Primary Immunodeficiency Disorders: Survey of Pediatricians in Kuwait

Al‐Herz, Waleed; Zainal, Mohammad; Salama, Mohammad; Al-Ateeqi, Wafa; Husain, Khalid; Abdul-Rasoul, Majeda; Al-Mutairi, Bander; Badawi, Mona; Aker, Najwa; Kumar, Subodh; Al-Khayat, Haitham

doi:10.1007/s10875-008-9191-6

Cited by 22 publications

(20 citation statements)

References 19 publications

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“…The mean overall score was 60% (95% confidence interval = 58% to 61%), the clinical presentation score was 63%, the syndromes associated with immunodeficiency score was 58%, and the laboratory investigation score was 51%. It was uncertain, however, whether these results are applicable to other countries and repeated studies are recommended [13]. Another study from Turkey investigated only the awareness of pediatricians to important PID indicators.…”

Section: Discussionmentioning

confidence: 99%

“…Six questions looked at personal information, 24 at PID signs and symptoms, 9 at associated syndromes, 11 at screening tests, 6 at laboratory interpretations and 7 at management. The implemented questions were a modification of those developed by Al-Herz et al [13]. …”

Section: Methodsmentioning

confidence: 99%

“…Also, we thank sincerely the pediatricians at different hospitals who took the responsibility to follow up on the completion of the survey: Dr Anwar Sallam, Al-Mafraq Hospital, Dr Amin Abu Bakr, Al-Rahba Hospital, Drs Aisha Al-Shattaf and Sabeeha Al-Tunaiji, Tawam Hospital, Dr Khalid Abu Ahmad, Madinat Zayed Hospital, Dr Wahib Marzouq, Ruwais Hospital, Dr Saif Al-Kaabi, Zayed Military Hospital, Dr Alla Shaheen, Al-Baraha hospital, Dr Mohamed Anwar Tufail, Al-Qasimi Hospital, Dr Raef El-Gamal, Sheikh Khalifa Hospital, Dr Fawzeya Abu Al-Assaad, Umm Al-Quwain Hospital, Dr Abdelmalik A Razik, Fujairah Hospital, Dr Saad Alaani, Khorfakkan hospital, and all the pediatricians who gave time to participate in this study. Last, but not least, Dr Walid Al-Herz for sharing with us the main structure of the survey used in his study [13]. …”

Section: Acknowledgmentsmentioning

confidence: 99%

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Attentiveness of pediatricians to primary immunodeficiency disorders

et al. 2012

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BackgroundPrimary immunodeficiency (PID) is a cluster of serious disorders that requires special alertness on the part of the medical staff for prompt diagnosis and management of the patient. This study explored PID knowledge and experience among pediatricians of wide educational backgrounds, practicing in the United Arab Emirates (UAE).MethodA self-administered questionnaire was used to determine the competency of pediatricians in their knowledge of PID disorders. This study questionnaire included questions on PID signs and symptoms, syndromes associated with immunodeficiency, screening tests, interpreting laboratory tests and case management. The participants were 263 pediatricians of diverse education working in the 27 governmental hospitals in all regions of UAE.ResultsThe overall performance of the pediatricians did not differ based on their age, gender, origin of certification, rank, or years of experience. Of the 50 questions, 20% of pediatricians answered correctly <60% of the questions, 76% answered correctly 60 to 79% of the questions, and 4% answered correctly ≥80% of the questions. Seventeen of the 19 PID signs and symptoms were identified by 55 to 97%. Four of 5 syndromes associated with immunodeficiency were identified by 50 to 90%. Appropriate screening tests were chosen by 64 to 96%. Attention to the laboratory reference range values as function of patient age was notably limited.ConclusionsThere was a noteworthy deficiency in PID work-up. Therefore, implementing effective educational strategies is needed to improve the competency of pediatricians to diagnose and manage PID disorders.

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Section: Discussionmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Acknowledgmentsmentioning

confidence: 99%

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Attentiveness of pediatricians to primary immunodeficiency disorders

et al. 2012

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“…The upper estimates suggest that 6 million people worldwide may be living with a primary immunodeficiency disorder. 8 Despite the increasing numbers of primary immunodeficiency disorders diagnosed in the last decade 9 and a better understanding of immune system functions, many physicians lack knowledge regarding these disorders and do not often consider the possibility of immunodeficiency early enough in the differential diagnosis, 10,11 resulting in a significant delay in diagnosis and an increased morbidity and mortality. [12][13][14][15][16][17][18] Primary immunodeficiency disorder in Kuwait, a small country located in the Arabian Peninsula, is relatively common with a prevalence of 11.98 in 100,000 children, an incidence of 10.06 in 100,000 children, and an estimated occurrence of 1 in 1,000 live births.…”

Section: Introductionmentioning

confidence: 99%

Sinopulmonary Complications in Subjects With Primary Immunodeficiency

Owayed

Al‐Herz

2016

Respir Care

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BACKGROUND: The aim of this work was to describe the frequency and spectrum of sinopulmonary complications among subjects with primary immunodeficiency disorders. METHODS: The subjects included all patients with primary immunodeficiency who were registered prospectively between January 2004 and December 2013 in the Kuwait National Primary Immunodeficiency Disorders Registry. RESULTS: A total of 202 subjects were registered during the study period. Subjects with combined immunodeficiencies were the most prevalent (65 subjects, 32.1%), followed by well-defined syndromes with immunodeficiency (45 subjects, 22.2%) and predominantly antibody deficiencies (35 subjects, 17.3%). A total of 295 sinopulmonary manifestations were observed in 127 subjects (63%); 157 manifestations (53.2%) were observed among the presenting symptoms, and 138 manifestations (46.8%) occurred after establishment of the primary immunodeficiency disorder diagnosis. Sinopulmonary manifestations were more common in subjects with predominantly antibody deficiencies (2.3 manifestations/subject), followed by subjects with combined immunodeficiencies (1.75 manifestations/subject). Pneumonia was the most common manifestation (108 episodes affecting 80 subjects), followed by otitis media (81 episodes affecting 59 subjects), bronchiectasis in 28 subjects (13.8%), and asthma in 22 subjects (11%). Microbial organisms were isolated during 46 episodes of pneumonia (42.5%) (cytomegalovirus and Pneumocystis jirovecii were the most common). There were 57 deaths (28%) during the study period. Twenty-four deaths (42%) were due to pulmonary complications as follows: pneumonia (16 subjects, 8%), pulmonary hemorrhage (6 subjects, 3%), and aspiration pneumonia (2 subjects, 1%). CONCLUSIONS: Sinopulmonary complications are common in subjects with primary immunodeficiency. They can be serious and continue to occur even after proper treatment is initiated. The pulmonologist should play an important role in the management of subjects with primary immunodeficiency disorder.

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“…Early diagnosis of PID is crucial because often lifesaving interventions can be provided to decrease the rate of morbidity and mortality [2]. A major proportion of affected infants and children are offspring of consanguineous marriage; in addition, family medical history is very common in PID and it is an essential component in the diagnosis [3].…”

Section: Introductionmentioning

confidence: 99%

The Value of Family History in Diagnosing Primary Immunodeficiency Disorders

Hendaus

Al-Hammadi

Adeli

et al. 2014

Case Reports in Pediatrics

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Eliciting proper family medical history is critical in decreasing morbidity and mortality in patients with primary immunodeficiency disorders (PIDs). Communities with a common practice of consanguinity have a high rate of PIDs. We are presenting 2 cases where digging deeply into the family medical history resulted in the diagnosis of Omenn syndrome, a possibly fatal entity if not managed in a reasonable period.

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Primary Immunodeficiency Disorders: Survey of Pediatricians in Kuwait

Cited by 22 publications

References 19 publications

Attentiveness of pediatricians to primary immunodeficiency disorders

Attentiveness of pediatricians to primary immunodeficiency disorders

Sinopulmonary Complications in Subjects With Primary Immunodeficiency

The Value of Family History in Diagnosing Primary Immunodeficiency Disorders

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