out of which 7 sessions had to be terminated due to circuit clotting (3.3%). Intradialytic hypotension or need for inotrope escalation was seen in 31 (15%) sessions but termination of the session for drop in BP was required in only 20 (9%) sessions.SLED is a feasible method of providing renal replacement in critically ill pediatric patients.