Two cases of children of homosexuals

The literautre is reviewed with reference to parental attitudes related to homosexuality, sex role typing, and object choice. Two cases of children of opposite-sexed homosexual parents are presented with projective testing indicating difficulties with gender role identity. It is our impression that the manifestation of sexual conflict in these homosexual parents expressed in attitudes and behavior toward the child is not unique and does not differ significantly from that of the heterosexual parent who has sexual conflicts. More long-term data are necessary to help us to understand the influences of parental homosexuality on the growing child.     

Massive osteolysis in a girl with agenesis of thyroid C cells

A rare case of massive osteolysis affecting the pelvis of a young girl is presented. The clinical, radiographic, and histopathological features are described in detail. Septicemia complicated the clinical course and the patient eventually died. Histopathological examination of the pelvic lesion revealed massive osteolysis characterized by prominent osteoclastic activity with extensive bone resorption. A thorough post-mortem histological examination of the thyroid gland showed no C cells. This is an interesting observation, since it is known that thyroid C cells are the primary source of calcitonin. Since the main biological effect of calcitonin is to inhibit osteoclastic bone resorption, there is a possibility that massive osteolysis in our case could have been related to the lack of this hormone. There is a clear need for further investigation regarding the role of thyroid C cells and calcitonin in this puzzling disease.     

Rare localization of extrarenal nephroblastoma in 1-month-old female infant

Extrarenal occurrence of Wilms' tumor is exceptional and the diagnosis is almost always made after surgery. The exact mechanism whereby a Wilms' tumor occurs in extrarenal tissue is unknown. The tumor is most commonly located in the retroperitoneum or inguinal region. Localization in subcutaneous tissue is extremely rare. In this paper, the case of a 1-month-old female infant with an extrarenal Wilms' tumor located in the lumbosacral region is presented. Surgical excision is the treatment of choice, and the same general therapeutic rules should be followed as when the kidney is affected.     

Is the prevalence of arterial hypertension in rheumatoid arthritis and osteoarthritis associated with disease?

In this study, we compare the prevalence of arterial hypertension (HT) in rheumatoid arthritis (RA) and osteoarthritis (OA) patients, exposed to high- and low-grade chronic inflammation, respectively, to assess the possible association between chronic inflammation and HT. A total of consecutive 627 RA and 352 OA patients were enrolled in this multicentric study. HT was defined as a systolic blood pressure (BP) ≥ 140 and/or diastolic BP ≥ 90 mmHg or current use of any antihypertensive drug. Overweight/obesity was defined as body mass index (BMI) ≥ 25, and patients ≥65 years were considered elderly. The prevalence of HT was higher in the OA group than in the RA group [73.3 % (95 % CI, 68.4, 77.7) and 59.5 % (95 % CI, 55.6, 68.4) P < 0.001, respectively]. When the results were adjusted for age and BMI, the HT prevalence was similar in both groups [RA 59 % (95 % CI, 55.1, 63.8) OA 60 % (95 % CI, 58.4, 65.0)]. In both groups, the prevalence of HT was higher in the elderly and those who were overweight than in the younger patients and those with a BMI < 25. Overweight (BMI ≥ 25) and age ≥65 were independent predictors of HT in multivariate logistic regression model, which showed no association between HT and the disease (RA or OA). The results indicate a robust association of age and BMI with HT prevalence in both RA and OA. The difference in HT prevalence between RA and OA is due rather to age and BMI than to the features of the disease, putting into question specific association of HT with RA.     

A novel and efficient one-pot synthesis of symmetrical diamide (bis-amidate) prodrugs of acyclic nucleoside phosphonates and evaluation of their biological activities

A novel and efficient method for the one-pot synthesis of diamide (bis-amidate) prodrugs of acyclic nucleoside phosphonates, starting from free phosphonic acids or phosphonate diesters is reported. The approach from phosphonate diesters via their bis(trimethylsilyl) esters is highly convenient, eliminates isolation and tedious purification of the phosphonic acids, and affords the corresponding bis-amidates in excellent yields (83-98%) and purity. The methodology has been applied to the synthesis of the potent anticancer agent GS-9219, and symmetrical bis-amidates of other biologically active phosphonic acids. Anti-HIV, antiproliferative, and immunomodulatory activities of the compounds are discussed including the bis-amidate prodrugs 14 and 17 that exhibited anti-HIV activity at submicromolar concentrations with minimal cytotoxicity.     

Alveolar echinococcosis in patient after cadaveric kidney transplantation

52-years old man years following the kidney transplantation from deceased donor was admitted to the hospital with fever and progressive abdominal pain. The patient was diagnosed with chronic hepatitis C seven years before admission. Graft function in posttransplant period was stable and optimal, the patient was treated with standard maintenance immunosupresive protocol (cyclosporine A, mycophenolate mofetil and low-dose prednison), metylprednisolon bolus therapy (1 g/m² body surface area), was administered two months prior to admission due to creeping creatinine (suspection of acute rejection was not confirmed by biopsy). Empiric antibiotic treatment due to febrile status was ineffective. Abdominal ultrasound and computer tomography (CT) scan revealed three tumorous lesions in the liver, radical surgical intervention was not executable. Histological examination of the tissue from the lesions demostrated alveolar echinococcosis, serology for Echinoccocus multilocularis was positive. Long-term treatment by mebendazol 200 mg twice daily led to disappearance of the clinical symptoms, but after the therapy cessasion patient was again hospitalized with fever and progression of cystic lesions in CT scan. Following the mebendazol therapy reinstalation the clinical course of echinococcosis was improved and remained stable, transplant kidney failure occurred due to progression of interstitial fibrosis/tubular atrophy and chronic haemodialysis was initiated one year later.     

Modified Marcy repair for indirect inguinal hernia in children: a 24-year single-center experience of 6826 pediatric patients

Purpose

To evaluate the management and outcomes of modified Marcy repair for inguinal hernia in a large series of children.

Methods

We analyzed the case records of 6826 pediatric patients who underwent surgery for inguinal hernia between January, 1991 and January, 2015 at Split University Hospital in Croatia. The following parameters were examined: sex, age, location of the hernia, intraoperative or postoperative complications, recurrence, and surgical method.

Results

The 6826 patients included 4751 boys and 2075 girls operated on for inguinal hernia. The mean age was 3.5 years, and mean followup was 14 years. Right-side predominance was noted with 59.50 % right hernia repairs, 33.72 % left hernia repairs, and 6.78 % bilateral hernia repairs. There were 6410 (93.90 %) elective procedures and 416 (6.10 %) emergency procedures for incarceration. The mean duration of surgery was 26 min (14–90 min), and the mean hospital stay was 1 day. Marcy repair was the most commonly performed operation (95.76 %), whereas Ferguson’s technique was performed in only 3.98 % of the children. The overall recurrence rate was 0.43 %, with a recurrence rate of 0.36 % for Marcy repair and 1.83 % for Ferguson repair (p = 0.0003).

Conclusion

Modified Marcy hernia repair is a safe and effective procedure for inguinal hernia in children with excellent outcomes and a low incidence of recurrence.

     

Which questionnaires should we use to evaluate quality of life in patients with chronic graft-vs-host disease?

AimTo investigate the ability of two standard quality of life (QOL) questionnaires – The Short Form (36-item) Health Survey (SF-36) and The European Organisation for Research and Treatment of Cancer Quality of Life Questionnaire-Core 30 (EORTC QLQ C30) to evaluate QOL in patients with chronic graft-vs-host disease (cGVHD) graded according to National Institutes of Health (NIH) consensus criteria.MethodsIn this cross-sectional study, QOL was assessed in patients who underwent allogeneic stem cell transplantation (allo-SCT) at the University Hospital Centre Zagreb and were alive and in complete remission for more than one year after allo-SCT.ResultsThe study included 58 patients, 38 patients with cGVHD and 20 controls, patients without cGVHD. Patients with cGVHD scored according to the NIH criteria had significantly lower scores of global health status and lower QOL on all SF-36 subscales and most of QLQ C30 functional subscales (P < 0.050 for all comparisons). Furthermore, patients with active cGVHD had significantly lower QOL scores than patients with inactive cGVHD, and this difference was most evident in physical functioning subscale of SF-36 (P = 0.0007) and social functioning subscale of QLQ C30 (P = 0.009).ConclusioncGVHD scored according to the NIH criteria is correlated with patient-reported QOL, particularly in the physical domains as detected by SF-36. QLQ C30 questionnaire adds more information on social functioning and should be used as a valuable tool in the evaluation of social domains in cGVHD patients.Although it is potentially lifesaving for a variety of hematological malignant and non-malignant disorders, allogeneic stem cell transplantation (allo-SCT) carries a significant risk of acute and late post-transplant complications. Improvements in transplantation techniques and supportive care have resulted in a reduction of early transplant-related mortality (1,2). However, the burden of late complications remains high, and two thirds of long-term allo-SCT survivors experience at least one chronic health condition (3). These complications occur due to treatment exposures before and during allo-SCT, cause substantial mortality, and severely impair patients’ functional status and quality of life (QOL). This is why today the aim of the treatment is not just to cure the primary hematological disease, but to facilitate the recovery of the physical and emotional functioning and improve QOL and social reintegration in family and work environment.Health-related QOL is now considered to be one of the relevant treatment outcomes because it provides a broader understanding of the patient’s status beyond simple disease-free survival. It is a multi-dimensional construct comprised of several related domains including physical, emotional, social, and role functioning, as well as a person''s overall evaluation of his or her well-being and ability to function (4,5). Better understanding of QOL in long-term survivors is necessary to provide adapted pre-transplant counseling and recommendations for post-transplant follow-up.With a cumulative incidence of 40%-70% and significant mortality, chronic GVHD (cGVHD) represents the most important late complication following allo-SCT (6,7). Moreover, it seems that the incidence of cGVHD in the recent years has been increasing, probably due to the fact that much older patients undergo allo-SCT, as well as due to the increased use of peripheral blood stem cell grafts and matched unrelated donors, all known risk factors for cGVHD (8). In a series of publications originating from 2005 National Institutes of Health (NIH) Consensus Conference, investigators proposed means to standardize diagnosis, scoring, histopathology, biomarkers, response assessment, and research in cGVHD (9-14). These criteria were developed to advance clinical trials and consequently improve the management of cGVHD and long-term survivorship after allo-SCT.As one of the important treatment outcomes, QOL is increasingly being subjected to the same degree of rigorous study as other significant allo-SCT outcomes. Most of the studies so far have reported a negative, significant association between cGVHD and QOL after allo-SCT (15-20). However, some of the studies have found no association (21-25), and this relationship still needs to be elucidated. The awareness of the relationship between QOL and cGVHD is necessary to further facilitate the prevention and treatment of cGVHD.The aim of this study was to examine the effect of cGVHD on QOL in our cohort of long-term allo-SCT survivors with the use of two standard QOL questionnaires; The Short Form (36-item) Health Survey (SF-36) and The EORTC Quality of Life Questionnaire (QLQ C30). Furthermore, we assessed QOL according to cGVHD severity and activity defined by the NIH consensus criteria.