Good Morning! THURSDAY, MARCH 26, 2015

Transcription

1 Good Morning! THURSDAY, MARCH 26, 2015

2 Prep A female newborn is noted to have a sacral subcutaneous lump with a fatty consistency and a slightly lateral deviation of the superior aspect of the inner gluteal fold. Her physical examination findings were otherwise normal, and there was no patellar hyper-reflexia. MRI of the thoracic and lumbar spine was obtained and showed a lipomeningomyelocele. It is unclear if the cord is tethered. The infant was referred to a multidisciplinary spina bifida clinic where renal ultrasonography was normal and urodynamic assessment showed no evidence of detrusor hyper-reflexia or bladder-sphincter dyssynergia. Of the following, the symptom or sign that you are most likely to look for during health supervision visits is A. Chronic progressive diarrhea B. Development of an ataxic gait C. Development of a sacral hemangioma D. Loss of patellar reflexes E. Urinary retention and leakage

3 Let s hear from our patient 2 1/2 yo female with diarrhea X 1 ½ weeks Loose (>10/day), frequency increasing? Blood-tinged today No fever, no emesis, no obvious abdominal pain Decreased PO; still has good UOP, although decreased from normal x2 days Has tried Pedialyte at home No other URI symptoms

4 Our patient BHx: 41 week vaginal delivery, prenatal care +, no complications, birthweight 7lb 12oz PMH: ALL diagnosed at 2 years Last Chemotherapy (VCR and Doxorubicin 1 week prior) PSH: PETs at 19 months, PAC placed at time of ALL diagnosis Meds: Biotene, Gabapentin, Bactrim for PCP prophylaxis, Zantac, Zofran Immunizations: UTD Allergies: NKDA FHx: paternal great-grandfather with skin cancer

5 Our patient (remember she s 2 ½) Vitals: T 98.1 P125 R24 BP 98/69 Gen: awake, alert, fussy but consolable Head: alopecia W11.7kg HEENT: conjunctivae clear but eyes appear sunken; OP clear but lips dry and mucous membranes tacky; unable to visualize TMs bilaterally due to cerumen CV/Resp: RRR, no murmur, cap refill <2sec, CTA B Abd/GU: BS x4, soft and nontender without guarding or rebound, no HSM, + diaper rash LAD: normal Skin: no rashes Neuro: grossly intact

6 What are you considering? Remember, she is immunocompromised! Medications Thyrotoxicosis

7 What do you want to do? (and what we did!) CBC CMP, Mg, Phos Stool Studies WBC Ova/Parasite Stool Culture C diff Rota/Adeno

8 Rehydration What % dehydrated is she?** Wt 11.7 kg She needs 700cc replaced Maintenance = 45cc/hr Bolus x1 700cc-220cc =480 cc to replace 240 over 8 hours = 30cc/hr + 45 cc/hr main 75cc/hr 240 over 16 hours = 15cc/hr + 45cc/hr main 60cc/hr

9 Results S/1B/49L/12M/1E/9ATL ANC 470 FOBT + Stool WBCs negative Ova/Parasite negative Adenovirus negative Rotavirus negative C. difficile toxin positive Ca 7.9 Mg 2.2 Phos /2.4/0.3/48/118/82

10 Clostridium difficile Anaerobic, spore-forming bacillus (spores are resistant to everything!) 30-70% of infants are asymptomatic carriers. Why? Disturbance of normal colonic flora allows C diff to grow spores produce toxins (A and B), causing mucosal damage Predisposing factors: Hospitalization, prolonged antibiotics, abdominal surgery, IBD, repeated enemas, immune deficiency Asymptomatic carriage mild diarrhea severe colitis toxic megacolon** Fever; crampy abdominal pain; foul-smelling, watery, often green stools** Blood or mucous present in those with colitis**

11 Clostridium difficile Stool studies: Toxin A and B by enzyme immunoassay** Toxin A- enterotoxin Toxin B- cytotoxin Studies show Toxin B is most important, those with strains lacking toxin A have disease similar to those with both toxins CBC: leukocytosis, possibly anemia Endoscopy:** Classic pseudomembrane is a white or yellow plaque along hyperemic and inflamed colonic mucosa Mucosa may be friable and erythematous without pseudomembrane

12 Clostridium difficile Contact precautions** Alcohol-based products do not kill Soap and water best for hand hygiene, bleach for cleaning surfaces Discontinue offending ABX if possible! (duh!) Flagyl 20mg/kg/day divided QID for days (max of 1g/day)** Relapse** 10-20% relapse in 4 weeks of stopping therapy due to: re-infection, persistent spores, chronic ABX, pre-disposing underlying disease First relapse: repeat Flagyl Second relapse: vancomycin 40mg/kg/day PO divided QID or days Chronic relapsing- PO vancomycin for 1-3 months + one off the following: cholestyramine, lactobacillus, GOLYTELY bowel irrigation, IVIG, saccharomyces boulardii

13 But wait Our patient developed abdominal pain with grossly bloody stools Hypotension Fever Now what are you concerned about, and what do you want to do?

14 New Results Blood Culture Gram Negative 0.5days Klebsiella pneumoniae Urine Culture No growth Abdominal CT Extensive submucosal and subserosal colonic pneumatosis (cecum, ascending colon, sigmoid, rectum) Extensive air expanding the submucosal space present in sigmoid and rectum wall with free air dissecting into retroperitoneal soft tissues in pelvis No free intra-abdominal air Residual colon with wall edema with mucosal hyperenhancement indicative of colitis

15 Neutropenic Enterocolitis Life-threatening, necrotizing enterocolitis Most common in those with hematologic malignancies who are neutropenic and have breakdown of gut mucosa secondary to cytotoxic chemo Typhlitis NE of ileocecal region Pathophysiology involves combination of mucosal injury by drugs, profound neutropenia, impaired host defense to invasion by microorganisms Microbial infection leads to necrosis of various layers of bowel wall Involved parts: Cecum most commonly involved Extends into ascending colon and terminal ileum

16 Typhlitis Micro seen: Pseudomonas aeruginosa Escherichia coli Klebsiella spp Viridans group strep Enterococci Bacteroides spp Clostridium spp Candida spp Risk factors: Initially seen in those receiving induction chemo for ALL Increased associated now in those with mucositis, HSCT, and chemo within past 2 weeks Frequency is increasing with use of cytotoxic drugs: Taxanes Cytarabine Idarubicin Vinorelbine 5-FU Cyclophosphamide Ifosfamide Cisplatin Carboplatin

17 Typhlitis Must consider in ANY severely neutropenic patient (ANC <500) who presents with fever and abdominal pain Often RLQ Rule out appendicitis!!, GVHD, colonic pseudoobstruction Fever typically presents during 3 rd week after chemo Distension, cramping, tenderness, NV, watery or bloody diarrhea, frank hematochezia Peritoneal signs and shock suggest perforation CT is preferred imaging (attempt po and iv contrast) Bowel wall thickening (100%) Mesenteric stranding, bowel dilatation, mucosal enhancement, pneumoatosis C diff toxin if not already done!

18 Typhlitis Bowel rest, NG suction, IVFs, Blood product support Antibiotics Active against: pseudomonas, E coli, other GNR, anaerobes Regimens 1. Piperacillin/tazobactam (Zosyn) 2. Cefepime or Ceftazidime + Flagyl 3. Imipenem or Meropenem (if allergic or know bug is resistant) Cover for C diff if it has not been excluded Antifungal? If fever present >72 hours after antibiotics started If no signs of severe disease, continue for 14 days following recovery from PO if no bacteremia and doing clinically well AF x 2 days and counts >500 (Augmentin or Cipro + Flagyl) Surgery?

19 Typhlitis May remain febrile until recovery from neutropenia, despite antibiotics G-CSF? Delay further chemo until full recovery Early recognition and progress in management have reduced mortality Initially 50% mortality No major studies demonstrating this Prone to develop again during subsequent treatments Finishing our patient

20 Noon conference: Dr Creel. Delivering Bad News, Room 3302 ACC Have a great day!