We report a case of hearing rehabilitation following combined cochlear implantation and ossiculoplasty. A 71-year-old patient visited the clinic for right-sided mixed hearing loss. We targeted neural and conductive components, performing two different operations simultaneously. At two months post-operative, the patient showed satisfactory results with respect to hearing threshold and speech comprehension. Our experience suggests that careful evaluation of patients and consideration of the diverse array of available treatment strategies can be used to provide personalized rehabilitation with maximal effectiveness. To the best of our knowledge, this is the first report to take such an integrated approach to treat hearing impairment and is thus likely to have clinical importance for otologists.

Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by progressive destruction of the intrahepatic bile ducts, leading to cholestasis. PBC is known to have both hepatic and extrahepatic manifestations. Extrahepatic manifestations are seen in up to 73% of patients with PBC, with the most common being Sjogren’s syndrome, thyroid dysfunction and systemic sclerosis. It is thought that patients with PBC are at increased risk of developing these extrahepatic manifestations, almost all of which are autoimmune, because patients with autoimmune disease are at higher risk of developing another autoimmune condition. Due to the high prevalence of extrahepatic diseases in patients with PBC, it is important to complete a thorough medical history at the time of diagnosis. Prompt recognition of extrahepatic disease can lead to improved patient outcomes and quality of life. The following review summarizes the most common extrahepatic conditions associated with PBC.
Autoimmune Diseases ; Bile Ducts, Intrahepatic ; Cholangitis* ; Cholestasis ; Diagnosis ; Humans ; Liver Diseases ; Prevalence ; Quality of Life ; Scleroderma, Systemic ; Thyroid Gland

Objective: This study aimed to investigate the risk factors of post-contrast acute kidney injury (PAKI) and the usefulness of the Mehran score for predicting PAKI in patients who underwent contrast-enhanced abdominopelvic computed tomography (CE-APCT) in the emergency department (ED). Methods: This was a retrospective observational study. Patients who underwent CE-APCT and had a follow-up creatinine test within 72 hours in the period January to June, 2017, were enrolled for the study. PAKI is defined as a 25% or higher increase in the level of serum creatinine (sCr) within 72 hours after receiving contrast, or an increase in the level of sCr by 0.5 mg/dL. The odds ratio (OR) of risk factors and incidence of PAKI after CE-APCT were analyzed according to the Mehran risk group, and compared to expected incidence. Univariate and multivariate logistic regression analyses were performed for each risk factor. Results: A total of 1,718 patients were enrolled in the study. Of these, 203 patients (11.8%) developed PAKI, and 2 patients (0.1%) required dialysis. Hypotension (systolic blood pressure <80 mmHg) was determined to be statistically significant (P=0.029; OR, 3.181) among the considered risk factors of PAKI. In the group having abnormal estimatedglomerular filtration rate (<90 mL/min/1.73 m2), the age and rate of the underlying disease (congestive heart failure, hypertension) was found to be higher in the PAKI group. The receiver operating curve of Mehran score (area under the curve: 0.521 in model A, 0.520 in model B) was statistically not significant in the univariate analysis. A higher Mehran score was associated with a higher proportion of patients who underwent prophylactic treatment. Conclusion There are no definite useful risk factors, including the Mehran score, for predicting PAKI in patients who underwent contrast-enhanced computed tomography in the ED.

Objective: This study aimed to investigate the risk factors of post-contrast acute kidney injury (PAKI) and the usefulness of the Mehran score for predicting PAKI in patients who underwent contrast-enhanced abdominopelvic computed tomography (CE-APCT) in the emergency department (ED). Methods: This was a retrospective observational study. Patients who underwent CE-APCT and had a follow-up creatinine test within 72 hours in the period January to June, 2017, were enrolled for the study. PAKI is defined as a 25% or higher increase in the level of serum creatinine (sCr) within 72 hours after receiving contrast, or an increase in the level of sCr by 0.5 mg/dL. The odds ratio (OR) of risk factors and incidence of PAKI after CE-APCT were analyzed according to the Mehran risk group, and compared to expected incidence. Univariate and multivariate logistic regression analyses were performed for each risk factor. Results: A total of 1,718 patients were enrolled in the study. Of these, 203 patients (11.8%) developed PAKI, and 2 patients (0.1%) required dialysis. Hypotension (systolic blood pressure <80 mmHg) was determined to be statistically significant (P=0.029; OR, 3.181) among the considered risk factors of PAKI. In the group having abnormal estimatedglomerular filtration rate (<90 mL/min/1.73 m2), the age and rate of the underlying disease (congestive heart failure, hypertension) was found to be higher in the PAKI group. The receiver operating curve of Mehran score (area under the curve: 0.521 in model A, 0.520 in model B) was statistically not significant in the univariate analysis. A higher Mehran score was associated with a higher proportion of patients who underwent prophylactic treatment. Conclusion There are no definite useful risk factors, including the Mehran score, for predicting PAKI in patients who underwent contrast-enhanced computed tomography in the ED.

Hypereosinophilic syndrome (HES) is defined as peripheral eosinophilia, organ involvement of eosinophils and exclusion of other disorders or dysfunction. HES is subclassified as myeloproliferative variant, T-lymphocytic, overlap, idiopathic, familial and associated type according to the new classification using molecular and immunologic markers. HES presenting with gastrointestinal symptoms is very rare but characterized by peripheral eosinophilia, eosinophilic infiltration of the gastrointestinal tract with other organs such as liver and bone marrow. We report a rare case of idiopathic HES in 18-year-old adolescent who presented with gastrointestinal symptoms including abdominal pain, diarrhea and ascites which resolved rapidly with corticosteroid therapy. HES should be included in the differential diagnosis of abdominal pain and ascites in children and adolescents.
Abdominal Pain ; Adolescent* ; Ascites ; Biomarkers ; Bone Marrow ; Child ; Classification ; Diagnosis, Differential ; Diarrhea ; Eosinophilia ; Eosinophils ; Gastrointestinal Tract ; Humans ; Hypereosinophilic Syndrome* ; Liver

The aims of this study were to investigate whether early arterial blood gas analysis (ABGA) could define the severity of disease in infants with congenital diaphragmatic hernia (CDH). We conducted a retrospective study over a 21-yr period of infants diagnosed with CDH. Outcomes were defined as death before discharge, and extracorporeal membrane oxygenation requirements (ECMO) or death. A total 114 infants were included in this study. We investigated whether simplified prediction formula [PO2-PCO2] values at 0, 4, 8, and 12 hr after birth were associated with mortality, and ECMO or death. The area under curve (AUC) of receiver operating characteristic curve was used to determine the optimum ABGA values for predicting outcomes. The value of [PO2-PCO2] at birth was the best predictor of mortality (AUC 0.803, P < 0.001) and at 4 hr after birth was the most reliable predictor of ECMO or death (AUC 0.777, P < 0.001). The value of [PO2-PCO2] from ABGA early period after birth can reliably predict outcomes in infants with CDH.
*Algorithms ; Area Under Curve ; *Blood Gas Analysis ; *Extracorporeal Membrane Oxygenation ; Female ; Hernia, Diaphragmatic/*congenital/mortality/physiopathology ; Humans ; Infant, Newborn ; Male ; Predictive Value of Tests ; ROC Curve ; Retrospective Studies ; Survival Rate ; Treatment Outcome

Methotrexate (MTX) is used in the reproductive aged females for the management of medical conditions such as ectopic pregnancy, autoimmune diseases and malignancies. Because of its antimetabolite effect, exposure to MTX during the fetal period can cause multiple anomalies. The most common anomalies related to intrauterine MTX exposure include growth retardation, craniofacial dysmorphism, central nervous system anomalies, cardiac anomalies and skeletal defects. We report a premature baby boy born after 27(+5) weeks of gestation who presented intrauterine growth restriction, single umbilical artery, small chest and anomalies of rib and thoracic vertebra. His mother had received 50 mg of MTX for the treatment of misdiagnosed ectopic pregnancy at 5th week of gestation. During the hospitalization, he was ventilator dependent and pulmonary hypertension persisted despite medical treatment including nitric oxide and sildenafil. Open lung biopsy revealed nonspecific findings suggestive of lung hypoplasia. He died at 141 days after birth due to respiratory failure.
Aged ; Autoimmune Diseases ; Biopsy ; Central Nervous System ; Female ; Hospitalization ; Humans ; Hypertension, Pulmonary ; Infant, Newborn ; Infant, Premature ; Lung ; Methotrexate ; Mothers ; Nitric Oxide ; Parturition ; Piperazines ; Pregnancy ; Pregnancy, Ectopic ; Purines ; Respiratory Insufficiency ; Ribs ; Single Umbilical Artery ; Spine ; Sulfones ; Thorax ; Ventilators, Mechanical ; Sildenafil Citrate

OBJECTIVES: Iodine deficiency causes multiple health problems. Previously we reported that 96% of high school students in Tuguegarao, Philippines had adequate iodine levels. However, iodine deficiency-associated problems remain among adults in the Philippines. Therefore, we evaluated iodine nutritional status and goiter prevalence among adults, including pregnant women, in Tuguegarao, Philippines. METHODS: A total of 245 adults, including 31 pregnant women, provided samples for urinary iodine analysis, and all pregnant women completed a questionnaire about iodine deficiency. RESULTS: The median urinary iodine level was 164.0 ± 138.4 µg/L; 38.4% of the participants were iodine deficient, according to the International Council for Control of Iodine Deficiency Disorders (ICCIDD) criteria. No severe iodine deficiency was observed. Among the 31 pregnant women, 24 (77.5%) fell into the iodine deficient category defined by a stricter World Health Organization (WHO) guideline, in which iodine deficiency is considered when urinary iodine levels are below 150 µg/L. Almost half (42%) of the pregnant women were unaware of the harmful effects of iodine deficiency on the human body and their fetus. CONCLUSIONS: Although iodine nutritional status in the Philippines has improved, iodine deficiency still exists among adults, especially among pregnant women. Therefore, our study strongly suggests that a better strategy should be established to monitor iodine nutritional status among adults continually, and to focus on populations susceptible to iodine deficiency, including pregnant women and women of reproductive age, to achieve the total elimination of iodine deficiency.
Adult* ; Female ; Fetus ; Goiter ; Human Body ; Humans ; Iodine* ; Nutritional Status* ; Philippines* ; Pregnant Women* ; Prevalence ; World Health Organization

Objectives: . To compare the surgical outcomes of externally monitored and conventional buried flaps with the goal of determining the usefulness of external monitoring of buried flaps. Methods: . In this case-control study with propensity score matching, 30 patients were evenly divided into externally monitored buried flap and conventional buried flap groups. The total operative time for free flap reconstruction, the flap survival rate, the length of hospital stay, the initial time of a reliable visual assessment, complications, the final diet achieved, and the duration until diet initiation were compared between the groups. Results: . The mean operative time for reconstruction was 115 minutes (interquartile range, 85–150 minutes) and 142 minutes (interquartile range, 95–180 minutes) in the externally monitored and conventional groups, respectively (P= 0.245). The median length of hospital stay was 24 days (interquartile range, 18–30 days) and 27 days (interquartile range, 20–41 days) in the externally monitored and conventional groups, respectively (P=0.298). The median duration until diet initiation was 15 days (interquartile range, 15–21 days) and 18 days (interquartile range, 15–34 days) in the externally monitored and conventional groups, respectively (P=0.466). The final diet, initial time of a reliable visual assessment, and complications were comparable between the groups, but the external skin paddle provided an excellent visual assessment immediately postoperatively in all cases. Conclusion . The outcomes were comparable between the groups, indicating that externalization of the cutaneous component of a buried flap may be a straightforward and useful technique for monitoring a buried anterolateral thigh free flap in laryngopharyngeal reconstructions. The salvage and false-positive rates of compromised flaps should be compared in large subject groups in future studies to prove that the use of an external skin paddle improves flap monitoring.

PURPOSE: Feeding desaturation is a common problem among preterm infants which can result in prolonged hospital stays, long-term feeding difficulties and growth delay. The purpose of this study was to identify the characteristics of premature infants with feeding desaturation and to examine the effect of orocutaneous stimulation on oral feeding. METHODS: During the first phase of this study, 125 extremely low birth weight infants were reviewed retrospectively. Characteristics between infants with feeding desaturation (n=34) and those without feeding desaturation (n=91) were examined. During the second phase, 29 infants recruited from March, 2009 to May, 2010 were subjected to orocutaneous stimulation. The results of orocutaneous stimulation were compared to a control group (n=81). RESULTS: The first phase of the study revealed that extremely low birth weight infants with feeding desaturation were significantly lower in gestational ages at birth, and had lower 5 minute apgar scores, more gastroesophageal refluxes and bronchopulmonary dysplasia. Infants without feeding desaturation reached full enteral feeding significantly earlier and showed shorter duration of hospital stay. At the second phase, infants in the intervention group showed shorter days to achieve initiation of bottle feeding, shorter days in achievement of full bottle feeding, last episodes of feeding desaturation and length of hospital stay compared to the control group of similar characteristics. CONCLUSION: Orocutaneous stimulation among extremely low birth weight infants results in earlier achievement of full bottle feedings without episodes of feeding desaturation hence shortens the length of hospital stay.
Achievement ; Bottle Feeding ; Bronchopulmonary Dysplasia ; Enteral Nutrition ; Gastroesophageal Reflux ; Gestational Age ; Humans ; Infant ; Infant, Low Birth Weight ; Infant, Newborn ; Infant, Premature ; Length of Stay ; Parturition ; Retrospective Studies