This chapter focuses on biochemical and sensory aspects, with data on the microbiology of cheeses. It defines sensory perceptions as the odor perceived by the nose, with no introduction of the food into the mouth, while flavor is the perception of the food during mastication either retronasaly or by the tongue. It summarizes the differences between the flavor and odor attributes reported for R, P or MF cheeses. Cheese is a biochemically dynamic product and undergoes significant changes during ripening. Proteins, carbohydrates, and fat are metabolized by both microbial activities, and by the action of indigenous milk enzymes and residual coagulant. Proteolysis has a direct and indirect role in the formation of texture and flavor of cheeses. Other biochemical reactions such as lipolysis, metabolism of residual lactose, lactate and citrate, and the formation of volatile compounds are also extremely important in the development of flavor compounds. Further, Raw milk cheeses ripen faster than cheeses made from milk, the microflora of which has been removed. Therefore, R cheeses tend to develop a stronger odor/flavor at the same age than those made from P or MF milk. This has been observed in all types of cheese studied: Cheddar, Manchego, Raclette, other hard and semi-hard cheeses, Bergkase, Swiss-type cheeses and soft goats' milk cheese. In all cases, this phenomenon seems to be directly linked to the activity of the indigenous microflora of the milk.

Condensed tannins (CT) may affect ruminal biohydrogenation of dietary polyunsaturated fatty acids. A feeding experiment was conducted with 24 Holstein cows to evaluate whether diets containing CT from different forage legumes can increase polyunsaturated fatty acids, especially n-3 fatty acid content in milk and cheese, without affecting negatively their physicochemical and sensorial properties. Cows were assigned to 4 treatment groups (n=6) for 52 d, divided into 2 periods: a control period (CoP) and an experimental period (ExP). During the CoP, cows received a basal diet composed of hay, corn silage, ExtruLin (Trinova Handel & Marketing AG, Wangen, Switzerland), concentrate, and alfalfa (AF) in a ratio of 45:25:5:7:18. In the ExP, in 3 of the 4 groups AF was replaced by either sainfoin (SF; 19% CT in dry matter) or 1 of 2 cultivars of birdsfoot trefoil [Polom (BP), 3% CT; Bull (BB), 5% CT]. At the end of each period, milk was collected on 3 consecutive days and analyzed for milk gross composition and fatty acid profile and was processed to Gruyère-type cheese. A trained panel assessed the sensory quality of raw milk and cheese using discriminative and descriptive tests. This experimental design consisting of AF in both the CoP and ExP allowed us to quantify effects due to lactation stage and experimental diets. In both the CoP and ExP, dry matter intake and milk yield did not differ among treatment groups. From the CoP to the ExP, milk urea content was reduced by 23% with SF, remained unchanged with BP, and tended to increase with AF and BB. The odor of the raw BB milk was judged to be different from AF milk. With SF, switching from the CoP to the ExP resulted in a 17% increase of the 18:3n-3 proportion in milk and cheese lipids. In BP cheese, the increase was 3%, whereas it tended to decrease in BB cheese. Additionally, the 20:5n-3 and 22:5n-3 proportions tended to increase in SF cheese from the CoP to the ExP. Compared with the AF cheeses, cheeses from cows fed CT-containing legumes were judged harder and tended to be less adhesive to the palate. In addition, SF and BP cheeses had less rind. In conclusion, feeding SF compared with BB and BP increased the content of 18:3n-3 in the milk and the cheese without a negative effect on flavor of the cheese. Despite a similar CT content, the 2 birdsfoot trefoil cultivars had opposite effects on milk urea and 18:3n-3 deposition, suggesting that, besides the content, the chemical structure may have had an important effect on the CT efficacy.

Eye formation is an important quality parameter in the dairy industry for (semi-)hard cheeses in Switzerland. To monitor the formation of eyes in cheese, radiography and, more recently, computed tomography (CT) technology, are employed. In the present study, two quality indicators (eye number and total eye volume) are calculated from radiographs by two recent procedures and compared with the results from CT data. The radiograph analysis procedures underestimate both quantifiers systematically and the deviation increases with the number of eyes. In contrast to radiographic data, the distribution of the eye volumes and positions as well as their geometric shape can be calculated from the CT data. These data allowed the observation that the eyes in the considered cheeses accumulate in the centre of the cheese, are predominately of spherical shape and non-spherical eyes can be extracted by a simple geometrical criterion. These findings illustrate the advantages of the CT technology.

The angiotensin-converting enzyme (ACE) inhibitory activity and the concentration of the 2 ACE-inhibiting tripeptides Val-Pro-Pro (VPP) and Ile-Pro-Pro (IPP) were studied during cheese ripening in 7 Swiss cheese varieties. The semi-hard cheeses Tilsiter, Appenzeller 1/4 fat, Tête de Moine, and Vacherin fribourgeois and the extra-hard and hard cheeses Berner Hobelkäse, Le Gruyère, and Emmentaler were investigated. Three loaves of each variety manufactured in different cheese factories were purchased at the beginning of commercial ripeness and investigated at constant intervals until the end of the usual sale period. Good agreement was found between ACE-inhibitory activity and the total concentration of VPP and IPP at advanced ripening stages. In most of the investigated varieties ACE-inhibitory activity and the concentration of the 2 tripeptides initially increased during the study period. A decline in the concentration of VPP and IPP was obtained toward the end of the investigated period for Tilsiter and Gruyère. The ratio of VPP/IPP decreased during ripening in all varieties with the exception of Emmentaler. However, large variations were observed among the cheese varieties as well as the individual loaves of the same variety. Chemical characterization of the investigated cheeses revealed that qualitative differences in the proteolysis pattern, not quantitative differences in the degree of proteolysis, are responsible for the observed variations in the concentrations of VPP and IPP. The presence of Lactobacillus helveticus in the starter culture was associated with elevated concentrations of VPP and IPP. The results of the present study show that concentrations of VPP and IPP above 100 mg/kg are attainable in semi-hard cheese varieties after ripening periods of about 4 to 7 mo and that stable concentrations of the 2 antihypertensive tripeptides can be expected over several weeks of cheese ripening.

The contents of the 2 antihypertensive peptides Val-Pro-Pro (VPP) and Ile-Pro-Pro (IPP) were determined in 101 samples from 10 different Swiss cheese varieties using HPLC with subsequent triple mass spectrometry. In the category of extra hard and hard cheeses, the Protected Denomination of Origin cheeses Berner Alpkäse and Berner Hobelkäse, L’Etivaz à rebibes, Le Gruyère, Sbrinz, Emmentaler (organic and conventional) and in the category of semihard cheeses, the varieties Tilsiter, Appenzeller 14 fat and full fat, Tête de Moine, and Vacherin fribourgeois were screened in the study. The average concentration of the sum of VPP and IPP in the screened cheese varieties varied to a large extent, and substantial variations were obtained for individual samples within the cheese varieties. The lowest average concentration of the 2 tri-petides was found in L’Etivaz à rebibes (n = 3) at 19.1mg/kg, whereas Appenzeller 14 fat (n = 4) contained the greatest concentration at 182.2mg/kg. In individual samples, the total concentration of VPP and IPP varied between 1.6 and 424.5mg/kg. With the exception of a 10-yr-old cheese, VPP was always present at greater concentrations than IPP. Milk pretreatment, cultures, scalding conditions, and ripening time were identified as the key factors influencing the concentration of these 2 naturally occurring bioactive peptides in cheese. The results of the present study show that various traditional cheese varieties contain, on average, similar concentrations of the 2 antihypertensive peptides to the recently developed fermented milk products with blood pressure–lowering property. This may serve as a basis for the development of a functional cheese with blood pressure–lowering property.

To theEditor:We readwith interest the report ofBurkitt lymphoma in a child with Joubert syndrome [1], which highlights the observation that patients with congenital malformations may be at increased risk of developing malignances [2,3]. We recently cared for a 5-year-old boy with osteogenesis imperfecta who also developed a Burkitt lymphoma. The family history was significant for a father and two older siblings with a known diagnosis of osteogenesis imperfecta type I. The patient had sustained only one fracture at age 3, involving the left radius and ulna. He presented with progressive abdominal distention, tachypnea, and shortness of breath. Chest Xray showed bilateral pleural effusions, and a computed tomography scan revealed soft tissue masses at the right cardiophrenic angle and at the right postero-lateral aspect of the thoraco-abdominal junction. There was a large amount of ascites throughout the abdomen with thickened areas of peritoneum and confluent lymph node enlargement. Malignant fluid removed from a thoracentesis and paracentesis revealed a clonal B cell population expressing CD10 (dim), CD19, CD20, FMC7, and lambda light chains, consistent with aggressive B cell lymphoma. Histologically, the fluid sample revealed markedly atypical cells with prominent nucleoli and darkly basophilic cytoplasm with very prominent cytoplasmic vacuolization. Cytogenetic analysis revealed a t(8;14) translocation. There was no bone marrow or central nervous system involvement. The patient was diagnosed with stage III Burkitt lymphoma and was treated according to Children’s Cancer Group protocol no. 5961, regimen B1. Induction chemotherapy was complicated by profound abdominal ascites and mild respiratory compromise which required several thoracenteses and paracenteses. He tolerated the remainder of his 4 months chemotherapy course without problems. He remains in clinical remission 2 years following diagnosis. Since the diagnosis of Burkitt lymphoma, he has sustained several additional fractures secondary to trauma: bilateral mandible fractures, T5 and L2 compression fractures, and a T9 burst fracture. Osteogenesis imperfecta is a rare congenital connective tissue disorder with considerable phenotypic variability. Prognosis ultimately depends on the degree of severity. The incidence of fractures varies widelywith age [4], level of activity, and success of surgical interventions [5]. There is no known increased risk for developing a malignancy in children with osteogenesis imperfecta. Rare pediatric patients with osteogenesis imperfecta have developed osteosarcoma [6] or familial leukemia [7]. There have also been case reports of adults with osteogenesis imperfecta who have developed osteosarcoma [8], breast carcinoma [9], ovarian serous carcinoma [10], andmultiple myeloma [11]. Osteogenesis imperfecta is associated with deletion of ormutation in the collagen type I, alpha-1 or alpha-2 genes (COL1A1 or COL1A2) [12,13], located at chromosomes 17q21.31-q22 and 7q22.1, respectively. Burkitt lymphoma results from chromosome translocations that involve the c-MYC oncogene (chromosome 8q24) and regulatory regions of immunoglobulin (Ig) heavy chains (14q32), kappa (2p12), or lambda light chains (22q11) [14], resulting in t(8;14), t(8;2), and t(8;22). Since there have not been anyprevious reports of Burkitt lymphoma in osteogenesis imperfecta patients, it is likely that this association is a randomoccurrence. However, since collagen is a key component of the hematopoietic microenvironment, it is possible that alteration of the extracellular matrix could have perturbed hematopoiesis and contributed to malignant transformation. From a clinical perspective, our patient’s underlying condition did not affect his ability to tolerate and respond to chemotherapy. Should future cases arise, this knowledge may be of interest.

Purpose: Total body irradiation (TBI) as part of a conditioning regimen before hematopoietic stem cell transplant (HSCT) is an important component in the management of acute lymphoblastic leukemia (ALL) that has relapsed or has other certain high-risk features. Controversy exists, however, as to whether a cranial boost in addition to TBI is necessary to prevent central nervous system (CNS) recurrences in these high-risk cases. Previous national trials have included a cranial boost in the absence of data to justify its use. Therefore, the aim of this study was to assess risk of CNS recurrence in ALL patients treated with TBI, to identify subsets of these high-risk patients at an increased or decreased risk of CNS recurrence after TBI, and to investigate whether regimens with higher doses of cranial irradiation further reduce the risk of CNS recurrence.Methods and Materials: Charts of 67 consecutively treated patients with ALL who received TBI before HSCT were reviewed. Data including patient demographics, clinical features at presentation, conditioning regimen, donor source, use of a cranial boost, remission stage at transplant, histologic subtype, cytogenetics, and extramedullary site of presentation were retrospectively collected and correlated with the risk of subsequent CNS recurrence.Results: At the time of analysis, 30 (45%) patients were alive with no evidence of disease, 8 (12%) were alive with recurrence of leukemia, 7 (10.5%) had recurrent ALL but with successful salvage, 7 (11%) died subsequent to recurrence, 14 (21%) died from complications related to HCST, and I patient was lost to follow-up (1.5%). Of the patients who recurred after HSCT, the relapses were hematologic in 13 (57%), CNS with or without simultaneous marrow involvement in 3 (13%), and other sites in 7 (30%). Forty-one (61%) patients did not receive an extracranial boost of irradiation with TBI. Two of these patients (4.9%) suffered CNS failures compared with 1 of 26 (3.8%) who received a cranial boost (p = 0.84). None of the 40 patients who presented only with hematologic disease developed a CNS recurrence despite the fact that only 13 of 40 of these patients received a cranial boost after TBI. Cranial boost was therefore not associated with a reduction in CNS recurrence, especially in patients with only hematologic disease at presentation for which there were no failures regardless of the use of additional cranial radiotherapy.Conclusions: Patients who present with hematologic disease only at the time of HSCT have a low risk of CNS recurrence after TBI regardless of the use of a cranial boost, suggesting that a cranial boost may not be necessary in these patients. (c) 2005 Elsevier Inc.

An infant who is brought to an emergency department with vague symptoms poses a difficult problem for the pediatrician. The presence of specific findings on laboratory screening tests often helps to narrow the differential diagnosis, enabling appropriate treatment. Unexpected laboratory results, however, may lead the physician astray in making the correct diagnosis. It is important to interpret laboratory results within the context of the entire clinical picture and to keep in mind the limitations of certain of these tests. We report a case of apparent proteinuria in a 7-week-old infant caused by the addition of baking soda to his formula, and point out that careful history-taking may help to avoid an extensive diagnostic workup.