Entomophthoromycosis: a challenging emerging disease.

Entomophthoromycosis is a rare fungal infection that may affect immunocompetent hosts; predominantly in tropical and subtropical regions. Recently, the importance of this emerging mycosis has increased and the scope of its manifestations has been expanded. These manifestations; however, may masquerade as other clinical entities. Prompt diagnosis of this infection requires a high index of suspicion. Although histopathological examination and cultures are the gold standard diagnostic tools; molecular diagnosis is now available and started to play an important role. The cornerstone treatment is prolonged anti-fungal therapy along with surgical debridement. More awareness of this mycosis is warranted for definitive diagnosis and implementation of early proper therapeutic strategies.

Effect of nutrition-related infodemics and social media on maternal experience: A nationwide survey in a low/middle income country.

BACKGROUND: Undernutrition is a crucial cause of morbidity and mortality among children in low- or middle-income countries (LMICs). A better understanding of maternal general healthy nutrition knowledge, as well as misbeliefs, is highly essential, especially in such settings. In the current era of infodemics, it is very strenuous for mothers to select not only the right source for maternal nutrition information but the correct information as well. AIM: To assess maternal healthy nutritional knowledge and nutrition-related misbeliefs and misinformation in an LMIC, and to determine the sources of such information and their assessment methods. METHODS: This cross-sectional analytical observational study enrolled 5148 randomly selected Egyptian mothers who had one or more children less than 15 years old. The data were collected through online questionnaire forms: One was for the general nutrition knowledge assessment, and the other was for the nutritional myth score. Sources of information and ways of evaluating internet sources using the Currency, Relevance, Authority, Accuracy, and Purpose test were additionally analyzed. RESULTS: The mean general nutrition knowledge score was 29 ± 9, with a percent score of 70.8% ± 12.1% (total score: 41). The median myth score was 9 (interquartile range: 6, 12; total score: 18). The primary sources of nutrition knowledge for the enrolled mothers were social media platforms (55%). Half of the mothers managed information for currency and authority, except for considering the author's contact information. More than 60% regularly checked information for accuracy and purpose. The mothers with significant nutrition knowledge checked periodically for the author's contact information (P = 0.012). The nutrition myth score was significantly lower among mothers who periodically checked the evidence of the information (P = 0.016). Mothers dependent on their healthcare providers as the primary source of their general nutritional knowledge were less likely to hold myths by 13% (P = 0.044). However, using social media increased the likelihood of having myths among mothers by approximately 1.2 (P = 0.001). CONCLUSION: Social media platforms were found to be the primary source of maternal nutrition information in the current era of infodemics. However, healthcare providers were the only source for decreasing the incidence of maternal myths among the surveyed mothers.

Value of Nutritional Screening Tools Versus Anthropometric Measurements in Evaluating Nutritional Status of Children in a Low/Middle-Income Country.

Purpose: Pediatric patients in low-income countries are at a high risk of malnutrition. Numerous screening tools have been developed to detect the risk of malnutrition, including the Subjective Global Nutritional Assessment (SGNA), Pediatric Yorkhill Malnutrition Score (PYMS), Screening Tool for the Assessment of Malnutrition in Pediatrics (STAMP), and Screening Tool for Risk of Nutritional Status and Growth (STRONGkids). However, anthropometry remains the main tool for assessing malnutrition. We aimed to identify the value of four nutritional screening tools versus anthropometry for evaluating the nutritional status of children. Methods: We conducted a cross-sectional study of 1,000 children aged 1-12 years who visited the outpatient clinic of Cairo University Pediatric Hospital. Each participant was evaluated using anthropometric measurements (weight, length/height, and weight for length/height) as well as the PYMS, STAMP, STRONGkids, and SGNA screening tools. The sensitivities and specificities of these four tools were assessed using anthropometry as the gold standard. Results: Of the patients, 1.7% were underweight, 10.2% were wasted, and 35% were stunted. STRONGkids demonstrated the highest sensitivity (79.4%) and a high specificity (80.2%) for detecting malnutrition compared with weight for height, followed by STAMP, which demonstrated lower sensitivity (73.5%) but higher specificity (81.4%). PYMS demonstrated the lowest sensitivity (66.7%) and the highest specificity (93.5%), whereas SAGA demonstrated higher sensitivity (77.5%) and lower specificity (85.4%) than PYMS. Conclusion: The use of nutritional screening tools to evaluate the nutritional status of children is valuable and recommended as a simple and rapid method for identifying the risk of malnutrition in pediatric patients.

Case Report: Dubin-Johnson Syndrome Presenting With Infantile Cholestasis: An Overlooked Diagnosis in an Extended Family.

Dubin-Johnson syndrome (DJS) is an often-missed diagnosis of neonatal cholestasis. We report two patients with DJS, who presented with neonatal cholestasis. The first patient underwent extensive investigations for infantile cholestasis with no definitive etiology reached; the diagnosis of DJS was missed until the age of 14 years old. The diagnosis was confirmed genetically with c.2273G > T, p.G758V mutation in exon 18 of the ABCC2 gene. The 2nd patient is a 7-day-old baby, the son of the 1st patient who gave birth to him at the age of 21 years old. He was diagnosed with DJS at the age of 2 weeks based on normal clinical and laboratory workup apart from direct hyperbilirubinemia. He had the same mutation as his mother in homozygous status. The husband was heterozygous for the same mutation. DJS is one of the often-missed differential diagnoses of neonatal cholestasis. It should be suspected in patients of infantile cholestasis, who have an, otherwise, normal physical examination, and laboratory investigations to avoid unnecessary lengthy, invasive, and expensive workups.

Efficacy of single phototherapy with low-cost reflective sheets versus single phototherapy alone in mild-to-moderate unconjugated hyperbilirubinaemia in full-term neonates.

BACKGROUND: An economical alternative method of increasing the light intensity of phototherapy for neonatal jaundice is the use of reflective sheets placed on the sides of the incubator. AIM: To determine whether reflective sheets in addition to phototherapy increase the reduction of bilirubin levels and the duration of hospital stay. METHODS: The study was undertaken in the neonatal intensive care unit of Cairo University Children's Hospital. There were two groups: a study group of 90 full-term neonates with neonatal jaundice who received single phototherapy in incubators covered with white plastic reflective sheets and a control group of 90 full-term neonates with neonatal jaundice who received single phototherapy without the reflective sheets. RESULTS: The mean (SD) rate of bilirubin decline in the first 24 hours of phototherapy was greater in the study group [3.7 (0.86) µmol/L/hr] than in the control group [2.2 (0.14) µmol/L/hr] (p<0.001). CONCLUSION: Use of reflective sheets decreases the total duration of phototherapy and the cost and duration of hospitalisation without any added complications.Abbreviations: AAP: American Academy of Pediatrics; HIDS: high-intensity double-surface; LMIC: low- and middle-income countries; MCTP: mirror-covered tunnel phototherapy; NICU: neonatal intensive care unit; TSB: total serum bilirubin.

Consensus statement on the epidemiology, diagnosis, prevention, and management of cow's milk protein allergy in the Middle East: a modified Delphi-based study.

BACKGROUND: This study aimed to develop an expert consensus regarding the epidemiology, diagnosis, and management of cow's milk protein allergy (CMPA) in the Middle East. METHODS: A three-step modified Delphi method was utilized to develop the consensus. Fifteen specialized pediatricians participated in the development of this consensus. Each statement was considered a consensus if it achieved an agreement level of ≥ 80%. RESULTS: The experts agreed that the double-blind placebo-controlled oral challenge test (OCT) should be performed for 2-4 weeks using an amino acid formula (AAF) in formula-fed infants or children with suspected CMPA. Formula-fed infants with confirmed CMPA should be offered a therapeutic formula. The panel stated that an extensively hydrolyzed formula (eHF) is indicated in the absence of red flag signs. At the same time, the AAF is offered for infants with red flag signs, such as severe anaphylactic reactions. The panel agreed that infants on an eHF with resolved symptoms within 2-4 weeks should continue the eHF with particular attention to the growth and nutritional status. On the other hand, an AAF should be considered for infants with persistent symptoms; the AAF should be continued if the symptoms resolve within 2-4 weeks, with particular attention to the growth and nutritional status. In cases with no symptomatic improvements after the introduction of an AAF, other measures should be followed. The panel developed a management algorithm, which achieved an agreement level of 90.9%. CONCLUSION: This consensus document combined the best available evidence and clinical experience to optimize the management of CMPA in the Middle East.

Perinatal transmission of hepatitis C virus: an update.

Infection with hepatitis C virus (HCV) is a major health problem worldwide. A large proportion of perinatal HCV infections are silent and may present later in adulthood with long-term complications. HCV has no effective immune prophylaxis and hence appropriate follow-up of all infants born to HCV-infected mothers is necessary. Universal antenatal screening for HCV is largely debatable. Intrauterine and partum transmission of HCV are both possible and higher rates are associated with a high maternal serum viral load (> 106 copies per milliliter), concomitant HIV infection, prolonged or difficult delivery, and invasive fetal monitoring during delivery. Infection during pregnancy and infancy needs to be investigated more in order to design management strategies for perinatal transmission of HCV most effectively. The recently approved new-generation, oral, direct-acting antiviral drugs may open a new era in HCV therapy for pregnant women and infected infants if proved to be safe during conception and infancy.

Congenital chloride losing diarrhea: A single center experience in a highly consanguineous population.

Congenital chloride losing diarrhea (CCLD) is a rare type of chronic watery diarrhea due to mutations in SLC26A3 gene leading to defective chloride-bicarbonate exchanges with the resultant loss of chloride and retention of bicarbonate.We aim to define pediatric Saudi CCLD patients' characteristics to achieve prompt diagnosis, management, follow up with good quality of life, and prevention of complications in these patients.We carried retrospective data review of demographic, clinical, laboratory, radiographic, and outcome of all pediatric patients fulfilling the criteria of CCLD over 10 years from 2004 to 2014 from a single center in Taif region, Saudi Arabia.Forty-nine patients fulfilled the criteria of CCLD from 21 families with more than one affected patient in the same family in 90% of them and positive consanguinity in 91% of the cohort. Most patients were born preterm with intrauterine growth restriction and usually neonatal intensive care unit (NICU) admissions with prematurity and its complications. Thirteen patients were discharged without diagnosis of CCLD and 3 were misdiagnosed as intestinal obstruction with unnecessary surgical intervention. Many complications do existed with renal complications being the most common with three patients received renal transplantation.Prematurity with abdominal distension and stool like urine were the commonest presentation of CCLD in Saudi children. Positive consanguinity and more than one affected sibling are present in most of our cohort.High index of suspicion by clinicians is a cornerstone for early diagnosis with subsequent favorable outcome.A multicenter national incidence study of CCLD in KSA and its genetic attributes is recommended. Premarital screening should be implemented specially for consanguineous marriage.

Predictive accuracy of serum hyaluronic acid as a non-invasive marker of fibrosis in a cohort of multi-transfused Egyptian children with ß-thalassaemia major.

BACKGROUND AND STUDY AIM: Liver disease remains a major cause of morbidity and mortality in patients with ß-thalassaemia major (ß-TM); therefore, its identification at an early stage is of great significance. Serum hyaluronic acid (HA) is considered as a non-invasive marker that appears early before pathological changes occur. We aim to determine the predictive accuracy of HA in detecting and staging hepatic fibrosis in ß-TM patients. PATIENTS AND METHODS: 30 Egyptian children with ß-TM, and 15 age and sex-matched controls were studied. All had abdominal ultrasonography (US), measurement of serum amino-transferases (ALT, AST); hepatitis C, B and human immunodeficiency viruses (HCV, HBV, HIV) sero-markers, serum ferritin and HA. Liver biopsy was done for patients and fibrosis was scaled using Metavir scoring system and liver iron concentration (LIC) was measured. RESULTS: Twenty patients (67.7%) had sero-markers of HCV, none had HBV or HIV. Serum HA was significantly higher in patients (90.78±28.79 ng/ml) compared to controls (21.1±13.24 ng/ml) with p<0.05. No difference between HCV infected and non-infected patients was detected. Positive significant correlation was detected between serum HA and stages of fibrosis by histopathology and US. No correlation was found between serum HA and age, sex, weight, height, haemoglobin level, platelet count, AST, serum ferritin, necro-inflammatory grade, and LIC. CONCLUSIONS: Serum HA is a valuable non-invasive marker that may contribute to the assessment of liver fibrosis in multi-transfused children and adolescents with ß-TM, irrespective of concomitant HCV infection.

Gastrointestinal basidiobolomycosis in children: an overlooked emerging infection?

Basidiobolus ranarum is a known cause of chronic subcutaneous zygomycosis. During the past decade, many cases have been reported with extracutaneous basidiobolomycosis. We aimed to review the medical literature on gastrointestinal basidiobolomycosis (GIB) as an emerging fungal infection causing a serious, and occasionally fatal, paediatric disease. We reviewed all reported cases of visceral basidiobolomycosis with special focus on the gastrointestinal involvement in children. Twenty-six cases of GIB have been reported worldwide, in 14 adults and 12 children. All cases presented with fever, abdominal pain with mass and high blood eosinophilia and were misdiagnosed as other chronic granulomatous diseases or malignancies. A few cases of retroperitoneal, pulmonary, nasal and disseminated basidiobolomycosis have also been reported. Basidiobolomycosis mostly affects young males as a subcutaneous infection. The visceral form of infection is rare. GIB has been scarcely reported in the medical literature, but recently it has been increasingly recognized. GIB poses diagnostic difficulties. Abdominal pain with mass and eosinophilia were present in all cases, highlighting the necessity of considering GIB in the differential diagnosis of this presentation. B. ranarum has been reported to cause disseminated fatal disease in both immune-competent and -compromised individuals. Culture is the gold standard for diagnosis, but the characteristic histopathological picture of chronic granulomas rich in eosinophils and the Splendore-Hoeppli phenomenon are the usual diagnostic tools. Surgery plus long courses of itraconazole treatment, up to 1 year, appear to be the best management options. GIB is an emerging infection that might lead to diagnostic confusion, morbidity and mortality. Diagnosis requires a high index of suspicion in the differential diagnosis of patients with fever, abdominal pain with mass and eosinophilia.

Medical management of chronic liver diseases in children (part I): focus on curable or potentially curable diseases.

The management of children with chronic liver disease (CLD) mandates a multidisciplinary approach. CLDs can be classified into 'potentially' curable, treatable non-curable, and end-stage diseases. Goals pertaining to the management of CLDs can be divided into prevention or minimization of progressive liver damage in curable CLD by treating the primary cause; prevention or control of complications in treatable CLD; and prediction of the outcome in end-stage CLD in order to deliver definitive therapy by surgical procedures, including liver transplantation. Curative, specific therapies aimed at the primary causes of CLDs are, if possible, best considered by a pediatric hepatologist. Medical management of CLDs in children will be reviewed in two parts, with part I (this article) specifically focusing on 'potentially' curable CLDs. Dietary modification is the cornerstone of management for galactosemia, hereditary fructose intolerance, and certain glycogen storage diseases, as well as non-alcoholic steatohepatitis. It is also essential in tyrosinemia, in addition to nitisinone [2-(nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione] therapy, as well as in Wilson disease along with copper-chelating agents such as D-penicillamine, triethylenetetramine dihydrochloride, and ammonium tetrathiomolybdate. Zinc and antioxidants are adjuvant drugs in Wilson disease. New advances in chronic viral hepatitis have been made with the advent of oral antivirals. In children, currently available drugs for the treatment of chronic hepatitis B virus infection are standard interferon (IFN)-α-2, pegylated IFN-α-2 (PG-IFN), and lamivudine. In adults, adefovir and entecavir have also been licensed, whereas telbivudine, emtricitabine, tenofovir disoproxil fumarate, clevudine, and thymosin α-1 are currently undergoing clinical testing. For chronic hepatitis C virus infection, the most accepted treatment is PG-IFN plus ribavirin. Corticosteroids, with or without azathioprine, remain the basic strategy for inducing remission in autoimmune hepatitis. Ciclosporin (cyclosporine) and other immune suppressants may be used for patients who do not achieve remission, or who have significant side effects, with corticosteroid/azathioprine therapy. The above therapies can prevent, or at least minimize, progression of liver damage, particularly if started early, leading to an almost normal quality of life in affected children.

Medical management of chronic liver diseases (CLD) in children (part II): focus on the complications of CLD, and CLD that require special considerations.

Treatment of the causes of many chronic liver diseases (CLDs) may not be possible. In this case, complications must be anticipated, prevented or at least controlled by the best available therapeutic modalities. There are three main goals for the management of portal hypertension: (i) prevention of the first episode of variceal bleeding largely by non-selective ß-adrenoceptor antagonists, which is not generally recommended in children; (ii) control of bleeding by using a stepwise approach from the least to most invasive strategies; (iii) and prevention of re-bleeding using bypass operations, with particular enthusiasm for the use of meso-Rex bypass in the pediatric population. Hepatic encephalopathy management also consists of three main aspects: (i) ruling out other causes of encephalopathy; (ii) identifying and treating precipitating factors; and (iii) starting empiric treatment with drugs such as lactulose, rifaximin, sodium benzoate, and flumazenil. Treatment of mild ascites and peripheral edema should begin with the restriction of sodium and water, followed by careful diuresis, then large-volume paracentesis associated with colloid volume expansion in severe cases. Empiric broad spectrum antimicrobial therapy should be used for the treatment of spontaneous bacterial peritonitis, bacterial and fungal sepsis, and cholangitis, after taking appropriate cultures, with appropriate changes in therapy after sensitivity testing. Empirical therapies continue to be the standard practice for pruritus; these consist of bile acid binding agents, phenobarbital (phenobarbitone), ursodeoxycholic acid, antihistamines, rifampin (rifampicin), and carbamazepine. Partial external biliary diversion can be used in refractory cases. Once hepatorenal syndrome is suspected, treatment should be initiated early in order to prevent the progression of renal failure; approaches consist of general supportive measures, management of concomitant complications, screening for sepsis, treatment with antibiotics, use of vasopressin analogs (terlipressin), and renal replacement therapy if needed. Hepatopulmonary syndrome and portopulmonary hypertension are best managed by liver transplantation. Provision of an adequate caloric supply, nutrition, and vitamin/mineral supplements for the management of growth failure, required vaccinations, and special care for ensuring psychologic well-being should be ensured. Anticoagulation might be attempted in acute portal vein thrombosis. Some CLDs, such as extrahepatic biliary atresia (EHBA), Crigler-Najjar syndrome, and Indian childhood cirrhosis, require special considerations. For EHBA, Kasai hepatoportoenterostomy is the current standard surgical approach in combination with nutritional therapy and supplemental fat and water soluble vitamins, minerals, and trace elements. In type 1 Crigler-Najjar syndrome, extensive phototherapy is the mainstay of treatment, in association with adjuvant therapy to bind photobilirubin such as calcium phosphate, cholestyramine, or agar, until liver transplantation can be carried out. Treating Indian childhood cirrhosis with penicillamine early in the course of the disease and at doses similar to those used to treat Wilson disease decreases the mortality rate by half. New hopes for the future include extracorporeal liver support devices (the molecular adsorbent recirculating system [MARS®] and Prometheus®), hepatocyte transplantation, liver-directed gene therapy, genetically engineered enzymes, and therapeutic modalities targeting fibrogenesis. Hepapoietin, a naturally occurring cytokine that promotes hepatocyte growth, is under extensive research.

Gastrointestinal basidiobolomycosis: an emerging fungal infection causing bowel perforation in a child.

Basidiobolomycosis is an unusual fungal skin infection that rarely involves the gastrointestinal (GI) tract. We report a 10-year-old boy diagnosed as suffering GI basidiobolomycosis after being misdiagnosed first as suffering intestinal malignancy then schistosomiasis. The patient presented with fever, abdominal pain, vomiting, abdominal tenderness and rigidity with marked blood eosinophilia. Abdominal ultrasonographic and computed tomographic scans revealed a large caecal mass. Biopsy of the mass showed transmural granulomatous inflammation interpreted as schistosomal granuloma, ruling out lymphoma. The patient's condition deteriorated despite anti-schistosomal therapy. Emergency surgery was then performed, and caecal perforation was found. The mass was excised; cultures were negative and histopathological examination was suggestive of schistosomal granuloma. The mass recurred 3 weeks post-operatively. Second-opinion histopathological examination diagnosed Basidiobolus ranarum infection. Treatment with itraconazole produced marked improvement, with diminution of the mass. B. ranarum was unequivocally identified in the archival formalin-fixed and paraffin-embedded (FFPE) tissue by PCR. This case emphasizes the need to consider GI basidiobolomycosis in children presenting with fever, abdominal mass and eosinophilia, especially those complicated by bowel perforation.

Diagnosis of spontaneous bacterial peritonitis in infants and children with chronic liver disease: A cohort study.

BACKGROUND: Spontaneous bacterial peritonitis (SBP) is a serious complication in infants and children with chronic liver disease (CLD); however its diagnosis might be difficult. We aimed to study the feasibility of diagnosing SBP by routine ascitic fluid tapping in infants and children with CLD. METHODS: We enrolled thirty infants and children with biopsy-proven CLD and ascites. Ascitic fluid was examined for biochemical indices, cytology and cell count. Aerobic and anaerobic bacteriological cultures of ascitic fluid were preformed. Direct smears were prepared from ascitic fluid deposit for Gram and Zheil-Nelson staining. RESULTS: Patients were divided into three groups: Group I included five patients with SBP in which the cell count was ≥ 250/mm3 and culture was positive (16.7%), Group II, eight patients with culture negative neutrocytic ascites (CNNA) with cells ≥ 250/mm3 and negative culture (26.7%) and Group III, seventeen negative patients (56.6%) in which cells were <250/mm3 and culture was negative. None of our patients had bacteriascites (i.e. culture positive with cells <250/mm3). Presence of fever was significantly higher in SBP and CNNA. The mean lactate dehydrogenase (LDH) level was significantly higher in ascitic fluid in the infected versus sterile cases (p < 0.002). A ratio of ascitic/serum LDH ≥ 0.5 gave a sensitivity of 80%, specificity of 88%, positive predictive value (PPV) of 66.7%, negative predictive value (NPV) of 93.7% and accuracy of 63.3%. The mean pH gradient (arterial - ascitic) was significantly higher in SBP and CNNA cases when compared to the negative cases (p < 0.001). Ascitic fluid protein level of ≤ 1 gm/dl was found in 13/30 (43.3%) of studied cases with a sensitivity of 100%, specificity of 64.7%, PPV of 45.5%, NPV of 100% and diagnostic accuracy of 53.3% (p = 0.0001). CONCLUSIONS: SBP is a rather common complication in children with CLD. Culture of the ascitic fluid is not always diagnostic of infection. Biochemical parameters of the ascitic fluid definitely add to the diagnostic accuracy. LDH ascitic/serum ratio ≥ 0.5, an arterial-ascitic pH gradient ≥ 0.1 and total ascitic fluid protein ≤ 1 gm/dl are the most significant parameters suggesting infection.