18F-FDG PET/CT Findings Overlapping Lymphoma in a Patient with Systemic Juvenile Idiopathic Arthritis.

Systemic juvenile idiopathic arthritis (sJIA) is an important autoinflammatory disease whose first symptom is usually fever, and life-threatening conditions such as macrophage activation syndrome can develop when diagnosis and treatment is delayed. sJIA is an exclusion diagnosis, and there is no specific test that distinguishes it from other febrile diseases. We report the positron emission tomography/computed tomography (PET/CT) findings of sJIA in a 12-year-old girl who presented with fever, rash, and arthralgia. 18F-fluorodeoxyglucose (FDG) uptake was observed in the spleen, bone marrow, and lymph nodes in 18F-FDG PET/CT performed to investigate the etiology of fever of unknown origin. The result of excisional biopsy performed with the suspicion of lymphoma from the left cervical lymph node with intense 18F-FDG uptake was reported as reactive hyperplasia. PET/CT is an alternative diagnostic method for patients with fever of unknown origin. In this case report, we emphasize that in patients with sJIA, there may be intense fluorodeoxyglucose-avid lymph nodes that may lead to the consideration of lymphoproliferative disease, and PET/CT findings along with spleen and bone marrow involvement may overlap with lymphoma.

Paediatric systemic lupus erythematosus: A single referral centre experience.

In this study, the clinical and laboratory findings, management and follow-up of 32 children with paediatric systemic lupus erythematosus (pSLE) were evaluated to determine the prognostic factors in pSLE. Of the 32 patients, 25 (78.1%) were females. Age at onset of symptoms and diagnosis in the patients were 147.6 ± 49 months and 154.3 ± 48 months, respectively. The most common symptom on admission were joint problems, seen in 25 (78.1%) patients. Haematological alterations were seen in 25 (78.1%) cases during follow-up. Lupus nephritis was diagnosed in 10 (31.2%) patients. Malar rash was seen in a total of 12 (37.5%) patients during follow up, however it had been noted in five (15.6%) patients on admission. Antinuclear antibody and anti-dsDNA were positive in all patients and 31 (96.8%) patients, respectively. Decreased complement 3 and 4 levels were noted in 23 (71.8%) patients. Antiphospholipid antibody was studied in 27 patients and it was found to be positive in 13 (48.1%) patients. In conclusion, based on our findings, we would like to emphasize that pSLE has a large and remarkable clinical and laboratory findings.

Early kidney injury in immunoglobulin A vasculitis: Role of renal biomarkers.

BACKGROUND: We aimed to determine whether urine kidney injury molecule 1 (KIM-1) and neutrophil gelatinase-associated lipocalin (NGAL) can be used as early noninvasive biomarkers of kidney injury in immunoglobulin A vasculitis. METHODS: Patients who were diagnosed with immunoglobulin A vasculitis were included in the study. Urine samples were collected for determination of urine KIM-1 and NGAL levels. The control group consisted of age-matched healthy children. RESULTS: Sixty-one patients who were diagnosed with immunoglobulin A vasculitis were included in the study; 37.7% of these patients were determined to have renal involvement. Median KIM-1 was found to be significantly higher in the patient group (69.59 pg/mL) than the control group (40.84 pg/mL) (P = 0.001). Median NGAL was determined to be statistically significantly higher in the patient group (59.87 ng/mL) compared with the control group (44.87 ng/mL) (P = 0.013). In 23.6% of the patients without renal involvement at admission renal involvement developed within the following 6 months. When median KIM-1 and NGAL at admission of these patients were compared with the control group, they were determined to be statistically significantly higher (P = 0.001, P = 0.003). CONCLUSIONS: The fact that our patients with late-term nephropathy had no hematuria and / or proteinuria and that KIM-1 and NGAL levels were determined to be high indicates that these biomarkers might be potentially reliable, noninvasive and early determinants of kidney injury.

Use of propranolol in children with primary nocturnal enuresis.

In nocturnal enuresis, motivational therapy, alarm therapy, and drug therapy, such as anticholinergics, imipramine, and sertraline, are the mainstay of treatment. In the present study, we used motivational therapy, oxybutynin, and propranolol in children with primary nocturnal enuresis to determine if propranolol is an effective treatment. Fifty-two children with primary nocturnal enuresis were included in the study. Firstly, motivational therapy was given for 1 month to all patients. Patients who failed the motivational therapy were randomly given oxybutynin or propranolol. The patients were re-evaluated after 1 month of drug therapy. There was not a significant difference between oxybutynin and propranolol groups for initial frequency of nocturnal enuresis ( p > 0.05). Of 52 patients, 28 (53.8%) patients improved by motivational therapy. There were 14 patients in the oxybutynin group. One patient was excluded from the study because facial flushing and mouth drying developed in the first week of oxybutynin therapy. In oxybutynin group, 12 of 13 (92.3%) patients improved. There were 10 patients in the propranolol group. In the propranolol group, while nine (90%) patients did not improve, one patient had significant remission (90%, p < 0.001). No significant adverse reaction was noted during propranolol therapy. There was no significant difference between oxybutynin and propranolol groups for initial frequency of nocturnal enuresis (p > 0.05). A significant difference was found between the groups for the remission of nocturnal enuresis ( p < 0.001). Our findings showed that motivational therapy is the first line treatment in primary nocturnal enuresis, and oxybutynin but not propranolol is effective in patients who failed with the motivational therapy.

Canakinumab treatment in four children with colchicine resistant familial mediterranean fever.

Familial Mediterranean Fever (FMF) is an autosomal recessive and autoinflammatory disease, characterized with inflammation of serous membranes such as peritoneum, pleura, synovium with fever and pain. Colchicine is the main treatment of FMF, but 5-10 % of patients are unresponsive to colchicine. We report using anti-interleukin-1 agents anakinra and canakinumab in four colchicine-resistant patients who were successfully treated. Three of the patients were siblings.

Spontaneous rupture of the spleen in a patient with systemic lupus erythematosus initially presented as Evans syndrome.

BACKGROUND: Although splenic abnormalities are common in patients with lupus, spontaneous rupture of spleen is extremely rare. OBSERVATIONS: A 15-year-old boy with new-onset Evans syndrome subsequently diagnosed as systemic lupus erythematosus developed spontaneous rupture of spleen during the course of his illness. Despite the severe thrombocytopenia, he was managed conservatively with gradual regression of hematoma without further complication. CONCLUSIONS: Splenic rupture may occur spontaneously in the course of systemic lupus erythematosus. We conclude that conservative treatment of splenic rupture may be preferred especially in immunocompromised patients to avoid surgical complications.

Frequency of congenital hypothyroidism in neonates in the Konya region, Turkey.

Congenital hypothyroidism (CH) resulting from deficient production of thyroid hormone is one of the most commonly encountered diseases in pediatric endocrinology. Thyroid hormones play a crucial role in normal cerebral and growth maturation. These harmful effects on the cerebral and growth maturation can be prevented by early diagnosis and sufficient treatment in the first weeks of life. Diagnosis must be determined immediately within days after birth and effective treatment must begin. Unfortunately, despite the presence of national neonatal screening programs, CH cases are still rarely seen. In our study, it was aimed to assess the outcome of having determined an early diagnosis of CH and initiating treatment with thyroid stimulating hormone (TSH) screening test on live born babies over a period of 7 years in our hospital. With this aim, 93,897 live births were evaluated in the Doctor Faruk Sükan Obstetrics and Pediatrics Hospital between the years of 1999 and 2007. All neonates were screened with the TSH test. CH was determined in 43 (1/2183) of all cases and treatment was begun. The importance of this test was emphasized in that the test should be performed routinely on all neonates to obtain an early diagnosis and so that treatment for CH can begin.

Frequency of congenital hearing loss in 43,503 healthy newborn infants in Konya, Turkey.

OBJECTIVE: To determine the frequency of congenital hearing loss (CHL) in healthy newborn infants in Konya, Turkey. METHODS: A total of 43,503 healthy neonates born at Doctor Faruk Sükan Obstetrics and Children's Hospital between the years of 2006 and 2011 were evaluated, prospectively. Hearing screening test was carried out using Transient Evoked Otoacoustic Emission (TEOAE) method. Hearing test was also repeated three times in neonates with suspected hearing loss in first month of life. Infants with abnormal hearing test were referred to Department of Ear, Nose and Throat Selçuk University, Meram Faculty of Medicine, Konya, Turkey. Evoked auditory brainstem responses (E-ABR) were performed in these cases. Infants with abnormal E-ABR were referred to further centers for cochlear implantation. RESULTS: Hearing test was found to be abnormal in 226 (5.19/1,000) infants based on the results of TEOAE, repeated three times. CHL was diagnosed in 216 (4.97/1,000) of 226 infants at the end of E-ABR. These 216 infants were referred for cochlear implantation. CONCLUSION: Our findings showed that TEOAE method is highly effective in neonatal hearing screening and frequency of CHL was 4.97/1,000 in our region.

Chronic mercury poisoning: Report of two siblings.

Mercury exists as organic inorganic and elementary forms in nature and is one of the most toxic metals that are poisonous for human beings. Mercury is commonly used in many different sectors of industry such as in insects formulas, agriculture products, lamps, batteries, paper, dyes, electrical/electronic devices, jewelry, and in dentistry. In this study, two siblings (one a 7-year-old boy and the other a 13 years old girl) are reported who developed chronic mercury poisoning as a result of long-term contact with batteries. Our aim is to emphasize the importance of mercury poisoning that is extremely rarely seen in childhood.

The prevalence of urinary lithiasis in children in Van Region, Turkey.

We aimed to determine the prevalence and etiology of urinary lithiasis in childhood in our region, Van, Turkey. A total of 1120 children were included in the study. Urinary ultrasonography was performed in all the children between April 2003 and June 2003. During the study, the kidneys were examined longitudinally and transversally using a 3.75 MHz convex probe in a Hitachi EUB -315 ultrasonography machine. The children's ages ranged from 7 years to 14 years (10.16 +/- 1.92 years) and 572 (51.1%) were males and 548 (48.9%) females. Urinary ultrasonography showed that 19 (1.7%) children had urinary lithiasis, which was in the right kidney in 15 children and in the left kidney in four children. Urinary lithiasis was in the upper urinary tract in all children. The etiological studies showed metabolic disorder in 14 children, and congenital renal anomaly in one child, but no underlying cause was diagnosed in four children. In conclusion, we found that was the prevalence of urinary lithiasis was 1.7% in school-aged children in our region. It was also noted that all urinary lithiasis was in the upper urinary system and its most common cause was metabolic disorder.

Lymphocytes subsets in children with febrile convulsions.

In this study, lymphocytes subsets including blood CD3, CD4, CD8, CD16, CD19, and CD56 values were analyzed in children with febrile convulsion (FC) to determine whether there was the association of lymphocytes subsets in the pathogenesis of FC. The study includes 48 children with FC, and 55 healthy age matched control subjects, followed in Yüzüncü Yil University, Faculty of Medicine, Department of Pediatrics between October 2003 and June 2004. Blood CD3, CD4, CD8, CD16, CD19, and CD56 values were examined in the study and control groups. The analyses were performed in the Hematology Laboratory, Yüzüncü Yil University Faculty of Medicine, with flow cytometer device (Coulter Epics XL2, Flow Cytometer). A total of 48 children [17 girls (35.5%) and 31 boys (64.5%)], aged 6 months to 60 months (mean 22.20 +/- 13.75 months) with FC and 55 healthy children [28 girls (51%) and 27 boys (49%)], aged 6 months to 60 months (mean 28.87 +/- 17.04 months) were included in the study. When compared with the control group, the study found significantly decreased blood CD3 and CD4 values in the study group (p <.05). However, there was not significant difference in CD8, CD16, CD19, and CD56 values between the control and study groups (p >.05). When comparing the children with and without positive family history for FC, the study did not find any difference for all CD values between the groups (p >.05). Similarly, there was not significant difference in CD values between the children with simple and complex FC (p >.05). The findings suggested that decreased blood CD3 and CD4 values might be responsible for the infections connected with FC or that they might be related to the pathogenesis of FC in some children.

Bilateral brachial plexopathy complicating Henoch-Schönlein purpura.

An 11-year-old boy presented with convulsion, fever, rash, abdominal pain, swelling on the eyelids, elbow and wrists, oliguria and hematuria. Based on the abnormal findings the patient was diagnosed with Henoch-Schönlein purpura. On the 3rd day of admission, neurological examination showed ataxic gait, loss of deep tendon reflexes, and decreased (4/5) of muscle strength on all extremities. Additionally, bilateral loss of touch, pain and temperature sensation in a glove, from the elbows to distal region (on C5-T1 level) was diagnosed. Cerebrospinal fluid examination and cranial magnetic resonance imaging (MRI) were normal. The patient was discharged with oral prednisolone on the 7th day of admission. One week after discharging from the hospital, he was re-admitted with vertigo and seizures. He was in coma. MRI of cranial, cervical and cervical plexus were normal. Electromyography showed severe bilateral brachial plexopathy. Prednisolone and intravenous immunglobulin (IVIG) therapy were given without significant improvement. He was discharged from the hospital on the 17th day of admission. On the second month of follow-up, a second cure of IVIG was given because of no clinical improvement. Now, he is on the 4th month of follow-up, unfortunately, no improvement was noted on his muscle strength and sensorial abnormalities on the upper extremities.

Cranial MRI findings in children with protein energy malnutrition.

In this study, cranial magnetic resonance imaging (MRI) findings were investigated in children with moderate and severe protein energy malnutrition (PEM) to determine cerebral abnormalities in malnutrition in childhood. A total of 20 children aged 3 months to 36 months were included in the study. Thirteen (65%) children had severe malnutrition and seven (35%) children had moderate malnutrition. Fifteen (75%) children had abnormal MRI findings: all of them had cerebral atrophy, and 10 (75%) children had cerebral atrophy plus ventricular dilatation. None of the children had abnormality in the brain stem or cerebellum. The authors did not find statistically significant differences between the groups when comparing the MRI findings for degree of malnutrition, head circumference, iron deficiency anemia, and serum albumin levels. In conclusion, the findings showed that most (75%) children with moderate/severe PEM had abnormal MRI findings. Therefore, it is suggested that children with PEM should be evaluated for cerebral atrophy.

Four children with colchicine poisoning.

Colchicine poisoning is a rare event. It is characterized by multiorgan involvement and by poor prognosis associated with overdose. In this article we present four children with colchicine poisoning to emphasize that colchicine poisoning has a large spectrum in childhood. The children's ages ranged between 1 year and 3.5 years. The ingested dosage of colchicine was between 0.37 and 1.72 mg/kg. Most of the findings of colchicine poisoning such as gastrointestinal symptoms, hepatotoxicity, cardiotoxicity, bone marrow suppression, hypocalcaemia and hair loss were diagnosed in our patients. Two children receiving 0.37 mg/kg and 1 mg/kg colchicine and admitted 13 and 19 hours after poisoning, respectively, died. Our findings suggest that in addition to amounts of the drug, mortality was also related to the duration between drug ingestion and admission to hospital.

Measles is still a severe problem in Eastern Turkey.

In this study, we reviewed the demographic and clinical findings of 143 children with measles to draw attention the importance of measles and its complications in Eastern Turkey. Of 143 patients, 75 (52.5%) were boys, 68 (47.5%) were girls. The patients' age ranged from 5 months to 13 years (3.97 +/- 3.11 years). The peak of admissions (37%) occurred in the age range five to 24 months. The majority of the cases (84.7%) were not immunized against measles. Of 143 cases, 104 (72.7%) cases were malnourished. Of 143 children, 57 (40%) children had one or more complication of measles and the most common complication was pneumonia. Two (1.3%) children died. In conclusion, our findings showed that measles and its complications were severe problem in our country. We think that it is primarily related to very low socioeconomic status of our region.

Cranial computed tomography in purulent meningitis of childhood.

The cranial computed tomography (CT) findings of 48 children with purulent meningitis were examined, prospectively, to determine the importance of cranial CT findings on the prognosis of childhood meningitis, in a developing country. The age of children ranged from 2 months to 13 years. Of 48 patients, 29 (60.5%) survived without sequelae, 13 (27%) survived with sequelae, and six (12.5%) died. Cranial CT was normal in 21 (43%) patients of 48 children with meningitis at admission. Abnormal CT findings were detected in 10, 11, and 6 children in the groups of survived without sequelae, survived with sequelae, and deaths, respectively, at admission (p <.05) We found that CT scan results were correlated with neurological signs (p <.05). At least one or more cranial CTs were was re-taken in children in whom the first CT revealed abnormal findings; we did not find a statistically significant difference for the follow-up CT findings between the groups (p >.05). Hydrocephalus and subdural effusion were the commonest abnormal CT findings. In conclusion, our findings showed that cranial CT may safely be used to detect intracranial complications of meningitis in childhood and the ratio of sequelae and death were more common in children with abnormal cranial CT than those of normal cranial CT findings. Additionally, there was a positive correlation between CT scan results and neurological signs.

PFAPA syndrome mimicking familial Mediterranean fever: report of a Turkish child.

The PFAPA (Periodic Fever, Aphthous stomatitis, Pharyngitis, Adenitidis) syndrome is characterized by periodic fever, adenitis, pharyngitis, and aphthous stomatitis. Herein, we present a Turkish child with PFAPA syndrome mimicking familial Mediterranean fever because of a rare presentation. A 9-year-old boy was admitted with recurrent fever, aphthous stomatitis, sore throat, headache, and general body pains, lasting 2 to 3 days since 3.5 years of age. He was completely symptom-free between the attacks. He was diagnosed as having familial Mediterranean fever according to the clinical findings when he was 6 years of age and Colchicum tablet was administrated. Despite colchicines therapy for 8 months, his attacks did not subside; therefore, the drug was discontinued. He had high fever, a painful cervical lymphadenopathy, aphthous stomatitis, and tonsillo-pharyngitis. The patient was then diagnosed as having PFAPA syndrome. He was given a single dose of prednisolone (0.35 mg/kg/dose). His complaints dramatically and completely disappeared 3 h after administration of the drug. During the 8th month of follow-up, a similar febrile attack lasting only 1 day was noted and it was controlled with a single dose of prednisolone (0.5 mg/kg/day). At this writing the patient is in the 12th month of follow-up, and there have been no symptoms after the second attack. In conclusion, our patient shows that PFAPA syndrome can be confused with familial Mediterranean fever. We also would like to emphasize that the typical PFAPA syndrome can be easily diagnosed by detailed history-taking and physical findings.