First case recognized as autoimmune polyglandular syndrome type 2 with myasthenia gravis in Palestine: A case report and literature review.

BACKGROUND: Myasthenia gravis is an organ specific autoimmune disorder that is potentially serious but treatable. It is characterized by fatigability of the voluntary muscles and weakness caused by antibodies against the nicotinic acetylcholine receptor (AChR) on the postsynaptic membrane at the neuromuscular junction.Sometimes, and in very rare cases, it can be associated with other autoimmune conditions in a so called autoimmune polyglandular syndrome type 2, which consists mainly of autoimmune adrenal insufficiency (Addison's disease) with autoimmune thyroid disease and/or type 1 diabetes mellitus. CASE PRESENTATION: We describe a case of a 47-year-old male patient presenting with weakness, difficulty swallowing (mainly liquids) and dysarthria. He was discovered to have low cortisol and TSH levels with high T4 and T3. These findings lead to the suspicion of a more complex disease process and through a thorough research of literature we discovered an association between myasthenia gravis and autoimmune polyglandular syndrome specifically type 2 which fits with our patients' presentation. CONCLUSION: In any autoimmune disease, it is important to keep in mind associations and susceptibilities to other autoimmune processes and syndromes in order to reach a correct diagnosis and treatment preventing life threating events.

Misdiagnosis of persistent asthma of a patient suffering from acute bronchopulmonary aspergillosis (ABPA).

BACKGROUND: Allergic bronchopulmonary aspergillosis (ABPA) is a condition characterized by an exaggerated immune response (a hypersensitivity response) to the fungus Aspergillus (most commonly Aspergillus fumigatus).ABPA causes airway inflammation that if left untreated can lead to bronchiectasis (an abnormal dilation of the airways) due to the immune system and fungal spores damaging sensitive lung tissues and ultimately leading to scarring. CASE PRESENTATION: We present a case of a 32-year-old female patient who was misdiagnosed with persistent asthma and treated accordingly for several months until a reexamination was done and a diagnosis of ABPA was concluded. Treatment was altered which led to the successful recovery of the patient. CONCLUSION: A reevaluation of the patients' condition was needed to arrive to the correct diagnosis and to put her on the correct treatment as an ABPA patient instead of persistent asthma, concluding that the medical history and physical examination are both of vital significance to stipulate a correct diagnosis.

Endoscopic Drainage of Giant Pancreatic Pseudocysts Using Both Lumen-Apposing Metal Stent and Plastic Stent: A Report of Two Cases and Review of the Current Literature.

INTRODUCTION: A pancreatic pseudocyst (PP) with major diameter equal to 10 cm or more is called a giant pseudocyst. The ideal management for giant PPs is controversial. Endoscopic drainage is an alternative nonsurgical approach for PP management. Only a few cases of giant PPs were reported to be managed by endoscopic drainage. Case Presentation. We reported two cases of giant PPs following an episode of acute pancreatitis. Both were resolved following endoscopic cystogastrostomy using metallic and double-pigtail stents with excellent outcomes. There was no history of recurrence or complications on follow-up. In addition, we extensively reviewed all available literature studies of giant pancreatic pseudocyst presentation, management, and complications. We summarized all reported cases and presented them in a comprehensive table. CONCLUSION: The endoscopic cystogastrostomy approach is cost saving, can avoid surgical complications, and offers an early hospital discharge.

Splenic Vein as an Autologous Graft in Traumatic SMV Injuries: A Missed Valuable Alternative.

Traumatic superior mesenteric artery (SMA) and vein (SMV) injuries are rare but often lethal. The ideal management options of traumatic SMV injury are still controversial. Management options include venous repair and ligation. Splenic vein turndown procedure (SVTP) is a rare procedure that has been described in only 6 cases in the literature. Here, we reviewed the literature on the usage of the splenic vein turndown procedure (SVTP) as an alternative option in patients with traumatic SMV injury. METHODS: We performed a narrative review for the available literature on the usage of the splenic vein as an autologous graft in the management of the SMV injury. We included all studies of SVTP in traumatic SMV injuries only. RESULTS: We included only 5 studies. In total, 7 patients underwent SVTP. Five patients presented with a penetrating abdominal vascular trauma (AVT) and 2 patients with a blunt AVT. The advantages of the SVTP include no need for additional incisions to harvest potential autologous grafts, minimally increased operative time, and 1 less anastomotic site compared to other conduit options. CONCLUSIONS: In cases of traumatic SMV injuries with associated splenic or pancreatic injuries that need distal pancreatosplenectomy, surgeons may consider SVTP as an ideal management option rather than primary repair or ligation.

Long-Term Mortality and Morbidity Related to Congestive Heart Failure with Reduced Ejection Fraction (CHFrEF) in Palestinian Patients Maintained on Submaximal Sacubitril/Valsartan Doses: A Pilot Study.

BACKGROUND: The efficacy of sacubitril/valsartan, a newly introduced combination drug for heart failure with reduced ejection fraction (HFrEF), was demonstrated in the PARADIGM-HF trial conducted in Western countries. However, these findings need to be verified in the Middle Eastern context, where patients may exhibit a different response due to different environmental and racial factors. OBJECTIVES: The goal of this study was to evaluate the efficacy of submaximal sacubitril/valsartan doses in terms of improving the disease symptoms, as measured by the New York Heart Association (NYHA) classification and left ventricular ejection fraction (LVEF) percentage, as well as establish long-term morbidity and mortality associated with HFrEF among Palestinian patients administered target doses of an angiotensin-converting enzyme inhibitors (ACEI) or angiotensin II receptor blockers (ARBs). Material and Methods. This study involved a retrospective review of charts related to patients with HFrEF maintained on sacubitril/valsartan and was conducted in a referral cardiology clinic in Palestine. The inclusion criteria were age 18+, HFrEF diagnosis, sacubitril/valsartan usage for at least six months during the period between January 1, 2016, and June 30, 2019, and LVEF < 40%. The exclusion criteria included LVEF ≥ 40% and drug administration duration < 6 months. The collected data included NYHA class, as well as LVEF, serum sodium (Na), potassium (K), serum creatinine (Cr), and blood urea nitrogen (BUN) levels and the mortality rate before and after the minimum treatment duration. IBM SPSS STATISTICS for Windows, version 20.0, Armonk, NY: IBM Corp. IBM Corp., released 2012, was used for data analysis, whereby T score was calculated for comparisons between numerical groups, and p < 0.05 was considered statistically significant. RESULTS: The initial study sample comprised of 205 consecutive patients with HFrEF maintained on sacubitril/valsartan for at least six months from January 1, 2016, to June 30, 2019. Three patients were excluded due to attrition, along with further 12 patients with LVEF ≥ 40% (based on the PARADIGM-HF trial criteria). Throughout the treatment period, most patients showed escalating improvement in terms of the LVEF and NYHA classification, as LVEF = 29.8% and NYHA = 3 were obtained on average before initiating sacubitril/valsartan, compared to 41% and 1.7, respectively, after 6-month treatment (p = 0.0003 and 0.046, respectively). These improvements in LVEF and NYHA class were noted across all sacubitril/valsartan doses (50-400 mg). However, 23 patients (12%) died while undergoing sacubitril/valsartan treatment. CONCLUSION: A significant long-term reduction in the mortality and morbidity rates was observed in Palestinian patients with HFrEF maintained on submaximal doses of sacubitril/valsartan.

Prognosis and Treatment of Liver Transplant Recipients in the COVID-19 Era: A Literature Review.

Liver transplantation during the COVID-19 pandemic is challenging. Both donor and recipient issues can be influenced by the risks attributed to the pandemic. Allocation policy may need to be modified and criteria may be influenced by local infection rates and availability of medical facilities. Modifying immunosuppression (IS) protocols is controversial and is not evidence-based. In this study, we review the published literature on liver transplant recipients who were infected with COVID-19. A literature review was performed using PubMed, ScienceDirect, and WHO databases to identify relevant English-language articles published up to May 20, 2020. Fifteen articles reported 120 liver transplant recipients who were infected with COVID-19. Only 10 papers with 22 patients reported full encounter characteristics. Four papers reported 23, 17, 13, and 6 patients, respectively, but with minimal data. One paper reported the authors' own 39 patients' characteristics and demographics. The mean age was 58.2 years with 66% males. The most commonly reported presentations in descending order were fever (91%), cough (36.7%), shortness of breath (SOB) (31.8%), and diarrhea (31.8%). Liver transplant patients infected with COVID-19 were maintained on Tac (79%), mycophenolate (MMF) (48.4%), and Prednisone (29.6%) and were managed by reducing MMF in 14.3% of patients and reducing Tac in 14.3% of patients; 28.6% of patients needed ICU admission, 13.6% of patients had died, and the reported general population COVID-19 mortality rate was 3.4%. The clinical presentation of COVID-19 in liver transplant recipients may be different from the general population, with higher rates of severe disease, complications, and mortality.

Sutureless Approach for Gastroschisis Patients in Palestine.

Gastroschisis is a ventral abdominal wall congenital defect with bowel herniation outside the abdominal cavity. Gastroschisis traditional management is the primary operative closure surgery (POCS), but the sutureless silo approach (SSA), a novel alternative, gains wide acceptance in the developed countries and across nations. This study describes the first-ever gastroschisis patient managed with the sutureless silo approach in Palestine. In addition, we shall use this case as the very first nucleus for the upcoming gastroschisis management in our referral hospital because the SSA yields a reduced hospital stay which is fundamental to our institution due to the limited number of beds and lower management costs to the hospital and families.

Kidney Transplantation in the Times of COVID-19 - A Literature Review.

Kidney transplantation at the time of the COVID-19 pandemic is challenging. Modifying the immunosuppression protocols is controversial and not evidence based. In this study, we aim to review the published literature of kidney transplant recipients who encountered COVID-19. A literature review was performed using PubMed, ScienceDirect, and World Health Organization databases to identify relevant English-language articles published up to May 7, 2020. There were 24 articles that reported 129 kidney transplant recipients who encountered COVID-19. The age mean was 54.2 years with 73.7% as males. The most commonly reported presentations in order were fever (82.3%), cough (58%), shortness of breath (33.2%), and fatigue (30.7%). Acute kidney injury was observed in 34.1% of patients. Kidney transplant patients encountered COVID-19 were maintained on tacrolimus (Tac, 92%), mycophenolate mofetil (MMF, 78.8%), and prednisone (Pred, 77%) and were manage by holding MMF in 79.1% of patients and holding Tac in 34.4% of patients. In all, 20% of patients needed Intensive Care Unit (ICU) admission and 24.6% of patients required mechanical ventilation. In all, 18.8% of patients had died compared to the reported general population COVID-19 mortality of 3.4%. The clinical presentation of COVID-19 in kidney transplant recipients may be different from the general population with a higher rate of severe disease, complications including renal failure, and mortality.

Familial Mediterranean Fever Complicated by a Triad of Adrenal Crisis: Amyloid Goiter and Cardiac Amyloidosis.

BACKGROUND: FMF is a common disease in the Mediterranean populations and may be complicated by AA amyloidosis. The coexistence of adrenal and thyroid amyloidosis in AA amyloidosis secondary to familial Mediterranean fever (FMF) is an extremely rare reported condition. We presented a previously unreported triad of adrenal, thyroid, and cardiac amyloidosis secondary to FMF. Presentation of Case. We reported a 23-year-old Palestinian male patient presented with hypotension, vomiting, diarrhea, and abdominal pain. The patient was subsequently diagnosed to have an adrenal crisis with both amyloid goiter and cardiac amyloidosis. CONCLUSION: It is crucial to recognize the adrenal crisis in patients with AA amyloidosis secondary to FMF who present similarly to acute FMF inflammatory episodes. The adrenal crisis has high morbidity and mortality, especially if not recognized early in the course of the disease.

Congenital arrhinia: A case report and literature review.

INTRODUCTION: Underdevelopment of the nose is a wide spectrum entity ranges from partial absence of the nose to congenital arrhinia (CA). CA is the congenital absence of the external nose, nasal cavities, and/or nasal nostrils±olfactory apparatus, and is an extremely rare entity as less than 50 cases reported in the literature. CA can be isolated and idiopathic in origin or be a part of genetic-linked certain syndromes. Of note, the isolated CA can be inherited as an autosomal dominant condition with incomplete penetrance. PRESENTATION OF CASE: We report a 13-month-old Palestinian female patient with isolated CA complicated with recurrent lower and upper respiratory tract infections (URTI). The family history is significant for the mother with incomplete and uncomplicated underdevelopment of the external nose and nasal nostrils. The patient uses a tracheostomy to breathe and is waiting for the optimal age for surgical correction. In addition, we reviewed the available literature using PubMed and summarized all CA cases reported from 2016 to 2019 since two studies had presented the literature before 2016, and presented them in a very comprehensive table. DISCUSSION: CA is largely idiopathic and not well understood. Although CA can be inherited and runs in families with incomplete penetrance, no causative genetic abnormalities had been found in most of the reported cases. CA frequently presents with upper airway obstruction and respiratory distress, recurrent lower and URTI, and feeding difficulties. CA is managed initially with tracheostomy and should be followed by a surgical correction at the appropriate age. CONCLUSION: CA may be sporadic, familial, or a part of a syndrome. CA carries a significant rate of morbidity and mortality and a tracheostomy should be inserted initially to alleviate the early CA complications and followed by surgical reconstruction when the patient reaches the age of preschool/school age. More studies are required to determine CA inheritance.

Systemic Lupus Erythematosus with Multiple Autoimmune Disease Presented with Extensive Peripheral Gangrene.

Systemic lupus erythematosus (SLE) is an autoimmune disease and can be associated with other autoimmune diseases. SLE usually presents with skin change and rarely presents with gangrene. SLE gangrene usually involves the digits of upper extremities. We report the first case of SLE associated with an extremely rare constellation of neuromyelitis Optica (NMO) and diabetes mellitus type 1, presented with a rare form of the SLE gangrene which involves bilateral lower extremities up to midlegs, a case that has not yet been reported in the literature. Although SLE gangrene may respond to immunosuppressants, it has a high risk of complications that can end up with amputations.

IVIG and under Burn Unit Care Yield Favorable Outcomes in Pediatric Patients with Toxic Epidermal Necrolysis: A Case Report and Literature Review.

Body reactions to drugs can manifest as Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). TEN is the most severe form of cutaneous reactions with an incidence rate of 1-2 per million cases per year. Despite TEN being a critical and life-threatening condition, there is little to no evidence of clear management protocol. We reported a 5-year-old male child who presented with lamotrigine-induced TEN and was successfully treated with intravenous immune globulin (IVIG) with a burn unit care level, while TEN treatment with IVIG is an appropriate approach with predictable good outcomes, burn unit care is also effective in creating highly favorable effects. Upon reviewing the literature, several studies indicate that TEN patients treated with the combination of IVIG and burn unit care lead to decreased levels of morbidity and mortality than when treated with IVIG or burn unit care alone. Therefore, treatment involving both IVIG and burn unit care should be considered for TEN patients.

Appendicitis-Like Picture Induced by Foreign Body in a 2-Year-Old Boy.

Background and Aim. Appendicitis is unusual in toddlers and foreign body- (FB-) induced appendicitis is rare. We present a FB-induced appendicitis in a toddler with no suggestive history of FB ingestion. Case Presentation. A 2-year-old healthy boy presented to the emergency department with irritability for 3 days duration associated with fever of 39°C, nausea, anorexia, and vomiting. There was no history of foreign body ingestion. The abdomen was distended and diffusely tender. An abdominal ultrasound (US) was suggestive of perforated appendicitis with appendicular mass formation. An abdominal X-ray showed a pin-like foreign body in the abdomen. An emergent appendectomy was performed. Intraoperatively, a sealed small cecal perforation was noticed. A 5 cm pin-like metallic foreign body was found to obstruct the appendicular lumen. The appendix was grossly normal without inflammatory changes. Conclusion. FB-induced perforations or appendicitis albeit in patients with no history of FB ingestion or infants and toddlers need a high clinical suspicion to prevent the delay in diagnosis and the subsequent complications.

Paraduodenal hernias in children: Etiology, treatment, and outcomes of a rare but real cause of bowel obstruction.

INTRODUCTION: Paraduodenal Hernia (PDH) is the most common variant of internal hernia and occurs most often in males during their 4th-6th decades of life. PDH in pediatric age group has rarely been reported in literature with only five cases of age up to 10 years were reported. PDH is a rare cause of intestinal obstruction, which may lead to subsequent strangulation and perforation of the bowel. PRESENTATION OF CASE: We reported a 1.5 year-old male child presented with intestinal obstruction. The patient experienced abdominal pain, vomiting and irritability. Abdominal x-ray showed distal intestinal obstruction which was discovered to be a result of left PDH incidentally during the surgery. In addition, we performed a literature search using PubMed to identify the published cases of PDH. We also compared our case with the characteristics of all reported PDHs in toddlers and children up to 10 years of age in a concise table. DISCUSSION: Despite its congenital origin, PDH has been reported in childhood age group in very rare occasions rendering the accurate incidence of PDH in infancy and childhood unknown. PDHs can be asymptomatic or can present most commonly with recurrent upper abdominal pain. Diagnosis is quite difficult in the absence of symptoms but could be achieved using a computed tomography (CT-scan) in non-emergency symptomatic patients. Surgical repair is mandatory to avoid potential complications. CONCLUSION: As PDH can lead to major and life threatening complications, it must remain in our minds as a possible cause of intestinal obstruction.

Postoperative intestinal intussusception in children, an easily missed culprit of postoperative intestinal obstruction: Case series and literature review.

BACKGROUND: Postoperative intestinal intussusception (POI) is a rare cause of intestinal obstruction with POI after surgical reduction of ileocolic intussusception being an extremely rare variant. POI was reported to follow many abdominal and non-abdominal operations. A late diagnosis can risk ischemia and necrosis. POI also increases the morbidity and mortality, rendering an early diagnosis and prompt management as lifesaving. METHODS: We reviewed the medical charts retrospectively for the last ten years for patients with POI at Palestine Red Crescent Society Hospital, Hebron, Palestine. We reviewed the literature and presented the characteristics of the most reported cases of POI following surgical reduction of ileocolic intussusception. RESULTS: We presented three cases of ileoileal POI and one case of ileocolic POI followed different primary operations. All but one patient presented in the first two weeks. The delayed presentation came two months after revision of a prolapsed colostomy. All patients managed successfully with operative manual reduction with no postoperative complications. Initially, we had struggles in the diagnosis of POI largely due to a low suspicion for this rare entity, but thereafter we kept POI in mind and managed the after-coming cases in an expeditious manner. CONCLUSION: Frequently, POI is misdiagnosed as postoperative adhesive obstruction. POI is challenging in diagnosis and needs a very high index of suspicion, mainly due to its rarity and atypical presentation. By keeping the possibility of POI in mind, one can easily diagnose it and prevent its consequences.