Exploration of the relationship between functional motor and communication performance levels and amount of use of the more affected upper extremity based on the caregivers' perceptions in children with hemiplegic cerebral palsy: A cross-sectional study.

OBJECTIVE: To investigate relationships between amount of use of the more affected upper extremity and functional motor and communication performance classification systems. MATERIAL AND METHODS: The study comprised 95 children with congenital hemiplegic cerebral palsy (CP) aged 6-15 years (52 males, 43 females; mean age 9.53, SD 3.1) and their parents/caregivers. The amount of use of the more affected upper extremity was assessed using Pediatric Motor Activity Log-Revised-How Often subscale (PMAL-R HO). Functional levels of the enrolled children were defined by the parents/caregivers using Manual Ability Classification System (MACS), Gross Motor Function Classification System-Expanded and Revised (GMFCS-E&R), and Communication Function Classification System (CFCS). RESULTS: A strong and negative correlation was found between PMAL-R HO subscale score and MACS (r=-0.819), suggesting that children with lower MACS levels are more likely to use their more affected upper extremity spontaneously. Additionally, negative and moderate associations between PMAL-R HO subscale score and GMFCS and CFCS were revealed (r1=-0.549 and r2=-0.567). CONCLUSION: The amount of use of the more affected upper extremity is more sensitive to MACS than GMFCS-E&R and CFCS. Children with a given MACS level had a wide range of PMAL-R HO subscale score. In addition to MACS, a score on the PMAL-R HO subscale related to the more affected upper extremity should be included as an inclusion criterion in clinical trials to avoid misleading effects of intervention approaches aimed at improving the amount of use of the more affected upper extremity in children with congenital hemiplegic CP.

Activity-based hand-function profile in preschool children with obstetric brachial plexus palsy.

Obstetric brachial plexus palsy (OBPP) leads to various hand-function disorders. The present study aimed to determine activity-based hand function profiles in preschool children with different OBPP functional levels. 112 children aged 44-77 months were included, and grouped on the Narakas classification: group 1, Narakas type 1; group 2, type 2: and group 3, types 3 + 4. Hand function was evaluated on the Raimondi scale, stereognosis, and the elbow/forearm and wrist/finger/thumb sections of Brachial Plexus Outcome Measure (BPOM). Groups 1 and 2 successfully and comparably completed the BPOM hand activities (p > 0.05), while Group 1 had better results than Group 2 for BPOM elbow/forearm activities (p < 0.001): Group 2 had difficulty in activities involving elbow flexion, supination and pronation, whereas Group 1 had difficulty only in activities involving supination. Comparatively, Group 3 had lower scores for Raimondi scale (p < 0.001), BPOM-hand (p < 0.001), BPOM-elbow/forearm (p < 0.001) and stereognosis (p < 0.001). According to the literature, hand functions are conserved in upper-root brachial plexus injury and there is no need to evaluate them, but our results showed activity restrictions related to hand functions involving forearm rotation. In children with total plexus injury, grasp was absent and thumb function was deficient. The present showed that these restrictions can be identified by population-specific activity-based assessment.

Requirements for tele-health in children with obstetric brachial plexus palsy during Covid-19-like situations.

The purpose of the study was to investigate families' concerns and service requirements during the Covid-19 lockdown. In case of tele-consultation, we also aimed to assess the effects of this service on coping and the family's worries. At end of lockdown, we contacted the parents of 67 obstetric brachial plexus palsy patients (0-10 years age) by e-mail. During lockdown, 42 of the families had had a tele-consultation with our team, while 25 reported that not receiving any service. A questionnaire consisting of 6 questions was sent to the families, and data were analyzed according to 4 age-groups. Parents' concerns varied according to the children's age group (p = 0.001). All families replied that their children should receive remote services during Covid-19-like situations (p = 0.173). Parents of the 42 children who had tele-consultations reported that this had alleviated their worries, independently of age-group (p = 0.160). The usefulness of tele-consultation to manage the lockdown situation differed according to age-group (p = 0.002). The parents of under-3-year-olds experienced more worry during lockdown, but all respondents reported needing remote services. Although the tele-consultation alleviated the worries of almost all families, it was most useful in managing lockdown in families with under-3-year-olds.

Urinary complications of gynecologic surgery: iatrogenic urinary tract system injuries in obstetrics and gynecology operations.

OBJECTIVES: To evaluate iatrogenic urinary tract system injuries in obstetrics and gynecology operations and compare the results with the literature. PATIENTS AND METHODS: We examined the records of patients who had obstetric and gynecology operations at the Ministry of Health, Dr. Zekai Tahir Burak Women's Health, Training and Research Hospital between June 2007 and June 2010. All the patients who were diagnosed as having urinary system injuries in either the intraoperative or postoperative period were determined. RESULTS: During this period, 25,998 gynecologic and obstetrical operations were performed, 0.03% ureteric, 0.20% bladder, and one case of urethral injury, in a total of 0.24% urinary tract injuries were observed. The bladder was the most frequently injured organ. Total urinary tract injury rates were 0.79% (0.49% bladder, 0.24% ureteral) in gynecologic operations and 0.19% (0.18% bladder and 0.01% ureteral) in obstetric operations. CONCLUSION: Urinary system injuries are seen in approximately 1% of all gynecologic and obstetric surgeries. The complication rates observed in our patients were comparable with the other studies in the literature. A gynecologic surgeon must become familiar with the anatomy of the urinary tract and must be aware of common intraoperative and postoperative complications to decrease the risk of morbidity.

rac-Diethyl 9-hy-droxy-9-methyl-7-phenyl-1,4-diaza-spiro-[4.5]decane-6,8-dicarboxyl-ate.

The title mol-ecule, C(21)H(30)N(2)O(5), is chiral with four stereogenic centres. The crystal is a racemate and consists of enanti-omeric pairs with the relative configuration rac-(6S*,7R*,8R*,9S*). The ethyl fragment of the eth-oxy-carbonyl group at position 6 is disordered in a 0.46 (3):0.54 (3) ratio. The crystal structure features inter-molecular N-H⋯O. Intra-molecular O-H⋯N and N-H⋯O hydrogen bonds also occur.

The effects of hand deformity on upper-limb function and health-related quality of life in children with spastic hemiplegic cerebral palsy.

The aim of this study was to investigate the effect of hand deformity on upper-limb function and health-related quality of life (HRQOL) in children with hemiplegic cerebral palsy (CP). The study included 44 children with hemiplegic CP between the ages of 6 and 14 years (mean age, 10.04 years; SD, 3.1; 23 males, 21 females). The Manual Ability Classification System (MACS) and Gross Motor Function Classification System (GMFCS) were used, with the Zancolli classification to characterize hand deformities on the more affected side. Upper-limb function was assessed in terms of unilateral capacity (Quality of Upper Extremity Skills Test: QUEST) and bimanual performance (Children's Hand-use Experience Questionnaire: CHEQ), while HRQOL was evaluated on the KIDSCREEN-27 questionnaire. Comparison of bimanual performance and unilateral capacity in children with Zancolli level 1 and 2a hemiplegic CP found statistically significant differences (p < 0.01). There was also a significant difference on the HRQOL 'physical activities and health' subdomain, in favor of Zancolli level I deformity (p = 0.003), but not on the other HRQOL domains (p > 0.05). Upper-limb function and the HRQOL physical health domain were poorer with greater hand deformity in children with hemiplegic CP.

Human hypertension caused by mutations in WNK kinases.

Hypertension is a major public health problem of largely unknown cause. Here, we identify two genes causing pseudohypoaldosteronism type II, a Mendelian trait featuring hypertension, increased renal salt reabsorption, and impaired K+ and H+ excretion. Both genes encode members of the WNK family of serine-threonine kinases. Disease-causing mutations in WNK1 are large intronic deletions that increase WNK1 expression. The mutations in WNK4 are missense, which cluster in a short, highly conserved segment of the encoded protein. Both proteins localize to the distal nephron, a kidney segment involved in salt, K+, and pH homeostasis. WNK1 is cytoplasmic, whereas WNK4 localizes to tight junctions. The WNK kinases and their associated signaling pathway(s) may offer new targets for the development of antihypertensive drugs.

Azoospermia and cryptorchidism in a male with a de novo reciprocal t(Y;16) translocation.

An apparently balanced reciprocal translocation between the long arm of the Y chromosome and the long arm of the chromosome 16 t(Y;16)(q12;q13) is described in an infertile man with azoospermia and cryptorchidism. The patient was phenotypically normal and had bilateral inguinal hernia repair with orchidopexy at the age of 8 years. Histological examination of testicular biopsies revealed maturation arrest. Y/autosome translocations in the literature are relatively rare and mostly associated with infertility. To our knowledge, this is the sixth report about the reciprocal t(Y;16) translocation in the literature but the first presenting with cryptorchidism.

Sulbactam/ampicillin in the treatment of pediatric infections.

Seventy eight pediatric patients (43 males, 35 females) aged 34 days to 17 years were treated with intravenous or intramuscular sulbactam/ampicillin 3 or 4 times daily for skeletal system infection (10 cases), systemic salmonellosis (2 cases), intrathoracic infection (12 cases), and soft tissue or miscellaneous infections (54 cases). The dose used to treat the majority of patients was 200 mg of ampicillin plus 100 mg of sulbactam per kg/day. The duration of treatment ranged from 8 to 23 days. Sulbactam/ampicillin alone was used in 68 patients. Ten patients were treated with an additional antibacterial agent. The overall cure rate was 98.7% for all 78 study patients. One patient with an abscess in the neck was shown to be infected with a strain of Escherichia coli resistant to sulbactam/ampicillin. Only one patient experienced a rash, but it did not necessitate discontinuation of therapy. This study shows that sulbactam/ampicillin is a safe and effective agent in the treatment of various pediatric infections.

Vasoactive intestinal polypeptide and its receptor changes in human temporal lobe epilepsy.

The distribution of the VIP receptor in the human hippocampus was studied by receptor autoradiography using [3-iodotyrosyl-125I]Vasoactive Intestinal Peptide (VIP) as a ligand, and the relationship of receptor distribution to the distribution of the peptide (visualized by immunocytochemistry) was examined in hippocampi surgically removed from patients with medically intractable temporal lobe epilepsy (TLE) and hippocampi obtained at autopsy from neurologically normal subjects. In the autopsy hippocampi and hippocampi from TLE patients with extrahippocampal temporal lobe lesions [125I]VIP binding was highest in the dentate molecular layer, with lower levels in the fields of Ammon's Horn (CA fields) and the subiculum. In hippocampi from patients with no temporal lobe lesions but considerable hippocampal neuronal loss there were significant elevations in the levels of ligand binding in all CA fields and the subiculum. Ligand binding densities in all CA fields of the patient hippocampi were strongly negatively correlated with neuronal numbers. Immunocytochemical localization of VIP shows no obvious change in the distribution patters of VIP immunoreactivity in the patient groups. This is the first demonstration of VIP and its receptor distribution in the human hippocampus. It is suggested that the elevated levels of receptor binding in the hippocampal seizure focus may indicate a mechanism for greater excitability of neurons and/or for their survivability in the face of the increased excitation and potential for injury in a seizure focus.

Genetic heterogeneity of inherited cerebral cavernous malformation.

OBJECTIVE: Cerebral cavernous malformation (CCM) is frequently an inherited disorder showing autosomal dominant transmission. Genetic analysis has localized a gene causing CCM to a segment of the long arm of human chromosome 7 (7q). This evidence derives from investigation of a small number of families, mostly of Hispanic American descent. In this study, we have tested whether inherited CCM is always due to mutation in this 7q gene, or whether mutation in other genes can cause CCM. METHODS: We have studied subjects from two non-Hispanic families with inherited CCM. The clinical features of CCM in these families are indistinguishable from those in kindreds in which CCM is due to mutation in the 7q gene. To test whether CCM in these kindreds is caused by a mutation on 7q, we compared the inheritance of CCM to the inheritance of genetic markers on 7q. RESULTS: Genetic analysis demonstrates independent inheritance of CCM and markers on 7q in both families studied. This evidence excludes mutation in the 7q gene as the cause of CCM in these families, with odds against CCM being due to mutation in 7q in each family of more than 100,000:1 and 100:1, respectively. CONCLUSION: These findings demonstrate that inherited CCM is not always caused by a mutant gene on 7q, indicating the presence of at least a second gene in which mutation can cause CCM. These results have implications for genetic testing and the pathogenesis of this disorder.

Intraoperative endovascular treatment as an adjunct to microsurgical clipping of paraclinoid aneurysms.

OBJECT: The endovascular procedure can provide proximal control, suction decompression, and prompt intraoperative angiography during microsurgical clipping of aneurysms of the paraclinoid segment of the internal carotid artery (ICA). The authors assess the safety and feasibility of this method in 24 consecutive cases. METHODS: Frontotemporal craniotomy and radical pterionectomy were performed with the patient's head immobilized in a radiolucent frame while femoral artery catheterization was achieved. Before dural opening, a balloon catheter with a coaxial lumen was positioned and tested in the ICA, after which microsurgical exposure was completed, including intradural clinoid drilling and optic canal decompression. Trapping of the lesion was achieved by inflating the balloon and placing a temporary clip beyond the aneurysm neck. The catheter was gently aspirated to achieve suction decompression and to facilitate clip application. Intraoperative digital subtraction angiography was then performed. Twenty-two aneurysms were larger than 10 mm, and 11 of them were giant. Six patients presented with subarachnoid hemorrhage and nine with visual symptoms. Balloon occlusion and suction decompression were performed in 16 cases (67%), and proximal control alone in 1 case. Intraoperative angiography was performed in every case. Subsequent clip readjustment was necessary in seven cases, including three cases of residual aneurysm filling and four of ICA compromise. Complete aneurysm obliteration was achieved in 20 cases, and greater than 90% obliteration in 22. One major infarct likely related to catheter thromboembolism was found. There were no instances of visual deterioration or other complications attributable to the endovascular procedure. CONCLUSIONS: The endovascular method allows safe and reliable proximal control, suction decompression, and intraoperative angiography in microsurgical treatment of large paraclinoid aneurysms.

Carotid endarterectomy prevention strategies and complications management.

Perioperative complications of carotid endarterectomy (CEA) are uncommon but potentially devastating. The authors review strategies aimed at minimizing morbidity of surgical treatment of carotid occlusive disease. Multiple components of the perioperative course of patients who undergo CEA must be tightly controlled to maintain an acceptably low complication rate. These factors comprise appropriate patient selection, including careful assessment of techniques aimed at prevention and monitoring of intraoperative complications and postoperative care.

[A clinical trial with ampicillin and sulbactam in various infections]. / Cesitli enfeksiyonlarda ampisilin ve sulbaktamin klinik etkinligi.

Eighty four patients with various infections were treated with parenteral ampicillin and sulbactam. Twenty seven patients had meningitis five septic arthritis and osteomyelitis, two systemic salmonellosis, nine intrathoracic infections, five of which were complicated with pleural empyema; thirty patients had infection of the deep tissues of the neck, and the remaining eleven had soft tissue infection in different localizations. The clinical and microbiological results were interpreted together and the overall rate of success in treatment with ampicillin and sulbactam was found to be 98.8%.

Mapping a gene causing cerebral cavernous malformation to 7q11.2-q21.

Cerebral cavernous malformation is a common disease of the brain vasculature of unknown cause characterized by dilated thin-walled sinusoidal vessels (caverns); these lesions cause varying clinical presentations which include headache, seizure, and hemorrhagic stroke. This disorder is frequently familial, with autosomal dominant inheritance. Using a general linkage approach in two extended cavernous malformation kindreds, we have identified linkage of this trait to chromosome 7q11.2-q21. Multipoint linkage analysis yields a peak logarithm of odds (lod) score of 6.88 with zero recombination with locus D7S669 and localizes the gene to a 7-cM region in the interval between loci ELN and D7S802.