A review of 43 cases of tetanus neonatorum.

Forty-three patients with neonatal tetanus admitted to the Infectious Diseases Ward of the Istanbul University Faculty of Medicine Children's Hospital at Capa from 1982-1989 are presented. Thirty-two (74.4%) of the subjects were boys. The overall mortality was found to be 60 percent and the mean incubation period 6.1 +/- 20 days. The mean period between onset of symptoms and hospital admission was 1.7 days. Thirty-nine (90.7%) of the 43 patients were unhygienic home deliveries and only two cases were delivered with a midwife in attendance. None of the mothers of the children in our series had been immunized with tetanus toxoid. The incidence of neonatal tetanus in Turkey is on the decline, but a fraction of the population, the so-called urban poor, is still at high risk for preventable diseases. Every contact an unvaccinated person has with a health care professional should be viewed as an opportunity for tetanus immunization.     

Dynamic muscular endurance in primary fibromyalgia compared with chronic myofascial pain syndrome.

The objective of the study was to quantify the voluntary dynamic muscular endurance (DME) in patients with primary fibromyalgia (PF) since easy fatigability of muscles is one of the major symptoms in this syndrome. Consecutive outpatients referred to a rheumatology clinic specializing in PF were investigated. Thirty-six patients with PF were compared with 18 patients with chronic myofascial pain (CMP) syndrome. Subjects were matched according to gender, age, height, weight, peak torque, and contractional work. The DME was defined as the number of repeated knee extensions necessary for the contractional work in two successive knee extensions to be equal to or below 70% of the initial value measured with an isokinetic dynamometer. A significantly lower DME was found in the PF group than in the CMP group: 11 (7-13) and 18 (11-25), respectively (median and quartiles), p less than .005. Patients with PF have a low voluntary muscular endurance compared to CMP patients.     

Individual chaperones required for Yop secretion by Yersinia.

Pathogenic yersiniae secrete anti-host proteins called Yops, by a recently discovered Sec-independent pathway. The Yops do not have a classical signal peptide at their N terminus and they are not processed during membrane translocation. The secretion domain is nevertheless contained in their N-terminal part but these domains do not resemble each other in the different Yops. We have previously shown that YopE secretion requires SycE, a 15-kDa acidic protein acting as a specific cytosolic chaperone. Here we show that the gene downstream from yopH encodes a 16-kDa acidic protein that binds to hybrid proteins made of the N-terminal part of YopH and either the bacterial alkaline phosphatase or the cholera toxin B subunit. Loss of this protein by mutagenesis led to accumulation of YopH in the cytoplasm and to a severe and selective reduction of YopH secretion. This protein thus behaves like the counterpart of SycE and we called it SycH. We also engineered a mutation in lcrH, the gene upstream from yopB and yopD, known to encode a 19-kDa acidic protein. Although this mutation was nonpolar, the mutant no longer secreted YopB and YopD. The product of lcrH could be immunoprecipitated together with cytoplasmic YopD. lcrH therefore seems to encode a YopD-specific chaperone, which we called SycD. Determination of the dependence of YopB on SycD requires further investigation. SycE, SycH, and SycD appear to be members of a new family of cytosolic chaperones required for Yop secretion.     

Perineal ectopic testis

The case of a 24-year-old patient is presented who was erroneously presumed to have unilateral testicular aplasia following inguinal exploration in early childhood. At the age of 24 years he underwent surgery for a soft perineal mass, which was found to be perineal testicular ectopia associated with a congenital hernia. Histological examination of the excised testicle revealed atrophy of the germinal epithelium. A survey of the literature yielded approximately 175 more cases of perineal testicular ectopia. This is the most frequent from of testicular ectopia with a relative incidence of less than 1% of all cases of undescended testes. Ectopic testes seem only to differ from undescended testes in terms of topography. Little difference can be observed in terms of histology or the incidence of concomitant hernias.     

Vaginal cones: a conservative method of treating genuine stress incontinence

Summary. A set of weighted vaginal cones designed to exercise the pelvic floor muscles was used by a group of 39 premenopausal patients with genuine stress incontinence who were awaiting corrective surgery. The effectiveness of the exercises in reducing urinary loss was assessed with a standard urine pad test before and after 1 month of training. Of the 30 women who completed 1 month of the exercises 70% felt they were improved or cured and 90% found it an acceptable method of treatment. Of the 30 women only 11 (37%) opted for surgery after training. There was a highly significant correlation (P<0·001, r_s= -0·21) between decreased urine loss and increase in retained cone weight.     

Non-Hodgkin's lymphoma with protein S deficiency]

Venous thrombosis is rare in children. It can be either acquired or of constitutional origin. Thrombosis during non-Hodgkin lymphoma remains exceptional and is usually locally associated to the tumoral process, raising the issue of its tumoral or cruoric nature. The treatment is based on anticoagulation concomitantly to chemotherapy. We report on a 4-year-old boy admitted for mediastinal non-Hodgkin lymphoma, who developed a thrombosis of the superior vena cava associated to protein S-deficiency. The mechanism of thrombosis may have been multifactorial: associated protein S-deficiency, vascular compression, tumoral process and chemotherapy.     

Recombinant activated factor VII for intractable bleeding post splenectomy in a patient with myeloproliferative disorder.

Recombinant activated factor VII has been Food and Drug Administration approved to treat hemorrhages in hemophiliac patients with inhibitors and in acquired hemophilia patients. Recombinant activated factor VII use has also been considered for the management of uncontrolled bleeding in a number of congenital and acquired hemostatic abnormalities. The myeloproliferative disorders are a group of clonal hematologic diseases where, frequently, abnormal platelet function is considered a hallmark. This is the first case report addressing the clinical benefit of off-label use of recombinant activated factor VII in an attempt to control intractable bleeding in a patient with a myeloproliferative disorder after splenectomy.