A 4-month-old male baby who presented in a moribund condition with seizures was found to have hepatomegaly, hypoglycemia and milky serum. Serum triglycerides were markedly elevated (3168 mg/dL) with cholesterol being 257 mg/dL and high density lipoprotein levels were low (19 mg/dL). The possibility of glycogen storage disease type I was considered in the diagnosis. Infants with glycogen storage disease type I may present like sepsis. The association of hepatomegaly, hypoglycemia and abnormal lipid profile stated above should alert the physician to consider glycogen storage disease type I in the diagnosis.

Intracranial hypotension syndrome is an uncommon manifestation of shunt overdrainage; characterized by a triad of postural headache, diffuse pachymeningeal gadolinium enhancement and low cerebrospinal fluid opening pressure. We describe a young female with recurrent episodes of postural headaches and reversible dorsal midbrain syndrome due to intracranial hypotension as a complication of shunt overdrainage, and a subsequent improvement following shunt ligation.

Phenytoin is one of the commonly used antiepileptic drugs. The common dose dependent and reversible neurological side effects of phenytoin are nystagmus, diplopia, dysarthria, ataxia, incoordination, chorioathetosis, orofacial dyskinesias and drowsiness. Persistent cerebellar dysfunction with cerebellar atrophy is a well known complication of long term phenytoin use. There are several mechanisms proposed including hypoxia due to frequent seizures or toxic effects of phenytoin on cerebellar Purkinje cells. However, irreversible cerebellar dysfunction following acute phenytoin intoxication is rare. We report a 20 year old female who presented with nystagmus, dysarthria, limb and truncal ataxia with orofacial dyskinesias and chorea. She also had cognitive and affective symptoms in the form of reduced attention, slow responses, lalling speech, blunting of affect, inappropriate laughter, reduced self care and executive dysfunction. The symptoms started 2 weeks following the initiation of phenytoin 300mg/ day, given prophylactically following left basal ganglia bleed. Her serum phenytoin was in toxic range, hence phenytoin was stopped. Her PET scan revealed bilateral cerebellar hypometabolism. At 6 months follow up, she had persistent ataxia with cognitive and affective dysfunction and follow up MRI showed diffuse cerebellar atrophy. The clinical and radiological fi ndings suggest that acute phenytoin intoxication is responsible for persistent ataxia and cerebellar cognitive affective syndrome.

Progressive multifocal leukoencephalopathy (PML) is a progressive lethal demyelinating disease of the brain, caused by JC virus. Reactivation of JC virus due to reduction of cellular immunity especially in setting of AIDS, is the commonest underlying cause. PML has classically been described in individuals with profound cellular immunosuppression such as patients with AIDS, haematological malignancies, organ transplant recipients or those treated with immunosuppressive or immunomodulatory medications for autoimmune diseases. Rarely it has also been diagnosed in cases with no or minimal immunosuppression. Here, we report a 50 year-old man who presented with sudden onset multiple neurologic defi cits. Neuroimaging, histopathology, and virology studies confi rmed the diagnosis of PML. We could not however demonstrate any underlying immunodefi ciency state. Our case suggests that absence of immunodefi ciency does not exclude the possibility of PML and should be considered in immunocompetent patients with a typical clinical course and neuroimaging fi ndings.

OBJECTIVES: This article describes our experience with neck dissection in 10 patients with oral squamous cell carcinoma. MATERIALS AND METHODS: Between January 2007 and October 2009, 10 patients underwent primary surgery for the treatment of squamous cell carcinoma of the oral cavity. For patients with N0 disease on clinical exam, selective neck dissection (SND [I-III]) was performed. In patients with palpable cervical metastases (N+), modified radical neck dissections were performed, except in one patient in whom SND (I-III) was performed. The histopathologic reports were reviewed to assess the surgical margins, the presence of extra-capsular spread, perineural invasion, and lymphatic invasion. RESULTS: On histopathologic examination, positive soft tissue margins were found in three patients, and regional lymph node metastases were present in five of the ten patients. Perineural invasion was noted in five patients, and extra nodal spread was found in four patients. Regional recurrence was seen in two patients and loco-regional recurrence plus distant metastasis to the tibia was observed in one patient. During the study period, three patients died. Seven patients remain free of disease to date. CONCLUSION: Histopathological evaluation provides important and reliable information for disease staging, treatment planning, and prognosis. The philosophy of neck dissection is evolving rapidly with regard to the selectivity with which at-risk lymph node groups are removed. The sample size in the present study is small, thus, caution should be employed when interpreting these results.
Carcinoma, Squamous Cell* ; Humans ; Lymph Nodes ; Mouth ; Neck Dissection* ; Neck* ; Neoplasm Metastasis ; Philosophy ; Prognosis ; Recurrence ; Sample Size ; Tibia

Background/Aims: Opioid-induced bowel dysfunction includes nausea, vomiting, constipation and abdominal distension. We describe patients presenting with gastrointestinal (GI) ulcers and ulcerated strictures secondary to opioid abuse, an entity not well described in literature. Methods: This retrospective observational study included patients with opioid abuse gastroenteropathy presenting to Dayanand Medical College and Hospital, Ludhiana, India between January 2013 and December 2018. Opioid abuse gastroenteropathy was defined as gastric or small bowel ulcers and ulcerated strictures in patients abusing opioids, where all other possible etiologies of GI ulcers/strictures were excluded. Clinical, biochemical, endoscopic, radiological and histological parameters as well as response to treatment were assessed. Results: During the study period, 20 patients (mean age, 38.5±14.2 years; 100% males) were diagnosed to have opioid induced GI ulcers and/or ulcerated strictures. The mean duration of opioid consumption was 6.2±3.4 years. The mean duration of symptoms at presentation was 222.1±392.3 days. Thirteen patients (65%) had gastroduodenal involvement, 6 (30%) had a jejunoileal disease and 1 (5%) had an ileocecal stricture. Two patients (10%) presented with upper GI bleeding, 11 (55%) had features of gastric outlet obstruction and 7 (35%) presented with small bowel obstruction. Abdominal pain and iron deficiency anemia were the most common presentations. Only 1 patient (5%) responded to proton pump inhibitors, 3 (15%) had a lasting response to endoscopic balloon dilatation, while all other (80%) required surgical intervention. Conclusions Opioid abuse gastroenteropathy presents as ulcers and ulcerated strictures which respond poorly to medical management and endoscopic balloon dilatation. A majority of these cases need surgical intervention.

Background/Aims: Exclusive enteral nutrition (EEN) is recommended for induction of remission in pediatric Crohn’s disease (CD). However, it is not currently recommended for inducing remission in adults. This report describes the use of 12-week EEN for induction of remission in anti-tumor necrosis factor (anti-TNF) refractory adult CD. Methods: This is a retrospective analysis of adults with moderate to severe active (Crohn’s Disease Activity Index [CDAI] >220) anti-TNF refractory CD, who received EEN for 12 weeks between April 2018 and March 2019 at Dayanand Medical College and Hospital, Ludhiana, India. Primary outcomes included achievement of clinical remission and fistula healing at 12 weeks. Improvement in inflammatory markers and nutritional status were the secondary end points. Results: Out of 23 patients who received anti-TNF agents, 7 (30.4%) were refractory and were offered EEN as a salvage therapy. Six patients (66.7% females, mean age 25.6±6.5 years) consented. Four patients (66.6%) achieved clinical remission (CDAI <150). Mean CDAI of patients decreased significantly after 12 weeks of EEN (388.8±74.8 vs. 160.0±25.2, P<0.001). Perianal fistulas showed clinical response (drainage decreased by >50%), though none achieved remission. Entero-enteric fistulae showed complete healing. Mean body mass index improved from 15.6±3.1 to 18.9±1.9 kg/m2 at week 12 (P=0.003). Hemoglobin and serum albumin also improved from 8.2±1.1 g/dL and 2.8±0.3 g/dL at baseline to 12.6±0.6 g/dL and 3.6±0.5 g/dL post-EEN respectively (P<0.001 and P=0.006 respectively). Conclusions EEN appears to be an effective and well tolerated therapy for induction of remission in anti-TNF refractory adult CD. More data from prospective trials with larger number of patients is required.

BACKGROUND AND OBJECTIVES: The bilateral choanal atresia is a medical emergency for which surgery is the only and definite treatment. We performed an atretic bone perforation using Hagers dilators. The purpose of this study, then, is to evaluate the efficacy of this simple technique of perforation, dilation and stenting. MATERIAL AND METHODS: We studied five patients who underwent surgery for a complete bilateral bony atresia from 1994 to 2004. All the patients were newborn: two males and three females babies. The male female ratio was 1 : 1.5. Their age varied from 1 day to 4 days. The surgery was performed with Hagers dilators using the puncture and dilation technique. Stenting was carried out in all cases using a non cuffed endotracheal tube. RESULTS: The reestablishment of the nasal passage through a perforating atretic plate was considered a successful criteria of surgery. All the neonates had good nasal air passage after the perforation, dilation and stenting. Two of the patients who were with the CHARGE association died within 7 days due to causes unrelated to surgery. The other three patients had successful removal of stent after six weeks. There was no follow-up afterwards. CONCLUSION: The puncture, dilation and stenting technique by Hagers dilator is safe, quick and simple. It involves minimum blood loss and is without any complications. Stenting, therefore, is essential for preventing restenosis.
CHARGE Syndrome ; Choanal Atresia* ; Emergencies ; Female ; Follow-Up Studies ; Humans ; Infant, Newborn ; Male ; Punctures* ; Stents*

BACKGROUND: Nasal myiasis is very distressing condition of the nose that occurs among patients with atrophic rhinitis. This condition is frequently found among those belonging to the lower socioeconomic status suffering from bad nasal hygienic conditions. A retrospective study was done of 58 cases of patients admitted for nasal myiasis. All the patients belonged to the lower socio- economic class and suffered from poor hygienic condition. The majority of the patients were over the age of 60 years and male female ratio was 1 : 1.5 .The primary complaints of this condition were epistaxis, foul smelling nasal discharge, passage of worms from the nose and pain. The majority of the patients were suffering from primary atrophic rhinitis and a few from the secondary atrophic rhinitis due to syphilis and leprosy. TREATMENT: As a first step of treatment, the maggots were removed manually and endoscopically. All the patients became maggot free in 2-3 days time. Alkaline nasal douching was then started along with 25% glucose in glycerine nasal drop. Complications such as septal perforation, saddling nose, palatal perforation were observed in a few cases. The incomplete closure of nose (modified Young's operation) was performed in a few cases. CONCLUSION: Manual and endoscopic removal should be done repeatedly. Antimeningitis prophylaxis should be started at once. And, to prevent recurrence, mosquito net should be used while sleeping, and incomplete closure of both the nasal cavity (modified Young's operation) should be carried out.
Epistaxis ; Female ; Glucose ; Glycerol ; Humans ; Larva ; Leprosy ; Male ; Mosquito Nets ; Myiasis* ; Nasal Cavity ; Nose ; Recurrence ; Retrospective Studies ; Rhinitis, Atrophic ; Smell ; Social Class ; Syphilis ; Therapeutic Irrigation

The inflammatory bowel disease (IBD)-disk is a validated, visual, 10-item, self-administered questionnaire used to evaluate IBD-related disability. The present study aimed to evaluate IBD-disk in assessment of IBD daily life burden and its relation with disease activity. Methods: A cross-sectional study was conducted between June 2021 and December 2021. Patients with IBD were asked to complete the IBD-disk and a visual analogue scale of IBD daily-life burden (scored from 0–10, score >5 indicative of high burden). The internal consistency of IBD-disk, correlation with IBD daily life burden and disease activity (assessed by partial Mayo score and Harvey Bradshaw Index in patients with ulcerative colitis [UC] and Crohn’s disease [CD], respectively) and diagnostic performance of IBD-disk to detect high burden were analyzed. Results: Out of the 546 patients (mean age 40.33±13.74 years, 282 [51.6%] males) who completed the IBD-disk, 464 (84.98%) had UC and the remaining (n=82, 15.02%) had CD. A total of 311 patients (291 UC and 20 CD; 56.95%) had active disease. The mean IBD-disk total score and IBD daily life burden were 18.39±15.23 and 2.45±2.02, respectively. The IBD-disk total score correlated strongly with the IBD daily life burden (ρ=0.94, P<0.001), moderately with partial Mayo score (ρ=0.50) and weakly with Harvey Bradshaw Index (ρ=0.34). The IBD-disk total score >30 predicted high IBD daily-life burden. Conclusions: The IBD-disk accurately predicts the daily life burden and parallels disease activity in patients with IBD and can be applied in clinical practice. (Intest Res, Published online)