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.

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.

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.

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.

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.

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.

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.

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.

Do not overlook acute isoniazid poisoning in children with status epilepticus.

A previously healthy 2-year-old girl was admitted with generalized convulsive status epilepticus. She was in a stupor and could respond only to painful stimuli. She also had severe metabolic acidosis. Although initial liver function tests were normal, they were found to be moderately high on the fifth day of admission; however, they dropped to their normal ranges on the twelfth day of admission. Initially, the patient was diagnosed as having idiopathic status epilepticus, and classic anticonvulsant agents, including diazepam, phenytoin, and then phenobarbital, were given. However, her seizures did not subside, and diazepam infusion was initiated. After initiation of diazepam infusion, the seizures were completely controlled. On the fourth day of admission, her parents said that she had accidentally received 20 tablets (a total dose of 2000 mg) of isoniazid just before admission to our hospital. Later, we injected 200 mg of pyridoxine intravenously. During follow-up, her general condition improved, and anticonvulsant agents were discontinued because an electroencephalogram was found to be norma. She was discharged from the hospital on the twelfth day of admission. At the fourth month of follow-up, she was seizure free. Because of this case, we would like to re-emphasize that acute isoniazid poisoning should also be considered in a child with unexplained status epilepticus.

Isolated left oculomotor nerve palsy following measles.

A 10-month-old boy was admitted with ptosis on the left eyelid, which rapidly occurred following a disease with rash about 20 days before admission to our hospital. By history, none of the vaccinations had been performed. On physical examination, his vital signs were stable, and he had marasmus. Isolated left oculomotor nerve palsy was diagnosed. Cranial magnetic resonance imaging was normal. Serum IgM antibody to measles virus was positive. Oculomotor nerve palsy markedly improved on the 15th day of follow-up, and complete improvement was noted on the second month of follow-up. To our knowledge, this is the first case of oculomotor nerve palsy following measles.

Seizures in a boy with subacute sclerosing panencephalitis during high-dose intrathecal interferon-alpha therapy.

A 27-month-old boy was admitted with speech abnormality, inability to walk, and involuntary movements. He was diagnosed with subacute sclerosing panencephalitis based on clinical and laboratory findings. Inosiplex (100 mg/kg/day orally) plus intrathecal interferon-alpha (3 million units/dose twice per week) in a standard regime were given. After four doses of interferon it was prescribed as 6 million units/dose/week because he had been admitted from a remote district. One day after giving the second dose of 6 million units of interferon, two generalized tonic-clonic seizures that occurred within an hour, associated with high fever, which lasted approximately 5 minutes were observed. An antiepileptic agent was not administered because electroencephalogram results did not indicate epileptic discharges. After this condition we returned to the first treatment protocol of interferon (3 million units/dose twice per week). At the current time, he is in the fifth month of follow-up and remains convulsion-free. To the best of our knowledge, seizures as a result of high-dose intrathecal interferon in subacute sclerosing panencephalitis has not been reported in the literature. Our patient demonstrated that it is reasonable to avoid the use of high-dose intrathecal interferon-alpha in childhood.

Onset of generalized seizures after intrathecal interferon therapy of SSPE.

An 11-year-old male was admitted with inability to walk and speech abnormality. He was diagnosed with subacute sclerosing panencephalitis on the basis of clinical and laboratory findings. Therapy with inosiplex (100 mg/kg/day orally) plus intrathecal interferon-alpha (3 million units/dose twice per week) and ribavirin (15 mg/kg/day orally) was initiated. Ribavirin was given orally because of a lack of parenteral form in our country. During follow-up, he complained about fever and widespread body pains after intrathecal therapy. On the sixth month of follow-up, generalized tonic-clonic seizures, associated with high fever, and lasting approximately 1-2 minutes occurred about 6 hours after giving interferon-alpha. Four days after the first seizures, a similar seizure attack reoccurred after intrathecal IFN-alpha. An antiepileptic agent was not administered because electroencephalogram results did not indicate epileptic discharges. At the current time, he is in the ninth month of follow-up and remains seizure-free. In conclusion, our case demonstrated that standard dose intrathecal interferon-alpha might cause seizures in children. We think that this unfortunate condition was more common in subacute sclerosing panencephalitis children treated with intrathecal interferon-alpha.

Prognostic factors in children with purulent meningitis in Turkey.

In this study the clinical and laboratory findings of 48 children with purulent meningitis were examined, prospectively, to determine the prognostic factors in childhood meningitis in a developing country. Patients were examined for the following variables: history of antibiotic use; period between onset of symptoms and hospital admission; age at presentation; sex; fever; convulsion; level of consciousness; malnutrition; anemia; leukocyte and thrombocyte counts; erythrocyte sedimentation rate; serum C-reactive protein (CRP) level; and cerebrospinal fluid (CSF) including white blood cell count; glucose, protein, and CRP concentrations; antibiotic treatment; neurological sequelae; and fatality rate during the hospital stay. Most of these parameters were re-evaluated in all patients 36-48 h after admission. Patients were divided into 3 groups: surviving without sequelae, surviving with sequelae, and not surviving (deceased). A total of 48 children, 19 girls (39.5%) and 29 boys (60.5%), aged 2 months to 13 years, were included in the study. Of the 48 patients, 29 (60.5 %) survived without sequelae, 13 (27%) survived with sequelae and 6 (12.5%) died. In a comparison among groups, we found that absence of anemia, low (< 1,000) CSF white blood cell (WBC) count, and high CRP level at admission were the indicative of poor prognosis. Thirty-six to 48 h after admission, the presence of fever, depressed level of consciousness, high (> 1,000) CSF WBC count, and low CRP level were also poor prognostic factors. In addition, we observed that mortality rate was lower in the penicillin G + chloramphenicol group than in the ampicillin-sulbactam + cefotaxime group (P < 0.05). The mean period between onset of symptoms and hospital admission was longer in the surviving with sequelae and in the not surviving groups than in the surviving without sequelae group (P < 0.05).

Report of eight infants with acute infantile hemorrhagic edema and review of the literature.

Acute infantile hemorrhagic edema (AIHE) is a cutaneous leukocytoclastic vasculitis, clinically characterized by the symptom triad of fever, large purpuric skin lesions, and edema. The clinical picture has a violent onset, a short benign course, and spontaneous complete recovery. In this article, we present eight patients who were admitted with rashes on the skin and edema on the eyelids and extremities, and were diagnosed with AIHE according to their clinical and histopathological features (immunohistological study was also performed in three of them). Our purpose was to emphasize that, aside from Henoch-Schönlein purpura, meningococcemia, septicemia, and purpura fulminans, AIHE benign disorder should also be considered in the differential diagnosis to determine the clinical course and treatment protocol in patients with purpuric rashes.

Report of eight children with amitraz intoxication.

Many pesticides are formulated in organic solvents. An example is amitraz, one of the formamidine groups of pesticidal chemicals. It is commonly used for the treatment of generalized demodicosis in dogs and for the control of ticks and mites in cattle and sheep. In this article, the clinical and laboratory findings of eight children with amitraz intoxication are reviewed. The purpose was to enlighten the findings of amitraz intoxication in children. Of the eight patients, five (62.5%) were boys, three (37.5%) were girls, and the ages ranged from 1 to 4 years. All children accidentally ingested amitraz orally, with no dermal exposure. The most common observed signs were decreased consciousness and bradycardia. Leukocytosis, hyperglycemia, hypernatremia, increased serum aspartate transaminase level, and prolonged partial prothrombin time were diagnosed in children. None of the children had hypothermia, hypotension, or convulsion and none of the patients died. The findings show that the initial signs and symptoms of acute amitraz intoxication appeared severe but they disappeared, with only supportive care needed in most cases within a few days.

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.

Hyperkalemia most likely associated with massive cephalhematoma in a newborn infant who was treated with urgent peritoneal dialysis: case report.

Cephalhematomas rarely lead to serious complications such as infection, osteomyelitis and skull fractures. However, we present a newborn infant with hyperkalemia in the context of a serious complication believed to be caused by hemolysis of a large cephalhematoma. The patient was treated with urgent peritoneal dialysis and discharged with a successful outcome. In conclusion, neonates with massive cephalhematoma should be closely examined in terms of bilirubin counts as well as electrolyte counts.

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.