Clinics and Practice

Poor management of either type 1 diabetes or haemophilia A can lead to complications such as organ dysfunction and haemarthropathy. Here, we describe the case of an 8-year-old boy diagnosed with severe haemophilia A shortly after birth. At 2 years old, he was also diagnosed with type 1 diabetes. After six years, the haemophilia treatment was changed from a plasma-derived factor VIII (FVIII) concentrate (octanate^®, Octapharma, Lachen, Switzerland) to Nuwiq^® (simocotocog alfa, Octapharma, Lachen, Switzerland), a recombinant FVIII (rFVIII) product from a human cell line, which allowed for a personalised treatment schedule that supported good adherence. The dosing regimen could be reduced to two weekly rFVIII infusions. The patient has experienced no spontaneous bleeds since switching to rFVIII and shows no signs of joint damage after over seven years of FVIII prophylaxis. rFVIII was well tolerated, with no treatment-related adverse events observed. This case illustrates the importance of treatment personalisation for young patients and their families managing concomitant diseases. Full article

Oral health-related quality of life (OR-QoL) measurement in patients with oral lichen planus (OLP) can provide valuable information for the optimal management of their clinical conditions. The main objective of the present study was to assess the OR-QoL of patients with OLP as measured by the short-form Oral Health Impact profile-14 (OHIP-14) questionnaire. PubMed/MEDLINE, ISI/Web of Science, clinical trial registry, Embase, Scopus, and grey literature (via Google Scholar and Scilit) were searched. Reviewers independently screened titles/abstracts, assessed full-text articles, extracted data, and appraised their quality. Random effect analysis along with subgroup analysis for age, gender, and clinical type was performed. Seventeen studies were included. Mean overall OH-QoL was 15.20, [95% CI 12.176, 18.231]; a higher OHIP-14 score was seen in OLP patients, resulting in poor OH-QoL. The impact of OLP on OH-QoL life was moderate as compared to healthy subjects. However, medical treatment of the disease improved the OH-QoL and thus reduced the impact of OLP on it. OH-QoL among patients with OLP is generally poor. Clinicians and physicians should consider the OH-QoL of these patients as part of patients’ evaluation and modulate the administered treatment based on the OH-QoL response. Full article

The surge in gestational diabetes mellitus (GDM) globally requires a health system tailored approach towards prevention, detection and management. We estimated the prevalence of GDM using diverse recommended tests and diagnostic thresholds, and also assessed the risk factors and obstetric outcomes, including postpartum glycemia. Using a prospective cohort design, 446 singleton pregnant women without pre-existing diabetes did GDM tests in five hospitals in Ghana from 20–34 weeks using fasting plasma glucose (FPG), one-hour and 2-h oral glucose tolerance test (OGTT). Birth outcomes of 403 were assessed. GDM was diagnosed using six international diagnostic criteria. At 12 weeks postpartum, impaired fasting glucose (6.1–6.9 mmol/L) and diabetes (FPG ≥7.0 mmol/L) were measured for 100 women. Per FPG and 2-h OGTT cut-offs, GDM prevalence ranged between 8.3–23.8% and 4.4–14.3%, respectively. Risk factors included overweight (OR = 2.13, 95% CI: 1.13–4.03), previous miscarriage (OR = 4.01, 95% CI: 1.09–14.76) and high caloric intake (OR = 2.91, 95% CI: 1.05–8.07). Perineal tear (RR = 2.91, 95% CI: 1.08–5.57) and birth asphyxia (RR = 3.24, 95% CI: 1.01–10.45) were the associated perinatal outcomes. At 12 weeks postpartum, 15% had impaired fasting glucose, and 5% had diabetes. Tackling modifiable risk factors is crucial for prevention. Glycemic monitoring needs to be integral in postpartum and well-child reviews. Full article

Recurrent aphthous stomatitis (RAS) is an oral condition characterized by painful oral ulcerations. While the clinical features of this disease are easily defined, the etiology remains unclear. Thus, existing treatments are still unsatisfactory in reducing the severity, healing, and recurrence rate; however, there is no permanent and definitive treatment. Effective treatment for aphthous stomatitis is not available, and those treatments available mainly focus on suppressing its symptoms. We are reporting a case of a 17-year-old boy who presented with a 3-year history of multiple recurrent major ulcers in the oral cavity. Levamisole with steroids has been used in many clinical trials to treat aphthous ulcers, showing an improvement in pain, discomfort, healing time, and reduction in the number of ulcers. The same method was used to treat our patient, who showed promising results, with no recurrence for one year. Levamisole is a safe, easily tolerable and promising drug for the treatment of RAS. Full article

A 26-year-old man with symptomatic SARS-CoV-2 infection developed a sudden-onset acute testicular pain. The echo-doppler images showed massive testicular infarction, so orchiectomy was performed. On gross examination, the surgical specimen showed complete testicular necrosis and diffuse thickening of the testicular coverings. Under the microscope, a severe obliterative arteritis was evidenced. SARS-CoV-2 spike antibody was detected by immunohistochemistry in the arterial endothelium. Electron microscopy displayed intracytoplasmic spiky viral particles in endothelial cells. The patient was treated with corticoids and was asymptomatic at last contact. Full article

Physical examinations are critical for diagnosis and should be differentiated into static and dynamic categories. One of the static findings is white nail, such as Terry’s and Lindsay’s nails. Here, we report the cases of two older patients with acute diseases who had nail changes that aided evaluation of their clinical course. Two elderly women who presented with acute conditions were initially thought to have normal serum albumin levels. They were found to have white nail with differences in nail involvement of the first finger, which subsequently revealed their hypoalbuminemia. The clinical courses were different following the distribution of nail whitening. Our findings show that examination of a white nail could indicate the previous clinical status more clearly than laboratory data. It can be useful for evaluating preclinical conditions in patients with acute diseases. Further evaluation is needed to establish the relationship between clinical outcomes and the presence of white nail in acute conditions among older patients. Full article

Anti-MDA5 antibodies-associated amyopathic dermatomyositisis a rare autoimmune disease that involve polyarthritis, cutaneous and pulmonary manifestations. The development of rapidly progressing interstitial lung disease is a life-threatening complication. We report the case of a 45-year-old woman without medical history, who was addressed to the Pulmonary Department for a polyarthritis with dry cough and hypoxemic dyspnea. Initially there was neither cutaneous manifestation nor interstitial lung disease on chest CT scan. After a few days, the patient developed fatal acute respiratory failure with diffuse ground glass opacities. Identification of anti-MDA5 antibodies allowed establishing diagnosis, despite the fact that the first immunological assessment was negative. Corticosteroid bolus of 1 g for three days and immunosuppressive treatment by cyclophosphamide was only initiated at the acute respiratory distress syndrome stage. Given the rapidly unfavorable prognosis of this entity of amyopathic dermatomyositis, the testing for anti-MDA5 antibodies should be recommended in case of progressive pulmonary symptoms associated with joint signs in order to identify this disease at an early stage and to begin rapid and adequate management. Full article

Non-tuberculous mycobacterial (NTM) infections of the breast are rare. These infections present as cellulitis of the breast or breast abscess. Their diagnosis poses a challenge as they manifest signs of acute inflammation, unlike tuberculous mycobacterial infections which present in a chronic pattern. However, on aspiration of pus from the site of infection, primary smear may show acid fast bacilli. This poses a diagnostic dilemma. The present case is that of a 34-year-old woman who presented with recurrent mastitis. She had history of right breast swelling, for which surgical excision had been performed three months prior at another facility. Her histopathology had showed cystic granulomatous neutrophilic mastitis (CNGM). The patient again presented with right breast abscess which was confirmed on ultrasonography. Incision and drainage along with removal of necrotic tissue was done. Primary smear of pus showed acid fast bacilli on Ziehl–Neelson staining. Bacterial culture and line probe speciation revealed non-tuberculous mycobacterium M. abscessus, which responded well to prolonged anti-microbial therapy. These rapidly growing NTM require prolonged treatment and are quite often recurrent. M. abscessus is a rare cause of CNGM, with this being only the third reported case in literature. A brief case report with a review of literature is presented. Full article

Patients diagnosed with thrombotic thrombocytopenic purpura (TTP) typically present with microangiopathic hemolytic anemia (MAHA) and thrombocytopenia; these two clinical manifestations were often believed to be essential indicators of TTP. However, such indicators are not always present in every case. Here, we present a patient affected by TTP but showing no distinctive schistocytes on blood smear review. TTP was diagnosed through a critically low level of a disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13 (ADAMTS13) activity. Awareness of such an atypical presentation of TTP is essential for timely treatment to prevent serious and even fatal outcomes for patients. Full article

A 67-year-old female patient with a past medical history of menopause, migraines, and gastro-esophageal disease presented with palpitation, fatigue, and shortness of breath. One month prior to her presentation, she reported having flu-like symptoms. Her EKG showed sinus tachycardia with no other abnormality. Laboratory findings, along with imaging, showed normal results. The event monitor failed to detect any arrythmias. We report a case of inappropriate sinus tachycardia secondary to viral infection as a diagnosis of exclusion. Full article

Sodium-glucose cotransporter 2 inhibitors (SGLT2i) are the most recently approved drug class for the treatment of type 2 diabetes mellitus (T2D). Although they are largely well-tolerated, their intake has been associated with euglycemic diabetic ketoacidosis (DKA) in some rare cases. We report the case of a 70-year-old male with type 2 diabetes and no history of DKA, who started therapy with empagliflozin one day before presenting with acute pancreatitis and laboratory findings consistent with euglycemic DKA. SGLT2i can induce euglycemic DKA from the first dose. Given the atypical presentation, a high degree of clinical suspicion is required to recognize this complication. Full article

Introduction: Phyllodes tumor (PT) of the breast, particularly malignant phyllodes tumor (mPT), is a rare fibroepithelial neoplasm. A complex diagnosis is based on pathologic, radiologic, and clinical findings, with controversies about what is the best therapeutic strategy. Objective: Our objective was to provide an overview of the clinical, pathologic, and therapeutic aspects of this rare tumor. Conclusions: mPT is a rare presentation of breast cancer and a challenge in clinical practice. A multidisciplinary approach should take into account some aspects like pathogenic mutations and hereditary syndromes. Oncologic surgery is the fundamental approach, and the use of adjuvant therapies is still controversial due to the lack of clinical trials. Treatment recommendations should be individualized according to patient risk and preferences. Prospective studies are fundamental to clarifying the best treatment for these tumors. Full article

Gas forming liver abscess (GFLA) though rare is seen in diabetic patients. Rupture of such abscesses usually requires surgical intervention. These cases are associated with high morbidity and mortality due to sepsis. Tuberculous liver abscesses are more often silent in presentation. GFLA formed in the background of a tuberculous liver abscess is rare. We present a case of ruptured GFLA with underlying tuberculous pathology in a normoglycemic patient. The abscess was managed by image guided intervention. A brief case report along with review of literature is presented. Full article

(1) Background: Several techniques for the treatment of pilonidal sinus disease (PSD) are in vogue, though none have emerged as the gold standard. Laying open (deroofing) and curettage under local anesthesia is one of the most straightforward procedures to treat PSD. In this study, the long-term follow-up in a large series was analyzed. (2) Methods: The laying open approach was performed for all types of consecutive PSD patients—simple, complicated, and abscess. The primary outcome parameter of the study was the healing rate. The secondary outcome parameters were operating time, hospital stay, time to resumption of normal work, and healing time. (3) Results: 111 (M/F–92/19, mean age-22.9 ± 5.7 years) consecutive patients were operated on and followed for 38 months (6–111 months). Of these, 24 had pilonidal abscesses, 87 had chronic pilonidal disease, while 22 had recurrent disease. Operating time and hospital stay were 24 ± 7 min and 66 ± 23 min, respectively. On average, patients could resume normal work in 3.6 ± 2.9 days and the healing time was 43.8 ± 7.4 days. Three patients were lost to follow-up. Complete resolution of the disease occurred in 104/108 (96.3%) patients, while 4 (3.7%) had a recurrence. One recurrence was due to a missed tract, while three recurrences presented after complete healing had occurred. Two patients with recurrence were operated on again with the same procedure, and both healed completely. Thus, the overall success rate of this procedure was 98.1% (106/108) with a recurrence rate after first surgery of 3.7% over a median follow-up of 38 months. (4) Conclusions: Pilonidal disease managed by laying open (deroofing) with curettage under local anesthesia is associated with a high cure rate. This procedure is effective in treating all kinds of pilonidal disease (simple, complicated, and abscess). Full article

Regional anesthesia is an important aspect of the overall anesthetic plan for patients. It has the potential to offer superior benefits compared to opioid-based treatment and is an important component of Enhanced Recovery after Surgery (ERAS) protocols. The use of the pectoral type I (PECS I) and pectoral type II blocks (PECS II) has been shown to reduce postoperative pain and opioid consumption in patients undergoing complete mastectomy and breast surgery. We describe the use of dexamethasone and dexmedetomidine to prolong the analgesic effects of these regional blocks in a patient undergoing total mastectomy with lymph node dissection. Full article

Esophagitis dissecans superficialis (EDS) is a rare and underdiagnosed esophageal lesion characterized by sloughing of the esophageal mucosa that has been associated with medications, various autoimmune disorders, and exposure to some chemical irritants. Anatomically, EDS is most commonly seen in the middle and distal thirds of the esophagus. When present, EDS is best treated by discontinuing the offending agent and initiating pharmacologic therapy with proton pump inhibitors. Steroids may also be effective if the etiology is autoimmune in nature. Our case highlights a 65-year-old female diagnosed with EDS after incidental ingestion of hair dye containing resorcinol and para-phenylenediamine (PPD). Full article

The aim of this study is to compare laparoscopic and conventional techniques following Total Pharyngo-laryngo-esophagectomy (TPLE) with respect to perioperative morbidity and mortality and postoperative recovery in post cricoid cancer patients. This is a prospective study, which was undertaken in Gujrat Cancer Research Institute (GCRI) in the period of July 2007 to March 2010. Fifteen consecutive patients who underwent laparoscopic TPLE were compared to that of 18 consecutive patients who underwent open TPLE. Laparoscopic and open TPLE procedure were compared with respect to patient characteristics, intra operative and complications present. The average duration was observed to be 3.5 h in the MIS (Minimally Invasive Group) group and was 5.3 h in the open group. The average blood loss was 300 mL in the MIS group and 500 mL in the open group. Average duration of the hospital stay in the MIS group was 13 days and 16 days in the open group. In the MIS group, one patient (6.7%) had a pneumonic complication and two patients (13%) had wound complications. In the open group, six patients (33%) had pneumonic consolidation and four patients (22%) had wound infections. In both groups, one patient each suffered mortality. Laparoscopic TPLE has been found to be much safer with less morbidity as compared with open surgery. Full article

Secondary organizing pneumonia refers to a disease process caused by pulmonary tissue injury. Various insults can cause secondary organizing pneumonia, including multiple types of infections and cancer. The mainstay of diagnosis is a combination of imaging and lung biopsy showing inflammatory changes, specifically plugs with granulated tissue and fibrosis. Clinical suspicion needs to be raised for secondary organizing pneumonia when a patient is requiring increasing amounts of oxygen in the presence of treatment for pneumonia or another underlying lung disease. Here, we present the case of a 65-year-old male who presented with acute hypoxemic respiratory failure in the setting of previously having been tested positive for influenza B. Aggressive steroids with eventual tapering of his O₂ requirements led to a successful outcome. While influenza has been reported as a cause of secondary organizing pneumonia after proceeding infection, these cases are usually represented by type A, rather than B. Full article

Viral infections, alcohol, hepatic steatosis, autoimmunity medications and herbal supplements are common etiologies of hepatitis. Khat (Catha Edulis) is a commonly used recreational substance in East African and Middle Eastern countries. Khat has been reported in the literature to be associated with hepatotoxicity, which can present in several forms, including chronic liver disease. The possible pathogenesis of liver injury could be secondary to biochemical components of Khat itself or additives such as pesticides or preservatives. An autoimmune mechanism of liver injury has also been postulated, supported by sparse evidence. We present a case of a Yemeni immigrant with acute hepatitis whose fear about social norms and breaching confidentiality made it challenging to identify Khat as being the underlying cause. A 34-year-old man from Yemen presented with right upper quadrant pain of one day duration. He had predominantly elevated transaminases with mild elevation in bilirubin. His investigations were negative for the viral, metabolic or biliary etiology. A persistent focus on clinical history and the well-established physician–patient relationship revealed a history of Khat use. The liver biopsy finding of lobular hepatitis was compatible with drug-induced liver injury and established the finding of Khat hepatotoxicity. Subsequently, the patient improved with conservative management. Full article