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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.

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.

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 concept of intra-abdominal hypertension and abdominal compartment syndrome is relatively new, but increasing awareness and evolving management strategies have been instrumental in reducing mortality by nearly half in critically ill patients with this condition. The thresholds for intraabdominal hypertension and abdominal compartment syndrome in children are different from adults. When intraabdominal pressure increases, it affects both abdominal and extra-abdominal organs. This needs to be recognised, the vicious cycle of poor perfusion and increasing intraabdominal pressure which ultimately leads to ischemic cellular necrosis. Focus has gradually shifted from treatment to prevention of progress of intraabdominal hypertension. Various non invasive methods can be used effectively to decrease intra-abdominal pressure, but surgical decompression is essential when organ dysfunction and abdominal compartment syndrome set in. This review describes the definitions, risk factors, pathophysiology and management strategies for increased intra-abdominal pressure 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.

The severe acute respiratory syndrome coronavirus 2 (SARS-CoV2) pandemic has brought the world to a standstill and is the largest public health crisis in the world in the present generation. As the cases continue to increase globally, and more patients are developing severe disease, large volumes of clinical data collection and aggressive research is being carried out to find effective medical therapies for this disease. No definitive proven treatment option exists till date. Various immunomodulatory and anti-viral drugs have shown potential, and are being studied extensively through randomized trials. The most available literature is based on the adult study population, with few/no children being included. This review attempts to summarize the pharmacotherapeutic options presently in consideration in children in the treatment of SARS-CoV2.

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).

Background In critically sick adults, sustained low efficiency dialysis [SLED] appears to be better tolerated hemodynamically and outcomes seem to be comparable to CRRT. However, there is paucity of data in critically sick children. In children, two recent studies from Taiwan (n = 11) and India (n = 68) showed benefits of SLED in critically sick children. Aims and objectives The objective of the study was to look at the feasibility and tolerability of sustained low efficiency daily dialysis-filtration [SLEDD-f] in critically sick pediatric patients. Material and methods Design: Retrospective study Inclusion criteria: All pediatric patients who had undergone heparin free SLEDD-f from January 2012 to October 2017. Measurements: Data collected included demographic details, vital signs, PRISM III at admission, ventilator parameters (where applicable), number of inotropes, blood gas and electrolytes before, during, and on conclusion of SLED therapy. Technical information was gathered regarding SLEDD-f prescription and complications. Results Between 2012–2017, a total of 242 sessions of SLEDD-f were performed on 70 patients, out of which 40 children survived. The median age of patients in years was 12 (range 0.8–17 years), and the median weight was 39 kg (range 8.5–66 kg). The mean PRISM score at admission was 8.77±7.22. SLEDD-f sessions were well tolerated, with marked improvement in fluid status and acidosis. Premature terminations had to be done in 23 (9.5%) of the sessions. There were 21 sessions (8.6%) terminated due to hypotension and 2 sessions (0.8%) terminated due to circuit clotting. Post- SLEDD-f hypocalcemia occurred in 15 sessions (6.2%), post- SLEDD-f hypophosphatemia occurred in 1 session (0.4%), and post- SLEDD-f hypokalemia occurred in 17 sessions (7.0%). Conclusions This study is the largest compiled data on pediatric SLEDD-f use in critically ill patients. Our study confirms the feasibility of heparin free SLEDD-f in a larger pediatric population, and even in children weighing

Pediatric liver transplantation has evolved over the last few decades from an experimental therapy to standard of care in end stage liver disease. Apart from surgical expertise, the success of liver transplantation largely depends on perioperative care of the patient. Intensive care management plays a key role in supporting the pre-operative sick patient through the surgery and till the new liver graft starts to function and there is resolution of pre-operative morbidities. Pediatric patients are more challenging than the adult counterparts and demand minute attention to fluid volume status, hemodynamics and various organ-specific and graft specific issues. This review describes the various principles and challenges in the post-operative intensive care management of pediatric patients undergoing liver transplantation.

Pediatric liver transplantation both in India and across the world is on the rise. This is due to better advances in both medical and surgical techniques and also better recognition and understanding of diseases that benefit from liver transplantation. The indications of liver transplantation in children differ from adults. Biliary atresia is the leading indication for pediatric liver transplantation throughout the world. Various metabolic disorders are now clearly identified as conditions that will benefit from liver transplantation. Apart from being a life saving measure, liver transplantation in children has evolved to a therapeutic step which improves quality of life. Hence timely recognition and preparation for transplant is essential before irreversible consequences of the primary disease set in. This review describes the various indications and unique aspects to be considered while planning liver transplantation in a pediatric patient.

Craniopharyngioma is associated with a wide and interesting variety of sodium states both by itself and following surgical resection. These are often challenging to diagnose, especially given their dynamic nature during the perioperative course. We present the case of a boy with craniopharyngioma who had hyponatremia due to cerebral salt wasting preoperatively, developed diabetes insipidus (DI) intraoperatively and proceeded to develop hypernatremia with adipsic DI.Cerebral salt wasting is a rare presenting feature of craniopharyngioma. Postoperative DI can be associated with thirst abnormalities including adipsia due to hypothalamic damage; careful monitoring and a high index of suspicion are required for its detection. Adipsic DI is a difficult condition to manage; hence a conservative surgical approach is suggested.

There is no firm evidence that any one catecholamine is more effective or safer than other in the treatment of shock. The choice of an agent depends on the underlying hemodynamic state. Understanding the pathophysiology, metabolic effects and altered responsiveness to catecholamines during critical illness is useful for management of the same. Titration of catecholamine is essential to avoid harmful side-effects.

A cu te Li ve r Fa ilu re from malnutrition, a host factor known to cause reduced immunocompetence leading to greater risk of multiple viral infections. Aims: To evaluate the role of maternal nutritional factors in acute viral hepatitis (AVH) and acute liver failure (ALF) patients with HEV infection during pregnancy. Methods: The study included 180 pregnant cases of which 72 HEV infected pregnant cases, 78 Non-HEV pregnant cases and 30 control cases were recruited in the study during February 2011 to December 2012. Serological evaluation of virological markers like IgM anti-HAV, HBsAg, anti-HCV Ab and IgM anti-HEV was done by ELISA. Nutritional factors were evaluated by measuring the anthropometric parameters such as body mass index (BMI), height, weight, triceps skin fold thickness, mid arm circumference (MAC), arm span using appropriate readings. Results: The meanmaternal age, gestational age at delivery and hemoglobin levels of HEV infected pregnant patients were 24.32+4.25years, 33.41+3.94weeks and 10.4+1.87 g/ dl respectively. BMI, mid arm circumference and tricep skin fold thickness were found to be significantly lower in ALF pregnant patients compared to their AVH counterparts in the HEV group (P