Thiol disulfide homeostasis in ionizing radiation and chemotherapeutic drug exposure.

OBJECTIVE: This study aims to reveal the oxidant and antioxidant status in nurses with chemotheropathic drug exposure and radiology unit workers exposed to ionizing radiation (IR). METHODS: Nineteen radiology unit workers, 14 nurses, and 15 controls were included the study. All of the participants using antioxidants, vitamin supplements, smokers, any therapeutic drugs, and exposed therapeutic or diagnostic X-ray or chemotherapeutic drugs in 12 months were excluded from the study. Total and native thiols, disulfide/native thiol percent ratios (SS/SH), disulfide/total thiol percent ratios, disulfide amounts, and native thiol/total thiol percent ratios, ischemia-modified albumin (IMA) were determined. RESULTS: Disulfide levels, disulfide/total thiol ratio, and disulfide/native thiol ratio of serum samples of both radiology unit workers and nurses were significantly higher and ratio of native thiol/total thiol was lower than the control group. The radiation dose in radiology unit workers was mean±SD: 0.02±0.009, median (min-max): 0.02 (0.001-0.04). Thiol-disulfide homeostasis was disturbed and the balance shifted in the direction of oxidant damage, even at low-dose IR exposure and normal range. CONCLUSION: As far as we know, the current findings first demonstrate an apparent chronic oxidative stress in the subjects who were occupationally exposed to antineoplastic drugs and radiation even if annual radiation exposure dose measurements are normal.

A novel mutation in the proteoglycan 4 gene causing CACP syndrome: two sisters report.

BACKGROUND: Camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome, caused by biallelic pathogenic mutations in the proteoglycan 4 (PRG4) gene, is characterized by early-onset camptodactyly, noninflammatory arthropathy, coxa vara deformity, and rarely, pericardial effusion. This syndrome can mimic juvenile idiopathic arthritis. CACP syndrome is caused by mutations in the proteoglycan 4 (PRG4) gene. To date, only 36 pathogenic mutations have been reported in this gene, but none have been reported from Azerbaijan. CASE PRESENTATION: Herein, we report two siblings presented with chronic polyarthritis, had a prior diagnosis of juvenile idiopathic arthritis, but was subsequently diagnosed as CACP syndrome with novel mutation in the PRG4 gene. CONCLUSION: Our report expands the knowledge of PRG4 mutations, which will aid in CACP patient counseling.

Evaluation of radiologic evidence of metabolic bone disease in very low birth weight infants at fourth week of life.

AIM: The aim of this study is to determine the incidence of metabolic bone disease (MBD) and assess the risk factors for development radiologic evidence of MBD. METHODS: Preterm infants with gestational age ≤32 weeks and birth weight ≤1500 g were included in this prospective study. Metabolic bone disease was defined as hypophosphatemia (phosphorus levels <4 mg/dl), ALP levels >450 U/L, or radiologic findings of MBD at four weeks of age. RESULTS: The study included 254 infants (gestational age: 29 (27-30) weeks, birth weight: 1130 g (960-1300)). Metabolic bone disease was diagnosed in 96 patients (37%); 48 infants had only radiologic evidence of MBD, 24 infants had only biochemical diagnosis of MBD, and 24 infants had both radiologic evidence of MBD and biochemical diagnosis of MBD. CONCLUSIONS: Our results showed that radiologic evidence of MBD existed in some infants with normal biochemical results. That finding may guide further development of screening programs for MBD.

Chronic recurrent multifocal osteomyelitis: a case report.

Chronic recurrent multifocal osteomyelitis is a rare autoinflammatory, immunologic disorder. It may involve recurrent inflammatory bone pain associated with aseptic osteomyelitis. If the diagnosis is delayed, it negatively influences quality of life by leading to persistent symptoms or joint damage. Herein, we report a 16-year-old male patient who presented with left hip and right knee pain lasting for the last two months and was diagnosed as having chronic recurrent multifocal osteomyelitis as a result of further evaluation. Improvement was achieved with prednisolone and methotrexate treatment that was initiated when no response to ibuprofen treatment could be obtained. Chronic recurrent multifocal osteomyelitis must be considered in the differential diagnosis in patients presenting with joint symptoms when clinical and radiologic bone lesions are found, no microorganism growth is observed, and no response to antibiotic treatment is obtained.

Ultrasonographic clues for diagnosis of spina bifida occulta in children.

BACKGROUND: The aim of the current study was to find out if spinal ultrasonography might have a predictive potential for detection of spina bifida occulta (SBO) in pediatric nocturnal enuresis patients. METHODS: A total of 108 children (58 females, 50 males) with a mean age of 8 (range, 6-15) years diagnosed for nocturnal enuresis in our tertiary care center were included in this cross-sectional analysis. Half of the cases (n=54, 50%) were found to have SBO, while the other half did not have SBO. After obtaining radiographs and computed tomography examinations of L5-S1 vertebra, patients were examined by spinal ultrasound regarding radiologic clues which may aid in the detection of SBO. RESULTS: The clues of "single and double echogeneous cap signs and the V-shaped tip of spine" were found useful for diagnosing SBO at levels of L5 and S1 in pediatric patients suspected for SBO. Receiver operating curve (ROC) curve analysis of CT and ultrasonographic clues for diagnosis of SBO on S1 level revealed that these clues yielded a comparable diagnostic accuracy to CT. Areas under curve for CT and studied ultrasonographic clues were are 0.667±0.053 and 0.907±0.032 (P<0.001) respectively. CONCLUSIONS: Ultrasonography seems to be a useful and practical diagnostic tool for diagnosing spina bifida. However, to implement our ultrasonographic criteria in routine radiological practice, further studies in larger series are warranted.

Miliary tuberculosis disease complicated by Pott's abscess in an infant: Seven year follow-up.

A 20-month-old boy presented with 1-year history of persistent fever, cough, and progressive abdominal distention. Abdominal ultrasonography showed hepatomegaly and multiple calcifications in the liver and spleen. Thoracic computed tomography showed multiple mediastinal lymph nodes and consolidation in both lungs. Additionally, there was a 2-cm thick retroperitoneal soft tissue mass destroying the T7-8 and L1-L2 vertebral bodies. The patient was preliminarily diagnosed with miliary tuberculosis (TB) and Pott's disease, and began administering anti-TB treatment consisting of isoniazid, rifampin, ethambutol, and pyrazinamide. Acid-resistant bacilli analysis and mycobacterial culture of the biopsy specimen of Pott's abscess were positive. Mycobacterial culture and PCR of gastric aspirate were also positive. The patient's condition progressively improved with anti-TB treatment and he received 12 months of antiTB therapy. At the end of the treatment all of the patient's symptoms were relieved and he was well except for kyphosis. Miliary TB complicated by Pott's abscess is a very rare presentation of childhood TB. The presented case shows that when Pott's abscess is diagnosed and surgically corrected without delay, patients can recover without squeal.

Antral gastritis caused by Helicobacter pylori infection in the pediatric age group is associated with increased mesenteric lymph node dimension observed by ultrasonography.

BACKGROUND: To find out if transabdominal ultrasonography (US) may have a predictive role for detection of antral gastritis and Helicobacter pylori (HP) infection in the antrum of pediatric age group. METHODS: A total of 91 (63.6%) patients and 52 (36.4%) controls were allocated into two groups as follows: Group 1 (n=91): patients with complaints and endoscopic findings consistent with gastritis and documented HP infection; Group 2 (n=52): patients with complaints and endoscopic findings consistent with gastritis in the absence of documented HP infection. These two groups were compared in terms of demographics and biggest mesenteric lymph node detected, muscularis mucosa thickness, submucosal thickness, muscularis propria thickness, and total gastric wall thickness. RESULTS: The two groups exhibited no statistically significant difference with respect to age (P=0.747), and presenting symptoms (P=0.982). However, the mesenteric lymph node dimension was significantly increased in Group 1 (P=0.039). Median mesenteric lymph node dimension was 12.9 (±15.4) mm in Group 1, while 11.0 (±12.8) mm in Group 2. No significant difference was observed between groups in terms of muscularis mucosa thickness (P=0.243), submucosal thickness (P=0.174), muscularis propria thickness (P=0.356), and total gastric wall thickness (P=0.223). CONCLUSIONS: Antral gastritis caused by HP infection in the pediatric age group is associated with increased mesenteric lymph node dimension observed by US.

Acute disseminated encephalomyelitis associated with acute Toxoplasma gondii Infection.

Acute disseminated encephalomyelitis is an acute demyelinating disorder of the central nervous system, which principally affects the brain and spinal cord. It usually follows a benign infection or vaccination in children. Although a number of infectious agents have been implicated in acute disseminated encephalomyelitis, Toxoplasma gondii infection has not been described previously in children. Acquired T. gondii infection presents with lymphadenopathy and fever and usually spontaneously resolves in immunocompetent patients. We describe a previously healthy 10-year-old boy with acute disseminated encephalomyelitis associated with acute acquired Toxoplasma gondii infection, the symptoms of which initially began with nuchal stiffness, difficulty in walking, and urinary and stool incontinence; he later had development of motor and sensory impairment in both lower extremities and classical magnetic resonance imaging lesions suggestive of the disease. The patient recovered completely after the specific therapy for acquired T. gondii infection and pulse prednisolone. Although acute acquired Toxoplasma gondii infection has not been reported previously in association with acute disseminated encephalomyelitis, clinicians should keep in mind this uncommon cause of a common disease when evaluating a patient with acute disseminated encephalomyelitis.

Value of pelvic sonography in the diagnosis of various forms of precocious puberty in girls.

PURPOSE: To evaluate the role of pelvic sonography (US) in the diagnosis and differentiation of various forms of precocious puberty in girls, and to explore reliable cutoff points of pelvic US measurements for differentiating between these conditions. METHODS: Uterine length, uterine cross-sectional area, uterine volume, fundocervical ratio, and ovarian volumes of 143 healthy girls were compared with those of 125 girls with different forms of sexual precocity. Ovaries were morphologically classified as homogenous, paucicystic, multicystic, macrocystic, and isolated cystic. RESULTS: Almost all US measurements of girls with central precocious puberty in the 0- to 8-year age group were significantly increased compared with controls and girls with premature thelarche and adrenarche (p < 0.05, except for ovarian volumes in premature thelarche group), whereas no statistically significant difference was found in the 8- to 10-year group (p > 0.05). Ovarian morphology distributions did not differ significantly between patient subgroups, but all had more mature forms compared with controls. Due to the wide overlap between the measurements, no reliable cutoff points could be determined by charting receiver operating characteristics curves. CONCLUSIONS: Pelvic US can improve the diagnosis of central precocious puberty in girls of 0-8 years of age but provides no reliable type differentiation alone. It provides no valuable information for this diagnosis in girls of 8-10 years of age.

Neurologic manifestations of novel influenza A (H1N1) virus infection in childhood.

The neurologic manifestations and prognoses of a novel influenza A (H1N1) virus infection in previously healthy children were evaluated. Nose and throat swabs were retrieved from all patients who met the criteria of influenza-like illness. A real time reverse-transcriptase polymerase chain reaction assay was used to confirm the novel influenza A (H1N1) virus. This viral infection was evident in 240 children between October 10 and December 22, 2009. Neurologic findings were evident in 17 (7.08%) patients, aged between 4 months and 8 years. Nine were boys. Five patients manifested simple febrile seizures, seven manifested complex febrile seizures or additional afebrile seizures, and three manifested encephalopathy. Febrile status epilepticus and flaccid paralysis were diagnosed in one patient each. All were treated with oseltamivir. Fifteen of 17 patients demonstrated complete recovery. One undergoing follow-up with a diagnosis of Guillain-Barré syndrome manifested sequelae. One patient died because of septic shock and disseminated intravascular coagulation. We suggest that neurologic manifestations occur quite often in children aged less than 5 years with novel influenza A (H1N1) virus infection. Most infections were benign, although a severe course is possible, and sequelae may be encountered.

Multiple visceral hematomas in a child with familial Mediterranean fever: polyarteritis nodosa.

A 14-year-old girl was diagnosed with familial Mediterranean fever (FMF) with homozygous for M694V mutation of the MEFV gene and was started on colchicine therapy 4 years before admission to our hospital. She was uncompliant to therapy and was admitted to a local hospital with complaining of fever, malaise, abdominal pain and artralgia lasting for 2 months. Multiple hypoechogenic mass lesions were detected on liver and kidneys with ultrasonography (US) and diagnosed to be hematomas by laparoscopic examination. She was referred to our hospital because of development of convulsions. On physical examination her blood pressure was 140/90 mmHg and body temperature was 39 degrees C. She was pale and extremely cachectic, with atrophic muscles of the extremities. She had diffuse abdominal tenderness and hepatosplenomegaly. Laboratory investigations revealed a hemoglobin of 9.8 g/dl, white blood cell count 9,900/mm3, platelets 213,000/mm3, erythrocyte sedimentation rate (ESR) 112 mm/h, C- reactive protein (CRP) 78 mg/L (normal < 2 mg/L) and fibrinogen 500 mg/dl. Electrolytes, renal and hepatic functions and urinalysis were normal. Examinations of peripheric blood smear and bone marrow aspiration were normal. X-rays of bones and chest showed no pathological finding. Protrombine, partial thromboplastine and bleeding times were normal. Bacterial cultures of blood, urine and stool grew no organisms. Serological tests for hepatitis B and C, cytomegalovirus, salmonella and brucella were negative.