Mehmet Kose

Rickettsialpox in Turkey.

To the Editor: Rickettsialpox is often described as a chickenpox-like disease and is caused by Rickettsia akari, a spotted fever group Rickettsia that is transmitted to humans by the bite of mites (Liponyssoides sanguineus). Although the mite host (typically a mouse) is widely distributed in cities, the disease is infrequently diagnosed. It is typically characterized in patients by the appearance of a primary eschar at the site of a mite bite followed by fever, headache, and development of a papulovesicular rash. Symptoms normally appear 9–14 days after the mite bite and are often unnoticed by the affected person. In documented rickettsialpox cases, the presence of a papule that ulcerates and becomes a scar approximately 0.5–3.0 cm in diameter is reported (1–3). Three to 7 days later, symptoms are more pronounced, with patients experiencing the sudden onset of chills, fever, and headache followed by myalgia and the appearence of generalized vesicular skin rashes. Less frequently, photophobia, conjunctival injection, cough, generalized lymphadenopathy, and vomiting are reported. The first well-described clinical case of rickettsialpox was documented in New York City in 1946 (1). Historically, most documented rickettsialpox cases have occurred in large metropolitan areas of the United States (2), where the causative agent, R. akari, circulates primarily between the house mouse (Mus musculus) and its mite (Liponyssoides sanguineus). Recently, rickettsialpox cases have been reported from Croatia, Ukraine, South Africa, Korea, and North Carolina (3,4). R. akari was isolated from the blood of a patient suspected of having Mediterranean spotted fever rather than rickettsialpox; this was the first human isolate of R. akari reported in >40 years (4). Recent reports of a rickettsialpox case in North Carolina (3), R. akari seropositivity found in HIV-positive intravenous drug users in the inner city of Baltimore, Maryland (5), and in Central and East Harlem, New York City (6), as well as rickettsialpox cutaneous eruption in an HIV patient in New York (7), indicate that R. akari rickettsiosis is more common than previously thought and presents the risk of sporadic outbreaks worldwide. We describe the clinical presentation of rickettsialpox in a 9-year-old boy from Nevpehir, located in the middle region of Turkey. Previously, a report from the Antalya area of Turkey described the prevalence of serum immunoglobulin (Ig) G antibodies in humans directed against R. conorii (spotted fever group Rickettsia) (8); however, rickettsialpox was not reported in Turkey. This report of what we believe to be the first described rickettsialpox case from Turkey further extends the recognized geographic distribution of R. akari. A 9-year-old boy was admitted to the Kayseri hospital with fever >39°C and generalized papulovesicular exanthema. One week before admission, fever, profuse sweating, headache, and dysuria were present. On admission, physical examination indicated generalized vesicular, bullouse, and papular exanthema involving the lips and oral cavity. Notable pathologic findings at admission included a black eschar on the boy’s penis, bilateral prominent conjunctival ejection, and bilateral lower pulmonary rales. The leukocyte count was 13,300/mm3, hemoglobin was 14.49 mg/dL, and the platelet count was 544,000/mm3. Serum electrolytes and blood urea nitrogen levels and results of coagulation study and urine analysis were normal. Routine blood cultures taken 24 hours postadmission were sterile. Specific antibodies (IgG; IgM) against Varicella were not detected in serum samples (Duzen Laboratories, Ankara, Turkey). Additionally, the patient reported mice on the family’s farm. A diagnosis of rickettsialpox was made and doxycycline treatment (200 mg/kg) was initiated. The patient serum sample was tested by indirect immunofluorescence assay (IFA) for IgG and IgM antibodies reactive with R. akari (Kaplan strain), R. typhi (Wilmington), R. rickettsii (Sheila Smith), and R. conorii (Malish 7). Serum IgG titers of 1/1280 and IgM of 1/40 to R. akari were detected and confirmed through cross-adsorption with rickettsial antigens (R. rickettsii, R. conorii) (9,10). Higher reciprocal titers were obtained against R. akari antigens than against R. rickettsii and R. conorii antigens (reciprocol titers of 1,024 vs. 512 and 512, respectively). We observed a difference in reduction in antibody titers against R. akari after adsorption with R. akari (Kaplan) (<16), R. rickettsii (256), and R. conorii (256). Antibodies against R. typhi were not detected. The IFA result confirmed the clinical diagnosis of R. akari infection. After 2 days of doxycycline treatment, the patient was afebrile, and the rickettsialpox infection resolved without scars or complications. In summary, we present a case in which the presence of an eschar on the patient’s penis, the failure of lesions to appear in crops, the sparsity of lesions, and mice on the family’s farm led to a diagnosis of rickettsialpox, which was confirmed by cross-adsorption serologic findings. This case indicates that rickettsialpox is an emerging infectious disease in Turkey. We recommend further studies to define the prevalence of R. akari and the worldwide distribution of rickettsialpox.

Management of Hyperleukocytosis and Prevention of Tumor Lysis Syndrome with Low-Dose Prednisone Continuous Infusion in Children with Acute Lymphoblastic Leukemia

Introduction: The standard management of childhood acute lymphoblastic leukemia with hyperleukocytosis is unclear and its treatment has focused on prompt leukocytoreduction. Cytoreductive therapies have been used for the prevention of tumor lysis syndrome, but the outcomes have been variable and their benefits have not been proven in controlled clinical trials. This condition needs further investigation to develop effective therapeutic strategies. Methods: In the present prospective trial, 15 children with acute lymphoblastic leukemia and hyperleukocytosis (range 101–838 × 109/l) were treated with intravenous low-dose prednisone continuous infusion (6 mg/m2/24 h). Doses were increased daily and on approximately the fifth day, the full dose of prednisone (60 mg/m2/day) was applied. Results: The mean reduction in white blood cell count achieved by this treatment was 34.4% on first day, 56.9% on second day and 76.6% on third day. The treatment was well tolerated. None of the 15 patients developed life-threatening metabolic disorders or required dialysis. Conclusions: Intravenous low-dose prednisone continuous infusion treatment can prevent the progression to tumor lysis syndrome and it may be used for the patients presenting with white blood cell counts between 100 and 400 × 109/l in centers where leukoapheresis is not readily available.

Thoracic MRI evaluation of sarcoidosis in children.

Childhood sarcoidosis is a very rare granulomatous disorder with an unknown etiology. Stage 1 disease is the most common whereas stages 2, 3, and 0 are rare in children.

Transverse myelitis and acute motor sensory axonal neuropathy due to Legionella pneumophila: A case report

Guillain–Barré syndrome is a rapidly progressive symmetrical muscle weakness associated with acute inflammatory disease. Transverse myelitis (TM) is the inflammation of the spinal cord characterized by rapidly evolving muscle weakness in the lower extremities, defects in sensory level and sphincter dysfunction. Guillain–Barré syndrome, and TM association occurs very rarely in childhood. A 7‐year‐old girl presented with complaints of neck pain, spout‐style vomiting, cough, shortness of breath, and acute paraparesis with sensory and sphincter disturbance. The patient was intubated because of increased respiratory distress. A positive direct fluorescein antigen test in bronchoalveolar lavage confirmed Legionella pneumophila infection. Imaging and neurophysiologic studies were diagnostic for TM with acute motor and sensory axonal neuropathy. She was treated with a combination of high‐dose methylprednisolone and intravenous immunoglobulins, and we observed incomplete recovery. The presented case is the first child with concomitant TM and acute motor and sensory axonal neuropathy related to L. pneumophila infection.

Predictive value of gelsolin for the outcomes of preterm neonates: A pilot study

Plasma gelsolin is a circulating actin‐binding protein that has a protective role against tissue injuries. Our aim was to compare the baseline levels of gelsolin in premature infants with neonatal outcomes.

Isotretinoin-Induced Agranulocytosis

Abstract:  Isotretinoin is used to treat a spectrum of dermatologic conditions. The present article reports the case of a 15‐year‐old female patient who exhibited agranulocytosis after the initiation of isotretinoin administration. Agranulocytosis is a rare but life‐threatening side effect of isotretinoin use, and clinicians must be aware of agranulocytosis.

Protective effect of vitamin D against hyperoxia-induced lung injury in newborn rats.

Preterm infants have risks of developing vitamin D deficiency. Thus we aimed to investigate the effect of vitamin D on hyperoxia‐induced lung injury in newborn rats.

Cervicothoracolumbar spinal epidural abscess and cerebral salt wasting

BACKGROUND CONTEXT Spinal epidural abscess (SEA) is a rare infectious disease. However, if left unrecognized and untreated, the clinical outcome of SEA can be devastating. PURPOSE To report a rare clinical presentation of a cervicothoracolumbar SEA with cerebral salt wasting (CSW). STUDY DESIGN Case report. METHODS Clinical history, physical and laboratory findings, and magnetic resonance imaging studies of a patient with cervicothoracolumbar SEA and CSW. RESULTS We report the case of a 15-year-old boy with cervicothoracolumbar SEA complicated with CSW and treated with conservative methods. CONCLUSIONS In conclusion, CSW can be seen at the follow-up period of the SEA and the clinicians must be aware of this entity.

Comparing the Efficacy of 7%, 3% and 0.9% Saline in Moderate to Severe Bronchiolitis in Infants

BACKGROUND There is no standard treatment option in acute bronchiolitis. 3-7% hypertonic saline (HS) seems to be the effective treatment choice for reducing the hospitalization day. AIMS To compare the effect of nebulized 7% HS/salbutamol and 3% HS/salbutamol to 0.9% saline/salbutamol. The primary outcome measure was the effect of study drugs on the length of hospital stay (LOS). Secondary outcome measures were safety and efficacy in reducing the clinical severity score (CSS) at the 24 hours of the study. STUDY DESIGN Prospective, double-blinded randomized clinical study. METHODS The study consists of 104 infants. Groups were constituted according to the treatment they received: These are, group A - 0.9% saline/salbutamol, group B -3% HS/salbutamol and group C-7% HS/salbutamol. Heart beat, Bronchiolitis CSS and oxygen saturation of the patients were determined before and after nebulization. The patients were monitored for adverse reactions. RESULTS Length of hospital stay in group A, B and C were as follows; 72.0 (20-288) hours in group A, 64.0 (12-168) hours in group B and 60.0 (12-264) hours in group C. No significant differences was observed among three groups (p>0.05). CONCLUSION 7% HS and 3% HS does not have any effect to decrease LOS for infants with bronchiolitis.

Clinical and Microscopic Hair Features of Griscelli Syndrome Associated with Asymmetric Crying Facies in an Infant

Griscelli syndrome (GS) is a rare autosomal recessive disorder characterized by immunodeficiency and partial albinism. Asymmetric crying facies (ACF) is caused by congenital hypoplasia or agenesis of the depressor anguli oris muscle. Asymmetric crying facies may be isolated or it may be associated with various anomalies. We report a 3-month-old Turkish boy who had clinical and microscopic hair features of GS associated with ACF. To the best of our knowledge, this is the 1st case of this association to be reported in the literature.