Clinico-Epidemiological Characteristics of Children with Cystic Fibrosis: A Tertiary Care Experience

Background: Cystic fibrosis (CF) is considered to be rare among individuals from Bangladesh. The objective of the study was to delineate the clinico-epidemiological characteristics of pediatric cystic fibrosis cases.Method: This observational study included pediatric patients (up to 14 years of age) with a clinical diagnosis of CF. Data were collected within the period from February 2021 and October 2021. Written informed consent was obtained from the accompanying parent. Clinical and epidemiological characteristics were analyzed on the basis of demographic data, medical history, laboratory tests, and outcome information. Data analysis was done with SPSS 26.Result: A total of 50 patients (66% male) with a mean age of 39.7 ±30.75 (SD) months were included. Twenty-eight patients (57.14%) had siblings with CF, and 41.67% of parents had a history of consanguineous marriage. The majority of them were stunted (86%) and underweight (86%), and half of them had wasting (54%). Median disease duration was 12 months (range: 2 – 72). Cough (100%) and purulent sputum (100%) were the predominant respiratory symptoms, while failure to thrive (98%) and bulky offensive stools (86%) were prime gastrointestinal symptoms. Among the signs, malnutrition (94%), short stature (72%), digital clubbing (64%), and bronchiectasis (40%) were most frequent. Pulmonary hypertension (48%, n=24) was the most common comorbidity identified in the study participants. In hospital, mortality was 16% (n=8). Digital clubbing, bronchiectasis, pancreatic insufficiency, and abnormal liver function tests were significantly higher in the patient who died.Conclusion: Children with cystic fibrosis most commonly present with undernutrition and respiratory symptoms. Failure to thrive was almost a global phenomenon. Pulmonary hypertension was the most common complication found in echocardiography.

Acute chest syndrome may not have an atherosclerotic background in sickle cell diseases

Background: We tried to understand whether or not there is a significant relationship between acute chest syndrome (ACS) and atherosclerosis in sickle cell diseases (SCD). Methods: All patients with the SCD were included. Results: The study included 434 patients (222 males) with similar mean ages in male and female genders (30.8 versus 30.3 years, respectively, p>0.05). Smoking (23.8% versus 6.1%, p<0.001) and alcohol (4.9% versus 0.4%, p<0.001) were higher in males, significantly. Transfused units of red blood cells (RBC) in their lives (48.1 versus 28.5, p=0.000) were also higher in males, significantly. Similarly, disseminated teeth losses (<20 teeth present) (5.4% versus 1.4%, p<0.001), chronic obstructive pulmonary disease (COPD) (25.2% versus 7.0%, p<0.001), ileus (7.2% versus 1.4%, p<0.001), cirrhosis (8.1% versus 1.8%, p<0.001), leg ulcers (19.8% versus 7.0%, p<0.001), digital clubbing (14.8% versus 6.6%, p<0.001), coronary heart disease (CHD) (18.0% versus 13.2%, p<0.05), chronic renal disease (CRD) (9.9% versus 6.1%, p<0.05), and stroke (12.1% versus 7.5%, p<0.05) were all higher in males but not ACS (2.7% versus 3.7%, p>0.05) in the SCD. Conclusion: SCD are severe inflammatory processes on vascular endothelium, particularly at the capillary level since the capillary system is the main distributor of hardened RBC into the tissues. Although the higher smoking and alcohol-like strong atherosclerotic risk factors and disseminated teeth losses, COPD, ileus, cirrhosis, leg ulcers, digital clubbing, CHD, CRD, and stroke-like obvious atherosclerotic consequences in male gender, ACS was not higher in them, significantly. In another definition, ACS may not have an atherosclerotic background in the SCD. Key words: Sickle cell diseases, chronic endothelial damage, atherosclerosis, acute chest syndrome, male gender, smoking, alcohol

Pachydermoperiostosis (PDP): A Case Report

Pachydermoperiostosis (PDP) is the primary form of hypertrophic osteoarthropathy which accounts for 5% of all cases of the disorder. It is a rare hereditary disorder that is associated with digital clubbing, polyarthritis, cutis verticis gyrata, Seborrhea, eyelid ptosis, and hyperhidrosis. In this case report, we discussed a case of an incomplete form of primary hypertrophic osteoarthropathy characterised by evidence of bone abnormalities without pachydermia. Keywords: Pachydermoperiostosis, hypertrophic osteoarthropathy, periostosis.

Digital Clubbing and Hodgkin Disease in Children: A Case Report and Review of Literature

Introduction: Digital clubbing (hypertrophic osteoarthropathy) as the initial presentation of lymphoma is rarely reported, particularly in children. In this study, we report a patient with intrathoracic Hodgkin Disease (HD) and digital clubbing as the first presentation, and we will review the literature regarding the same condition. Case Presentation: A 10-year-old boy presented with a 2-month history of cough, mild dyspnea, and night sweats, with prominent digital clubbing. A chest x-ray and a computed tomography scan of the chest showed multiple mediastinal masses. A mediastinal lymph node biopsy was done. Pathologic examination was indicative of nodular sclerosis HD. Conclusions: In patients with digital clubbing, intrathoracic malignancies should be considered a differential diagnosis and must be ruled out by precise examination and paraclinical help.

AVASCULAR NECROSIS MAY NOT HAVE AN ATHEROSCLEROTIC BACKGROUND IN SICKLE CELL DISEASES

Background: We tried to understand whether or not there is a significant association between avascular necrosis (AVN) and atherosclerosis in sickle cell diseases (SCD). Methods: All patients with the SCD were included. Results: The study included 434 patients (212 females) with similar mean ages in male and female genders (30.8 versus 30.3 years, respectively, p>0.05). Smoking (23.8% versus 6.1%, p<0.001) and alcohol (4.9% versus 0.4%, p<0.001) were higher in male gender, significantly. Transfused units of red blood cells (RBC) in their lives (48.1 versus 28.5, p=0.000) were also higher in male gender, significantly. Similarly, disseminated teeth losses (<20 teeth present) (5.4% versus 1.4%, p<0.001), chronic obstructive pulmonary disease (COPD) (25.2% versus 7.0%, p<0.001), ileus (7.2% versus 1.4%, p<0.001), cirrhosis (8.1% versus 1.8%, p<0.001), leg ulcers (19.8% versus 7.0%, p<0.001), digital clubbing (14.8% versus 6.6%, p<0.001), coronary heart disease (CHD) (18.0% versus 13.2%, p<0.05), chronic renal disease (CRD) (9.9% versus 6.1%, p<0.05), and stroke (12.1% versus 7.5%, p<0.05) were all higher in male gender but not AVN (24.3% versus 25.4%, p>0.05), significantly. Conclusion: SCD are severe inflammatory processes on vascular endothelium, particularly at the capillary level since the capillary system is the main distributor of hardened RBC into the tissues. Although the higher smoking and alcohol-like strong atherosclerotic risk factors and disseminated teeth losses, COPD, ileus, cirrhosis, leg ulcers, digital clubbing, CHD, CRD, and stroke-like obvious atherosclerotic consequences in male gender, AVN was not higher in them, significantly. In another definition, AVN may not have an atherosclerotic background in the SCD. Key words: Sickle cell diseases, chronic endothelial damage, atherosclerosis, avascular necrosis, male gender, smoking, alcohol

August 2021 - Volume 15, Issue 3 AUTOSPLENECTOMY MAY NOT HAVE AN ATHEROSCLEROTIC BACKGROUND IN SICKLE CELL DISEASES

Background: We tried to understand whether or not there is a significant relationship between autosplenectomy and atherosclerosis in sickle cell diseases (SCD). Methods: All patients with the SCD were included. Results: The study included 434 patients (222 males and 212 females) with similar mean ages in male and female genders (30.8 versus 30.3 years, respectively, p>0.05). Smoking (23.8% versus 6.1%, p<0.001) and alcohol (4.9% versus 0.4%, p<0.001) were higher in males, significantly. Transfused units of red blood cells (RBC) in their lives (48.1 versus 28.5, p=0.000) were also higher in males, significantly. Similarly, disseminated teeth losses (<20 teeth present) (5.4% versus 1.4%, p<0.001), chronic obstructive pulmonary disease (COPD) (25.2% versus 7.0%, p<0.001), ileus (7.2% versus 1.4%, p<0.001), cirrhosis (8.1% versus 1.8%, p<0.001), leg ulcers (19.8% versus 7.0%, p<0.001), digital clubbing (14.8% versus 6.6%, p<0.001), coronary heart disease (CHD) (18.0% versus 13.2%, p<0.05), chronic renal disease (CRD) (9.9% versus 6.1%, p<0.05), and stroke (12.1% versus 7.5%, p<0.05) were all higher in males but not autosplenectomy (50.4% versus 53.3%, p>0.05) in the SCD. Conclusion: SCD are severe inflammatory processes on vascular endothelium, particularly at the capillary level since the capillary system is the main distributor of hardened RBC into the tissues. Although the higher smoking and alcohol-like strong atherosclerotic risk factors and disseminated teeth losses, COPD, ileus, cirrhosis, leg ulcers, digital clubbing, CHD, CRD, and stroke-like obvious atherosclerotic consequences in male gender, autosplenectomy was not higher in them, significantly. In another definition, autosplenectomy may not have an atherosclerotic background in the SCD. Key words: Sickle cell diseases, chronic endothelial damage, atherosclerosis, autosplenectomy, male gender, smoking, alcohol

Atherosclerotic Background of Cirrhosis in Sickle Cell Patients

Background: We tried to understand the presence of any atherosclerotic background of cirrhosis in patients with sickle cell diseases (SCDs). Methods: The study was performed in the Hematology Service of the Mustafa Kemal University on SCDs patients between March 2007 and June 2012. Results: The study included 256 patients with SCDs (127 females). Their mean age was 29.3 years. Cirrhosis was detected in 5.8% (15) of the SCDs patients without any gender difference (6.2% of females versus 5.4% of males, p>0.05). There were 15 (5.8%) patients with chronic obstructive pulmonary disease with a highly significant male predominance (3.1% versus 8.5%, p<0.001). Digital clubbing and pulmonary hypertension were also higher in males, but the differences were nonsignificant in between (4.7% versus 6.2% and 11.0% versus 12.4%, respectively). Similarly, the leg ulcers were significantly higher in males, too (5.5% versus 16.2%, p<0.001). The significant male predominance was also observed in stroke and smoking (3.1% versus 6.2%, p<0.05 and 3.9% versus 11.6%, p<0.001, respectively). There were 14 (5.4%) mortal patients during the five-year follow-up period (6.2% of females and 4.6% of males, p>0.05), and the mean ages were 31.0 and 26.8 years, respectively (p>0.05). Conclusion: Probably cirrhosis is a systemic inflammatory process prominently affecting the hepatic vasculature, and an eventual accelerated atheroscerotic process is the main underlying cause of characteristics of the disease. SCDs are accelerated systemic atherosclerotic processes, too, and the higher prevalence of cirrhosis in SCDs patients may indicate the underlying atherosclerotic background of cirrhosis. Key words: Atherosclerosis, metabolic syndrome, cirrhosis, sickle cell diseases