ACQUIRED NEONATAL SEVERE FACTOR II DEFICIENCY- TWO CASES REPORT

In present study two cases of transient acquired and isolated factor II deficiency associated with severe bleeding are reported. Two infants were involved in severe coagulopathy. The blood clot time (CT) in case 2 was excessively prolonged over 16 hours. One-stage prothrombin time (PT) was remarkable prolonged. Haemostatic markers analysis showed an isolated deficiency of factor II at 2.5% and 4.5% respectively. No inhibitory activity against factor II could be detected. We successfully treated the deficiency with vitamin K1 during 15 days. It was interesting that in the case 2 female baby the cause of vitamin K deficiency might be breast feed problem (nutrition deficiency) and/or poor absorption from bowel. Physiopathological laboratory results and therapeutic aspects of two patients were presented. Peer Review History: Received: 1 November 2021; Revised: 9 December; Accepted: 28 December, Available online: 15 January 2022 Academic Editor: Dr. Sally A. El-Zahaby, Pharos University in Alexandria, Egypt, [email protected] UJPR follows the most transparent and toughest ‘Advanced OPEN peer review’ system. The identity of the authors and, reviewers will be known to each other. This transparent process will help to eradicate any possible malicious/purposeful interference by any person (publishing staff, reviewer, editor, author, etc) during peer review. As a result of this unique system, all reviewers will get their due recognition and respect, once their names are published in the papers. We expect that, by publishing peer review reports with published papers, will be helpful to many authors for drafting their article according to the specifications. Auhors will remove any error of their article and they will improve their article(s) according to the previous reports displayed with published article(s). The main purpose of it is ‘to improve the quality of a candidate manuscript’. Our reviewers check the ‘strength and weakness of a manuscript honestly’. There will increase in the perfection, and transparency. Received file: Reviewer's Comments: Average Peer review marks at initial stage: 6.0/10 Average Peer review marks at publication stage: 7.0/10 Reviewers: Prof. Dr. Hassan A.H. Al-Shamahy, Sana'a University, Yemen, [email protected] Dr. Ogbonna B. Onyebuchi, Nnamdi Azikiwe University, Awka, Nigeria, [email protected]

Prenatal predisposing factors associated with neonatal lupus erythematosus

Objectives To identify the prenatal predisposing factors related to neonatal lupus erythematosus (NLE). Materials and Methods A retrospective case-control study was made of 131 pregnant women with positive anti-Ro or anti-La autoantibodies and known neonatal outcomes between January 2002 and December 2019 at Siriraj Hospital, Bangkok, Thailand. There were 101 unaffected neonates and 30 NLE cases confirmed postnatally. Demographic and clinical data of the mothers and neonates with and without NLE were statistically compared. Results NLE was diagnosed in 30 out of 131 cases. A multivariate analysis identified the following significant factors for NLE: maternal anti-La antibodies (odds ratio (OR), 3.591; p = 0.030); and maternal treatment with either hydroxychloroquine (OR, 0.082; p = 0.001) or prednisolone (OR, 0.136; p = 0.017). Of the significant variables examined in the multivariate analysis models, high levels of maternal anti-La antibodies were found to be the strongest predictor of noncardiac NLE (OR, 4.558; p = 0.032), while a female baby was significantly higher in pregnancies complicated by cardiac NLE (OR, 5.374; p = 0.046). Hydroxychloroquine still provided a protective effect for both cardiac and noncardiac NLE ( p = 0.039 and 0.032, respectively). Conclusions The maternal anti-La antibodies were a beneficial predictor for NLE, especially as their high titers were influentially associated with noncardiac features. A female fetus seemed to present an increased risk for developing a congenital heart block. Nevertheless, the treatment with hydroxychloroquine during the pregnancies demonstrated a potentially protective factor against both cardiac and noncardiac manifestations.

Mesenteric cystic lymphangioma – A rare case report; National Center for Pediatric Surgery, Gezira State, Sudan; 2021

Cystic lymphangioma is a benign congenital malformation of the lymphatic vessels. At the National Center for Pediatric Surgery, Gezira State, Sudan; 2021; a female baby of 15 months old age presented complaining of abdominal distention of 10 days duration. Patient Looks unwell, not febrile, and not pale or jaundice; abdomen was distended with full flanks, there was a palpable mass in the epigastric region, no area of tenderness and no palpable organs. Abdomino-pelvic U/S showed very huge abdomino –pelvic cystic mass with multiple thick septa. CT abdomen showed huge abdomino-pelvic mass with multiple cysts that having variable sizes measuring up to 6*7 cm. An elective explorative laparotomy was done. Intra operative findings: Multiple cystic masses arise from the root of the small bowel mesentery matted together with multiple enlarged mesenteric lymph nodes involving segment of distal jejunum with multiple adhesions. Release of adhesion, resection of the masses along with 57 cm of the involving jejunum, anastomosis was done, and homeostasis was secured. Histopathology result showed dilated cystic spaces; lined by flat endothelial cells and multiple reactive lymph nodes, no evidence of malignancy. Features are in line with lymphangioma and associated mesenteric lymphadenitis. Discussion and Conclusion: Our case was 15 months old which is the age of presentation as mentioned internationally. Mesenteric cystic Lymphangiomas are more common among male unlike our case. Ultrasonography and CT abdomen were highly recommended to achieve early diagnosis and treatment of such cases.

Case Report on Sacroccygeal (Teratomas) Germ Cell Tumor

Sacrococcygeal teratomas are a type of germ cell tumour (GCTs) accounting for 40% of all GCTs of all GCT,s in children. Interestingly 75% occurs in females. Reporting here a case of 3 years old female baby hospitalized for surgical excision of sacrococcygeal teratoma. A female (3-year-old) patient was referred to the pediatric outpatient department at Acharya Vinoba Bhave Rural Hospital Wardha on 19th May 2021. Complaining of swelling on right gluteal region Since 6 month and swelling is increases day by day, pain in back side, fever (Temperature - 101˚F) and tenderness in back side, leg weakness, constipation and she was admitted to Pediatric ward. Surgical excision of sacrocogygeal teratoma was done during her hospitalization. She received one cycle of chemotherapy with antineoplastic medicines. The client discharged after 10 days stay in hospital.

Conjoined twins in dichorionic diamniotic triplet pregnancy: a report of three cases and literature review

Background Conjoined twins are a rare and serious complication of monochorionic twins. The total incidence is 1.5 per 100,000 births, and about 50% are liveborn. Prenatal screening and diagnosis of conjoined twins is usually performed by ultrasonography. Magnetic resonance imaging can be used to assist in the diagnosis if necessary. Conjoined twins in dichorionic diamniotic triplet pregnancy are extremely rare. Case presentation We reported three cases of dichorionic diamniotic triplet pregnancy with conjoined twins. Due to the poor prognosis of conjoined twins evaluated by multidisciplinary teams, selective termination of conjoined twins was performed in three cases. In case 1, selective reduction of the conjoined twins was performed at 16 gestational weeks, and a healthy female baby weighing 3270 g was delivered at 37 weeks. In case 2, the conjoined twins were selectively terminated at 17 weeks of gestation, and a healthy female baby weighing 2760 g was delivered at 37 weeks and 4 days. In case 3, the conjoined twins were selectively terminated at 15 weeks and 2 days, and a healthy female baby weighing 2450 g was delivered at 33 weeks and 6 days. The babies of all three cases were followed up and are in good health. Conclusion(s) Surgical separation is the only treatment for conjoined twins after birth. Early determination of chorionicity and antenatal diagnosis of conjoined twins in triplet gestations are critical for individualized management options and the prognosis of normal triplets. Expecting parents should be extensively counseled by multidisciplinary teams. If there are limitations in successful separation after birth, early selective termination of the conjoined twins by intrathoracic injection of potassium chloride may be a procedure in dichorionic diamniotic triplet pregnancy to improve perinatal outcomes of the normal triplet.

A rare case of recurrent paroxysmal supraventricular tachycardia in pregnancy managed with adenosine, a wonder drug

Paroxysmal supraventricular tachycardia (PSVT) is the most common sustained arrhythmia during pregnancy and a challenging situation due to lack of evidence based guidelines. About 50% of PSVT, who fail to respond to vagal maneuvres, responds to therapies as pharmacologic agents as adenosine and electrocardioversion. We reported a case of 29 years old primigravida women with no organic heart disease who presented at 21 weeks of period of gestation with complaints of palpitations and shortness of breath. Her ECG revealed PSVT for which she received adenosine as anti-arrhythmic for conversion to sinus rhythm. She was started prophylactically on tablet metoprolol 25 mg twice daily, as advised by cardiologist. In third trimester, she had recurrent episodes of PSVT for which she received adenosine in emergency department. She delivered a healthy female baby by an elective caesarean section done under spinal anesthesia. Fortunately, her intraoperative and postpartum was uneventful with no recurrence of PSVT during hospital stay. She was discharged on day 4 of caesarean section on tablet metoprolol 12.5 mg twice daily and followed in postpartum period for complications. To summarize, PSVT occurring during pregnancy, labour or at caesarean section is not uncommon. Treatment remains a challenge though, as clinical decision must be tackled with appropriate consideration of both maternal and fetal factors. So, multi-disciplinary approach is needed for treatment including obstetrician, cardiologists, physician and neonatologists. Our case highlighted the necessity of keeping anti-arrhythmic drugs such as adenosine readily available on the labour ward.

Successful Outcome Following Hysteroscopic Septoplasty in a Woman with Seven Consecutive Pregnancy Losses

Anomalies of the reproductive tract are common and present in about 3%–5% of the general population and in about 3% of infertile women. In general, they are asymptomatic, but they can also be associated with recurrent abortions or infertility. This is the case of a 27-year-old nulliparous woman presenting with the history of seven consecutive first trimester spontaneous abortions. Since her marriage 10 years back she conceived on repeated times but all the pregnancies ended in spontaneous miscarriage at around 10–12 weeks. Hysterosalpingography revealed a bicornuate/septate uterus. Hysteroscopic septoplasty with concurrent laparoscopy was planned after normalization of the endocrine parameters. She conceived spontaneously 7 months after the surgery. She had luteal phase support with progesterone, cervical cerclage and management of gestational diabetes, and pregnancy-induced hypertension. At 37 weeks of gestation, elective caesarean section was done with the delivery of a healthy female baby.

A 24-Year-Old Woman First Diagnosed as a Sickle Cell Anemia during her Second Pregnancy: A Case Report

Introduction: Sickle cell anemia (SCA) is generally diagnosed in the early childhood and the disease is usually diagnosed in the first years of life by its clinical manifestations. The crises of this disease worsens during pregnancy. In this report, a 24-year-old pregnant woman with gestational age of 29 weeks and six days, G2P2A0L1, is presented. She complained of severe back and lower extremity pain. Considering the refractory pain, severe anemia, and absence of delivery process signs, further tests detected SCA for her. Preterm termination of pregnancy were performed by cesarean section regarding severe non-controlled pain, severe IUGR (intrauterine growth retardation), severe oligohydramnios, and previous caesarian section. A female baby was born with Apgar score of 8 and a birth weight of 1250 grams. Considering the high probability of vascular occlusion crisis and severe anemia associated with SCA during pregnancy, it is reasonable to seek sickle cells in all the pregnant patients with severe unexplained anemia or pain.

A rare case of intestinal obstruction in pregnancy

Intestinal obstruction (IO) in pregnancy is rare at 1 in 2500 to 1 in 16709 deliveries with an incidence of 0.001% to 0.003%. Although uncommon, IO in pregnancy carries significant maternal (6%) and fetal (26%) mortality. A 30-year-old, primigravida, at 33weeks 4 days of gestation presented to the emergency obstetrics department of Swaroop Rani Nehru Hospital, Prayagraj, Uttar Pradesh, India with a 2-day history of diffuse abdominal pain, distension and obstipation. The decision of emergency caesarean section was taken and a 2.5kg healthy female baby was delivered. Surgical opinion was sought intraoperatively. Segmental resection and anastomosis, adhesiolysis and appendicectomy was done. Patient discharged on 10 post-operative day along with her baby in satisfactory condition. Diagnosis of SBO can be difficult to make as symptoms are often attributed mistakenly to pregnancy and there can be a reluctance to request plain films owing to the risks of ionising radiation. Both of these factors can lead to a delay in diagnosis and initiating treatment. Clinical suspicion is vital and joint management between surgeons and obstetricians is crucial. In agreement with previous literature reviews regarding the need for prompt laparotomy in most cases of SBO, we recommend that patients with confirmed adhesional obstruction may be managed conservatively in the first instance but with a low threshold for progressing to laparotomy.

Bilateral idiopathic club foot in baby of a rheumatoid mother: A rare case report and its management

Congenital clubfoot has a multifaceted etiology, with several hypotheses offered in its etiopathogenesis. The clubfoot has rarely been reported in babies born to women who have rheumatoid arthritis (RA). We present a rare case of a 31-year-old lady with RA on disease-modifying anti-rheumatoid drugs who delivered a child with bilateral congenital clubfoot. She had previously been using Methotrexate, Hydroxychloroquine, and Sulfasalazine regularly, but Methotrexate was stopped seven months before pregnancy. A full-term female baby was born through the cesarean section with bilateral clubfoot deformity and a modified Pirani score of eight out of 10. The deformity correction was done with the Ponseti serial casting method. The final modified Pirani score was two out of ten. In newborns born to rheumatoid arthritis mothers, the club foot deformity was effectively treated with serial Ponseti corrective casts, as was idiopathic clubfoot in babies born to non-rheumatoid mothers. Our findings validate the Ponseti serial casting method for these kinds of patients.