Efficacy of therapeutic plasma exchange in pediatric cases of acute liver failure as an extracorporeal liver support system.

BACKGROUND: Acute liver failure in the pediatric population is often accompanied by deranged metabolism, severe encephalopathy and coagulopathy. A liver transplant is the most viable option for the management of such patients. Therapeutic plasma exchange (TPE) is helpful in improving the liver biochemistry profile, thereby, increasing their likelihood of undergoing a liver transplant METHOD: The study was conducted over a period of 3 years (January 2018 to December 2021). Indications mainly consisted of ALF with hepatic encephalopathy, worsening liver parameters in spite of medical management, and candidacy for undergoing a liver transplant. Plasma exchange was performed daily or alternatively until the patient recovered, succumbed, or was stable enough to undergo a transplant. Biochemical parameters serum bilirubin, ALT, AST serum ammonia serum urea, serum creatinine were recorded before and after TPE sessions. RESULTS: The study group comprised 14 patients of which a total of 28 TPE was performed. There were a total of 5 cases of cryptogenic ALF, 4 of Wilson disease, 2 cases each of infection-related ALF and autoimmune hepatitis, and a single case of drug-induced hepatitis. A total of 5 out of 14 patients underwent a liver transplant and amongst the 9 who did not undergo a transplant, 4 patients expired due to septic shock syndrome; the remaining 5 were discharged in a stable condition following TPE sessions. The disease-free survival was 78.9% and the transplant-free survival was 35.71%. CONCLUSION: TPE plays a crucial role in improving the biochemistry profile of the liver in children with liver failure.

Dominant Fronto-temporal Lobectomy for Refractory Intracranial Hypertension following an Acute Arterial Ischemic Stroke in a Child.

Fronto-temporal lobectomy for refractory intracranial hypertension following an acute arterial ischemic stroke in a child is rarely performed following failed conventional measures including decompressive craniectomy. We present a case of a 10-year-old child who presented with acute ischemic stroke with intractable cerebral edema and failed conventional measures including decompressive craniectomy and had significant neurological recovery following frontotemporal lobectomy.

Acute Liver Failure in Dengue: A Common but Overlooked Entity in Pediatric Patients in Tropical Countries.

OBJECTIVE: The objective of the study is to estimate the incidence of acute liver failure (ALF) in dengue infection, understand the demographic and biochemical profiles, and identify prognostic factors associated with mortality. METHODOLOGY: This is a retrospective observational study. We evaluated the data of all pediatric dengue patients admitted over the last 5 years in our hospital to identify patients who fulfilled the criteria for pediatric ALF. Demographic profile, and biochemical and radiological parameters were assessed. Their outcomes and mortality data were analyzed to identify prognostic factors. RESULTS: Thirty children with dengue infection were identified to have developed a during the ALF study period which was 29.1% (30 of 103) of all our ALF admissions. A total of 189 children with dengue infection needed admission during the same period and 15.8% (30 of 189) of them developed ALF. The mean duration of onset of ALF was 5.4 days after fever onset. Twenty-two patients (73%) survived, and 8 patients expired. High creatinine, low albumin level, and multisystemic involvement were identified as poor prognostic markers in those patients who did not survive. CONCLUSION: ALF is common in admitted severe dengue patients. A significant proportion of acute liver patients in endemic countries can be attributed to dengue infection. Low serum albumin, high creatinine, and multi-organ dysfunction during acute illness can be used as prognostic markers in these children. Multicentric prospective studies are needed to validate these results.

COVID-19-associated Multisystem Inflammatory Syndrome in Children: A Multicentric Retrospective Cohort Study.

BACKGROUND: Multisystem inflammatory syndrome in children (MIS-C) associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is a new entity affecting a small percentage of children during the COVID-19 pandemic. MATERIALS AND METHODS: Demography, clinical, and laboratory variables of children admitted from April to September 2020 with MIS-C were studied retrospectively at eight hospitals in Delhi, India. RESULTS: We identified 120 patients [median age: 7 years (interquartile range (IQR): 4-10)] with male-to-female ratio of 2.3:1. Overall, 73 out of 120 children (60.8%) presented with shock, 63 (52.5%) required inopressor support, and 51 (43%) required respiratory support. We categorized the cohort into three observed clinical phenotypes: MIS-C with shock (n = 63), MIS-C with Kawasaki disease (KD) (n = 23), and MIS-C without shock and KD (n = 34). Atypical presentations were hypothermia, orchitis, meningoencephalitis, demyelination, polyneuropathy, pancreatitis, and appendicitis. Ninety-four percent had laboratory evidence of SARS-CoV-2 (78.3%, seropositive and 15.8%, RT-PCR positive). The median C-reactive protein (CRP) was 136 mg/L (IQR, 63.5-212.5) and ferritin was 543 ng/mL (IQR, 225-1,127). More than 90% received immunomodulatory therapy (intravenous immunoglobulins and/or steroids) with an excellent outcome (96% survived). CRP and absolute neutrophil count (ANC) were correlated statistically with severity. CONCLUSION: MIS-C data from Delhi are presented. Rising CRP and ANC predict the severe MIS-C. HOW TO CITE THIS ARTICLE: Mehra B, Pandey M, Gupta D, Oberoi T, Jerath N, Sharma R, et al. COVID-19-associated Multisystem Inflammatory Syndrome in Children: A Multicentric Retrospective Cohort Study. Indian J Crit Care Med 2021;25(10):1176-1182.

Viral Bronchiolitis in Children: Less is More.

Jerath N. Viral Bronchiolitis in Children: Less is More. Indian J Crit Care Med 2021;25(11):1219-1220.

Severe life-threatening hypersensitivity reaction to polidocanol in a case of recurrent aneurysmal bone cyst.

Aneurysmal bone cysts (ABC) are expansile lytic lesions constituting around 1% of all benign bone tumors with an annual incidence of 1.4/100000. A variety of treatments are available ranging from curettage with or without bone grafting (autologous or allogeneic), curettage with use of adjuvants [Polymethylmethacrylate (PMMA) bone cement, high speed burr, phenol, liquid nitrogen], wide en-block excision with or without reconstruction, selective arterial embolization of the feeding vessels, radiation therapy, high precision megavoltage radiotherapy and percutaneous radio-nuclide ablation, sclerotherapy (ethibloc, aetoxisclerol, alcohol gel, polidocanol). The optimal treatment is debatable due to various indications and contraindications of different modalities of treatment. Recent data suggest that percutaneous sclerotherapy with polidocanol is safe and effective alternative to surgery for treatment of ABCs as it has minimal side effects. We are reporting the first case of life-threatening adverse reaction to intra-lesional polidocanol in a three-year-old boy with a proximal femoral aneurysmal bone cyst. The importance of reporting this case is to make people aware regarding the adverse reaction of polidocanol and to highlight the precautions one should follow while using polidocanol for aneurysmal bone cysts.

Endoscopic esophageal substitution for pure esophageal atresia and wide gap esophageal atresia: A report of five cases with minimum follow-up of twelve months.

AIM: The aim of the study is to report feasibility and safety of endoscopic esophageal substitution in infants with pure esophageal atresia and wide gap tracheoesophageal fistula with a minimum one year follow-up. MATERIALS AND METHODS: This prospective study was conducted from January 2012 for twenty four consecutive months at Apollo Hospital, New Delhi. All babies either followed up or referred for esophageal substitution without any history of mediastinitis or associated major congenital anomaly and weighing greater than 6kg were to be included in the study. The indication, intraoperative details, operative approach, conversion to open, esophageal substitute, postoperative ventilation, ICU and hospital stay, time to solid foods, morbidity and mortality were recorded. Informed consent was obtained from all the parents and ethical clearance was obtained for the study from the hospital ethical committee. Postoperatively babies were followed up monthly for first six months, 3 monthly for next six months and annually thereafter. RESULTS: Between January 2012 and December 2013, in the two year period six infants were admitted for laparoscopic gastric transposition. In five patients the procedure was completed by the laparoscopic approach and one required conversion to open surgery owing to dense adhesions. The age range at the time of surgery was from 8months to 12months with a mean age of 10months. Four patients had pure esophageal atresia (type A) and two had wide gap esophageal atresia with distal tracheoesophageal atresia (type C). Five had primary esophagostomy and gastrostomy as a newborn, the sixth had postoperative anastomotic leak and required subsequent diversion. The mean operating time was 194minutes (range 170-210minutes). The mean stay in ICU was 7days with a range of 4-12days. All patients were ventilated in the postoperative period for an average of 5days with a range of 4-7days. One patient had prolonged gastric ileus which delayed the oral feeds by 14days. The mean time to start the oral feeds was 8days with a range of 6-14days. The mean hospital stay was 19.6days (range 16-23days). Early complications were pneumonia and pleural effusion in one patient. One patient developed anastomotic stricture which was amenable to dilatation. One patient had leak from esophagogastric anastomosis which healed spontaneously. All children are now orally fed, swallow without difficulty, and parents report an excellent cosmetic outcome. The follow-up ranges from 12 to 36months. CONCLUSION: The initial results of endoscopic esophageal substitution are encouraging and easily comparable to the outcome of open surgery with all the attendant benefits of minimally invasive approach.

Living Related Liver Transplantation for Biliary Atresia in the Last 5 years: Experience from the First Liver Transplant Program in India.

OBJECTIVE: To study the clinical, biochemical profile and outcome of patients with biliary atresia (BA) who underwent living related liver transplantation (LRLT) at authors' institute in the last 5 y (2008-2013). METHODS: Case records of the 20 patients diagnosed with biliary atresia who had undergone living related liver transplantation at authors' centre in the last 5 y were analysed. RESULTS: Eighteen patients with BA with a failed Kasai procedure and 2 without a prior Kasai's portoenterostomy received a liver transplant. At a median follow up of 2 y and 6 mo, both the patient and graft survival rates were 90 %. The median age of the recipients at the time of LRLT was 8 mo and 12 (60 %) of the transplanted children were less than or equal to 1 y of age. The male-female ratio was 1.8:1. The median weight was 7.3 kg (5.8-48 kg); two thirds were less than 10 kg. The median pre-transplant total serum bilirubin (TSB) and international normalized ratio (INR) were 12.98 (0.5-48.3) mg/dl and 1.3 (1.0-3.9) respectively. All patients received a living related graft and there was no donor mortality. The median duration of postoperative ventilation was 14 h. The post-operative complications were infection (30 %), vascular complications (20 %) and acute rejection (20 %). The median duration of postoperative hospital stay was 21 d (17-42). Two patients died of combined hepatic and portal vein thrombosis in the early postoperative period. Late rejection was encountered in 1 patient and another developed chronic kidney disease necessitating a renal transplant. There were no late vascular occlusions or development of post transplant lymphoproliferative disease. CONCLUSIONS: Thus, LRLT for BA with or without a prior portoenterostomy, is a feasible and successful treatment modality with good outcomes attained despite the challenges of age and size. This treatment modality is now well established in India.

Experience of 100 solid organ transplants over a five-yr period from the first successful pediatric multi-organ transplant program in India.

To analyze the clinical profile and outcome of pediatric patients who had undergone a liver and/or RT at our center over a five yr period, case records of all the patients who had undergone a liver or RT were analyzed retrospectively. One hundred solid organ transplants were performed at our center between January 2007 and January 2012. These included 50 liver, 44 renal, one sequential liver and renal, and two CLKT. BA was the most common indication for an LT (38%). At a median follow-up of two yr three months, the patient survival was 88%. The most common indication for an RT was chronic glomerulonephritis (54.5%). At a median follow-up of three yr, the survival was 91%. The CLKT were performed for hyperoxaluria. Two yr post LT, a sequential RT was performed for ESRD resulting from transplant associated microangiopathy. All patients received a living related graft. The common post-operative complications were infections, vascular complications, and graft dysfunction. Survival rates for liver and RT at our center are comparable to those in the established centers in the West.

Outcome of live donor liver transplantation in Indian children with bodyweight <7.5 kg.

This case-series analyzed the outcome of live donor liver transplantation (LT) performed in children <7.5 kg from January 2008 to June 2009 at our center. Five patients (3 males, 2 females, mean age, 8.2 ± .4 months; mean weight 6.8 ± 0.4 kg) underwent LT. The indications of LT included biliary atresia (3) and idiopathic neonatal hepatitis (2). Postoperative complications included acute rejection (1), portal venous thrombosis (1), bile leak (1), severe hypertension (1) and bacterial sepsis (4). There were no donor related complications. The median follow-up duration is 11 months with patient and graft survival rates of 100% each, respectively.

Bilateral lung abscesses in a 9-month-old healthy infant.

Multiple lung abscesses are extremely rare in healthy children. We report a case of polymicrobial bilateral lung abscess in a 9-month-old previously well infant presenting with a short history of fever and respiratory distress. The management options and outcome are discussed.

Clinical impact of vasopressin infusion on hemodynamics, liver and renal function in pediatric patients.

OBJECTIVE: To study effects of vasopressin on hemodynamic, clinical, and laboratory variables in children with advanced vasodilatory shock. DESIGN AND SETTING: Retrospective study in a multidisciplinary tertiary pediatric critical care unit. PATIENTS AND PARTICIPANTS: Patients (n = 117; 32 noncardiac, 85 postcardiac surgery) requiring intravenous vasopressin infusion longer than 60 min for advanced shock (January 2004 to December 2005). INTERVENTIONS: Vasopressin infusion (n =157). MEASUREMENTS AND RESULTS: Both cardiac and noncardiac patients showed a significant decrease in inotrope requirement without change in central venous saturation or lactate during infusion. Both groups had increased urea and creatinine and decreased urine output with longer duration/higher cumulative dose of vasopressin. There was a significant increase in conjugated bilirubin level in the noncardiac group during vasopressin infusion; noncardiac patients showed higher AST levels with higher cumulative dose or longer duration of infusion. Postcardiac surgical patients showed a trend towards normal INR values which persisted after vasopressin infusion. Platelet counts were significantly lower during infusion in both groups. CONCLUSIONS: Vasopressin infusion improved the hemodynamic state in advanced shock without compromising cardiac function. Urine output and creatinine levels were adversely affected but were reversible. This effect was more pronounced with higher dose or duration of infusion. There was no major effect on liver function but a significant reduction in platelet counts. These data suggest that vasopressin is useful in states of vasodilatory shock with limitations regarding to its adverse renal effects and on platelet counts.

Redefining the management of pediatric tonsillopharyngitis with cefprozil.

Tonsillopharyngitis is very common in children, with Group A Streptococci being the most common bacterial etiology. Effective antibacterial treatment is imperative due to risk of rheumatic fever. Cephalosporins have been used successfully for the treatment of Group A beta-hemolytic Streptococcal (GABHS) tonsillopharyngitis. Cefprozil is a novel broad-spectrum oral cephalosporin. Cefprozil is rapidly absorbed from the gastrointestinal tract with high bioavailability. The excellent penetration of cefprozil into tonsillar and adenoidal tissue corresponds well with the clinical outcome. The drug provides excellent coverage against both gram-negative and -positive bacteria that may cause pharyngitis/tonsillitis. The beta-lactamase stability of cefprozil appears to exceed that of other oral cephalosporins for important gram negative pathogens. In clinical trials, cefprozil appears to be at least as effective as commonly used comparison agents such as cefaclor and cefixime. Additionally, cefprozil is better tolerated than the latter, especially with regard to gastrointestinal adverse effects. Thus cefprozil can be considered a safe and reliable drug for the treatment of Streptococcal tonsillopharyngitis in children.