Pulmonary Radiological Manifestations of Humoral and Combined Immunodeficiencies in a Tertiary Pediatric Center.

Respiratory diseases are considered as significant causes of morbidity and mortality in primary immunodeficiencies. This study aimed to reveal the radiologic patterns of thoracic involvement in these disorders. A total of 58 patients, including 38 cases with combined cellular-humoral and 20 cases with humoral immunodeficiencies, were enrolled in this study. The "combined" group consisted of 12 cases with severe combined immunodeficiency (SCID) and 26 cases with combined immunodeficiency. The "humoral" group included seven patients with Hyper IgM syndrome (HIGMs), seven cases with common variable immunodeficiency (CVID), three patients with X-linked agammaglobulinemia, and three patients with other types of humoral primary immunodeficiencies (PIDs). The mean age of patients at the time of evaluation was 3.3±3.8 and 5.3±3.9 years in combined and humoral groups, respectively. The findings of chest X-rays and CT scans were interpreted and compared. There was a significant difference for alveolar opacification between combined and humoral immunodeficiencies (58% vs. 30%). The bronchopneumonia-like pattern was detected as a significant finding in patients with SCID (42%) and HIGMs (43%). Atrophy of the thymus was detected significantly often in cases of SCID (67%). Two patients with CVID and lipopolysaccharide-responsive and beige-like anchor protein deficiency showed parenchymal changes of granulomatous lymphocytic interstitial lung disease. No significant difference was detected for bronchiectasis, bronchitis/bronchiolitis patterns, pleural effusion, and thoracic lymphadenopathy. Distinct subtypes of primary immunodeficiency may provoke differing and comparable radiological patterns of thoracic involvement; which can clue the clinician and radiologist to the diagnosis of the disease.

Prognostic values of fluctuations in serum levels of alanine transaminase in inactive carrier state of HBV infection.

BACKGROUND: Current guidelines introduce periodic monitoring of serum alanine transaminase (ALT) as the first-line modality in follow-up patients, with a hepatitis B virus (HBV) inactive carrier state. OBJECTIVES: This study aimed to determine the incidence rate and patterns of ALT fluctuations and prognostic values for the development of chronic HBV e antigen (HBeAg)-negative hepatitis B (CHB), HBV surface antigen (HBsAg) seroclearance, and liver-related complications. PATIENTS AND METHODS: Treatment-naïve patients with a chronic HBV infection, HBeAg(-)/HBeAb(+), normal ALT levels, and HBV DNA < 2000 IU/mL, were followed-up every 6-12 months by assessing serum ALT levels. Serum HBV DNA was measured in cases of elevated ALT levels. RESULTS: A total of 399 patients were followed-up for 8.9 years; ALT > upper limit of normal (ULN, i.e. 40 IU/L) was detected in 103 (25.8%) patients, with an annual incidence rate of 2.9%. ALT elevation was associated with; male gender, age, and higher serum ALT levels at study entry. Among the cases of ALT elevations, 16 (15.5%) patients had ALT levels > 2 × ULN. There were 38 (36.9%) patients who had ALT levels that remained > ULN over six months, and 21 (20.4%) patients experienced at least two episodes of ALT elevations. In 15 (14.6%) patients, elevated ALT levels were associated with increased HBV replication (i.e. HBV DNA > 2 000 IU/mL) and these were considered as CHB. However, elevation of ALT levels, even in the absence of HBV replication, increased the risk for the development of CHB up to 8-fold in prospective follow-ups. HBsAg seroclearance, cirrhosis, and hepatocellular carcinoma were detected in 43 (10.8%), 4 (1%), and 1 (0.25%) patients, respectively. CONCLUSIONS: Fluctuations in serum ALT levels may change the prognosis of a HBV inactive carrier state.

Shunt-type and stop-type varicocele in adolescents: prognostic value of these two different hemodynamic patterns.

OBJECTIVE: To investigate different varicocele hemodynamic patterns (shunt type vs. stop type) as predictors of new-onset testicular hypotrophy or recurrence after varicocelectomy. DESIGN: Prospective clinical study. SETTING: Department of pediatric urology, children's hospital medical center. PATIENT(S): Seventy-four children and adolescents with varicocele. INTERVENTION(S): Based on ultrasound findings, patients were classified into shunt-type or stop-type varicocele groups. All patients with stop-type varicocele and testicular volume discrepancy of ≥20% underwent retroperitoneal varicocelectomy and internal spermatic vein ligation. Patients with shunt-type varicocele and ≥20% testicular asymmetry were randomly divided to undergo either retroperitoneal varicocelectomy (ligation of internal spermatic vein) or inguinal varicocelectomy with ligation of both internal and external spermatic veins. Patients with testicular volume discrepancy of <20% were put on conservative management. MAIN OUTCOME MEASURE(S): Occurrence of new-onset asymmetry among untreated patients and recurrence rate among operated patients. RESULT(S): Patients with shunt-type varicocele who did not undergo surgery revealed a higher risk of developing asymmetry during follow-up (odds ratio 10.5). Ligation of both internal and external spermatic veins in shunt-type patients was associated with significantly decreased rate of recurrence (6.3%) compared with retroperitoneal approach (30.8%). CONCLUSION(S): Shunt-type varicocele is associated with a higher risk of testicular hypotrophy among untreated patients. In addition, recurrence rate in shunt-type patients who underwent inguinal varicocelectomy was lower compared with retroperitoneal approach.