Patients with CVID had a greater likelihood of developing lung disease, possibly due to delayed diagnosis and immune dysregulation, as compared with XLA patients. Early diagnosis of patients with primary antibody deficiencies and adequate i.v. immunoglobulin replacement therapy substantially reduces the number of pulmonary infections. However, CVID patients are prone to progression of lung disease despite optimal immunoglobulin therapy because of the nature of the disease. This important issue should be addressed in further studies.
Echocardiographic evaluation of right ventricular function and noninvasive estimation of pulmonary artery pressure could have an important diagnostic role in the follow-up and therapeutic management of patients with primary immune deficiency.
Background: There are several factors may have an impact on the prognosis of developmental dysplasia of the hip (DDH). They may change the outcome and treatment if they are present in a patient. Objectives: The objective of this study is to determine the association of those factors with the outcome. Patients and Methods: The study was performed on 74 infants with DDH in Children’s Medical Centre. After obtaining informed consent and ethical approval, patients with DDH confirmed by ultrasound were included, and possible risk factors including severity of DDH based on the Graf criteria, follow-up length, age, gender, laterality, type of delivery, amniotic fluid index, fetal anomalies, birth order of children, and methods of treatment were collected and compared against treatment response. Results: There were 58 female and 19 male patients with a mean age of 6.54 weeks. Bilateral DDH was found in 31 females and nine males. The average alpha and beta angles were 53.19 ± 5.7 and 66.53 ± 6.6 degree, respectively. The severity of DDH had a significant association with treatment response (P = 0.003). Recovery was better in females than in males (P = 0.031). In addition, the first‐born infants had a better response (P = 0.001). A meaningful connection (P = 0.01) was noted between the amniotic fluid index as well as renal/limb anomalies and treatment response. Type of delivery (cesarean versus vaginal) or fetal presentation had no significant association with treatment response (P > 0.05). Conclusion: Factors with a negative impact on DDH outcomes include the severity of DDH, male gender, higher birth order, oligohydramnios, renal and limb anomalies.
Objective: An estrogen-only-producing adrenal tumor is a rare etiology of isosexual precocious puberty (PP) in girls. Methods: We describe a 2.5-year-old girl who presented with signs and symptoms of isosexual PP. In primary laboratory and imaging investigations, serum estradiol level was found to be increased, while follicle-stimulating hormone, luteinizing hormone, adrenocorticotropic hormone, cortisol, testosterone, and 17-hydroxyprogesterone levels were shown to be within normal ranges. Abdominopelvic ultrasound and abdominal computed tomography revealed a right-sided adrenal mass, which was initially assumed to be an incidentaloma. Diagnosis of an adrenal cortical tumor was confirmed by tumor resection. Histologic examination revealed the tumor to be a benign adenoma with scattered areas of necrosis. Results: Tumor resection resulted in normalized serum estradiol and diminished clinical signs after 3 weeks. The child received no additional treatment and remains symptom free after 30 months of close observation. Conclusion: With the intent to spread the awareness of adrenocortical tumors as a potentially malignant cause of PP in children, and due to the rarity of an estrogen-producing adrenal mass, we discuss the clinical and biochemical features of our patient and a brief review of the literature.
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