Actions speak louder than words: Home visits and its effect on dietary adherence in patients with phenylketonuria.

AIM: Phenylketonuria (PKU) is an inherited metabolic disorder in which accumulation of phenylalanine (Phe) leads to poor neurological outcomes without treatment. Dietary therapy is the main treatment and nonadherence is associated with elevated blood Phe levels and correspondingly poor neuropsychiatric outcomes. This study aimed to examine the effect of home visits on blood Phe levels in PKU patients. METHODS: Sixty-five paediatric PKU patients who were on low-phenylalanine diet were visited monthly at home for 6 months. At each visit, dietary education was provided, patients' height and weight were measured and blood samples were collected. RESULTS: Twenty-eight (43.1%) patients had classic PKU and 37 (56.9%) had moderate PKU. Blood Phe levels decreased statistically significant at first, second, fifth, and sixth months compared with screening visit. Blood Phe levels in moderate PKU patients decreased significantly at the last visit unlike classic PKU patients. A significant decrease in blood Phe levels was observed in patients older than 10 years. Anthropometric parameters improved. CONCLUSIONS: Dietary nonadherence is the main treatment failure in PKU. Home visits for education are a promising way to improve treatment outcomes by providing quality education, better assessment, and correction of mistakes but they should be ongoing and supported by different interventions that address patients' special needs.

Talaromyces marneffei infection in an HIV infected patient with hematological malignancy - first report from Turkey.

T.marneffei, encountered mostly in Southeast Asia, leads to a systemic infection, especially in immunocompromised individuals such as HIV-infected patients with low CD4 level. A 32-year-old male patient, residing in Hong Kong for the last two years, admitted with fever, cough, weakness, and weight loss. Physical examination revealed bilateral cervical and axillary multiple lymph nodes and hepatosplenomegaly. Screening of the pancytopenic patient revealed HIV infection. Histopathological examination of the cervical lymph node revealed plasmoblastic lymphoma. Blood and urine cultures remained sterile. Antiretroviral therapy was started. Fungal hyphae were detected in Gram staining of hemocultures taken in the third week due to ongoing fever, and antifungal therapy was started empirically. Red pigment around colonies on Sabouraud dextrose agar and microscopic appearance arose suspicion of Talaromyces spp. T.marneffei was identified by ITS 1-4 sequence analysis. Chemotherapy was started when fungemia was controlled. On the fifth day of chemotherapy, the patient's general condition deteriorated, broad-spectrum antibiotics were started and the patient was transferred to ICU. The cultures remained sterile and he expired five days later. In conclusion, although talaromycosis is not endemic in Turkey, it should be considered in patients with travel history to endemic regions and/or an underlying immunosuppressive disease such as HIV infection.

The place of a ketogenic diet in the treatment of resistant epilepsy: a comprehensive review.

Objective: The ketogenic diet (KD) is a high-fat, adequate-protein, and low-carb diet. Ketone bodies increase in the blood due to low carbohydrate content and high-fat content in the diet. The most important feature of the ketogenic diet is that it causes the production of ketone bodies in the liver. Ketone bodies are an alternative fuel to glucose for the brain and form the structure necessary for the cell membrane and biosynthesis of triglycerides. The ketogenic diet provides evidence on seizure control with anticonvulsant effects. In this review, the positive/negative effects of KD on seizure control, place, importance, quality of life, cognition, and behavior in the treatment of resistant epilepsy were examined.Methods: Scientific information on the subject was obtained from the literature accessed through databases such as MEDLINE, Embase, Web of Science, Cochrane Central, www.ClinicalTrials.gov, PubMed, Science Direct, and Google Scholar.Results: Although it has started to be used as a treatment method in many diseases today, the main area of effect of KD is drug-resistant epilepsy. In order for the ketogenic diet to be successful in these patients, it is necessary to choose the appropriate patient, medical treatment and diet plan, inform the patient sufficiently, and perform frequent monitoring in accordance with the follow-up criteria. It is argued that KD is one of the most effective treatments for epilepsy.Conclusion: The fact that KDs generally have a restricted diet pattern, the need for supplementation, biochemical findings and possible side effects raise the issue of diet sustainability. More clinical studies are needed to generalize.

Discrimination between non-functioning pituitary adenomas and hypophysitis using machine learning methods based on magnetic resonance imaging­derived texture features.

PURPOSE: Hypophysitis is a heterogeneous condition that includes inflammation of the pituitary gland and infundibulum, and it can cause symptoms related to mass effects and hormonal deficiencies. We aimed to evaluate the potential role of machine learning methods in differentiating hypophysitis from non-functioning pituitary adenomas. METHODS: The radiomic parameters obtained from T1A-C images were used. Among the radiomic parameters, parameters capable of distinguishing between hypophysitis and non-functioning pituitary adenomas were selected. In order to avoid the effects of confounding factors and to improve the performance of the classifiers, parameters with high correlation with each other were eliminated. Machine learning algorithms were performed with the combination of gray-level run-length matrix-low gray level run emphasis, gray-level co-occurrence matrix-correlation, and gray-level co-occurrence entropy. RESULTS: A total of 34 patients were included, 17 of whom had hypophysitis and 17 had non-functioning pituitary adenomas. Among the 38 radiomics parameters obtained from post-contrast T1-weighted images, 10 tissue features that could differentiate the lesions were selected. Machine learning algorithms were performed using three selected parameters; gray level run length matrix-low gray level run emphasis, gray-level co-occurrence matrix-correlation, and gray level co-occurrence entropy. Error matrices were calculated by using the machine learning algorithm and it was seen that support vector machines showed the best performance in distinguishing the two lesion types. CONCLUSIONS: Our analysis reported that support vector machines showed the best performance in distinguishing hypophysitis from non-functioning pituitary adenomas, emphasizing the importance of machine learning in differentiating the two lesions.

The Role of Obesity in Predicting the Clinical Outcomes of COVID-19.

INTRODUCTION: The aim of this was to describe the predictors of mortality related to COVID-19 infection and to evaluate the association between overweight, obesity, and clinical outcomes of COVID-19. METHODS: We included the patients >18 years of age, with at least one positive SARS-CoV-2 reverse transcriptase-polymerase chain reaction. Patients were grouped according to body mass index values as normal weight <25 kg/m2 (Group A), overweight from 25 to <30 kg/m2 (Group B), Class I obesity 30 to <35 kg/m2 (Group C), and ≥35 kg/m2 (Group D). Mortality, clinical outcomes, laboratory parameters, and comorbidities were compared among 4 groups. RESULTS: There was no significant difference among study groups in terms of mortality. Noninvasive mechanical ventilation requirement was higher in group B and D than group A, while it was higher in Group D than Group C (Group B vs. Group A [p = 0.017], Group D vs. Group A [p = 0.001], and Group D vs. Group C [p = 0.016]). Lung involvement was less common in Group A, and presence of hypoxia was more common in Group D (Group B vs. Group A [p = 0.025], Group D vs. Group A [p < 0.001], Group D vs. Group B [p = 0.006], and Group D vs. Group C [p = 0.014]). The hospitalization rate was lower in Group A than in the other groups; in addition, patients in Group D have the highest rate of hospitalization (Group B vs. Group A [p < 0.001], Group C vs. Group A [p < 0.001], Group D vs. Group A [p < 0.001], Group D vs. Group B [p < 0.001], and Group D vs. Group C [p = 0.010]). CONCLUSION: COVID-19 patients with overweight and obesity presented with more severe clinical findings. Health-care providers should take into account that people living with overweight and obesity are at higher risk for COVID-19 and its complications.

Evaluation of empathy and biopsychosocial approaches of medical faculty assistant doctors.

INTRODUCTION AND AIM: Biopsychosocial approach suggests that the individual's health condition is partly influenced by the interactions of biological, psychological, and social processes. It also concludes that the clinical method should be patient-centered, which is based on the physician's involvement in the patient's world and the physician's ability to see the illness from the patient's view. In this study, we aimed to determine the extent to which medical faculty assistant doctors use the biopsychosocial approach in their clinical practice. MATERIALS AND METHODS: Our study is a cross-sectional type, descriptive, and analytical research. The sample size consisted of assistant doctors who met the inclusion criteria which were accepted to participate in the study between December 1, 2017, and March 1, 2018, at the medical faculty hospital of Dicle University. Two hundred and three physicians participated in our study. The Patient-Physician Orientation Scale (PPOS), The Jefferson Scale of Physician Empathy (JSPE), and a survey study including questions in relation with sociodemographic characteristics were conducted in our study. The reliability of the Patient-Physician Orientation Scale (PPOS) and JSPE used in our study was tested with Cronbach's alpha and found to be 0.878 and 0.931, respectively. Questionnaires were administered by a face-to-face interview method during interview. Information on the purpose and method of the study was given. RESULTS: A total of 203 physicians participated in our study. One hundred and forty-three (70%) participants were male and 60 (30%) were female. According to medical disciplines, eight physicians (3, 9%) from psychiatry, 21 (10, 3%) from family medicine, 104 (51, 2%) from other internal medical disciplines, and 70 (34, 5%) from surgical medical disciplines participated in the study. In the first subgroup of the PPOS, which is used in our study, the average score of psychiatry physicians was 3.67 ± 0.99, family medicine assistant doctors 3.92 ± 0.67, other internal medical disciplines 3.98 ± 0.65, and surgical medical discipline 3.83 ± 0.61. In the second subgroup, which is defined as the care in the same scale, the average score of psychiatry assistant doctors was found to be 3.20 ± 0.45, family medicine assistant doctors 3.68 ± 0.54, other internal medical disciplines 3.49 ± 0.60, and surgical medical discipline 3.68 ± 0.58. The results of the Jefferson Scale used to determine the level of empathy in our study are found as psychiatry 78.25 ± 14.78, family physician 67.71 ± 14.69, other internal medical disciplines 72.03 ± 13.46, and surgical medical discipline 70.87 ± 15.24. CONCLUSIONS: The biopsychosocial approach is primarily an elementary approach model in medical discipline such as family medicine and should be applied to other internal and surgical medical disciplines as well. With conducting this study, the awareness of this area will be increased and related issues in the literature will be enriched.

Reference ranges for sonographic renal dimensions in preterm infants.

BACKGROUND: Ultrasonographic measurement of kidney dimensions is important in evaluation of renal disease in preterm infants who have multiple comorbidities that affect renal function. OBJECTIVE: The purpose of this study was to determine the reference ranges of kidney dimensions in preterm newborns and to provide a chart to use easily in daily practice. MATERIALS AND METHODS: We evaluated renal dimensions in 498 preterm infants with a gestational age of <37 weeks using sonography within the first week of life. We statistically analyzed the relationships between all dimensions and gender, gestational age (based on the last menstrual period), height and weight. Minimum and maximum values of dimensions were defined. RESULTS: All dimensions of the kidneys were statistically different in boys and girls (P < 0.05). Both longitudinal and anteroposterior dimensions of the right and left kidneys showed high correlation with gestational age, weight and height in girls and boys. Weight correlated best with dimensions. CONCLUSION: Nomograms from these data can be used to determine an abnormality in kidneys of preterm newborns.

Reference ranges for sonographic dimensions of the liver and spleen in preterm infants.

BACKGROUND: Preterm infants usually have multiple comorbidities that affect spleen and liver. Ultrasonographic measurement of organ sizes is an important and reliable parameter in evaluation of spleen and liver pathology in preterm newborns. OBJECTIVE: The purpose of this study was to determine reference values of ultrasonographic measurements of the liver and spleen in preterm newborns. MATERIALS AND METHODS: We prospectively performed sonography on 498 preterm newborns in the first week of life. We measured spleen and liver dimensions and statistically analyzed relationships between the dimensions and gender, gestational age (based on mother's last menstrual period), height and weight. Reference ranges of dimensions were defined. RESULTS: Longitudinal and anteroposterior dimensions of the liver and spleen were statistically significantly different between the boys and girls (P < 0.05) and showed high correlation with the gestational age, weight and height. Weight was the parameter best correlated with the dimensions. CONCLUSION: Nomograms from these data are useful for sonographic evaluation of the liver and spleen in preterm newborns.

Homocysteine levels and its association with intraventricular hemorrhage in preterm infants born to preeclamptic mothers.

OBJECTIVE: To investigate the relation between serum homocysteine levels and intraventricular hemorrhage (IVH) in preterm infants born to preeclamptic mothers. METHOD: This study included 84 preterm infants (42 born to preeclamptic mothers and 42 born to normotensive healthy mothers) who were admitted to Izmir Tepecik Training and Research Hospital Neonatology Clinic on the postnatal first day. The measurement of homocysteine levels in all samples were performed with an Immulite 2000 analyzer, using the chemiluminescence method. Cranial ultrasounds were performed on the fourth day and in the 1 month of age. RESULTS: The mean plasma levels of homocysteine in infants born to preeclamptic mothers and in the control group were 8.2 ± 5.9 µmol/L and 5.3 ± 2.7 µmol/L, respectively. The plasma levels of homocysteine were significantly higher in the study group (p = 0.006). There was no association between the plasma homocysteine levels and IVH or other neonatal complications including necrotizing enterocolitis, retinopathy of prematurity, bronchopulmonary dysplasia and mortality. CONCLUSION: Our data suggest that plasma levels of homocysteine are higher among infants born to preeclamptic mothers, but these high levels are not associated with IVH and other neonatal complications in preterm infants.

Incarcerated femoral hernia containing the right uterine tube. A pre-operative diagnosis is possible.

The incarcerated femoral hernia containing the right uterine tube is very rare to see. The case report is important to accumulate knowledge of very rare cases. The diagnosis of the case was established pre-operatively with abdominal computerized tomography (CT) On CT examination, the mass in the hernia sac was not connected with the intestines and a tubal structure on the right side of the uterus was shown to extend out of the abdomen. In the operation, the right uterine tube(RUT) was reduced into the abdomen after its blood supply was shown to be normal. A hernia repair was performed. Sometimes in obese patients, incarcerated femoral or inguinal hernias may not been noted. Ultrasonography, CT and magnetic resonance imaging (MRI) is used frequently to diagnose abdominal wall hernias. The organs in the incarcerated sac must be examined carefully and the viability must be checked. The surgeon must decide whether or not to resect the organs. In our case, strangulation was not found and polypropylene mesh was not used for hernia repair due to a fear of infection of the prosthesis. Incarceration of the uterine tube in the sac is traumatic and this condition may lead to infection. Such conditions may lead to ectopic pregnancy. The patient must be informed about ectopic pregnancy due to a previous incarceration of the tube because ectopic pregnancy may be fatal.