Epidemic management in a measles outbreak in 2023,the return of vaccine-preventable diseases:A single center,retrospective observational study

Objective:To investigate the importance of immunization in preventing measles infection and to determine the most useful laboratory tests for confirmation of measles.Methods:This study included pediatric cases evaluated with a presumed diagnosis of measles between December 2022 and June 2023,at Marmara University Pendik Training and Research Hospital.The effects of vaccination status and underlying disease on the clinical course,treatments,and complications were evaluated.Results:In total,117 patients were enrolled in the study with a median age of 80 months(IQR:32.5-125.0).Twelve patients with contact history were asymptomatic and had an underlying disorder,and intravenous immunoglobulin was given to them for post-exposure prophylaxis.Fifty-one patients had confirmed measles diagnosis.Ribavirin treatment was given to three patients(a newborn,a girl with rhabdomyosarcoma,and a healthy boy)with respiratory distress.Seventy-eight percent of confirmed measles cases were unvaccinated,and all hospitalized cases were unvaccinated or under-vaccinated.Four full-vaccinated children had confirmed measles infection.Measles PCR from nasopharyngeal swabs was negative in all of them,and their diagnosis was established with anti-measles IgM positivity.Conclusions:The measles vaccine is the most effective way to protect from measles and measles-related complications.Although measles can also occur in fully vaccinated patients,the disease is milder than in unvaccinated patients.Using ELISA and RT-PCR tests together may be beneficial in patients with high clinical suspicion for early diagnosis.

Magnetic resonance imaging evaluation of head and neck involvement in IgG4-related disease

Objective:To evaluate the radiological features of IgG4-related disease(IgG4-RD)in the head and neck region.Methods:In this radiology-based study,radiological features,clinical,laboratory,pathological findings,and prognosis of nine patients with head and neck involvement diagnosed with IgG4-RD were investigated retrospectively.Results:The median age of the patients was 38 years(range:2.5-79 years),and there were six males and three females.The most common symptoms and clinical findings of the patients were eyelid and lacrimal gland swelling,painless exophthalmos,and ophthalmoplegia.The most common site of involvement on MRI was the orbit.Orbital involvement was followed by branches of the trigeminal nerve,sinonasal cavity,cervical lymph nodes,and dural involvement.The most common and remarkable imaging features were T2 hypointensity and diffuse homogeneous contrast enhancement.Conclusions:Head and neck involvement of the IgG4-RD,has specific imaging features that can help with diagnosis.Thus,early diagnosis and better outcomes can be achieved with increasing awareness of these features of this relatively new pathology.

Evaluation of Interferon-Gamma Release Assay Testing and Tuberculin Skin Test for Early Diagnosis of Tuberculosis in Children and Adolescents

Background: This study aimed to evaluate the diagnostic value of interferon-γ release assay (IGRA), a sensitive microbiological diagnostic method, in children and adolescents with suspected tuberculosis in a country with a high burden of tuberculosis. Method: This study included 581 children and adolescents aged 4 - 19 years who were suspected of having tuberculosis, were latently infected with Mycobacterium tuberculosis, and had received at least one dose of BCG vaccine between April 17, 2019, and February 24, 2021. The study evaluated the TST results of 106 patients who had a positive Quantiferon test and were suspected of having tuberculosis. Results: The study included 581 patients aged between 4 and 19 years. Of these, 106 patients tested positive for the Quantiferon test, while 19 were indeterminate and 456 were negative. The Quantiferon test positivity rate was 18.24%. Among the 106 QFT-Plus-positive cases, 23 patients also tested positive for TST. The difference in distribution was found to be statistically significant. Conclusion: The QFT-Plus test is considered an alternative to TST and other microbiological diagnostic methods for early tuberculosis diagnosis, particularly in children and adolescents.

Ethanol Lock Therapy as additional treatment of catheter-related bloodstream infections in pediatric patients receiving home parenteral nutrition

Background: Catheter related bloodstream infections (CRIs) are a major cause of morbidity, mortality and prolonged hospitalization in pediatric patients with home parenteral nutrition (HPN). In recent years, new prophylactic and treatment options have been evaluated to reduce the incidence of CRIs and infection related catheter removals. In children, two retrospective studies have shown the efficacy of ethanol lock treatment (ELT) in reducing the incidence of CRIs in pediatric oncology patients. However, prospective data are missing. Our aim of the present study was to evaluate prospectively the effect of ELT on the cure and recurrence rate of CRIs, duration of hospitalization and antibiotic use in HPN pediatric patients. Methods: HPN children older than three months with a clinical suspicion of a CRI were included and randomised to ELT or standard flushing treatment (heparin or taurolidine). The primary outcomes were persistence and recurrence of the bacteraemia and Central Venous Access Device (CVAD) removal. Secondary outcomes included duration of antibiotic use, duration of hospitalization and side effects. Results: Data from eight HPN children experiencing 14 suspected CRI were analysed. Seven patients had proven CRIs by positive blood cultures. Four were treated with ELT and three with their standard flushing treatment. One CRI persisted in the control group. In one single patient, bacteraemia recurred three times within 24 weeks. This patient had been enrolled in both study arms. A CVAD removal was needed in one patient because of occlusion. Conclusions: This small study showed no additional efficacy of the use of ELT on cure rate or recurrences of CRIs compared to standard therapy in HPN treated children. There were also no differences in duration of hospitalization, the use of antibiotics or CVAD removal between both groups.

Autoimmune complications and clinical outcomes of herpes simplex encephalitis in children: A case series

Objective:To report the neurologic prognosis and autoimmune complications of 16 cases of childhood herpes simplex virus encephalitis.Methods:The study was conducted atŞanlıurfa Training and Research Hospital,Turkey from June 2017 to August 2019.The study included 16 pediatric patients aged between 6 months and 17 years(median age 77.7 months)who were diagnosed with herpes simplex virus type 1 encephalitis by pediatric infectious disease and pediatric neurology clinics.Patients were followed using patient records,and interviews at the pediatric neurology clinic or via the telephone.Clinical and demographic data,received therapies,neurologic prognosis and complications were evaluated.Results:Patients with and without autoimmune encephalitis were compared in terms of age,sex,symptom duration before treatment,initial cerebrospinal fluid protein,glucose,red blood count and white blood count but no significant difference was found.Autoimmune complications were seen in four patients.N-methyl-D-aspartate encephalitis was observed in three patients and choreoathetosis was seen in one patient.The average follow-up period was 48.3 months.Twenty-five percent of the patients were receiving multiple antiepileptic drug(AED)treatment,43.8%were receiving single AED treatment and 31.3%were not receiving AED treatment at the end of the follow-up.Motor disability was observed in 12.5%and drug-resistant epilepsy was observed in 6.3%who had autoimmune complications.Conclusions:Seizures and movement disorders were controlled with immunotherapy and autoantibodies should be studied routinely.Treatment should be started early upon recognition of autoimmune complications through follow-up by measuring autoantibody levels and clinical examination results.Effective prevention and curative treatment modalities are needed to avoid herpes simplex virus encephalitis complications.

Absorption Characteristics of Novel Compound Calcium Carbonate Granules:Effects of Gastric Acid Deficiency and Exogenous Weak Acids

Calcium carbonates are commonly administered as supplements for conditions of calcium deficiency.We report here pharmacokinetic characteristics of a novel formulation,calcium carbonate compound granules(CCCGs).forming complexes of calcium carbonate and calcium citrate in water.CCCGs were compared to a kind of commonly?used calcium carbonate preparation(CC)in the market in 5-week-old mice that had been treated with omeprazole,to suppress gastric acid secretion,and in untreated control mice.The results showed that:(1)CCCGs had better water solubility than CC in vitro;(2)In control mice,calcium absorption rates after CCCGs administration were comparable to those after CC administration;(3)Inhibition of gastric acid secretion did not affect calcium absorption after CCCGs,but moderately decreased it after CC;(4)The presence of phytic acid or tannin did not affect calcium absorption rates after CCCGs but did for CC;and(5)In nonnal mice,CCCGs did not inhibit gastric emptying and intestinal propulsion,and did not alter the gastrointestinal honnones.The results suggest that CCCGs may be therapeutically advantageous over more commonly used calcium supplement formulations,particularly for adolescents,because of their stable calcium absorption characteristics and their relatively favorable adverse effect profile.

Horizontal transmission of hepatitis B virus in children with chronic hepatitis B

AIM: To determine the possible routes of intrafamilial transmission pattern in pediatric cases of chronic hepatitis B virus (HBV) infection.METHODS: In this descriptive retrospective study, 302 children with chronic HBV infection from 251 families and their parents attending the Social Security Children's Hospital and Doctor Sami Ulus Children's Hopsital in Ankara between December 1998 and May 2000, were enrolled in. Screenings and diagnosis of chronic HBV infections were established according to the Consensus 2000.RESULTS: In the studied 302 children with chronic HBV infection, mothers of 38% and fathers of 23% were HBsAg positive. The HBsAg positivity in at least two siblings of the same family was 61% when both parents were HBsAg positive.CONCLUSION: It is well known that horizontal transmission is quite common in countries where Hepatitis B Virus is moderately endemic. To our best knowledge, this is the largest series observed regarding the horizontal transmission in pediatric chronic HBV infection in Turkey. It is necessary to expand the preventive programs to target not only the newborn period but also all stages of childhood.

Selecting suitable solid organ transplant donors: Reducing the risk of donor-transmitted infections

Selection of the appropriate donor is essential to a successful allograft recipient outcome for solid organ transplantation. Multiple infectious diseases have been transmitted from the donor to the recipient via transplantation. Donor-transmitted infections cause increased morbidity and mortality to the recipient. In recent years, a series of high-profile transmissions of infections have occurred in organ recipients prompt-ing increased attention on the process of improving the selection of an appropriate donor that balances the shortage of needed allografts with an approach that mitigates the risk of donor-transmitted infection to the recipient. Important advances focused on improving donor screening diagnostics, using previously excluded high-risk donors, and individualizing the selection of allografts to recipients based on their prior infection history are serving to increase the donor pool and improve outcomes after transplant. This article serves to review the relevant literature surrounding this topic and to provide a suggested approach to the selection of an appropriate solid organ transplant donor.

A Comparative Trial: The Safety and Clinical Efficacy of PEG 3350 and Liquid Paraffin in Management of Chronic Functional Constipation in Children

Background: Constipation is one of the most common problems in the pediatric age group. In the vast majority, chronic constipation is idiopathic or functional. In addition to education and behavioral changes, disimpaction and maintenance treatment with medications are the main pillars of successful outcome. The medications are variably effective and have potential side effects. Objectives: The current clinical trial was designed to evaluate clinical efficacy of polyethylene glycol (PEG) 3350 and liquid paraffin in chronic functional constipation. Besides, subjects were followed carefully for the side effects. Material and Methods: This study was involved 110 children suffering from chronic functional constipation. They were placed into two different groups, randomly. Each group was treated with PEG 3350 or liquid paraffin. The clinical efficacy and side effects were monitored. Results: After disimpaction with bisacodyl suppositories or combined with Mineral oil enemas in hard fecal impaction, maintenance therapy with PEG 3350 or liquid paraffin was started. The treatment duration and efficacies were not different between the two groups. Furthermore, serious adverse drug reaction was not observed. The family history of constipation and presence of incontinence were two worse prognostic factors. Conclusion: No difference was observed between PEG 3350 and liquid paraffin in chronic functional constipation in children in terms of their efficacies. They are safe and effective medications in this age group.

Human bocavirus infection in children hospitalized with lower respiratory tract infections:Does viral load affect disease course?

Objective:To examine the effects of human bocavirus type 1(HBoV1)on the course of lower respiratory tract infections in cases of monoinfection and coinfection,and the effects of HBoV1 viral load on the disease in children under six years old hospitalized with a diagnosis of HBoV1-associated lower respiratory tract infections.Methods:Children under six years of age,who were hospitalized with the diagnosis of lower respiratory tract infection due to HBoV1 between 1 January 2021 and 1 January 2022 were included in the study.Laboratory confirmation of the respiratory pathogens was performed using polymerase chain reaction(PCR).Results:Fifty-four(16.4%)children with HBoV1 among 329 children whose PCR was positive with bacterial/viral agent in nasopharyngeal swab samples were included in the study.There were 28(51.9%)males and 26(48.1%)females with a median age 23.4 months[interquartile range(IQR):13.2,30.0 months](min-max:1 month-68 months).HBoV1 was detected as a monoinfecton in 26(48.1%)children,and as a coinfection with other respiratory agents in 28 children(51.9%).In multiple regression analysis,coinfection(P=0.032)was associated with the length of hospitalization(P<0.001;R^(2)=0.166).There was a negative correlation(r=−0.281,P=0.040)between cough and cycle threshold.Fever was found to be positively correlated with C-reactive protein(r=0.568,P<0.001)and procalcitonin(r=0.472;P=0.001).Conclusions:Although we found a higher HBoV1 viral load in children with more cough symptoms in our study,it had no effect on the severity of the disease,such as length of hospital stay and need for intensive care.Coinfection was found to affect the length of hospitalization.

Etiology of granulomatous inflammation:A retrospective study of 174 children in a tertiary care center

Objective:To investigate granulomatous inflammation etiology based on clinical history and ancillary tests.Methods:Children aged<18 years with biopsy proven granulomatous lesions in any tissue specimens between January 2014 and January 2022 were included in the study.The diagnosis was based on the results of immunohistochemical staining,molecular tests,culture,serology,radiological and other auxiliary laboratory tests.Diagnoses were categorized into infectious and noninfectious causes.Results:In total,174 patients with granulomatosis inflammation confirmed by histopathology were analyzed.Approximately 59.2%patients were males,and the median age was 4.48(IQR 2.36-6.39)years(range:16 months-18 years).The tissues/organs that were most commonly biopsied were lymph node,bone,skin,and lung(51.1%,17.8%,9.2%,and 5.7%,respectively).Infectious and non-infectious causes were identified in 73.0%and 12.6%patients,respectively,in terms of granulomatosis inflammation etiology;however,no cause was identified in 14.4%patients.The most common infectious cause was tuberculosis(in 51.7%patients),followed by toxoplasmosis,aspergillosis,mucormycosis,leishmaniasis,and cat-scratch disease(in 8.6%,5.7%,1.7%,1.7%,and 1.1%patients,respectively).The common non-infectious cause was chronic granulomatous disease.Histopathological evaluation revealed granulomatosis inflammation in 33.3%patients,necrotizing granulomatosis inflammation in 30.5%patients,and caseating granulomatosis inflammation in 12.1%patients.When the pathology results of patients with and without tuberculosis were compared,the incidence of caseating granulomatosis inflammation(P=0.003)and necrotizing granulomatosis inflammation(P=0.005)was higher in patients with tuberculosis.Conclusions:Chronic granulomatous disease is the most common non-infectious cause in children.Moreover,primary or secondary immune deficiencies may cause granulomatosis inflammation,especially in pediatric patients.

Hematological profile of COVID-19 infected children before and after the spread of the Omicron variant in Istanbul

Objective:To examine the effect of the COVID-19 virus,especially the Omicron variant,on hematological parameters of hospitalized pediatric patients during the COVID-19 pandemic.Methods:Medical records of pediatric COVID-19 patients hospitalized at Kartal Dr.Lütfi K?rdar City Hospital in Istanbul,Turkey,between March 2020 and May 2022 were retrospectively reviewed to analyze data regarding demographics,SARS-Co V-2 infection polymerase chain reaction(PCR)test results,reversetranscriptase(RT)-PCR for other respiratory agents,duration of hospital stay,and hematological and biochemical laboratory findings.Results:Out of 467 children with a confirmed diagnosis of SARSCo V-2 infection,94(20.1%)had Omicron infection and 373(79.9%)were infected with other variants;the Omicron group had younger patients than the remaining samples(P<0.001).The most frequent clinical symptoms in all children were cough(53.5%)and fever(32.3%),followed by vomiting(20.8%).Lung involvement in the Omicron group(10.6%)was significantly lower than in the remaining samples(29.8%)(P<0.001).Hemoglobin and lymphocyte levels were lower in the Omicron-infected group(both P<0.001),while prothrombin time,activated partial thromboplastin time,international normalized ratio,and D-dimer levels were significantly higher in this group(P<0.001,P<0.001,P<0.001,and P=0.023,respectively).In terms of lung involvement,those with lung involvement were significantly older(P<0.001).Conclusions:Although lung involvement was less common with Omicron infection,this group had greater hematological system involvement,such as anemia,lymphopenia,D-dimer elevation,and coagulation disorders.

Chryseobacterium/Elizabethkingia species infections in children

Objective:To investigate the clinical and epidemiological features and outcome of Chryseobacterium and Elizabethkingia spp.infections in children,together with antimicrobial susceptibilities.Methods:This retrospective study was conducted at a tertiary pediatric hospital in Turkey.All patients infected with Chryseobacterium/Elizabethkingia spp.among those presenting to Ankara City Hospital between March 2014 and March 2022 were included.Results:A total of 49 cases were included and 29 cases were identified as Elizabethkingia.The median age was 14(0.2-185.0)months.The majority(89.8%)of these patients had an underlying disease,including malignancy(42.9%).Bacteremia(46.9%)and central line-associated bloodstream infection(28.6%)were the most common infections.The thirty-day all-cause mortality rate was 12.2%.The most commonly used antibiotics were ciprofloxacin and trimethoprim-sulfamethoxazole(TMP-SMX).Forty-five(91.8%)isolates were susceptible to ciprofloxacin,44(91.6%)to TMP-SMX,and 21(87.5%)to levofloxacin.Conclusions:Chryseobacterium and Elizabethkingia spp.are emergent,nosocomial pathogens and the majority of cases were older than the neonatal period.They were mainly seen in patients with long hospital stays,indwelling devices,and those who have received antibiotics within the last month,especially carbapenems.In addition,they were associated with bloodstream infection and malignancy.The most commonly useful antibiotics according to the resistance patterns were ciprofloxacin and TMP-SMX.

Brucellosis,a diagnostic dilemma,presenting atypically in a child with terminal ileitis:A case report

BACKGROUND Brucellosis is endemic in India with seropositivity rates as high as 10%in children in the eastern states,yet the disease is not on the radar when a differential diagnosis of pyrexia of unknown origin(PUO)is being considered,especially in children in urban set-up.This may be because of the non-specific multitude of systemic symptoms seen in this disease and the lack of awareness among clinicians.CASE SUMMARY We present a case of a 13-year-old boy,who came with a history of undulating fever for the past three and a half months,loss of appetite,and abdominal pain.The child had visited several pediatricians and was even admitted to a tertiary care hospital for PUO evaluation,but to no avail.He presented to us after three and half months of suffering and weight loss of more than 10%of body weight.His ultrasonography revealed thickening of the terminal ileum.His blood culture grew Brucella melitensis.A diagnosis of Brucellosis with terminal ileitis was made.Brucella serology by enzyme-linked immunoassay(ELISA)was positive for both IgG and IgM.He was treated with doxycycline and Rifampicin along with syrup multivitamin and zinc,for 6 wk.There was remarkable improvement with gain in 4 kg body weight within 2 mo of completing treatment.History revealed consumption of unpasteurized milk and contact with cattle.CONCLUSION Clinical suspicion,detailed history,appropriate laboratory investigations are the three pillars for diagnosing Brucellosis in patients presenting with vague symptoms.

昏迷诱导治疗狂犬病的生存情况

近日我们得知石家庄某大型医院对1例53岁男性狂犬病患者进行了昏迷诱导治疗。该患者为16天前酒后被小狗咬伤鼻子,之后狂犬病发作,就诊该院,进行了昏迷诱导治疗,但于昏迷的第6天死亡。日后据主治医师孙主任介绍,该院对类似病例所进行的治疗均未获成功。我们翻译了2005年美国Willoughby博士的病例报告,并带着问题通过电子邮件向其进行了咨询。现将Willoughby等报道的病例摘要及来信摘译如下,以飨读者。

Burkholderia cepacia outbreak in immunocompetent children in a tertiary hospital in Turkey:A case series

Objective:To report an outbreak of Burkholderia(B.)cepacia related to contaminated surface cleaner in the pediatric ward of a tertiary hospital in Turkey.Methods:This study retrospectively reported the outbreak occurred between January 16,2018 and January 23,2018.Twelve immunocompetent patients who developed a bloodstream infection a few days after the hospitalization and who were positive for B.cepacia were included.Environmental samples were collected from various areas in the hospital to find the source of the outbreak.Results:All patients had clinical and biochemical evidence of sepsis.None of the patients had an underlying disease or had a central venous catheter as a risk factor.B.cepacia was isolated from the samples taken from the surface cleaners.The antibiotic susceptibilities of B.cepacia isolates were identical in the surface cleaners with the isolates from the patients’blood cultures.The outbreak was controlled after removing the surface cleaners from use.None of the infected patients died during the outbreak.Conclusions:Nosocomial B.cepacia outbreak may occur in immunocompetent children as well.Rapid identification of the outbreak,defining the source and taking appropriate measures to control the outbreak are the key points in the management.

Comorbidities,clinical characteristics and outcomes of COVID‑19 in pediatric patients in a tertiary medical center in the Netherlands

Having pre-existing comorbidities is described as a risk factor for more severe disease in adult corona virus disease 2019(COVID-19)and in infections with SARS-CoV-1 and MERS-CoV[1].In adult SARS-CoV-2 infections,patients with pre-existing underlying comorbidities,such as chronic obstructive pulmonary disease,cardiovascular disease,diabetes and obesity,are more likely to have severe disease compared to healthy adults[2].An inconsistency is seen in current fndings on the association with comorbidities and pediatric COVID-19 severity.An important limitation in currently available studies is limited data:severe disease is rare in children compared to adults,and most studies describe COVID-19 severity merely by reporting intensive care unit(ICU)admission or mortality rates instead of detailed data on clinical presentation and outcomes.

昏迷诱导疗法治疗狂犬病后存活1例

We report the survival of a 15- year-old girl in whom clinical rabies developed one month after she was bitten by a bat. Treatment included induction of coma while a native immune response matured; rabies vaccine was not administered. The patient was treated with ketamine, midazolam, ribavirin, and amantadine. Probable drug-related toxic effects included hemolysis, pancreatitis, acidosis, and hepatotoxicity. Lumbar puncture after eight days showed an increased level of rabies antibody, and sedation was tapered. Paresis and sensory denervation then resolved. The patient was removed from isolation after 31 days and discharged to her home after 76 days. At nearly five months after her initial hospitalization, she was alert and communicative, but with choreoathetosis, dysarthria, and an unsteady gait.

Fever without a source in children:international comparison of guidelines

Background Fever without a source(FWS)in children poses a diagnostic challenge.To distinguish a self-limiting infection from a serious infection,multiple guidelines have been developed to aid physicians in the management of FWS.Currently,there is no comparison of existing FWS guidelines.Methods This comparative review describes consistencies and differences in guideline definitions and diagnostic and therapeutic recommendations.A literature search was performed to include secondary care FWS guidelines of high-income countries,composed by national or regional pediatric or emergency care associations,available in English or Dutch.Results Ten guidelines of five high-income countries were included,with varying age ranges of children with FWS.In children younger than one month with FWS,the majority of the guidelines recommended laboratory testing,blood and urine culturing and antibiotic treatment irrespective of the clinical condition of the patient.Recommendations for blood culture and antibiotic treatment varied for children aged 1–3 months.In children aged above three months,urine culture recommendations were inconsistent,while all guidelines consistently recommended cerebral spinal fluid testing and antibiotic treatment exclusively for children with a high risk of serious infection.Conclusions We found these guidelines broadly consistent,especially for children with FWS younger than one month.Guideline variation was seen most in the targeted age ranges and in recommendations for children aged 1–3 months and above three months of age.The findings of the current study can assist in harmonizing guideline development and future research for the management of children with FWS.

Ataxia telangiectasia in Turkey:multisystem involvement of 91 patients

Background:Ataxia telangiectasia (AT) is a genetically based multisystemic disorder.We aimed to make a comprehensive evaluation of multisystem involvement in AT by describing clinical features and outcome of 91 patients.Methods:Medical records of the patients who were diagnosed and followed by a multidisciplinary approach during a 27-year period (1988-2015) were reviewed retrospectively.Results:Forty six female and 45 male patients with a mean follow-up period of 39.13±4.28 months were evaluated.The mean age at the time of symptom onset and diagnosis were 15.4±1.09 months and 73.6144.11 months,respectively.Neurological abnormalities were progressive truncal ataxia,nystagmus,dysarthria,oculomotor apraxia and choreoathetosis.Thirty one patients (34.1%) became dependent on wheelchair at a mean age of 12.1±2.8 years.Eleven patients (12.1%) became bedridden by a mean age of 14.7±1.8 years.Cranial magnetic resonance imaging revealed pathological findings in 47/66 patients.Abnormal immunological parameters were determined in 51/91 patients:immunoglobulin (Ig)A deficiency (n=38),lymphopenia (n=30),IgG (n=15) and IgG2 (n=11) deficiency.Occurrence of recurrent sinopulmonary infections (n=45) and bronchiectasis (n=22) were found to be more common in patients with impaired immunological parameters (P=0.029and P=0.023,respectively).Malignancy developed in 5 patients,being mostly lymphoreticular in origin and resulted in death of 4 patients.Conclusions:AT is a long lasting disease with muitisystem involvement necessitating multidisciplinary follow up,as described in our cohort.Early diagnosis of malignancy and supportive treatments regarding pulmonary and neurological health may prolong survival and increase the quality of life.