Sjogren-Larsson syndrome (SLS) is a rare autosomal recessive neurocutaneous disorder with worldwide incidence of 0.4 per 100,000 people. It is characterized by the triad of congenital ichthyosis, spastic diplegia or quadriplegia, and mental retardation. Herein we report a 2-year-old male child with SLS, asthma, and recurrent pneumonia. SLS was confirmed by a molecular genetics study that revealed a deletion mutation in the ALDH3A2 gene. An ALDH3A2 gene mutation results in dysfunction of the microsomal enzyme fatty aldehyde dehydrogenase and impaired metabolism and accumulation of leukotriene B4, which is a key molecule and a pro-inflammatory mediator in developing allergic diseases, especially asthma. An increased level of leukotriene B4 has been reported in SLS patients. As far as we are aware, this is the first report of SLS associated with asthma and recurrent pneumonia. In conclusion, pediatricians should be aware of and evaluate patients with SLS for possible associated asthma and allergic disorders.
Aldehyde Dehydrogenase ; Asthma* ; Cerebral Palsy ; Child ; Child, Preschool ; Humans ; Ichthyosis ; Incidence ; Intellectual Disability ; Leukotriene B4 ; Male ; Metabolism ; Molecular Biology ; Neurocutaneous Syndromes ; Pneumonia* ; Quadriplegia ; Sequence Deletion ; Sjogren-Larsson Syndrome*

Visceral leishmaniasis or kala-azar is an endemic parasitic disease in some parts of the world which is characterized by fever, splenomegaly, and pancytopenia in most of the cases. Herein we report an 11 month-old male infant with diagnosis of kala-azar who presented with pallor, hepatosplenomegaly, failure to gain weight, and no history of fever. Surprisingly, fever started after beginning of meglumine antimoniate treatment in this patient. As far as we are aware of, this is a rare presentation of visceral leishmaniasis. Therefore, clinicians especially in endemic areas are highly recommended to include kala-azar among differential diagnosis of unexplained anemia without fever to prevent misdiagnosis of this potentially fatal, but treatable condition.
Amphotericin B/therapeutic use ; Anemia/*diagnosis/parasitology ; Antiprotozoal Agents/*therapeutic use ; Deoxycholic Acid/therapeutic use ; Diagnosis, Differential ; Drug Combinations ; Endemic Diseases ; *Fever ; Humans ; Infant ; Iran ; Leishmania infantum/pathogenicity ; Leishmaniasis, Visceral/*diagnosis/*drug therapy/parasitology ; Male ; Meglumine/therapeutic use ; Organometallic Compounds/therapeutic use ; Splenomegaly/parasitology

BACKGROUND: The aim of this study was to evaluate the antibiotic susceptibility of Group A streptococcus (GAS) to antibiotics usually used in Iran for treatment of GAS pharyngitis in children. MATERIALS AND METHODS: From 2011 to 2013, children 3-15 years of age with acute tonsillopharyngitis who attended Mofid Children's Hospital clinics and emergency ward and did not meet the exclusion criteria were enrolled in a prospective study in a sequential manner. The isolates strains from throat culture were identified as GAS by colony morphology, gram staining, beta hemolysis on blood agar, sensitivity to bacitracin, a positive pyrrolidonyl aminopeptidase (PYR) test result, and the presence of Lancefield A antigen determined by agglutination test. Antimicrobial susceptibility was identified by both disk diffusion and broth dilution methods. RESULTS: From 200 children enrolled in this study, 59 (30%) cases were culture positive for GAS. All isolates were sensitive to penicillin G. The prevalence of erythromycin, azithromycin, and clarithromycin resistance by broth dilution method was 33.9%, 57.6%, and 33.9%, respectively. Surprisingly, 8.4% of GAS strains were resistant to rifampin. In this study, 13.5% and 32.2% of the strains were resistant to clindamycin and ofloxacin, respectively. CONCLUSION: The high rate of resistance of GAS to some antibiotics in this study should warn physicians, especially in Iran, to use antibiotics restrictedly and logically to prevent the rising of resistance rates in future. It also seems that continuous local surveillance is necessary to achieve the best therapeutic option for GAS treatment.
Agar ; Agglutination Tests ; Anti-Bacterial Agents ; Azithromycin ; Bacitracin ; Child* ; Clarithromycin ; Clindamycin ; Diffusion ; Drug Resistance, Microbial ; Emergency Service, Hospital ; Erythromycin ; Hemolysis ; Humans ; Iran* ; Logic ; Ofloxacin ; Penicillin G ; Pharyngitis* ; Pharynx ; Prevalence ; Prospective Studies ; Rifampin ; Streptococcus pyogenes ; Streptococcus* ; Tonsillitis

BACKGROUND: Acute Epstein-Barr virus (EBV) infection could lead to atherogenic lipid profile changes in adults; while there is no evidence about the children with Infectious mononucleosis (IM). The aim of this study was to evaluate the lipid profile of the children in acute phase of mononucleosis and two months after the recovery. MATERIALS AND METHODS: From 2010 through 2012, 36 children with IM aged 1-10 years were enrolled in a prospective cross-sectional study. Fasting serum total cholesterol, high density lipoprotein (HDL), low density lipoprotein (LDL), and triglyceride level were measured during acute phase of the disease and after 2 months of the recovery. RESULTS: From 36 patients enrolled, 25 (69.4%) cases were male and the mean age of the patients was 4.1 ± 2.0 years. The mean of the total cholesterol level in the acute phase and 2 months after the recovery were149.5 ± 35.3 mg/dL and 145.7±30.6, respectively (P = 0.38). However, the serum level of HDL cholesterol in patients after 2 months of recovery was significantly increased (37.9 ± 9.3 mg/dL vs. 28.5 ± 10.6 mg/dL, P <0.001). The mean value of serum LDL cholesterol was significantly reduced, two months after recovery (81.4 ± 19.5 mg/dL, vs. 92.6 ± 28.8 mg/dL, P = 0.009). Furthermore, the serum triglyceride level was significantly reduced after the recovery (108.7 ± 36.9 mg/dL) compared with the acute phase (163.8 ± 114.3 mg/dL) (P = 0.004). CONCLUSION: EBV infection in children could change lipid profile which is partially restored 2 months after the recovery.
Adult ; Child ; Cholesterol ; Cholesterol, HDL ; Cholesterol, LDL ; Cross-Sectional Studies ; Epstein-Barr Virus Infections ; Fasting ; Herpesvirus 4, Human ; Humans ; Infectious Mononucleosis ; Lipoproteins ; Male ; Pediatrics ; Prospective Studies ; Triglycerides

Acinetobacter baumannii is one of the most important etiologies of nosocomial infections in recent years mainly because of increasing in frequency of multidrug and pan-resistant pathogens. Meningitis caused by this organism is a dilemma; because polymyxins are the only effective antibiotics against pan-resistant serotypes, but have poor penetration via blood brain barrier; however, it has still remained uncertain whether the intravenous therapy with these agents is an effective treatment with the sufficient concentration of the drug in the cerebrospinal fluid. Herein, we report a neonate who suffered from pan-resistant A. baumannii nosocomial meningitis successfully treated with intravenous colistin combined with meropenem and rifampin. It seems that intravenous colistin at least in combination with rifampin and meropenem might be considered as an option to try in patients in whom daily intrathecal injection or insertion of intraventricular device is not possible.

Purpose: The aim of this study was to compare the hepatitis B surface antibody (HBs Ab) titer 1 month after the 4th dose of hepatitis B vaccine administration on the large and appropriate for gestational age infants. Materials and Methods: This cross-sectional study was conducted on 7-month-old cases (n=132) divided into two groups of 2–4 kg (group 1: appropriate for gestational age, 63 cases) and >4 kg (group 2: large for gestational age, 69 cases), whom were vaccinated with a fourdose schedule of hepatitis B vaccine in 2016, Tehran, Iran. Results: Mean birth weight of the groups was 2.98±0.528 and 4.19±0.190 kg, respectively.Hepatitis B surface antigen and hepatitis B core antibody were negative in all cases. HBs Ab level in group 1 and 2 was 13,701.00±11,744.439 and 8,997.15±2,827.191, respectively (95% confidence interval of difference, -7,607.44 to -1,800.25). There was a significant difference between the two groups in antibody titration and antibody logarithm level (p=0.002, p=0.0001). Conclusion Birth weight may affect the response to the hepatitis B virus vaccine administration.

Acinetobacter baumannii is one of the most important etiologies of nosocomial infections in recent years mainly because of increasing in frequency of multidrug and pan-resistant pathogens. Meningitis caused by this organism is a dilemma; because polymyxins are the only effective antibiotics against pan-resistant serotypes, but have poor penetration via blood brain barrier; however, it has still remained uncertain whether the intravenous therapy with these agents is an effective treatment with the sufficient concentration of the drug in the cerebrospinal fluid. Herein, we report a neonate who suffered from pan-resistant A. baumannii nosocomial meningitis successfully treated with intravenous colistin combined with meropenem and rifampin. It seems that intravenous colistin at least in combination with rifampin and meropenem might be considered as an option to try in patients in whom daily intrathecal injection or insertion of intraventricular device is not possible.