Clinical features of Candidiasis in patients with inherited interleukin 12 receptor ß1 deficiency.

BACKGROUND: Interleukin 12Rß1 (IL-12Rß1)-deficient patients are prone to clinical disease caused by mycobacteria, Salmonella, and other intramacrophagic pathogens, probably because of impaired interleukin 12-dependent interferon γ production. About 25% of patients also display mucocutaneous candidiasis, probably owing to impaired interleukin 23-dependent interleukin 17 immunity. The clinical features and outcome of candidiasis in these patients have not been described before, to our knowledge. We report here the clinical signs of candidiasis in 35 patients with IL-12Rß1 deficiency. RESULTS: Most (n = 71) of the 76 episodes of candidiasis were mucocutaneous. Isolated oropharyngeal candidiasis (OPC) was the most common presentation (59 episodes, 34 patients) and was recurrent or persistent in 26 patients. Esophageal candidiasis (n = 7) was associated with proven OPC in 2 episodes, and cutaneous candidiasis (n = 2) with OPC in 1 patient, whereas isolated vulvovaginal candidiasis (VVC; n = 3) was not. Five episodes of proven invasive candidiasis were documented in 4 patients; 1 of these episodes was community acquired in the absence of any other comorbid condition. The first episode of candidiasis occurred earlier in life (median age±standard deviation, 1.5 ± 7.87 years) than infections with environmental mycobacteria (4.29 ± 11.9 years), Mycobacterium tuberculosis (4 ± 3.12 years), or Salmonella species (4.58 ± 4.17 years) or other rare infections (3 ± 11.67 years). Candidiasis was the first documented infection in 19 of the 35 patients, despite the vaccination of 10 of these 19 patients with live bacille Calmette-Guérin. CONCLUSIONS: Patients who are deficient in IL-12Rß1 may have candidiasis, usually mucocutaneous, which is frequently recurrent or persistent. Candidiasis may be the first clinical manifestation in these patients.

Oesophageal squamous cell carcinoma in a young adult with IL-12R beta 1 deficiency.

Genetic defects in the IL-12-IL-23/IFN-gamma circuit confer Mendelian susceptibility to mycobacteria and salmonella. The IL-12/IFN-gamma axis is essential for anti-tumoral immunity in mice. Cancer susceptibility has not been recognised in these patients so far. We report three relatives with IL-12R beta 1 deficiency. At the age of 25 years old, one patient presented with oesophageal squamous cell carcinoma (OSCC). The patient had no previous risk factors for OSCC. He died at the age of 29 years. OSCC is exceedingly rare in individuals under 30 years and frequently relates to alcohol intake and smoking. Disorders of the IL-12-IL-23/IFN-gamma axis may predispose to cancer.

Skin and soft tissue infections (patera foot) in immigrants, Spain.

An unusual skin and soft tissue infection of the lower limbs has been observed in immigrants from sub-Saharan Africa who cross the Atlantic Ocean crowded on small fishing boats (pateras). Response to conventional treatment is usually poor. Extreme extrinsic factors (including new pathogens) may contribute to the etiology of the infection and its pathogenesis.

[Automated erythrocytapheresis as treatment of severe malaria. Study of 6 patients]. / Eritrocitaféresis automatizada en el tratamiento de la malaria grave: estudio de 6 pacientes.

BACKGROUND AND OBJECTIVE: The high mortality attributable to severe malaria by Plasmodium falciparum is related to the grade of parasitemia. Automated erithrocytapheresis (AE) is a safe alternative to exchange transfussion, with the same potential benefits but less undesirable side effects. Literature on this technique is scarce, consisting of isolated reports or short series. The objective of this study is to describe the clinical picture and outcome observed in 6 severely ill malaria patients in whom EA was applied as complimentary therapeutic technique. PATIENTS AND METHOD: An observational prospective descriptive study was carried out of all inpatients with severe malaria in a single hospital between 1996 and 2006 in whom clinical, epidemiological and parsitological data were analyzed. RESULTS: This series included 2 women and 4 men, with a median age of 43 years. In all cases, the infection was acquired in West Sub-Saharan Africa. No patient had received antimalarial prophylaxis and all were infected by Plasmodium falciparum. The grade of parasitemia was between 10% and 35%. The number of severity criteria was between one and 4, the more frequent being hyperbilirrubinemia. All patients received conventional intravenous treatment. The total length of admission oscillated between 5 and 37 days, while the length of stay in the Intensive Care Unit varied between one and 17 days. All patients survived. CONCLUSIONS: AE is a safe technique, with the same advantages that blood exchange but lacking many of its disadvantages. A isolated parasitemia above 10%, or when a parasitaemia above of 5% is associated with any additional World Health Organization-2000 criteria of clinical severity, should constitute an indication for AE.

Immune Reconstitution Inflammatory Syndrome in HIV-Infected Immigrants.

Immune reconstitution inflammatory syndrome (IRIS) includes a group of potentially serious inflammatory processes that may be present in HIV-infected patients after initiating highly active antiretroviral therapy (HAART). Paradoxical IRIS is a worsening of symptoms, after an overwhelming response to a previously diagnosed opportunistic infection (OI); unmasking IRIS reveals a previously occult OI. The main objective of the study was to describe the epidemiological, clinical, and outcome data of HIV-infected immigrants, stratified according to high- or low-income countries of origin, who developed IRIS and to compare them with native-born Spanish patients. This retrospective study reviewed all patients with HIV infection admitted to the Unit of Infectious Diseases and Tropical Medicine between 1998 and 2014. IRIS was identified in 25/138 (18%) immigrant patients and 24/473 (5%) native-born Spanish patients infected with HIV. Most cases, 19/25 (76%), were of unmasking IRIS. The time elapsed between initiation of HAART and development of IRIS was significantly longer in patients with unmasking versus paradoxical IRIS. OIs, in particular due to mycobacteria, were the most frequently involved processes. Twenty percent of patients died. The comparison of immigrant and native-born patients found significant differences for both IRIS type (higher incidence of paradoxical forms among immigrants) and for the absence of malignancies in native-born patients. No significant differences were found when the data of immigrants from low- and high-income countries were compared.

Seroprevalence of Rickettsia typhi and Rickettsia conorii infections in the Canary Islands (Spain).

OBJECTIVE: The aim of this work was to evaluate the prevalence of past infection due to Rickettsia typhi and Rickettsia conorii in the Canary Islands (Spain). METHODS: A representative sample of the population of the seven islands, formed of 662 people aged between 5 and 75 years (368 females, 294 males), was analyzed. Epidemiological data were obtained by direct survey. The detection of serum IgG antibodies against both microorganisms was based on an indirect immunofluorescence test, considered positive if the titers were ≥ 1/80. RESULTS: Of the analyzed population 3.9% had IgG antibodies against R. typhi and 4.4% against R. conorii. Out of these positive samples, only three were positive for both species. The seroprevalence was similar in both sexes. Positive results were found in all age groups, but a higher rate was noticed in those aged 46 years and older (p<0.05). R. typhi was found to be more prevalent in rural areas of all islands, as well as in farmers. CONCLUSIONS: Our results confirm the presence of antibodies against the causative agents of murine typhus and Mediterranean spotted fever in the Canary Islands. Indirect data suggest that the detection of antibodies to R. conorii might be due to a cross-reaction between these species.

Mumps orchitis in the post-vaccine era (1967-2009): a single-center series of 67 patients and review of clinical outcome and trends.

Since the introduction of the mumps vaccine, the age of appearance of mumps infection has shifted from children to adolescents and young adults, groups with a higher incidence of disease complications and sequelae. During the years 2000-2001, the Gran Canaria Island was part of an epidemic of mumps. In that period, our institution attended 67 cases of serologically confirmed acute mumps orchitis, the most serious complication of mumps infection in young postpubertal males. We conducted a descriptive and prospective study of this cohort and extensively reviewed the literature from 1967 (the year the first mumps vaccine was introduced) to 2009. Fifty-six patients were admitted because of general impairment and were treated with alpha-interferon. Sixty-six patients presented parotitis previous to orchitis (interval from parotitis to orchitis, 4.9 d). Orchitis was unilateral in 89.5% and bilateral in 10.4% of cases. More than 98% of patients had orchitis-associated fever. Nine patients had clinical and biochemical data showing acute mumps meningitis, and 11 had subclinical pancreatitis. The mean duration of symptoms was 4.6 days (range, 1-9). During the acute phase, more than 41% of the evaluated testes had a volume >25 mL. Acute hormonal disturbances were highly prevalent. These included decreased levels of testosterone and inhibin B with low or normal levels of gonadotropins in 35% of subjects, and, to our knowledge not previously reported, an atypical hormonal pattern consisting of low levels of free testosterone and inhibin B, along with increased measures of luteinizing hormone but low or normal follicle-stimulating hormone levels (11% of cases). During the follow-up period (mean, 331 d) a high incidence of sperm disturbance was found.

Helminth-related Eosinophilia in African immigrants, Gran Canaria.

Of 788 recent African adult immigrants to Las Palmas de Gran Canaria, 213 (27.0%) had eosinophilia. The most frequent causes were filariasis (29.4%), schistosomiasis (17.2%), and hookworm infection (16.8%). Stool microscopy and filarial and schistosomal serologic tests gave the highest diagnostic yield. Country of origin and eosinophil count were associated with specific diagnoses.

Human Rickettsia felis infection, Canary Islands, Spain.

We report the first cases of human infection by Rickettsia felis in the Canary Islands. Antibodies against R. felis were found in 5 adsorbed serum samples from 44 patients with clinically suspected rickettsiosis by Western blot serology. Fleas from 1 patient's dog were positive for R. felis by polymerase chain reaction.

Streptococcus bovis bacteremia from a venous access port in a patient with AIDS.

We report the first case of S. bovis bacteremia related to endoluminal colonization of a venous access port in a setting of advanced HIV infection, neutropenia and co-infection with HBV. The patient had no bowel abnormalities. The clinical picture was mild and was resolved by removal of the device.

[Q fever in Gran Canaria: 40 new cases]. / Fiebre Q en Gran Canaria. Aportación de 40 nuevos casos.

INTRODUCTION: The aim of this study was to describe the clinical and epidemiologic features of Q fever in the southern area of the island of Gran Canaria (Spain). METHODS: We conducted a retrospective analysis of the clinical and epidemiological data of the cases of Q fever diagnosed by the Microbiology Laboratory of the Hospital Universitario Insular in Gran Canaria between 1998 and 2000. Antibodies against phase II Coxiella burnetii antigens were detected using an indirect immunofluorescence test. The diagnosis of acute Q fever was established by IgG titers > or = 1:320 and IgM titers > or = 1:80, or by seroconversion. RESULTS: During the period of study 59 cases of acute Q fever were diagnosed, making an incidence of 5 cases/100,000 inhabitants/year. The seroprevalence (IgG > or = 1:80) in the patients for whom Q fever serology was requested during that period was 23.9%. Clinical and epidemiologic data were available for 40 patients. All were sporadic cases and 57% were hospitalized. The mean age of the patients was 40.6 6 13.3 years (range 20-74 years), 85% were males and 67.5% came from a rural background. The majority of cases (65%) clustered from April to July. The most frequent clinical presentation was an acute febrile process with elevated liver enzymes (87.5%). Pneumonia was infrequent (only three cases). CONCLUSIONS: In our area Q fever is mainly manifested as an acute febrile illness with subclinical hepatic involvement. This fact and the small number of cases with pneumonia and chronic forms suggest the etiological involvement of C. burnetii strains different from those in other geographic areas.

Murine typhus with renal involvement in Canary Islands, Spain.

Murine typhus and "murine-typhus-like" disease are reemerging infectious diseases. In Canary Islands (Spain), a rather distinct clinical pattern characterized by higher incidence of complications, especially renal damage (including acute failure and urinalysis abnormalities), is apparent and highly suggestive. It could be related to different strains of Rickettsia typhi or other cross-reactive species.