Hepatic hydrothorax in a child and its management.

Hepatic hydrothorax is a rare complication of portal hypertension. The optimal treatment for this condition is liver transplantation. Liver transplantation is significantly more manageable in children who weigh more than 8 kg. Here, an implantable pleural access device was used in a 5-month-old infant for painless iterative punctures to relieve respiratory symptoms, while waiting for liver transplantation and the patient's growth. The patient underwent successful transplantation 3 months later with a more optimal weight.

Uterine rupture during pregnancy.

OBJECTIVE: A review of contemporary knowledge about uterine rupture during pregnancy, followed by a case-report of a patient with uterine rupture during pregnancy without an uterine scar. DESIGN: Review and case report. SETTING: Clinic of Obstetrics and Gynecology, University Hospital, Hradec Králové; Department of Gynecology and Obstetrics, Hospital Náchod. CASE REPORT: We present a case of an uterine rupture of a uterus without a scar from previous surgery. A patient in 33. week of pregnancy with stillborn fetus was administred to our hospital. While inducing the labor, the patient showed signes of shock, fetus was no longer present in uterus. An C-section was performed, but the stillborn baby was placed in abdominal cavity, with an abrupted placenta. Large uterine rupture was spotted, therefore a hysterectomy was performed. CONCLUSION: Uterine rupture during pregnancy is an urgent state. The incidency of uterine rupture is rising accordingly with the growing number of C-sections. However, it is important to include uterine rupture into differential diagnostics also in cases with other risk factors. The key to successful diagnosis is ultrasound examination and correct evaluation of clinical state, other imaging methods are less suitable because of time delay. Together with the change of major cause of uterine rupture, the approach to treatment has changed as well. If possible, a uterus-saving procedure is preferred. The aim of this case-report is presentation of a rare case of uterine rupture in an scar-free uterus. It also shows how troublesome diagnostics of uterine ruptures can be.

Long-term outcome of liver transplantation in childhood: A study of 20-year survivors.

We report the results of a study of survival, liver and kidney functions, and growth with a median follow-up of 24 years following liver transplantation in childhood. From 1988 to 1993, 128 children underwent deceased donor liver transplantation (median age: 2.5 years). Twenty-year patient and graft survival rates were 79% and 64%, respectively. Raised serum aminotransferase and/or γ-glutamyl transferase activities were present in 42% of survivors after a single transplantation. Graft histology (35 patients) showed signs of chronic rejection in 11 and biliary obstruction in 5. Mean total fibrosis scores were 4.5/9 and 3/9 in patients with abnormal and normal serum liver tests, respectively. Glomerular filtration rate was <90 mL·min-1 in 35 survivors, including 4 in end-stage renal disease who were undergoing dialysis or had undergone renal transplantation. Median final heights were 159 cm for women and 172 cm for men; final height was below the target height in 37 patients. Twenty-year survival after childhood liver transplantation may be close to 80%, and final height is within the normal range for most patients. However, chronic kidney disease or altered liver biochemistries are present in over one third of patients, which is a matter of concern for the future.

Exchangeable copper: a reflection of the neurological severity in Wilson's disease.

BACKGROUND AND PURPOSE: The severity of Wilson's disease (WD) is linked to free copper accumulating in the liver and brain. Exchangeable copper (CuEXC) is a new technique to determine plasmatic copper and is useful in the diagnosis of WD. It is hypothesized that it may also enable a good evaluation of extra-hepatic involvement and its severity. METHODS: Forty-eight newly diagnosed WD patients were prospectively evaluated using hepatic, neurological, ophthalmological and brain magnetic resonance imaging (MRI) scores. Three phenotypic presentations were distinguished: pre-symptomatic, hepatic and extra-hepatic. CuEXC was determined in addition to standard copper assays before decoppering therapy. Correlations between biological parameters and the different scores were determined and compared in the hepatic and extra-hepatic groups. RESULTS: Extra-hepatic patients had significantly higher CuEXC values than those with the hepatic form (P < 0.0001). The overall ability of CuEXC to separate the two forms was satisfactory, with an area under the curve of 0.883 (95% confidence interval 0.771-0.996) and an optimal threshold for extra-hepatic diagnosis of 2.08 µmol/l (sensitivity 85.7%; specificity 94.1%). In extra-hepatic patients, CuEXC was the only biological marker to be positively correlated with the Unified Wilson Disease Rating Score (r = 0.45, P = 0.016), the Kayser-Fleischer ring score (r = 0.46, P = 0.014) and the brain MRI score (r = 0.38, P = 0.048), but it was not correlated with the hepatic score. CONCLUSIONS: Exchangeable copper determination is useful when diagnosing WD as a value >2.08 µmol/l is indicative of the severity of the extra-hepatic involvement. In the case of purely hepatic presentation, atypical or mild neurological signs, it should encourage physicians to search for lesions in the brain and eyes.

Adherence of preventive oral care products in the Syrian market to evidence-based international recommendations.

No study has investigated the availability and adherence of preventive oral care products on the Syrian market to evidence-based international recommendations. Data were collected in 2012, and updated in 2016, in terms of availability, characteristics and adherence to evidence-based international recommendations. Few preventive products adhered to the recommendations. Despite the large decrease in the number of oral care products on the Syrian market, due to the Syrian crisis, nonadherence of some of the available products is still present. A multisectorial approach at a policy level is needed to address such important limitations. The Syrian Ministry of Health should reform regulations for fluoride products to become subject to drug monitoring systems; the Syrian Arab Committee for Measurements and Standards needs to update its standards; and the Syrian General Dental Association should distribute a preventive booklet to dental practitioners.

Adherence of preventive oral care products in the Syrian market to evidence-based international recommendations

No study has investigated the availability and adherence of preventive oral care products on the Syrian market to evidence-based international recommendations. Data were collected in 2012, and updated in 2016, in terms of availability, characteristics and adherence to evidence-based international recommendations. Few preventive products adhered to the recommendations. Despite the large decrease in the number of oral care products on the Syrian market, due to the Syrian crisis, nonadherence of some of the available products is still present. A multisectorial approach at a policy level is needed to address such important limitations. The Syrian Ministry of Health should reform regulations for fluoride products to become subject to drug monitoring systems; the Syrian Arab Committee for Measurements and Standards needs to update its standards; and the Syrian General Dental Association should distribute a preventive booklet to dental practitioners

Aucune étude n'a examiné à ce jour la disponibilité et la conformité des produits de soins dentaires préventifs sur le marché syrien avec les recommandations internationales fondées sur des données probantes. Des données ont été collectées en 2012, et mises à jour en 2016, en ce qui concerne la disponibilité, les caractéristiques et la conformité de ces produits avec les recommandations internationales fondées sur des données probantes. Peu de produits préventifs étaient conformes aux recommandations. Malgré une forte diminution du nombre de produits de soins dentaires sur le marché syrien du fait de la crise actuelle dans le pays, la non-conformité de certains produits disponibles reste d'actualité. Une approche multisectorielle au niveau politique est nécessaire pour remédier à ces lacunes importantes. Le ministère syrien de la Santé doit réformer les réglementations existantes sur les produits à base de fluor afin que ceux-ci soient soumis à des systèmes de suivi des médicaments ; le Comité arabe syrien des mesures et des normes doit mettre à jour ses normes ; et l'Association de dentisterie générale syrienne doit distribuer une plaquette de prévention à l'intention des praticiens dentaires

Rationale for monitoring cyclosporine concentration at 2 hours after administration in infants posttransplantation.

Therapeutic drug monitoring is critical to avoid overimmunosuppression or underimmunosuppression in young pediatric transplant recipients. The objective of this study was to examine cyclosporine (CsA) trough (C0) and 2-hour post-dose (C2) concentrations in the early period after liver transplantation (OLT) to determine whether CsA C2 monitoring is justified. Seventeen infants younger than 2 years treated with CsA (Neoral) were monitored at C0. The biopsy-proved acute rejection rate was 65% at 3 months post-OLT. No correlation was observed between values at C0 and C2. Poor absorption of CsA was observed in most infants during the first 2 weeks post-OLT, as well as interindividual variability in CsA clearance. Exposure to CsA could not be estimated using either C0 or C2 determinations in the early post-OLT period. As a marker of poor absorption, C2 is useful but does not indicate delayed or rapid clearance of drug without simultaneous measurement of concentration at C0. We suggest the use of both C0 and C2 monitoring, or AUC monitoring on an individual basis during at least the first 2 weeks post-OLT.

Orthotopic liver transplantation for mitochondrial respiratory chain disorders: a study of 5 children.

BACKGROUND: Liver involvement in mitochondrial respiratory chain disorders (MRCD) frequently ends in liver failure and death. Because of the high risk of extrahepatic, particularly neuromuscular, manifestations of the disease, the indication of orthotopic liver transplantation (OLT) in these patients remains controversial. We report on 5 such children in whom OLT was carried out, in an attempt to help clarify the matter. PATIENTS: Patients 1 and 2 presented with fulminant liver failure at ages 7 and 6 months respectively. Emergency liver transplantation was performed before etiological investigations were completed. Retrospective examination of the explanted livers showed defects in complexes I, III and IV. In patient 1, severe neurological deterioration occurred 2 months after OLT with fatal outcome 9 months later. Patient 2 is alive 22 months after OLT with moderate motor impairment. Patients 3, 4 and 5 presented with progressive liver failure before 6 months of age. Surgical liver biopsies displayed a 50% defect in complex IV (patient 3), a defect in complexes I, IV (patient 4) and in complexes I, III, IV (patient 5). Because there was no clinical extrahepatic involvement on investigations, OLT was carried out in these patients. Patient 3 died of multiple organ failure soon after OLT, patients 4 and 5 are alive respectively 21 months and 12 months after OLT with normal neurological examination. CONCLUSION: OLT may be a valid therapeutic option in infants with delayed liver cell failure due to MRCD, only after performing in emergency a thorough inves tigation to exclude clinically significant extrahepatic, especially neuromuscular, involvement.

Toxicity of boric acid to Blattella germanica (Dictyoptera: Blattellidae) and analysis of residues in several organs.

Pestiferous cockroach species are associated closely with humans and are important from medical and public health points of view. Conventional insecticides have been used widely to control cockroaches which have developed resistance to these compounds. Thus, interest has again centered on lesser-used compounds such as boric acid. Boric acid has been used as an insecticide for many years, especially against cockroach. Its mode of action on insects has not been satisfactorily established. In Algeria, Blattella germanica (Dictyoptera: Blattellidae) is a serious pest in the urban environment and their infestation were controlled for many years by organophosphate, carbamate or pyrethroid insecticides. In order to obtain more information on the mode of action of boric acid, we first evaluated the oral toxicity of boric acid on B. germanica adults. Then, the compound was determined in several organs by an colorimetric method. This insecticide was incorporated into the diet and orally administered at different concentrations ranging from 1 to 40% (w/w) to newly emerged adults. Mortality was recorded at different times during treatment (24, 48, 72 and 144 h). Treatment resulted in a dose-dependent mortality since the LD50 (%) recorded are 85 at 24 h, 67 at 48 h, 39 at 72 h and 8 at 144 h, respectively. Then the quantity of boric acid accumulated in several organs (hemolymph, gut, ovaries, testicles and fat body) was determined as function the duration of treatment (1 to 5 days) for two doses (LD50 and LD90). Results revealed that bioaccumulation of residues in these organs increased as function the duration of treatment. In addition, relatively important amounts of residues, are detected in fat body.

[Macrophage activation syndrome in primary human herpes virus-6 infection: a rare condition after liver transplantation in infants]. / Syndrome d'activation macrophagique au cours de la primo-infection pat le 6e virus herpétique humain: une affection certainement méconnue au cours de la transplantation hépatique chez l'enfant.

Human herpes virus-6 primary infection generally occurs during the first three years of childhood and is generally asymptomatic. The virus has been identified as the causal agent of exanthemum subitum in children or mononucleosis-like disease in adults, and may also cause several disorders in immunocompromised patients. We report a clinical case of acute rejection observed 29 days after orthotopic liver transplantation in a 22-month-old child associated with acute hepatitis and a hemophagocytic syndrome on day 38. Human herpes virus-6 primary infection was identified based on several virological tests: seroconversion, detection of viral DNA in bone marrow and peripheral blood after polymerase chain reaction, and detection of viral replication in peripheral blood. Tests for Epstein-Barr virus, cytomegalovirus or Parvovirus B19 infections were negative. After treatment by ganciclovir (Cymévan(R)), clinical status improved.

Percutaneous endoscopic gastrostomy for continuous feeding in children with chronic cholestasis.

BACKGROUND: Malnutrition associated with chronic cholestasis in children often requires continuous enteral feeding through a nasogastric tube, which may be poorly tolerated. METHOD: Percutaneous endoscopic gastrostomy was performed in five children (age range, 20 months to 13 years) with severe cholestasis (Alagille syndrome in four; biliary atresia in one) and severe malnutrition (mean weight, -2.6 standard deviations; mean height, -2.7 standard deviations) who were awaiting liver transplantation. The pull-through technique was used in patients under general anesthesia, and the button was set within 2 months. RESULTS: Minor wound infection required antibiotic therapy in one patient. In the four children with Alagille syndrome, enteral feeding by means of percutaneous endoscopic gastrostomy was used until liver transplantation for a mean period of 14 months with a mean weight gain of 350 g/mo and a mean height gain of 0.53 cm/mo. Seventeen months to 3 years, 3 months after liver transplantation, all four children were alive and in good clinical condition with normal readings in liver function tests. The technique had to be discontinued in the child with biliary atresia because of secondary occurrence of ascites, gastric intolerance, and refractory wound infection. CONCLUSION: Percutaneous endoscopic gastrostomy may be a valuable alternative to nasogastric tube for nutritional support in children with cholestasis and mild portal hypertension.

[Favorable outcome of treatment with NTBC of acute liver insufficiency disclosing hereditary tyrosinemia type I]. / Evolution favorable sous traitement par NTBC de l'insuffisance hépatique aiguë révélatrice de la tyrosinémie héréditaire de type I.

BACKGROUND: Hereditary tyrosinemia type I is a disease with a severe prognosis. Main causes of death are acute liver failure, neurologic crises and hepatocarcinoma. NTBC, which acts as an inhibitor of the 4-hydroxyphenylpyruvate dioxygenase, prevents the formation of toxic metabolites involved in hepatic, renal and neurologic lesions. CASE REPORTS: Results of NTBC therapy used in three infants with type I tyrosinemia who presented with acute liver failure are reported. The diagnosis relied on the finding of high plasmatic levels of tyrosine and methionine, and abnormal urinary excretion of succinyl acetone and delta aminolevulinic acid. Treatment with NTBC was initiated within 2 to 8 days from onset of symptoms. Signs of liver failure resolved after 3 weeks therapy. After 12 to 39 months of follow-up, outcome remains favorable. CONCLUSION: The results reported here highlight the efficiency of NTBC in type I tyrosinemia with early acute onset. However, the long term outcome needs to be determined with regards to prevention of hepatocarcinoma and toxicity of the drug.

An electromyographic study of strength and upper extremity muscle activity in simulated meat cutting tasks.

Meat cutting has long been associated with a high incidence rate of upper extremity musculoskeletal disorders. This study examined upper extremity muscle activities and force exertion capabilities to identify postures which have potential for causing overexertion injuries. Fifteen subjects exerted force against a handle in postures similar to those observed in the meatpacking industry. Exertion level, direction of exertion, handle height, reach distance and grip type were varied. Activity in the posterior deltoid, biceps brachii, triceps brachii, extensor digitorum and flexor digitorum superficialis was monitored via surface electromyography (EMG). The ratio of normalized EMG activity to force produced during the exertion was computed for each muscle under each condition. The results showed that handle position had a significant effect on force exertion capability and the EMG/force ratio in all muscles. Force exertion capability was maximized, and the EMG/force ratio was generally minimized when participants pulled downward on a handle positioned at full arm's reach above the shoulder. For vertical cuts, force decreased and muscle activity generally increased as the handle height was lowered. For horizontal cuts, the full reach distance tended to allow greater force exertion with lower EMG/force ratios. The stab grip also tended to be associated with higher forces and lower EMG/force ratios than the slice grip. This study supports the premise that musculoskeletal stresses in meatpacking tasks can be altered through tool and workstation redesign. The data provided herein may be useful in selecting design modifications that reduce biomechanical stress on the upper extremities.

Ursodeoxycholic acid therapy in pediatric patients with progressive familial intrahepatic cholestasis.

Progressive familial intrahepatic cholestasis (PFIC) is a lethal inherited childhood cholestasis of hepatocellular origin. Different subtypes of PFIC have been described according to serum gamma-glutamyl transpeptidase (GGT) activity. There is currently no effective medical therapy available for children with PFIC. We report on 39 patients with PFIC who received ursodeoxycholic acid (UDCA) orally (20-30 mg/kg b.w./day) for a period of 2 to 4 years. Group 1 (n = 26) consisted of children with normal GGT activity, and group 2 (n = 13) of children with high GGT activity. Within group 1, liver tests normalized in 11 children, improved in 5, and stabilized or worsened in 10. Within group 2, liver tests normalized in six children, improved in four, and stabilized or worsened in three. Improvement of parameters was associated with an enrichment of the circulating pool of bile acids with UDCA. Hepatosplenomegaly and pruritus disappeared or diminished in children in whom liver tests normalized. In nine of these children, liver tests worsened and normalized again after stopping and restarting UDCA. Liver histology assessed in four children after normalization of liver tests and 2 years of treatment showed a decrease in fibrosis. We conclude that UDCA should be considered in the initial therapeutic management of children with PFIC, because it appears effective in resolving or improving the liver function and the clinical status of a fair proportion of children. Chronic UDCA therapy might thus avoid the need for liver transplantation in some children with PFIC.

Work activities and musculoskeletal complaints among preschool workers.

The potential for musculoskeletal trauma among preschool workers has been largely unexplored in the United States. This case report describes an investigation conducted to identify and evaluate possible causes of back and lower extremity pain among 22 workers at a Montessori day care facility. Investigators met with and distributed a questionnaire to school employees, and made measurements of workstation and furniture dimensions. Investigators also recorded the normal work activities of school employees on videotape, and performed a work sampling study to estimate the percentage of time employees spend performing various tasks and in certain postures. Questionnaire results from 18 employees indicated that back pain/discomfort was a common musculoskeletal complaint, reported by 61% of respondents. Neck/shoulder pain, lower extremity pain and hand/wrist pain were reported by 33, 33 and 11% of respondents, respectively. Observation and analysis of work activities indicated that employees spend significant periods of time kneeling, sitting on the floor, squatting, or bending at the waist. Furthermore, staff members who work with smaller children (i.e. six weeks to 18 months of age) performed more lifts and assumed more awkward lower extremity postures than employees who work with older children (3-4 years of age). Analysis of two lifting tasks using the revised NIOSH lifting equation indicated that employees who handle small children may be at increased risk of lifting-related low back pain. Investigators concluded that day care employees at this facility are at increased risk of low back pain and lower extremity (i.e. knee) injury due to work activities that require awkward or heavy lifts, and static working postures. Recommendations for reducing or eliminating these risks by modifying the workplace and changing the organization and methods of work are presented.

An analysis of scanning postures among grocery cashiers and its relationship to checkstand design.

Mounting evidence suggests that musculoskeletal disorders are prevalent among US retail food workers. Cashiers who use electronic scanners appear to be at especially high risk for upper extremity musculoskeletal disorders. Checkstand design has been implicated as a contributor to musculoskeletal injury among cashiers because workstation design can significantly impact working posture. The present study examines working posture among two groups of cashiers to determine if checkstand design is associated with substantial differences in posture and movement during scanning. The work activities of twenty grocery cashiers using one of two checkstand designs (front-facing and right-hand takeaway) were examined. Videotapes of cashiers performing scanning tasks were observed and associated postures and movements were visually coded. The right-hand takeaway design was associated with a significantly higher percentage of non-neutral trunk postures than the front-facing design. However, there were no significant differences in shoulder posture, grasp, or scanning motion associated with checkstand/scanner design. Factors that appeared to affect cashier work posture during scanning included stature, order size, and product type. Although improving the checkstand design may reduce the occurrence of certain awkward postures and static muscle loading conditions among cashiers, the success of these interventions is likely to be limited unless follow-up programmes are instituted to ensure that cashiers are able to use these designs effectively. Furthermore, fundamental changes in cashier work may be required to fully eliminate hazards for musculoskeletal disorders from this job.