George Imvrios

New nucleos(t)ide analogue monoprophylaxis after cessation of hepatitis B immunoglobulin is effective against hepatitis B recurrence

New nucleos(t)ide agents (NAs) [entecavir (ETV) and tenofovir (TDF)] have made hepatitis B immunoglobulin (HBIG)‐sparing protocols an attractive approach against hepatitis B virus (HBV) recurrence after liver transplantation (LT). Twenty‐eight patients transplanted for HBV cirrhosis in our centre were prospectively evaluated. After LT, each patient received HBIG (1000 IU IM/day for 7 days and then monthly for 6 months) plus ETV or TDF and then continued with ETV or TDF monoprophylaxis. All patients had undetectable HBV DNA at the time of LT, and they were followed up with laboratory tests including glomerular filtration rate (GFR) after LT. All patients (11 under ETV and 17 under TDF) remained HBsAg/HBV DNA negative during the follow‐up period [median: 21 (range 9–43) months]. GFR was not different between TDF and ETV groups of patients at 6 and 12 months and last follow‐up (P value >0.05 for all comparisons). The two groups of patients were similar regarding their ratio of maximum rate of tubular phosphate reabsorption to the GFR (TmP/GFR). In conclusion, in this prospective study, we showed for the first time that maintenance therapy with ETV or TDF monoprophylaxis after 6 months of low‐dose HBIG plus ETV or TDF after LT is highly effective and safe.

Primary Intestinal Lymphangiectasia: Is It Always Bad? Two Cases with Different Outcome

Primary intestinal lymphangiectasia (PIL) or Waldmanns disease is a rare protein-losing gastroenteropathy of unknown etiology. Less than 200 cases have been reported globally. Patients may be asymptomatic or present edema, lymphedema, diarrhea, ascites and other manifestations. We report two pediatric cases with PIL with extremely different outcome in a 3-year follow-up period. The first patient presented with persistent diarrhea, hypoalbuminemia and failure to thrive, while the second patient presented with an abrupt eyelid edema. Hypoproteinemia was the common laboratory finding for the two patients and upper gastrointestinal endoscopy established the diagnosis. The first patient relapsed five times during the follow-up period after the diagnosis had been made and required intravenous albumin administration and micronutrient supplementation. The second patient revealed normal gastrointestinal endoscopy 4 months after the diagnosis had been established; he followed an unrestricted diet and remained asymptomatic throughout the follow-up period. PIL can be either severe, affecting the entire small bowel, leading to lifetime disease, or sometimes affects part of the small bowel, leading to transient disorder.

Successful, combined long-term treatment of cerebral aspergillosis in a liver transplant patient.

Invasive aspergillosis has long been recognized as one of the most significant and often fatal opportunistic fungal infections in liver transplant recipients. We report a case of a liver transplant recipient who developed an Aspergillus fumigatus brain abscess that produced significant neurologic symptoms. The patient was managed successfully with a combination of surgery and medical treatment with Voriconazole. To our knowledge, this is the second such case reported in the literature.

Atopic Dermatitis: Insights on Pathogenesis, Evaluation and Management

Background: Atopic Dermatitis (AD) is a highly pruritic chronic inflammatory skin disease that commonly presents during early childhood. Several studies indicate that the disease is very common in western world with lifetime prevalence in children of 10% to 20%. Data sources: We make recommendations regarding the evaluation and management of AD in children based on systematic literature searches using the best-available evidence from PubMed, Medline, Google Scholar, NICE, the American Academy of Dermatology and the World Allergy Organization. Results: AD has an immunologic basis. Both innate and adaptive immune responses are impaired in AD patients. Intense pruritus is a hallmark of the disease which leads to extensive scratching and further breakdown of the skin barrier. Treatment of AD may be topical or systemic. Topical corticosteroids and calcineurin inhibitors are used as topical anti-inflammatory agents. Patients should be carefully instructed in the use of topical glucocorticoids to avoid side effects. Conclusions: Treatment of atopic eczema should be based on a “stepped-care plan” where treatments are stepped up or down depending on the assessment of the state of the child’s skin by the physician. Physicians who treat children with mild to moderate AD should tell children and their caregivers that AD is a lifelong illness.

Long term outcome of acquired food allergy in pediatric liver recipients: a single center experience

Food induced sensitization has been reported in pediatric liver recipients. However long term follow up has not been established so far. We report here our experience regarding 3 pediatric patients who developed acquired food allergy after liver transplantation. The first patient suffered from persistent diarrhea and eczema. The second one presented with abdominal pain with no signs of rejection, abdominal discomfort, vomiting when ingesting milk proteins and responded well to the elimination diet. The third patient presented with facial angioedema and hoarseness of voice. She had multiple food allergies and reacted to milk, egg and sesame. All the patients had elevated total Immunoglobulin E (IgE) and elevated specific IgE antibodies to the implicated food allergens. The first patient presented clinical manifestations of allergy when she was 19 months old. The second patient became allergic at the age of 16 and the third patient at the age of 3. The symptoms of food allergy persisted for 8 years in the first case and for 2 years in the other two cases. Low levels of specific IgE antibodies to the implicated food allergens and an enhanced T-helper 1 cell immune response toward interferon-gamma production were markers of tolerance acquisition. The long term prognosis in our cases was excellent. Food allergy resolved in all the patients. The long term prognosis of acquired food allergy after liver transplantation is currently obscure. More studies would be needed including greater number of patients to determine whether acquired food allergy is transient in pediatric liver recipients.

Wireless Capsule Endoscopy Detects Meckel's Diverticulum in a Child with Unexplained Intestinal Blood Loss

Meckel’s diverticulum (MD) is the most common congenital anomaly of the gastrointestinal (GI) tract, affecting about 2% of the population. Most cases of Meckel’s diverticula are asymptomatic. The diagnosis of symptomatic MD is often difficult to make. We report the case of an 8-year-old boy who presented with GI bleeding due to MD. The diagnostic difficulties after an initial negative endoscopic evaluation and the diagnostic value of the various endoscopic procedures are discussed. The patient had suffered from bright red stools for 20 h before hospital admission. GI scintigraphy with 99mTc-Na-pertechnetate was negative for heterotopic gastric tissue in the small bowel area. Colonoscopy performed in order to exclude Crohn’s disease was also negative. He was placed on ranitidine at a dose of 6 mg/kg body weight twice daily. The patient remained asymptomatic over a period of 6 months before he was readmitted due to macroscopic rectal bleeding. Upper endoscopy and colonoscopy used to investigate the source of bleeding showed normal macroscopic findings. Radiolabeling of blood constituents with 99mTc on delayed imaging showed radionucleotide concentration in the ascending and transverse colon suggestive of a lesion in the ileocecal area. Further investigation with the use of wireless capsule endoscopy revealed a MD. Wireless capsule endoscopy may thus be indicated for patients with GI blood loss when other diagnostic methods, such as upper and lower endoscopy and colonoscopy, have failed to identify the source of bleeding.

Assessment of IgE-mediated food allergies in children with atopic dermatitis.

BACKGROUND Atopic dermatitis (AD) is an inflammatory disease of the skin, which is characterised by a chronic relapsing course. AIM The aim of the study was to assign the prevalence of clinically active food allergies among a group of children between 3 months and 7 years of age, with AD. METHODS Eighty-eight children with AD were screened for specific IgE antibodies to food proteins. All patients with AD and specific IgE antibodies to food proteins were subjected to Oral Food Challenges (OFCs) with the relevant foods. RESULTS Food-sensitised patients with moderate levels of sIgE had clinically active food allergy to milk (39.28%) and egg (42.34%) on the basis of positive OFCs. High IgE and eosinophilia had a prevalence of almost 80% and 25%, regardless of concomitant food sensitisation and disease severity. CONCLUSIONS In this study, clinically active food allergies were recognised in 26.13% of children with AD. Nevertheless, no association was confirmed between food sensitisation and AD severity. High IgE and peripheral eosinophilia have not been found more prevalent among children with severe AD nor among children with food sensitisation. Infants and younger children with AD should be screened for an underlying food allergy, regardless of disease severity.

Heterozygous liver transplantation for maple syrup urine disease: First European reported case.

MSUD is an autosomal recessive metabolic disorder that results from a defect in the BCKDH enzyme. This enzyme is essential for the second step in the metabolism of the branched‐chain amino acids, leucine, isoleucine, and valine. Patients with MSUD are subject to severe, irreversible neurologic injury unless closely managed with a specialized metabolic formula and a diet restricted in leucine throughout their lifetime. During times of illness, patients with MSUD can suffer from severe metabolic derangement, acute cerebral edema, and untimely death. Deceased donor liver transplant restores the ability to metabolize branched‐chain amino acids, even on an unrestricted diet, and prevents metabolic derangements during times of illness. We report a successful case of living donor (parental) transplant for a child with MSUD. The donor was the childs father. This approach has been controversial as parents of children with MSUD are obligate heterozygotes for the condition and have diminished levels of BCKDH activity. If effective, living‐related donor transplant provides a promising alternative for deceased donor liver transplant, which often requires a prolonged waiting period and may not be feasible in areas with limited medical resources.

HCC influence on patient survival after liver transplantation for HDV cirrhosis.

BACKGROUND/AIMS The effect of hepatocellular cancer (HCC) in patients transplanted for hepatitis B and D virus (HB/DV) cirrhosis is not well studied. Our aim was to study the long-term survival outcomes of patients who underwent liver transplantation for HB/DV cirrhosis with and without HCC. METHODOLOGY A total of 231 primary, adult, single- organ liver transplants were performed from 1990 to 2007. HB/DV was the cause of cirrhosis in 36 patients. Nine patients died during the first 3 postoperative months from surgical complications. The study group comprised the remaining 27 patients. The median follow-up was 1515 days. RESULTS The mean patient survival was 3760 days (95% CI: 3013-4507). Six patients were diagnosed with HCC. The mean patient survival was 3011 days (95% CI: 2344-3679) and 4036 days (95% CI: 3002-5070) for recipients without and with HCC, respectively. For the same groups, the incidence of microbial infections was 61.9% and 33.3%, respectively (p=0.219). HCC has not recurred in any of the six patients. CONCLUSIONS The mean long-term survival after liver transplantation for HB/DV and HCC surpassed 11 years. The superior survival of HCC patients is difficult to explain. The increased number (almost double) of microbial infections in the non- HCC population might be held accountable.

Validation of the Children's Sleep Habits Questionnaire in a sample of Greek children with allergic rhinitis

BACKGROUND Obstructive respiratory disorders, such as allergic rhinitis and asthma may impair sleep quality. The aim of this study is to validate the Childrens Sleep Habits Questionnaire (CSHQ) for Greek children from 6 to 14 years of age. No validated tool has been developed so far to assess sleep disturbances in Greek school-aged children. METHODS We examined the reliability and validity of the CSHQ in a sample of children with allergic rhinitis (AR) and a non-clinical population of parents of these children as a proxy measure of childrens AR quality of life (QoL) as evaluated by the Pediatric Allergic Rhinitis Quality of Life (PedARQoL) questionnaire. RESULTS The CSHQ questionnaire Childs Form (CF) had a moderate internal consistency with a Cronbachs alpha 0.671 and Guttman split-half coefficient of 0.563 when correlated with the PedARQoL (CF). There was also a moderate intraclass correlation of ICC=0.505 between the responses to both questionnaires in the two visits. The CSHQ Parents Form (PF) had a very good internal consistency with a Cronbachs alpha of 0.928 and Guttman split-half coefficient of 0.798. There was a high intraclass correlation of 0.643 between the responses in the two visits. CONCLUSIONS The Greek version of the CSHQ CF, but particularly the PF has proved to be a very reliable clinical instrument, which can be used in clinical trials for assessing sleep quality in school-aged children with sleep disturbances because of obstructive airway disorders, such as AR.