Human PLCG2 haploinsufficiency results in a novel natural killer cell immunodeficiency.

BACKGROUND: Although most individuals effectively control herpesvirus infections, some suffer from severe and/or recurrent infections. A subset of these patients possess defects in natural killer (NK) cells, lymphocytes that recognize and lyse herpesvirus-infected cells; however, the genetic etiology is rarely diagnosed. PLCG2 encodes a signaling protein in NK-cell and B-cell signaling. Dominant-negative or gain-of-function variants in PLCG2 cause cold urticaria, antibody deficiency, and autoinflammation. However, loss-of-function variants and haploinsufficiency have not been reported to date. OBJECTIVES: The investigators aimed to identify the genetic cause of NK-cell immunodeficiency in 2 families and herein describe the functional consequences of 2 novel loss-of-function variants in PLCG2. METHODS: The investigators employed whole-exome sequencing in conjunction with mass cytometry, microscopy, functional assays, and a mouse model of PLCG2 haploinsufficiency to investigate 2 families with NK-cell immunodeficiency. RESULTS: The investigators identified novel heterozygous variants in PLCG2 in 2 families with severe and/or recurrent herpesvirus infections. In vitro studies demonstrated that these variants were loss of function due to haploinsufficiency with impaired NK-cell calcium flux and cytotoxicity. In contrast to previous PLCG2 variants, B-cell function remained intact. Plcg2+/- mice also displayed impaired NK-cell function with preserved B-cell function, phenocopying human disease. CONCLUSIONS: PLCG2 haploinsufficiency represents a distinct syndrome from previous variants characterized by NK-cell immunodeficiency with herpesvirus susceptibility, expanding the spectrum of PLCG2-related disease.

Sensitization to Common Allergens Among Children with Asthma and Allergic Rhinitis in Qatar.

BACKGROUND: Childhood asthma (A) and allergic rhinitis (AR) are common in Qatar. Aeroallergens sensitization is integral in disease pathogenesis and clinical presentation. Determining sensitization patterns assists clinicians in tailoring an efficient medical management. OBJECTIVE: To determine the aeroallergen sensitization pattern and relationship to clinical parameters. METHODS: A retrospective review of children (2-14-years) files with i) Pediatric Allergist/or Pulmonologist confirmed-diagnosis of A, and AR, and ii) positive skin prick test (SPT). RESULTS: Among 473 patients (69.1% males; 30.9% females), aged 7.6 years, family history was positive in 66.3%: 59.4% in A, 64.2% AR, and 78.2% A-AR. The number of allergens/patients was 2.1±1.7. Median eosinophil count was 400 cells/ul and IgE 287 KU/L. Rates of A, AR, and A-AR varied significantly in children ≤5 years compared to >10 years: A was 43.2% vs 17.8%, and AR 34.5% vs 16.4%. Two hundred and four children (43.1%) were mono-sensitized, 215 (45.5%) oligosensitized (2-3 allergens), and 54 (11.4%) polysensitized (≥4 allergens). A-AR ranked the top number of positive allergens. The commonest aeroallergen was Der p1 (38.1%), followed by Der f (29.0%), cat (22.6%), alternaria (18.8%), American cockroach (18.4%), and dog (14.0%). House dust mite (HDM) and American cockroach were commoner in ≤5 years than older >10-year children (52.5%, 24.1%), while cat and dog allergens were commoner in older ones (37.1%, 21.6%). CONCLUSION: Family history is quite positive in patients with A and AR. Common aeroallergens include HDM, cats, and alternaria in the young children, while animal allergens were commoner in the older children.

A founder RAB27A variant causes Griscelli syndrome type 2 with phenotypic heterogeneity in Qatari families.

Griscelli syndrome type 2 (GS2) is a rare autosomal recessive disorder caused by pathogenic variants in the RAB27A gene and characterized by partial albinism, immunodeficiency, and occasional hematological and neurological involvement. We reviewed and analyzed the medical records of 12 individuals with GS2 from six families belonging to a highly consanguineous Qatari tribe and with a recurrent pathogenic variant in the RAB27A gene (NM_004580.4: c.244C > T, p.Arg82Cys). Detailed demographic, clinical, and molecular data were collected. Cutaneous manifestations were the most common presentation (42%), followed by neurological abnormalities (33%) and immunodeficiency (25%). The most severe manifestation was HLH (33%). Among the 12 patients, three patients (25%) underwent HSCT, and four (33%) died. The cause of death in all four patients was deemed HLH, providing evidence for this complication's fatal nature. Interestingly, two affected patients (16%) were asymptomatic. This report highlights the broad spectrum of clinical presentations of GS2 associated with a founder variant in the RAB27A gene (c.244C > T, p.Arg82Cys). Early suspicion of GS2 among Qatari patients with cutaneous manifestations, neurological findings, immunodeficiency, and HLH would shorten the diagnostic odyssey, guide early and appropriate treatment, and prevent fatal outcomes.

Biallelic mutations in DNA ligase 1 underlie a spectrum of immune deficiencies.

We report the molecular, cellular, and clinical features of 5 patients from 3 kindreds with biallelic mutations in the autosomal LIG1 gene encoding DNA ligase 1. The patients exhibited hypogammaglobulinemia, lymphopenia, increased proportions of circulating γδT cells, and erythrocyte macrocytosis. Clinical severity ranged from a mild antibody deficiency to a combined immunodeficiency requiring hematopoietic stem cell transplantation. Using engineered LIG1-deficient cell lines, we demonstrated chemical and radiation defects associated with the mutant alleles, which variably impaired the DNA repair pathway. We further showed that these LIG1 mutant alleles are amorphic or hypomorphic, and exhibited variably decreased enzymatic activities, which lead to premature release of unligated adenylated DNA. The variability of the LIG1 genotypes in the patients was consistent with that of their immunological and clinical phenotypes. These data suggest that different forms of autosomal recessive, partial DNA ligase 1 deficiency underlie an immunodeficiency of variable severity.

Camel's milk allergy.

INTRODUCTION: Camel's milk is a safe and therapeutic nutrient. Camel's milk allergy is almost unknown. OBJECTIVE: To identify the clinical and laboratory features of camel's milk allergy. METHODS: In this retrospective study, the records of patients with camel's milk allergy were reviewed. Data collected included age, sex, clinical presentation, concomitant allergies, family history, laboratory tests (complete blood cell count [CBC], white blood cell [WBC] count, total immunoglobulin E [IgE], food specific IgE), and skin-prick tests (SPT) to camel's milk and other foods. RESULTS: Nine patients (four male patients, five female patients; mean age ± SD 4.3 ± 2.4 years) presented with cutaneous urticaria and/or angioedema (five patients [55.6%]) and anaphylaxis (four patients [44.4%]). Allergic reactions occurred within the first 15 minutes of ingesting camel's milk in all the patients (100%). Concurrent allergies were observed in 77.8% of the patients, of whom, five patients (71.4%) had atopic dermatitis (AD) and two patients (22%) had cow's milk allergy that exacerbated AD. All the patients (100%) had a family history of allergies. The family farm was the source of camel's milk in all the patients (100%). The WBC count was 9425 ± 1452.8 (mean ± SD) cells/µL, and eosinophils was 612 ± 455.4 (mean ± SD) cells/µL, and the median IgE was 301.5 kU/mL. A camel's milk SPT resulted in a wheal of 8.7 ± 4.9 (mean ± SD) mm. CONCLUSION: Camel's milk allergy is a distinct, yet very rare, disease entity. Cutaneous and systemic allergic reactions are the main clinical manifestations. Concomitant other allergies, viz., AD, and positive family history are risk factors. Early life exposure to camel's milk is a possible risk factor. High blood eosinophil counts and total IgE levels were observed in patients with camel's milk allergy. In the presence of a consistent and specific clear-cut history of camel's milk-related symptoms, a SPT was a dependable confirmatory test.

Allergic diseases among children: nutritional prevention and intervention.

Allergic diseases comprise a genetically heterogeneous group of chronic, immunomediated diseases. It has been clearly reported that the prevalence of these diseases has been on the rise for the last few decades, but at different rates, in various areas of the world. This paper discusses the epidemiology of allergic diseases among children and their negative impact on affected patients, their families, and societies. These effects include the adverse effects on quality of life and economic costs. Medical interest has shifted from tertiary or secondary prevention to primary prevention of these chronic diseases among high-risk infants in early life. Being simple, practical, and cost-effective are mandatory features for any candidate methods delivering these strategies. Dietary therapy fits this model well, as it is simple, practical, and cost-effective, and involves diverse methods. The highest priority strategy is feeding these infants breast milk. For those who are not breast-fed, there should be a strategy to maintain beneficial gut flora that positively influences intestinal immunity. We review the current use of probiotics, prebiotics, and synbiotics, and safety and adverse effects. Other dietary modalities of possible potential in achieving this primary prevention, such as a Mediterranean diet, use of milk formula with modified (hydrolyzed) proteins, and the role of micronutrients, are also explored. Breast-feeding is effective in reducing the risk of asthma, allergic rhinitis, and atopic eczema among children. In addition, breast milk constitutes a major source of support for gut microbe colonization, due to its bifidobacteria and galactooligosaccharide content. The literature lacks consensus in recommending the addition of probiotics to foods for prevention and treatment of allergic diseases, while prebiotics may prove to be effective in reducing atopy in healthy children. There is insufficient evidence to support soy formulas or amino acid formulas for prevention of allergic disease. A healthy diet, such as the Mediterranean diet, may have a protective effect on the development of asthma and atopy in children. In children with asthma and allergic diseases, vitamin D deficiency correlates strongly with asthma, allergic rhinitis, and wheezing.

Linear growth and endocrine function in children with ataxia telangiectasia.

INTRODUCTION: Ataxia telangiectasia (AT) is a rare, genetic, primary immune deficiency disease characterized by immunodeficiency and neurological manifestations, with an increased tendency to infection, malignancy, and autoimmune diseases. Both growth delay and endocrine abnormalities are occasionally reported in these patients. PATIENTS AND METHODS: We studied growth parameters height (Ht), weight, body mass index (BMI) and calculated the Ht standard deviation scores (HtSDS) of 13 patients (age 7.7 ± 3.5 years-age range: 3-14.5 years) with AT in relation to their mid-parental Ht SDS (MPHtSDS). We measured their serum calcium (Ca), phosphorus (PO4), alkaline phosphatase, alanine transferase (ALT), serum ferritin, creatinine and albumin concentrations. Endocrine investigations included the assessment of serum free thyroxine (FT4), thyrotropin (TSH), insulin-like growth factor-I (IGF-I) and morning cortisol. Complete blood count and serum immunoglobulins (IgG, IgM and IgA antibodies) were also measured. Growth data were correlated to hormonal and immune data. RESULTS: About 31% of patients with AT had short stature (HtSDS <-2). However, their MPHtSDS denoted that their short stature was familial because four out of 13 had MPHtSDS <-2. They had low BMI, and two of them had low serum albumin and IGF-I, denoting malnutrition or disturbed growth hormone secretion. Elevated serum ALT and ferritin in some patients suggest immune-related inflammation in the liver. 30% of patients had high TSH, two of them had low FT4 diagnosing overt (15%) and sub-clinical (15%) hypothyroidism. Anti-thyroid peroxidase antibodies were high in two out of 13 patients denoting immune-related thyroid aggression. Eight out of 13 patients had Vitamin D deficiency (<20 ng/ml) however, their serum Ca and PO4 levels were in the normal range. One adolescent girl (14.5 years) had hyper-gonadotropic hypogonadism (low estradiol and high follicle stimulating hormone). All patients had normal 8 AM cortisol and renal function. None of the growth parameters were correlated with the IgG, IgM or IgA levels. IN SUMMARY: Patients with AT had a high prevalence of growth retardation and endocrine dysfunction in the form of low IGF-I, overt and subclinical hypothyroidism and hypogonadism. Physicians should be aware of these possible endocrinopathies for an early diagnosis and proper treatment.

The impact of Vitamin D deficiency on asthma, allergic rhinitis and wheezing in children: An emerging public health problem.

BACKGROUND: Vitamin D deficiency has been declared a public health problem for both adults and children worldwide. Asthma and related allergic diseases are the leading causes of morbidity in children. The objective of this study was to investigate the potential role of Vitamin D deficiency in childhood asthma and other allergic diseases such as allergic rhinitis and wheezing. MATERIALS AND METHODS: This cross-sectional study was conducted in Primary Health Care Centers (PHCs), from March 2012 to October 2013. A total of 2350 Qatari children below the age of 16 were selected from PHCs, and 1833 agreed to participate in this study giving a response rate of (78%). Face-to-face interviews with parents of all the children were based on a questionnaire that included variables such as socio-demographic information, assessment of nondietary covariates, Vitamin D intake, type of feeding, and laboratory investigations. Their health status was assessed by serum Vitamin D (25-hydoxyvitamin D), family history and body mass index. RESULTS: Most of the children who had asthma (38.5%), allergic rhinitis (34.8%) and wheezing (35.7%) were below 5 years. Consanguinity was significantly higher in parents of children with allergic rhinitis (48.6%), followed by those with asthma (46.4%) and wheezing (40.8%) than in healthy children (35.9%) (P < 0.001). The proportion of severe Vitamin D deficiency was significantly higher in children with wheezing (23.4%), allergic rhinitis (18.5%), and asthma (17%) than in healthy children (10.5%). Exposure to the sun was significantly less in Vitamin D deficient children with asthma (60.3%), allergic rhinitis (62.5%) and wheezing (64.4%) than in controls (47.1%) (P = 0.008). It was found that Vitamin D deficiency was a significant correlate for asthma (odds ratio [OR] =2.31; P < 0.001), allergic rhinitis (OR = 1.59; P < 0.001) and wheezing (relative risk = 1.29; P = 0.05). CONCLUSION: The study findings revealed a high prevalence of Vitamin D deficiency in children with asthma and allergic diseases. Vitamin D deficiency was a strong correlate for asthma, allergic rhinitis and wheezing.

Is high prevalence of vitamin D deficiency a correlate for attention deficit hyperactivity disorder?

The aim of the study was to determine the association between vitamin D and attention deficit hyperactivity disorder (ADHD), and difference in the level of vitamin D in ADHD children and control. This a case-control study carried out in school health and primary health care clinics. A total of 1,331 children and adolescents who were diagnosed with ADHD based on clinical criteria and standardized questionnaires were enrolled in this study and were matched with 1,331 controls, aged 5-18 years old. Data on body mass index (BMI), clinical biochemistry variables including serum 25-hydroxyvitamin D were collected. The study found significant association between ADHD and vitamin D deficiency after adjusting for BMI and sex (adj. OR 1.54; 95 % CI 1.32-1.81; P < 0.001). Majority of the ADHD children were in the age group 5-10 years (40.7 %), followed by 11-13 years (38.4 %). The proportion of BMI <85th percentile was significantly over represented in ADHD group as compared to healthy control (87.8 vs. 83 %; P < 0.001, respectively), while on the other hand, BMI >95th percentile was over represented in the control than ADHD group (7.6 vs. 4.6 %; P < 0.001, respectively). Mean values of vitamin D (ng/mL) were significantly lower in ADHD children (16.6 ± 7.8) than in healthy children (23.5 ± 9.0) (P < 0.001). There was significant correlation between vitamin D deficiency and age (r = -0.191, P = 0.001); calcium (r = 0.272, P = 0.001); phosphorous (r = 0.284, P = 0.001); magnesium (r = 0.292, P = 0.001); and BMI (r = 0.498, P = 0.001) in ADHD children. The vitamin D deficiency was higher in ADHD children compared to healthy children.

Primary immunodeficiency diseases in children: 15 year experience in a tertiary care medical center in Qatar.

BACKGROUND: Primary immunodeficiency diseases (PID) are a group of heterogeneous, rare, genetic, mainly childhood disorders that affect specific components of immune system leading to serious complications. OBJECTIVES: This study is aimed at describing the prevalence and the categories of PID, the ages of onset and the diagnosis, the clinical presentations, the treatment modalities and the overall outcome of affected patients. MATERIALS AND METHODS: A retrospective study was conducted on 131 pediatric patients (aged 0-14 years) diagnosed with PID at Hamad General Hospital during a 15-year period (1998-2012). RESULTS: Data of 131 patients (75 males & 56 females) was analyzed with an estimated prevalence of 4.7 PID patients per 100,000 children younger than 14 years of age. The most common type of PID was predominantly antibody deficiency (23.7 %), followed by other well-defined immunodeficiency syndromes (22.9 %), 19.1 % combined T and B cell immunodeficiency, but rare CVID, and no cases of complement deficiency. The mean onset age was 24.01 months and diagnosis age was 42.2 months. Recurrent infections, particularly pneumonia (48.9 %), failure to thrive (34.4 %), otitis media (26 %), sepsis (23.7 %), and chronic diarrhoea (21.4 %) were commonest presenting conditions. P. aeruginosa (15.7 %), Salmonella species (13.2 %), and Non-TB mycobacteria (13.2 %) were the most common bacterial isolates. The overall mortality rate was 21.4 % with combined immunodeficiency's accounting for 53.4 % of deaths. CONCLUSIONS: This study reveals that PIDs are not rare in children in Qatar; and like other studies predominantly antibody deficiencies are the most common. Strategies that reinforce awareness and education of practicing physicians, bone marrow transplantation, and establishing PID national registry should be adopted to reduce mortality and morbidity of PID patients in Qatar.

Vitamin D deficiency as a strong predictor of asthma in children.

BACKGROUND: Epidemiological studies suggest a link between vitamin D deficiency in early life and development of asthma in later life. AIM: The aim of this study was to measure serum vitamin D levels in asthmatic children and to compare these to healthy non-asthmatic controls. METHODS: Asthmatic (n = 483) and healthy control (n = 483) children were recruited from the Pediatric Allergy-Immunology Clinics of Hamad General Hospital and the Primary Health Care Clinics in Qatar from October 2009 to July 2010. All children were below 16 years of age and asthma was diagnosed by a physician. Parents of all children completed extensive questionnaires documenting demographics, child's feeding practice and vitamin D intake. Serum vitamin D (25-hydroxyvitamin D), calcium, phosphorus, alkaline phosphatase, magnesium, creatinine and parathyroid hormone assays were performed. Subjects with serum containing less than 20 ng/ml vitamin D were deemed deficient. RESULTS: Asthmatic children had significantly reduced serum vitamin D levels compared to non-asthmatic children (p < 0.001); 68.1% of all asthmatics were vitamin D deficient. Asthmatic children had significantly higher degrees of moderate (41.8 vs. 25.1%) and severe (26.3 vs. 11.0%) vitamin D deficiency compared to healthy controls (p < 0.001). Positive familial history of vitamin D deficiency (35.6%, p = 0.005) and asthma (36.4%, p = 0.009) were significantly higher in asthmatic children. Along with vitamin D deficiency, asthmatics also had reduced phosphorus (p < 0.001) and magnesium (p = 0.001) levels but elevated serum alkaline phosphatase (p < 0.001) and IgE (p < 0.001). The majority of asthmatic children had less exposure to sunlight (66.7%, p = 0.006) and less physical activity (71.3%, p < 0.001). Vitamin D deficiency was the strongest predictor of asthma in this population (OR 4.82; 95% CI 2.41-8.63, p < 0.001). CONCLUSION: The present study revealed that the majority of asthmatic children had vitamin D deficiency compared to control children. Vitamin D deficiency was the major predictor of asthma in Qatari children.

Co-existing paradoxical vocal cord motion and asthma in a young child.

We are reporting a 7½-year-old child who had coexisting paradoxical vocal cord motion (PVCM) and asthma. There are only a few reported cases of PVCM in children younger than 10 years of age. Thus diagnosis mainly relies on high index of suspicion for these children. Missing PVCM diagnosis can lead to unnecessary diagnostic and therapeutic interventions to control asthma. We hope that this case report would increase the awareness of such coexistence in such a young age group.

Camel milk: an alternative for cow's milk allergy in children.

Treatment of cow's milk allergy (CMA) in children includes avoidance of cow's milk and providing a milk substitute. This study was designed to determine whether CMA children could safely consume camel's milk as an alternative, and skin-prick test (SPT) to camel's milk could be a reliable tool in selecting them. Between April 2007 and February 2010, children with confirmed CMA seen at the Allergy-Immunology Clinic, Hamad Medical Corp., were enrolled into this prospective cohort study. Subjects had a detailed history and medical examination, complete blood count with differential count, total serum IgE, and specific IgE test and SPT to cow's milk. Patients with positive SPT and an elevated cow's milk-specific IgE had negative SPT to camel's milk. Of 35 children (23 male and 12 female children) aged 4-126 months (median, 21 months), 23 patients (65.7%) presented with acute urticaria, 17 (48.6%) with atopic dermatitis, 9 (25.7%) with anaphylaxis, 8 (22.9%) with failure to thrive, and 5 (14.3%) with chronic vomiting. Twenty-eight patients (80%) had family history of allergy. Twenty-six patients (74.3%) were breast-fed for ≤18 months. Mean white blood cell count was 9860.5 cells/µL, absolute eosinophil count was 1219 cells/µL, IgE was 682 IU/mL, and cow's milk-specific IgE was 22.01 kU/L. Only 7 patients (20%) had positive SPT to camel's milk and 28 (80%) were negative to camel's milk. All patients with negative SPT took camel's milk without any reactions. In children with CMA, SPT is a reliable clinical test in ruling out reactivity to camel's milk so these children could safely take camel's milk as an alternative nutrient.

Exclusive breast feeding and prevention of diarrheal diseases: a study in Qatar / Aleitamento materno exclusivo na prevenção de doenças diarréicas: um estudo realizado no Qatar

OBJECTIVE: to explore the relationships between breast feeding and diarrhea and to assess the effect of exclusive breast feeding (EBF) on reducing the risk of diarrhea in Qatar. METHODS: this is a cross sectional survey carried out at the Well baby clinics and Pediatric clinics in the 11 Primary Health Care Centers and Hamad General Hospital, Hamad Medical Corporation, in Qatar. A multistage sampling design was used and a representative sample of 1500 Qatari infants and preschool children with an age range of 0-3 years and mothers agedbetween 18 to 47 years were surveyed during the period from October 2006 to September 2008 in Qatar. Out of the 1500 mothers, 1278 agreed to participate in this study, with a response rate of 85.2 percent. Questionnaires were administered to women who were attending Primary Health Care (PHC) Centers for child immunization. Data about the child gathered included date of birth, gender, birth order, consanguinity, socio-economic conditions, age of mother, level of education of mother, occupation, mode of breast feeding, sterilization of bottle and incident of diarrhea during the interview. Univariate statistical methods were performed for statistical analysis. RESULTS: of the 1278 infants studied, more than half (59.3 percent) were exclusively breastfed, and the mean duration was11.4 (SD=6.7). The risk for presenting diarrhea was higher in formula fed (48.7 percent) and partiallybreastfed children (37.3 percent) when compared to EBF(32.5 percent). CONCLUSION: EBF plays an important role in reducing the incidence and severity of infantile diarrhea.

OBJETIVOS: investigar a relação entre a amamentação e a diarréia e avaliar o efeito do aleitamento maternao exclusivo (AME) na redução do risco de diarréia noQatar. MÉTODOS: um estudo transversal realizado nos Well Baby Clinics and Pediatric Clinics dos 11 centros de atenção básica do Hamad General Hospital, Hamad Medical Corporation, no Qatar. Um desenho amostral de múltiplos estágios foi empregado com uma amostra representativa de 1500 recémnascidos e crianças de idade pré-escolar catarianos entre outubro de 2006 esetembro de 2008 no Qatar. Entre as 1500 mães, 1278 aceitaram participar neste estudo, uma taxa de resposta de 85,2 por cento. Os questionários foram aplicados a mulheres atendidas pelos centros de atenção básica para imunização infantil. Dados colecionados sobre as crianças incluíram data de nascimento, gênero, ordem de nascimento, consanguinidade, condições socioeconômicas, idade maternal, escolaridade maternal, ocupação maternal, método de amamentação, esterilização dos biberões, e incidência de diarréia durante a entrevista. Métodos univariados e foram utilizados para realizar a análise estatística. RESULTADOS: dos 1278 recém-nascidos estudados, mais que metade (59.3 por cento) foram exclusivamente amamentados e a duração média foi de 11,4 (DP=6,7). O risco de apresentar a diarréia foi mais alto nas crianças aleitadas com fórmulas (48.7 por cento) e nas que foram parcialmente amamentadas (37.3 por cento) em comparação com a AME(32.5 por cento). CONCLUSÕES: o AME desempenha um papel importante na redução da incidência e da gravidade da diarréia infantil.

Camel milk is a safer choice than goat milk for feeding children with cow milk allergy.

Background. Various sources of mammalian milk have been tried in CMA. Objectives. To determine whether camel milk is safer than goat milk in CMA. Methods. Prospective study conducted at Hamad Medical Corporation between April 2007 and April 2010, on children with CMA. Each child had medical examination, CBC, total IgE, cow milk-specific IgE and SPT. CMA children were tested against fresh camel and goat milks. Results. Of 38 children (median age 21.5 months), 21 (55.3%) presented with urticaria, 17 (39.5%) atopic dermatitis, 10 (26.3%) anaphylaxis. WBC was 10, 039 ± 4, 735 cells/µL, eosinophil 1, 143 ± 2, 213 cells/µL, IgE 694 ± 921 IU/mL, cow's milk-specific-IgE 23.5 ± 35.6 KU/L. Only 7 children (18.4%) tested positive to camel milk and 24 (63.2%) to goat milk. 6 (15.8%) were positive to camel, goat, and cow milks. Patients with negative SPT tolerated well camel and goat milks. Conclusions. In CMA, SPT indicates low cross-reactivity between camel milk and cow milk, and camel milk is a safer alternative than goat milk.

Treatment outcome of acne vulgaris with oral isotretinoin.

OBJECTIVE: To determine the clinical efficacy of oral isotretinoin in the treatment of severe acne and assess its effect on total serum cholesterol, triglycerides, HDL-cholesterol and Low-Density Lipoprotein-cholesterol (LDL-cholesterol). STUDY DESIGN: A cohort, descriptive, hospital-based study. PLACE AND DURATION OF STUDY: Al-Ain Medical District, Tawam Hospital, United Arab Emirates, from 1994 to 2002. METHODOLOGY: A total of 198 patients seen at Tawam Hospital, referred with acne vulgaris for a minimum of 6 weeks, were treated by isotretinoin for the first time, were included in the study. Variables studied were as per objectives apart from demographics and distribution. RESULTS: The study included 63 (32%) males and 135 (68%) females of mean age (+/-SD) of 21.3+/-5.6 years. Majority (81%) of patients was under 25 years. Of them, 26 patients had family history of acne. The most common site of acne was on face (66.7%), followed by trunk (26.2%) and neck (9.1%). Of 198 patients treated, 32.8% were cured, 19.1% markedly improved, 11.1% moderately improved and 24.2% of patients were advised for further treatment. There was no marked change in total and LDL-cholesterol, while LDL and triglycerides changed markedly. CONCLUSION: In acne patients, isotretinoin is effective in producing remission. In addition, it was safe and its effect on serum lipids was transient, especially in healthy and young patients with normal liver functions.

Protective effect of breastfeeding on diarrhea among children in a rapidly growing newly developed society.

In developed communities, the effect of exclusive breastfeeding (EBF) is encouraged since it has been found to be protective against infantile diarrhea. In a newly developing Qatar, modern water supply and sanitation facilities have become available to everyone during the last two decades. The objectives of the current study were to explore the relationships between breastfeeding and diarrhea and to assess the effect of EBF on the risk reduction of diarrhea in children aged 1-5 years. This is a cross-sectional survey conducted in the Well-Baby clinics and pediatric clinics in the 11 Primary Health Care (PHC) Centers and Hamad General Hospital, Hamad Medical Corporation, Qatar. A multistage sampling design was used, and a representative sample of 1500 Qatari infants and pre-school children in the age group of 1-5 years and mothers aged between 18 to 47 years were surveyed during the period from October 2006 to September 2007; 1,278 mothers agreed to participate in this study, with a response rate of 85.2%. The sociodemographic characteristics, feeding modes and diarrhea morbidity were collected from the parents of the children during the interview. Of the 1,278 infants studied, more than half (59.3%) were EBF, followed by those partially breastfed (28.3%), and finally the formula fed (12.4%). The duration of EBF was 11.4 +/- 6.7 months (mean +/- SD) and the duration of partial breastfeeding with bottled milk was 9.2 +/- 4.1 months (mean +/- SD), and the difference was statistically significant (p<0.0010). When compared to the EBF infants, the risk of diarrhea was higher and statistically significant in both the partially breastfed (48.7% vs 32.5%) and in the non-EBF (37.3% vs 32.5%, p<0.001). Upper respiratory tract infection (URTI), short duration of breastfeeding, level of maternal education, and sterilization of bottles were considered as predictors. These results indicate that in Qatar, breastfeeding plays an important role in reducing the incidence and severity of infantile diarrhea. This observation is particularly important given the growing concern that, as an unwanted effect of 'modernization', breastfeeding is on the decline in Qatar and comparable populations elsewhere.