Turkish Journal of Pediatrics最新文献

Background: The clinical presentation of mercury (Hg) intoxication may mimic rheumatic diseases. Hg exposure is associated with systemic lupus erythematosus (SLE)-like disease in genetically susceptible rodents and Hg is among the environmental factors in the development of SLE in humans. Herein, we presented a case with clinical and immunological features suggestive of SLE but diagnosed with Hg intoxication.

Case: A thirteen-year-old female with myalgia, weight loss, hypertension and proteinuria was referred to our clinic for the evaluation of possible SLE. Physical examination of the patient was unremarkable except for a cachectic appearance and hypertension, laboratory investigation revealed positive anti-nuclear antibody, dsDNA antibody and hypocomplementemia with nephrotic range proteinuria. Inquiry for toxic exposures revealed a continuous exposure to an unknown silverly shiny liquid for a month which was thought to be Hg. Due to the fulfillment of Systemic Lupus International Collaborating Clinics (SLICC) classification criteria for SLE, a percutaneous kidney biopsy was performed whether proteinuria resulted because of the Hg exposure or flare of lupus nephritis. Blood and 24-hour urine Hg levels were high, and no findings associated with SLE were observed in the examination of the kidney biopsy. The patient was diagnosed with Hg intoxication and, clinical and laboratory findings, including hypocomplementemia, positive ANA and anti-dsDNA antibody, improved with chelation therapy. Also, no findings associated with SLE were observed in the follow-up of the patient.

Conclusions: In addition to the toxic effects, Hg exposure may cause autoimmune features. As far as we know, this is the first-time Hg exposure was associated with hypocomplementemia and anti-dsDNA antibody in a patient. Also, this case highlights the inconvenience of the use of classification criteria for diagnostic purposes.

Background: Mass lesions of the umbilical cord are rare anomalies. There have been rare reports of hemangiomas of the umbilical cord, but the co-occurrence of omphalocele and hemangioma of the umbilical cord has not been previously reported. Nonetheless, the condition is clinically significant as it may cause the disturbance of intrauterine fetal circulation, retardation of fetal growth and development, non-immune hydrops fetalis, morbidity and mortality.

Case: Here we aim to report a case that was prenatally diagnosed with an omphalocele and that presented after birth with a hemangioma on the omphalocele sac.

Conclusions: When dealing with umbilical mass lesions in the prenatal and postnatal periods, a hemangioma on the omphalocele sac should be considered in the differential diagnosis of patients when an omphalocele is suspected.

Background: Kidney involvement related to infective endocarditis (IE) may present with different clinical findings. The most common histopathological finding of renal involvement is a combination of proliferative and exudative glomerulonephritis. However, severe acute kidney injury (AKI) induced by crescentic glomerulonephritis (CGN) is extremely rare in children with IE. To date, only 4 pediatric cases with IE-induced CGN had been reported. We present a 14-year old girl with IE-induced CGN.

Case: A 14-year old girl with fever, macroscopic hematuria, oliguria, and acute kidney injury (AKI) was admitted to our clinic. The medical history revealed that the patient had undergone several cardiac interventions due to truncus arteriosus type 1, and she recovered from IE-induced glomerulonephritis following antibiotherapy six months ago. During admission, the patient was diagnosed with IE according to one major (positive imaging finding) and three minor (fever, predisposing cardiac disease, and immunological criterion) criteria. Immediate antibiotic treatment was initiated. A kidney biopsy was performed, which showed crescentic glomerulonephritis (CGN with crescents, > 50%). Daily pulse steroid (3 days), monthly pulse cyclophosphamide (6 doses), and oral steroid (2 mg/kg/day) therapy were initiated with gradual dose tapering. The patient underwent 12 hemodialysis sessions until the 38 < sup > th < /sup > day of the treatment. She was discharged on the 45th day of treatment with normal kidney function tests and negative acute phase reactants. Treatment was maintained with mycophenolate mofetil (MMF) after a 6-month course of cyclophosphamide. MMF was discontinued in the 12th month. At the 18thmonth follow-up visit the patient had mild proteinuria, and was on ramipril therapy.

Conclusions: The occurrence of CGN should be considered in children with predisposing cardiac disease, who develop hematuria, proteinuria, and severe AKI. Although antibiotic therapy alone is often sufficient in this immune complex GN induced by infection, early initiation of additional immunosuppressive therapy in the presence of CGN may be beneficial for long term preservation of kidney functions.

Background: Multisystem inflammatory syndrome (MIS-C) is the most important complication of COVID-19 in the pediatric population. Unfortunately, this problem is an unpredictable situation in patients with COVID-19. We aimed to evaluate the effects of MIS-C on thymus dimensions in pediatric patients.

Methods: We retrospectively analyzed the files of 368 pediatric patients aged 2-18 years, who were diagnosed with COVID-19. Computer Tomography (CT) images of 22 patients diagnosed with COVID-19 and 10 patients diagnosed with MIS-C were evaluated in detail by two board-certified radiologists. Eighteen age and sexmatched patients who applied to the emergency department of our hospital for any reason and had a CT scan for any reason were selected as the control group. The data of both groups were statistically compared.

Results: Considering the differences between the groups in terms of laboratory data, monocytes, hemoglobin, and platelet were significantly lower in the MIS-C group than the other groups. Procalcitonin, C- reactive protein, neutrophil to lymphocyte ratio (NLR), platelet to lymphocyte ratio (PLR), and proBNP levels were statistically significantly higher in the MIS-C group compared to the other groups Regarding the differences in thymus dimensions, thymus AP diameter, transverse diameter, length, thickness, and volume were significantly higher in the MIS-C group than in the other groups There was a significant positive correlation between the transverse diameter of the thymus and CRP, procalcitonin, pro-brain natriuretic peptide (proBNP), and NLR levels.

Conclusions: Our study shows that thymus dimensions and acute phase reactants are higher in pediatric patients in the MIS-C group. Also, thymus transverse diameter, thymus thickness, and PLR values pose a risk for the development of MIS-C. More research is needed on the role of the thymus gland in the pathogenesis and diagnosis of MIS-C.

Background: All drugs may cause hypersensitivity reactions. Anaphylaxis is a medical emergency that rarely occurs in newborns due to immature immunity. Early diagnosis and treatment are life-saving. Vancomycin, a glycopeptide antibiotic with bactericidal action against Gram-positive bacteria, is commonly used for neonatal nosocomial sepsis.

Case: We hereby present a premature infant (born at the 33rd week of gestation, birth weight: 1745 grams) who was started on vancomycin on postnatal day 7. He had severe circulatory failure and stridor during infusion on day 7 of vancomycin treatment and his tryptase level was elevated to 64.60 micrograms/L Conclusions. To the best of our knowledge, there is no neonatal case of anaphylaxis due to vancomycin in the literature. Neonatologists should keep in mind that an anaphylactic reaction with a fatal course may develop during vancomycin infusion.

Background: The aim was to assess the success of a three-drug regimen, consisting of cefazoline, metronidazole and gentamicine, for the antimicrobial treatment of complicated appendicitis and to investigate predictors of failure.

Methods: This retrospective study included patients who had undergone appendectomy for complicated appendicitis from 2013 to 2018. The shift to second-line antibiotics was considered a failure. The choice was based upon clinical deterioration. Patients were grouped into 2 groups: localized complicated appendicitis (LCA) and extensively complicated appendicitis (ECA) for the study purpose. Univariate and multivariate analysis were performed to identify predictors of failure.

Results: Ninety patients (65.2%) with LCA and 48 patients (35%) with ECA were included. Three-drug regimen failed in 50 patients (36%) with a higher rate in the ECA group (50%, p=0.017). In a multivariate analysis, this failure was found to be associated with ECA (adjusted OR 3.00 [1.2-7.4], p=0.041). Children with ECA experienced a longer hospital stay (median length 8 days, p < 0.001) and antimicrobial therapy (median length 8 days, p < 0.001). However, no difference in the rate of surgical site infections was found (p=0.514).

Conclusions: The institutional antibiotic stewardship program highlighted a high failure rate for the old threedrug regimen. A new protocol should be recommended, especially for the patients affected by ECA.

Background: Hepatic glycogen storage diseases are a group of diseases manifesting mainly with hypoglycemia and hepatomegaly. The patients require frequent daytime and nocturnal feedings. Hypoglycemia may cause sensorineural hearing loss and nocturnal feeding is a risk factor for the development of gastroesophageal reflux that may cause chronic otitis media and hearing loss consequently. We aimed to determine the prevalence and characteristics of hearing loss in hepatic glycogen storage diseases.

Methods: A total of 24 patients with hepatic glycogen storage disease (15 glycogen storage disease type I and 9 non type I) and 24 age/sex matched healthy controls were enrolled in the study. Pure tone audiometer, immitansmetry, acoustic reflex measurement, otoacoustic emission test (OAE) and auditory brainstem response (ABR) tests were applied to all participants.

Results: Hearing loss was determined in 17/24 patients (12 glycogen storage disease type I and 5 non type I) with pure tone audiometer. Interpretation of all the findings revealed a total of 8 patients had conductive and 9 had mixed hearing loss. All parameters were significantly different than the control group.

Conclusions: This is the first study to comprehensively assess the auditory functions of patients with hepatic glycogen storage disease. Audiological findings determined a significantly increased prevalence of conductive/ mixed type hearing loss in the patient group which is a new finding in the literature. Further studies with extended patient numbers are required to enlighten the underlying pathophysiology.