Hepatic Encephalopathy: Early Diagnosis in Pediatric Patients With Cirrhosis

How to Cite This Article: Dara N, Sayyari AA, Imanzadeh F. Hepatic Encephalopathy: Early Diagnosis in Pediatric Patients With Cirrhosis. Iran J Child Neurol. 2014 Winter; 8(1):1-11.ObjectiveAs acute liver failure (ALF) and chronic liver disease (cirrhosis) continue to increase in prevalence, we will see more cases of hepatic encephalopathy.Primary care physician are often the first to suspect it, since they are familiar with the patient’s usual physical and mental status. This serious complication typically occurs in patients with severe comorbidities and needs multidisciplinary evaluation and care. Hepatic encephalopathy should be considered in any patient with acute liver failure and cirrhosis who presents with neuropsychiatric manifestations, decrease level of consciousness (coma), change of personality, intellectualand behavioral deterioration, speech and motor dysfunction.Every cirrhotic patient may be at risk; potential precipitating factors should be addressed in regular clinic visits. The encephalopathy of liver disease may be prominent, or can be present in subtle forms, such as decline of school performance, emotional outbursts, or depression.“Subtle form” of hepatic encephalopathy may not be obvious on clinical examination, but can be detected by neurophysiologic and neuropsychiatric testing.References:Ferenci P, Lockwood A, Mullen K, Tarter R, Weissenborn K, Blei AT. 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COVID-19 pneumonia in a child with hepatic encephalopathy: A case study

Coronavirus disease 2019 (COVID-19) is caused by the seventh coronavirus, known as the severe acute respiratory syndrome coronavirus 2(SARS-CoV-2). Children often have milder diseases than adults with very rare mortality. Gastrointestinal manifestations and a mild increase in liver enzymes have been reported in 8.8% to 53% of COVID-19 cases. However, liver failure is extremely rare and has not been reported so far in the literature. The prevalence of comorbidities is not clear in children with COVID-19. Here, we reported a fatal case of simultaneous pneumonia secondary to SARS-CoV-2and acute liver failure in a 14-year-old boy with liver cirrhosis. &nbsp

High Dose Botox Injection for Patients with Internal Anal Sphincter Achalasia Persistent to Posterior Internal Anal Sphincter Myectomy

Introduction: one of the most common reported chief complaints in visits to pediatricians is constipation. Although in a majority of cases with no anatomical defect, dietary manipulation, stool softeners, and oral laxatives are successful, some patients fail to respond. Internal Anal Sphincter (IAS) Achalasia is one of the causes of constipation.It results in failure of IAS relaxation and has similar clinical presentation to Hirschsprung’s disease with absence of rectosphincteric inhibitory reflex on anorectal manometry (ARM) and presence of ganglion cells on rectal biopsy. Although posterior internal anal sphincter myectomy (ISM) is considered the standard treatment for IASA, some cases fail to respond and present with intractable constipation which may be associated with soiling. This research aims to assess the role of botox injection for treatment of patients who presented with intractable constipation and have already beentreated by posterior IAS myectomy. Materials and Methods:Internal anal sphincter Botox injection was performed (with a dose of 20 U/Kg) in 14 patients with internal anal sphincter achalasia (IASA); who had presented with intractable constipationafter being treated by posterior internal anal sphincter myectomy. Patients were followed for 2 years after injection. Result: of all 14 patients with persistent constipation (resistant to oral laxatives), 12 patients (85.7%) had regular bowel function for more than 6 months after botox injection therapy (P < 0.05). Of all 14 patients that had needed rectal enema for defecation, no patient needed rectal enema after botox injection therapy (P < 0.05). Of 5 patients with fecal soiling before botox injection therapy (4 had occasionalsoiling, and 1 had soiling every day without social problems) only a single patient experienced transient fecal soiling for 2 weeks after botox injection therapy (P < 0.05). No patient needed another botox injection in 2 years of follow up. Conclusion: IAS Botox injection therapy (BIT) was successfully used to manage IASA patients who had presented with intractable constipation after posterior myectomy. This method significantly reduces the need for laxatives and rectal enema; and improves constipation, fecal soiling and bowel movements at the same time

Autoimmune pancreatitis as a very rare cause of recurrent pancreatitis in children; a case report and review of literature

Autoimmune pancreatitis as chronic inflammation of the pancreas due to an autoimmune mechanism is a rare type of pancreatitis. A 14 years old girl presented with multiple episodes of abdominal pain, nausea with elevation of amylase and lipase suspicions of acute recurrent pancreatitis since 3 years of age. After through evaluation about secondary causes of recurrent and familial pancreatitis finally she responded to corticosteroid treatment. Although very rare but autoimmune processes should be considered in teenagers with recurrent pancreatitis

It's time to revise the role of positive D- penicillamine challenge test in diagnosis of Wilson disease

Hepatic involvement in Wilson disease consists of isolated elevated liver enzyme, asymptomatic hepatomegaly, cirrhosis and acute liver failure. Here, we report three patients with unexplained elevated liver enzymes. By considering the level of urinary excretion of copper after penicillamine challenge test, we had some problems in the process of diagnosis. Therefore, we thought of cautiously applying the diagnostic cut-off in the mentioned challenge tes

The Late presenting Bochdalek hernia; A Case report and review of literature

We report a 22-month-old boy who referred due to nausea, vomiting, abdominal pain and watery non-bloody diarrhea and after thorough evaluation, a large defect in the left postero-lateral side of diaphragm and presence of bowel loops, spleen, stomach and left lobe of liver in the left hemi-thorax were detected. So, he was operated and managed with the impression of Bochdalek hernia. We have also reviewed the similar case reports in the past 10 years, briefly, in order to map the presentations and clinical course of cases with Bochdalek hernia which were diagnosed late, for giving physicians a better insight on this issue

A Rare Case Report of Duodenal Adenocarcinoma Accompanied by Cerebellar Medulloblastoma

A case of a 6-year-old female with a diagnosis of Duodenal Adenocarcinoma is presented by the authors, in which 24 months subsequent to undergoing radiation therapy for a Cerebellar Medulloblastomas, the mentioned diagnosis has been confirmed. Although, the chance of a secondary malignancy has been recognized to be elevated in children formerly undergone radiation therapy, the incidence of duodenal adenocarcinoma is a highly infrequent condition. As a consideration, observing manifestations like weight loss, vomiting, and icterus in a pediatric patient who has been administered earlier on with radiation therapy, must alert physicians that the risk of a gastrointestinal malignancy exists

Assessment of Serum Amino Acid Chromatography in Children with Inflammatory Bowel Diseases

Background: Given the important role of amino acids in regulating many metabolic pathways of the body and considering the scarcity of markers for the diagnosis of inflammatory bowel disease (IBD) and its differentiation, we aimed to investigate the status of serum amino acids chromatography in children with IBD. Materials and Methods This case-control study was conducted among children with primary diagnosis of IBD who referred to Mofid Children's hospital in Tehran, Iran. Children with a definite diagnosis of chronic IBD on the basis of endoscopy and biopsy were enrolled. In addition, 100 children without any history of predisposing, chronic, or inflammatory disease who referred to the same hospital during the period of the study were also selected. All samples underwent serum amino acids chromatography via HPLC method. Results Of all the patients in the IBD group, 18 patients (18%) suffered from Crohn's disease and 82 patients (82%) had ulcerative colitis; the disease was active in 54 patients (54%). The results of serum amino acids chromatography showed that several amino acids were significantly higher in patients with IBD. Considering the normal serum levels of amino acids, only the levels of two amino acids of histidine and tryptophan were significantly different in the IBD group compared the control group. In total, of all the subjects, 30 children (15%) had abnormal amino acid serum chromatography; hence, its prevalence was significantly higher in the IBD group (P=0.048). Conclusion The current study showed that serum amino acid chromatography in children with IBD were different from that in healthy children. More specifically, the decrease in tryptophan level was more observed in patients with active disease

Creatinine Phosphokinase (CPK) Elevation in the Coexistence of Wilson's Disease and Autoimmune Hepatitis with Atypical Presentation: A Diagnostic Dilemma

Background: Wilson's disease (WD) is a genetic disorder with various clinical presentations due to excessive accumulation of copper in the liver and other organs. It can present as acute/chronic hepatitis, liver failure, extrahepatic and neuromuscular manifestations. Autoimmune hepatitis (AIH) is a necroinflammatory disease of the liver, which affects a lot of people particularly the children population. AIH has a broad clinical presentation that is similar to WD. Coexistence of WD with elevated creatinine phosphokinase (CPK) and AIH, may be a diagnostic dilemma. Case Report: We presented a 6 years old boy with dysarthria, aggressive behavior, weak attention, concentration and weight loss with abnormal physical examination. Laboratory, histochemical, genomic studies, muscle/liver biopsy and atomic absorption test confirmed the diagnosis of both WD and AIH in the boy. Conclusion Although CPK and liver enzyme elevation is a rare presentation of chronic hepatitis with dominant feature of WD and AIH; however, simultaneous therapy with immunosuppressive drugs and Penicillamine may have superior benefit with a significant response

Evaluation of Clarithromycin and Metronidazole Resistance of Helicobacter Pylori Infection in Symptomatic Iranian Children

Helicobacter Pylori (H. pylori) as a gram-negative bacterium is the most common infection of the gastrointestinal tract, and worldwide it affects the children over three years of age. H. pylori could cause gastrointestinal and extra-intestinal manifestations. Antibiotic resistance can happen primarily and occurs during treatment. We aimed to evaluate the resistance gene of H. pylori obtained from gastric biopsy by polymerase chain reaction (PCR) method in Iranian children over 3 years old. Materials and Methods This study was a cross-sectional to evaluate the resistance gene of H. pylori obtained from gastric biopsy by polymerase chain reaction method for metronidazole and clarithromycin in children over three years old referring to the Mofid Children's Medical Center in Tehran, Iran. Results: Finally, data from seventy-nine samples included (mean age=10.7 years and male gender = 60.8%). Beta Globulin (BG) gene were detectable in 75 (94.93%) specimens of 79 (100%). Seventeen out of 75 specimens showed positive results for molecular detection of H. pylori. The results of RFLP-PCR technique showed that mutation of RdxA gene in seven of 17 (41.1%) for Metronidazole resistance and one case of 17 (5.8%) mutation of 23Y RNA gene that leads to clarithromycin resistance. Conclusion: Regarding the results of our study, it is better to check microbial resistance by culture and antibiogram for the antibiotic regimen of the first and second line of H. pylori treatment in children