Role of Itolizumab in the Treatment of COVID-19 Patients, Admitted to ICU at a Tertiary Care Hospital

Background: Clinical studies have correlated severe deterioration of COVID-19 patients due to excessive and uncontrolled production of cytokines. There is a pressing need to explore therapies, which could prevent the cytokine storm rather than terminating it. Aims and objectives: The aim of the study is to evaluate the effect of itolizumab on clinical outcomes of patients with moderate-severe COVID-19 disease admitted to ICU. The primary aim of the current study is to find out any mortality benefit in 14 days. The secondary aim is to assess the morbidity outcomes in terms of reduction in inflammatory markers and also the duration of hospital stays to assess the prognostication. Materials and methods: It is a retrospective case-control study in which laboratory-confirmed COVID-19 patients admitted to ICU were taken. A total of 62 patients were recruited, 31 patients received itolizumab (cases/treatment group) and 31 patients didn’t receive itolizumab (designated as controls). Results: Among the total patients recruited, 68% of the study population was male and 32% were female. A total of 12 patients expired among cases and 13 expired among controls. Overall mortality in both groups was noted to be almost similar. The control group showed mortality at lower computed tomography (CT) scores compared to the cases. There is a significant reduction in inflammatory markers, like interleukins-6 (IL-6) and D-dimer in cases compared to the control group. Conclusion: In conclusion, treating patients with cytokine storms before they require intubation/mechanical ventilation is crucial to preventing deaths. Itolizumab has shown no clinical benefit in critically ill COVID-19 patients, however, timely initiation of itolizumab therapy may serve as a key therapeutic option in preventing the mortality and morbidity outcomes in moderate-severe COVID-19 patients.

Colorectal carcinoma up to the second decade of life: An 8‑year experience in a tertiary care center.

AIMS AND OBJECTIVES: To evaluate the demographic pattern, incidence, and histological characteristics of colorectal carcinomas (CRCs) in very young adults diagnosed in the center. MATERIALS AND METHODS: We retrieved and reviewed slides and data pertaining to all the cases of CRCs and “segregated into decade wise age‑groups” from the archives of Department of Pathology. Patients with age ≤20 years diagnosed during the last 8 years (2006–2013) were further evaluated. RESULTS: Totally, 590 cases of CRCs diagnosed over last 8‑year period, of which 4.2% (25 cases) presented in the study group (age ≤20 years) with a mean age of 17 years. About 50% of the tumors were either signet ring cell, mucin‑secreting or poorly differentiated carcinomas. Four cases occurred in a background of familial adenomatous polyposis (FAP), three of which showed high‑grade dysplasia, while in one case, carcinoma‑in‑situ was diagnosed. In all but two cases, rectum was the site of involvement except FAP cases in which colorectal location was noted. CRCs show a sharp rise in earlier age onset (≤40 years) and an increasing trend was followed in patients between age groups third, fourth, and fifth decades of life over the last 8 years. CONCLUSION: Colorectal carcinomas show an increasing trend in young age (≤40 years). This change may be attributed to dietary, lifestyle changes, and newer genetic alterations in developing countries. In very young age group (≤20 years), a higher grade and stage at the time of diagnosis and predominantly rectal involvements are the distinct features.

Oral and oropharyngeal squamous cell carcinoma in our population: the clinic-pathological and morphological description of 153 cases / Carcinoma de células escamosas oral y orofaríngeo en nuestra población: descripción clínico-patológica y morfológica de 153 casos

Oral cavity and oropharynx are one of the commonest sites for cancers in our population due to a high prevalence of tobacco use, especially chewing, in our country. The objective of this study was to prospectively assess the clinico-pathological and morphological aspects of the patients presenting to us with these malignancies. We studied 153 patients with oral and oropharyngeal squamous cell carcinoma who were managed in the department of otolaryngology and head and neck surgery between January 2006 and December 2007 at Maulana Azad Medical College and associated hospitals in New Delhi. There were 127 male patients (83 percent) and 26 females (17 percent) with ages ranging from 22 years to 70 years. One hundred and eleven patients (73 percent) presented with oral cavity and 42 patients (27 percent) with oropharyngeal carcinomas. Most common site of presentation in the oral cavity was the buccal mucosa, whereas, base of tongue was the commonest site in the oropharynx. Amongst the oral cavity cancers, 51 each (46 percent each) were well differentiated and moderately differentiated, whereas, 9 (8 percent) were poorly differentiated cancers. However, amongst the oropharyngeal cancers, 27 (64 percent) were moderately differentiated and 15 (36 percent) were poorly differentiated. Overall, 73 patients presented with lymphadenopathy on presentation, out of which, 44 patients were those with oral cavity and 29 with oropharyngeal cancers. There was a significant correlation between the site (i.e. oral cavity or orophaynx) and lymphadenopathy on presentation. Fifty nine patients (39 percent) presented to us with early stage disease (i.e. stage I and II), whereas, 94 patients (61 percent) presented with late stage disease (i.e. stage III and IV). There was a significant correlation between the site (i.e. oral cavity or oropharynx) and stage at presentation.

En nuestro país, la cavidad oral y orofaringe son los sitios más comunmente afectados por cáncer, debido a la alta prevalencia del consumo de tabaco, especialmente, el tabaco de mascar. El objetivo de este estudio fue evaluar prospectivamente los aspectos clínico-patológicos y morfológicos de los pacientes que acuden con estas malignidades. Se estudiaron 153 pacientes con carcinoma de células escamosas de la cavidad oral y orofaringe que fueron tratados en el Servicio de Otorrinolaringología y Cirugía de Cabeza y Cuello entre enero del 2006 y diciembre del 2007 en Maulana Azad Medical College y hospitales asociados en Nueva Delhi. Se evaluaron pacientes de ambos sexos, 127 hombres (83 por ciento) y 26 mujeres (17 por ciento) con edades entre 22 a 70 años. Ciento once pacientes (73 por ciento) presentaron carcinoma de la cavidad oral y 42 (27 por ciento) carcinomas de la orofaringe. El sitio más común de presentación en la cavidad oral fue la mucosa bucal, mientras que la base de la lengua fue el sitio más común de la orofaringe. Entre los cánceres de la cavidad oral, 51 (46 por ciento cada uno) estaban bien diferenciados y moderadamente diferenciados, mientras que 9 (8 por ciento) fueron escazamente diferenciado. Sin embargo, entre los cánceres de la orofaringe, 27 (64 por ciento) fueron moderadamente diferenciados y 15 (36 por ciento) fueron pobremente diferenciados. En total, 73 pacientes se presentaron con adenopatías, de los cuales, 44 pacientes fueron en la cavidad oral y 29 en la orofaringe. Se observó una correlación significativa entre el sitio (es decir, la cavidad oral u orofaringe) y la presentación de adenopatías. Cincuenta y nueve pacientes (39 por ciento) se presentaron con enfermedad en estadio temprano (estadios I y II), mientras que 94 pacientes (61 por ciento) se presentaron con enfermedad en estadio tardío (etapas III y IV). Se observó una correlación significativa entre el sitio (cavidad oral u orofaringe) y la etapa en la presentación.

Intraparotid facial nerve neurofibroma: an uncommon neoplasm / Neurofibroma intraparotídeo del nervio facial: una neoplasia poco frecuente

Intraparotid facial nerve neurofibromas are a rare entity, and are grossly and histopathologically distinct from the comparatively more prevalent schwannomas. We report a rare case of an intraparotid facial nerve neurofibroma with intratemporal extension in a 48-year-old female. The tumor was surgically excised with cable grafting of the facial nerve. Electroneurography may eventually assist in the preoperative diagnosis. Facial nerve resection should be more readily contemplated in a patient with neurofibroma than in a patient with schwannoma; however, facial nerve preservation with serial follow-up electroneurography and computerized tomography remains a viable option in patients with small facial nerve neurofibromas when electrical testing reveals minimal evidence of progressive neural degeneration. Close monitoring, especially in neurofibromas associated with Von Recklinghausen's disease, is mandatory because of an increased risk of sarcomatous degeneration.

Los neurofibromas intraparotídeos del nervio facial son una entidad poco frecuente, macroscópicamente e histopatológicamente diferentes de los comparativamente más frecuentes schwannomas. Se presenta un caso raro de un neurofibroma del nervio facial con extensión intraparotídea intratemporal en una mujer de 48 años de edad. El tumor se extirpó quirúrgicamente con injerto del nervio facial. La electroneurografía puede llegar a ayudar en el diagnóstico preoperatorio. La resección del nervio facial debe ser realizada con mayor facilidad en un paciente con neurofibroma que en un paciente con schwannoma, sin embargo, la preservación del nervio facial con electroneurografía de seguimiento y tomografía computarizada sigue siendo una opción viable en pacientes con pequeños neurofibromas del nervio facial, cuando las pruebas eléctricas revelan mínima evidencia de degeneración neuronal progresiva. Una estrecha vigilancia, especialmente en los neurofibromas asociados con la enfermedad de Von Recklinghausen es fundamental debido al aumento del riesgo de degeneración sarcomatosa.

Primary tuberculosis of nose with intracranial extension: A rare presentation.

Tuberculosis continues to be a major public health problem. This disease has varying presenting features. We here present a case of primary tuberculosis of the nose with intracranial extension, a very rare presenting feature of tuberculosis

Preoperative computed tomographic evaluation of inferior nasal concha hypertrophy and its role in deciding surgical treatment modality in patients with deviated nasal septum / Evaluación preoperatoria con tomografía computada de la hipertrofia de la concha nasal inferior y su rol en la decisión de la modalidad de tratamiento quirúrgico en pacientes con tabique nasal desviado

Deviated nasal septum is associated with compensatory hypertrophy of the inferior nasal concha on the contralateral side. In the past conventional septoplasty was done for the deviated septum, which would improve the patency on the side of deviation but would worsen it on the opposite side due to reallocation of the septum to the midline. The present study of 50 patients unfolds the anatomical composition of the inferior nasal concha based on the computed tomographic scan and help the otolaryngologist to determine whether to add turbinoplasty to standard septoplasty procedure or not.

Un tabique nasal desviado está asociado con una hipertrofia compensatoria de la concha nasal inferior del lado contra lateral. En el pasado se realizaba septoplastía convencional para el tabique desviado, lo que mejoraraba la permeabilidad en el lado de la desviación, pero agravaba ésta en el lado opuesto, debido a la reacomodación del tabique en el plano mediano. En el presente estudio se evaluó en 50 pacientes la composición anatómica de las conchas nasales inferiores, basado en la exploración a través de tomografía computarizada, y de esta manera ayudar al otorrinolaringólogo a determinar si es necesario o no, añadir la plastía de la concha nasal inferior al procedimiento de septoplastía estándar.

Deafness: Burden, prevention and control in India.

The high burden of deafness globally and in India is largely preventable and avoidable. According to the 2005 estimates of WHO, 278 million people have disabling hearing impairment. The prevalence of deafness in Southeast Asia ranges from 4.6% to 8.8%. In India, 63 million people (6.3%) suffer from significant auditory loss. Nationwide disability surveys have estimated hearing loss to be the second most common cause of disability. A lack of skilled manpower and human resources make this problem a huge challenge. The Government of India has launched the National Programme for Prevention and Control of Deafness. This article highlights the major components of the programme with a focus on manpower development and ear service provision including rehabilitation. Since the programme is also being implemented at the primary healthcare level, it envisages a reduction in the burden of deafness and prevention of future hearing loss in India.

Prevalence of Coronary Risk Factors in Type 2 Diabetics without Manifestations of Overt Coronary Heart Disease.

Aims : To find the prevalence of cardiovascular risk factors in type-2 diabetics without manifestations of overt coronary heart disease and to estimate the prevalence of silent myocardial ischaemia in these patients. Methods : Seventy seven patients of type 2 diabetes were recruited for the study (one patient lost after recruitment; 76 completed the study). History and physical examination were recorded. Laboratory investigations included fasting and 2-hour post-prandial blood sugar, blood urea, serum creatinine, lipid profile, glycated haemoglobin, and microalbuminuria. Ultrasonographic scanning of the carotid arteries was performed to measure the carotid IMT. For identification of cases of silent ischaemia, treadmill test (TMT) was performed. Results : The study group was divided into a non-CAD group (n=54), and a silent CAD group (n=22). Twenty-two diabetics were found to have silent CAD as evidenced by a positive TMT result (28.9%). The prevalence of silent myocardial ischaemia was almost similar in both males and females. Serum LDL levels more than 140 mg% had a significant correlation with the prevalence of silent CAD (p=0.04). The difference in CCA-IMT values was found to be statistically significant between the silent CAD and non-CAD groups (p=0.019). Conclusion : High LDL level and greater carotid intima-media thickness are particularly important parameters that can predict if a patient of type 2 diabetes is at risk for silent ischaemia. A high carotid IMT is a surrogate and reliable marker of higher risk of CAD amongst type 2 diabetic patients, even in those without overt CAD. The study also underlines the utility of carotid IMT as a simple, non-invasive, safe, and cheap screening test for the assessment of risk of CAD in type 2 diabetics. ©

Carotid intimomedial thickness in type 2 diabetic patients and its correlation with coronary risk factors.

AIMS: To compare the carotid intimal-medial thickness in type 2 diabetics with and without coronary artery disease (CAD), and to correlate the intimal-medial thickness (IMT) with known coronary risk factors. METHODS: One hundred and eleven patients of type 2 diabetes were recruited for the study. History and physical examination were recorded. Laboratory investigations included fasting and 2-hour post-prandial blood sugar, blood urea, serum creatinine, lipid profile, glycated haemoglobin, and microalbuminuria. Ultrasonographic scanning of the carotid arteries was performed to measure the carotid IMT. For identification of cases of silent ischaemia, treadmill test (TMT) was performed. RESULTS: The study group was divided into a non-CAD group (n=40), and a CAD group (n=71). The mean carotid IMT of the group as a whole, was 0.840 +/- 0.2 mm. The mean carotid IMT was significantly higher (p<0.0001) in type 2 diabetics with CAD (both overt and silent) than in those without CAD. In diabetics with CAD, the systolic blood pressure, diastolic blood pressure and triglycerides were found to be predictors of high mean carotid IMT. On subgroup analysis of the cases with silent ischaemia, the variables affecting carotid IMT were serum creatinine, total cholesterol, microalbuminuria/proteinuria, serum triglyceride levels, and diastolic blood pressure. CONCLUSION: A high carotid IMT is a surrogate and reliable marker of higher risk of CAD amongst type 2 diabetic patients, even in those without overt CAD. The study underlines the utility of carotid IMT as a simple, non-invasive, safe, and cheap screening test for the assessment of risk/prognosis of CAD in type 2 diabetics. We have also demonstrated the usefulness of measuring IMT, as a means to detect silent CAD among type 2 diabetics.

Clear cell myoepithelioma of the hard palate

Salivary gland myoepitheliomas are rare tumours. We report the case of a 40-year-old female who presented to us with a hard palatal mass. The tumour was excised with a healthy rim of surrounding tissue. Histological and immunohistochemical analyses revealed the tumour to be a myoepithelioma of the clear cell variety.

Pneumatization of Mastoid Air Cells: Role of Acquired Factors / Neumatización de las Células Mastoideas: Rol de Factores Adquiridos

The exact mechanism of the pneumatization of the mastoid air cell system and the factors influencing the pneumatization are poorly understood. Both genetic as well as acquired factors have been implicated to influence this pneumatization process. Since pneumatization of the mastoid air cell system is considered an important prognostic factor in the outcome of reconstructive ear surgeries, a cohort study was carried out to assess the role of two important acquired factors i.e. duration of chronic otitis media and auditory tube functional status on the pneumatization of mastoid air cells. 50 individuals with unilateral chronic otitis media underwent assessment of their mastoid air cell system using planimetry and auditory tube functions using flourescein dye nasopharyngoscopy. The results were in accordance with the previous studies indicating a definite influence of chronic middle ear disease on the pneumatization process, although the duration of disease was not significant. In contrast to some of the previous studies, no influence of auditory tube functional status was found on the mastoid pneumatization.

El mecanismo exacto de la neumatización del sistema de células mastoideas y los factores que influencian este proceso es pobremente comprendido. Factores genéticos, como también adquiridos, han sido implicados en el proceso de neumatización del sistema de células mastoideas. Esta neumatización es considerada un factor de importante pronóstico en la conducción de las cirugías reconstructivas de oído. Hemos llevado a cabo un estudio para explicar el rol de dos importantes factores adquiridos en la duración de las otitis media crónica y el status funcional de la tuba auditiva en la neumatización de las células mastoideas. En 50 individuos, con otitis media crónica unilateral, se estudió su sistema de células aeríferas mastoideas usando la planimetría y las funciones de la tuba auditiva usando fluorescina, vía nasofaringoscopía. Los resultados concuerdan con estudios previos, indicando una influencia definida de enfermedades crónicas del oído medio en el proceso de neumatización, aunque la duración de la enfermedad no fue significativa. En contraste con los estudios previos, no se encontró influencia del status funcional de la tuba auditiva en la neumatización mastoidea.

Extensive gas-forming infection of the deep neck and laryngeal spaces

We report the case of a 46-year-old diabetic male who presented to us with swelling in the neck associated with dysphagia and respiratory discomfort following dental infection. The CT-scan and soft tissue neck radiograms of the patient revealed an extensive head and neck abscess with gas pockets. Prompt surgical intervention with ntibiotic coverage and control of diabetes led to recovery of the patient. The absence in the literature of any previous report of a laryngeal gasforming abscess resulting from a primary dental infection prompted us to report this case

Extensive gas-forming infection of the deep neck and laryngeal spaces%Infecção formada por gás extenso na profundidade do pescoço e espaços laríngeos

We report the case of a 46-year-old diabetic male who presented to us with swelling in the neck associated with dysphagia and respiratory discomfort following dental infection. The CT-scan and soft tissue neck radiograms of the patient revealed an extensive head and neck abscess with gas pockets. Prompt surgical intervention with ntibiotic coverage and control of diabetes led to recovery of the patient. The absence in the literature of any previous report of a laryngeal gasforming abscess resulting from a primary dental infection prompted us to report this case

Tubercular pansinusitis presenting as a nasal polyp.

Tuberculosis of the paranasal sinuses is an uncommon presentation. We present a case of a 16 year old female who presented with a polypoidal nasal mass and symptoms consistent with chronic sinusitis. The computerized tomography scan of the paranasal sinuses was consistent with pansinusitis and sinonasal polyp. The polyp was excised and the histopathological evaluation of the surgically removed tissue revealed it to be tuberculous. The patient responded to four drug anti-tuberculous treatment.

Solitary tuberculous ulceration of Pinna: A rare presentation.

Tuberculosis of pinna is a rare presentation. We present a case of a 25 year-old woman who presented with a chronic, non-healing ulceration of pinna. On histopathological evaluation of the incisional biopsy from the lesion, she was found to have tuberculosis of pinna. She responded to 4-drug anti-tubercular treatment. To our knowledge, there has been no previous report of any similar case in the past.

Study of Sphenoid Sinus Anatomy in Relation to Endoscopic Surgery / Estudio Anatómico del Seno Esfenoidal en Relación con la Cirugía Endoscópica

For radical surgery of the past, the precise definition of the clefts of the paranasal sinuses was of limited significance. In the recent years, the transsphenoidal approach to the pituitary gland has come into practice and the study of the surgical anatomy and relationships of the sphenoid sinus has gained increased significance. To better define this anatomy, 20 sphenoid sinuses in cadavers were dissected to reveal important anatomical relationships, dimensions, volume and pneumatization of the sphenoid sinus. The results of the study are discussed with particular reference to the important surgical anatomical features of the sphenoid sinus. The results also point towards the possibility of racial variations in terms of relationships of the sphenoid sinus to internal carotid artery and optic nerve in the Indian population.

En las cirugías radicales del pasado, la definición precisa de las hendiduras de los senos paranasales fueron de importancia limitada. En los últimos años, el abordaje transesfenoidal de la hipófisis y las relaciones del seno esfenoidal han tenido en la práctica y en el estudio de la anatomía quirúrgica, un significativo incremento. Para definir mejor esta anatomía, 20 senos esfenoidales fueron disecados en cadáveres para revelar importantes relaciones anatómicas, dimensiones, volumen y neumatización del seno esfenoidal. Los resultados de este estudio son discutidos con referencia particular para la anatomía quirúrgica del seno esfenoidal. Los resultados también apuntan hacia la posibilidad de variaciones raciales, en términos de relaciones del seno esfenoidal con la arteria carótida interna y el nervio óptico, en la población de la India.

Ultrasonographic evaluation of gallbladder volume in diabetics.

AIMS AND OBJECTIVE: Ultrasonographic determination of gallbladder volume in diabetics [both type I and type 2], it's comparison with a control group, and correlation of gallbladder volume in diabetics with parameters such as age, sex, body mass index, parity, hyperlipidaemia, and autonomic neuropathy. MATERIALS AND METHOD: Ninety one cases of diabetes mellitus and 40 healthy controls were recruited for the study. A detailed history and physical examination were recorded. Laboratory investigations done were--fasting and postprandial blood sugar, glycosylated haemoglobin, microalbuminuria, and serum lipid profile. Autonomic neuropathy was determined by using simple non-invasive bedside tests. Fasting gallbladder volume was measured by ultrasonography (calculated by ellipsoid formula). RESULTS: The mean fasting gallbladder volume was 18.20 +/- 2.54 ml in type I diabetics and 25.87 +/- 13.90 ml in type 2 diabetics, with a minimum value of 9.30 ml and maximum value of 88 ml. When type 2 diabetics were subgrouped according to the presence of autonomic neuropathy, higher gallbladder volumes were seen in patients with autonomic neuropathy. CONCLUSIONS: Cholecystomegaly, to a significant degree, was found in type 2 diabetics in the present study. It was significantly correlated with age, body mass index, and the severity of autonomic neuropathy. In male type 2 diabetics, gallbladder volume was significantly correlated with LDL cholesterol levels. In female type 2 diabetics, gallbladder volume was significantly correlated with waist-hip ratio. Gallbladder volume also had significant correlation with proliferative diabetic retinopathy, but not with glycaemic control, microalbuminuria, hypertension, or the duration of diabetes.

Aetiology and clinical profile of osteomalacia in adolescent girls in northern India.

BACKGROUND: The adolescent age group is particularly prone to nutritional rickets/osteomalacia due to an increased demand for nutrients, especially calcium and vitamin D. Osteomalacia presents with non-specific signs and symptoms because of which diagnosis may be delayed. Vitamin D deficiency is unexpected in India, which is a tropical country with abundant sunshine. METHODS: We prospectively studied the clinical presentation, aetiology and social factors contributing to adolescent rickets/ osteomalacia in our region. RESULTS: We saw 21 symptomatic adolescents with osteomalacia during the study period (November 2000-July 2002). All were girls. Only 1 practised purda and 4 belonged to a low socioeconomic class. The mean (SD) duration of illness before correct diagnosis was 2.8 (2.1) years. Bone pains and muscular weakness were universally present. Non-specific complaints (especially limb pains being mistaken for joint involvement) led to a delay in diagnosis with consequent morbidity. All but 1 patient had low serum 25-hydroxyvitamin D levels (<10 ng/ml), with the mean (SD) being 4.9 (2.7) ng/ml. Their mean dietary calcium intake was low 1265 (199) mg/day, range 40-810 mg/day]. Restricted outdoor activities (n = 19) and the traditional dress code (n = 21) were contributory factors, as they led to poor exposure to sunshine. CONCLUSIONS: Nutritional osteomalacia among adolescents is a poorly recognized entity. Even in non-purda practising communities in the tropics, poor exposure to sunshine due to social factors, compounded by low dietary calcium intake, can lead to osteomalacia in adolescents.

Spectrum of congenital heart defects associated with Down Syndrome in high consanguineous Omani population.

OBJECTIVE: To identify the pattern of congenital heart defects (CHD) in children with Down Syndrome (DS) in an Omani population, and compare it with CHD in children without DS and with historical cohorts from populations with low prevalence of consanguinity. SETTING: Open-access Paediatric Echocardiography Clinic at Sultan Qaboos University Hospital, Muscat, Oman. DESIGN: Prospective study. METHODS: All children with DS referred to the Clinic from 1995-1998 formed the subjects (Group I). Children without DS or other known associations of CHD seen at the clinic during the same period served as controls (Group II). Two-dimensional echo-Doppler studies were performed on both groups and the results compared. RESULTS: CHD were detected in 54/90 (60%) children in Group I, compared to 698/2122 (32.9%) in Group II (P<0.001). The common CHD in Group I included secundum atrial septal defect (ASD; 18/54), atrioventricular septal defect (AVSD; 15/54) and ventricular septal defect (VSD; 14/54), and in Group II included ASD (175/698), VSD (175/698), patent ductus arteriosus (123/698), pulmonary stenosis (PS; 76/698) and AVSD (35/698). AVSD was more common (P <0.001) and PS less common (P = 0.03) in Group I. Aortic stenosis, coarctation of aorta, transposition of great arteries and complex heart diseases were not detected. Compared to several studies from populations with low prevalence of consanguineous marriages, our study showed a higher frequency of CHD in DS (P <0.05). CONCLUSION: A high frequency of CHD was documented in DS-children from a population with widely prevalent consanguinity. AVSD was most frequent in DS. An interesting observation was the relative rarity of some CHD in the DS population studied.