Immunological Memory and Affinity Maturation After Vaccination in Patients With Propionic Acidemia.

Earlier studies have recommended routine childhood immunization in patients with propionic acidemia (PA); however, the literature presents insufficient data on the response to vaccines, notably specific IgG concentrations and avidity maturation, after measles, mumps, rubella (MMR), and diphtheria/tetanus (DiphtTe) vaccinations in this population. In patients with PA, cellular and humoral changes of the immune system (e.g. a decreased CD4+ T cell count, with a reversal of CD4/CD8 T cell ratio, a deficient gamma-globulin fraction, and in one case a decreased lymphocyte blastogenesis) have been reported. Former reports also detected pancytopenias accompanying febrile infections in PA patients. In the current study, we analyzed vaccine-specific IgG concentrations and avidity maturation after MMR and DiphtTe vaccinations in 10 patients with PA. Compared to gender and age matched controls, all 10 had protective IgG concentrations for at least one tested antigen, and in 6 out of 10 patients high relative avidity indices for measles and rubella were detected. In summary, the present study revealed a sufficient immune response and outcome, indicating an acceptable humoral memory in patients with PA after booster vaccinations.

Diagnosing lactose malabsorption in children: difficulties in interpreting hydrogen breath test results.

Lactose malabsorption (LM) is caused by insufficient enzymatic degradation of the disaccharide by intestinal lactase. Although hydrogen (H2) breath tests (HBTs) are routinely applied to diagnose LM, false-negative results are not uncommon. Thirty-two pediatric patients (19 females, 13 males) were included in this prospective study. After oral lactose administration (1 g kg(-1) bodyweight to a maximum of 25 g), breath H2 was measured by electrochemical detection. HBT was considered positive if H2 concentration exceeded an increase of ⩾20 ppm from baseline. In addition to H2, exhaled methane (CH4), blood glucose concentrations and clinical symptoms (flatulence, abdominal pain, diarrhea) were monitored. A positive HBT indicating LM was found in 12/32 (37.5%) patients. Only five (41.7%, 5/12) of these had clinical symptoms during HBT indicating lactose intolerance (LI). Decreased blood glucose concentration increments (⩽20 mg dL(-1) (⩽1.1 mmol L(-1))) were found in 3/5 of these patients. CH4 concentrations ⩾10 ppm at any time during the test were observed in 5/32 (15.6%) patients and in 9/32 (28.1%) between 1 ppm and 9 ppm above baseline after lactose ingestion. In patients with positive HBT 10/12 (83.3%) showed elevated CH4 (>1 ppm) above baseline in breath gas, whereas in patients with negative HBT this figure was only 4/17 (23.5%). In addition to determining H2 in exhaled air, documentation of clinical symptoms, measurement of blood glucose and breath CH4 concentrations may be helpful in deciding whether in a given case an HBT correctly identifies patients with clinically relevant LM.