Comparison of pneumococcal vaccination response in children with sickle cell disease: HbSS and HbSC.

INTRODUCTION: Sickle cell disease (SCD) children are at increased risk of invasive pneumococcal disease and rely on penicillin prophylaxis and vaccination for infection prevention. Post-vaccination antibody levels in SCD may wane overtime. HbSC are believed to have better immunological response than HbSS. OBJECTIVE: To compare antibody response to 23-valent pneumococcal polysaccharide vaccine (PPSV-23) between HbSS and HbSC. METHODS: Patients with HbSS (n=33) and HbSC (n=11), aged 7-18 years, were prospectively recruited. Luminex pneumococcal antibody levels were measured for 23-serotypes, after two PPSV-23 doses. RESULTS: Absolute median titer for 20 of the 23 serotypes was higher in HbSC than HbSS and significantly higher for serotypes 22 (3.9 vs. 1.6mcg/ml; p=0.039) and 43 (2.9 vs. 0.8mcg/ml; p=0.007). HbSC mounted a better immune anti-pneumococcal response compared to HbSS (≥1.3mcg/ml) for 18 of 23 serotypes, albeit not significant for any of the serotypes. More HbSC (64%) than HbSS (42%) were good vaccine responders (p=0.303). Two of 21 (10%) good vaccine responders and nine of 23 (39%) poor vaccine responders SCD participants subsequently developed acute chest syndrome or pneumonia (p=0.036). None of the HbSC patients developed ACS after receiving PPSV-23. HbSS poor vaccine responders were at increased future recurrence risk for ACS (p=0.003), pneumonia (p=0.036) or both (p=0.011), compared to good vaccine responders. CONCLUSION: HbSC possess better pneumococcal vaccine response than HbSS. Poor vaccine response is concerning for future acute pulmonary events. Current vaccination strategy for SCD sub-types are lacking, therefore further study to evaluate utility of vaccine boosters is necessary.

Severe Hepatopulmonary Syndrome in a Child with Caroli Syndrome.

Hepatopulmonary Syndrome (HPS) is a potential complication of chronic liver disease and is more commonly seen in the adult population. Caroli Syndrome is a rare inherited disorder characterized by intrahepatic ductal dilation and liver fibrosis that leads to portal hypertension. In children with liver disease, HPS should be considered in the differential diagnosis of prolonged, otherwise unexplained, hypoxemia. The presence of HPS can improve patient priority on the liver transplantation wait list, despite their Pediatric End-Stage Liver Disease (PELD) score. We present a 6-year-old girl with Caroli Syndrome and End-Stage Renal Disease who presented with persistent hypoxemia. The goal of this report is to increase awareness of HPS in children.

First report of a family with Lynch syndrome type II in Puerto Rico

Colorectal cancer (CRC) incidence in Puerto Rico has increased prodigiously since incidence figures were first recorded in 1950. Implications for hereditary nonpolyposis colorectal cancer (HNPCC) in concert with this increased CRC incidence are discussed. A family with the Amsterdam-positive criteria of the Lynch syndrome II variant, identified in the eastern area of Puerto Rico, is described. As far as we can determine, this is the first such report of this disorder in Puerto Rico

First report of a family with Lynch syndrome type II in Puerto Rico.

Colorectal cancer (CRC) incidence in Puerto Rico has increased prodigiously since incidence figures were first recorded in 1950. Implications for hereditary nonpolyposis colorectal cancer (HNPCC) in concert with this increased CRC incidence are discussed. A family with the Amsterdam-positive criteria of the Lynch syndrome II variant, identified in the eastern area of Puerto Rico, is described. As far as we can determine, this is the first such report of this disorder in Puerto Rico.

Hereditary colorectal carcinoma syndromes and their implications for colorectal carcinoma in Puerto Rico

During the last 41 years cancer of the colon in Puerto Rico has markedly increased as reported by the Puerto Rico Cancer Registry. The etiology of this increase remains elusive, but possibilities include an increase in reporting new cases, dietary modifications, exposure to carcinogens, as well as an hereditary predisposition. The importance of Hereditary Nonpolyposis Colorectal Cancer (HNPCC) in Puerto Rico, its molecular genetics, diagnostic criteria, and management will be discussed

Hereditary colorectal carcinoma syndromes and their implications for colorectal carcinoma in Puerto Rico.

During the last 41 years cancer of the colon in Puerto Rico has markedly increased as reported by the Puerto Rico Cancer Registry. The etiology of this increase remains elusive, but possibilities include an increase in reporting new cases, dietary modifications, exposure to carcinogens, as well as an hereditary predisposition. The importance of Hereditary Nonpolyposis Colorectal Cancer (HNPCC) in Puerto Rico, its molecular genetics, diagnostic criteria, and management will be discussed.

Prevalence of Helicobacter pylori at antral mucosa of patients with dyspepsia at University District Hospital

A total of 120 patients with dyspepsia were evaluated by endoscopy to determine the prevalence of Helicobacter pylori in the gastric antrum in 1988. It was found that duodenal ulcer and gastric ulcer were highly associated with this bacteria. Also statistically significant association between H.p. and active gastritis was observed

Prevalence of Helicobacter pylori at antral mucosa of patients with dyspepsia at University District Hospital.

A total of 120 patients with dyspepsia were evaluated by endoscopy to determine the prevalence of Helicobacter pylori in the gastric antrum in 1988. It was found that duodenal ulcer and gastric ulcer were highly associated with this bacteria. Also statistically significant association between H.p. and active gastritis was observed.