Presentation and non-surgical endodontic treatment of two patients with X-linked hypophosphatemia: a case report.

AIM: To describe two patients with X-linked hypophosphatemia presenting with spontaneous signs of pulpal necrosis in multiple intact teeth. The presentation and management are discussed, along with the diagnostic and endodontic treatment challenges. SUMMARY: Two young male patients with X-linked hypophosphatemia were referred to the Department of Restorative Dentistry at the Edinburgh Dental Institute, UK for management of dental infection. Both patients were referred due to their unusual clinical presentation and abnormal root canal morphology. They subsequently presented on multiple occasions with pain or sinus tracts over a 3- and 5-year period whilst under care. Clinical examination revealed intact teeth with buccal swellings, draining buccal sinuses and negative responses to pulp sensibility testing. Radiographic examination, generally consisting of periapical radiographs, revealed intact teeth with a range of unusual morphological features including large pulp chambers, wide canals, short roots and open apices, all with associated periapical radiolucencies. Due to the unusual morphology, some teeth required apexification with a mineral trioxide aggregate plug. Patient 1 underwent root canal treatment on teeth 21 and 43 over a 3-year period. Patient 2 underwent root canal treatment on 10 permanent teeth over a 5-year period. At follow-up, both patients were asymptomatic and clinically the teeth had no signs of infection or periapical inflammation. Radiographic examination confirmed complete resolution of the apical radiolucencies on 11 out of 12 teeth. Favourable outcomes have been demonstrated up to a follow-up of 4.5 years. KEY LEARNING POINTS: Patients with X-linked hypophosphatemia may present with 'spontaneous' signs of pulp necrosis in multiple teeth in the absence of caries and trauma posing a diagnostic challenge. Abnormal morphological features, including wide canals and open apices, may present challenges during root canal treatment. Outcomes demonstrate that an appropriate root canal treatment protocol, including the application of apexification procedures, may be implemented to successfully manage such cases.

Prevalence, predictive factors and clinical course of persistent pain associated with teeth displaying periapical healing following nonsurgical root canal treatment: a prospective study.

AIMS: To investigate the prevalence, pain catastrophizing and other predictive factors and clinical course of persistent pain/discomfort associated with teeth displaying periapical healing following nonsurgical root canal treatment (NSRCT). METHODOLOGY: One hundred and ninety-eight patients (264 teeth) who had NSRCT were reviewed at 5-14 months, postoperatively. Teeth with persistent post-treatment pain or discomfort, plus evidence of periapical healing were further monitored 0.5, 4 and 10 years later. Pain Catastrophizing Scale (PCS) and Short Form of the McGill Pain Questionnaire (SF-MPQ) were completed. Predictive factors were investigated using logistic regression models. RESULTS: Twenty-four per cent (60/249) of teeth displaying periapical healing at first review were associated with persistent pain or discomfort. Fifty-five teeth monitored 6-7 months later were associated with reduction in pain (17/30) or discomfort (7/25). Cone beam computed tomography (CBCT) of eight teeth with persistent symptoms and complete periapical healing (by conventional radiographs) revealed distinct, small apical radiolucencies (n = 3) or root apex fenestration through the buccal plate (n = 2). History of chronic pain (headache, temporo-mandibular joint, masticatory muscle, neck, shoulder or back pain; P = 0.005), preoperative pain (P = 0.04), responsive pulp (P = 0.009), tooth crack (P = 0.05) and small periapical radiolucency (P = 0.005) were significant predictive factors. The PCS revealed 16 patients (22 teeth) were catastrophizers (PCS ≥ 30), but this had no influence on post-treatment symptoms (P = 0.5). CONCLUSIONS: Persistent pain or discomfort associated with teeth showing periapical healing at the first review after NSRCT, decreased in intensity in most cases over the following 6 months. Longer-term follow-up revealed spontaneous improvement or symptom resolution in the majority of those with confirmed radiographic the absence of periapical disease. Five predictive factors (history of chronic pain, teeth with responsive pulps, association with pain, diagnosis of tooth crack before treatment and diameter of preoperative radiolucency) were identified.

Gait disturbance in Alzheimer's disease: a clinical study.

Gait disturbance is common in patients with Alzheimer's disease (AD). The aim of this study was to analyse the clinical gait syndromes of patients with AD using Nutt's classification. Fifty-five patients who satisfied the NINCDS-ADRDA criteria for probable AD and 55 controls were recruited from a geriatric and a psychogeriatric unit. Patients with classical musculoskeletal or neurological syndromes causing gait disturbance were excluded. A standardized neurological examination was carried out in all subjects. Twenty-two (40%) patients and ten (18%) controls had a higher level gait disorder (p < 0.01). The pattern of gait disturbance in AD patients varied according to the stage of the disease. Cautious gait was the commonest gait disorder in AD patients with mild dementia, while frontal gait disorder was the commonest disturbance in patients with severe dementia. The prevalence of frontal release signs (gegenhalten or any primitive reflex) was highest among patients with frontal gait disorder.

Intrauterine pressure studies in multigravid patients in spontaneous labour: effect of oxytocin augmentation in delayed first stage.

Uterine activity was assessed by measuring the uterine activity integral (UAI) in 116 black multigravid patients in spontaneous active labour at term. Although individual patients showed a wide range of mean UAI levels, normal labour (group I, n = 54) was associated with a mean UAI level of 1640 kPas/15 min which was higher than the mean level observed in patients in whom delay in cervical dilatation occurred in active labour. The patients who failed to progress in labour were treated with oxytocin infusion and 32 of them made good progress in labour and achieved vaginal delivery (group IIa: mean UAI pre-oxytocin treatment 1040 (SD 424) kPas/15 min, post-oxytocin 1890 (SD 559) kPas/15 min). The other 23 patients required operative delivery (group IIb) despite correction of uterine activity after oxytocin treatment (pre-oxytocin mean UAI 1230 (SD 570) kPas/15 min, post-oxytocin 1815 (SD 650) kPas/15 min). The rate of oxytocin infusion varied between patients from 2 to 16 mU/min but in 75% uterine activity was corrected to normal levels by a dose of less than or equal to 8 mU/min. It is concluded that delay in progress in labour is associated with lower intrauterine pressures than are present in normal labour, and that management of patients presenting with inefficient uterine action may benefit from the use of uterine activity integral measurements.

Comparison of labetalol and dihydralazine in hypertensive emergencies of pregnancy.

Labetalol (Trandate; Allen & Hanburys), a combined alpha- and beta-adrenergic blocking agent, was compared with the more commonly used peripheral vasodilator, dihydrallazine (Nepresol; Ciba), each administered as an infusion, in the treatment of severe hypertension in 20 primigravidas at greater than or equal to 32 weeks' gestation. With the dosage regimen used in this study there was a tendency towards more effective blood pressure control with dihydrallazine. The pulse rate was unaffected by labetalol therapy and there were no harmful effects on the neonate or fetus directly attributable to either drug.

Changes in amniotic fluid prostaglandins with oxytocin-induced labor.

Amniotic fluid prostaglandin levels were measured serially in 15 patients who underwent successful induction of labor and compared with those of patients presenting in spontaneous labor. At comparable cervical dilation the induced group demonstrated significantly lower prostaglandin levels. Four of these patients delivered without any increment in prostaglandins while in the remaining patients increases in prostaglandins followed the attainment of efficient uterine contractions by several hours. These data support the hypothesis that oxytocin acts directly on myometrial cells and not primarily by prior generation of prostaglandin synthesis in the membranes.

Platelet alpha 2-adrenoreceptor binding in elderly depressed patients.

Specific binding of 3H-clonidine to platelet membranes was measured in depressed elderly patients and in an elderly control group. Maximum specific binding was significantly higher in depressed patients than in the control group, whereas the binding affinity was not significantly different.

Rupture of the gravid uterus.

A detailed analysis of 129 cases of rupture of the gravid uterus between 1980 and 1983 is presented and compared with two previous reports from King Edward VIII Hospital, Durban, over the last 2 decades. Statistically significant decrease in the occurrence of this condition (from 2,7 to 1,06/1000 deliveries (P less than 0,001; chi 2 = 20,38)) and in the maternal mortality rate (from 12% to 3%) have taken place since the initial study. The most probable reasons for this improvement are the provision of a community-based obstetric service, the early detection of cephalopelvic disproportion, awareness of the condition, immediate and adequate resuscitation once the diagnosis is established, and the performance of a total abdominal hysterectomy as definitive treatment in most cases.

Abnormal concentrations of prostaglandins in amniotic fluid during delayed labour in multigravid patients.

Concentrations of prostaglandins E (PGE), F2 alpha (PGF), 13,14-dihydro-15-keto prostaglandin F2 alpha (PGFM), 6-keto F1 alpha and thromboxane B2 were measured by specific radioimmunoassay in samples of amniotic fluid from 22 multigravid patients during labour. Normal labour in 10 patients was associated with a significant increase of PGE, PGF and PGFM with close correlation to cervical dilatation (P less than 0.05). In the 12 patients with clinically delayed labour, in the absence of cephalopelvic disproportion, there were significantly lower PGF (P less than 0.002) and PGFM (P less than 0.05) concentrations obtained while no differences were observed in the other prostanoids measured. Administration of oxytocin to the latter group to enhance labour did not have any effect on the concentrations of prostaglandins obtained in spite of an improvement in intrauterine pressures and accelerated progress of labour.

Obstructed labour.

PIP: The major cause of obstructed labor is cephalopelvic disproportion, which may be due to a small pelvis, a large baby, fetal malpresentation, a tight perineum, or abnormalities or tumors of the uterus, ovary, or vagina. Prior to obstruction in primigravidae the rate of cervical dilation in the active 1st phase of labor slows to less than 1 cm/hr. If intervention is not done, fetal anoxia, pressure necrosis, fetal death, and vesicovaginal fistula will occur. In multigravidae the cervix may achieve full dilation, but the fetus fails to descend in the birth canal, and the uterine contractions continue until the uterus ruptures. The primary treatment for obstructed labor is prevention through adequate prenatal care. In communities without hospital facilities, midwife-run satellite clinics with adequate transport for referrals are the next best option. Another option is the provision of mothers' waiting areas near the clinic or hospital, where expectant mothers can receive intrapartum supervision. Labor should be monitored with a partogram, so that abnormal cervical dilation can be detected early, and the patient can be transferred to hospital. Once there, the patient will require immediate parental fluids and antibiotics before surgery. If an anesthetist is not available, the surgeon should administer an epidural block. A stomach tube and antacid premedication should be used to prevent aspiration of gastric contents. The surgeon must decide on the most apposite mode of delivery: episiotomy, ventouse and forceps delivery, symphysiotomy, or cesarean section. Various cesarean section methods may be appropriate: extraperitoneal cesarean section when there is intrauterine infection in cephalic presentation, De Lee incision where there is transverse cephalic presentation or an uncorrectable constriction ring, or lower segment transverse incision. If the fetus is dead and the uterus has not ruptured, vaginal destruction of the fetus is preferable to cesarean section. If the uterus has ruptured, cesarean hysterectomy is the recommended action. Cesarean section is the recommended procedure for vertex, brow, and transverse presentations if the fetus is alive. A live fetus with hydrocephaly may be delivered vaginally after the head is drained, but symphysiotomy may be required.^ieng

Intrauterine-pressure studies in African nulliparae: normal labour progress.

Uterine activity was measured in 29 nulliparous African women, who were in the active phase of spontaneous labour, and who were delivered vaginally without supplementary oxytocin. A fluid-filled intrauterine catheter, an external strain gauge and a pressure-curve integrator were used to quantitate uterine activity. The rate of dilatation of the cervix from entry to the study to full dilatation of the cervix was greater than 1 cm/h; this constituted normal labour progress. In nulliparous African women the minimum level of uterine activity likely to be associated with a cervical dilatation rate of 1 cm/h was 1200 kPas/15 min and the median uterine activity level in the active phase of normal labour was 1824 kPas/15 min.

Intrauterine-pressure studies in African nulliparae: delay, delivery and disproportion.

Uterine activity was measured in 43 African nulliparae who were in the active phase of spontaneous labour and in whom delay in cervical dilatation had occurred. Details of labour were recorded on a partogramme, on which an alert and an action line had been drawn, and which were 2 h apart. Delay was defined as a cervical dilatation rate of less than 1 cm/h. Uterine activity was measured with a fluid-filled intrauterine catheter, an external strain gauge and a pressure-curve integrator. The uterine activity levels which were observed in association with delay are considerably less than those associated with normal labour progress. The uterine activity levels that were associated with pitocin augmentation after the action line and which resulted in vaginal delivery have been analysed. They were similar to the levels observed in normal labour. The uterine activity levels which were associated with 'failure to progress in labour' beyond the action line have also been analysed. It is argued that a minimum uterine activity level of 1800 kPas/15 min is required to fully test the cephalopelvic relation.