Efficacy of spironolactone in treating hidradenitis suppurativa in women of childbearing age: a single-center retrospective analysis.

Background: Hidradenitis suppurativa (HS) is a chronic skin disease characterized by recurrent nodules that affect areas with a high density of apocrine sweat glands, such as the axillae and groin. Androgens are implicated in the pathophysiology of HS. Therefore, spironolactone, an antiandrogen therapy, is recommended. However, data on its use in women of childbearing age are limited, especially since its antiandrogenic effects may affect menstruation, fertility, and pubertal development. Objective: To evaluate the efficacy and safety of spironolactone in the treatment of hidradenitis suppurativa in women of childbearing age and to identify factors associated with treatment response. Methods: A retrospective analysis was conducted on female patients aged 12 to 50 with HS treated with spironolactone at Michigan Medicine dermatology clinics from 2000 to 2021. The patients' demographic data, HS characteristics, and spironolactone responses were examined. Statistical assessments were performed to determine the efficacy indicators. Results: Of the 157 patients reviewed, 31 showed an improvement in treatment. Variables such as axillary involvement, previous treatment failures, and use of intralesional steroids were linked to a lack of improvement in spironolactone. Through adjusted multiple logistic regression analysis, a significant association was observed between improvement status and Hurley stage 3 (odds ratio = 0.15 [95% CI: 0.02-0.79], P = .036), suggesting that patients with Hurley stage 3 were 85% less likely to exhibit improvement in spironolactone therapy. Limitations: The study's retrospective nature and reliance on single-center data can limit generalizability. The sample size is limited and therefore affects the study's statistical power. Conclusion: Thus, spironolactone may offer therapeutic benefits for HS in women of childbearing age. However, patients with severe disease (Hurley stage 3) had reduced response rates. Further prospective studies are recommended to validate these findings and determine the most suitable patient profile for spironolactone therapy for HS.

Point-of-care blood clotting test and its correlation with fibrinogen level: Potential in goal-directed transfusion in postpartum hemorrhage.

OBJECTIVE: To study the correlation of conventional point-of-care clotting test (POCCT) of whole blood with laboratory fibrinogen levels in women with primary major postpartum hemorrhage (PPH) to generate evidence regarding its potential in rationalizing transfusion of blood components for supplementing fibrinogen. METHODS: A total of 68 samples were studied: 40 from women with primary major PPH, 20 from women without PPH, and eight samples of Fresh Frozen Plasma (FFP). POCCT was performed in the PPH and non-PPH groups and at the same time, sample for laboratory fibrinogen was sent. Values were correlated using Pearson's correlation coefficient (r). Depending upon the laboratory fibrinogen values, POCCT in the PPH group was divided into three subgroups of less than 7, 7-11, and more than >11 min as subgroups 1, 2, and 3, respectively. RESULTS: Women in the PPH group (n = 40) had a mean fibrinogen level of 346.15 ± 143.37 mg% with a significant negative correlation with POCCT (r = -0.69, P < 0.001). Mean fibrinogen levels of non-PPH group and FFP samples were 602.1 ± 169.72 and 286.75 ± 103.42 mg%, respectively. PPH subgroups 1, 2, and 3 had mean fibrinogen of 452.2 ± 141.8, 332.58 ± 138.51, and 158.71 ± 145 mg%, respectively. These values may potentially guide FFP transfusions. CONCLUSION: POCCT is easy, costs nothing and has a potential role in rationalizing FFP transfusion in low- and middle-income countries.

Early detection by magnetic resonance imaging of fetal cerebral damage in a fetus with hydrops and cytomegalovirus infection.

We discuss the use of magnetic resonance imaging (MRI) to reveal early fetal neurological involvement of cytomegalovirus (CMV) infection. A woman presented at 21 weeks of pregnancy with active CMV infection. Cerebral ultrasound examination had been normal. An MRI scan revealed a thickened germinal matrix, which was histologically confirmed, associated with underdevelopment of the gyri. Brain MRI proved particularly useful in identifying the findings not disclosed by routine ultrasound during pregnancy and subsequently confirmed at histology.

Efficacy and safety of nadroparin in the treatment of pregnant women with antiphospholipid syndrome: a prospective cohort study.

The optimal therapeutic management of patients with antiphospholipid syndrome (APS) during pregnancy is debatable. In the present prospective cohort study the use of a low molecular weight heparin (LMWH) (nadroparin), administered alone twice daily in 30 pregnant women who were diagnosed with APS on the basis of the current classification criteria, is evaluated. Dosage was adjusted according to anti-Xa levels in the patients as the pregnancies progressed. Three women, in whom an important gradual fall in platelet count in the first trimester did not respond to increased nadroparin doses, were shifted to a second-line treatment protocol. Fetal loss occurred in two of the 27 remaining women (7.40%), while 25 (92.59%) delivered 25 live infants, between the 32nd and 40th weeks of gestation. No fetal problems were registered during pregnancies, while maternal complications occurred in two of the 25 patients (8%). Moreover, there were no thrombotic events in any of the women during the study. Patient compliance was good and only minor side-effects were reported. The results of this study indicate that nadroparin alone is useful and safe in the management of pregnant patients with APS. However, in consideration of the good pregnancy outcome obtained in patients with only pregnancy morbidity when heparin and aspirin were used together, other studies comparing nadroparin twice daily with once daily plus Aspirin would be useful to ascertain which is more effective in these patients.

EXIT procedure in a twin gestation and review of the literature.

Prenatal diagnosis can show masses of the fetal neck, mouth, and face that can potentially cause respiratory distress at birth. To prevent such an emergency, the EXIT (ex utero intrapartum technique) is performed: it is the intrapartum intubation of the fetus at term while still connected to the placenta. The EXIT procedure was first performed in a case of cervical teratoma. Up to now a total of 34 cases are described, mostly cervical teratomas (13 cases), lymphangiomas (7), epignathus (3); babies' outcome has been successful in 25 of them, with one death related to the procedure. Among the reported cases we are aware of only one where EXIT was performed in a twin gestation, in which the normal twin was delivered first. In our case the normal fetus was posterior to the twin with cervical malformation, requiring us to work on the latter while the former was still in the uterus. After having safely secured the airway in twin A, twin B was prompt delivered with excellent general conditions. Our limited experience enlarges the possibility to perform this prenatal procedure even in "nonstandard" conditions, such as a twin gestation, and may prove useful to those who are going to deal with such issues.

[The EX-utero Intrapartum Technique (EXIT) procedure in Italy]. / Il parto ex-utero intrapartum technique (EXIT) in Italia.

Aim of the study was to present the first two Italian cases of C-section performed with the EXIT procedure (EX-utero Intrapartum Technique). Deliveries were performed at the Division of Obstetrics and Gynecology of the Hospital of Padua in cooperation with the Pediatric Surgery Department, both tertiary care centers. The first case was a twin with a huge neck mass (cystic hygroma) and the second a fetus with an oropharyngeal mass (epignathus). Airway patency could have been compromised at birth in both of them. EXIT procedure consists in securing the airway of the fetus partially delivered and still connected with the placenta. This technique leaves an intact feto-placental circulation and guarantees a normal fetal oxygenation while fetal airway patency is secured. Both the fetuses were successfully intubated and the C-section ended up in a short period of time without maternal and fetal complications. The EXIT technique, performed for the first time in 1989 and now in many centers abroad, can be considered a safe procedure as long as a multidisciplinary approach is carried out. The EXIT procedure is indicated whenever fetal airways can be compromised at birth, that is when oropharyngeal masses, laryngeal atresia, cystic hygroma and goiter are encountered during prenatal ultrasound.

Neonatal ovarian cysts: management and follow-up.

Twenty-seven neonatal ovarian cysts were diagnosed in utero during a 13-year period; 9 were complicated and 18 were initially simple, but 8/18 showed evidence of complications in utero or soon after birth, leaving only 10 simple cysts. One simple and 4 complicated cysts were surgically excised early in the study period; 2 simple cysts were treated by needle aspiration at birth because of their large size. The remaining 20 cases were initially managed conservatively. All simple cysts and 10/13 complicated cysts regressed completely within 12 months; 3 that failed to regress were surgically excised. Nineteen patients who did not undergo a laparotomy had ultrasonographic (US) examinations from 3 months to 9 years after birth. Echography showed both ovaries in all 9 girls who had simple cysts at birth and in 2 of the 10 with complications. In the other 8 complicated cases only one ovary was detected at follow-up. Pre- or post-natal aspiration of simple cysts 4 cm or more in diameter is recommended, while conservative management seems appropriate in the absence of symptoms for simple cysts of less than 4 cm and complicated cysts of any size tending to involute after birth.

Outcome of fifty-five newborns of antiphospholipid antibody-positive mothers treated with calcium heparin during pregnancy.

OBJECTIVE: The outcome of 55 infants born to 53 antiphospholipid antibody (aPL)-positive mothers treated during pregnancy with calcium heparin is described. METHODS: The clinical state of the children was evaluated immediately after delivery by a clinical examination, and a neonatological check-up was performed no later than 24 hours after birth. Neonates with problems were transferred to the neonatal intensive care unit. After their discharge from hospital the clinical state of the babies was followed by means of interviews with the pediatricians and mothers for a period varying between 1.33 and 5.66 years (mean 2.51 +/- 0.92 SD). RESULTS: The newborns comprised 30 females and 25 males, including 2 sets of twins, delivered between the 25th and 40th weeks of gestation (mean 36.69 +/- 2.91 SD). They had a mean birth weight of 2.828 g +/- 706.50 SD (range 800-4.000) and a mean Apgar score at 5 minutes of 9.60 +/- 0.68 SD (range 7-10). Soon after delivery, 12 children (21.81%) were admitted to the neonatal intensive care unit for periods varying between 2 and 120 days (mean 30.33 +/- 33.40 SD), after which the clinical course was normal. All of these neonates suffered from complications exclusively due to prematurity. Malformations and signs of thrombosis or other aPL-related disorders were not observed in any of the newborns. During the follow-up, none of the diseases suffered by the 55 children differed from those of the normal pediatric population; in particular, aPL-related manifestations were never observed. CONCLUSION: These data indicate the absence of aPL-related problems in the offspring of aPL-positive mothers treated during pregnancy with calcium heparin.

Oral iron chelating therapy. A single center interim report on deferiprone (L1) in thalassemia.

BACKGROUND AND OBJECTIVE: Deferiprone (L1) is a largely studied oral chelator in clinical setting, however, no definite conclusions concerning efficacy and toxicity still could be drawn. In an ongoing prospective trial with L1, we evaluated the efficacy and tolerance-toxicity in patients with thalassemia major previously treated by desferrioxamine (DFO); the specific aim of the study is to demonstrate that L1 could be an alternative to DFO in some patients with an acceptable toxicity. DESIGN AND METHODS: Sixty-nine patients over 13 years of age with poor compliance to DFO were considered for the study. The design included a liver biopsy before starting L1 in all patients in order to define liver siderosis either by histologic grading or by hepatic iron concentration (HIC); only patients with a minimum HIC of 4 mg/g dry weight entered the study. A repetition of the liver biopsy after one year of L1 was planned; further evaluations included serum ferritin, plasma iron, transferrin TIBC and iron urine excretion. L1 was given at 70 mg/kg/day in three divided doses. Toxicity was monitored either clinically or by controlling liver, kidney and marrow function by specific tests. Concerning clinical characteristics 52 patients showed hypogonadism (78%), 39 growth retardation (58%), 6 diabetes (9%), 4 cardiomyopathy (6%), 9 hypothyroidism (12%); 45 patients had chronic liver damage (65%). RESULTS: We focus this report on data collected in a group of 29 patients with a minimum follow-up of one year (14-33 months). The mean ferritin value was 3748 ng/mL (range: 200-10,000) and 2550 ng/mL (range: 80-14,500), before and while on L1 therapy, respectively (p = 0.001); the mean sideruria changed from 17.25 mg/dL (range: 5.4-50) to 20.98 mg/dL (range: 10-40), on DFO and L1, respectively (p = 0.078); the ratio between plasma iron (sideremia) and transferrin TIBC changed from 0.96 with DFO to 0.86 with L1 (0.014). A correlation with grade of liver siderosis and serum ferritin (p = 0.069) and iron urine excretion (p = 0.008) was recorded. The judgement of efficacy showed that L1 was effective (EF) in 9 patients, no assessable (UN) in 11 patients, not effective (NE) in 2 patients and with no advantages with respect to DFO in 7 patients. Liver biopsy was repeated in 20 patients showing a reduction of grade of liver siderosis and iron content in 7 patients. Clinical toxic effects of L1 were gastric intolerance (one patient), joint pain (three patients) and mild and temporary neutropenia (one patient). INTERPRETATION AND CONCLUSIONS: This preliminary experience shows that L1 is effective in several patients with thalassemia with poor compliance to DFO and to improve iron burden and iron excretion with generally minor side effects. L1 could be an alternative to DFO in some patients, however the recognition of neutropenia warrants a careful evaluation of patients and efforts finalized to early recognition of those to be addressed with this new and still experimental therapy.

Fetal gallstones: sonographic and clinical observations.

Fetal gallstones were visualized sonographically in two cases at 30 and 37 weeks' gestation; follow-up scans at 2 and 3 months, respectively, disclosed their spontaneous resolution. The hypotheses advanced to explain the formation of echogenic material in the fetal gallbladder are reviewed, and the clinical utility of postnatal long-term sonographic follow-up is stressed.

Spontaneous resolution of cystic hygroma in a 46,XX normal female.

We report a case of nuchal cystic hygroma with spontaneous resolution detected by ultrasound examination at 13 weeks' gestation. Fetal karyotype and amniotic fluid alpha-fetoprotein levels were normal. Extreme caution in evaluating this situation is stressed.

Prevalence of polycystic ovaries in women with acne.

The prevalence of polycystic ovaries was determined by pelvic ultrasound imaging in 119 women (mean age, 23.6 +/- 6.06 years; range, 14-45 years) with acne but with no menstrual disorders, obesity, or hirsutism, and in 35 healthy controls (mean age, 25 +/- 5.8 years; range, 21-40 years). Polycystic ovaries were found in 54 out of 119 patients with acne (45.37%) and in 6 out of 35 controls (17.14%). The results of this study indicate that polycystic ovaries are common in women with acne and not necessarily associated with menstrual disorders, obesity, or hirsutism.

[The phenomenon of a permanent spontaneous articular vacuum (or anti-vacuum effect) in neonatology]. / Il fenomeno del "vacuum" articolare (o effetto antivacuum) spontaneo, duraturo in neonatologia.

Natural permanent pneumarthrography (NPP) is due to entrance of gases in-to the joint spaces following a sudden increase in intra articular volume and lowering of intra articular pressure by sudden stresses. A case of N.P.P. of the hip occurred in one of two stillborn twins (breech delivery); the causes of death were some amniotic bridles of the cord with consequent stop in the blood flow.

[Precurarization using pancuronium in male subjects. Evaluation of certain clinical effects]. / La précurarisation par pancuronium chez le sujet de sexe masculin. Evaluation de certains effets cliniques.

The authors studied certain effects in the male subject of precurarisation using different doses of pancuronium. The results obtained show that the maximum doses used, corresponding to 0.01 Omg/kg of pancuronium, were associated with subjective symptoms which were well tolerated by the conscious patient, a decreases in the incidence of fasciculations, antagonistic effects against succinylcholine and a decreases in postoperative myalgia. In their conclusion, the authors confirm that the maximum doses of pancuronium used require an increase in doses of succinylcholine in order to avoid the antagonism phenomenon, and that doses ranging from 0.010 to 0.020 mg/kg of pancuronium could be used in order to completely eliminate postoperative myalgia related to succinylcholine.