Developmental epileptic encephalopathy in DLG4-related synaptopathy.

Objective: The postsynaptic density protein of excitatory neurons PSD-95 is encoded by discs large MAGUK scaffold protein 4 (DLG4), de novo pathogenic variants of which lead to DLG4-related synaptopathy. The major clinical features are developmental delay, intellectual disability (ID), hypotonia, sleep disturbances, movement disorders, and epilepsy. Even though epilepsy is present in 50% of the individuals, it has not been investigated in detail.

Pre-notifications increase retention in a 17-year follow-up of adolescents born very preterm.

Objective: Retention is essential in follow-up studies to reduce missing data, which can cause bias and limit the generalizability of the results. We investigated whether pre-notification letters would increase the response rates of approval forms and questionnaires and reduce the need for post-notifications in a prospective follow-up study of 17-year-old adolescents.

Study Design: and settings This long-term follow-up study included 269 adolescents were randomized (1:1) into a pre-notification group (n = 132) and a no pre-notification group (n = 137).

Motor Performance in Association with Perceived Loneliness and Social Competence in 11-Year-Old Children Born Very Preterm.

Background: Very preterm birth may affect motor performance and social competence up to adulthood. Our objective was to describe perceived loneliness and social competence in children born very preterm in relation to motor impairment. Methods: 165 children born very preterm (birth weight ≤ 1500 g and/or gestational age < 32 weeks) were assessed at 11 years of age.

Modeling the Impact of Newcastle Disease Virus Vaccinations on Chicken Production Systems in Northeastern Madagascar.

Chickens are a key source of nutrition for rural Malagasy communities. Due to high endemic rates of Newcastle disease, it remains challenging to raise sustainable chicken flocks as a consistent food source. Here, we explore the impact of triannual Newcastle disease virus (NDV) vaccine interventions on the growth and herd immunity acquisition of Malagasy chicken flocks.

Pronator teres selective neurectomy in children with cerebral palsy.

Unlabelled: The objective of this study was to evaluate the results after selective pronator teres (PT) neurectomy in children with spastic hemiplegia. Patients with PT spasticity without contracture and an active supination improvement after PT botulinum toxin injection were included. Hand function and deformities were evaluated with the House score, Gschwind and Tonkin pronation deformity classification and Zancolli's classification.

Does the PFNA™ nail limit impaction in unstable intertrochanteric femoral fracture? A 115 case-control series.

Introduction: Femoral neck shortening after dynamic fixation of extra-capsular fracture may impair functional results, but is rarely assessed. The present study measured impaction in stable and unstable fractures (on the Ender classification) treated by PFNA™ nail. The objectives were: 1) to validate the Ender classification to assess fracture stability; 2) to determine whether neck shortening and head purchase quality varied with stability; and 3) to determine the functional impact of femoral neck shortening.

Algorithm for Quantifying Frontal EMG Responsiveness for Sedation Monitoring.

Introduction: To study stimulation-related facial electromyographic (FEMG) activity in intensive care unit (ICU) patients, develop an algorithm for quantifying the FEMG activity, and to optimize the algorithm for monitoring the sedation state of ICU patients.

Methods: First, the characteristics of FEMG response patterns related to vocal stimulation of 17 ICU patients were studied. Second, we collected continuous FEMG data from 30 ICU patients.

Conjunctival inflammatory cells and their predictive role for deep sclerectomy in primary open-angle glaucoma and exfoliation glaucoma.

Purpose: To investigate the conjunctival inflammatory alterations of patients with primary open-angle glaucoma (POAG) and exfoliation glaucoma (ExG) and correlate the findings with the success of deep sclerectomy (DS) surgery and with the patients' medical history.

Methods: Altogether 25 POAG and ExG patients of the prospective DS study were divided, based on the diagnosis and success of the operation, into 4 groups, POAG S (success), POAG F (failure), ExG S, and ExG F. Controls were obtained from other ophthalmologic surgery patients who did not have glaucoma, and their conjunctiva was examined to be normal.

Early laparoscopic fundoplication and gastrostomy in infants with spinal muscular atrophy type I.

Background/purpose: Spinal muscular atrophy (SMA) in children leads to progressive muscle weakness, dysphagia, aspiration, and death. We hypothesized that early laparoscopic fundoplication and gastrostomy in infants with SMA type I could be performed safely perhaps leading to fewer aspiration events and improved nutritional status.

Methods: Children diagnosed with SMA type I from 2002 through 2005 were included (n = 12).

Phospholipases A2 in normal human conjunctiva and from patients with primary open-angle glaucoma and exfoliation glaucoma.

Background: Chronic situations like long-term use of topical medications induces conjunctival inflammation and is also a significant risk factor for failure of filtering surgery. We evaluated conjunctival expression of group IIA secretory PLA(2) (sPLA(2)-IIA), group V secretory PLA(2) (sPLA(2)-V), calcium-independent PLA(2) (iPLA(2)) and cytosolic PLA(2) (cPLA(2)).

Methods: Samples were obtained from non-glaucomatous patients (control subjects), and patients with either primary open-angle glaucoma (POAG) or exfoliation glaucoma (ExG).

Acute diverticulitis after Duhamel-Martin procedure for total colonic Hirschsprung's disease.

A 28-year-old man with a history of total colonic Hirschsprung's disease treated with a Duhamel-Martin procedure in infancy, presented with signs and symptoms of acute colonic diverticulitis. Flexible sigmoidoscopy demonstrated a 20 cm jejunocolonic anastomosis, consistent with his previous operation in childhood, with a large diverticulum at the proximal end of the anastomosis containing fecal concretions. The patient returned one month later after a course of antibiotics for definitive resection of the diverticulum.

Time-resolved detection of hot electron-induced electrochemiluminescence of fluorescein in aqueous solution.

Strong electrogenerated chemiluminescence (ECL) of fluorescein is generated during cathodic pulse polarization of oxide-covered aluminum electrodes and the resulting decay of emission is so sluggish that time-resolved detection of fluorescein is feasible. The present ECL in aqueous solution is based on the tunnel emission of hot electrons into the aqueous electrolyte solution, which probably results in the generation of hydrated electrons and hydroxyl radicals acting as redox mediators. The successive one-electron redox steps with the primary radicals result in fluorescein in its lowest excited singlet state.

Perforation of the appendix from intestinal mucormycosis in a neonate.

A premature neonate had pneumoperitoneum 5 days after discontinuation of extracorporeal membrane oxygenation therapy. A perforated appendix was found at exploratory laparotomy. Pathologic examination of the appendix found mucormycosis.

Efficacy and tolerability of isradipine and metoprolol in treatment of hypertension: the Finnish Isradipine Study in Hypertension (FISH).

Eight hundred seventy-six men and women with diastolic blood pressure (DBP) of 95-115 mm Hg during a 4-week placebo period were included in a multicenter trial; 479 patients had previously been treated for hypertension. The patients were randomized to receive isradipine or metoprolol; both groups were comparable for age, weight, height, smoking habits, and duration of hypertension. By the end of the placebo period, 79 patients did not fulfill the final entry criteria and were withdrawn.

Clinical outcome and left ventricular function 23 years after acute coxsackie virus myopericarditis.

The clinical outcome of 18 patients, who presented with a Coxsackie virus myopericarditis in 1965, was evaluated 23 years later. Five patients had died, two of them had had heart failure. Thirteen patients and 23 healthy control subjects underwent exercise testing with gas exchange analysis.

Imprint cytology in immunocytochemical analysis of oestrogen and progesterone receptors of breast carcinoma.

Cytological imprint material from 26 mammary carcinomas was stained with monoclonal antibodies to oestrogen and progesterone receptors in an immunoperoxidase procedure. The staining result was compared with that of parallel stainings of frozen tissue sections of the same tumours. The peroxidase reactions in both techniques were semiquantitatively assessed (histoscore).

Proliferative activity and steroid receptors determined by immunohistochemistry in adjacent frozen sections of 102 breast carcinomas.

Adjacent frozen sections of 102 consecutive female breast carcinomas were examined for the expression of the Ki-67 antibody-reactive proliferation-associated nuclear antigen and of estrogen and progesterone receptors with the use of monoclonal antibodies and peroxidase histochemistry. The results of steroid receptor stainings were semiquantitatively assessed (histoscore) on the basis of nuclear staining intensity and the percentage of positively stained carcinoma cell nuclei. Carcinomas negative for either receptor had significantly higher percentages of Ki-67-positive cells.

Clinical characteristics and coronary anatomy in refractory unstable angina pectoris leading to coronary artery bypass grafting. The Kuopio experience.

One hundred patients with high-risk unstable and medication-resistant angina pectoris underwent coronary artery by-pass grafting. In 35 cases the angina was of early post-infarction type, in 60 it was progressive after previous stability and in five it was of recent onset. All had abnormal ECG in association with anginal attacks (ST depression in 76, ST elevation in 5, T-wave inversion in 15 and left bundle branch block in 4).