Background. Infantile cholestasis continues to represent a diagnostic challenge. It is very impor... more Background. Infantile cholestasis continues to represent a diagnostic challenge. It is very important to diagnose surgically correctable disorders, such as biliary atresia, in a timely manner to prevent progressive damage to the liver. It has been recently suggested that the triangular cord (TC) sign is a simple and useful tool in the diagnosis of biliary atresia. Methods. We prospectively studied 65 infants presenting with conjugated hyperbilirubinemia (age range: 32–161 days). All patients underwent ultrasonographic examination with a 7.0-MHz transducer (Acuson, Mountain View, CA). The TC was defined as a triangular, or tubular, echogenic density seen immediately cranial to the portal vein bifurcation. Results. The TC sign was identified in 25 infants, and all of them had histologic features suggestive of biliary atresia; the diagnosis was confirmed at surgery by gross morphology of hepatobiliary system, and liver biopsy, with or without intraoperative cholangiogram. Among the 40 patients who did not have the TC sign, 6 had paucity of the intrahepatic bile ducts. Three had alph-1-antitrypsin deficiency, and 31 had neonatal hepatitis. None of the 40 patients who did not have the TC sign developed acholic stools. Seven patients with biliary atresia were followed by ultrasonographic examination for 6 months after the Kasai procedure. The TC sign disappeared in all patients after the surgery; however, the TC sign reappeared in 3 patients who developed progressive cholestasis after the procedure. Conclusion. The TC sign is a simple, timesaving, and reliable diagnostic tool in the evaluation of infants with infantile cholestasis. The TC sign may also prove to be helpful in following patients after hepatoportoenterostomy. We suggest a new diagnostic strategy for patients suspected to have biliary atresia. When the TC sign is visualized, the patient should undergo intraoperative cholangiogram to confirm the diagnosis of biliary atresia, reserving percutaneous liver biopsy for those patients in whom the TC sign could not be detected.
Background: Ursodeoxycholic acid is a bile acid that was found to increase bile flow, protect hep... more Background: Ursodeoxycholic acid is a bile acid that was found to increase bile flow, protect hepatocytes, and dissolve gallstones. Purpose: The objective of this study is to review ursodeoxycholic acid in infants and children with extrahepatic biliary atresia. Methods: We used a statistical analysis of data of records of infants and children having extrahepatic biliary atresia who underwent Kasai portoenterostomy and attended Hepatology Clinic, New Children's Hospital, Cairo University, Egypt, from May 1985 until June 2005. Results: Of 141 infants with extrahepatic biliary atresia, 108 received ursodeoxycholic acid for mean duration ± SD of 252.6 ± 544.9 days in a dosage of 20 mg/kg per day. The outcome of infants who did not receive ursodeoxycholic acid and those who did was the following: 8 (24.2%) and 11 (10.18%) had a successful outcome (P = .043), 0 (0%) and 7 (6.4%) improved (P = .148), 25 (75.7%) and 84 (77.7%) had a failed outcome (P = .489), and none vs 5 died (4.6%) (P = .135), respectively. The predictors of successful outcomes were age less than 65 days at portoenterostomy (P = .008) and absence of ursodeoxycholic acid intake (P = .04) with a likelihood of a successful outcome that was 2.8, that associated with ursodeoxycholic acid intake. Conclusion: In this cohort of infants with extrahepatic biliary atresia, ursodeoxycholic acid was not shown to be effective, and its use was associated with a plethora of hepatic and extrahepatic complications.
Biliary atresia (BA) is the most common indication for liver transplantation among children globa... more Biliary atresia (BA) is the most common indication for liver transplantation among children globally (1). This Research Topic aimed to shed light on the BA possible etiology(s), pathogenesis, underlying genetic susceptibility, screening, diagnosis, and challenges that need to be addressed for its elimination and eradication.
Journal of the Egyptian Society of Parasitology, 2018
Parasitic infections in children with liver diseases could be a serious health problem. This stud... more Parasitic infections in children with liver diseases could be a serious health problem. This study documented a true prevalence of intestinal parasitosis in children with pediatric liver diseases and estimate risk factors for parasitic infection. Faecal samples were collected from 95 children suffering liver diseases. Samples were examined microscopically for parasitic stages and coproscopy of acid fast (AF) stained fecal smear for Cryptosporidium oocysts, by ELISA for E. histolytica and Giardia and by PCR genotyping for E. histolytica and Cryptosporidium spp. copro-DNA. G. intestinalis was the prevailing protozoa (14.7%) followed by Hymenolepis nana (8.4%) and C. hominis (4.2%). The pathogenic E. histolytica was not detected by genetic differentiation or ELISA. Only age group (P value .004) was a risk factor for intestinal parasitosis among these children. Giardiasis, hymenolepiasis nana and cryptosporidiosis are the most prevailing parasites in children with liver disease that phy...
Background: Cardiopulmonary surgery is associated with risk of liver dysfunction due to hypoperfu... more Background: Cardiopulmonary surgery is associated with risk of liver dysfunction due to hypoperfusion, release of mediators by macrophages and drug induced liver injury. Aim of the work: to assess liver functions and sonographic changes post-cardiopulmonary bypass in children. Methods: Fifty children with congenital heart diseases who underwent cardiac surgery were assessed. Liver function tests, abdominal ultrasound and echocardiography were performed for those with impaired liver functions at 6 months and up to 18months postoperatively. Results: Among 50 studied children (30 males and 20 females); 40 underwent cardiopulmonary bypass (CPB) and 10 underwent closed heart surgery. Twelve (24%) patients had elevated liver transaminases (aspartate transaminase (AST) and alanine transaminase (ALT)). They all proved to have anti-hepatitis C virus (HCV) antibodies and positive HCV by polymerase chain reaction (PCR). Brightness of liver (20 cases), hepatomegaly (12 cases) and congested hepatic veins (10 cases) were the most important sonographic findings. Significant relation was found between elevated liver enzymes and HCV (p=0.012). There was no correlation between CPB or congenital structural defect or surgical procedure and hepatic affection (p>0.05). Conclusion: Open heart surgery may be associated with increased risk of hepatitis C infection. Increased liver brightness is the most common sonographic finding. Pre-cardiac surgery screening for HCV is mandatory in children.
Introduction: Contemporary stethoscope has limitations in diagnosis of chest conditions, necessit... more Introduction: Contemporary stethoscope has limitations in diagnosis of chest conditions, necessitating further imaging modalities. Methods: We created 2 diagnostic computer aided non-invasive machine-learning models to recognize chest sounds. Model A was interpreter independent based on hidden markov model and mel frequency cepstral coefficient (MFCC). Model B was based on MFCC, hidden markov model, and chest sound wave image interpreter dependent analysis (phonopulmonography (PPG)). Results: We studied 464 records of actual chest sounds belonging to 116 children diagnosed by clinicians and confirmed by other imaging diagnostic modalities. Model A had 96.7% overall correct classification rate (CCR), 100% sensitivity and 100% specificity in discrimination between normal and abnormal sounds. CCR was 100% for normal vesicular sounds, crepitations 89.1%, wheezes 97.6%, and bronchial breathing 100%. Model B's CCR was 100% for normal vesicular sounds, crepitations 97.3%, wheezes 97.6%, and bronchial breathing 100%. The overall CCR was 98.7%, sensitivity and specificity were 100%. Conclusion: Both models demonstrated very high precision in the diagnosis of chest conditions and in differentiating normal from abnormal chest sounds irrespective of operator expertise. Incorporation of computer-aided models in stethoscopes promises prompt, precise, accurate, cost-effective, non-invasive, operator independent, objective diagnosis of chest conditions and reduces number of unnecessary imaging studies.
Biliary atresia (BA) is the most common indication for pediatric liver transplantation. We descri... more Biliary atresia (BA) is the most common indication for pediatric liver transplantation. We describe The BA variant: Kotb disease. Liver tissue in the Kotb disease BA is massively damaged by congenital aflatoxicosis resulting in inflammation, adhesions, fibrosis, bile duct proliferation, scarring, cholestasis, focal syncytial giant cell transformation, and typical immune response involving infiltration by CD4+, CD8+, CD68+, CD14+, neutrophil infiltration, neutrophil elastase spill, heavy loads of aflatoxin B1, accelerated cirrhosis, disruption of p53 and GSTPi, and have null glutathione S transferase M1 (GSTM1). All their mothers are heterozygous for GSTM1. This inability to detoxify aflatoxicosis results in progressive inflammatory adhesions and obliterative cholangiopathy early in life. The typical disruption of both p53 and GSTPi causes loss of fidelity of hepatic regeneration. Hence, regeneration in Kotb disease BA typically promotes accelerated cirrhosis. The immune response in Kotb disease BA is for damage control and initiation of regeneration, yet, this friendly fire incurs massive structural collateral damage. The Kotb disease BA is about actual ongoing hepatic entrapment of aflatoxins with lack of ability of safe disposal due to child detoxification-genomics disarray. The Kotb disease BA is a product of the interaction of persistent congenital aflatoxicosis, genetic lack of GSTM1 detoxification, ontogenically impaired activity of other hepatic detoxification, massive neutrophil-elastase, immune-induced damage, and disturbed regeneration. Ante-natal and neonatal screening for aflatoxicosis, avoiding cord milking, and stringent control of aflatoxicosis content of human, poultry and live-stock feeds might prove effective for prevention, prompt diagnosis and management based on our recent understanding of its patho-genomics.
Epidermoid cysts are encapsulated sebaceous cysts that contain keratin. They present at any age, ... more Epidermoid cysts are encapsulated sebaceous cysts that contain keratin. They present at any age, yet they are commoner in adults. They mainly affect face, neck, trunk and to a lesser extent groin, but can potentially arise as a subepidermal nodule anywhere on the body. They develop from the follicular infundibulum, and are different from dermoid cysts as epidermoid cysts do not contain other adnexal structures of embryonic origin. Epidermoid cysts are mostly silent but can rupture, get inflamed, become infected or develop daughter cysts. Very rarely epidermoid cysts develop in proximity to testicles. They can be mistaken for a testicular tumor. The preoperative diagnosis is essential to avoid unindicated testicular surgery. Magnetic resonance imaging (MRI) is vital for diagnosis. We report a 6-year-old child with a long standing painless scrotal swelling thought by the parents to be polyorchidism. They were alarmed by its recent rapid growth. By examination it was a median raphe cyst. Pre-operative imaging and operative surgical removal proved it to be an inflamed epidermoid cyst. The surgery was uncomplicated and the child had an uneventful recovery. Testicular epidermoid cysts in children are exceptionally rare, yet they can present as median raphe cysts. Inflammation within the epidermoid cyst presents clinically as rapid painless growth. Clinical examination, high index of suspicion and pre-operative MRI avoid unnecessary testicular surgery.
An Amyand hernia is defined as when the appendix is trapped within the sac of an inguinal hernia ... more An Amyand hernia is defined as when the appendix is trapped within the sac of an inguinal hernia through a patent vaginal process. It was first described by Claudius Amyand, in 1735 during an appendectomy of a perforated appendix inside a hernial sac of an 11-year old child. Amyand hernia rare, constituting less than 1% of inguinal hernias. Amyand hernia is 3 times more likely to be diagnosed in children compared to adults due to the patency of vaginal process. Appendicitis or its perforation in Amyand hernia, complicate 0.1% of cases. Other variants include the presence of ileum, caecum or both in addition to the appendix. Isolated acute appendicitis in neonates is extremely rare, with a high mortality rate, and higher among those within Amyand hernia up to 30%. Perforated appendix in Amyand hernia is difficult to diagnose, as its clinical picture is not specific. We report a neonate 26 days old with perforated appendix in an Amyand inguinal hernia who presented by a picture of obstructed oblique inguinal hernia. He underwent emergency open inguinal exploration, herniotomy and appendicectomy. Despite the high mortality rate of Amyand hernia due to be peritoneal spread of sepsis, the boy survived complication free. Perforated appendix in Amyand hernia is exceptionally rare, and can present in the early neonatal period, its diagnosis and outcome rely on high index of suspicion and prompt surgical intervention.
Background and objectives: Liver damage in cholestasis is multifactorial, yet bile acid-mediated ... more Background and objectives: Liver damage in cholestasis is multifactorial, yet bile acid-mediated hepatotoxicity is pivotal. Honey consumption has many physiological effects; it influences detoxification process (phase I, II and III), has antioxidant, anti-inflammatory, immune-stimulant, anti-ulcer, wound/burn healing effects and others. The bile acid ursodeoxycholic acid (UDCA) is currently used off-label to treat neonatal cholestasis. This study aimed to assess the effectiveness of spore-free natural honey to treat neonatal nonobstructive cholestasis. Methods: Thirty infants with cholestasis received spore-free natural honey. Their progression was compared to a control group with cholestasis (28 infants) and a historical group (31 infants) on UDCA. Results: The mean ± standard deviation (SD) follow-up duration was 29.68 ± 18.68 months. At presentation, the total bilirubin concentrations were 9.5 ± 5.9 mg/dL, 10.9 ± 7 mg/dL and 14 ± 9 mg/dL in the honey, control and UDCA groups, respectively (p = 0.064), and their direct bilirubin concentrations were 6 ± 4 mg/dL, 6.7 ± 4 mg/dL and 8.4 ± 6.9 mg/dL, respectively (p = 0.169). The final total bilirubin concentrations were 1.2 ± 1.7 mg/dL, 4.6 ± 8.2 mg/dL and 7.3 ± 8.9 mg/dL, (p = 0.006), and their final direct bilirubin concentrations were 0.8 ± 1 mg/dL, 2.77 ± 5.2 mg/dL and 5.1 ± 7.2 mg/dL (p = 0.008), with cure achievement in 25, 16 and 16 (p = 0.023), improvement in 3, 5 and 3 (p = 0.565), failure in 2, 3 and 10 (p = 0.016), and death in 0, 4 and 2, respectively (p = 0.095). None suffered from botulism or flaccid paralysis. Conclusions: Spore-free honey is effective in clearing cholestasis in neonates and infants. UDCA use in cholestasis in pediatric age should be abandoned as it is less effective and is associated with a worse outcome.
Advances in Intelligent Systems and Computing, 2018
Heart sounds recognition is essential for heart defects diagnosis. Diagnosis of structural heart ... more Heart sounds recognition is essential for heart defects diagnosis. Diagnosis of structural heart defects is not always possible using the contemporary stethoscope and need further assessment using high cost devices such as X-ray, electrocardiogram (ECG), echocardiography (ECHO) and computed tomography (CT)). Automatic computer assisted auscultation may be used as a clinical decision support tool. In an attempt to develop an automatic computer aided diagnostic modality for heart conditions that is sensitive, specific, non-invasive we created two automatic computer cardiac auscultation (ACCA) models that provide heart sound analysis and we aimed to improve the sensitivity and correct classification rate (CCR) of recognition of heart sounds, thus we developed model A ACCA recognition system (machine learning (interpreter independent)) and model B ACCA recognition system (machine learning and interpreter dependent visual analysis). We used machine learning based on mel frequency cepestr...
Introduction Acute alcohol toxicities are mainly due to intake of big amounts of strong alcoholic... more Introduction Acute alcohol toxicities are mainly due to intake of big amounts of strong alcoholic drinks, i.e. spirits like whisky and brandy, while industrial cases are considered limited (Driesbach, 1983). Mild, moderate and severe toxicities are expressed when blood alcohol levels reach 0.15%, 0.3% and 0.5% respectively (Shoemaker et al., 1984). Alcoholic coma is accompanied with severe respiratory and circulatory depression and subnormal temperature (Arena, 1979).
Primary hyperoxaluria type 1 (PH1) is a rare disease that is challenged by the overproduced oxala... more Primary hyperoxaluria type 1 (PH1) is a rare disease that is challenged by the overproduced oxalate and commonly presented with radiopaque renal stones or obstructive uropathy. This study aimed to report clinical presentations, renal replacement therapy (RRT), and outcome of PH1 in end stage kidney disease (ESKD) children. This is an observational cohort study. Data of 22 patients with ESKD due to PH1 were analyzed at Pediatric Nephrology Unit, Faculty of Medicine Cairo University. Infantile onset patients (n = 10) had worst renal outcome (80% with ESRD at presentation, p = 0.019) and worse patient outcome (mortality 40%, p = 0.016) than juvenile (n = 9) and late onset (PH1 n = 3) patients. RRT modalities include peritoneal dialysis (PD) in 7 (31.8%), hemodialysis (HD) in 11 (50%), and combined liver kidney transplantation (CLKT) in 4 (18.2%) patients. Infectious complications were encountered in 42.8% of PD patients. Better HD adequacy was observed with frequent HD (n = 6) and/or H...
8544 Background: This is a retrospective study of 140 nephroblastoma patients newly diagnosed and... more 8544 Background: This is a retrospective study of 140 nephroblastoma patients newly diagnosed and treated at the National Cancer Institute between January 1991 and December 2000. The aim was to report results of combined modality treatment and to investigate the impact of biopsy, presence of blastema and preoperative chemotherapy on outcome Methods: Chemotherapy consisted of Vincristine 1.5mg/m2 and Actinomycin-D 45μg/kg for stage I and stage II favorable. Doxorubicin 40mg/m2 was added in alternation with Actinomycin-D to stage II unfavorable and stages III-V. Duration of therapy was 6 months for stage I and stage II favorable, 12 months for stages II unfavorable, III-V favorable and 18 months for stages III-V unfavorable. Rhabdoid tumors were given Etoposide 150mg/m2×3days and Carboplatin 500mg/m2 day1 alternating with Ifosfamide 1800mg/m2×5days and Doxorubicin 75mg/m2 day1. Postoperative radiotherapy was given to tumor-bed (10-27Gy) in stage II unfavorable, stages III-V and to metastatic lung site (12Gy). Preoperative chemotherapy was employed in massive or bilateral tumors or presence of metastases Results: Using NWTS staging system 21.4%, 36.4%, 20.7%, 15.7% and 5.7% were stages I-V respectively. Histology was favorable (FH) in 93 (67%) patients [31/93 showed blastema] and unfavorable (UH) in 46 (33%). Preoperative chemotherapy resulted in down staging of 30/54 (55.6%) patients to stages I and II, of which only 4 relapsed. Local recurrence was reported in 4/32 patients (12.5%) with core biopsy, 0/15 fine needle biopsy and 10/93 (10.75%) without biopsy. With median follow up of 63 months, overall 5-year survival and disease free survival (DFS) were 72.3±4% and 69±3.9% respectively. For FH, 5-year DFS was 78.2±4.3% [stages I-V being 92%, 74.3%, 74%, 60%, 80%] and 51.4±7.9% for UH (p= .0007, log rank test) [stages I-V being 75%, 72.7%, 63.6%, 8.3%, 33.3%] Within FH, 5-year DFS was 76.6% for blastema versus 83.3% for non blastema (p= .46, log rank) Conclusions: This study suggests that biopsy and down staging after preoperative chemotherapy did not lead to increased risk of recurrence. Presence of blastema had adverse impact on outcome; however it needs to be studied on larger scale No significant financial relationships to disclose.
Superior mesenteric artery (SMA) syndrome is a rare condition resulting in small bowel partial or... more Superior mesenteric artery (SMA) syndrome is a rare condition resulting in small bowel partial or complete obstruction. The third part of the duodenum becomes compressed between the aorta and the SMA once a subject loses weight rapidly. The patients at risk of SMA syndrome are those with eating disorders, trauma and substance abuse. We present a case of a 13-year old female that complained of jaundice 3 months earlier, of recurrent attacks of vomiting and progressive weight loss. The vomiting was bilious and non-projectile following meals, 3-4 times per day. Upon presentation her weight was below the third percentile for age, with jaundice and hepatomegaly. Hepatitis proved to be autoimmune in nature and computerized tomography (CT) angiography confirmed SMA. The weight loss that preceded the SMA proved to be due to autoimmune thyroiditis and thyrotoxicosis. Duodenojejunostomy was not performed as she responded to carbimazole and nutritional support. Vomiting was controlled within a week. Hyperthyroidism can masquerade as SMA. Search for the underlying cause of weight loss leading to SMA is essential. We support the initial trial of conservative treatment for SMA.
Background. Infantile cholestasis continues to represent a diagnostic challenge. It is very impor... more Background. Infantile cholestasis continues to represent a diagnostic challenge. It is very important to diagnose surgically correctable disorders, such as biliary atresia, in a timely manner to prevent progressive damage to the liver. It has been recently suggested that the triangular cord (TC) sign is a simple and useful tool in the diagnosis of biliary atresia. Methods. We prospectively studied 65 infants presenting with conjugated hyperbilirubinemia (age range: 32–161 days). All patients underwent ultrasonographic examination with a 7.0-MHz transducer (Acuson, Mountain View, CA). The TC was defined as a triangular, or tubular, echogenic density seen immediately cranial to the portal vein bifurcation. Results. The TC sign was identified in 25 infants, and all of them had histologic features suggestive of biliary atresia; the diagnosis was confirmed at surgery by gross morphology of hepatobiliary system, and liver biopsy, with or without intraoperative cholangiogram. Among the 40 patients who did not have the TC sign, 6 had paucity of the intrahepatic bile ducts. Three had alph-1-antitrypsin deficiency, and 31 had neonatal hepatitis. None of the 40 patients who did not have the TC sign developed acholic stools. Seven patients with biliary atresia were followed by ultrasonographic examination for 6 months after the Kasai procedure. The TC sign disappeared in all patients after the surgery; however, the TC sign reappeared in 3 patients who developed progressive cholestasis after the procedure. Conclusion. The TC sign is a simple, timesaving, and reliable diagnostic tool in the evaluation of infants with infantile cholestasis. The TC sign may also prove to be helpful in following patients after hepatoportoenterostomy. We suggest a new diagnostic strategy for patients suspected to have biliary atresia. When the TC sign is visualized, the patient should undergo intraoperative cholangiogram to confirm the diagnosis of biliary atresia, reserving percutaneous liver biopsy for those patients in whom the TC sign could not be detected.
Background: Ursodeoxycholic acid is a bile acid that was found to increase bile flow, protect hep... more Background: Ursodeoxycholic acid is a bile acid that was found to increase bile flow, protect hepatocytes, and dissolve gallstones. Purpose: The objective of this study is to review ursodeoxycholic acid in infants and children with extrahepatic biliary atresia. Methods: We used a statistical analysis of data of records of infants and children having extrahepatic biliary atresia who underwent Kasai portoenterostomy and attended Hepatology Clinic, New Children's Hospital, Cairo University, Egypt, from May 1985 until June 2005. Results: Of 141 infants with extrahepatic biliary atresia, 108 received ursodeoxycholic acid for mean duration ± SD of 252.6 ± 544.9 days in a dosage of 20 mg/kg per day. The outcome of infants who did not receive ursodeoxycholic acid and those who did was the following: 8 (24.2%) and 11 (10.18%) had a successful outcome (P = .043), 0 (0%) and 7 (6.4%) improved (P = .148), 25 (75.7%) and 84 (77.7%) had a failed outcome (P = .489), and none vs 5 died (4.6%) (P = .135), respectively. The predictors of successful outcomes were age less than 65 days at portoenterostomy (P = .008) and absence of ursodeoxycholic acid intake (P = .04) with a likelihood of a successful outcome that was 2.8, that associated with ursodeoxycholic acid intake. Conclusion: In this cohort of infants with extrahepatic biliary atresia, ursodeoxycholic acid was not shown to be effective, and its use was associated with a plethora of hepatic and extrahepatic complications.
Biliary atresia (BA) is the most common indication for liver transplantation among children globa... more Biliary atresia (BA) is the most common indication for liver transplantation among children globally (1). This Research Topic aimed to shed light on the BA possible etiology(s), pathogenesis, underlying genetic susceptibility, screening, diagnosis, and challenges that need to be addressed for its elimination and eradication.
Journal of the Egyptian Society of Parasitology, 2018
Parasitic infections in children with liver diseases could be a serious health problem. This stud... more Parasitic infections in children with liver diseases could be a serious health problem. This study documented a true prevalence of intestinal parasitosis in children with pediatric liver diseases and estimate risk factors for parasitic infection. Faecal samples were collected from 95 children suffering liver diseases. Samples were examined microscopically for parasitic stages and coproscopy of acid fast (AF) stained fecal smear for Cryptosporidium oocysts, by ELISA for E. histolytica and Giardia and by PCR genotyping for E. histolytica and Cryptosporidium spp. copro-DNA. G. intestinalis was the prevailing protozoa (14.7%) followed by Hymenolepis nana (8.4%) and C. hominis (4.2%). The pathogenic E. histolytica was not detected by genetic differentiation or ELISA. Only age group (P value .004) was a risk factor for intestinal parasitosis among these children. Giardiasis, hymenolepiasis nana and cryptosporidiosis are the most prevailing parasites in children with liver disease that phy...
Background: Cardiopulmonary surgery is associated with risk of liver dysfunction due to hypoperfu... more Background: Cardiopulmonary surgery is associated with risk of liver dysfunction due to hypoperfusion, release of mediators by macrophages and drug induced liver injury. Aim of the work: to assess liver functions and sonographic changes post-cardiopulmonary bypass in children. Methods: Fifty children with congenital heart diseases who underwent cardiac surgery were assessed. Liver function tests, abdominal ultrasound and echocardiography were performed for those with impaired liver functions at 6 months and up to 18months postoperatively. Results: Among 50 studied children (30 males and 20 females); 40 underwent cardiopulmonary bypass (CPB) and 10 underwent closed heart surgery. Twelve (24%) patients had elevated liver transaminases (aspartate transaminase (AST) and alanine transaminase (ALT)). They all proved to have anti-hepatitis C virus (HCV) antibodies and positive HCV by polymerase chain reaction (PCR). Brightness of liver (20 cases), hepatomegaly (12 cases) and congested hepatic veins (10 cases) were the most important sonographic findings. Significant relation was found between elevated liver enzymes and HCV (p=0.012). There was no correlation between CPB or congenital structural defect or surgical procedure and hepatic affection (p>0.05). Conclusion: Open heart surgery may be associated with increased risk of hepatitis C infection. Increased liver brightness is the most common sonographic finding. Pre-cardiac surgery screening for HCV is mandatory in children.
Introduction: Contemporary stethoscope has limitations in diagnosis of chest conditions, necessit... more Introduction: Contemporary stethoscope has limitations in diagnosis of chest conditions, necessitating further imaging modalities. Methods: We created 2 diagnostic computer aided non-invasive machine-learning models to recognize chest sounds. Model A was interpreter independent based on hidden markov model and mel frequency cepstral coefficient (MFCC). Model B was based on MFCC, hidden markov model, and chest sound wave image interpreter dependent analysis (phonopulmonography (PPG)). Results: We studied 464 records of actual chest sounds belonging to 116 children diagnosed by clinicians and confirmed by other imaging diagnostic modalities. Model A had 96.7% overall correct classification rate (CCR), 100% sensitivity and 100% specificity in discrimination between normal and abnormal sounds. CCR was 100% for normal vesicular sounds, crepitations 89.1%, wheezes 97.6%, and bronchial breathing 100%. Model B's CCR was 100% for normal vesicular sounds, crepitations 97.3%, wheezes 97.6%, and bronchial breathing 100%. The overall CCR was 98.7%, sensitivity and specificity were 100%. Conclusion: Both models demonstrated very high precision in the diagnosis of chest conditions and in differentiating normal from abnormal chest sounds irrespective of operator expertise. Incorporation of computer-aided models in stethoscopes promises prompt, precise, accurate, cost-effective, non-invasive, operator independent, objective diagnosis of chest conditions and reduces number of unnecessary imaging studies.
Biliary atresia (BA) is the most common indication for pediatric liver transplantation. We descri... more Biliary atresia (BA) is the most common indication for pediatric liver transplantation. We describe The BA variant: Kotb disease. Liver tissue in the Kotb disease BA is massively damaged by congenital aflatoxicosis resulting in inflammation, adhesions, fibrosis, bile duct proliferation, scarring, cholestasis, focal syncytial giant cell transformation, and typical immune response involving infiltration by CD4+, CD8+, CD68+, CD14+, neutrophil infiltration, neutrophil elastase spill, heavy loads of aflatoxin B1, accelerated cirrhosis, disruption of p53 and GSTPi, and have null glutathione S transferase M1 (GSTM1). All their mothers are heterozygous for GSTM1. This inability to detoxify aflatoxicosis results in progressive inflammatory adhesions and obliterative cholangiopathy early in life. The typical disruption of both p53 and GSTPi causes loss of fidelity of hepatic regeneration. Hence, regeneration in Kotb disease BA typically promotes accelerated cirrhosis. The immune response in Kotb disease BA is for damage control and initiation of regeneration, yet, this friendly fire incurs massive structural collateral damage. The Kotb disease BA is about actual ongoing hepatic entrapment of aflatoxins with lack of ability of safe disposal due to child detoxification-genomics disarray. The Kotb disease BA is a product of the interaction of persistent congenital aflatoxicosis, genetic lack of GSTM1 detoxification, ontogenically impaired activity of other hepatic detoxification, massive neutrophil-elastase, immune-induced damage, and disturbed regeneration. Ante-natal and neonatal screening for aflatoxicosis, avoiding cord milking, and stringent control of aflatoxicosis content of human, poultry and live-stock feeds might prove effective for prevention, prompt diagnosis and management based on our recent understanding of its patho-genomics.
Epidermoid cysts are encapsulated sebaceous cysts that contain keratin. They present at any age, ... more Epidermoid cysts are encapsulated sebaceous cysts that contain keratin. They present at any age, yet they are commoner in adults. They mainly affect face, neck, trunk and to a lesser extent groin, but can potentially arise as a subepidermal nodule anywhere on the body. They develop from the follicular infundibulum, and are different from dermoid cysts as epidermoid cysts do not contain other adnexal structures of embryonic origin. Epidermoid cysts are mostly silent but can rupture, get inflamed, become infected or develop daughter cysts. Very rarely epidermoid cysts develop in proximity to testicles. They can be mistaken for a testicular tumor. The preoperative diagnosis is essential to avoid unindicated testicular surgery. Magnetic resonance imaging (MRI) is vital for diagnosis. We report a 6-year-old child with a long standing painless scrotal swelling thought by the parents to be polyorchidism. They were alarmed by its recent rapid growth. By examination it was a median raphe cyst. Pre-operative imaging and operative surgical removal proved it to be an inflamed epidermoid cyst. The surgery was uncomplicated and the child had an uneventful recovery. Testicular epidermoid cysts in children are exceptionally rare, yet they can present as median raphe cysts. Inflammation within the epidermoid cyst presents clinically as rapid painless growth. Clinical examination, high index of suspicion and pre-operative MRI avoid unnecessary testicular surgery.
An Amyand hernia is defined as when the appendix is trapped within the sac of an inguinal hernia ... more An Amyand hernia is defined as when the appendix is trapped within the sac of an inguinal hernia through a patent vaginal process. It was first described by Claudius Amyand, in 1735 during an appendectomy of a perforated appendix inside a hernial sac of an 11-year old child. Amyand hernia rare, constituting less than 1% of inguinal hernias. Amyand hernia is 3 times more likely to be diagnosed in children compared to adults due to the patency of vaginal process. Appendicitis or its perforation in Amyand hernia, complicate 0.1% of cases. Other variants include the presence of ileum, caecum or both in addition to the appendix. Isolated acute appendicitis in neonates is extremely rare, with a high mortality rate, and higher among those within Amyand hernia up to 30%. Perforated appendix in Amyand hernia is difficult to diagnose, as its clinical picture is not specific. We report a neonate 26 days old with perforated appendix in an Amyand inguinal hernia who presented by a picture of obstructed oblique inguinal hernia. He underwent emergency open inguinal exploration, herniotomy and appendicectomy. Despite the high mortality rate of Amyand hernia due to be peritoneal spread of sepsis, the boy survived complication free. Perforated appendix in Amyand hernia is exceptionally rare, and can present in the early neonatal period, its diagnosis and outcome rely on high index of suspicion and prompt surgical intervention.
Background and objectives: Liver damage in cholestasis is multifactorial, yet bile acid-mediated ... more Background and objectives: Liver damage in cholestasis is multifactorial, yet bile acid-mediated hepatotoxicity is pivotal. Honey consumption has many physiological effects; it influences detoxification process (phase I, II and III), has antioxidant, anti-inflammatory, immune-stimulant, anti-ulcer, wound/burn healing effects and others. The bile acid ursodeoxycholic acid (UDCA) is currently used off-label to treat neonatal cholestasis. This study aimed to assess the effectiveness of spore-free natural honey to treat neonatal nonobstructive cholestasis. Methods: Thirty infants with cholestasis received spore-free natural honey. Their progression was compared to a control group with cholestasis (28 infants) and a historical group (31 infants) on UDCA. Results: The mean ± standard deviation (SD) follow-up duration was 29.68 ± 18.68 months. At presentation, the total bilirubin concentrations were 9.5 ± 5.9 mg/dL, 10.9 ± 7 mg/dL and 14 ± 9 mg/dL in the honey, control and UDCA groups, respectively (p = 0.064), and their direct bilirubin concentrations were 6 ± 4 mg/dL, 6.7 ± 4 mg/dL and 8.4 ± 6.9 mg/dL, respectively (p = 0.169). The final total bilirubin concentrations were 1.2 ± 1.7 mg/dL, 4.6 ± 8.2 mg/dL and 7.3 ± 8.9 mg/dL, (p = 0.006), and their final direct bilirubin concentrations were 0.8 ± 1 mg/dL, 2.77 ± 5.2 mg/dL and 5.1 ± 7.2 mg/dL (p = 0.008), with cure achievement in 25, 16 and 16 (p = 0.023), improvement in 3, 5 and 3 (p = 0.565), failure in 2, 3 and 10 (p = 0.016), and death in 0, 4 and 2, respectively (p = 0.095). None suffered from botulism or flaccid paralysis. Conclusions: Spore-free honey is effective in clearing cholestasis in neonates and infants. UDCA use in cholestasis in pediatric age should be abandoned as it is less effective and is associated with a worse outcome.
Advances in Intelligent Systems and Computing, 2018
Heart sounds recognition is essential for heart defects diagnosis. Diagnosis of structural heart ... more Heart sounds recognition is essential for heart defects diagnosis. Diagnosis of structural heart defects is not always possible using the contemporary stethoscope and need further assessment using high cost devices such as X-ray, electrocardiogram (ECG), echocardiography (ECHO) and computed tomography (CT)). Automatic computer assisted auscultation may be used as a clinical decision support tool. In an attempt to develop an automatic computer aided diagnostic modality for heart conditions that is sensitive, specific, non-invasive we created two automatic computer cardiac auscultation (ACCA) models that provide heart sound analysis and we aimed to improve the sensitivity and correct classification rate (CCR) of recognition of heart sounds, thus we developed model A ACCA recognition system (machine learning (interpreter independent)) and model B ACCA recognition system (machine learning and interpreter dependent visual analysis). We used machine learning based on mel frequency cepestr...
Introduction Acute alcohol toxicities are mainly due to intake of big amounts of strong alcoholic... more Introduction Acute alcohol toxicities are mainly due to intake of big amounts of strong alcoholic drinks, i.e. spirits like whisky and brandy, while industrial cases are considered limited (Driesbach, 1983). Mild, moderate and severe toxicities are expressed when blood alcohol levels reach 0.15%, 0.3% and 0.5% respectively (Shoemaker et al., 1984). Alcoholic coma is accompanied with severe respiratory and circulatory depression and subnormal temperature (Arena, 1979).
Primary hyperoxaluria type 1 (PH1) is a rare disease that is challenged by the overproduced oxala... more Primary hyperoxaluria type 1 (PH1) is a rare disease that is challenged by the overproduced oxalate and commonly presented with radiopaque renal stones or obstructive uropathy. This study aimed to report clinical presentations, renal replacement therapy (RRT), and outcome of PH1 in end stage kidney disease (ESKD) children. This is an observational cohort study. Data of 22 patients with ESKD due to PH1 were analyzed at Pediatric Nephrology Unit, Faculty of Medicine Cairo University. Infantile onset patients (n = 10) had worst renal outcome (80% with ESRD at presentation, p = 0.019) and worse patient outcome (mortality 40%, p = 0.016) than juvenile (n = 9) and late onset (PH1 n = 3) patients. RRT modalities include peritoneal dialysis (PD) in 7 (31.8%), hemodialysis (HD) in 11 (50%), and combined liver kidney transplantation (CLKT) in 4 (18.2%) patients. Infectious complications were encountered in 42.8% of PD patients. Better HD adequacy was observed with frequent HD (n = 6) and/or H...
8544 Background: This is a retrospective study of 140 nephroblastoma patients newly diagnosed and... more 8544 Background: This is a retrospective study of 140 nephroblastoma patients newly diagnosed and treated at the National Cancer Institute between January 1991 and December 2000. The aim was to report results of combined modality treatment and to investigate the impact of biopsy, presence of blastema and preoperative chemotherapy on outcome Methods: Chemotherapy consisted of Vincristine 1.5mg/m2 and Actinomycin-D 45μg/kg for stage I and stage II favorable. Doxorubicin 40mg/m2 was added in alternation with Actinomycin-D to stage II unfavorable and stages III-V. Duration of therapy was 6 months for stage I and stage II favorable, 12 months for stages II unfavorable, III-V favorable and 18 months for stages III-V unfavorable. Rhabdoid tumors were given Etoposide 150mg/m2×3days and Carboplatin 500mg/m2 day1 alternating with Ifosfamide 1800mg/m2×5days and Doxorubicin 75mg/m2 day1. Postoperative radiotherapy was given to tumor-bed (10-27Gy) in stage II unfavorable, stages III-V and to metastatic lung site (12Gy). Preoperative chemotherapy was employed in massive or bilateral tumors or presence of metastases Results: Using NWTS staging system 21.4%, 36.4%, 20.7%, 15.7% and 5.7% were stages I-V respectively. Histology was favorable (FH) in 93 (67%) patients [31/93 showed blastema] and unfavorable (UH) in 46 (33%). Preoperative chemotherapy resulted in down staging of 30/54 (55.6%) patients to stages I and II, of which only 4 relapsed. Local recurrence was reported in 4/32 patients (12.5%) with core biopsy, 0/15 fine needle biopsy and 10/93 (10.75%) without biopsy. With median follow up of 63 months, overall 5-year survival and disease free survival (DFS) were 72.3±4% and 69±3.9% respectively. For FH, 5-year DFS was 78.2±4.3% [stages I-V being 92%, 74.3%, 74%, 60%, 80%] and 51.4±7.9% for UH (p= .0007, log rank test) [stages I-V being 75%, 72.7%, 63.6%, 8.3%, 33.3%] Within FH, 5-year DFS was 76.6% for blastema versus 83.3% for non blastema (p= .46, log rank) Conclusions: This study suggests that biopsy and down staging after preoperative chemotherapy did not lead to increased risk of recurrence. Presence of blastema had adverse impact on outcome; however it needs to be studied on larger scale No significant financial relationships to disclose.
Superior mesenteric artery (SMA) syndrome is a rare condition resulting in small bowel partial or... more Superior mesenteric artery (SMA) syndrome is a rare condition resulting in small bowel partial or complete obstruction. The third part of the duodenum becomes compressed between the aorta and the SMA once a subject loses weight rapidly. The patients at risk of SMA syndrome are those with eating disorders, trauma and substance abuse. We present a case of a 13-year old female that complained of jaundice 3 months earlier, of recurrent attacks of vomiting and progressive weight loss. The vomiting was bilious and non-projectile following meals, 3-4 times per day. Upon presentation her weight was below the third percentile for age, with jaundice and hepatomegaly. Hepatitis proved to be autoimmune in nature and computerized tomography (CT) angiography confirmed SMA. The weight loss that preceded the SMA proved to be due to autoimmune thyroiditis and thyrotoxicosis. Duodenojejunostomy was not performed as she responded to carbimazole and nutritional support. Vomiting was controlled within a week. Hyperthyroidism can masquerade as SMA. Search for the underlying cause of weight loss leading to SMA is essential. We support the initial trial of conservative treatment for SMA.
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