The overwhelmed intestine syndrome

We studied 54 patients who, after small intestinal resection, developed a massive protracted diarrhea with a daily fecal loss greater than 2 kg, status we defined as the "overwhelmed intestine syndrome" (OIS). Median length of residual small bowel was 120 cm, 19 patients had a definitive stoma (jejunostomy, n = 9; colostomy, n = 10), 26 patients had a provisional jejunostomy. Fecal weight greater than 2 kg was related to enteral hyperalimentation (greater than 3,500 Kcal) in 19 patients (induced OIS) and was clearly independent in 16 others who had fecal weight over 3 kg while receiving approximately 2,000 Kcal (obligatory OIS); the last 19 patients had fecal weight between 2 and 3 kg during normoalimentation. Hypocalcemia and hypomagnesemia were common in the three groups. The other complications were seen mostly in patients with obligatory OIS: in those patients, parenteral nutrition was maintained in 9 cases out of 16 (vs. 0 in other groups), nutritional gain was scanty, sodium equilibrium was difficult to obtain in spite of a large sodium intake (380 mmol/day), hospitalization lasted several months and autonomy via the enteral route could not be achieved in 7 out of the 9 patients with definitive short bowel (vs. 0 in other groups). This study shows that the OIS is an unique functional entity. Complications and prognosis are dependent on the obligatory or induced pattern of the syndrome. Only patients with obligatory OIS require definitive home parenteral nutrition.     

A 13-year longitudinal analysis of risk factors and clinic visitation patterns of patients with repeated gonorrhea

A retrospective study of 2498 patients who made multiple visits to a sexually transmitted disease clinic over a 13-year period analyzed risk factors, default patterns, and repeated infections associated with gonococcal urethritis. An analysis of visitation patterns found that being young, black, and male and having a history of gonococcal urethritis before visiting the clinic was positively related to the total time a patient remained involved with the clinic. Default rates for all patients increased with successive clinic visits. A focused analysis was carried out on the records of 146 patients who returned to the clinic within 12 months with a second diagnosis of gonococcal urethritis. It was found that this group of "repeaters," who comprised 15% of the total population with gonococcal urethritis, accounted for approximately 29% of all diagnoses of this infection over the 13-year study period. Repeaters were found to be more frequently male, black, single, and to be less likely to return for a test-of-cure culture. Longitudinal analysis found that the median time repeaters remained involved with the clinic was approximately 130 days. The relatively brief clinic "half-life" and rapid rates of removal of repeaters are discussed in terms of the development of strain-specific immunity to Neisseria gonorrhoeae in a closed population of patients.     

A study on OX39, a murine anti-rat interleukin 2 receptor antibody

OX39, a murine IgG1 monoclonal antibody (MoAb) that recognizes the 55 kDa alpha chain of the rat interleukin 2 receptor (R-IL2), was studied in vitro for its ability to interfere with IL2 binding and IL2-induced proliferation on rat concanavalin A (ConA) blasts and in vivo in a model of rat heart allografts. In vitro studies indicated that OX39 MoAb interacts with a single class of sites on the alpha chain of the rat R-IL2 with a high affinity (K_D=0.8 nm) and competes with IL2 binding on this chain (K_I=0.53 nm). In contrast, OX39 MoAb was found to be 10–20 times less efficient in competing with IL2 binding to the high-affinity R-IL2 (K_I10 nm). It is proposed that the epitope recognized by OX39 on the alpha chain (low-affinity R-IL2) is modified on (or buried in) the high-affinity R-IL2 configuration. Accordingly, OX39 was found to be a weak inhibitor in vitro on IL2-induced proliferation and in vivo on allograft rejection. Allograft survival was unaffected by doses of OX39 of 20 and 50 g/rat for 9 days; only a borderline effect was noted when doses as high as 250 g/rat were used. A significant, but restricted, effect of OX39 could be further detected when combined with low doses of cyclosporine A (1.5 mg/kg), which were ineffective by themselves. Together, our data suggest that in order to be efficient in vivo, anti-R-IL2 MoAbs must bind with high affinity to epitopes involved in the high-affinity IL2 binding site.     

Solitary plasmacytoma of the spine. Long-term clinical course

The data for 19 patients with solitary plasmacytoma of the spine were reviewed with regard to clinical course and prognosis (median follow-up, 96 months). Eight patients presented with spinal cord compression. A monoclonal immunoglobulin was initially detected in seven of 15 evaluable patients. Treatment included radiotherapy (18 of 19) and/or surgery (11 of 19) and chemotherapy (eight of 19). Spinal cord compression was reversed in every patient. The expected survival rate was 85% at 10 years after diagnosis. Local recurrence or dissemination was observed in 13 patients. It occurred within 5 years of diagnosis in 11 patients and was localized (that is, local recurrence or single bone metastasis) in eight patients. It was always associated with the appearance or an increase of the M component. Dissemination frequently had a "metastatic" pattern with no diffuse bone marrow plasmacytosis. The incidence of local recurrence (five patients) and leukemia (four patients) was high. Local recurrence and/or dissemination were significantly more frequent in patients with the M component at diagnosis than in those without it (P less than 0.05; relative risk, R = 4). The effectiveness of surgery and chemotherapy combined with radiotherapy is also discussed.     

Spectrum of NSD1 mutations in Sotos and Weaver syndromes

Interestingly, mental retardation was consistently more severe in patients with NSD1 deletions. Macrocephaly and facial gestalt but not overgrowth and advanced bone age were consistently observed in Sotos syndrome patients. We suggest therefore considering macrocephaly and facial gestalt as mandatory criteria for the diagnosis of Sotos syndrome and overgrowth and advanced bone age as minor criteria.     

A severe form of amyloidotic polyneuropathy in a Costa Rican family with a rare transthyretin mutation (Glu54Lys)

Four affected siblings in a Costa Rican family presented an aggressive polyneuropathy with widespread involvement of many visceral organs and onset during the third decade of life with rapid loss of muscle mass in the lower limbs and severe dysautonomy. The medical histories include vitreous opacity, cardiac enlargement, dermal and gastrointestinal infiltration, and autonomic dysfunction including circulatory compromise and gastrointestinal disturbances. Histological studies using Congo red stain and immunohistochemical assays with antibodies against the transthyretin (TTR) protein showed widespread deposition of amyloid in extracellular areas, including dermis and gastrointestinal lamina propia, endo- and perineural spaces, and vascular walls. A mutation search in the transthyretin (ttr) gene was performed seeking the cause of this severe form of familial amyloidotic polyneuropathy (FAP). We applied single-stranded conformational polymorphism (SSCP)-analyses followed by sequencing of the four exons of the ttr gene, revealing a point mutation in exon 3, a G to A transition that causes a Glu54Lys codon change. Western blots of plasma proteins incubated with anti-transthyretin antibodies after gel electrophoresis provided separation of wild-type and mutant TTR protein in affected family members.     

Serological studies of monoclonal RH1(D) antibodies with RH1(D) variants

In this paper we chose to emphasize three aspects of our work. First we underlined that “low grade and high grade” D weak red blood cells studied at the DNA level could, when monoclonal antibodies were used, give patterns of positive and negative reactions like partial RH1(D) cells. Secondly, we showed the importance of the technical conditions of the study which are essential for establishing a pattern of reactivity defining an epitope. It appears that the use of papain treated cells at room temperature can be misleading for the definition of epitope especially with IgM antibodies. Lastly we pointed out the interest of Rh variant cells, defined at the gene level, to study the expression of RH1(D) epitopes on the external part of the membrane.     

Pathology of inverted Takotsubo cardiomyopathy

Myocardial dysfunction without coronary involvement may occur in acute cerebral diseases. The inverted Takotsubo pattern has been recently recognized as a novel heart neurologic stress-related syndrome. We report on the case of a 40-year-old woman presenting with massive subarachnoid hemorrhage and brain death. Echocardiography revealed an extensive left ventricular circumferential akinesis except at the apex. Histologic analysis of the heart confirmed the absence of myocardial infarction and revealed only sparse foci of myocyte necrosis with contraction bands in the akinetic areas.