How women are treated during facility-based childbirth: development and validation of measurement tools in four countries - phase 1 formative research study protocol.

BACKGROUND: Every woman has the right to dignified, respectful care during childbirth. Recent evidence has demonstrated that globally many women experience mistreatment during labour and childbirth in health facilities, which can pose a significant barrier to women attending facilities for delivery and can contribute to poor birth experiences and adverse outcomes for women and newborns. However there is no clear consensus on how mistreatment of women during childbirth in facilities is defined and measured. We propose using a two-phased, mixed-methods study design in four countries to address these research gaps. This protocol describes the Phase 1 qualitative research activities. METHODS/DESIGN: We will employ qualitative research methodologies among women, healthcare providers and administrators in the facility catchment areas of two health facilities in each country: Ghana, Guinea, Myanmar and Nigeria. In-depth interviews (IDIs) and focus group discussions (FGDs) will be conducted among women of reproductive age (15-49 years) to explore their perceptions and experiences of facility-based childbirth care, focused on how they were treated by healthcare workers and perceived factors affecting how they were treated. IDIs will also be conducted with healthcare providers of different cadres (e.g.: nurses, midwives, medical officers, specialist obstetricians) and facility administrators working in the selected facilities to explore healthcare providers' perceptions and experiences of facility-based childbirth care and how staff are treated, colleagues and supervisors. Audio recordings will be transcribed and translated to English. Textual data will be analysed using a thematic framework approach and will consist of two levels of analysis: (1) conduct of local analysis workshops with the research assistants in each country; and (2) line-by-line coding to develop a thematic framework and coding scheme. DISCUSSION: This study serves several roles. It will provide an in-depth understanding of how women are treated during childbirth in four countries and perceived factors associated with this mistreatment. It will also provide data on where and how an intervention could be developed to reduce mistreatment and promote respectful care. The findings from this study will contribute to the development of tools to measure the prevalence of mistreatment of women during facility-based childbirth.

HLA-B51 subtypes in Turkish patients with Behçet's disease and their correlation with clinical manifestations.

Behçet's disease (BD) is a multisystemic inflammatory disease believed to be triggered by microbial or environmental factors on a genetic platform. Clinically, it may have an impact on many body systems, including the mucocutaneous, ocular, articular, vascular, and neurological systems. In this study, we aimed to determine the HLA-B51 subtypes and their correlations with the clinical findings of BD. Fifty-one patients with BD and 44 gender- and age-matched healthy subjects were included in this study. The HLA-B51 subtypes of all participants were determined, and the correlations of the clinical manifestations of the disease with the HLA-B51 subtypes were analyzed. HLA-B51 positivity was found to be significantly higher in the patient group (P < 0.001, RR = 15.20), which had significantly more frequent HLA-B5101, HLA-B5102(01), HLA-B5109, and HLA-B5122 subtypes than the healthy subjects (all P < 0.05). Furthermore, considering the correlation between the genetic makeup and clinical findings, the HLA-B5109 subtype was found to be less frequent in patients with papulopustular skin lesions (P = 0.042). The frequency of HLA-B5103 was significantly higher in patients with central nervous system involvement (P = 0.015). There may be a relationship between HLA-B5102(01), HLA-B5109, and HLA-B5122 in addition to HLA-B51 and HLA-B5101(01) in Turkish patients with BD. The HLA-B5109 subtype can be protective against papulopustular lesion development; however, the HLA-B5103 subtype may pose a risk for neuro-Behçet development in BD.

Evaluation of total oxidant and antioxidant status in localized and generalized vitiligo.

BACKGROUND: Vitiligo is an acquired depigmentation disorder, and oxidative stress is suggested to have a major role in its aetiopathogenesis. AIM: To assess whether oxidative stress has a greater role in generalized than in localized vitiligo. METHODS: We assessed 31 patients with active vitiligo (17 localized, 14 generalized) and 38 healthy controls. Serum total oxidant status (TOS), total antioxidant status (TAS) and oxidative stress index (OSI) were determined. RESULTS: Patients with vitiligo had significantly lower TAS and higher TOS and OSI values than controls. Both localized and generalized vitiligo were associated with lower TAS and higher TOS and OSI values, compared with controls, and all three did not differ with vitiligo type. CONCLUSIONS: A systemic oxidative stress exists in patients with vitiligo. These results indicate that the global antioxidant capacity of patients might have been exhausted through a defence mechanism against oxidative processes. The imbalance in TOS/TAS status may have an important role in the aetiopathogenesis of vitiligo, regardless of the clinical variant of the disease.

The relationship between oxidative stress, smoking and the clinical severity of psoriasis.

BACKGROUND: Recent studies suggested that increased oxidant products and decreased antioxidant system functions may be involved in the pathogenesis of psoriasis. In this study, we investigated total oxidative status, Paraoxonase (PON)1/arylesterase enzyme activities and severity of the disease in smoker and non-smoker psoriatic patients. METHODS: Fifty-four patients with plaque type psoriasis (28 smokers and 26 non-smokers) and 62 healthy volunteers (16 smokers and 46 non-smokers) were enrolled in the study. Serum total oxidant status (TOS), total antioxidant capacity (TAC) and arylesterase levels were measured, and oxidative stress index (OSI) was calculated in all participants. RESULTS: Psoriasis Area and Severity Index scores were significantly higher in smoker patients than in non-smoker patients (P = 0.014). Both smoker and non-smoker patients had significantly increased TOS levels and OSI values and decreased TAC levels than healthy subjects (all P values = 0.000). The TAC and TOS levels, OSI values and arylesterase activities were similar between smoker and non-smoker patients. The levels of triglyceride (TG), total cholesterol (TC), low-density lipoprotein (LDL) and high-density lipoprotein (HDL) were not significantly different between smoker and non-smoker psoriasis patients. When compared with non-smoking controls, only smoking psoriasis patients had significantly higher TG (P = 0.005), lower HDL (P = 0.022) and lower arylesterase levels (P = 0.015). There were no significant correlations with Psoriasis Area and Severity Index (PASI) scores and TAC, TOS, OSI, TG, TC, HDL and LDL levels in all psoriasis patients. CONCLUSIONS: Oxidative stress is increased in psoriasis patients regardless of their smoking status. The decreased arylesterase activity in smoker psoriasis patients suggested that smoking may be a considerable risk factor that increases the severity of psoriasis by increasing oxidative stress in these patients.

Knowledge and attitudes of primary care physicians regarding food allergy and anaphylaxis in Turkey.

BACKGROUND: Food allergy, which becomes an important public health problem, can lead to important morbidity and mortality. Patients with food allergies are more likely to first present to their primary care physicians. We aimed to determine the knowledge of primary care physicians with regard to management of food allergies and anaphylaxis. METHODS: Primary care physicians were surveyed via a questionnaire aimed to document their knowledge and attitudes about food allergy and anaphylaxis management. RESULTS: A total of 297 participants completed questionnaires, 55.6% of which were female. Participating physicians had a mean of 17.0 ± 6.1 years of experience. Participants answered 47.2% of knowledge-based items correctly. Overall, participants fared poorly with regard to their knowledge on the treatment of food allergies and anaphylaxis. For example while 60.7% knew that a child can die from the milk allergy reaction, only 37.5% were aware that a child with IgE mediated milk allergies cannot eat yoghourts/cheese with milk. Besides, 53.1% of them chose epinephrine as their first treatment of choice in case of anaphylaxis, yet only 16.6% gave the correct answer about its dosage. Nearly a third of participants (36.7%) felt they were knowledgeable enough regarding the management of patients with food allergies, while 98.2% extended their request for future periodic educational meetings on allergic disorders. CONCLUSION: Knowledge of food allergy and anaphylaxis among primary care physicians was unsatisfactory. Provision or periodic educational programmes should be aimed at improving the standard of practice as acknowledged by the participants.

Using a Caesarean Section Classification System based on characteristics of the population as a way of monitoring obstetric practice.

OBJECTIVE: to compare the distribution of caesarean rates in the Robson's 10 groups classification in order to see if any change occurred after the implementation of an audit and feedback intervention. DESIGN: cross sectional, before and after an audit and feedback study. SETTING: a university hospital in Brazil. METHODS: clinical records of all births during two three months-periods were evaluated. Each case of CS was classified into one of ten mutually exclusive categories according to obstetric characteristics. The proportion of CS in each group was compared in both periods. RESULTS: total number of deliveries and the high rate of CS were similar in both periods. Group 3 (multiparous excluding previous CS, single, cephalic, >/= 37 weeks, spontaneous labour) accounted for the largest proportion of deliveries, 28.5 and 26.8% in both periods. Group 1 (nulliparous, single, cephalic, >/= 37 weeks, spontaneous labour) was the second largest one, while Group 5 (previous caesarean section, single, cephalic, and >/= 37 weeks) was the third but the largest contributor to CS, accounting for 16.6 and 14.9% among all deliveries in both periods. Groups 2 (nulliparous, single, cephalic, >/= 37 weeks, induction or CS before labour) and 4 (multiparous excluding previous CS, single, cephalic, >/= 37 weeks, induction or CS before labour) were less prevalent, however had higher rates of CS. Only in Group 10 (All single, cephalic,

Six different CYBA mutations including three novel mutations in ten families from Turkey, resulting in autosomal recessive chronic granulomatous disease.

BACKGROUND: One of the rarest forms of autosomal recessive chronic granulomatous disease (AR-CGD) is attributable to mutations in the CYBA gene, which encodes the alpha polypeptide of cytochrome b(558), (also known as p22-phox), a key transmembrane protein in the phagocyte NADPH oxidase system. This gene is localized on chromosome 16q24, encompasses 8.5 kb and contains six exons. MATERIALS AND METHODS: We report here the clinical and molecular characterization of 12 AR-CGD patients from 10 consanguineous, unrelated Turkish families with clinical CGD and positive family history. The ages of the six male and six female patients were between 1and 18 years. Before mutation analysis, subgroup analysis of patients was made by flow cytometry with antibodies against NADPH-oxidase components and with the DHR assay (flow cytometric assay of NADPH oxidase activity in leucocytes). RESULTS: Mutation analysis of CYBA showed six different mutations: a frameshift insertion in exon 3 (C after C166); a missense mutation in exon 2 (p.Gly24Arg), a splice-site deletion in intron 1 (4-bp deletion +4_+7 AGTG), a novel nonsense mutation in exon 6 (p.Cys113X), a novel large deletion of exons 3-6 and a novel 1-bp deletion in exon 6 (c.408delC). All mutations were present in homozygous form and all parents investigated were found to be heterozygotes for these mutations. CONCLUSIONS: In our series of 40 CGD families, approximately 25% of the families have p22-phox defects, with six different mutations, including three novel mutations. The high rate of consanguineous marriages seems to be the underlying aetiology.

Four different NCF2 mutations in six families from Turkey and an overview of NCF2 gene mutations.

BACKGROUND: One of the rarest forms of autosomal recessive chronic granulomatous disease (AR-CGD) is attributable to mutations in the NCF2 gene, which encodes the polypeptide p67(phox), a key cytoplasmic protein in the phagocyte NADPH oxidase system. NCF2 is localized on chromosome 1q25, encompasses 40 kb and contains 16 exons. MATERIALS AND METHODS: We report here the clinical and molecular characterization of six patients with CGD from six consanguineous Turkish families. The ages of the five female patients were between 3 and 22 years and a male patient was 2 years old; all patients showed clear clinical symptoms of CGD. RESULTS: The mothers of the patients did not show a bimodal histogram pattern specific for X-CGD in the dihydrorhodamine-1,2,3 (DHR) assay. Moreover, p67(phox) protein expression was not detectable using flow cytometric analysis of the patients' neutrophils except in those from patient 6, which had a diminished expression. Mutation analysis of NCF2 revealed four different homozygous mutations: a novel nonsense mutation in exon 3 c.229C>T, p.Arg77X; a novel missense mutation in exon 4 c.279C>G, p.Asp93Glu; a nonsense mutation in exon 4 c.304C>T, p.Arg102X; and a novel missense mutation in exon 6 c.605C>T, p.Ala202Val. The parents were found to be heterozygotes for these mutations. CONCLUSIONS: The prevalence of NCF2 mutant families is approximately 15% in our series of 40 CGD families. This high incidence of A67 CGD in Turkey is undoubtedly caused by the high incidence of consanguineous marriages. We found three new mutations in NCF2 and one previously described. These are presented together with an overview of all NCF2 mutations now known.

AA-861 appears to suppress leukocyte infiltration induced by traumatic brain injury in rats.

OBJECTIVE: To study the effect of 2,3,5-Trimethyl-6-(12-hydroxy-5,10-dodecadiynyl)-1,4-benzoquinone (AA-861) on intercellular adhesion molecule 1 (ICAM-1) and P-selectin expression, leukotriene B4 (LTB4) level, and myeloperoxidase (MPO) activity 24 hours after traumatic brain injury (TBI). METHODS: This study was carried out in the laboratory of the Department of Clinical Pharmacology, Osmangazi University, Eskisehir, Turkey in 2006. Traumatic brain injury was induced in 2 sets of animals using Feeney`s weight-drop method. The first set was used to study the expression of ICAM-1, P-selectin, CD11a, and mouse anti-rat granulocyte monoclonal antibody (HIS48). The second was used to study tissue changes in LTB4 level, and MPO activity. The rats were sacrificed at 0.5, 4, 24, 48, and 72 hours post-injury. RESULTS: Intercellular adhesion molecule (p=0.000001) and P-selectin expression (p=0.00002) peaked at 24 hours, remained high at 48 hours (p=0.00012 for ICAM-1, and p=0.00002 for P-selectin), and 72 hours (p=0.000008 for ICAM-1, p=0.0011 for P-selectin). The HIS48 intensity was significantly increased at 24-72 hours (p=0.022), while the intensity of CD11a became significant only at 72 hours (p=0.040). Myeloperoxidase activity increased notably at 24 hours (p=0.00077), and peaked at 48 hours (p=0.00001). The LTB4 increased markedly at 4 hours (p=0.000004), and peaked at 24 hours (p=0.000001). Pretreatment with AA-861 considerably suppressed the expression of ICAM-1 (p=0.0053), and P-selectin (p=0.0018) on microvascular endothelium, and lowered MPO activity (p=0.0007), and LTB4 level (p=0.008) at 24 hours. CONCLUSION: The present results suggest that AA-861 might be a potential mediator in the treatment of brain inflammation in TBI.

Frontoethmoid sinus osteoma as a cause of subperiosteal orbital abscess.

A 65-year-old woman presented with severe periorbital pain and swelling of the left eye, with complete ptosis, proptosis, and conjunctival chemosis. The eye was in a hypotropic position, and activity in the left superior rectus was inadequate. A firm, elastic, 2-cm mass was palpated near the superior orbital rim. Computed tomography revealed a subperiosteal abscess (SPA) at the superior portion of the orbit and a large frontoethmoidal sinus osteoma. After the SPA had been surgically drained and the osteoma completely removed, the patient recovered, with resolution of proptosis, ptosis, and motility limitations. Osteomas of the paranasal sinuses are usually asymptomatic and rarely cause SPA and orbital cellulitis. Therefore, osteoma cases must be closely followed to ensure that early diagnosis and treatment of SPA are successful in preventing severe visual loss and rescuing the patient's vision.

Oxidative stress parameters in localized scleroderma patients.

Localized scleroderma (LS) (morphea) is a chronic, inflammatory skin disease with unknown cause that progresses with sclerosis in the skin and/or subcutaneous tissues. Its pathogenesis is not completely understood. Oxidative stress is suggested to have a role in the pathogenesis of localized scleroderma. We have aimed to determine the relationship of morphea lesions with oxidative stress. The total oxidant capacity (TOC), total antioxidant capacity (TAC), paroxonase (PON) and arylesterase (ARES) activity parameters of PON 1 enzyme levels in the serum were investigated in 13 LS patients (generalized and plaque type) and 13 healthy controls. TOC values of the patient group were found higher than the TOC values of the control group (p < 0.01). ARES values of the patient group was found to be higher than the control group (p < 0.0001). OSI was significantly higher in the patient group when compared to the control (p < 0.005). Oxidative stress seems to be effective in the pathogenesis. ARES levels have increased in morphea patients regarding to the oxidative stress and its reduction. Further controlled studies are required in wider series.

A stop codon in xeroderma pigmentosum group C families in Turkey and Italy: molecular genetic evidence for a common ancestor.

Xeroderma pigmentosum family G from Van, Turkey had two severely affected children: a son with multiple skin cancers who died at age 10 (XP67TMA), and an 8 y old daughter who began developing skin cancer before 3 y of age (XP68TMA). XP67TMA and XP68TMA cells were hypersensitive to killing by ultraviolet and the post-ultraviolet DNA repair level was 12-16% of normal. Host cell reactivation of an ultraviolet-treated reporter plasmid cotransfected with a vector expressing wild-type XPC cDNA assigned XP67TMA to xeroderma pigmentosum complementation group C. The XPC mRNA level was markedly reduced. Sequencing of the 3.5 kb XPC cDNA from XP67TMA showed a C-T mutation in XPC exon 8 at base pair 1840. This mutation converts the CGA codon of arginine at amino acid 579 to a UGA stop codon resulting in marked truncation of the 940 amino acid xeroderma pigmentosum C protein. Restriction fragment length polymorphism analysis of XPC exon 8 DNA in XP67TMA and XP68TMA showed that both affected children had a homozygous mutation and that both parents had heterozygous normal and mutated sequences at the same position consistent with a history of consanguinity in the family. The mutated allele also contained two XPC single nucleotide polymorphisms. The same mutated XPC allele was reported in an Italian family. Studies of 19 microsatellite markers flanking the XPC gene on chromosome 3 suggest that the XPC allele passed between Italy and Turkey approximately 300-500 y ago. This XPC allele containing a nonsense mutation is associated with severe clinical disease with multiple skin cancers and early death.

Successful bone marrow transplantation in a case of Griscelli disease which presented in accelerated phase with neurological involvement.

Griscelli disease (GD) is a rare disorder characterized by pigment dilution, immunodeficiency and occurrence of accelerated phase consisting of hemophagocytosis, pancytopenia and neurological manifestations. Allogeneic BMT in the early period is an important modality of treatment for GD. We carried out an alloBMT from an HLA-identical sibling donor on a 4-year-old girl who presented in accelerated phase with neurological manifestations including convulsions, strabismus, severe dysarthria, ataxia and clonus. She was treated with etoposide, methylprednisolone and intrathecal methotrexate for 8 weeks and underwent alloBMT after receiving a conditioning regimen including ATG (rabbit, 10 mg/kg x 5 days), Bu/Cy. 8 x 108/kg nucleated bone marrow cells were given. Engraftment occurred early and the post-BMT period was uneventful. Currently, she is at 18 months post BMT with sustained engraftment and with a normal neurological examination except for minimal clonus. Long-term follow-up will determine the prognosis regarding the neurological findings.

Otorhinolaryngological aspects of Xeroderma pigmentosum.

OBJECTIVE: to evaluate the probable presence of otorhinolaryngological pathology accompanied by head and neck region skin findings in patients with Xeroderma pigmentosum. METHODS: a total of 19 patients with Xeroderma pigmentosum were investigated for otorhinolaryngological findings. The patients gave their anamnesis and underwent physical examination, audiological tests and endoscopic examination. RESULTS: various malignancies developed in 14 patients on the sun-exposed areas of the head and neck region. Multiple malignancies were found in six of them. There was no other pathological condition secondary to this rare clinical entity. CONCLUSION: Xeroderma pigmentosum causes skin lesions. Some otolaryngological findings such as rhinitis, sinusitis etc. were thought to be coincidental.

Juvenile hyaline fibromatosis in one Turkish child.

We describe a case of juvenile hyaline fibromatosis (JHF) in a Turkish child. Only about 40 cases of juvenile hyaline fibromatosis had been reported in English literature as of March 1998, and it had not been reported in English literature from Turkey as of November 1998. Juvenile hyaline fibromatosis characterized by multiple cutaneous masses is a rare hereditary disorder. This disease is usually found in children, and a malfunction of collagen synthesis is considered as the pathogenetic cause. In the presented case, light microscopy demonstrated an abundance of a homogeneous, amorphous, eosinophilic extracellular matrix in which fibroblasts were embedded. Well-formed collagen fibers could not be demonstrated with Gieson's method or with reticulin preparation. The hayalin material periodic acid-Schiff-positive and diastase-resistant, whereas the Congo red method was negative. Immunohistochemically, the spindle-shaped cells were actin (smooth muscle) negative.

Bare lymphocyte syndrome with lack of HLA class I and II antigens. Presentation of two cases.

Bare lymphocyte syndrome (BLS) is a rare disorder characterized by deficient expression of human leukocyte antigens (HLA antigens) and combined immunodeficiency to various degrees. Recurrent severe infections especially due to opportunistic organisms are common. Here, we present two patients with BLS who lack both class I and II antigens (Type III). They had the typical clinical and immunologic findings of BLS. The first patient showed marked improvement in pulmonary symptoms resulting from cytomegalovirus infection by means of gancyclovir treatment. However, intramuscular injections of interferon-alpha (IFN-alpha) had no beneficial effect in either the expression of HLA antigens or the clinical status. The second patient died of septicemia while being prepared for bone marrow transplantation.

Gastric antral stricture in a patient with chronic granulomatous disease.

Chronic granulomatous disease (CGD) is a rare disorder of phagocytic cell oxidative metabolism. Patients have recurrent infections with catalase-positive organisms and granulomatous lesions throughout the body. Gastric antrum can be an occult site of involvement. We describe a four-year old boy with chronic granulomatous disease who was admitted with the complaints of persistent vomiting and weight loss. Gastric antral narrowing was diagnosed according to radiological findings. Treatment with steroid and antibiotics yielded a good clinical response in 15 days with a relief of the obstruction. This case report emphasizes the beneficial effect of this form of therapy in preventing life-threatening obstruction of vital organs in CGD.

Cyclic neutropenia complicated by renal AA amyloidosis.

Cyclic neutropenia is a rare disease characterized by regular cyclic fluctuations in the numbers of neutrophils. Patients with the disease suffer from recurrent infections at regular intervals of nearly three weeks. Recently, recombinant human granulocyte colony-stimulating factor (rhG-CSF) was reported to be an effective treatment for this disease. here we describe 17-year-old cyclic neutropenic female patient with a very rare association of renal amyloidosis of AA type who was under intermittent rhG-CSF treatment for the previous one and a half years. We conclude that although the disorder is usually benign, reactive amyloidosis may rarely develop in cases who remain untreated for a long period of time. However familial Mediterranean fever (FMF) type II should also be born in mind, particularly in predisposed populations.

Diclofenac sodium suppository in the treatment of primary nocturnal enuresis.

The prostaglandin synthesis inhibitor diclofenac sodium was given as suppository in the treatment of primary nocturnal enuresis in 24 patients. The statistically significant difference in response to diclofenac sodium and to placebo leads us to use diclofenac sodium in primary nocturnal enuresis.

Relaxation degree: a new concept in erectile dysfunction.

PURPOSE: To investigate the clinical application of the new parameter' Relaxation Degree. PATIENTS AND METHODS: Chart reviews of 150 patients were evaluated retrospectively of whom 96 had been diagnosed as pure cavernoocclusive dysfunction (COD) by using corpus cavernosum electromyography (CC-EMG) and cavernosometry and of whom 54 had been diagnosed as psychogenic erectile dysfunction by normal CC-EMG; cavernosometry and pulse doppler scanning. Relaxation degree was defined as the percent decrease in the amplitudes of the electrical activity of corpus cavernosum (EACC). RESULTS: Mean relaxation degree measurements were found as 66.5%, 66.28% and 16% in minimal, moderate and severe COD groups respectively. This value was 80% in psychogenic erectile dysfunction group. Statistical comparisons were found significant between minimal and severe and moderate and severe COD groups. All three organic erectile dysfunction groups were also compared with psychogenic erectile dysfunction group where differences were found significant. If the normal relaxation degree was accepted as more than 80%, COD could be diagnosed with 93% specificity and 98% sensitivity without performing cavernosometry. CONCLUSION: Relaxation degree parameter could be used in diagnosis of COD showing the percent relaxation ability of the cavernous smooth muscle as a non invasive method when compared with cavernosometry.